Key points about sleep and breathing problems in neuromuscular conditions
- if your child has a neuromuscular condition, talk with your health professional about symptoms to watch for
- your health professional will talk you through what tests your child may need
- keep your child away from people who are unwell or who smoke
- wash your hands well and often - encourage teachers and carers to do the same
- strongly consider yearly flu vaccination for your child, whānau (family) and caregivers
- check if your child needs a booster pneumococcal pneumonia vaccine
- have an emergency plan for when your child becomes unwell with breathing problems
Flu vaccination for your child, whānau and caregivers every year is important. The Muscular Dystrophy Association may be able to help if it is not free for your child.
What are neuromuscular conditions?
Neuromuscular conditions (NMC) affect the nerves that control the body and muscles.
Some examples of NMC in tamariki (children) include:
- spinal muscular atrophy
- Duchenne muscular dystrophy
- congenital muscular dystrophy
- cerebral palsy
- myopathies
How weak muscles affect breathing and sleep
Make sure you have an emergency plan for when your child becomes unwell with their breathing.
Neuromuscular weakness can affect different muscle groups needed for breathing. This includes:
- the upper airway
- chest muscles
- back muscles
- the diaphragm which helps bring air in and out of the chest and also helps with coughing
Back muscles help keep the spine straight. This is important for chest size and expansion with breathing.
Tamariki with neuromuscular weakness have:
- slightly lower oxygen levels during sleep
- higher carbon dioxide levels during sleep
During sleep, muscles relax. During rapid eye movement (REM) sleep, the eyes move, but the rest of the body's muscles are paralysed. In tamariki with neuromuscular problems, this can cause sleep disordered breathing.
This can lead to:
- snoring or noisy breathing
- night waking
- poor quality sleep
Breathing problems may develop at different ages and often worsen over time.
Even when there is no cure for a neuromuscular condition, there are ways to help manage breathing problems that may improve your child's health.
Symptoms of sleep and breathing problems
Symptoms can include:
- fast, shallow breathing
- using neck or chest muscles to breathe (sucking in between ribs or around the neck)
- snoring or noisy breathing
- tiredness
- headaches - especially in the morning
- weak cough
- choking
Some serious breathing problems may not cause clear symptoms. Your child may not complain of shortness of breath, even with a severe NMC. Tests can help find problems early.
Tests for sleep and breathing problems
Chest x-ray
A chest x-ray can show:
- lung infection (pneumonia)
- signs of mucus in the lungs
- chest size
If chest muscles are weak, the chest may appear smaller. A normal x-ray does not rule out breathing problems.
Lung (pulmonary) function tests
All tamariki with NMC should have breathing tests when they are old enough (usually from 6 years) and able to.
These tests measure lung function while awake. They help to show the risk of breathing problems during illness.
Testing may need to happen every 6 months if weakness worsens, especially if your child cannot walk independently.
Tests may include:
- spirometry
- peak cough flow
- oximetry
Oximetry measures oxygen levels. It may be done:
- during hospital visits
- overnight in hospital
- overnight at home
Carbon dioxide levels may also be monitored.
Gas test
A small finger-prick blood test can check carbon dioxide levels. This can be done early in the morning or at random times.
Polysomnography sleep test
Your child's medical team may consider polysomnography (PSG) at least once a year if your child:
- has frequent lung infections
- has reduced lung function
- has abnormal gas test results
- has symptoms of hypoventilation
Hypoventilation means breathing that is too shallow or too slow. Symptoms include:
- morning headaches
- daytime sleepiness
- unrefreshing sleep
- poor growth not explained by their condition
Sometimes a PSG sleep test is not available. Your health professional may organise overnight oximetry with carbon dioxide monitoring. This may help find out if your child has abnormal breathing due to sleep disordered breathing or hypoventilation.
Swallow study (VFSS)
A speech-language therapist can assess your child's swallowing and airway muscle function.
Aspiration happens when food or drink goes into the lungs instead of the food pipe.
If there are worries that your child may be aspirating, the speech-language therapist will set up a swallow function study. This is an x-ray taken while your child eats. If this shows aspiration, your child may need a flexible tube (laryngoscopy) by ear, nose and throat (ENT) specialists. This looks at the airway and voice box.
Spinal x-rays
The health professional will check your child for scoliosis (curved spine). They will do this by examining your child and if necessary, by arranging spine x-rays.
