Bone fragility in patients affected by congenital diseases non skeletal in origin
A Vet's Guide To Von Willebrand Disease In Dogs
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Von Willebrand disease in dogs is the most common inherited blood disorder our canine friends suffer with, and it also happens in humans and many other species. It's a bleeding disorder – a failure of the blood to clot – with symptoms that can vary from mild to severe.
Luckily, von Willebrand Disease is rare enough that I haven't had to deal with many dogs with the disorder in my eight years of being a vet, but I still keep a close lookout for it.
In this article, I'll explain what von Willebrand Disease is and – if your dog is unlucky enough to have it – what the likely outcome will be. Just to reassure you though, that the most common form of von Willebrand disease in dogs is mild and has a near-normal life expectancy.
What is Von Willebrand disease in dogs?Von Willebrand Disease (vWD) is an inherited blood clotting disorder, similar (but different to) hemophilia. It is known to affect dogs, humans, and many other species, but it's not contagious – it's genetic, with the type of inheritance pattern determined by the gene mutation that causes the problem.
When there's an injury that's bleeding, platelets gather at the site of the injury, forming a mesh to reduce the blood flow. Von Willebrand's factor is a protein complex made up of several small and large proteins that together act like glue to hold the platelets together.
Dogs with von Willebrand's Disease have a von Willebrand factor (vWF) deficiency. They don't have enough of the proteins to hold their platelets together when they're bleeding. The type of deficiency they have determines the 'type' of vWD they have – and therefore how serious the disease is.
Golden retriever being assessed at the vets
Type 1 von Willebrand Disease in dogsType 1 von Willebrand Disease is the most common type, and also the mildest. It occurs when dogs have a mild deficiency in all the proteins making up their von Willebrand factor, but the proteins have a normal structure. These dogs may have no symptoms or have slow clotting that doesn't cause major issues.
Type 2 von Willebrand Disease in dogsType 2 von Willebrand Disease happens when dogs have no large vWF proteins and have low levels of the smaller proteins. This can slow down clotting a lot, meaning these dogs can have severe bleeding episodes.
Type 3 von Willebrand Disease in dogsType 3 von Willebrand disease is when dogs have low or no von Willebrand factor at all – less than 1% of normal. This slows clotting significantly, meaning this is the most severe form of von Willebrand disease in dogs.
What dog breed is most affected by von Willebrand's disease?Because von Willebrand Disease is a genetic defect, it's inherited. However, inheritance patterns are complicated in vWD, with different genes mutations possible and different inheritance patterns for each. It's thought that Type 1 vWD is incompletely dominant, while Type 2 and 3 vWD are recessive.
In terms of actual proportions, the dog breed most affected by von Willebrand Disease is the Doberman Pinscher, which tend to get Type I disease. Other dog breeds affected by von Willebrand include:
Shetland Sheepdog (Type 1 and Type 3)
German Shepherd Dog (Type 1)
Standard Poodle (Type 1)
Airedale (Type 1)
Bernese Mountain Dog (Type 1)
Golden Retriever (Type 1)
Corgi (Type 1)
Greyhound (Type 1)
Schnauzer (Type 1)
German Short-Haired Pointer (Type 2)
German Wire-Haired Pointer (Type 2)
Scottish Terrier (Type 3)
Chesapeake Bay Retrievers (Type 3)
Other breeds have also been recorded to have von Willebrand disease, but these are usually sporadic and/or isolated cases.
Schnauzer being held by a vet
What are the symptoms of von Willebrand disease in dogs?Dogs with von Willebrand Disease can have a range of symptoms. Some dogs with the mild form of the disease can be asymptomatic for much of their lives until another disease pushes them into clinical symptoms. This is especially true if they aren't neutered and haven't had any injuries that would bleed. Most dogs, however, have symptoms in the first year or two of life, including:
Bruising after surgery
Slow to stop bleeding after an injury
Excessive bleeding when the puppy teeth are lost
Excessive bleeding during heats (seasons) or birth
Spontaneous bleeding from the nose or mouth
Bloody urine
Bloody stool (see our blood in dog stool article for more information)
Unexplained bruising
Spotty dog gums (see our dog gums article for more information on what unhealthy gums look like)
Uncontrollable bleeding during surgery or after injury, which may be fatal
If your dog has any of the symptoms above, it's sensible to talk to your vet, especially if your dog is listed in the susceptible breeds.
