Miss Mira

Neurology Appointment

2011-05-08T21:16:00.002-05:00

Last week, we had a long standing neurology appointment, and it turned out to be probably one of the shortest appointments I think we have had to date. This is the second time we have seen this particular neurologist, and Sarah and I are pretty comfortable with him. He is direct, which is great, but more importantly, he recognizes that our family had been on this journey for almost 6 years and he is merely acting as a guide, acting in our best interest, on how to continue the journey.

We discussed medications first and foremost. With all of the changes coming up (mainly Mira's transition into her new summer schedule with the school district and our potentially slow and gradual departure from IDC) that we have no intention of rocking the boat with medication changes on top of everything else. Mira has been on a combination of Lyrica and Clorazepate for some time now and we think it is the best balance that we can achieve for her. She is still having daily seizures (all types - myoclonics, tonic-clonics, head drops, absence, etc.) but nothing new or out of the ordinary. Lyrica helps with the seizures while the clorazepate helps with the irritability. We briefly discussed clobazam, which is only one of two medications that Mira hasn't tried, but I think we will only look to alternatives if things are getting worse.

Genetics is always woven into the dialogue at every neurology appointment and this past meeting was no exception. We ran down the list of isolated gene testing and her neuro keeps coming back to Dravet's Syndrome (SMEI - Severe Myoclonic Epilepsy in Infancy) and the genetic anomalies associated with it, focusing on the SCN1A gene, which Mira has already tested negative for, years ago. There are now additional genes, including SCN1A and SCN1B that are being tied to kids with Dravet-like symptoms - the genetic connection(s) keep expanding. Based on Mira's history, I'm not sure I necessarily agree that Dravet's is accurate in terms of a diagnosis, but anything is possible I suppose. The spectrum with any diagnosis is so varied and unpredictable that it is pointless to theorize any more about which gene causes what. Based on what I have read, I think Dravet's is less likely in Mira's case - again, just based on history and symptoms of the disorder.

Regardless, we are taking the approach that it is fruitless to test for isolated genes (Mira has done 5-10 different genes to date) particularly when the testing at this stage, would be very, very low. Thus, we are going to wait until Children's Mercy advanced genetic testing becomes available, which will be in another 6-9 months. I don't recall the name of the specific test, but I do know that it will test for over 300+ genes, instead of single isolated genes. The technology keeps advancing exponentially it seems.

We also decided during our appointment that we should reduce our neurology visits to just once a year now. Mira has been pretty steady in terms of her seizure activity over the past 2 years and there really is no compelling reason why we would need to continue our 6 month visits. All of her medication tweaks over the past few years have been either over the phone or through email.

Unless of course, we enjoy paying our new copay at CMH, which TRIPLED since our last visit. Ah, insurance...........sometimes it is pointless.

Ramp

2011-04-17T21:06:00.037-05:00

As an architect, I never thought I would have the ADA hit so close to home, but nonetheless, here we are. We are having to make modifications to the house, most of which are efforts just to get her chair around the house smoothly. We had used ADA guidel ines as a reference, incorporating some of these concepts into the addition (i.e. 3' doors, wider clearances in the kitchen, etc.) but the existing parts of the house prove to be a little more complicated. Modifying a 1953 ranch to meet any sort of ADA compliance is not an easy task. On the interior, there are 2' wide doors and if you can find a 3' turning radius anywhere but the center of the living room, you're ecstatic. The exterior is equally as challenging.

We have been getting accustomed to Mira's new chair over the past few months and one of the challenges we noticed right from the start was just getting the chair in and out of the house. The KidCart (her former system) was easy, since we could simply detach the chair from the base and either carry it out or practically bounce it down the two steps on our front stoop, since it was so light. A majority of the time, the base stayed in the van, so it was really a non-issue. The Zippie (her new chair) weighs considerably more and has anti-tip brackets on the back, which makes it more difficult to get down even a couple stairs.

Back in January, we had a Band-Aid solution, which was to buy a folding ramp until we could modify the front walkway of the house permanently. The folding ramp was suffice for a few months, but running down the 5' incline with the Zippie ended up being a 30 degree dive downward that you learned to prep for with a broad stance and a firm grip on the storm door when stepping onto the stoop. Unfortunately, we could only fit a 5' run from the stoop before we ended up in the bushes, based on the curved walkway.

The challenge with a permanent ramp was to not take on yet another costly and lengthy home improvement exercise - we have met our quota for the next 10 years. So we decided the easiest and most cost effective solution was to simply pour a concrete slope directly over our existing walk. There was some minor demolition, in order to get good concrete coverage and to fan out the end of the path in order to get around the van when parked. We also used geofoam (lightweight foam) to reduce the weight of the concrete at the deeper sections toward the house. In addition to the ramp itself, we also had a small 3' x 20' pad poured on the opposite side of the driveway to get around the van more easily.

It was the obvious and least-invasive solution - the whole project went off without a hitch and was finished quickly. The ramp is only slightly steeper than a typical ADA slope, but it works beautifully for us. We will have some landscape work to do in the future, but ironically, you can barely notice the ramp from the street, as it is right now.

I wanted to extend my eternal thanks to our neighbor Jim Rothberg, who sacrificed his time, materials, and expertise in getting this done for us. We are tremendously grateful for all of your efforts and hard work. Sarah and I cannot thank you enough.

Baby Day Photography

2011-03-05T08:45:00.008-06:00

Mira's former therapist and friend Shannon Lockwood at IDC came in to our house this past week to document our family, as a sort of 'day in the life' through candid pictures. They turned out to be nothing short of amazing. As somebody who really appreciates a good photo eye, I think the images she took are incredible. Our pictures are on the March 4th, 2011 blog post here on her website. With so many images to choose from, we have our work cut out for us in which ones we want to have framed. We cannot thank her enough for taking such great shots.

Shannon also compiled a video of all the images in this video:

Update, Pictures, + Videos

2011-01-31T23:12:00.020-06:00

For weeks and months now, I have been telling myself (for some odd reason it seems every Sunday evening about 11:30pm when the weekend is finally winding down and Sarah and I are gearing up for the week ahead, that I ‘might possibly be on the verge of planning to maybe, perhaps, certainly, positively update Mira’s blog this week, at some point’ which by mid-week turns to ‘this is it – I am GOING to post something this week’ which ultimately leads to yet another Sunday. So, where were we………..Mira’s 5^th birthday…………………

Well, first, I have to have rant and I will apologize in advance for immediately hijacking the first 8 or 9 paragraphs about Mira’s chair. As I rewind and look at Mira’s last post, I had to chuckle when I read about where we were with her chair back in June. Alas, the eleven month saga has finally come to closure, only a few weeks ago. I will try to provide (don’t hold me to this) only the Cliff Notes version of the endless phone calls, emails, and absolute fuming on our end that ensued throughout the entire process of obtaining a single piece of durable medical equipment.

Having navigated only the surface of the icy waters of health care, insurance, seating clinics, clearinghouses, DME caps, manufacturers, middle-men, markups, documentation, premiums, processing and mainly, excuses, over the past year, it has become painfully clear why health care in this country is broken or at least, the lug nuts are loose and it’s about to lose a wheel. It is a host of factors, yet in this case, it is a story of broken communication, internally and externally, of just one segment of one simple, single transaction – we just wanted to order a chair for our daughter. The process was absurd. There are too many people involved for one transaction, too many channels to navigate, too many documents, too many decisions (and non-decisions alike) too much waiting, and too much money involved. So many people are trying to get paid for one single transaction, which ultimately means, making the entire process TOO CONVOLUTED.

When we first started this, in January of 2010, we felt optimistic and empowered, thinking we were on top of it all – it’s early in the year, we were getting Mira fitted and sized, and on track to have her in a new system by spring. It’s January. She gets measured, the order is rolling. Or is it……..we don’t know. Behind the scenes, the paperwork sits, and gets shuffled, and pushed to someone else, a second party, who sits on it, and reshuffles it, then sends it back, in the wrong direction. Then the company resends paperwork to secondary internal group, again, who finally correctly routes it to insurance. When did insurance actually see this paperwork? Not sure. Is this the first time they have actually seen it? Not sure. Internal finger pointing ensues and a calming assurance is issued from the company that they will resolve this communication problem. They will fix this in the future. Insurance company now needs time to think about the claim, now that they have it. Perhaps more documentation might be needed to help render a decision. One month goes by, two months, three months. No answers. No direction. Severed communication string continues to unravel. Secondary clearinghouse company (funded by the seating company) gets the paperwork back from insurance – the request is to resend it because they need more documentation in regards to Mira’s condition. They can’t make a decision based on what they currently have.

Fast forward to summer 2010, we were no further along than we were in January. By this time, we begin to question whether or not the system we ordered would even work for her by the time we actually get it. At Mira’s current growth rate, she is poised to outgrow the chair in a year. One thing was for certain – we do NOT want to go through this entire process again in another twelve months. We just spent five months getting nowhere. In August, after finally getting in contact with her seating company, we came to the conclusion that the system wouldn’t work for her for the long term. At the same time, it was leaking out that (after nearly 7 months later, endless documentation, and overwhelming frustration in trying to communicate with the seating company) that the system we were ordering was possibly going to be denied by insurance. At this point, we had to make another critical decision. Do we continue this process with this seating company or do we start over completely somewhere else? Let’s give them one more chance to make amends. Bad decision.

Again, we leaned on our insurance to make a decision. We couldn’t place a separate order for a different system until the initial claim was processed and cleared, regardless of where we went - we just needed documentation from them to move forward. In early September, they denied the claim over the phone, as we expected. Twenty minutes later, they called me back and said it had been ‘overturned’ and that they would now approve the claim. It took you two months to make a decision and twenty minutes to overturn it? Really? Ironically, it didn’t matter, since we were ordering a different chair. At that point, we went back to the seating company (the same one, complete with atrocious customer service – we failed to learn our lesson at this point) to resize Mira for a new system and headed BACK down this path, only this time, we requested a case manager through insurance in an effort to expedite the process.

In late September, Mira was measured and sized for the new system and the process starts over ... this time with a proactive insurance case manager on-board. Then we wait, again. One week, two weeks, three weeks, and the cycle of crappy, non-communicative customer service escalates, all over again. October goes by. Finally, just before Halloween, insurance approves Mira’s new chair. We call our rep at the seating company and relay our exciting news. They assure us they will act expeditiously. And again, we wait. It’s mid-November. We can’t stand it anymore and it’s almost Thanksgiving. We remember the quote from the sales rep weeks ago, something along the line of ‘we will have Mira in her new chair by Thanksgiving’ and I laugh sadistically.

We pick up the phone. Good morning, seating company: WHAT IN THE F%*K IS THE STATUS ON MIRA’S CHAIR?!?! (in a more rational tone than I am portraying, but nevertheless thinking). Response: Oh, they tell us, we have been waiting for you to give us a verbal commitment that you will pay the out of pocket costs before we will order her chair and NO her chair isn’t ordered. Cue the sound of screeching tires ... WHAT??!?!?!!!

I cannot believe I am hearing this. I ask for them to send the itemized order for her chair to me via email, so I can review it. I hang up the phone. Ten minutes later I receive the quote via email. Eyes squinting at the screen, I read the cost of the chair and steam begins to whistle in Popeye-esque fashion from both ear canals. Well, this is interesting, Horribly-Communicating-Seating-Company. It says here, based on what you are now deciding to charge us for the chair, after our applied annual DME cap, my out-of-pocket costs are going to be $4,200. That’s almost double what you quoted us TWO MONTHS AGO WHEN YOU WERE SUPPOSED TO ORDER HER CHAIR. At this point, with gnashing teeth, I call the seating company back and said to NOT order the chair, because not only is the quote considerably higher than what you told us before, but yet again, you failed to do your job properly. Let’s remember that you are actually in customer service. I have no idea how you survive as a business, but somehow you do. We are officially done with you. I could be cliché and say ‘strike three’ but the reality is I lost count after 7 or 8 strikes.

Two days before Thanksgiving, we took Mira’s chair order in hand, went to a DIFFERENT seating company, placed the order, had it re-approved by our case manager through insurance (who is a rock-star in this process), and took possession of her chair, for $2,000 less than what we were quoted by the first company, all by the end of the year. We accomplished more with this company in 6 weeks than we did in 10 months with the first company.

One month later after all of this dust has settled, I am slowly calming my frustration. I have never, in my entire life, in dealing with any transaction, with any company, firm, office, or individual, ever dealt with such poor customer service. Ironically, they went ahead and ordered the chair (without our consent – without a phone call, email, or ANY sort of gesture to try and contact us, yet again) in early December, and now they are stuck with it. In the end, poor communication and karma bit them in the ass and they get to deal with a $6,700 chair that we don’t need, and personally, I could not care any less. I am done with my rant and am now moving on.

The chair itself is awesome and everything that we need for Mira. As she gets bigger, the chair will accommodate. We are currently making some modifications to the house, getting bids on pouring a ramp up to the front stoop. Right now, we are having to lift her chair in and out of the back of the van, but we do have a folding ramp. Ultimately, we will look into getting a converted van, but that is another adventure, hopefully in 2012.

In other news…………….Mira’s neurologist left last summer and we had our first appointment with her new neuro, which went very well. He is approachable, direct, and has an open mind. We went through a lengthy review of all Mira’s history and medications, ultimately focusing on two things: 1. seizure control and 2. minimizing/weaning anything out of her routine that may not be helping. Clearly, Lyrica works for Mira – we have no intentions of weaning her off of pregabalin. We have ventured down this road, with poor results. What all of us did question, however, was clorazepate. This is an older benzodiazepine, with very sedating properties, that may or may not be doing anything for her. We originally used it for her irritability and anxiety, but now questioned whether or not she should 1. be at this dosage and 2. whether it was doing anything for seizure control. Collectively, we agreed to try weaning her off it.

It is all trial and error and we found out the hard way that she needs clorazepate. The weaning process took several weeks and by the time she was completely off of it, Mira was a mess. Her irritability gradually increased to the point where she was crying inconsolably for most of the day. Then she started getting up again at night, screaming, crying, and was not a happy girl. She was extremely irritable to the point that NOTHING would help. We have tactics, we tried them all and now, we are done with this – put her back on it, right now. The answer to this particular question would be YES, Mira needs to be on clorazepate. Within 3 or 4 days, Mira was back to her old self without the constant irritability, fussing, and crying. There was no finger-pointing, questioning, or anything. Just put her back on it and let’s move on.

With her new neurologist, we also discussed genetics. His thought was that there is no need to go on a fishing expedition and continue to test for isolated genes, based on the literature that is out there now. Genetic deletions, mutations, rearrangements, and mosaicisms are being discovered every other day – some relevant; most are not. All of these genes: MECP2, CDKL5, STXBP1, FOXG1, SCN1A, and the list keeps growing that all link back to intractable epilepsy – Mira has been tested for most, but not all of them. Her neurologist basically said that until a whole genome sequencing effort was commercially available and/or affordable, there was no valid reason for entertaining additional testing for specific genes. We are fine with this. There really is no compelling reason other than to point to a particular gene (ABC123) and say ‘there it is’ – congratulations, you found it. It obviously doesn’t help Mira’s day-to-day quality of life or will help her per se, but it would certainly make it easier to explain.

Completely switching gears, Mira has a new name as of late – Shark Teeth. Mira lost her first two teeth this past week. One of them we have and the other is lost. As her lower permanent teeth were pushing up, her new ones hadn’t worked themselves out, thus the two rows of teeth for a few weeks. So Shark Teeth gets filed in our mental Rolodex of names for Mira’s nuances, mannerisms, toys and the like: there’s the dragon, the Bunz, happy claps, Meerz, and now Shark Teeth.

Mira continues to grow. At her last appointment in September, she was weighing in at 60 pounds and is still in the 90^th percentile for height. As she increases in size, our diaper options decrease. We officially had to give up the Stage 7 diaper route (available only online) and move to an adult-type diaper. I was flabbergasted at the size of these things. We ordered a sample of the extra-smalls, based on her waist and they were a little snug still, so we quickly moved up to the small size. The word small is a misnomer – there is nothing small about them, in fact, they remind me of a trash bag, only with a light green hue. The flaps are huge, the tabs are unforgiving and they are expensive. Yet, they work, so no complaints. We are using up the last of the Stage 7 order and transitioning into them successfully, but not without some initial jaw-dropping.

The house is all but done for now – some minor trim work and some painting is all that remains. When the weather warms up, we can finish painting the interior windows an actually start on some landscaping in the spring.

I am posting a bunch of random images from June through this past weekend, which is how our lives seem to function these days – a potpourri of kids, family, housework, occasional travels (Sarah surprised me for my 40^th in September and we took and extended weekend in Chicago) and toy repairs. Mira broke her fifth Tomy toy, but we were able to salvage parts from old ones and rebuild it. We also found another backup on Ebay, so I think we are in the clear for a while.

I also posted a bunch of random videos on Mira’s YouTube site here. They aren’t all that exciting, but it gives you a good idea of Mira’s mobility these days. Hope everyone is doing well and thanks for all of your thoughts, prayers, help, and generosity over the past year!

Mira Turns Five!

2010-06-29T22:12:00.023-05:00

Well, where to begin. First the big news. Mira turned 5 yesterday! I cannot believe she is already celebrating her 5th birthday, when I swear it was yesterday she just turned 4. We celebrated her birthday at Avelluto’s for dinner, went swimming, and had pink frosted cupcakes for dessert. Mira enjoyed a couple of scoops of pink frosting after we opened presents, then the sugar rush ensued. I think she would have eaten the entire tub of frosting had we let her – it was a big hit. Thanks to everyone for all of the cards and gifts!

Things have finally settled down over the past few months, after having some medication and irritability struggles with Mira in March and April. Over the course of a few months, Mira’s demeanor had become progressively worse – more seizures, more crying, more fussiness, more twitching, and just some all around rough times with her around the beginning of May. She had a couple of longer, intense seizures during this stretch as well, which we haven’t seen in a while. It basically came down to her needing an increase in her Lyrica dosage, which we ultimately did and things have gotten back on track. It’s frankly so difficult to tell what is happening with her and ultimately, it is a guessing game most of the time. We have attributed this latest bout to Lyrica. Something in that medication works for her and we need to just make sure we are at a therapeutic dosage for her all the time. She is now holding on a steady dosage, along with clorzaepate.

We did get some unsettling news about a month ago that Mira’s primary neurologist is leaving Children’s Mercy. After 3+ years as her only neurologist, we are very saddened to see him go. We were able to schedule a final appointment with him next week before he departs to the east coast for good, then we begin a new search for someone else in the clinic. Over the years, Mira has seen three other neuros in clinic (in KC and Saint Louis) and I have corresponded with countless other neurologists, doctors, nutritionists, geneticists, parents, theorists, and quacks alike in our search for help and guidance. He has always been on target with Mira and very understanding. We will miss him.

Since our last post, ironically, we are no further along in obtaining Mira’s new mobility system. The combination of endless insurance red tape and ongoing seating clinic miscommunication equals frustration for us. While Mira continues to kick apart (literally) the connections on her KidCart, we continue to prod the seating clinic and insurance company to ensure the process is moving forward. It is painful at times, but we are frankly at the mercy of those who are pushing (albiet very, very slowly) this funding paper trail around. We did start this back in early January…….and now it’s almost July.

In terms of therapy, Mira is doing excellent. She is still getting weekly OT, PT, swimming, feeding, and we are still doing hippotherapy (weather permitting) nearly every week. Mira has made great strides over the past six months in weight bearing on her legs. During some of her therapy sessions, her therapist(s) have been getting her in a gait trainer (which Mira loves) and having her get used to the idea of putting pressure through her legs while on a very slow-moving treadmill. She is also starting to not only grasp her bottles, but able to hold it in place during drinking! Most of the time, she is grabbing at the bottle and will hold the bottle (predominantly with her right hand) and we have been letting her drink it unassisted! She is doing so well.

