Making Sickle Cell Disease a Manageable Illness

New York Times (July 7, 2011)- On most days Giovanna Poli acts like a typical 12-year-old. She enjoys riding bikes with her brother, likes learning about the planets in science class and wants to be a pediatrician when she grows up.

But Giovanna, a brown-haired girl from West Palm Beach, Fla., also suffers from debilitating pain, recurring infections and organ damage. She was born with sickle cell disease, a genetic disorder with unpredictable complications that causes the red blood cells to assume an abnormal sickle shape, making it difficult for blood to flow through vessels and deliver oxygen throughout the body.

“I usually feel good,” she says. “But some days it hurts so much in my legs and arms. I can’t really walk, and I don’t like people touching me. If it’s really bad, I have to go to the hospital.”

Every year, nearly 1,000 babies in the United States are born with sickle cell disease, which is thought to affect about 100,000 Americans. Forty years ago, the outlook for babies born with the disease was pretty bleak; the average child lived to be only 14. However, over the last four decades, new treatments, early intervention techniques and newborn screening programs have helped turn this disease, which was once a death sentence, into a manageable chronic illness.

“Our entire approach to treating sickle cell disease has changed,” said Dr. Clinton H. Joiner, director of the division of hematology at Cincinnati Children’s Hospital Medical Center. He notes that today, most patients live to see their 40th birthday.

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