Classics of Paradigm Paralysis• “There is no reason for any individual to have a computer in their home." --- Kenneth Olsen, 1977 President and

founder of Digital Equipment Corp.• "Airplanes are interesting toys but of no military value." --- 1911, J. Marshal Ferdinand Foch, French Military

Strategist and future World War I commander• "Man will never reach the moon regardless of all future scientific advances." --- February 25, 1967, Dr. Lee de Forest,

inventor of the Audio Tube (Television), and Father of Radio.• "[Television] won't be able to hold on to any market it captures after the first six months. People will soon get tired of

staring at a plywood box every night." --- 1946, Darryl F. Zanuck, head of 20 th Century-Fox.• "We don't like their sound. Groups of guitars are on the way out.”---1962, Decca Records rejecting the Beatles.• "For the majority of people, the use of tobacco has a beneficial effect." --- November 18, 1969, Dr. Ian G. MacDonald,

Los Angeles surgeon, as quoted in Newsweek.• "This 'telephone' has too many shortcomings to be seriously considered as a means of communication. The device is

inherently of no value to us." --- 1876, Western Union Internal memo.• "The earth is the center of the universe." --- Popes of the Roman Catholic Church• "Nothing of importance happened today." --- July 4, 1776, written by King George III of England• “Everything that can be invented has been invented." --- 1899, Charles H. Duell, U.S. Commissioner of patents.

• To this can now be added:• “Daytime noninvasive ventilation via a mouthpiece should not be viewed as an alternative to tracheostomy” • “Determining the best date for tracheostomy in patients with DMD remains a challenge.”---2006, J.-C. Raphael, Paris,

as written in the European Respiratory Journal, volume 28, pages 468-469.

“…some of our physical therapists, in struggling with (iron lung) patients, noticed that they could simply take the positive pressure attachment, apply a small plastic mouthpiece..., and allow that to hang in the patient's mouth….We even had one patient who has no breathing ability who has fallen asleep and been adequately ventilated by this procedure, so that it appears to work very well, and I think does away with a lot of complications of difficulty of using (invasive) positive pressure. You just hang it by the patients and they grip it with their lips, when they want it, and when they don't want it, they let go of it. It is just too simple….."

Post-polio syndrome?

• Vent use from acute polio

• Acute to late onset

• Late onset only

• Conclusions: Stable patients receiving prolonged mechanical ventilation (PMV) without clinical pneumonia have a high alveolar burden of bacteria. The bacterial burden in most patients exceeds the commonly accepted threshold for diagnosing ventilator associated pneumonia. The utility of quantitative bronchoscopic culture in the diagnosis of ventilator associated pneumonia in this patient population requires further study.

• Baram D, Hulse G, Palmer LB. Stable patients receiving prolonged mechanical ventilation (PMV) have a high alveolar burden of bacteria. Chest 2005;127:1353-57.

Mechanical Ventilation via a Tubedecreases diaphragm contractile properties

Le Bourdelles et al. Am J Respir Crit 1994;149.

Survival with Tracheostomy Tubes

• 100 ALS patients, 40 died in 5 years, about 32/40 due to the tube and the others died suddenly

• Bach JR. Amyotrophic lateral sclerosis: communication status and survival with ventilatory support. Am J Phys Med Rehabil 1993;72:343-349.

Treatment Goals

•Optimize chest wall/lung ROM and growth

•Optimize cough flows

•Maintain normal ventilation

Optimize Cough Flows and Airway Secretion Elimination

BRAVO!

November 22, 1988 In-ExsufflatorBach JR, Zaniewski R, Lee H. Cardiac arrhythmias from a malpositioned Greenfield filter in a traumatic tetraplegic. Am J Phys Med Rehabil 1990; 69(5):251-253.

• Department of Health and Human Services, Food and Drug Administration, Orphan Products Development Grant #FD-R-000649-01. Bach JR. Mechanical exsufflation for ventilatory support without tracheostomy, $51,222, 9/30/90-9/29/91.

