Sickle cell disease (SCD) affects about 100,000 Americans as an inherited genetic disorder with intermittent exacerbations requiring hospitalization. SCD is also a painful and complicated disease with no single physician specialist that can provide pain relief. While SCD pain is similar in severity to cancer pain, patients struggle to find adequate pain relief because they are often labeled as “doesn’t appear in pain” as the imaging scans may not show actual pathology.
Some palliative colleagues draw the comparison that cancer is visible in scans and SCD is not, which gives mental ease to clinicians as cancer patients’ pain is easier to believe and treat appropriately. However, the source of SCD pain is typically micro-vascular because the disease generates pain due to occlusion in the micro-vascular. It can’t be “seen” on scans except when worse complications like acute chest syndrome or avascular necrosis of a bigger joint are present.
Patients with SCD, who are likely dealing with untreated pain, are typically perceived as “difficult, demanding, and verbally aggressive,” especially in situations when their pain is under-treated. In some cases, the entire medical teams, including nurses, are offended by the “attitudes” of patients with SCD. It often results in “Discharge against medical advice (AMA),” which is notoriously high in the SCD patient subset, widening the mistrust gap and negatively impacting medical care for patients with SCD.
Clinicians’ limited knowledge about treatment options further reduces positive pain management experience for patients with SCD, and primary care physicians may hesitate to prescribe opioids. Opioids are the mainstay medications and lifeline for SCD pain, but they can be a double-edged sword that opens a pandora’s box of problems and side effects, including addiction, tolerance, pseudo-addiction, respiratory depression, and death. Hematologists are also uncomfortable prescribing high doses of pain medicine that are usually needed for managing SCD pain. Hydroxyurea does have proven efficacy for SCD pain, but it is only prescribed to 25% of the adults with SCD to assist with disease management and pain crisis episodes.
Patients with SCD usually start using intravenous pain medicines for their vaso-occlusive crisis (VOC) very early in life, with the majority around six months of age. The Pediatrics sickle cell programs are comprehensive with extensive support for the patient and caregivers, but the pediatric SCD program fails to prepare SCD patients for disease-related adulthood challenges.
Despite calls for a palliative approach to manage sickle cell disease, patients continue to struggle to receive appropriate palliative services because SCD has not been considered a “palliative disease,” as the majority of the palliative program services are restricted to cancer patients only.
We offer the option of following in the palliative clinic for ongoing chronic pain management. Our palliative team collaborates with SCD hematologists and the urgent care center to provide comprehensive care to the SCD patient population.
We followed one SCD patient’s life trajectory from 2011- to 2020. From 2011- to 2015, the patient has spent 343 days of life with an average of 68.6 days/year inside the hospital. Two more charts were reviewed, and they showed similar results. The patient’s acute and chronic pain was appropriately managed by using long-acting and short-acting opioids with opioid adjuvants. Along with medications, non-pharmacologic measures, like meditation and mindfulness, were utilized to assist with pain control and resources for social issues.
A significant worry in the current environment of opioid deprescribing is that it may push patients with SCD to use illicit street drugs for pain relief. The need of the hour is to take aggressive and urgent steps to increase disease awareness, educate the clinicians, and develop coordinated care models to better serve this patient population.
Palliative care providers know how and when to discuss SCD care goals and better manage pain. Increased outpatient access to palliative care would lower emergency department visits and hospital admissions for uncontrolled SCD pain and result in reduced health care utilization. Palliative care can remedy the mistrust of patient-reported pain, fear of opioid use disorder, and growing provider discomfort with using high-dose opioids.
By developing coordinated palliative care models (as described above), steps could be taken to increase disease awareness and educate clinicians to effectively manage SCD pain while supporting patients with SCD in enjoying a higher quality of life outside of the hospital.
Ramandeep Kaur is a palliative medicine physician.
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