by: Parent Project Muscular Dystrophy
The PPMD team was recently joined by pulmonologist Dr. Janaki Paskaradevan, psychologist Dr. Allison Clarke, and Maria Gonzalez, MSN, APRN, FNP-BC, CPN, from the Division of Pulmonary and Sleep Medicine at Ann & Robert H. Lurie Children’s Hospital, where they led a discussion on the critical role of BiPAP use in Duchenne and Becker muscular dystrophy. BiPAP (Bilevel positive pressure ventilation) is a machine that provides two different levels of air pressure to help support breathing in individuals who have weaker breathing muscles. This is often prescribed for individuals with Duchenne and Becker and can sometimes pose challenges with its use. The speakers emphasized the importance of consistent use of BiPAP, even when individuals feel well, and highlighted the potential long-term impact of not wearing prescribed breathing support.
The team also outlined common physical and psychosocial challenges families often face when starting BiPAP and shared practical strategies to support better compliance. Some of the strategies used in their multidisciplinary PAP Clinic that can be helpful in supporting successful BiPAP use include patient education, behavioral approaches, caregiver engagement, and regular follow-ups with the neuromuscular team. These solutions help encourage adherence and improve outcomes for individuals living with Duchenne and Becker muscular dystrophy. The Lurie team outlined the following techniques to support families in overcoming challenges with BiPAP use:
Physical Challenges of BiPAP Use
| Problem | Solution |
|---|---|
| Mask leaking or discomfort | Mask/headgear adjustment; Re-fitting with a new mask |
| Nasal congestion/epistaxis | Adjust humidification and ensure heated tubing; Maintenance of clean equipment; Consider intranasal steroid, saline nasal gel |
| Skin irritation | Apply a skin barrier (e.g., Mepilex); Alternate masks and proper mask maintenance |
| Dry mouth | Increase humidity; Check for leaks (excessive leak can exacerbate dry mouth); Proper hydration |
| Aerophagia | Mild symptoms may be treated with simethicone; Reduce pressure settings; Can consider increasing inspiratory time (i-time) and rise time |
| PAP rainout (condensation in tubing) | Use heated tubing; Position device lower than the mask; Adjust temperature and humidifier settings |
| Mouth leak | Use a chin strap |
Psychosocial Challenges of BiPAP Use
| Problem | Solution |
|---|---|
| Anxiety or fear about using the mask | Systematic desensitization, counter-conditioning; Relaxation techniques; Reassurance and education on safety |
| Frustration | Motivational interviewing; Positive reinforcement |
| Low self-efficacy | Hands on training and follow-ups; Frequent check-ins |
| Lack of perceived benefit | Share objective data (oxygen and carbon dioxide levels); Education |
| Difficult incorporating into routine | Habit stacking; Use reminders/alarms |
| Limited space or inconvenient | Optimize bedroom layout; Organizational aids for tubing and mask |
It’s important to talk openly with your neuromuscular team about any challenges you or your child may be experiencing with BiPAP use, as well as any questions you may have about the machine. Your team can work with you to find individualized solutions to ensure you are getting the breathing support needed.
For additional information on BiPAP, pulmonary and emergency care, please visit PPMD’s website.
Watch the recorded webinar:
