Sunday, December 14, 2014

New Swing

We have had the pleasure of living next to a park for the past 14+ years. It's always been a strange mix of green space, play equipment, and at the time we bought the house, an enormous water tower. The water tower, which had been a monument in the landscape since the early 1950's, was decommissioned and dismantled several years ago. Shortly after it was taken down, the park received some minor renovations.

Earlier this year, the city announced they would be giving the park an entire overhaul, which included updated play equipment, a tricycle track, a park shelter, and new swings. I was invited, along with several other adjacent neighbors, to provide input on what we would like to see in the park.

Well, that's an easy question. Mira loves to swing. Having an ADA or an accessible swing would be ideal to have right next door. The only other ADA swing for kids with special needs in the entire area is about 20 miles away, so having one practically in our back yard, would be a dream.

The parks and recreation department was receptive to the idea and incorporated the swing into the plans, along with a host of other comments from neighbors. Construction finally ended late yesterday and the park was opened to the public. Mira was the first one to break in the new swing and she loved it!

I am thankful for living in such a great community, who involves its residents in the decision making process of even the smallest of projects. My deepest thanks to the City of Prairie Village and thanks to Jori Nelson, who helped facilitate the process.

Sunday, December 7, 2014

The Year of the Seizure

It seems like this year will always be remembered as the year of the seizure. While Mira has been battling seizures for the past 9 1/2 years, this year was marked by having them every single day. Her myoclonics seem to have slowly ticked upward throughout the entire year, with no relief in sight.

Let's start with medications. She began the year on Lyrica, which she has been taking for years, while also taking Onfi, which she had been on for about a year. Neither one seem to be doing anything for seizure control. In fact, Onfi caused incredible drowsiness, drooling, and a lot of sedation with her. While she was on Onfi, we struggled with Mira's head control. It seemed to cause a lot of lethargy with her and she was really out of it - struggling just to keep her head up. Ironically, she still was having a ton of myoclonic seizures while on it. After about 18 months of being on Onfi, we decided to wean her off it back in March and April.

We witnessed a consistent flow of myoclonics throughout the entire summer that we just could not control. Mira also continued to average 3-4 involved tonic-clonic seizures a week, sometimes upwards of 6-10 a week. The myoclonics were averaging 100+ a day. Frankly, they are difficult to track, since she has so many. Recently, we tried increasing her Lyrica and it did not go well. Mira had a couple of really positive days with the increase and we were hoping we were headed in the right direction, but she quickly went back to baseline. She started having even more seizures with the increase.

We did have a neurology appointment a few months back and we discussed our options and a plan with her neurologist, in terms of what we can try. Unfortunately, Mira has few options left to try. We first discussed doing a trial of Leucovorin, in the very rare likelihood that Mira might have a CFD (cerebral folate deficiency). A brief trial with folinic acid (a derivative of folic acid) pretty much ruled this out. She is still taking it now, but it is clear that she does not have a CFD or anything related to MTHFR (methylenetetrahydrofolate reductase deficiency) and if she does, it wouldn't be remedied with Leucovorin. We have discussed MTHFR and CFD possibilities with several of her past neurologists, so we figured it was worth a trial.

Our second option was to increase her current Lyrica dosage and unfortunately, we have considered the increase a failure at this point, as it ironically only seemed to cause more seizure activity. After a few more discussions with her neurologist over the past week, we have decided to abandon Lyrica all together, We feel collectively, that it lost its efficacy long ago and the fewer ineffective medications in her system, the better, She is in the process of weaning right now, which makes us nervous since she has been on it for so long.

While she is weaning the Lyrica, we are trying Vimpat (lacosamide) to help control her seizures. Lacosamide is a sodium-channel inhibitor, which she has tried before, so we are not very optimistic. It is one of the few medications she hasn't tried though. If it proves to be ineffective or worsens her seizures, we aren't sure what to consider. One step at a time.

One of the big issues this year also has to do with Mira's weight. She had lost a considerable amount over the past 9-10 months, enough to drop her from the 90% percentile to the 30% range. While her neurologist wasn't overly concerned, he did recommend a consult with a dietitian. We met with someone in clinic to get Mira on a decent calorie intake and she has already gained back a few pounds over the past month.

