The Health Psychologist

Society for Health Psychology

What Health Psychologists Should Know about Sickle Cell Anemia

2023 Spring, Rare diseases and disorders, The Health Psychologist

Shawn M. Bediako, Ph.D., MPA
Inclusive Learning Group
Center for the Advancement of Science Leadership and Culture
Howard Hughes Medical Institute
Health psychologists utilize a sophisticated understanding of the mental and social impact of disease and illness to help patients cope, adapt, and adjust to specific conditions. However, there is a significant shortage of health psychologists with training in the psychological and social aspects of sickle cell anemia – the world’s most common genetic blood disorder (Osakonor & Tsitsikas, 2022). Consequently, specialized psychological services for individuals living with sickle cell disease are limited and rare.

Sickle cell anemia is a genetic condition that occurs when a substitution of two amino acids – valine and glutamic acid – creates an abnormal form of hemoglobin, a vital blood protein. This substitution causes hemoglobin to produce red blood cells that are deoxygenated, sticky, and crescent-shaped (like the blade of a sickle). Sickled red blood cells carry less oxygen than typical cells and adhere to the blood vessel wall, causing blockages and occlusions that prevent nutrients transported through blood from reaching major organ systems (Steinberg, 2008). Sickle cell causes a wide range of health issues, but the most common complaint is unpredictable episodes of severe, acute pain. Although the precise location of pain varies by individual, one epidemiologic study of sickle cell pain suggests that it frequently occurs in the lower back and lower extremities (McClish et al., 2009).

Health psychologists and health psychologists-in-training should know a few key things about sickle cell anemia:

  • Chronic Pain: Beyond its contribution to acute pain, the sickling of red blood cells can cause tissue damage that leads to chronic pain (i.e., pain that lasts for more than three months). Tissue damage can occur at a localized somatic site (e.g., the eye, lung, spleen, etc.) or affect multiple sites throughout the body such as the hips, shoulders, and knees. Regardless of the chronic pain site(s), sustained experiences with it can lead to depression, anxiety, or other physical and mental health challenges (Darbari et al., 2020; Wallen et al., 2014).
  • Stigma: People living with sickle cell often face discrimination and negative evaluations from others, which can lead to feelings of isolation and an impaired self-concept (Bulgin et al., 2018; O’Brien et al., 2022). Stigma in sickle cell is characterized by both intrapersonal (e.g., internalized stigma and disclosure concerns) and interpersonal (e.g., social exclusion and expected discrimination) aspects that are associated with health care use (Bediako et al., 2016).
  • Coping Strategies: People living with sickle cell may use a range of coping strategies to manage pain and other symptoms related to the condition. A limited research literature suggests that relaxation techniques, meditation, music, and other self-management programs may be beneficial for patients (Rodgers-Melnick et al., 2022).
  • Social Support: People living with sickle cell can benefit from strong and empathic networks from family, friends, support groups, and health care professionals (Desine et al., 2021). Interventions that focus on increasing both emotional and instrumental support may be particularly helpful, as might those that enhance a strong psychological sense of community.
  • Healthcare Utilization: People living with sickle cell may require frequent hospitalizations and medical treatments that interfere with one’s ability to maintain gainful employment. Further, repeated engagement with healthcare systems and providers that are not perceived to convey respect and trust may have a negative impact on patients’ quality of life (Haywood et al., 2010). In addition, ancillary aspects of recurrent hospitalizations may lead to other psychological issues such as algophobia (fear of pain), thanatophobia (fear of death), kinesiophobia (fear of movement), or fear of hospitals and/or needles (Pells et al., 2007).
  • Culture and Cultural Background: Contrary to most thinking, sickle cell is not a “Black” disease; it affects people from all ethnic backgrounds. Therefore, it is important for health psychologists to avoid “race-based” thinking about sickle cell and, instead, be aware of broader cultural nuances that shape the disease experience. For example, the ways in which people negotiate living with sickle cell may differ in Little Rock, Lagos, or Lahore; even within these locales, an individual’s cultural context and cultural beliefs may uniquely shape their perception of the disease and how they respond to or seek treatment (Bediako & Haywood, 2009; Bediako &Moffitt, 2011; Creary, 2018).
  • Education and Awareness: There is a general lack of knowledge – in both the public and in the sickle cell community – about the disease, available treatments, and its management (Pecker et al., 2021; Smith & Brownell, 2018). Health psychologists can play a critical role in educating patients about the disease and how to manage symptoms. They can also work with health communication experts to design interventions that shape public and/or patient perceptions by mitigating stigma and improving negative attitudes about sickle cell (Jean-Baptiste et al., 2022).

Health psychologists who wish to learn more about sickle cell anemia can consult the extant research literature or reach out to the Sickle Cell Disease Association of America’s Medical and Research Advisory Committee for more information. It may also be helpful to contact the local comprehensive sickle cell center in your community if there is one. The National Alliance of Sickle Cell Centers lists its Member and Associate Member centers here.


