American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain

Brandow, Amanda M.; Carroll, C. Patrick; Creary, Susan E; Edwards-Elliott, Ronisha; Glassberg, Jeffrey; Hurley, Robert W.; Kutlar, Abdullah; Seisa, Mohamed O.; Stinson, Jennifer; Strouse, John J.; Yusuf, Fouza; Zempsky, William T.; Lang, Eddy

doi:10.1182/bloodadvances.2020001851

Cited by 252 publications

(322 citation statements)

References 169 publications

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“…The American Society of Hematology recently published the 2020 guidelines for sickle cell disease management of acute and chronic pain. 38 They suggest that given the high prevalence of psychologic comorbidities that often coexist in the context of pain, routinely screening for depression and anxiety, and targeted screening for other psychologic comorbidities is considered good clinical practice.…”

Section: Psychiatric Disorders and Chronic Painmentioning

confidence: 99%

Optimizing the management of chronic pain in sickle cell disease

2020

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Chronic pain in sickle cell disease (SCD) refers to pain present on most days lasting over six months. It can start during childhood and the prevalence increases with age. By adulthood, over 55% of patients experience pain on over 50% of days; 29% reporting pain on 95% of days. The true prevalence of chronic pain in SCD is likely underappreciated as it is mostly managed at home. Patients with chronic pain and SCD frequently seek acute care for exacerbation of underlying chronic pain difficult to distinguish from their usual acute vaso-occlusive crises. When treating chronic pain in SCD, the challenge is distinguishing between non-SCD related etiologies versus chronic pain resulting from SCD pathophysiological processes. This distinction is important to delineate as it will drive appropriate management strategies. Chronic pain in SCD has profound consequences for the patient; is often associated with comorbid psychiatric illnesses (depression and anxiety), not dissimilar from other chronic pain syndromes. They may also experience challenges with sleep hygiene, various somatic symptoms, and chronic fatigue that impair quality of life. How best to treat chronic pain in SCD is not definitively established. Both acute and chronic pain in SCD is typically treated with opioids. Emerging data suggests that chronic opioid therapy (COT) is a suboptimal treatment strategy for chronic pain. This review will discuss the complexity of managing chronic pain in SCD; pain that may be dependent or independent of the underlying SCD diagnosis. We will also describe alternative treatment approaches to high-dose COT.

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Section: Psychiatric Disorders and Chronic Painmentioning

confidence: 99%

Optimizing the management of chronic pain in sickle cell disease

2020

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“…There is mounting evidence demonstrating that some SCD patients will develop chronic pain, which can be punctuated with acute pain crises. In addition to recurrent episodes of pain crises, complications of SCD, such as skin ulcers and avascular necrosis can be associated with acute and chronic pain 28–30. This heterogeneity of pain phenotypes among SCD patients can certainly lead to therapeutic challenges and add complexity to the interpretation of results of clinical trials examining analgesic therapies.…”

Section: Sickle Cell Disease a Painfully Complex Disordermentioning

confidence: 99%

“…The SCD experts in that working group proposed the existence of three common subtypes of chronic pain in SCD patients: chronic pain not resulting from SCD complications, chronic pain derived from a complication of SCD, and chronic pain with mixed presentation 34. In turn, recently, the American Society of Hematology issued a set of specific guidelines for the management of acute and chronic pain in SCD patients 28. Further, recognizing that SCD patients have such high pain burden, scientific societies as well as regulatory and funding agencies have dedicated great effort and resources to improve our understanding of the underlying mechanisms of SCD pain and to develop effective mechanism-based non-opioid pain therapies.…”

Section: Sickle Cell Disease a Painfully Complex Disordermentioning

confidence: 99%

Nitric oxide and sickle cell disease—Is there a painful connection?

Hallmark

Almeida

Kamimura

et al. 2020

Exp Biol Med (Maywood)

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Sickle cell disease is the most common hemoglobinopathy and affects millions worldwide. The disease is associated with severe organ dysfunction, acute and chronic pain, and significantly decreased life expectancy. The large body of work demonstrating that hemolysis results in rapid consumption of the endogenous vasodilator nitric oxide, decreased nitric oxide production, and promotion of vaso-occlusion provides the basis for the hypothesis that nitric oxide bioavailability is reduced in sickle cell disease and that this deficit plays a role in sickle cell disease pain. Despite initial promising results, large clinical trials using strategies to increase nitric oxide bioavailability in sickle cell disease patients yielded no significant change in duration or frequency of acute pain crises. Further, recent investigations showed that sickle cell disease patients and mouse models have elevated baseline levels of blood nitrite, a reservoir for nitric oxide formation and a product of nitric oxide metabolism, regardless of pain phenotype. These conflicting results challenge the hypotheses that nitric oxide bioavailability is decreased and that it plays a significant role in the pathogenesis in sickle cell disease acute pain crises. Conversely, a large body of work demonstrates that nitric oxide, as a neurotransmitter, has a complex role in pain neurobiology, contributes to the development of central sensitization, and can mediate hyperalgesia in inflammatory and neuropathic pain. These results support an alternative hypothesis: one proposing that altered nitric oxide signaling may contribute to the development of neuropathic and/or inflammatory pain in sickle cell disease through its role as a neurotransmitter.

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“…EBIs needed to manage SCD exist. The American Society of Hematology Sickle Cell Disease Guidelines provide SCD management recommendations including broad pain management approaches, pharmacological and nonpharmacological interventions and analgesic delivery [ 24 ] blood transfusion support [ 25 ], and prevention, diagnosis, and treatment of cerebrovascular disease in children and adults with SCD [ 26 ], which recognizes the challenges and opportunities that are unique to LMICs including cost-effective approaches. Further, dearth of SCD management resources, and limited number of physicians with expertise in hematology and implementation science research training are significant barriers to providing effective SCD management in many LMICs including Africa.…”

Section: Introductionmentioning

confidence: 99%

Implementation science research for the scale-up of evidence-based interventions for sickle cell disease in africa: a commentary

Gyamfi¹,

Ojo²,

Iwelunmor

et al. 2021

Global Health

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Background The burden of sickle cell disease (SCD) is greatest among African nations. Effective scalability of evidence-based interventions (e.g., newborn screening, health education, prophylaxis for infection, optimal nutrition and hydration, hydroxyurea therapy, blood transfusions, and transcranial Doppler (TCD) screening) is urgently needed particularly in these settings for disease management. However, Africa is constrained by limited resources and the lack of capacity to conduct implementation science research for proper understanding of context, and assessment of barriers and facilitators to the uptake and scalability of evidence-based interventions (EBI) for SCD management. Main Body We outline implementation science approaches to embed EBI for SCD within the African context and highlight key implementation research programs for SCD management. Building implementation research capacity will meet the major need of developing effective life-long and accessible locally-tailored interventions for patients with SCD in Africa. Conclusion This commentary communicates the importance of the application of implementation science methodology to scale-up evidence-based interventions for the management of SCD in order to reduce pain, prevent other morbidities and premature death experienced by people with SCD in Africa, and improve their overall quality of life.

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American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain

Cited by 252 publications

References 169 publications

Optimizing the management of chronic pain in sickle cell disease

Optimizing the management of chronic pain in sickle cell disease

Nitric oxide and sickle cell disease—Is there a painful connection?

Implementation science research for the scale-up of evidence-based interventions for sickle cell disease in africa: a commentary

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