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Von Willebrand disease (also called pseudohemophilia) is the most common inherited bleeding disorder in humans and dogs. The disease rarely occurs in cats. The blood of those with this genetic disease does not clot normally. When blood doesn’t clot, especially in cases of trauma and surgery, excessive blood loss can lead to anemia and worse ... life threatening blood loss. Signs include bleeding from the gums, nose bleeds, and excessive bruising. There is no cure for von Willebrand disease, but knowing if your pet has the disease is important so that the proper precautions can be taken to prevent bleeding episodes.
Coagulation (blood clotting) is a defensive physiological process that occurs — in part — as a sequential biological cascade. But the action of vessel walls and their interaction with blood components called platelets is another crucial mechanism in coagulation.
The interaction between platelets and the vessel wall requires a molecule called von Willebrand factor. When there is not enough of this protein, the ability of the blood to clot is compromised — typically mildly, but often severely enough to be life threatening in the event of trauma or surgery. Von Willebrand disease is the term veterinarians apply to this condition.
Mildly affected dogs may experience prolonged bleeding only after surgery or trauma, or may even live an entire lifetime with the disease undetected. Severely affected dogs may present as pups with uncontrollable bleeding from their gums (after losing puppy teeth, for example), spontaneous bleeding from their noses, gastrointestinal tracts, or under their skin. Bleeding into joints, as sometimes occurs with active pups, inevitably causes lameness.
More uncommon signs may include difficulty breathing (bleeding into the airways), paralysis (bleeding in the spinal cord), and sudden death due to massive blood loss following simple trauma.
Once any clotting disease is suspected, your veterinarian may recommend blood tests to determine the degree of clotting dysfunction and degree of platelet deficiency. Diagnosis is made with a very specific test to measure the von Willebrand factor deficiency. There is also a test for the presence of the gene in certain breeds.
A simple screening test can be performed for dogs of at-risk breeds: the buccal mucosal bleeding time. In this test, a veterinarian makes a small cut on the dog’s inner lip (sedation may be needed for some pets) and measures the time required for the bleeding to stop. A prolonged bleeding time may indicate a bleeding disorder. The veterinarian can then test for von Willebrand disease.
All dogs diagnosed with von Willebrand disease should also be tested for other disorders that may contribute to bleeding tendencies.
While this disease has occurred in more than 50 breeds of dogs, Doberman Pinschers, German Shepherds, Golden Retrievers, Poodles and Shetland Sheepdogs are commonly affected. Any breed, including mixed breeds, may theoretically inherit the disease, too.
There is no cure for von Willebrand disease. In the event of a bleeding problem, dogs can be treated with transfusions of blood or plasma products to increase the amount of von Willebrand factor in the system. A synthetic hormone called desmopressin acetate may also be given to help the dog increase its level of von Willebrand factor.
The ideal mode of prevention involves careful screening of breeding dogs that are known to be genetically predisposed to this disease.
Transfusions may be given before, and if necessary, after surgery to help prevent excessive bleeding. After treatment, the dog should be kept on stick cage rest and monitored until all bleeding has resolved.
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