Sickle Cell Center at Cincinnati Children's Receives NIH Grant
CINCINNATI -- Cincinnati Children's Hospital Medical Center has received a 5-year, $9.5 million grant from the National Institutes of Health (NIH) to fund the Cincinnati Comprehensive Sickle Cell Center (CCSCC) in the Division of Hematology/Oncology.
The CCSCC was founded in 1972 and is dedicated to the research and treatment of sickle cell anemia, a genetic blood disease that affects more than 70,000 Americans, primarily individuals of African descent. The new funding, from the National Heart Lung and Blood Institute, a division of the NIH, will support patient care, community outreach, and both clinical and basic research efforts aimed at improving current treatments and discovering new therapies for sickle cell disease.
The CCSCC is one of only 10 nationally funded sickle cell centers. The CCSCC receives 1,000-plus outpatient visits, with an average of more than 200 admissions to the hospital each year. The CCSCC staff follows approximately 240 children and adolescents, and the University of Cincinnati Hospital staff follows approximately 115 adults with sickle cell disease. About 600 children and adults in the Cincinnati area have sickle cell disease, and about 20,000 more carry the gene but have no symptoms.
"This grant fosters our goal of being in the forefront of medical research for children," says Clinton Joiner, MD, PhD, director of the CCSCC. "The overriding goal of those affected by sickle cell disease, their families and those who provide their medical care is to find a cure. It's clear that additional knowledge is needed to accomplish this goal. As we work toward a cure, we are also trying to design more effective treatments."
Sickle cell disease is a genetic disorder of hemoglobin -- the oxygen-carrying pigment of red blood cells, which has a tendency to gel in the body and block circulation, causing pain and organ damage. Symptoms begin in infancy and persist throughout life. Strokes are a major and devastating complication in children.
In the past decade, however, medical advances have improved the outlook for people with sickle cell disease. These include newborn diagnosis and prophylactic penicillin to reduce infections; the use of transfusions to prevent stroke in children who are at risk; drug therapy (hydroxyurea, Hydrea") to make patients feel better, reduce the rate of pain episodes, and decrease the need for transfusion; improved technology of blood transfusion, including ways to reduce the side effects of iron overload; and curative bone marrow transplantation in select patients. The CCSCC has participated in many of the clinical research studies that helped develop these new treatments.
The new grant builds on thirty years of nearly continuous NIH funding for the CCSCC. During the next few years, researchers at the center will focus on an area in which Cincinnati Children's investigators already have made important contributions: the tendency of sickle red blood cells to become dehydrated. Dehydration of sickle cells worsens their functional characteristics, making pain and organ damage more likely. Several basic science projects will explore the molecular basis for this dehydrating tendency in sickle cells; another will examine this cellular process in the body; and a clinical research study will employ two commonly available drugs, dipyridamole and magnesium salts, to prevent sickle cell dehydration in patients.
The CCSCC also will participate in a major new initiative of the NIH program: a series of clinical research projects in which all of the 10 funded Sickle Cell Centers collaborate. "This is an exciting opportunity to quicken the pace of developing new treatments for sickle cell disease," says Dr. Joiner. "By pooling our resources and working together at Centers across the country, we can more efficiently answer questions about what treatment strategies work the best.
NIH funding also helps to support clinical teams that provide care to patients and families at Cincinnati Children's and at University Hospital. For example, one special project helps teens and young adults with sickle cell disease to assume responsibility for their own health and to make the transition from pediatric to adult care providers. And, collaboration with the Sickle Cell Awareness Group of Greater Cincinnati, affiliated with the Urban League, provides community awareness activities, education, social services and support for families and individuals with sickle cell disease.
Contact Information
Jim Feuer, 513-636-5637,
jim.feuer@cchmc.org
