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A boy cries as he recuperates after surgery during "Operation Smile" at a hospital in Manila's Makati financial district October 26, 2009. Operation Smile aim to provide free surgery for about a hundred children inflicted with cleft lips, cleft palates, and other facial deformities over a period of five days in Makati.  REUTERS/Cheryl Ravelo

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    Sickle cell drug underused, experts agree

    WASHINGTON
    Wed Feb 27, 2008 4:54pm EST

    WASHINGTON (Reuters) - An inexpensive cancer pill that can relieve the painful symptoms of sickle cell disease is used far too little and patients are needlessly suffering as a result, a panel of U.S. experts agreed on Wednesday.

    U.S.  |  Health

    They said as many as half of all patients with sickle cell disease could be helped by hydroxyurea, but no more than 3 percent to 5 percent are likely using the drug.

    A shortage of doctors specializing in sickle cell disease, along with a batch of rumors about the drug's safety, worsens the problem, the National Institutes of Health consensus panel agreed.

    "One of the first problems is we don't even know how many people have sickle cell disease," Dr. Otis Brawley, who chaired the meeting at the NIH, said in a telephone interview.

    "You can find data that says the number of sickle cell patients is between 50,000 and 75,000 and you can find other papers that say it is between 50,000 and 120,000," added Brawley, a professor of hematology, oncology and epidemiology at Emory University in Atlanta and chief medical officer of the American Cancer Society.

    Hydroxyurea, which is now available generically, was approved by the U.S. Food and Drug Administration in 1998 for use in adults with sickle cell anemia, a disease caused by abnormally shaped red blood cells.

    The cells die early, or get stuck in blood vessels, blocking blood flow and causing pain and organ damage. Hydroxyurea can stop the severe pain and prevent the need for blood transfusions.

    "The compelling benefits of hydroxyurea warrant increased adoption of this drug as a front-line therapy in adults with sickle cell disease," Brawley said.

    "We suspect that about half or more patients with sickle cell disease would benefit from hydroxyurea treatment."

    SHORTAGE OF DOCTORS

    NIH Consensus panels draw together experts on controversial medical subjects to give unbiased, impartial guidance.

    One barrier to wider use of the drug is worries about its safety, Brawley said.

    "If you do an Internet search for hydroxyurea, there are all kind of negative things there that say that it causes cancer ... decreased sperm count," Brawley said.

    "All of these things are really overblown."

    Another advantage of the drug is that it is available generically.

    "There are 20 companies that manufacture the drug and it is a generic so nobody can make money off it so you don't have any drug company that is out there hyping the drug, that is out there pushing the drug," Brawley added.

    Sickle cell patients must inherit the trait from both parents, and the genetic susceptibility is most common in people of Mediterranean, African and Hispanic origin.

    There is a shortage of doctors willing to see the patients likely to have the disease, who include minorities and people with little or no health insurance.

    "The number of doctors who are interested in specializing in sickle cell disease is going down dramatically," Brawley said.

    (Editing by Will Dunham and Stuart Grudgings)



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