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Exercise and Rehabilitation in Individuals with Ehlers-Danlos Syndrome and Generalized Hypermobility Spectrum Disorders (G-HSD): A Pulmonary Perspective

*Jillian Dhawan BHSc,1,3 Noor Al Kaabi MSc,2,3 and Dmitry Rozenberg MD, PhD 2-4
1)University of Ottawa, Faculty of Medicine, Ottawa, Canada  
2)Temerty Faculty of Medicine, University of Toronto, Toronto, Canada  
3)Toronto General Hospital Research Institute, GoodHope Ehlers-Danlos Syndrome Program, Toronto, Ontario, Canada  
4)Division of Respirology, University Health Network, Toronto, Ontario, Canada  

*All three authors contributed equally

Ehlers-Danlos syndrome (EDS) and Generalized Hypermobility Spectrum Disorders (G-HSD) are a heterogeneous group of hereditary connective tissue disorders characterized by joint hypermobility, skin hyper-extensibility, and tissue fragility.1,2 Both are multi-systemic conditions and can involve gastrointestinal, cardiovascular, musculoskeletal, and respiratory systems.2 The prevalence varies by EDS/G-HSD subtypes and across the globe, with estimates ranging between 1/5000 to 1/20,000 for some subtypes.3 

Respiratory manifestations in EDS/G-HSD are gaining increased recognition and are associated with functional limitations.4,5  The collagen connective tissue abnormalities may lead to structural (i.e. scoliosis, pneumothorax), functional (airway obstruction, sleep apnea, and vocal cord dysfunction), and inflammatory conditions (i.e. asthma, mast cell activation), which can contribute to dyspnea, chest tightness, and exercise intolerance.4 These respiratory manifestations may impair physical function and quality of life, in addition to the musculoskeletal limitations experienced by individuals with EDS and G-HSD.6,7              

Exertional dyspnea has been described to be prevalent in individuals with EDS and G-HSD8,9 and is multifactorial, including but not limited to thoracic cage abnormalities, respiratory muscle dysfunction, deconditioning, and autonomic dysfunction.4,5  Depending on the institution, some of these EDS/G-HSD individuals may be referred to a pulmonary rehabilitation (PR) program given their underlying dyspnea, if a specialized EDS rehabilitation program is not available which has traditionally focused on physical and musculoskeletal fitness in this population.10,11  It is possible that PR may be beneficial for some individuals with EDS/G-HSD as PR is “a comprehensive intervention based on a through patient assessment followed by patient-tailored therapies that include, but are not limited to exercise training, education, and behavior change, designed to improve the physical and psychological condition of people with chronic respiratory disease and to promote the long-term adherence to health-enhancing behaviors.”12 However, it is important to highlight that rehabilitation programs for EDS/G-HSD are usually individually tailored therapies designed to improve physical and mental well-being and can be performed in hospital and outpatient rehab clinics, home, or a combination of both settings. 

The rehabilitation modalities such as endurance/aerobic (walking, swimming, stationary bike), resistance/strength (free weights, resistance bands, core stability), stretching/flexibility (dynamic warm up exercises, thoracic mobility, yoga), and balance/proprioception (neuromuscular stabilization, body alignment, posture) training modalities have been shown to improve cardiorespiratory fitness, chronic pain, muscle health, joint stabilization, and fatigue in EDS/G-HSD.11 Further, inspiratory muscle training (IMT) has been evaluated among individuals with hypermobility EDS and shown to be effective in improving respiratory muscle strength, lung function and exercise capacity in one study of 20 randomized participants.8  Our center has an ongoing trial of IMT evaluating the feasibility and underlying mechanisms of IMT in both hypermobility EDS and G-HSD participants.13 Chest physical therapy modalities such as diaphragmatic breathing exercises and energy conservation techniques are important considerations in this population as well.10   

Additional components include medication management, nutrition, education, and psychosocial support, which are essential modalities to cope with episodic musculoskeletal flare-ups, relieve any fears or anxiety associated with exercise, and help promote long-term exercise adherence.14  Importantly, EDS/G-HSD patients report fear of injury, often described as kinesiophobia, and fatigue as barriers to physical activity.15  Collectively, kinesiophobia, fatigue, pain, and respiratory symptoms may lead to exercise-avoidance,16 which can perpetuate physical deconditioning in these patients leading to more difficulty in performing physical activity and exercise.15,17

Educational components in rehabilitation programs may be beneficial for EDS/G-HSD patients in addressing these barriers. Safety considerations include regular medical supervision, monitoring exertional tolerance, avoidance of high impact activities, and balancing co-morbidities such as postural orthostatic tachycardia syndrome.10  More importantly, working with centers of EDS excellence is important to harness the expertise in managing the multi-systemic nature of EDS/G-HSD.   

In summary, respiratory symptoms are prevalent in individuals with EDS and G-HSD and rehabilitation programs addressing aerobic, strength, flexibility/stretching, and respiratory muscle training may be opportunities to improve respiratory symptoms in addition to musculoskeletal function, energy conservation strategies, and quality of life. Rehabilitation programs addressing educational components can help with disease management and promote an active lifestyle. Thus, it is possible that some EDS/G-HSD patients, especially those presenting with respiratory symptoms, may be managed in cardiac or pulmonary rehabilitation programs if a specialized EDS rehab program is not available, but close collaboration with EDS specialists is critical in managing this multi-systemic condition in rehabilitation.            

Conflicts: None to Report

Funding: DR receives research salary support from the Sandra Faire and Ivan Fecan Professorship in Rehabilitation Medicine and Temerty Faculty of Medicine, University of Toronto.


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