Excerpt

Motor neurone disease (MND) is a fatal neurodegenerative disease. It is characterised by the onset of symptoms and signs of degeneration of primarily the upper and lower motor neurones. This leads to progressive weakness of the bulbar, limb, thoracic and abdominal muscles. Respiratory muscle weakness resulting in respiratory impairment is a major feature of MND, and is a strong predictor of quality of life and survival. Non-invasive ventilation can improve symptoms and signs related to respiratory impairment and hence survival.

There is currently no evidence-based guideline for use in England, Wales and Northern Ireland that addresses the use of non-invasive ventilation in patients with MND. This guideline considers the signs and symptoms that can be used for predicting respiratory impairment in patients with MND, the diagnostic accuracy of investigations for detecting and monitoring respiratory impairment, the clinical and cost effectiveness of non-invasive ventilation for treating respiratory impairment and the information and support needs of patients and their families and carers relating to the use of non-invasive ventilation.

Contents

• Introduction
• Patient-centred care
• 1. Summary
  • 1.1. List of all recommendations
  • 1.2. Care pathways
  • 1.3. Overview
• 2. How this guideline was developed
  • 2.1. The identification and assessment of respiratory impairment in patients with motor neurone disease: clinical symptoms and signs
  • 2.2. The identification and assessment of respiratory impairment in patients with motor neurone disease: respiratory function tests
  • 2.3. Clinical and cost effectiveness of non-invasive ventilation for the treatment of respiratory impairment in patients with motor neurone disease
  • 2.4. Key elements in the management of the use of non-invasive ventilation for patients with motor neurone disease
  • 2.5. Information and support needs of patients with motor neurone disease and their families and carers
• 3. Research recommendations
  • 3.1. Cost effectiveness of non-invasive ventilation for patients with MND
  • 3.2. Withdrawing non-invasive ventilation at the end of life
  • 3.3. Communication with patients, families and carers
  • 3.4. Non-invasive ventilation for patients with MND with severe bulbar impairment
  • 3.5. Training and education needs
• 4. Other versions of this guideline
  • Quick reference guide
  • ‘Understanding NICE guidance’
• 5. Related NICE guidance
  • Published
• 6. Updating the guideline
• 7. References, glossary and abbreviations
  • 7.1 References
  • 7.2 Glossary
  • 7.3 Abbreviations
• 8. Contributors
  • 8.1. The Guideline Development Group
  • 8.2. The short clinical guidelines technical team
  • 8.3. The short clinical guidelines team
  • 8.4. Centre for Clinical Practice
  • 8.5. The Guideline Review Panel
  • 8.6. Declarations of interest
  • 8.7. Authorship and citation
• 9 Appendices
  • Appendix 9.1. Scope
  • Appendix 9.2. Key clinical issues, review questions and review protocol
  • Appendix 9.3. Results of GDG short questionnaires
  • Appendix 9.4. Lists of excluded studies
  • Appendix 9.5. Literature search and search strategies
  • Appendix 9.6. Evidence tables
  • Appendix 9.7. Health economic evaluation

NICE clinical guidelines are recommendations about the treatment and care of people with specific diseases and conditions in the NHS in England and Wales.

This guidance represents the view of NICE, which was arrived at after careful consideration of the evidence available. Healthcare professionals are expected to take it fully into account when exercising their clinical judgement. However, the guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, and informed by the summary of product characteristics of any drugs they are considering.

Implementation of this guidance is the responsibility of local commissioners and/or providers. Commissioners and providers are reminded that it is their responsibility to implement the guidance, in their local context, in light of their duties to avoid unlawful discrimination and to have regard to promoting equality of opportunity. Nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties.