Inspiratory Muscle Training in Patients With Duchenne Muscular Dystrophy

Wanke, T.; Toifl, K.; Merkle, Monika; Formanek, D.; Lahrmann, Heinz; Zwick, Hartmut

doi:10.1378/chest.105.2.475

Cited by 121 publications

(120 citation statements)

References 29 publications

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“…As well as in patients with obstructive lung disease, there is a theoretical rationale for strengthening the inspiratory muscles of any patient who experiences inappropriate breathlessness, abnormal respiratory mechanics and/or inspiratory muscle weakness/fatigue. Furthermore, there are a small number of randomised, controlled trials in which IMT has elicited a positive effect upon neuromuscular diseases [23,24] as well as less rigorously designed and executed studies in conditions such as kyphoscoliosis [10]. Although the number of our patients is small, the efficacy of preoperative respiratory training using Threshold IMT is shown clinically in this study.…”

Section: Discussionmentioning

confidence: 85%

Surgical management of severe scoliosis with high-risk pulmonary dysfunction in Duchenne muscular dystrophy

Takaso

Nakazawa

Imura

et al. 2009

International Orthopaedics (SICOT)

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Between 2005 and 2007, 14 patients who had severe scoliosis in Duchenne muscular dystrophy (DMD) and a poor forced vital capacity (FVC) of <30% at admission underwent scoliosis surgery. FVC on admission was 21.6% (range, 16-27%). The patients were given respiratory muscle training using a pulmonary trainer (Threshold IMT, Philips Respironics, Inc.) for six weeks before operation. FVC increased to 26.2% (range, 22-31%) the day before operation. The mean preoperative scoliosis was 98°(range, 81°-130°). All patients underwent posterior fusion and allscrew construction and were extubated on the operative day. No patients developed any respiratory complications. The postoperative scoliosis was 34°(range, 20°-40°) (65%). FVC remained stable at six weeks after operation. FVC decreased to 19.8% (range, 16-25%) and the mean scoliosis was 35°(range, 23°-40°)(64%) at two years after operation. DMD patients with severe scoliosis and FVC considered too low to permit reasonable surgical risk could undergo surgery and could benefit from surgery.

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Section: Discussionmentioning

confidence: 85%

Surgical management of severe scoliosis with high-risk pulmonary dysfunction in Duchenne muscular dystrophy

Takaso

Nakazawa

Imura

et al. 2009

International Orthopaedics (SICOT)

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“…ESTRUP et al [14] and recently WANKE et al [11] showed improvements in strength and endurance of the inspiratory muscles in patients with progressive muscular dystrophy. Only the latter was a controlled study.…”

Section: In Ns Sp Pi Ir Ra At To or Ry Y Mmentioning

confidence: 97%

“…The results from other studies should be considered preliminary and interpreted with great caution. In general, most studies show improvements in strength or endurance of the inspiratory muscles [4][5][6][7][8][9][10][11]. The transfer to outcome variables relevant to patients, such as reduced dyspnoea, improved exercise tolerance, better performance of activities of daily living (ADL), reduced postoperative pulmonary complications, or successful weaning from mechanical ventilation, is less obvious.…”

Section: In Ns Sp Pi Ir Ra At To or Ry Y Mmentioning

confidence: 99%

Inspiratory muscle training: where are we?

Gosselink¹,

Decramer²

1994

Eur Respir J

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“…However, some patients with hypercapnia or a FVC < 25% had no improvement. 104 Similarly, Koessler et al assessed the effect of inspiratory muscle training in patients with DMD and spinal muscular atrophy. 105 The training consisted of breathing maneuvers against variable inspiratory resistance for endurance, and of maximal inspiratory efforts against a nearly occluded resistance for strength training.…”

Section: Respiratory Muscle Trainingmentioning

confidence: 99%

“…69,[71][72][73]128 In selecting patients for an exercise program of respiratory muscle training, strength training or aerobic exercise, one should consider the patients' limitations. For example, inspiratory muscle training may not be beneficial in patients who are already hypercapnic, 70,104 or have a vital capacity < 25% predicted, 104 and cardiovascular training may be difficult to achieve in patients with significant limb weakness. Additionally, several of the neuromuscular disorders, the dystrophies in particular, may be associated with cardiac impairment, and training in those conditions should proceed with caution and close supervision.…”

Section: Exercise Prescriptionmentioning

confidence: 99%

Mechanisms of exercise limitation and pulmonary rehabilitation for patients with neuromuscular disease

Aboussouan

2009

Chron Respir Dis

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Indications for exercise and pulmonary rehabilitation extend to neuromuscular diseases tough these conditions pose particular challenges given the associated skeletal muscle impairment and respiratory muscle dysfunction. These challenges are compounded by the variety of exercise prescriptions (aerobic, muscle strengthening, and respiratory muscle training) and the variety of neuromuscular disorders (muscular, motor neuron, motor nerve root, and neuromuscular transmission disorders). Studies support a level II evidence of effectiveness (i.e., likely to be effective) for a combination of aerobic exercise and strengthening exercises in muscular disorders, and for strengthening exercises in amyotrophic lateral sclerosis. The potential deleterious effects of work overload in the dystrophinopathies have not been confirmed in Becker muscular dystrophy. Adjunctive pharmacologic interventions (e.g., theophylline, steroids, PDE5 inhibitors, creatine), training recommendations (e.g., interval or lower intensity training) and supportive techniques (e.g., noninvasive ventilation, neuromuscular electrical stimulation, and diaphragm pacing) may result in more effective training but require more study before formal recommendations can be made. The exercise prescription should include avoidance of inspiratory muscle training in hypercapnia or low vital capacity, and should match the desired outcome (e.g., extremity training for task-specific performance, exercise training to enhance exercise performance, respiratory muscle training where respiratory muscle involvement contributes to the impairment).

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Inspiratory Muscle Training in Patients With Duchenne Muscular Dystrophy

Cited by 121 publications

References 29 publications

Surgical management of severe scoliosis with high-risk pulmonary dysfunction in Duchenne muscular dystrophy

Surgical management of severe scoliosis with high-risk pulmonary dysfunction in Duchenne muscular dystrophy

Inspiratory muscle training: where are we?

Mechanisms of exercise limitation and pulmonary rehabilitation for patients with neuromuscular disease

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