cEDS can cause a variety of symptoms in many different areas of the body, so people with cEDS may require providers in multiple different specialties to manage their care.
Key aspects of care include:
Skin protection
People with cEDS should consider the injury risk of activities and avoid contact sports to prevent injury. Extra layers of clothing, particularly close-fitting layers of clothing next to the skin, can provide some protection.
Wound management
People with cEDS have very fragile skin that can be wounded by even minor trauma. Wounds may be difficult to suture and heal slowly. Poor wound healing leads to wide, atrophic scars in people with cEDS. Skin injuries should be repaired promptly, preferably by a plastic surgeon, to minimize scarring, improve healing, and prevent further complications.
Wound care recommendations include:
-
- Wounds should be closed without tension.
- Deep wounds should be sutured in layers.
- Multiple small stitches with a fine gauge suture can be used to avoid tension.
- After repair, the wound area should be immobilized with Steri-Strips, tape (which requires careful removal), or a tubular bandage.
- Sutures should be left in twice as long as usual.
Sun protection
People with cEDS may notice their skin has a prematurely aged appearance. Regular use of sun protection from a young age can reduce the chance of premature skin aging. Vitamin D supplementation may be considered due to reduced sun exposure.
Pain and fatigue management
Management of pain and fatigue should focus on the underlying causes and may include:
-
- Lifestyle changes
- Physical therapy and exercise
- Medications
- Assistive devices
- Cognitive behavioral therapy
Cardiac screening
People with cEDS should have an echocardiogram when they are diagnosed, and any findings should be followed up on. If the results are normal during childhood, the echocardiogram should be repeated in adulthood.
Pregnancy management
Specialist obstetric care is recommended for people with cEDS, and all healthcare professionals should be aware of the diagnosis. Pelvic organ prolapse may occur after delivery due to tissue fragility and extensibility. Premature rupture of membranes and breech presentation are more likely when the baby has cEDS.
Surgical care
All medical professionals involved in a person’s care should be aware of their cEDS diagnosis. Surgical wounds can heal well when care is taken to suture without tension. During surgeries, self-retaining retractors should be used carefully because excessive retraction can cause tissue tears and hematomas. Surgery may be difficult due to tissue fragility.
Gastrointestinal considerations
Procedures such as endoscopy and colonoscopy should be performed with care and awareness of the diagnosis due to a possibly increased risk of mucosal bleeding, tissue fragility, and perforation.
Genetic counseling
cEDS is inherited in an autosomal dominant pattern, so people with cEDS have a 50% chance of passing the condition on to each child they have. People with cEDS and their partners may benefit from discussing what this means for them with a genetic counselor when considering having children.
Support
Mental health support can be beneficial for anyone with a lifelong medical condition. There are many ways to get mental health support, including through doctors, therapists, charity organizations, and support groups. The Ehlers-Danlos Society offers virtual support groups for people with all types of EDS and HSD.