Hi there, my name is Ada and I am the mum of Sama, my beautiful 5 year old boy who has started in Carlow Educate Together school this year. Sama was born with a very rare genetic skin condition called Bullous Congenital Ichthyosiform Erythroderma. Bit of a mouthful that, but I could spell it backwards for you at this stage! It’s not contagious so you can’t catch it.
Sama has thickened scaly skin all over his body that causes him to stand out from the crowd. This is because, due to a defect of one of the proteins in his skin, his skin grows 200% faster than normal and also tends not to shed, which results in a “traffic jam” effect of the skin on the top layers of his body. This causes Sama pain on a daily basis (of which he rarely complains) due to the tightness of the
skin especially around joints. His skin blisters really easily as well so minor trauma like wearing most shoes, or getting hit by a football or knocked into by another child at play can cause him lacerations on his body and leave him in severe pain and stinging when the air hits the open wound. This is due in part to the fact that the top layer of skin does not attach to the bottom layers, so he has sliding effect of his skin upon relatively small traumas. He needs dressings and pain relief for this.
I have to lance blisters on his body with a sterile needle on a regular basis and he is so so good, he tolerates it so well because he knows it will feel better soon after. Due to harmless bacteria trapped between the skin particles, there can be an odour from his skin at times. This is controlled as much as possible with medications and daily bathing, but cannot always be eliminated. Sama is heat intolerant and CANNOT SWEAT at all because of his thickened skin blocking the pores for the sweat.
This makes it hard for Sama to participate in sports without having to stop and take a drink or just stop playing altogether especially if the weather is mild. He tends not to stay in the sun at all due to this. He also loses water through his skin as the barrier function does not work very well, so he always needs to drink fluids to stop dehydrating.
Sama does not sleep well at night because of the severity of his itching, but he still always manages to smile and crack a joke. He just loves life so much, he makes me feel ashamed sometimes for the little things I moan about, though I tend to do that less now, and he inspires me every day with his brave witty and happy nature. He makes me laugh so much all the time. I was telling him the other day that his cousin Isla is coming home from New Zealand soon and that she will be one in a few weeks to which he replied “does that mean she is no age?”.
So when you see my little boy don’t stare at him, he would love it if you went up to him and said hi, he is very social and loves to chat. I wanted to write this information so that you can understand why Sama looks like he does, and also so that you can explain this to your children so they too can understand. And answer any questions that they ask as honestly and age appropriate as possible.
I encourage you to watch the following video link with your children as it is very informative and was made by children with Icthyosis for other children so they could understand it better.
Also check out www.scalyskin.org
as these will have a lot of very informative articles etc to help your understanding.