Prevention of Pulmonary Morbidity

“No DMD person should ever require a trache tube or develop any respiratory complications.  If people follow closely what we describe here, respiratory difficulties can be eliminated.”

Dr. John R. Bach

 

Summary by Rich Clingman:

It has been estimated that 55% to 90% of DMD patients die from respiratory failure between 16.2 and 19 years old. The use of the Protocol described below has been shown to increase the DMD patient’s quality of life, decrease respiratory-related hospitalization from 21 days/year to less than 2 days/year, and prolong life by at least 4½ years.

A “Pulse-Oximeter” measures heart rate and blood oxygen level (SaO2, oxyhemoglobin saturation) by placing a sensor on the patient’s finger. An SaO2 reading below 95% indicates either hypoventilation (under ventilation) or the presence of congestion that can lead to lung infections.

The use of supplemental O2 (oxygen) should never be prescribed to the DMD patient to treat low SaO2 levels without testing to determine if the patient suffers from hypoventilation. End-tidal CO2 (carbon dioxide) levels or blood gas CO2 should be checked to determine if the patient is retaining CO2. Improper use of supplemental oxygen can mask hypoventilation, decrease breathing rate, and cause breathing to stop!

Monitoring the SaO2 level while having the DMD patient take several deep breaths can often act as a quick check for hypoventilation. If the SaO2 level rises above 95%, hypoventilation due to weak chest muscles is a likely cause. (Bach JR, Quest 1995; 2:1)

Through the proper use of in-home SaO2 monitoring and assisted coughing, serious problems and hospitalizations can often be avoided. An in-home pulse-oximeter should be prescribed well before respiratory problems are discovered during hospitalization.

Once part-time (usually nighttime) breathing assistance is required, non-invasive intermittent positive pressure ventilation (NIV/IPPV) should be utilized. The use of a Bi-PAP (Bi-Level Positive Airway Pressure) device is not recommended by Dr. Bach since a Bi-PAP cannot be used for “air stacking”. A high-quality portable volume ventilator should be prescribed since full-time use of a volume ventilator will almost certainly be required eventually. Though a volume ventilator is more expensive, reduced health care costs obtained by following the Protocol described below justifies its early purchase.

 

In-Home Respiratory Equipment Needs:

When fatigued, short of breath, or ill:

Pulse-oximeter (as low as $400) [specs] [supplier]

When unassisted Peak Cough Flow (PCF) < 270L/min:

“Air stacking” and manual-assisted coughing training and aids.

Emerson Cough Assist (AKA In-Exsufflator or Coffilator) [specs]

When hypoventilation diagnosed (baseline SaO2 < 95%):

Portable IPPV Volume Ventilator such as

Pulmonetic LTV 950 (12.4 lbs) (about $10,000) [specs]

Respironics PLV 100 (28.2 lbs) (as low as $9,749) [specs] [supplier]

Properly fitting non-invasive ventilation (NIV) nasal or mouth mask.

 

The following is reprinted with the express permission of its corresponding author, Dr. John R. Bach.

 

Click to view PDF version of original Chest article

Contents

Table 1 - Hospitalization Rate Comparisons for Non‑Protocol Pre‑Ventilator Use High Risk Patients Vs. Protocol Part‑Time Noninvasive IPPV Usersa 2

Out‑Patient Protocol 2

Prevention of Pulmonary Morbidity for Patients with Duchenne Muscular Dystrophy. 2

Corresponding Author 2

Abstract 3

Study Objective. 3

Design. 3

Methods. 3

Results. 3

Conclusion. 3

Key words. 3

Abbreviations. 3

Introduction. 3

Patients and Methods. 3

Results. 4

Discussion. 5

References. 6

 

Table 1 - Hospitalization Rate Comparisons for
Non‑Protocol Pre‑Ventilator Use High Risk Patients
Vs. Protocol Part‑Time Noninvasive IPPV Usersa

 

Entire Population

Non‑Protocol
Protocol
P

Patients

17

24

 

Hospitalizations/patient

2.411.84

0.51.0

<0.005

Hospitalizations/year/patient

2.254.75

0.20.5

<0.005

Hospitalizations avoided/patient

 

1.81.7

 

Hospitalizations avoided/year/patient

 

0.81.0

 

Hospitalizations days/patient

35.466.3

3.68.7

<0.005

Hospitalizations days/year/patient

21.437.8

1.85.2

<0.005

Years

3.62.7

3.13.2

0.21

a—This does not include the hospitalizations for introduction of definitive ventilator use

