Outcomes of Research or Clinical Trials Activity Levels Acute Flaccid Paralysis Ageing Anaerobic Threshold Anaesthesia Assistive Technology Brain Cardiorespiratory Cardiovascular Clinical Evaluation Cold Intolerance Complementary Therapies Continence Coping Styles and Strategies Cultural Context Diagnosis and Management Differential Diagnosis Drugs Dysphagia Dysphonia Epidemiology Exercise Falls Fatigue Fractures Gender Differences Immune Response Inflammation Late Effects of Polio Muscle Strength Muscular Atrophy Orthoses Pain Polio Immunisation Post-Polio Motor Unit Psychology Quality of Life Renal Complications Respiratory Complications and Management Restless Legs Syndrome Sleep Analaysis Surgery Vitality Vocational Implications

Title order Author order Journal order Date order
Category: Respiratory Complications and Management

Title: 1246: Missed chronic respiratory failure in a post-polio syndrome patient
Author: Aljasmi M, Agarwal K, Uppalapati S, Bazan L
Affiliation: Not stated
Journal: Critical Care Medicine
Citation: Crit Care Med. 2015 Dec;43(12 Suppl 1):313
Publication Year and Month: 2015 12

Abstract: This is a brief Case Report which does not have an abstract. This is an extract:

INTRODUCTION: Prior to the introduction of the polio vaccine, paralytic poliomyelitis was a major cause of morbidity and death. Twenty-five to fifty percent of the survivors are known to develop post-polio syndrome. Symptoms include fatigue, insidious respiratory failure, obstructive sleep apnea, bulbar neuropathy, central ventilatory abnormalities, hemi-diaphragmatic paralysis and progressive functional decline with new onset weakness, among others. We present a case of post-polio syndrome presenting with hypercapnic respiratory failure.

Conclusions:

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Airway Secretion Clearance by Mechanical Exsufflation for Post-Poliomyelitis Ventilator-Assisted Individuals
Author: John R. Bach, William H. Smith, Jennifier Michaels, Lou Saporito, Augusta S. Alba, Rajeev Dayal, Jeffrey Pan.
Affiliation:
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil Vol 74:170-177, February 1993.
Publication Year and Month: 1992 03

Abstract: Pulmonary complications from impaired airway secretion clearance mechanisms are major causes of morbidity and mortality for post-poliomyelitis individuals. The purpose of this study was to review the long-term use of manually assisted coughing and mechanical insufflation-exsufflation (MI-E) by post-poliomyelitis ventilator-assisted individuals (PVAIs) and to compare the peak cough expiratory flows (PCEF) created during unassisted and assisted coughing. Twenty-four PVAIs who have used noninvasive methods of ventilatory support for an average of 27 years, relied on methods of manually assisted coughing and/or MI-E without complications during intercurrent respiratory tract infections (RTIs). Nine of the 24 individuals were studied for PCEF. They had a mean forced vital capacity (FVC) of 0.54 ± 0.47L and a mean maximum insufflation capacity achieved by air stacking of ventilator insufflations and glossopharyngeal breathing of 1.7L. The PCEF were as follows: unassisted, 1.78 ± 1.16L/sec; following a maximum assisted insufflation, 3.75 ± 0.73L/sec; with manual assistance by abdominal compression following a maximum assisted insufflation, 4.64 ± 1.42L/sec; and with MI-E, 6.97 ± 0.89L/sec. We conclude that manually assisted coughing and MI-E are effective and safe methods of airway secretion clearance for PVAIs with impaired expiratory muscle function who would otherwise be managed by endotracheal suctioning. Severely decreased maximum insufflation capacity but not vital capacity indicate need for a tracheostomy.

Conclusions: Post-poliomyelitis individuals using noninvasive methods of ventilatory support have greater PCEF produced by manually assisted coughing and by MI-E than can be produced by unassisted coughing. Thus, if intubation and tracheostomy are to be avoided, it becomes crucial for these individuals and their caregivers to learn and have access to these methods, particularly during RTIs when expiratory muscle weakness is exacerbated.[55] These principles may also apply to individuals with severe expiratory muscle weakness due to other neuromuscular conditions. We believe that it is largely because of the lack of general knowledge of and access to MI-E that other centers are more likely to resort to intubation and tracheostomy for patients with advanced neuromuscular disease and RTIs. We conclude that loss of FVC in itself does not mandate the need for intubation or tracheostomy for ventilatory support. A maximum insufflation capacity below 500mL may be a better indicator. MI-E should be further explored as a convenient and probably more effective alternative to endotracheal suctioning for ventilator-supported neuromuscular individuals.

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Category: Respiratory Complications and Management

Title: Bi-level positive airway pressure ventilation maintains adequate ventilation in post-polio patients with respiratory failure.
Author: Gillis-Haegerstrand C, Markström A, Barle H.
Affiliation: Department of Anaesthesiology and Intensive Care, Karolinska Institutet, Danderyd Hospital, Stockholm, Sweden. [email protected]
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Acta Anaesthesiologica Scandinavica 2006 May;50(5):580-5
Publication Year and Month: 2006 05

Abstract: BACKGROUND:
Patients suffering from post-polio syndrome still contribute significantly to the number of patients with chronic respiratory failure requiring home mechanical ventilation (HMV). Many of these patients are treated either with invasive (tracheostomy) or non-invasive (nasal mask) controlled mechanical ventilation i.e. volume-controlled ventilation (VCV). In this group of patients, we have previously shown that bi-level pressure support ventilation (bi-level PSV) decreases the oxygen cost of breathing. The aim of this study was to compare the effect of bi-level PSV, with special regard to the adequacy of ventilation and the oxygen cost of breathing, during the patients' ordinary VCV and spontaneous breathing.

METHODS:
Eight post-polio patients on nocturnal VCV were investigated. Five of them were tracheostomized and three of them used a nasal mask. Work of breathing was analysed by assessing differences in oxygen consumption (VO2) using indirect calorimetry. Blood gases were obtained regularly to assess adequacy of ventilation.

RESULTS:
Bi-level PSV decreases the oxygen cost of breathing in post-polio patients with respiratory failure without decreasing ventilation efficiency. Furthermore, PaCO2 decreased significantly using this mode of ventilation (P < 0.05).

Conclusions: In this study, it was shown that bi-level PSV reduces the oxygen cost of breathing and gave a significant decrease in PaCO2 in PPS patients. These data suggest that bi-level PSV ventilation maintains adequate ventilation in patients who suffer from post-polio syndrome with respiratory failure.

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Category: Respiratory Complications and Management

Title: Comparison of activity and fatigue of the respiratory muscles and pulmonary characteristics between post-polio patients and controls: A pilot study
Author: David Shoseyov, Tali Cohen-Kaufman, Isabella Schwartz, Sigal Portnoy,
Affiliation: Pediatric department, Hadassah Mount Scopus, Jerusalem, Israel
Physical Medicine and Rehabilitation department, Hadassah Mount Scopus, Jerusalem, Israel
Department of Physiotherapy, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Department of Occupational Therapy, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
National Yang-Ming University, TAIWAN
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: PLoS One. 2017; 12(7): e0182036.
Publication Year and Month: 2017 07

Abstract: Objectives
To compare pulmonary function measures, maximal respiratory pressure and fatigue of respiratory muscles between patients with Post-Polio Syndrome (PPS) and controls.

Design
Cross-sectional study.

Patients
Patients with PPS (N = 12; age 62.1±11.6 years) able to walk for 6 minutes without human assistance; age-matched controls with no history of polio or pulmonary dysfunction (N = 12; age 62.2±6.5 years).

Measurements
A body plethysmograph was used to quantify Residual Volume (RV), Total Lung Capacity (TLC), and Thoracic Gas Volume (TGV) etc. A manometer was used to measure Maximal Inspiratory Pressure (MIP) and Maximal Expiratory Pressure. A spirometer was used to measure Maximal Voluntary Ventilation (MVV). Surface electromyography (sEMG) recorded diaphragmatic muscle activity while performing MVV.

Results
The control group had significantly higher TGV and showed improvement in MIP following the effort (difference of 5.5±4.0cmH2O) while the PPS group showed deterioration in MIP (difference of -2.5±5.0cmH2O). Subjects with scoliosis had significantly higher RV/TLC values compared with subjects without scoliosis. The 25th frequency percentile of the sEMG signal acquired during MVV was reduced in the PPS group.

Conclusions: Maximal respiratory pressure test and sEMG measurements may identify fatigue of respiratory muscles in patients with PPS. Early diagnosis of respiratory impairment may delay respiratory decline and future need of invasive respiratory aids.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Feasibility of lung volume recruitment in early neuromuscular weakness: a comparison between amyotrophic lateral sclerosis, myotonic dystrophy, and postpolio syndrome
Author: Kaminska M (1,2), Browman F (3), Trojan DA (4), Genge A (4), Benedetti A (2,5), Petrof BJ (6,7)
Affiliation: (1) Respiratory Division, McGill University Health Centre, 1001 Decarie Blvd, D05.2504 Montreal, Quebec, Canada H4A 3J1; (2) Respiratory Epidemiology and Clinical Research Unit, McGill University, Montreal, Quebec, Canada; (3) Department of Respiratory Therapy, Montreal Chest Institute, McGill University Health Centre, Montreal, Quebec, Canada; (4) Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University Health Centre, Montreal, Quebec, Canada; (5) Departments of Medicine and Epidemiology, Biostatistics & Occupational Health, McGill University Health Centre, Montreal, Quebec, Canada; (6) Meakins-Christie Laboratories, McGill University and Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada; (7) Respiratory Division, McGill University Health Centre, 1001 Decarie Blvd, D05.2506 Montreal, Quebec, Canada H4A 3J1
Journal: PM&R: The Journal of Injury, Function, and Rehabilitation
Citation: PM R. 2015 Apr;1-8
Publication Year and Month: 2015 04

Abstract: BACKGROUND: Lung volume recruitment (LVR) is a cough assistance technique used in persons with neuromuscular disorders (NMDs), most typically in those requiring noninvasive ventilation (NIV). Whether it may be useful in persons with NMDs who have milder respiratory impairment is unknown.

OBJECTIVE: To assess the feasibility, impact on quality of life (QOL), and preliminary physiological effects of daily LVR in different categories of persons with NMDs who have an early stage of respiratory impairment.

DESIGN: Feasibility study.

SETTING: Academic tertiary care center.

PARTICIPANTS: Outpatients diagnosed with amyotrophic lateral sclerosis (n = 8), postpolio syndrome (n = 10), and myotonic dystrophy (n = 6) who had restrictive respiratory defects but were not yet using NIV.

METHODS: Participants were asked to perform LVR up to 4 times daily and log their LVR use in a diary. Physiological measurements and questionnaires were completed at baseline and after 3 months.

MAIN OUTCOME MEASUREMENTS: Compliance with LVR use was assessed, along with QOL and willingness to continue the treatment. Physiological measurements included forced vital capacity (FVC), lung insufflation capacity (LIC), and the LIC minus FVC difference.

RESULTS: Of the 24 recruited subjects, 7 with amyotrophic lateral sclerosis, 7 with postpolio syndrome, and 5 with myotonic dystrophy completed the study (n = 19). At baseline, mean values for FVC and spontaneous peak cough flow were 59.9% predicted and 373.1 L/min, respectively. For subjects completing the study, 74% were willing to continue long-term LVR use, and QOL scores were not adversely affected by LVR in any NMD subgroup. The LIC-FVC difference increased from baseline to follow-up by a mean of 0.243 L (P = .006) in all subjects (n = 19), suggesting a possible improvement in respiratory system mechanics.