We invested in a pool pass this year and are getting her in the pool as much as we can, since it is always a hit with her. She LOVES being in the pool – lots of smiles, lots of splashing, and it wears her out. Jonah on the other hand HATES the water – bath time is terrible and he despises the pool. He is slowly coming around thanks to Mira’s ecstatic nature in the pool. Eli is taking lessons again this year and is increasingly confident in the water. He has made great strides over the past year and is really enjoying the water slides at all the pools. He now is eyeing the diving boards.

We have been getting some help a few days a week for the summer from a graduate student who works at Mira’s school. She is fantastic with Mira, very enthusiastic, and we are extremely thankful to have the help. We wish we could pay her more!

We have taken a few weekend trips already this summer, to Saint Louis and to Omaha, and are tentatively planning a few more trips toward the end of the summer, but right now, it is good to just take a break. With all of the major renovation and addition work on the house out of the way, we are just in painting and trim work mode. We have a roll-off scheduled for delivery (our third one for this project) on Friday, mainly to clean out and throw away all of the leftover construction material and yard debris. We lost yet ANOTHER tree this year, after a 50 foot limb broke off and came crashing down in our front yard, destroying part of the park fence, gouging the yard, and taking out a couple of shrubs. Fortunately, it didn’t fall on the house, but cost us a small fortune to have it removed.

On a high note, Sarah and I celebrated our 10 year wedding anniversary this past month. We were able to have a small getaway weekend to celebrate and had dinner at the best restaurant in the world (well, at least in Kansas City and it is our favorite) called Le Fou Frog. In the 12+ years we have been going there, they have never disappointed and the mussels and lobster tails WILL make you drool, guaranteed. Thanks Auntie Aura for watching the kids – we are forever in your debt.

Speaking of Sarah – she is becoming quite the photographer. She took most of these pictures. The close-ups of Mira with her new dress are stunning at high-res. Kudos to my wonderful wife, who is really taking some great shots. The video at the end isn’t very exciting (I did it), but gives you an idea of what Mira does most of the time in her chair. Notice the double-banding of her toy, so there is NO WAY for her to pull it off her tray and shove it on the floor. Now she pulls the entire tray with it, so it all comes crashing down togehter on the hardwoods, making the most awful noise in the world. Needless to say, Mira LOVES that toy - thanks to Erin for donating Fletcher’s old one – that was very thoughtful and we appreciate it very much!

We are looking forward to a quieter summer than last year and now that we have more room, please visit!

Mira's Toy

2010-03-29T13:56:00.006-05:00

Mira broke her third (and last) TOMY piano toy. We have been trying to find one on eBay and just missed the bidding for one yesterday. We are trying to find another one ANYWHERE. If anyone knows where we can get one, please let us know. It is a TOMY brand, sometimes called a globe toy, bulb toy, piano toy, or play by light toy. It was made in 1999. It is one of the few toys that Mira consistently interacts with. They are really hard to find now but if any parent is willing to part with one, we will buy it!

Finally.......An Update!

2010-01-24T22:23:00.013-06:00

It’s official. I have personally adopted September as ‘Merry-Go-Round Month’. I think it all started a few years ago, when Eli was in full Preschool mode and Mira was in full-therapy mode. And Jonah makes three (although technically not in school – three just adds to the daily chaos).

The first week in September starts off fun and exciting, with the start of school of course - new beginnings, new friends, and school pictures, much like the euphoria you feel of first getting on a merry-go-round. Lights flashing, music blaring, the ride slowly starts. The second week in September is much like the first, quickly accelerating – twice as fast as the first. Cub Scout meetings. Popcorn sales. IEP meetings. Spinning. Ups and downs. Up and downs. Toward the middle of the ride, you realize the cuddly blue dolphin you chose to sit on is loose from its pole on the ride. It squeaks a little (much like a dolphin would squeak in real life I suppose) but this squeak sounds desperate and maniacal. Things are speeding up and you realize upon further inspection that the dolphin is missing a flipper and in its place, painted over duct tape. The ride spins faster and faster. You are too distracted to actually enjoy the ride, focusing on the funnel cake stands spinning wildly around you. You find a focal point – perhaps the floor. The mirrored ceiling, the child barfing next to you and the parent trying to get the operator’s attention in a desperate attempt to bring the ride to a screeching halt. The dolphin keeps squeaking. Now in full motion, the pinnacle of the ride hits you like a lead balloon. You are nauseous, breathing heavy, over stimulated by noise, red lights, green lights, laughing clowns and scary unbalanced MIDI music. The ride starts to decelerate and it’s not soon enough. You start mapping your exit strategy during this winding down phase, gradually distancing yourself from the squeaking dolphin, which has caused your ears to seemingly bleed. Thank God, it’s the end of the ride and the end of September. The winding down of pancake breakfasts, wood floor deliveries, fundraisers, Boy Scout campouts, drywall contractors, and medication trials is officially here. It’s October. Finally.

Then, procrastination sets in. (I started this post in October. It’s now January 2010.) So, so bad. I am still spinning from September.

So where to begin. In early September, Mira had a routine neurology visit, which went as expected, I suppose. Every time Sarah and I go in with a certain degree of expectation for one of Mira’s neuro appointments, we walk out an hour later (or 3 hours later in some cases) wondering how we were spun 90 degrees from the mindset with which we had expected to leave. Sarah and I call it the ‘90 degree effect’. One would think we would have learned by now. The main topic of discussion this visit was Mira’s irritability. The past few months have been rough with her. We tried Banzel briefly, which seemed to help some initially, but quickly turned sour after a week or so. She was really cranky and having lots of seizures. We gave it a few weeks, but it seemed to noticeably cause more harm than good after a week. Strike one.

One of her neuro’s other suggestions to combat the irritability factor was to try Risperdal (risperidone) which is an antipsychotic sometimes used to treat schizophrenia. Knowing it would do nothing to stop her seizures; we tried it as a potential relief from her seemingly constant uncomfortable disposition. After a few weeks of that, we abandoned it. We were told it either works or it doesn’t for her. It didn’t. Strike two. Then we tried Carbatrol (carbamazepine), yet another antiepileptic. Still very cranky and no change in seizure activity. Strike three to finish out 2009. Our batting average with meds this year is about .125, and that’s being generous.

In mid-December, we tried Clorazepate, (in the benzodiazepine family of drugs, i.e. sedating similarly to valium) which she is currently taking. We are trying it mainly to aid in resolving her irritability and if it were to decrease her seizures, all the better. We started at a lower dosage and saw some modest improvement, then increased her dosage several weeks later, which is where she stands now. She is still having seizures, but they are far less frequent and her irritability has decreased considerably since increasing her dosage. All in all, we feel this is the best we could hope for right now, so we aren’t rocking the boat. We’re trying to find the elusive sweet spot in between overly medicated/non-functioning and extremely irritable/non-functioning; finding that balance is difficult.

Nothing else significant surfaced in her appointment in September. We talked genetics and testing for a bit, but there are several fundamental issues that come up with this discussion every time. First, genetics is moving at such an incredible pace that it is difficult to keep up with the constant gene deletion and mutation discoveries that are coming to light. Literally, hundreds of genetic anomalies are being discovered every month. Second, there are so many genetic markers associated with particular developmental and neurological issues; you could not possibly test for every one relevant to Mira’s situation. CMH is in the process of implementing some new genetic technology screens (one in particular called a ‘Whole Genome Exon Sequencing’ screen) will be available some time this year and Mira will be one of the first kids tested at the hospital. It is supposed to scan for an exponentially higher number of mutations and deletions than even 2 years ago.

The first week in October brought us a stomach bug too. Mira caught it first, threw up in her bed numerous times, and quickly passed it on to Jonah. They passed it on to me, where I went home one day after hugging the bathroom sink at work a few times. Eventually it passed. Otherwise we’ve just been weathering winter’s standard onslaught of various cold virus’ and mild fevers, with the most recent round of congestion, phlegm and breathing treatments (for Jonah) ending two weeks ago with everyone thankfully turning a corner. We’re hunkering down and hoping to avoid any more major illnesses. Fingers crossed!

November and December simply flew by. We spent Thanksgiving with the Flora’s, who were just getting settled in after their recent move to Omaha. I think we will be being seeing more of each other from now on, since we are only 3 hours away and not 6 – it is fantastic to be that much closer! Mira loved hanging out at their new house, since they have acres of room (all carpeted so she Mira can roll around) downstairs in their entertainment room.

December was a big month for Jonah, who officially started walking around Christmas. He had been scooting around, always pushing with his right arm, for months. I don’t think he was interested in walking for a long time for two reasons. First, he was faster and more efficient at just scooting – lightning fast and second, he realized that if he just waits or screams it out long enough, someone will pick him up and just carry him ALL THE TIME! He had perfected the art of getting people to pick him up and still takes full advantage of it when he is tired. A month later, he is cruising around the house and quickly getting into everything.

Christmas was very low key this year, perhaps due to the fact that we had 18 inches of snow dumped on us here in Kansas City and constant single digit temperatures for nearly three weeks. With Christmas break and the weather, I think Eli and Mira were home from school and therapy for a total of 2 days during that time. Lots of school cancellations and trying to find things to do indoors. In early January, the temperatures came back up we all got back into the full swing of 2010.

Mira’s diet has shifted to pretty much all liquid all the time, with the exception of feeding therapy and the occasional applesauce, where her therapist can usually get her to eat some solids. Otherwise, she is pretty content and her diet of combination of warm soy and rice milk, mixed with protein powder and supplements, topped off with a Pediasure (sometimes two) at some point during the day. We have found that since abandoning cow’s milk all together, her skin has cleared up dramatically. We aren’t sure whether or not she has an allergic reaction with dairy or cow’s milk in particular, but she doesn’t seem to miss it and her skin is definitely better. She’s also taking Zyrtec everyday too, so that may be helping her eczema as well.

As always, every prepared drink has to be the right temperature or she will reject it. Mira has now perfected the art of taste-testing each bottle when you put it to her lips. She chews on the end for a few seconds, to get a little but in her mouth, and then decides whether or not it meets her very specific temperature requirements. Warm, but not hot. Never cold or she will spit it out with vigor and push the bottle right out of her face. She knows what she likes.

Sleep has been much better, although far from perfect. Mira had some weeks back in November and December where we were using trazodone practically every night, but she has settled back into her routine of having one rough night every few weeks. We aren’t exactly sure how much she is awake in the middle of the night; some nights we can hear making noise for hours and other nights not so much as a peep. We gave up on using melatonin for good, finding that the trazodone is much more effective for her. The last few weeks Mira has been getting up consistently about midnight or 1:00am, taking a bottle and slowly going back to sleep, after an hour of bouncing around in her bed.

This past week we started the wheels in motion (no pun intended) to get Mira a new chair system. Her KidCart has worked great for the past two years, but unfortunately, she is out growing it. The system she has now works well, since it is so flexible. It is a three-piece system: a detachable chair that sets in both a high-low base to use around the house, as well as a separate stroller base to everywhere else. Happily, her school has a spare high-low base for her to use during her preschool time, enabling her to be face to face with her peers. We are actively searching for a similar system, but they are very expensive. The process to get her KidCart several years ago took about 10 months from start to finish. We may be looking at a similar timeframe to get her a new chair, thus we are starting early. One of the systems we are looking at is the Panda Futura (http://www.snugseat.com).

Further down the road (yet another bad pun) we are looking at conversion options for the van. One approach would be to convert our existing Odyssey, which is already 3 years old. The other option would be buying a new van that is already been converted. Sarah started doing all the research on Mira’s new chair and the van options this past week, so we are still becoming educated, exploring options and costs.

We are officially moved into the addition, despite being only 80% complete. There is a laundry list of ongoing construction tasks that still need to be completed, but we are trying to prioritize what happens next. Anyone who wants to help me do some demolition work, I will take you up on the offer in about 2 seconds. We will be getting a roll-off in about 2 or 3 weeks to start ‘Phase 2’, the existing part of the house. The plan is to demo our old kitchen and turn it into a laundry room, then demo the existing bathroom, making it more accessible and bringing the 1970’s finishes up to date. Finally, the existing hardwoods will be refinished to match the new addition. We are still aiming to be done with the entire project by June 1st, which will be one full year from when we started. It’s been a long haul, but will all be worth it once we finish.

Thanks to everyone for all of the wonderful Christmas gifts, thoughts, and prayers for our family. Hope to see more of everyone in 2010!

The Long, Long Summer

2009-08-29T22:02:00.018-05:00

Such a long, long month and an end to the seemingly longest summer in history. I’m not sure what it is about the end of summer, but it always seems like a whirlwind of pool parties, doctor appointments, and open house nights that consume us all until we reach that glorious finish line around the middle of August. Yes, God bless the starting of school. Eli finished his first full week of first grade and Mira started preschool at IDC this week. I don’t think it could be any sooner to get back into some resemblance of a routine to keep our sanity these days.

So, starting from where I left off in the middle of July…….. Mira’s MRI results came back a few weeks ago and it was completely normal. No indication of anything abnormal or any sort of hint as to why she is still having daily seizures. It’s not as though we were expecting anything out of it – it was more of an update to see if anything has changed since her last MRI, which was three years ago.

Mira’s eating habits over the past few months have really gone downhill. She refuses to eat anything by spoon now. This is no longer a hunger strike – it is a flat out refusal to eat and she has perfected it. Mira has now learned to clench her teeth together and avoid getting ANY food in her. Up until this past week (which things have improved slightly) I would say that Sarah and I have successfully gotten her to eat about 2 jars of food total over the past month. She gets plenty of fluid, in fact that all she gets. She is on target for height and weight so we have little concern that she is getting the calories that she needs. We continue to supplement with protein powders mixed with milk, Pediasure, and watered down juice, which she never seems to get tired of.

Mira’s irritability was hitting an all-time high a few weeks ago. As I’m sure you have read in other posts, the number one issue we have with Mira is her irritability and how there are days where it is just relentless, and completely exhausts everyone in the house. Mira spends some part of her day, every single day, crying, screaming and/or fussing. If she has a bad day, she usually follows up with a bad night - which she has had several screaming/crying nights over the past few weeks. There are days that NOTHING will make her happy. It’s always a guessing game for us as to what is causing her to have such an irritable demeanor every single day, but there is no sense in trying to guess. We usually just chalk it up to something neurological.

We end up having to play junior neurologists during these periods of increased crying and monitor her seizures more closely. Typically, we see an increase in seizures and twitching, which always leads to the conversation of changing medications. We had that conversation a few weeks ago with her neurologist and we decided to put Mira on Banzel (Rufinimide) last week. So far, it has decreased her seizures significantly since she started it, which is fantastic. Her demeanor is improved slightly, but she is still having her daily screaming/crying fits, which are now limited to the late afternoon for some reason (probably because she refuses to eat all day). Her Banzel dosage gets increased tomorrow and we have a standing appointment with her neurologist on Friday to talk about how she is doing on it.

We did try reducing her Lyrica dosage weeks ago with disastrous results. In our efforts to curb her irritability, we talked to her neurologist about setting up a weaning schedule and slowly began to drop her dosage to see if there was a correlation between her crankiness and the Lyrica. Mira was great for a few days but immediately began having really intense seizures and even a few longer tonic-clonic seizures (mostly when waking up) that really took a toll on her. We had not seen intense seizures like those for at least a year or more. Needless to say, we put her back on her regular dosage with the understanding that the Lyrica is doing SOMETHING for her – maybe not controlling her seizures completely, but helping in some capacity. It’s all trial and error.

On the house front…………the addition is completely done on the outside and we have been plugging away and installing the mechanical, electrical, and plumbing systems over the past month or so, which has been fairly slow going. We plan on getting the rough-in inspected this week and have a drywall crew on board to start in two weeks. Being in the role(s) of owner, architect, and contractor is exhausting, yet has been extremely cost-effective for us to say the least. Yet we are growing tired of the enormous plastic tarp in our dining area that separates the existing house from the addition. We are slowly getting there. A million thanks to Aura and Ware for taking Sarah and the kids in for a week while I did all of the demolition – there was no way I could have accomplished that much work without your extreme generosity and hospitality!

While Eli is getting back into a groove with school, Jonah is finding his own groove by scooting everywhere around the house. His official new nickname is ‘Scooter’. He is pulling up on everything now and it’s just a matter of time before the chaos begins. That kid has such an infectious smile and is an unbelievably even-tempered go with the flow sort of kid. If we could only get him to quite waking up every 2 hours in the middle of the night! We are trying some new bedtime routines with him to see if that will help. Wish us luck!

Hope you don't mind if I go off on a tangent...............It’s interesting through this journey of Infantile Spasms that there are days that are so draining and exhausting that you just ache - emotionally, physically, spiritually, and everywhere in between. Then we read the daily updates from Reagan Leigh’s blog and the struggles that their family deals with every day make our lives seem pretty easy. Our family is at a different point in this journey in some ways, perhaps a little further down the road and we can remember those awful sleepless nights, the insomnia, the nighttime screaming and crying, the terrible eczema, the relentless multiple seizure days, and constant medication trials. The irony I suppose is that it may sound like we are still dealing with those same issues years later (and don’t get me wrong – we still are) but they seem so much less severe now than they did just 2 years ago. I don’t know whether we have just modified our coping strategies and learned to just 'live with it' or perhaps we have simply become numb to it all and are living in a constant state of high-functioning pseudo-PTSD . It’s only been 4 years for us. Who knows what the next 4 will bring. One thing is for sure is that our hearts go out to all of those families dealing with a seizure disorder, whatever stage or severity.

MRI

2009-07-14T22:12:00.002-05:00

Mira's MRI yesterday went off without a hitch. She had a rough morning, probably because she wasn't allowed to eat anything 6 hours prior to the MRI and only clear liquids (she has never cared for plain old water) up until a few hours before. She was really hungry and cranky by the time we got to the hospital. Fortunately, everything was running on time and Mira did great during the scan. They said the results would probably be ready toward the end of the week. If nothing remarkable comes from the MRI, chances are we won't hear anything from neurology until our appointment in September.

Happy Birthday - Part Deux

2009-07-08T22:04:00.020-05:00

Last weekend we had our official combined birthday party for Mira, Jonah, Beau, and Evie (friends of ours) at a local swimming/community center in Mission. The party had all of the grandeur and splendor that one could hope for - balloons, hats, goofy sunglasses, and of course, lots of cake. A special thanks to Erin for organizing the party and getting the festivities in order and to Aura, Ware, Addy, and Truman for making the trip in to celebrate with us.

The 4th was a lot of fun - despite the Marching Cobras not being in the lineup this year at Villagefest. Budget cuts are hitting hard this year to Prairie Village apparently. I didn't think that Eli was going to run into his archenemy, Sluggerrrr (the Royal's Mascot) for their third annual Villagefest Smackdown. As luck would have it, Eli and Sluggerrr did eventually meet up on the concourse at Kaufman Stadium that afternoon at the Royals game. Sluggerrr gave Eli a big hug and no one got punched or kicked - I think this year, it was a draw.

This past week has been pretty hectic to say the least. Sarah and I have been on the fence about taking Mira to see a specialist, Dr. Harry Chugani, in Detroit. Dr. Chugani is a neurologist with extensive experience and exposure with children with Infantile Spasms. I have corresponded with him through email over the past few years, asking questions, which he always responds to very quickly. Prior to actually seeing Dr. Chugani in clinic, Mira would be required to have a 24 hour EEG and a PET scan, neither of which she has had in the past. We originally had everything (EEG, PET, and consult) scheduled for the first week in August, but have since postponed the trip.

