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

Authors

• Robert I Liem
• Sophie Lanzkron
• Thomas D Coates
• Laura DeCastro
• Ankit A Desai
• Kenneth I Ataga
• Robyn T Cohen
• Johnson Haynes
• Ifeyinwa Osunkwo
• Jeffrey D Lebensburger
• James P Lash
• Theodore Wun
• Madeleine Verhovsek
• Elodie Ontala
• Rae Blaylark
• Fares Alahdab
• Abdulrahman Katabi
• Reem A Mustafa

Publication/Presentation Date

12-10-2019

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.

Volume

3

Issue

23

First Page

3867

Last Page

3897

ISSN

2473-9537

Published In/Presented At

Liem, R. I., Lanzkron, S., D Coates, T., DeCastro, L., Desai, A. A., Ataga, K. I., Cohen, R. T., Haynes, J., Osunkwo, I., Lebensburger, J. D., Lash, J. P., Wun, T., Verhovsek, M., Ontala, E., Blaylark, R., Alahdab, F., Katabi, A., & Mustafa, R. A. (2019). American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease. Blood advances, 3(23), 3867–3897. https://doi.org/10.1182/bloodadvances.2019000916

Disciplines

Business Administration, Management, and Operations | Health and Medical Administration | Management Sciences and Quantitative Methods

PubMedID

31794601

Department(s)

Administration and Leadership

Document Type

Article