Identifying Clinical and Research Priorities in Sickle Cell Lung Disease An Official American Thoracic Society Workshop Report

• Type of Material: Article
• Authors: A. Parker Ruhl, National Institute of Allergy and Infectious Diseases; S. Christy Sadreameli, Johns Hopkins School of Medicine; Julian L. Allen, Childrens Hospital of Philadelphia; Debra P. Bennett, Independent Patient Advocate; Andrew D. Campbell, Childrens National Medical Center; Thomas D. Coates, Childrens Hospital Los Angeles; Dapa A. Diallo, University of Sciences, Techniques and Technologies of Bamako (USTTB); Joshua J. Field, Medical College of Wisconsin; Elizabeth K. Fiorino, Cohen Children's Medical Center; Claudia Morris, Emory University
• Language: English
• Date: 2019-09-01
• Publisher: American Thoracic Society
• Publication Version: Final Published Version
• Copyright Statement: © 2019 by the American Thoracic Society
• Final Published Version (URL): http://dx.doi.org/10.1513/AnnalsATS.201906-433ST
• Title of Journal or Parent Work: Annals of the American Thoracic Society
• ISSN: 2329-6933
• Volume: 16
• Issue: 9
• Start Page: E17
• End Page: E32
• Keywords: ACUTE CHEST SYNDROME; Science & Technology; Respiratory System; Life Sciences & Biomedicine; pulmonary hypertension; ENVIRONMENTAL TOBACCO-SMOKE; AIRWAY HYPERRESPONSIVENESS; acute chest syndrome; OBSTRUCTIVE SLEEP-APNEA; sickle cell disease; TRICUSPID REGURGITATION VELOCITY; ACUTE PULMONARY COMPLICATIONS; PLACENTA GROWTH-FACTOR; FORCED EXPIRATORY VOLUME; CAPILLARY BLOOD-VOLUME; RISK-FACTORS; asthma; sleep disorders
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

Background: Pulmonary complications of sickle cell disease (SCD) are diverse and encompass acute and chronic disease. The understanding of the natural history of pulmonary complications of SCD is limited, no specific therapies exist, and these complications are a primary cause of morbidity and mortality. Methods: We gathered a multidisciplinary group of pediatric and adult hematologists, pulmonologists, and emergency medicine physicians with expertise in SCD-related lung disease along with an SCD patient advocate for an American Thoracic Society–sponsored workshop to review the literature and identify key unanswered clinical and research questions. Participants were divided into four subcommittees on the basis of expertise: 1) acute chest syndrome, 2) lower airways disease and pulmonary function, 3) sleep-disordered breathing and hypoxia, and 4) pulmonary vascular complications of SCD. Before the workshop, a comprehensive literature review of each subtopic was conducted. Clinically important questions were developed after literature review and were finalized by group discussion and consensus. Results: Current knowledge is based on small, predominantly observational studies, few multicenter longitudinal studies, and even fewer high-quality interventional trials specifically targeting the pulmonary complications of SCD. Each subcommittee identified the three or four most important unanswered questions in their topic area for researchers to direct the next steps of clinical investigation. Conclusions: Important and clinically relevant questions regarding sickle cell lung disease remain unanswered. High-quality, multicenter, longitudinal studies and randomized clinical trials designed and implemented by teams of multidisciplinary clinician-investigators are needed to improve the care of individuals with SCD.