Respiratory Care in Muscular Dystrophy Jonathan Finder, MD Professor of Pediatrics University of Pittsburgh School of Medicine
Respiratory complications are: Unnecessary Predictable Preventable and treatable
Respiratory specialists are important in MD care Most serious illness is respiratory New technologies & paradigms of management Specialized testing/care required
Kevin, age 14
It ain’t rocket science. 1. Support airway clearance 2. Support breathing Preferably using a non-invasive approach
The 4 stages of respiratory dysfunction in MD 1. Initially: normal respiratory function
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Birth to age 10 or so
2. Normal breathing, but weak cough
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Early teens
3. Normal breathing during daytime, but inadequate breathing asleep
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Mid teens
4. Inadequate breathing awake and asleep
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Mid-late teens/20’s
Stage 1: Normal Physical therapy directed to chest wall A good idea to get immunized against influenza annually and to receive Pneumovax once Annual screening Pulmonary function tests > age 6 Identify the respiratory specialist for your child
Stage 2: Inadequate COUGH May not know until you get a cold Easily predictable with pulmonary function testing Main risk is PNEUMONIA You will notice a lot of difficulty clearing secretions with a cold
Managing weakened cough Manually assisted cough: deep insufflation (AMBU bag) followed by an abdominal thrust or thoracic squeeze. Mechanically assisted cough: The Respironics CoughAssistTM -- A fantastic device, a gift to the MD community (courtesy of John Bach). Others – Nippy Clearway;
http://www.healthcare.philips.com/main/homehealth/respiratory http://www.nippyventilator.com/about-us/nippy-clearway/
Manually assisting cough
Abdominal thrust while stabilizing chest
In-exsufflator
The New CoughAssist E70 CoughAssist E70 Smaller, portable Smarter, patient triggered Can run off car power No knobs!
http://coughassiste70.respironics.com
Respironics CoughAssist
Pegaso Cough by Dima Italia
PREVENTION Assisted cough (CoughAssistTM) USE IT DAILY and especially with colds The best way of preventing pneumonia, most reliable means‌
Immunizations (influenza and Pneumovax) Prompt medical attention and resp. support with colds/lower resp. infections
Pulse oximetry All patients requiring assisted cough should have pulse oximeter in the home. Saturation < 95% = aggressive airway clearance (CoughAssist) DO NOT USE O2 as a substitute for cough or weak breathing muscles
Stage 3: Nightime breathing problems Symptoms may be subtle fatigue, lack of restful sleep, morning headache, nightmares, increased awakenings
Easy to detect (overnight, in-home pulse oximetry or sleep study in hospital) Most common solution is BiPAP Bilevel Positive Airway Pressure
BiPAP/VPAP BiPAPTM (also called VPAP) can support breathing in sleep Nasal mask or face mask Uncomfortable to use continuously, so not a good option for 24 hr support
Getting the interface to match
limited options in pts < 1 yr Payer limitations
Stage 4: Inadequate breathing awake This stage often occurs following a severe infection, like a pneumonia Respiratory insufficiency can be shown with PFTâ&#x20AC;&#x2122;s No longer is tracheostomy mandated at this stage Nearly all patients at this stage will require nutritional support via gastrostomy
Non-invasive breathing support
Portable ventilator with a mouthpiece attached (like a microphone) to wheelchair E.g., Pulmonetic LTV 950 = 22 lbs
Tremendous improvement in energy level and quality of life
Patrick, age 26, graduating from Pitt Law, 2004
PPMD/MDA-supported consensus statement, 2004
AJRCCM, 2004. Finder, et al.
ATS 2004 Consensus Statement ANTICIPATORY approach to care NON-INVASIVE approach to care COLLABORATIVE approach to care: Pulmonologist Nutritional support Cardiologist Orthopedist Physical, speech, and occupational therapists; psychiatry, pastoral care as needed
In summary: BE AN ADVOCATE Anticipate respiratory needs Donâ&#x20AC;&#x2122;t wait for a crisis Take a preventive approach Get the technology you need to stay healthy Identify a respiratory care professional interested in the care of MD patients