We are often asked what are the risks for Annabelle and others suffering with Vascular EDS, please share to help raise awareness of the risks so others can understand what we are going through: The major risks for patients with vascular EDS are arterial (including aortic) dissection, rupture and aneurysm. The sigmoid colon is the commonest site for bowel rupture - this can be seen at all ages. Obstetric complications include uterine and arterial rupture as well as massive postpartum haemorrhage and severe lacerations from tearing at vaginal delivery. The most important complication in vascular EDS is rupture of internal organs. This most commonly affects arterial blood vessels, which are fragile and prone to aneurysms (dilatations). Arterial rupture accounts for the majority of deaths in vascular EDS and any artery may be affected, although 50% occur in the chest and abdomen, 25% in the head and neck and the rest in the extremities. The bowel is another site prone to rupture (occurs in 25% of individuals) and involves predominantly the lower bowel (colon). Catastrophic events can potentially occur at any age but are rare before puberty. A quarter of individuals experience a significant medical complication by age 20 years and more than 80% by age 40 years. Type III collagen predominates in blood vessels, uterus and bowel. It is also present in skin and tendons. Mutations (faults) in the COL3A1 gene responsible for collagen III causes reduced, or abnormal, production of collagen which in turn leads to the clinical features of vascular EDS. Vascular EDS is a serious condition that is associated with a risk of sudden death due to arterial rupture at any age. annabelleschallenge.org
Posted on: Fri, 01 Aug 2014 18:00:01 +0000
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