Nursing care for patients with the Ehlers-Danlos and other hypermobility syndromes

Abstract

Ehlers-Danlos syndrome is a rare genetic disorder, with mainly three symptoms: hypermobility, fragile skin and fragile veins. In this article we discuss the syndrome and we consider the consequences for nursing care of the three most common types of EDS. Starting point are the health patterns of Gordon in combination with peri-operative situations. Ehlers-Danlos syndrome (EDS) comprises a clinically and genetically heterogeneous group of inherited connective tissue disorders, which are mainly characterized by a variable degree of generalized joint hypermobility, skin hyperextensibility, easy bruising and skin fragility. Two facts have to be taken into consideration: firstly, the severity of the manifestation of the syndrome varies among patients, even among members of the same family. It is not possible to make sharp distinctions based on clinical findings between the types. Secondly, it is impossible to clearly distinguish the different types based on clinical findings, as the syndrome manifests itself in many different ways. So far, there are no simple and reliable clinical tests or laboratory test. Furthermore, patients often have additional problems, which are not characteristics of the known types of Ehlers Danlos. As a nurse it is important to be aware of the strongly varying needs of patients both during the day and night and their pain and fatigues, which all patients report, and report as being as very troublesome. It is essential that nurses, during hospi- talization, should bear in mind that patients may have to cope with hypermobility of the joints, fragile skin, possible dysfunctional vasoconstriction and the often present generalized autonomous disregulation. Because the pattern of complaints varies markedly from patient to patient, good communication between nurse and patient is very important.