Sabin Russell Science Writer


Bandaging Ileana

                One of the joys of the troubled business of journalism is to come in contact with the heroism of people who are living their day to day lives out of the spotlight. I was about to write “the heroism of ordinary people,”  but…they are extraordinary people.

                Stephanie Peralta is a single mom, and her daughter Ileana, 15 years old, knows more about life than this aging baby boomer blogger. Ileana's skin, her body’s largest organ, has betrayed her since birth, but she perseveres as if to say to the world that life is about optimism and defiance, and maybe more so when things are undeniably tough. I wrote a piece about her, and the disease she has, epidermolysis bullosa, in the Bay Area section of the Sunday New York Times.

                Epidermolysis bullosa is also an intellectual challenge for medical scientists who try to set their emotions aside while they puzzle over the genetic mechanisms that cause it. They are looking for ways to  restore a missing gene that codes for Collagen VII, the glue that keeps the rest of us neatly and painlessly packaged in an envelope of healthy tissue.

                I wrote about this disease in December, when I first heard about it, because it is a target of the taxpayer-supported California stem cell initiative. I felt then that it was such a compelling story that  it was worth a closer look. The New York Times, thankfully, offered me another opportunity to do so.

           That same compelling story had already attracted the attention of Hollywood, where skin is a more important organ than in most places on the planet. The Epidermolysis Bullosa Medical Research Foundation – started by a Piedmont, California couple, Lynn and Gary Anderson, who lost two children to EB – is now headed by Paul Joseph, a Los Angeles stockbroker, and his wife Andrea Pett-Joseph, whose six-year-old son, Brandon, has EB. Andrea is a talent manager in Los Angeles. They care about their son just as deeply as Stephanie Peralta, the unemployed single mom from Livermore, loves her daughter. EBMRF now has assets of $2 million and an advisory board listing celebrities who have been moved by the tragedy of this disease:  Brad Pitt, Jennifer Aniston, Steve Young, Gwen Stefani, and Courteney Cox-Arquette and Adam Sandler, to name a few.

                The foundation supports research, including that of Dr. Alfred Lane at Stanford. Because Dr. Lane conducts medical research on these children, the pediatrician makes a point that another Stanford physician, Dr. Anna Bruckner, provides their clinical care. He recognizes that the interests of a researcher and that of a treating physician may not always coincide. Dr. Bruckner is their caregiver and their advocate. “They have to cope with so much,” Dr. Bruckner told me. “Once you go beyond what you see…there’s a real person in there just waiting to come out.”

                Another parent-founded group DebRA (Dystrophic epidermolysis bullosa Research Association of America), in New York, raises money to educate the public and the parents of EB children about the disease. When a child is born with EB, DebRA arrives with a kit to help the deal with what has just happened to their lives. It helps to pay for the gauze bandages that are the only reliable treatment for EB. Each year, parents wrap their children in literally miles of bandages costing thousands of dollars. Here is their web site:

                Stephanie Peralta was a scared teenager when she gave birth to a daughter whose skin had sloughed off from her right calf when she was born. Stephanie had no idea she was a genetic carrier of the disease, nor did Ileana’s biological father, who played no part in their lives after the pregnancy. Stephanie has since had three other children since Ileana’s birth. None of them have EB. They are a warm and loving family, and Ileana’s biggest regret at home is that she can’t roughhouse with her siblings. It’s just too dangerous.

                Ileana visits Dr. Bruckner at Stanford on a regular basis for treatment of anemia, a common symptom of EB. Like others with severe EB, Ileana lost her nails when she was an infant, and her fingers have curved inward and partially fused.  But she can grab a pencil and write with impeccable cursive script. She can draw.

                “I’d like to try to become an artist,” she told me. “To get a job and earn money, to pay for my own phone bill, to get a car and drive it to the movies.”

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Posted by Sabin Russell

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  1. This is very moving. You were born to blog.

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