Your vet will have a suspicion of von Willebrand disease from your dog's symptoms and history. A Buccal Mucosal Bleeding Time test can help to confirm slow clotting – a standardized tool makes a small incision in your dog's gums, and your vet times how long it takes to stop bleeding.
If this test confirms that clotting time is abnormal in your dog, your vet may recommend a von Willebrand factor assay – a test to measure the amount of von Willebrand factor your dog has in their blood. The result is given as a percentage of normal. Less than 70% is considered affected, and less than 50% is likely to cause symptoms. Dogs with Type 3 vWD have values of 1% or less.
Doberman having his gums assessed by a male vet
DNA tests can also tell whether your dog has vWD, whether they're unaffected, or whether they're a carrier somewhere in the middle – unaffected but able to pass on dodgy genes. However, it's important to remember that the relationship between genes and disease is complicated – testing your dog's percentage of von Willebrand factor helps to determine if your dog is likely to be symptomatic for the disease.
Before breeding, owners of affected breeds should consider a the best DNA dog test to determine whether either parent carries a von Willebrand gene. The same von Willebrand factor assay above can also give an indication of how affected a dog is, and how likely they are to pass on a bad gene.
What is the best treatment for von Willebrand disease in dogs?Canine von Willebrand Disease can't be treated as it is a genetic disorder, but we can help manage the risk of a bleed, and treat that bleed aggressively if it happens. This means avoiding rough play, being careful clipping nails, and monitoring your dog closely for bruising or bleeding. Injuries will need to be treated rapidly, with skin glue and bandages used to help staunch the flow of blood, and plasma transfusions if the bleeding isn't stopping.
It's also important to let your veterinary practice know if your dog has von Willebrand Disease. If dogs are known to have vWD, it may limit their options for treatment of other conditions, as there are several medications that dogs may not be able to have if they've got vWD, for example:
Aspirin
Heparin
Some antibiotics
NSAIDS
Having von Willebrand Disease also means that surgery is a lot more risky, and it should be avoided in any dogs with symptoms. If surgery is needed, dogs will need to be transfused with plasma products, which contain the proteins necessary to help stop bleeding. This can be very effective, but the effect only lasts about four hours before wearing off, so it isn't a long-term option.
You might see a hormone called desmopressin acetate (DDAVP) mentioned when reading about treating von Willebrand Disease in dogs. DDAVP is only a temporary solution, as it only lasts for about two hours and long-term use of this hormone causes side effects.
Female vet assessing a brown sheepdog
What's the prognosis for dogs with von Willebrand disease?Despite the lack of good treatment options, most dogs with Type 1 vWD will have a normal life expectancy, unless they're unlucky enough to have a severe injury or a disease that requires risky surgical treatment. Dogs with Type 2 and Type 3 vWD may also make it to old age, but the more severe their disease the less likely they can be kept safe enough to prevent bleeds.
At-home dog DNA tests mean that more people are aware of their dog's vWD status than before. While it's sensible to limit risky activities for these dogs, they may never be clinically affected and it may never impact their quality of life or life expectancy.
Von Willebrand Disease is thankfully fairly rare, and many dogs will have the mildest form of the disease. If your dog has symptoms suggesting slow clotting or spontaneous bleeding, they will need investigations. Dogs found to have abnormal concentrations of von Willebrand factor are at risk of symptoms and steps should be taken to avoid surgery and keep them safe.
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Vonvendi Gains Expanded Approval In Von Willebrand Disease
The Food and Drug Administration (FDA) has expanded the approval of Vonvendi® (von Willebrand factor [recombinant]) to include routine prophylaxis to reduce the frequency of bleeding episodes in adults with von Willebrand Disease (VWD), including those with Type 1 and Type 2 disease, and on-demand and perioperative management of bleeding in pediatric patients with VWD.
Vonvendi is a recombinant von Willebrand factor previously only approved for on-demand and perioperative use in adults with VWD and routine prophylactic use in adults with severe Type 3 VWD receiving on-demand therapy.
The expanded approval was based on data from multiple clinical trials in patients with VWD, including a phase 3 pediatric study (Study 3; ClinicalTrials.Gov Identifier: NCT02932618), a phase 3 adult study (Study 5; ClinicalTrials.Gov Identifier: NCT02973087), and a phase 3b continuation trial in adults and children (Study 6; ClinicalTrials.Gov Identifier: NCT03879135).
