SICKLE CELL
PAIN MANAGEMENT IN Sickle Cell Disease
James Patrick Murphy, MD, MMM
Patients with sickle cell disease( SCD) must deal with a wide range of painful conditions, e. g. orthopedic, visceral, neuropathic, chronic and acute. Their treatment is often complicated by tolerance to medications, physical dependence and in a small number of cases the development of a substance use disorder. Patients with SCD often encounter underestimation, misunderstanding and inadequate management of their pain. While SCD is a chronic hematologic disorder, often treated in oncology clinics, patients with SCD rarely are afforded the exemption from state pain regulations that patients with cancer-related pain invoke, making prescribers reticent to treat SCD-related pain adequately. These are among the many reasons that SCD patients may receive inadequate pain care, all the more troubling seeing as pain management is perhaps the most salient aspect of caring for the patient afflicted with SCD.
SCD is an inherited disorder manifested by the distortion of hemoglobin when blood oxygen level is compromised, causing the red blood cells to become sickle-shaped. These altered cells adhere to each other and vessel walls, causing decreased circulation to body tissues, which can lead to organ damage, inflammation and pain. Acute pain crises( also known as pain episodes, sickle cell crises or vaso-occlusive crises) are typically about 10 days in duration, unpredictable and occur throughout the life of a SCD patient as frequently as weekly. Each acute painful episode may build upon the next and lead to devastating complications and extensive organ damage such as: bone infarction, avascular necrosis of joints, spinal infarction, intervertebral disk protrusion, leg ulcers, osteomyelitis, multiorgan failure and sudden death. The chronic pain associated
with SCD can lead to comorbid insomnia, anxiety, helplessness, loneliness, despair and depression. The chronic pain component of SCD may be debilitating for months, years, or a lifetime.
Acute exacerbations of chronic pain are the primary reasons for hospital admissions and readmissions, particularly in young adults with SCD. The acute pain of a sickle cell crisis can either be localized or migratory and is often described as continuous and throbbing. SCD crisis pain is severe and will typically cause patients to grunt, groan, cry and contort their bodies. Pain locations may include the head, neck, chest, back, abdomen or limbs.
Optimal pain care is supported by the caregiver’ s acknowledgment that SCD patients have generally acquired expertise in understanding their own pain as, by way of experience, they often know what treatments work best for themselves. Thus, SCD patients should be asked for their input regarding specific medications( including opioids) that have been successful in managing their pain in the past. It is widely accepted that pain care can be more successful when administered in collaboration with the patient’ s suggestions.
Although the severe pain of a SCD crisis demands the most attention, effective management of SCD pain begins at home. Selfcare strategies include analgesics( opioid and nonopioid), physical medicine techniques( e. g., heat, hydration, massage), proper nutrition, avoidance of alcohol and illicit drugs, mindfulness, spirituality and social support. When pain becomes severe enough to warrant transport to a clinic or hospital, patients will often prefer to be treated in an outpatient clinic rather than the emergency department in order to avoid extensive wait times. Regardless of the clinical setting, treatment with parenteral short-acting opioids is usually prescribed, with the goal of promptly and effectively treating the pain in an effort to decrease the overall frequency of hospital admissions and emergency department visits. A typical pain medication regimen
20 LOUISVILLE MEDICINE