
HOPE FOR SICKLE CELL DISEASE
Too much iron in the blood is a common problem for patients with sickle cell disease. The blood disorder affects about 72,000 people in the United States, mainly African Americans. Now, researchers have developed a drug that may reduce the iron levels.
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Sunshine, a soft breeze and a book. Just what the doctor ordered for college student Sylvonna Cooper.
Sylvonna Cooper
Has sickle cell disease
"I stay in school a lot because my school environment is very low stress."
Sylvonna needs to avoid stress because it brings on excruciating pain. She has sickle cell disease -- a hereditary, chronic blood disorder.
Zahida Yasin, M.D.
Hemotological Oncologist
University of Cincinnati Medical Center
Cincinnati, OH
"Blood transfusions are the mainstay of treatment in sickle cell disease."
Doctor Zahida Yasin says the problem with transfusions is iron overload, which can damage multiple organs like the liver, the heart, the lungs, and the kidneys.
Zahida Yasin, M.D.
"There should be an effective means of removing this excess iron, and we don't have a very good drug to do this."
Now this drug is being studied. ICL 670 extracts excess iron from the body, making transfusions safer for sickle cell sufferers. Patients dissolve the tablets in water and drink up. So far, no one has complained of major side effects.
Zahida Yasin, M.D.
"We're hoping that it will be a breakthrough drug. It's early at this point in time."
Sylvonna Cooper
"I hope it works because I would love to just take the pills in the morning at the beginning of my day."
The drug is still under study, but it's working for Sylvonna. She says her iron counts have already gone down.
Patients say the liquid mixture is tasteless, which makes it easy to take. Currently, the only FDA approved drug for iron overload is infused several hours a day for several days a week.
BACKGROUND: Sickle cell disease (SCD) is the most common blood disorder in the United States, affecting approximately 72,000 Americans. The disease is prevalent in African Americans, with one in 12 carrying the sickle cell trait. SCD is caused by a mutation in the hemoglobin-Beta gene found on chromosome 11. Hemoglobin is responsible for transporting oxygen from the lungs to other areas of the body. In people who suffer from SCD, abnormal hemoglobin molecules attract to one another, forming long, rod-like structures. The structures cause red blood cells to stiffen, forming a sickle shape. Hence, the mostly commonly known name, sickle cell anemia (SCA). Red blood cells pile up, causing blockages that damage vital organs and tissue. The cells also block the flow of blood through the blood vessel, causing lung damage, pain episodes, strokes, and spleen, kidney and liver damage.
There is no known universal cure for sickle cell anemia. However, over the years doctors have learned a great deal about the disease and know how to treat it. People diagnosed with SCA require regular medical care. The goal of treatment is to relieve a patient's pain, prevent infections and control any complications that occur. Blood transfusions are a common treatment for sickle cell patients. To prevent iron overload, doctors currently use the drug deferoxamine, which is infused subsutaneously several hours a day for several days.
A NEW OPTION: With chronic transfusions, iron in the hemoglobin can begin to build up. Researchers at the University of Cincinnati are testing ICL 670 -- a new oral chelator. The drug latches on to the excess iron in the blood and helps to eliminate it. The drug is dissolved in water, and the study participants drink the tasteless substance once a day. Zahida Yasin, M.D., a hematological oncologist at the University of Cincinnati Medical Center, believes the drug could drastically change the quality of life for SCD patients because it will reduce the complications of the disease. She reports there have been no major problems with the drug.
LIVING WITH SICKLE CELL DISEASE: Maintaining a healthy lifestyle is an important factor for anyone with sickle cell disease. Doctors recommend patients with the disease should:
- Maintain a healthy diet.
- Drink plenty of water.
- Get enough sleep and rest.
- Schedule regular check-ups and treatments.
- Prevent infection: Keep up to date with flu shots and other vaccinations.
- Avoid overexertion, stress and extreme heat or cold.
David Bracey
Public Relations and Communications
University of Cincinnati Medical Center
165 Health Professions Building
Eden Ave and Albert Sabin Way
Cincinnati, OH 45267
(513) 558-4559
david.bracey@uc.edu
http://www.medcenter.uc.edu
Copyright © 2005 Ivanhoe Broadcast News, Inc.
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