Study 3 was an open-label trial that evaluated the efficacy of Vonvendi with or without Advate for on-demand treatment of non-surgical bleeding episodes in 18 pediatric patients (median age, 11). The primary endpoint was the number of patients with treatment success, defined as a mean efficacy rating score of less than 2.5 for all bleeding episodes using the pre-specified 4-point efficacy rating scale.
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Children and adults living with VWD face unique challenges, including bleeds that can disrupt daily activities like school, work or spending time with loved ones, often leading to physical and emotional burdens.
Findings showed there were 104 nonsurgical bleeding episodes treated with Vonvendi, with or without Advate. Among the 98 evaluable bleeding episodes, the hemostatic efficacy rating count was "Excellent" (1 rating score) in 99% of bleeding episodes and "Good" (2 rating score) in 1%.
Additionally, Study 3 evaluated the efficacy of Vonvendi with or without Advate for the management of surgical bleeding from elective and emergency surgeries in 4 pediatric patients aged 6 to 12 years with severe VWD. Four surgeries (3 elective and 1 emergency) were treated with Vonvendi.
Results showed that all 4 surgeries had overall and intraoperative hemostatic efficacy ratings of "Excellent" (95% CI, 39.8-100). The blood loss rating was also "Excellent" for all surgeries.
Study 5 was a single-arm, open-label trial that evaluated the efficacy and safety of prophylactic treatment with Vonvendi in reducing the frequency of bleeding episodes in adults diagnosed with VWD. Patients who received on-demand treatment only (n=12; 1 with Type 1, 1 with Type 2, and 10 with Type 3 VWD) were enrolled into 1 treatment arm while those who had received previous prophylactic treatment with plasma-derived von Willebrand factor (n=10; 1 with Type 1, 1 with Type 2, and 8 with Type 3 VWD) were enrolled into a switch arm.
Findings showed in the prior on-demand treatment arm, 91.7% achieved annualized bleeding rate (ABR) reduction success, defined as at least 25% reduction in the ABR for spontaneous bleeding episodes requiring factor treatment from the patient's historical to the on-study period. In the Switch arm, 90% of patients achieved preservation success, defined as achieving an on-study ABR for spontaneous bleeding episodes requiring factor treatment that was no greater than the patient's historical ABR for treated bleeding episodes.
The most common adverse reactions reported with Vonvendi were headache, vomiting, nausea, dizziness, and generalized pruritus.
"Children and adults living with VWD face unique challenges, including bleeds that can disrupt daily activities like school, work or spending time with loved ones, often leading to physical and emotional burdens," said Cheryl Schwartz, Senior Vice President, US Rare Disease Business Unit Lead and US Commercial Operations at Takeda. "We are proud to have supported adults with VWD since Vonvendi's initial approval in 2015 and will now be able to help even more patients manage their condition."
