Saturday, August 29, 2009

The Long, Long Summer

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.

Tuesday, July 14, 2009


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.

Wednesday, July 8, 2009

Happy Birthday - Part Deux

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!!!

Sunday, June 28, 2009

Happy Birthday Mira!

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!

Monday, June 1, 2009

Gerber Goop

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.

Monday, April 27, 2009


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.

Thursday, March 5, 2009

Mira's New Bed

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!