Mira has had some positive days, as shown in the video below, but they are few and far between. Most days, she is having so many drop seizures and myoclonics, that it is difficult for her to focus on anything. It's heartbreaking to watch her suffer and there is little left to try and seizures have just taken over her routine as of late.

video


Mira has always battled irritability. It's a vicious cycle of seizures, irritability, and sedation. She gets irritable from the constant neurological static, then has a big seizure, and is knocked out for hours afterwards. She tries to play with her toy, has countless myoclonics while she is trying to play, then becomes irritable and frustrated because it is nearly impossible for her to focus or enjoy it with all of the interference.

So where are we now? Besides trying to advocate for legalization of medicinal cannabis in the State of Kansas, we are researching anything and everything to try and help Mira, as we have been doing for the past 9 1/2 years. Reading and researching countless articles, studies, and trials, while trying to remain hopeful that something will help her. We are open to suggestions and input, so feel free to email me at marcus1988001@yahoo.com.

Sunday, February 2, 2014

Very Brief Update

We just finished weaning Mira off of Onfi, after being on it for 18 months or so - I forget how long its been, but nonetheless, she is done with it. We tried increasing the dosage on several occassions, but it failed to have a positive effect on her seizures. It frankly just increased her drooling and made her more lethargic. Mira continues to take Lyrica, which she has been on for 5+ years. It doesn't completely control her seizures, but it is the best we can hope for, for now. 

With all of the buzz (no pun intended) with CBD/THC, we thought it might be worth a shot to try holy basil, which supposedly has some of the same effects/mechanisms as CBD, yet is legal. Mira has been on a low dosage since the end of December, but it has failed to helped. We will give it a little longer until we completely abandon it.

Will update pics soon.

Friday, November 2, 2012

Mira's New Chair + Equipment

Mira's new chair arrived late last week! Mira had grown so much over the past 18 months that she quickly outgrew her old Zippie Iris. Her new chair is a larger scale Zippie Iris, with additional side supports (to hopefully keep her from scooting and slouching in her chair) and better lateral supports. The new larger head rest will also help Mira with head and neck support. We also ordered her a new bath chair and a new car seat, both of which she had  grown too big for. All three pieces are working great - thank you Variety KC!!!



On a side note, Mira is now completely off of clorazepate, a medication she had been on for several years. We have not noticed any major side affects and are happy to have her off of it. On the direction of her neurologist, we had originally added it to her daily medications to help with some severe irritability. She is now on a dosage of clobazam, which is much less sedating. Mira seems to be more engaged and active now.

Sunday, October 7, 2012

Mira's New Chair is Arriving Soon

Mira's new chair is on it's way and should be here in a few weeks! I cannot be more excited for two main reasons. Number one, the process of getting her new chair went so smoothly this time, that I am still in disbelief. The staff and communication with the seating company has been a quantum leap better than what we had to deal with in obtaining her current Zippie. So, kudos to the seating company for being proactive. Secondly, Variety of Greater Kansas City is paying for Mira's new wheelchair! I cannot thank Variety enough for this incredible gift in helping pay for her chair. This will make an enormous difference for our family and for Mira, most of all. THANK YOU VARIETY!!!!!!!

Sunday, September 23, 2012

The Slow Wean

We have been slowly weaning Mira off of clorazepate (Tranxene) for a few weeks now and we are not quite half way through it. Benzodiazepines are very sedating and will not only cause the body to build up a tolerance to these types of medication, but weaning can cause significant and severe withdrawal symptoms if someone has been on it for a long time, thus the very slow wean for Mira.

On the direction of her neurologist, we are dropping her a quarter tab every week, which will (fingers crossed) get her completely rid of it in 6 weeks. This comes after dropping her a full tab over the past 6 weeks. We have been seeing an increase in larger tonic-clonic seizures and a ton of twitching/myoclonic type seizures the day after a decrease in her dosage. For instance, we dropped her dosage last night and this morning she had two big seizures almost back to back, then several hours of twitching, which was making her pretty irritable. It seemed to wear off over the day.

We hope to get her off of this medication altogether, but we are taking it slow. Mira added clorazepate to her seizure medication regiment about 2 1/2 years ago, only because we were dealing with some serious irritability with her. So far, we haven't seen too much change in her demeanor. Her seizure activity is questionable right now.

On a high note, we received a letter back from Mira's insurance on her new chair and it was approved! Phase one complete. Now, if we can only get the seating company moving forward, we might be in a good position to have her chair in 4-6 weeks.