Conclusions: In patients with NMDs who have early restrictive respiratory defects but do not yet require NIV, regular use of LVR is feasible with no negative impact on QOL over a 3-month period and may have physiological benefits. Further work is needed to determine whether early institution of LVR can improve respiratory system mechanics and help delay ventilatory failure in persons with NMDs.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Lung function in post-poliomyelitis syndrome: a cross-sectional study
Author: Lira CABD, Minozzo FC, Sousa BS, Vancini RL, Andrade MDS, Quadros AAJ, Oliveira ASB, Silva ACD.
Affiliation: Federal University of Goiás at Jataí, Jataí, Brazil - [email protected]
Journal: Jornal Brasileiro de Pneumologia (Brazilian Journal of Pulmonology)
Citation: J Bras Pneumol. 2013 Jun-Aug; 39(4): 455–460. doi: 10.1590/S1806-37132013000400009
Publication Year and Month: 2013 08

Abstract: OBJECTIVE: To compare lung function between patients with post-poliomyelitis syndrome and those with sequelae of paralytic poliomyelitis (without any signs or symptoms of post-poliomyelitis syndrome), as well as between patients with post-poliomyelitis syndrome and healthy controls.

METHODS: Twenty-nine male participants were assigned to one of three groups: control; poliomyelitis (comprising patients who had had paralytic poliomyelitis but had not developed post-poliomyelitis syndrome); and post-poliomyelitis syndrome. Volunteers underwent lung function measurements (spirometry and respiratory muscle strength assessment).

RESULTS: The results of the spirometric assessment revealed no significant differences among the groups except for an approximately 27% lower mean maximal voluntary ventilation in the post-poliomyelitis syndrome group when compared with the control group (p = 0.0127). Nevertheless, the maximal voluntary ventilation values for the post-poliomyelitis group were compared with those for the Brazilian population and were found to be normal. No significant differences were observed in respiratory muscle strength among the groups.

Conclusions: With the exception of lower maximal voluntary ventilation, there was no significant lung function impairment in outpatients diagnosed with post-poliomyelitis syndrome when compared with healthy subjects and with patients with sequelae of poliomyelitis without post-poliomyelitis syndrome. This is an important clinical finding because it shows that patients with post-poliomyelitis syndrome can have preserved lung function.

Outcome of Research: Not applicable.

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Category: Respiratory Complications and Management

Title: Poliomyelitis: late respiratory complications and management
Author: Fischer DA
Affiliation: Not stated
Journal: Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):891-4
Publication Year and Month: 1985 07

Abstract: One hundred forty-six respiratory polios have been reviewed for complications and current respiratory aids. One hundred thirty-eight of these people required respirator assistance at the onset of their poliomyelitis infection. Fifteen patients have kyphoscoliosis. Seventy-five percent of the total patients require some assisted ventilation. Fifty-two percent have tracheostomies. Most of these people have been followed at Rancho Los Amigos Medical Center for many years. The literature regarding late respiratory complications of polio is reviewed.

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Category: Respiratory Complications and Management

Title: Protocol for diaphragm pacing in patients with respiratory muscle weakness due to motor neurone disease (DiPALS): a randomised controlled trial.
Author: McDermott C, Maguire C, Cooper CL, Ackroyd R, Baird WO, Baudouin S, Bentley A, Bianchi S, Bourke S, Bradburn MJ, Dixon S, Ealing J, Galloway S, Karat D, Maynard N, Morrison K, Mustfa N, Stradling J, Talbot K, Williams T, Shaw PJ.
Affiliation: Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385A Glossop Road, Sheffield, S10 2HQ, UK.
Journal: BioMed Central Neurology
Citation: 2012 Aug 16;12:74.
Publication Year and Month: 2012 08

Abstract: BACKGROUND:
Motor neurone disease (MND) is a devastating illness which leads to muscle weakness and death, usually within 2-3 years of symptom onset. Respiratory insufficiency is a common cause of morbidity, particularly in later stages of MND and respiratory complications are the leading cause of mortality in MND patients. Non Invasive Ventilation (NIV) is the current standard therapy to manage respiratory insufficiency. Some MND patients however do not tolerate NIV due to a number of issues including mask interface problems and claustrophobia. In those that do tolerate NIV, eventually respiratory muscle weakness will progress to a point at which intermittent/overnight NIV is ineffective. The NeuRx RA/4 Diaphragm Pacing System was originally developed for patients with respiratory insufficiency and diaphragm paralysis secondary to stable high spinal cord injuries. The DiPALS study will assess the effect of diaphragm pacing (DP) when used to treat patients with MND and respiratory insufficiency.

METHOD/DESIGN:
108 patients will be recruited to the study at 5 sites in the UK. Patients will be randomised to either receive NIV (current standard care) or receive DP in addition to NIV. Study participants will be required to complete outcome measures at 5 follow up time points (2, 3, 6, 9 and 12 months) plus an additional surgery and 1 week post operative visit for those in the DP group. 12 patients (and their carers) from the DP group will also be asked to complete 2 qualitative interviews.

DISCUSSION:
The primary objective of this trial will be to evaluate the effect of Diaphragm Pacing (DP) on survival over the study duration in patients with MND with respiratory muscle weakness. The project is funded by the National Institute for Health Research, Health Technology Assessment (HTA) Programme (project number 09/55/33) and the Motor Neurone Disease Association and the Henry Smith Charity.

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Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Pulmonary dysfunction and its management in post-polio patients
Author: John R. Bach, Margaret Tiltona.


Affiliation: Department of Physical Medicine and Rehabilitation; UMDNJ-New Jersey Medical School, Director of the Center for Ventilator Management Alternatives, University Hospital, Newark.
Journal: NeuroRehabilitation
Citation: NeuroRehabilitation 8 (1997) 139-153
Publication Year and Month: 1997

Abstract: Respiratory dysfunction is extremely common and entails considerable risk of morbidity and mortality for individuals with past poliomyelitis. Although it is usually primarily due to respiratory muscle weakness, post-poliomyelitis individuals also have a high incidence of scoliosis, obesity, sleep disordered breathing, and bulbar muscle dysfunction. Although these factors can result in chronic alveolar hypoventilation (CAH) and frequent pulmonary complications and hospitalizations, CAH is usually not recognized until acute respiratory failure complicates an otherwise benign upper respiratory tract infection. The use of non-invasive inspiratory and expiratory muscle aids, however, can decrease the risk of acute respiratory failure, hospitalizations for respiratory complications, and need to resort to tracheal intubation. Timely introduction of non-invasive intermittent positive pressure ventilation (IPPV), manually assisted coughing, and mechanical insufflation-exsufflation (MI-E) and non-invasive blood gas monitoring which can most often be performed in the home setting, are the principle interventions for avoiding complications and maintaining optimal quality of life.

Conclusions: The failure to make timely appropriate management decisions often leads to episodes of acute respiratory failure and unnecessary hospitalizations, endotracheal intubations, bronchoscopies, and tracheostomies. The use of these invasive approaches adversely affects quality of life and can increase the risk of pulmonary complications and mortality for post-poliomyelitis patients [47]. Episodes of acute respiratory failure which most often result from otherwise benign upper respiratory tract infections can be reversed by the timely use of non-invasive respiratory muscle aids.

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Pulmonary Dysfunction and Sleep Disordered Breathing as Post-Polio Sequelae: Evaluation and Management
Author: John R. Bach, MD and Augusta S. Alba, MD
Affiliation: New Jersey Medical School of the University of Medicine and Dentistry of New Jersey, and the Department of Physical Medicine and Rehabilitation, University Hospital; Department of Physical Rehabilitation Medicine, New York University and Godwater Memorial Hospital, New York, NY.
Journal: Orthopedics
Citation: Orthopedics December 1991 Vol 14 No 12 1329-1337.
Publication Year and Month: 1991

Abstract: Post-polio sequelae can include sleep disordered breathing and chronic alveolar hypoventilation (CAH). Both conditions develop insidiously and can render the post-polio survivor susceptible to cardiopulmonary morbidity and mortality when not treated in a timely and appropriate manner. These conditions can be diagnosed by a combination of spirometry, noninvasive blood gas monitoring, and ambulatory polysomnography. Sleep disordered breathing is most frequently managed by nasal continuous positive airway pressure, while tracheostomy ventilation is the most common treatment for ventilatory failure. We report the more effective and comfortable techniques recently made available for managing sleep disordered breathing and the use of noninvasive treatment options for CAH, respiratory failure, and impaired airway clearance mechanisms. One hundred forty-three cases are reviewed.

Conclusions:

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Sleep disordered breathing in adult with polio sequelae: A case control study of predictive factors
Author: A.Leotard, A.Pages, M.Salga, G.Genet, J.Levy, M.A.Quera-Salva, F.Genet
Affiliation: Hopital R.-Poincaré, Sleep unit, Garches, France

Hopital R.-Poincaré, Department of Physical Medicine and Rehabilitation, Garches, France
Journal: Annals of Physical and Rehabilitation Medicine
Citation: Volume 61, Supplement, July 2018, Page e72

Publication Year and Month: 2018 07

Abstract: Introduction/Background
Estimates of sleep disorder breathing (SDB) prevalence are higher in patients with polio sequelae than in general population, ranging from 50% to 65%. No specific predictive factors have been described in our knowledge. The aim of this study was to identify SDB predictive factors among polio survivors.

Material and method
A case control study including patients with polio sequelae with confirmed SDB (apnea hypopnea index (AHI) ≥ 5; n = 38) compared to polio patients with AHI ≤ 5 or negative Berlin questionnaire (n = 114). Matching criteria were: sex, age at evaluation, and body mass index (BMI). For each patient, age at acute polio, bulbar, trunk, and lower limbs involvement, scoliosis, current walking abilities and history of arthrodesis, brace or iron lung were assessed using preexisting database and medical charts. SDB symptoms, Berlin questionnaire and AHI using polysomnographic reports were also assessed retrospectively.

Results
Among the 362 polio patients from our systematic database 152 (38 cases and 114 controls) were matched for comparison. SDB ratio was significantly higher in patients with bulbar involvement at acute polio (100% vs. 22.5%), trunk involvement at acute polio (41.9% vs. 18.4%), bulbar involvement at evaluation (100% vs. 23%), scoliosis (38.6% vs. 13.8%) and non-walking patients (50% vs. 22.5%) compared with polio controls. Multivariate analysis only shown scoliosis to be associated with SDB in those patients (OR = 2.72 (95% CI: 1.10–6.95); P = 0.03).

Conclusions: Despite there was an increased ratio of SDB in patients with bulbar and trunk involvement, only scoliosis seems to specifically increase the risk of SDB occurrence among polio survivors. In those patients, symptoms suggestive of SDB should be searched for systematically, especially in case of trunk deformities.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Sleep-disordered breathing in neuromuscular disease
Author: Aboussouan LS
Affiliation: Respiratory Institute, Cleveland Clinic, Cleveland, Ohio
Journal: American Journal of Respiratory and Critical Care Medicine
Citation: Am J Respir Crit Care Med. 2015 May 1;191(9):979-89. doi: 10.1164/rccm.201412-2224CI
Publication Year and Month: 2015 05

Abstract: Sleep-disordered breathing in neuromuscular diseases is due to an exaggerated reduction in lung volumes during supine sleep, a compromised physiologic adaptation to sleep, and specific features of the diseases that may promote upper airway collapse or heart failure. The normal decrease in the rib cage contribution to the tidal volume during phasic REM sleep becomes a critical vulnerability, resulting in saw-tooth oxygen desaturation possibly representing the earliest manifestation of respiratory muscle weakness. Hypoventilation can occur in REM sleep and progress into non-REM sleep, with continuous desaturation and hypercarbia. Specific characteristics of neuromuscular disorders, such as pharyngeal neuropathy or weakness, macroglossia, bulbar manifestations, or low lung volumes, predispose patients to the development of obstructive events. Central sleep-disordered breathing can occur with associated cardiomyopathy (e.g., dystrophies) or from instability in the control of breathing due to diaphragm weakness. Mitigating factors such as recruitment of accessory respiratory muscles, reduction in REM sleep, and loss of normal REM atonia in some individuals may partially protect against sleep-disordered breathing. Noninvasive ventilation, a standard-of-care management option for sleep-disordered breathing, can itself trigger specific sleep-disordered breathing events including air leaks, patient-ventilator asynchrony, central sleep apnea, and glottic closure. These events increase arousals, reduce adherence, and impair sleep architecture. Polysomnography plays an important role in addressing pitfalls in the diagnosis of sleep-disordered breathing in neuromuscular diseases, identifying sleep-disordered breathing triggered by noninvasive ventilation, and optimizing noninvasive ventilation settings.

