From the Guidelines
Ehlers-Danlos syndrome (EDS) patients should prioritize low-resistance exercise and physical therapy to improve joint stability and manage chronic pain, as recommended by the most recent guidelines 1. The management of EDS focuses on symptom control and prevention of complications. Key aspects of management include:
- Physical therapy to strengthen muscles around unstable joints and improve joint stability
- Pain management with a pain management specialist, as patients with EDS hypermobile type often experience chronic pain
- Lifestyle modifications to prevent injury, such as avoiding activities that put excessive strain on joints
- Use of braces or mobility aids to support joints and improve mobility
- Regular monitoring by a multidisciplinary team, including cardiologists for those with vascular EDS due to risk of arterial rupture The diagnosis of EDS, hypermobility type, is based on criteria such as 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. In terms of specific interventions, low-resistance exercise is recommended to improve joint stability by increasing muscle tone, and physical therapy for myofascial release is often necessary to facilitate participation in low-resistance exercise 1. Additionally, vitamin C may be beneficial as a cofactor for cross-linking of collagen fibrils and may improve hypermobility 1.
From the Research
Ehlers-Danlos Syndrome Overview
- Ehlers-Danlos syndrome (EDS) is a multifaceted disease that can present with a variety of types of pain, including both nociceptive and neuropathic pain 2, 3.
- The syndrome is characterized by joint hypermobility, elastic skin, and often chronic pain, especially in the hypermobility variant 3.
Treatment Options
- The proposed treatments for the various musculoskeletal pain syndromes in EDS have had variable success, and it becomes much more imperative to better define and evaluate the current treatment modalities in treating this debilitating disease 2.
- Treatment options may include non-opioid medications, physical therapy, and psychological support, as well as complimentary/alternative treatments, opioids/opioid-like medications, nonsteroidal anti-inflammatory drugs, muscle relaxants, neuropathic modulators, steroids, surgery/procedures, and acetaminophen 2.
- Occupational therapy and bracing were found to be the most effective options, with 70% of patients reporting improvement 2.
- Opioids should be used cautiously in EDS patients because of connective tissue vulnerabilities and potential side effects 3.
Exercise and Rehabilitation
- Exercise and rehabilitation may be beneficial for various physical and psychological outcomes in people with EDS 4.
- A systematic review of 10 eligible studies found that all studies showed some improvement in a physical and/or psychological outcome after the intervention period 4.
- However, adequately powered and rigorous randomized controlled trials of exercise and rehabilitation interventions for people with EDS are needed 4.
Challenges and Future Directions
- Diagnosing EDS is difficult due to symptom overlap with other connective tissue disorders 3.
- Managing EDS-related chronic pain requires a tailored, multidisciplinary approach, and early and accurate diagnosis and specialized healthcare providers familiar with EDS are crucial for effective pain management 3.
- Ongoing research and evidence-based pain management approaches are vital to address the unique needs of EDS patients, promoting better pain relief and overall well-being 3, 5.