From the Guidelines
Preventative measures for Ethylene Danolos syndrome, also known as Ehlers-Danlos syndrome (EDS), should focus on managing symptoms, preventing complications, and improving quality of life, with a particular emphasis on cardiovascular monitoring for vascular EDS, as recommended by the 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines 1.
Key Preventative Measures
- Physical therapy to strengthen muscles around hypermobile joints and improve proprioception
- Low-impact exercises such as swimming, walking, and stationary cycling to maintain mobility without causing joint damage
- Proper posture and body mechanics to reduce strain on joints
- Pain management using over-the-counter medications like acetaminophen or NSAIDs, with monitoring by a healthcare provider for long-term NSAID use
- Skin care for those with fragile skin, including the use of mild soaps, regular moisturizing, and protection from trauma
- Cardiovascular monitoring, particularly for vascular EDS, with regular echocardiograms and blood pressure management as needed, as the vascular form of EDS is characterized by rupture of arteries, uterus, or intestines, leading to reduced life expectancy 1
- Genetic counseling for family planning
- Use of medical alert bracelets and regular follow-ups with healthcare providers specializing in EDS management
Considerations for Vascular EDS
- The diagnosis of vascular Ehlers-Danlos syndrome is based on DNA or protein studies identifying a defect in type III collagen, encoded by the COL3A1 gene 1
- Noninvasive vascular imaging is preferred due to the risk of fatal complications associated with invasive imaging in these patients 1
- Surgical repair of ruptured arteries can be complicated by tissue fragility, tendency to hemorrhage extensively, and poor wound healing, but successful aortic surgery can be achieved with careful handling of tissues and resewing of anastomoses with pledgeted sutures 1
From the Research
Preventative Measures for Ethylene Danolos Syndrome
- There are no specific medical or genetic therapies available for any type of Ehlers-Danlos syndrome (EDS) 2
- Preventative and protective measures starting at birth can help preserve joint function and improve infant outcomes 3
- Management of EDS consists of a care team responsible for surveillance of major and organ-specific complications, integrated physical medicine, and rehabilitation 2
Medication and Treatment
- Celiprolol, a beta blocker, has been shown to have a protective effect in patients with vascular Ehlers-Danlos syndrome (vEDS) 4
- Treatment with celiprolol is tolerated in most patients with vEDS, despite some fatal vascular events 4
Exercise and Rehabilitation
- Exercise and rehabilitation may be beneficial for various physical and psychological outcomes in people with EDS 5
- 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 5
- Physical therapy plays a central role in the management of individuals with hypermobility-related disorders, including EDS 6
- The International Classification of Functioning, Disability and Health (ICF) can be used as a framework for assessing and managing individuals with EDS 6