Respiratory-Swallow Coordination Training and Voluntary Cough Skill Training: A Single-Subject Treatment Study in a Person With Parkinson's Disease.

Purpose Airway protective disorders are common in Parkinson's disease (PD), yet effective methods to rehabilitate these life-threatening impairments are limited. This study examined the effects of two skill-based treatments aimed at improving swallowing and cough in a severely dysphagic person with PD: respiratory-swallow coordination training (RSCT) and voluntary cough skill training (VCST). It was hypothesized that (a) RSCT would improve respiratory-swallow coordination and swallowing safety and efficiency and (b) VCST would improve reflex and voluntary cough effectiveness. Method An 81-year-old man with midstage PD and severe dysphagia was recruited for study participation. The study utilized a multiple-baseline ABACA experimental design with a 2-month delayed retention assessment. Measures of respiratory-swallow coordination, swallowing safety and efficiency, and cough effectiveness were collected at each assessment using respiratory inductive plethysmography, flexible endoscopic evaluations of swallowing, and spirometry. Data were analyzed descriptively using baseline corrected tau and standard mean difference effect sizes (d). Results Large effect sizes were observed immediately following RSCT for respiratory-swallow coordination (d = 9.17), penetration-aspiration (d = 12.88), vallecular residue (d = 1.75), piriform residue (d = 4.15), and overall dysphagia severity (d = 1.83). Large effect sizes were also observed immediately following VCST for single voluntary cough (d = 4.30), sequential voluntary cough (d = 3.28), and reflex cough (d = 5.58). Improvements were maintained 2 months later for all outcome measures except single voluntary cough. Discussion This is the first study to examine the effects of RSCT and VCST in a person with PD. Robust improvements in respiratory-swallow coordination and swallowing safety and efficiency were achieved following four sessions of RSCT, and significant improvements in reflex and voluntary cough strength were seen following four sessions of VCST. Future work is needed to study these treatments in larger cohorts of people with PD.

Minimal Clinically Important Differences for Patient-Reported Outcome Measures of Cough and Sputum in Patients with COPD.

Background: Cough and sputum are highly prevalent in patients with chronic obstructive pulmonary disease (COPD). Pulmonary rehabilitation (PR) has shown to be effective in managing these symptoms. However, the interpretation of the magnitude of PR effects is hindered by the lack of minimal clinically important differences (MCIDs). Purpose: This study established MCIDs for the Leicester cough questionnaire (LCQ) and the cough and sputum assessment questionnaire (CASA-Q), in patients with COPD after PR. Patients and Methods: An observational prospective study was conducted in patients with COPD who participated in a 12-weeks community-based PR program. Anchor- (mean change, receiver operating characteristic curves and linear regression analysis) and distribution-based methods [0.5*standard deviation; standard error of measurement (SEM); 1.96*SEM; minimal detectable change and effect size] were used to compute the MCIDs. The anchors used were: i) patients and physiotherapists global rating of change scale, ii) COPD assessment test, iii) St. George's respiratory questionnaire and iv) occurrence of an exacerbation during PR. Pooled MCIDs were computed using the arithmetic weighted mean (2/3 for anchor- and 1/3 for distribution-based methods). Results: Forty-nine patients with COPD (81.6% male, 69.8±7.4years, FEV150.4±19.4%predicted) were used in the analysis. The pooled MCIDs were 1.3 for LCQ and for CASA-Q domains were: 10.6 - cough symptoms; 10.1 - cough impact; 9.5 - sputum symptoms and 7.8 - sputum impact. Conclusion: The MCIDs found in this study are potential estimates to interpret PR effects on cough and sputum, and may contribute to guide interventions.

The effects of respiratory muscle training on peak cough flow in patients with Parkinson's disease: a randomized controlled study.

