Sickle Cell Disease and Its Respiratory Complications

Cureus. 2022 Aug 29;14(8):e28528. doi: 10.7759/cureus.28528. eCollection 2022 Aug.

Abstract

Sickle cell disease (SCD) is a hematological disorder that is inherited in an autosomal recessive (AR) fashion. It is caused by mutations in the genes encoding for the globin apoprotein of hemoglobin (Hb), leading to diminished oxygen-carrying ability. Its pathophysiologic mechanism affects multiple organ systems, making it crucial to understand the complications of SCD and find the best ways to prevent and treat them. Some important ways that SCD manifests in the respiratory system are acute chest syndrome (ACS), pulmonary hypertension (PH), asthma, and venous thromboembolism (VTE). This article summarizes their salient features, including pathogenesis related to the adverse outcomes, screening practices, and management guidelines, with the intent to provide greater insight into forming better practices that increase the quality of life in SCD patients.

Keywords: acute chest syndrome (acs); asthma; pulmonary; pulmonary hypertension; pulmonary screening; respiratory; sickle cell disease complications; sickle cell disease: scd; venous thromboembolism (vte).

Publication types

  • Review