Going to Detroit would require us to be there for nearly a week, which isn't that big of a stretch from a travel standpoint, it frankly has more to do with putting Mira through additional questionable testing. It's all speculative at this point - looking for a needle in a haystack to a certain degree. For now, we are going to put the trip on hold and see Mira's regular neurologist again, before we potentially reschedule something for Detroit. We are also considering a trip to the Mayo Clinic, which we also want to discuss with her neurologist.

One of tests that Mira has not had in several years is a MRI. She has had two in the past - her first when she was originally diagnosed at 11 weeks old and another when she turned one. Her neurologist thinks it would be a good idea to have another MRI prior to seeing him, to see if there have been any significant changes over the past 3 years. We have the MRI scheduled for this upcoming Monday.

Mira's latest hunger strike has been going on seemingly forever. She is drinking plenty, but snubs absolutely everything from a spoon. The Gerber Goop, oatmeal, and her usual staples have lost their appeal for some reason, thus we resorted to our old standby, fresh baked sweet potatoes. She wouldn't eat a bite yesterday, but we managed to get a half of a bowl in her this evening, which was her first 'full' meal in over a week.

On the flip side, the tooth fairy has been paying (literally and figuratively speaking) more visits to Eli over the past few weeks. Eli's left front tooth fell out last week to which he replied, 'aren't front teeth worth more money to the Tooth Fairy and shouldn't I get a bigger toy for this one?' Yes, I suppose they are and I will relay that message to the old TF and see that you are adequately compensated. Eli is already a chief negotiator at age 6.

We are finally seeing some progress with the addition. After 6 weeks of frustration with our concrete sub, he finally had his crew pouring the walls today. It has been a comedy of errors with the foundation guy and I cannot wait to get him off of the site - he is unprofessional, unreliable, and frankly, just dishonest. The good news is that he is almost done. Good riddance. The framers will start in a few days.

In all of our hustle, craziness, and headaches over the past week with the house, I not only forgot my Dad's birthday last week, but even forgot to wish him a belated birthday on the phone last night when we talked. Sorry, Dad - Happy Birthday!!!

Happy Birthday Mira!

2009-06-28T23:55:00.011-05:00

Mira turned 4 years old today and it was only fitting that after having a week of brutal humidity and upper 90's temperatures, some relief finally arrived, with the weather and with Mira's eating. I say that because after 4 or 5 days of not eating more that two bites at every meal, Mira finally relented and ate a jar of food over lunch. I think we had some good vibes from Tom, Nikki, Sophia, and Siena who were visiting from Saint Louis for the weekend. Mira started one of her hunger strikes earlier this week and has been very, very irritable since. She falls into one of her viscous cycles of crying because she's ravenous, yet refuses to eat patterns until everyone is exhausted and frustrated. Hopefully she turned a corner today and will get back on the eating train.

We have a party planned for Mira on Friday, but in the meantime, we had a small celebration with just Sarah, Eli, Jonah and I, with cake from Avelluto's. Mira quickly snubbed the cake and ultimately ended up in tears. She didn't care much for dinner either, but ended the day on a good note.

The one thing that Mira will consistently respond to is the Bunz (her pull-string bunny) only the original Bunz is in need of serious repair. The string is worn thin and pulled out of its socket, only playing a few seconds of the song before needing to be pulled again. Sarah managed to find a hippo (which is pink like the Bunz) and plays the exact same song! Tonight was our first trial with the faux-Bunz hippo and she loved it. Again, it is one of those moments that Sarah and I enjoy every evening where we know we can get a smile out of Mira at the end of a long and often trying day. If we are really lucky, we get to see 'the dragon', where Mira sticks her tongue out and makes a funny breathing noise when she is really happy - you can barely see a small faint 'dragon' face about 45 seconds into the video I posted.

We did have a great weekend, despite Mira's eating woes - a huge thanks to Tom and Nikki for coming up to hanging out, the birthday gifts, and for watching the kids last night, so Sarah and I could escape to celebrate our 9th anniversary (which was almost 2 weeks ago) at Le Fou Frog. We loved seeing all of you and really appreciated getting out of the house!

Gerber Goop

2009-06-01T00:09:00.012-05:00

If there is one thing we have discovered over the past month is that we should do some serious investing in Gerber stock. When I was a kid, we called it Gerber Goop. It looks terrible, smells strange, and is all around, pretty bland. Mira’s typical diet up until now has been fairly predictable. Sweet potatoes, oatmeal, warm bottles of milk, and the occasional pureed leftovers. We found months ago that Mira loves warm oatmeal, with maple and brown sugar being one of her favorites. That has been working well for her. Breakfast typically goes off without a hitch, topping the morning off with some warm milk mixed with protein powder to get her day going. After breakfast though, everything is hit or miss with her. She can go on a hunger strike that may last for days, with little food wise that will spark her interest. Until now.

Sarah was out of sweet potatoes a few weeks ago and was desperate around lunch time for Mira, when she figured she would feed her one of Jonah’s baby food jars. Mira gobbled it right up and several more jars right after it. This could not be any more convenient. The next day, we loaded up on various 6 ounce Gerber concoctions, including spaghetti and meat sauce, chicken noodle dinner, squash, and anything else that wasn’t green or just didn’t deserve to be pureed and jarred. Through trial and error, we found that Mira’s new found Gerber love is the chicken noodle dinner, which she is now known to throw back 3 jars in a single sitting.

Why she loves bland baby food is beyond us, but she has been consistently eating it for a few weeks now, with a whole lot less hunger strikes. Baby food is bland, but we think she enjoys the texture. Whatever the case, I must admit that it is EXTREMELY convenient. Instead of constantly cooking, reheating, and preparing food that she may or may not eat (which becomes embarrassingly wasteful and expensive), we just take a few jars of Gerber Goop with us and go. We will continue to experiment with some less expensive alternatives, seeing if she might try some canned soups and vegetables. The stage 3 jars can sometimes run $1.50 a piece and Mira easily eats two jars at a sitting, sometimes three.

We also discovered a good organic ‘off the shelf’ milk that Mira is really enjoying. We have always struggled with warming up milk for her and trying to keep it at her desired temperature when we are out running around, but now we can just take the prepackaged milk containers with us. We found them in bulk at Costco over the weekend. Yet another convenient way to keep Mira happy – room temperature milk that won’t spoil on the go.

As far as seizures are concerned, Mira is still having a few involved ones every day. We had lunch in Lawrence this afternoon and she had one at the table that lasted less than 30 seconds or so, but the ‘aftershocks’ and twitching went on for several minutes afterwards. She has a lot of subtle jerks and twitches throughout the day, but typically only 1 or 2 longer seizures.

We are still struggling some days to keep her happy and today was no exception. Mira was pretty cranky all afternoon, so I took all three kids for a walk after we got back from Lawrence (hey Aura and Ware, did we mention we had La Familia sweet salsa and Free State Beer?!?!); I think Mira enjoyed just getting out again. I could tell, since the minute we came back in the house, she started crying again. It’s difficult to keep her entertained all the time, but we try to break up the day for her with trips and outings.

We managed to take a brief road trip last weekend to Saint Louis and were able to watch the Royals snap their losing streak against the Cards at Busch Stadium. (They are since back to their losing ways at Kauffman Stadium.) It was a beautiful day at the Cardinals game and we managed to meet up with some family before, during, and after the game. We also briefly met a mom (who is also named Sarah) at the game who has a daughter nearly identical to Mira, from her age, to her medical history, and whose daughter continues to struggle with seizures, just like Mira. It is great for us to run into other families who you can immediately relate to and know exactly what you are dealing with. You always seem to make an instant connection with them.

Summer break is officially here, with last Tuesday being Eli’s last day of Kindergarten. I cannot believe this year has flown by so quickly. Eli is getting so tall and by my estimation, he is going pass me by the fourth grade. He also talks nonstop. I bet he spits out 25,000 words a day. Some days, I seriously wish he had a pause button so I could get a few words in. His new favorite love these days is playing Lego Indiana Jones on the Wii; amazingly he is capable of talking nonstop while playing. He is a great kid with an unbelievable amount of energy.

Jonah is also getting enormous. He has been getting his fair share of tummy time in an effort to entice him to crawl, walk or just throw his weight around so he can understand his potential mobility. It hasn’t really motivated him to either crawl or walk, but once you put him in his walker, he will flatten your toes in 2 seconds. Sarah and I wear heavy shoes around the house when he is on a tear in that walker, since he knows no boundaries.

The house project is finally moving forward, after a lot of ongoing frustration with our lender. Unfortunately, the frustration and anxiety has not subsided completely with the bank, but it will all fall into place this week. The good news is that we did actually start construction this past Wednesday. Currently, we have an enormous partially dug hole to show off, but that is about it. While the foundation was being dug, we had a few dilapidated trees removed from our back yard. This week, we are having some electrical work going on and the footings are supposed to be poured by mid-week.

Update

2009-04-27T00:50:00.019-05:00

How time gets away from us all – I have been meaning to update Mira’s blog for the past month and just now am getting around to it. I swore April just started a few days ago and already we are heading into May.

I first wanted to give an update on what has been happening in Mira’s world over the past month and a half. It’s hard to explain, but most of days, it seems the more things change, the more they stay the same. Mira continues to have seizures every day, having only one day since my last post where Sarah and I didn’t see a single one. Yet, the seizures that she is having are more intense and are lasting longer. She is getting back in to this cycle of having intense seizures again. It feels as though it’s 2007 all over again – same intense seizures that slowly and steadily get worse until we have to jump on the medication merry-go-round in hopes of giving her relief.

We started the discussion with her neurologist about Rufinamide, which is pretty new to the market, but the clinic only has 2 kids on it currently and don’t seem too eager to put anyone else on it. I have all but given up on Lyrica at this point, despite Mira being seizure free for a period of time last year on it. It certainly isn’t doing much now but possibly adding to her irritability.

It is really difficult to explain to people what it is like to live with Mira most days, unless you spend some time with us here in the house. One of the things that we battle with most is the crying and irritability with Mira. She spends a part of her day, every single day, crying and screaming. Some days it is not difficult to distract her with bottles of milk, providing a change of scenery, taking her for a walk, or something to break the cycle of boredom, hunger, or whatever it is you think is ailing her at the moment. Other days, like today, she is just crying, fussy, and inconsolable and there is nothing you can do to snap her out of it. It’s very frustrating and sad to watch her. The best way to describe it is to imaging living with a 3 month old baby, all the time. It makes it tough when you have two other kids to take care of on top of it.

I have had several neurologists in the past tell me that the seizures don’t hurt her, with which I have to disagree. I think about 90 percent of the time, there is something neurological (and/or medications that exacerbate her issues) that causes her to be irritable and that the seizures themselves are actually causing her pain or at least discomfort. For instance, I was 10 minutes into our stroller ride with Mira and Jonah this afternoon (an attempt to improve her mood after a particularly cranky morning) and she had a very intense seizure. She was pretty quiet afterwards for about 10 or 15 minutes, then became extremely fussy and by the time we got home, she was crying so hard I thought she was going to vomit all over me. It took her another 45 minutes to stop crying.

It’s a vicious cycle of seizures, medications, and trying to figure out what is ailing her that it is beyond exhausting for Sarah and I sometimes. We are now debating whether to keep her on the Lyrica, take her off meds altogether or just try something else, which as history has proven to us repeatedly, may be more of a hindrance than helpful to the situation.

Despite all of the constant daily crying drama, things continue to move along slowly with her therapies. We were able to get her 6 weeks of hippotherapy, which were supposed to start last week, but as luck would have it, the therapist was sick. Mira is scheduled to go tomorrow for a session, but it is calling for thundershowers, which usually warrants a cancellation. We haven’t made any changes to any of her other therapies and she is making some very small gains (in her gross motor skills especially) through all of it over the past year.

We did get Mira’s final CDKL5 labs back about a month ago, which were normal. No surprises there. While this doesn’t ‘officially’ rule out a Rett Syndrome diagnosis, it does ensure that she absolutely does not have a typical or atypical mutation or deletion of almost all of the known genetic abnormalities. There is one final genetic screen that has surfaced over the past year that is known to cause Rett-like symptoms, called the FOXG1 mutation, but we don’t really have any intention of testing her for it, unless her neurologist advises it.

Mira is still enjoying the bed and seems very confident in it. We often walk in on her lunging into the sides of the mesh – don’t know whether she is testing the durability or trying to rip a hole in it. Needless to say, both Sarah and I are so happy to have it. It gives me some piece of mind that she is safe at night in it and won’t injure herself. Some of her other equipment isn’t holding up so well. I had to repair the footrest on her Kid Cart (again). She tends to kick it so hard and so often that she knocks the entire assembly out of its socket and it needs to be reassembled and bolted together again. This is the third time she has done this and the footrest is starting to show its frustration with Mira’s feet. It’s starting to rebel by requiring more bolts to hold it together.

Although it is nearly May and school is drawing to a close in another month, Sarah and I were glad to get some vacation time in the last month and managed to take a couple trips. Our first was to Illinois to visit Auntie Aura and Uncle Ware for a week over spring break. We took all of the kids and even successfully managed a day trip to Chicago and visited the Shedd Aquarium. The following weekend, Sarah and I took the Eli and Jonah to San Diego and spent some quality time with family and at Legoland. We also visited the USS Midway and a quick trip to the Pacific Ocean. I think it was a much needed excursion for Eli to have a couple of days where it was just about him. We get so preoccupied with taking care of Mira and Jonah that Eli often has to patiently (or not so) wait his turn. It is a tough balance for all of us sometimes. It was the first trip we have taken without Mira. It was sad not to have her with us, but at the same time, it would have been a rough trip for her all around, from airplane rides to car rides to sleeping somewhere new for a few days. She was well cared for back at home with friends and family.

Outside of our trips back in March and early April, things have been pretty quiet. Jonah’s baptism was last Sunday, which went off almost without a hitch - only one baptismal candle burning incident during the entire ceremony and one KidCart malfunction (yes, the footrest issue). Jonah didn’t mind the water too much, but the oil sent him over the edge – I think he was mad because it messed up his hair. He spent all morning on it.

Nothing has been happening on the renovation. We are still waiting on the loan. Now that it’s warming up outside, contractors (including my foundation sub) are getting busy and I am finding it tough to get on their schedules. Oh well, we are optimistic that eventually we will start building – might be 2010 at this point.

Mira's New Bed

2009-03-05T21:27:00.018-06:00

After months of anticipation, we are excited to announce that Mira's bed is finally here. The mobility supply company came this afternoon and set it up in about an hour. We did have to play some musical furniture prior to it being delivered. First, we had to get the crib out of Mira's room and into Eli's room (where Jonah will start taking naps and eventually start sleeping) and took all the miscellaneous pieces of furniture out, just to get the bed in. Frankly, we had no idea it was going to be so tall. We did take some initial measurements when we ordered it, but the bed pretty much spans from door to window (see pictures below) and comes within inches of the ceiling fan. It's tight, but it works just fine. Prairie Village houses are not known for their size - the bedrooms are really small to begin with and the bed makes it look even smaller, but who cares.

Needless to say, we love it and Mira loves it. No more banging her head into the crib, kicking the rails out, and the best part of all, we can change her in the bed. We currently have a baby changing table that we use to change her on, which works great for Jonah, but not a 3 foot tall, 35 pound wiggly girl. Now we can just swing the side rail down and change her right on the bed.

The bed basically has a very durable mesh fabric on all four sides, along with tilt-up side rails for protection against the wall. The mesh sides all have zippers, so the entire canopy becomes zipped tight and will keep her from falling out. The mesh is non-abrasive and gives just enough to cushion Mira when she falls, leans, or launches herself into it. The canopy posts and side rails are all heavily padded, so there is virtually no way of her knocking her head against anything. It is designed to keep her safe, which it will. The mattress is close to a twin size, which is plenty of room for growth and designed so that she will use it for years to come.

Eli's face sort of summarizes our eventful day - we are very excited to have the bed. Mira did a lot of jumping around and exploring for about a half hour once it was set up, eventually wearing herself out. We knew she was officially spent when she propped her feet up on the side rail and called it quits.

We wanted to personally thank the IDC, the Love Fund, and Dad and Dottie for helping us pay for it. We are eternally grateful - thank you!

Bedtime is Almost Here

2009-03-03T12:48:00.004-06:00

We are so excited that Mira's bed is finally coming this week! A technician is supposed to come on Thursday afternoon to deliver it and set it up. It could not be any sooner. Jonah will be graduating from the bassinet to Mira's crib. Fortunately, the crib is still in one piece, needing only minor periodic repairs from Mira kicking out the side rails. I have secretly nicknamed her feet 'Tiny Daggers' (named after the not-so-famous song by INXS - I am always very random), kicking and destroying everything within range. It's hard to believe that Mira has been using the crib for the past 3 1/2 years. We have some furniture rearranging to do and we are crossing our fingers that the new bed doesn't take up the entire room.

Now that it is March, I think the sickness is finally subsiding and everyone is feeling better. Jonah still has a slight cough but nearly there, Eli is back to his normal rambunctious self, and Sarah's flu symptoms have drifted away. Mira continues to have some good days and not so good ones - I think she just needs to get out of the house more often, which is difficult when there is snow on the ground and it's 15 degrees. Today is the start of a warming trend, so hopefully we all can get out and get some fresh air.

I spoke with neurology in regards to Mira's CDKL5 testing and they said it could be another 3-4 weeks. Some testing takes longer than others I suppose.

I will post bed pictures Thursday hopefully!

Mira..........Volume Control Please!

2009-02-21T21:52:00.005-06:00

Mira was in an exceptionally good mood all day today. Perhaps it was the riveting trip we took to Home Depot this morning that inspired her. She actually slept through the night last night and didn't get up until after 7:00am. Since Eli has had the flu all week (missing 4 days of school) and now Jonah coming down with a high fever (and Sarah too) Mira and I are turning out to be the only ones avoiding the sickness that has perpetrated our house this past week. Knock on wood, Mira hasn't had so much as a sniffle and has been in a fantastic mood. Go figure?!

One of things Sarah and I noticed today in particular was her vocal talent. She either needs to stop watching American Idol or she is trying to tell us she wants to go out for karaoke night. She has some serious lung capacity these days. The combination of vocals, tray kicking and her globe toy is enough for us to consider ear plugs. All joking aside, it is great to hear her making new sounds and having fun!

Mira tends to do a lot of hand-mouthing throughout the day, but today she would stick her hand in her mouth and was purposely making noise (see video below) which was something new for her. She was consistently energetic today in her chair (much like in the video) and was doing a ton of vocalization, all day long. Enjoy!

Testing

2009-02-20T09:10:00.001-06:00

Testing blog.

Video Test

2009-02-19T23:07:00.006-06:00

So we bought another gadget - a 'Flip' video camera that holds up to 30 minutes of video and is no bigger than a cell phone. It was marked down from $150 to $60 on Amazon, so I'm not sure how great it works. I just created a little Mira video of her to see how it does. The resolution looks great on our laptop(s), but comes out pretty poor here and it's jumpy - not sure what the deal is.

Real Life Groundhog Day

2009-02-12T20:19:00.026-06:00

Just wanted to give a quick update on what has been happening over the past month. Mira has had a quiet couple of weeks and some great stretches of quality sleep. She hasn't been getting up much in the middle of the night and when she does, she usually goes back down without an additional dose of melatonin. The past week hasn't been so great, but no complaints after a few solid weeks of scream-free evenings.

I wish I could say the same for Jonah, who is still getting up every two hours throughout the night. He also caught another cold virus last week, with another round of gagging/vomiting, fever, and nebulizer treatments. He has been feeling better and vomiting less over the past few days, so he is on the mend, we think. I must admit that it all feels exactly like Groundhog Day (the movie - one of my all time favorites) as if the beginning of February is exactly like the beginning of January. Where have the last 4 weeks gone?!

We finally ordered Mira's bed today. Thanks so much to the IDC, the Love Fund, and Dad and Dottie for helping us out - we appreciate the support tremendously! It should arrive in a few weeks and frankly, it couldn't be soon enough. Mira continues to get her legs and feet caught between the rails of the crib.

