Evolution of sickle cell disease from a life-threatening disease of children to a chronic disease of adults: The last 40 years

Am J Hematol. 2016 Jan;91(1):5-14. doi: 10.1002/ajh.24235.

Abstract

Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimistic that the next 40 years will be focused on meeting current challenges in SCD by addressing chronic complications of SCD to reduce mortality and improve quality of life in a growing population of adults with SCD in high-income countries, while simultaneously decreasing the disparity of medical care between high and low-income countries.

Publication types

  • Review

MeSH terms

  • Adult
  • Anemia, Sickle Cell / complications
  • Anemia, Sickle Cell / mortality
  • Anemia, Sickle Cell / prevention & control
  • Anemia, Sickle Cell / therapy*
  • Anti-Bacterial Agents / administration & dosage
  • Anti-Bacterial Agents / therapeutic use*
  • Antibiotic Prophylaxis
  • Antisickling Agents / administration & dosage
  • Antisickling Agents / therapeutic use*
  • Blood Transfusion*
  • Child
  • Child, Preschool
  • Chronic Disease
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Hydroxyurea / therapeutic use
  • Infant
  • Infant, Newborn
  • Mortality / trends
  • Neonatal Screening
  • Penicillins / therapeutic use
  • Stroke / etiology
  • Stroke / prevention & control

Substances

  • Anti-Bacterial Agents
  • Antisickling Agents
  • Penicillins
  • Hydroxyurea