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    • AAHPM |  Publications |  AAHPM Quarterly | Winter 2024 |  The Role of Palliative Care in Caring for Patients with Sickle Cell Disease

    The Role of Palliative Care in Caring for Patients with Sickle Cell Disease

    Arshia Madni, MD FAAP, Rushil V. Patel, MD, and Jon P. Furuno, PhD FSHEA FAAHPM

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    Sickle cell disease (SCD) comprises a group of life-limiting, inherited blood disorders characterized by abnormal formation of red blood cells. An estimated 100,000 persons in the US have SCD, of which approximately 90% are Black or African American and 3% to 9% are Hispanic, with smaller numbers of South Asian or Middle Eastern ancestry.1,2 In this article, we describe a clinical case of a young man with SCD and opportunities for palliative care to improve the patient, family, and provider experience.

    Clinical Case

    Don is a 20-year-old male with hemoglobin SS SCD admitted to the hospital for pain crisis. He has had many inpatient encounters as a child, including bouts of vaso-occlusive crisis, and has had a history of acute chest syndrome as well. Don and his family have felt the transition to the adult world from pediatrics has not been smooth and are frustrated by the lack of communication with his clinical team. Today he is in his hematologists’s office talking about the possibility of gene therapy as a next step. The hematologist wants to discuss this next step and, in the setting of the recent communication stressors, would like to consult palliative care.  

    Impact of Sickle Cell Disease on Patients and Their Families

    Patients with SCD are living longer than before but continue to face the burdens and challenges of those living with a chronic medical condition.3 Life-threatening symptoms related to vaso-occlusive crisis, hemolysis, and pain can also lead to repeated hospital admissions.3,4 Along with the physical discomfort, patients face psychological sequalae related to chronic pain and life disruptions including missing work or school.5

    An additional challenge can be ineffective communication and counseling from providers, leading to suboptimal delivery of essential information around anticipatory guidance, preventive care, and future therapies. Racism, bias, and discrimination embedded in clinical encounters can also lead to miscommunication and poor health outcomes.5 This can manifest as clinicians viewing patients’ expression of pain with skepticism, especially if they do not “look” like they are in pain or have been in the hospital frequently for pain crises.5,6

    Opportunities for the Palliative Care Clinician

    Improving the experience of patients with SCD and their families and addressing inequities in the provision of their care should be institutional priorities. A recent analysis reported that fewer than 1% of patients with SCD admitted to the hospital received a palliative care consultation.5 Furthermore, several disparities were identified, including that patients who are Black, Hispanic, female, using Medicaid, or receiving care at a rural or urban nonteaching hospital were significantly less likely to be seen by palliative care.6 Additionally, although hematologists generally find that palliative care is beneficial for patients with SCD, they do not consult as frequently as they would like, especially in end-of-life issues.7 Stigma and misunderstanding of palliative care services among both patient and providers (hematology and palliative care alike) are potential barriers.

    Advocating at your institutions to have palliative care embedded in the care of patients with SCD can help bridge the gap with anticipatory guidance and aid in shared decision making, leading to more equitable and patient-centered care. Palliative care skills that can be utilized include initiating goals-of-care conversations, addressing total pain and suffering, and aiding in shared decisions around advanced therapies (eg, gene therapy).8

    Return to Don

    The palliative care team was able to meet with Don and his family and hear about their experience with sickle cell disease, their hopes, and their worries. They were able to provide Don’s hematologist with psychosocial context that allowed them to better connect with Don and build a more trusting relationship. The palliative care team explored Don’s thoughts about gene therapy and found that Don and his family’s hesitation about gene therapy was related to inadequate information about the risks and benefits as well as feeling unheard. With the ability to build a therapeutic, trusting relationship and feel heard, along with being armed with more information, Don and his family began to feel that gene therapy was in line with their goals.

    References

    1. Kayle M, Blewer AL, Pan W, et al. Birth prevalence of sickle cell disease and county-level social vulnerability—sickle cell data collection program, 11 states, 2016-2020. MMWR Morb Mortal Wkly Rep. 2024;73(12):248-254. doi:10.15585/mmwr.mm7312a1.
    2. Brousseau DC, Panepinto JA, Nimmer M, Hoffman RG. The number of people with sickle-cell disease in the United States: national and state estimates. Am J Hematol. 2010;85(1):77-78.
    3. Patel RV, Nwogu-Onyemkpa E, Kanter J, Osunkwo I. Palliative care for adults with sickle cell disease: an unrecognized opportunity. The Hematologist. 2024;21(5).
    4. Sickle Cell Disease. American Society of Hematology. Accessed October 18, 2024. https://www.hematology.org/education/patients/anemia/sickle-cell-disease.
    5. Aurora T, Bridges C, Porter A, Madni A. Pediatric sickle cell disease: a case for improved clinician–family partnerships. J Hosp Med. 2024;19(2):146-148.
    6. Nwogu-Onyemkpa E, Dongarwar D, Salihu HM, et al. Inpatient palliative care use by patients with sickle cell disease: a retrospective cross-sectional study. BMJ Open. 2022;12(18):e057361. doi:10.1136/bmjopen-2021-057361.
    7. Nwogu-Onyemkpa E, Saleem N, Amspoker A. Sickle cell disease providers’ perspectives on palliative care. J Pain Symptom Manage. 2024;67(5):e539-e540.
    8. Patel RV, Johnston EE. End-of-life care for people with sickle cell disease: barriers to and facilitators of high-quality care. Hematology Am Soc Hematol Educ Program. 2024;2024(1):355-362.

    Arshia Madni, MD FAAP, is an assistant professor of pediatrics and associate program director of the hospice and palliative medicine fellowship at the University of Tennessee Health Sciences Center/Le Bonheur Children’s Hospital. She also is chair of AAHPM’s DEI Committee.

    Rushil V. Patel, MD, is an assistant professor of medicine in the division of hematology and oncology and the division of geriatrics, gerontology, and palliative medicine at the O’Neal Comprehensive Cancer Center at The University of Alabama at Birmingham.

    Jon P. Furuno, PhD FSHEA FAAHPM, is a professor in the department of pharmacy practice at Oregon State University College of Pharmacy.