Guidelines for Management of Acute and Chronic Pain in Sickle Cell Disease (ASH, 2020)

American Society of Hematology (ASH)

This is a quick summary of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

July 29, 2020

The guidelines on management of acute and chronic pain in sickle cell disease (SCD) were published on June 23, 2020, by the American Society of Hematology (ASH).[1]

Use of Standardized Protocols to Treat Acute Pain in Acute Care Setting

For adults and children with SCD presenting to an acute care setting with acute pain related to SCD, rapid (≤1 hour of emergency department [ED] arrival) assessment and administration of analgesia with frequent reassessments (every 30-60 minutes) to optimize pain control are recommended.

For adults and children with SCD presenting to an acute care setting with acute pain related to SCD for whom opioid therapy is indicated, tailored opioid dosing is recommended, based on consideration of baseline opioid therapy and prior effective therapy.

Nonopioid Pharmacologic Therapies for Acute Pain

For adults and children with acute pain related to SCD, a short course (5-7 days) of nonsteroidal anti-inflammatory drugs (NSAIDs) in addition to opioids is suggested for acute pain management.

For adults and children presenting for acute pain related to SCD, avoidance of corticosteroids for acute pain management is suggested.

For adults and children presenting with acute pain related to SCD who are hospitalized, a subanesthetic (analgesic) ketamine infusion is suggested as adjunctive treatment of pain that is refractory or not effectively treated with opioids alone.

For adults and children presenting with acute pain related to SCD, regional anesthesia approaches are suggested for localized pain that is refractory or not effectively treated with opioids alone.

No recommendation is made for or against intravenous (IV) fluids in addition to standard pharmacologic management for the treatment of acute pain.

Nonpharmacologic Therapies for Acute Pain

For adults and children who seek treatment of acute pain, massage, yoga, transcutaneous electrical nerve stimulation (TENS), virtual reality (VR), and guided audiovisual (AV) relaxation are suggested in addition to standard pharmacologic management.

No recommendation is made for or against acupuncture or biofeedback for the treatment of acute pain in addition to standard pharmacologic management.

Pain Management in SCD-Specific Hospital-Based Acute Care Facility

For adults and children with acute pain episodes requiring hospital care, use of SCD-specific hospital-based acute care facilities (ie, day hospitals and infusion centers with appropriate expertise to evaluate, diagnose, and treat pain and other SCD complications) is suggested over typical ED-based care.

Continuous Basal Opioid Infusion for Acute Pain

For children and adults with SCD who seek treatment of acute pain in the hospital, no recommendation is made for or against basal opioid dosing in conjunction with on-demand dosing or scheduled intermittent dosing.

Nonopioid Pharmacologic Therapies for Chronic Pain With Another Identifiable Cause Besides SCD

For adults with SCD who have chronic (as opposed to episodic) pain from the SCD-related identifiable cause of avascular necrosis (AVN) of bone, use of duloxetine (and other serotonin and norepinephrine reuptake inhibitors [SNRIs]) is suggested as a management option, in the context of a comprehensive disease and pain management plan.

For adults with SCD who have chronic (as opposed to episodic) pain from the SCD-related identifiable cause of AVN of bone, use of NSAIDs is suggested as a management option, in the context of a comprehensive disease and pain management plan.

For children with SCD who have chronic (as opposed to episodic) pain from the SCD-related identifiable cause of AVN of bone, no recommendation is made for or against the use of SNRIs, NSAIDs, or both.

For adults and children with SCD who have chronic (as opposed to episodic) pain from the SCD-related identifiable cause of leg ulcers, no recommendation is made for or against any specific nonopioid pharmacologic management strategy.

Nonopioid Pharmacologic Therapies for Chronic Pain With No Identifiable Cause Beyond SCD

For adults who have SCD-related chronic pain with no identifiable cause beyond SCD, SNRIs (eg, duloxetine and milnacipran) are suggested as options for pain management.

For adults who have SCD-related chronic pain with no identifiable cause beyond SCD, tricyclic antidepressants (TCAs; eg, amitriptyline) are suggested as options for pain management.

For adults who have SCD-related chronic pain with no identifiable cause beyond SCD, gabapentinoids (eg, pregabalin) are suggested as options for pain management.

Nonpharmacologic Therapies for Chronic Pain

For adults and children with SCD who have chronic pain related to SCD, cognitive and behavioral pain management strategies are suggested, in the context of a comprehensive disease and pain management plan.

For adults with SCD who have chronic pain related to SCD, other provider-delivered integrative approaches (eg, massage therapy and acupuncture) are suggested, as available, as tolerated, and conditional on individual patient preference and response. They should be delivered in the context of a comprehensive disease and pain management plan.

For adults and children with SCD who have chronic pain related to SCD, no recommendation is made for or against a number of physical activities, exercise, or combined meditation/movement programs (eg, aerobic exercise, yoga, and Pilates) to improve pain and disability.

Chronic Opioid Therapy (COT) for Chronic Pain

For adults and children with SCD and emerging or recently developed chronic pain, it is suggested that COT not be initiated unless pain is refractory to multiple other treatment modalities.

For adults and children with chronic pain from SCD who are receiving COT, are functioning well, and have perceived benefit, shared decision making is suggested for continuation of COT.

For adults and children with chronic pain from SCD who are receiving COT, are functioning poorly, or are at high risk for aberrant opioid use or toxicity, it is suggested that COT not be continued.

Chronic Transfusion Therapy for Recurrent Acute Pain or Chronic Pain

For adults and children with SCD and recurrent acute pain, it is suggested that chronic monthly transfusion therapy not be used as a first-line strategy to prevent or reduce recurrent acute pain episodes.

For adults and children with chronic pain from SCD, no recommendation is made for or against chronic monthly transfusion therapy as an option for pain management.

For more information, please go to Sickle Cell Anemia.

For more Clinical Practice Guidelines, please go to Guidelines.

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