OBJECTIVE: To compare the effects of an inspiratory versus and expiratory muscle-training program on voluntary and reflex peak cough flow in patients with Parkinson disease. DESIGN: A randomized controlled study. SETTING: Home-based training program. PARTICIPANTS: In all, 40 participants with diagnosis of Parkinson's disease were initially recruited in the study and randomly allocated to three study groups. Of them, 31 participants completed the study protocol (control group, n = 10; inspiratory training group, n = 11; and expiratory training group, n = 10) Intervention: The inspiratory and expiratory group performed a home-based inspiratory and expiratory muscle-training program, respectively (five sets of five repetitions). Both groups trained six times a week for two months using a progressively increased resistance. The control group performed expiratory muscle training using the same protocol and a fixed resistance. MAIN MEASURES: Spirometric indices, maximum inspiratory pressure, maximum expiratory pressure, and peak cough flow during voluntary and reflex cough were assessed before and at two months after training. RESULTS: The magnitude of increase in maximum expiratory pressure ( d = 1.40) and voluntary peak cough flow ( d = 0.89) was greater for the expiratory muscle-training group in comparison to the control group. Reflex peak cough flow had a moderate effect ( d = 0.27) in the expiratory group in comparison to the control group. Slow vital capacity ( d = 0.13) and forced vital capacity ( d = 0.02) had trivial effects in the expiratory versus the control group. CONCLUSIONS: Two months of expiratory muscle-training program was more beneficial than inspiratory muscle-training program for improving maximum expiratory pressure and voluntary peak cough flow in patients with Parkinson's disease.

Participation in a randomised controlled feasibility study of a complex intervention for the management of the Respiratory Symptom Distress Cluster in lung cancer: patient, carer and research staff views.

This paper reports finding from a nested qualitative study designed to elicit the views and perceptions of those who participated in a randomised controlled feasibility trial testing a non-pharmacological intervention, Respiratory Distress Symptom Intervention (RDSI), for the management of the breathlessness-cough-fatigue symptom cluster in lung cancer. Semi-structured interviews were conducted with 11 lung cancer patients, three caregivers and seven researchers involved in recruitment, consent, RDSI training and delivery and participant follow-up. Thematic analysis identified key considerations including: the importance of informed consent emphasising commitment to completion of paperwork and raising awareness of potential sensitivities relating to content of questionnaires; ensuring screening for the presence of symptoms reflects the language used by patients; appreciation of the commitment required from participants to learn intervention techniques and embed them as part of everyday life; conduct of interviews with patients who decline to participate; and conduct of serial interviews with those receiving RDSI to further inform its routine implementation into clinical practice. This study will inform the development of a fully powered follow-on trial testing the hypothesis that RDSI plus usual care is superior to usual care alone in the effective management of this symptom cluster in lung cancer.

Rehabilitation of Swallowing and Cough Functions Following Stroke: An Expiratory Muscle Strength Training Trial.

OBJECTIVE: To determine the effect of expiratory muscle strength training (EMST) on both cough and swallow function in stroke patients. DESIGN: Prospective pre-post intervention trial with 1 participant group. SETTING: Two outpatient rehabilitation clinics. PARTICIPANTS: Adults (N=14) with a history of ischemic stroke in the preceding 3 to 24 months. INTERVENTION: EMST. The training program was completed at home and consisted of 25 repetitions per day, 5 days per week, for 5 weeks. MAIN OUTCOME MEASURES: Baseline and posttraining measures were maximum expiratory pressure, voluntary cough airflows, reflex cough challenge to 200µmol/L of capsaicin, sensory perception of urge to cough, and fluoroscopic swallow evaluation. Repeated measures and 1-way analyses of variance were used to determine significant differences pre- and posttraining. RESULTS: Maximum expiratory pressure increased in all participants by an average of 30cmH2O posttraining. At baseline, all participants demonstrated a blunted reflex cough response to 200µmol/L of capsaicin. After 5 weeks of training, measures of urge to cough and cough effectiveness increased for reflex cough; however, voluntary cough effectiveness did not increase. Swallow function was minimally impaired at baseline, and there were no significant changes in the measures of swallow function posttraining. CONCLUSIONS: EMST improves expiratory muscle strength, reflex cough strength, and urge to cough. Voluntary cough and swallow measures were not significantly different posttraining. It may be that stroke patients benefit from the training for upregulation of reflex cough and thus improved airway protection.

