From the Guidelines
Hypermobile Ehlers-Danlos syndrome (hEDS) should be managed with a multidisciplinary approach, focusing on symptom control, preventing complications, and improving quality of life, as there is no specific cure for the condition. The diagnosis of hEDS is established by meeting specific criteria, including joint hypermobility confirmed by a score of 5 or more on the 9-point Beighton scale, soft or velvety skin with normal or slightly increased extensibility, and absence of skin or soft tissue fragility 1. Treatment typically includes physical therapy to strengthen muscles around hypermobile joints, with exercises tailored to avoid joint overextension, as well as pain management with acetaminophen or NSAIDs like ibuprofen (400-800mg every 6-8 hours) for mild to moderate pain, while some patients may require stronger medications such as tramadol (50-100mg every 4-6 hours) for severe pain.
Key Management Strategies
- Physical therapy to improve joint stability and strengthen muscles around hypermobile joints
- Pain management with medications such as acetaminophen or NSAIDs
- Use of braces, splints, or orthotic devices to stabilize hypermobile joints during activities
- Avoidance of high-impact activities that could damage joints
- Engagement in low-impact exercises like swimming or cycling
- Psychological support to address chronic pain and physical limitations
- Management of gastrointestinal symptoms, which are common in hEDS, with specific dietary modifications and medications, as patients with hEDS often experience acid reflux, abdominal pain, and constipation 1.
Importance of Multidisciplinary Care
Regular monitoring by a multidisciplinary team is essential, as complications can affect multiple body systems, and patients with hEDS often have associated conditions such as postural tachycardia syndrome (PoTS) and autonomic dysregulation 1. The condition results from genetic mutations affecting collagen or related proteins, which provide strength and elasticity to connective tissues throughout the body. A comprehensive management plan, incorporating the latest evidence and guidelines, is crucial to improve the quality of life and reduce morbidity and mortality in patients with hEDS.
From the Research
Hypermobile Ehlers-Danlos Syndrome
- Hypermobile Ehlers-Danlos syndrome (EDS) and hypermobility spectrum disorders are the most common symptomatic joint hypermobility conditions seen in clinical practice 2.
- The 2017 International Classification of the Ehlers-Danlos syndromes replaced previous terms for symptomatic joint hypermobility with hypermobile EDS and introduced the term hypermobility spectrum disorders for patients not meeting diagnostic criteria for hypermobile EDS 2, 3.
- Hypermobile EDS is inherited in an autosomal dominant pattern, but it does not have a known genetic mutation to help with diagnosis 2.
Clinical Features
- Clinical features of hypermobile EDS include joint hypermobility, skin findings, and joint pains or recurrent dislocations 2.
- Hypermobile EDS and, less commonly, hypermobility spectrum disorders may also be associated with several extra-articular symptoms, including anxiety disorders, chronic pain, fatigue, orthostatic intolerance, functional gastrointestinal disorders, and pelvic and bladder dysfunction 2.
Management
- The central goals of therapy are managing symptoms, preventing joint injury, and educating patients about their condition 2.
- Patients with hypermobile EDS/hypermobility spectrum disorders may benefit from physical and occupational therapy, psychological support, and self-management 2, 3.
- Physical therapy interventions, such as therapeutic exercise and motor function training, are efficacious methods to treat individuals with hypermobile EDS and generalized hypermobility spectrum disorder 4.
- Complementary therapies, including traditional Chinese therapies, herbal medications, and marijuana, are widely used by patients with EDS/HSD, with some reporting them to be extremely helpful in alleviating pain and improving quality of life 5.