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Health

Von Willebrand’s Disease: Inconvenience or Deal Breaker?
January 25, 2016 (published)
Rebekah Gunn-Christie, DVM, DACVP


(A note before we begin: The specific details of the owner and dog in this story are fabricated. The account is woven from the threads of stories of dogs affected by von Willebrand’s disease that I’ve encountered throughout my career. I could have told the story of just one dog…but then the story would have lacked important details. The scientific details are not in any way fabricated.)


Willa searched and searched for the perfect Doberman pinscher puppy. She’d had a Doberman when she was a young girl and, while she’d had many other dogs since that time, none had quite compared. Willa met with several breeders, trying to find just the right one with just the right dog. Finally, she found her perfect match, a beautiful adolescent puppy with a quiet sweetness conveyed from the limpid depths of her soft brown eyes. The breeder had wanted to keep the puppy, but then decided to get out of the breeding industry. Willa brought the puppy home and named her Vonnie. Vonnie quickly became the apple of Willa’s eye. Eager to please and quick to learn commands, Willa had a demeanor that bespoke of the quality of a long line of fine Dobermans. They lived happily ever after.

Until…

One day shortly after bringing Vonnie home, Willa accidentally cut Vonnie’s nail too short as she was trimming it. The “quicked” nail bled. And bled. And bled. After four hours the bleeding eventually stopped. Vonnie hadn’t lost much blood (really, it was more oozing than bleeding) and was feeling sassy after enjoying an afternoon with Willa cuddling her and holding her paw. However, Willa and her blood-stained floor were in a state of distress. Certainly, this was not normal. With a sinking feeling, Willa remembered a condition that she had come across in her search for Doberman pinscher breeders: von Willebrand’s disease, a bleeding disorder most commonly seen in certain breeds of dogs, including Doberman pinschers. Vonnie’s “new puppy” exam was scheduled for later that week. A worried Willa moved Vonnie’s appointment up to the next day.

Willa and Vonnie arrived early for their appointment with Dr. Kind. Vonnie charmed the staff and impressed them with her stellar behavior as she got her weight and temperature taken. Obviously, Vonnie was an extraordinary puppy! Dr. Kind examined Vonnie and agreed that she was an exceptional specimen of a Doberman. Vonnie’s temperament and conformation were exemplary. However, Dr. Kind’s normally jovial demeanor turned more serious when he began to discuss Vonnie’s bleeding episode. He voiced Willa’s concern: That Vonnie could have von Willebrand’s disease.

Dr. Kind had been Willa’s veterinarian for years. He'd cared for every sick or injured rescue that Willa had taken in. Willa had total confidence in his judgment.

He explained to Willa that there are three forms of vWD, an autosomal recessive disorder, all of which are inherited.  If one parent has it, and one parent is a carrier, the chance is 50% that the dog will get the faulty gene from the carrier parent. If both parents have it, the puppy will have it.

Type I is the least affected, and the proteins, or von Willebrand factors, that make the vWD factor are all there, just in small amounts. It is the most common type, accounting for 90 percent of cases. In Type II, the big factors are missing but the small proteins try to keep up, and Type II accounts for less than 10 percent of cases. In Type III, the dog, or cat, doesn't have any factors at all. Thus the dog either doesn't have any of those proteins or only has defective ones, and thus the blood cannot clot normally. Less than two percent of reported cases are Type III.

Dr. Kind said there are two tests, either of which they could choose to determine if Willa had the dreaded vWD. The von Willebrand factor assay is a blood test that measures the concentration of factors. Although it's widely used and an adequate test in many instances, it has a significant drawback: it has a large gray zone, meaning that concentrations in the mid-range are difficult to interpret.

The other is a genetic test that determines whether a dog is free, a carrier, or clinically affected by the mutation resulting in vWD. Five mutations have been identified that cause vWD in dogs, and there are genetic (DNA) tests for all five. However, at the time of this writing the genetic test is only available for 21 dog breeds. Besides Dobertmans, vWD has been found in Scottish terriers, Shetland sheepdogs, Chesapeake Bay retrievers, Dutch kookiers, Bernese mountain dogs, Manchester terriers, Pembroke Welsh corgis, poodles and several other dog breeds. Occasionally it's seen in cats.

