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Neuromuscular diseases cause weakness of the muscles of your body including the respiratory muscles that help in breathing.  This happens because the nerves supplying these muscles are affected or the when the brain is affected so that it is not able to send messages to the muscles. As a result, breathing becomes difficult and the effort is insufficient for appropriate breathing.

Which conditions cause respiratory muscle weakness?

Neuromuscular disorders, genetic conditions affecting the brain and patients of cerebral palsy can have muscle weakness. Some examples of disorders affecting the respiratory muscles are

  • Muscular dystrophies
  • Myasthenia Gravis
  • Spinal Muscular Atrophy
  • Down’s syndrome
  • Genetic disorders affecting the neuromuscular system
  • Cerebral palsy

How can neuromuscular disorders affect my breathing?

  • Muscle weakness in the chest and diaphragm makes breathing difficult and the child is unable to take deep full breaths. As less air exchanges in the lungs, the oxygen level drops and carbon dioxide level rise and leads to respiratory failure.
  • Weakened back muscles lead to postural changes like kyphosis and scoliosis. As a result, the chest becomes smaller and lung capacity also reduces.
  • Coughing is protective reflex that helps in expelling out secretions from the lungs. Weakness of the muscles lead to accumulation of secretions in the lungs and makes it more susceptible to infections.
  • Weakness of swallowing muscles lead to feeding difficulties and increases chances of aspiration.
  • Weakness of upper respiratory muscles leads to collapse of upper airways during sleeps causing obstructive sleep apnea.

What are the early signs of breathing problems in these children?

Early signs of breathing problems with neuromuscular disease include:

  • Rapid and shallow breathing
  • Low energy
  • Difficulty breathing
  • Wakes up with a headache that goes on waking up
  • Colds that last more than 10 days

As the condition progresses, oxygen levels start falling and when they are chronically low, the following additional symptoms arise:

  • Daytime sleepiness
  • Shortness of breath at rest
  • Morning headaches

How do we help these children with breathing?

  • Non-Invasive ventilation with CPAP or BiPAP can be given to support the respiratory muscles and help in deep breathing. This allows the body to maintain optimum oxygen levels and remove excess carbon dioxide.
    These devices can be used at home continuously or while sleeping. Numerous nasal masks and devices are available to suit the patient’s need. Parents are trained to use these machines and take care of children on home ventilation.
  • Respiratory muscle exercises and deep breathing exercises help in strengthening of these muscles
  • Physiotherapy for airway clearance is advised to clear the airway and reduce chances of infection
  • In case spine deformities, surgical correction can be done