27 May 2013

Ichthyosis Awareness Month: Courtney and Harry's story ~ "I am proud of how good looking my children are. I can’t bear that some people only see Harry's ichthyosis and not how beautiful he is and won't ever know what a sweetheart he is."

Courtney is an Australian mum to Harry, a little boy with Ichthyosis. Harry loves ball sports and has a gorgeous smile. Courtney worries what the future will hold for Harry, and I have told her that while life with Ichthyosis is hard, it does get better. Harry is pictured below with Courtney and his twin sister.

"Harry is two and a half, he has EHK/EI Ichthyosis. It turned my world upside-down... I had wanted to be a mother for years and finally I was not just pregnant, but carrying twins. It was a perfect pregnancy, the scans and tests came back spot-on for healthy babies. In the end though, after twelve hours of a breech-breech labour that wasn't progressing, I had a c-section and was told "you have a daughter!" She was checked over and put on my chest, perfect. Next I was shown enough of my son to know he was a boy then he was whisked away. A hazy voice told me that there was something wrong and that he needed extra attention. The next words I heard floating around the theatre were "glad wrap"... Why were they glad wrapping my baby?

The longest hour or two later I was finally allowed up to NICU (neonatal intensive care unit) where (I'm told) I asked "appropriate" questions. I didn't remember going there that night until I saw photos of it a couple of weeks later.

My son looked like he had been boiled (and it took a long time, and a visit to the genetic counsellor, to stop blaming myself for taking such hot showers during pregnancy... if only it was that simple!); like a huge blister had covered his torso and had popped, leaving red raw fragile skin over a lot of his body; with other areas seemingly covered in a thin membrane or extra layer. I later read that babies with EHK/EI have been described as "enfant brûlée", from the French for "burned child", quite a fitting description.

Over the four weeks he stayed in NICU we started to learn to care for his skin, but in choosing to avoid the internet for that time, our knowledge remained limited. Harry was a couple of months old when, at a dermatology appointment, we were told he had Lamellar ichthyosis ... the same appointment at which he almost managed to wee on the dermatology registrar. The same registrar who had removed his newly applied head to toe dressings back in NICU (a very long and distressing process to apply) and then just left him like that after an unannounced examination - Go Harry, good try!

After changing hospitals and finding a dermatologist we preferred we discovered that he actually has Epidermolytic ichthyosis (EI), previously (and still to us) known as EHK or Epidermolytic hyperkeratosis. What that really means is that his skin is fragile and prone to blistering but reproduces significantly faster than it can shed (possibly up to 200x faster than normal), which results in thick, hard scales.

He spends a lot of time in the bath to soften and remove excess skin and we apply acid-based cream to exfoliate and moisturise (no, the one we use doesn't sting). It doesn't sound too bad, but try and explain cause and effect to a two year old ... if you have your five minutes of cream, you will feel better all day. When half an hour of crying twice a day happens most days, when before he even gets in the bath he begs not to have creams, no matter how you approach it, your heart aches. Harry's twin finds it distressing to see him so upset too. So I compensate, I try to make them the most kissed and cuddled, most fiercely protected babies in the world; but they also have occasional days when they are the ones to say, "Stop crying Mama, you ok, you be happy now".

I admit to a certain level of vanity, and am proud of how good looking my children are. I can’t bear that some people only see Harry's ichthyosis and not how beautiful he is and won't ever know what a sweetheart he is. It is a strange experience to see the "normal" reactions and comments that people make towards his twin, compared to the hesitancy as people decide which approach to take when meeting Harry ... say nothing, pity, empathy, assumption, curiosity, "helpful advice."

One of my biggest fears is school ... will they look after him? He overheats within minutes, how will a teacher keep him safe and make sure he doesn't get potentially deadly heatstroke? Of course the other fear is bullying, exclusion, seeing his gorgeous heart saddened if he is left out because of something that is no fault of his own.

We've already encountered some mean children and had some parents "subtly" remove their own kids from where he was playing; but we have also met some brave children who visibly brace themselves and allow him to hold their hand or hug them even though they find it confronting. And some really sweet children who take him under their wing and encourage and play with him, concerned only that they might hurt him. But what happens when he isn't little and cute anymore? Well, he is intelligent and if I manage to do my job as a parent and foster his compassion, his empathy, keep his incredible interest and enthusiasm alive, as well as help him develop skills and interests that will help him make friends, then I can only hope he'll be OK.

Harry's dad played a lot of sport growing up and always talked about coaching our kids and which sports he hoped they might get involved in. Harry loves anything that involves a ball especially golf, cricket and soccer; as well as riding his scooter and going to kindy-gymnastics; he is a natural sportsman. What slows him down is sore skin, blisters, skin grazes/tears and of course the fact that he doesn't perspire. When it is much over 22C or he is running around for more than a couple of minutes he wears a neck cooler and we wet him down. Just recently we bought him a cooling vest that has special icepacks all around his torso, he'll get a new one every year or two as he grows. I don't know how much sport he will be able to play, but he loves it so I'm sure he'll find a way. EHK skin loves water so here's hoping he is a keen swimmer or even a surfer... at thirty-six I've just started surfing lessons, so if I can I'm sure he can!"

(Harry and Courtney with former Australian cricketer Steve Waugh)

This post is part of the Ichthyosis Awareness Month project. For all posts in this project, click here.

For Ichthyosis and appearance diversity resources, click here.


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