Von Willebrand Disease - Ask The Pharmacist

Apr 15, 2014

Specialist pharmacists provide medication management tips for patients with von Willebrand disease.

Most bleeding disorders are rare. However, von Willebrand Disease (vWD) is a relatively common – if not well understood – bleeding disorder. Approximately 1 in 100 people are affected by vWD but many patients go undiagnosed because their symptoms may be very mild. It is a genetic disorder that affects men and women equally.

Von Willebrand is one of the diseases in which the specialist pharmacists in the Express Scripts Bleeding Disorder Therapeutic Resource Center, offered through Accredo, have disease-specific expertise. We help patients understand and manage their condition and treatments.

What is vWD?

Von Willebrand factor (vWf) is produced in the bone marrow and the lining of the blood vessels where it is stored. VWf is released into the blood when an injury occurs and works with platelets to start the clotting process, which ultimately stops the bleeding. Inadequate stores of vWf can result in uncontrollable or poorly controlled bleeding.

Symptoms of vWD

Symptoms of vWD can range widely depending on the person’s gender and type of vWD. They can be as minor as light bruising or small knots under a bruised area. More serious symptoms include bleeding from the gums and prolonged bleeding after a dental extraction, blood in the urine (kidney bleeding) or bleeding in the stomach or intestines.

Female patients can have additional symptoms including prolonged or excessively heavy menstrual bleeding, which in some cases, are very serious – requiring blood transfusion until the symptoms are controlled. Post partum bleeding is of concern as well.

Types of vWD

There are three types of vWD. A vast majority – nearly 75% – of those diagnosed have Type 1 vWD. Patients with Type 1 have reduced levels of vWf and typically experience mild symptoms. People with Type 2 have enough clotting factor but it does not function correctly. Type 3 is the most serious and rarest type of vWD. Patients with Type 3 have the most severe deficiency of vWf and therefore experience the most significant bleeding including joint bleeding and require treatment with intravenous clotting factor containing vWf.

Treatment

There is no cure for vWD, but the symptoms can be treated and managed. Treatment varies depending on the patient and the severity of symptoms. Symptoms of Type 1 and some forms of Type 2 are often treated with an intranasal spray (Stimate), also available in injectable form.

Aminocaproic acid and tranexamic acid are oral medications that help prevent clots that have formed from breaking down. They are often used to prevent bleeding from dental procedures and for heavy menstrual bleeding in girls and women. Some other forms of Type 2 and Type 3 are treated with intravenous plasma-derived clotting factor.

For patients with any form of vWD, here are a few useful medication management tips to keep in mind to ensure that their condition is well controlled:

Use as directed: Nasal sprays, injectables and intravenous medications for vWD require a hematologist’s supervision and approval and should be used exactly as directed. Over-dosing or under-dosing can be dangerous and potentially fatal.

Watch what you eat: Sharp foods such as corn chips may scratch/pierce the oral mucosa and cause bleeding. Chewing gum may result in tongue bites. Hot foods and beverages can cause vasodilation, causing bleeding to worsen.

Do not self-adjust your medications: Always consult a hematologist or an appropriate clinician for questions about dosing changes. Missing doses or discontinuing the medication could cause the bleeding to resume.

Have first-aid handy: Keep popsicles in the freezer as a first line of care for oral bleeding until medication can be administered as ordered by the hematologist. The frozen treat aids in vasoconstriction, which is part of the first stage of coagulation. Ice packs for topical use should be in the freezer as well.

Cover all contingencies: In case of young patients, have a plan and discuss it with the school nurse and teachers. Ensure that medication is available in the school clinic for bleeding disorders patients.

Talk to your clinician: Heavy menstrual bleeding can be dangerous and must be reported to the hematologist or clinician. Let your hematologist know at least 2 weeks prior to surgery or dental work. Report any symptoms or changes.

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