About 3-4 weeks ago Ben started having spasms. We thought they were the startle reflex that babies have. Or at least that's what I wanted to think they were. They happened a couple times a day and just a few at a time. About two weeks ago they started to get worse and his eyes would roll the the upper left corner when it happened. I called his neurologist and asked if I could send a video of him doing it so we could see if it was anything. I was still telling myself it was nothing to be worried about. Even if it was a seizure they have medicine for that, no big deal.
This is the video I shared with Dr. T:
The neurologist reviewed the video and called me last Friday to discuss it. He felt like what Ben was having was something called Infantile Spasms. He told be NOT to Google it. Why do they say that? Once someone tells you NOT to Google something is it even possible NOT to do it? Anyway, I tried to stick to the more legitimate sites and learned some about infantile spasms.
Basically, infantile spasms are a somewhat rare type of seizure. They are often related to brain injury (like Ben's stroke) or a genetic disease called tuberous sclerosis. They happen in clusters and are more likely to occur upon awakening or after eating. This describes Ben's perfectly. They have increased over the last couple weeks and he now has them about 4 times a day and he has around 10-20 spasms per cluster.
Let me pause here and say that it is heartbreaking to hold your child while this is happening.
So, our neurologist wanted an EEG to verify what was happening. We went to Blank on Tuesday and had that done. While an EEG is a painless test, it was exhausting. Ben had 35 electrodes connected to his head with stinky glue. Then we had to soothe him to sleep because they want to watch the brain activity during sleep. Then they wake him up to watch the brain activity while awake. The goal is to capture 65 minutes total of brain activity. He had a couple of spasms while the technician was attaching his electrodes. Then, when he woke up he had about 15 spasms which is the most we had ever seen him have. After they stopped the test we had to walk around for about 30-40 minutes while they waited to hear back from the neurologist that he had all the information he needed.
This is what Ben looked like while we walked around:
Finally, Dr. T (the neurologist) called back and they had everything they needed so we were able to take the electrodes off. That may have been the worst part. They had to use acetone to get the glue off and I'm sure it pulled his hair and didn't feel the greatest.
Next we had an appointment with Dr. T but had time to grab lunch first, which Ben slept through.
We met with Dr. T and basically got no information because neither he nor the other pediatric neurologist in his office have treated this before or felt comfortable treating it so they decided to send us to Iowa City. By this time it was after 3pm so we would go the next day.
Wednesday we went to Iowa City and met with two neurologists there. They confirmed Ben's diagnosis of infantile spasms and Dr. Joshi did a wonderful job explaining what was happening in his brain and how we would treat it.
So, first I'll tell you what's happening. Babies with infantile spasms have chaotic brain waves called hypsarrhythmia. It looks like this:
(This is not Ben's EEG, this image is from Wikipedia)
A normal EEG looks like this:
Next, why is this such a big deal? Well, if untreated, infantile spasms and hypsarrhythmia WILL cause mental retardation. I'm going to pause here and say that I HATE that word - the "R" word. However, it is unavoidable and it is the clinical diagnosis for many children with infantile spasms. The neurologist yesterday, Dr. Joshi, said that there is a 5-7 point decrease in IQ for every two week period the spasms continue. Ben has been having spasms for about 4 weeks and as you can imagine I am torturing myself with why questions. Why didn't I take him in sooner. Why didn't I take it more seriously? Why was I in denial that they were seizures?
What are we doing? Ben is home, he does not have to stay in the hospital. He is being treated with a round of high-dose steroids. He is getting 40mg of prednisolone daily. There are lots of side effects related to high-dose steroids and he will be followed at least weekly to check for increased blood pressure, blood glucose and to make sure his electrolytes are not out of balance. This means going to our pediatrician once or twice a week and we will be making a trip to Iowa City every other week. It also means Ben will have to have his blood drawn at least weekly which I'm already dreading. He will undoubtedly also be irritable, hungry, gain weight, retain fluid, and have an upset tummy from the steroids.
The goal is to completely stop the seizures as quickly as possible to avoid further damage to his brain. The steroids completely stop the seizures within two weeks in 70% of patients. We are praying that Ben is in that 70%. If not, we'll increase the dose of steroids in two weeks. If in two weeks he still is not spasm free we will go to a drug called ACTH.
ACTH is Adrenocorticotropic Hormone. It is produced synthetically as Acthar Gel. It is an injectable medication given daily - we would have to give him a shot twice a day. It has more side effects than the steroids and is expensive. Like $39,000 per 5ml vial expensive. Yes, you are reading that right. Thirty-nine THOUSAND dollars. Dr. Joshi did say that insurance covers it but I shudder to think what the copay would be on that.
Where are we at right now? Well, we are trying to he hopeful. The statistics say that only 10-20% of kids with infantile spasms will be "normal" cognitively speaking. The odds are in your favor though if the child was developing normally before the spasms started. While Ben was by no means developing normally because of his stroke, his cognitive function was great and we have affirmation of that from his last visit with his developmental specialist. Ben is behind in gross motor function. He sits up but doesn't roll over, much less crawl. I haven't really shared this information before because I expect that when Ben and Ainsley are older they will read this blog and I don't want Ben to feel bad because he wasn't doing things when he should be. I may do another post on his physical delays caused by his stroke, again so I can remember and see how far we've come once we get through this.
We are struggling to be positive. We are struggling with they "why" questions. Why us? Why Ben? Haven't we been through enough already? We are disheartened and struggling to find our faith right now. It comes and goes.
I am thankful to our friends who have already reached out to us. It means so much. We welcome all your thoughts and prayers right now.