Your child may need to see an orthopaedic surgeon (a doctor who specialises in bones and joints) to find out if they need surgery.
Managing breathing problems in neuromuscular conditions
Even in tamariki where there is no cure for a NMC, there are ways to help manage breathing problems that may improve your child's health.
Chest physiotherapy
Tamariki with a NMC may have a weak cough that limits their ability to get mucus out of their lungs. Chest physiotherapy can help tamariki who find it hard to clear mucus from their airways.
There are various therapies which can help your child cough out mucus, such as:
- percussion
- breath stacking
- cough assist device
- suctioning
Talk to your health professional about what is best for your child.
Vaccination
Good handwashing and avoiding sick people helps prevent infection.
All tamariki with NMC should have a flu vaccination every year. Your child may also benefit from a booster pneumococcal vaccine.
Some vaccinations may not be free. If you need to pay, contact Muscular Dystrophy Association NZ. They may be able to help.
Feeding tubes
Some tamariki cannot swallow safely and are at risk of aspiration. This can cause repeated chest infections. These tamariki may need a feeding tube. Feeding tubes can help prevent these problems and provide nutrition.
Feeding tubes may be:
- a nasogastric (NG) tube
- a tube placed into the stomach (PEG)
Sleep disordered breathing in neuromuscular conditions
Sleep can be a time when breathing is more difficult for tamariki with NMC. This is because their body position changes, muscles relax and breathing becomes shallower. Because of this, breathing problems may first show up during sleep. Health professionals call this ‘sleep disordered breathing’.
Sleep disordered breathing can range from mild to severe and may change over time.
One type of sleep disordered breathing happens when the breathing muscles aren’t strong enough during sleep. This leads to shallow breathing. Health professionals call this ‘hypoventilation’.
Another type of sleep disordered breathing happens when the airway narrows at times during sleep. This can lead to noisy breathing or snoring. This can cause poor quality sleep. Health professionals call this ‘obstructive sleep apnoea’.
Other things that can make breathing more difficult during sleep include:
- large adenoids
- large tonsils
- obesity
Snoring Or Noisy Breathing In Children
Your health professional will ask you about your child’s breathing during sleep. They can arrange tests if needed.
If your child has breathing problems during sleep, your health professional will talk with you about treatment. This may include:
- lifestyle changes
- medicines
- surgery
- machines or devices to support breathing
There are a range of different devices that your health professional may recommend depending on your child’s breathing problems. These devices may include:
- high-flow therapy
- CPAP (continuous positive airway pressure)
- BPAP (bilevel positive airway pressure)
These treatments can relieve symptoms and improve your child’s health and quality of life.
Continuous Positive Airway Pressure (CPAP)
Respiratory emergency action plan for children with neuromuscular conditions
It is important to have a plan for when your child becomes unwell. This is especially important if your child uses breathing support.
The plan should include:
- how to care for your child at home
- where to take your child for assessment
- treatments that may help in hospital (such as physiotherapy)
- equipment your child may need in hospital (such as a cough assist machine)
Your child may need help with breathing using a machine and mask during a severe chest infection or pneumonia.
Long-term use at home, usually overnight, only begins after careful discussion with you and your child. This discussion will include how your child is likely to do in the future (their prognosis).
Your health professional will also talk with you about ventilation options if your child has a sudden severe infection, or if their breathing slowly gets worse over time.
The Muscular Dystrophy Association (MDA) is a nationwide organisation. It supports New Zealanders living with neuromuscular conditions by providing information, personal support, and advocacy. MDA offers a range of services to help people living with a neuromuscular condition.
Acknowledgements
The content on this page has been developed and approved by the Paediatric Sleep Medicine Clinical Network, Paediatric Society New Zealand.
References
Muscular Dystrophy Association of New Zealand. [Accessed 26/08/2022]
Hull J, Aniapravan R, Chan E, et al. 2012. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax. [Accessed 26/08/2022]
Paediatric Sleep Medicine Clinical Network. Paediatric Society New Zealand. 2015. Sleep and sleep disordered breathing in children. [Accessed 26/08/2022]
Elizabeth Edwards. Paediatric Respiratory Service, Starship Children's Health. 2020. Starship clinical guidelines: Pre-operative assessment of children with neuromuscular disease for major surgery including scoliosis. [Accessed 26/08/2022]
