Evaluation and Management of Dyspnea in Hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorder (HSD)
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 34
- 试验地点
- 2
- 主要终点
- Maximal Inspiratory Pressure
研究概览
简要总结
Individuals with hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorders (HSD) often experience dyspnea. Inspiratory Muscle Training (IMT) has been shown to improve dyspnea and respiratory muscle function; however, the impact of IMT in combination with whole-body exercise training on respiratory muscle strength, dyspnea, and daily function remains unknown. The objectives of this research are i) to evaluate dyspnea, respiratory muscle strength and function, ventilatory parameters, and health-related quality of life (HRQL) in individuals with hEDS and HSD compared to healthy age and sex-matched controls, ii) to explore the contributors to dyspnea during exercise with a specific focus on respiratory muscle structure and function in hEDS and HSD patients and healthy controls, and iii) to assess whether the combination of IMT and whole-body exercise training will be more effective than whole-body exercise training alone in improving participant outcomes in hEDS and HSD participants. It is hypothesized that i) hEDS and HSD participants will have lower respiratory muscle strength, higher peripheral airway resistance, lower HRQL, and higher anxiety and depression levels compared to healthy controls, ii) the contributors to increased exercise induced dyspnea will include decreased respiratory muscle strength, increased airway resistance, and greater prefrontal cortical neural activity, and iii) the combination of IMT and whole-body exercise training will be superior to whole-body training alone for improving dyspnea, respiratory muscle strength and endurance, aerobic capacity, HRQL, anxiety, and depression.
详细描述
Thirty-four hEDS and HSD participants will be recruited from the University Health Network (Toronto, Canada). Participants will have baseline evaluations of dyspnea, pulmonary function tests (i.e., oscillometry, spirometry, and lung volumes), respiratory muscle structure and function (i.e., diaphragm ultrasound and respiratory muscle strength), HRQL, anxiety and depression. Symptom limited cardiopulmonary exercise testing will be utilized to quantify exertional dyspnea using the 10-item Borg dyspnea scale, 18-point qualitative dyspnea assessment scale, peak oxygen uptake (peak VO2), and assessment of dynamic ventilatory parameters. Neural activity in the pre-frontal cortex will be measured during cardiopulmonary exercise testing via functional near-infrared spectroscopy. These baseline measures will be compared to 17 age and sex-matched healthy controls. After baseline assessments, hEDS and HSD participants will be randomized to one of the following groups: i) Usual Care plus IMT: 8 weeks of whole-body exercises (standard care provided to hEDS and HSD patients at the University Health Network) plus a concurrent IMT intervention 5 times per week); ii) Usual Care: 8 weeks of whole-body exercises (as outlined above). After four and eight weeks, participants will have repeat assessments as described below.
Exertional dyspnea is a prevalent and troublesome symptom experienced by individuals living with hEDS and HSD. This study will provide a better understanding of the underlying contributors of dyspnea in hEDS and HSD patients, with a focus on respiratory muscle structure and function, prefrontal cortical neural activity, and ventilatory parameters, both at rest and with exertion.
研究设计
- 研究类型
- Interventional
- 分配方式
- Randomized
- 干预模型
- Parallel
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Adult participants (≥ 18 years of age)
- •New referral to the GoodHope Exercise and Rehabilitation (GEAR) Program at the University Health Network
- •Diagnosis of hEDS based on the 2017 diagnostic criteria or diagnosis of HSD (defined as generalized joint hypermobility, chronic pain, and systemic findings to suspect connective tissue underpinnings) with clinical verification by the EDS medical team
排除标准
- •Genetic testing confirming the diagnosis of another type of EDS (i.e., vascular EDS, classical EDS)
- •Any contraindication to exercise testing (i.e., unstable cardiac disease). Presence of cardiac pacemaker/implantable defibrillator or structural cardiac abnormalities on echocardiogram
- •Recent respiratory infection (< 1 month) or known diagnosis of obstructive (i.e., asthma, chronic obstructive pulmonary disease) or restrictive parenchymal lung disease
- •History of pneumothorax, otitis media (fluid behind the ear drum), or rupture of tympanic membranes given risk with IMT
- •Recent participation in formal exercise training or IMT program (within the last 3 months)
- •Persistent symptoms or difficulty tolerating IMT (i.e., breathing difficulties and/or chest pain)
- •Diagnosis of severe autonomic dysfunction or postural orthostatic tachycardia syndrome limiting daily physical activity or exercise
- •Neuromuscular disease (i.e., myositis, diaphragm paralysis) that may interfere with IMT
- •Insufficient English fluency to provide informed consent or ability to follow study protocols
- •Self-reported pregnancy
- •Inability to connect to the internet
结局指标
主要结局
Maximal Inspiratory Pressure
时间窗: Change from baseline at 8 weeks
Maximal inspiratory pressure will be evaluated using standard methods in the pulmonary function laboratory, with the highest value (cm H2O) taken within 10 percent of the other readings as per American Thoracic Society recommendations.
次要结局
- Inspiratory Muscle Training Load(Through study completion, an average of 8 weeks)
- Inspiratory Muscle Training Mean Power(Through study completion, an average of 8 weeks)
- Health-Related Quality of Life(Change from baseline at 8 weeks)
- Participant Study Recruitment(Over the study period (8 weeks))
- Respiratory Muscle Endurance(Change from baseline at 4 weeks and 8 weeks)
- Lung Volumes (Residual Volume)(Change from baseline at 8 weeks)
- Maximal Expiratory Pressure(Change from baseline at 8 weeks)
- Borg Dyspnea Scale(Change from baseline at 8 weeks)
- Oscillometry(Change from baseline at 8 weeks)
- Spirometry (Forced Vital Capacity)(Change from baseline at 8 weeks)
- Spirometry (Forced Expiratory Volume in 1 Second)(Change from baseline at 8 weeks)
- Lung Volumes (Total Lung Capacity)(Change from baseline at 8 weeks)
- Diaphragm Thickening Fraction(Change from baseline at 8 weeks)
- Peak Oxygen Uptake During Cardiopulmonary Exercise Testing(Change from baseline at 8 weeks)
- Ventilatory Response During Cardiopulmonary Exercise Testing(Change from baseline at 8 weeks)
- Pre-frontal Cortical Neural Activity During Cardiopulmonary Exercise Testing(Change from baseline at 8 weeks)
- Inspiratory Muscle Training Workload(Through study completion, an average of 8 weeks)
- Inspiratory Muscle Training Mean Volume(Through study completion, an average of 8 weeks)
- Medical Research Council Dyspnea Scale(Change from baseline at 8 weeks)
- Qualitative Assessment of Dyspnea(Change from baseline at 4 weeks and 8 weeks)
- Heart Rate Response During Cardiopulmonary Exercise Testing(Change from baseline at 8 weeks)
- Physical Activity Levels(Change from baseline at 4 weeks and 8 weeks)
- Participant Retention in the Study(Over the study period (8 weeks))
- Lung Volumes (Inspiratory Capacity)(Change from baseline at 8 weeks)
- Diaphragm Thickness(Change from baseline at 8 weeks)
- Leisure-Time Exercise(Baseline)
- Depression, Anxiety and Stress Scale(Change from baseline at 8 weeks)
- Adherence to Inspiratory Muscle Training Days(Over the intervention period (8 weeks))
- Adverse Events During Inspiratory Muscle Training Practice (Safety and Tolerability)(Over the intervention period (8 weeks))
- Adherence to Inspiratory Muscle Training Load(Over the intervention period (8 weeks))
- Participant Satisfaction with the IMT Intervention(Change from baseline at weeks 1, 4 and 8)