Infantile Spasms

Infantile Spasms…

I have read so many stories online about how other parents noticed something a little off about their child’s jerky movements but thought that maybe it was just a normal, wacky thing or in my case I thought it was because Thomas has jerky movements anyway due to his increased muscle tone.

I noticed a repetitive jerky movement that just wouldn’t go away and at first I thought that maybe it was that he was turning around to look at something he heard. After about a month of this repetitive movement I started to notice that he would jerk quite involuntarily and it didn’t seem deliberate at all. Often it would happen during feeding or when lying down. It was this strong involuntary jerk and the deviation of his eyes that made me think that perhaps it was what I thought it was but what I’d dread it would be. Infantile spasms or otherwise known as West Syndrome. I had seen many videos on YouTube because I was looking up info on seizures. I had read that Infantile Spasms were an uncommon seizure disorder with devastating results leading to possible loss of acquired milestones, autism, developmental delays and other forms of epilepsy. The prognosis was especially not positive if there was already an underlying developmental brain disorder or injury which is true in Thomas’ case.

Diagnosis

Thankfully, the diagnosis was fairly straight forward. Unfortunately the lead up was extremely stressful. Thomas had a long episode of infantile spasms followed by a prolonged tonic-clonic seizure. This eventuated in an ambulance ride straight to Starship hospital where we waited in ED. Not long after arrival, Thomas presented with more spasms and seizures requiring sedation. That night, Thomas required sedation 3 times. We remained in resus for many hours before being admitted to the neurology ward. The next day Thomas had an EEG (Electroencephalography) which confirmed Infantile Spasms.

Treatment

From what I’ve read, the two most common treatments include:

• Steroid therapy (adrenocorticotropic hormone [ACTH by injection] or prednisone)
• A seizure medication called Sabril (vigabatrin)

In Thomas’ case, we could not treat with Sabril because of his eye condition, CVI. A possible side effect of Sabril is permanent loss of peripheal vision. With CVI, Thomas predominantly uses his peripheal as his preferred visual field. Yup, this meant steroid therapy. Prednisone.

I HATE Prednisone! It is horrible horrible stuff. I was not provided with any information to read about steroid treatment from the hospital but just a quick verbal rundown from the neurologist. I have hunted on the net to find out about other parents’ experiences with the steroid treatment and couldn’t find much! This is one of the reasons I’m launching this blog.

As part of Thomas’ treatment, he receives a high dose of prednisone for a number of weeks. The side effects kicked in within days. He changed from being happy, smiley, extremely good sleeper, set amount of routine feeds, talkative bubba to:

• No smiles or talking
• Insomniac requiring an additional hospital stay. He was sleeping 3-5 hours a day and that was broken sleep.
• Irritable, grumpy, sad, moody, crying sometimes non-stop
• Hungrier and fussier
• Clingier – wanted to be held ALL THE TIME!

The good news is that Thomas’ spasms stopped occurring after a few days. We have been told that they can return with a vengeance but we live in hope that they stay far far away!

The other good news is that when Thomas began to be weaned, gradually, off the Prednisone, he slowly started to become less of all the above nasty side effects. However, the sleeping is still questionable.

So Thomas required an additional hospital stay to be treated for his insomnia. It was so so bad. The poor wee man was in quite a state and between my husband, my mum, and I, it was still really hard. I stayed with Thomas in hospital for as long as we needed to trial how Thomas responded to a stronger sedative than melatonin. The melatonin did work but unfortunately it does not sustain sleep. Thomas would get 1/2 an hour to 2 hours sleep max from the melatonin which was just not enough in the end. Thomas responded really well to the stronger sedative, chloral hydrate. We were able to take some home for the week. Unfortunately, it doesn’t last as you can build an immunity towards it.

What I haven’t mentioned is that the steroid treatment leaves Thomas with immunosuppression for up to 6 months!