Pulmonary management

Looking after the breathing muscles

Usually boys do not have trouble breathing or coughing while they are still walking. Because the breathing muscles become affected, as boys with DMD get older they are at risk of chest infections, often due to an ineffective cough. Later on they develop problems with their breathing when sleeping. When they are older, they may require help with breathing during the day as well. As this is a staged progression of problems, a planned and proactive approach to respiratory care is possible based around appropriate surveillance, prophylaxis and interventions. The team must include a doctor and therapist with skill in looking after the delivery of noninvasive ventilation and associated techniques for increasing the amount of air that can enter the lungs (lung volume recruitment), and manual and mechanically assisted cough.

Important facts to remember

  1. Keep a copy of your son’s latest breathing tests to show any doctor who takes care of him.
  2. Your son should never be given inhaled anaesthesia or the drug succinylcholine.
  3. Your son’s lung function should be checked before surgery. It is good to pick up on silent problems so that they can be treated promptly.
  4. Your son will need help with coughing and antibiotics if he has a chest infection.
  5. Symptoms of hypoventilation and weak cough should be monitored for and be reported to the medical caregivers so that therapy can be initiated.
  6. If your son’s oxygen level drops when he is ill or injured, the doctor must be very careful giving him oxygen because this can cause a situation where his own urge to breathe is decreased.

Information based on consensus statement (published in January 2010)