Pulmonary (Lung) Care

Lung monitoring and treatment

Lung problems are often a major concern, but they are treatable. Especially once boys can no longer walk, it is important that healthcare providers team up to notice, prevent, and treat lung problems. Early signs to look for include morning headache, more daytime sleepiness, and not sleeping soundly at night. Chest infections need not be a problem and can be almost entirely prevented by proper use of assisted coughing such as with a combination of the CoughAssist(TM) augmented by a manual assist. Healthcare providers should help families get CoughAssist set up properly, and should train families in its use. Providers should advise using CoughAssist every day, not only when a person is sick. To reduce the chance of illness, children should have an annual influenza vaccination starting at 6 months of age.

If there is large enough decrease in lung function, healthcare providers should talk to the family about non-invasive intermittent positive pressure ventilation (BiPAP), which is often used at home during the night. As children get older, ventilation may need to be used during the day, too.

How often to have monitoring, and what is involved

Regular monitoring is important. Different healthcare providers may have different monitoring plans, but many plans look something like this:

  • Children between ages 4 and 6 have at least one visit with specialist in pediatric respiratory care.
  • Children who are 12 years or older, or children who are in a wheelchair, or children who have a predicted fall in vital capacity below 80% see a specialist in pediatric respiratory care twice a year.
  • People who need mechanically-assisted airway clearance therapy or mechanically assisted ventilation see a pulmonologist at least every 3 to 6 months.

Lung tests should include a medical history that focuses on symptoms of breathing problems during sleep, a physical exam, and tests of lung function. When there is a concern about breathing problems during sleep, the healthcare provider may suggest special tests like a sleep study or test of oxygen or carbon dioxide levels. Pulmonary function (how well the lungs are working) is checked by using tests like peak cough flow and forced vital capacity (FVC).

To learn more:

  • Read PPMD's Fact Sheet on lung health.
  • “Respiratory Care” section of the TREAT-NMD recommendations
  • American Thoracic Society. Respiratory care of the patient with Duchenne muscular dystrophy: American Thoracic Society Consensus Statement. Am J Respir Crit Care Med 2004 170: 456-465.