Homeschool Posts

Friday, October 10, 2008

National Ichthyosis Awareness Week

National Ichthyosis Awareness Week is a time when we try to help people become more aware and understanding of this group of misunderstood skin conditions. Our youngest child, Peter, has Lamellar Ichthyosis. It is a very rare condition, estimated to afflict about 1 in 200-300,000. It is a genetic condition caused by a recessive gene---it is not catching, but he will have it for his entire life.

When Peter was born, he was covered in a collodian membrane, a thick, tight, brittle "skin" that made him look as though he were tightly wrapped in plastic. His eyelids were slightly everted and he could not close them, the skin around his mouth was so tight that he could not close it, nor could he breastfeed, in fact he could barely suck through a preemie nipple. Over the next few months, the collodian membrane would crack and eventually peel off, causing fissures in his delicate skin. His entire body had to be kept well lubricated with a thick ointment applied about 7-8 times a day, to help reduce his skin cracking and protect him from bacterial infection. Clothes were a real bane to him...we usually just wrapped him in thin blankets because sleeves and things would pull on his skin and since he had to be totally undressed several times a day anyway...

After the collodian membrane came off, Peter's "real" skin slowly emerged. In "normal" skin, there is an enzyme that causes the microscopic, dead skin cells to shed constantly. With Lamellar Ichthyosis, that enzyme is either missing or malfunctioning so that the dead skin cells collect as plate-like scales. This skin is extremely inflexible and itchy. When the scales do come off, they can tear the thin skin beneath. The inflexibility of the scales can also cause fissures in the skin. (Click on the pictures for a closeup of Peter's skin) Because of this, Lamellar patients have a greatly increased risk of skin infections. We try to keep Peter's skin well moisturized with heavy ointments to help protect it, but he greatly dislikes feeling greasy all the time and often resists being creamed. We use medicated creams to help remove the scales, which are very uncomfortable, but sometimes these creams burn, and so we have to be careful. All of his creams leave residue in his clothes that even after soaking them and washing them in hot water does not completely come out. He wears out clothes even faster than most boys!

The thickness of the scales also makes Peter prone to overheating...there are parts of his body that cannot sweat, so there are many hot summer days he must stay inside.

Many people we know are not aware of Peter's condition...his face, arms and legs are almost clear of scales (he seems to have a sub-type of Lamellar the doctors call "bathing suit Ichthyosis" because it primarily affects the trunk of the body), but he still often gets stares and sometimes rude questions. I don't want you to feel sorry for Peter...he is truly a wonderful, happy child and we are very blessed to have him. And his skin is much easier to manage now that he's a little older. But I would like to take this opportunity to educate the public a little more about this little known and much misunderstood condition. For more information on Lamellar or other types of Ichthyosis, go to the F.I.R.S.T. (Foundation for Ichthyosis and Related Skin Types) website.

And please comment on this post:->

9 comments:

  1. I hadn't heard of Ichthyosis Awareness before meeting Peter, but I was well aware of the diligent attention his skin required early on. I'm glad to hear it's easier to take care of now. And from the birthday party video you guys posted a few weeks back, he's definitely a joyful little guy!

    Be assured of my prayers for you all.

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  2. Oh, and lest I forget: congrats on the pregnancy!!

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  3. Wow! I have never heard of that! I thought I had it bad dealing with my son's eczema. Thank God for good parents! You are obviously one of them!!! :)

    TOS The Crazy Mom

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  4. Josh: Yes, he definitely loves life!

    Crazy Mom: Thank you for the encouragement! I totally sympathize with the eczema, I've seen how severe cases can very traumatizing it can be for small children and sometimes a lifelong problem...you're a good parent, too!

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  5. Wow Susan! You are like super-Mom! Everything about you amazes me. Peter is so cute that I often forget he has this condition. Love, Jessica

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  6. I had not heard of Ichtyosis until now -- and I googled "celiac" + "ichthyosis" and immediately got a few hits--might be worth a try to go gluten free for a couple of months to see if it improves the itching. Genetics can be influenced positively or negatively by environment (including food).

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  7. It's a thought, and I'll definitely look into it (it won't be the first time Peter's Dermatologist looked at me funny, but I just ignore that now). Diet and natural remedies are things I'm always looking into to improve his condition, and I would do anything to help the itching, though I think a lot of it is kind of like scratching a scab:->

    However, if you take a closer look at these links, some are false hits (the two terms don't actually appear in the same article together) and some are showing that ichthyosis or acquired ichthyosis could be a symptom of celiac. "Ichthyosis" is also a descriptive term used by doctors. It simply means dry, scaly skin. So, if a doctor says you have ichthyotic skin, all she is actually saying is you have dry, scaly skin. Acquired ich is actually fairly common and can have all kinds of different causes (most of them are nutritional deficiencies that I have investigated for Peter, again his Doc thought I was crazy, but what do I care:).

    Thank you very much for your concern, it's definitely appreciated. I'm always looking for ideas. -Susan

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  8. Susan,

    Great explanation for a great child. Keep up the good work!

    ajb

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  9. Blessings to Peter!
    He's a handsome boy!
    blessings
    cheryl
    SisterTipster
    Homeschoolblogger.com/SisterTipster/

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