Flu immunisation for your child, family and caregivers every year is important. The Muscular Dystrophy Association may be able to help if it is not free for your child.
Neuromuscular conditions (NMC) are disorders of the nerves that control the body and muscles.
Some examples of NMC in children are:
Make sure you have an emergency plan for when your child becomes unwell with their breathing.
Neuromuscular weakness can affect different muscle groups necessary for breathing:
The muscles in the back help keep the spine straight, which is also important for chest size and expansion with breathing.
Children with neuromuscular weakness have oxygen levels that are a little lower and carbon dioxide levels that are higher during sleep. For everyone, when you sleep your muscles relax and during one stage of sleep (rapid eye movement or REM sleep) your eyes continue to move but the rest of the body's muscles are paralysed. In children with neuromuscular problems this can cause 'sleep disordered breathing'. This makes breathing harder and causes children to snore or have noisy breathing. It may also mean your child wakes during the night and has disrupted sleep.
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.
NMCs can cause breathing problems which may appear at different ages and get worse over time.
Symptoms can include:
There can also be breathing problems that are serious but do not cause immediate symptoms. Your child may not complain about feeling short of breath or working hard to breathe, even with a severe NMC. This is why some tests can help find sleep and breathing problems.
Chest x-rays can help show up:
If the chest wall muscles don't work well, the chest appears smaller on an x-ray. But even if the x-ray shows no changes, your child may still have a breathing problem.
All children with an NMC should have a breathing function test when they are old enough. Children usually need to be at least 6 years old to be able to cooperate with the test. These tests measure how much lung function your child has when they are awake. The results may predict how likely it is your child will have serious breathing difficulties during respiratory illnesses.
These tests need to happen more often (every 6 months) when your child's neuromuscular weakness gets worse, and especially when they are unable to walk independently.
Lung function testing may include the following:
This is a blood gas from a finger prick to check the carbon dioxide level.
Your child's medical team may consider polysomnography (PSG) at least once a year if your child:
Or, if a PSG is not available, your child should have a basic overnight oximetry together with carbon dioxide monitoring. This may help find out if your child has abnormal breathing due to sleep disordered breathing, or breathing that is too shallow or slow for the needs of their body (hypoventilation).
Find out about polysomnography [3]
Find out about overnight oximetry [2]
By watching your child's eating and drinking, a speech language therapist can find out about your child's:
If there are concerns about food or liquid 'going down the wrong way' into the lungs (aspiration), the speech language therapist will arrange for a swallow function study. The technical term for this is video fluoroscopy swallow study (VFSS). It is an x-ray imaging study while your child is eating. If this shows aspiration, your child may need a flexible tube (laryngoscopy) by ear, nose and throat (ENT) specialists. This tube looks at the upper airway and voice box.
Your child's doctor will check your child for a scoliosis (curved spine). They will do this by examining your child and if necessary, by spine x-rays. Your paediatrician will talk to you about the need for spinal x-rays. Your child may need to see an orthopaedic surgeon (a doctor who specialises in conditions related to bones, ligaments, tendons and muscles) to find out if they need surgery for their scoliosis.
Even in children where there is no cure for a NMC, there are ways to help manage breathing problems that may improve your child's health.
Children with a NMC may have a weak cough that limits their ability to get mucus out of their lungs. Chest physiotherapy may help children who have difficulty clearing mucus from their breathing passages because of an ineffective or weak cough. There are various therapies which can help your child cough out mucus, such as:
Talk to your doctor about what therapy is best for your child.
Good hand washing and staying away from people who are ill can help avoid infections. All children with a NMC should have a flu immunisation every year. Your child may also benefit from a booster pneumococcal pneumonia immunisation to help prevent infection caused by different types of Streptococcus pneumoniae bacteria.
Find out more about immunisation for children [4]
Find out more about flu immunisation [5]
If immunisations aren't free for your child with a neuromuscular condition, you could contact the Muscular Dystrophy Association NZ. They may be able to help with the cost.
