Treatment for Sleep-Disordered Breathing in Muscular Dystrophy

Written by Alexandra Childs – Clinical Measurements Scientist (Sleep and Respiratory) and PhD student

Metro North Health & The University of Queensland

Sleep-disordered breathing is common in people living with muscular dystrophy and other neuromuscular conditions and can have a significant impact on sleep quality, daytime functioning and overall health. Understanding the available treatment options is an important step in managing symptoms, improving breathing during sleep and supporting long-term wellbeing.

Sleep requires an individualised approach, and not everything in this article will apply to you. If any information here raises concerns, please speak to your doctor.

What are the different types of treatments for sleep-disordered breathing?

The goal of treatment for sleep-disordered breathing is to correct or compensate for ineffective breathing patterns during sleep. This is usually achieved using specialised machines or ventilators that support the airway and breathing.

For conditions such as sleep apnoea and sleep hypoventilation, non-invasive positive pressure ventilation is most commonly used. This involves a machine gently blowing air into the lungs through an external interface, such as a mask worn over the nose or mouth. Unlike invasive ventilation, this support sits outside the body rather than using a tube placed inside the airway.

In some situations, non-invasive ventilation may not be suitable. This can include people who have ongoing aspiration issues, are unable to tolerate non-invasive support, or cannot be weaned from ventilation following emergency intubation. In these cases, long-term invasive ventilation using a tracheostomy tube may be considered. More information on this type of treatment is available on the Parent Project Muscular Dystrophy website.

This article focuses on long-term non-invasive treatments only and does not cover emergency treatments, mouthpiece ventilation or invasive ventilation.

What is CPAP and BiPAP?

The two main types of non-invasive positive pressure treatment are Continuous Positive Airway Pressure (CPAP) and Bilevel Positive Airway Pressure (Bilevel). While both therapies support breathing during sleep, they work in different ways and are used to treat different types of sleep-disordered breathing.

Continuous positive airway pressure (CPAP)

CPAP is described as continuous pressure because it delivers a single, steady level of air pressure throughout the night. This treatment is most commonly used for sleep apnoea.

CPAP works by gently splinting the airway open from the outside in, preventing it from collapsing during sleep. By keeping the airway open, CPAP reduces pauses in breathing and improves airflow while you sleep.

Bilevel positive airway pressure (Bilevel)

Bilevel therapy is known by several names, including BPAP, BiPAP, VPAP and NIV. These terms all refer to the same type of treatment, which delivers two different levels of air pressure during sleep.

The lower pressure, called EPAP (expiratory positive airway pressure), is applied during breathing out. It works in a similar way to CPAP by helping to keep the airway open, but is timed with exhalation so the lungs do not have to push against higher pressure.

The higher pressure, called IPAP (inspiratory positive airway pressure), is delivered during breathing in. While EPAP maintains airway openness, IPAP helps expand the lungs and deepen each breath.

Bilevel therapy is effective for treating sleep apnoea, but it is also commonly used when breathing weakness affects the exchange of oxygen and carbon dioxide. This makes it particularly important for people living with neuromuscular conditions.

Can the treatment I need change over time?

Yes. As you get older and the strength of your breathing muscles changes, the type of therapy you need may also change. This is why regular review of your treatment is so important.

Many people start with CPAP when sleep apnoea is the main issue. Over time, breathing can become shallower, meaning the body is less effective at balancing oxygen and carbon dioxide during sleep. When this happens, bilevel therapy may be recommended to provide additional breathing support.

Needing bilevel therapy does not mean you are getting sicker, and in most cases it is not end of life support. In fact, research shows that using bilevel therapy regularly can be life extending and can reduce the risk of lung infections such as pneumonia, which can cause serious damage to the lungs.

Moving from CPAP to bilevel therapy simply reflects a change in how your breathing needs to be supported so your lungs can work as effectively as possible during sleep and sometimes during the day.

Adjusting to bilevel therapy can take time, as it feels different from CPAP. However, bilevel therapy continues to provide the airway support you are used to, with the added benefit of helping deepen breathing and balance oxygen and carbon dioxide levels in the body.

What types of masks are there and what should I use?

When starting treatment, finding the right mask can be one of the biggest challenges. One of the main goals of a sleep study is to identify a mask that works well for you, as comfort and fit play a major role in successful treatment.

Your mask should:

1. Fit your face well, create a good seal and minimise air leaks
2. Feel comfortable and be something you can put on and use independently, or with support, at home

There are two main types of masks used during sleep: nasal masks and full face masks.

Nasal masks cover the nose only and are commonly used by people who breathe comfortably through their nose during sleep. Full face masks cover both the nose and mouth. They were originally designed for people who cannot breathe well through their nose due to nasal blockage, but many people prefer them because they find breathing through the mouth more comfortable.

There are many different mask designs available from a range of brands, each with its own advantages and limitations. Depending on how your mask is sourced, it can be costly. Once you have a general idea of what feels right for you, it can be helpful to do some research or speak with your local supplier to find an option within your price range.

Some of the different mask designs and interfaces are shown below.

Figure 4: Different types of masks and interfaces used for non-invasive ventilation (PAP therapy). Adapted from https://doi.org/10.4187/respcare.089

For some people living with muscular dystrophy, weakness or wasting of the facial muscles can affect how well a mask seals. If you notice changes in the shape of your face over time, or if your mask no longer fits as well as it once did, it may be worth reviewing your mask with your sleep clinic or equipment provider.

What is a chinstrap? Will I need one?

A chinstrap is a soft fabric strap that wraps around your head and under your jaw to help keep your mouth closed while you sleep. During your sleep study, you might be fitted with a chinstrap if the team notices you are breathing through your mouth or if air is leaking from your mask.

Chinstraps aren’t suitable for everyone, and not everyone will need one. If your treating team thinks a chinstrap could help you, they will discuss it with you and work with you to find a style that’s comfortable or suggest an alternative if needed.

What does treatment feel like? What should I expect?

CPAP or Bilevel treatment can feel unusual at first because it’s different from other therapies.

• CPAP provides a single, steady level of air pressure. When you breathe in, it may feel like air is rushing into your lungs until your body adjusts.
• Bilevel offers two levels of pressure that sync with your breathing. The machine increases pressure as you inhale and decreases it as you exhale, making it feel more natural once you and the machine are in tune.

Here are a few things to keep in mind:

1. An adjustment period is normal – it won’t feel perfect immediately. Your body needs time to get used to it.
2. Go at your own pace – you don’t need to use PAP for the whole night from the start. Many people begin by simply sitting with the mask on while watching TV or reading to get comfortable. Gradually increase your usage over time.
3. Your feedback matters – if it’s still difficult, talk to your treating team. They can help improve comfort and address issues such as pressure levels, mask fit, side effects (like dry mouth) or feelings of anxiety or claustrophobia. Letting them know what’s challenging allows them to tailor the treatment to your needs.

Summary

Sleep-disordered breathing is common in people living with muscular dystrophy, but effective treatments like CPAP and Bilevel therapy can make a huge difference. Masks, chinstraps, and the right pressure settings help support breathing, and adjustment takes time and practice.

Your treatment may change as your needs evolve, and your feedback is essential to make it comfortable and effective. Regular review and support from your sleep or respiratory team ensure you get the most benefit, helping improve sleep, overall health and quality of life.

If you’d like to talk to someone about any of this information, please call us on 07 3243 9700.

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