Mechanical Assisted Cough “A historical perspective”

…Since 1954 to 2010

The cof-flator

The “Paint Can”Insufflator-Exsufflator

The In-Exsufflator

The Cough Assist

Maintain normal alveolar ventilationaround the clock

IDEAL INTERFACE

• LEAK FREE

• COMFORTABLE

• MAINTENANCE FREE

Outpatient Protocol

• Maintain Spo2 > 94% at all times, especially during colds

• How? By using Mechanically assisted coughing (MAC) and noninvasive ventilation

• If Spo2 < 95%, you have either hypoventilation, mucus, or pneumonia

Should all patients who fail SBT, fail extubation..???

“…We conclude that the ability to generate CPF of at least 160L/min is necessary for the successful extubation or tracheostomy tube decannulation of patients with neuromuscular disease irrespective of ability to breathe…”

Extubation After Weaning Failure

• 157 intubated patients failed SBTs both before and after extubation to full-setting NIV/MAC

• Before hospitalization 98 (61%) had no experience with NIV, 39 (26%) used it nocturnally, and 20 (15%) were continuously NIV dependent.

• First attempt protocol extubation success 96% (147 patients). • All 98 extubation attempts on patients with assisted CPF ≥ 160 L/m

were successful. • Continuous NIV dependence and duration of NIV dependence prior to

intubation correlated with extubation success (p<0.005). • Six of 7 patients who initially failed extubation succeeded on

subsequent attempts, so 1 underwent tracheotomy despite continuous post-extubation ventilator dependence.

• Bach JR, Gonçalves MR, Hamdani I, Winck JC. Extubation of unweanable patients with neuromuscular weakness: a new management paradigm. Chest (in press), EPub PMID 20040608,

• http://chestjournal.chestpubs.org/content/early/2009/12/24/chest.09-2144.

Decanulation of Unweanable Patients

• Bach JR, Alba AS. Noninvasive options for ventilatory support of the traumatic high level quadriplegic. Chest 1990;98:613-619.

• Bach JR. New approaches in the rehabilitation of the traumatic high level quadriplegic. Am J Phys Med Rehabil 1991;70:13-20.

“… It was concluded that, in general because of their youth, intact mental status and bulbar musculature, and abscence of obstructive lung disease, patients with traumatic high level spinal cord injury are candidates to benefit from continous noninvasive ventilation and assited coughing techniques even with no ventilatory autonomy…”

NIV Support Consensus, April 2010

22 Centers in 18 countries760 continuous NIV dependent patients with DMD, ALS, SMA type 1

for 3000 patient-years

• Duchenne muscular dystrophy• Age Duration Age Duration Age Still Ext Decan Deaths Tt• # N ptNIV ptNIV N ftNIV ftNIV Current Alive • 1. 120 20.3±2.8 2.0±2.1 101 22.3±5.9 7.0±5.9 30.3±6.1 63/128 29 9 14/63 0*• 2. 10 22.7±3.2 1.7±1.9 10 24.3±4.1 4.6±1.6 28.4±4.6• 3. 24 18.0±1.6 9.9±4.5 24 28.1±4.6 4.2±2.8 32.3±2.8 24/24 0 0 0 0• 4. 9 18.7+5.2 4.6+2.2 4 23.3+6.5 4.0+2.8 27.0+5.0 4/4 0 0 0 1• 5. 42 21.9 4.1±2.5 42 26 5 31 32/42 0 0 3/10 1• 6. 6 22.0+2.8 3.01.1 6 25.0+2.4 4.93.2 29.9+3.6 4/6 0 0 1/2 0 • 7. 25 18.3+4.0 3.52.4 11 21.92.4 5.04.4 26.94.3 8/11 2 1 1/3 0

• ALS• Age Duration Age Duration Age Still Ext Decan Deaths Tt• # N ptNIV ptNIV N ftNIV ftNIV Current Alive • 1. 176 52.5±5.6 0.9±1.1 109 53.3±5.3 0.8±2.2 54.6±5.7 67/109 15 6 42 44*• 2. 4 67.8±6.9 1.3±0.8 4 69.3±6.6 2.0±1.9 70.5±8.4 • 4. 78 59.5+9.4 0.9+1.1 27 60.3+1.3 0.6+0.5 62.2+9.1 14/27 0 0 5/14 6• 6 1 0/1 0 • 7. 83 56.1+9.0 0.9+0.9 19 55.5+9.0 1.1+2.1 6/19 0 0 7 6

