From the Patient's Perspective
From The Patient’s Perspective
Journal of Dermatology for Physician Assistants (JDPA)
By Kelsey Julian
When my son Ian was born, I had no idea what it meant to be diagnosed with ichthyosis, a genetic skin disorder characterized by dry, scaling skin. As a new mom, I couldn’t help but wonder how this might impact his life and the day-to-day routine of caring for my child. During his infant and toddler years, we were living in South Eastern New Mexico and the dryness in the air was having a severe impact on Ian’s skin. Every aspect of his care had to be considered and, unfortunately, that meant a lot of trial and error. We were struggling with his skin regimen, especially finding diapers that didn`t cause awful rashes. Ian would constantly get methicillin-resistant Staphylococcus aureus (MRSA) in the diaper area and the routine skin care and dealing with infections was beginning to drain me.
I eventually learned that Calmoseptine® Ointment created the best barrier to protect his skin from diaper rashes and using dye and fragrance-free diapers also helped. In the warmer months, allowing him to go without a diaper when MRSA was not present kept him healthy too. In my attempt to find the best products and strategies for keeping him comfortable, I quickly discovered that Aquaphor® Healing Ointment worked in improving his skin and was essential to his care regimen. I would go through almost two jars of it per week. We ended up moving from New Mexico to my hometown in Texas and while this change in environment helped to decrease the amount needed to manage the ichthyosis, the financial burden of it all was noticeable, especially during those early days.
Ian was a round one year old when I saw a pediatric dermatologist. She told me about the Foundation for Ichthyosis & Related Skin Types (FIRST) and encouraged us to research it. I am so thankful she did because that is when I felt the floodgates of support open. If it wasn’t for FIRST, I would have never known about the wealth of resources available, like a program offered by the manufacturers of Aquaphor that sent care pack ages to families who use large amounts of their products due to chronic skin issues. Qualifying for this program was truly a weightlifted off our shoulders. I remember getting our first case in the mail. It felt like Christmas. When I saw how many containers were in the box, I wept tears of joy.
Looking back, I realize that the challenges of managing ichthyosis extended beyond Ian’s skin regimen. He was considered under-weight, an issue brought up to us by pediatricians and professionals from The Special Supplemental Nutrition Program for Women, Infants, and Children (WIC). We were trying everything to help him grow at a healthy rate and keep his skin under control, so it was frustrating to receive criticism from medical professionals and others outside of my circle. When out in public, complete strangers would comment about him being tiny for his age, suggesting that I should feed him more and a sked why I allowed him to get so badly sunburned. Sometimes I wanted to scream, "It's ichthyosis, look it up!
Though he protested and squirmed like most toddlers do, Ian was surprisingly tolerant of the extensive skin care routine and never showed a desire to scratch or rub off his lotions and creams. Maybe he associated early on that the lotions made his skin feel better or more comfortable. I'm not sure. I did find that applying the ointment at night, although not necessarily the most effective strategy, was extremely important. Nighttime required a heavier application due to clothes and blankets rubbing against the skin. Another consideration in his care was potential mess and stains created by using such a large amount of skin creams. I found that fleece was the most forgiving material and stocked up on inexpensive jammies at Walmart® and re-sale shops.
We did have a season where his skin went through some changes. When Ian was four-years old, his skin changed and became drier, more sensitive, and more inflamed than before. One night after a bath, I was applying ointment and he was crying. He slapped my hand and screamed, "Why do you like hurting me? It burns, mommy. Please stop!" My heart sank. I called in reinforcement and excused myself to my bedroom for a heartfelt cry. I had no idea how to explain to a 4-year old that when I apply skin products, it is out of the deepest love and care. I knew the burning was momentarily and long-term effects of not applying those products would cost much more pain and aggravation than a few moments of burning. But again, how do you get a four-year old to understand that? Thankfully, we moved past it. His skin improved and I never had to experience that situation again.
Even though on the surface Ian’s case is much milder than others of ichthyosis, he struggles tremendously with heat and cold intolerance. This is because individuals with ichthyosis do not sweat normally. The condition does not allow their sweat to reach the surface of their skin and cool them effectively. In very hot and humid weather they are at risk for overheating, heat exhaustion, and heat stroke, so we had to work hard to keep him comfortable and look for any warning signs.
Ian was around three years old when we received a gift that made it possible for him to enjoy the outdoors—a cooling vest. Some dear friends with whom we connected with through FIRST learned that Ian overheated the previous summer during Memorial Weekend when the high was only 76° F. This couple didn`t want him to go through another summer of having to stay indoors all the time. I would have never known about the cooling vest if it wasn’t for the FIRST families. Receiving the cooling vest opened so many doors of opportunities for Ian to participate in outdoor activities—going to the park and participating in things like zoo trips, Easter egg hunts, school field trips. He was even able to take part in recess and physical education class.
Since those difficult infant and toddler years, I’ve slowly been able to take a back seat and allow Ian to be responsible for managing his skin care routine. When he is really dry, he will ask for help getting his back or other areas of concern. Ian fully understands ichthyosis and the importance of keeping up with care. He lets me know when he is getting low on products and when he finds something that works well. Right now, his favorite products are Hempz Moisturizing lotion and Sweet Girl Farm Tallow Butter, which is made right here in Forney, Texas. He also understands he is at risk for overheating during exercise if he does not have his cooling vest and that clothing plays a role in keeping him comfortable. Now that he is a preteen, he mostly wears jersey net shorts, white cotton undershirts, and fleece pajama pants during the winter.
I used to think having ichthyosis would prevent Ian from being able to do “normal” childhood activities like playing outdoors and learning sports. As he grew up, his strong will and determined spirit showed me how wrong I was. We just celebrated Ian’s 12th birthday and, if we stay on track, he will be going for his black belt, which signifies mastery of the fundamentals of Taekwondo. We are so thankful to have found FIRST as their resources and support have eased so much of the visible and invisible burdens of growing up with ichthyosis.