Exacerbation of Sickle Cell Disease by Obstructive Sleep Apnea

Abstract

\s=b\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. Postoperative 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.

Early recognition of pulmonary complications of sickle cell disease

“…Tonsillectomy and adenoidectomy were carried out, subsequently improving the vaso-occlusive crises markedly, which in turn resolved her pain in around 2 years and cured her OSA. 40 Sunil et al 41 carried out a cohort study that included 32 adult patients with SCD whose Epworth sleepiness scale score was ≥10 and who underwent a full sleep evaluation. Of the patients, 44% had sleep-disordered breathing )SDB( documented by polysomnography, and more than 50% had insomnia and delayed sleep phase syndrome.…”

“…Tonsillectomy and adenoidectomy were carried out, subsequently improving the vaso-occlusive crises markedly, which in turn resolved her pain in around 2 years and cured her OSA. 40 …”

COVID-19 and ENT Surgery

“…The indications to carry out T & A without delay in the current context of pandemic are enlarged tonsils and adenoids with severe obstructive sleep apnoea syndrome (OSAS). In children, an additional argument to maintain the scheduling of this operation is the existence of comorbidities such as sickle cell anaemia where there is a risk of vaso-occlusive crisis aggravated by OSAS [23]. In adults, tonsillectomy for severe OSAS can still go ahead, without prior sleep endoscopy, where there is a high probability of therapeutic success.…”

Periodic limb movement in sleep and sickle cell disease: a neglected association?

“…Since the first description of an association between exacerbation of clinical manifestation of sickle syndromes, also referred to as sickle cell disease (SCD), and obstructive sleep apnoea (OSA) in a child in 1988, there have been further studies demonstrating the importance of OSA in children with SCD (Sidman & Fry, 1988;Kirkham et al, 2001). These studies have led to the finding that OSA, and the consequent nocturnal hypoxemia, might be associated with severe comorbidities, including stroke (Kirkham et al, 2001).…”