Abstract

Vaso-occlusive sickle cell crises have been described with onset during sleep. Nineteen sleep studies were performed on 14 children with sickle cell disease to evaluate oxygen saturation during sleep. The patients, age 4 to 17 years, were selected based upon the number and frequency of crises, and history of nocturnal crises. Total sleep time (TST) and percent TST as rapid eye movement (REM) were comparable to normals for age. Changes in respiratory dynamics (apnea and hypopnea) and oxygen saturation were monitored during sleep. Four individuals experienced 1 to 2 episodes of apnea per night. Six children in 8 studies demonstrated 1 to 48 hypopneic events. Oxygen desaturation was defined as a ≥ 4% drop from baseline or ≤ 90% actual saturation. The duration of desaturation was quantitated in 14 of 19 studies. Desaturation associated with apnea or hypopnea was appreciated in only four studies. The total accumulative duration of desaturation was 1 to 9 minutes per study. Oxygen desaturation not associated with changes in respiratory dynamics occurred in all but one patient. The average duration of desaturation was 20 minutes (range 3 to 100 min.) These data reveal marked oxygen desaturation during sleep in a subset of patients with sickle cell disease, and suggest a relationship between desaturation and increased frequency of vaso-occlusive crises.

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