Back to resultsDyspnea in Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder
Primary Purpose
Hypermobile EDS (hEDS), Hypermobility Syndrome
Status
Recruiting
Phase
Not Applicable
Locations
Canada
Study Type
Interventional
Intervention
Usual Care plus Inspiratory Muscle Training (IMT)
Sponsored by
About this trial
This is an interventional treatment trial for Hypermobile EDS (hEDS) focused on measuring Hypermobile Ehlers Danlos Syndrome, Hypermobility Spectrum Disorder, Dyspnea, Inspiratory Muscle Training, Exercise
Eligibility Criteria
Inclusion Criteria:
- 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
Exclusion Criteria:
- 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
Sites / Locations
- University Health NetworkRecruiting
Arms of the Study
Arm 1
Arm 2
Arm Type
Experimental
No Intervention
Arm Label
Usual Care plus Inspiratory Muscle Training (IMT)
Usual Care
Arm Description
Participants will attend the standard of care rehabilitation program offered to hEDS and HSD patients at the University Health Network. The program consists of i) an individualized home-based rehabilitation and exercise program (twelve weeks of aerobic, neuromotor, and resistance-based exercises), ii) a self-management education intervention, and iii) a community resource engagement plan. Furthermore, patients attend four on-site sessions (a baseline assessment and three follow-up visits). Participants in this group will also be provided with a personalized prescription for an IMT program for eight weeks to be performed in the home environment (two daily IMT sessions of 30 breaths, five days per week). Participants will be virtually supervised by the study team weekly with any adverse events closely monitored. Participants will receive instructions and feedback on how to optimize their home training efforts with direct observation of their IMT practice.
Participants will participate in the standard of care rehabilitation program offered to hEDS and HSD patients at the University Health Network.
Outcomes
Primary Outcome Measures
Maximal Inspiratory Pressure
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.
Secondary Outcome Measures
Maximal Expiratory Pressure
Maximal expiratory 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.
Respiratory Muscle Endurance
Respiratory muscle endurance (endurance time measured in seconds) will be assessed using a manual threshold loading device (Philips Threshold trainer).
Inspiratory Muscle Training Workload
Total IMT workload per session (product of inspiratory muscle force and inhaled volume expressed in Joules) will be extracted from the IMT device in the experimental group.
Inspiratory Muscle Training Load
Inspiratory muscle training Load (cm H2O) will be abstracted from the IMT device in the experimental group.
Inspiratory Muscle Training Mean Power
Inspiratory muscle training mean power per breath (Watts) will be abstracted from the IMT device in the experimental group.
Inspiratory Muscle Training Mean Volume
Inspiratory muscle training mean volume per breath (Litres) will be abstracted from the IMT device in the experimental group.
Medical Research Council Dyspnea Scale
The Medical Research Council Dyspnea Scale will be used to assess the effect of breathlessness on daily activities. The scale comprises five statements that describe the range of respiratory dyspnea from none (Grade 1) to severe with difficulties performing self-care (Grade 5).
Borg Dyspnea Scale
The 10-point Borg Dyspnea Scale will be used throughout the study to adjust the intensity of inspiratory muscle training sessions The lowest score (0) corresponds to the sensation of normal breathing (absence of dyspnea) and the highest score (10) corresponds to the maximum possible sensation of dyspnea.
Borg Dyspnea Scale
The 10-point Borg Dyspnea Scale will be used during cardiopulmonary exercise testing to characterize degree of dyspnea. The lowest score (0) corresponds to the sensation of normal breathing (absence of dyspnea) and the highest score (10) corresponds to the maximum possible sensation of dyspnea.
Qualitative Assessment of Dyspnea
The 18-point Qualitative Dyspnea Scale will be ascertained at the end of cardiopulmonary exercise testing. Participants will be presented with a list of 18 descriptors characterizing their dyspnea and will be asked to rank the top three.
Qualitative Assessment of Dyspnea
The 18-point Qualitative Dyspnea Scale will be ascertained at the end of respiratory muscle endurance testing. Participants will be presented with a list of 18 descriptors characterizing their dyspnea and will be asked to rank the top three.
Oscillometry
Oscillometry will be performed in the pulmonary function laboratory following the protocol from the European Respiratory Society.
Spirometry (Forced Vital Capacity)
Spirometry (forced vital capacity) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Spirometry (Forced Expiratory Volume in 1 Second)
Spirometry (forced expiratory volume in 1 second) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Lung Volumes (Total Lung Capacity)
Lung volumes (i.e., Total Lung Capacity) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Lung Volumes (Inspiratory Capacity)
Lung volumes (i.e., inspiratory capacity) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Lung Volumes (Residual Volume)
Lung volumes (i.e., Residual Volume) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Diaphragm Thickness
The right hemi-diaphragm thickness will be measured using a 13 megahertz ultrasound transducer.
Diaphragm Thickening Fraction
The right hemi-diaphragm thickening fraction will be measured using a 13 megahertz ultrasound transducer.
Peak Oxygen Uptake During Cardiopulmonary Exercise Testing
Cardiopulmonary exercise test-based assessment of peak oxygen uptake (VO2 peak) will be performed on a cycle ergometer.
Ventilatory Response During Cardiopulmonary Exercise Testing
Ventilatory response will be assessed during cardiopulmonary exercise testing (on a cycle ergometer) with exhaled gas measurements captured breath by breath.
Heart Rate Response During Cardiopulmonary Exercise Testing
Heart rate response will be assessed during cardiopulmonary exercise testing on a cycle ergometer.
Pre-frontal Cortical Neural Activity During Cardiopulmonary Exercise Testing
Oxygenated and total hemoglobin of the prefrontal cortex will be measured with Functional Near Infrared Spectroscopy during cardiopulmonary exercise testing.
Physical Activity Levels
Physical activity (daily steps) will be measured via a tracking device (Fitbit, San Francisco, USA) for a 7-day period.
Leisure-Time Exercise
Leisure-time exercise will be measured via the 3-item Godin Leisure-Time Exercise Questionnaire - Leisure Score Index. The Leisure Score Index is used to rank individuals from the lowest to highest exercise levels: sedentary (score less than 14 units); moderately active (score of 14-23 units); and active (24 units or more).
Health-Related Quality of Life
The Short-Form 36 Health Survey (SF-36) will be used to assess general health-related quality of life. The SF-36 consists of eight scaled scores (total score ranging from 0 to 100), with lower scores representing lower health-related quality of life.
Depression, Anxiety and Stress Scale
The Depression, Anxiety and Stress Scale (DASS-21) questionnaire has 21 items to assess mood (anxiety, depression, and stress). Each statement for the three domains is given a score of 0 (not applicable) to 3 (applicable most of the time) with scores categorized as follows: Normal, Mild, Moderate, Severe, and Extremely Severe.
Participant Study Recruitment
Recruitment-success percentage and reasons for participation/non-participation in the study will be collected.
Participant Retention in the Study
Participant retention will be assessed by measuring participant follow-up with testing throughout the study period.
Adherence to Inspiratory Muscle Training Load
Adherence to the IMT intervention will be tracked electronically via the IMT device (i.e., percentage of the maximal inspiratory pressure prescribed).
Adherence to Inspiratory Muscle Training Days
Adherence to the IMT intervention will be tracked using a participant log to record the number of training days completed each week.
Adverse Events During Inspiratory Muscle Training Practice (Safety and Tolerability)
Any adverse events (i.e. prolonged muscle soreness, pneumothorax) with IMT will be assessed throughout the study period.
Participant Satisfaction with the IMT Intervention
A multiple-choice and free-form questionnaire assessing the participants' satisfaction with the IMT intervention will be completed by study participants.
Full Information
NCT ID
NCT04972565
First Posted
June 25, 2021
Last Updated
November 3, 2022
Sponsor
University Health Network, Toronto
1. Study Identification
Unique Protocol Identification Number
NCT04972565
Brief Title
Dyspnea in Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder
Official Title
Evaluation and Management of Dyspnea in Hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorder (HSD)
Study Type
Interventional
2. Study Status
Record Verification Date
November 2022
Overall Recruitment Status
Recruiting
Study Start Date
August 16, 2021 (Actual)
Primary Completion Date
December 31, 2023 (Anticipated)
Study Completion Date
December 31, 2023 (Anticipated)
3. Sponsor/Collaborators
Responsible Party, by Official Title
Sponsor
Name of the Sponsor
University Health Network, Toronto
4. Oversight
Studies a U.S. FDA-regulated Drug Product
No
Studies a U.S. FDA-regulated Device Product
No
Data Monitoring Committee
No
5. Study Description
Brief Summary
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.
Detailed Description
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.
6. Conditions and Keywords
Primary Disease or Condition Being Studied in the Trial, or the Focus of the Study
Hypermobile EDS (hEDS), Hypermobility Syndrome
Keywords
Hypermobile Ehlers Danlos Syndrome, Hypermobility Spectrum Disorder, Dyspnea, Inspiratory Muscle Training, Exercise
7. Study Design
Primary Purpose
Treatment
Study Phase
Not Applicable
Interventional Study Model
Parallel Assignment
Model Description
Hypermobile EDS and HSD participants will be randomized to an inspiratory muscle training group versus usual care. Baseline measures will be compared to 17 age and sex-matched healthy controls.
Masking
None (Open Label)
Allocation
Randomized
Enrollment
34 (Anticipated)
8. Arms, Groups, and Interventions
Arm Title
Usual Care plus Inspiratory Muscle Training (IMT)
Arm Type
Experimental
Arm Description
Participants will attend the standard of care rehabilitation program offered to hEDS and HSD patients at the University Health Network. The program consists of i) an individualized home-based rehabilitation and exercise program (twelve weeks of aerobic, neuromotor, and resistance-based exercises), ii) a self-management education intervention, and iii) a community resource engagement plan. Furthermore, patients attend four on-site sessions (a baseline assessment and three follow-up visits).
Participants in this group will also be provided with a personalized prescription for an IMT program for eight weeks to be performed in the home environment (two daily IMT sessions of 30 breaths, five days per week). Participants will be virtually supervised by the study team weekly with any adverse events closely monitored. Participants will receive instructions and feedback on how to optimize their home training efforts with direct observation of their IMT practice.
Arm Title
Usual Care
Arm Type
No Intervention
Arm Description
Participants will participate in the standard of care rehabilitation program offered to hEDS and HSD patients at the University Health Network.
Intervention Type
Behavioral
Intervention Name(s)
Usual Care plus Inspiratory Muscle Training (IMT)
Intervention Description
Participants will receive usual care and will also be provided with a personalized prescription for an IMT program for 8 weeks. Participants will perform two daily IMT sessions of 30 breaths (< 5 minutes/session), 5 days per week in their home environment. IMT intensity will be progressed weekly by 5-10% of the baseline maximal inspiratory pressure if the Borg Dyspnea score is < 7.
Primary Outcome Measure Information:
Title
Maximal Inspiratory Pressure
Description
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.
Time Frame
Change from baseline at 8 weeks
Secondary Outcome Measure Information:
Title
Maximal Expiratory Pressure
Description
Maximal expiratory 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.
Time Frame
Change from baseline at 8 weeks
Title
Respiratory Muscle Endurance
Description
Respiratory muscle endurance (endurance time measured in seconds) will be assessed using a manual threshold loading device (Philips Threshold trainer).
Time Frame
Change from baseline at 4 weeks and 8 weeks
Title
Inspiratory Muscle Training Workload
Description
Total IMT workload per session (product of inspiratory muscle force and inhaled volume expressed in Joules) will be extracted from the IMT device in the experimental group.