Conclusions:

Outcome of Research: Effective.

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There are currently 12 papers in this category.

Category: Respiratory Complications and Management

Title: Sleep disordered breathing in adult with polio sequelae: A case control study of predictive factors
Author: A.Leotard, A.Pages, M.Salga, G.Genet, J.Levy, M.A.Quera-Salva, F.Genet
Affiliation: Hopital R.-Poincaré, Sleep unit, Garches, France

Hopital R.-Poincaré, Department of Physical Medicine and Rehabilitation, Garches, France
Journal: Annals of Physical and Rehabilitation Medicine
Citation: Volume 61, Supplement, July 2018, Page e72

Publication Year and Month: 2018 07

Abstract: Introduction/Background
Estimates of sleep disorder breathing (SDB) prevalence are higher in patients with polio sequelae than in general population, ranging from 50% to 65%. No specific predictive factors have been described in our knowledge. The aim of this study was to identify SDB predictive factors among polio survivors.

Material and method
A case control study including patients with polio sequelae with confirmed SDB (apnea hypopnea index (AHI) ≥ 5; n = 38) compared to polio patients with AHI ≤ 5 or negative Berlin questionnaire (n = 114). Matching criteria were: sex, age at evaluation, and body mass index (BMI). For each patient, age at acute polio, bulbar, trunk, and lower limbs involvement, scoliosis, current walking abilities and history of arthrodesis, brace or iron lung were assessed using preexisting database and medical charts. SDB symptoms, Berlin questionnaire and AHI using polysomnographic reports were also assessed retrospectively.

Results
Among the 362 polio patients from our systematic database 152 (38 cases and 114 controls) were matched for comparison. SDB ratio was significantly higher in patients with bulbar involvement at acute polio (100% vs. 22.5%), trunk involvement at acute polio (41.9% vs. 18.4%), bulbar involvement at evaluation (100% vs. 23%), scoliosis (38.6% vs. 13.8%) and non-walking patients (50% vs. 22.5%) compared with polio controls. Multivariate analysis only shown scoliosis to be associated with SDB in those patients (OR = 2.72 (95% CI: 1.10–6.95); P = 0.03).

Conclusions: Despite there was an increased ratio of SDB in patients with bulbar and trunk involvement, only scoliosis seems to specifically increase the risk of SDB occurrence among polio survivors. In those patients, symptoms suggestive of SDB should be searched for systematically, especially in case of trunk deformities.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Sleep-disordered breathing in neuromuscular disease
Author: Aboussouan LS
Affiliation: Respiratory Institute, Cleveland Clinic, Cleveland, Ohio
Journal: American Journal of Respiratory and Critical Care Medicine
Citation: Am J Respir Crit Care Med. 2015 May 1;191(9):979-89. doi: 10.1164/rccm.201412-2224CI
Publication Year and Month: 2015 05

Abstract: Sleep-disordered breathing in neuromuscular diseases is due to an exaggerated reduction in lung volumes during supine sleep, a compromised physiologic adaptation to sleep, and specific features of the diseases that may promote upper airway collapse or heart failure. The normal decrease in the rib cage contribution to the tidal volume during phasic REM sleep becomes a critical vulnerability, resulting in saw-tooth oxygen desaturation possibly representing the earliest manifestation of respiratory muscle weakness. Hypoventilation can occur in REM sleep and progress into non-REM sleep, with continuous desaturation and hypercarbia. Specific characteristics of neuromuscular disorders, such as pharyngeal neuropathy or weakness, macroglossia, bulbar manifestations, or low lung volumes, predispose patients to the development of obstructive events. Central sleep-disordered breathing can occur with associated cardiomyopathy (e.g., dystrophies) or from instability in the control of breathing due to diaphragm weakness. Mitigating factors such as recruitment of accessory respiratory muscles, reduction in REM sleep, and loss of normal REM atonia in some individuals may partially protect against sleep-disordered breathing. Noninvasive ventilation, a standard-of-care management option for sleep-disordered breathing, can itself trigger specific sleep-disordered breathing events including air leaks, patient-ventilator asynchrony, central sleep apnea, and glottic closure. These events increase arousals, reduce adherence, and impair sleep architecture. Polysomnography plays an important role in addressing pitfalls in the diagnosis of sleep-disordered breathing in neuromuscular diseases, identifying sleep-disordered breathing triggered by noninvasive ventilation, and optimizing noninvasive ventilation settings.

Conclusions:

Outcome of Research: Effective.

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Category: Respiratory Complications and Management

Title: 1246: Missed chronic respiratory failure in a post-polio syndrome patient
Author: Aljasmi M, Agarwal K, Uppalapati S, Bazan L
Affiliation: Not stated
Journal: Critical Care Medicine
Citation: Crit Care Med. 2015 Dec;43(12 Suppl 1):313
Publication Year and Month: 2015 12

Abstract: This is a brief Case Report which does not have an abstract. This is an extract:

INTRODUCTION: Prior to the introduction of the polio vaccine, paralytic poliomyelitis was a major cause of morbidity and death. Twenty-five to fifty percent of the survivors are known to develop post-polio syndrome. Symptoms include fatigue, insidious respiratory failure, obstructive sleep apnea, bulbar neuropathy, central ventilatory abnormalities, hemi-diaphragmatic paralysis and progressive functional decline with new onset weakness, among others. We present a case of post-polio syndrome presenting with hypercapnic respiratory failure.

Conclusions:

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Comparison of activity and fatigue of the respiratory muscles and pulmonary characteristics between post-polio patients and controls: A pilot study
Author: David Shoseyov, Tali Cohen-Kaufman, Isabella Schwartz, Sigal Portnoy,
Affiliation: Pediatric department, Hadassah Mount Scopus, Jerusalem, Israel
Physical Medicine and Rehabilitation department, Hadassah Mount Scopus, Jerusalem, Israel
Department of Physiotherapy, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Department of Occupational Therapy, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
National Yang-Ming University, TAIWAN
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: PLoS One. 2017; 12(7): e0182036.
Publication Year and Month: 2017 07

Abstract: Objectives
To compare pulmonary function measures, maximal respiratory pressure and fatigue of respiratory muscles between patients with Post-Polio Syndrome (PPS) and controls.

Design
Cross-sectional study.

Patients
Patients with PPS (N = 12; age 62.1±11.6 years) able to walk for 6 minutes without human assistance; age-matched controls with no history of polio or pulmonary dysfunction (N = 12; age 62.2±6.5 years).

Measurements
A body plethysmograph was used to quantify Residual Volume (RV), Total Lung Capacity (TLC), and Thoracic Gas Volume (TGV) etc. A manometer was used to measure Maximal Inspiratory Pressure (MIP) and Maximal Expiratory Pressure. A spirometer was used to measure Maximal Voluntary Ventilation (MVV). Surface electromyography (sEMG) recorded diaphragmatic muscle activity while performing MVV.

Results
The control group had significantly higher TGV and showed improvement in MIP following the effort (difference of 5.5±4.0cmH2O) while the PPS group showed deterioration in MIP (difference of -2.5±5.0cmH2O). Subjects with scoliosis had significantly higher RV/TLC values compared with subjects without scoliosis. The 25th frequency percentile of the sEMG signal acquired during MVV was reduced in the PPS group.

Conclusions: Maximal respiratory pressure test and sEMG measurements may identify fatigue of respiratory muscles in patients with PPS. Early diagnosis of respiratory impairment may delay respiratory decline and future need of invasive respiratory aids.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Poliomyelitis: late respiratory complications and management
Author: Fischer DA
Affiliation: Not stated
Journal: Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):891-4
Publication Year and Month: 1985 07

Abstract: One hundred forty-six respiratory polios have been reviewed for complications and current respiratory aids. One hundred thirty-eight of these people required respirator assistance at the onset of their poliomyelitis infection. Fifteen patients have kyphoscoliosis. Seventy-five percent of the total patients require some assisted ventilation. Fifty-two percent have tracheostomies. Most of these people have been followed at Rancho Los Amigos Medical Center for many years. The literature regarding late respiratory complications of polio is reviewed.

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Category: Respiratory Complications and Management

Title: Bi-level positive airway pressure ventilation maintains adequate ventilation in post-polio patients with respiratory failure.
Author: Gillis-Haegerstrand C, Markström A, Barle H.
Affiliation: Department of Anaesthesiology and Intensive Care, Karolinska Institutet, Danderyd Hospital, Stockholm, Sweden. [email protected]
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Acta Anaesthesiologica Scandinavica 2006 May;50(5):580-5
Publication Year and Month: 2006 05

Abstract: BACKGROUND:
Patients suffering from post-polio syndrome still contribute significantly to the number of patients with chronic respiratory failure requiring home mechanical ventilation (HMV). Many of these patients are treated either with invasive (tracheostomy) or non-invasive (nasal mask) controlled mechanical ventilation i.e. volume-controlled ventilation (VCV). In this group of patients, we have previously shown that bi-level pressure support ventilation (bi-level PSV) decreases the oxygen cost of breathing. The aim of this study was to compare the effect of bi-level PSV, with special regard to the adequacy of ventilation and the oxygen cost of breathing, during the patients' ordinary VCV and spontaneous breathing.

METHODS:
Eight post-polio patients on nocturnal VCV were investigated. Five of them were tracheostomized and three of them used a nasal mask. Work of breathing was analysed by assessing differences in oxygen consumption (VO2) using indirect calorimetry. Blood gases were obtained regularly to assess adequacy of ventilation.

RESULTS:
Bi-level PSV decreases the oxygen cost of breathing in post-polio patients with respiratory failure without decreasing ventilation efficiency. Furthermore, PaCO2 decreased significantly using this mode of ventilation (P < 0.05).

Conclusions: In this study, it was shown that bi-level PSV reduces the oxygen cost of breathing and gave a significant decrease in PaCO2 in PPS patients. These data suggest that bi-level PSV ventilation maintains adequate ventilation in patients who suffer from post-polio syndrome with respiratory failure.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Pulmonary dysfunction and its management in post-polio patients
Author: John R. Bach, Margaret Tiltona.


Affiliation: Department of Physical Medicine and Rehabilitation; UMDNJ-New Jersey Medical School, Director of the Center for Ventilator Management Alternatives, University Hospital, Newark.
Journal: NeuroRehabilitation
Citation: NeuroRehabilitation 8 (1997) 139-153
Publication Year and Month: 1997

Abstract: Respiratory dysfunction is extremely common and entails considerable risk of morbidity and mortality for individuals with past poliomyelitis. Although it is usually primarily due to respiratory muscle weakness, post-poliomyelitis individuals also have a high incidence of scoliosis, obesity, sleep disordered breathing, and bulbar muscle dysfunction. Although these factors can result in chronic alveolar hypoventilation (CAH) and frequent pulmonary complications and hospitalizations, CAH is usually not recognized until acute respiratory failure complicates an otherwise benign upper respiratory tract infection. The use of non-invasive inspiratory and expiratory muscle aids, however, can decrease the risk of acute respiratory failure, hospitalizations for respiratory complications, and need to resort to tracheal intubation. Timely introduction of non-invasive intermittent positive pressure ventilation (IPPV), manually assisted coughing, and mechanical insufflation-exsufflation (MI-E) and non-invasive blood gas monitoring which can most often be performed in the home setting, are the principle interventions for avoiding complications and maintaining optimal quality of life.

Conclusions: The failure to make timely appropriate management decisions often leads to episodes of acute respiratory failure and unnecessary hospitalizations, endotracheal intubations, bronchoscopies, and tracheostomies. The use of these invasive approaches adversely affects quality of life and can increase the risk of pulmonary complications and mortality for post-poliomyelitis patients [47]. Episodes of acute respiratory failure which most often result from otherwise benign upper respiratory tract infections can be reversed by the timely use of non-invasive respiratory muscle aids.

