"I wonder if 2011 will be boring?"

These were the words out of my husbands mouth last night. I paused for a minute and wondered what boring meant and thought to myself: "Oh, he meant no major anythings!"   My answer: "If we look at the last 3 years the percentages are not on our side. Lets hope for normal, whatever that is?"

Bad major things the last three years (............. means I'm not telling you! lol)

Autism Diagnosis
Type 1 Diabetes Diagnosis
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Gall bladder surgery
Unplanned Pregnancy (Such a blessing that I could not imagine life without)
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Birth of our third and last child, which led to....
Pulmonary Embolism and DVT. Good chance of dying.
Recovery of the above PE and DVT (So much pain)
Infantile Epilepsy
Trips to Boston
Failure to Thrive
G-Tube Surgery
Weight Gain (Me)

Good things:

My husband: He is healthy (as far as I know) continues to be able to provide for us, is a great father and my rock. Things get rough, but we always make it through it.

My children: Sam has made improvements by leaps and bounds. No one would know he was diagnosed with plain Moderate Autism 3 years ago.  His report card was a pleasant surprise!! We are getting use to life with Type 1 Diabetes. We would rather Kailyn not to have to deal with this, but she is thriving in and out of school. Keira: The fact that she was born!! I can not imagine life without her. She is so much fun, an easy baby besides the whole epilepsy thing, and just pure joy!! Just as recently as 3 weeks ago was the last time we saw a seizure and she is now in the THRIVING category. Gaining new skills every day. We got the all clear after an upper GI yesterday to get her therapy to try and get rid of the G-Tube. We hope to see continued Physical development.

My Extended Family: We could not have made it through the above without them. We love them very much and are thankful for EVERYTHING that they have done for us.

Here's to a non-chaotic and Happy New Year!!! :)

-Kristen

Keira saying spider, trying hand movements, saying all done and momma! Huge Developments!!

I am holding my breath that this is the beginning of more to come!!!  Notice her saying spider, momma and all done. It does not have to be said correctly for it to be counted as a word.  She also tries to do the hand movements and screams at me when I stop usually, but this was not caught on the video. :)  This all took place around 1am because her teeth are hurting. You can see her put her fingers in her mouth too.




PS: No seizures still except for when she was sick with a very high fever!!  Starting Stage 1 baby food, but she refuses to drink using any sort of cup or bottle.

Some Serious Christmas Preparations

Our house is decorated. Thanks to the kids. I have decided it is a kid themed Christmas and they did the decorating. (with a little direction from me ;) )We have chains linked all the way around the living room, in the playroom, Sam's room and Kailyn/Keira's room. Paintings hung in different areas too. The kids went with their grandparents to get the 'real' tree that we put on our porch and decorate for the birds today. We would have done the traditional Gingerbread house, but I decided to quit that tradition. It is way too hard to count the carbs and little fingers like to steal the candy off of the house at random times.  That was part of the fun (at least in my opinion). Last year was a mess with blood sugars out of control.  We will move back to sugar cookies (probably the precut kind) ;)
I have all the presents, stocking stuffers, wrapping paper and the cards are made. Now we just need to wrap the presents, send those card and for Christmas to come. I am usually doing all of this at the last minute, but I want to be prepared this year. "Expect the unexpected"  Kailyn was shocked I was letting them put the tree up right after Thanksgiving. She said, "I did not think you would really say yes!" lol  A lot of my time when the kids are in school goes into therapy and Doctors appointments for Keira so it will be a nice load lifted off my shoulders when everything is done. Hopefully, Grammie will make us one of her beautiful wreaths again this year ;)
What do I want for Christmas?  Tranquility for Seth and I. Joy for my children.

On a hunch

Call it mother's intuition or just a gut feeling. I decided that the Zonisamide was not helping Keira's seizures.  Her seizures are triggered by sleep (among other things) and the Z med is not only known for making kids sleepy, but for making them have more trouble thinking/concentrating.  I got Seth on board and we slowly titrated her down to zero. Her last dose of  Z was on Tuesday and the last seizure we saw was on Tuesday so far. I am knocking on some serious cyber wood. I have been hesitant to say anything only to have them return the next day. BUT, I DO want to say something!! She has had a few random days without seizures, but none were consecutive!! This is 4 days without any noticable seizure activity.
We have seen a few other developments and go to the Neurologist on Monday.  She is only on Depakene 3x's a day now. Still a hard core drug for her little body, but she has made great strides since her surgery on October 20.  The most notable being her chubby little cheeks.

