Below are some suggestions for increasing the pulmonary health of clients with Neuro-Muscular Diseases (NMD), Post Polio Syndrome(PPS), or Spinal Cord Injury (SCI). Links are included that may assist in educating your clients.
Also see:
Diagnostic Criteria for Neuromuscular Disorders European Neuro-Muscular Centre
Prevention of Pulmonary Morbidity in Duchenne Muscular Dystrophy (Bach)
SMA Type 1 - Noninvasive Respiratory Management Approach (Bach)
Intensive Care Protocol (Bach)
Outpatient Protocol (Bach)
Respiratory Care in Muscular Dystrophy
(Jonathan Finder, MD, Pediatric Pulmonologist)
Pulmonary care issues (including assisted ventilation) do not need to be "life threatening" and should not be seen as "end stage." By understanding their predictable course and preparing to handle situations as they arise, pulmonary care issues are easily addressed.
Anticipation causes anxiety.
Preparation removes fear.
Ignorance is only bliss
Until the inevitable arrives.
Train your clients to plan for, monitor for, and minimize preventable respiratory complications related to their disease.
Also see:
Be Proactive In Your Respiratory Care
Medical Alert Card and Brochures
Respiratory Care in Muscular Dystrophy
(Jonathan Finder, MD, Pediatric Pulmonologist)
Ensure your clients know the difference between Chronic Obstructive Pulmonary Disease (COPD) and the Restrictive Lung Disease (RLD) that affects most people with MND, PPS, or SCI.
Inform them that RLD (caused by weak respiratory muscles or poor respiratory muscle control) causes shallow breathing (hypopnea). This can result in excessive CO2 being retained in the blood stream (hypercapnia).
Also see:
Restrictive Lung Disease (RLD)
Breathing Latin
Emergency Room Precautions
Obtain baseline Pulmonary Function Test (PFT) results early. In Duchenne, SMA, ALS, and other progressive diseases, pulmonary function can diminish rapidly--for those with Duchenne, beginning early in their teenage years.
At least annual PFTs should be performed until a decrease in pulmonary function is noted. PFTs should then be performed biennially or quarterly until full time ventilatory assistance is prescribed.
Also see: Pulmonary Function Tests (PFTs)
Rather than relying on invasive blood gas CO2 readings, use real-time end tidal CO2 (EtCO2) readings obtained from a capnograph. Peak EtCO2 (obtained after fully exhaling a deep breath) should not be above 43 mmHg after ___ minutes of relaxed monitoring.
A sustained hypercapnic reading (EtCO2 > 43 mmHg) indicates a need for ventilatory assistance.
Likewise, a pulse-oxymeter allows real-time monitoring of blood oxygen saturation (SpO2) rather than relying on a single historical measurement (SaO2) where the client was likely hyperventilating in anticipation of having blood drawn. This hyperventilation could cause erroneous readings for both O2 and CO2.
For an otherwise healthy and properly ventilated pulmonary system, a sustained SpO2 reading below 95% is indicative of retained secretions or aspirated particles (saliva, food, or liquids) interferring with pulmonary effectiveness.
Also see:
What's a Capnograph?
Using a Pulse-Oxymeter
Because most people with NMD, PPS, or SCI generally experience hypopnea caused by RLD rather than COPD, the long term use of supplemental O2 is contraindicated and should be avoided unless EtCO2 is constantly monitored.
Although supplemental O2 can bring SpO2 to normal, it can also mask hypercapnia and supress spontaneous breathing, further increasing hypercapnia. Sustained hypercapnia (EtCO2 > 43 mmHg) indicates the need for ventilatory assistance. Sustained hypercapnia can lead to coma and death.
Also see:
Oxygen, The Dangerous Cure-All
Severe hypercapnia after low-flow oxygen therapy...
As supine vital capacity (VCS) diminishes, the client will be highly susceptable to nighttime hypopnea resulting in CO2 retention. Symptoms include: Morning headaches, fitful sleep, increased daytime napping, lethargy, loss of appetite.
Also see:
Rather than waiting until a formal in-hospital sleep study is required, provide for the client to perform an in-home study utilizing a recording pulse-oxymeter and recording capnograph.
Blood oxygen saturation (SpO2) should not be sustained below 95% for longer than ____ seconds.
EtCO2 should not be sustained above 43 mmHg for longer than ____ seconds.
Also see: Informal Sleep Study
Prescribe and teach the client to utilize an Ambubag for air stacking combined with manually-assisted coughing to increase cough efficacy and to maintain lung elasticity.
Train the client in the use of a CoughAssist device for clearing stubborn secretions.
