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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