Bediako, S. M., & Haywood, C., Jr. (2009). Sickle cell disease in a “postracial” America. Journal of the National Medical Association, 101, 1065-1066.

Bediako, S. M., & Moffitt, K. R. (2011). Race and social attitudes about sickle cell disease. Ethnicity & Health, 16, 423-429.

Bediako, S. M., Lanzkron, S., Diener-West, M., Onojobi, G., Beach, M. C., & Haywood, C., Jr. (2016). The measure of sickle cell stigma: Initial findings from the improving patient outcomes through respect and trust study. Journal of Health Psychology, 21, 808-820.

Bulgin, D., Tanabe, P., & Jenerette, C. (2018). Stigma of sickle cell disease: A systematic review. Issues in Mental Health Nursing, 39, 675-686.

Creary, M. S. (2018). Biocultural citizenship and embodying exceptionalism: Biopolitics for sickle cell disease in Brazil. Social Science & Medicine, 199, 123-131.

Darbari, D. S., Sheehan, V. A., & Ballas, S. K. (2020). The vaso-occlusive pain crisis in sickle cell disease: Definition, pathophysiology, and management. European Journal of Haematology, 105, 237-246.

Desine, S., Eskin, L., Bonham, V. L., & Koehly, L. M. (2021). Social support networks of adults with sickle cell disease. Journal of Genetic Counseling, 30, 1418-1427.

Haywood, C., Jr., Lanzkron, S., Ratanawongsa, N., Bediako, S. M., Lattimer-Nelson, L., & Beach, M. C. (2010). Hospital self-discharge among adults with sickle cell disease: Associations with trust and interpersonal experiences with care. Journal of Hospital Medicine, 5, 289-294.

Jean-Baptiste, D. M., Wassef, M., Bolyai, S. S., & Jenerette, C. (2022). Individuals with sickle cell disease using SBAR as a communication tool: A pilot study. International Journal of Environmental Research in Public Health, 24, 138

McClish, D. K., Smith, W. R., Dahman, B. A., Levenson, J. L., Roberts, J. D., Penberthy, L. T., Aisiku, I. P., Roseff, S. D., & Bovbjerg, V. E. (2009). Pain site frequency and location in sickle cell disease: The PiSCES project. Pain, 145, 246-251.

O’Brien, J. A., Hickman, Jr., R. L., Burant, C., Dolansky, M., & Padrino, S. (2022). Health literacy, perceived stigma, self-efficacy, and HRQOL in sickle cell disease. Western Journal of Nursing Research, 45(4), 335-343. doi: 10.1177/01939459221135331.

Osakonor, D., & Tsitsikas, D. A. (2022). Reflections from a psychologist working with sickle cell and thalassaemia patients during the COVID-19 pandemic. Medicina, 58, 1286.

Pecker, L. H., Sharma, D., Nero, A., Paidas, M. J., Ware, R. E., James, A. H., & Smith-Whitley, K. (2021). Knowledge gaps in reproductive and sexual health in girls and women with sickle cell disease. British Journal of Haematology, 194, 970-979.

Pells, J., Edwards, C. L., McDougald, C. S., Wood, M., Barksdale, C., Jonassaint, J., Leach- Beale, B., Byrd, G., Mathis, M., Harrison, M. O., Feliu, M., Edwards, L. Y., Whitfield, K., & Rogers, L. (2007). Fear of movement (kinesiophobia), pain, and psychopathology in patients with sickle cell disease. Clinical Journal of Pain, 23, 707-713.

Rodgers-Melnick, S. N., Lin, L., Gam, K., de Santana Carvalho, E. S., Jenerette, C., Rowland, D. Y., Little, J. A., Dusek, J. A., Bakshi, N., & Krishnamurti, L. (2022). Effects of music therapy on quality of life in adults with sickle cell disease (MUSIQOLS): A methods feasibility study. Journal of Pain Research, 15, 71-91.

Smith, M., & Brownell, G. (2018). Knowledge, beliefs, attitudes, and behaviors regarding sickle cell disease: Implications for prevention. Social Work and Public Health, 33, 299-316.

Steinberg, M. H. (2008). Sickle cell anemia, the first molecular disease: Overview of molecular etiology, pathophysiology, and therapeutic approaches. Scientific World Journal, 8, 1295-1234.

Wallen, G. R., Minniti, C. P., Krumlauf, M., Eckes, E., Allen, D., Oguhebe, A., Seamon, C., Darbari, D. S., Hildesheim, M., Li, Y., Schulden, J. D., Kato, G. K., & Taylor, J. G. 6th (2014). Sleep disturbance, depression, and pain in adults with sickle cell disease. BMC Psychiatry, 14, 207.