UMEM Educational Pearls

Sickle Cell Disease (SCD) is a hemoglobinopathy that is considered a relatively rare disease in the United States, affecting about 90,000-100,000 individuals.

Globally, SCD affects millions, primarily in West and Central Africa.

 

Acute presentations of SCD include:

  • Acute Pain (Sickle Cell or Vaso-occlusive) Crisis
    • Most common presentation in emergency departments
  • Severe Anemia
    • Splenic sequestration crisis
    • Aplastic crisis
    • Hemolytic crisis
  • Infections
    • Particularly from encapsulated organisms because of a damaged spleen (functional asplenia)
  • Acute Chest Syndrome
    • From damaged lung tissues leading to hypoxia
    • A leading cause of death for patients SCD
  • Stroke
  • Priapism
  • Other organ dysfunction including kidney failure and eye problems (retinopathy)

The bottom line: 

  • Sickle Cell Disease is a serious, painful and potentially life threatening disease that can cause major damage to multiple organ systems.

References

 

http://www.cdc.gov/ncbddd/sicklecell/data.html

http://www.nhlbi.nih.gov/health/health-topics/topics/sca/signs

http://emedicine.medscape.com/article/205926-clinical