I’m not really sure why Noah, our four year old, is wearing a helmet … although, it would probably be better for him if he wore one more often! I blogged about Noah’s biopsy here and here for those who need a recap of the buildup to the biopsy. We got the results last week but it’s taken me a while to blog about it – I think I’m still processing and dealing with a lot of disappointment and worry.
The explanation is that he has morphea scleroderma, an incredibly rare disorder that literally changes the skin on a cellular level, transforming it into something thicker and harder. By rare, only 5000-7000 kids will be diagnosed with forms of scleroderma this year; only something like 25 out of a million people (all ages) contract it. Very little is known about it, there is no cure, and it will get a lot worse before it gets better. How it has happened with others is that the skin will slowing transform (for him, most likely where it is red, meaning from above his eye, down his nose and into his nostril) into a thick, white, scar like skin before (maybe) changing back. This process will literally take years and years, which does have a lot of impact on children in particular who are not emotionally or psychologically prepared for this kind of issue – especially where it’s on his face and he rarely goes out in public without someone commenting on it – usually strangers! To say it’s disappointing to find out it’s a condition that doesn’t have a cure, will most certainly get worse before it gets better, and in the best case scenario will take years to heal (most likely leaving scars), would be an understatement.
Adding to the stress of it, though, is that it’s one of the two forms of scleroderma. He has the surface form, which in theory only impacts skin – but not much is known about it still. The other form, systematic scleromderma, does the same thickening and hardening transformation on the inside of the body – effectively shutting down organs and blood vessels, and it is always fatal. It can take twenty years to kill someone, but it always does because there is no cure. Very little is known about it as well – those earlier numbers I quoted include all forms of scleroderma, not just Noah’s. It’s a shock for us, though, because my grandmother died of the systematic scleroderma a few years ago. Because of her age (it normally strikes people my age, not children or elderly), it only took a few years to take her life, but the memories of it hitting her and her suffering are still very fresh for our family so hearing any kind of diagnosis that includes the word ‘scleroderma’ is scary – even though the doctors keep assuring us that people only get one or the other (not exactly accurate; about 1% of victims of scleroderma get both kinds … so the potential is there, but incredibly slim odds). It is not supposed to be hereditary (although it hasn’t been proven), but there is thought that there could be a genetic susceptibility that could be passed on. Noah’s recent heart arrhythmia diagnosis also has us worried because that can be a symptom of the systematic version.
All that being said, we’re going to get him to some specialists to hopefully rule out the more serious version for our own peace of mind. It’s not the answers we were hoping to get, and we haven’t really spoken about it with our kids – he hasn’t been diagnosed with what my grandma had, so we’re definitely not putting those thoughts into our kids’ minds. We will have to have the conversation with Noah that it’s going to take a while to heal, but he has a good attitude. And prayers for Noah and our peace of mind are definitely welcome!