Sleep in children and young adults with cystic fibrosis

Brigitte Fauroux1, Karen Waters2, Joanna E MacLean3

  • 1Pediatric Noninvasive Ventilation and Sleep Unit, Necker University Hospital, AP-HP, Paris, France; Université de Paris, EA 7330 VIFASOM, F-75004 Paris, France.

Insights

Sleep disturbances are common in cystic fibrosis (CF), impacting quality of life and potentially worsening CF complications. Routine sleep evaluation and interventions are needed to improve sleep duration and quality in CF patients.

Area of Science:

  • Pulmonary Medicine
  • Sleep Medicine
  • Cystic Fibrosis Research

Background:

  • Cystic fibrosis (CF) management has improved, shifting focus from severe respiratory issues to broader health aspects.
  • Early CF sleep studies concentrated on nocturnal respiratory problems like hypoxia and hypoventilation.
  • Recent research includes patients with better lung function, allowing for more detailed examination of sleep patterns and quality.

Purpose of the Study:

  • To review current understanding of sleep disturbances in cystic fibrosis.
  • To identify factors contributing to poor sleep in CF patients.
  • To highlight the potential consequences of sleep problems in CF and the need for further research and intervention.

Main Methods:

  • Literature review of studies on sleep in cystic fibrosis patients.
  • Analysis of factors affecting sleep quality and duration.
  • Examination of the relationship between sleep disturbances and CF symptoms/comorbidities.

Main Results:

  • Reduced sleep duration and poor sleep quality are prevalent in CF.
  • Contributing factors include chronic pain, cough, gastrointestinal issues, nasal obstruction, and medications.
  • Sleep problems are associated with depression, reduced academic performance, and lower quality of life, particularly in adolescents.
  • Restless legs syndrome is also noted in adult CF patients.
  • Sleep issues occur across a spectrum of lung function, though potentially more pronounced in those with lower function.

Conclusions:

  • Poor sleep is a significant issue in cystic fibrosis, affecting physical and mental well-being.
  • The multi-organ impact of CF may be exacerbated by sleep disturbances.
  • Routine sleep assessment in CF patients is recommended.
  • Prospective studies are urgently needed to evaluate interventions aimed at improving sleep in CF.

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