Nocturnal Pain Crises in an Adult with Sickle Cell Disease

TL;DR

A 62-year-old sickle cell disease patient experienced worsening nighttime pain due to undiagnosed sleep apnea, which improved with treatment.

Contribution

Highlights the under-recognized link between sleep-disordered breathing and nocturnal pain in adult sickle cell disease patients.

Findings

Obstructive sleep apnea was identified as a cause of worsening nocturnal pain in a sickle cell disease patient.

Treatment with continuous positive airway pressure resolved the patient's symptoms.

Sleep-disordered breathing may contribute to hypoxia and sickle cell complications in adults.

Abstract

Sickle cell disease is the most common genetic hemoglobinopathy worldwide, characterized by a single-nucleotide mutation that predisposes to hemoglobin polymerization and erythrocyte sickling in hypoxic states. This report describes a 62-year-old male obese patient with a history of sickle cell disease, who presented with worsening nocturnal pain crises without any apparent triggering factor. A thorough evaluation at the outpatient department revealed obstructive sleep apnea. Airway obstruction or decreased respiratory effort during sleep may induce hypoventilation and hypoxia in the context of sleep-disordered breathing, with severe cardiopulmonary complications. Sleep-disordered breathing is considered common in children with sickle cell disease, but the prevalence in adults has not been sufficiently documented. Our patient responded favorably to treatment with continuous positive airway pressure during sleep, showing complete resolution of his symptoms. Timely diagnosis and management are fundamental to improve outcomes and prevent severe complications.

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