Our Visit to Children's Hospital Boston: Synopsis and Questions.

Keira's current diagnosis:
Infantile Myoclonic Epilepsy

She has a sister with Type 1 Diabetes (age 8) dx two years ago who has history of reflex anoxic seizures and a brother with a dx of Autism (age 6) 3 yrs ago. He is doing very well and in a typical classroom without supports. (Title one for Math and Reading)


Keira's first symptoms started around June 5, 2010, when she got her first cold with a slight fever. She did not have a febrile seizure, it looked more like a startle response. The Pediatric MD assured us that it was a startle response at the time. It would happen with different sounds, when she looked at different lighting and then evolved into being spontaneous in clusters of 3 with her eyes rolling to the right side for no apparent reason at all. It also started to happen when she was drinking. At that point she was 7.5 months old and had started on chunky solid foods with no problems.

This is when we got concerned and called the Ped MD again (July 13, 2010). She had us come in and confirmed that they were, in fact, seizures (because she saw them herself) and called one of the Neurologists on staff at Maine Med so she could get an EEG ASAP. That would not happen until three PM the next day.

She had her first one hour VEEG and MRI at almost 9 months old on July 14, 2010 and was diagnosed with Benign Myoclonic Infantile Epilepsy(which meant she would outgrow it with no permanent damage). She had an MRI on July 15, 2010 that came back completely normal. At that point, she was not only meeting her milestones, but was ahead of them in the communication areas. She did not show the EEG pattern for Infantile Spasms (hypsarrhythmia). She was having the seizures anywhere from 80-100 times a day. We were advised that we should put her on meds because she was having them so frequently, so we did. She started on Keppra on July 15, 2010.

Keppra only reduced the seizures in half. She was then taken off of Keppra after a couple of weeks and put on Depakote, which reduced them to 10-20 a day, most of them happening when she ate/drank, when sleepy, and riding in the car. She at one time started to refuse the bottle and was down to 5 ozs of formula a day. We got her back up to taking a reasonable amount because we forced her to drink through the seizures using a bigger nipple and cutting more holes in them (12-15 ozs a day). The Depakote was then increased on August 24, 2010, when her therapeutic blood levels were tested and she was still only at 83. Optimal levels are between 50-100. After this increase we still saw no reduction from the 10-20 seizures a day and she was almost always having them when eating/drinking, sleepy and riding in the car backwards. Then around Sept 15th we realized that she was not meeting milestones and was even losing skills she had already mastered.

She used to do these things easily and can no longer :

Give us high fives. Clap and say "YAY". When you asked her how big is Keira, raised her hands for "SO BIG". Wave hi and bye bye easily (we have it on tape). Use her hand to sing a Native American song. Eat foods that were chunky, holding the foods and putting foods in her mouth with a pincher grasp. Say Momma (looking at me) Dada and Hi appropriately. She started to give a few sporatic high fives and make the momma, dada and hi sounds again as of (Oct 9, 2010), but it does not come easily to her.

Keira can:

Respond to her name. Laugh, giggle, and smile when people are playing with her. She screams sometimes to either hear her own voice, get what she wants or to try and fit into our noisy, chaotic house. Shakes her head No. She shakes her head no regularly because she does not want to eat. She knows it will trigger a seizure. Gets up to a sitting position and down from one. She does an army crawl. Has a pincher grasp, but won't put things in her mouth to eat in fear of a seizure. She will chomp on things that are not food, but not swallow. Bangs toys together. Picks up utensils. Crosses the mid line with objects and transfers an object from one hand into the other. She gives (cave) kisses when you put her right up to your cheek. Likes to read books. Likes to play with toys/experiment. Her fine motor skills might be right on track and she does have sensory issues. Hates baths, being touched, etc.(we have an evaluation for this coming up soon)

Keira has had a hard time with:

Since birth she would not apply pressure on her legs and would fold them up so we could not try and make her stand. We now make her stand and she curls her feet up most of the time, but yesterday she did it without crying or curling her feet up for the first time (Oct 7, 2010). 12 days from 12 months old. It is not a preferred activity, but we do it every day.