Sunday, September 16, 2012

The Cliff Notes Version Update


It’s been such a long time………… I can’t possibly go into every single detail since the date of my last post, which has been nearly 18 months, but I will try to give you the Cliff Notes version.

Let me first start off by telling you how much Mira has grown. Mira turned seven back in June and she now weighs 80 lbs. Still in the 95th percentile for height and weight, she is getting much more difficult to transfer. Currently, we transfer her from her chair into the van, then lift her chair into the back of the van. This process is exhausting and really bad on our backs. We are in the process of getting her a new chair (see previous posts about the ordeal we endured getting her Zippie Iris and insert frustration rant here) as she continues to grow, but it may be awhile. A newer chair really means a heavier chair – it will be another Zippie-type chair, one that tilts, only with a bigger frame.

Mira is a scoocher these days. She is constantly slouching down in her chair. We are getting some special attachments for her new chair that will hopefully keep her from pushing down in her seat, but for now, it is a battle of keeping her lap belt tight. When she slouches, she swings her legs out of the chair, which nearly pulls her sideways out of her seat. We find ourselves constantly having to reposition her to keep her from wiggling out. The chair will also have additional head supports on the sides, which will hopefully keep her from tilting her head as well. In addition to slouching, Mira is tilting her head (almost exclusively to the left side) and pushing her neck between the seat backing and the underside of her headrest. It looks uncomfortable for her and we find ourselves tilting her chair way back into an inclined position to try and keep her from doing it.

Mira’s rapid growth over the past 18 months has also prompted us to start searching for a lift van. After visiting with a sales rep a few weeks ago, we know what we need and want in terms of a van, now we just need $65,000 to pay for it. Small problem. We have no money. We are holding off until next year and hopefully be in a better position to at least reevaluate what we might be able to afford. For now, we are going to try and get her a new car seat system, as she has maxed out her Britax seat, but that process will take a little while with insurance, as it always does. With the combination of a new chair and a new car seat, we will see if we can get through another year of transferring her in and out of our current minivan.

The biggest adjustment we have had over the past year is Mira starting school. Mira started kindergarten with the school district last fall, going half days for most of the school year. Toward the end of the spring semester, she started going full days and she has adjusted well to the extended day. She is now a month into first grade and all is going well. It was difficult to send her off on the bus for the first few days, but Mira loves the ride to school. The movement and the vibration of the bus ride usually gets her in a good mood. The drivers and the staff have been great and I love her teacher and her aides at the school, as they are very caring and attentive.

In terms of medications, Mira has been on a constant regiment of Lyrica and Tranxene. She has been on Lyrica for almost five years now and we think that is it contributing significantly to her weight gain over the past 18 months or so. We are in the process of weaning her off of the Tranxene, as she is also taking clobazam. The hope is that we can fully transition her from Traxene to clobabzam, which is supposed to be less sedating. Once we have cleared that hurdle, we can talk about reducing or eliminating her the Lyrica. We did try weaning her a few years back and it was a disaster. One step at a time. We are actively working with a dietician through Children's Mercy in an effort to try and manage her weight.

We have a had a series of routine neurology appointments over the past 18 months, which seem to always brush on genetic testing and so forth, but nothing revealing has ever come up. Mira’s neurologist has basically told us that Mira has something very similar to Dravet Syndrome (SMEI), although genetic markers thus far have yielded nothing. Mira is currently enrolled in a broader study through Children’s Mercy that will look at over 300+ potential gene mutations, which only a handful might be relevant to Mira specifically. We enrolled her in the study about 10 months ago, but we have not heard anything back.

I think spiritually and mentally our family is in a good place right now and we all seem to be in a groove with three kids in school. Physically, we are often worn out as the logistics of getting Mira around are taxing, but we are actively working on that.

I did start a simple website at http://thefirebreathingduck.com/ as my daily mantra as a parent of a child with special needs. If you enjoy it, please pass it on.

Hope everyone is well with life and family and I will post when I can!

Sunday, May 8, 2011

Neurology Appointment

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.

Sunday, April 17, 2011

Ramp

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 guidelines 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.









Saturday, March 5, 2011

Baby Day Photography

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:


Monday, January 31, 2011

Update, Pictures, + Videos

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 5th 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 90th 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 40th 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!


Tuesday, June 29, 2010

Mira Turns Five!

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!