Out‑Patient Protocol

Patients are considered to be at risk for respiratory failure particularly during chest colds when they have assisted PCF below 270 liters per minute.  They are prescribed oximeters and trained in air stacking insufflated volumes via mouth and nasal interfaces, manually assisted coughing, MI‑E at +35 to +50 to ‑35 to ‑50 cm H2O pressure drops with abdominal thrusts ap­plied during exsufflations, and given rapid (less than 1 hour) access to a portable volume ventilator, a Cough Assist (J. H. Emerson Co., Cambridge, MA), and to various mouth pieces and nasal interfaces.

The patients and care providers are instructed that any decreases in SaO2 below 95% indicate either hypoventilation or the presence of airway mucus accumulation that must be cleared to prevent atelectasis and pneumonia.  They are told to use oxyhemoglobin saturation (SaO2) monitoring whenever fatigued, short of breath, or ill.  They use noninvasive IPPV and manually and mechanically assisted coughing as needed to maintain normal SaO2 at all times.

Patients with elevated end‑tidal blood carbon dioxide levels or periods of daytime SaO2 below 95% undergo nocturnal SaO2 monitoring.  When symptomatic or nocturnal means are below 94% a trial of nocturnal nasal IPPV is provided.  People continue to use nocturnal nasal IPPV when they felt less fatigue and noctur­nal mean SaO2 increases.  Most young patients use noninvasive IPPV for the first time to assist lung ventilation during chest infections.

Prevention of Pulmonary Morbidity for Patients with Duchenne Muscular Dystrophy

John R. Bach, M.D., F.C.C.P., F.A.A.P.M.R., Professor of Physical Medicine and Rehabilitation, University of Medicine and Dentistry of New Jersey (UMDNJ)‑New Jersey Medical School, Newark, N.J., Co‑Director of the Jerry Lewis Muscular Dystrophy Association Clinic, UMDNJ‑New Jersey Medical School, Newark, N.J.; Director of the Center for Ventilatory Management Alterna­tives, University Hospital, Newark, N.J. and Kessler Institute for Rehabilitation, West Orange, N.J.

Yuka Ishikawa, M.D., Department of Pediatrics, National Yakumo Hospital, Yakumo‑cho Yamakoshi‑gun, Hokkaido, Japan and Department of Pediatrics, Sapporo University of Medicine, Hok­kaido, Japan.

Heakyung Kim, M.D., Clinical Professor of Physical Medicine and Rehabilitation, Yonsei University College of Medicine, Seoul, Korea and the Department of Physical Medicine and Rehabilitation, UMDNJ‑The New Jersey Medical School

Corresponding Author

John R. Bach, M.D.

Department of Physical Medicine and Rehabilitation

University Hospital B‑403

UMDNJ‑The New Jersey Medical School

150 Bergen Street

Newark, N.J.   07103

Phone: 1‑201‑982 4393

Fax: 1‑201‑982 5725

Abstract

Study Objective

To evaluate the effects of a new respiratory management protocol on respiratory morbidity and hospitalization rates for patients with Duchenne muscular dystrophy (DMD).

Design

A retrospective cohort study.

Methods

Using a protocol in which oxyhemoglobin desaturation was prevented or reversed by the use of noninvasive intermittent positive pressure ventilation (IPPV) and assisted coughing as needed, the hospitalization rates and days for 24 protocol DMD ventilator users were compared with those of 22 non‑protocol DMD tracheostomy IPPV users.

Results

The 22 conventionally managed patients were hospitalized a mean of 72.2112 days when undergoing tracheostomy.  This in­cluded a 16.15.4 day period of translaryngeal intubation.  The 24 protocol patients were hospitalized a mean of 6.02.4 days (p<0.005) when beginning ventilator use.  Over their next 126.2 patient‑years of ventilator use, the 24 protocol patients had significantly lower rates of hospitalization (p<0.008) and hospitalization days (p<0.005) than had the tracheostomy IPPV users over a 167.2 patient‑year period.  This is true although 14 of the 24 protocol patients went on to require 24 hour nonin­vasive IPPV for 4.53.6 years.  Five of the 14 have yet to be hospitalized.

Conclusion

The use of inspiratory and expiratory aids can prolong survival while significantly decreasing the pulmonary morbidity and hospitalization rates associated with conventional resort to tracheostomy IPPV.

