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