The purpose of this article is to provide a comprehensive review of wheezing in sickle cell disease (SCD), including epidemiology, pathophysiology, associations between wheezing and SCD morbidity and finally the clinical approach to evaluation and management of individuals with SCD who wheeze.
Wheezing is common in SCD and in some individuals represents an intrinsic component of SCD-related lung disease rather than asthma. Emerging data suggest that, regardless of the cause, individuals with SCD and with recurrent wheezing are at increased risk for subsequent morbidity and premature mortality. We believe individuals who acutely wheeze and have respiratory symptoms should be managed with a beta agonist and short-term treatment of oral steroids, typically less than 3 days to attenuate rebound vaso-occlusive disease. For those who wheeze and have a history or examination associated with atopy, we consider asthma treatment and monitoring per National Heart, Lung and Blood Institute asthma guidelines.
Wheezing in SCD should be treated aggressively both in the acute setting and with controller medications. Prospective SCD-specific clinical trials will be necessary to address whether anti-inflammatory asthma therapies (leukotriene antagonists, inhaled corticosteroids) can safely mitigate the sequelae of wheezing in SCD.
aEmergency Medicine, Hematology and Medical Oncology, Mount Sinai School of Medicine, New York
bWashington University School of Medicine in St Louis, St Louis, Missouri
cVanderbilt-Meharry Center of Excellence in Sickle Cell Disease, Monroe Carell Jr Children's Hospital, Vanderbilt, Nashville, Tennessee, USA
Correspondence to Michael R. DeBaun, MD, MPH, Professor of Pediatrics and Medicine, JC Peterson Endowed Chair in Pediatrics, Vice Chair for Clinical Research, Pediatrics, Director, Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease, Monroe Carell Jr Children's Hospital at Vanderbilt, 2200 Children's Way, Room 11206 DOT, Nashville, USA. Tel: +1 615 875 3040; e-mail: firstname.lastname@example.org