Key words

 Cough; Duchenne; Exsufflation; Mechanical ventila­tion; Muscular dystrophy; Respiratory failure; Respiratory paralysis; Respiratory therapy.

Abbreviations

DMD=Duchenne muscular dystrophy

EtcO2=end‑tidal carbon dioxide tension

IPPV=intermittent positive pressure ven­tilation

SaO2=oxyhemoglobin saturation

PCF=peak cough flows

URTIs=upper respiratory tract infections

VC=vital capacity

Introduction

It has been estimated that 55%1,2 to 90%3‑5 of DMD patients die from respiratory failure between 16.2 and 19 years of age and uncommonly after age 25.4,6  For patients with DMD as well as for patients with other progressive neuromuscular diseases, acute respiratory failure most often occurs during otherwise benign up­per respiratory tract infections (URTIs).7  During these episodes, already severe pulmonary dysfunction is further com­promised by bronchial mucus plugging and by further weakening and fatigue of inspiratory and expiratory muscles.8  Such episodes can result in repeated pneumonias, hospitalizations, tracheal in­tubations, and ultimately, in tracheostomy or death.

Up to 24 hour use of noninvasive IPPV has prolonged the sur­vival of over 700 patients with neuromuscular ventilatory failure.9  For noninvasive IPPV to be effective long‑term, however, the ability to clear the airway of secretions is criti­cal, especially during intercurrent URTIs.  We have found that at least 160 L/m of peak cough flows (PCF) is the minimum required to clear airway debris10,11 and that this appears to be possible for the great majority of patients with DMD.  Also, in our ex­perience, patients for whom at least 270 L/m of PCF can be gener­ated have little risk of developing respiratory failure during URTIs.  Further, since both hypercapnia and bronchial mucus plug­ging cause decreases in oxyhemoglobin saturation (SaO2) below 95%, we hypothesized that if we could identify DMD patients at risk of developing acute respiratory failure on the basis of low PCF and train and equip them to maintain sufficient alveolar ven­tilation and airway mucus elimination to prevent oxyhemoglobin desaturation, we could prevent pulmonary morbidity and acute respiratory failure.

Patients and Methods

Of all patients referred to a regional Jerry Lewis Muscular Dystrophy Association clinic since 1977, 92 were diagnosed with DMD on the basis of onset and progression of weakness before age 5, calf muscle pseudohypertrophy, loss of unassisted ambulation before age 13,1 and characteristic elevations of serum creatinine kinase, muscle biopsy in the patient or male relative, and electrodiagnostic examination.  More recently, documentation of a Duchenne‑type mutation or identical haplotype involving closely linked markers in other family members has been established to eliminate the need for a biopsy or electrodiagnostic examination to confirm the diagnosis.12  In addition, all 92 patients were seen in our clinic after 11 years of age.  No patients had sub­stance abuse problems or chronic lung disease. 

Of these 92 patients, five were lost to follow‑up before 26 years of age without having experienced acute respiratory dis­tress, and 39 patients were between 11 and 26 years of age but, although four were using nocturnal nasal IPPV to reverse symptomatic chronic alveolar hypoventilation and seven patients in all had PCF below 270 L/m putting them at high risk for respiratory failure, none had as yet had any episodes of acute respiratory distress.  These patients were no longer considered in our data analysis.

Prior to 1983 no respiratory muscle aids were used to prevent respiratory failure.  Since 1983, patients were routinely screened every 3 to 6 months for symptoms of chronic alveolar hypoventilation13 and the following were measured: vital capacity (VC), maximum insufflation capacity (maximum volume of air stacked breaths) (Collins Survey Spirometer, Collins, Inc., Braintree, MA),11 assisted and unassisted PCF (Access Peak Flow Meter, HealthScan, Inc., Cedar Grove, N.J.), and SaO2 (Ohmeda Model #3760, Louisville, CO).  An oral‑nasal interface or lip seal (Nelcor‑Puritan‑Bennett, Boulder, CO) was used for spirometry when lip muscles were too weak to grab a mouth piece.  For as­sisted PCF measurements, the patients were insufflated to their maximum insufflation capacities then the expiratory muscles were assisted by coordinating an abdominal thrust to glottic opening.14  In 1986, end‑tidal carbon dioxide (EtcO2) monitoring (Microspan 8090 Capnograph, Biochem International, Waukesha, WI) was added to the screening. 

