Duchenne Pulmonary Care for Adults (Webinar Summary & Recording)

The lungs require the strength of many muscles to help a person to breathe and cough. As people with Duchenne grow older, the respiratory muscles, like the skeletal muscles, become weaker. Like the heart, every person with Duchenne will have pulmonary issues of some type.

The goal of pulmonary care is to allow both the prevention and management of respiratory complications. This is such an important area of care and concern that we pulled together pulmonary experts from across the US to address these important issues.

This fall, PPMD presented a three part series on the pulmonary care of patients living with Duchenne. Click here to review summaries & recordings of all three webinars.


  • Dr. Daniel Sheehan, State University of New York Buffalo, Women and Children’s Hospital of Buffalo
  • Dr. Lisa Wolfe, Northwestern University, Northwestern Memorial Hospital



1) Need for assisted cough: after loss of ambulation (12-16yo)

  • Signs of weak cough: maybe no signs, may have persistent chest congestion, throat clearing

  • Evaluation of cough: cough peak flow, MEP (mean expiratory pressure), FVC (forced vital capacity)

  • Red Flags: Peak cough flow is <270 LPM or if cough feels/seems weaker

  • Management of weak cough: assisted cough with ambu bag, mechanical cough assist

  • When to use a cough assist:

    • If you are able to walk and have a strong cough when you are well, then you are less likely to need a cough assist unless you are ill.

    • You should begin using a cough assist machine when your cough peak flow (a measurement of how hard you can cough) is <270 Lpm (1).

    • If you are no longer able to walk on your own, you should use the cough assist machine daily to keep your lungs clear, chest muscles flexible, and use it more frequently during a respiratory illness/cold.

    • Read “how to use a cough assist during a respiratory illness"

  • Continued evaluation of cough: once - twice yearly cough peak flow until you are prescribed a mechanical cough assist, episodes of upper respiratory infections/pneumonias

2) Need for Nocturnal (night time) assisted ventilation (BiPAP): generally mid-late teens

  • Signs that nocturnal assisted ventilation may be needed: restless sleep, frequent repositioning, nightmares, snoring, sweating, am headaches, need for daytime naps, mood changes, school performance changes in older (high school, college) students

  • Evaluation of nighttime oxygenation: FVC, PETCO2 (end tidal carbon dioxide level), sleep evaluation or home overnight (oxygen only or oxygen + CO2 evaluation)

  • Red Flags: FVC <30-50% predicted, MIP (mean inspiratory pressure) <-60cm H2O, daytime PaCO2 >45 mmHg, daytime SpO2 (oxygen saturation) <95%

  • Management: “high span BiPAP:” BiPAP assists breathing using 2 pressures: an inspiratory pressure (breathing in, higher pressure) and an expiratory pressure (breathing out; lower pressure that keeps airways open between breaths); a “back up rate” (which starts a breath if breaths are not occurring frequent enough) is important; there are many interfaces (masks, nasal pillows, etc.) – you many need to try 2-3 different interfaces to find one that works, so don’t give up!

  • Continued evaluation of nighttime breathing: sleep studies may be needed to adjust the BiPAP settings

3) Daytime assisted ventilation needed late teens, early 20’s

  • Signs that daytime assisted ventilation may be needed: short of breath, anxious, soft voice, needing to breathe during even short sentences.

  • Evaluation of daytime oxygenation: awake PETCO2 or O2 saturation

  • Red Flags: FVC <680 ml (15-20% predicted), awake PETCO2 or PaCO2 >45 mmHg despite nighttime assisted ventilation

  • Management: Non-invasive ventilation (NIV, “sip vent”): many types, many mouthpieces Invasive ventilation (tracheostomy): consider if this is the patient’s preference, cannot successfully use NIV, local medical infrastructure does not support NIV, 3 failed attempts to extubate to NIV (despite optimal use of cough assist), failure of NIV to prevent aspiration of secretions; recent data (MD STARnet preliminary data) supports that more patients receive invasive ventilation (tracheostomy) during an acute respiratory illness (maybe optimal use of NIV and extubation protocols could delay or prevent invasive ventilation), and most of those patients used a cough assist or NIV before invasive ventilation (maybe there is a weaker population of patients who need invasive ventilation, or this may fail to provide adequate ventilation over time).

    • Eg. Extubation Protocol for Neuromuscular Patients:

Disaster Preparedness

  • Have a back up generator

  • Have back up batteries and know how long your equipment will run on the batteries

  • NIV and/or mechanical cough assist might be needed on transport to the hospital

  • Know your EMS; request an EMS van with electrical outlets; educate them about your disease

  • If you have an older cough assist and ventilator, they require an inverter for car/van use

Going to the Hospital or ED, ALWAYS:

  • Bring your own equipment

  • Bring the PPMD emergency card 

  • Bring your healthcare proxy

  • Bring your 1 page health history

  • Call your neuromuscular, pulmonary and cardiology providers and respiratory therapist

