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Peak Cough Flow in Children with Neuromuscular Disorders

Nidhi Kotwal1,2, Prateek J Shukla3, Geovanny F Perez4,5

  • 1Division of Pulmonology and Allergy, Department of Pediatrics, University of Maryland, Maryland, 737 W Lombard St, Ste 314, Baltimore, MD, 21201, USA. nkotwal@som.umaryland.edu.

Lung
|February 26, 2020
PubMed

Insights

Pediatric patients with neuromuscular disease (NMD) show lower peak cough flow (PCF) than adults, especially younger children. These findings highlight the need for pediatric-specific PCF values to guide assisted cough therapies in NMD.

Area of Science:

  • Pediatric Pulmonology
  • Neuromuscular Diseases
  • Respiratory Physiology

Background:

  • Neuromuscular disease (NMD) causes progressive respiratory muscle weakness, impairing cough effectiveness.
  • Peak cough flow (PCF) is a key metric for assessing cough function and guiding interventions in adults.
  • Current PCF guidelines are derived from adult populations and may not be suitable for pediatric NMD patients.

Purpose of the Study:

  • To characterize peak cough flow (PCF) values in a pediatric population diagnosed with neuromuscular disease (NMD).
  • To establish baseline PCF measurements for children with various types of NMD.
  • To inform the development of age-appropriate assisted cough therapy recommendations.

Main Methods:

  • Retrospective chart review of pediatric patients with NMD treated between 2010 and 2016.
  • Inclusion of demographic data (age, gender) and clinical variables (ambulation status, specific NMD diagnosis).
  • Analysis of peak cough flow (PCF) measurements across different NMD subtypes and age groups.

Main Results:

  • 366 pediatric NMD patients were analyzed, with Duchenne muscular dystrophy (DMD) being the most common diagnosis.
  • Mean PCF values were significantly lower in DMD and congenital muscular dystrophy (CMD) compared to Charcot Marie Tooth disease (CMT).
  • Children under 10 years old exhibited significantly lower PCF values than older children, irrespective of ambulation status.

Conclusions:

  • Baseline PCF values in young pediatric NMD patients are below established adult thresholds for initiating assisted cough techniques.
  • Existing adult-derived PCF values are insufficient for guiding cough assistance in pediatric NMD.
  • Longitudinal studies are essential to establish reliable, pediatric-specific reference values for PCF in NMD.
Abstract

Keywords:
Becker muscular dystrophyCharcot Marie Tooth diseaseCongenital muscular dystrophyDuchenne muscular dystrophyNeuromuscular diseasePeak cough flow

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