Reprinted with permission from her blog Adult Living with EB (Epidermolysis Bullosa)
I’ve decided to “come out”…….. come out to tell my history, my experience, my life of living with Epidermolysis Bullosa (EB).
Epidermolysis Bullosa is a rare genetic disease that is characterized by the presence of fragile skin and recurrent blister formation that can result from minor to mechanical friction or trauma. I am a 54-year-old African-American female; one of several older adults in the U.S. living with this disease. One can only imagine how this could have been a parents’ worst nightmare after giving birth to a child diagnosed with this disorder 54 years ago. Life was great then for Tom and Mary Hughes, having established their lives, already with two children. On October 25th, their youngest child was born in Lincoln Hospital in Detroit, Michigan, Marsha Lynn Hughes. The medical staff was CLUELESS as to what disorder this infant could possibly have as this newborn was basically one whose entire body looked like “raw meat” (as quoted by the nurses). The nurses (and the docs) were absolutely baffled by this newborn. However, they did what THEY thought was best including placing a humidifier in the room, which they felt would help relieve the blistering. The humidifier only made matters worse, however. The doctors actually felt that I would not “live long”, only because they had no clue with what they were dealing with as no one in this hospital had ever encountered this disease. My mother overheard the staff conversing outside of the room saying, “we might as well let the mother take the child home as she won’t live long anyway.” Of course, they did not know Mary!!! Her motherly instincts and true determination kicked in and here I am today educating others about the disease.
There are many factors that helped me along the way with a disease that has no discrimination to race, creed, nationality or gender. The main factor in keeping me alive was mom and dad’s strong faith in God, as they knew they worshiped a God who would never put more on them than they could bear.
My parents communicated well and worked well with one another; they ended up being married for 64 years! They discussed and agreed on how they would handle my disease, simply with love and care, no different than parents would treat any of their children. Dr. Gall, my pediatrician, explained to my parents that I would not be able to survive any major infection and they would need to take extra precautions to prevent that from happening.
My EB as a child was determined as RDEB because back then only the recessive or the dominant types of EB were known. There was very little other known information regarding different subtypes of EB.
In the 1990s, I officially had a biopsy to try to determine which form of EB I actually had, which is still determined to be an ‘inconclusive’ form of EB. The only clear-cut facts about my illness at the time of my birth was that I had a rare skin disorder called epidermolysis bullosa, which entailed blistering inside the mouth, entire body and heavy scar formation.
Thanks to incredibly strong and loving parents as well as many positive people in my life, I never really looked at myself as being “different”, especially as a child. It wasn’t until after hitting my late teens/early 20’s that I really understood that I was “different.” People called me cruel names and referred to me as that woman with leprosy, the woman with that ‘stuff’, or the “woman with those bumps”, and I could go on, but most things do not merit repeating. I realize that children can be cruel but in my lifetime, I have experienced the cruelest behavior from adults.
The main thing I must stress to anyone with EB is cleanliness and keeping your environment sanitized. Our environment must be extraordinarily clean so as to prevent infection from occurring. The medical staff suggested to my mother to bandage the blisters; however, we learned that this actually hindered the healing process. There are many who choose to bandage their wounds, but it is definitely MY experience that allowing the wounds to be exposed to air as much as possible will speed up recovery from the outbreaks. Of course, one must always be certain to be in a sterile environment when allowing the blisters to “air out.”
As a newborn my entire body actually looked like ‘raw meat.’ My mother changed my sheets 3-4 times per day with white cotton sheets, no added perfumes, etc. When at home, I only wore cloth diapers because something as simple as elastic would cause friction to my skin, resulting in large blisters. After bathing me with either pHisoHex or pHisoderm (which was the choice of antibacterial soap used at that time), my mother would lay me on clean, white sheets with baby powder to prevent my skin from sticking to the sheets and apply Bacitracin ointment 3-4 times daily. Today, I will use Hibiclens antimicrobial soap for serious breakouts of blisters. I would only be fully clothed when I had to leave the house.
Protein foods are also essential for the physical well being of someone with EB. As I had blisters in my mouth as a newborn until 2-3 years of age, my mother would give me everything in liquid form, especially meat which she would grind up in a blender; this was one of the recommendations of my pediatrician. EB can be very draining on the physical body due to the blisters and pain, along with the inability to sleep. As there is an extreme loss of energy while enduring the pain as the result of an outbreak, the body requires as much protein and iron as possible for it to continue to thrive. Staying physically active is also a necessity as joints, extremities and muscles could stiffen up and toes could sometimes web together.
As far as emotionally, I never allowed myself to feel as if I was a victim. I was ALWAYS a rambunctious child. I had always known there was someone out there worse off than me. I actually felt that God chose me to have something different than the average individual because I am strong and can help others be just as strong, no matter the circumstances.
Though I faced many challenges as a youngster, a teen and mostly as an adult, I have lived and continue to live a productive life. I’ve been married and the mother of 3 sons (only one of whom has EB).
Even though there is still not a cure for this disease, many children growing up CAN live to adulthood with EB and have a successful life with a career and family. I am writing this to encourage others with EB as well as as those who have loved ones with EB. Many with EB can live a productive life; just educate yourself, always have high self-esteem and be strong. It can be done; I am living proof of that!