This article originally appeared on MPR
U.S. FDA Approves Expanded Indication For VONVENDI® [von Willebrand Factor (Recombinant)] For Adults And Children With Von Willebrand Disease
− Approval Expands Use of VONVENDI to Include Routine Prophylaxis to Reduce the Frequency of Bleeding Episodes in Adults with Type 1 and 2 Von Willebrand Disease (VWD) and On-Demand and Perioperative Management of Bleeding in Pediatric Patients with VWD1
− VONVENDI is the Only Recombinant Von Willebrand Factor Replacement Therapy with Approved Indications in Adults and Children with VWD1
− VWD, the Most Common Bleeding Disorder, Impacts More Than 3 Million People in the U.S.2
OSAKA, Japan & CAMBRIDGE, Mass., September 06, 2025--(BUSINESS WIRE)--Takeda (TSE:4502/NYSE:TAK) today announced that the U.S. Food and Drug Administration (FDA) has approved the supplemental Biologics License Application (sBLA) for VONVENDI® [von Willebrand factor (Recombinant)], expanding the indication to include routine prophylaxis to reduce the frequency of bleeding episodes in adults with von Willebrand Disease (VWD), including those with Type 1 and 2 disease, and on-demand and perioperative management of bleeding in pediatric patients with VWD. VONVENDI was previously approved for on-demand and perioperative use in adults with VWD and routine prophylactic use in adults with severe Type 3 VWD receiving on-demand therapy.1
VONVENDI is now available for use in a broad range of VWD patients and is the only recombinant von Willebrand Factor (VWF) replacement therapy with approved indications in both adults and children with VWD.1
VWD is the most common bleeding disorder impacting more than 3 million people in the U.S. Alone. It occurs equally among men and women. People with VWD have low levels of or dysfunctional von Willebrand factor (VWF), a protein that helps blood clot. This can lead to life-threatening bleeding – either from an injury or after surgery, gastrointestinal bleeding, dental procedures or childbirth – prolonged nosebleeds, heavy or long menstrual bleeding and easy bruising, which can have a significant impact on quality of life.2,3
VONVENDI is an infused medicine designed to replace the body's missing or dysfunctional VWF. VONVENDI has a uniquely long half-life (22.6 hours [50 IU/kg] for adults and 14.3 hours for children). In clinical trials, the majority of non-surgical bleeds were treated with one VONVENDI infusion in both adults (157/192) and children (80/104).1
"The FDA's approval of VONVENDI for routine prophylactic use in adults with all VWD types marks a pivotal milestone in advancing care for this challenging condition. Prophylactic treatment is critical to reduce the frequency of bleeds in adults, including spontaneous and joint bleeds," said Jonathan C. Roberts, M.D., associate medical and research director at the Bleeding and Clotting Disorders Institute, associate professor of pediatrics and medicine at the University of Illinois College of Medicine at Peoria, and an investigator in the VONVENDI prophylaxis study. "Establishing prophylaxis as the standard of care for all adults with VWD, regardless of subtype, represents a significant step forward in helping to manage bleeding risk and the burdens that bleeds can have on patients' daily lives."
This approval is based on data from three clinical trials – a Phase 3 trial in adults with VWD (NCT02973087), a Phase 3 study in children with VWD (NCT02932618) and a Phase 3b continuation trial in adults and children with VWD (NCT03879135) – as well as supportive real world data.1 Please see the VONVENDI full Prescribing Information for details on the established clinical profile.
"Children and adults living with VWD face unique challenges, including bleeds that can disrupt daily activities like school, work or spending time with loved ones, often leading to physical and emotional burdens," said Cheryl Schwartz, Senior Vice President, U.S. Rare Disease Business Unit Lead and U.S. Commercial Operations at Takeda. "We are proud to have supported adults with VWD since VONVENDI's initial approval in 2015 and will now be able to help even more patients manage their condition. This milestone highlights Takeda's unwavering commitment to the blood and bleeding disorders community as we strive to deliver innovative therapies."
Takeda submitted applications seeking approval to expand the indications of VONVENDI for on-demand and perioperative management of bleeding in pediatric patients with VWD in Japan and VEYVONDI™ (vonicog alfa) for on-demand use in pediatric patients with VWD in the European Union.
VONVENDI [von Willebrand factor (Recombinant)] Important Information
IndicationsVONVENDI [von Willebrand factor (recombinant)] is indicated in adult and pediatric patients with von Willebrand disease (VWD) for:
For adult patients only:
Detailed Important Risk Information
CONTRAINDICATIONSVONVENDI is contraindicated in patients who have had life-threatening hypersensitivity reactions to VONVENDI or constituents of the product (tri-sodium citrate-dihydrate, glycine, mannitol, trehalose-dihydrate, polysorbate 80, and hamster or mouse proteins).
WARNINGS AND PRECAUTIONSThromboembolic EventsThromboembolic events have occurred with VONVENDI. These events can include disseminated intravascular coagulation (DIC), venous thrombosis, pulmonary embolism, myocardial infarction, and stroke. Patients with known risk factors for thrombosis, including low ADAMTS13 levels are at a higher risk. Monitor patients for signs and symptoms of thrombosis such as pain, swelling, discoloration, dyspnea, cough, hemoptysis, and syncope. Institute prophylaxis measures against thromboembolism according to current clinical practice and standard of care.
In patients requiring frequent doses of VONVENDI in combination with rVIII, monitor plasma levels for FVIII:C activity because sustained excessive factor VIII plasma levels can increase the risk of thromboembolic complications.