I thought I would post of few pics of all the kids - they are growing so fast. Mira got a desperately needed haircut late last week, which is always an adventure. Mira HATES to have her hair brushed and screams bloody murder whenever Sarah tries to comb the tangles out, so hopefully snipping a few inches off will lessen her daily hair-brushing tantrums. We have noticed that she gets ten times more upset having her hair brushed as she does when she gets a blood draw.

We haven't heard back on the CDKL5 deletions testing as of yet, but we will check in with genetics in a few weeks to make sure things are moving along. Mira's seizures have been pretty steady and consistent over the past month. Although some seizures seem to be more intense than others, that has never really meant anything. She still is having several dozen small seizures a day, which hasn't changed since last summer. She is holding steady on 75mg of Lyrica still and we don't plan on changing her meds anytime soon.

New Pictures and Updates

2009-01-18T07:42:00.016-06:00

Now that the RSV fiasco is almost completely over, things feel as though they might actually be reaching a level of normalcy around the house. Jonah still coughs every so often (and at night wakes himself up in the process), but I think we are in the clear. We took the nebulizer back to the pediatrician's office on Friday, so the breathing treatments are officially over.

Mira is doing alright. She has had a few nights of screaming and crying, but nothing out of the ordinary. She woke up Wednesday night and had a lot of difficulty decompressing for a few hours, but eventually went back down. Sarah and I decided on nights like that, we probably need to break out the trazadone to help her calm down and get some rest. The trazadone tends to work quickly. She ends up having a rough day through therapy and eating after a patchy night of sleep.

We are talking with neurology and genetics (and have been for weeks - it's a slow process) about getting some additional Rett's testing. As it turns out, there are four different testing scenarios in regards to Rett's. Mira has had 3 of the 4. She has been tested for the MECP2 variant ('typical' Rett's - both mutations and deletions) and CDKL5 ('atypical' - early onset Rett's - but only for mutations, not deletions). Therefore, CDKL5 testing for deletions, would be the last test. Neurology and genetics need to discuss what the right testing procedure is for the right variant. It all takes time.

We are hopefully going to order Mira's bed in the next couple of weeks, which is exciting. We are working on getting the funding in place through several (generous) local organizations. The director of Mira's school has been an IMMENSE asset in this endeavor, pointing us in the right directions and being our liaison to several orgs. On the other hand, insurance has been no help in this whatsoever. The approach insurance often takes is to wear you down through paperwork and processing time so that you ultimately give up in trying to navigate the 'system' (dare I call it that). Insurance is exhausting and frustrating sometimes, but at the end of the day, I am grateful that they have paid for just about everything else.

I may or may not have mentioned it before, but Sarah and I have been planning on putting an addition on the house for quite some time. Now that we officially are out of bedrooms and tripping over our own feet in our 900 square foot mansion, it's time to move forward with it. I met with several contractors last week and we are in the process of getting bids back. The market is soft and I think most GCs are hungry, which works in our favor. The way this will probably pan out is that we will have the shell and framing put up on the house and we will take on the rest of the interior construction. I suppose it will be a good learning experience for Eli to learn how to hang drywall and pull electrical conduit. Sarah will also have to invest in a good tool belt. Anyway, it's all moving forward and we hope to have some bids back by the end of the week.

And yes, we are still having fun with the camera - we found that one of the great things is getting really close up shots of facial expressions and lately, lots of drool. Enjoy!

New Pictures

2009-01-18T07:42:00.001-06:00

Now that the RSV has

Happy New Year! (Cough)

2009-01-05T22:49:00.013-06:00

If there is one thing that the holidays provide in our household, it's adventure. While Christmas itself came and went without much fanfare and excitement, the week after was pure crazytown. Jonah contracted RSV and is just now starting to get over it. He has been vomiting off an on for a week or so, with today being his first vomit-free day (although technically it's 11:30pm - there's still time)...... The coughing and sickness made its way around to everyone else eventually, including Mira, who is now battling lots of phlegm and periodic coughing fits. Needless to say, we are all pretty wiped out from all of the fevers, sneezing, coughing, gagging, breathing treatments, and of course, non-stop laundry from cleaning up puke. Delicious.

One of Mira's big gifts this Christmas was a piano/keyboard, which has light up keys. She has always been fond of her globe toy (which has seen better days) but we figured we could get her something to bang on that offers a little more visual and musical excitement. She wasn't too enthusiastic about if on Christmas Day, but we are hoping she will warm up to it.

One of the highlights of the holiday season (prior to the RSV onslaught) was getting a surprise visit from Auntie Aura, Uncle Ware, Addy and Truman! Friday evening I answered a knock at the door and to my surprise, Addy was standing on our porch yelling 'Merry Christmas'! It took a few seconds for it to register as to why and how my nephew from Illinois was standing there, but eventually it sunk in. We did get to enjoy a few days hanging out between Christmas and New Year's with the whole gang.

As far as medications go, we had Mira on an increased dosage of Lyrica for over two weeks and it did not go well. The increase really knocked her out. She started sleeping a ton - most of it during the day. When she was up, she was pretty irritable. She has been really cranky over the past few days in particular, probably from all of the congestion and phlegm. her first experience with Lyrica was pretty dramatic in halting her seizures (within a few days), but the increase this time didn't help at all. We didn't see any difference in seizure activity over the past few weeks. Lots of head drops and myoclonics throughout the day.

Sarah and I were having a conversation about Mira and seizures and I think we've come to some harsh realities in this journey with her. I think 18 months ago, I would have thought that there is a 50% chance that something would stop them. Now, I can't help but think that the likelihood of ever gaining seizure control with her is close to zero. We have exhausted all of our options with medications and are frankly just plain exhausted with it all sometimes. It's the days when she is screaming her head off and you can't figure out why or what to even do for her, that it all feels so helpless. We just continue to take it day by day. Sorry for the mildly depressing rant - it's late and I am sleep deprived. I will post new pictures soon.

Christmas is Almost Here!

2008-12-23T06:41:00.007-06:00

Nothing much has been happening around here, other than we are all excited about it being 2 days away from Christmas! It's funny how I just remember it being December 1st 15 minutes ago. December always burns by in a flash and suddenly it's Christmas?!

We moved Mira up to 100mg on the Lyrica over this past week and frankly, it doesn't seem to make a bit of difference. She seems significantly sleepier and overall a little doped up. She's having just as many seizures and the irritability factor is still present. We will give it a little while longer to see if the increased dosage will 'kick in' (maybe another week or so) but we are not optimistic. Mira's neuro didn't seem to think it would work, but it is certainly worth a try, since it's the only medication to ever work for her.

Mira is still taking the melatonin and still getting up in the middle of the night. We could either bump her dosage up or try a time-released brand of melatonin. The fact that she is able to get in a few solid blocks of sleep throughout the night is a dream (no pun intended) in comparison to what we were all going through a few months ago.

On the bed front, insurance denied our claim (the correct claim this time) with the explanation that our policy has an "exclusion" for anything but a 'regular hospital bed.' It's comical almost to imagine Mira sleeping SAFELY in a bed like that. Why yes, that seems safe to put my 3 1/2 year old child with severe dyspraxia and hypotonia in a bed with 12" side rails elevated off of the floor several feet. That makes sense.

So, we are currently looking into alternative funding sources available to us here in the area, through Mira's therapy group/school and some other local organizations. From what we have been told, there is funding available, we just need to apply. We were able to get part of Mira's stander and KidCart funded through similar groups in the past. We will see what happens and hopefully have Mira in a new bed not too far into January.

I did have a brief conversation with Mira's neuro about having her screened for a recently discovered Rett's Syndrome genetic variant called FOXG1. He is doing some research on it to see if it makes sense to have her screened for it. Mira was screened for MECP2 and CDKL5 (and the results were negative), the only two known and well documented genetic screens for Rett's. FOXG1 seems to be the third that is only recently coming into focus. At this stage, we will leave it up to her neuro to make the call whether it makes sense to have the screen done.

Moving on Up

2008-12-07T22:24:00.010-06:00

We didn't really notice any difference in adding the ranitidine over the past week, so we suspect that Mira's irritability is not due to reflux, but most likely, it's all just neurological. We increased her Lyrica dosage to 75mg daily and will hold there for a week, most likely moving up to 100mg next week. We will wait and see what happens.

Sarah and I are still having fun with the camera. We tend to get some good pictures (catching Mira is strange poses as though she is telling some 'big fish' story) but we still need some guidance on getting the most out of the camera. The beauty of digital is that you can take as many shots as you want without it costing a thing.

Mira is fairly consistent in her sleep these days. We have stopped giving her the trazadone and opted to just give her melatonin only. We give her 2.5mg before bedtime and she sleeps pretty solid until about 1am, then wakes up crying. We give her another 2.5mg and she sleeps until about 5:00am-6:00am and usually wakes up crying again, probably because she is hungry. She has been consistently doing this most of this week, which is actually a nice change of pace. I am personally glad the nightly inconsolable screaming fits have stopped for now, knock on wood!

Neurology Visit

2008-12-01T22:41:00.003-06:00

Sarah called neurology this morning in hopes to schedule an appointment, which usually takes about 2-3 months just to get on the books. As luck would have it, someone cancelled this morning and we were able to get in this afternoon! We gave her neurologist an information dump as to what has been going on over the past few months. We all agreed to do a trial of ranitidine (Zantac) in hopes of targeting some of Mira's possible ailments. Since Mira can't tell us what is going on, we have to play 'Name that Fuss'. So at this point, our best guess is that all of her irritability could be reflux. So, either the ranitidine will give her some relief or it will do nothing. I hope and pray that it helps her and it's something very simple.

As for the seizures, we are going to do an increased trial of Lyrica after a few days of the ranitidine and see what happens. No one seemed to have any definitive suggestions at the appointment as what to try next in terms of seizure control, so this is our best option for now.

Update

2008-11-29T20:39:00.008-06:00

Mira's sleep over the past few nights hasn't improved much. It took her and hour last night to stop screaming and fall asleep, only to get up for several hours again, from about 3:30am until 5:30am. Part of the problem as of late is that we have a few variables in motion, which included adding Gabapentin, which I will get into later. The other thing is that Mira has been battling constipation for months now and when she has trouble clearing out her system, it only adds to her sleeping problems and irritability. Needless to say, we have been giving her Miralax nearly every day to help with the constipation and it seems that when we miss a day or two, it really throws her whole 'chi' off and we ultimately pay for it days later. Well today was the day for her to clear out her system. This has happened several times over the past month and the result is the same. The only detail that you need to know is that another outfit has been officially ruined.

The trial with Gabapentin has been another medication disaster. I think we are now 0 for 4 with post-Lyrica medication trials. She has been on it for about 10 days now and not only have we seen a marked increase in her irritability (yes this could be attributed to her ongoing constipation, but not for 10 straight days.......) but Sarah and I are seeing a substantial increase in seizures. I swear she was consistently having one every 15 minutes today. Lots of hand wringing on her right hand side and funny movements for a few moments after every seizure too. I thought the premise of the medication was to DECREASE her seizures?! I don't know where we are going to look next, but we have discontinued trying Gabapentin for now. It was a very low dosage and there wasn't anything positive about it.

We did receive a letter back from our insurance last week, giving us written confirmation that they have denied our claim for Mira's bed. The problem is that they denied the claim based on a motorized bed, not the manual one we had requested. I'm not sure what happened during the claim process, but basically they denied the claim based on the wrong type of bed. Now we have to try to get our insurance back on track and make sure they are processing the claim for the right bed this time. More paperwork and phone calls.

After years of fighting to get decent pictures of the kids with our point-and-shoot digital camera, Sarah and I finally invested in a digital SLR. After two days, I love it. No more 5 second delays, takes and retakes, and trying to capture decent shots with the kids! The SLR is so slick and has so many foolproof options that it's hard to take a bad picture with the right lighting. Mira posed like a rockstar this afternoon in her 80's shades for us to test out the new camera.

The Long and Winding Road

2008-11-18T21:53:00.018-06:00

Sorry it's been such a long time between posts, but things have been fairly hectic over the past few weeks. Unfortunately, our positive melatonin experience(s) had pretty much subsided after my last post on the 3rd. Mira had a couple of great nights at 1mg, but it soon wore off and she returned to her poor sleeping pattern. She started getting up earlier and earlier, so we increased her dosage to 3mg and it was still only helping her for a few 2-3 hour stretches every night. She continued to scream relentlessly during the night, so we ended up calling neurology for some relief. No one seems to be getting quality sleep these days.

After pleading with neurology for some help, her neurologist recommended trazodone, which is a sedative. It's not uncommon to use for kids with developmental disorders and sleep issues, so we figured it is worth a shot. We started her on a low dosage and have since moved up to 25mg at bedtime. It seems to work for a while, letting her sleep solid usually until midnight or so. She consistently gets up and has a few of her screaming fits throughout the evening, in which we give her melatonin again to help her settle back down. It's a vicious cycle of sleep deprivation and irritability with Mira at night and we are just frankly exhausted from it all. We will be starting her on gabapentin (brand name is called Neurontin) which has a nearly identical mechanism of action as Lyrica, to help combat her seizures. I still believe that the root of all of her sleeping issues are neurological and anything within reason that might help is certainly worth trying.

We have been putting Mira in her stander every day, with her AFOs and she doesn't seem to mind it. She loves to try and throw herself backwards once upright, but eventually settles down and gets used to being in a standing position. It turns out to be a two-person job just to get her in the stander, since she is the queen of wiggling, squirming, and kicking. One has to take extra precaution when she is wearing her AFOs, since they are basically pointed plastic weapons once they are on her feet. They are rough on the chins (mine and Sarah's that is).

We did hear back from our insurance this morning on Mira's bed and our company denied the request. We could appeal, but having heard from other parents requesting similar equipment, it's a long painful battle and unlikely to end positively. Therefore, we are looking at $4850 for the bed. We are planning on calling the manufacturer directly tomorrow to ask them specifics on the bed and see what options are necessary and whether we might be able to reduce the cost some.

A few weekends ago we made the trek to Illinois for a few days at Auntie Aura + Uncle Ware's house. We went to an awesome pirate birthday pool party for Addy and had a wonderful time. Did someone say scurvy dog?!

Despite all of the broken sleep and ongoing trials with Mira, she seems to be taking it all in stride. Some days she does really well in therapy, is happy, and seems to be a chatterbox, vocalizing all day long. The next day, she might be on the opposite end of the spectrum, crying and fussing all day. If there is one thing consistent about Mira is that she is inconsistent - you never know what the day is going to bring. Kudos to my wonderful wife Sarah, who endures the daily nuances and at times, complete chaos in our house (appropriately referred to as 'crazytown' more often than not) day in and day out. You are awesome and I love you.

Melatonin Day 2

2008-11-03T20:31:00.003-06:00

A big thank you to all the positive feedback and good vibes I received from everyone regarding melatonin. We gave Mira a 1mg tablet last night and I only heard her once, again at 4am very briefly, and she was laughing! She went back to sleep and I didn't hear her stirring around until 6:15am, which was when Eli came storming up the stairs wanting to tell me all about his nightmare about giraffes. Note to self: no more nature shows before bed. Anyway, I think we will stay at 1mg before bedtime and go up if need be. Right now, melatonin is our friend!

I have been corresponding via email with Mira's neurologist about what we might try next, but I think we are not going down that path for at least a week or so to give Mira a break. Her neuro wants to think for a while on what might make sense at this point to control her seizures.

Let the good nights continue!

Melatonin

2008-11-02T21:53:00.004-06:00

After weeks of poor sleep, we gave melatonin a try with Mira. We gave her 0.5mg (very low dosage) before bed last night and she slept from about 7:30pm to 4am, even with daylight savings time! That was probably the best night of sleep she has had in weeks. She did get up crying at 4am, so we gave her another 0.5mg and she slept in another few hours.

We haven't seen any sort of withdrawal symptoms after stopping the ethosuximide, nor have we noticed any spike in seizure activity, which is all very promising so far. Let's hope she can go two nights in a row!

This Past Week

2008-11-01T21:18:00.011-05:00

We have had a pretty tumultuous time with Mira and her sleep over the past week. Frankly, things are really bad with her getting up in the middle of the night. She has been consistently getting up and staying up for hours, crying and screaming. Last night was a very bad night for her. She continues to be inconsolable during these episodes too - nothing seems to help. All of the insomnia, screaming, and terrible sleep cycles began when she started on the ethosuximide.

While Sarah and I think we have been seeing slightly less seizures with Mira over the past week, the constant screaming and crying in the middle of the night is more than we can stand. I have said this time and time again - either the medication works or it doesn't and in the case of ethosuximide, it was only partially working and the side effects for Mira were horrendous. We took her off of it today. We gave her a small dosage of melatonin this evening to help her hopefully sleep better.

We are so tired of the medication-go-round over the past few months. It is so exhausting for everyone, especially for Mira. We basically have few options left in terms of medications and are jaded by all of Mira's poor responses to everything we have tried since Lyrica. We will probably push on her neuro to try Gabapentin (which has nearly the exact mechanism of action as Lyrica) if things get worse again, but we are going to give Mira a break for a while. She has to be exhausted.

On a positive note, Halloween turned out to be picture perfect for all of the kids. Great weather and great costumes. Mira was a monkey, Jonah was a banana, and Eli was a Star Wars Jedi. Sarah rigged up Mira's switch on her Kid Cart tray so that when she smacked it, it said 'trick or treat'. Worked like a charm!

This Past Week

2008-11-01T21:18:00.000-05:00

We have had a pretty tumultous time with Mira

Still Crying

2008-10-27T23:21:00.002-05:00

Mira has been fussing in her bed for the last hour off and on. Her crabby episodes at night are becoming very routine, unfortunately. I have no idea what to do for her. Any suggestions would most appreciated!!!

Bad Nights Continue

2008-10-26T22:36:00.004-05:00

I don't know whether it's the increase in medications or what, but Mira's nights continue to be pretty rough as of late. Last night she was up for hours and just could not find her happy place to calm down. She just screams and cries. During the day, she tends to have her moments in terms of being fussy, but it's her sleep that is really erratic. She has been taking some power naps throughout the day to make up for it, but I really don't think she is getting enough sleep overall since she is up so much during the evening.

We increased her meds to 3ml twice daily on the ethosuximide last week and I suppose we are seeing less seizures. However, we always seem to trade one symptom for another with many of the medications, whether it be irritability, insomnia, or something worse. I just wish SOMETHING would work positively for her like Lyrica did.

We did manage to find something that Mira enjoys to eat lately (besides her usual daily quota of sweet potatoes) which is pintos and cheese from Taco Bell. Go figure. She gobbles it right up. We figured we could make the equivelant at home with refried beans, cheese and some salsa so we aren't making daily trips to TB and the fact that who knows what is actually in it. I think she tends to get bored with the usual food fare we put in front of her, so perhaps Mira figures ANYTHING is better than sweet potatoes, applesauce, yogurt, or oatmeal.

Better Days, Worse Nights

2008-10-19T21:15:00.008-05:00

After being on the ethosuximide for the past 10 days or so, it's really difficult to say if it's helping or hurting. Some days we seem to see a lot less seizures, as in today. I've personally only seen 2 or 3, but I haven't been staring at her all day waiting for something to happen. Other days, it's a wash as to whether she is having less or not. It's just hard to tell. The upside is that Mira is doing better during the day than she was on the Keppra or Depakote, although nights are another story.

Last night she was up for several hours just crying and screaming. There is little we can do to calm her down when she is on a roll - she is just inconsolable. There is always a correlation between her appetite and her poor sleeping. Yesterday, she wouldn't eat a bite at breakfast, lunch or dinner. She finally came around at lunch today and ate a decent meal. Tonight as I am writing, she is still having a tough time winding down and is crying and fussing herself to sleep.

