Improved respiratory (breathing) management over the past 10 years has significantly improved the quality and length of life for people living with Duchenne. In October 2018, articles discussing specific areas of Duchenne care were released. One article “Respiratory Management of the Patient With Duchenne Muscular Dystrophy” was written by several pulmonologists who specialize in Duchenne. This article endorses the pulmonary guidelines published in the 2018 Care Considerations as well as discuss additional recommendations for providing optimal pulmonary care for people living with Duchenne.

 

Ambulatory

Seated forced vital capacity (FVC) is one reading that is collected during a pulmonary function test, and remains the “gold standard” for monitoring respiratory function in Duchenne. While visits with the pulmonology team should begin at diagnosis, pulmonary function testing with measurements of seated FVC should begin at 5 to 6 years of age so children can “practice” the test, and their course of pulmonary function can start to be tracked. Serial measurements of the FVC are extremely important for your pulmonologist to see what pulmonary function looks like over time.

Leading pulmonologists explain the importance of “anticipatory respiratory care.” This means that even if respiratory function is adequate, your pulmonologist may still want you or your child to start using assistive equipment, such as a long volume recruitment appliance or assisted coughing. Use of assistive devices has shown to keep people with Duchenne breathing optimally and feeling their best, as well as prevents potential complications from happening.

Early Non-Ambulatory

When the FVC measures less than 60% predicted, “lung volume recruitment” should be started.  “Lung volume recruitment,” or breath stacking, is a maneuver that can be taught to you by your pulmonologist and/or respiratory therapist to help to keep your lungs flexible, expanded and able to hold as much air as possible.

Coughing and keeping your airways clear is extremely important for maintain healthy lungs. Assisted cough should be started when FVC is less than 50% predicted, or the MEP (maximal expiratory (breathing out) pressure) is <60 cm H2O.  When assisted cough is started, a pulse oximeter should be available to assess the oxygen levels in the blood, diagnosing early mild hypoxemia that could be managed with airway clearance/assisted cough.

Late Non-Ambulatory

The article also discusses assessing the need for nocturnal and daytime respiratory assistance. Indications for sleep studies, nighttime ventilation and daytime ventilation are all discussed in the article and on our website.

Preventing Illnesses and Pneumonia

To prevent respiratory illnesses from happening, it is important to receive all vaccines, including the inactivated influenza (flu) vaccine yearly and pneumococcal vaccines, according to the recommended schedule.  When illness does occur, warnings about using oxygen without measuring carbon dioxide (CO2) or giving oxygen with non-invasive ventilations (Bi-PAP) were again stressed.

As people living with Duchenne grow older, coughing muscles become progressively weaker. Weak coughing muscles can put people with Duchenne at risk for mucus plugging, atelectasis (section of the lung collapsing), and pneumonia. One way to prevent these problems is to use assisted coughing more frequently when you or your child are sick.

In addition, during acute respiratory illness, such as a cold or bronchitis, early antibiotic therapy should be prescribed for patients who have 3 of the following 5 clinical signs of pneumonia:

1. Fever
2. Elevated white blood cell count or C-reactive protein level
3. Sputum (thick mucus or “phlegm”) development
4. Pulmonary infiltrate (dense area) on chest x-ray
5. Hypoxemia (low blood oxygen) or respiratory distress

By following the guidelines for pulmonary care, you should be able to keep your lungs as clear and as healthy as possible.

Share Your Data

Your pulmonary journey can help tell the story of pulmonary health in Duchenne and increase the understanding of experts in the field. That’s why we are asking you to update your Breathing/Respiratory module in The Duchenne Registry. Log into your Registry account, go to your Dashboard, and click on the Breathing/Respiratory module link. Either complete the survey for the first time, or select “Update survey” if you are updating your information.

Not in the Registry?

Join today and become a Citizen Scientist, adding your data to the already powerful Duchenne Registry! Please take 5 minutes today to complete this survey today!