Von Willebrand's Disease

What is von Willebrand's disease?

von Willebrand's disease (vWD) is the most common inherited bleeding disorder of both man and animals. It is caused by a deficiency in the amount of a protein needed to help platelets (a blood cell used in clotting) seal broken blood vessels. The deficient protein is called "von Willebrand factor antigen".

Which breeds are most commonly affected by vWD? 

About 30 different breeds are known to be affected but the Dobermann is the breed most commonly associated with this disease. A 1995 study found 61% of Dobermanns in Australia to be carriers of the disease. Fortunately, most of these are not clinically affected (i.e. we see no evidence of bleeding). However, the number of Dobermanns with a history of bleeding appears to be on the increase. Although Dobermanns are commonly affected, they usually have the mildest form of the disease. Dobermanns are, on average, four years old before diagnosis is made.

One study showed that 30% of Scottish Terriers and 28% of Shetland Sheepdogs had abnormally low concentrations of von Willebrand factor. The German Shepherd dog can also be affected with the disease.

What are some of the signs of vWD?

Many dogs with vWD never show outward evidence of having the disease. Others may haemorrhage from the nose, vagina, urinary bladder or oral mucous membranes; prolonged bleeding after trauma or surgery is common. Females may bleed excessively while in oestrus or after giving birth. In affected dogs with uncontrolled haemorrhage, death may occur.

"Many dogs with vWD never show outward evidence of having the disease. Others may haemorrhage from the nose, vagina, urinary bladder or oral mucous membranes; prolonged bleeding after trauma or surgery is common."

How is vWD diagnosed?

A screening test, called the buccal mucosal bleeding time, may be performed by your veterinarian. Prolonged bleeding on this test can raise the suspicion of the disease, especially in breeds known to be at risk. For owners who wish to confirm the diagnosis, it is possible to determine the exact amount of von Willebrand protein present in the blood. 

Owners of Dobermanns often report that the pet has undergone routine ovariohysterectomy (spay) or castration, or trim, and tail docking as a pup. An uncomplicated recovery from such procedures does not eliminate the possibility that a dog may be affected; some dogs do not become obvious "bleeders" until later in life. 

Are there any situations which pose an increased risk if my dog is affected?

The avoidance of certain medications is critical for the dog with vWD. Drugs that may precipitate a bleeding crisis in the dog include the following:

• Ampicillin/Amoxicillin
• Aspirin
• Antihistamines
• Heparin
• Ibuprofen
• Oestrogens
• Penicillin
• Phenothiazine tranquilizers
• Phenylbutazone
• Sulphur-based antibiotics
• Theophylline

These drugs should be avoided when possible, but especially if the dog is in a bleeding crisis.

Emotional stress is thought to precipitate bleeding in humans with the disease. The subjective nature of such a finding makes it difficult to know if there is a similar association in dogs but it remains a possibility.

What can be done to treat dogs with vWD?

In an emergency situation, transfusion of blood or fresh frozen plasma may stabilise the patient. The dog donating blood may be treated with a drug called DDAVP prior to blood collection which will raise the level of von Willebrand factor in the donor's blood, an obvious benefit to the recipient. 

Some dogs with vWD are able to increase the amount of protein in circulation after the administration of DDAVP, although the response is variable. At this time, it is not recommended to use this drug on a regular basis. The drug is expensive, and not all dogs will respond. 

If I own a Dobermann that has always been healthy, should I do something?

Since many affected Dobermanns will never have bleeding problems, any recommendation to do routine testing is debatable. However, identification of dogs that have abnormal bleeding times can be very valuable if surgery is planned. Additionally, knowing that your dog is a carrier of vWD can be very important if an injury occurs.