The Food and Drug Administration (FDA) of the U.S. permitted Von Willebrand in December 2015 and it is designated for on-demand treatment and management of bleeding episodes in persons aged 18 years and above with VWD. Von Willebrand is the first and only Von Willebrand treatment for persons living with VWD. The trials are projected to end in 2019 and 2020, respectively.
In the U.S., von Willebrand recombinant is known by the brand name vonvendi. Von Willebrand recombinant is available as generics which are cheaper than the original brand.
Von Willebrand factor is available in powder form and has to be diluted with a liquid before use. Patients need to understand how to mix and store the drug well, especially if they use it at home. The diluted von Willebrand factor should be used within three hours if it has been stored at room temperature.
Vonvendi is a filtered recombinant von Willebrand factor expressed in Chinese Hamster Ovary cells. It is formulated and produced without the addition of any exogenous raw materials either human or animal origin in the purification, cell culture, or formulation of the last product.
The only proteins available in the finished container product other than rVWF are small quantities of hamster or mouse immunoglobulin from the host cell protein, and recombinant factor VIII.
Von Willebrand is administered via IV through the vein.
Side effects are rare. However, a patient needs to call a medical practitioner when they see the following outlined signs and symptoms:
Consider the following before use of VWF recombinant:
If you store VWF at room temperature:
Storing the mixed (reconstituted) product
The manufacturer has reported no drug interactions. There are no specific foods that should be avoided or excluded from the diet when getting the medication.
However, there are a number of drugs that may combine with von Willebrand factor, including over the counter medicines, prescription, herbal and vitamins products.
Von Willebrand factor is contraindicated in people who have had ruthless hypersensitivity response to Von Willebrand or ingredients of the drug (glycine, mannitol, tri-sodium citrate-dihydrate, polysorbate 80, trehalose-dihydrate, and mouse or hamster).
Before taking this medication, inform your doctor about all of your medical conditions. Especially if you:
Thromboembolic reactions, including disseminated intravascular coagulation (DIC), myocardial infarction, pulmonary embolism, venous thrombosis and stroke is likely to occur, mainly in patients with recognized threat factors for thrombosis.
Early symptoms and signs of thrombosis should be monitored such as swelling, pain, cough discoloration, syncope, dyspnea, and hemoptysis.
In patients that need frequent doses of von Willebrand with recombinant factor VIII, plasma levels for FVIII: C. Activity should be monitored since an undue rise in factor VIII levels can add to the risk of thromboembolic difficulty.
Hypersensitivity reactions, including anaphylaxis, are likely to occur. Symptoms may consist of generalized urticaria, anaphylactic shock, chest tightness, angioedema, hypotension, nausea, shock, lethargy, vomiting, restlessness, paresthesia, pruritus, and wheezing as well as acute respiratory distress.
Immediately discontinue administration of von Willebrand if symptoms and signs of harsh allergic reactions arise, and provide proper supportive care.
Neutralizing antibodies to von Willebrand factor and factor VIII is likely to happen. If the anticipated plasma levels of VWF activity are not attained, an appropriate assay is supposed to be performed to determine if anti-VWF or anti-FVIII inhibitors are there. Other therapeutic option should be considered. The patient should then be referred to a physician with understanding in the care of both von Willebrand disease and hemophilia A.
For the most part, widespread unpleasant result seen in 2% of subjects in clinical trials (n=66) was generalized pruritus as no safety alarm was identified.
Von Willebrand disease is the most frequent inherited bleeding disorder, distressing 1% of the people in the U.S., according to the FDA. A defect in von Willebrand factor, which is a protein vital for clotting, causes it.
Patients with von Willebrand disease can cause severe bleeding from the gums, nose, and intestines, as well as into joints and muscles. Women who have von Willebrand disease are likely to have substantial menstrual periods that last longer than average and also experience too much bleeding after childbirth.
Von Willebrand resolves bleeding episodes, with a median of only two infusions to take care of severe bleeds.