Challenges of Management of Sickle Cell Disease with Pulmonary Hypertension in Resource Scarce Settings

Abstract

Sickle cell disease (SCD) is a genetic hemoglobinopathy endemic to sub-Saharan Africa and parts of the Middle East and other resource-scarce areas throughout the world. Pulmonary hypertension (PH) is now one of the most severe complications of SCD, affecting 6-30% of adult patients. SCD-PH carries high morbidity and mortality. The past decade has been characterized by great progress in understanding the epidemiology, pathophysiology, diagnosis, and management of SCD-PH. However, much work remains to be done to improve outcomes for patients with SCD-PH who reside in resource-limited areas. This narrative review will cover the epidemiology, pathophysiology, diagnosis, and treatment of SCD-PH while focusing on the unique challenges that exist within these settings. Diagnosis and screening remain poor in these areas, and confirmatory testing with right heart catheterization is often limited. Access to disease-modifying therapies such as hydroxyurea is poor, and PH-specific therapies are not available. These gaps in care present several opportunities for task-sharing, decentralization of care, adaptation of novel screening algorithms, and policy change.