The worst thing you can tell a hypochondriac such as myself, is that there is another illness out there waiting to be experienced. Case in point: The little-known bleeding disorder, von Willebrand Disease (vWD). While it sounds like something you can only catch from effete German aristocrats, it is, in fact, named after Erik Adolf von Willebrand, a Finnish pediatrician who first discovered it in 1926. And the only reason I am writing about it now, is that it turns out that I know someone who actually suffers from it, and she recently described her symptoms to me. (Note: When innocents start doing this and my husband, The Lawyer, is present, he desperately, tries to stop them before I personally start to manifest the symptoms!) Amazingly, it is remarkably common – if not well understood – and by some estimates, affects as many as 1 in 100 people. A genetic disorder, it is non gender-specific, meaning men and women are afflicted equally. Most cases, though, go undiagnosed because the symptoms tend to be very mild.
In order to find out more about vWD, I consulted with the specialty-trained clinicians in the Express Scripts Bleeding Disorder Therapeutic Resource Center, offered through Accredo, who have disease-specific expertise. They help patients understand and manage their condition and treatments. In other words, they are good for me to get to know. (Both the experts and the treatments, that is!)
Here is what Leslie Oygar, a clinical nurse liaison in the TRC, tells me:
Von Willebrand factor (vWf) is one of the key components of the process that helps blood clot. It is produced in the bone marrow and the lining of the blood vessels, where it is also stored. In 99% of us, VWf is released into the blood when an injury occurs and works with platelets to start the clotting process, which ultimately stops the bleeding. If the supplies of vWf are inadequate, it can result in uncontrollable, or poorly controlled, bleeding.
There are three types of vWD. Fortunately, a vast majority –roughly 75% – of patients have Type 1. These patients typically experience less severe symptoms. Type 2 is caused by a qualitative dysfunction of the vWf. There’s enough clotting factor but it does not function the way it should. Type 3 is the most serious, and rarest, of vWD. These patients have a a severe deficiency of clotting factors, and experience significant bleeding –including joint bleeding. Type 3 requires treatment with an intravenous clotting factor containing vWF.
Now for the scary part as far as I’m concerned! That would be the actual symptoms. According to Oygar, these 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,” Oygar says.
The more alarming ones 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, as one would imagine, can have additional signs including prolonged or excessively heavy menstrual bleeding, which are in some cases very serious, requiring blood transfusions until it is under control. Postpartum bleeding is of concern as well.
The good news? vWD is treatable, Oygar points out. How? Glad you asked, because Oygar has answers.
• Symptoms of Type I and some forms of Type 2 are often treated with an intranasal spray, Stimate, also offered in injectable form.
• Oral medications, like Aminocaproic acid and tranexamic acid, are available to help prevent the clot formed from breaking down after the bleeding stops. They are often used before and after dental procedures.
• Some other forms of Type 2 and Type 3 may call for an intravenous plasma-derived clotting factor.
For patients with any form of vWD, however, below are six useful medication management tips to help ensure that their condition is well controlled. They are important suggestions to keep on hand on your journey to feeling Better Than Before:
1) Be on the alert: If you have vWD, as with so many conditions, the first step is prevention. Similar to people with hemophilia, obviously avoid activities that can lead to cuts and internal bleeding. Less obvious is food-intake related actions. Sharp-edged foods, such as corn chips, may scratch or pierce the soft tissue inside your mouth and cause bleeding. Chewing gum can lead to biting your tongue. And if you are already bleeding, avoid hot foods and beverages, as they can cause vasodilation, causing bleeding to worsen.
2) 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 ice-cold treat aids in vasoconstriction, which is part of the first stage of coagulation. Ice packs for topical use on other areas of the body should be in the freezer as well.
3) Cover all contingencies: Especially in the case of your children who may have vWD, have a plan and discuss it with the school nurse or teachers, and other caregivers. Ensure that medication is available in the school clinic for any episodes of bleeding.
4) Use as directed: Nasal sprays, injectables and intravenous medications for vWD require a hematologist’s supervision and approval and should be used exactly as prescribed. Over-dosing or under-dosing can be dangerous and potentially fatal. 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.
5) Monitor fluid intake: Over-hydration in patients on a nasal spray or injectable form of medication can cause seriously low sodium levels which could lead to seizures.
6) 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. Be sure to report any symptoms or changes.
As for me, I’ve decided that from now on I probably should stay away from sharp knives; and just to be sure, all cooking utensils! The Lawyer will just have to fend for himself in the kitchen. (Who cares if the odds are 99 to 1 that I don’t have vWD!)