Weaving palliative principles into sickle cell disease care

If you log on to PubMed.gov, an online repository of all published scientific research, and search for literature including the terms “palliative care” and “cancer,” more than 40,000 articles pop up. When Griffin Collins, MD, Department of Oncology, ran a similar search, replacing “cancer” with “sickle cell disease,” the result was just 83 articles. Considering sickle cell disease is a major cause of severe symptoms and even death in children and young adults, the lack of literature on the subject was startling.

Collins is an expert in palliative care — therapies designed to improve a patient’s quality of life during treatment for serious illness. He began by exploring its impact on patients undergoing stem cell transplantation during a fellowship at the University of California, San Francisco. Believing that palliative care could offer longitudinal, person-centered support for patients with sickle cell disease, he wanted to expand the scope of his work. He found a like-minded collaborator at St. Jude in Erica Kaye, MD, MPH, Department of Oncology.

The pair quickly teamed up with Mgbechi Erondu, MD, Stanford University, and Eberechi Nwogu-Onyemkpa, MD, Washington University in St. Louis, physician-scientists with a demonstrated history of advocating for integrating palliative care into sickle cell disease treatment. The team drew from their own experiences working in palliative care and interacting with patients with sickle cell disease to characterize the problem. They called for a change in clinical approaches to improve palliative care access, published as a call-to-action in the New England Journal of Medicine.

“Patients with sickle cell disease are an important group of people who can benefit from palliative care — really meeting them where they are and supporting them through their illness — regardless of prognosis,” said Collins.

Palliative care’s unique role

Sickle cell disease is a genetic disorder leading to an aberrant hemoglobin molecule that turns the flexible surface of a red blood cell into a sticky sickled shape. These malformed cells block blood vessels and poorly distribute oxygen throughout the body. Sickle cell disease can result in complications, including acute pain crises, severe infections, multisystem organ damage and early mortality. Despite its global prevalence, afflicting approximately 7 million people worldwide, the disease has been a historically underappreciated condition. While anyone can carry the gene that causes sickle cell, it primarily affects people of African, Hispanic, Middle Eastern, Indian and Mediterranean descent.

“The connection between sickle cell disease and race can lead to implicit biases and systemic and structural racism that touch clinical care, education and research,” said Kaye.

Communities that experience these burdens can lack trust in health care, but this barrier to adequately supporting patients with sickle cell disease is surmountable. In their call-to-action, the researchers posit that palliative care teams are uniquely situated to mitigate differences in health care experience for patients from underrepresented backgrounds with serious illness. They achieve this through intentional relationship-building.

“Palliative care specialists are experts in caring for persons who experience severe chronic illness,” explained Erondu. “Due to the interdisciplinary nature of their field, they are particularly equipped to respond to the biological, psychological, social, spiritual or total pain some persons living with sickle cell experience.”

“The secret sauce is the longitudinal relationship,” said Collins. “If you come into a room and you see me doing my job, it might look like I’m just hanging out with a patient, but what I’m doing is being intentional about demonstrating that I’m a trustworthy person and developing that relationship.

“This means that when challenges come, when tough conversations are happening, it’s not happening with a stranger,” he continued. “It’s happening with someone they already know and trust.”

An extra layer of support

This past June, St. Jude hosted the inaugural National Palliative Care in Sickle Cell Disease Summit. At the summit, palliative care and sickle cell experts interested in optimizing care met to discuss the best practices and share concerns. Some summit attendees expressed trepidation about palliative care, citing concerns about taking away from the efforts to find curative therapies and conflating palliative with end-of-life care.

The call-to-action research team suspects these are common concerns among those unfamiliar with palliative care work, and they aim to allay such apprehensions. Nwogu-Onyemkpa emphasized that “palliative care does not take away from the current care that individuals with sickle cell disease receive. Rather, it adds an extra layer of support for those experiencing life-altering changes due to this insidious disease. Palliative care can enhance the management of factors that reduce the quality of life for those affected by this condition.”

For patients with sickle cell disease, those factors vary daily. While the team did not wish to be overly prescriptive in their call to action, according to Collins, palliative care teams often offer patients and their families support. They help manage symptoms, such as pain crises or symptoms associated with intensive therapies; assist in coordinating care, especially during care milestones such as transitioning to adult care; and facilitate clear communication between the patients and their clinical care team.

Reaching more people

It will take time to fully integrate palliative approaches into sickle cell disease care. Ensuring that there are enough trained palliative care practitioners will require a shift in training and culture so that clinicians can incorporate palliative principles into their practice and, thus, reach more patients and families.

Additionally, Collins expressed interest in implementation models for other institutions to adapt. “My hope is that, with our resources here at St. Jude, we can develop models for integrating palliative care for people with sickle cell disease that are easily adaptable and implemented, even in settings where resources aren’t as readily available. Through such models, we can help not just the patients who are right here and trusting us with their care, but also patients across the country and the globe.”