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Medicine: Discriminating Disease

2 minute read

Sickle-cell anemia is a truly discriminating disease: 99% of its U.S. victims are black. The result of a genetic mutation that occurred in Africa centuries ago, it reduces susceptibility to malaria in the 8% to 10% of U.S. Negroes who carry it. But in those (about 1%) actually harmed by it, periodic crises distort the normally spherical red blood cells into crescent-like (“sickle”) structures, which then block the narrow capillaries. This deprives nearby tissues of needed oxygen and causes severe pain. The disease kills at least half its victims before the age of 20; only a handful live beyond 40, and most are crippled long before death.

Medicine still has no cure for this inherited illness. But a team of Michigan medical researchers has just announced a discovery that offers new relief for sickle-cell sufferers. By treating the patient with a solution of urea and invert sugar, say the researchers, the sickling tendency can be reversed and the misshapen cells returned to normal.

Cautious Encouragement. The Michigan team, led by Dr. Robert Nalbandian of Blodgett Memorial Hospital in Grand Rapids, owes its discovery to the work of another researcher, Makio Murayama of the National Institutes of Health. Murayama discovered that the sickle-cell shape is caused by an abnormal bonding between hemoglobin molecules in the red cells. Using this knowledge, Nalbandian’s team decided to try urea, a waste substance produced by the normal human liver and excreted in the urine. As they knew, urea can dissolve certain types of molecular bonds. Their experiment worked: urea broke the bond between the hemoglobin molecules, halted the sickling effect, and relieved the victims’ pain.

Nalbandian’s team is cautiously optimistic about its discovery. Earlier attempts to treat sickle-cell anemia with alkalis and antihistamines either failed or produced undesirable side effects. But Nalbandian’s treatment, tested on 25 patients at four major hospitals, has thus far proved safe and effective.

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