Time Frame
Through study completion, an average of 8 weeks
Title
Inspiratory Muscle Training Load
Description
Inspiratory muscle training Load (cm H2O) will be abstracted from the IMT device in the experimental group.
Time Frame
Through study completion, an average of 8 weeks
Title
Inspiratory Muscle Training Mean Power
Description
Inspiratory muscle training mean power per breath (Watts) will be abstracted from the IMT device in the experimental group.
Time Frame
Through study completion, an average of 8 weeks
Title
Inspiratory Muscle Training Mean Volume
Description
Inspiratory muscle training mean volume per breath (Litres) will be abstracted from the IMT device in the experimental group.
Time Frame
Through study completion, an average of 8 weeks
Title
Medical Research Council Dyspnea Scale
Description
The Medical Research Council Dyspnea Scale will be used to assess the effect of breathlessness on daily activities. The scale comprises five statements that describe the range of respiratory dyspnea from none (Grade 1) to severe with difficulties performing self-care (Grade 5).
Time Frame
Change from baseline at 8 weeks
Title
Borg Dyspnea Scale
Description
The 10-point Borg Dyspnea Scale will be used throughout the study to adjust the intensity of inspiratory muscle training sessions The lowest score (0) corresponds to the sensation of normal breathing (absence of dyspnea) and the highest score (10) corresponds to the maximum possible sensation of dyspnea.
Time Frame
Through study completion, an average of 8 weeks
Title
Borg Dyspnea Scale
Description
The 10-point Borg Dyspnea Scale will be used during cardiopulmonary exercise testing to characterize degree of dyspnea. The lowest score (0) corresponds to the sensation of normal breathing (absence of dyspnea) and the highest score (10) corresponds to the maximum possible sensation of dyspnea.
Time Frame
Change from baseline at 8 weeks
Title
Qualitative Assessment of Dyspnea
Description
The 18-point Qualitative Dyspnea Scale will be ascertained at the end of cardiopulmonary exercise testing. Participants will be presented with a list of 18 descriptors characterizing their dyspnea and will be asked to rank the top three.
Time Frame
Change from baseline at 8 weeks
Title
Qualitative Assessment of Dyspnea
Description
The 18-point Qualitative Dyspnea Scale will be ascertained at the end of respiratory muscle endurance testing. Participants will be presented with a list of 18 descriptors characterizing their dyspnea and will be asked to rank the top three.
Time Frame
Change from baseline at 4 weeks and 8 weeks
Title
Oscillometry
Description
Oscillometry will be performed in the pulmonary function laboratory following the protocol from the European Respiratory Society.
Time Frame
Change from baseline at 8 weeks
Title
Spirometry (Forced Vital Capacity)
Description
Spirometry (forced vital capacity) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Time Frame
Change from baseline at 8 weeks
Title
Spirometry (Forced Expiratory Volume in 1 Second)
Description
Spirometry (forced expiratory volume in 1 second) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Time Frame
Change from baseline at 8 weeks
Title
Lung Volumes (Total Lung Capacity)
Description
Lung volumes (i.e., Total Lung Capacity) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Time Frame
Change from baseline at 8 weeks
Title
Lung Volumes (Inspiratory Capacity)
Description
Lung volumes (i.e., inspiratory capacity) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Time Frame
Change from baseline at 8 weeks
Title
Lung Volumes (Residual Volume)
Description
Lung volumes (i.e., Residual Volume) will be measured in the pulmonary function laboratory following the American Thoracic Society Guidelines.
Time Frame
Change from baseline at 8 weeks
Title
Diaphragm Thickness
Description
The right hemi-diaphragm thickness will be measured using a 13 megahertz ultrasound transducer.
Time Frame
Change from baseline at 8 weeks
Title
Diaphragm Thickening Fraction
Description
The right hemi-diaphragm thickening fraction will be measured using a 13 megahertz ultrasound transducer.
