Related Experiment Videos
The painful crisis of homozygous sickle cell disease: clinical features
G R Serjeant1, C D Ceulaer, R Lethbridge
1MRC Laboratories University of the West Indies, Kingston, Jamaica.
Insights
Sickle cell painful crises often occur between 3 PM and midnight, triggered by factors like cold and stress. Fever is common, but not always a sign of infection in these crises.
Area of Science:
- Hematology
- Clinical Medicine
- Public Health
Background:
- Sickle cell disease (SCD) is a genetic blood disorder causing painful crises.
- Understanding triggers and symptoms of SCD crises is crucial for patient management.
Purpose of the Study:
- To prospectively record details of painful crises in homozygous sickle cell (SS) disease.
- To identify onset patterns, precipitating factors, symptoms, and pain distribution.
Main Methods:
- Prospective recording of 183 painful crises in 118 patients at a day-care center in Kingston, Jamaica.
- Data collection included crisis onset, perceived triggers, associated symptoms, and pain location.
Main Results:
- Crises peaked between 3 PM and midnight, affecting 15-29 year olds equally between sexes.
- Precipitating factors included skin cooling (34%), emotional stress (10%), physical exertion (7%), and pregnancy (5%).
- Common pain sites were lumbar spine (49%), abdomen (32%), femoral shaft (30%), and knees (21%); fever was frequent, irrespective of infection.
Conclusions:
- Painful crises in sickle cell disease have distinct temporal patterns and triggers.
- Fever is a characteristic symptom of sickle cell crises, not necessarily indicating infection.
- Day-care management facilitated over 90% of patients returning home post-treatment.
Abstract:
The details of onset, perceived precipitating factors, associated symptoms, and pain distribution in the painful crisis of homozygous sickle cell (SS) disease have been prospectively recorded in 183 painful crises in 118 patients admitted to a day-care centre in Kingston, Jamaica. Painful crises developed most frequently between 3 p.m. and midnight, most commonly affected patients aged 15-29 years, affected the sexes equally, and were not obviously influenced by menstrual cycle. Of the perceived precipitating factors, skin cooling occurred in 34%, emotional stress in 10%, physical exertion in 7%, and pregnancy in 5% of women of child-bearing age. Cold as a precipitant was not less common in patients with more subcutaneous fat. Pain affected the lumbar spine in 49%, abdomen in 32%, femoral shaft in 30%, and knees in 21%. There was a highly significant excess of bilateral involvement in limb and rib pain. Recurrent painful crises occurred in 40 patients but showed no evidence of involving similar sites on successive occasions. Abdominal painful crises were associated with abdominal distention in 18 (31%) and with referred rib pain in a further 15 (26%) of crises. Fever was common even in apparently uncomplicated painful crises, suggesting that fever is characteristic of the painful crisis itself and not necessarily indicative of infection. Following investigation and treatment in a day-care centre, over 90% of patients returned home.