I have VWD Type 2A and recently spent 5 days hospitalized with a GI Bleed. It took 8 doses of Humate P to get control of the bleeding. I carry 2 doses with me when travelling, but am thinking that isn’t enough. We spend time in 3rd world countries…ie Africa, Honduras, Equador where the drug does not exist. My internist suggests carrying 6 doses…enough to stabilize and support me enough for transport home in case of emergency. For those of you who travel extensively, how many doses do you carry? My insurance covers the cost, so thankfully, that hasn’t been an issue.
Hi, my 5yr old son was diagnosed with VWD2A, I actually don’t have an answer for your question, rather I have a question for you. How did you get a GI bleed? Is this common for type 2A, and do you not respond to DDAVP and that’s why you take the humate?
The gene comes from my side and I was adopted but it is known that the I could have had it but I do not but I passed it to my son, so I don’t know anything about any family members that have it. I appreciate the information.
I think I’m unusual in that I wasn’t diagnosed until I was 62!! Lots of incidents of bleeding throughout my life, but back in those days it was so uncommon to be worked up for a clotting disorder. I never had children or any major surgery to create complications.
I went through 2 DDAVP trials; both failed. Sadly, I just don’t respond. Did your son go through the challenge to see if DDAVP spray works? I do respond to Amicar which I take before dental cleaning; apparently we have a chemical in our saliva that impairs clotting. The Amicar helps inhibit that chemical.
So, the only drug I really respond to is the Humate P. I’m not sure why I got the GI bleed; it’s more common to have spontaneous bleeds with Type 3. Maybe because I’m older, and my systems are more fragile?
I guess my only suggestion is to be sure your son has gone through the DDAVP challenge to be sure the drug works. It often doesn’t with Type 2A.
I went through my local hematologist, but got so few answers. I took myself to a Hemophilia Research Center for re-evaluation…learned a lot about living a normal life with this odd disease.
Long winded…I hope it helped. Best of luck to you guys. Don’t hesitate if you think I can help in any way.
Thanks so much, he has had the DDAVP challenge he responds to it, we also take the amicar for mucosal bleeds. They said when he’s 7 they will give him the nasel spray challenge. He sees the head hematologist at Cohens children’s hospital she only does hemophilia, we are very lucky to live where it is easy to access excellent medical care. His biggest issue has been healing after an injury, he is 5 so he regularly runs into walls lol. Thanks again for the info!
My mom has the exact same problem with the GI bleeds. I’m bombarding Johntheelder with questions on a different thread. My mom just found out that she has type 2a and she’s 67.
The specialist wants her to attempt the Stimate challenge on Tuesday. May I ask what specialist or hospital you went to? We live in Los Angeles and there is one specialist here - My mom is throwing her for a loop.
Do You have any pointers regarding the GI bleeds? I’ve taken my mom off of whole foods and we blend everything (mainly bone broth a and vegetables) but that seems to be failing at the moment. My mom did OK on Amicar but the specialist asked her to take tranexamic acid instead… and I don’t know what to do.
My apologies for not answering sooner; we were out of town. I feel so bad for your mom. But take a deep breath…this is manageable. I was diagnosed at 62; had my teeth cleaned and hemorrhaged into my mouth. I was admitted after passing out…and the rest is history. In retrospect, I think I had the disease my whole life, but it was mild. Long menstrual cycles, nosebleeds. Nothing too serious. I think menopause created a lot on a lot of changes for me, including worsening of the VWD.
My hematologist is at a local hospital…I remember being in panic at the diagnosis. We travel the world, had a home in a very remote location without hospitals…how could this be manageable. My hematologist told me I was being “emotional”. That set me on a quest…to get some real answers. I made an appt at the Hemophilia Center in Philadelphia for eval. They were a wealth of information. One book they gave me was “100 Questions and Answers about Von Willebrand Disease” by Andra H. James, MD. They also did retesting…I am 2A like your mom, and like most of us of this type, I don’t respond well to Stimate. Some people do, but not by any worthwhile extent. I take Amicar before simple dental cleanings. I think Tranexamic Acid is similar? but I’ve never taken it. The only drug most Type 2As respond to is Humate P, which is the human factor. It’s administered only by intravenous push. I carry 4 doses when I travel; both my husband and I have been instructed in mixing the drug, starting an IV on me, and administering the drug. It sounds overwhelming, I know, but it isn’t. It gives me the freedom to decide where and when to travel. Humate P is expensive; however under Medicare and my Plan F policy, I have no copay.
Re the GI bleeds, I had one very major one that hospitalized me for a week. It was “occult” meaning they never found the source. So I watch my stool for darkening. That’s all I can do. I have blood work (CBC and Iron levels) once every 6 months. I haven’t changed my diet much; I don’t think it makes much of a difference unless I’m actively bleeding. I’m a vegetarian, eat tons of salads and beans. After my bleed, I avoided roughage for about a week or so.
Going into a major hemophilia center for evaluation and teaching was probably the best thing I did and my biggest recommendation. They taught me how to live with the disease, and manage it. We travel the world…spent a month in Africa last year, and sailed alone in the Greek Isles not long ago. But the initial shock is tough.
Please drop a line any time if you think I can help. My best to both you and your mom.
PS…I did go back to my original hematologist who told me I was “emotional”. I gave him a copy of the book. Also had a discussion of his knowledge of VWD and how everyone is “emotional” with a new diagnosis. He’s learned a lot, handled my GI bleed beautifully. I go back into Philadelphia only as needed.
My rule of thumb is to always travel with a month a medication above and beyond what I need while on the actual trip, particularly for international travel when you don’t know if you’ll have access to what you need. Heck, once I was stranded in NYC for a week without my checked luggage! Luckily I had two weeks worth of meds in my carry on.
I don’t have VWD myself, I other chronic issues, but I definitely like the suggestion of going to a hemophilia center for education. They may answer questions you didn’t know to ask.