Time Frame
Change from baseline at 8 weeks
Title
Peak Oxygen Uptake During Cardiopulmonary Exercise Testing
Description
Cardiopulmonary exercise test-based assessment of peak oxygen uptake (VO2 peak) will be performed on a cycle ergometer.
Time Frame
Change from baseline at 8 weeks
Title
Ventilatory Response During Cardiopulmonary Exercise Testing
Description
Ventilatory response will be assessed during cardiopulmonary exercise testing (on a cycle ergometer) with exhaled gas measurements captured breath by breath.
Time Frame
Change from baseline at 8 weeks
Title
Heart Rate Response During Cardiopulmonary Exercise Testing
Description
Heart rate response will be assessed during cardiopulmonary exercise testing on a cycle ergometer.
Time Frame
Change from baseline at 8 weeks
Title
Pre-frontal Cortical Neural Activity During Cardiopulmonary Exercise Testing
Description
Oxygenated and total hemoglobin of the prefrontal cortex will be measured with Functional Near Infrared Spectroscopy during cardiopulmonary exercise testing.
Time Frame
Change from baseline at 8 weeks
Title
Physical Activity Levels
Description
Physical activity (daily steps) will be measured via a tracking device (Fitbit, San Francisco, USA) for a 7-day period.
Time Frame
Change from baseline at 4 weeks and 8 weeks
Title
Leisure-Time Exercise
Description
Leisure-time exercise will be measured via the 3-item Godin Leisure-Time Exercise Questionnaire - Leisure Score Index. The Leisure Score Index is used to rank individuals from the lowest to highest exercise levels: sedentary (score less than 14 units); moderately active (score of 14-23 units); and active (24 units or more).
Time Frame
Baseline
Title
Health-Related Quality of Life
Description
The Short-Form 36 Health Survey (SF-36) will be used to assess general health-related quality of life. The SF-36 consists of eight scaled scores (total score ranging from 0 to 100), with lower scores representing lower health-related quality of life.
Time Frame
Change from baseline at 8 weeks
Title
Depression, Anxiety and Stress Scale
Description
The Depression, Anxiety and Stress Scale (DASS-21) questionnaire has 21 items to assess mood (anxiety, depression, and stress). Each statement for the three domains is given a score of 0 (not applicable) to 3 (applicable most of the time) with scores categorized as follows: Normal, Mild, Moderate, Severe, and Extremely Severe.
Time Frame
Change from baseline at 8 weeks
Title
Participant Study Recruitment
Description
Recruitment-success percentage and reasons for participation/non-participation in the study will be collected.
Time Frame
Over the study period (8 weeks)
Title
Participant Retention in the Study
Description
Participant retention will be assessed by measuring participant follow-up with testing throughout the study period.
Time Frame
Over the study period (8 weeks)
Title
Adherence to Inspiratory Muscle Training Load
Description
Adherence to the IMT intervention will be tracked electronically via the IMT device (i.e., percentage of the maximal inspiratory pressure prescribed).
Time Frame
Over the intervention period (8 weeks)
Title
Adherence to Inspiratory Muscle Training Days
Description
Adherence to the IMT intervention will be tracked using a participant log to record the number of training days completed each week.
Time Frame
Over the intervention period (8 weeks)
Title
Adverse Events During Inspiratory Muscle Training Practice (Safety and Tolerability)
Description
Any adverse events (i.e. prolonged muscle soreness, pneumothorax) with IMT will be assessed throughout the study period.
Time Frame
Over the intervention period (8 weeks)
Title
Participant Satisfaction with the IMT Intervention
Description
A multiple-choice and free-form questionnaire assessing the participants' satisfaction with the IMT intervention will be completed by study participants.
Time Frame
Change from baseline at weeks 1, 4 and 8
Other Pre-specified Outcome Measures:
Title
Electrocardiogram
Description
A 12-Lead Electrocardiogram will be performed in all study participants. The following electrocardiogram parameter (i.e. ST segment) will be assessed to ensure within normal limits before cardiopulmonary exercise testing to ensure no signs of active ischemia.
