Assessment of painful episode frequency in sickle-cell disease

Frequency of painful episodes in sickle-cell disease is considered to be related to clinical severity and possibly to other aspects of the disease. Measurements of frequency often include only hospital-related or more severe, longer-lasting episodes. Since painful episodes, however, may regularly occur in nonhospital settings or be shorter-lasting with possible different pathologic effects, we measured all painful episodes in 10 adults with sickle-cell disease for 1.0-3.8 years, using a daily questionnaire. The results were related to other indices of disease severity and to possible precipitating factors, such as cold weather and menses. Sixty-one percent (on average) of the total number of episodes (243) were nonhospital-related, and 33% (on average) were shorter-lasting. Episode frequencies, whether determined as total, hospital-related, nonhospital-related, or shorter-lasting, were not related to each other or to other indicators of disease severity. The highest incidence of episode frequency occurred in the winter. The association of episodes with menses was moderately close in individual patients. The findings suggest that nonhospital-related painful episodes and shorter-lasting episodes may contribute significantly to episode frequency. Measurement of frequency of all painful episodes would require consideration when evaluating episode frequency and its relationship to disease severity, to possible precipitating factors of episodes, and to treatment of the disease, and for study of the natural course of the disease.