When presented with a new object she sometimes hovers her hand over it for a long time. It seems like she knows what to do, but her brain won't let her hand move. Once she gets into it she has no problems banging around etc.

Keira has never fed herself with a sippy cup or a bottle. She can hold them, but won't do it.

She has regressed to eating only stage 2 foods and formula from a bottle.

She does not use gestures, one after another, to get needs met, like giving, showing, waving, and pointing. She does reach for what she wants. She does not play peek-a-boo, patty cake, or other social games. Make sounds like "ma," "ba," "na," "da," and "ga" on a regular basis. (sporadic ma, da and ha sounds) Sometimes you can catch her saying Momma, but it has no meaning anymore like it did when she was looking at me and saying it and it is not on a regular basis.

After the realization that she was not meeting milestones and that she was having seizures almost always whenever sleepy, riding in the car, eating and drinking we rushed her down to the ER at Children's Hospital in Boston September 17, 2010. These seizures can last anywhere from seconds to three minutes in length now. The longest ones are when she is eating. She also tightens up and curls her toes whenever drinking a bottle. I had mentioned the loss of skills and eating problems with the Neuro in Maine, but he replied with an unhelpful, "Huh, that is strange." Children's Hospital Boston saw the seizures on video and in person and admitted her to do another longer VEEG. The first MD said they would do the VEEG for 24 hours and that they would check her Depakote levels to see if there was room for an increase and come up with some new meds based on the VEEG.

The second MD said that the VEEG showed the Myoclonic pattern, but was non-committal about identifying the seizures as myoclonic and said that they could also be clonic. He said that she looked like she was having reflex seizures and to talk to our MD and Dr. Expert (at Children's). We were scheduled to see Dr. Expert in Nov (that was in Sept) and this MD wanted us to see what Dr. Expert had to say about what meds she should try. He never checked her Depakote levels and did not want to step on anyone's toes. We wanted to make sure she was not having Infantile Spasms, but on the other hand were disappointed that he did not check her thoroughly--he never touched her. Especially, since we were talking about our daughter's developing brain and our difficulty in keeping her properly nourished. He did mention that she could still have developmental delays with myoclonic seizures and doubted that the Depakote was the problem.

We went back to Maine and called her Neurologist promptly on Monday Sept 20, 2010 as directed. After 2 days and 3 calls he called back and I had him look at the video of her seizing and he ordered an immediate increase of Depakote (without checking her levels). After 7 days with no change he ordered blood work checking her levels and other blood counts. Her Depakote level was 150, 50 levels higher than it should be. He ordered us to take her down to the first level of Depakote because the seizures never got better with the increases and started her on a new med called Zonisamide. After 3 days on the Z med she physically could not move. I called the Neuro again on October 1, 2010 in a panic and he had us decrease it in half to titrate her up even slower. She started to move again, her seizures when being tired have reduced, but otherwise we have not seen any improvement in her seizure activity. Some days we think we do and then the next day slaps us back in the face with even more seizures. Sometimes I feel like I can snap her out of the seizures and other times not. I never can when she is eating, drinking, or in the car. Just when she has spontaneous ones from being tired.

Our questions for the Epilepsy MD:

Are you 100% sure the diagnosis is correct and if it is not, what could it potentially be?

Should she have other testing done to see what is going on?

Such as, PET, (Blood Testing) Metabolic and any other type of disorders that cause seizures?

What do her toes curling while drinking and seizing mean?

I read that seizures with eating are typically focal motor seizures and are symptomatic?

Is she on the right medications?

What are her percentiles today for height and weight?

If eating epilepsy is treatment resistant (which I have read), should we put her on a feeding tube to make sure she is getting the right amount of nourishment and does not have to force herself to have seizures? This way we can avoid more medication that may be causing developmental delays? We are very concerned with the eating/seizure component and it causing her to not gain weight.