Some children cannot swallow well and are at risk of food or liquid going down the wrong way into the lungs (aspiration). This can lead to repeated chest infections. These children may need a feeding tube. Feeding tubes prevent these problems and provide nutrition. Feeding tubes can be:
Find out more about tube feeding [6]
Children with NMC can be more likely to have sleep disordered breathing. Risk factors and treatments are similar to healthy children.
Find out about snoring or noisy breathing [7]
Large tonsils and adenoids can block the airway. If your child has large tonsils or adenoids, or both, they may benefit from surgery to remove them.
Find out about tonsillectomy and adenotonsillectomy [8]
But children wtih an NMC are at higher risk of breathing problems when they sleep (such as sleep apnoea or obstructive sleep apnoea). So your child may need to have a polysomnography 2 ro 3 months after surgery to make sure the sleep disordered breathing has got better.
Children who are very overweight are more likely to have sleep disordered breathing. If your child is overweight, talk with your family doctor or paediatrician about seeing a dietitian to discuss a weight management program.
Children can have trouble breathing during sleep. This is because the throat muscles relax and block the breathing passages. Children with an NMC may need support for breathing at night.
If your child has difficulty getting enough air and has low oxygen levels, there may be ways to help provide extra oxygen or other support for breathing. It's best to talk to a paediatric sleep medicine specialist about what options are possible for your child and how well they may work.
Some children may need a breathing machine to help them breathe (such as continuous positive airway pressure - CPAP).
This is usually through a mask on the nose or a larger mask covering the nose and mouth (full face mask). Some children use this support only when they sleep. These children are likely to have one or more of the following:
It is important to have an emergency plan for when your child becomes unwell with their breathing. This is especially important if your child uses a ventilator or breathing support. Make sure you have a plan for what to do when your child becomes unwell with what might seem like a simple cold, a more severe chest infection, or develops breathing difficulties during sleep. The plan should include:
Your child may need help with their breathing with a machine and a mask during a severe chest infection or pneumonia. But, long-term overnight use at home only starts after discussion with parents and patients. Discussion will focus on:
Muscular Dystrophy Association of New Zealand [10]. [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 [11]. [Accessed 26/08/2022]
Paediatric Sleep Medicine Clinical Network. Paediatric Society New Zealand. 2015. Sleep and sleep disordered breathing in children [12]. [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 [13]. [Accessed 26/08/2022]
The content on this page has been developed and approved by the Paediatric Sleep Medicine Clinical Network, Paediatric Society New Zealand.
This page last reviewed 26 August 2022.
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Links
[1] https://www.kidshealth.org.nz/cerebral-palsy-overview
[2] https://www.kidshealth.org.nz/overnight-oximetry
[3] https://www.kidshealth.org.nz/polysomnography-sleep-test
[4] https://www.kidshealth.org.nz/immunisation-overview
[5] https://www.kidshealth.org.nz/flu-immunisation
[6] https://www.kidshealth.org.nz/tube-feeding
[7] https://www.kidshealth.org.nz/snoring-or-noisy-breathing-children
[8] https://www.kidshealth.org.nz/tonsillectomy-adenotonsillectomy
[9] https://www.kidshealth.org.nz/continuous-positive-airway-pressure-cpap
[10] https://www.mda.org.nz/
[11] https://thorax.bmj.com/content/67/Suppl_1/i1.long
[12] https://starship.org.nz/guidelines/sleep-and-sleep-disordered-breathing-in-children/
[13] https://starship.org.nz/guidelines/scoliosis-in-neuromuscular-disorders/
[14] https://www.kidshealth.org.nz/node/2140?language=rar
[15] https://www.kidshealth.org.nz/contact?from=http%3A%2F%2Fwww.kidshealth.org.nz%2Fprint%2F2128%3Flanguage%3Drar