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Dr. Jonathan Finder made the following presentation at the 7th Annual Parent Project Muscular Dystrophy Conference (June 22-24, 2001). This outline is
reprinted with Dr. Finder’s permission. |
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Respiratory Care in Muscular Dystrophy
Jonathan D. Finder, M.D.,
Pediatric Pulmonologist
University of Pittsburgh
School of Medicine
Children’s Hospital of Pittsburgh
Respiratory Issues...
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Ultimately the most important issues!
· Do not have to be life shortening
o I never use the phrase “end stage”
· Predictable in their course
· Critical to anticipate
Be aware of new technologies
Four Respiratory Stages in MD
I. Initially: Normal respiratory function
II. Normal breathing, but weak cough
III. Normal breathing during daytime, but inadequate breathing asleep
IV. Inadequate breathing awake and asleep
Stage I: Normal Respiratory Function
· No special needs
· A good idea to
o Get fully immunized against influenza annually, and
o Receive Prevnar against pneumonia
· Annual screening
o Pulmonary Function Tests (PFT’s) for boys over age 6
Stage II: Weakened Cough
· May not know until you get a cold
· Predictable with semi-annual PFT’s
· Main risk is pneumonia
Cough
· Two Kinds of Respiratory Muscles
o Inspiration
o Expiration
· Inhaling (inspiration)
o Breathing-in requires muscle force, like stretching a spring
o Uses diaphragm and intercostal muscles
· Exhaling (expiration)
o Generally, breathing-out is passive, like the recoil of a spring
· Coughing (forceful expiration)
o Uses abdominal muscles to increase pressure
How Do We Cough?
1. Take a deep breath (diaphragm/intercostals)
2. Close the voice box
3. Squeeze the abdominal muscles to increase pressure inside the chest
4. Open the voice box
5. Air rushes out at a high velocity, carrying with it mucus and debris
Why MD Patients Get Pneumonia
· Inability to take a deep breath
o Weak diaphragm/intercostals
· Inability to generate good expiratory flow rate to expel mucus
o Weak abdominal muscles
· Retention of secretions
o “Lunch for bugs” (rich medium for bacterial growth)
· So a cold can lead to pneumonia
Weakened Cough: What Can YOU Do?
· A lot, it turns out!
· Manually assisted cough:
o Abdominal thrust following deep breath (or breath assisted with ambu bag or other device)
o Fair at best
· Mechanically assisted cough:
o The Emerson Cough Assist™ (In-Exsufflator, Cofflator)
o A fantastic device
o A gift to the MD community
Emerson Cough Assist™
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Click on photo
for more information. |
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The Key: PREVENTION!
· Assisted cough (Cough Assist™) with colds
o ABSOLUTELY CRITICAL!
o The best way of preventing pneumonia
o Most reliable means
· Immunizations
o Influenza
o Prevnar
· Percussion and drainage
o Only useful if you can get those secretions out!
· Nebulized medications when prescribed
· Prompt medical attention and respiratory support with colds and lower respiratory infections
Stage III: Inadequate Breathing in Sleep
· Symptoms may be subtle:
o Fatigue
o Lack of restful sleep
o Morning headache
· Easy to detect with overnight, in-home study with pulse-oximeter
· May require follow-up study in hospital
· Most common solution is BiPAP – Bi-level Positive Airway Pressure
BiPAP –
Bi-level Positive Airway Pressure
· BiPAP can support breathing in sleep
· 
Nasal
mask or face mask is used (non-invasive)
· Uncomfortable to use continuously, so
· Not a good option for 24-hour support
Stage IV: Inadequate Breathing All The Time
· This stage often occurs following a severe infection like pneumonia
· Respiratory insufficiency can be shown with PFT’s
· No longer is tracheostomy mandated at this stage
Non-invasive Breathing Support
· Portable ventilator with a mouthpiece attached (like a microphone) to wheelchair has been successful for daytime or 24-hour support
· Pulmonetic LTV 950
o Weighs only 12 lbs with 1-hour internal battery
o 3-hour and 9-hour external batteries available
Pulmonetic LTV series
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Mouth Piece Ventilators
· A standard ventilator, wheelchair-mounted
· Great for folks who
o Need some help during the day, but
o Can breathe without help some of the time
· Non-invasive
· Take a deep breath every minute or two
· Tremendous improvement in
o Energy level
o Quality of life
Invasive Support of Breathing
· Consider when a person
o Cannot breathe sufficiently, and
o Cannot use mouthpiece vent
· Tracheostomy—a big decision!
· Ventilator can be attached to wheelchair
o Goal is mobility!
o Lightweight vent is key to this
· Trache can
o Facilitate suctioning
o Can be used with Cough Assist™ (In-Exsufflator, Cofflator)
In summary:
· Key to good health is anticipating respiratory needs
· Don’t wait until a crisis occurs!
· Take an interventional approach
· Get the technology you need to stay away from the hospital!
· Identify a respiratory care professional interested in the care of MD patients
The
breathing animation is used by permission of Michael Grant White of www.breathing.com.
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Dr. Bach's Articles: ©2000-2004, John R. Bach, MD, used by permission.
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