Evan's Skin

When people see Evan, many say that his skin looks great, including his Dermatologist. About 95% of the time they do not notice that he is scaly. There are many reasons for this and the number one reason for it, is his skin care. A few other reasons are that his scales are white and he does not have the most severe form of Harlequin Ichthyosis. I was told by one of his doctors that since they found his mutation at the end of the protein, it was likely, that it was a milder form of HI. And if the mutation was found in the beginning of the protein it would be a more severe case. (Don't quote me on that.. still have A LOT to learn in the genetics department.) Also, Evan was born at 30 weeks 5 days. I can't imagine how much MORE skin would grow if he was born 9 weeks later. He started receiving skin care when he was 31 weeks old. I see how much skin he grows in one day so I can't imagine another 9 weeks of untreated skin manipulating his features, digits or the tightness of his chest.

Things obviously happen for a reason. Evan fought and thrived through his NICU days and continues to thrive in his own way. Though, the help he has been given from his Mommy and Daddy are helping to contribute to his success (not to talk myself up or anything ;-p). Joe and I work very hard on skin care. We have been consistent with our routine and Evan has been cooperating, for the most part. We have been very, very, very lucky that he has not gotten a skin infection (knock on wood) and has only had two mild eye infections. Evan still tolerates the 40-60 minute baths with exception to exfoliation of his hands or feet. He will relax for the most part, play with a toy, splash his feet and hands, giggle, babble or sign along to songs. Once the hands are scrubbed he will fuss, whine or even scream. And once you let his hand go, he smiles and goes back to what he was doing. He'll react the same way when we scrub his feet. His hands and feet are very scaly (very scaly to me, is when I struggle to get it off since it is thicker than card stock). Not being able to properly exfoliate his hands or feet due to his intolerance allows for quick build up. We have been using Tazorac (topical skin retinoid which helps breakdown scale) on his palms and feet a few times a week when necessary.

Before a bath & not even that scaly

If I didn't have Joe, Evan's feet and hands would be even worse. Joe ignores the fussing and I help distract Evan so he can exfoliate these areas. Usually, Joe will not stop until his feet and hands are 'smooth'. We obviously will not let exfoliation really upset Evan. If he is actually crying we will stop to avoid making it a completely negative experience. Not only is Joe great during bath time he is a constant picker. He seems to have an addiction with peeling scale. There are obvious pieces that will come off easy with no harm. But there are pieces that I would not dare attempt and Joe does. Evan doesn't fuss because it probably feels good to get the tight & itchy 'flakes' off. This picking contributes enormously to the quality of his skin but does have its risks. Besides moisturizing, descaling is the next most important thing.

During a bath splashing away

We use A LOT of Aquaphor (healing ointment). We are fortunate that the company supplies us with 50% of what we use. It is amazing that this company helps those with Ichthyosis by providing a case every three months (read more about that here). When Evan was little, it was enough but now we will go through at least two cases every three months, which averages out to at least 2 jars a week. I can't imagine having to pay for all of that ourselves. Having that reliance from this company is a major relief. I use a lot of Aquaphor because it is the only thing that keeps his skin moist. If Evan's skin is left dry it could easily break, rip or tear leaving him susceptible to infection. Lotions are not effective for Evan. Seconds after putting it on was as if you never put anything on his skin. He sucks it up like a sponge and then its gone. So things are pretty sticky around here! Evan obviously likes the Aquaphor since he acts like goof ball during applications and diaper changes. It makes a mess and is hard to clean up but it is helping to keep my son alive so I can deal with the greasy clothes, toys, blankets and smudges around the house. But it will be interesting once he is motor!

After a bath chillin' out

The only time I let Evan get really dry, is before a bath. Trying to bathe him with a fresh coating of Aqauphor on is near impossible and a waste of time. Once he is dry and very flakey we are much more successful with exfoliation and he is able to easily soak up moisture. We are lucky that Evan has been so well behaved with baths and skin care and I hope he continues to be tolerant. I have been holding off on compound creams or other types of retinoids (which help with thick scaling) unless is it absolutely necessary. I cannot imagine Evan putting up with all of the scrubbing when he is at an age where he'd rather play. So when exfoliating or descaling is unsuccessful we do have options to help with it.

Being home with Evan has been a huge benefit for both of us. I am fortunate to be able to take care of Evan, participate in all of his therapies and be in control of his skin care and feedings. I feel most comfortable being responsible for his complete care. In less than a year he will be headed to preschool where he will receive his therapies. This stresses me out beyond belief. I know I will eventually have to let go and let someone else care for him. I just hope that when we make that step towards preschool he will be communicating better and will be moving around more on his own. We have a lot of goals to reach and achieve in the next 10 months and I am optimistic that we will succeed.