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Understanding the Clinical Burden of Sickle Cell Disease
Sickle cell disease (SCD) is an inherited hemoglobinopathy affecting over 100,000 individuals in the United States and more than 7 million globally. It is caused by a specific point mutation in the beta-globin gene that promotes polymerization of deoxygenated hemoglobin, transforming normally flexible erythrocytes into rigid, sickle-shaped cells. This fundamental change in cellular morphology drives a relentless cycle of chronic hemolysis and vaso-occlusive crises, precipitating acute and often life-threatening complications. Clinicians managing SCD must navigate a spectrum of high-acuity events, ranging from the rapid respiratory decline seen in acute chest syndrome to the devastating neurological impact of ischemic strokes and the profound physical distress of vaso-occlusive pain.
From Acute Crises to Chronic Organ Damage
The burden of the disease extends far beyond these acute episodes, as cumulative ischemic injury eventually manifests as multi-system organ dysfunction. Over time, patients often develop chronic sequelae such as avascular necrosis of major joints, heart failure, and chronic kidney disease, frequently complicated by secondary iron overload from necessary transfusion therapies. Despite the advancements in newborn screening, the expansion of penicillin prophylaxis, and the emergence of disease-modifying therapies like Hydroxyurea, a staggering mortality gap persists. In the United States, the average life expectancy for an individual with SCD remains approximately 20 years shorter than that of the general population, a disparity that is even more pronounced in resource-limited global settings. Even as survival improves, the threat of sudden, unexpected death remains an ever-present reality for people living with this insidious disease.
The Human Toll: Living with “Total Pain”
Individuals with SCD typically experience their first symptoms in early infancy and face the consequences of this chronic, progressive illness throughout their lives. This clinical reality creates a state of “total pain” for the patient—a confluence of physical, psychological, social, spiritual, and existential distress. The unpredictability of the disease frequently disrupts education and career trajectories, while the transition from pediatric to adult care often exposes patients to fragmented medical support. While the ultimate treatment goal reported by patients is an improved quality of life, those living with SCD consistently report lower quality-of-life scores than patients with other chronic illnesses. This deficit highlights a critical underutilization of Palliative Care (PC) services. Historically, the integration of PC has been hindered by the misconception that it is synonymous with end-of-life care, yet its interprofessional and patient-centered approach is uniquely suited to the SCD population.
The Case for Early Palliative Care Integration
By integrating PC early in the disease trajectory, medical teams can provide more robust symptom management, facilitate complex serious illness conversations, and offer the psychosocial support necessary to mitigate caregiver burden. Furthermore, the inclusion of PC helps address the deep-seated health disparities and structural inequities that have long plagued SCD care. Patients, predominantly of African, Southeast Asian, and Mediterranean descent, often face systemic racism and provider bias that stigmatize their pain and marginalize their clinical needs. Palliative Care clinicians have a unique opportunity to model a person-centered, goal-directed standard of care that treats the whole person rather than just the pathology.
With tomorrow being World Sickle Cell Day, we wanted to raise awareness of the global burden of SCD and underscore the urgent need to improve care, reduce disparities, and expand access to comprehensive support. As we move toward a future defined by high-risk curative interventions like gene therapy, the role of PC as an essential layer of support is more vital than ever to ensure that every patient achieves the quality of life they deserve.
Want to continue the conversation? Members can participate in the Sickle Cell Disease Discussion Group on Connect. To join the discussion group, follow these steps:
- Login to your My Account on aahpm.org
- Click My Communities on the left‑hand menu.
- Check the box next to the Sickle Cell Disease Discussion Group
- Click Save to confirm your selections.
Meet the Authors
Eberechi Nwogu-Onyemkpa, MD MHS FACP FAAHPM
Dr. Eberechi Nwogu-Onyemkpa is an Assistant Professor of Medicine in the Division of Palliative Medicine at Washington University in St. Louis. She is committed to expanding access to palliative care and hospice for patients with non-malignant diseases. Her clinical and research interests focus on integrating palliative care into the management of advanced heart failure and sickle cell disease.
Jemella Raymore, MD
Dr. Jemella Raymore is a board-certified Internal Medicine and Palliative Care physician who completed training at New York-Presbyterian Hospital/Columbia University Medical Center and Weill Cornell Medical Center. She serves as a Palliative Medicine specialist in both inpatient and outpatient clinical care settings. Her interests focus on increasing the pipeline of students at all academic levels interested in pursuing careers in medicine or research and decreasing burnout via the arts and humanities.