The American Society of Hematology (ASH) released evidence-based 2020 guidelines on the management of acute and chronic pain in pediatric and adult patients with sickle cell disease (SCD). The guidelines aim to support patients, clinicians, caregivers, hematologists, and other healthcare workers with decisions associated with pain management in SCD.

The lack of understanding of the biological complexities associated with acute and chronic pain, combined with the lack of evidence on which to base recommendations, has made the management of pain for children and adults with SCD a significant clinical challenge. The administration of opioids is the current treatment standard for acute pain in patients with SCD who seek care for these symptoms. Consequently, few studies have been conducted on the use and role of nonopioid pharmacological and nonpharmacological treatments for acute and chronic SCD pain. Ultimately, these gaps in knowledge and treatment have led to barriers in effective and targeted interventions.

ASH formed a multidisciplinary panel to develop guidelines on this topic, which included 2 patient representatives. The process for developing the guidelines, including updates and systematic review, was supported by the Mayo Evidence-Based Practice Research Program.

Ranging from pharmacological to nonpharmacological interventions and analgesic delivery, the panel decided on 18 recommendations for the management of acute and chronic pain; however, due to low certainty evidence, most recommendations made are conditional following close balance of benefits and harms.


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The guideline recommendations were either labeled as strong or conditional, the interpretations of which are labeled below for patients and clinicians (Table).

Table. Interpretation Definitions of Strong and Conditional Recommendations

 For PatientsFor Clinicians
Strong recommendationsMost patients would want the recommended course of action; only some would not.The majority of patients would want the recommended course of action, but many would not. Decision aids may be helpful to aid patients to make decisions that align with their values and preferences.
Conditional recommendationsMost clinicians should adhere to the recommended course of action. Formal decision aids are most likely not required to help patients make decisions that align with their values and preferences.Clinicians must recognize that individual patients may require different choices that reflect their values and preferences, and that not all options are appropriate for all patients. Formal decision aids may be beneficial for clinicians to help individual patients make decisions.

Using the Grading of Recommendations Assessment, Development and Evaluation approach to assess evidence and make recommendations, the panel categorized recommendations into 4 levels of certainty: high certainty, moderate certainty, low certainty, and very low certainty. For the full list of recommendations, refer to the complete guidelines, published in Blood Advances.

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

For pediatric and adult patients with SCD who present to an acute care setting with SCD-related acute pain, the ASH panel recommended the assessment and administration of analgesia within 1 hour of emergency department arrival with frequent reassessment every 30 to 60 minutes to optimize pain control (strong recommendation based on low certainty in evidence about effects).

For pediatric and adult patients with SCD who present to an acute care setting with SCD-related acute pain for whom opioid therapy is indicated, the ASH panel suggested tailored opioid dosing. Dosing should be based on baseline opioid therapy and previous effective therapy (conditional recommendation based on moderate certainty in evidence about effects for adults; conditional recommendation based on low certainty in the evidence about effects for children).