More than ‘Just’ Fragile Skin and Loose Joints: A Nurse’s Guide to Ehlers-Danlos Syndrome- Term life

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You might not have heard of Ehlers–Danlos syndrome (EDS), but chances are you’ve had a patient with this hereditary connective tissue disorder, which affects an estimated one in 5,000 people worldwide. The real number is probably higher. There are six types of EDS with widely varying presentations—and given the lack of consensus regarding diagnostic criteria, underrecognition seems likely.

Primary symptoms can include skin hyperelasticity, joint hypermobility, and general tissue fragility; the effects can be disabling and even life threatening. In one of this month’s CE features,“Nursing Management of Patients with Ehlers–Danlos Syndrome,”. 

Joint hypermobility is often characteristic of EDS. Photo © 2015 Suzbah / Dreamstime.com.

Overview: EDS has historically been misunderstood … Because of the high degree of phenotypic variability, patients are often correctly diagnosed only after years of seemingly unrelated but debilitating injuries and illnesses. Specific genetic mutations have been identified for some, but not all, EDS types; patients presenting with a high index of suspicion should be referred to a geneticist. As awareness and recognition of the syndrome improve, nurses are increasingly likely to care for patients with EDS. This article gives a brief overview of the syndrome and provides guidance on ways to manage symptoms, recognize and prevent serious complications, and improve patients’ quality of life.

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