This week is purple cupcake week. If you see a purple cupcake for sale at your favorite bakery, please buy it and smile. It is The Great Purple Cupcake Project, a sweet epilepsy awareness initiative this week. It is an effort to increase awareness about epilepsy, a condition that affects over 3 million Americans, including our 6 year old Kendal.
She was diagnosed with "infantile spasms," a severe form of epilepsy. Infantile spasms is one of the “catastrophic childhood epilepsies” because of the difficulty in controlling seizures and the association with mental retardation. However, early recognition, a careful diagnostic evaluation, and proper treatment may allow some children to attain seizure control and to achieve a normal, or at least much improved, level of development.
Most children are diagnosed with infantile spasms between the ages of 4 and 8 months old, and Kendal was 20 months old when the seizures began. That is a good thing, as 70% of infantile spasms cases never mentally progress beyond the age when the seizures began. Unlike treatment of other seizure types, there is only one goal for treatment of infantile spasms: the complete control of spasms. If spasms cannot be controlled, the child is unlikely to do well developmentally, and even a 90% reduction of seizures does not provide for this possibility.
Kendal was put on a anti-convulsion medication which reduced the seizures from about 17 or 18 a day to around 9 or 10. I was grateful as a parent, as I read up on infantile spasms and realized some kids never get any better at all. The seizures usually occur when the child is tired or just waking up, and sometimes, that is how I knew Kendal was awake in the morning and awake from her naps - I could hear her banging into the wall in her bedroom on her bed when she woke up while having a seizure.
|Kendal in March of 2007, my happy sweet girl about a week before the first seizure.|
The anti-convulsant and the ACTH had some crazy side effects - high blood pressure (obviously), she couldn't sweat and could overheat very easily (makes for a hard summer!), her immune system plummeted, horrible kidney stones, increased appetite, no speech nor emotion, and ACTH has a prednizone effect, so she ballooned right up. I am sure there are a few more, but I must have blocked them out!
She stopped talking completely, and for about 6 weeks the only sound she would make is when she would waddle over to her high chair, and shake it while whimpering - telling me she wanted to eat. I really don't even recall her crying during this time. She was miserable, and looking back at these pictures, makes me realize just how crazy and uncertain life seemed to be then.
What a good experience it all was and how grateful I am to have the resources available to us to help our little Kendal. She attended all day Kindergarten last year and did very well, considering the statistics.
Infantile spasms is associated with a significant risk of mortality and morbidity. Riikonen, an infantile spasm specialist, has followed 214 infantile spasms patients for 20–35 years and has accumulated the best long-term follow-up studies of these patients. In her series, nearly one third of the patients died during the follow-up period, many in the first 3 years of life. Eight of the 24 patients who died by age 3 died of complications of therapy with ACTH. Of the 147 surviving patients, 25 (17%) had a favorable developmental outcome with an IQ of 85 or greater. Eleven others were in the dull–normal range, with an IQ of 68–84. Thus, of the 214 patients diagnosed with infantile spasms, 31% died, 45% were retarded, but 24% had a reasonably favorable outcome.
Wear purple this week, and go make a batch of purple cupcakes for epilepsy awareness!