Sickle Cell Disease

OT Dude Academy’s Free NBCOT® Exam Prep Course Hematology Sickle Cell Disease
  • An inherited blood condition caused by a genetic disorder that changes the behavior of hemoglobin molecules, which tend to polymerize on deoxygenation. The red blood cells become less pliable and become deformed into the characteristic sickle shape. These cells may block blood flow (vaso-occlusion) and lead to tissue ischemia, and organ failure.1 
  • Results in painful vaso-occlusive crisis (VOC). More VOCs lead to decreased quality of life2 3 4 5 6 
  • First recognized in people of West African ancestry.
  • Also occurs in other parts of the world including Italy, Greece, Turkey, Saudi Arabia, and throughout central India.
  • People who know about the genetics of the disease and their genetic status may avoid relationships between carriers.7 
  • Complications7 8 
    • VOC episodes
    • Infections
    • Acute chest syndrome
    • Strokes (overt or silent) which may affect global IQ9 10 11 
    • Chronic pulmonary hypertension
    • Growth development – lowered weight and height
    • Delayed sexual development
    • Pregnancy risks and symptoms
    • Bone pain

Occupational Therapy

  • May affect participation in academic, extracurricular, and everyday activities.12 
  • Decreased cognitive functioning combined with reduced participation in ADLs can negatively affect the transition from pediatric to adult health care and independent living.13 
  • Adolescents with CVA from SCD have additional needs in personal skills, housekeeping, and health care skills compared to controls.
  • Categories requiring more practice were emergencies, money management, and living arrangements.
  • Implications: healthcare management, money management, specific vocational skills, and empowering adolescents for learning12  Barriers to meaningful goals from one study included cumulative effects of hospitalizations, VOC episodes, unsafe residence locations, and transportation.14 
  • Interventions: Parental education, targeting the home environment15 , early intervention, educational handouts16 , transition programs, support groups, and comprehensive and holistic care.17 
  • Improving long-term outcomes by providing appropriate evaluation and cognitive interventions.18 
  • Psychosocial approaches: cognitive restructuring, problem-solving, emotional regulation, seeking social support, and using coping strategies.19 
  1. Piel FB, Steinberg MH, Rees, DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561–73. pmid:28423290[↩]
  2. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018–31. pmid:21131035[↩]
  3. Habara A, Steinberg MH. Minireview: genetic basis of heterogeneity and severity in sickle cell disease. Exp Biol Med (Maywood). 2016;241(7):689‐96.[↩]
  4. Zhang D, Xu C, Manwani D, Frenette PS. Neutrophils, platelets, and inflammatory pathways at the nexus of sickle cell disease pathophysiology. Blood. 2016;127(7):801–9. pmid:26758915[↩]
  5. McClish DK, Penberthy LT, Bovbjerg VE, Roberts JD, Aisiku IP, Levenson JL, et al. Health related quality of life in sickle cell patients: the PiSCES project. Health Qual Life Outcomes. 2005;3:50. pmid:16129027[↩]
  6. Kater AP, Heijboer H, Peters M, Vogels T, Prins MH, Heymans HS. Quality of life in children with sickle cell disease in Amsterdam area. Ned Tijdschr Geneeskd. 1999;43(41):2049–53.[↩]
  7. Serjeant, G. R. (1997). Sickle-cell disease. The Lancet, 350(9079), 725-730.[↩][↩]
  8. Mehta SR, Afenyi-Annan A, Byrns PJ, Lottenberg R. Opportunities to improve outcomes in sickle cell disease. Am Fam Physician. 2006;74(2):303–10. pmid:16883928[↩]
  9. Hogan A. M., Pit-ten Cate I. M., Vargha-Khadem F., Prengler M., & Kirkham F. J. (2006). Physiological correlates of intellectual function in children with sickle cell disease: Hypoxaemia, hyperaemia and brain infarction. Developmental Science, 9, 379–387.[↩]
  10. Schatz J., Brown R. T., Pascual J. M., Hsu L., & DeBaun M. R. (2001). Poor school and cognitive functioning with silent cerebral infarcts and sickle cell disease. Neurology, 56, 1109–1111. http://dx.doi.org/10.1212/WNL.56.8.1109[↩]
  11. Vichinsky E. P., Neumayr L. D., Gold J. I., Weiner M. W., Rule R. R., Truran D., … Armstrong F. D.; Neuropsychological Dysfunction and Neuroimaging Adult Sickle Cell Anemia Study Group. (2010). Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA, 303, 1823–1831. http://dx.doi.org/10.1001/jama.2010.562[↩]
  12. Abel, R. A., Cho, E., Chadwick-Mansker, K. R., D’Souza, N., Housten, A. J., & King, A. A. (2015). Transition needs of adolescents with sickle cell disease. American Journal of Occupational Therapy, 69(2), 6902350030p1-6902350030p5.[↩][↩]
  13. Anie K. A., & Telfair J.; Sickle Cell Disease Transition Study Working Group. (2005). Multi-site study of transition in adolescents with sickle cell disease in the United Kingdom and the United States. International Journal of Adolescent Medicine and Health, 17, 169–178.[↩]
  14. Berg, C., King, A., & Edwards, D. F. (2018). Mentoring program for young adults with sickle cell disease. Occupational therapy in health care, 32(2), 124-136.[↩]
  15. Fields, M. E., Hoyt‐Drazen, C., Abel, R., Rodeghier, M. J., Yarboi, J. M., Compas, B. E., & King, A. A. (2016). A pilot study of parent education intervention improves early childhood development among toddlers with sickle cell disease. Pediatric blood & cancer, 63(12), 2131-2138.[↩]
  16. Calhoun, C. L., Abel, R. A., Pham, H. A., Thompson, S., & King, A. A. (2019). Implementation of an educational intervention to optimize self‐management and transition readiness in young adults with sickle cell disease. Pediatric blood & cancer, 66(7), e27722.[↩]
  17. Okpala, I., Thomas, V., Westerdale, N., Jegede, T., Raj, K., Daley, S., … & Abbs, I. (2002). The comprehensive care of sickle cell disease. European Journal of Haematology, 68(3), 157-162.[↩]
  18. Varughese, T., Varnum, M., Hull, E., Abel, R., & King, A. (2019). Cognitive and Functional Outcomes in Adults With Sickle Cell Disease. American Journal of Occupational Therapy, 73(4_Supplement_1), 7311500032p1-7311500032p1.[↩]
  19. Gold, J. I., Mahrer, N. E., Treadwell, M., Weissman, L., & Vichinsky, E. (2008). Psychosocial and behavioral outcomes in children with sickle cell disease and their healthy siblings. Journal of behavioral medicine, 31(6), 506-516.[↩]
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