Lissencephaly

The other day I mentioned that Emmett had lissencephaly and Josh said, "I thought he had cerebral palsy".  It surprised me that even my own kids don't know what Emmett's diagnosis is. We tell people he has cerebral palsy because, well, he does, but it is because of his main condition.  So, I thought this blog might be a good way to educate our people about Emmett and help you get to know him better.  

Lissencephaly, which literally means smooth brain, is a rare brain formation disorder characterized by the lack of normal folds in the brain.  It is caused by defective neuronal migration (the process in which nerve cells move from their place of origin to their permanent location.)  Causes can include viral infections of the uterus or the fetus during the 1st trimester or pregnancy, or insufficient blood supply to the fetal brain early in pregnancy.  There are also a number of genetic causes of lissencephaly.  We don't know what caused Emmett's lissencephaly --but we do know there were some abnormal things about the pregnancy.  

Symptoms of the disorder may include unusual facial appearance, difficulty swallowing, failure to thrive,  sever psychomoter retardation, muscle spasms, and seizures.

We feel extremely blessed that Emmett is quite a healthy boy--he takes just one medication regularly (to keep him regular) and that's it.  He had a few seizures when he was two and three but has been seizure free, aside from his little "zoning out" episodes that we think are seizure activity, ever since.  He can't do anything physically for himself, doesn't roll, sit, or crawl.  He doesn't say words, though he does communicate using his voice and his eyes--we definitely know he has opinions and a sense of humor.  He eats pureed foods by mouth and he has a feeding tube to supplement his fluid intake and for medicine when he's sick.  He's had his share of sickness and surgeries but overall he is pretty healthy.  

We don't know how long he will be with us.  Life expectancy is different for every child.  Most end up dying from respiratory illness so it is always  important for us to keep him well.  He is such a sweet and pleasant boy.  His (and my) favorite thing is to cuddle and kiss.  I kiss him on his soft cheeks and then it's his turn to kiss me and we go back and forth.  I could do that all day long.  He is 8 years old and in the third grade and spends most of the school day in his life skills class but goes to a regular 3rd grade classroom for various activities throughout the day.  He is a very smart boy.  He has an excellent teacher who pushes him to succeed.  Currently he is learning to tell time and count money.  

Having Emmett Dean has changed the dynamics of our family dramatically and added mounds and mounds of stress and responsibilities on our shoulders.  But we wouldn't trade him for the world.  He is our Mickey-Dean, our Meeto-benini, our sweet Deano, String Dean, Deano-magoo.  Three words to describe his personality:  patient, silly, gentle.