Current and emerging treatments for sickle cell disease

JAAPA. 2019 Sep;32(9):1-5. doi: 10.1097/01.JAA.0000578812.47138.99.

Abstract

Sickle cell disease (SCD) is a group of inherited blood disorders affecting the hemoglobin, shortening the lifespan of erythrocytes, and causing them to take on a distinctive sickled shape that can lead to vaso-occlusion. Current treatment aims to reduce morbidity and mortality through hydroxyurea, erythrocyte transfusion, and hematopoietic stem cell transplantation. This article reviews the disease process, typical presentations, complications, and acute and chronic treatment options.

Publication types

  • Review

MeSH terms

  • Acute Chest Syndrome / diagnosis
  • Acute Chest Syndrome / etiology
  • Acute Chest Syndrome / therapy
  • Acute Disease
  • Analgesics, Opioid / therapeutic use*
  • Anemia, Sickle Cell / complications
  • Anemia, Sickle Cell / physiopathology
  • Anemia, Sickle Cell / therapy*
  • Anti-Bacterial Agents / therapeutic use
  • Antisickling Agents / therapeutic use*
  • Chronic Disease
  • Disease Progression
  • Erythrocyte Transfusion*
  • Fluid Therapy
  • Genetic Therapy
  • Glutamine / therapeutic use
  • Hematopoietic Stem Cell Transplantation
  • Humans
  • Hydroxyurea / therapeutic use
  • Infant, Newborn
  • Infection Control
  • Infections
  • Mesenteric Ischemia / diagnosis
  • Mesenteric Ischemia / etiology
  • Mesenteric Ischemia / therapy
  • Neonatal Screening
  • Oxygen Inhalation Therapy
  • Pain Management
  • Patient Education as Topic
  • Pneumococcal Infections / prevention & control
  • Pneumococcal Vaccines / therapeutic use
  • Stroke / diagnosis
  • Stroke / etiology
  • Stroke / therapy

Substances

  • Analgesics, Opioid
  • Anti-Bacterial Agents
  • Antisickling Agents
  • Pneumococcal Vaccines
  • Glutamine
  • Hydroxyurea