One of things Mira has been enjoying of late is her globe toy. She has had it for ages (she actually has a spare one thanks to her former vision therapist who snagged another one for her on eBay) and has always enjoyed playing with it. Lately, she has been getting extremely intense with it, banging and smacking it so hard I think she is going to pulverize it into a thousand pieces. She will go for 45 minute stretches just beating it into submission until she is exhausted. She then sneaks in a 10 minute catnap in her chair, then lights it up for another half hour. She never gets tired of it.

Mira's orthotics (AFOs) came in last week and she had them fitted and adjusted at Children's Mercy. She will probably wear them for an hour a day, mainly during therapy. They should last a little less than a year.

We are still waiting to hear back on her bed at the moment. I called the beginning of October and they told me it was was going to be another 6-8 weeks before insurance will process it. This doesn't mean they will even approve it. If they deny it, we may have to appeal or go another route. I'm not sure why it takes so long - more insurance red tape I suppose. In the meantime, Mira keeps trying to sit up in her crib, which often results in her banging her head into the rails when she launches forward. She is just getting to big and too mobile for it to make sense for her.

The plan for now is to hold steady on the medication fromt. Mira has some room to go up on the ethosuximide, so we are optimistic at this point that it will help in some capacity. She is currently on about 30mg/kg and can in theory go up to 60mg/kg. We are taking it week by week, as usual.

Ethosuximide

2008-10-08T22:43:00.008-05:00

Sarah took the boys to Decatur over the weekend for a visit with Auntie Aura, while Mira and I enjoyed a nice long four day weekend here. As quiet as it was around the house, Mira seemed to be pretty irritable all weekend, so we managed to go on quite a few walks. I got our money's worth out of the double stroller and practically wore the tires flat in the process.

One of the things I did notice being home with Mira all day is just how many seizures she is having. I seriously lost count after 20 or 30. Most of them rarely last more than 5 seconds - where she lunges forward with arms extended for a few seconds, then comes out of it and pretty much goes back to what she was doing. It's the fact that she is having so many of them that is disconcerting.

There definitely seems to be a direct correlation between the seizure activity and her irritability. More seizures - more crying. There were a few moments over the weekend where she was just screaming and kicking like crazy and I could not calm her down, other than get her outside and go for a walk with her - just a change of scenery works wonders as a distraction for her. Her fussiness has continued into this week. The past several nights haven't been all that great too - getting up screaming and crying for 45 minutes to an hour each night.

We did take Mira off of Keppra last week, due to the fact that it wasn't doing anything and seemed to be agitating her. She had a very similar reaction with the Depakote - a poor one. So, we are essentially back in full swing on the medication merry-go-round. I spoke and emailed neurology most of last week, trying to determine what to do next. Mira's neuro agreed to try ethosuximide (brand name Zarontin - there is a generic equivalent which Mira is on) only because it has a very similar mechanism of action as Lyrica. Since Lyrica is basically the ONLY medication that has ever helped, we need to try and find similar medications with similar mechanics, but there just aren't many out there. She has been on the ethosuximide for only two days, so it's too early to tell anything.

Sarah took Eli and Mira to the dentist this morning and both did extremely well. No cavities, no problems, and no screaming!

On the Lighter Side

2008-10-03T21:09:00.006-05:00

In the chronic state of worry and anxiety that surrounds Mira every day, it's time for a little comic relief. Eli (as well as Mira) take Coromega every day, which is a fish oil supplement (omega 3 fatty acids - good for the brain), masked with orange flavor and a hint of chocolate. In all honesty, it's the best tasting fish oil you can find and you pay the price for good taste. A month's supply costs upward of $50-60 for the both of them. Sarah and I tried to mask the fact that it's fish oil by calling it 'orange pudding', but nonetheless, Eli hates it. Every morning I get the exact same reaction from him, which is 'blah!'. Enjoy!

'What's the Prommo?!'

2008-09-29T22:05:00.010-05:00

When Eli was about 2 years old, he couldn't quite get the word 'problem' out, so when he was frustrated or concerned about something, he posed the question 'what's the problem', which loosely translated into 'what's the prommo?!' It's just stuck with me over the years and I now find myself staring at Mira every day when she is screaming her head off asking 'what IS the prommo?!' It's so difficult to understand what exactly is going on in Mira's world and becomes so frustrating when you feel that there is nothing you can do for her. It's really a helpless feeling as a parent and you can't help but feel like a failure at times. Sarah and I find ourselves every day wondering what she is feeling.......is she tired, is she hungry, is she bored, does her stomach hurt, is it something neurological, is it the medication(s), or all of the above?!

Mira spends some part of her day, every day, being irritable. Some days it's a half hour; other days it's sun up to sun down. Other days, nothing makes or keeps her happy and we feel like we are always two steps behind whatever she wants or needs. Sarah and I call it LGF (Low Grade Fuss) where she will just whine, whimper, or all out cry and there is seemingly nothing we can do about it.

On a good note, my Mom came in from Saint Louis for the weekend and was able to see Mira's gymnastics moves, live in action. Mira's mobility increases a little every week. She is still popping right off of the floor into a sitting position. She will do it over and over until she is exhausted. It's great exercise for her and she seems pretty excited when she is in an upward position. Who knows - maybe she can solidify a spot on the 2016 Olympic squad soon.

All that aside, she seems to be adjusting to the Keppra fine - no increased irritability for the past few days, then again, no decrease in seizure activity. I thought I didn't see anything for the first two days, then yesterday I swore I saw a half dozen before noon. We will just keep watching and waiting.

We did add some vitamins back into her diet, since going back on additional medications. We added B12 and folic acid, added to the B6 and omega 3 she has been taking for some time.

Keppra

2008-09-21T22:52:00.004-05:00

We started Mira on a low dose of Keppra on Saturday and so far so good. She's been off and on irritable today, but I chalk it up to her getting up Saturday morning at 4:00am, crying and fussing for 2 1/2 hours. She slept a lot yesterday and today we were running around in the car all day, so she wasn't able to get her usual naps in. Haven't heard much out of her this evening - I suspect she was pretty exhausted overall.

We did get a double stroller today, which is something Sarah desperately needed, just to get around with errands and such. Lugging around Jonah and Mira (even while Eli is in school) is a challenge to say the least. The double holds up to 50 lbs. per seat, so I think we have some breathing room with Mira. It also has a decent five point harness in it to keep Mira contained and upright. We already have a Jeep double stroller, but it's more of an umbrella type and isn't very accommodating for Mira or Jonah, at this point. Eli outgrew it a while ago too.

Tomorrow, all three kids gets caught up on their vaccinations, including Mira. Wish us luck - it's going to be fun!

AFOs

2008-09-19T21:22:00.005-05:00

Mira's appointment to get fitted for her AFOs went really well today. Basically, they did a fiberglass wrap on both of her legs (one at a time of course) which is similar to getting fitted for a cast. The hardest part was actually holding Mira's legs in place while they did the wrapping and cutting. It took several minutes for the cast to firm up and Mira did some serious squirming throughout the entire process. Either she is getting really strong or I am just weak, but it took a couple pairs of hands just to keep her from forcing her way out of the mold with each leg. The therapist then cut the wraps, which will be used to form and custom fit her actual AFOs. We even got to select colors for the brace, velcro straps, and accent banding. It's just a given that they will be pink and lavender, with floral banding. Isn't everything Mira wears pink and lavender?!

The AFOs should be ready in about 3-4 weeks and we have an appointment scheduled in mid-October to pick them up and try them out. We will see if insurance is going to cover them - so far we have been extremely fortunate in that they have covered just about everything.

Quiet

2008-09-17T21:19:00.011-05:00

All has calmed down since taking Mira off of the Depakote. She really turned around after only a few days of being Depakote-free, despite feeling under the weather. Mira immediately started eating better too once she was off it - we have been getting her back on the morning oatmeal train again.

We are still seeing quite a few head drops from her everyday, which has been going on for months now. No better, no worse - they just happen. Now that she has been sitting up (and staying up for 20-30 seconds stretches) I am always nervous about her having a seizure while sitting up an falling flat on her face. The few times I have seen it, she just sort of bends forward, but still stays up. They don't appear to be strong enough to topple her over.

Mira's neuro recommended starting her on a low dose of Keppra to help with the head drops, but we are holding off until she gets over the congestion she has been battling over the past few days. After her latest bout with Depakote, we aren't too anxious to spin the medication wheel again, but will try if we think it will help.

On Friday, Mira is going to get fitted for some orthotics, also referred to as AFO's - Ankle and Foot Orthotics. Basically what AFOs are leg braces that will help Mira to maintain alignment of the bones in her feet and ankles. As she becomes more mobile, the AFOs will also help keep her from rolling her ankles and provide needed stability, should she try to stand. She won't be wearing them all the time, but will easing into them gradually for a few hours a day. We should learn more on Friday on the AFOs.

Goodbye Depakote, It's Been Nice (Not Really)

2008-09-08T21:20:00.006-05:00

I couldn't come up with anything better than to reference an old Supertramp song. Obscure, yes.

Less than 2 weeks after starting Depakote, we took Mira off it. We noticed a drastic change in her mood soon after starting it. She became increasingly irritable all of last week, which escalated to her screaming Friday night and turning purple she was getting so upset. She had a lot of trouble decompressing at night and did a lot of screaming all week. It did nothing for her seizures so we decided Friday was her last day on it. Today she is pretty much back to her old self.

We had a similar episode when she tried Felbatol in early 2007, only we took her off of that after only a few days. That was really awful for her. We haven't seen such a poor reaction to a medication since Felbatol, thankfully, but Depakote was a good contender for worst meds.

We put a call in to neurology to see what her neuro suggests. Frankly, there isn't much left that she hasn't tried, other than Neurontin (Gabapentin), Zarontin (Ethosuximide), Gabatril (Tiagabine) and Inovelon (Rufinamide), which isn't available in the U.S. Since Lyrica is about the only thing that has worked for her, it would be nice to find a medication that has a similar mechanism of action.

Back on Wheels!

2008-09-04T22:37:00.008-05:00

After much delay, we finally got Mira's Kidcart up and running again. Pushing her around in a stroller over the past few weeks has been a hassle, but we survived. The seating company rep came over this afternoon to install the cable and to give an assessment on Mira's bed situation, so we were able to get everything accomplished in one day.

Mira's crabbiness has subsided somehwat over the past few days. Perhaps it is her system getting acclimated to the Depakote and/or the fact that she is eating a little better these days.

I thought I would post some video of Mira sitting up again - this one is pretty amazing. After venting some frustration, she managed to maintain some balance once she was up for about 20 seconds! I thought she was going to a faceplant at first, but she kept right on bouncing around. Go Mira!

Mira Watched Too Many Olympic Events

2008-08-31T13:12:00.010-05:00

Mira has been moving around so much lately, that I think all of the Olympics exposure over the past few weeks has led to her attempting some of the events. As in the video in the last post, Mira has been getting up into a sitting position (for 10 seconds at a time on rare occasions) whenever we put her on the floor. This has led to her crossing her legs and quickly pushing forward, which ends up in a face plant! It's looks really painful, but it is hard to catch her in time to lessen the impact. Mira thinks it's funny most of the time. She is rolling around on a blanket, which is over a carpet, so it's not as though she is hitting the hardwoods directly, but it looks bad nonetheless. I caught it once on tape - all of her movements look like some choreographed floor exercise routine I saw in the Olympics, minus the whole landing on your face part.

We started Mira on a small dosage of Depakote last week and it doesn't seem to be agreeing with her. She has been much more irritable since starting it, waking up screaming in the middle of the night 3 out of the last 4 nights. I'm not sure what affect it has on appetite, but I'm sure it's not good, Her eating is still hit or miss, which doesn't help the nighttime screaming when you are hungry on top of it. Then again, this isn't really new behavior for her, she was pretty much the same before starting the additional meds. Only now, she seems more irritable, more often. We are going to give the Depakote a few weeks (our neuro says at least 6 weeks) and hopefully it will help with her seizures.

We are still waiting on the cable for her KidCart, which was supposed to be in on Friday. We also have someone from the company that we ordered the chair through to come out and give us an assessment on a new bed for her. Mira has been periodically kicking out the rails on her crib (which she is just too big for anyway) and is constantly hitting her head against the sides when she is moving around. We looked into this bed and are going to see if we can work something out with insurance, if not, we have several alternate plans.

Kidcart Debacle

2008-08-22T20:45:00.007-05:00

We have managed to get Mira to get at least 3 or 4 meals over the past few days, which is better than nothing. She is still only eating less than half of the time you offer her something. The nighttime screaming has subsided a little too - she only woke up screaming once in the past three days, which happened to be last night.

The biggest hurdle we have had of late is Mira's Kidcart debacle. Her Kidcart is basically a three part system - a chair (which is the actual seat she sits in) a high-low base (for getting her around the house) and a stroller base, for getting her around outside. There is an intricate cable system that broke on the chair that enables the seat to lock into the high-low base, as well as her stroller base. It is critical that have the chair working so that we can just get her around without having to carry her everywhere. The cable started to unravel and fall apart several weeks ago. We were proactive, calling the company that services her chair and placed the order for a new cable. As it turns out, the cable broke completely yesterday morning, with the chair locked into her high-low base. Good and bad. Good news is that it locked in the upright position. Bad news is that you can't get her around in the high-low base, other than in the house.

So we called the company to find out the status on the cable and as luck would have it, they hadn't even ordered it yet, citing it was pending insurance approval. Sarah explained our dilemma so they are going to expedite our order. By 'expediting' they mean you still won't get it until sometime next week. Even if we were somehow able to get a hold of a cable, we still need someone to install it. Basically we are stuck trying to get Mira around in a stroller (which offer no support) and lugging her locked chair/high-low base combo, which is clumsy and awkward to get in and out of the car, not to mention weighs 40 lbs.

The Good, The Bad, and the Screaming

2008-08-17T21:05:00.006-05:00

It's always good to start off on a positive note, so I wanted to tell everyone how well Mira has been doing at sitting up. Sarah and I have seen her up for 10-15 seconds at a stretch sometimes! She has learned to get into a sitting position in a couple of different ways. The first is the 'super abdominal crunch', where she will thrust herself upward leaning forward (as if doing a sit up) which is a very effective method for her - Mira now boasts some serious ab muscles from doing this. Her second way is to push up off of her side with her arms, although doesn't seem to work as well for her. She ends up spinning around and gets her arms trapped underneath her. She seems to be working on a third method, which I caught her doing in her crib last week. While laying on her stomach, she will tuck her knees inward toward her chest, then cross her legs and lean backward. I was astonished when I saw her do it, partly because it looked so painful, but mainly because I have never seen her bring her legs together (in unison so to speak) in a purposeful manner. I did manage to capture some of her sitting gymnastics on video:

And now for the bad. Mira pretty much stopped eating 4 days ago. Her hunger strikes have increased in frequency and intensity over the past few months, but this latest one is a whopper. Sarah and I cannot get her to eat anything these days. She will only drink from a bottle. I don't know whether it's regression, stubbornness or my poor cooking, but she flat out will not eat anything. It is incredibly frustrating. We have tried every tactic outside of force-feeding, but have been unsuccessful for 4 days now. We usually manage to get a few bites (at best) and then the snubbing begins. We just wait another meal or another day until she decides she wants a solid meal.

The third and final issues these days is the screaming. I have no doubt that Mira's eating woes go hand in hand with her sleeping problems. Over the past few nights, she has woken up screaming and inconsolable at various times throughout the night. The first 2 nights were at 2am, then 3-4am the following night, then for several hours last night just before midnight. She has had a really difficult time decompressing before she goes to bed which is not uncommon, but lately has been waking up screaming her lungs for out until she is exhausted. We haven't seen her do this consistently (as in multiple nights in a row) in quite some time. We didn't hear a peep out of her after midnight last night, so a 6 hour stretch scream-free is a good sign of things to come we hope.

On a side note - Eli is really excited about starting kindergarten tomorrow. I can't believe he is already starting school! Jonah is also doing very well - Sarah and I got some great 'cooing' and smiles out of him last night - too bad it was at 1:30am!

GABA Land

2008-08-07T22:44:00.000-05:00

We have been doing the GABA for a week now and haven't noticed any difference, but nothing bad either. Some irritability - same amount of seizures. Mira seems to have a lot of energy these days and gotten back on the food train, well at least for the past two days. I think she makes up for lost time by inhaling some of her meals. For dinner, I couldn't feed her fast enough. She ate a sweet potato in about 2 minutes flat, then took 2 bottles of milk and without batting an eye. We have just been pouring the GABA capsules in her milk and she is taking them fine. They basically have no flavor.We have also gotten her back on her protein powder shakes. Since her calorie and protein intake can vary greatly (when she won't eat) we have been doing the shake mixture in the morning.

Mira has been sitting up a lot this week, while not uncommon, she has been doing it for longer stretches. Sarah told me that yesterday Mira propped her self up and stayed up for about 15 seconds! I took some video of her doing some good 5 second stretches, which is great. She is starting to gain some balance when she swings upward and almost gets into a sitting position. The coordination is there - she just needs to use those arms as props!

Back at Home

2008-07-29T21:26:00.005-05:00

After a wonderful trip to Saint Louis toward the end of last week for my Dad's surprise 70th birthday party, we are getting settled back in at home. I took Mira and Eli with me for the trip, while Sarah stayed at home with Jonah. Things could have gone a lot smoother at the party, but it was fun nonetheless. Mira never sleeps well when we travel, frankly because she usually spends most of the time either in her Kidcart or in the van while driving, so she is easily bored. She didn't get to sleep until 10:30pm on Thursday night, which threw her patterns off significantly. On top of that, she sleeps in the Pack + Play, which she is almost too big for. I'm not sure what to use for future trips. At one point I woke up to the sound of her kicking her feet out so hard against the side of it, that she was rocking the whole thing across the room at my brother's house.

The three of us returned Saturday afternoon and Mira was pretty crabby all day, screaming and crying the last 50 miles home. Her irritability continued into Sunday, when I think she finally was able to catch up on some sleep Sunday night. As I said before, her sleeping is usually pretty poor on trips, which tends to affect her eating as well. The combination of poor sleep and her hunger strikes makes for a rough time for her.

We have decided to try a trial of GABA supplements with Mira before we dive straight into the Depakote, again. Mira tried Depakene (same medication, different form - liquid versus capsules) back in November of 2005 and it didn't do anything, so we are not in a huge hurry to try it again. GABA is a simple amino acid that is used for anxiety and seizures, although its efficacy is questionable and unproven (as are many medications mind you), since supplemental GABA doesn't cross the BBB (blood-brain barrier). GABA itself acts as a neurotransmitter in the central nervous system and is suppose to regulate neuron activity. We were going to try GABA supplements last year, but side tracked with medications before we tried it long-term. GABA is pretty safe and harmless, so a trial of it for a few weeks is worth a shot.

Update

2008-07-21T22:18:00.022-05:00

Sorry to have been so long between posts - it's that whole brand new baby thing where you are in constant sleep deprivation mode and are trying to remember what day it is. Things are getting better and we all seem to be getting into some sort of groove - a rather dysfunctional one at times, but a groove nonetheless.

I figured the easiest way to give an update is to pick up where I left off, which was right after Mira's birthday. We actually had a larger birthday party for Mira in the park, a few days after her actual birthday. Turned out to be a beautiful day and Mira managed to take some nibbles of of a cupcake. Thanks to everyone for all of the nice gifts, cards, flowers, and balloons. I know Mira really enjoyed having everyone there.

