Sickle cell disease is caused by a mutation in the hemoglobin-ß 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, however, 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, leading to anemia. This anemia is what gives the disease its commonly known name - sickle cell anemia. The sickle cells also block the flow of blood through vessels, resulting in lung tissue damage, and injury to the spleen, kidneys and liver. Sickle cell disease is the most common inherited blood disorder in the United States, with approximately 80,000 Americans having the disease. In the United States, sickle cell disease is most prevalent among African Americans. About one in 12 African Americans and about one in 100 Hispanic Americans carry the sickle cell trait, which means they are carriers of the disease.

Given the severity of sickle cell disorder, is it any wonder that self-management is being introduced as a strategy to help prevent the pain and harm of disease? The movement is only in its nascent stage but the approach has promise. In a nice study, Yoon and Goodwin set out to see whether playing a simple CD-ROM educational game, specifically developed for children with sickle cell disease, would improve children's knowledge and confidence in selected symptom management and practice. In the investigation, twenty-two eligible children completed a pretest to determine knowledge and confidence levels, played the Sickle Cell Slime-O-Rama Game, then completed an identical posttest. Yoon and Goodwin found significant increases in knowledge and confidence in the ability to apply the knowledge between pre- and posttests.

COMMENTS: The authors concluded that a simple, interactive CD-ROM game allowed children with sickle cell disease to quickly acquire knowledge about the disease and symptom management, and increased their confidence to apply this knowledge. As the technology is relatively simple, the approach has great potential for application to other populations of patients with sickle cell disorder. There is a great need for such application as we could find only three references on MEDLINE on the use of self-management with the sickle cell disorder. There may not be an overwhelming number of patients with the chronic illness, but this should never dismiss the critical need for more studies on self-management and sickle cell disorder. Just ask those with the disease.

S.L. Yoon and A. Goodwin. Enhancing self-management in children with sickle cell disease through playing a CD-ROM educational game: a pilot study. Pediatric Nursing, 2007;33:60-63,72.