Lissencephaly

Most parents don't think about how or when their children will die. In the back of your mind, every parent knows it can happen, but it's not really something you think about on a daily basis. Until something happens, that makes it a daily possibility.
I can't describe how I felt when the doctor told me my precious little baby would most likely have less than 10 years to live. Although it did literally take my breath away for a moment. Can you imagine that? Being told you'll have less than 10 years with your child? Maybe 10 years sounds like quite a while to some, but to a mother ...... a lifetime is still too short with her children. He may as well have told me I only had 10 days with him for how hard it hit me, and how it made me feel. That's about how long it sounded to me.

Lissencephaly means smooth brain. Most people's brains have grooves and folds. Reese's is smooth. If you look up Lissencephaly on the internet you get a very cold, bleak, clinical outlook.
Children with this disorder have severe mental and physical retardation, seizures, muscle spasticity (super tight hard to move muscles) or hypotonia (very low muscle tone, not able to use them much or have control over them), and swallowing difficulties. Reese has all of these things.

There is broad spectrum with this disease, ranging from mild to severe. Depending on how much of the brain is smooth. Reeses brain is completely smooth, with just two little bumps of undeveloped gyri towards the front of his brain. His case was determined to be moderate to severe. Most children with this disorder seem to be moderate to severe, I haven't heard of too many with a mild case.
Many children will not develop beyond the mentality or mobility of a 5 month old.
If you look up Lissencephaly on wikipedia, it tells you most children with this die within several months of birth. Upon further investigation, you might be able to find reports of many children being able to make it into the first 2 or 3 years of life, but always coming with the hardships of severe medical problems the whole time. Like I said, cold, bleak, and not a lot of hope from the clinical standpoint.
There are also many different types of Lissencephaly that can come with a whole barrage of different syndromes causing even more medical issues for the child.
Luckily Reese doesn't seem to have any accompanying syndromes with his case. The neurologist assured me that with the advances in the medical world over even just the last five years, the life span of the children with this disorder was increasing to more like 10 to 20 years, maybe.

So the next few weeks were pretty hard. Trying to come to acceptance of this new world we were a part of. Reeses first seizures started the day after his MRI. The neurologist thought the medication used to put him to sleep for it triggered them to start. For the first week, they weren't too bad. Just one every few days. But they quickly increased to EVERY single day after about a week. Over the next short week they progressed in their length also. Finally about two weeks in he had one that lasted 15 minutes. The type of seizure he was having, although not looking too severe on outward appearances, was extremely damaging to his brain.
So although we had tried to give him some time in hopes that the little cold he had and getting the MRI medication out of his system would make them go away, it was not to be. We had  to get him on medication.
Many people think "well duh!! Why didn't you do that right away?!" And my educated defense is, seizure medication is not good for the body. It is in fact VERY hard on the body, especially one so little. But as the neurologist counseled me, it would get to the point where the seizures would be much worse for his little body than the medication would.
So he was put on an enormously high dose of a steroid (high for even an adult) to get rid of these types of seizures. Luckily it worked, and very quickly. With surprisingly mild side effects. He only had to be on this medication for a month and it was able to wipe the seizures out completely. I realize we were VERY lucky with this. This is not the case with all Liss children. Many times several different medications need to be tried before the one that works is found. Many times the children are not able to go off the medication ever, or the seizures will come back. And many times, no medication is found to get rid of the seizures, only help "control" them so they're not as violent or happening quite as often. As I said, we've been very lucky with how well Reese has done over the last 11 months.
Some people may think, well, it's only a seizure. That's not so bad to deal with right? Just lie them down, make sure there's nothing around for them to get hurt on, and wait it out. I say this because I used to be one of those uninformed people who thought I knew everything.
People can die from having a seizure. Many times liss children will stop breathing. Did you know you're not supposed to do CPR on a person who has stopped breathing that is having a seizure?
I learned about that quite quickly. Also, because of the excess phlegm problem and difficulty swallowing due to complications of this disease, Reese would gag, then choke, then stop breathing sometimes while having a seizure. Do you know how hard it is to try and suction thick gooey phlegm from deep in a babies throat while their having a seizure and their head and chest are jerking around?
I'll tell you IT'S NOT EASY! Not at all, not even a little bit.

So through these hardships of the first few weeks, adjusting to our new life I learned something very poignant. It came to me kind of suddenly, in the midst of my fears and incomprehension. And that ..... will be my next post. About my little Angel here on earth, Reese.