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Pulmonary Dysfunction and Sleep Disordered Breathing as Post-Polio Sequelae: Evaluation and Management
Author: John R. Bach, MD and Augusta S. Alba, MD
Affiliation: New Jersey Medical School of the University of Medicine and Dentistry of New Jersey, and the Department of Physical Medicine and Rehabilitation, University Hospital; Department of Physical Rehabilitation Medicine, New York University and Godwater Memorial Hospital, New York, NY.
Journal: Orthopedics
Citation: Orthopedics December 1991 Vol 14 No 12 1329-1337.
Publication Year and Month: 1991

Abstract: Post-polio sequelae can include sleep disordered breathing and chronic alveolar hypoventilation (CAH). Both conditions develop insidiously and can render the post-polio survivor susceptible to cardiopulmonary morbidity and mortality when not treated in a timely and appropriate manner. These conditions can be diagnosed by a combination of spirometry, noninvasive blood gas monitoring, and ambulatory polysomnography. Sleep disordered breathing is most frequently managed by nasal continuous positive airway pressure, while tracheostomy ventilation is the most common treatment for ventilatory failure. We report the more effective and comfortable techniques recently made available for managing sleep disordered breathing and the use of noninvasive treatment options for CAH, respiratory failure, and impaired airway clearance mechanisms. One hundred forty-three cases are reviewed.

Conclusions:

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Airway Secretion Clearance by Mechanical Exsufflation for Post-Poliomyelitis Ventilator-Assisted Individuals
Author: John R. Bach, William H. Smith, Jennifier Michaels, Lou Saporito, Augusta S. Alba, Rajeev Dayal, Jeffrey Pan.
Affiliation:
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil Vol 74:170-177, February 1993.
Publication Year and Month: 1992 03

Abstract: Pulmonary complications from impaired airway secretion clearance mechanisms are major causes of morbidity and mortality for post-poliomyelitis individuals. The purpose of this study was to review the long-term use of manually assisted coughing and mechanical insufflation-exsufflation (MI-E) by post-poliomyelitis ventilator-assisted individuals (PVAIs) and to compare the peak cough expiratory flows (PCEF) created during unassisted and assisted coughing. Twenty-four PVAIs who have used noninvasive methods of ventilatory support for an average of 27 years, relied on methods of manually assisted coughing and/or MI-E without complications during intercurrent respiratory tract infections (RTIs). Nine of the 24 individuals were studied for PCEF. They had a mean forced vital capacity (FVC) of 0.54 ± 0.47L and a mean maximum insufflation capacity achieved by air stacking of ventilator insufflations and glossopharyngeal breathing of 1.7L. The PCEF were as follows: unassisted, 1.78 ± 1.16L/sec; following a maximum assisted insufflation, 3.75 ± 0.73L/sec; with manual assistance by abdominal compression following a maximum assisted insufflation, 4.64 ± 1.42L/sec; and with MI-E, 6.97 ± 0.89L/sec. We conclude that manually assisted coughing and MI-E are effective and safe methods of airway secretion clearance for PVAIs with impaired expiratory muscle function who would otherwise be managed by endotracheal suctioning. Severely decreased maximum insufflation capacity but not vital capacity indicate need for a tracheostomy.

Conclusions: Post-poliomyelitis individuals using noninvasive methods of ventilatory support have greater PCEF produced by manually assisted coughing and by MI-E than can be produced by unassisted coughing. Thus, if intubation and tracheostomy are to be avoided, it becomes crucial for these individuals and their caregivers to learn and have access to these methods, particularly during RTIs when expiratory muscle weakness is exacerbated.[55] These principles may also apply to individuals with severe expiratory muscle weakness due to other neuromuscular conditions. We believe that it is largely because of the lack of general knowledge of and access to MI-E that other centers are more likely to resort to intubation and tracheostomy for patients with advanced neuromuscular disease and RTIs. We conclude that loss of FVC in itself does not mandate the need for intubation or tracheostomy for ventilatory support. A maximum insufflation capacity below 500mL may be a better indicator. MI-E should be further explored as a convenient and probably more effective alternative to endotracheal suctioning for ventilator-supported neuromuscular individuals.

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Category: Respiratory Complications and Management

Title: Feasibility of lung volume recruitment in early neuromuscular weakness: a comparison between amyotrophic lateral sclerosis, myotonic dystrophy, and postpolio syndrome
Author: Kaminska M (1,2), Browman F (3), Trojan DA (4), Genge A (4), Benedetti A (2,5), Petrof BJ (6,7)
Affiliation: (1) Respiratory Division, McGill University Health Centre, 1001 Decarie Blvd, D05.2504 Montreal, Quebec, Canada H4A 3J1; (2) Respiratory Epidemiology and Clinical Research Unit, McGill University, Montreal, Quebec, Canada; (3) Department of Respiratory Therapy, Montreal Chest Institute, McGill University Health Centre, Montreal, Quebec, Canada; (4) Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University Health Centre, Montreal, Quebec, Canada; (5) Departments of Medicine and Epidemiology, Biostatistics & Occupational Health, McGill University Health Centre, Montreal, Quebec, Canada; (6) Meakins-Christie Laboratories, McGill University and Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada; (7) Respiratory Division, McGill University Health Centre, 1001 Decarie Blvd, D05.2506 Montreal, Quebec, Canada H4A 3J1
Journal: PM&R: The Journal of Injury, Function, and Rehabilitation
Citation: PM R. 2015 Apr;1-8
Publication Year and Month: 2015 04

Abstract: BACKGROUND: Lung volume recruitment (LVR) is a cough assistance technique used in persons with neuromuscular disorders (NMDs), most typically in those requiring noninvasive ventilation (NIV). Whether it may be useful in persons with NMDs who have milder respiratory impairment is unknown.

OBJECTIVE: To assess the feasibility, impact on quality of life (QOL), and preliminary physiological effects of daily LVR in different categories of persons with NMDs who have an early stage of respiratory impairment.

DESIGN: Feasibility study.

SETTING: Academic tertiary care center.

PARTICIPANTS: Outpatients diagnosed with amyotrophic lateral sclerosis (n = 8), postpolio syndrome (n = 10), and myotonic dystrophy (n = 6) who had restrictive respiratory defects but were not yet using NIV.

METHODS: Participants were asked to perform LVR up to 4 times daily and log their LVR use in a diary. Physiological measurements and questionnaires were completed at baseline and after 3 months.

MAIN OUTCOME MEASUREMENTS: Compliance with LVR use was assessed, along with QOL and willingness to continue the treatment. Physiological measurements included forced vital capacity (FVC), lung insufflation capacity (LIC), and the LIC minus FVC difference.

RESULTS: Of the 24 recruited subjects, 7 with amyotrophic lateral sclerosis, 7 with postpolio syndrome, and 5 with myotonic dystrophy completed the study (n = 19). At baseline, mean values for FVC and spontaneous peak cough flow were 59.9% predicted and 373.1 L/min, respectively. For subjects completing the study, 74% were willing to continue long-term LVR use, and QOL scores were not adversely affected by LVR in any NMD subgroup. The LIC-FVC difference increased from baseline to follow-up by a mean of 0.243 L (P = .006) in all subjects (n = 19), suggesting a possible improvement in respiratory system mechanics.

Conclusions: In patients with NMDs who have early restrictive respiratory defects but do not yet require NIV, regular use of LVR is feasible with no negative impact on QOL over a 3-month period and may have physiological benefits. Further work is needed to determine whether early institution of LVR can improve respiratory system mechanics and help delay ventilatory failure in persons with NMDs.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Lung function in post-poliomyelitis syndrome: a cross-sectional study
Author: Lira CABD, Minozzo FC, Sousa BS, Vancini RL, Andrade MDS, Quadros AAJ, Oliveira ASB, Silva ACD.
Affiliation: Federal University of Goiás at Jataí, Jataí, Brazil - [email protected]
Journal: Jornal Brasileiro de Pneumologia (Brazilian Journal of Pulmonology)
Citation: J Bras Pneumol. 2013 Jun-Aug; 39(4): 455–460. doi: 10.1590/S1806-37132013000400009
Publication Year and Month: 2013 08

Abstract: OBJECTIVE: To compare lung function between patients with post-poliomyelitis syndrome and those with sequelae of paralytic poliomyelitis (without any signs or symptoms of post-poliomyelitis syndrome), as well as between patients with post-poliomyelitis syndrome and healthy controls.

METHODS: Twenty-nine male participants were assigned to one of three groups: control; poliomyelitis (comprising patients who had had paralytic poliomyelitis but had not developed post-poliomyelitis syndrome); and post-poliomyelitis syndrome. Volunteers underwent lung function measurements (spirometry and respiratory muscle strength assessment).

RESULTS: The results of the spirometric assessment revealed no significant differences among the groups except for an approximately 27% lower mean maximal voluntary ventilation in the post-poliomyelitis syndrome group when compared with the control group (p = 0.0127). Nevertheless, the maximal voluntary ventilation values for the post-poliomyelitis group were compared with those for the Brazilian population and were found to be normal. No significant differences were observed in respiratory muscle strength among the groups.

Conclusions: With the exception of lower maximal voluntary ventilation, there was no significant lung function impairment in outpatients diagnosed with post-poliomyelitis syndrome when compared with healthy subjects and with patients with sequelae of poliomyelitis without post-poliomyelitis syndrome. This is an important clinical finding because it shows that patients with post-poliomyelitis syndrome can have preserved lung function.

Outcome of Research: Not applicable.

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Category: Respiratory Complications and Management

Title: Protocol for diaphragm pacing in patients with respiratory muscle weakness due to motor neurone disease (DiPALS): a randomised controlled trial.
Author: McDermott C, Maguire C, Cooper CL, Ackroyd R, Baird WO, Baudouin S, Bentley A, Bianchi S, Bourke S, Bradburn MJ, Dixon S, Ealing J, Galloway S, Karat D, Maynard N, Morrison K, Mustfa N, Stradling J, Talbot K, Williams T, Shaw PJ.
Affiliation: Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385A Glossop Road, Sheffield, S10 2HQ, UK.
Journal: BioMed Central Neurology
Citation: 2012 Aug 16;12:74.
Publication Year and Month: 2012 08

Abstract: BACKGROUND:
Motor neurone disease (MND) is a devastating illness which leads to muscle weakness and death, usually within 2-3 years of symptom onset. Respiratory insufficiency is a common cause of morbidity, particularly in later stages of MND and respiratory complications are the leading cause of mortality in MND patients. Non Invasive Ventilation (NIV) is the current standard therapy to manage respiratory insufficiency. Some MND patients however do not tolerate NIV due to a number of issues including mask interface problems and claustrophobia. In those that do tolerate NIV, eventually respiratory muscle weakness will progress to a point at which intermittent/overnight NIV is ineffective. The NeuRx RA/4 Diaphragm Pacing System was originally developed for patients with respiratory insufficiency and diaphragm paralysis secondary to stable high spinal cord injuries. The DiPALS study will assess the effect of diaphragm pacing (DP) when used to treat patients with MND and respiratory insufficiency.

METHOD/DESIGN:
108 patients will be recruited to the study at 5 sites in the UK. Patients will be randomised to either receive NIV (current standard care) or receive DP in addition to NIV. Study participants will be required to complete outcome measures at 5 follow up time points (2, 3, 6, 9 and 12 months) plus an additional surgery and 1 week post operative visit for those in the DP group. 12 patients (and their carers) from the DP group will also be asked to complete 2 qualitative interviews.

DISCUSSION:
The primary objective of this trial will be to evaluate the effect of Diaphragm Pacing (DP) on survival over the study duration in patients with MND with respiratory muscle weakness. The project is funded by the National Institute for Health Research, Health Technology Assessment (HTA) Programme (project number 09/55/33) and the Motor Neurone Disease Association and the Henry Smith Charity.

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Outcome of Research: More research required

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There are currently 12 papers in this category.