Indications for Tracheostomy

When the Spo2 decreases below 95%

and can not be normalized by NIV or MAC

DOCTORBACH.COM

Duchenne muscular dystrophy

• 120 patients used NIV, MAC/oximetry for 10.56.1 years• 103 nocturnal-only NIV users extended to continuous NIV

for 7.06.1 years to age 30.36.1 • 56 patients still alive. • 28 of the 103 were not hospitalized• 8 tracheostomy continuous users were decanulated to NIV• 29 unweanable intubated patients were extubated to

NIV/MAC. • Of 57 deaths, 26 (46%) were probably cardiac, 14 (34%)

probably respiratory, and 17 (30%) of other etiology with 8 patients dying from CHF before vent use.

• 8 of 12 deaths of TIV users were tube related.

Spinal Muscular Atrophy

• 72 SMA-1 patients using NIV are alive at mean age 86.1 (range 13 to 196) months; 13 died at 52.3 (range 13 to 111) months.

• 67 of the 75 could speak.

• The NIV patients had significantly more hospitalizations than tracheostomy patients until age 3 (p<0.001) but not thereafter.

CONVENTIONAL PROTOCOL

1. Oxygen administrated arbitrarily in concentrations that maintain SpO2 well above 95%.

2. Frequent airway suctioning via the tube.

3. Supplemental oxygen increased when desaturations occur.

4. Ventilator weaning attempted at the expense of hypercapnia.

5. Extubation not attempted unless the patient appears to be ventilator weaned.

6. Extubation to CPAP or low span bi-level positive airway pressure and continued oxygen therapy.

7. Deep airway suctioning by catheterizing the upper airway along with postural drainage and chest physical therapy.

8. With increasing CO2 retention or hypoxia supplemental oxygen is increased and ultimately the patient is reintubated.

9. Following re-intubation tracheostomy is thought to be the only long-term option ...or following successful extubation bronchodilators and ongoing routine chest physical therapy are used.

10. Eventually discharged home with a tracheostomy, often following a rehabilitation stay for family training.

PROTOCOL – Dr. Bach1. Oxygen administration limited only to approach 95% SpO2.

2. Mechanical insufflation-exsufflation used via the tube at 25 to 40 cm H2O to -25 to -40 cm H2O pressures up to every 10 minutes as needed to reverse oxyhemoglobin desaturations due to airway mucus accumulation and when there is auscultatory evidence of secretion accumulation. Abdominal thrusts are applied during exsufflation. Tube and upper airway are suctioned following use of expiratory aids as needed.

3. Expiratory aids used when desaturations occur.

4. Ventilator weaning attempted without permitting hypercapnia.

5. Extubation attempted whether or not the patient is ventilator weaned when meeting the following:

A. Afebrile

B. No supplemental oxygen requirement to maintain SpO2 >94%

C. Chest radiograph abnormalities cleared or clearing

D. Any respiratory depressants discontinued

E. Airway suctioning required less than 1-2x/eight hours

F. Coryza diminished sufficiently so that suctioning of the nasal orifices is required less than once every 6 hours (important to facilitate use of nasal prongs/mask for post-extubation nasal ventilation)

6. Extubation to continuous nasal ventilation and no supplemental oxygen.

7. Oximetry feedback used to guide the use of expiratory aids, postural drainage, and chest physical therapy to reverse any desaturations due to airway mucus accumulation.

8. With CO2 retention or ventilator synchronization difficulties nasal interface leaks were eliminated, pressure support and ventilator rate increased or the patient switched from BiPAP-ST™ to using a volume cycled ventilator. Persistent oxyhemoglobin desaturation despite eucapnia and aggressive use of expiratory aids indicated impending respiratory distress and need to re-intubate.

9. Following re-intubation the protocol was used for a second trial of extubation to nasal ventilation ...or following successful extubation bronchodilators and chest physical therapy were discontinued and the patient weaned to nocturnal nasal ventilation.

10. Discharge home after the SpO2 remained within normal limits for 2 days and when assisted coughing was needed less than 4 times per day.