I have the G20210A prothrombin gene mutation. Could she have something similar to this causing her problems?

The diagnosis for infantile spasms can be made even if one of the 3 elements is missing (according to the international classification). She does not have hypsarrhythmia, but has the other components? Does Boston not follow this classification and only rely on the H pattern?

Do you feel comfortable working with our Neurologist in ME and if not is there someone you can recommended to us?

Our appointment is Tuesday at 10:30 in Boston with the Child Epilepsy Specialist

I have not decided if I am going to give this to the specialist. If I do I will have to do some rewording. ;)

Adventures in.... What the heck is going on here seizures?

We rushed to Children's Hosptial Boston on Friday Morning because Keira is starting to not meet milestones and her seizures are increasing and evolving.  These symptoms all pointed to Infantile Spasms. If they are infantile spasms time is of the essence and you DO NOT fool around. They admitted her to do a 24 hour EEG.  I have been told this is the #1 hospital for child neurology/ epilepsy in the country if not in the entire world.  People come from all over the globe to go there for an opinion. 
We left the next day with good news and bad news.  We were told that her seizures were clonic (not myoclonic), but the Epilepsy specialist did not seem convincing. The major problem with Keira's seizures is that they are very rare. The other problem is that Neurology is not an exact science and every Epilepsy specialist will have a different opinion on what track to take. I think he wants to wait for the MD with more experience to give more info and decide the course of medication.  He was convinced they were not Infantile Spasms (which is a good thing), but also told us that other seizures can cause delays.   He said to start services locally, call our regular Neurologist to get med changes and to work with the other Children's Hospital Epilepsy Specialist to come up with a plan.  He did not want to step on anyones toes and add meds that may change in a month. We meet with the other specialist in Boston on Nov 9.  So close, yet so far away.
Our local pediatrician called this am and has already called in a referral for an eating clinic at Maine Medical Center. Keira will chew now, but now swallow. She is not gaining weight and has lost a little bit.  She also made a  referral to a Physical Therapy and Occupational Therapy clinic for an evaluation.
I feel good that we are doing all that we can to make sure that she can develop normally.  Anxious to hear what Mr. Expert has to say.
Thrilled that Keira is still the happy go lucky, easiest baby in the world. (besides seizures ;) ) 
Here are a few pics of our stay in Boston and video of what her seizures look like now are on youtube. Let me know if you would like the link.

Daddy Reading Pat the Bunny

The EEG machine with Audio and Video recording. You can see the little picture of her in the upper right hand corner. The squiggles are the activity being recorded in her brain.

Our view of Boston if you looked left.

The sign that says not to swear Daddy.  I also was recorded because I ended up crawling into the crib and slept with her because the cot was like sleeping on the floor.

 

Keira's 9 month check up

Went really well.  She is developmentally right on track, if not ahead in some areas.(YAY)  Her only problems were that she has lost some weight and won't bear weight on her legs. The thought is that it is a sensory issue because she has the strength, but chooses not to bear weight on her legs at all.  It is a wait and see situation that I will be all over until she does it. She gets all over the place by rolling, rotating her body and pushing off on her foot. She scoots backwards and gets stuck in furniture too. Completely normal for now, but it won't be if it does not change soon. We are supposed to ask the Pediatric Neurologist about the weight bearing too. Our pediatrician does not seem to think it is a big deal at this point.  I told her Seth walked late so it could be all his fault-haha.
Her weight is down because she is refusing bottles. Whenever she drinks a bottle she has multiple seizures and she only has them now when she drinks. Sippy cups too because you have to suck out of them. Some days we have only gotten 5ozs into her.  I have to make a call into the Ped Neuro to update her progress tomorrow and will report this, but I was also told to wean her onto a cup.  With persistence we will be able to do this (so I am told).  I don't believe it.  The kid is awake 8 hours of the day and I can't see myself now trying to get her to down 24ozs out of a cup for 4 of those hours. I don't feel like I have time to be persistant.  I will try it, but with two other older active children I am not hopeful for success. That and the fact that she does not even hold a bottle, Yeah. lol  In the meantime, I bought new sippy cups and will take out the stoppers and got the fastest flow nipples for her bottle to see if that works.
No vaccines for a long while now.  People with Epilepsy are not supposed to get certain ones and her Doctor asked if she was getting vaccines today (she said because she has too, AAP), but agreed with me that it would not be a good idea to get them for a long time. Her system is too sensitive.
It feels good knowing that she is ahead of the game cognitively given all the challenges she has had so far.  SO, good appointment.
Now off to try the fast flow nipple. :)