Category: Respiratory Complications and Management

Title: Sleep-disordered breathing in neuromuscular disease
Author: Aboussouan LS
Affiliation: Respiratory Institute, Cleveland Clinic, Cleveland, Ohio
Journal: American Journal of Respiratory and Critical Care Medicine
Citation: Am J Respir Crit Care Med. 2015 May 1;191(9):979-89. doi: 10.1164/rccm.201412-2224CI
Publication Year and Month: 2015 05

Abstract: Sleep-disordered breathing in neuromuscular diseases is due to an exaggerated reduction in lung volumes during supine sleep, a compromised physiologic adaptation to sleep, and specific features of the diseases that may promote upper airway collapse or heart failure. The normal decrease in the rib cage contribution to the tidal volume during phasic REM sleep becomes a critical vulnerability, resulting in saw-tooth oxygen desaturation possibly representing the earliest manifestation of respiratory muscle weakness. Hypoventilation can occur in REM sleep and progress into non-REM sleep, with continuous desaturation and hypercarbia. Specific characteristics of neuromuscular disorders, such as pharyngeal neuropathy or weakness, macroglossia, bulbar manifestations, or low lung volumes, predispose patients to the development of obstructive events. Central sleep-disordered breathing can occur with associated cardiomyopathy (e.g., dystrophies) or from instability in the control of breathing due to diaphragm weakness. Mitigating factors such as recruitment of accessory respiratory muscles, reduction in REM sleep, and loss of normal REM atonia in some individuals may partially protect against sleep-disordered breathing. Noninvasive ventilation, a standard-of-care management option for sleep-disordered breathing, can itself trigger specific sleep-disordered breathing events including air leaks, patient-ventilator asynchrony, central sleep apnea, and glottic closure. These events increase arousals, reduce adherence, and impair sleep architecture. Polysomnography plays an important role in addressing pitfalls in the diagnosis of sleep-disordered breathing in neuromuscular diseases, identifying sleep-disordered breathing triggered by noninvasive ventilation, and optimizing noninvasive ventilation settings.

Conclusions:

Outcome of Research: Effective.

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Category: Respiratory Complications and Management

Title: Sleep disordered breathing in adult with polio sequelae: A case control study of predictive factors
Author: A.Leotard, A.Pages, M.Salga, G.Genet, J.Levy, M.A.Quera-Salva, F.Genet
Affiliation: Hopital R.-Poincaré, Sleep unit, Garches, France

Hopital R.-Poincaré, Department of Physical Medicine and Rehabilitation, Garches, France
Journal: Annals of Physical and Rehabilitation Medicine
Citation: Volume 61, Supplement, July 2018, Page e72

Publication Year and Month: 2018 07

Abstract: Introduction/Background
Estimates of sleep disorder breathing (SDB) prevalence are higher in patients with polio sequelae than in general population, ranging from 50% to 65%. No specific predictive factors have been described in our knowledge. The aim of this study was to identify SDB predictive factors among polio survivors.

Material and method
A case control study including patients with polio sequelae with confirmed SDB (apnea hypopnea index (AHI) ≥ 5; n = 38) compared to polio patients with AHI ≤ 5 or negative Berlin questionnaire (n = 114). Matching criteria were: sex, age at evaluation, and body mass index (BMI). For each patient, age at acute polio, bulbar, trunk, and lower limbs involvement, scoliosis, current walking abilities and history of arthrodesis, brace or iron lung were assessed using preexisting database and medical charts. SDB symptoms, Berlin questionnaire and AHI using polysomnographic reports were also assessed retrospectively.

Results
Among the 362 polio patients from our systematic database 152 (38 cases and 114 controls) were matched for comparison. SDB ratio was significantly higher in patients with bulbar involvement at acute polio (100% vs. 22.5%), trunk involvement at acute polio (41.9% vs. 18.4%), bulbar involvement at evaluation (100% vs. 23%), scoliosis (38.6% vs. 13.8%) and non-walking patients (50% vs. 22.5%) compared with polio controls. Multivariate analysis only shown scoliosis to be associated with SDB in those patients (OR = 2.72 (95% CI: 1.10–6.95); P = 0.03).

Conclusions: Despite there was an increased ratio of SDB in patients with bulbar and trunk involvement, only scoliosis seems to specifically increase the risk of SDB occurrence among polio survivors. In those patients, symptoms suggestive of SDB should be searched for systematically, especially in case of trunk deformities.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Airway Secretion Clearance by Mechanical Exsufflation for Post-Poliomyelitis Ventilator-Assisted Individuals
Author: John R. Bach, William H. Smith, Jennifier Michaels, Lou Saporito, Augusta S. Alba, Rajeev Dayal, Jeffrey Pan.
Affiliation:
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil Vol 74:170-177, February 1993.
Publication Year and Month: 1992 03

Abstract: Pulmonary complications from impaired airway secretion clearance mechanisms are major causes of morbidity and mortality for post-poliomyelitis individuals. The purpose of this study was to review the long-term use of manually assisted coughing and mechanical insufflation-exsufflation (MI-E) by post-poliomyelitis ventilator-assisted individuals (PVAIs) and to compare the peak cough expiratory flows (PCEF) created during unassisted and assisted coughing. Twenty-four PVAIs who have used noninvasive methods of ventilatory support for an average of 27 years, relied on methods of manually assisted coughing and/or MI-E without complications during intercurrent respiratory tract infections (RTIs). Nine of the 24 individuals were studied for PCEF. They had a mean forced vital capacity (FVC) of 0.54 ± 0.47L and a mean maximum insufflation capacity achieved by air stacking of ventilator insufflations and glossopharyngeal breathing of 1.7L. The PCEF were as follows: unassisted, 1.78 ± 1.16L/sec; following a maximum assisted insufflation, 3.75 ± 0.73L/sec; with manual assistance by abdominal compression following a maximum assisted insufflation, 4.64 ± 1.42L/sec; and with MI-E, 6.97 ± 0.89L/sec. We conclude that manually assisted coughing and MI-E are effective and safe methods of airway secretion clearance for PVAIs with impaired expiratory muscle function who would otherwise be managed by endotracheal suctioning. Severely decreased maximum insufflation capacity but not vital capacity indicate need for a tracheostomy.

Conclusions: Post-poliomyelitis individuals using noninvasive methods of ventilatory support have greater PCEF produced by manually assisted coughing and by MI-E than can be produced by unassisted coughing. Thus, if intubation and tracheostomy are to be avoided, it becomes crucial for these individuals and their caregivers to learn and have access to these methods, particularly during RTIs when expiratory muscle weakness is exacerbated.[55] These principles may also apply to individuals with severe expiratory muscle weakness due to other neuromuscular conditions. We believe that it is largely because of the lack of general knowledge of and access to MI-E that other centers are more likely to resort to intubation and tracheostomy for patients with advanced neuromuscular disease and RTIs. We conclude that loss of FVC in itself does not mandate the need for intubation or tracheostomy for ventilatory support. A maximum insufflation capacity below 500mL may be a better indicator. MI-E should be further explored as a convenient and probably more effective alternative to endotracheal suctioning for ventilator-supported neuromuscular individuals.

Outcome of Research:

Availability of Paper: The full text of this paper has been generously made available by the publisher.

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Category: Respiratory Complications and Management

Title: Protocol for diaphragm pacing in patients with respiratory muscle weakness due to motor neurone disease (DiPALS): a randomised controlled trial.
Author: McDermott C, Maguire C, Cooper CL, Ackroyd R, Baird WO, Baudouin S, Bentley A, Bianchi S, Bourke S, Bradburn MJ, Dixon S, Ealing J, Galloway S, Karat D, Maynard N, Morrison K, Mustfa N, Stradling J, Talbot K, Williams T, Shaw PJ.
Affiliation: Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385A Glossop Road, Sheffield, S10 2HQ, UK.
Journal: BioMed Central Neurology
Citation: 2012 Aug 16;12:74.
Publication Year and Month: 2012 08

Abstract: BACKGROUND:
Motor neurone disease (MND) is a devastating illness which leads to muscle weakness and death, usually within 2-3 years of symptom onset. Respiratory insufficiency is a common cause of morbidity, particularly in later stages of MND and respiratory complications are the leading cause of mortality in MND patients. Non Invasive Ventilation (NIV) is the current standard therapy to manage respiratory insufficiency. Some MND patients however do not tolerate NIV due to a number of issues including mask interface problems and claustrophobia. In those that do tolerate NIV, eventually respiratory muscle weakness will progress to a point at which intermittent/overnight NIV is ineffective. The NeuRx RA/4 Diaphragm Pacing System was originally developed for patients with respiratory insufficiency and diaphragm paralysis secondary to stable high spinal cord injuries. The DiPALS study will assess the effect of diaphragm pacing (DP) when used to treat patients with MND and respiratory insufficiency.

METHOD/DESIGN:
108 patients will be recruited to the study at 5 sites in the UK. Patients will be randomised to either receive NIV (current standard care) or receive DP in addition to NIV. Study participants will be required to complete outcome measures at 5 follow up time points (2, 3, 6, 9 and 12 months) plus an additional surgery and 1 week post operative visit for those in the DP group. 12 patients (and their carers) from the DP group will also be asked to complete 2 qualitative interviews.

DISCUSSION:
The primary objective of this trial will be to evaluate the effect of Diaphragm Pacing (DP) on survival over the study duration in patients with MND with respiratory muscle weakness. The project is funded by the National Institute for Health Research, Health Technology Assessment (HTA) Programme (project number 09/55/33) and the Motor Neurone Disease Association and the Henry Smith Charity.

Conclusions:

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: 1246: Missed chronic respiratory failure in a post-polio syndrome patient
Author: Aljasmi M, Agarwal K, Uppalapati S, Bazan L
Affiliation: Not stated
Journal: Critical Care Medicine
Citation: Crit Care Med. 2015 Dec;43(12 Suppl 1):313
Publication Year and Month: 2015 12

Abstract: This is a brief Case Report which does not have an abstract. This is an extract:

INTRODUCTION: Prior to the introduction of the polio vaccine, paralytic poliomyelitis was a major cause of morbidity and death. Twenty-five to fifty percent of the survivors are known to develop post-polio syndrome. Symptoms include fatigue, insidious respiratory failure, obstructive sleep apnea, bulbar neuropathy, central ventilatory abnormalities, hemi-diaphragmatic paralysis and progressive functional decline with new onset weakness, among others. We present a case of post-polio syndrome presenting with hypercapnic respiratory failure.

Conclusions:

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Lung function in post-poliomyelitis syndrome: a cross-sectional study
Author: Lira CABD, Minozzo FC, Sousa BS, Vancini RL, Andrade MDS, Quadros AAJ, Oliveira ASB, Silva ACD.
Affiliation: Federal University of Goiás at Jataí, Jataí, Brazil - [email protected]
Journal: Jornal Brasileiro de Pneumologia (Brazilian Journal of Pulmonology)
Citation: J Bras Pneumol. 2013 Jun-Aug; 39(4): 455–460. doi: 10.1590/S1806-37132013000400009
Publication Year and Month: 2013 08

Abstract: OBJECTIVE: To compare lung function between patients with post-poliomyelitis syndrome and those with sequelae of paralytic poliomyelitis (without any signs or symptoms of post-poliomyelitis syndrome), as well as between patients with post-poliomyelitis syndrome and healthy controls.

METHODS: Twenty-nine male participants were assigned to one of three groups: control; poliomyelitis (comprising patients who had had paralytic poliomyelitis but had not developed post-poliomyelitis syndrome); and post-poliomyelitis syndrome. Volunteers underwent lung function measurements (spirometry and respiratory muscle strength assessment).

RESULTS: The results of the spirometric assessment revealed no significant differences among the groups except for an approximately 27% lower mean maximal voluntary ventilation in the post-poliomyelitis syndrome group when compared with the control group (p = 0.0127). Nevertheless, the maximal voluntary ventilation values for the post-poliomyelitis group were compared with those for the Brazilian population and were found to be normal. No significant differences were observed in respiratory muscle strength among the groups.