Time Frame
Baseline
Title
Chest X-Ray
Description
Chest x-ray will be performed to characterize possible etiologies of dyspnea and exclude any evidence of pneumothorax prior to commencing an IMT program.
Time Frame
Baseline
Title
Body Mass Index
Description
Body mass index data (kg/m^2) will be ascertained from clinical records.
Time Frame
Change from baseline at 4 weeks and 8 weeks
Title
Waist Circumference
Description
Waist circumference (cm) will be ascertained from clinical records.
Time Frame
Change from baseline at 4 weeks and 8 weeks
Title
Demographic Data
Description
A demographic questionnaire will be administered to collect information on age, sex, and ethnicity of the study participants.
Time Frame
Baseline
10. Eligibility
Sex
All
Minimum Age & Unit of Time
18 Years
Accepts Healthy Volunteers
No
Eligibility Criteria
Inclusion Criteria:
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
Exclusion Criteria:
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
Central Contact Person:
First Name & Middle Initial & Last Name or Official Title & Degree
Dmitry Rozenberg, MD, PhD
Phone
416-340-4800
Ext
7358
Email
Dmitry.Rozenberg@uhn.ca
Overall Study Officials:
First Name & Middle Initial & Last Name & Degree
Dmitry Rozenberg, MD, PhD
Organizational Affiliation
University Health Network/University of Toronto
Official's Role
Principal Investigator
Facility Information:
Facility Name
University Health Network
City
Toronto
State/Province
Ontario
ZIP/Postal Code
M5G 2C4
Country
Canada
Individual Site Status
Recruiting
Facility Contact:
First Name & Middle Initial & Last Name & Degree
Dmitry Rozenberg, MD, PhD
Phone
416-340-4800
Ext
7358
Email
dmitry.rozenberg@uhn.ca
12. IPD Sharing Statement
Plan to Share IPD
No
Citations:
PubMed Identifier
30569502
Citation
Reychler G, Liistro G, Pierard GE, Hermanns-Le T, Manicourt D. Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers-Danlos syndrome: A randomized controlled trial. Am J Med Genet A. 2019 Mar;179(3):356-364. doi: 10.1002/ajmg.a.61016. Epub 2018 Dec 20.
Results Reference
background
PubMed Identifier
15317957
Citation
Hakim AJ, Grahame R. Non-musculoskeletal symptoms in joint hypermobility syndrome. Indirect evidence for autonomic dysfunction? Rheumatology (Oxford). 2004 Sep;43(9):1194-5. doi: 10.1093/rheumatology/keh279. No abstract available.
Results Reference
background
PubMed Identifier
4023980
Citation
Ayres JG, Pope FM, Reidy JF, Clark TJ. Abnormalities of the lungs and thoracic cage in the Ehlers-Danlos syndrome. Thorax. 1985 Apr;40(4):300-5. doi: 10.1136/thx.40.4.300.
Results Reference
background
PubMed Identifier
17412739
Citation
Morgan AW, Pearson SB, Davies S, Gooi HC, Bird HA. Asthma and airways collapse in two heritable disorders of connective tissue. Ann Rheum Dis. 2007 Oct;66(10):1369-73. doi: 10.1136/ard.2006.062224. Epub 2007 Apr 5.
Results Reference
background
PubMed Identifier
12867232
Citation
Gazit Y, Nahir AM, Grahame R, Jacob G. Dysautonomia in the joint hypermobility syndrome. Am J Med. 2003 Jul;115(1):33-40. doi: 10.1016/s0002-9343(03)00235-3.
Results Reference
background
PubMed Identifier
26263828
Citation
Chatzoudis D, Kelly TJ, Lancaster J, Jones TM. Upper airway obstruction in a patient with Ehlers-Danlos syndrome. Ann R Coll Surg Engl. 2015 Apr;97(3):e50-1. doi: 10.1308/003588414X14055925061793.
Results Reference
background
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Dyspnea in Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder
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