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Respiratory support for the severely handicapped child with neuromuscular disease: ethics and practicality

Anita K Simonds1

  • 1Academic Department of Sleep and Breathing, Royal Brompton Hospital, London, United Kingdom. A.Simonds@rbht.nhs.uk

Seminars in Respiratory and Critical Care Medicine
|June 15, 2007
PubMed

Insights

Advances in respiratory care, including noninvasive ventilation, are transforming outcomes for children with severe neuromuscular disease. These innovations offer improved symptom management and enhanced quality of life, though ethical considerations remain.

Area of Science:

  • Pediatric Pulmonology
  • Neuromuscular Diseases
  • Respiratory Care

Background:

  • Severe neuromuscular diseases significantly impact pediatric respiratory function and natural history.
  • Recent advancements in diagnosis and respiratory support have emerged.
  • Improved functional scoring systems aid in evaluating muscle function and treatment efficacy.

Purpose of the Study:

  • To review recent respiratory developments impacting the management of children with severe neuromuscular disease.
  • To discuss the implications of enhanced ventilatory support options on decision-making and ethical considerations.
  • To evaluate the role of noninvasive ventilation in improving outcomes and quality of life.

Main Methods:

  • Review of recent literature on respiratory developments in pediatric neuromuscular disease.
  • Analysis of the impact of improved diagnostic tools and ventilatory support techniques.
  • Evaluation of functional scoring systems for assessing muscle function and treatment effects.

Main Results:

  • Noninvasive ventilation, particularly when combined with cough assistance, is often preferred over invasive methods.
  • These techniques are associated with reduced respiratory morbidity.
  • Quality of life may be independent of functional ability or need for ventilatory support.

Conclusions:

  • Enhanced respiratory care, including noninvasive ventilation, offers significant benefits for children with severe neuromuscular disease.
  • Careful assessment of benefits and burdens is crucial for informed decision-making regarding ventilatory support.
  • Noninvasive ventilation can palliate symptoms and facilitate home care, even when survival extension is limited.

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