What can trigger vaso-occlusive crisis in sickle cell disease (SCD)?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Approximately half the individuals with homozygous HbS disease experience vaso-occlusive crises. The frequency of crises is extremely variable. Some individuals have as many as 6 or more episodes annually, whereas others may have episodes only at great intervals or none at all. Each individual typically has a consistent pattern for crisis frequency. Triggers of vaso-occlusive crisis include the following:

  • Hypoxemia: May be due to acute chest syndrome or respiratory complications
  • Dehydration: Acidosis results in a shift of the oxygen dissociation curve
  • Changes in body temperature (eg, an increase due to fever or a decrease due to environmental temperature change)

Many individuals with HbSS experience chronic low-level pain, mainly in bones and joints. Intermittent vaso-occlusive crises may be superimposed, or chronic low-level pain may be the only expression of the disease.

See Presentation for more detail.


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