The day after (or the day before I don't remember - again the baby thing as my default) we went to a 4th of July celebration for the city (called Villagefest) where it turned out to be another perfect day out weather wise. Who expected back to back days in the beginning of July at 82 degrees?! I just remember sweating bullets last year this same time at Villagefest and it being nearly 100. Anyway, Eli had a blast that day for two reasons. First, he was able to indulge in one of his new found interests, which is anything and everything to do with the military, particularly guns. The police department has a display of assault rifles, handguns, masks, and other weaponry for kids of all ages to enjoy, I guess.

The annual Village fest 4th of July celebration wouldn't be complete for Eli though, without running into his old pal Sluggerrr. Eli somehow new that this bitter rivalry would come to a head that day, so he opted to wear his Cardinal's shirt, just to taunt Sluggerrr if he saw him. The showdown finally took place about high noon, where Eli caught him out of the corner of his eye. He first threatened to kick him, but Sluggerrr stood tall, picked Eli up and gave him a big bear hug. Eli immediately took off running after Sluggerrr got the best of him. Final score: Sluggerrr 2, Eli 1.

The following week we headed to the air show in nearby Gardner, Kansas, where Jonah got his first taste of real summer heat. We didn't last long and tried to stay in the shade (which often meant under the shadow of the Chinook helicopter propeller blades) but survived. Again, Eli whose love these days of weaponry, old cars, and military vehicles knows no boundaries, exclaimed "this is my luckiest day of war" as we passed through the gate. He saw an old 1932 Roadster, a gigantic Chinook helicopter, and a plane armed with guns, all in the first 30 seconds.

Last week was fairly uneventful, although we were dealing with one of Mira's hunger strikes most of the week. She went a couple days and we realized that she was probably constipated. At the same time, she was battling more phlegm issues off and on for a few days during the week. I woke up one morning, started preparing a bottle for her and heard her making some funny sounds. Sarah and I both ran in to find her vomiting everywhere, probably due to the mucus and being so upset. It's funny to know your child's noises and mannerisms so well that you can immediately sense when something is not right. Mira makes a lot of noises and sounds throughout the day, but we know when the sound she makes is not right. Just tonight she let out this weird cry, again we both rushed into her room to find her with her binky caught in her mouth. Time to be done with the binky at night. Most of the time when we hear a strange cry and she has her leg or arm wedged between the rails in her crib. Time to modify her bed.

Now that she is more active, we are finding it really difficult to find places for her to roll around on the floor. Our house is only so big (yes - a whopping 900 square feet) and she rolls into everything. She often gets stuck in weird positions - between the couch and chair, getting tangled in her blanket, etc.

So as it goes, we were getting into a little bit of a rhythm this past week, until we had Mira's EEG last Thursday. The expectation for an EEG is that you keep her up as late as possible (Children's Mercy says to keep her up until midnight, which is impossible, we rarely get to 10:00pm) and then wake her up at 4:00am (again, impossible if we can get her up and keep her up earlier than 5:00am) so that she is sleep deprived (a scenario we are all experiencing at the moment) for the EEG itself and will fall asleep for the beginning of it. This EEG, being her 12th or so, we were actually able to keep to that schedule fairly well and Mira turned out to be a real trooper for the whole thing. I ended up staying with her at CMH until our neurologist appointment afterwards, while Sarah stayed home with the baby. The EEG was scheduled for 7:30am, which went fine, but getting into neurology is a whole other ballgame. We were almost an hour late by the time we were sitting down with her neurologist, which seems to be the norm with that department. Great people, great doctors and nurses - just never on schedule.

Despite having a seizure free period of nearly six months, Mira's EEG hasn't changed much since last November. I don't quite understand the specifics of her EEG (ironically this is the first time someone actually tried to explain it to us) other than she is having frequent spiking and bursts, which interrupts her ability to focus and learn though we have seen considerable progress since she started on the Lyrica. Why does her EEG look like this is the million dollar question. Mira did manage to have one head drop toward the end of the EEG, which they caught on tape. Unfortunately, it doesn't offer any insight as to why - it's just that she has the potential for seizures all the time, but we are only seeing a fraction of them on the surface.

We discussed genetics again and believe we have exhausted all of the testing at this point in time. There is one final test to perform for now, which is a mosaicism of idic15, which would completely rule out any sort of idic15-related scenario for her. A mosaicism of idic15, as opposed to the 'typical' inverted or duplication of chromosome 15 is extremely unlikely, occurring in about 1:100,000 births. Children with a mosaicism (which is merely a mutation, inversion, or duplication occurring in a small percentage of cells as opposed to every cell in the body) are usually much more high functioning than kids with the 'typical' version of the genetic disorder. Our neuro thinks it would be very unlikely, nonetheless, he thinks it's a good idea to finish out the testing. We basically are banking her DNA for future testing and should something come up that they believe she should be tested for in the future but for now, we are done.

As far as seizures are going, Mira is doing about the same. We are still seeing several dozen head drops a day, but nothing more major than those small myoclonics. Our neuro is very hesitant to go any higher on the Lyrica, based on how little is known about this kind of use of the drug. Possibly, Lyrica has lost some of its efficacy, which is not uncommon with AEDs. Our plan is a conservative one regarding her meds. For now we will drop Mira back down to 50mg daily of lyrica and add a small dosage of Depakote in about two weeks. Depakote is in the same family of GABA-related AEDs as Lyrica, so the theory is that it will work in conjunction with the Lyrica to help combat the head drops. It's all trial and error with the medications and it will always continue to be at this point. We will only try the Depakote for 6 weeks unless she is not tolerating it well or it stops her seizures.

Despite the seizures and the occasional hunger strikes, Mira is doing very well overall. Thanks to everyone for your continued support, gifts, and prayers.

Mira Turns 3

2008-06-29T23:01:00.010-05:00

On Saturday, Mira turned three years old. I couldn't help from having a small pity party for her as I was putting her to bed for the night. I thought about what Mira and our family have gone through over the past three years and what the future holds for her. It has been an emotional roller coaster to say the least, one that I would hope and pray that no one ever has to endure. What really made me most sad is that Mira didn't even realize that it is her birthday, which is a time of great happiness for most kids - getting presents, blowing out candles, eating lots of your favorite cake, and running off a sugar high for the next three hours. I can't help sometimes to ponder various scenarios with Mira, wondering what life would be like if she hadn't been so affected by all of this and how different things would be if she just were able to be aware of people and engage with her surroundings. I don't know how to explain it. It's watching other three year old kids running around in the park and desperately wishing the same will happen for your child. It's a constant craving as a parent to want to have a connection with your child somehow. It's a hope that your child recognizes you in some fashion, whether through a smile, laugh, or a simple visual gesture. It's just wanting your child to be happy and constantly questioning whether or not they truly are.

Settling In

2008-06-26T21:44:00.004-05:00

It's been a few days since we left the hospital and we are finally getting settled in. Eli has been back at school for 2 days a week and Mira is back at therapy. I was able to take her to PT and OT sessions yesterday and she had vision in-house today. Her week wraps up with feeding and group therapy tomorrow morning, which I also get to attend. Sarah gets a 'break' from Mira's therapies (which translates to taking care of Jonah at home) for the week while I am off work.

Eli is adjusting to the baby very well. He seemed pretty freaked out when Jonah was screeching at the top of his lungs the first day back from the hospital, but has gotten used to it by now. Mira cackles every so often when Jonah is crying, since his cry is reminiscent of a mix between a high-pitched baby pterodactyl and a squeak toy. It's distinct to say the least.

Mira started one of her hunger strikes over the weekend and she finally caved at dinner this evening. I was able to get her to finish off a small bowl of sweet potatoes, which is the first 'full' meal she has had in days. Nothing seems to break her cycle of not wanting to eat - we try and switch her meals up and offer up new things, but nothing makes a difference. She just decides that she wants to start eating again and we are forced to wait it out.

Since we have been seeing an increase in her head drops and jerks, we decided to move her dosage back up to 75mg daily. It hasn't been a dramatic increase, but a slow and steady one over the past few months in the frequency and intensity of her spasms/episodes. Sitting through her 1 1/2 hour therapy session yesterday, I saw at least a half dozen. I spoke to neurology yesterday and we agreed to move her dosage up over the next few days. We may just hold steady at 75mg until our appointment in the middle of July and see where she is at then. Still, no full blown tonic-clonics or anything, which is good.

Pictures

2008-06-24T13:14:00.011-05:00

Some quick pictures from Jonah's arrival this weekend More to come!

New Arrival

2008-06-22T20:44:00.003-05:00

Jonah Frederick was born early this morning, weighing in at 8 pounds, 7 ounces. Mommy and baby are both doing great. We hope to be out of the hospital tomorrow. I will post pics and give a longer update when we get settled in at home.

Mira has been really fussy the past 2 or 3 days, going on one of her hunger strikes and getting up in the middle of the night again for hours. Poor Auntie Aura stayed up with her last night while we were in the hospital. It might take a while to get back into a rhythm once the new baby is home, so wish us luck!

Lyrica Trials

2008-06-13T23:37:00.002-05:00

I spoke with neurology about Mira's ongoing head-drops and what look like myoclonic seizures. We basically haven't really seen any improvement in the frequency or intensity of Mira's head drops, so we decided to actually decrease Mira's dosage back down to 25mg 2xs daily. We are going to see if things get worse and if so, we will increase it again, most likely above and beyond her current dosage, which is 25mg 3xs daily. All of this is on the direction of our neurologist of course.

Our neurologist (and the neurology clinic in general) doesn't have the experience with kids taking Lyrica, since it is so new to the market. We agreed that it has really been the miracle medication for Mira and we are not about to abandon it yet. This sort of 'reverse' trial dosage is to really see if Mira is really reacting to the Lyrica or to see if we need to look at alternatives or adjunct therapies. I hope that it is just that we need to do some tweaking with her dosage and the aim is to not have her so over-medicated that she isn't able to do anything. There's some action or mechanism at the cellular level (I guess) with Lyrica that has been helping Mira, which could be a clue as to why she is having seizures. If she reacts to lowering the dosage, we will know for sure.

We are also going to push for another EEG, since Mira hasn't had one since last November and all of this new seizure activity would warrant another.

Updates + Microarray

2008-06-10T23:18:00.003-05:00

Well, first things first. I spoke to neurology yesterday and Mira's microarray came back normal. This was probably the last of the genetic screens that we will be doing, unless her neurologist has something specific in mind for additional testing. We have pretty much exhausted all of the reasonable screens and anything that we would be testing beyond this point would be a stab in the dark.

We have still been seeing a fair amount of head drops with Mira, so there is concern that the dosage increase hasn't done much yet. We are going to give it another week at the higher dosage and see what happens. If things don't improve, we will either take her back down or perhaps try something different. Mira's temperament has improved a little over the past few days, so there is hope that the increase is potentially helping in other ways.

Mira had her first haircut today! Her hair was getting seriously unruly and Sarah seemed to spend half her day getting knots and rats out of her hair, which Mira really detests. She did great, all things considered. We sat her on my lap while the stylist did some quick work, taking about 4 inches off the back. It took Sarah and I together, with all hands on Mira, just to keep her from flinging and thrusting backwards. Mira is getting very strong and has developed 'abs of steel' from squirming and arching so much.

I posted a few videos on Mira's site (see link to Youtube.com at the right). One is of Mira's new swing that we installed on the trellis in the back yard, which she loves. Sarah managed to find a swing for her that puts her in more of a reclined position and it works great. The other video is of Mira's new found love - hearing the trumpets from Sarah Vaughn's 'Whatever Lola Wants'. Yes - completely random how we discovered it. We had the iPod on one day and Mira was over in her chair just chuckling away, but only at certain parts in the song. We discovered it was the trumpets that really cracked her up.

Last but not least, the countdown continues for the baby. Sarah had some contractions over the weekend, but nothing after that. Still waiting!

Doing Better

2008-06-05T21:02:00.003-05:00

Mira has had a slightly better few days, but has still been fairly crabby. We managed to get a few chuckles out of her for dinner, despite her ongoing hunger strike - perhaps she thinks it's funny to not eat?! She managed to gag on her meds and vitamins this morning, ending up with a lap full of milk and a warm bath afterwards. (She laughed during this episode too.) She has been tolerating the increased dosage well so far and we haven't seen any side affects as of yet.

Crabby, Crabby, Crabby

2008-06-03T19:02:00.003-05:00

After having a great week last week, Mira has been really, really crabby over the past week. She is fussing a lot and we cannot figure out why. She has been taking power naps in the middle of the afternoon and when she isn't sleeping, she is fussing. We have been seeing about the same amount of head drops as the week before, so perhaps it's something else that is bothering her.

She also started one of her hunger strikes a few days ago, where she won't eat anything and it is a struggle just to get her meds in her. She is getting really good at resisting pills, spoons, and is partial only to one thing - her bottle. And yes, the bottle MUST be warm or she will snub it before you can say 'fussbot'.

Speaking of meds, we decided to up her dosage today, since she is completely snot-free and still having some sort of seizure activity. We are hoping that bumping her up will help get rid of the head drops, but we have been down this path many times before. For 6 months, Lyrica was the miracle medication for her and we hope that it continues to be. It will take a few days for her to feel the increase, so we will watch her closely.

Have to go - the fussing continues.......

Pictures

2008-05-23T23:31:00.013-05:00

Mira has been having a great week - lots of smiling, giggling, and being in a great mood all around. The weather has been great, so we have been able to spend a lot of time at the park and have the windows open at night (with the attic fan on it seems to help Mira sleep better) which is a bonus. Mira has been able to make it to hippotherapy for three weeks in a row with out getting rained out! She has been doing really well on the horse, yet still being very particular about facing forward.

Mira's skin has cleared up dramatically since we started giving her acidophilus every day. I don't know what it is about probiotics, but if we stop giving it to her, the rash and irritation comes back the next day. You have to love the friendly bacteria. This rash on her face has been around for a long, long time and it worsens when she is rolling around on the floor.

Speaking of rolling, we have been spreading a quilt out on the floor for her every day and she loves having roll time. The trouble is, she is getting increasingly quick and will end up on the hardwood floor in no time. Sarah also ordered a swing online that we are going to start using, in an effort to get her excited about moving around. She is constantly moving and squirming and we want her to be able to be as active as possible out of her chair and not be on the floor so much. We occasionally put her in the swing at the park, but she can only lean forward and it's a lot of work for her to keep her head up and forward in those swings. This swing is in more of a reclined position so hopefully it will work better.

Other than that, not much else happening here. Still seeing some head drops and other quirks, but nothing major with Mira. Sarah is really exhausted these days, hitting the last month before the baby gets here!

Thumper

2008-05-13T21:30:00.006-05:00

I spoke with Mira's neurologist (or at least his nurse) about raising Mira's dosage and we agreed to wait it out another week to see if Mira can get all of the congestion out of her system. It's been lingering around for a few weeks now and she still has loads of snot - I don't know of a delicate way to put it. We stocked up on Kleenex at Costco last week and have since gone through half of it!

Sarah and I have been seeing a lot of head drops lately, maybe up to a half dozen or so a day that we catch. I saw a couple this morning and one when Eli and I were out in the park with Mira. Despite this, Mira is still in a great mood, eating well, and drinking well - all of the things she should be doing.

Mira has taken on the name 'Thumper' since one of her favorite things to do these days is kick. If you hold her in your arms, with her facing away from you, she kicks and 'thumps' like a miniature tornado - arms and legs going everywhere. She is also constantly arching her back, which makes it nearly impossible to sit her on your lap, let alone try and hold her. She seems to be in constant squirm mode.

Changing her diaper has become a real challenge, since all she wants to do is throw her legs up in the air (usually at you) all while twisting, turning, squirming, and generally trying to kick her way out position. Don't get me wrong - it's fantastic to see her moving so much and exerting an incredible amount of energy, but it is a total 5 minute workout for Sarah or I just to get her changed.

This Week

2008-05-07T21:19:00.002-05:00

Mira has been doing great this week. She was cranky during PT/OT, but did well. She is finally getting a lot of the phlegm out of her system and has turned a corner with her sleeping. Her sleep patterns are much, much better when she is feeling well. She slept through the night last night for the first time in weeks!

We are crossing our fingers that we don't get rained out for hippotherapy tomorrow, but we have been getting rain off and on today and more is forecast for tomorrow.

We moved forward with the aCGH (genetic micro-array) testing on Monday. Children's Mercy is sending it through a private genetic company for testing, so as it was with the other genetic screens, it will be back in about 6-8 weeks.

I had the pleasure of sitting through a 4 hour city planning commission meeting last night, for yet another cell tower proposal. Cingular threatened back in 2005 to put a cell tower about 50 feet from our house, in the middle of a park no less. After an exhausting fight, they were denied a special use permit and eventually the issue went away in 2006. Now, T-Mobile wants to put a massive 120' monopole about three blocks from us, which we could still see from our back yard. Again, the application was denied. Fortunately, we have really proactive neighbors (some of which are attorneys, conveniently) who are always willing to fight such proposals.

6 Months Seizure Free*

2008-05-03T22:45:00.002-05:00

Yesterday officially marks Mira being seizure free for 6 months, but I say that with an asterisk. We have been seeing what we think are small seizures or at least something over the past few weeks. Mira has been battling this cold off and on for a while, so we are chalking it up to her being sick (which lowers one's seizure threshold) or considering the possibility that we need to increase her meds. I contacted her neurologist about it and we are taking a wait and see approach for now, at least until she gets over the cold and phlegm out of her system.

As I was getting her ready for bed tonight, she had a small head drop and her eyes fluttered very briefly. All of it lasts less than a second. I know it was something, because I have seen it thousands of times before - I was just hoping they would never return. Anyway, Sarah and I have noticed these small twitches/episodes every so often over the past week or so, but we aren't getting too worried at this point. If it happens, it will happen and we have a plan of attack.

Sarah is enjoying a very brief girls' weekend in Saint Louis with her sister and Auntie Tisha. Daddy gets to stay at home in KC and do laundry!

Hippotherapy - Finally!

2008-05-01T19:47:00.003-05:00

After waiting for 6 months for an opening and getting rained out for 5 weeks in a row, Mira finally had her first full hippotherapy session this afternoon. She did wonderful, even grabbing onto the ring saddle on the horse a few times. Her therapist said that the grabbing was unprompted and she did it on her own. Mira definitely didn't care for riding backwards, crying every time they tried to turn her around. Her sessions were supposed to start in the first week in April, but were rained out every single Thursday until today. The only bad news about the hippo is that it's a 45 minute drive to Baldwin City from our house, but well worth the drive and it's only once a week.

I was able to take the day off (Sarah still isn't feeling well) and actually saw what Mira goes through on her heavier therapy days. This morning she had PT in house, then vision therapy in-house after lunch, then hippo in the late afternoon.

SMEI Results

2008-04-29T21:57:00.003-05:00

I received confirmation this morning on Mira's SMEI/Dravet's testing and it came back normal. This genetic test was one of the more exhaustive (and expensive at $6,200) genetic tests out there. The lab was looking at a very specific deletion/mutation of a very specific gene called SCN1A. She has pretty much gone through all of the major testing - Angleman's, Rett's, and now Dravet's.

At this point, we are probably going to do one more additional screen, called an Oligo Array CGH (Cognitive Genomic Hybridization). Basically, Mira had some similar genetic screens back in 2005, soon after her seizures started, but it was at a very low resolution, meaning it only picked up major genetic organization. The technology for genetic screening has advanced so much over the past 2-3 years, that they can now perform a much more exhaustive scan at a much lower cost. This scan (neurology refers to it as a 'micro-array') is an extremely high resolution scan that is able to identify many more deletions/mutations in DNA than ever. Before this can happen, we need to clear it with insurance, since it costs about $4,000.

Mira has been battling a cold over the past few days, but is doing alright. Lots of snot. Need I say more? Sarah has been dealing with the same thing now for two weeks. I think she is exhausted from it. I am hoping that Spring is just around the corner........

