Sickle cell disease pain: acute crisis and chronic pain

Treat a vaso-occlusive crisis fast: analgesia within 1 hour, reassessment every 30–60 minutes, individualized opioids, plus short NSAIDs; add ketamine or regional techniques for refractory pain, and avoid corticosteroids for the pain itself.

1. Speed comes first. ASH recommends assessment and analgesia within 1 hour of emergency department arrival, with reassessment every 30–60 minutes; subcutaneous and intranasal routes help when IV access is delayed ¹.
2. Tailor opioid doses to the patient. ASH suggests dosing based on baseline opioid therapy and prior effective treatment, ideally from an individualized care plan written with the sickle cell team ¹.
3. Use multimodal adjuncts. ASH suggests a 5–7 day NSAID course added to opioids, and massage, yoga, TENS, virtual reality or guided relaxation alongside standard drugs ¹.
4. Escalate refractory pain without steroids. For hospitalized patients with opioid-refractory pain, ASH suggests subanesthetic ketamine, and epidural or peripheral nerve catheters for localized refractory abdominal, hip or leg pain; it suggests against corticosteroids for acute pain ¹.
5. Chronic pain needs a different plan. For chronic pain without another identifiable cause, ASH suggests SNRIs, TCAs or gabapentinoids, largely from fibromyalgia data, plus cognitive and behavioral strategies ¹.
6. Be cautious starting chronic opioids. ASH suggests against starting chronic opioid therapy for new chronic pain unless it is refractory to multiple other treatments, and shared decision-making for patients already functioning well on it ¹.

Steroids for the crisis pain itself are suggested against, but should still be given for other indications such as asthma. Crisis pain is also excluded from CDC opioid guidance, so outpatient limits do not apply ¹³.

• Subanesthetic ketamine starts at 0.1–0.3 mg/kg per hour, maximum 1 mg/kg per hour, in centers with expertise ¹.
• Reported ketamine harms in sickle cell disease include nystagmus, hallucinations, dizziness and dysphoria ¹.
• For avascular necrosis pain, ASH suggests duloxetine or another SNRI, or NSAIDs ¹.
• ASH suggests day hospitals or infusion centers over typical emergency department care for crises ¹.
• Chronic monthly transfusion is suggested against as first-line prevention of recurrent pain ¹.
• NHLBI strongly recommends hydroxyurea for adults with three or more severe crises in 12 months ².
• NHLBI strongly recommends incentive spirometry for patients hospitalized with a crisis ².
• The CDC 2022 opioid guideline excludes pain related to sickle cell disease ³.

• Delaying opioids until an NSAID has "failed"; NSAIDs are added to opioids ¹.
• Applying CDC 2022 outpatient limits to sickle cell crisis pain ³.
• Using corticosteroids to shorten a pain crisis ¹.
• Expecting a fixed definition of "refractory" pain; ASH notes none is standardized or widely accepted ¹.