Low Blood Sugars and Seizures.

are making for not quite sleepless nights, but almost.  Keira's seizures seem to be getting better. We don't see any spontaneous ones lately, they are all brought on by stimuli or stress. For example, I gave her a bath this am and she had four seizures because she was scared of the running water and of getting her hair rinsed out.  After a while she was fine, but we want to minimize these for her so we are laying low. If she gets scared she has a seizure, if she is touched a certain way when tired she has a seizure and if she drinks a bottle she has major seizures. Major seizures don't seem to come anymore unless drinking her bottle, which is not optional.  She also always has more the more tired she is, but like I said, they are reduced. Anywhere from 50-70% on a given day. This is a GOOD thing. It means the meds are working.  We start taking a full dose tonight so we will be watching her like a hawk this pm-am. We try to make sure Keira gets her naps and does not get over tired, which means she does not go places very much, nor do we.(as a family)  It is making for a low key summer, but the kids don't seem to mind. Thank God for the person who invented Intex Easy Set Pools.

Saying Momma after her tubby

Naturally curly baby Mohawk

SO tired

Kailyn had REALLY low blood sugars the last two nights in a row. Last night was the worst at a 38. We get scared when she is in the thirties during sleep. Our worst fear would be a diabetic coma. She was really good at waking up during lows, but is not anymore. This means Seth or I will be getting up at one hour and two hour intervals throughout the night until she is completely back on track. I adjusted her carb ratios for night time and hopefully tonight she will be back within her blood glucose range 80-150. Thankfully, Seth can wake up multiple times a night and go to work and still function. I am also thankful for my Mommy Radar. I always seem to feel deep down in my bones that she is low.  Of course, Kailyn woke up late this am acting like a bear because she was in the 300's. Over correction.  It's quite unbelievable how blood glucose levels can effect ones mood.  Can you imagine this pretty face grumpy?

I don't want to offend anyone so this title is "Damné si vous faites damné si vous ne faites pas"

Hope you don't know french if you are offended by the phrase. lol   Anyway, Keira's Keppra med is not working. Her seizures have reduced, but not enough to make any of us feel comfortable (including Keira) Sometimes she cries after one and has refused to eat or drink because she knows that it is a trigger. The Ped Neuro is worried that she will a) suffer brain damage if we don't get the seizures under control or b) move on to the diagnosis of severe myclonic or some other Epilepsy if they don't stop. I knew that he said this was a possibility when he first diagnosed her, but we were so happy it was not infantile spasms and the MRI came out normal, any other form of Epilepsy was ignored.  We just felt relief.
At this point, she is not showing any signs of developmental delays, but she has lost skills in verbal areas. She was ahead in that area anyway. Regardless, she is still on track in all areas.  I was on top of that pre-diagnosis anyway.
The next line of medications to take on her type of seizures is Depakote.  This medication comes with a string of side effects with the most alarming one being liver failure. 1 in 1,000 infants end up with it.  He said the majority that develop liver failure have developmental delays already or an underlying metabolic disorder. It also can lead to pancreas problems (yes, he knows about K's T1D)  Knowing that our family is not favored when it comes to statistics, this makes me even more worried.  I just can't naively say, "What are the chances?" in my head, because my thought train is pretty good at predicting that ANYTHING can happen now.  Let's face it, lightening (so to speak) has struck us quite a bit lately.
After talking about it at length with Seth though, we have decided that we would rather get the seizures under control than risk permanent developmental delays. We will watch her like a hawk and get liver testing constantly if we have to. According to the Ped Neuro, he is pretty sure she has none of the underlying disorders.  So, tonight she starts Depakote. A hard core drug that is used as an anti-psychotic for bi-polar disorder etc.. too.  It's first use was for Epilepsy though. Here's to hoping it does not change our sweet babies personality, or makes her sick and gets the seizures under control.

