Ehlers-Danlos syndrome, classical type: case management

As the field of genetics expands, there will be more need for health care professionals to possess basic knowledge of genetic conditions and patterns of inheritance to assist their patients and to make the proper referrals. Ehlers-Danlos syndrome (EDS) is a group of genetic connective tissue disorders that affects approximately 1 in 5,000 live births, including males and females of all racial and ethnic groups. The main characteristics of EDS are skin hyperextensibility, tissue fragility, andjoint hypermobility. Diagnosis of EDS is often difficult due to the complexity of symptoms and lack of specific genetic tests. However, once a diagnosis is suspected or confirmed, nurses play a vital role in assisting the patient andfamily to manage the disorder. In this article, EDS symptoms and genetic basis, suggestions for management, and resources available for health care providers, families, and patients with EDS are provided.