Although either test would have been sufficient, Willa elected the genetic test. The test results came back: Willa had two defective copies of the gene. Distraught, Willa contacted the breeder with the results. The breeder told Willa that neither Vonnie’s dam nor her sire had showed the bleeding disorder, but reassured Willa that she would take the puppy back as part of her health guarantee. Willa did not understand why neither the dam nor sire had a clinical bleeding disorder when her darling Vonnie did.

Dr. Kind explained that having one defective gene for the disorder often does not cause problems, but having two defective genes does. Both of Vonnie’s parents had a mutated gene, but neither showed abnormal bleeding tendencies because they each also had a “normal” copy of the gene. Vonnie’s parents were carriers. However, Vonnie inherited a mutated gene from each parent and was affected.

Willa was at a crossroads. The breeder would take Vonnie back and refund Willa’s money, but Vonnie was the perfect puppy except for her one tragic flaw. Willa wasn’t sure what to do.

Willa reached out to other Doberman owners in her community as well as to Dr. Kind. Everyone basically said the same thing: there is no cure or daily medication. Living with a vWD dog boils down to special care that's needed during surgical procedures, including spaying and neutering, although it may be unecessary in some minor procedures such as dental cleanings.

Transfusion with cryoprecipitate can be used to control major bleeding episodes or in conjunction with surgical procedures. Cryoprecipitate is preferred over plasma because it contains five to ten times more von Willebrand's factor than plasma. However, if cryoprecipitate is not available, fresh frozen plasma can be given. Cost involves the basic cost of the product as well as administration costs, which are variable depending on whether it is given intravenously, such as for surgery, or applied topically for dental prophylaxis procedures such as cleanings. Planned surgical procedures would need to take into account time for delivery of cryoprecipitate from a veterinary blood bank (typically a few days, as most general practitioner veterinarians do not stock the product) and additional cost for the procedures beyond the "normal" baseline cost. As long as the veterinarian feels comfortable with the protocol for administration of cryoprecipitate and their surgical skills, many surgeries (such as spays and neuters) can be performed by general practitioners, and do not automatically need to be sent to a boarded surgeon.

Dr. Kind also pointed out that non-steroidal anti-inflammatories (NSAIDs) should be avoided, even for arthritis, because they increase bleeding tendencies. Chondroprotectant nutriceuticals, such as glucosamine, should also be avoided since they contain heparin-type substances that can also promote bleeding. 

In affected dogs, bleeding in the mouth is the most common sign, and can be seen when teething or vigorously chewing on toys. Excessive bleeding, although uncommon, can also occur with blood draws for routine tests.

Many vWB dogs have a good quality of life, but it is an undesirable condition even though it's nowhere near as severe as hemophilia. Although affected dogs don't have to live in a plastic bubble, caution is called for in situations where excessive roughhousing (such as dog parks, where fights may occur) or injury (such as taking a spill during an agility competition) are more likely to happen. Accidents such as getting hit by a car, or disease that causes internal bleeding, can wreak havoc on the best-laid plans. The real stress in living with a vWD dog is preventing injury, and sometimes that isn't possible. Willa would be justified in returning Vonnie to the breeder.

That evening, she and Vonnie curled up on the couch to watch the evening news and Vonnie reached up to lick her face. Willa knew her decision was made and Vonnie was staying. Vonnie wasn’t the perfect dog, but Willa felt neither was any other dog she would have found. Every pet has health issues of one kind or another. She didn’t plan to breed Vonnie, so there was no concern of passing on a defective gene to future generations. In Willa's case, she wanted to keep Vonnie no matter what her health issues were.

2 Comments

Karen Thompson
March 22, 2016

I'm a long-time Dobie lover and have had 2 vWD dogs.  Fortunately neither were terribly bad but one did have a lot of bleeding after a mammary tumor removal (rescue dog). The real issue is, will the breeder of "vonnie" make sure she never breeds that pair of dogs again?  More likely, she'll just match them with other "normal" dogs and hope for the best.


Holly
January 25, 2016

We never know how long our dogs will live. I, too, would have kept the dog/puppy and just appreciated every day I had with her.



 
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