Sitters

Breathing problems are less frequent in sitters, but it is still recommended that they have a physical examination at least once every six months. At this appointment, if they are able, they should have breathing tests (spirometry) to measure lung size and breathing muscle strength. Sleep studies to check on breathing during sleep should be completed for all who have any symptoms, or suspicion of ‘insufficient breathing’. Examples of symptoms are poor sleep quality, morning headaches, and daytime sleepiness.

If a sitter has a weak cough they should be given chest physiotherapy. Parents and carers should be shown techniques and a cough assist device (e.g. Cough Assist®, Vital Cough®) should be provided with a demonstration and clear instructions as to how and when to use it. Non-invasive Ventilation (NIV) should be used for everyone who shows symptoms that suggest they are not breathing well enough during sleep (poor sleep quality, headaches, and daytime sleepiness).

Sleep studies should be used to confirm if breathing issues are causing the problems, and to determine optimal settings for the NIV. NIV should be set to give a big enough breath so that oxygen is not needed. (Read more about the NIV and how it works in the non-sitters/management Section or in the glossary).

Other breathing supports such as ‘continuous positive airway pressure’ (CPAP) are not recommended because they do not facilitate carbon dioxide exchange for oxygen.

Colds can lead to chest infections which are common and are made worse when someone is unable to cough up mucus and other secretions well enough. Chest physiotherapy combined with a cough assist machine should be available to all sitters. Those with an ineffective cough and swallow should also be provided with a machine to suck out (suction) their oral secretions. Parents and carers should be given training and support so that they can follow through with advice and use machines effectively.

Medications used to open the airways (nebulised bronchodilators) should be available if there is high suspicion of asthma or if a clear improvement in breathing is seen after it has been given. Medications to reduce salivary secretions (such as glycopyrrolate) should be used carefully and, with medical guidance, the dose changed as needed. This is to avoid the possibility of the secretions drying out too much, which makes them harder to remove. Long term daily use of medications to break down secretions (Pulmozyme® or hyptertonic saline) is not recommended. Antibiotic use during illness should be discussed on an individual basis with the medical team.