As a child, doctors thought I might have VonWillebrand disease.
As an adult, I've been diagnosed with Vascular EDS. Is this common?
In childhood many individuals with Vascular EDS are first thought to
have a coagulation disorder. Bleeding disorders can arise from
disorders of the: vessel wall defects, platelets, and coagulation.
There are some patients with EDS, however, who have been diagnosed with
Von Willebrand, Factor deficiencies, and even Haemophilia. Most
laboratory studies reveal bleeding and clotting times to be normal,
even though most require blood transfusions and intravenous
alimentation. Recent studies have shown that the increased bleeding
tendency probably is due to a defect in the collagen structure of the
vascular and perivascular tissues. Possible capillary fragility and
venous varicosities may also occur. There is also a reduction in the
ability of abnormal collagen in patients with EDS to attract platelets,
which may contribute to the bleeding tendency. (Karaca.et.al "Abnormal
platelet collagen reaction in EDS" J Haematol 1972; 9:465-9. Wesley TA.
"Multiple surgical problems in two patients with EDS" Surgery 1980;87