Milestones in Sickle Cell Disease
Dec 05
2008
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. |