Hypersensitivity and Infusion-Related ReactionsHypersensitivity reactions and infusion-related reactions have occurred with VONVENDI. These reactions can include anaphylactic shock, generalized urticaria, angioedema, chest tightness, hypotension, shock, lethargy, nausea, vomiting, paresthesia, pruritus, restlessness, blurred vision, wheezing and /or acute respiratory distress. If signs and symptoms of severe allergic reactions occur, immediately discontinue administration of VONVENDI and provide appropriate supportive care.
VONVENDI contains trace amounts of mouse immunoglobulin G (MuIgG) and hamster proteins less than or equal to 2 ng/IU VONVENDI. Patients treated with this product may develop hypersensitivity reactions to non-human mammalian proteins.
Neutralizing AntibodiesNeutralizing antibodies to VWF and/or factor VIII can occur with VONVENDI. If the expected plasma levels of VWF activity (VWF:RCo) are not attained, perform an appropriate assay to determine if anti-VWF or anti-factor VIII inhibitors are present. Consider other therapeutic options and direct the patient to a physician with experience in the care of either VWD or hemophilia A.
In patients with high levels of inhibitors to VWF or factor VIII, VONVENDI therapy may not be effective, and infusion of this protein may lead to severe hypersensitivity reactions. Since inhibitor antibodies can occur concomitantly with anaphylactic reactions, evaluate patients experiencing an anaphylactic reaction for the presence of inhibitors.
Monitoring Laboratory TestsMonitor plasma levels of VWF:RCo and factor VIII activities in patients receiving VONVENDI to avoid sustained excessive VWF and/or factor VIII activity levels, which may increase the risk of thrombotic events, particularly in patients with known clinical or laboratory risk factors.
Monitor for development of VWF and/or factor VIII inhibitors when suspected. Perform appropriate inhibitor assays to determine if VWF and/or factor VIII inhibitors are present if bleeding is not controlled with the expected dose of VONVENDI.
ADVERSE REACTIONSThe most common adverse reactions observed in greater than or equal to 2% of patients in clinical trials with VONVENDI (n=132) were headache, vomiting, nausea, dizziness, and generalized pruritus.
Please see VONVENDI full Prescribing Information.
About von Willebrand Disease (VWD)VWD is the most common inherited bleeding disorder, affecting up to one percent of the U.S. Population. VWD is caused by a deficiency or dysfunction of von Willebrand factor (VWF), one of several types of proteins in the blood that are needed to facilitate proper blood clotting. Due to this defect or deficiency in VWF, blood is not able to clot effectively in people with VWD which may result in heavy menstrual periods, easy bruising, or frequent nose bleeds. Bleeding caused by VWD varies greatly among patients with this disease.2
About TakedaTakeda is focused on creating better health for people and a brighter future for the world. We aim to discover and deliver life-transforming treatments in our core therapeutic and business areas, including gastrointestinal and inflammation, rare diseases, plasma-derived therapies, oncology, neuroscience and vaccines. Together with our partners, we aim to improve the patient experience and advance a new frontier of treatment options through our dynamic and diverse pipeline. As a leading values-based, R&D-driven biopharmaceutical company headquartered in Japan, we are guided by our commitment to patients, our people and the planet. Our employees in approximately 80 countries and regions are driven by our purpose and are grounded in the values that have defined us for more than two centuries. For more information, visit www.Takeda.Com.
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References:
VONVENDI [von Willebrand factor (recombinant)] Prescribing Information; 2025. Https:/www.Shirecontent.Com/PI/PDFs/VONVENDI_USA_ENG.Pdf
What is von Willebrand disease? Centers for Disease Control and Prevention website. Accessed July 9, 2025. Https://www.Cdc.Gov/von-willebrand/about/?CDC_AAref_Val=https://www.Cdc.Gov/ncbddd/vwd/facts.Html
Nicholas L.J. Chornenki, Edwin Ocran, Paula D. James; Special considerations in GI bleeding in VWD patients. Hematology Am Soc Hematol Educ Program 2022; 2022 (1): 624–630. Doi: https://doi.Org/10.1182/hematology.2022000390
View source version on businesswire.Com: https://www.Businesswire.Com/news/home/20250905840795/en/
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Media ContactLindsey ColegroveLindsey.Colegrove@takeda.Com+1 617 306 5718
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