Respiratory Muscle Training in Duchenne Muscular Dystrophy

Martin, Andrew; Stern, L. M.; Yeates, J.; Lepp, D.; Little, James P.

doi:10.1111/j.1469-8749.1986.tb03879.x

Cited by 49 publications

(22 citation statements)

References 11 publications

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“…A common issue with both IOPD and LOPD is progressive restrictive ventilatory failure (1,26). Several forms of respiratory muscle exercise training have been successfully described in MD (27)(28)(29). More recently, several studies have shown that respiratory exercise training is helpful in PD (30)(31)(32).…”

Section: Introductionmentioning

confidence: 99%

Nutrition and exercise in Pompe disease

Tarnopolsky¹,

Nilsson²

2019

Ann. Transl. Med

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The current standard of care for Pompe disease (PD) is the administration of enzyme replacement therapy (ERT). Exercise and nutrition are often considered as complementary strategies rather than "treatments" per se. Nutritional assessment is important in patients with locomotor disability because the relative hypodynamia limits energy expenditure and thus the total amount of energy must be reduced to avoid obesity. A lower total energy intake often leads to lower protein and micronutrient intake. Consequently, ensuring that Pompe patients are tested for and replaced for deficiencies (protein, vitamin D, vitamin B12, etc.) is an important aspect of care. Furthermore, given the role of autophagy in the pathophysiology of PD and the fact that fasting induces autophagy, it is important that strategies such as nutritional timing and amino acid intake (L-arginine, L-leucine) be evaluated as therapies. Exercise interventions have been shown to improve six-minute walk testing distance by more than what was seen in the seminal ERT study in late-onset PD. Exercise therapy can also activate autophagy, and this is likely another component of its efficacy. The current review will evaluate the theoretical and practical aspects of nutrition and exercise as therapies for patients with PD.

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Section: Introductionmentioning

confidence: 99%

Nutrition and exercise in Pompe disease

Tarnopolsky¹,

Nilsson²

2019

Ann. Transl. Med

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“…Previous ments in both strength and endurance were possible. Our own pilot study (Martin et al 1986) suggested that respiratory muscle training could improve endurance, but not strength or vital capacity. In that study improved endurance was based on the time that resistance to airflow could be tolerated, whereas in the present trial the Nickerson valve provided a more objective means of measuring endurance.…”

Section: Discussionmentioning

confidence: 93%

“…The physiotherapeutic management of these respiratory problems have included breathing exercises and 'blowing' games (Houser andJohnson 1971, Siege1 1975)' but there was little objective evidence that these measures had a significant effect on respiratory function or on eventual outcome. In order to assess whether it was possible to increase respiratory muscle strength and endurance by respiratory muscle training, we carried out a pilot project involving 18 boys with Duchenne dystrophy (Martin et al 1986). It showed that there was significant improvement in ventilatory muscle endurance, but not in strength.…”

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confidence: 99%

Training Inspiratoryresistance in Duchenne Dystrophy Using Adapted Computer Games