Exploratory mixed methods study of respiratory physiotherapy for patients with lower respiratory tract infections.

OBJECTIVES: To assess the outcomes of respiratory physiotherapy for patients with lower respiratory tract infections (LRTI). DESIGN: Parallel group mixed-methods study. SETTING: Patients were recruited from a general hospital. Respiratory physiotherapy took place in a community setting. PARTICIPANTS: Fifty-four patients aged ≥18 years and diagnosed with LRTI completed the study. Twenty-seven patients were allocated to the control group {CG: 10 male, mean age 53.3 [standard deviation (SD) 17.4] years} and 27 patients were allocated to the experimental group [EG: 10 male, mean age 58.6 (SD 17.2) years]. INTERVENTION: The CG received conventional medical treatment and the EG received conventional medical treatment plus respiratory physiotherapy for 3 weeks. OUTCOME MEASURES: Patients in both groups undertook the 6-minute walk test (6MWT), modified Borg scale (MBS), modified Medical Research Council questionnaire (mMRC), and Breathlessness, Cough and Sputum scale (BCSS) before and after the intervention. A telephone follow-up survey was performed 3 months after the first hospital visit. Interviews were conducted immediately after the intervention in the EG. RESULTS: In the EG, the distance walked in the 6MWT increased by more than the minimally important difference (P=0.001), and significantly more than the CG {EG: mean change 76m [standard deviation (SD) 63], 95% confidence interval (CI) 51 to 101; CG: mean change 27m (SD 56), 95% CI 5 to 49; mean difference between groups: 49m 95% CI 16 to 82; partial η(2)=0.15}. No differences in the MBS, mMRC and BCSS were found between the two groups. The EG reported high levels of satisfaction with the intervention (27/27; 100%) and with the physiotherapist (20/27; 74%). The intervention improved patients' symptoms (19/27; 70%) and their self-management skills to control/prevent future LRTI (19/27; 70%). Health service use was significantly less in the EG (P=0.04). CONCLUSIONS: Respiratory physiotherapy appears to be effective for the management of patients with LRTI. CLINICALTRIAL. GOV REGISTRATION NUMBER: NCT02053870.

Efficacy of a respiratory rehabilitation exercise training package in hospitalized elderly patients with acute exacerbation of COPD: a randomized control trial.

CLINICAL TRIALS IDENTIFIER: NCT02329873. BACKGROUND: Acute exacerbation (AE) of COPD is characterized by a sudden worsening of COPD symptoms. Previous studies have explored the effectiveness of respiratory rehabilitation for patients with COPD; however, no training program specific to acute exacerbation in elderly patients or unstable periods during hospitalization has been developed. OBJECTIVE: To evaluate the effects of a respiratory rehabilitation exercise training package on dyspnea, cough, exercise tolerance, and sputum expectoration among hospitalized elderly patients with AECOPD. METHODS: A randomized control trial was conducted. Pretest and posttest evaluations of 61 elderly inpatients with AECOPD (experimental group n=30; control group n=31) were performed. The experimental group received respiratory rehabilitation exercise training twice a day, 10-30 minutes per session for 4 days. The clinical parameters (dyspnea, cough, exercise tolerance, and sputum expectoration) were assessed at the baseline and at the end of the fourth day. RESULTS: All participants (median age =70 years, male =60.70%, and peak expiratory flow 140 L) completed the study. In the patients of the experimental group, dyspnea and cough decreased and exercise tolerance and sputum expectoration increased significantly compared with those of the patients in the control group (all P<0.05). Within-group comparisons revealed that the dyspnea, cough, and exercise tolerance significantly improved in the experimental group by the end of the fourth day (all P<0.05). CONCLUSION: Results of this study suggest that the respiratory rehabilitation exercise training package reduced symptoms and enhanced the effectiveness of the care of elderly inpatients with AECOPD.

Air stacking: effects on pulmonary function in patients with spinal muscular atrophy and in patients with congenital muscular dystrophy.

