[Congressional Record Volume 141, Number 53 (Wednesday, March 22, 1995)]
[Extensions of Remarks]
[Page E660]
From the Congressional Record Online through the Government Publishing Office [www.gpo.gov]


  DR. MARTIN STEINBERG MAKES SIGNIFICANT ADVANCES IN THE TREATMENT OF 
                           SICKLE CELL ANEMIA

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                      HON. G.V. (SONNY) MONTGOMERY

                             of mississippi

                    in the house of representatives

                        Wednesday, March 22, 1995
  Mr. MONTGOMERY. Mr. Speaker, I am pleased to call to the attention of 
my colleagues an article that recently appeared in the February 13-19, 
1995 edition of The Stars and Stripes. The article features Dr. Martin 
Steinberg, the associate chief of staff for research at the Jackson, 
MS, VA Medical Center and his work in a nationally-recognized study of 
drug that may be the first successful treatment for severe cases of 
sickle cell anemia. Dr. Steinberg has been with the Jackson VA Medical 
Center since October 1967. He is well known for his expertise and is 
VA's sickle cell program director.
  Dr. Steinberg's accomplishments in this area are another example of 
the tremendous research that is being done by the Department of 
Veterans Affairs, and all of us are extremely proud of Dr. Steinberg's 
work and his association with the Jackson VA Medical Center.
                [From the Stars and Stripes, Feb. 1995]

      VA Researcher Key Figure In Sickle-Cell Anemia Breakthrough

                           (By Dick Maggrett)

       A researcher at the Jackson, MS, VA Medical Center has 
     played a key role in a nationwide study of a cancer drug that 
     proved to be the first successful treatment for severe cases 
     of sickle-cell anemia, a blood disorder affecting 72,000 
     mostly black Americans.
       Physician Martin Steinberg, an associate chief of staff for 
     research, led a group studying hydroxyurea and its effects on 
     sickle-cell patients. ``This is a significant advance,'' he 
     said.
       Steinberg and his fellow scientists believe that 
     hydroxyurea may work by stimulating the production of fetal 
     hemoglobin, which is present in fetuses and newborn babies. 
     By about four months of age, fetal hemoglobin has been 
     replaced by adult hemoglobin.
       Steinberg, who also is a professor of medicine at the 
     University of Mississippi Medical Center, where some of the 
     research was conducted, said hydroxyurea isn't a cure but 
     that its administration was ``the first effective treatment 
     for this serious illness and may greatly improve the quality 
     of life of sickle-cell anemia patients.''
       In patients with the disease, hemoglobin molecules stick to 
     one another, forming long rods inside red blood cells and 
     causing them to take on a sickle-like shape and become rigid. 
     The cells, unable to squeeze through tiny blood vessels, 
     deprive tissue of an adequate blood supply and cause pain.
       In the $500,000 National Institutes of Health (NIH)-
     sponsored study that examined genetic analyses of patients, 
     half received the drug and half a placebo. In this phase of 
     the work, Steinberg examined the genetic determinants linked 
     to the sickle hemoglobin gene.
       Between January 1992 and April 1993 the study enrolled 299 
     adult sickle-cell anemia patients, 18 years of age and older, 
     at 21 clinics in the United States. All patients had 
     experience at least three pain crises within 12 months.
       The only side effect was mild reversible bone marrow 
     suppression, which caused lowering of blood counts.
       The study showed that daily doses of hydroxyurea reduced 
     the frequency of painful episodes and hospital admissions for 
     sickle-cell crises by about 50 percent. Recurrent painful 
     episodes are the most disabling feature of the illness and 
     interfere with education, jobs and social development.
       Hydroxyurea therapy also reduced the frequency of acute 
     chest syndrome, a life-threatening complication characterized 
     by chest pain, fever and an abnormal chest X-ray. Test 
     patients taking the drug had about 50 percent fewer episodes 
     of acute chest syndrome than those taking a placebo.
       And patients on hydroxyurea also required about 50 percent 
     fewer units of blood transfused than those on the placebo. 
     This finding has ``important'' public health implications, 
     according to the Jackson VAMC.
       Hydroxyurea proved effective in dramatically reducing pain 
     in adult patients with sickle-cell anemia, and NIH recently 
     stopped drug trials four months early and notified 5,000 
     doctors of the treatment.
       Steinberg hopes his research will discover the means of 
     predicting which patients will respond best to the drug. He 
     said he will attempt to determine whether it might be 
     possible to foretell the response of fetal hemoglobin to 
     hydroxyurea.
       Steinberg cautioned that hydroxyurea may not be appropriate 
     for all sickle-cell patients.
       ``The drug should not be used in patients likely to become 
     pregnant,'' Steinberg said. ``Long-term safety in adults and 
     safety and effectiveness of treatment in children have not 
     been determined.''
       And, Steinberg said, hydroxyurea also has the potential to 
     cause life-threatening decreases in blood counts called 
     ``cytopenia.''
       Hydroxyurea hasn't been approved by the Food and Drug 
     Administration for treatment of sickle-cell anemia, although 
     physicians can prescribe it for that purpose. The FDA may 
     consider approving hydroxyurea for sickle-cell anemia after 
     Bristol-Myers Squibb, the drug's manufacturer, gets the 
     study's results.
       The VA facility couldn't say when that might be.
       Hydroxyurea currently is used for treating polycythemia 
     vera, a disease in which too many red blood cells are 
     produced.
       Sickle-cell anemia is an inherited disease most common in 
     people with ancestors from Africa, the Middle East, the 
     Mediterranean basin and India.
       One in 12 African-Americans carries the sickle-cell trait.


       

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