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.
Jonathan D. Finder, M.D., Pediatric Pulmonologist
University of Pittsburgh School of Medicine
Children’s Hospital of Pittsburgh
· 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
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
· 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
· May not know until you get a cold
· Predictable with semi-annual PFT’s
· Main risk is pneumonia
· Two Kinds of Respiratory Muscles
· 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
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
· 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
· 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
Click on photo
for more information.
· Assisted cough (Cough Assist™) with colds
o ABSOLUTELY CRITICAL!
o The best way of preventing pneumonia
o Most reliable means
· 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
· Symptoms may be subtle:
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
· Uncomfortable to use continuously, so
· Not a good option for 24-hour support
· 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
· 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
· 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
· Take a deep breath every minute or two
· Tremendous improvement in
o Energy level
o Quality of life
· 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)
· 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.
You are welcome to print or email the articles on this website provided you reference "www.DoctorBach.com". For any other use, please contact Rich Clingman prior to use.