Sickle cell disease: painful crises occurred in many sites.

Clinical bottom line (level 4)

  1. In patients with sickle cell disease, exposure to cold was associated with a third of painful crises.
  2. Most patients had two or three sites of pain- the commonest sites being the lumbar spine, abdomen and femur.
Serjeant et al: British Journal of Haematology 1994; 87: 586-591
Expires May 2004

The study

Case series with objective outcomes, not adjusted for confounding factors, not validated in an independent set of patients.

Setting: sickle cell clinic, university hospital, Jamaica

118 patients (aged range 3 to 58 years; median 22, 61% female) sickle cell disease presenting with painful crises (episodic pain without other pathology, requiring narcotic analgesia)

Excluded if
  • <3.5 years old





    • Outcomes studied:
  • one crisis
  • two crises
  • three crises
  • >4 crises
  • precipitating factor cold
  • precipitating factors emotional stress
  • physical exertion
  • alcohol
  • pregnancy
  • site of crisis lumbar spine
  • site of crisis abdomen
  • site of crisis femur
  • knees
  • sternum
  • ribs
  • shoulder
  • elbows
  • one site
  • two sites
  • three sites
  • four sites
  • more than five sites

    • There were 183 crises in 118 patients.

    The evidence

    outcome time to outcome number of patients/total number %
    (95% CI)
    one crisis ? 78/118 66%
    (58% to 75%)
    two crises ? 26/118 22%
    (15% to 30%)
    three crises ? 9/118 8.0%
    (2.8% to 12%)
    >4 crises ? 3/118 3.0%
    (0.0% to 5.4%)
    precipitating factor cold ? 63/183 34%
    (28% to 41%)
    precipitating factors emotional stress ? 18/183 9.8%
    (5.5% to 14%)
    physical exertion ? 13/183 7.1%
    (3.4% to 11%)
    alcohol ? 7/183 3.8%
    (1.0% to 6.6%)
    pregnancy ? 4/183 2.2%
    (0.067% to 4.3%)
    site of crisis lumbar spine ? 89/183 49%
    (41% to 56%)
    site of crisis abdomen ? 58/183 32%
    (25% to 38%)
    site of crisis femur ? 54/183 30%
    (23% to 36%)
    knees ? 38/183 21%
    (15% to 27%)
    sternum ? 33/183 18%
    (12% to 24%)
    ribs ? 32/183 17%
    (12% to 23%)
    shoulder ? 32/183 17%
    (12% to 23%)
    elbows ? 31/183 17%
    (12% to 22%)
    one site ? 37/183 20%
    (14% to 26%)
    two sites ? 62/183 34%
    (27% to 41%)
    three sites ? 49/183 27%
    (20% to 33%)
    four sites ? 14/183 8%
    (3.8% to 12%)
    more than five sites ? 17/183 9.3%
    (5.1% to 14%)

    • 42% of patients had a fever.
    • 90% were discharged home after six hours of treatment and observation.

    Comments

    1. The sickle cell centre sees about 90% of all sickle cell painful crises, so the authors believe that the study population is representative.

    Citation

    1. Serjeant GR, Ceulaer CDE, Lethbridge R, et al: The painful crisis of homozygous sickle cell disease: clinical features. British Journal of Haematology 1994; 87: 586-591
    Contributor: Chris Ball and Clare Wotton, May 2000
    Reviewer:

    Clinical Question.
    Patient sickle cell disease
    Intervention or Exposure prevalence
    Outcome precipitating factors