As people with Duchenne muscular dystrophy (DMD) grow older, breathing becomes harder: respiratory failure is one of the two main causes of mortality in DMD.
People with DMD often have trouble breathing at night (when breathing is naturally shallower) long before they have trouble breathing during the day. Non-invasive nighttime ventilation can help support breathing during sleep to improve sleep quality and overall wellbeing.
Why is nocturnal ventilation important in DMD?
Most of the time, the body breathes with the help of the diaphragm, the intercostal muscles (found between the ribs) and with other muscles in the chest, neck and abdomen. However, during REM sleep, breathing changes and the diaphragm is the only muscle keeping the body breathing.
In children and young adults with DMD, muscle weakness puts a strain on the diaphragm and other respiratory muscles. This can lead to shallow breathing (hypoventilation) during REM sleep; as a result, less oxygen is circulated around the body. Low oxygen levels and a buildup of carbon dioxide can cause many health issues.
The consequences of poor-quality sleep include daytime sleepiness, headaches, poor concentration, irritability, increased risk of respiratory infections, strain on the heart and general fatigue. The long-term impact includes respiratory failure.
Sleep-disordered breathing in DMD is exacerbated by other contributing factors, including obesity, the use of corticosteroids and scoliosis.
Nocturnal ventilation is used to support the respiratory system and maintain adequate breathing during the night. Its use also has benefits for daytime breathing and energy levels.
Learn more about DMD treatment and care
How is nocturnal ventilation administered in DMD?
When a care team starts to suspect that nighttime breathing is being impacted, they’ll generally recommend a sleep study. Oxygen and carbon dioxide levels are measured, as well as interruptions in breathing, called apnea. If breathing is too shallow, they will likely recommend noninvasive ventilation.
The most common form of nocturnal ventilation is a bilevel positive airway pressure (BiPAP) machine. A BiPAP machine delivers pressurized air through a face mask worn overnight. It supports the respiratory system via two pressure settings:
- Inhalation pressure (IPAP): Helps the lungs draw in air.
- Exhalation pressure (EPAP): Helps keep the airways open during exhalation.
It might take time to get used to using a BiPAP machine, as the mask can feel tight. Yet nighttime ventilation is worth it: regular use of BiPAP improves not only nighttime breathing and sleep but also daytime respiratory function, which can help delay the need for daytime ventilation.
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