Inspiratory Muscle Training for Ehlers-Danlos Syndrome

Overseen By Dmitry Rozenberg, MD, PhD

Age: 18+

Sex: Any

Trial Phase: Academic

Sponsor: University Health Network, Toronto

Disqualifiers: Other EDS, Cardiac disease, Lung disease, Neuromuscular disease, Pregnancy, others

No Placebo Group All trial participants will receive the active study treatment (no placebo)

Compensation: Varies

Number of Visits: 5

Duration: 8 weeks

What You Need to Know Before You Apply

What is the purpose of this trial?

This trial explores how adding Inspiratory Muscle Training (IMT) to regular exercise might help individuals with hypermobile Ehlers-Danlos Syndrome (hEDS) or Hypermobility Spectrum Disorders (HSD) breathe more easily and improve their quality of life. IMT consists of specific exercises to strengthen the muscles used for breathing. The study compares two groups: one engaging in regular exercise and another combining regular exercise with IMT. This trial suits adults newly referred to the GoodHope Exercise and Rehabilitation Program who have a clinical diagnosis of hEDS or HSD. As an unphased trial, it offers a unique opportunity to contribute to research that could enhance treatment options for others with similar conditions.

Will I have to stop taking my current medications?

The trial information does not specify whether you need to stop taking your current medications. It's best to discuss this with the trial coordinators or your doctor.

What prior data suggests that Inspiratory Muscle Training is safe for individuals with Ehlers-Danlos Syndrome?

Research shows that Inspiratory Muscle Training (IMT) is generally safe and well-tolerated for people with hypermobile Ehlers-Danlos Syndrome (hEDS). Studies have found that IMT can improve breathing and lung function in these patients. In one study, participants who did IMT for six weeks handled 13% more exercise and exhaled 10% more air in one second. The study reported no serious safety issues, indicating that the treatment is safe. Participants did not experience significant negative effects, making IMT a promising option for those considering this type of training. ^{1 2 3 4 5}

Why are researchers excited about this trial?

Researchers are excited about Inspiratory Muscle Training (IMT) for Ehlers-Danlos Syndrome (EDS) because it offers a novel way to potentially improve respiratory function in these patients. Unlike the standard rehabilitation programs that focus on general exercise and self-management, IMT specifically targets the muscles involved in breathing, which could enhance respiratory strength and endurance. This approach is personalized and home-based, making it accessible and convenient, while still providing virtual supervision to ensure effectiveness and safety. These features could make IMT a promising complement to existing treatments for EDS, addressing a unique aspect of the condition that isn't the primary focus of current care options.

What evidence suggests that Inspiratory Muscle Training is effective for Ehlers-Danlos Syndrome?

Research has shown that Inspiratory Muscle Training (IMT) can improve breathing problems in people with hypermobile Ehlers-Danlos Syndrome (hEDS) and Hypermobility Spectrum Disorders (HSD). Studies have found that individuals with hEDS often have weaker breathing muscles, but IMT can strengthen these muscles and improve lung function. This trial will compare two groups: one receiving Usual Care plus IMT and another receiving Usual Care alone. By focusing on strengthening the breathing muscles, IMT helps patients breathe more easily and carry out daily activities. Overall, IMT shows promise in enhancing respiratory health for those with these conditions. ^{1 6 7 8 9}

Who Is on the Research Team?

Dmitry Rozenberg, MD, PhD

Principal Investigator

University Health Network/University of Toronto

Are You a Good Fit for This Trial?

Adults diagnosed with hypermobile Ehlers-Danlos Syndrome (hEDS) or Hypermobility Spectrum Disorders (HSD), who are new referrals to the GEAR Program. Participants must meet specific diagnostic criteria and can't have conditions like unstable heart disease, recent respiratory infections, other types of EDS, neuromuscular diseases, severe autonomic dysfunction, or be pregnant.

Inclusion Criteria

You have been diagnosed with hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorder (HSD) by a medical team that specializes in these conditions.

You have been diagnosed with hypermobile Ehlers-Danlos syndrome (hEDS) based on specific criteria or with hypermobility spectrum disorder (HSD) confirmed by a medical team specializing in Ehlers-Danlos syndrome.

You have recently been referred to the GoodHope Exercise and Rehabilitation (GEAR) Program at the University Health Network.

