Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by sickle-shaped red blood cells that lead to blood vessel occlusion, infarction, organ damage, and inflammation. According to the Centers for Disease Control and Prevention, approximately 90% of the 100,000 people affected with SCD in the United States are Black. Healthcare disparities among Blacks and other racial/ethnic minority groups in the United States are well known and directly related to poor outcomes, with Blacks experiencing the highest rates of illness and lower life expectancy. The purpose of this commentary is to present the assertion that palliative care offers an opportunity to reduce health disparities for individuals with serious illnesses, such as SCD. We support the assertion with exemplar statements from a person with SCD who experienced the benefits of palliative care.
For individuals with SCD, the health disparities are profound. First, as a disease group, SCD receives less federal research dollars than other groups with genetic disorders such as cystic fibrosis. Individuals with SCD also have less access to comprehensive care than other conditions. There is also a scarcity of adult SCD providers. Despite advances in care, DeBaun and colleagues note the median age at death for persons with severe SCD genotypes is 48 years, approximately three decades less than the general Black population. Brousseau and colleagues found the highest rates of acute care encounters and rehospitalizations occur among 18- to 30-year-olds, with pain control being the most common reason individuals with SCD seek care. However, care-seeking is often delayed until the pain is unbearable due to previous adverse experiences in the healthcare system.
“It felt like a hand reached right up my spine, grabbed my chest, and it grabbed the back of my head. And I learned later it was because my spine was actually swollen and inflamed. Everything. Every tendon and connective tissue was inflamed. It was full body, systemic, acute pain.”
The pain of SCD is unimaginable and recurrent over the individual’s lifetime. Yusef and colleagues report pain was the most common reason for the estimated 197,333 annual emergency department (ED) visits by persons with SCD between 1999-2007. Although ED visits may be frequent, persons with SCD may avoid seeking ED care, going only when they have exhausted all treatments at home because they doubt they will successfully obtain pain relief because of previous adverse care experiences. Jenerette and colleagues found that by the time young adults with SCD seek treatment, they report average pain intensity of 8.7 ± 1.2 on a 1 to 10 scale. The unpredictable acute pain episodes of SCD typically last 9 to 11 days; treatment typically includes I.V. fluids and opioids.
“The pain was already bad enough to take me to the ED. And that’s not a thing I do. That’s not a thing we tend to do. Research has shown we go in when it is the absolute worst pain. I was already there. And then my worst pain, what I thought was a ten, I learned very quickly, was really more of a six or seven. I learned what a ten is, and it hit me so fast and so hard that I could barely breathe. I couldn’t speak. My mother says I didn’t open my eyes for days anyway.”
Individuals with SCD endure the psychological and physiological toll of repetitive pain episodes, pain-related symptoms, and the side effects of the pain treatments. For many individuals living with SCD, pain catastrophizing increases with more intense pain, suggesting that persons with SCD feel helpless and less activated in controlling their pain as it intensifies. Persons with SCD experience health-related stigmatization and have described feeling less than human when providers ignore, discount, or deem their pain complaints false. The quality of treatment of a painful SCD crisis currently depends on providers’ abilities to assess the patient’s pain, ultimately deciding whether an individual’s report of pain is credible and requires treatment. There are no objective markers of a pain crisis, so it is important to believe patients.
Additionally, treatment should be based on individualized pain plans, but if unavailable, patients should be treated using institutional SCD-specific pain plans. The treatment plan needs to be informed by the patient. Navigating the intersection between pain and communication is important to achieving health equity in managing SCD pain.
Braveman and colleagues describe health equity as everyone having a fair opportunity to be as healthy as possible, which requires removing obstacles to health, such as access to healthcare. Optimal health for individuals living with SCD requires managing complex challenges, including acute and chronic pain, health-related stigma, and psychosocial issues that impact the quality of life (QoL). Wilkie and colleagues noted more than a decade ago that palliative care for individuals with a serious illness, such as SCD, that’s focused on symptom relief and improved QoL, can help lessen inequities in SCD. Health equity implores that individuals with SCD have access to palliative care to improve their pain outcomes and QoL by providing strategies for SCD management to include cue recognition and response, communication, as well as coping with loss, grief, and planning for the future.
Currently, most individuals with SCD do not have access to palliative care and are not knowledgeable about it. In contrast to a multi-faceted palliative care approach, opioids are the cornerstone of treatment for SCD. However, the current opioid crisis has increased the likelihood that providers will prescribe even less pain medication to this population, who are already at significant risk for inadequate pain management due to race, negative attitudes, and distrust. Improved communication to convey pain and pain-related symptoms with healthcare providers is even more crucial for individuals with SCD in the opioid crisis. Palliative care can remove many of the challenges for individuals with SCD and potentially decrease their suffering.
“I was in excruciating pain all weekend. Monday came around, and I insisted on palliative care. Palliative care came in with a phenomenal woman named Doctor L. I legitimately believe she and my mother’s prayers saved my life. She got me on the right level of pain control.”
The palliative care approach offers strategies to reduce the challenges confronting adults with SCD. Palliative care is a multidisciplinary approach to relieve suffering, increase QoL, and improve the quality of care for individuals with serious illness and their families. Many people equate palliative care with end-of-life care, but palliative care is most effective when integrated with the patient’s care early in the care trajectory, according to a study by McDonald and colleagues. While research is limited, palliative care can improve QoL for individuals with SCD by providing strategies to manage symptoms associated with SCD. Using a retrospective review, Alsirafy and colleagues concluded that palliative care needs of individuals with SCD were similar to those of people with cancer. Based on research by Agarwal and Epstein, palliative care interventions for patients with cancer have been shown to improve outcomes such as QoL, depression, satisfaction with care, and survival. Often, individuals who would benefit from palliative care do not understand it and are resistant to these services because they equate palliative care with hospice and impending death.
“Palliative care is a secret I think everybody needs to know in this community. Palliative care can increase your doses if it is absolutely necessary. I insisted on palliative care. Palliative care came in. Palliative care got me on the amounts I needed.”
The focus of palliative care is to provide aggressive multidisciplinary symptom management, psychosocial and spiritual support, and advance care planning for individuals with serious illnesses, including SCD. Individuals with SCD need symptom management to live their best lives. Nurses and other healthcare providers must educate individuals and families living with SCD about palliative care. In genetic conditions like SCD, where symptoms often begin in childhood, palliative care should also start at this time. Interventions are needed to determine best practices to incorporate palliative care into SCD care and evidence-based practice to support the workforce growth of palliative care to better manage patients over their disease trajectory. Nurses can lead the way in connecting patients with SCD to palliative care by educating them and increasing awareness.
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