Conclusions: With the exception of lower maximal voluntary ventilation, there was no significant lung function impairment in outpatients diagnosed with post-poliomyelitis syndrome when compared with healthy subjects and with patients with sequelae of poliomyelitis without post-poliomyelitis syndrome. This is an important clinical finding because it shows that patients with post-poliomyelitis syndrome can have preserved lung function.

Outcome of Research: Not applicable.

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Category: Respiratory Complications and Management

Title: Pulmonary dysfunction and its management in post-polio patients
Author: John R. Bach, Margaret Tiltona.


Affiliation: Department of Physical Medicine and Rehabilitation; UMDNJ-New Jersey Medical School, Director of the Center for Ventilator Management Alternatives, University Hospital, Newark.
Journal: NeuroRehabilitation
Citation: NeuroRehabilitation 8 (1997) 139-153
Publication Year and Month: 1997

Abstract: Respiratory dysfunction is extremely common and entails considerable risk of morbidity and mortality for individuals with past poliomyelitis. Although it is usually primarily due to respiratory muscle weakness, post-poliomyelitis individuals also have a high incidence of scoliosis, obesity, sleep disordered breathing, and bulbar muscle dysfunction. Although these factors can result in chronic alveolar hypoventilation (CAH) and frequent pulmonary complications and hospitalizations, CAH is usually not recognized until acute respiratory failure complicates an otherwise benign upper respiratory tract infection. The use of non-invasive inspiratory and expiratory muscle aids, however, can decrease the risk of acute respiratory failure, hospitalizations for respiratory complications, and need to resort to tracheal intubation. Timely introduction of non-invasive intermittent positive pressure ventilation (IPPV), manually assisted coughing, and mechanical insufflation-exsufflation (MI-E) and non-invasive blood gas monitoring which can most often be performed in the home setting, are the principle interventions for avoiding complications and maintaining optimal quality of life.

Conclusions: The failure to make timely appropriate management decisions often leads to episodes of acute respiratory failure and unnecessary hospitalizations, endotracheal intubations, bronchoscopies, and tracheostomies. The use of these invasive approaches adversely affects quality of life and can increase the risk of pulmonary complications and mortality for post-poliomyelitis patients [47]. Episodes of acute respiratory failure which most often result from otherwise benign upper respiratory tract infections can be reversed by the timely use of non-invasive respiratory muscle aids.

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Comparison of activity and fatigue of the respiratory muscles and pulmonary characteristics between post-polio patients and controls: A pilot study
Author: David Shoseyov, Tali Cohen-Kaufman, Isabella Schwartz, Sigal Portnoy,
Affiliation: Pediatric department, Hadassah Mount Scopus, Jerusalem, Israel
Physical Medicine and Rehabilitation department, Hadassah Mount Scopus, Jerusalem, Israel
Department of Physiotherapy, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Department of Occupational Therapy, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
National Yang-Ming University, TAIWAN
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: PLoS One. 2017; 12(7): e0182036.
Publication Year and Month: 2017 07

Abstract: Objectives
To compare pulmonary function measures, maximal respiratory pressure and fatigue of respiratory muscles between patients with Post-Polio Syndrome (PPS) and controls.

Design
Cross-sectional study.

Patients
Patients with PPS (N = 12; age 62.1±11.6 years) able to walk for 6 minutes without human assistance; age-matched controls with no history of polio or pulmonary dysfunction (N = 12; age 62.2±6.5 years).

Measurements
A body plethysmograph was used to quantify Residual Volume (RV), Total Lung Capacity (TLC), and Thoracic Gas Volume (TGV) etc. A manometer was used to measure Maximal Inspiratory Pressure (MIP) and Maximal Expiratory Pressure. A spirometer was used to measure Maximal Voluntary Ventilation (MVV). Surface electromyography (sEMG) recorded diaphragmatic muscle activity while performing MVV.

Results
The control group had significantly higher TGV and showed improvement in MIP following the effort (difference of 5.5±4.0cmH2O) while the PPS group showed deterioration in MIP (difference of -2.5±5.0cmH2O). Subjects with scoliosis had significantly higher RV/TLC values compared with subjects without scoliosis. The 25th frequency percentile of the sEMG signal acquired during MVV was reduced in the PPS group.

Conclusions: Maximal respiratory pressure test and sEMG measurements may identify fatigue of respiratory muscles in patients with PPS. Early diagnosis of respiratory impairment may delay respiratory decline and future need of invasive respiratory aids.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Bi-level positive airway pressure ventilation maintains adequate ventilation in post-polio patients with respiratory failure.
Author: Gillis-Haegerstrand C, Markström A, Barle H.
Affiliation: Department of Anaesthesiology and Intensive Care, Karolinska Institutet, Danderyd Hospital, Stockholm, Sweden. [email protected]
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Acta Anaesthesiologica Scandinavica 2006 May;50(5):580-5
Publication Year and Month: 2006 05

Abstract: BACKGROUND:
Patients suffering from post-polio syndrome still contribute significantly to the number of patients with chronic respiratory failure requiring home mechanical ventilation (HMV). Many of these patients are treated either with invasive (tracheostomy) or non-invasive (nasal mask) controlled mechanical ventilation i.e. volume-controlled ventilation (VCV). In this group of patients, we have previously shown that bi-level pressure support ventilation (bi-level PSV) decreases the oxygen cost of breathing. The aim of this study was to compare the effect of bi-level PSV, with special regard to the adequacy of ventilation and the oxygen cost of breathing, during the patients' ordinary VCV and spontaneous breathing.

METHODS:
Eight post-polio patients on nocturnal VCV were investigated. Five of them were tracheostomized and three of them used a nasal mask. Work of breathing was analysed by assessing differences in oxygen consumption (VO2) using indirect calorimetry. Blood gases were obtained regularly to assess adequacy of ventilation.

RESULTS:
Bi-level PSV decreases the oxygen cost of breathing in post-polio patients with respiratory failure without decreasing ventilation efficiency. Furthermore, PaCO2 decreased significantly using this mode of ventilation (P < 0.05).

Conclusions: In this study, it was shown that bi-level PSV reduces the oxygen cost of breathing and gave a significant decrease in PaCO2 in PPS patients. These data suggest that bi-level PSV ventilation maintains adequate ventilation in patients who suffer from post-polio syndrome with respiratory failure.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Poliomyelitis: late respiratory complications and management
Author: Fischer DA
Affiliation: Not stated
Journal: Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):891-4
Publication Year and Month: 1985 07

Abstract: One hundred forty-six respiratory polios have been reviewed for complications and current respiratory aids. One hundred thirty-eight of these people required respirator assistance at the onset of their poliomyelitis infection. Fifteen patients have kyphoscoliosis. Seventy-five percent of the total patients require some assisted ventilation. Fifty-two percent have tracheostomies. Most of these people have been followed at Rancho Los Amigos Medical Center for many years. The literature regarding late respiratory complications of polio is reviewed.

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Category: Respiratory Complications and Management

Title: Pulmonary Dysfunction and Sleep Disordered Breathing as Post-Polio Sequelae: Evaluation and Management
Author: John R. Bach, MD and Augusta S. Alba, MD
Affiliation: New Jersey Medical School of the University of Medicine and Dentistry of New Jersey, and the Department of Physical Medicine and Rehabilitation, University Hospital; Department of Physical Rehabilitation Medicine, New York University and Godwater Memorial Hospital, New York, NY.
Journal: Orthopedics
Citation: Orthopedics December 1991 Vol 14 No 12 1329-1337.
Publication Year and Month: 1991

Abstract: Post-polio sequelae can include sleep disordered breathing and chronic alveolar hypoventilation (CAH). Both conditions develop insidiously and can render the post-polio survivor susceptible to cardiopulmonary morbidity and mortality when not treated in a timely and appropriate manner. These conditions can be diagnosed by a combination of spirometry, noninvasive blood gas monitoring, and ambulatory polysomnography. Sleep disordered breathing is most frequently managed by nasal continuous positive airway pressure, while tracheostomy ventilation is the most common treatment for ventilatory failure. We report the more effective and comfortable techniques recently made available for managing sleep disordered breathing and the use of noninvasive treatment options for CAH, respiratory failure, and impaired airway clearance mechanisms. One hundred forty-three cases are reviewed.

Conclusions:

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Feasibility of lung volume recruitment in early neuromuscular weakness: a comparison between amyotrophic lateral sclerosis, myotonic dystrophy, and postpolio syndrome
Author: Kaminska M (1,2), Browman F (3), Trojan DA (4), Genge A (4), Benedetti A (2,5), Petrof BJ (6,7)
Affiliation: (1) Respiratory Division, McGill University Health Centre, 1001 Decarie Blvd, D05.2504 Montreal, Quebec, Canada H4A 3J1; (2) Respiratory Epidemiology and Clinical Research Unit, McGill University, Montreal, Quebec, Canada; (3) Department of Respiratory Therapy, Montreal Chest Institute, McGill University Health Centre, Montreal, Quebec, Canada; (4) Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University Health Centre, Montreal, Quebec, Canada; (5) Departments of Medicine and Epidemiology, Biostatistics & Occupational Health, McGill University Health Centre, Montreal, Quebec, Canada; (6) Meakins-Christie Laboratories, McGill University and Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada; (7) Respiratory Division, McGill University Health Centre, 1001 Decarie Blvd, D05.2506 Montreal, Quebec, Canada H4A 3J1
Journal: PM&R: The Journal of Injury, Function, and Rehabilitation
Citation: PM R. 2015 Apr;1-8
Publication Year and Month: 2015 04

Abstract: BACKGROUND: Lung volume recruitment (LVR) is a cough assistance technique used in persons with neuromuscular disorders (NMDs), most typically in those requiring noninvasive ventilation (NIV). Whether it may be useful in persons with NMDs who have milder respiratory impairment is unknown.

OBJECTIVE: To assess the feasibility, impact on quality of life (QOL), and preliminary physiological effects of daily LVR in different categories of persons with NMDs who have an early stage of respiratory impairment.

DESIGN: Feasibility study.

SETTING: Academic tertiary care center.

PARTICIPANTS: Outpatients diagnosed with amyotrophic lateral sclerosis (n = 8), postpolio syndrome (n = 10), and myotonic dystrophy (n = 6) who had restrictive respiratory defects but were not yet using NIV.

METHODS: Participants were asked to perform LVR up to 4 times daily and log their LVR use in a diary. Physiological measurements and questionnaires were completed at baseline and after 3 months.

MAIN OUTCOME MEASUREMENTS: Compliance with LVR use was assessed, along with QOL and willingness to continue the treatment. Physiological measurements included forced vital capacity (FVC), lung insufflation capacity (LIC), and the LIC minus FVC difference.

RESULTS: Of the 24 recruited subjects, 7 with amyotrophic lateral sclerosis, 7 with postpolio syndrome, and 5 with myotonic dystrophy completed the study (n = 19). At baseline, mean values for FVC and spontaneous peak cough flow were 59.9% predicted and 373.1 L/min, respectively. For subjects completing the study, 74% were willing to continue long-term LVR use, and QOL scores were not adversely affected by LVR in any NMD subgroup. The LIC-FVC difference increased from baseline to follow-up by a mean of 0.243 L (P = .006) in all subjects (n = 19), suggesting a possible improvement in respiratory system mechanics.

Conclusions: In patients with NMDs who have early restrictive respiratory defects but do not yet require NIV, regular use of LVR is feasible with no negative impact on QOL over a 3-month period and may have physiological benefits. Further work is needed to determine whether early institution of LVR can improve respiratory system mechanics and help delay ventilatory failure in persons with NMDs.

Outcome of Research: More research required

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There are currently 12 papers in this category.

Category: Respiratory Complications and Management

Title: Sleep disordered breathing in adult with polio sequelae: A case control study of predictive factors
Author: A.Leotard, A.Pages, M.Salga, G.Genet, J.Levy, M.A.Quera-Salva, F.Genet
Affiliation: Hopital R.-Poincaré, Sleep unit, Garches, France

Hopital R.-Poincaré, Department of Physical Medicine and Rehabilitation, Garches, France
Journal: Annals of Physical and Rehabilitation Medicine
Citation: Volume 61, Supplement, July 2018, Page e72

Publication Year and Month: 2018 07

Abstract: Introduction/Background
Estimates of sleep disorder breathing (SDB) prevalence are higher in patients with polio sequelae than in general population, ranging from 50% to 65%. No specific predictive factors have been described in our knowledge. The aim of this study was to identify SDB predictive factors among polio survivors.