OBJECTIVE: Respiratory complications are the main causes of morbidity and mortality in patients with neuromuscular disease (NMD). The objectives of this study were to determine the effects that routine daily home air-stacking maneuvers have on pulmonary function in patients with spinal muscular atrophy (SMA) and in patients with congenital muscular dystrophy (CMD), as well as to identify associations between spinal deformities and the effects of the maneuvers. METHODS: Eighteen NMD patients (ten with CMD and eight with SMA) were submitted to routine daily air-stacking maneuvers at home with manual resuscitators for four to six months, undergoing pulmonary function tests before and after that period. The pulmonary function tests included measurements of FVC; PEF; maximum insufflation capacity (MIC); and assisted and unassisted peak cough flow (APCF and UPCF, respectively) with insufflations. RESULTS: After the use of home air-stacking maneuvers, there were improvements in the APCF and UPCF. In the patients without scoliosis, there was also a significant increase in FVC. When comparing patients with and without scoliosis, the increases in APCF and UPCF were more pronounced in those without scoliosis. CONCLUSIONS: Routine daily air-stacking maneuvers with a manual resuscitator appear to increase UPCF and APCF in patients with NMD, especially in those without scoliosis.

Air stacking: effects on pulmonary function in patients with spinal muscular atrophy and in patients with congenital muscular dystrophy, / Efeitos do treinamento de empilhamento de ar na função pulmonar de pacientes com amiotrofia espinhal e distrofia muscular congênita

OBJECTIVE: Respiratory complications are the main causes of morbidity and mortality in patients with neuromuscular disease (NMD). The objectives of this study were to determine the effects that routine daily home air-stacking maneuvers have on pulmonary function in patients with spinal muscular atrophy (SMA) and in patients with congenital muscular dystrophy (CMD), as well as to identify associations between spinal deformities and the effects of the maneuvers. METHODS: Eighteen NMD patients (ten with CMD and eight with SMA) were submitted to routine daily air-stacking maneuvers at home with manual resuscitators for four to six months, undergoing pulmonary function tests before and after that period. The pulmonary function tests included measurements of FVC; PEF; maximum insufflation capacity (MIC); and assisted and unassisted peak cough flow (APCF and UPCF, respectively) with insufflations. RESULTS: After the use of home air-stacking maneuvers, there were improvements in the APCF and UPCF. In the patients without scoliosis, there was also a significant increase in FVC. When comparing patients with and without scoliosis, the increases in APCF and UPCF were more pronounced in those without scoliosis. CONCLUSIONS: Routine daily air-stacking maneuvers with a manual resuscitator appear to increase UPCF and APCF in patients with NMD, especially in those without scoliosis. .

OBJETIVO: As complicações respiratórias são as principais causas de morbidade e mortalidade em pacientes com doenças neuromusculares (DNM). Os objetivos deste estudo foram determinar os efeitos que o treinamento diário domiciliar com manobras de empilhamento de ar tem na função respiratória de pacientes com amiotrofia espinhal (AE) e distrofia muscular congênita (DMC), e identificar possíveis associações entre deformidades na coluna vertebral e os efeitos das manobras. MÉTODOS: Dezoito pacientes com DNM (dez com DMC e oito com AE) foram submetidos a treinamento diário domiciliar de empilhamento de ar com ressuscitador manual por um período de quatro a seis meses e submetidos a testes de função pulmonar antes e após tal período. Os testes de função pulmonar incluíram medidas de CVF, PFE, a capacidade de insuflação máxima (CIM) e a medida do pico de fluxo de tosse não assistido e assistido (PFTNA e PFTASS, respectivamente). RESULTADOS: Após o uso das manobras de empilhamento de ar no domicílio, houve uma melhora significativa na PFTNA e PFTASS. Nos pacientes sem escoliose, houve também um aumento significativo na CVF. No grupo de pacientes sem escoliose, o ganho na PFTNA e PFTASS foi superior ao do grupo com escoliose. CONCLUSÕES: A utilização rotineira diária de manobras de empilhamento de ar com ressuscitador manual parece melhorar a PFTNA e PFTASS em pacientes com DNM, especialmente naqueles sem escoliose. .