How things are

Things are going relatively well given all the excitement that has gone on.  Keira still has seizures, but they seem to have been reduced by the medication.  I think I counted around 20 today. Much better than the 80-100 a day I was counting.  The goal is to eradicate them altogether, however.  We had to increase the dosage and if they are not almost gone within 4 more days she is going to try another one. I would rather not do this because she does not seem to have any side effects from the Keppra. We notice the seizures the most when she has her bottle and this makes her not want to drink it. Not a good thing and all the more reason to make sure they stop.  I feel like the medication is allowing her to think more clearly and she has learned to clap and say yay, gotten back the ability to say bye bye, gives great kisses, high fives and says Dada like a pro.  I have tried repeatedly to get her to say "no no dada" like Kailyn, but my efforts seem to be fruitless so far. That was Kailyn's first official sentence.  She gets all over the place by rotating her body, rolling and occasionally pushing off on her feet to reach.
Kailyn is turning out to be quite the baker and little homemaker this summer.  She is trying to earn money for a Jessie doll (she actually has, just waiting for "payday" to buy it) and can now do the dishes, laundry, organize closets and drawers and do almost any chore for money.  I don't want her to do the toilets at this point.  Her blood sugar is good overall as long as we keep on top of her. She wants to do it on her own, but she forgets or calculates the carbs wrong.  The baking throws it off, but I am not going to deprive her of the experience just to keep blood sugar levels in check.  I just need to drive home that she needs to measure out what part of the bowl she licks or to not do it at all.  She made sugar cookies from scratch all by herself yesterday. I only put them in the oven.  I thought they were great, Seth thought he had an aftertaste of baking soda.  After she baked I had her check her blood sugar and she was 360 so I corrected and she ended up 38. Sooooo, we definitely have to watch her.
Sam had a ton of fun at basketball camp last week.  He did really well for never playing before and from what I could tell he was one of only a couple that had never done it before.  He came in last in the skills challenge (mainly because he can't reach the hoop), but it did not phase him and he wants to play this winter.  He did get the ball during one of the games and ran the ball all the way to the hoop (forgot to dribble), but when Kailyn did Kindergarten basketball this is how the majority of kids did it at first.  They don't call traveling at that age. It encouraged him to know that most of the other kids had played basketball for either 1 or 2 years.  It was fun to listen to other parents talk about how their child was a basketball prodigy (insert rolling eyes here).  We are talking 5-7 year olds. lol
We are working on getting healthier as a family.  No butter or treats in the house (unless Kailyn bakes) and the peanut butter will be hidden. We are also getting rid of extended cable/dvr.  Sam got in a habit this spring of eating out of boredom and watching more tv than he usually does.  Changing our cable package will only allow for mostly boring tv(with an added bonus of saving $35.00 a month).  We are making them go outside and I am walking again.  I got a 40 minute walk in today and my leg did NOT hurt. Woohoo!! My leg hurting afterwards was a huge deterrent for me.  That and sheer exhaustion.  Now that both have passed I look forward to my walks again. The kids start swimming again on Friday and then in the Fall Sam will do soccer and I am looking into Jukado for both of them.
I almost forgot to say that Keira has four bottom teeth now and is working on her top teeth.  She is pretty good natured about it.  I still say she is the best baby ever.  I love my other two just as much as her, but she is so easy I am in awe sometimes.

Interesting Statistics

1 in 400-600 kids have Type 1 Diabetes (Kailyn), 1 in 100 kids have Autism (Sam) and 1 in 40,000 have Benign Myoclonic Epilepsy (Keira). I find myself wondering sometimes if there are any other people in the world like us. Not in a sad, this is not fair, woe is me kind of way. It is more like "Wow, what are the chances?"  There are groups on the Internet for Diabetes and Autism, but barely any Epilepsy ones let alone one for children with Keira's condition. If anyone knows how to calculate the statistics please feel free to do so and let me know the results.  I'm thinking it is in the millions. lol