Thursday, October 8, 2009

National Ichthyosis Awareness Week 2009

For those of you who don't already know, our youngest son, 4-year-old Peter, has a congenital skin condition known as Lamellar Ichthyosis. This is a rare and little known or understood condition that can sometimes make it difficult for people who don't know him to see past the surface and become acquainted with the sweet, loving, energetic boy that he is.

Peter is a happy, healthy boy who loves to do all the things other boys his age do, like riding his bike, playing on the playground, running, jumping, sliding, and swinging. He loves to draw dinosaurs and dragons, and to sing songs by the Beatles. He loves books, especially the original Winnie-the-Pooh stories (Eeyore's his favorite). He loves building (and tearing down) Lego. He loves his brother and sisters.

But Peter has some physical challenges. In "normal" skin, there's an enzyme that causes dead, microscopic skin cells to shed constantly. If you have Lamellar Ichthyosis, that enzyme is either missing or malfunctioning. The result is thick, scaly, inflexible skin. It afflicts a significant portion of Peter's body, including his trunk, neck, scalp, forehead, hands, insides of his ears, and the insides of his elbows and knees. In addition to being itchy and uncomfortable, the scales are stiff and can cause new skin to crack leaving him open to risk of developing skin infections. He constantly scratches, leaving a trail of skin flakes behind him, and his skin raw and vulnerable to injury. He has trouble sweating, making him prone to overheating in the summertime. The scale in his ears affects his hearing to some degree and he has to visit an ENT (Ear, Nose and Throat) specialist every few months to have his ear canals cleaned out.

Peter wears heavy creams on his skin to protect it, help maintain some flexibility, and reduce the itch. The creams leave him feeling sticky and greasy (his clothes stick to him and any gnat that alights on him is doomed). Regular adhesive bandages fall off. Some of his creams are medicated to help remove some of the scale and they can burn. None of his creams completely wash out of his clothes, even in a hot water soak and wash. Yard sales and clearance racks are a wonderful way for me to keep a constant supply of new clothes for him as the old ones wear out every few months.

Our family is truly blessed by Peter's presence. He's an amazing little boy and I have a feeling he's going to grow to be an amazing young man.

This post is my annual update on Peter's condition in honor of National Ichthyosis Awareness Week. You can read last year's post with closeup pictures of Peter's skin here. To find out more about Lamellar and other forms of Ichthyosis, please visit F.I.R.S.T. (Foundation for Ichthyosis and Related Skin Types).

4 comments:

  1. I had never heard of this, thank you for informing us.

    Our son suffered a little known birth injury, "Erb's Palsy" so I know what it is like for others to not get it. We have been blessed in that Nicholas has made an excellent recovery and it is now barely noticeable, but many children do not have as positive an outcome.

    Praying for blessings for you and Peter today.

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  2. Kimberly Kovach-TOS CREWOctober 9, 2009 at 2:46 PM

    Poor little guy. Never heard of this before. Thanks for enlightening us.

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  3. Thank you for sharing and raising awareness of this life changing skin disease.

    As an NICU RN, I will never forget the first time we had a little one born with Ichthyosis. Unlike Peter her outcome was not as positive. It also affected the lining of her lungs.

    As you know infection is a huge issue because our skin is our first line of defense.

    Peter is blessed to have you as his mother.

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  4. Thank you everyone.

    Linda- yes, it's truly heartbreaking when these little ones are born. Peter's first year is kind of a blur, full of uncertainty, full of his physical pain. We are blessed that he has never developed a serious infection. We try not to relive those days too much---pictures from that time period are hard to look at. It's taken a lot of healing...

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