In muscular dystrophy, progressive weakness of respiratory muscles can result in varying degrees of breathing difficulty.

One of the most important respiratory muscles is the diaphragm, which sits just below the lungs and helps in the process of inhalation, or breathing in, which supplies oxygen to the lungs. The weakening of the diaphragm in people with muscular dystrophy results in reduced oxygen intake and decreased lung function.

Different muscles help in exhalation, or removal of carbon dioxide. Contraction of muscles in the abdomen during activity supports exhalation, while at rest, lung elasticity aides in the removal of carbon dioxide. In muscular dystrophy patients, the excessive workload on the lungs, due to poor diaphragm function and the weakening of abdominal muscles, hinders the elimination of carbon dioxide.

Weakness in the muscles of the upper respiratory tract, or the nose and throat, causes difficulty breathing during sleep. This makes muscular dystrophy patients, therefore, prone to breathing problems while they are sleeping.

Respiratory muscles also support coughing, and their breakdown causes coughing difficulties.

Scoliosis, or the abnormal curvature of the spine, affects the structure of the chest wall, which also can contribute to breathing problems.

Symptoms

Monitoring breathing and coughing ability are vital for people with muscular dystrophy to support the early identification and management of breathing problems.

Symptoms of breathing problems in MD patients include:

  • Shallow breathing and snoring
  • Difficulty sleeping
  • Wheezing
  • Shortness of breath, especially at rest
  • A cold that lasts for more than 10 days
  • Morning headaches
  • Daytime sleepiness

Testing

When breathing problems are suspected, a trained pulmonologist may perform a series of tests to determine the strength of respiratory muscles to perform their function. These tests include:

  • Pulmonary function tests that are usually performed in children over age 5
  • Sleep studies to determine nighttime breathing patterns
  • Pulse oximetry to measure oxygen levels in the blood

Treatment and management

The treatment plan to address breathing problems in people with muscular dystrophy is based on the breathing symptoms observed.

Children with muscular dystrophy are prone to respiratory infections such as pneumonia. Therefore, it is recommended that they receive pneumonia vaccination as a preventive measure. For acute respiratory infections, antibiotic therapy is prescribed.

Chest physiotherapy in consultation with trained respiratory therapists, and the use of assistive devices such as vests, can help in clearing mucus, and prevent recurrent infections.

Bronchodilators to open the airways can help in alleviating wheezing. They can be delivered using an inhaler or nebulizer.

Ventilation is useful for patients with respiratory failure or hypoventilation (excessively slow breathing). Ventilation is the use of a machine (a ventilator) that helps the individual to breathe normally. There are non-invasive as well as invasive ventilation options available. Non-invasive ventilation is either through the nose, mouth, or full-face masks. Invasive ventilation involves inserting a tube into the windpipe, either through the patient’s mouth or nose, or through an incision in the neck.

In some cases, surgery to correct scoliosis may be required to relieve the pressure on respiratory muscles and ease breathing.

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