Patients with symptoms of alveolar hypoventilation, VCs below 600 ml, elevated EtcO2, or periods of daytime SaO2 below 95% underwent nocturnal SaO2 monitoring.  Symptomatic patients and those with nocturnal SaO2 means below 94% underwent trials of nocturnal nasal IPPV using a portable volume ventilator (PLV‑100, Respironics Inc., Murrysville, PA).  The patients were encouraged to use nocturnal nasal IPPV nightly when they had symptomatic relief or when nocturnal mean SaO2 was demonstrated to have in­creased.  With time, more than nocturnal use became necessary.  Seven to 16 hours per day was considered part‑time, and greater than 16 hours per day was considered full‑time use.  Most patients used noninvasive IPPV for the first time, however, during an URTI.

Patients were considered to be at risk for URTI‑associated respiratory failure when they had maximum assisted PCF below 270 L/m.  Since their VCs were below 1000 ml at this point, they were trained in air stacking manual resuscitator delivered volumes to their maximum insufflation capacities.  They were also prescribed oximeters and trained in manually assisted coughing and in mechanical insufflation‑exsufflation (mechanically assisted coughing).14  If not already using noninvasive IPPV, they were provided with rapid access (less that 2 hours) to a portable volume ventilator, to various mouth pieces15 and nasal inter­faces, and to a mechanical insufflator‑exsufflator (In‑Exsufflator, J. H. Emerson Co., Cambridge, MA).13  The patients and care providers were instructed to monitor SaO2 whenever fatigued, short of breath, or ill.  They were instructed that any decreases in SaO2 below 95% indicate either hypoventilation or bronchial mucus plugging and that these must be corrected to prevent atelectasis, pneumonia, and respiratory failure.  Thus, the protocol consisted of using noninvasive IPPV and manually and mechanically assisted coughing as needed to maintain normal SaO2, particularly during intercurrent URTIs.  No supplemental oxygen was provided for any patient in the community.  No patients who were regularly evaluated failed to be properly trained and equipped or refused the protocol. 

Averted hospitalizations were defined as acute episodes of respiratory distress relieved by 24 hour ventilator use along with the use of assisted coughing and mechanical insufflation‑ exsufflation to expel mucus and to immediately reverse oxyhemoglobin desaturation‑associated mucus plugging.  When baseline SaO2 decreased below 92% or dyspnea persisted despite continuous ventilator use and aggressive assisted coughing, or when clinical dehydration was suspected, high fevers persisted, or lethargy occurred, the patients were instructed to present for evaluation and possible hospitalization.

High risk pre‑ventilator use periods were defined by the period of time following an initial episode of acute respiratory failure or averted hospitalization until the onset of daily definitive ventilator use.  These periods were compared for hospitalization rates and days for the protocol and non‑protocol patients.  Patients requiring daily ventilator use from the first episode of respiratory failure or averted hospitalization were not considered to have had high risk pre‑ventilator use periods.  Likewise, hospitalization rates and days were compared for non‑protocol tracheostomy, and protocol noninvasive IPPV users.  The Mann Whitney nonparametric T‑test was used to compare hospitalization rates and days for the protocol and non‑protocol groups.  P <0.05 was considered to represent statistical sig­nificance.

Results

Forty‑eight patients required treatment for respiratory failure.  Two patients became dependent on 24 hour noninvasive IPPV without being equipped with oximeters or the expiratory aids of this protocol.  Both died from pneumonia and respiratory failure complicating intercurrent URTIs.  They and are no longer considered.  The remaining 46 patients included: (1) 22 non‑protocol tracheostomy IPPV users who were referred before 1983, referred already using tracheostomy IPPV and for whom the tube had not been removed, or patients who were not trained in nonin­vasive methods because of failing to return regularly to the clinic; (2) 10 protocol noninvasive IPPV users; and (3) 14 patients who had experienced multiple hospitalizations before being referred to us and were then placed on our protocol.  The latter included 3 patients who were extubated and one whose tracheostomy tube was removed, all despite requiring continuous ventilatory support. 

The 22 non‑protocol patients experienced initial episodes of respiratory failure at 23.75.0 years of age and underwent tracheostomy at 24.64.9 years of age.  It was not always pos­sible to separate tracheostomy IPPV use into part‑time or full‑time use.  However, most patients continued to use tracheostomy IPPV 24 hours a day from the point at which they underwent tracheostomy.  The 10 protocol patients first had acute respiratory failure prevented at 19.84.4 years of age, began ongoing part‑time noninvasive IPPV at 19.94.4 years of age and full‑time IPPV at 24.66.1 years of age.  The 14 initially con­ventionally managed patients initially experienced respiratory failure at 16.92.8 years of age, began ongoing part‑time nonin­vasive IPPV at 19.43.8 years of age, and full‑time noninvasive IPPV at 23.36.1 years of age.

