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Milestones in Sickle Cell Disease

An article on sickle cell disease and thalassemia by Elliot Vichinsky, MD, and these accompanying milestones were published in December 2008 as part of the special ASH anniversary brochure, 50 Years in Hematology: Research That Revolutionized Patient Care.

1910 James Herrick notes "peculiar, elongated sickle-shaped erythrocytes" in a patient with anemia.
1930s Lemuel Whitley Diggs suggests that pain in sickle cell patients is due to sickle cells clogging up small blood vessels.
1949 Linus Pauling discovers that sickle cell disease is caused by an abnormal hemoglobin.
1957 Vernon Ingram discovers that a change in one amino acid in hemoglobin S causes sickling.
1970s Sickle cell anemia becomes the first human disease to be explained at the level of a single nucleotide mutation: Using recombinant DNA technology techniques, scientists find that the nucleotide change in the DNA for sickle hemoglobin results from an A to T substitution.
The civil rights movement calls attention to racial inequality in health care. Charles Whitten establishes the Sickle Cell Disease Association of America to improve research, education, and health care for sickle cell patients.
1972 The Sickle Cell Anemia Control Act allocates government funding for screening, research, and treatment. With these funds, the National Heart, Lung, and Blood Institute establishes the Cooperative Study of Sickle Cell Disease.
1980 Robert P. Hebbel observes that sickle cells stick to the lining of blood vessels and shows that this correlates with severity of illness.
1983 The Prophylactic Penicillin Study (PROPS) finds that treatment of well sickle cell patients with penicillin could prevent death related to serious infections.
1995 The Multicenter Study of Hydroxyurea proves the usefulness of hydroxyurea in preventing complications in patients with sickle cell disease.
1996 The Multicenter Bone Marrow Transplant Study demonstrates a cure for children with sickle cell disease.
1998 The Stroke Prevention in Sickle Cell Disease clinical trials show that transcranial Doppler ultrasonography, a method of analyzing blood flow in the brain, is an effective screening tool.
2000 A published study by Lennette J. Benjamin demonstrates that a day hospital for the treatment of pain improves quality of life and prevents hospitalizations.
Hemolysis (a breakdown of red blood cells) results in free hemoglobin, which decreases the availability of nitric oxide (an important signaling molecule), causing widespread pathologic consequences, including pulmonary hypertension. Further studies determine that pulmonary hypertension is common in sickle cell disease and a strong predictor of death.
2001 Gene therapy successfully cures a sickle-cell mouse.
2005 Genetic methods are developed to predict complications of sickle cell disease.
2007 Techniques are developed in sickle-cell mice to convert normal cells into stem cells to be used for gene therapy and transplant.