Material and method
A case control study including patients with polio sequelae with confirmed SDB (apnea hypopnea index (AHI) ≥ 5; n = 38) compared to polio patients with AHI ≤ 5 or negative Berlin questionnaire (n = 114). Matching criteria were: sex, age at evaluation, and body mass index (BMI). For each patient, age at acute polio, bulbar, trunk, and lower limbs involvement, scoliosis, current walking abilities and history of arthrodesis, brace or iron lung were assessed using preexisting database and medical charts. SDB symptoms, Berlin questionnaire and AHI using polysomnographic reports were also assessed retrospectively.

Results
Among the 362 polio patients from our systematic database 152 (38 cases and 114 controls) were matched for comparison. SDB ratio was significantly higher in patients with bulbar involvement at acute polio (100% vs. 22.5%), trunk involvement at acute polio (41.9% vs. 18.4%), bulbar involvement at evaluation (100% vs. 23%), scoliosis (38.6% vs. 13.8%) and non-walking patients (50% vs. 22.5%) compared with polio controls. Multivariate analysis only shown scoliosis to be associated with SDB in those patients (OR = 2.72 (95% CI: 1.10–6.95); P = 0.03).

Conclusions: Despite there was an increased ratio of SDB in patients with bulbar and trunk involvement, only scoliosis seems to specifically increase the risk of SDB occurrence among polio survivors. In those patients, symptoms suggestive of SDB should be searched for systematically, especially in case of trunk deformities.

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Category: Respiratory Complications and Management

Title: Comparison of activity and fatigue of the respiratory muscles and pulmonary characteristics between post-polio patients and controls: A pilot study
Author: David Shoseyov, Tali Cohen-Kaufman, Isabella Schwartz, Sigal Portnoy,
Affiliation: Pediatric department, Hadassah Mount Scopus, Jerusalem, Israel
Physical Medicine and Rehabilitation department, Hadassah Mount Scopus, Jerusalem, Israel
Department of Physiotherapy, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Department of Occupational Therapy, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
National Yang-Ming University, TAIWAN
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: PLoS One. 2017; 12(7): e0182036.
Publication Year and Month: 2017 07

Abstract: Objectives
To compare pulmonary function measures, maximal respiratory pressure and fatigue of respiratory muscles between patients with Post-Polio Syndrome (PPS) and controls.

Design
Cross-sectional study.

Patients
Patients with PPS (N = 12; age 62.1±11.6 years) able to walk for 6 minutes without human assistance; age-matched controls with no history of polio or pulmonary dysfunction (N = 12; age 62.2±6.5 years).

Measurements
A body plethysmograph was used to quantify Residual Volume (RV), Total Lung Capacity (TLC), and Thoracic Gas Volume (TGV) etc. A manometer was used to measure Maximal Inspiratory Pressure (MIP) and Maximal Expiratory Pressure. A spirometer was used to measure Maximal Voluntary Ventilation (MVV). Surface electromyography (sEMG) recorded diaphragmatic muscle activity while performing MVV.

Results
The control group had significantly higher TGV and showed improvement in MIP following the effort (difference of 5.5±4.0cmH2O) while the PPS group showed deterioration in MIP (difference of -2.5±5.0cmH2O). Subjects with scoliosis had significantly higher RV/TLC values compared with subjects without scoliosis. The 25th frequency percentile of the sEMG signal acquired during MVV was reduced in the PPS group.

Conclusions: Maximal respiratory pressure test and sEMG measurements may identify fatigue of respiratory muscles in patients with PPS. Early diagnosis of respiratory impairment may delay respiratory decline and future need of invasive respiratory aids.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: 1246: Missed chronic respiratory failure in a post-polio syndrome patient
Author: Aljasmi M, Agarwal K, Uppalapati S, Bazan L
Affiliation: Not stated
Journal: Critical Care Medicine
Citation: Crit Care Med. 2015 Dec;43(12 Suppl 1):313
Publication Year and Month: 2015 12

Abstract: This is a brief Case Report which does not have an abstract. This is an extract:

INTRODUCTION: Prior to the introduction of the polio vaccine, paralytic poliomyelitis was a major cause of morbidity and death. Twenty-five to fifty percent of the survivors are known to develop post-polio syndrome. Symptoms include fatigue, insidious respiratory failure, obstructive sleep apnea, bulbar neuropathy, central ventilatory abnormalities, hemi-diaphragmatic paralysis and progressive functional decline with new onset weakness, among others. We present a case of post-polio syndrome presenting with hypercapnic respiratory failure.

Conclusions:

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Sleep-disordered breathing in neuromuscular disease
Author: Aboussouan LS
Affiliation: Respiratory Institute, Cleveland Clinic, Cleveland, Ohio
Journal: American Journal of Respiratory and Critical Care Medicine
Citation: Am J Respir Crit Care Med. 2015 May 1;191(9):979-89. doi: 10.1164/rccm.201412-2224CI
Publication Year and Month: 2015 05

Abstract: Sleep-disordered breathing in neuromuscular diseases is due to an exaggerated reduction in lung volumes during supine sleep, a compromised physiologic adaptation to sleep, and specific features of the diseases that may promote upper airway collapse or heart failure. The normal decrease in the rib cage contribution to the tidal volume during phasic REM sleep becomes a critical vulnerability, resulting in saw-tooth oxygen desaturation possibly representing the earliest manifestation of respiratory muscle weakness. Hypoventilation can occur in REM sleep and progress into non-REM sleep, with continuous desaturation and hypercarbia. Specific characteristics of neuromuscular disorders, such as pharyngeal neuropathy or weakness, macroglossia, bulbar manifestations, or low lung volumes, predispose patients to the development of obstructive events. Central sleep-disordered breathing can occur with associated cardiomyopathy (e.g., dystrophies) or from instability in the control of breathing due to diaphragm weakness. Mitigating factors such as recruitment of accessory respiratory muscles, reduction in REM sleep, and loss of normal REM atonia in some individuals may partially protect against sleep-disordered breathing. Noninvasive ventilation, a standard-of-care management option for sleep-disordered breathing, can itself trigger specific sleep-disordered breathing events including air leaks, patient-ventilator asynchrony, central sleep apnea, and glottic closure. These events increase arousals, reduce adherence, and impair sleep architecture. Polysomnography plays an important role in addressing pitfalls in the diagnosis of sleep-disordered breathing in neuromuscular diseases, identifying sleep-disordered breathing triggered by noninvasive ventilation, and optimizing noninvasive ventilation settings.

Conclusions:

Outcome of Research: Effective.

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Category: Respiratory Complications and Management

Title: Feasibility of lung volume recruitment in early neuromuscular weakness: a comparison between amyotrophic lateral sclerosis, myotonic dystrophy, and postpolio syndrome
Author: Kaminska M (1,2), Browman F (3), Trojan DA (4), Genge A (4), Benedetti A (2,5), Petrof BJ (6,7)
Affiliation: (1) Respiratory Division, McGill University Health Centre, 1001 Decarie Blvd, D05.2504 Montreal, Quebec, Canada H4A 3J1; (2) Respiratory Epidemiology and Clinical Research Unit, McGill University, Montreal, Quebec, Canada; (3) Department of Respiratory Therapy, Montreal Chest Institute, McGill University Health Centre, Montreal, Quebec, Canada; (4) Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University Health Centre, Montreal, Quebec, Canada; (5) Departments of Medicine and Epidemiology, Biostatistics & Occupational Health, McGill University Health Centre, Montreal, Quebec, Canada; (6) Meakins-Christie Laboratories, McGill University and Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada; (7) Respiratory Division, McGill University Health Centre, 1001 Decarie Blvd, D05.2506 Montreal, Quebec, Canada H4A 3J1
Journal: PM&R: The Journal of Injury, Function, and Rehabilitation
Citation: PM R. 2015 Apr;1-8
Publication Year and Month: 2015 04

Abstract: BACKGROUND: Lung volume recruitment (LVR) is a cough assistance technique used in persons with neuromuscular disorders (NMDs), most typically in those requiring noninvasive ventilation (NIV). Whether it may be useful in persons with NMDs who have milder respiratory impairment is unknown.

OBJECTIVE: To assess the feasibility, impact on quality of life (QOL), and preliminary physiological effects of daily LVR in different categories of persons with NMDs who have an early stage of respiratory impairment.

DESIGN: Feasibility study.

SETTING: Academic tertiary care center.

PARTICIPANTS: Outpatients diagnosed with amyotrophic lateral sclerosis (n = 8), postpolio syndrome (n = 10), and myotonic dystrophy (n = 6) who had restrictive respiratory defects but were not yet using NIV.

METHODS: Participants were asked to perform LVR up to 4 times daily and log their LVR use in a diary. Physiological measurements and questionnaires were completed at baseline and after 3 months.

MAIN OUTCOME MEASUREMENTS: Compliance with LVR use was assessed, along with QOL and willingness to continue the treatment. Physiological measurements included forced vital capacity (FVC), lung insufflation capacity (LIC), and the LIC minus FVC difference.

RESULTS: Of the 24 recruited subjects, 7 with amyotrophic lateral sclerosis, 7 with postpolio syndrome, and 5 with myotonic dystrophy completed the study (n = 19). At baseline, mean values for FVC and spontaneous peak cough flow were 59.9% predicted and 373.1 L/min, respectively. For subjects completing the study, 74% were willing to continue long-term LVR use, and QOL scores were not adversely affected by LVR in any NMD subgroup. The LIC-FVC difference increased from baseline to follow-up by a mean of 0.243 L (P = .006) in all subjects (n = 19), suggesting a possible improvement in respiratory system mechanics.

Conclusions: In patients with NMDs who have early restrictive respiratory defects but do not yet require NIV, regular use of LVR is feasible with no negative impact on QOL over a 3-month period and may have physiological benefits. Further work is needed to determine whether early institution of LVR can improve respiratory system mechanics and help delay ventilatory failure in persons with NMDs.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Lung function in post-poliomyelitis syndrome: a cross-sectional study
Author: Lira CABD, Minozzo FC, Sousa BS, Vancini RL, Andrade MDS, Quadros AAJ, Oliveira ASB, Silva ACD.
Affiliation: Federal University of Goiás at Jataí, Jataí, Brazil - [email protected]
Journal: Jornal Brasileiro de Pneumologia (Brazilian Journal of Pulmonology)
Citation: J Bras Pneumol. 2013 Jun-Aug; 39(4): 455–460. doi: 10.1590/S1806-37132013000400009
Publication Year and Month: 2013 08

Abstract: OBJECTIVE: To compare lung function between patients with post-poliomyelitis syndrome and those with sequelae of paralytic poliomyelitis (without any signs or symptoms of post-poliomyelitis syndrome), as well as between patients with post-poliomyelitis syndrome and healthy controls.

METHODS: Twenty-nine male participants were assigned to one of three groups: control; poliomyelitis (comprising patients who had had paralytic poliomyelitis but had not developed post-poliomyelitis syndrome); and post-poliomyelitis syndrome. Volunteers underwent lung function measurements (spirometry and respiratory muscle strength assessment).

RESULTS: The results of the spirometric assessment revealed no significant differences among the groups except for an approximately 27% lower mean maximal voluntary ventilation in the post-poliomyelitis syndrome group when compared with the control group (p = 0.0127). Nevertheless, the maximal voluntary ventilation values for the post-poliomyelitis group were compared with those for the Brazilian population and were found to be normal. No significant differences were observed in respiratory muscle strength among the groups.

