Sickle cell disease is the most common inherited blood disorder in the United States, with about 80,000 Americans afflicted with the disease. In the United States, sickle cell disease is most prevalent among African Americans. About one in 12 African Americans and one in 100 Hispanic Americans carry the sickle cell trait and are, therefore, carriers of the disease. Sickle cell disease is caused by a mutation in the hemoglobin-Beta gene found on chromosome 11. Hemoglobin transports oxygen from the lungs to other parts of the body. Red blood cells with normal hemoglobin (hemoglobin-A) are smooth and round and glide through blood vessels. In people with sickle cell disease, abnormal hemoglobin molecules - hemoglobin S - stick to one another and form long, rod-like structures. These structures cause red blood cells to become stiff, assuming a sickle shape. Their shape causes these red blood cells to pile up, causing blockages and damaging vital organs and tissue. Sickle cells are destroyed rapidly in the bodies of people with the disease, causing anemia. This anemia is what gives the disease its commonly known name - sickle cell anemia.

Jenerette and Murdaugh investigated factors predicting health outcomes in persons with sickle cell disease (SCD) within the framework of self-management. They proposed that vulnerability factors negatively affect health care outcomes and self-care management resources, and positively mediate the relationship between vulnerability factors and health care outcomes. A cross-sectional descriptive design was used to test the model with a sample of 232 African American adults with SCD. Results supported the negative effect of vulnerability factors on health outcomes. The overall model was supported, although self-management resources did not mediate the relationship between vulnerability and health care outcomes.

WHAT THE STUDY MAY MEAN TO YOU: The findings provide support for interventions to increase self-management resources to improve health care outcomes. We applaud the efforts of the investigators and hope that they not only continue their research, but that others pick up the torch to develop and implement effective self-management programs to relieve the pain of sickle cell patients.

CM Jenerette, C Murdaugh. Testing the theory of self-care management for sickle cell disease. Research in Nursing & Health, February 4, 2008.