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.

Read the brochure Sickle cell disease and thalassemia

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.
1970s	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.
2000	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.