Stern¹,

Martin

Jones

et al. 1989

Develop Med Child Neuro

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SUMMARY Computer games were modified to encourage respiratory effort by two groups of patients with Duchenne muscular dystrophy. One group trained during the first six months of the study, the other during the second six months. Inspiratory effort was increased by their having to breathe through a mask to both start and continue the games. Pulmonary function tests were done at the beginning of the trial and six, 12 and 18 months later, when forced vital capacity, respiratory muscle endurance and strength were measured. Although there were some individual improvements, over‐all the results were not statistically significant. RÉSUMÉ Entrainement á la resistance inspiratoire chez les myopathes de Duchenne, par jeux d'ordinateur adaptés Des jeux d'ordinateur ont été modifies pour encourager l'effort respiratoire dans deux groupes de myopathes de Duchenne. Un groupe fut entrainé durant les six premiers mois de l'étude, l'autre durant les seconds six mois. L'effort inspiratoire fut accru par une respiration à travers un masque, pour débuter et poursuivre les jeux. Les tests de fonction pulmonaire furent effectués au début de l'apprentissage et six, 12 et 18 mois plus tard, comprenant la capacité vitale maximale, 1'endurance des muscles respiratoires et leur force. Bien qu'il y ait eu quelques améliorations individuelles, les résultats globaux ne furent pas statistiquement significatifs. ZUSAMMENFASSUNG Training der inspiratorischen Resistenz bei Patienten mit Duchenne'scher Muskeldystrophie unter Verwendung von dafiir entwickelten Computerspielen Mit Hilfe von modifizierten Computerspielen wurde bei zwei Gruppen von Patienten mit Duchenne'scher Muskeldystrophie versucht, die Atemanstrengungen zu fördern. Eine Gruppe trainierte während der ersten sechs Monate der Studie und die andere während der zweiten sechs Monate. Die inspiratorischen Anstrengungen wurden dadurch erhöht, daß sie durch eine Maske atmen mußten, um die Spiele zu starten und zu spielen. Die Lungenfunktionstests wurden zu Beginn der Untersuchung und sechs, 12 und 18 Monate später, als auch die forcierte Vitalkapazität, die Atemmuskelausdauer und‐stärke gemessen wurden, durchgeführt. Obwohl einige individuelle Verbesserungen verzeichnet werden konnten, waren die Ergebnisse nicht statistisch signifikant. RESUMEN Entrenamiemto de la resistencia inspiratoria en pacientes con distrofia de Duchenne usando juegos de computadora adaptados Se modificaron juegos de computadora para facilitar el esfuerzo respiratorio en dos grupos de pacientes con distrofia muscular de Duchene. Un grupo se entrenó durante los primeros seis meses del estudio y el otro durante los siguientes seis meses. El esfuerzo inspiratorio aumentó al tener que respirar a través de una máscara para iniciar y continuar los juegos. Se hicieron pruebas de la función pulmonar al inicio del aprendizaje y a los seis, 12 y 18 meses después, midiendo la capacidad vital, la resistencia de los músculos respiratorios y su fuerza. Aunque hubo algunas mejorias individuates, en conjunto los resultados no fu...

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“…164 Several other authors also failed to induce gains of lung volumes in response to such training. [165][166][167][168] Possibly, the training period was too short. Winkler and associates 32 had 16 patients with neuromuscular dystrophy (13 with Duchenne dystrophy) undertake 9 months of home training, using a variable inspiratory resistance.…”

Section: Duchenne Muscular Dystrophymentioning

confidence: 99%

Lifestyle and the Respiratory Health of Children

Shephard

2010

American Journal of Lifestyle Medicine

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This article offers a review of the potential influences of personal lifestyle on respiratory health in children, looking at both healthy individuals and those with respiratory disorders. As with many aspects of health, regular physical activity, an appropriate diet, and avoidance of obesity and cigarette smoke all contribute to optimal development of the healthy child. An active lifestyle is associated with greater static and dynamic lung volumes, greater efficiency of the ventilatory process, and an optimization of breathing patterns. The risk of upper respiratory infections is also reduced in those maintaining a moderate level of physical activity. Maternal smoking during pregnancy, as well as active and passive smoking, all have an adverse influence on lung function in the child, the largest effects being on dynamic lung volumes. The risk of developing asthma seems reduced in children who maintain a normal body mass and are physically active. A program of graded physical activity is of therapeutic value in a number of established respiratory conditions, including asthma, cystic fibrosis, and ventilatory impairment from neuromuscular disorders. Exercise carries a slight risk of fatalities from asthma and anaphylactic reactions. In designing an optimal physical activity program, it is also important to guard against the hazards of deep oronasal breathing, including the precipitation of bronchospasm by the inhalation of cold, dry air and pollens; an increased exposure to atmospheric pollutants (reducing and oxidant smog, fine and ultra-fine particulates, and carbon monoxide); and possible long-term dangers from chlorine derivatives in the atmosphere of indoor swimming pools.

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Respiratory Muscle Training in Duchenne Muscular Dystrophy

Cited by 49 publications

References 11 publications

Nutrition and exercise in Pompe disease

Nutrition and exercise in Pompe disease

Training Inspiratoryresistance in Duchenne Dystrophy Using Adapted Computer Games

Lifestyle and the Respiratory Health of Children

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