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Pain in SCD-Many mechanisms and mysteries
Olufunke Y Martin1, Deepika S Darbari2
1Division of Pediatric Hematology-Oncology, Children's Medical Center, Center for Cancer and Blood Disorders, The University of Texas Southwestern Medical Center, Dallas, TX.
Seminars in Hematology
|July 2, 2026
Summary
Pain in sickle cell disease (SCD) encompasses acute vaso-occlusive crises and prevalent chronic pain. Understanding these pain types across the lifespan is crucial for effective, individualized, whole-person care approaches.
Area of Science:
- Hematology
- Pain Medicine
- Psychology
Background:
- Sickle cell disease (SCD) is characterized by significant pain complications.
- Acute vaso-occlusive crises and chronic pain are prevalent throughout the lifespan in SCD patients.
Purpose of the Study:
- To review the current understanding of pain in SCD across the lifespan.
- To integrate definitions, mechanistic categories, and management strategies within biologic, psychosocial, and environmental contexts.
Main Methods:
- Review of current understanding of pain mechanisms in SCD.
- Integration of biologic, psychosocial, and environmental factors.
- Evaluation of peripheral and central nervous system hyperexcitability using quantitative sensory testing and functional neuroimaging.
Main Results:
- Acute pain in SCD originates from vaso-occlusion, inflammation, and ischemia-reperfusion injury.
- Chronic pain in SCD involves nociceptive, neuropathic, and nociplastic mechanisms influenced by comorbidities and stressors.
- Evidence suggests peripheral and central nervous system hyperexcitability contributes to chronic pain development and maintenance.
Conclusions:
- Current SCD pain management guidelines have limited evidence; multimodal strategies are recommended.
- Multidisciplinary, patient-centered care models incorporating nonopioid and nonpharmacologic adjuncts are most effective.
- An individualized, whole-person care approach using patient-reported outcomes and biomarkers is key for advancing SCD pain interventions.
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