Chicago Woman Cured of Sickle Cell Disease

ScienceDaily (June 18, 2012) — Chicagoan Ieshea Thomas is the first Midwest patient to receive a successful stem cell transplant to cure her sickle cell disease without chemotherapy in preparation for the transplant.

University of Illinois Hospital & Health Sciences System physicians performed the procedure using medication to suppress her immune system and one small dose of total body radiation right before the transplant.

The transplant technique is relatively uncommon and is a much more tolerable treatment for patients with aggressive sickle cell disease who often have underlying organ disease and other complications, says Dr. Damiano Rondelli, professor of medicine at UIC, who performed Thomas's transplant.

The procedure initially allows a patient's own bone marrow to coexist with that of the donor. Since the patient's bone marrow is not completely destroyed by chemotherapy or radiation prior to transplant, part of the immune defense survives, lessening the risk of infection. The goal is for the transplanted stem cells to gradually take over the bone marrow's role to produce red blood cells -- normal, healthy ones.

Thomas, 33, had her first sickle cell crisis when she was just 8 months old. Her disease became progressively worse as an adult, particularly after the birth of her daughter. She has spent most of her adult life in and out of hospitals with severe pain and has relied on repeated red blood cell transfusions. Her sickle cell disease also caused bone damage requiring two hip replacements.

"I just want to be at home with my daughter every day and every night," said Thomas, who depends on family to help care for her daughter during her frequent hospitalizations.

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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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