Designed and created by Guideline Central in participation with the American Society of Hematology
Sickle Cell Disease Management of Acute and Chronic Pain
Patient Guideline Summary
Publication Date: June 1, 2020
Last Updated: March 3, 2023
Objective
Objective
This patient summary means to discuss key recommendations from the American Society of Hematology (ASH) for acute and chronic pain management in sickle cell disease.
Overview
Overview
- Sickle cell disease is an inherited genetic defect that causes red blood cells to deform under certain conditions. Those deformed cells plug small blood vessels, causing pain and tissue damage.
- We will use the abbreviation SCD throughout this summary to refer to sickle cell disease and ASH for the American Society of Hematology.
- This patient summary focuses on the management of pain in patients with sickle cell disease.
Treatment
Treatment
Acute Pain
Acute Hospital Care
Recurrent Acute Pain
Chronic Pain
Chronic opioid therapy (COT)
- SCD patients with acute pain require evaluation and treatment within an hour of arrival and re-evaluation every 30–60 minutes.
- Treatment options include:
- Opioids
- Added nonsteroidal anti-inflammatory drugs (NSAIDs)
- Avoidance of corticosteroids (cortisone)
- Analgesic (pain relieving) ketamine infusion if the above is not sufficient
- Regional anesthesia if the above is not sufficient
- Massage, yoga, transcutaneous electrical nerve stimulation (TENS), virtual reality (VR), and guided audiovisual (AV) relaxation added to the above.
- Treatment options include:
Acute Hospital Care
- Patients with SCD are best managed in facilities that have expertise in treating SCD.
Recurrent Acute Pain
- The ASH suggests other measures be tried before chronic monthly transfusion therapy.
Chronic Pain
- For adults who have SCD-related chronic pain, the ASH suggests a comprehensive disease and pain management plan that considers these options:
- SNRIs (eg, duloxetine and milnacipran)
- Gabapentinoids (eg, pregabalin)
- Tricyclic antidepressants (TCAs) eg, amitriptyline.
- Cognitive and behavioral pain management strategies
- Other provider-delivered integrative approaches (eg, massage therapy and acupuncture) as available and as tolerated and conditional upon individual patient preference and response.
- For adults with SCD who have chronic bone pain from avascular necrosis (bone death due to loss of blood supply), the ASH suggests duloxetine (and other serotonin and norepinephrine reuptake inhibitor (SNRI) medications) as an option.
Chronic opioid therapy (COT)
- Due to the high risk that long-term opioids present, the ASH prefers multiple other treatments be tried before initiating or continuing COT.
Abbreviations
- ASH: American Society Of Hematology
- AV: Audiovisual
- COT: Chronic Opioid Therapy
- NSAIDs: Non-Steroidal Anti-Inflammatory Drugs
- SCD: Sickle Cell Disease
- SNRI: Serotonin-norepinephrine Reuptake Inhibitor
- TCAs: Tricyclic Antidepressants
- TENS: Transcutaneous Electrical Nerve Stimulation
- VR: Virtual Reality
Source Citation
Brandow AM, Carroll CP, Creary S, Edwards-Elliott R, Glassberg J, Hurley RW, Kutlar A, Seisa M, Stinson J, Strouse JJ, Yusuf F, Zempsky W, Lang E. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Adv. 2020 Jun 23;4(12):2656-2701. doi: 10.1182/bloodadvances.2020001851. PMID: 32559294; PMCID: PMC7322963.
Disclaimer
The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.