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Adherence and barriers to hyperinsufflation in children with congenital muscular dystrophy

John E Pascoe1,2, Hemant Sawnani1, Oscar H Mayer3

  • 1Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Pediatric Pulmonology
|November 23, 2016
PubMed

Insights

Adherence to hyperinsufflation in children with Congenital Muscular Dystrophy (CMD) was low, around 40-44%. Families struggled to identify barriers, highlighting a need for better communication and interventions to improve adherence to respiratory therapies.

Area of Science:

  • Pediatric Pulmonology
  • Neuromuscular Disorders
  • Respiratory Therapy Adherence

Background:

  • Congenital Muscular Dystrophy (CMD) is a rare inherited neuromuscular disease causing progressive muscle weakness and respiratory failure.
  • Adherence to essential respiratory therapies in children with neuromuscular disorders remains largely uncharacterized.
  • This study focused on assessing adherence and barriers to hyperinsufflation in pediatric CMD patients.

Purpose of the Study:

  • To evaluate adherence rates to a prescribed hyperinsufflation regimen in children with CMD.
  • To identify and analyze potential barriers affecting adherence to respiratory therapy.
  • To compare adherence data obtained through different assessment methods.

Main Methods:

  • Eighteen children with CMD participated in the study.
  • Adherence was measured using both a daily phone diary (DPD) and electronic data from the hyperinsufflation device over 48 hours.
  • Personalized hyperinsufflation settings were determined for each participant.

Main Results:

  • Adherence to hyperinsufflation was found to be 40% (DPD) and 44% (electronic data), showing strong agreement between methods.
  • A significant portion (53%) of participants reported no specific barriers, despite low adherence.
  • Social distractions and family obligations were identified as barriers, but no significant difference in adherence was observed between those who did and did not report barriers.

Conclusions:

  • Adherence to hyperinsufflation presents a significant challenge for children with CMD and their families.
  • Families often face difficulties in recognizing and articulating adherence barriers.
  • Future interventions should focus on facilitating open dialogue about barriers and implementing strategies to enhance adherence behaviors.
Abstract

Keywords:
CMDchildrencough assistfacilitatorshyperinsufflationpulmonary functionrestrictive lung disease

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