Background Purpose Methods Formation of Consensus Committee Methodologies for Synthesizing Expert Consensus Evaluation and Anticipatory Guidance of the Patient with DMD Routine Evaluation of Respiratory Function End of Life Directives Nutrition Sleep Evaluation Cardiac Involvement Management Airway Clearance Respiratory Muscle Training Noninvasive Nocturnal Ventilation Daytime Noninvasive Ventilation Continuous Invasive Ventilation Scoliosis in DMD Corticosteroids in Management of DMD Patient Education Long-term Care Issues End of Life Care

Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement.

Evidence-based recommendations on the clinical use of cardiopulmonary exercise testing (CPET) in lung and heart disease are presented, with reference to the assessment of exercise intolerance, prognostic assessment and the evaluation of therapeutic interventions (e.g. drugs, supplemental oxygen, exercise training). A commonly used grading system for recommendations in evidence-based guidelines was applied, with the grade of recommendation ranging from A, the highest, to D, the lowest. For symptom-limited incremental exercise, CPET indices, such as peak O(2) uptake (V'O(2)), V'O(2) at lactate threshold, the slope of the ventilation-CO(2) output relationship and the presence of arterial O(2) desaturation, have all been shown to have power in prognostic evaluation. In addition, for assessment of interventions, the tolerable duration of symptom-limited high-intensity constant-load exercise often provides greater sensitivity to discriminate change than the classical incremental test. Field-testing paradigms (e.g. timed and shuttle walking tests) also prove valuable. In turn, these considerations allow the resolution of practical questions that often confront the clinician, such as: 1) "When should an evaluation of exercise intolerance be sought?"; 2) "Which particular form of test should be asked for?"; and 3) "What cluster of variables should be selected when evaluating prognosis for a particular disease or the effect of a particular intervention?"

https://erj.ersjournals.com/content/erj/29/1/185.full.pdf

Recommendations on the use of exercise testing in clinical practice

Background: Exercise-induced bronchoconstriction (EIB) describes acute airway narrowing that occurs as a result of exercise. EIB occurs in a substantial proportion of patients with asthma, but may also occur in individuals without known asthma.Methods: To provide clinicians with practical guidance, a multidisciplinary panel of stakeholders was convened to review the pathogenesis of EIB and to develop evidence-based guidelines for the diagnosis and treatment of EIB. The evidence was appraised and recommendations were formulated using the Grading of Recommendations, Assessment, Development, and Evaluation approach.Results: Recommendations for the treatment of EIB were developed. The quality of evidence supporting the recommendations was variable, ranging from low to high. A strong recommendation was made for using a short-acting β2-agonist before exercise in all patients with EIB. For patients who continue to have symptoms of EIB despite the administration of a short-acting β2-agonist before exercise, stron...

/pdf/an-official-american-thoracic-society-clinical-practice-2ev9b7wn92.pdf

An official American Thoracic Society clinical practice guideline: exercise-induced bronchoconstriction.

Clinical exercise testing with reference to lung diseases : indications, standardization and interpretation strategies

Deterioration of respiratory function in patients with neuromuscular disorders is primarily responsible for the high mortality associated with these diseases. A review of Duchenne muscular dystrophy and spinal muscular atrophy, the leading neuromuscular disorders affecting children, will be followed by a critical analysis of the various pathophysiological mechanisms underlying respiratory manifestations in these patients. Among such mechanisms, the role of muscular weakness in preservation of lung function, mucociliary clearance, gas exchange at rest and during exercise, and respiratory control during wakefulness and sleep will be examined in detail. In addition, the potential benefits of respiratory muscle training and of early diagnosis and clinical intervention will be delineated. This review underscores the importance of periodic assessment of pulmonary function during wakefulness and sleep in children affected by neuromuscular diseases as an essential component of multidisciplinary care aimed at improving long-term morbidity, survival, and quality of life.

Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy

Computerized respiratory muscle training in children with Duchenne muscular dystrophy

Recent studies have shown some evidence that exercise-induced asthma (EIA) may not be entirely explained by respiratory heat loss (RHL). We investigated the interrelationship between heat exchange, exercise intensity and EIA. In order to differentiate between the effects of RHL and exercise intensity, we arranged for tests to be performed with the same RHL, but with different intensities of exercise and inspired air conditions. Each of 8 asthmatic children exercised twice in random order for 6 min on a cycle ergometer. One test consisted of exercise performed at a greater level of effort while breathing room air, mean (+/- SE) air conditions being 25.0 +/- 0.4 degrees C and 15.7 +/- 0.2 mg H2O/L. The other test was performed at a lesser level of effort while breathing cold (0.0 +/- 0.5 degrees C) and dry air (O mg H2O/L). The mean ratio of minute ventilations in the 2 exercise tests was 1.78 +/- 0.03, but the RHL was similar in both tests. The EIA after the exercise at the greater level was more severe than after the lesser level, the percent fall in FEV2 from baseline being 36 +/- 7% and 21 +/- 5%, respectively (p less than 0.025). We conclude that the exercise level has a major role in determining the severity of EIA and that climatic conditions act as modifying factors.

Exercise intensity determines and climatic conditions modify the severity of exercise-induced asthma.

Thirteen children each exercised for 6 min by running on a treadmill and by tethered swimming, breathing air at room temperature and either 8% or 99% relative humidity continuously. Ventilation, gas exchange and heart rate were closely matched in all four tests in each child, with a mean oxygen consumption of 32.3 +/- 1.7 ml x min-1 x kg-1. The post-exercise fall in FEV1 expressed as a percentage of the baseline FEV1 (delta FEV1) was significantly greater after running compared with swimming breathing either humid or dry air. The delta FEV1 was also related to respiratory heat loss (RHL) calculated from measurements of inspired and expired gas temperature and humidity. At a standardised RHL, the difference between running and swimming was highly significant [delta FEV1 (%) +/- SE = 39 +/- 5 and 28 +/- 4 respectively, p less than 0.01]. These experiments suggest that the type of exercise influences the severity of exercise-induced asthma even under conditions of the same metabolic stress and respiratory heat loss.

Differences between swimming and running as stimuli for exercise-induced asthma.

To compare the refractory period that follows exercise and isocapnic hyperventilation, 10 asthmatic children performed two pairs of challenge tests in random order at least six hours apart. In pair A a hyperventilation challenge was followed by an exercise challenge and in pair B the order was reversed. Both pairs of tests were done while the children were breathing cold dry air. Tests were matched in terms of work load, ventilation, and end tidal carbon dioxide tension (PCO2). The mean percentage fall in FEV1 (delta FEV1) after the first challenge (hyperventilation) of pair A and the first challenge (exercise) of pair B were the same (30% (SEM 2%)) and 30% (4%) respectively). The mean delta FEV1 of the exercise test following hyperventilation in pair A and of hyperventilation following exercise in pair B was 22% (4%) and 18% (4%) respectively. Both these latter results were significantly lower than the respective delta FEV1 when the challenge was the first test of the pair. Although the mean refractoriness index (reduction in induced asthma in the second test of each pair compared with the first test) was greater when exercise was the first challenge, the difference was not significant.

https://thorax.bmj.com/content/thoraxjnl/38/11/849.full.pdf

Ilan Gur

Papers

Computerized respiratory muscle training in children with Duchenne muscular dystrophy

Exercise intensity determines and climatic conditions modify the severity of exercise-induced asthma.

Differences between swimming and running as stimuli for exercise-induced asthma.

Refractory period following induced asthma: contributions of exercise and isocapnic hyperventilation.

Vital signs analysis algorithm detects inflammatory response in premature infants with late onset sepsis and necrotizing enterocolitis.