A Long Hiatus

2008-04-27T23:41:00.005-05:00

Sorry it has been such a long time since my last post. We have been extremely busy over the past month, so without going into too much detail, I will give you the Cliff notes of what has happened with the house:

............Started searching for a new house. Looked at many, many houses and drove many, many neighborhoods. Fixed supposed cracked foundation to stop leaky basement to prepare our house. Put our the house on the market. Had almost 20 showing in 10 days with no offers on our house. Could not find a house worth putting an offer on in the neighborhoods we liked. Took our house off the market after 10 days and decided not to move. Basement flooded 3 times over the past two weeks since house has been off market. Still have a leaky house. Still have packed boxes in friend's garage and in storage...........

Sarah and I are pretty exhausted from this whole experience, but it has been a very eye-opening one at the same time. We are quite content with staying put and with the market as soft as it is currently, we felt like it was the best decision for us right now. We will deal with the bedroom situation as it comes. We figure we might be singing a different tune in 6 months (the baby is due in 8 weeks!) and might consider making another go at moving. But for now, it's nice not to have so many irons in the fire and it's just one less thing to worry about.

In addition to the house, the winter sickness and colds have been working their way around the house for seemingly forever. Sarah has been battling a cold and some congestion for over a week and it doesn't seem to be going away. It's getting passed around, with Mira and myself getting touches of it over the weekend. The weather isn't helping either - 65 one day then 30 the next.

As for Mira, she has been doing well too. I am happy to report that she is still seizure free, although she has been having what I swear I thought were head drops at one point. They are very subtle and you wouldn't know she was doing it, unless you have seen it as many times as Sarah and I have. She had a few (at least they looked like really quick ones - not completely sure) on Wednesday and then one today. I emailed her neuro and asked and his recommendation was just to keep a close eye on her and we could always up her Lyrica dosage. She has been on it for nearly 6 months and has not had to increase yet, so it isn't unreasonable to think that based on her growth and weight, that she might need to increase the dosage.

Her sleeping has not been so great as of late. She has been getting up at all hours of the night, usually crying out. Most of the time, she settles down after a few minutes, but other times she really gets worked up. It has also been difficult for her to 'decompress' at the end of the day and she will fuss in her crib for an hour off and on before she finally gets to sleep. None of this is out of the ordinary for her - she has had erratic sleep patterns her whole life. It comes and goes.

We don't have anything lined up on the books with neurology, since nothing has changed much over the past few months. We will probably get Mira in for a visit sometime over the summer, unless something changes drastically before then. We haven't heard anything back on the Dravet's testing, which was ordered at the end February. Apparently, it's not uncommon for the results to take 2 months or more. We will keep waiting.

I did manage to upload several videos (see 'Other Mira videos' link on the right side of the page) of Mira doing some funny things. She really gets wound up, often for no apparent reason - one episode I was able to capture on tape back in January. We also found that she gets particularly excited over the food processor and the dust buster. Not sure what it is about certain appliances and noises, but it cracks her up.

Hope everyone is doing well and apologies for not getting our ducks in a row to do the Trolley Run this year. There was so much on our plate over the past few weeks that we just couldn't get organized to do it. The actual run was today.

Pot Roast + Spring Cleaning

2008-03-10T22:19:00.004-05:00

Mira has been doing very well over the past week or so, with the exception of yesterday when she basically cried off an on for 4 hours - most of the afternoon. Sarah and I jokingly chalked it up to daylight savings time - it must have thrown her whole 'chi' off. She was pretty irritable all day, since every time she wanted to doze off, it was time to go somewhere, so she never had a chance to decompress. Today was better for her, although she still had trouble getting to sleep.

On the flip side, we discovered that one of Mira's new found friends is pot roast. Sarah found an excellent recipe for a roast and creamy potatoes and Mira cannot seem to get enough of it. I think she would eat is for three meals a day if we let her. She will snub yogurt all day long, but put a spoonful of pureed roast in front of her and she snaps right out of it and chomps it down. Needless to say, looks like we will be cooking it more often!

As of March 2nd, Mira has been seizure free for 4 months solid! She periodically rolls her eyes upwards, sometimes with a 'fluttering' motion, but it doesn't appear to be seizure related. It looks like it is more self-stimulating than anything. She is constantly squirming, so trying to hold her is no longer an option - her movements (back-arching and throwing herself backwards) make it increasingly difficult to carry her and get her in and out of the car sometimes. In her crib, she is often thrashing from side to side, flinging her legs up in the air, and is still putting her hands in her mouth, all the time. Most of this behavior surfaced after she became seizure free, so to us, it's a sign that she is making some progress.

We are doing some spring cleaning around the house, donating clothes and books, shredding old papers and getting the house in order to get on the market in about 2 weeks. In the meantime, we are narrowing our house search to a particular area. This after driving neighborhoods and checking out schools over the past month.

Quiet Times

2008-02-28T18:01:00.003-06:00

Not much has been happening over the past few weeks, thus the break in posts. Mira is still seizure free, going on almost 4 months! We did have Mira's neurology appointment on Monday, which lasted about 2 1/2 hours. Her final results on the Rett's testing, for the typical MECP2 type, came back negative. After much discussion, we decided to do some further genetic testing, for Dravet's Syndrome. The decision was based on Mira's poor reaction to Lamictal and Diamox last year, both of which act in a specific way in the body, through sodium channels. Her adverse reaction to particular medications is sometimes a clue.

Dravet's, also referred to SMEI (Severe Myoclonic Epilepsy in Infancy) is usually attributed to a specific gene, referred to as SCN1A (and also SCN2A). Mira will be tested for this, but as with all of the other genetic scenarios that she has been tested for, it is unlikely that she has it.

Weekend Events

2008-02-11T21:37:00.000-06:00

Aura, Addy, + Truman came into town for Eli's 'Batman Bowling Party Extravagganza Weekend'. (Eli turns 5 on Wednesday!) The festivities continued into Sunday when I took the boys to Monster Jam at the recently completed Sprint Center. Having never attended any sort of racing, car, truck, or motorcycle show, it was an eye-opening (and ear-ringing) experience to say the least. Eli and Addy were reved up for most of the show, but after 90 minutes of trucks, dirt, and noise, they were both pretty much spent. I'm not sure how anyone could possibly fall asleep during Monster Jam, but Addy managed to.

All things considered, it has been a quiet week for Mira. I plan on talking to neurology toward the end of the week so that we can make sure we have the remaining Rett's results for her appointment at the end of the month.

Three Months Seizure Free

2008-02-04T22:14:00.000-06:00

Saturday marked three full months of Mira being seizure free! She continues to do well, although her eating is still not up to par and hasn't been for quite some time. She tends to eat in waves, scarfing down yogurt and eating full meals for a few days, then won't eat a bite for a the next two days. We can't quite figure it out why she does this, but she is still getting plenty of fluids every day. Despite her mealtime issues, she is doing very well these days and continues to be very vocal and animated.

It was in the mid-60's this afternoon so everyone (well, almost everyone) was able to get out and enjoy the weather here. Sarah took the kids to the park and Eli was able to burn off some energy. Poor daddy was stuck inside all day and had to sit through boring meetings and talk to other nerd architects about exhibition hall rigging capacities imposed on structural mainframes.

A Much Better Night (and Monday)

2008-01-28T20:55:00.001-06:00

Mira rebounded Saturday night and slept from 7:00pm to 7:00am, only getting up briefly, once. I think she was making up for lost sleep. I think she might also be on the rebound with her eating habits as well. Up until this morning at therapy, she still wasn't eating much. She managed to down an entire yogurt with her feeding therapist, which is probably more than she has consumed in the last 4 days, combined.

We also managed to get an appointment (most likely due to a cancellation) with CMH neurology, toward the end of February, so we are on the books for Mira's next appointment.

Rough Night

2008-01-26T09:17:00.000-06:00

Mira tends to go through phases of not sleeping well and last night was 'one of those nights'. We usually chalk her poor sleeping up to several factors, including how much she sleeps during the day (little lately) or how she has been eating (very poorly lately) and/or how active she was during the day (not so much yesterday).

So, I put her down about 7:30pm last night and she slept soundly until about 11:30pm. After thrashing around and vocalizing (still very loudly and Eli somehow doesn't wake up in the room right next to her) in her crib until about 1:30am, she fell back asleep, woke up again at 3:30am, screaming. I then gave her a bottle and she stayed up until 5:30am, dozed off until 6:45am, then got up again for the day.

Mira also goes through bouts of not eating, which she is on day four of not eating much of anything. A few bites here and there to get her meds down, but that's where it ends. She plugs back a ton of liquids every day, but is snubbing everything we offer her these days.

We all have cabin fever lately, since we have been braving the 0-20 degree temperatures here for two or three solid weeks now. Sarah left for Chicago yesterday, for a much needed girls' weekend out and I returned from a Nashville day trip late Thursday night, to a balmy 7 degrees here in Kansas City. Unfortunately, it is just as cold in Chicago and Nashville as it is here - so much for a change of pace. It is supposed to be in the 50's today, so hopefully we can get outside!

No news on the Rett's testing yet - my weekly harassing phone calls to neurology seem to offer no new insight, other than 'genetics is still working on it'. I will try again next week I suppose. The neurology clinic at CMH is going through some rough transition - neurologists leaving and new ones coming in, which means shifting patients and often, frustrated parents. It is extremely difficult to even get an appointment set up now. The books are supposed to open up next week, so that we can actually set an appointment, which we are told is another 2-3 months out after the schedule is posted.

This Week

2008-01-22T22:32:00.000-06:00

Mira has been doing very well over the past week. She continues to vocalize a lot throughout the day, nearly shattering windows at times. It is seriously ear piercing. She is also trying to move around quite a bit - she seems to be in a state of perpetual motion, especially when she is in her crib or on the bed. It keeps diaper changing times fun and exciting. She will twist on her side as she is rolling around and it seems like she is trying to lift her head up, but it is so much work for her.

Mira is also starting to grab at toys. Tonight, she reached several times for her Abby Cadaby toy (from Sesame Street) which have bells tied around her waist to make noise. She has been responding to Abby consistently and she gets excited every time she hears the bells. Her movements most of the time seem uncoordinated and not very deliberate, but you can tell that she is really trying to do things. Over the past few weeks though, her movements are becoming increasingly purposeful. Sarah said that Mira has been pulling her bows out of her hair throughout the day too. She is making a little progress every day by doing new things.

Baptisms + Decibel Ranges

2008-01-14T20:52:00.000-06:00

We had Mira and Elias baptized yesterday at St. Michael's. It was something we had been planning to do for some time. We wanted to thank everyone so much for being there and for all of the wonderful gifts for the kids. A special thanks to Tom, Nikki, Sophia, and Siena for making the trip in from Illinois. I will post pictures soon.

Mira had a pretty rough night last night - who could blame her after such an eventful day. She was up most of the night, kicking and vocalizing. The interesting thing is, she has become REALLY vocal over the past two days. Yesterday evening, she seemed to strike a chord with everyone in the house - a really LOUD chord. She has been moving and squirming around a lot lately (which is progress!) and now has started vocalizing a ton, to such an extent I thought she was going to shatter some wine glasses tonight. I shot some video last night of her doing some of it.

Two Months Seizure Free!

2008-01-04T20:58:00.001-06:00

As of January 2nd (Wednesday) Mira has been seizure free for two solid months! Lyrica has turned out to be the miracle medication for her. She is now only on a single medication and has weaned off of the diet for the most part. She is able to now enjoy applesauce, pudding (with extreme caution - see yesterday's post) and she actually had some of my Mom's famous spaghetti sauce yesterday, which we had on reserve in the freezer. (Hint, hint, Mom - we need to restock.......). We have been helping Mira get reacquainted with oatmeal and juice as well, in moderation. She continues to do so well and we are all so proud of her!

Press Your Luck

2008-01-03T22:07:00.000-06:00

For those of you out there who remember the 'Whammy' from the game show 'Press Your Luck', this one is for you..........

Earlier this week, Mira wasn't eating very well and we were having a difficult time just trying to get her eat anything, even a few bites. She had been taking plenty of fluids and has gone through bouts of not eating before (see Thanksgiving posts) therefore, we weren't too concerned. Well, after a few days of nibbling at meals, I figured it wouldn't hurt to try and feed Mira in her KidCart, since it wasn't likely that she was going to eat anyway. The first few spoonfuls of applesauce went off without a hitch, so I graduated to vanilla pudding, figuring I was on a roll.

This is usually the point where I begin chanting 'no whammies' in hopes that Mira doesn't gag on the pudding, which is her way of saying "I'm REALLY not hungry so stop tyring to feed me." Well, the Whammy came out, meaning Mira proceeded to gag once and vomit everything she had drank in the past 48 hours, all down the sides and front of her KidCart. Her stomach holds a lot of fluid, just so you know.

Ahh, to clean the KidCart is so, so much fun. First, you strip all of the straps, belts, armrests, seat padding, neck padding, (separating the padding from the covers) and throw it all in the wash. Then, detach the seat from the stand, scrub the seat and stand down with soap and water, getting in all of the bolts, screws, and wheels. If it's too cold outside, you get to do all of this in the bathtub instead of spraying it down with the hose. Once all of the padding is scrubbed, washed, and dried, you get to reassemble the whole thing. Oh, yes, and Mira needs a bath too.

The irony is that this is certainly not the first time this has happened. It seems whenever I press my luck trying to feed her in the KidCart, I lose and end up an hour later with wrinkled hands, a bottle of 409 in my hand, crawling on all fours scrubbing the hardwoods.

The moral of this story is simple. Don't ever feed Mira in the KidCart. Ever.

Christmas Images

2007-12-30T21:52:00.000-06:00

Here are some highlights from our Christmas. Mira and Eli got to see Santa once again on Christmas Eve, getting out any last toy requests before the big day. Somehow, Santa looked a little different from the last time they saw him a few weeks ago at Mira's program, but who can tell. He brought gifts and cookies for all the kids. The week flew by too quickly, but we were able to spend lots of time with the Floras and grandma.

Anyway, the images above include the Christmas photo we sent out, Mira and Eli each with Santa on Christmas Eve, the kids at Union Station to see the trains, Eli, Addy, and Truman horsing around in the pack-n-play, and the three amigos watching TV.

Last but not least, Eli got a tiny bit of a raw deal for Christmas. Sarah frantically searched for 'old-school' G.I. Joe action figures and an army jeep, finally outbidding someone on EBay the week before Christmas. Only, the shipment didn't make it in time and Eli had to settle for a late delivery from Santa a few days later.

A Non-Stop Week

2007-12-23T19:59:00.000-06:00

This week, as all pre-Christmas onslaught weeks are, was crazy. We hit the ground running earlier in the week with Eli's Christmas program all the way to today, when we were trying to get in some last minute shopping with 25,000 other frantic people all lobbying for what is left on the shelves, which isn't much. From the grocery store to the department store - it was all crowded, as expected. We survived.

Mira has been doing extremely well over the past week, with the exception of today. She has some diaper rash that is bothering her, but other than that, she has been doing great. Her sleep has been decent over the past few nights too. She is still getting up at 4:00am, but able to put herself back to sleep soon after.

We haven't seen any negative effects from stopping the Zonegran, as of yet. It's one less medication to deal with and keep out of her system, so good riddance. Mira has also had the pleasure over the past week to get some variety in her diet, having beef stew a couple nights. She loved it! Since we are slowly weaning her off of the diet, we have been adding different vegetables into her meals, as well as offering her oatmeal and yogurt in the morning, which is hit or miss. She doesn't seem to be too keen on breakfast these days, but I think she is enjoying a change of pace with meals regardless.

And of course, the great news is that Mira continues to be seizure free. Tomorrow will be 50 days of being seizure free! We hope that everyone has a Merry Christmas and I will post pictures mid-week!

No More Zonegran

2007-12-15T21:45:00.001-06:00

After 8 months, today was Mira's last day on Zonegran. As with all medication weans, we are anticipating some potential changes over the next few weeks. You never know how she is going to react when she is ramping up or coming off a medication. We have been weaning her for two weeks, so her last dosage today was only 25mg, which is a very low dosage for her weight. It usually takes another few weeks for her system to completely flush it out.

One of the changes as of late is her sleep. She tends to get up in the middle of the night (last night it was 2am) and stays up for hours. She isn't really doing much during this time, although I know she is awake because I can hear her babbling through the monitor or just kicking around in her crib. She usually does this for an hour or more, then starts to get really tired and irritable for a few minutes. I end up giving her a bottle and she goes back to sleep, but by this time, it's 5am and more often than not, Eli decides that this would be a good time to get up. Mira then sleeps until 7:30am, then takes long power naps throughout the day. Sleep patterns are pretty screwy around here lately. We are taking it all in stride and thankful that she is still seizure free.

Partial Results

2007-12-13T22:03:00.001-06:00

Thanks for all of the suggestions for trying to clear up Mira's rash. We have taken several steps over the past week and it has cleared up significantly. As I posted earlier, we have tried multiple ointments and antibiotics, using a pretty potent anti-fungal/antibiotic cream on it over the months, which has helped a little. What has really helped though, is keeping her face dry. We change out her sheets every night, using a fresh towel as an 'added layer of protection' over the bed sheet. (Yes, I am beginning to sound like a bad diaper commercial.) We also started lathering her hands up with lotion, which not only helps her hands heal, but keeps her from sticking her hands in her mouth every 20 seconds. I don't think she likes the taste all that much after a while.

Mira's genetic screen for Rett Syndrome (the MECP2 typical variant) came back clean, meaning she didn't have any mutations on the MECP2 gene. A mutation occurs in about 85% of the typical cases of the MECP2 screening process. What they are now searching for are MECP2 deletion(s) on this gene. I don't quite understand how the process of recognizing a mutation versus a deletion occurs or why they have to be performed separately. I guess that's why I'm an architect and not a geneticist. We should get the results back on the deletions in another 2 weeks.

Thanks to Dad and Dottie for the Christmas flowers - they are wonderful! Eli has been eyeing the candy canes all day too..............

Rash Be Gone!

2007-12-07T20:11:00.000-06:00

Mira continues to do very well at home and in therapy. She is focusing and scanning the room much more lately. She also has been thrusting her tongue out a lot, as though she is spitting and making some silly faces on occasion. We have been able to get some really good giggles out of her by tickling her in just the right spot on her rib cage. laughing until she gets the hiccups. She can't seem to sit still any more when she is on your lap, constantly squirming and wanting to move.

She has been battling a rash on her face for nearly the entire year and it just won't heal. We have tried lotions, antibiotic creams, ultra-strength antibiotic creams, but it continues to linger. We recently discovered that it is a yeast infection, that started on her cheeks, but since she is now constantly putting her hands in her mouth, it has spread to the back of her hands and fingers. For yeast infections, it is supposed to dry out to get rid of it, which is nearly impossible since she is constantly drooling and and messing with her hands. Any suggestions would be great!

I received an email from Mira's neurologist and he said we should expect the Rett's testing back sometime next week from the lab. Thanks to everyone for your continued thoughts, prayers, and support.

One Month Seizure Free!

2007-12-04T20:10:00.000-06:00

Today officially marks Mira being seizure free for an entire month! Her last seizure was on November 4th. Way to go Mira!

Holiday Program + Neurologist Visit

2007-12-01T10:24:00.000-06:00

Mira had her annual holiday music program, sponsored by the Lee Anne Britain Center last night. Her class performed 'Jingle Bell Rock'. Despite being in a pretty cranky mood most of the evening, Mira managed to do well once up on stage with one of her therapists. I think all she really wanted to do was catch up on some sleep. Neither Mira or Eli were at all interested in having their picture taken - so it goes.........pictures of squirming and weird faces was the best we could muster out of the kids last night.

Eli became really excited once he saw Santa make his grand entrance at the end of the program. He was pretty direct with Santa and requested "Transformers" and an "Army Doll." Eli seems to be all about 'armies' these days, drawing pictures at school, telling his teachers all about them, etc., etc. If you would've asked him, "armies" were what he was the most thankful for this thanksgiving.

