Recently, Dr. Lisa Wolf (Assistant Professor, Pulmonary Medicine and Pulmonary and Neurology at Northwestern University, Feinberg School of Medicine, Chicago, IL) gave a wonderful presentation at Duchenne Foundation Australia’s Melbourne Symposium. While her presentation was titled, “DMD Respiratory Review 2014: The Younger Years,” she was able to cover a wide range of respiratory topics that span the life of a person living with Duchenne. Some of these topics include:

When to evaluate pulmonary status

  • Waiting for symptoms is too late!
  • Pulmonary testing should begin by age 5yrs

Is FVC (Forced Vital Capacity) the best measure of pulmonary status in Duchenne? (a discussion of some of the new measures)

  • “Tension Time Index,” 
  • “Work of breathing” 

Types of NIV (non-invasive ventilation) interfaces (masks, nasal options, etc.) and devices

  • Interface should enhance sleep quality
  • Devices must have a back up rate, should have battery back up and be portable, downloadable data assists with the review of breathing during sleep, it must be serviceable in your community
  • DO not use these devices: CPAP, Spontaneous Bi-Level ventilators (“servo ventilators” – these are for patients with heart failure and not neuromuscular disease), “Flex/EPR” (no back up rate) or any device with the word “auto” in the name (these are for obstructive sleep apnea)
  • Options for NIV
    • Self-titration: Volume Assured Pressure Support (VAPS)
    • Fixed setting: spontaneous timed, timed, pressure control and ventilators with classic ventilator settings

Do’s and Don’s of NIV and Polysomnography (PSG)(sleep studies)

  • Don’t delay the start of NIV awaiting PSG
  • Use labs with neuromuscular protocols
  • Use labs with accommodations for people with disabilities
  • If FVC is <50% or if CO2 levels are elevated, go straight to NIV
  • Do PSG if:
    • Person is symptomatic, with or without symptoms
    • Pulse oxymetry is abnormal
    • Person has history of obstructive sleep apnea (OSA) or is already using DPAP

Hints for making NIV work 

  • Desensitization: try the mask and device while awake
  • Mask: make sure the mask fits well without “air leak,” especially into the eyes
  • Humidity: the patient should be able to control this themselves
  • Tubing: as tubing has become heavier, rotating it up over the headboard is helpful
  • “Rainout:” water droplets accumulating in the tubing; keeping the tubing insulated (“tubing snuggy”) keeps the tubing warm and helps to prevent rainout
  • Insomnia
  • Use all counts
  • Sound
  • Adjust settings: adjust setting while awake; trial different settings and use the setting most comfortable to the patient
  • Aerophagia (air swallowing): can be prevented by keeping the mouth closed, shortening the inhalation time, raising the head of the bed; if it is a major problem, a volume assured automatic device with a lower MAP and very close monitoring might help
  • Sinus care
  • Other sleep issues

Breath stacking and cough assist

  • Can be done with an Ambu bag (the patient exhales) or a cough assist machine (the machine exhales for the patient)
  • Should start when the cough peak flow is between 300-400
  • Evidence that breath stacking 2 times per day improves pulmonary function and may change the course of the disease
  • Seating/body positioning is very important: if the pelvic floor and mouth are in alignment, breathing is maximized

Effects of scoliosis on pulmonary status and mortality

  • Spinal curves affect breathing and cough
  • Increased mortality with scoliosis is effected by decreased pulmonary function caused by poor body positioning and scoliosis
  • Steroid use strengthens core muscles and paraspinal muscles during growth, resulting in less scoliosis

Please click here to view Dr. Wolf’s presentation.

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