Issues with Swallowing

  • Usually increases during young adult/adulthood; can be due to
    • Fatigue with eating
      • Lungs that are not fully expanded make it harder to swallow; “breath stacking” or practice fully expanding the lungs helps; best to do just before eating so that lungs are fully expanded prior to eating
    • Aspiration with eating
      • Can be evaluated by: FEES (fiberoptic endoscopic evaluation of swallowing - tiny) or VFS (Videofluroscopic swallowing study)
  • Evaluation should be done if:
    • Adult (late non-ambulatory); earlier if indicated
    • 1 episode of chest infection plus: prolonged chewing, difficult swallowing
  • If patient has had a scary choking episode at home (choking, gagging, etc.), skip evaluation and go straight to therapy.
  • Swallowing/speech therapy interventions:
    • Change position of head/neck
    • Change consistency of the food (avoid: thin liquids, dry/crumbly foods, raw food)
    • Eat multiple small meals, rather than three larger meals
    • Consider: straws, cups, smaller spoons, etc.
  • If nothing helps and the patient is malnourished, consider a G-tube

Feeding Tubes

  • Goals:
    • Major advantage: **Reduce aspiration/chest infections
    • Increase quantity of nutrition (address weight loss)
    • Increase quality of nutrition (proteins for muscle, fat for respiration/energy (decreased Co2 with fat metabolism)
    • Improve hydration (thin secretions, reduce constipation)
  • Types of feeding tubes:
    • Placement options: using ultrasound imaging or using a tiny camera that is placed endoscopically (down the esophagus) that can monitor the placement of the tube)
    • G-tubes (gastric tubes –inserted into the stomach) or GJ tubes (button in stomach with a tube that runs into the intestine; may help with reflux/heartburn)
    • Feeding tube buttons: Mini (smaller), Mic-Key (larger), can have a long tube or be right against the abdomen 

Managing Nasal, Oral, and Chest Secretions

  • Rule: if it’s too wet, dry it; if it’s too dry, wet it!
  • If secretions are thick (dry): humidify, anti-histamines are ok (do NOT use decongestants, or medicines that include a “D” in the title (example: Claritin-D); use “little sucker” or cough assist to help clear (take breath in with cough assist through the mouth – breath out through the nose)
  • Oral hygiene is very important to prevent bacteria in the mouth that can cause chest infections; chest infections can effect heart valves and devices; consider using: sage kits, chlorhexadin toothpaste, floss (air floss); consider “disability dentist” especially if wisdom teeth are an issue
  • Chest secretions may be helped by:
    • Improved seating/posture (improved cough)
    • Scoliosis repair (Improved respiration and cough)
    • Abdominal binders (may help with cough)
    • Constipation management (constipation can distend the belly, making it hard to breathe an/or cough)
    • Reflux management (makes breathing harder)
    • Preventing “aerophagia” (swallowing air)

Managing Scoliosis

  • Scoliosis should be managed irrespective of skeletal maturation
  • Spine films:
    • Measure the “Cobb angle” – amount of curve in the spine
    • Yearly if curve is <15-20 degrees
    • Every 6 month if curve is >20 degrees
  • Consider surgery if:
    • Progression continues
    • Evidence of vertebral fractures
    • Pain
  • “30-30 Rule”
    • If the Cobb angle is >30 degrees and the FVC is >30% of normal, then repair the scoliosis
    • New data: use of NIV and airway clearance has made scoliosis repair possible with FVC’s down to 20%
    • Reason to repair scoliosis: longer life
  • Steroids:
    • Prolong ambulation
    • Greatly reduced the need for scoliosis repair

Transitioning to Adult Care

  • Things to consider:
    • When should planning start?
    • What are your goals?
    • Who should be on your medical team?
    • Who should be on your transition team?
    • What are your vocational choices?
    • What are your living options?
    • Home environment: living independently and managing airway clearance/NIV
    • Education: university vs Vocational Rehab
    • Exercise: direct ROM from personal assistant
    • Activities: outside the home, getting out of the house
    • Affect: address fears of dependence, anxiety, depression
    • Ambitions: start to talk about goals for the future as young as possible
    • Dental care: oral care to reduce risk of pneumonia, wisdom teeth
    • Diet: weight, calcium/vitamin D, fluids
    • Drugs, cigarettes, alcohol: peer pressure, taking responsibility for your medicines
    • Sexuality: role models (the Empowered Fe Fes, Pathfinders)
    • Sleep: differences between adolescent/young adult sleep patterns and adult/older adult sleep patterns; conflicts with care providers and parents
  • Learn to advocate for yourself
    • Deal with your DME company: you must call them (they will not call you!)
    • Learn your equipment and settings: know how to download settings/information from your device; make sure you are meeting your therapy goals (how large should your breath be? how many hours should you use bipap? does your mask fit?)
    • Learn to work with a care giver to better assure respiratory care at home
    • Learn to hire your own assistants: get hours through adult agencies; set schedules; learn how best to communicate your needs


  1. As a physical therapist, what exercises for pulmonary care would you suggest?
    Range of Motion (ROM) exercises; use of an ambu bag or cough assist to help maintain chest excursion (the ability to easily expand); Mary Massery’s book is recommended for all PT’s; exercises to help “open the chest” and help with stooped posture (stretching of the chest muscles, help to maintain strength of the muscles of the upper back)

  2. Are there abdominal binders available for those with G-tubes?
    Yes: there are abdominal binders with holes; they are generally made for those with an ostomy, but they work well for feeding tubes.

Other Webinars in This Series

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