Shared Decision-Making for Patients with Sickle Cell Disease and their Families
Sickle cell disease (SCD) is an inherited hemoglobin disorder that can lead to worsening and permanent damage to vital organs, excruciating episodes of pain, and ultimately a truncated life expectancy. Blood and marrow transplant (BMT) is the only curative therapy. Outcomes for patients with SCD who undergo BMT are excellent (>90%), especially when the patient is a child with a matched sibling donor.1,2
Despite proven outcomes and willingness by patients and families to accept the associated risks of mortality and graft-versus-host disease (GVHD), BMT remains significantly underutilized.3,4
With SCD, patients and families face ongoing treatment decisions
Shared decision-making (SDM) is a buzzword in hospitals and clinics throughout the U.S. and yet there are barriers to consistent application and evaluation. The practice sounds simple enough. SDM includes the process of communication, deliberation and decision-making.5 However effective communication of complex, and sometimes uncertain, information when there are myriad psychosocial and logistical barriers such as low health literacy and limited time, is no simple feat.
Sometimes SDM is straightforward because the evidence for BMT is clear. But for SCD, a chronic disease, SDM can be more challenging. SCD can be managed with hydroxyurea and supportive care, so in the absence of severe strokes and frequent pain crises, a person can lead a relatively normal life. Yet, when the disease progresses and causes worsening organ damage, BMT may be appropriate, or worse – too late.
A 2018 qualitative study by Khemani et al. found that key factors influencing patients and families to pursue BMT include heavy disease burden, worry about SCD, strong family support, and availability of a matched sibling donor.6
In the end, the decision to undergo BMT has serious implications for a patient’s health, finances and quality of life, so it must be the patient's decision with family support. Through patient education, advocacy, clarifying values, and facilitating communication, you can support the SDM process. And yet a key element of SDM may still be missing: trust.
How to increase shared decision-making? Promote trust in your communication with patients
Research suggests that patients’ mistrust of the healthcare system directly affects the quality of their relationship with their health care team and their clinical decisions.7,8 Trust is a fundamental component of every interaction with patients. To enhance trust with your patients, recommendations include:
- Participation in cultural competency trainings
- Learn how sociocultural factors affect your patients’ values, beliefs, behaviors and clinical decision-making
- Make time for your patients to tell you their thoughts and concerns about treatment options, including BMT
- Give your patients time to consider their options
- Involve family members in the teaching and discussion
- Be transparent about their treatment options
You play an important role to foster trust, support SDM and empower patients to make informed treatment decisions and actively participate in their own treatment plan. Finally, to build and demonstrate trust allow the patient and family to make the final decision.
Resources for you
- CME/CE Activity: Improving Sickle Cell Disease Outcomes. Treatment decision-making, curative therapies and overcoming barriers to care: BeTheMatchClinical.org/cureSCD
- SCD Decision Aid: SickleOptions.org
- Find SCD clinical trials through the Jason Carter Clinical Trials Program: JCCTP.org
- Sources for free, online cultural competency training:
- Think Cultural Health offered by the Department of Health & Human Services Office of Minority Health
- Cultural Competency and Health Equity Training, offered by Cigna
- Gluckman E, Cappelli B, Bernaudin F, et al. Sickle cell disease: an international survey of results of HLA-identical sibling hematopoietic stem cell transplantation. Blood. 2017;129:1548-1556.
- Walters MC, De Castro LM, Sullivan KM, et al. Indications and results of HLA-identical sibling hematopoietic cell transplantation for sickle cell disease. Biol Blood Marrow Transplant. 2016;22:207-211.
- Walters MC, Patience M, Leisenring W, et al. (1996). Barriers to bone marrow transplantation for sickle cell anemia. Biology of Blood and Marrow Transplantation, 2: 100-104.
- Roth M, Krystal J, Manwani D, Driscoll C, Ricafort R. (2012). Stem cell transplant for children with sickle cell anemia: parent and patient interest. Biology of Blood and Marrow Transplantation, 18(11): 1709–1715.
- Center for International Blood and Marrow Transplantation Research. Number of blood and marrow transplants for sickle cell disease 1990-2012. May 2013.
- Institute of Medicine. (2014). Shared Decision-Making Strategies for Best Care: Patient Decision Aids. Retrieved from https://nam.edu/wp-content/uploads/2015/06/SDMforBestCare2.pdf
- Khemani, K., Ross, D., Sinha, C., Haight, A., Bakshi, N. & Krishnamurti, L. (2018). Experiences and Decision Making in Hematopoietic Stem Cell Transplant in Sickle Cell Disease: Patients' and Caregivers' Perspectives. Biology of Blood and Marrow Transplantation, 24(5):1041 – 1048.
- Haywood C, Ratanawongsa N, et al. (2010). The Association of Provider Communication with Trust among Adults with Sickle Cell Disease. Journal of General Internal Medicine, 25(6): 543–548.
- Benkert R, Peters RM, Clark R, Keves-Foster K. (2006). Effects of perceived racism, cultural mistrust and trust in providers on satisfaction with care. Journal of the National Medical Association, 98(9): 1532–1540.