What is the treatment for Ehlers-Danlos Syndrome (EDS)?

From the Guidelines

The treatment for Ehlers-Danlos Syndrome (EDS) should focus on a multidisciplinary approach to manage symptoms and prevent complications, with careful consideration of the patient's specific EDS type and symptoms, as outlined in the 2010 guidelines for the diagnosis and management of patients with thoracic aortic disease 1. The management of EDS involves a range of strategies, including:

• Physical therapy to strengthen muscles and stabilize joints without causing injury
• Pain management using non-pharmacological approaches and medications such as acetaminophen, NSAIDs, or low-dose opioids for severe pain
• Use of braces, splints, or taping to support joints and prevent dislocations
• Assistive devices like canes or wheelchairs to reduce joint stress
• Cardiovascular monitoring, particularly for vascular EDS, where beta-blockers might be prescribed to reduce stress on blood vessels, as noted in the guidelines for thoracic aortic disease 1
• Dietary modifications, proton pump inhibitors for reflux, or medications for motility issues to manage gastrointestinal symptoms
• Psychological support to address chronic pain and physical limitations that can lead to anxiety and depression It is essential to avoid high-impact activities that could damage joints and to provide close monitoring during pregnancy and surgical procedures due to tissue fragility and potential complications with wound healing, as highlighted in the guidelines 1. In the case of vascular EDS, surgical repair of ruptured arteries can be life-saving, but it is crucial to handle tissues carefully and use pledgeted sutures to minimize complications, as recommended in the guidelines for thoracic aortic disease 1. Noninvasive vascular imaging is preferred to minimize the risk of fatal complications associated with invasive imaging in these patients 1.

From the Research

Treatment Options for Ehlers-Danlos Syndrome (EDS)

The treatment for EDS is multifaceted and depends on the specific subtype and symptoms of the patient. Some of the treatment options include:

• Complimentary/alternative treatments, such as physical therapy and occupational therapy, which were found to be effective in 70% of patients in a study by 2
• Medications, such as nonsteroidal anti-inflammatory drugs, opioids, and neuropathic modulators, which were used by 70-92% of participants in a study by 3
• Surgical interventions, such as elective surgical repair of blood vessels at risk of rupture, which was shown to be safe and effective in a study by 4
• Bracing and splints, which were found to be helpful in managing acute and chronic pain in a study by 5
• Avoidance of potentially dangerous activities, which was found to be helpful in managing acute and chronic pain in a study by 5
• Heat therapy, which was found to be helpful in managing acute and chronic pain in a study by 5
• Massage therapies, which were found to be helpful in managing chronic pain in a study by 5

Management of Vascular EDS

Vascular EDS is a subtype of EDS that is associated with a high risk of arterial rupture and other potentially lethal complications. The management of vascular EDS includes:

• Treatment with beta blockers, such as Celiprolol, which was shown to decrease the risk of arterial rupture in a study by 4
• Elective surgical repair of blood vessels at risk of rupture, which was shown to be safe and effective in a study by 4
• Endovascular therapy with coil embolization, which was shown to be effective in treating ruptured pseudoaneurysms, visceral aneurysms, and carotid-cavernous fistulas in a study by 4

Multidisciplinary Management

EDS is a complex condition that requires multidisciplinary management. A team of healthcare professionals, including physical therapists, occupational therapists, pain management specialists, and surgeons, should work together to develop a comprehensive treatment plan for each patient. This approach was emphasized in a study by 2, which highlighted the importance of a multidisciplinary approach to managing EDS. Additionally, a study by 6 emphasized the importance of appropriate diagnosis and multidisciplinary management in achieving better clinical outcomes for patients with EDS.