Sickle Cell Disease and the March of Dimes
by David Rose, March of Dimes Archivist
Sickle
cell disease is a genetic blood disorder in which red blood cells take on a
characteristic abnormal “sickle” shape. African-Americans and others from
tropical sub-Saharan Africa are most susceptible to this disorder, which can
cause intense pain, high blood pressure, stroke, damage to vital organs, and
the risk of serious infection. March of Dimes research involvement into the
causes and prevention of sickle cell disease dates back to the polio era, when the
chemist Linus Pauling discovered that the disease results from an abnormality
in molecules of hemoglobin, which carries oxygen from the lungs to the rest of
the body. Dr. Pauling’s finding that
sickle hemoglobin differs in a measurable way from normal hemoglobin introduced
the idea that heritable changes in the structure of a molecule could lead to
improper function and result in disease. Dr. Pauling received one of the
earliest basic research grants from the March of Dimes, and he was awarded the
Nobel Prize in Chemistry in 1954. His work laid the groundwork for techniques
used in newborn screening and the diagnosis of sickle cell disease today.
Sickle Cell screening program
After the March of Dimes changed its mission to birth defects prevention in 1958, sickle cell disease again came to the forefront of concern as a significant, but treatable, genetic disorder. The Foundation supported several lines of research: one was a medication that prevents red blood cells from “sickling;” another was giving daily antibiotics to affected infants and toddlers to prevent life-threatening bacterial infections; a third was bone marrow transplantation, used to cure other genetic blood disorders as well as severe sickle cell disease. The Foundation helped to establish one of the first pediatric clinics in the U.S. to care for infants and children with sickle cell disease at the Mt. Sinai Medical Center in New York City in the late 1970s. This center provided medical services, social and psychological support, genetic counseling and education needed by children with sickle cell disease as well as their families and communities. March of Dimes researchers investigated the effectiveness of innovative drug treatments and a multi-disciplinary team approach to caring for infants and children affected by the disease.
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| Image of a March of Dimes Birth Defects Center |
For over
50 years the March of Dimes has focused on treatment of sickle cell disease in
its quest to prevent all birth defects. In 1982, March of Dimes grants led to
the development of a safe and accurate prenatal test for the disease, and even
today our grants support cutting-edge medical research to understand the
molecular pathways of cellular development, to determine the risk factors
inherent in the disease to prevent other infections, and to explore innovative
gene therapies to eliminate the risks of leukemia in those affected by the
disease.
February 21, 2013 /
David Rose / March of Dimes Archives
