American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease

Abstract

Background: Prevention and management of end-organ disease represent major challenges facing providers of children and adults with sickle cell disease (SCD). Uncertainty and variability in the screening, diagnosis, and management of cardiopulmonary and renal complications in SCD lead to varying outcomes for affected individuals. Objective: These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in their decisions about screening, diagnosis, and management of cardiopulmonary and renal complications of SCD. Methods: ASH formed a multidisciplinary guideline panel that included 2 patient representatives and was balanced to minimize potential bias from conflicts of interest. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including performing systematic evidence reviews up to September 2017. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations, which were subject to public comment. Results: The panel agreed on 10 recommendations for screening, diagnosis, and management of cardiopulmonary and renal complications of SCD. Recommendations related to anticoagulation duration for adults with SCD and venous thromboembolism were also developed. Conclusions: Most recommendations were conditional due to a paucity of direct, high-quality evidence for outcomes of interest. Future research was identified, including the need for prospective studies to better understand the natural history of cardiopulmonary and renal disease, their relationship to patient-important outcomes, and optimal management.

Emergency Department Utilization for Patients Living With Sickle Cell Disease: Psychosocial Predictors of Health Care Behaviors

“…Provider stigma and insensitivity toward the sickle cell population, including lack of belief about the patient's self-reported pain and inclinations to suspect drug abuse and addiction, are all factors that serve as barriers to tailored SCD care. Previous literature has also shown that providers with negative attitudes are significantly less likely to repeat doses of opioids for sickle cell pain, 24 leading to poor quality of care and poor treatment outcomes.…”

The molecular basis for the prothrombotic state in sickle cell disease

“…Nonetheless, the high VTE recurrence rate in SCD patients appears to justify indefinite anticoagulation as an efficacious secondary prevention strategy, especially in patients with unprovoked or recurrent provoked thrombosis. 121 Even SCD patients with less severe disease have high VTE recurrence rates (18% at 5 years) with no difference according to whether the incident event occurred within or >90 days after hospitalization, 6,8 suggesting that most patients are likely to benefit from secondary prevention. However, caution may be warranted, as clinical and plas-ma biomarkers that reliably predict VTE recurrence in SCD patients have not been identified to guide decision making in this subgroup, unlike in the general population with unprovoked VTE.…”

A user guide to the American Society of Hematology clinical practice guidelines

“…3 Example recommendation 6 For children and adults with sickle cell disease (SCD) and albuminuria, the ASH guideline panel suggests the use of angiotensin-converting enzyme inhibitors (ACEi) or angiotensin II receptor blockers (ARB) (conditional recommendation based on low certainty in the evidence about effects ÅÅ ◯◯ ). 12 Remarks: (1) The initiation of ACEi and ARB for patients with SCD requires adequate follow-up and monitoring of side effects (eg, hyperkalemia, cough, hypotension).…”

“…Since November 2018, Blood Advances has published American Society of Hematology (ASH) clinical practice guidelines on venous thromboembolism (VTE), [1][2][3][4][5][6][7][8] immune thrombocytopenia, 9 and sickle cell disease. [10][11][12] More ASH guidelines on these and other topics are forthcoming. In this article, we describe why ASH guidelines are trustworthy, and how patients, clinicians, policymakers, researchers, and others may use them.…”