Although three of the protocol patients had gastrostomy tubes to supplement paraoral intake, bulbar muscle function was always sufficient to permit speech, assisted PCF over 160 L/m, and continued oral intake except for one patient who could no longer swallow.  Although no DMD ventilator user had PCF measure­ments before 1983, only three tracheostomy IPPV users had in­dwelling gastrostomy tubes, all received nutrition by mouth and could speak clearly; and clinically, this group had comparable bulbar muscle function to the protocol patients.

Seventeen non‑protocol patients were hospitalized 2.41.8 times for 35.466.3 days over 3.62.7 years before undergoing tracheostomy.  On the other hand, three protocol patients had 1 hospitalization avoided, each, by using the protocol over a mean 93 month period before using ongoing noninvasive IPPV.

At the time of undergoing tracheostomy the 23 tracheos­tomized patients were hospitalized a mean of 72.2112 days, and translaryngeally intubated for 16.15.4 days before tracheostomy.  This can be compared to the 24 noninvasive IPPV users who were hospitalized 6.02.4 days (p<0.0005) at onset of definitive daily ventilatory assistance.  In addition, over their next 65.4 patient‑years of part‑time, and 60.8 years of full‑time use of noninvasive IPPV, these 24 patients were intubated a mean of 0.30.2 times for 4.03.6 days over 5.53.8 total years per patient.  Upon extubation the patients returned to noninvasive ventilatory assistance.  The rates of hospitalizations, hospitalization days, and averted hospitalizations of the protocol noninvasive IPPV users and non‑protocol patients are noted in Tables 1 and 2. 

Life was prolonged a mean of at least 7.96.3 years for the 22 long‑term tracheostomy IPPV users, 13 of whom are still alive, to a current age of 33.77.4 years.  Life was prolonged at least a mean of 4.53.7 years for the 14 full‑time noninvasive IPPV users, 11 of whom are still alive, to a current age of 28.06.1 years.

Discussion

The initial objective, that of identifying DMD patients at risk of developing acute respiratory failure, appears to have been achieved because no patients with (assisted) PCF above 270 L/m developed acute respiratory distress.  All but 1 patient for whom assisted PCF were below 270 L/m had VCs below 1000 ml.  Despite 14 patients eventually requiring full‑time noninvasive IPPV, two with VCs under 100 ml, and three with severe dysphagia necessitating indwelling gastrostomy tubes, assisted PCF of at least 160 L/min were attainable for all protocol patients through the study period.  This is consistent with previous studies that indicated the feasibility of managing ventilatory failure without tracheostomy.10,11

Prior to initiating this protocol we informed patients to contact us at the first sign of an URTI, especially in the event of airway encomberment or respiratory distress, and we would provide them with an oximeter and respiratory muscle aids to avert hospitalization.  However, two consecutive patients presented to us only after they had already developed severe oxyhemoglobin desaturation and pneumonia and they required hospitalization and intubation.  We, therefore, felt that the presence of an oximeter in the home was especially important for immediate feedback to the patient during URTIs since we have not yet observed any DMD patient to have developed respiratory com­plications and require intubation when the SaO2 baseline was maintained above 92%.  All of the patients in this study, and their care providers, were properly trained and equipped and used oximetry and the respiratory aids appropriately.  However, these patients had very dedicated and capable family members who provided noninvasive IPPV and the cough aids, at times, every 10 to 15 minutes, and essentially around the clock, during some in­tercurrent URTIs.  Thus, it would be anticipated that patients without dedicated and effective care providers might not succeed in avoiding conventional management.  

This study demonstrated that, given effective care providers, the use of noninvasive inspiratory and expiratory aids with oximetry feedback can significantly decrease the incidence of respiratory hospitalizations and prolong survival for DMD patients without resort to tracheostomy.  There were only 3 protocol patients who had episodes that would have warranted hospitalization before requiring definitive ventilator use be­cause most protocol patients first used noninvasive IPPV during URTIs and weaned to nocturnal use of noninvasive IPPV after their first averted hospitalization, whereas non‑protocol patients tended to have repeated episodes of respiratory failure until un­dergoing tracheostomy.  The study also demonstrated that hospitalization rates and days can be significantly fewer both at onset of definitive ventilator use and subsequently for part‑time and for full‑time noninvasive IPPV users than for tracheostomy IPPV users.

