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Comprehensive plan for managing acute and chronic pain in sickle cell disease

Comprehensive plan for managing acute and chronic pain in sickle cell disease
Chronic pain management plan and strategies to reduce pain episodes
  • Take prescribed medications regularly, including long-acting opioid medications and/or methadone if appropriate
  • Have short-acting opioids available (and a clear plan for their use) for breakthrough pain
  • Maintain hydration
  • Avoid extremes of temperature and stress
  • Possible use of medications for neuropathic pain
  • Discussion of hydroxyurea or other disease-modifying therapies (eg, L-glutamine, regular transfusions) when well as an outpatient, if appropriate
  • Address psychosocial issues (eg, depression, social isolation)
Acute pain – Prompt assessment and treatment
  • Distinguish between acute vaso-occlusive pain events and non-SCD pain
    • Treat minor injuries with rest, heat, elevation, and over-the-counter analgesics (acetaminophen, NSAIDs)
    • Treat pain preceded by fever, dehydration, temperature extremes, or other stresses as vaso-occlusive (sickle cell) pain
  • Treat with escalating therapy
    • Start with nonpharmacologic therapies (eg, relaxation/breathing exercises)
    • Add nonopioid therapies (eg, acetaminophen, NSAIDs) if pain is not controlled
    • Add short-acting opioids
  • Avoid
    • Avoid application of ice or cold compresses, which can precipitate sickling
    • Avoid ketorolac in hospitalized patients and for prolonged periods, due to concerns about toxicities
  • Identify and treat other potential SCD complications
    • Pain with fever or cough may indicate respiratory infection or early acute chest syndrome and requires pain management and communication with sickle cell clinic
    • Pain with dyspnea, focal neurologic signs, splenic enlargement, and/or signs of severe anemia requires emergency medical attention and evaluation for possible acute chest syndrome, stroke, splenic sequestration, or aplastic crisis
Management of opioid side effects
  • Use stimulant laxatives for constipation; osmotic agents are not as effective in opioid-induced constipation 
  • Use nonsedating (H1-antagonist) antihistamines for pruritus if needed
  • Use antiemetics for nausea if needed; a selective 5-HT3 receptor antagonist such as ondansetron should be the first choice because of minimal CNS findings
  • Treat insomnia with nonpharmacologic or pharmacologic approaches as needed
Refer to UpToDate for additional information.
SCD: sickle cell disease; NSAIDs: nonsteroidal anti-inflammatory drugs; CNS: central nervous system.
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