Sleep & Breathing Problems In Neuromuscular Conditions

Key points about sleep and breathing problems in neuromuscular conditions

• if your child has a neuromuscular condition, talk with your health professional, paediatrician or neurologist about symptoms to watch for and what tests your child may need
• watch for and tell a health professional about cough, swallowing difficulties or choking, poor sleep and night waking, snoring or noisy breathing, morning headaches, curvature of the spine (scoliosis)
• keep your child away from people who are sick or who smoke
• wash your hands often and well when caring for your child and encourage teachers or carers to do the same
• strongly consider yearly flu vaccination for your child, whānau (family) and caregivers 
• check to see if your child needs a booster pneumococcal pneumonia vaccine
• make sure you have an emergency plan for when your child becomes unwell with their breathing - this should cover what to do if your child has what seems like a simple cold, as well as dealing with a more severe chest infection

What are neuromuscular conditions?

Neuromuscular conditions (NMC) are disorders of the nerves that control the body and muscles.

Some examples of NMC in tamariki (children) are:

• spinal muscular atrophy
• Duchenne muscular dystrophy
• congenital muscular dystrophy
• cerebral palsy
• myopathies

How weak muscles affect breathing and sleep

Neuromuscular weakness can affect different muscle groups necessary for breathing:

• the upper airway
• chest muscles
• the diaphragm which helps bring air in and out of the chest and also helps with coughing

The muscles in the back help keep the spine straight, which is also important for chest size and expansion with breathing.

Tamariki 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 tamariki with neuromuscular problems this can cause 'sleep disordered breathing'. This makes breathing harder and causes tamariki to snore or have noisy breathing. It may also mean your child wakes during the night and has disrupted sleep.

Sleep and breathing problems

NMCs can cause breathing problems which may appear at different ages and get worse over time.

Symptoms of sleep and breathing problems

Symptoms can include:

• fast shallow breathing
• increased use of other muscles such as in the neck or rib cage (sucking in between ribs or around the neck)
• snoring or noisy breathing
• tiredness (fatigue)
• headaches (especially in the morning)
• weak cough
• choking

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.

Tests for sleep and breathing problems

Chest x-ray

Chest x-rays can help show up:

• pneumonia
• whether there is any mucus in the breathing passages or lungs
• the size of a child's chest

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. 

Lung (pulmonary) function tests

All tamariki with an NMC should have a breathing function test when they are old enough. Tamariki 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:

• spirometry
• peak cough flow
• oximetry - the medical team may check your child's oxygen levels or saturations at hospital visits during the day, or overnight during sleep in hospital, or at home. In hospital, the medical team may also monitor carbon dioxide levels

Overnight Oximetry

Early morning or random gas test

This is a blood gas from a finger prick to check the carbon dioxide level. 

Polysomnography sleep test

Your child's medical team may consider polysomnography (PSG) at least once a year if your child:

• has frequent pneumonias or chest infections, especially if the episodes lead to time in hospital
• has symptoms of hypoventilation (such as morning headaches, daytime sleepiness or excessive tiredness, unrefreshing sleep, poor growth that can't be explained by their underlying disease) 
• has reduced lung function or an abnormal early morning or random gas test

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).

Polysomnography Sleep Test

Overnight Oximetry

Swallow study (VFSS)

By watching your child's eating and drinking, a speech language therapist can find out about your child's:

• upper airway muscle function
• swallowing ability

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.  

Spinal x-rays

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.

X-rays For Children

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 may help tamariki 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:

• percussion
• breath stacking
• cough assist device
• suctioning

Talk to your health professional about what therapy is best for your child. 

Vaccination

Good hand washing and staying away from people who are ill can help avoid infections. All tamariki with a NMC should have a flu vaccination every year. Your child may also benefit from a booster pneumococcal pneumonia vaccination to help prevent infection caused by different types of Streptococcus pneumoniae bacteria.

Vaccination Overview

Flu vaccination

If vaccinations 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.

Feeding tubes

Some tamariki 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 tamariki may need a feeding tube. Feeding tubes prevent these problems and provide nutrition. Feeding tubes can be:

• a nasogastric or NG tube or
• a tube surgically placed into the stomach ('PEG')

Tube Feeding

Managing sleeping problems in neuromuscular conditions

Tamariki with NMC can be more likely to have sleep disordered breathing. Risk factors and treatments are similar to healthy tamariki.

Snoring Or Noisy Breathing In Children

Children with large tonsils or adenoids

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.

Tonsillectomy & Adenoidectomy

But tamariki 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 with obesity

Tamariki who are very overweight are more likely to have sleep disordered breathing. If your child is overweight, talk with a health professional or paediatrician about seeing a dietitian to discuss a weight management program.

Children who have problems with muscle tone

Tamariki can have trouble breathing during sleep. This is because the throat muscles relax and block the breathing passages. Tamariki 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 tamariki may need a breathing machine to help them breathe (such as continuous positive airway pressure - CPAP).

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 tamariki use this support only when they sleep.  These tamariki are likely to have one or more of the following:

• low levels of oxygen
• carbon dioxide levels which are above normal
• symptoms of shallow or slow breathing (hypoventilation)

Respiratory emergency action plan for children with neuromuscular conditions

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:

• how to care for your child at home
• where to take your child for assessment
• what treatments (for example, chest physiotherapy) may support your child if they need to spend time in hospital 
• what equipment (for example, cough assist machine) your child might need if they need to spend time in hospital 

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:

• how well your child is likely to do long term (their prognosis)
• future ventilation choices if the need arises with a sudden, severe chest infection, or, if there is a period of gradual worsening in your child's breathing over a longer period of time