Respiratory Management in Neuromuscular Conditions

Written by Lynne Borgert, Allied Health Team Lead, and Stephanie Schrader, Senior Occupational Therapist

Breathing difficulties and sleep-disordered breathing are common complications in neuromuscular conditions (NMCs). These difficulties are often caused by weakness in the diaphragm, intercostal muscles (between the ribs), and upper airway muscles. Poor respiratory management can result in serious complications, injury, or death. For individuals with an NMC, proper respiratory care and ventilation are associated with longer life expectancy, improved quality of life, and reduced risk of pulmonary disease.

Individuals with an NMC may also have a weak cough due to muscular weakness. Coughing plays a crucial role in clearing the lungs of irritants such as smoke, mucus, fluid, germs, and pollen. A weak or ineffective cough increases the risk of hospitalisation and respiratory disease.

The following sections outline key aspects of respiratory management for people living with an NMC.

Baseline Respiratory Assessment

A baseline respiratory assessment is recommended at diagnosis if respiratory muscle involvement is likely. Best practice guidelines emphasise:

• Regular review with a respiratory physician and multidisciplinary team
• Sleep study testing
• Blood gas analysis
• Assessment of clinical signs

Recognising Signs and Symptoms

Awareness of respiratory muscle weakness and respiratory failure is critical.

Signs of respiratory muscle weakness requiring specialist attention:

• Fast, shallow breathing
• Limited chest movement during inhalation
• Use of accessory muscles (neck or shoulder) to breathe
• Quieter than normal breathing sounds
• Stomach moving inward instead of outward during inhalation
• Weak cough or weak sniff

Signs of respiratory failure requiring emergency support (call 000):

• Bluish colour around lips or fingertips
• Shaky hands or tremors
• Bulging neck veins
• Strong, pounding pulse
• Swelling around the eyes (papilledema)
• Confusion, drowsiness, or difficulty waking

Signs of inadequate or underventilation:

• Daytime drowsiness
• Morning headaches
• Tachycardia (increased heart rate)
• Fatigue
• Dizziness

Physiotherapy

Physiotherapy supports respiratory health by:

• Managing symptoms
• Optimising positioning for breathing through assistive technology
• Recommending cough augmentation techniques
• Providing exercises to enhance quality of life

Positioning

Correct positioning can open airways and improve breathing efficiency. Specialists and physiotherapists may recommend postural supports, such as power tilt seating, to maintain spinal alignment and reduce progression of spinal deformities like scoliosis.

Scoliosis is a lateral curvature and rotation of the spine that can stiffen the chest wall, reducing lung expansion and making breathing more difficult. Proper positioning is key to slowing scoliosis progression.

Cough Augmentation and Lung Recruitment

Cough augmentation and lung recruitment strategies help individuals with weak respiratory muscles to cough effectively and fully expand their lungs. These are taught by physicians and physiotherapists.

Cough augmentation is critical for preventing respiratory failure in NMCs, which often occurs due to ineffective coughing during recurrent chest infections. Techniques can be implemented by the individual or caregivers, and include:

• Use of an Ambu bag to teach breath stacking (pictured below)
• Glossopharyngeal breathing (GPB), or “frog breathing,” which uses mouth and pharynx muscles to push air into the lungs

Equipment Access Through Muscular Dystrophy Queensland

Cough assist devices, or mechanical insufflation-exsufflation (MI-E), are prescribed by a respiratory team. These devices:

• Provide positive pressure to inflate the lungs and negative pressure to induce a cough
• Clear lung secretions
• Reduce risk of lung infections and hospitalisation
• Maintain lung function by removing irritants

Evidence shows that using cough assist devices with non-invasive ventilation (NIV) reduces respiratory infection hospitalisations and can increase lifespan, particularly in clients with Duchenne muscular dystrophy. Respiratory physiotherapists assess individual needs, settings, and provide training.

At Muscular Dystrophy Queensland, we understand that obtaining this equipment can be challenging. Funding delays, unexpected illness or the need to trial equipment can leave people without timely access to what is often lifesaving support.

Through our Equipment Loan Program, MDQ offers short- or long-term loans of cough assist machines for Queenslanders with neuromuscular conditions.

This program can support people who:

• Need a machine urgently during or after a respiratory infection
• Require equipment while awaiting NDIS or alternative funding
• Would benefit from trialling a device prior to purchase
• Need short-term support during periods of illness or recovery

Our team provides the device, initial consumables and guidance on safe and effective use.

To learn more about the program, visit our website: mdqld.org.au/services/equipment-loan-program/

Suctioning

Suctioning helps manage airway secretions. Individuals with NMCs often struggle to clear secretions due to respiratory muscle weakness.

• Upper airway suctioning: Performed at home or in the community by trained caregivers or support workers using suction machines and catheters
• Lower airway suctioning: Performed in hospital via tracheostomy or bronchial tube when secretions cannot be cleared or during respiratory infections

Assisted Ventilation

Non-Invasive Ventilation (NIV)
NIV provides breathing support via masks without invasive procedures. It reduces muscle workload and supports breathing in both acute and chronic respiratory failure.

• Bi-Level Positive Airway Pressure (BiPAP): Supports inhalation and exhalation, commonly used for nocturnal ventilation and hypoventilation disorders
• Continuous Positive Airway Pressure (CPAP): Keeps airways open continuously, first-line treatment for moderate to severe obstructive sleep apnoea
• Daytime ventilation: Offered via mouthpiece and bilevel machine if hypoventilation occurs during the day

Masks for NIV
Masks vary in design and fit, including full-face, total-face, and nasal masks. Masks consist of:

1. Cushion (gel, foam, silicone, or flexible material)
2. Frame/shell connecting cushion to tubing and headgear
3. Headgear straps to secure mask

Discomfort or poor mask fit should be addressed with a respiratory physician for reassessment.

Invasive Mechanical Ventilation
Used in hospital when NIV fails or in acute respiratory failure, typically via endotracheal tube or tracheostomy. Mechanical ventilators take over breathing entirely. Once stabilised, clients may transition to home care.

Oxygen Therapy

Oxygen therapy is generally contraindicated in NMCs and should be prescribed with caution. Oxygen alone may cause hypercapnia (high blood CO₂), reducing the drive to breathe and worsening respiratory function.

Anaesthetic Considerations in DMD

Inhalational anaesthetics are contraindicated in people with Duchenne muscular dystrophy and other conditions involving ongoing muscle degeneration due to the risk of severe complications, including rhabdomyolysis and malignant hyperthermia. Always alert medical teams to the neuromuscular diagnosis prior to surgery.

Summary

Effective respiratory management is a crucial part of supporting someone living with a neuromuscular condition. Regular review with a respiratory physician, physiotherapist and multidisciplinary team ensures needs are identified early and managed appropriately.

At Muscular Dystrophy Queensland, we are committed to helping individuals access the equipment and support they need. Our equipment loan program provides vital access to cough assist machines when they are needed most, helping prevent hospitalisation and supporting safer respiratory care at home.

If you’d like to talk to someone about borrowing equipment please call us on 07 3243 9700.

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