Conclusions: With the exception of lower maximal voluntary ventilation, there was no significant lung function impairment in outpatients diagnosed with post-poliomyelitis syndrome when compared with healthy subjects and with patients with sequelae of poliomyelitis without post-poliomyelitis syndrome. This is an important clinical finding because it shows that patients with post-poliomyelitis syndrome can have preserved lung function.

Outcome of Research: Not applicable.

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Category: Respiratory Complications and Management

Title: Protocol for diaphragm pacing in patients with respiratory muscle weakness due to motor neurone disease (DiPALS): a randomised controlled trial.
Author: McDermott C, Maguire C, Cooper CL, Ackroyd R, Baird WO, Baudouin S, Bentley A, Bianchi S, Bourke S, Bradburn MJ, Dixon S, Ealing J, Galloway S, Karat D, Maynard N, Morrison K, Mustfa N, Stradling J, Talbot K, Williams T, Shaw PJ.
Affiliation: Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, 385A Glossop Road, Sheffield, S10 2HQ, UK.
Journal: BioMed Central Neurology
Citation: 2012 Aug 16;12:74.
Publication Year and Month: 2012 08

Abstract: BACKGROUND:
Motor neurone disease (MND) is a devastating illness which leads to muscle weakness and death, usually within 2-3 years of symptom onset. Respiratory insufficiency is a common cause of morbidity, particularly in later stages of MND and respiratory complications are the leading cause of mortality in MND patients. Non Invasive Ventilation (NIV) is the current standard therapy to manage respiratory insufficiency. Some MND patients however do not tolerate NIV due to a number of issues including mask interface problems and claustrophobia. In those that do tolerate NIV, eventually respiratory muscle weakness will progress to a point at which intermittent/overnight NIV is ineffective. The NeuRx RA/4 Diaphragm Pacing System was originally developed for patients with respiratory insufficiency and diaphragm paralysis secondary to stable high spinal cord injuries. The DiPALS study will assess the effect of diaphragm pacing (DP) when used to treat patients with MND and respiratory insufficiency.

METHOD/DESIGN:
108 patients will be recruited to the study at 5 sites in the UK. Patients will be randomised to either receive NIV (current standard care) or receive DP in addition to NIV. Study participants will be required to complete outcome measures at 5 follow up time points (2, 3, 6, 9 and 12 months) plus an additional surgery and 1 week post operative visit for those in the DP group. 12 patients (and their carers) from the DP group will also be asked to complete 2 qualitative interviews.

DISCUSSION:
The primary objective of this trial will be to evaluate the effect of Diaphragm Pacing (DP) on survival over the study duration in patients with MND with respiratory muscle weakness. The project is funded by the National Institute for Health Research, Health Technology Assessment (HTA) Programme (project number 09/55/33) and the Motor Neurone Disease Association and the Henry Smith Charity.

Conclusions:

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Bi-level positive airway pressure ventilation maintains adequate ventilation in post-polio patients with respiratory failure.
Author: Gillis-Haegerstrand C, Markström A, Barle H.
Affiliation: Department of Anaesthesiology and Intensive Care, Karolinska Institutet, Danderyd Hospital, Stockholm, Sweden. [email protected]
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Acta Anaesthesiologica Scandinavica 2006 May;50(5):580-5
Publication Year and Month: 2006 05

Abstract: BACKGROUND:
Patients suffering from post-polio syndrome still contribute significantly to the number of patients with chronic respiratory failure requiring home mechanical ventilation (HMV). Many of these patients are treated either with invasive (tracheostomy) or non-invasive (nasal mask) controlled mechanical ventilation i.e. volume-controlled ventilation (VCV). In this group of patients, we have previously shown that bi-level pressure support ventilation (bi-level PSV) decreases the oxygen cost of breathing. The aim of this study was to compare the effect of bi-level PSV, with special regard to the adequacy of ventilation and the oxygen cost of breathing, during the patients' ordinary VCV and spontaneous breathing.

METHODS:
Eight post-polio patients on nocturnal VCV were investigated. Five of them were tracheostomized and three of them used a nasal mask. Work of breathing was analysed by assessing differences in oxygen consumption (VO2) using indirect calorimetry. Blood gases were obtained regularly to assess adequacy of ventilation.

RESULTS:
Bi-level PSV decreases the oxygen cost of breathing in post-polio patients with respiratory failure without decreasing ventilation efficiency. Furthermore, PaCO2 decreased significantly using this mode of ventilation (P < 0.05).

Conclusions: In this study, it was shown that bi-level PSV reduces the oxygen cost of breathing and gave a significant decrease in PaCO2 in PPS patients. These data suggest that bi-level PSV ventilation maintains adequate ventilation in patients who suffer from post-polio syndrome with respiratory failure.

Outcome of Research: More research required

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Category: Respiratory Complications and Management

Title: Pulmonary dysfunction and its management in post-polio patients
Author: John R. Bach, Margaret Tiltona.


Affiliation: Department of Physical Medicine and Rehabilitation; UMDNJ-New Jersey Medical School, Director of the Center for Ventilator Management Alternatives, University Hospital, Newark.
Journal: NeuroRehabilitation
Citation: NeuroRehabilitation 8 (1997) 139-153
Publication Year and Month: 1997

Abstract: Respiratory dysfunction is extremely common and entails considerable risk of morbidity and mortality for individuals with past poliomyelitis. Although it is usually primarily due to respiratory muscle weakness, post-poliomyelitis individuals also have a high incidence of scoliosis, obesity, sleep disordered breathing, and bulbar muscle dysfunction. Although these factors can result in chronic alveolar hypoventilation (CAH) and frequent pulmonary complications and hospitalizations, CAH is usually not recognized until acute respiratory failure complicates an otherwise benign upper respiratory tract infection. The use of non-invasive inspiratory and expiratory muscle aids, however, can decrease the risk of acute respiratory failure, hospitalizations for respiratory complications, and need to resort to tracheal intubation. Timely introduction of non-invasive intermittent positive pressure ventilation (IPPV), manually assisted coughing, and mechanical insufflation-exsufflation (MI-E) and non-invasive blood gas monitoring which can most often be performed in the home setting, are the principle interventions for avoiding complications and maintaining optimal quality of life.

Conclusions: The failure to make timely appropriate management decisions often leads to episodes of acute respiratory failure and unnecessary hospitalizations, endotracheal intubations, bronchoscopies, and tracheostomies. The use of these invasive approaches adversely affects quality of life and can increase the risk of pulmonary complications and mortality for post-poliomyelitis patients [47]. Episodes of acute respiratory failure which most often result from otherwise benign upper respiratory tract infections can be reversed by the timely use of non-invasive respiratory muscle aids.

Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Airway Secretion Clearance by Mechanical Exsufflation for Post-Poliomyelitis Ventilator-Assisted Individuals
Author: John R. Bach, William H. Smith, Jennifier Michaels, Lou Saporito, Augusta S. Alba, Rajeev Dayal, Jeffrey Pan.
Affiliation:
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil Vol 74:170-177, February 1993.
Publication Year and Month: 1992 03

Abstract: Pulmonary complications from impaired airway secretion clearance mechanisms are major causes of morbidity and mortality for post-poliomyelitis individuals. The purpose of this study was to review the long-term use of manually assisted coughing and mechanical insufflation-exsufflation (MI-E) by post-poliomyelitis ventilator-assisted individuals (PVAIs) and to compare the peak cough expiratory flows (PCEF) created during unassisted and assisted coughing. Twenty-four PVAIs who have used noninvasive methods of ventilatory support for an average of 27 years, relied on methods of manually assisted coughing and/or MI-E without complications during intercurrent respiratory tract infections (RTIs). Nine of the 24 individuals were studied for PCEF. They had a mean forced vital capacity (FVC) of 0.54 ± 0.47L and a mean maximum insufflation capacity achieved by air stacking of ventilator insufflations and glossopharyngeal breathing of 1.7L. The PCEF were as follows: unassisted, 1.78 ± 1.16L/sec; following a maximum assisted insufflation, 3.75 ± 0.73L/sec; with manual assistance by abdominal compression following a maximum assisted insufflation, 4.64 ± 1.42L/sec; and with MI-E, 6.97 ± 0.89L/sec. We conclude that manually assisted coughing and MI-E are effective and safe methods of airway secretion clearance for PVAIs with impaired expiratory muscle function who would otherwise be managed by endotracheal suctioning. Severely decreased maximum insufflation capacity but not vital capacity indicate need for a tracheostomy.

Conclusions: Post-poliomyelitis individuals using noninvasive methods of ventilatory support have greater PCEF produced by manually assisted coughing and by MI-E than can be produced by unassisted coughing. Thus, if intubation and tracheostomy are to be avoided, it becomes crucial for these individuals and their caregivers to learn and have access to these methods, particularly during RTIs when expiratory muscle weakness is exacerbated.[55] These principles may also apply to individuals with severe expiratory muscle weakness due to other neuromuscular conditions. We believe that it is largely because of the lack of general knowledge of and access to MI-E that other centers are more likely to resort to intubation and tracheostomy for patients with advanced neuromuscular disease and RTIs. We conclude that loss of FVC in itself does not mandate the need for intubation or tracheostomy for ventilatory support. A maximum insufflation capacity below 500mL may be a better indicator. MI-E should be further explored as a convenient and probably more effective alternative to endotracheal suctioning for ventilator-supported neuromuscular individuals.

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Category: Respiratory Complications and Management

Title: Pulmonary Dysfunction and Sleep Disordered Breathing as Post-Polio Sequelae: Evaluation and Management
Author: John R. Bach, MD and Augusta S. Alba, MD
Affiliation: New Jersey Medical School of the University of Medicine and Dentistry of New Jersey, and the Department of Physical Medicine and Rehabilitation, University Hospital; Department of Physical Rehabilitation Medicine, New York University and Godwater Memorial Hospital, New York, NY.
Journal: Orthopedics
Citation: Orthopedics December 1991 Vol 14 No 12 1329-1337.
Publication Year and Month: 1991

Abstract: Post-polio sequelae can include sleep disordered breathing and chronic alveolar hypoventilation (CAH). Both conditions develop insidiously and can render the post-polio survivor susceptible to cardiopulmonary morbidity and mortality when not treated in a timely and appropriate manner. These conditions can be diagnosed by a combination of spirometry, noninvasive blood gas monitoring, and ambulatory polysomnography. Sleep disordered breathing is most frequently managed by nasal continuous positive airway pressure, while tracheostomy ventilation is the most common treatment for ventilatory failure. We report the more effective and comfortable techniques recently made available for managing sleep disordered breathing and the use of noninvasive treatment options for CAH, respiratory failure, and impaired airway clearance mechanisms. One hundred forty-three cases are reviewed.

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Outcome of Research: Not applicable

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Category: Respiratory Complications and Management

Title: Poliomyelitis: late respiratory complications and management
Author: Fischer DA
Affiliation: Not stated
Journal: Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):891-4
Publication Year and Month: 1985 07

Abstract: One hundred forty-six respiratory polios have been reviewed for complications and current respiratory aids. One hundred thirty-eight of these people required respirator assistance at the onset of their poliomyelitis infection. Fifteen patients have kyphoscoliosis. Seventy-five percent of the total patients require some assisted ventilation. Fifty-two percent have tracheostomies. Most of these people have been followed at Rancho Los Amigos Medical Center for many years. The literature regarding late respiratory complications of polio is reviewed.

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Outcomes of Research or Clinical Trials Activity Levels Acute Flaccid Paralysis Ageing Anaerobic Threshold Anaesthesia Assistive Technology Brain Cardiorespiratory Cardiovascular Clinical Evaluation Cold Intolerance Complementary Therapies Continence Coping Styles and Strategies Cultural Context Diagnosis and Management Differential Diagnosis Drugs Dysphagia Dysphonia Epidemiology Exercise Falls Fatigue Fractures Gender Differences Immune Response Inflammation Late Effects of Polio Muscle Strength Muscular Atrophy Orthoses Pain Polio Immunisation Post-Polio Motor Unit Psychology Quality of Life Renal Complications Respiratory Complications and Management Restless Legs Syndrome Sleep Analaysis Surgery Vitality Vocational Implications