Policy responses and statements

Name of organisation:

British Thoracic Society (BTS)

Name of policy document:

BTS Guideline for Respiratory Management of Children with Neuromuscular Weakness - Draft for public consultation

Deadline for response:

12 December 2011

Background: This document has been prepared by a sub-committee of the BTS Standards of Care Committee (SOCC) in line with the Society’s policy for Guideline production.

The draft Guideline is now available for public consultation, and the BTS formally invited comments from the Royal College of Physicians of Edinburgh.

Introduction

Clinical context and need for a guideline

Neuromuscular diseases as a group are relatively common with a prevalence of about 1 in 3000. The majority of these conditions are genetic and become clinically apparent during childhood. The likelihood of respiratory impairment varies greatly amongst the different conditions; with some notable exceptions, it is more likely in children with more severe global weakness. Acute respiratory failure associated with respiratory infection is the most frequent reason for unplanned hospital admission, and chronic respiratory failure is a frequent cause of death. With appropriate intervention, the incidence of unplanned hospital admission can be reduced and life-expectancy can be improved. These guidelines attempt to summarise the available evidence in this field and to provide recommendations that will aid the healthcare professional in delivering good quality patient care.

There are a number of excellent disease-specific guidelines and consensus statements on neuromuscular diseases, but none which focus specifically on the respiratory management. Many of the principles of respiratory management are not disease-specific and the objective of this guideline is to provide recommendations that can be applied to the all children with neuromuscular weakness. The evidence for much of current practice is weak and is based largely on observational studies. The guideline committee have attempted to identify and summarise the existing evidence, and where that is lacking, provide expert consensus opinion.

Target audience for the guideline

This guideline is aimed primarily at healthcare practitioners within the UK who care for children with neuromuscular weakness. This will include doctors, nurses, physiotherapists and other healthcare professionals.

Scope of the guideline

The guideline starts with a background overview of respiratory problems in children with neuromuscular weakness. A brief summary of the conditions covered by the guideline is provided in Appendix 2. The respiratory management of these children is then covered in the following sections:

• Identifying children at risk of respiratory complications
• Airway clearance and respiratory muscle training
• Assisted ventilation
• Planning for surgical procedures
• Scoliosis
• Other interventions that impact on respiratory health
• Transition to adult care
• Quality of life and palliative care

Details of individual studies that form part of evidence behind the recommendations are available in the evidence tables (which can be downloaded as a supplement from the BTS website).

Areas not covered by the guideline:

The following fall outside the scope of the guideline:

• Non-respiratory aspects of management of children with neuromuscular disease
• Detailed anaesthetic management of children with neuromuscular disease
• Adults with neuromuscular disease
• Children with cerebral palsy
• Children with myasthenia gravis

COMMENTS ON
British Thoracic Society (BTS)
BTS Guideline for Respiratory Management of Children with Neuromuscular Weakness - Draft for public consultation

The Royal College of Physicians of Edinburgh is pleased to respond to the British Thoracic Society on the Guideline for Respiratory Management of Children with Neuromuscular Weakness.  The College welcomes this guideline from recognised leaders in this field, thoroughly researched and thoughtfully constructed according to the best principles of guideline development.

The guideline should be invaluable both for clinicians contemplating provision of this specialised care and those involved in aspects of shared care, but it will undoubtedly also be a useful reference for well-established centres.  Furthermore, although a highly specialised field, advances in care means that many more healthcare professionals are likely to encounter such patients, particularly in unscheduled care situations.

The guide is well laid out and strikes a good balance between the necessary description of techniques, for example specialised physiotherapy, without making the document overly long for those seeking specific information.  The topics and layout allow for those seeking targeted information.  In an area of clinical activity where high quality evidence (according to conventional criteria) is inevitably lacking the authors are to be commended for providing a welcome number of good practice points and expert opinion.  The section with thumbnail sketches of the various relevant conditions is a valuable inclusion.

A few detailed and specific points follow:

1. Experience in adults would suggest that definite periods of REM hypoventilation can be seen in Duchenne patients without oxygen desaturation below Sp02 93%.  Therefore, there is minimal concern regarding total reassurance of a satisfactory oximetry alone.

2. The sections on transition, quality of life and palliative care are generally excellent.  Perhaps there might have been a little more specific focus on issues around continuing to use assisted ventilation, particularly NPPV, for symptom relief through the terminal phase.

3. p36.  The good practice point that overnight oximetry which shows that "saturation is maintained at 93% or above is sufficient to exclude clinically significant nocturnal hypoventilation" is based on expert opinion and perhaps something like is "usually sufficient" or is "considered sufficient” would be more apt.

4. p45. Good practice points. Humidification seems only to be mentioned here.  There does not seem to be discussion in the ventilation section of the need for humidification.  There is no guidance about the need for or benefits of using dry humidification with humidifying filters which can be used and are effective.

5. p54. Children with stable disease can be started on NIV without need for hospital admission - suggest adding: if adequate home support from specialist nurses is available.

6. p56. Tracheostomy.  There is no discussion about the use of cuffed versus non-cuffed tubes and the relative pros and cons.  It has been found that cuffed tubes can be very useful for managing some children with significant leaks causing ventilator alarms.

7. p60. It was felt that the good practice point about oxygen to correct hypoxaemia caused by hypoventilation needs strengthening.

In summary, the guideline is both very timely and likely to become a key document in the field of respiratory care of neuromuscular conditions – a great deal of the guideline’s recommendations/advice translates directly to clinical practice in the care of adults with neuromuscular conditions.

Copies of this response are available from:

Lesley Lockhart,
Royal College of Physicians of Edinburgh,
9 Queen Street,
Edinburgh,
EH2 1JQ.

Tel: 0131 225 7324 ext 608
Fax: 0131 220 3939

[13 December 2011]