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Hematology Am Soc Hematol Educ Program. 2017 Dec 8;2017(1):525-533. doi: 10.1182/asheducation-2017.1.525.

Optimizing the care model for an uncomplicated acute pain episode in sickle cell disease.

Author information

Centre for Genomics and Child Health, Blizard Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, United Kingdom; and.
Departments of Haematology and Paediatric Haematology, Royal London Hospital, Bart's Health National Health Service Trust, London, United Kingdom.


The pathophysiology, clinical presentation, and natural history of acute pain in sickle cell disease are unique and require a disease-centered approach that also applies general principles of acute and chronic pain management. The majority of acute pain episodes are managed at home without the need to access health care. The long-term consequences of poorly treated acute pain include chronic pain, adverse effects of chronic opioid usage, psychological maladjustment, poor quality of life, and excessive health care utilization. There is no standard protocol for management of an acute pain crisis in either the hospital or the community. The assumptions that severe acute pain must be managed in the hospital with parenteral opioids and that strong opioids are needed for home management of pain need to be questioned. Pain management in the emergency department often does not meet acceptable standards, while chronic use of strong opioids is likely to result in opioid-induced hyperalgesia, exacerbation of chronic pain symptoms, and opioid dependency. We suggest that an integrated approach is needed to control the underlying condition, modify psychological responses, optimize social support, and ensure that health care services provide safe, effective, and prompt treatment of acute pain and appropriate management of chronic pain. This integrated approach should begin at an early age and continue through the adolescent, transition, and adult phases of the care model.

[Available on 2018-12-08]
[Indexed for MEDLINE]

Conflict of interest statement

Conflict-of-interest disclosure: P.T. is on the board of directors or an advisory committee for Pfizer; has received research funding from Napp and Kyowakirin; has consulted for Novartis, Global Blood Therapeutics, and Bluebird Bio; and has received honoraria from Novartis, Global Blood Therapeutics, Apopharma, and Bluebird Bio. B.K. is on the board of directors or an advisory committee for Astra Zeneca and has received honoraria from Novartis.

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