Earlier today, Mira had her neurology visit, which we had on the books for a month or so. We talked a little about her EEG, which although improved from her all her previous EEGs, is still abnormal. Her neurologist couldn't elaborate on it any more than that - there wasn't anything revealing in the EEG that would offer any clues or further insight.

As with every neurology appointment lately, we seem to walk in with certain expectations and questions, and 90 minutes later, we walk out feeling spun 180 degrees. Sarah and I had decided that we wanted to eliminate unnecessary medications and diets that we felt aren't working for Mira and just stick with what is helping, which we feel is the Lyrica. Therefore, we have decided to first wean the Zonegran which will take about the next two weeks. We'll give her the rest of December without any changes and then around the beginning of January, we'll begin weaning her off of the MAD (Modified Atkins Diet).

We feel that neither is doing anything - Mira continued to have daily seizures while doing both, with her seizure activity spiking badly during October. Things actually became worse. She has been on the Zonegran for over eight months and there was no positive change during that time either. As far as the diet goes, we essentially have already boosted her carbohydrate count by giving her applesauce throughout the day, so far we haven't seen any adverse affects from this boost seizure-wise. She is up from 10 grams to probably around 20 grams daily at this point. For Mira, we feel the diet is not nutritionally adequate, causes bad constipation, high cholesterol, and can be hard on her stomach. It is not healthy for kids to be on it long term. By gradually tapering these two therapies off individually, we can determine what is helping and eliminate what is not. Her neurologist agreed, thus our plan of action is in place for the next month or so.

What really threw us for a loop this particular appointment stemmed from the video I had sent her neurologist just before Thanksgiving. During her first couple of seizure-free weeks Mira had some strange, somewhat repetitive movements and breathing patterns. I videotaped her, posted it on Youtube and sent him a link. He reviewed it and it triggered some ideas for him, one of which was Rett's Syndrome. Some of the movements were apparently reminiscent of past Rett's cases he has seen in some of his patients. Mira had already gone through a round of genetics testing soon after her seizures started (in December 2005 I believe) and they came up with nothing in regards to Rett's.

As it turns out, Mira was only tested for the atypical mutation of the CDKL2 gene (5% of Rett's cases) and not the MECP2 gene (95% of cases). The reason testing the atypical route was based on the symptoms she was presenting at the time, but now, based on other criteria, she fits more of the characteristics of typical Rett's cases. So, CMH is scanning her DNA and seeing if they can find a mutation of the MECP2 gene to see if she has Rett's. We went through a very similar and emotional scenario several months ago, when her neurologist suspected she might have Angelman's Syndrome, although she lacked some of the facial features evident in most kids with AS. Rett's, as with Angelman's is pretty cut and clear - she either has it or she doesn't. We expect to get the results back in a few weeks.

The great news is that Mira continues to be seizure free, nearing the one month mark on Tuesday!

Doing Much Better

2007-11-26T21:12:00.000-06:00

Mira had a much, much better day today. She ate a great lunch and dinner, keeping everything down. She didn't sleep as much today, probably due to having some food in her stomach and having more energy.

Thanksgiving Break

2007-11-25T22:18:00.000-06:00

We returned from Illinois this afternoon, after spending Thanksgiving with Auntie Aura + Uncle Ware - thanks for putting up with us for 4 straight days!

Mira was mildly cranky on Thanksgiving Day - why we don't really know. She was pretty irritable during the afternoon and couldn't seem to settle down for most of the day. That was also the last day she ate a decent meal. Friday and Saturday she wouldn't eat anything all day, gagging on some pudding both nights, scattering the hardwoods with her upset stomach. We don't know whether she has a stomach bug, is bored with the food, or just isn't hungry, but she has eaten very little over the past 3 days. She is drinking plenty of fluids and doesn't have a fever, so we aren't real concerned at this point. We were able to get her to take a few spoonfuls of applesauce today, without gagging or vomiting - always a bonus when there is nothing to clean up.

Speaking of cleaning up...........I have officially met my quota of cleaning up bodily fluids for the next 6-8 weeks. Friday night - lots of vomit, soaking her high chair. Saturday night - repeat of Friday night, this time thoroughly drenching her Kidcart. Tonight - overflowing diaper (not the 'clear' stuff mind you) - staining her recently cleaned and disinfected high chair, again, along with the bed, the floor, and of course, her clothes.

On a positive note, she is still seizure free and still in a good mood! We have her neurologist appointment on Friday around lunch, along with her Christmas program on Friday evening.

EEG Results

2007-11-21T20:31:00.001-06:00

Mira had her EEG yesterday and everything went very well, despite her not being able to completely fall asleep - she did a lot of rolling around, even giggling toward the end of it. This was her 10th or 11th EEG (sort of lost count) and they all have pretty much looked the same until now.

We did get a brief message back from her neurologist this afternoon and he noted the following: 'Her EEG is improved, although still abnormal. The movements and activity noted on the EEG are in fact not seizures or seizure activity.' This is very good news overall! We are going to get into a more in-depth discussion during Mira's standing appointment with her neurologist next Friday.

On a side note - it was great to see my sister and family yesterday evening in from Colorado, who graciously treated us to pizza -thanks so much! We hope to come out and visit soon. Hope everyone has a wonderful Thanksgiving!

This Past Week

2007-11-17T06:39:00.001-06:00

Mira continues to be seizure free, which is fantastic. On the flip side, she is having sleep issues again, getting up throughout the night. She also is having strange movements throughout the day, which to me are concerning. She tenses up and arches her back all the time, in this sort of rhythmic motion every 4 or 5 seconds and seems to do it throughout the day. While I don't think it's seizure activity per se, I do think there is something else going on with her. We have her EEG scheduled for Tuesday, which might shed some light on it.

One Week Seizure Free!

2007-11-12T19:48:00.001-06:00

Mira has not had a seizure since last Monday, which marks one week of being seizure free! She has been vocalizing all day and constantly moving around. So much so, that we are keeping her off of the floor (she usually 'rolls' around on a blanket in the living room) because she is chaffing her cheeks and nose really badly.

She only got up twice last night, which is a big improvement from over the weekend. Things are going well for her and we are hoping and praying that it continues!

Seizure Free Stretch

2007-11-10T20:58:00.000-06:00

I hope I don't jinx it by saying this, but Mira has been seizure free for five days straight. Her last seizure was on Monday. She has been doing very well and constantly squirming. Seems like she won't sit still and has been smacking her globe toy so much, that I think she is going to break it. I hope her seizure free streak continues!

On the negative side, she has been having difficulty sleeping again. She has been waking up periodically throughout the night, crying as if in pain. This morning she got up at 4:00am and was inconsolable for a while. Her sleep patterns have been getting thrown off and she has been taking long naps in the afternoon, which is a recent phenomenon for her. I theorize that the sleeping issues are neurological, since her crying out seems to happen at night or when she is extremely tired. Coincidentally, that is prime time for kids to have seizures - when they are falling asleep and when they are waking up.

We do have her scheduled for an EEG on the Tuesday before Thanksgiving and a neurology appointment at the end of the month. She has not had an EEG for almost a year.

Going Well

2007-11-06T21:19:00.000-06:00

Things have been going very well for Mira over the past few days. She has been having only one seizure every other day, since last Thursday, which is fantastic! She has been giggling and smiling more, and doing very well in therapy.

Once again, she got a REALLY big kick out of 'The Bunz' this evening, making some strange laughing noises which I have never heard before. She became very animated and excited as soon as I pulled the string!

Sunday night Sarah and I attended the 26th Annual Tiny Tim, a formal fundraiser for the Lee Ann Britain Development Center (also called the IDC) where Mira gets many of her therapies. It was amazing to see the turnout and generosity from so many people giving to such a great cause.

Skunk + Spiderman Sightings!

2007-11-01T22:19:00.000-05:00

We had a great Halloween last night and have enough candy to be on a sugar high until early 2012. Mira was a skunk, which she seemed to get a big kick out of most of the time she had it on (despite the frown in the pictures). The tail was the kicker for the costume!

Eli was Spiderman with exaggerated muscles and all. We made it up and down our block before he decided he didn't need to go door to door to get candy - we have plenty at home! I was even able to cash in on some of the candy, when Sarah picked up a bag of Bottle Caps. Now, my teeth hurt. Mmmmmmmmmm.........sugar.

We did finally get Mira's Kidcart on Wednesday as well. It is a three-piece interlocking system that has a seat, a 'cart' for around the house, and a stroller base, all for a mere $6,200 retail. We will find out from insurance how much of that we will end up paying. She was seen at the seating clinic at CMH over the summer to get fitted for it. She is just on the cusp of being too small for some of the straps, but she will grow into it.

Mira continues to do very well since being on the Lyrica. She had one seizure today, but was extremely active after dinner and even knocked her toy straight off the chair.

Vomiting

2007-10-31T20:45:00.000-05:00

Perhaps not the greatest topic of discussion...............Mira has been vomiting lately and we are not sure what to attribute it to. Saturday, she threw up late in the afternoon, fortunately she was upright and in her Tumbleform. Tonight though, we had just put her down for bed and we heard her making some funny noises about ten minutes later and she had completely soaked the sheets, getting it everywhere. It could be from the medication or the fact that she has been really congested and it dealing with some drainage or something.

Otherwise, she has been doing very well, even rebounding very quickly from her stomach issues both times. We did see a few breakthrough seizures yesterday, but none today. She has still been in a great mood and is doing well in therapy during the day.

Day 3

2007-10-29T20:35:00.000-05:00

Mira didn't have any seizures today either, which makes 3 days in a row seizure-free. This hasn't happened in a long, long time - in the last six months at least. I don't know whether it's the Lyrica or something else, but she has been in a great, great mood the past 2 days! She had a big belly laugh with 'The Bunz' tonight before bed - more animated than I have seen her in a long time.

Pumpkin Patch + More Pictures

2007-10-28T20:53:00.000-05:00

I had a few additional pictures from last weekend from the train ride and Maple Leaf Festival and a couple from this weekend's pumpkin patch excursion, just outside of Lawrence. The weather could not have been better, but unfortunately, the pumpkin selection was pretty lean. We managed to get a few decent ones (see Eli sitting on his prize possession above left). Mira also had a good time, enjoying the bumpy stroller ride through the pumpkin fields. Since we were so close to Lawrence, we had to make a pit stop at La Familia for lunch. The sweet salsa is so good, it's almost drinkable. By the time we got home, everyone was sleeping, including Mira, who couldn't muster the energy to play with her pink sponge ball.

Mira started a low dosage (25mg) of Lyrica on Thursday and has been tolerating it very well. On Friday she had 2 intense seizures, but hasn't had one since, which is always promising. She will go up to 50mg on Tuesday and then we will see if she is still tolerating it. The therapeutic dosage for Lyrica can vary, so assuming it is helping, she will probably just hold at 50mg for a while.

Lyrica

2007-10-25T20:56:00.000-05:00

We started Mira on a low dosage of Lyrica this evening and we will cross our fingers to see if it will give her some relief. She only had one tonic-clonic this morning, but she has been very irritable, having tons of twitches and small jerks all day, which is sort of where she was back in August, before she started the diet.

Recent Events

2007-10-23T22:11:00.001-05:00

Saturday we took Mira out to a PT who specializes in hippotherapy for a consult and a session to see how she is she could benefit from weekly therapy. At first, she was very floppy on the horse, but over time, became used to the rhythm and movement and was much more relaxed after a few minutes. She even giggled a few times. Ultimately, we think it could be very beneficial for her and we are actively trying to get on the therapist's schedule for the spring.

We went to the Maple Leaf Festival in Baldwin City (after Mira's session) on Saturday and had a great time and enjoyed some fantastic fall weather. We rode the train from the old depot, while Eli tried to verbally apprehend several of the 'bandits' (a staged robbery skit/re-enactment they do on the train) by simply scowling loudly at them. It didn't work, but it was convincing.

Unfortunately, Mira has been doing pretty poorly over the past week, having multiple seizures every day, especially over the weekend, when she had a dozen or so. Her seizures have gradually become worse over the past several weeks and we are often seeing clusters of activity. She has become more irritable as well and they are really bothering her much more than before.

We talked to her neurologist about starting Lyrica (pregabalin) which is one of the few medications she hasn't tried, to see if it will help. As with all of the other medications, it either works or it doesn't. We had talked about increasing her Zonegran (she is already on a maximum dosage) or just taking her off of it (could potentially make things worse) so in the end, we decided to try Lyrica and not have any other moving targets at this point.

Past 2 Days

2007-10-19T20:30:00.000-05:00

Mira has been off the Ketocal for a few days and her demeanor has improved. Although, yesterday she had another cluster of seizures which Sarah had to use the Klonopin to interrupt the cluster. She had 3 seizures within about 20 minutes. Today was better for her and we were able to get a few smiles out of her before bedtime.

Tomorrow we are going to meet with a therapist to potentially start hippotherapy with Mira every week.

Aspartame + Artificial Sweeteners

2007-10-17T20:24:00.000-05:00

Mira is rebounding from a rough few weeks and we have made some changes, starting yesterday. We noticed that she started becoming increasingly irritable soon after starting Ketocal, about two weeks ago. I really hate to sound so jaded, but I attribute her problems directly with Ketocal and what it has in it. It contains aspartame, which is an artificial sweetener. While there is some truly frightening literature out there (read this story or this one and why not another or get the book from Amazon.com, read them and form your own opinions) I can't help but believe that aspartame is a seizure trigger for her or at a minimum, causes some issues with her.

In addition to causing irritability issues, the Ketocal was also causing a lot of constipation, which stresses her out. We stopped the Ketocal yesterday and gave her several doses of Miralax over the past day and a half and she has been doing remarkably better than she was yesterday. She still had 2 seizures, but she is in a much better mood and stopped twitching. And yes, she pooped A TON today. If you want details, Sarah and I can provide.

Regardless of whether aspartame is a trigger or not, we are going to stay clear of it (along with other artificial sweeteners) all together and talk to neurology and her dietitian about alternative methods of boosting her ketones.

A Mixed Week

2007-10-13T08:30:00.000-05:00

Mira has had some difficulty this week, especially Thursday and Friday. We started seeing a bunch of seizures earlier in the week, which took a toll on her. We saw even more on Wednesday which prompted a call to neurology, since we were afraid that she was going to have another episode similar to last week. They gave us a prescription for clonazepam (Klonopin) wafers, to use as an additional 'temporary' medication to help her get through bad days. Mira had tried Klonopin a long time ago, without success, but now she will use it on occasion if she has frequent episodes or multiple seizures. She can still use the Diastat (Ativan) to stop the clusters, but not in conjunction with the Klonopin - the two have to be used independently.

Mira has also been vomiting some this week. She had an episode on Thursday night and again this morning, as I was posting this, ironically. It might be that she is becoming more ketotic, which tends to mark an increase in nausea, vomiting, and/or diarrhea. Sorry, Mira's diet topics are typically not a conversation to have over dinner.

Speaking of ketosis, we are still working with insurance to move forward on the Ketocal, but have been having difficulty pushing the paperwork through thus far. It is moving along, though.

A Positive Rebound

2007-10-08T20:57:00.001-05:00

Since her episode last Tuesday, Mira has been doing significantly better. We don't know what her status seizure could have been attributed to and chances are, we will never know. Her seizures and episodes are so completely random that we quit guessing.

Since last Tuesday, she has only had 3 seizures (in 6 days!), which is fantastic. We think the Keotcal boost has really been helping her, so we stocked up for the next few weeks. We should be getting our first shipment tomorrow. We had to make a run to Children's Mercy this afternoon for another can, just to get us by for the day. Although Mira is only supplementing, we will be going through a can every 5 days, which equates to over $2000 a year, just in Ketocal alone. Thanks to Sat Inder, Karta Purkh, and Auntie G. for contributing to Mira's Ketocal fund!

On a side note, Sarah and I celebrated Sarah turning 29 (for the 4th year in a row.....) at 40 Sardines. We decided to skip our usual favorite locale, Le Fou Frog and try something new. I can't say that I was all that impressed with our choice, which is disappointing, since I had heard rave reviews about the joint. Could have been an off night I suppose. After all, it was 'opera' night at the restaurant and I did win a CD out of the deal by Anna Netrebko. It would be great if I actually listened to opera and could pronounce her name, but I cannot do either. Oh well, guess I'll stick with Thievery Corporation.

Anyway, thanks to Auntie Aura who braved our evening out by watching all four kids!

A Scary Tuesday

2007-10-03T21:07:00.000-05:00

We had a very scary moment with Mira on Tuesday afternoon. After a fantastic weekend (2 seizures in three days with none on Sunday) she had 5 seizures within a couple of hours on Tuesday afternoon. After her sixth seizure around 3:30pm, she started twitching and jerking repeatedly for a while afterwards (not uncommon), but then began having what seemed like a status seizure. We are not sure exactly what it was, but it could have been what is referred to as complex partial status epilepticus. She began squeezing and tightening her hands, arms and legs repetitively and consistently every 30-45 seconds.

We didn't want to be too concerned at first, as her breathing wasn't labored and she didn't seem to be in any pain. Although she had been quite distressed after each tonic-clonic ("big") seizure she was really zoned out from the start of this "episode." It became apparent to Sarah after a few minutes that she was having clusters of activity and was staring off repeatedly, tensing her arms and legs over and over.

We called neurology to get a hold of Mira's nurse to try and understand if this was a prolonged seizure or if she was going into status or what. I had left work early and was headed to CMH at that point to pick up Mira's Keotcal and was able to discuss her situation directly with our neurology nurse, Lynn. She told me that it sounded like a status seizure of some sort and recommended administering Diastat regardless, which we have as a rescue/emergency medication. Sarah gave it to her as I was leaving the hospital and her clusters and squeezing stopped almost instantly. This episode went on for about an hour and 15 minutes total.

Needless to say, we have never seen this sort of seizure activity with her before. If there is one thing that is consistent with Mira is that her seizures are inconsistent. Different types, different times, ever changing patterns. We were watching her like a hawk today and ironically, she didn't have a single seizure or episode all day.

This Week

2007-09-29T16:32:00.000-05:00

Not much has really been happening over the past week. Mira is still averaging about 1-2 seizures a day, mainly when she is waking up or falling asleep, which is very common, according to her neurologist. We are still having difficulty getting her ketones up and within a 'therapeutic' range (so to speak) which could account for her seizures not being completely controlled. We are going to take her in to CMH next week to have her labs done again, just to see where she is with the diet and to see how ketotic she is. Chances are, we are going to have to supplement with Ketocal in order to boost her ketones. Overall, the diet is proving to be a success, we just need to get her into better ketosis. We are moving forward, slowly but surely.

We did get her Jenx Monkey Stander last week and she has been using it for PT at home. She broke it in the first day by throwing up on it! Probably not a good idea to get her in it right after breakfast........

Back in the Swing of Things

2007-09-21T21:18:00.000-05:00

After some travelling last weekend and more this week for business, things are settling back down for this weekend. I was trying to come up with a witty title and all I could come up was this? Secretly I have the 1985 'hit' by A-ha stuck in my head. Yes, random, very random.

Mira has been doing well over the past week. She is still averaging about a seizure or two a day, but has really been in a good mood all week. She seems to be handling the tweaks with the diet, especially the MCT oil. We are gradually increasing her intake to get to 1 oz. daily. She has been off of the Lamictal completely for a week and we haven't noticed any negative changes yet. We will start weaning the Zonegran very soon.

We all had a great time in Saint Louis last weekend and especially at Siena's (Mira's cousin) birthday party. Thanks to Tom and Nikki for the animal farm fun!

(Pics from left to right: Eli and the tortise; Siena on a wagon ride; Sophia with the rabbits; Siena ready for some cake)