It’s Complicated

FIRST member, Brittany A. was featured in the Summer 2011 issue of the Journal of Dermatology for Physician Assistants. The following is reprinted with permission of JDPA.

I define myself by my loving family, lasting friendships and passion for helping children with special medical conditions NOT by the only complicated part about me: my medical history. I rarely discuss the fact that for as long as I can remember, through every stage of my life, my body has always given me a bit of a hard time. Consider this perspective a reflective 22-year recap of what it is like to ‘wear’ two chronic incurable conditions daily. My brother sent me the song, Shine On by Eric Bibb, which I adore. The following lyrics summarize how I currently feel about life with these conditions,

My story starts on June 19, 1988, Father’s Day

I was born 6 weeks prematurely with one very noticeable difference: bright red skin. At the time of my birth, doctors were uncertain of the cause and condition that affected my skin. They sent me home to be carefully cared for by my loving, attentive family. However, when I was four months old my delicate skin developed a staph infection. I was omitted to the Children’s Hospital of Philadelphia where doctors still did not have an explanation for my rare condition. No one would have thought a clue to this complicated medical puzzle would arrive in the form it did: my brother, Matthew. He literally was a “Gift of God” born 15 months later than myself, a perfectly healthy 10 lb. baby boy with one visible difference; his skin. Doctors now knew our condition was genetic.

At Yale, we were finally diagnosed with CIE ichthyosis
I am affected with “50 cents” worth of ARCI-CIE ichthyosis (Congenital Ichthyosiform Erithroderma) while my brother has a “nickel’s worth.” CIE is a rare genetic recessive skin disorder characterized by redness, minor scaling, and heat intolerance. At the age of two, my finger swelled and my mother took me took the pediatrician and tests revealed I had juvenile rheumatoid arthritis (JRA). My JRA would hurt during intervals of medication, so daily I was more consumed with managing that pain rather than my skin. As a child I did not comprehend how much care my skin required because my mother seemingly effortlessly managed it. She took care of every doctor’s visit, bath, lotion application, scalp treatment, and vacuum clean up that my condition required for thirteen-plus years. Her daily dose of unconditional love was, and still is, the best medicine.

The unexpected allergic reactions
Each year around the holidays I always got very ill. My skin would turn the brightest shade of red, large scales fell off my body and because my skin was reproducing at such a rapid rate, I stayed under 3 blankets at all times. At the age of 5 while walking around a Christmas tree farm with my mother and grandmother hives spread to my face. It was then clear I was allergic to pine trees. My whole family invested in fake trees and I have been healthy during the holidays ever since. Although that allergy has been figured out for a few years, my list of allergies seems to grow every two years evidenced by horrific allergic reactions that happen when I least expect them. In high school, right before my uncle’s wedding, I took Sudafed for a cold and forty-five minutes later I had a fever, was vomiting, and my skin turned two shades from purple. I am sure that all of the guests were wondering if I had been burned, but only one guest (who had too much to drink) actually asked me about my appearance. During my senior year of college I came off of Accutane too quickly, which caused a painful rash to cover my body and left me confined to my room and unable to walk. My dermatologist prescribed a sulfa drug, which caused everything to get worse. I was in so much pain one day that I went over to the health clinic where the attending physician thought I had Stevens-Johnson Syndrome.

Treatments
As a child I cried any time my parents applied a lotion with acid, so it somehow ended up that my brother and I coated ourselves in plain old Vaseline petroleum jelly. Today, I prefer the Walgreens petroleum jelly with shea butter. I shampoo with T-Sal twice a week to reduce the buildup in my scalp. On my palms and feet I use Hydro 35 foam. To calm allergic reactions, I recently found that a hydrocortisone compound works if applied daily for a week or two. When I am fed up with dedicating so much time to topical treatments, I turn to Accutane. For years, my parents resisted allowing me to try the medication because they were so fearful of all the side affects.  I completely understand the dangers that come along with Accutane, but it cleared my skin for high school dances, proms and summer trips. Speaking of trips, when I studied abroad in England during my junior year of college the water completely cleared up my skin.

Turning the pain into a passion
I do have painful memories of what it feels like to be “different.” Classmates did not want to hold my hand during circle time, customers in the grocery store stared, comments of “look at that girl” floated out a crowd in Disney World, and most recently a guy at the bar asked me if I was holding a piece of sandpaper when I shook his hand. When moments like these happen, I smile, take a deep breath, and remove myself from the situation. I would rather not waste my time or energy on ignorant people. Instead I have discovered my passion lies in helping children who are also affected with special medical conditions. One day, I hope to facilitate programs for children with these medical conditions and to organize discussions about how their conditions make them feel. For many years I internalized the painful comments and became better at “covered up” my conditions to appear as normal as possible. I am not convinced this was the best way to deal with the psychological effects of “wearing” two chronic incurable conditions because when I initially meet people they can barely tell anything is wrong with my skin. However I’m currently struggling with the elephant in the room conundrum of when to address and acknowledge the only visibly complicated part about me, my medical history.  

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This information is provided as a service to patients and parents of patients who have ichthyosis.  It is not intended to supplement appropriate medical care, but instead to complement that care with guidance in practical issues facing patients and parents.  Neither FIRST, its Board of Directors, Medical & Scientific Advisory Board, Board of Medical Editors, nor Foundation staff and officials endorse any treatments or products reported here.  All issues pertaining to the care of patients with ichthyosis should be discussed with a dermatologist experienced in the treatment of their skin disorder.