Palliative Care for Adults With Sickle Cell Disease: An Unrecognized Opportunity

Although people with sickle cell disease (SCD) are living longer, their life expectancy remains 20 to 30 years shorter than that of the general public.¹  Recurrent vaso-occlusive crisis, chronic hemolysis, and vessel injury ultimately result in life-limiting complications of the heart, lungs, brain, and kidneys, as well as chronic pain.^1-3  While current therapies including hydroxyurea and chronic transfusion can delay some of these issues, the available treatment options have complications that limit their long-term efficacy.^4-6  Further, affected individuals may suffer from such functional limitations as impaired mobility^7-9  or cognitive issues,^1,10  resulting in decreased quality of life (QoL).

Palliative care (PC) is specialized medical care for people living with a serious illness. It is focused on providing relief from the symptoms and stress of the illness, with the goal of improving QoL for both patients and their families.¹¹  PC emphasizes a patient-centered approach by addressing the physical, emotional, and religious/spiritual needs of individuals with chronic illnesses like SCD. PC is not restricted to implementation during end-of-life (EOL) care; instead, it can be provided simultaneously with treatment to help people live as well and as long as possible. Early integration of PC has been shown to decrease symptom burden; improve QoL;¹²  decrease patient, family, and provider distress;¹³  and decrease health care costs for numerous chronic care conditions.¹⁴ 

Given the symptomology and related burden of SCD, management of the disease is an optimal area for integration of PC. However, despite PC’s benefits, there are barriers to its use in SCD, including limited access to services, stigma surrounding this type of care, and a lack of awareness among providers and patients about the benefits of PC, which can delay access to services and hamper opportunities to improve QoL.

The following cases illustrate how PC can be successfully integrated into SCD care.

Ms. Smith is a 49-year-old teacher with hemoglobin SC and end-stage renal disease (ESRD). Her nephrologist has recommended she initiate hemodialysis, but she has yet to start treatment. At her follow-up outpatient hematology appointment, Ms. Smith’s SCD doctor asks how she felt about her recent nephrology visit and the recommendation for hemodialysis. She says she is hesitant to proceed.

Aware of the increased risk of ESRD for those with SCD, Ms. Smith’s hematologist consults a PC clinician to meet with her and discuss her experiences, Ms. Smith reveals that several of her extended family members have also been afflicted with SCD:

• One of her cousins was admitted to the intensive care unit with acute chest syndrome and required hemodialysis. Unfortunately, he died several days later.

• An aunt with SCD also had ESRD but struggled with peritoneal dialysis. She opted to initiate hemodialysis as an outpatient but then had to rely on family members to transport her to the dialysis center. Between her dialysis sessions and office visits, she felt as though she was putting unnecessary burden on her family members.

• Ms. Smith has two adult children who are married with children of their own and does not want to inconvenience anyone. At the same time, she enjoys spending time with her grandchildren.

Upon eliciting this information, Ms. Smith’s hematologist, nephrologist, and PC physician clarify how hemodialysis can allow her to spend time with family. Relieved, she speaks with her family about coordinating transportation to the local dialysis center.

Mr. Jones is a 32-year-old man with hemoglobin SS and a history of chronic pain and increased hospitalizations over the past two years despite receiving chronic transfusion therapy and escalated doses of home opioids. He was readmitted and started on a patient-controlled analgesia pump with minimal relief. Mr. Jones’ health care providers reach out to the inpatient hematology consult service.

Mr. Jones’ inpatient PC service is consulted for psychosocial support:

• He reports generalized pain of 9/10 in severity, noting only minimal relief with intravenous (IV) opioids. He does not feel that any medications have offered long-lasting improvement.

• In exploring the impact of pain on Mr. Jones’ life, the PC clinician learns how pain has prohibited him from working, enjoying time with friends, and attending weekly church services.

• He reports feeling depressed and having lost interest in his previous hobbies.

• He copes with his pain through prayer, though wonders why God is punishing him.

Based on the information provided by Mr. Jones, the PC clinician recognizes the need to address his “total pain” and recommends additional consults to psychiatry, chaplaincy, and pain management. As Mr. Jones’ SCD therapy is already optimized with chronic transfusions, he is started on buprenorphine and antidepressant therapy and titrated off IV opioids over the next several days. During this interval, he meets with the chaplain to address his religious/spiritual distress.

Mr. Jones is discharged to weekly follow-up in the SCD clinic, where he reports reduced pain (6/10) and reveals that he attended church for the first time in months.

Adults with SCD often suffer from significant symptom burden and related complications. With a growing proportion living into adulthood, there is an urgent need for models that incorporate PC into care for this patient population. Together, PC clinicians can work with SCD centers to improve patients’ experiences and enhance their QoL.

Drs. Patel, Nwogu-Onyemkpa, Kanter, and Osunkwo indicated no relevant conflicts of interest.