Sickle cell disease: painful episodes are most common in 25-29yr olds.

Clinical bottom line (level 4)

Patients with sickle cell disease may be more likely to have a painful episode if they are aged between 25 and 29 years.

Platt et al: New England Journal of Medicine 1991; 325 (1): 11-16

Expires June 2003

The study

Prospective cohort study with ?objective ?blinded outcomes, adjusted for confounding factors, not validated in an independent set of patients.

Setting: 23 clinical centres, USA

3578 patients (aged range newborn to 66 years, ?% male) sickle cell disease

Excluded if

>10 closely spaced events

acute care given at a non-participating hospital

no diagnosis of sickle cell type

Poisson regression was used to adjust for confounding factors.

74% followed for 3 years
Outcomes studied:

The evidence

The pain rate over three years (episodes per year) was highest in 25-29 year olds (1.21), and lowest in 0-4 year olds (0.40). The pain rate increased between 0-4 years and 25-29 years, and then decreased again up to 45-49 years.
The average pain rate was 0.8 episodes per patient-year in sickle cell anaemia; 1.0 episode per patient-year in sickle ß ° -thalassaemia; 0.4 episodes in haemoglobin sickle cell disease.
39% of patients with sickle cell disease had no painful episodes and 1% had more than six per year.

Comments

No odds ratios were calculated for age 25-29 years being a risk factor for pain, so it is unclear how useful this is.
A study calculating odds ratios for age risk factors is needed.
Follow-up was low (74%), which makes the information less reliable.

Citation

Platt OS, Thorington BD, Brambilla DJ, et al: Pain in sickle cell disease: rates and risk factors. New England Journal of Medicine 1991; 325 (1): 11-16

Contributor: Clare Wotton and Musab Hayatli, June 2000
Reviewer:

_{Clinical Question.}

_Patient	_{sickle cell disease}
_{Intervention or Exposure}	_prevalence
_Outcome	_{painful episodes}