Finding the Right Care to Treat Rare Disorder

This article originally appeared in Star Chronicle, a newsletter published for the Children's Medical Center.

When their son, Jonathon, nine, was diagnosed with the rare skin disorder epidermolysis bullosa (EB) at five months old, Whitney Connolly and Jonathon Rose soon discovered the challenges before them. First was finding a dermatologist familiar with treating the disease. After several years of searching, Jonathon's parents found the right fit at UMass Memorial Children's Medical Center.

"EB is a group of inherited disorders characterized by extremely fragile skin where blisters develop in response to minor friction or trauma," explained Karen Wiss, MD, chief of pediatric dermatology.

Under the care of Dr.Wiss, Jonathon is now scheduled for visits every three months, a significant gain compared to his younger years when he had appointments at the UMass Memorial EB Clinic twice a month. Jonathon, who has moderate EB, is prone to daily outbreaks on his hands and anywhere there is contact to his skin.

The UMass Memorial Division of Pediatric Dermatology is a designated EB referral site serving New England. UMass Memorial is currently treating about 35 pediatric patients with the rare disease, which affects only 12,000 Americans. The division's two physicians - Dr.Wiss and Leah Belazarian, MD - along with other UMass Memorial dermatologists - see about 6,500 pediatric dermatology patients each year.

As an EB patient benefiting from care at UMass Memorial, Jonathon's young life has been plagued by setbacks caused by slips and falls, starting as an infant when he tumbled out of his car seat. At age five, a deep cut to his right middle finger almost ended in amputation.

Today, his treatment plan is less structured. His daily routine still requires wrapping his knees, feet and sometimes his elbows and wrists with bandages, but every-other-day bleach baths for fighting infection are now needed less than once a month. "As he gets older, he knows his boundaries and thinks of the consequences," said Ms. Connolly. "He is well educated about EB and how to handle things."

Despite his disease, Jonathon is very much a typical nine-year-old boy. An avid hockey fan, he enjoys watching "Cops" and visits to Chuck E. Cheese's.

For more information, call 508-334-5979.