Considering the fact that the ages at onset of tracheostomy IPPV and full‑time noninvasive IPPV were the same, the noninvasive IPPV users experienced their first episodes of acute respiratory distress before the tracheostomy group, and there were as many protocol as tracheostomy patients who required gastrostomy tubes, one can not explain the lower morbidity in the noninvasive IPPV users by their being less severely affected than the nonprotocol patients.

Besides guiding the use of respiratory muscle aids during URTIs, oximetry was also useful in guiding the daytime and noc­turnal use of noninvasive IPPV.13,16,17  For patients using only nocturnal noninvasive IPPV, daytime hypercapnia was usually mild and well tolerated when SaO2 remained within normal limits.  Oximetry was also useful for indicating the need for manually and mechanically assisted coughing during oral food intake for patients with severe dysphagia.  Although many patients were found to aspirate food to varying degrees, patients were per­mitted to continue oral intake provided that any aspiration‑associated oxyhemoglobin desaturations could be reversed by using manually and mechanically assisted coughing.  Mechanical insufflation‑exsufflation via an anesthesia mask became par­ticularly important when assisted PCF were marginal (about 160 L/min) and scoliosis prevented optimal abdominal thrusts.  Since all patients had normal SaO2 when awake and using ventilatory as­sistance as necessary, and all could reverse sudden desatura­tions, we considered any ongoing aspiration of airway secretions or food to be clinically insignificant. 

Although both tracheostomy and noninvasive IPPV can prolong life, tracheostomy IPPV has been reported to be associated with numerous complications and excessive expense.18  Tracheostomy necessitates more professional medical services than noninvasive ventilation and there are other expenses associated with wound care, tracheal suction catheters, and other supplies.  In addi­tion, tracheostomy is associated with an initial mean hospital stay of 72 days, and more hospitalization days subsequently, than is associated with noninvasive management.  Further, patients al­most invariably prefer noninvasive aids over tracheostomy for safety, convenience, appearance, comfort, facilitating effect on speech, sleep, swallowing, and general acceptability.19  The in­vasive nature of tracheostomy IPPV and tracheal suctioning also diminishes patients' quality of life.  This study further sug­gests that even full‑time need for noninvasive IPPV can be safer as well as being less expensive than tracheostomy IPPV.  Most of our protocol patients were trained in the use of respiratory muscle aids by a skilled respiratory therapist in the clinic and home settings and five noninvasive IPPV users went on to require definitive 24 hour ventilatory support without as yet ever being hospitalized.  Successful use of noninvasive alternatives, however, requires dedicated personal care providers and their ef­fort intensive interventions during intercurrent URTIs.

Despite the fact that survival can be extended, there con­tinue to be reports declaring that the prognosis for DMD remains unchanged,20 and DMD remains "untreatable".21,22  This is par­ticularly ironic since medical treatments that are more costly and less beneficial on function and survival are now being widely used to treat amyotrophic lateral sclerosis, another common severe neuromuscular disease.  Medications, however, are a familiar modality to physicians and usually are associated with high profile marketing campaigns.  Physical medicine applications that can prolong survival and optimize quality of life, on the other hand, tend to be misunderstood and underutilized.23  As a result, the conventional practices of offering elective tracheos­tomy, treating "sleep disordered breathing" with oxygen sup­plementation and minimal span nocturnal bi‑level positive airway pressure,24,25 or of simply waiting for acute respiratory failure to develop and then intubating and tracheostomizing patients are no longer necessary or advisable.26

References

1. Brooke MH, Fenichel GM, Griggs RC, et al. Duchenne muscular dystrophy: patterns of clinical progression and effects of sup­portive therapy. Neurol 1989; 39:475‑80

2. Mukoyama M, Kondo K, Hizawa K, Nishitani H, and the DMDR Group. Life spans of Duchenne muscular dystrophy patients in the hospital care program in Japan. J Neurol Sci 1987; 81:155‑58

3. Inkley SR, Oldenburg FC, Vignos PJ Jr. Pulmonary function in Duchenne muscular dystrophy related to stage of disease. Am J Med 1974; 56:297‑306

