Keppra

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

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

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)

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!

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

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

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

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

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

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

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

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.