Independent cough flow augmentation by glossopharyngeal breathing plus table thrust in muscular dystrophy.

OBJECTIVE: The purpose of the present study was to compare the unassisted cough peak flow (CPF) of patients affected by muscular dystrophy with CPF augmented by various techniques, including maximal depth glossopharyngeal breathing (GPB) combined with a subsequent self-induced thoracic or abdominal thrust. DESIGN: All of the motorized wheelchair-dependent patients with muscular dystrophy who had previously mastered GPB were trained at home to increase their cough efficacy. This training involved maneuvering their wheelchair against the edge of a specially built table to autonomously produce a thoracic and/or abdominal thrust timed to the opening of the glottis for an independently assisted cough. Both unassisted and variously assisted CPFs were compared. RESULTS: The 18 patients (17 men/1 woman) with muscular dystrophy, aged 21.1 ± 5.4 yrs, achieved variously assisted CPFs that were significantly higher than the spontaneous CPF (P < 0.001), with assisted CPFs but not unassisted CPFs that significantly exceeded a reported efficacious cough threshold value of 160 liters/min (P < 0.001). Moreover, increases in the CPFs by personal assistance including air stacking by manual resuscitator and thoracoabdominal thrust (326.4 ± 79.5 liters/min) or by GPB and thoracoabdominal thrust (326.4 ± 87.5 liters/min) were not significantly different (P = 0.07) from the CPFs independently attained by GPB plus independently maneuvering a wheelchair for a table thrust (310.3 ± 74.7 liters/min). CONCLUSIONS: The independently assisted (GPB plus table thrust) CPF was comparable to the CPFs that required personal assistance for air stacking and abdominal thrusts. Therefore, for patients with muscular dystrophy, this physical medicine technique and cough-assisted techniques that require personal intervention are strongly recommended.

Eficácia da eletroestimulação muscular expiratória na tosse de pacientes após acidente vascular encefálico / Effectiveness of electrical stimulation in expiratory muscle on cough of patients after stroke

Indivíduos com sequelas de acidente vascular encefálico (AVE) apresentam como complicação comum a hipotonia de tronco com consequente fraqueza da sua musculatura, em especial, o reto abdominal que é o principal músculo acionado para a tosse. As correntes elétricas têm sido amplamente utilizadas para recrutamento de fibras musculares nas mais diversas patologias. O presente estudo teve como objetivo avaliar a eficácia da eletroestimulação transcutânea de média frequência (ETMF) na força muscular expiratória e tosse de pacientes com sequela de AVE. Trata-se de um estudo quase-experimental (antes e depois) onde foram selecionados 11 indivíduos pós-AVE entre 40 a 65 anos, ambos os sexos e estáveis hemodinamicamente. Os pacientes foram avaliados quanto a parâmetros clínicos e pneumofuncionais (ventilometria, pico de fluxo expiratório (PFE) e manovacuometria) e submetidos ao protocolo, que constava de ETMF no músculo reto abdominal com o aparelho de corrente Russa, frequência portadora de 2.500 Hz modulada a 40 Hz, durante 15 minutos, por 15 sessões. Na avaliação inicial observou-se diminuição da força muscular inspiratória e expiratória (Pimáx; Pemáx) e do PFE quando comparados aos valores de normalidade preditos na literatura. Após a ETMF houve incremento da Pimáx e Pemáx sem significância estatística (p=0,18 e p=0,29) já o PFE teve um acréscimo de 283,73 L/minuto para 347,27 L/minuto (p=0,03). Pode-se observar que a ETMF foi eficaz no incremento dos parâmetros avaliados, com o PFE sendo o de maior impacto e significância estatística, no entanto, mais estudos com populações maiores se fazem necessários para análise dessa nova abordagem terapêutica.

