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Primary snoring and growth failure in a patient with cystic fibrosis

Kelvin D Macdonald1, Brian M McGinley, David J Brown

  • 1Eudowood Division of Pediatric Respiratory Sciences, 200 N Wolfe Street, Baltimore, MD 21287, USA. kmacdon3@jhmi.edu

Respiratory Care
|December 8, 2009
PubMed

Insights

Cystic fibrosis (CF) patients with enlarged tonsils and adenoids may experience growth problems due to snoring and sleep issues. Adenotonsillectomy can improve sleep, weight gain, and growth in these children.

Area of Science:

  • Pediatric Pulmonology
  • Sleep Medicine
  • Genetics

Background:

  • Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, including the lungs and pancreas.
  • Pancreatic insufficiency in CF often leads to malabsorption and growth issues.
  • Obstructive sleep apnea (OSA) symptoms like snoring can occur in children with CF.

Observation:

  • An 8-year-old female with CF and pancreatic insufficiency presented with recurrent pharyngitis, reduced BMI, and height velocity.
  • She exhibited symptoms suggestive of obstructive sleep apnea, including snoring and restless sleep, with tonsillar hypertrophy noted on examination.
  • Nocturnal polysomnography showed a normal respiratory disturbance index but revealed prolonged snoring associated with hypercapnia.

Findings:

  • Adenotonsillectomy was performed, leading to decreased snoring and improved sleep quality.
  • Over an 18-month follow-up, the patient demonstrated significant weight gain and improved growth velocity.
  • The findings suggest a link between adenotonsillar hypertrophy, snoring, and impaired growth in this CF patient.

Implications:

  • Adenotonsillar hypertrophy and associated sleep disturbances may be an underrecognized factor hindering growth in children with cystic fibrosis.
  • Early identification and management of upper airway obstruction, such as through adenotonsillectomy, could be crucial for optimizing growth outcomes in CF patients.
  • This case highlights the importance of a multidisciplinary approach to managing CF, considering respiratory and sleep-related factors alongside gastrointestinal issues.

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