Also see:
Air Stacking
Assisted Coughing
For clients experiencing aspiration problems, or when supine vital capacity (VCS) falls below _____ ml, prescribe a pulse-oxymeter for in-home monitoring of SpO2. Sustained drops in SpO2 indicate a decreased breathing efficiency, commonly caused by secretion buildup or aspiration of foods or liquids.
By self-monitoring SpO2, the client can utilize assisted coughing to clear retained secretions and aspirated particles before bacterial growth forms, dramatically reducing the likelihood of pneumonia, respiratory distress, and hospitalization.
Also see: Using a Pulse-Oxymeter
When unaided peak cough flow (PCF) falls below ____ ltr/min, prescribe ready access to a CoughAssist (in-exsufflator/cof-flator) for in-home use during colds and instances of stubborn secretion retention.
It is generally adequate to have a CoughAssist available on-call with a 2-hour delivery time. Where ready access is not available, prescribe a CoughAssist for in-home use.
Also see:
Using CoughAssist
Letters of Medical Necessity
Also see: Be Ventilator-Ready
Whenever possible, utilize Noninvasive Ventilation (NIV) to supply ventilatory support. In RLD, even a client with "zero vital capacity" can be effectively ventilated using NIV.
NIV has been shown to increase quality of life and decrease respiratory-related hospitalization when compared with tracheostomy. Of those who have utilized both a trach and NIV, an overwhelming majority prefer NIV. Additional benefits of NIV: Speech is not impared (no blockage of airway by trach or cuff), reduced secretions (decreased need for secretion removal), self-administerable CoughAssist for clearing lungs rather than invasive suctioning, no stoma to become infected.
For clients who lack bulbar function, a tracheostomy may be required.
Also see:
Prevention of Pulmonary Morbidity in Duchenne Muscular Dystrophy (Bach)
SMA Type 1 - Noninvasive Respiratory Management Approach (Bach)
Intensive Care Protocol (Bach)
Outpatient Protocol (Bach)
Brochures
Two ventilators are required for wheelchair-bound clients, one for bed-side and the other chair-mounted. Medicare covers these costs for "patient mobility", but not if one is listed as a "backup."
Also see: Letters of Medical Necessity
For clients with progressive NMD or PPS--unless contraindicated by the presence of COPD--, prescribe a volume ventilator for Intermittent Positive Pressure Ventilation (IPPV--typ. 1000 ml, rate 10, command/assist mode) rather than a Bi-PAP or VPAP... never a CPAP.
Even for clients where a ventilator will be utilized for nighttime-only assistance, an IPPV will allow
Air stacking is not possible when using a pressure limited device (CPAP/Bi-PAP/VPAP). A volume limited device (volume vent) allows air stacking through the client's acceptance of repeated boluses of air.
When a Bi-PAP or VPAP is prescribed, "high span" settings (typ. IPAP 20 mlH2O, EPAP 2 mlH2O) should be used to diminish exhaling back pressure.
Also see:
Ventilator Options
Air Stacking
For nighttime use: Typically an ADAM or BREEZE circuit with nasal mask or nasal pillows.
For daytime use: Typically a gooseneck-mounted mouthpice attached to a wheelchair-mounted ventilator.
Also see: NIV Mask Options
Also see:
www.DoctorBach.com
and these books by John R. Bach, MD:
Noninvasive Mechanical Ventilation (2002)
Evaluation and Management of Neuromuscular Disease (1999)
Pulmonary Rehabilitation: The Obstructive and Paralytic Conditions (1996)
For urgent care issues, Lou Saporito (the RT who has worked with Dr. Bach for 18 years) can be reached by pager: 1-973-466-9128.
Lou Saporito, RT, can be emailed at
Perinatal/Pediatric Clinical Specialist: Brian X. Weaver, BS, RT, RPFT ()
Millennium Respiratory Services
For client appointments at the Jerry Lewis MDA Clinic, Newark, NJ:
Ms. Keisha Brady:
1-973-972-7195
()
Dr. Bach sees patients at the Jerry Lewis MDA Clinic primarily on Wednesdays and Thursdays. The MDA Clinic is located at
Jerry Lewis MDA Clinic
Doctors Office Center
90 Bergen Street, Suite 3100
Newark, NJ 07103
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Dr. Bach's office is located at
John R. Bach, MD
UMDNJ-University Hospital
Physical Medicine and Rehabilitation
150 Bergen Street, Room B-403
Newark, NJ 07103
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For Dr. Bach's direct telephone or email address, email .
Prepared by: Rich Clingman
Reviewed by:
Updated: 07/30/02
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Dr. Bach's Articles: ©2000-2004, John R. Bach, MD, used by permission.
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