Individuals with sequelae of stroke present as a common complication hypotonia and weakness of trunk muscles, in special the rectus abdominal muscle which is the main muscle activated for cough. The electrical stimulation (ES) is being widely used to fiber recruitment in many muscle groups and pathologies. The present study aimed to evaluate the efficiency of electrical stimulation with median frequencies (ETMF) in expiratory muscles strength and cough in patients with sequels of stroke. It is a quasi-experimental study (before and after) where 11 individuals were selected after stroke between 40 and 65 years, both sexes and hemodynamically stable. Patients were evaluated about clinic and respiratory scores (ventilometer, peak of expiratory flow (PEF) and manometer (MIP and MEP) and submitted to the protocol, which consisted of ETMF in expiratory muscle (abdominals rectus) with the unit of Russian current with 2,500 Hz carrier frequency modulated at 40 Hz for 15 minutes by 15 sessions. In the initial assessment evidenced decreasing in inspiratory and expiratory muscular strength (MIP, MEP) and PEF when compared to predicted values in literature. In the end of ETMF it happened an increase in MIP, MEP without statistical significance (p=0.18 and p=0.29) but the PEF has had an increase of 283.73 L/minute to 347.27 L/minute (p=0.03). It can be seen that the ETMF was effective in the improvement of the parameters evaluated, with PEF being the greatest impact and statistical significance, however further studies with larger populations are necessary to analyze this new therapeutic approach.

Chronic aspiration without pulmonary complications after partial laryngectomy: long-term follow-up of two cases.

We report the long-term history of two patients with oropharyngeal dysphagia subsequent to partial resection of the larynx (supraglottic and subtotal laryngectomy, respectively). Both fiberendoscopic and videofluoroscopy swallowing studies demonstrated aspiration of liquids and solids in the lower respiratory tract. The patients underwent a swallowing rehabilitation program and respiratory exercises to improve the efficacy of their cough, and both continued oral feeding despite their persistent dysphagia. They were followed annually over the subsequent 12 and 9 years, respectively, which included overseeing the clinical status of their bronchopulmonary apparatus (i.e., whether a productive cough was present or not), nutritional status, spirometric parameters, arterial blood gas analysis, pulse oximetry, and measurement of cough effectiveness. They also underwent serial videofluoroscopy swallowing studies, which demonstrated persistent tracheobronchial aspiration of both liquids and solids. Based on the absence of respiratory hospitalizations as well as pulmonary deterioration during the follow-up period, we discuss the importance of cough effectiveness and its enhancement as a possible protective factor to avoid pulmonary morbidity in chronic aspiration.

Validation of a parent-proxy quality of life questionnaire for paediatric chronic cough (PC-QOL).

BACKGROUND: Quality of life (QOL) measures are an important patient-relevant outcome measure for clinical studies. Currently there is no fully validated cough-specific QOL measure for paediatrics. The objective of this study was to validate a cough-specific QOL questionnaire for paediatric use. METHOD: 43 children (28 males, 15 females; median age 29 months, IQR 20-41 months) newly referred for chronic cough participated. One parent of each child completed the 27-item Parent Cough-Specific QOL questionnaire (PC-QOL), and the generic child (Pediatric QOL Inventory 4.0 (PedsQL)) and parent QOL questionnaires (SF-12) and two cough-related measures (visual analogue score and verbal category descriptive score) on two occasions separated by 2-3 weeks. Cough counts were also objectively measured on both occasions. RESULTS: Internal consistency for both the domains and total PC-QOL at both test times was excellent (Cronbach alpha range 0.70-0.97). Evidence for repeatability and criterion validity was established, with significant correlations over time and significant relationships with the cough measures. The PC-QOL was sensitive to change across the test times and these changes were significantly related to changes in cough measures (PC-QOL with: verbal category descriptive score, r(s)=-0.37, p=0.016; visual analogue score, r(s)=-0.47, p=0.003). Significant correlations of the difference scores for the social domain of the PC-QOL and the domain and total scores of the PedsQL were also noted (r(s)=0.46, p=0.034). CONCLUSION: The PC-QOL is a reliable and valid outcome measure that assesses QOL related to childhood cough at a given time point and measures changes in cough-specific QOL over time.

[Clinical aspects of rehabilitation potential in patients with chronic obstructive pulmonary disease].

