Exacerbation of sickle cell disease by obstructive sleep apnea

It is difficult to recognize and treat the factors that exacerbate the vascular crises of sickle cell disease. We describe a 12-year-old black girl with sickle cell anemia who, since the age of 9 years, has been repeatedly hospitalized for pain associated with vaso-occlusive disease. A diagnosis of obstructive sleep apnea was suggested by the history and physical examination and was confirmed by polysomnography. Following preoperative transfusion, the patient underwent elective tonsillectomy and adenoidectomy. She has been free of vaso-occlusive pain and crises for over two years and has not been hospitalized since her surgery. Post-operative polysomnography has shown no evidence of obstructive sleep apnea. It is our hypothesis that repeated oxygen desaturation during periods of obstructive sleep apnea was the cause of this patient's frequent vaso-occlusive crises. She not only obtained complete relief of her symptoms, but objective sleep study measurements were normal following surgery. Obstructive sleep apnea is a recent diagnosis, and its pathologic effects are only beginning to be known. Physicians taking care of patients with hemoglobinopathies need to be aware of possible contributing factors to their patients' disease, and an aggressive approach to their diagnosis and possible relief should be sought.