Pulmonary Management Overview
People with Duchenne typically do not have trouble breathing or coughing while they are still walking. As you get older and coughing muscles weaken, you may be at risk for chest infections due to an ineffective cough. Later on, the muscles that support breathing may weaken as well, and you may develop problems with breathing during sleep. It is important to let your neuromuscular team know if you have headaches or morning fatigue, as these may be signs of breathing too shallowly during sleep, and indicate the need for a sleep study.
If your cough is weakening, it is extremely important to use devices to make your cough more effective, especially if you are sick. A sleep study evaluates how well you are breathing while asleep. If your oxygen levels are found to be too low while you are sleeping, you will need to use bi-level positive airway pressure (BI-PAP) to help improve your breathing during sleep. You may find, as you get older, that you may need help withbreathing during the day as well.
A planned and proactive approach to respiratory care is necessary and should be based around close monitoring, prevention of problems, and early intervention when indicated. Your pulmonary team should include a doctor (pulmonologist) and respiratory therapist with experience in evaluating breathing in people with Duchenne. Their treatment goals typically include implementing techniques that expand the lungs and keep the chest wall muscles supple (lung volume recruitment), manually and mechanically assist cough for effective airway clearance, and to deliver non-invasive or invasive nighttime and daytime ventilatory support.