Respiratory Management

Respiratory Management


Children and young people with myotubular and centronuclear myopathies more usually experience some breathing problems, with many requiring a tracheostomy (small incision by surgical procedure into the windpipe) to aid their breathing, and others sometimes require non-invasive ventilation (via a nose or face mask) for several hours of the day and/or night.

Getting on top of breathing problems can really help improve quality of life and it is important that children and young people have a good multidisciplinary team involved in their breathing care and access to modern equipment.

We hope that the information and personal perspectives provided here will help guide you on respiratory management. We have tried to give as wide a perspective as possible.

BTS Guidelines for Respiratory Management of Children with Neuromuscular Weakness

British Thoracic Society has now published "Guidelines for Respiratory Management of Children with Neuromuscular Weakness". The full document can be downloaded here

A lay, patient friendly version of these guidelines, which focusses on Non-Invasive Ventilation for the over 2's was recently published by South West Neuromuscular Network (NHS). You can download and read the leaflet here.


Webinar on Management of Breathing and Respiratory Issues, April 2012

Part 1: Pediatric Pulmonologist Hemant Sawnani, M.D., Cincinnati Children’s Hospital Medical Center: How we breathe and what goes wrong in congenital muscle disease?

Part 2:
Professor Anita Simonds, Royal Brompton Hospital, London: Respiratory issues in congenital muscle disease and what we can do about them

Part 3: Dr Michelle Chatwin, Royal Brompton Hospital, London: Secretion Management Techniques


Getting Your Baby Home From Hospital

Some families with newborns who need invasive ventilation (tracheostomy) or non-invasive ventilation (a ventilator and mask) can experience long delays in getting their baby home from hospital for the first time. In this short film made by the Royal Brompton Long Term Ventilation Team, three families, including the parents of Baby Elijah who has myotubular myopathy, talk about how a Long Term Ventilation team helped them to be able to get their baby home from hospital safely, smoothly and within a matter of months.

If you are keen to seek more information about long term ventilation, either as a patient, carer or professional please take a look at the Royal Brompton Hospital website

Personal Perspectives


Other Resources

Breathe On (UK)

Breathe On UK is a national charity dedicated to supporting the families and carers of young people who are life-dependent on mechanical breathing, or long term ventilation (LTV).