AIM: To reveal and evaluate clinical signs influencing rehabilitation potential in COPD patients. MATERIAL AND METHODS: Clinical findings in COPD patients were analysed at medical and social expert examination (MSEE). RESULTS: Progression of the pathological process is associated with aggravation of chest pain, fever, sputum discharge at coughing. Cough and dyspnea occurred in all the examinees irrespective of the disease etiology. Severity of respiratory failure correlated with severity of COPD. Respiratory and circulatory failure aggravate quality of life. The study determined clinical signs in patients with COPD of various etiology which influence rehabilitation potential of COPD patients. This helped specification of disability criteria and certification. Conclusion. In MSEE of COPD patients it is necessary to base on the patient's complaints, duration of the disease, severity of functional respiratory and circulation failure, complications, social problems.

[Non-invasive ventilation: possibilities and limitations in patients with reduced ability to cough]. / Nichtinvasive Beatmung: Möglichkeiten und Grenzen bei eingeschränkter Hustenfunktion.

BACKGROUND: Neuromuscular diseases cover a wide range of pathologies, which are slowly or rapidly progressive. Besides the reduced capacity of the inspiratory muscles there is a limitation of expiration which compromises coughing. Ventilatory respiratory insufficiency (VRI) develops in stages, beginning during sleep with hypercapnia manifesting later on. Several retrospective studies have shown an improved alveolar ventilation, survival and quality of life for most NMD, even in non-bulbar ALS. The initiation of NIV is seen as apropriate when VRI in sleep develops. But patient acceptance to use NIV for more than 4 hours must be taken into account. NIV is limited by the interface which may cause skin abrasions or leakage due to pressure. NIV may be used day and night in stable conditions and with assistance experienced in mechanically-assisted coughing (MAC). The discussion of whether to perform tracheostomy remains a preeminent topic especially in ALS. CONCLUSION: NIV is effective in NMD and improves and prolongs life in slowly progressive NMD. Mask problems, leakage and bulbar dysfunction can limit the effectiveness. MAC is a tool that is very useful for elimination of bronchial secretions.

[Assisted cough--physiotherapy to improve expectoration of mucus]. / Assisted cough--Physiotherapie zur Verbesserung der Sekretexpektoration.

Pulmonary complications are the most common causes of mortality in patients with severe inspiratory and/or expiratory muscle weakness. An inspiratory tidal volume below 1500 ml and a peak cough flow below 160 L/min result in mucus retention and increase the risk of pneumonia. An intact cough function is pivotal for airway clearance during acute and chronic airway infections with increased mucus production as well as for protection against endotracheal aspirations. Effective cough requires that all of its phases work or are effectively supported. Non-invasive assistance of inspiration can be provided by manual hyperinflation, air stacking, glossopharyngeal breathing or mechanical insufflation. Safe and effective methods of expiration assistance include manual thorax and abdominal compression, manual self-assistance as well as mechanical insufflation-exsufflation. The use of these non-invasive inspiratory and expiratory muscle aids can decrease the risk of pulmonary complications in patients with severe ventilatory muscle weakness.

[NIV--modes and prognosis]. / NIV--Modi und Prognose.

Non-invasive (NIV) as well as invasive mechanical ventilation are established for the treatment of ventilatory failure in neuromuscular diseases. The EUROVENT study provides data about mechanical ventilation at home. In this survey there is a trend towards pressure-derived ventilation in these patient groups. Volume-cycled ventilators had been used in 41% patients with neuromuscular diseases. During the past decade a remarkable change towards pressure-cycled ventilation has occurred. Two recently published, randomised cross-over studies demonstrated equal efficacy of volume-cycled ventilation compared to pressure-cycled ventilation in terms of gas exchange and sleep efficacy in chronic ventilatory failure. In both groups the median survival times were equal. In respiratory muscle failure, assisted ventilation, assist/controlled ventilation as well as controlled ventilation are applied. Between 1977 and 2001, long-term survival improved in patients with Duchenne muscular dystrophy (DMD) in Denmark based on a strict organisation of care, home ventilation as standard treatment and the establishment of centres for home ventilation. The proportion of ventilated patients rose from 0.9% to 43.4% of all DMD patients. The median survival of an untreated DMD patient is 9.7 months. Simonds demonstrated a 5-year survival rate of 73% in these groups of patients under home mechanical ventilation. In patients with the more rapidly deteriorating amyotrophic lateral sclerosis, the efficacy of home ventilation is less impressive and bulbar involvement serves as an independent negative predictor.

