Benjamin Joseph Wiley, Jr. was born on Oct 27th, 2011. Though it had been a normal, healthy pregnancy and delivery for Ben and his mom, it was immediately clear that something wasn’t right: baby Ben was missing the skin on his hands and his left foot, and had blisters in his mouth and on his lips. Doctors at the DeSoto Memorial Hospital in Arcadia, Florida where he was born had never seen a child born like this before, and did know how to treat him. Baby Ben was immediately rushed to All Childrens’ Hospital in St. Petersburg by ambulance, since the wind was too strong for an emergency flight.
Teams of doctors worked around the clock to treat Ben at the All Childrens’ Hospital NICU. He had to have special diapers, special treatment of his wounds… it was hard to find anyone who knew how to care for him. He was fed through his belly button until his pain could be controlled enough to give him a bottle, and even then the bottle nipple had to be covered in aquaphor/vasoline to stop blisters from forming. A layer of Aquaphor was kept all over his body in order to prevent any friction against his skin, because any friction against his skin at all would shear off the skin or create a blister that had to be popped and would leave a severe wound. He was the only baby that the hospital had seen born with his condition.
Ben Jr. was seen by over 19 doctors (plastic surgeons, GI specialists, dermatologists, skin teams, radiologists, a respiratory team, and neonatologist). They took skin specimens and on Nov 8th doctors diagnosed him with Epidermolysis Bullosa (EB). In a nutshell, EB is a connective tissue disease causing blisters in the skin and mucosal membranes, with an incidence of 1/50,000. Its severity ranges from mild to lethal. It is caused by a mutation in the keratin or collagen gene. People with EB lack these protein anchors, so when there is any friction on the skin, the two layers rub against each other and separate, resulting in painful sores and blisters.
In it mildest form the blistering is confined to hands and feet. In its severest form the whole body is affected, wounds heal extremely slowly, there is significant scarring, physical deformity, and disability. In its severest form EB is fatal in infancy. People with severe EB have a significantly higher risk of developing skin cancers.
The term Butterfly children is sometimes used for younger individuals with EB, because their skin is said to be as fragile as the wings of a butterfly.
EB affects both males and females equally, as well as all ethnic groups. EB is incurable and treatment focuses on relieving the symptoms of pain, infection risk and some other potential complications.
There are three main types of EB:
- EBS- Epidermolysis Bullosa- Simplex
- DEB- Dystrophic Epidermolysis Bullosa
- JEB-Juntional Epidermolysis Bullosa
There are over 25 known EB forms.
Ben Jr’s first biopsy was inconclusive as to the type of EB. It was not until September 20, 2012 that we received our son’s diagnosis with the worst type of EB – Junctional Epidermolysis Bullosa (JEB-NH). At this time we are going to have more testing done in order to know exactly what type of JEB-NH Ben Jr. has.
Baby Ben blisters every day, with all types of blisters: all over his body, head, scalp, mouth, throat, legs, arms, fingers, feet, toes, butt, private area, ears, neck, chest, and back.
He never gets a break and neither do we. He has not only brought us closer as parents, but he has taught us the true meaning of life. They say there is no cure, but we know that there is a cure and we trust that one day he will be completely healed by the blood of the Lamb! The Lord has healed our son, beyond anyone’s expectations for him already. He is a miracle in the making. Please join us in spreading the word about EB & celebrating our son’s life, & the everyday obstacles that our son overcomes.
Ben Jr.’s bandages have to be changed daily. Supplies are always needed! To continue testing to discover Ben Jr.’s exact type of EB, which will better inform his doctors about how best to treat him, we need to travel to University of Miami Miller School of Medicine, that is about a 4 hr drive that involves days off of work and the cost of staying in a hotels. Along with meals, tolls, and emergency items that may be needed. At this time we are currently pressing for genetic testing and a skin cell transplant/therapy for Ben Jr’s left foot. It has never been healed since birth.