The Great Purple Cupcake Project for Kendal

 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.

Epilepsy affects more than 3 million Americans and 50 million people worldwide. That's more people than multiple sclerosis, muscular dystrophy, cerebral palsy and Parkinson's disease combined! Additionally, each year in the United States, 200,000 new cases are diagnosed-45,000 of which are children under the age of 15.  Despite these statistics, epilepsy remains one of the most publicly misunderstood conditions, and misconceptions about the disorder persist.

A few months before Kendal's second birthday, she started having these strange upper body-only sharp movements over and over again -  a seizure.  I didn't think they were seizures, but I didn't know what it was.  It happened more frequently, usually when she was just waking up in the mornings and I was able to video one of her seizures.  I took the video to our local doctor who was stumped and he referred us to Primary Children's Hospital in Salt Lake City.  We were able to meet with a nuerologist there within about 2 weeks after the first seizure began.  By then, they were occuring around 10 times a day.

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.

 Her neurologist was not happy with just a reduction of seizures, he wanted them gone for good.  So after a few months on the anti-convulsant meds, he also had her on a 9 week ACTH therapy.  Wow, what an interesting summer we had that year, 2007.  Kendal turned 2 years old, started ACTH, stopped talking completely, and went into survival mode.  ACTH was a shot I had to administer every night and every morning, but not until we had driven to the local Emergency Room and checked her blood pressure.  If it was too high, we could not give her the shot.  This only happened about 4 or 5 times.  After we returned from the ER, I would give her the ACTH shot in the leg and go along with our day.
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.

The ACTH did what is was supposed to do - the seizures decreased and finally stopped.  Her last seizure was on the 4th of July 2007, Independence Day.  Since that time, it has been a long road.  She remained on the anti-convulsant drug for 4 more years to keep those brain waves under control.  She finally started talking again, but very delayed.  The anti-convulsant drug had its own side effects - decreased speech and thought processes, no sweating still, and decreased bone strength, and I am sure a few more, but that is what mattered to her.  In the fall of 2007, she was tested by the Early Intervention folks and we started getting an occupational therapist and a speech therapist in our home every week.  This happened until she turned 3, then she attended the Early Intervention Preschool.
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.

 So, Kendal is now finishing up 1st grade where she has done very well.  Her speech is amazing, although her 'Rs' are difficult (they were for me too!), she reads at a end of Kindergarten level, math is difficult for her, but doable, she is in the middle level spelling group in her class, and loves recess!  She played in the chess club this year, and that seems to be one of the activities that she can truly concentrate on, buckle down, and really enjoy.  We are not sure what it is about chess, but she loves it, can play it pretty dang well, and can sit still long enough to play a game.  She is an active little girl and will be playing soccer this spring and can't wait to start in a few weeks.  She is a sweetheart and a stinker at the same time.  She is so very willing to try to please me in what I ask her to do, she follows directions better than her siblings.  She is stubborn, oh so very stubborn, but she needed that stubborn independence to get through what she has been through. 

We are so happy we have the Kendal that we have today.  How very lucky and blessed we are as a family to have gone through hard times, knowing it could have been harder and much much worse.  How very blessed we are to have a whole and beautiful nearly 7 year old in a 7 year old body, rather than many of those that have suffered from epilepsy like she has and be a 20 month old in a 7 year old body.  She is an angel and we love her so very much.  She reminds me everyday how very blessed we are and that we can pull through almost anything.

Wear purple this week, and go make a batch of purple cupcakes for epilepsy awareness!