Forced expiratory technique, directed cough, and autogenic drainage.

In health, secretions produced in the respiratory tract are cleared by mucociliary transport, cephalad airflow bias, and cough. In disease, increased secretion viscosity and volume, dyskinesia of the cilia, and ineffective cough combine to reduce secretion clearance, leading to increased risk of infection. In obstructive lung disease these conditions are further complicated by early collapse of airways, due to airway compression, which traps both gas and secretions. Techniques have been developed to optimize expiratory flow and promote airway clearance. Directed cough, forced expiratory technique, active cycle of breathing, and autogenic drainage are all more effective than placebo and comparable in therapeutic effects to postural drainage; they require no special equipment or care-provider assistance for routine use. Researchers have suggested that standard chest physical therapy with active cycle of breathing and forced expiratory technique is more effective than chest physical therapy alone. Evidence-based reviews have suggested that, though successful adoption of techniques such as autogenic drainage may require greater control and training, patients with long-term secretion management problems should be taught as many of these techniques as they can master for adoption in their therapeutic routines.

Conventional chest physical therapy for obstructive lung disease.

Chest physical therapy (CPT) is a widely used intervention for patients with airway diseases. The main goal is to facilitate secretion transport and thereby decrease secretion retention in the airways. Historically, conventional CPT has consisted of a combination of forced expirations (directed cough or huff), postural drainage, percussion, and/or shaking. CPT improves mucus transport, but it is not entirely clear which groups of patients benefit from which CPT modalities. In general, the patients who benefit most from CPT are those with airways disease and objective signs of secretion retention (eg, persistent rhonchi or decreased breath sounds) or subjective signs of difficulty expectorating sputum, and with progression of disease that might be due to secretion retention (eg, recurrent exacerbations, infections, or a fast decline in pulmonary function). The most effective and important part of conventional CPT is directed cough. The other components of conventional CPT add little if any benefit and should not be used routinely. Alternative airway clearance modalities (eg, high-frequency chest wall compression, vibratory positive expiratory pressure, and exercise) are not proven to be more effective than conventional CPT and usually add little benefit to conventional CPT. Only if cough and huff are insufficiently effective should other CPT modalities be considered. The choice between the CPT alternatives mainly depends on patient preference and the individual patient's response to treatment.

Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness.

Adults and children with neuromuscular disease exhibit weak cough and are susceptible to recurrent chest infections, a major cause of morbidity and mortality. Mechanical insufflation/exsufflation may improve cough efficacy by increasing peak cough flow. It was hypothesised that mechanical insufflation/exsufflation would produce a greater increase in peak cough flow than other modes of cough augmentation. The acceptability of these interventions was also compared. Twenty-two patients aged 10-56 yrs (median 21 yrs) with neuromuscular disease and 19 age-matched controls were studied. Spirometry was performed and respiratory muscle strength measured. Peak cough flow was recorded during maximal unassisted coughs, followed in random order by coughs assisted by physiotherapy, noninvasive ventilation, insufflation and exsufflation, and exsufflation alone. Subjects rated strength of cough, distress and comfort on a visual analogue scale. In the neuromuscular disease group, mean +/- SD forced expiratory volume in one second was 0.8 +/- 0.6 L x s(-1), forced vital capacity 0.9 +/- 0.8 L, maximum inspiratory pressure 25 +/- 16 cmH2O, maximum expiratory pressure 26 +/- 22 cmH2O and unassisted peak cough flow 169 +/- 90 L x min(-1). The greatest increase in peak cough flow was observed with mechanical insufflation/exsufflation at 235 +/- 111 L x min(-1) (p<0.01). All techniques showed similar patient acceptability. Mechanical insufflation/exsufflation produces a greater increase in peak cough flow than other standard cough augmentation techniques in adults and children with neuromuscular disease.