According to the ASH guidelines, how should patients with sickle cell disease (SCD) be screened for sleep-disordered breathing?

Updated: May 12, 2021
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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For asymptomatic children and adults with SCD, the ASH guideline panel suggests against screening with formal polysomnography (sleep study) for sleep-disordered breathing. [109]

When appropriate, validated tools (eg, Epworth Sleepiness Scale or Pittsburgh Sleep Quality Index) should be used to further identify patients who should be considered for formal sleep testing. The following signs or symptoms may warrant a diagnostic sleep study for otherwise healthy patients to evaluate for sleep-disordered breathing:

  • Snoring
  • Witnessed apneas or respiratory pauses
  • Nonrestorative sleep and/or excessive daytime sleepiness
  • Obesity
  • Early morning headaches
  • Unexplained desaturation or hypoxemia during sleep, while awake, or with exertion
  • Carbon dioxide retention on arterial blood gas
  • History of poorly controlled hypertension or congestive heart failure
  • History of nocturnal enuresis in an older child (eg, ≥10 years old)
  • History of recurrent priapism or frequent daytime or nocturnal vaso-occlusive pain
  • History of PH confirmed by right-heart catheterization
  • History of ischemic stroke without evidence for vasculopathy
  • History of memory loss, difficulty with concentration, or unexplained episodes of mental confusion
  • Symptoms of attention deficit–hyperactivity disorder, poor academic achievement, and performance or behavior problems in children

For patients in whom a sleep study is warranted, the ASH panel notes that American Academy of Sleep Medicine guidelines currently recommend in-laboratory, “attended” sleep studies for children and for adults with chronic disease and known comorbidities, specifically cardiopulmonary. Additionally, it is important for formal sleep studies to be conducted in a certified sleep center that meets standards as required by accreditation groups.

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