Sickle cell disease: a malady beyond a hemoglobin defect in cerebrovascular disease.

ABSTRACT:

Introduction

Sickle cell disease (SCD) is a devastating monogenic disorder that presents as a multisystem illness and affects approximately 100,000 individuals in the United States alone. SCD management largely focuses on primary prevention, symptomatic treatment and targeting of hemoglobin polymerization and red blood cell sickling. Areas covered: This review will discuss the progress of SCD over the last few decades, highlighting some of the clinical (mainly cerebrovascular) and psychosocial challenges of SCD in the United States. In addition, focus will also be made on the evolving science and management of this inherited disease. Expert commentary: Until recently hydroxyurea (HU) has been the only FDA approved therapy for SCD. However, advancing understanding of SCD pathophysio

SUBMITTER: Ansari J 

PROVIDER: S-EPMC6117162 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature