Fact Sheet: Pediatric Sickle Cell Disease

Sickle cell disease (SCD) is a genetic, multisystem disease characterized by vascular injury that is caused by decreased oxygen and blood flow, shorter red blood cell lifespan and infectious complications.

Prevalence and Course

In the U.S., SCD has an estimated prevalence of 70,000 to 100,000 and predominantly affects individuals of African-American and Hispanic descent, but can also affect individuals from other ethnic groups. In 2007 the U.S. implemented universal newborn screening for SCD resulting in individuals now being diagnosed shortly following birth.

Health and Psychosocial Consequences

Health complications of SCD include: pain episodes, pulmonary complications, infections, stroke, anemia, priapism (painful erections) in males, kidney disease, bone and joint problems, and damage to the spleen. Health care utilization is increased, including the need for frequent hospital and emergency department visits and regular clinic appointments in multiple disciplines. Psychosocial consequences may include: lower health-related quality of life (HRQOL), interference from acute and chronic pain, school/work absences resulting in underachievement, cognitive impairment, and depressive and anxious symptomatology.

Evidence-based Assessment

Areas/domains that are important to assess include: adherence — facilitators and barriers; cognitive and social functioning; pain — frequency, intensity, coping and interference; and HRQOL. Disease-specific HRQOL measures are being developed.

Culture, Diversity, Demographic and Developmental Factors

The majority of children in the U.S. with SCD are African-American, but research examining the relationship between race and social attitudes is limited. African-American children are more likely to have low socioeconomic status, a predictor of limited access to health care, increased use of emergency departments, difficulties with transportation to/from appointments, and suboptimal adherence. Barriers may be magnified during the transition from pediatric to adult care, and developmental level should be considered to ensure successful transition, self-management, and adherence. As a result of school absenteeism, seeking care for acute pain, and decreased social activities, children and adolescents with SCD may experience stigmatization, low self-esteem, embarrassment, and depression. Positive family functioning may ameliorate the risk of poor physical and psychosocial outcomes for children and youth with SCD.

Evidence-based Interventions

Systematic reviews support the efficacy of cognitive-behavioral interventions, including relaxation and cognitive coping for symptom management.

References

Anie, K.A. & Green, J. (2012), Psychological therapies for sickle cell disease and pain. Cochrane Database of Systematic Reviews. 2 DOI: 10.1002/14651858.CD001916.pub2

Barakat, L.P., Patterson, C.A., Weinberger, B.S., Simon, K., Gonzalez, E.R., & Dampier, C. (2007). A prospective study of the role of coping and family functioning in health outcomes for adolescents with sickle cell disease. Journal of Pediatric Hematology/Oncology, 29(11):752-760.

Hassell, K.L. (2010). Population estimates of sickle cell disease in the U.S. American Journal of Preventive Medicine, 38(suppl 4), S512-S521.

National Heart Lung and Blood Institute. (2002). The management of sickle cell disease. Bethesda, MD: National Institute of Health. NIH publication No. 02-2117.

Panepinto, J.A., Pajewski, N.M., Foerster, L.M., Sabnis, S., & Hoffman, R.G. (2009). Impact of family income and sickle cell disease on the health-related quality of life of children. Quality of Life Research, 18(1), 5-13.

Treadwell, M., Telfair, J., Gibson, R.W., Johnson, S., & Osunkwo, I. (2010). Transitioning from pediatric to adult care in sickle cell disease: Establishing evidence-based practice and directions for research. American Journal of Hematology, 86(1), 116-120.