Exclusion Criteria

I have a muscle condition that could affect my breathing therapy.

My genetic test confirmed I have a specific type of EDS.

You are not able to do exercise testing due to unstable heart disease, or you have a pacemaker or implantable defibrillator, or certain heart abnormalities found on an echocardiogram.

Timeline for a Trial Participant

Screening

Participants are screened for eligibility to participate in the trial

2-4 weeks

Baseline Assessment

Baseline evaluations of dyspnea, pulmonary function tests, respiratory muscle structure and function, HRQL, anxiety, and depression

1 week

1 visit (in-person)

Treatment

Participants undergo 8 weeks of whole-body exercises with or without Inspiratory Muscle Training (IMT)

8 weeks

4 visits (in-person), weekly virtual supervision

Follow-up

Participants are monitored for safety and effectiveness after treatment

4 weeks

What Are the Treatments Tested in This Trial?

Interventions

• Usual Care plus Inspiratory Muscle Training (IMT)

Trial Overview The trial is testing if Inspiratory Muscle Training (IMT) combined with whole-body exercise improves breathing difficulties, muscle strength in the lungs, quality of life and mental health for those with hEDS/HSD compared to just exercise alone.

How Is the Trial Designed?

Group I: Usual Care plus Inspiratory Muscle Training (IMT) Experimental Treatment 1 Intervention

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.

Group II: Usual Care Active Control 1 Intervention

Participants will participate in the standard of care rehabilitation program offered to hEDS and HSD patients at the University Health Network.

Who Is Running the Clinical Trial?

University Health Network, Toronto

Lead Sponsor

Trials

1,555

Recruited

526,000+

Citations

1. pubmed.ncbi.nlm.nih.gov pubmed.ncbi.nlm.nih.gov/30569502/

Inspiratory muscle strength training improves lung function ... IM strength is significantly reduced in patients with hEDS. IMT improved IM strength, lung function, and exercise capacity.

2. atsjournals.org atsjournals.org/doi/pdf/10.1164/ajrccm-conference.2024.209.1_MeetingAbstracts.A2766?download=true

Inspiratory Muscle Training for the Management of ... This ongoing study will help inform the feasibility of IMT in hEDS/G-HSD and provide a better understanding of the respiratory physiological ...

3. onlinelibrary.wiley.com onlinelibrary.wiley.com/doi/abs/10.1002/ajmg.a.61016

Inspiratory muscle strength training improves lung function ... IM strength was significantly reduced in patients with hEDS, but it was improved by inspiratory muscle training similarly to lung function and exercise ...

4. clinicaltrials.gov clinicaltrials.gov/study/NCT04972565

Dyspnea in Hypermobile Ehlers-Danlos Syndrome and ... Inspiratory Muscle Training (IMT) has been shown to improve dyspnea and respiratory muscle function; however, the impact of IMT in combination with whole ...

5. centerwatch.com centerwatch.com/clinical-trials/listings/NCT04972565/dyspnea-in-hypermobile-ehlers-danlos-syndrome-and-hypermobility-spectrum-disorder

Dyspnea in Hypermobile Ehlers-Danlos Syndrome and ... Inspiratory Muscle Training (IMT) has been shown to improve dyspnea and respiratory muscle function; however, the impact of IMT in combination ...

6. pmc.ncbi.nlm.nih.gov pmc.ncbi.nlm.nih.gov/articles/PMC8312172/

A review of respiratory manifestations and their management ... Reduced respiratory muscle function in hEDS patients responds to inspiratory muscle training ... Ehlers-Danlos syndromes: results of a national cohort study on ...

7. sciencedirect.com sciencedirect.com/science/article/pii/S2590109522000143

Exercise and Rehabilitation in People With Ehlers-Danlos ... Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers-Danlos syndrome: a randomized controlled trial. Am J Med ...

8. researchprotocols.org researchprotocols.org/2023/1/e44832/

Evaluation and Management of Dyspnea in Hypermobile ... This study aims to evaluate dyspnea, respiratory muscle strength, and patient-reported outcome measures (PROMs) in hEDS or G-HSD compared with healthy controls.

9. mdpi.com mdpi.com/2077-0383/14/12/4126

Respiratory Manifestations and Their Physical ... The 6-week IMT group had a 13% improvement in exercise capacity and 10% increase in forced expiratory volume in one second in comparison to the non-IMT group.