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Recent Advances in Chest Medicine |

Chronic Pulmonary Complications of Sickle Cell Disease

Alem Mehari, MD; Elizabeth S. Klings, MD
Author and Funding Information

FUNDING/SUPPORT: Dr Mehari is supported by National Heart, Lung, and Blood Institute of the National Institutes of Health under Award Number P50HL118006 and Department of Medicine Academic Enrichment Fund.

CORRESPONDENCE TO: Elizabeth S. Klings, MD, Boston University School of Medicine, Pulmonary Center, R-304, 72 E Concord St, Boston, MA 02118


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;149(5):1313-1324. doi:10.1016/j.chest.2015.11.016
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Sickle cell disease (SCD), the most common genetic hemolytic anemia worldwide, affects 250,000 births annually. In the United States, SCD affects approximately 100,000 individuals, most of African descent. Hemoglobin S (HbS) results from a glutamate-to-valine mutation of the sixth codon of the β-hemoglobin allele; the homozygous genotype (HbSS) is associated with the most prevalent and severe form of the disease. Other SCD genotypes include HbSC, composed of one HbS allele and one HbC (glutamate-to-lysine mutation) allele; and HbS-β-thalassemia0 or HbS-β-thalassemia+, composed of one HbS allele and one β-thalassemia allele with absent or reduced β-chain production, respectively. Despite advances in care, median survival remains in the fifth decade, due in large part to chronic complications of the disease. Chronic pulmonary complications in SCD are major contributors to this early mortality. Although our understanding of these conditions has improved much over the past 10 to 15 years, there remains no specific treatment for pulmonary complications of SCD. It is unclear whether conventional treatment regimens directed at non-SCD populations have equivalent efficacy in patients with SCD. This represents a critical research need. In this review, the authors review the state-of-the-art understanding of the following pulmonary complications of SCD: (1) pulmonary hypertension; (2) venous thromboembolic disease; (3) sleep-disordered breathing; (4) asthma and recurrent wheezing; and (5) pulmonary function abnormalities. This review highlights the advances as well as the knowledge gaps in this field to update clinicians and other health care providers and to garner research interest from the medical community.

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