4. Rideau Y, Gatin G, Bach J, Gines G. Prolongation of life in Duchenne muscular dystrophy. Acta Neurol 1983; 5:118‑24

5. Vignos PJ. Respiratory function and pulmonary infection in Duchenne muscular dystrophy. Isr J Med Sci 1977; 13:207‑14

6. Emery AEH. Duchenne muscular dystrophy: genetic aspects, car­rier detection and antenatal diagnosis. Br Med Bull 1980; 36:117‑22

7. Bach JR, Pansit R, Ballanger F, Kulessa R. Neuromuscular ven­tilatory insufficiency: the effect of home mechanical ventilator use vs. oxygen therapy on pneumonia and hospitalization rates. Arch Phys Med Rehabil (submitted for publication)

8. Mier‑Jedrzejowicz A, Brophy C, Green M. Respiratory muscle weakness during upper respiratory tract infections. Am Rev Respir Dis 1988; 138:5‑7

9. Bach JR. Prevention of morbidity and mortality with the use of physical medicine aids. In: Bach JR, ed. Pulmonary rehabilita­tion: the obstructive and paralytic conditions. Philadelphia: Hanley & Belfus, 1996; 303‑29

10. Bach JR. Amyotrophic lateral sclerosis: predictors for prolongation of life by noninvasive respiratory aids. Arch Phys Med Rehabil 1995; 76:828‑32

11. Bach JR, Saporito LR. Criteria for extubation and tracheos­tomy tube removal for patients with ventilatory failure: a dif­ferent approach to weaning. Chest 1996; 110:1566‑71

12. Jennekens FGI, ten Kate LP, de Visser M, Wintzen AR. Duchenne and Becker muscular dystrophies. In: Emery AEH, ed. Diagnostic criteria for neuromuscular disorders. Baarn, The Netherlands: Eur Neuromuscular Centre, 1994; 9‑13

13. Bach JR, Alba AS. Management of chronic alveolar hypoventila­tion by nasal ventilation. Chest 1990; 97:52‑57

14. Bach JR. Mechanical insufflation‑exsufflation: comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest 1993; 104:1553‑62

15. Bach JR, Alba AS, Saporito LR. Intermittent positive pressure ventilation via the mouth as an alternative to tracheostomy for 257 ventilator users. Chest 1993; 103:174‑82

16. Hill NS, Eveloff SE, Carlisle CC, Goff SG. Efficacy of noc­turnal nasal ventilation in patients with restrictive thoracic disease. Am Rev Respir Dis 1992; 145:365‑71

17. Jimenez JFM, Sanchez de Cos Escuin J, Vicente CD, Valle MH, Otero FF. Nasal intermittent positive pressure ventilation: analysis of its withdrawal. Chest 1995; 107:382‑88

18. Bach JR, Intintola P, Alba AS, Holland I. The ventilator‑assisted individual: cost analysis of institutionalization versus rehabilitation and in‑home management. Chest 1992; 101:26‑30

19. Bach JR. A comparison of long‑term ventilatory support alter­natives from the perspective of the patient and care giver. Chest 1993; 104:1702‑06

20. Boland BJ, Silbert PL, Groover RV, Wollan PC, Silverstein MD. Skeletal, cardiac, and smooth muscle failure in Duchenne muscular dystrophy. Pediatr Neurol 1996; 14:7‑12

21. Hyser CL, Mendell JR. Recent advances in Duchenne and Becker muscular dystrophy. Neurol Clin 1988; 6:429‑38.

22. Matsuoka Y, Sakai M, Iida M, Takahashi A. Advance of dis­ability and prognosis in Duchenne muscular dystrophy ‑ a com­parison between institutionalized care and home care. Clin Neurol 1989;29:1000‑03

23. Bach JR. Ventilator use by muscular dystrophy association patients: an update. Arch Phys Med Rehabil 1992; 73:179‑83

24. Sanders MH, Kern N. Obstructive sleep apnea treated by independ­ently adjusted inspiratory and expiratory positive airway pressure via nasal mask: physiologic and clinical implications. Chest 1990; 98:317‑24

25. Smith PEM, Edwards RHT, Calverley PMA. Protriptyline treat­ment of sleep hypoxaemia in Duchenne muscular dystrophy. Thorax 1989; 44:1002‑05

26. Bach JR. Ventilator use by muscular dystrophy association patients: an update. Arch Phys Med Rehabil 1992; 73:179‑83


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