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Von Willebrand Disease (VWD) - Online Support Group

Frustration

VWD has to be the most frustrating disease there is!!!!!!!!!!!

Here I am thinking that going to back for my results would be the answer that I am looking for but no. It's not. All he can tell me is that I have low VWF which presents as having VWD but because your levels change daily he is unable to 100% diagnose it as being VWD but he is certain that is what I have but if I don't it is clear that I do in fact have a bleeding disorder.

My iron count is currently at 60 but when my GP tested it it was at 3. I know my levels can not fluctuate that much so im curious as to what he tested that she didn't? Im back to see my GP next week so hopefully she can give me some better answers.

He has allowed me to take aspirin for my heart problems but says if you start bleeding from anywhere or longer than normal stop taking it immediately and go to a hospital.

So basically now I have more questions and even less answers than I want.

I guess living in Perth Western Australia we do not have the specialists here that are required for specific bleeding disorders.

Dear PerthMom,

Just a little frustrating???? Not really confident in the GP advice? See if the GP can't get in contact with a Hematologist at a major hospital to answer your questions. All the vWD blood tests should be available in Perth. If not, the samples can be shipped frozen in dry ice to a major lab. There are at least four iron tests, ferritin level, TIBC , serum iron and iron saturation. The range of values can normally vary over as much as a two to one or three to one ratio so you can expect the values to change from test to test. I have difficulty absorbing iron from food so I get iron infusions every few months. If I am bleeding out they give me iron so I can make new Hemoglobin to build up the Hgb count in the CBC. The RDW and MCV numbers will change if you have lost some blood and are in the process of making new blood. Since bleeding can occur in the GI tract you should be aware of the color of stool. Dark to black stool indicates, usually, an upper GI bleed. Visible red blood is likely to be in the colon or rectal area, in general. I actually purchased Hemoccult test strips online and keep them in the Bathroom. It's an easy test to perform and interpret.

I would definitely read the aspirin side-effects literature very carefully. If you really have significant vWD then you are unlikely to suffer from blood clots. On the other hand if you have clogged arteries then you are in a hard place so question the Doctor carefully about the risk analysis factors. What would he or she do in your situation??? You have a very legitimate concern. Also note that anti-depressants like SSRI and SNRI typically have anti-coagulant side-effects. Buproprion (Wellbutryn) acts differently and does not have a tendency to increase bleeding incidents as far as I have been able to find. There are also herbal supplements which increase bleeding incidents.

If you have vWD test for vonWillebrand Factor which is low, then Factor VIII will also probably be low as the VWF molecules wrap around the Factor VIII molecules and protect them from other blood chemicals. To tell what type of vWD you have you need more tests, such as a "blood smear" or "multimere" test and Ristocetin Cofactor. An APTT and PT test check the coagulation time for your blood. APTT relates to Factor VIII levels and PT relates to Factor IX. My APTT typically runs from the high thirties to fifty or more. the top of the normal (reference range) is about 30. At 38 I can bleed out. My PT is normal. Hemophilia A is lack of Factor VIII and Hemophilia B is related to Factor IX. My VWF numbers are so low that I should be treated as a Hemophilia A patient. My multimere test shows that I don't have large VWF multimeres needed to protect the Factor VIII. My blood has anti-bodies which apparently eat the large multimeres for lunch as fast as my bone marrow produces them. That is why Stimate doesn't do a lot for me.

For many people, Stimate nasal spray is a real life-saver. It only takes about an hour to cause the release of VWF from cells that store the VWF. If you do check into the hospital for a bleeding incident, they are likely to give you an IV infusion of "desmopressin" which is the secret ingredient of Stimate. There is a simple test procedure to check whether or not Stimate will work in your case but it won't probably be done unless you are in the hospital. They take a blood sample to test for VWF, Rist.Cof. and FVIII, give you the IV desmopressin and two hours later take another sample for the VW tests. In my case I went from less than 5% to less than 20%. Enough to stop a mouth bleed or nosebleed, maybe, but not enough to stop a GI bleed or serious bleeding incident. You will probably have better results. I hope!

I wear a medical alert bracelet that says BLEEDING DISORDER and info about VWD. I also have a good collection of bandage making material I take with me in the car and the kayak. I usually carry the Stimate with me also.

You may run across reference to AMICAR or aminocaproic acid as a bleeding stopper. This material works for mouth bleeds where the saliva tends to break down the "fibrin" that makes up a blood clot. It is not applicable to all bleeds, like GI bleeds but is often used prior to dental work.

So if you read this far, you know I have had a few bleeding episodes and a lot of testing done. I have found that having written questions for the doctor, one copy for you and one copy for the doc really help to focus the discussion on what you need to know for your peace of mind. I always try to have my wife or daughter with me for important consultations, like now I am trying to avoid a prostate biopsy procedure that might be more dangerous than a slow-growing prostate cancer. So good luck and keep posting your questions and concerns!!! You are not alone.

Hoping this helps you formulate your questions for the doc,

John the elder

:frowning: Sorry to hear what you are dealing with at this time. Unfortunately, from my experience anyways, even when all the doctors know about the VWD diagnosis there is still a lot of confusion and miscommunication between them. Just this morning I had to go for an endoscopy which my Gastrologist normally does in her office clinic but last minute she moved it to the hospital out patient surgery centre because of the DDAVP infusion. I specifically questioned her that my VWD would probably needed to be treated at a hospital and not a clinic but she had said it was no problem. Well, apparently it turned out to be a problem, as I had expected my hematologist would say it would. So, I can’t imagine your level of frustration.

Question: I’m a little confused as to what you are calling " iron count." Your stored iron called serum ferritin is the iron not found in hemoglobin. Ferritin levels drop when stored iron decreases. For a female it should range from 12 ng/dL to 150 ng/dL. Hemoglobin levels are measured because that is where most of your iron is stored. Your hemoglobin level for a female should range from 11.5 g/dL to 15 g/ dL. Hematocrit levels are measured because not everything in your blood draw is red blood cells. The level is a measure of the percentage of red blood cells in your blood. Your hematocrit for a female should be 35 to 45%. Iron saturation and binding capacity are also usually measured. So when one doctor was saying your level was 60 and the other was saying 3 where they talking about the same thing? Also, if your level was truly a 3 why were you allowed to leave the doctor’s office and not immediately given a transfusion or iron infusion? I walked in with my hemoglobin level at 6 g/dL and my doctor couldn’t believe I was able to walk and told me if I waited any longer I would have been in big trouble. He immediately sent me for an iron infusion. It took several more over a period of months to get it above the normal 12 g/ dL range. My doctor is a hematologist/oncologist. He told me anytime I get it measured below 10 g/dL I need to come in. Are your numbers expressed differently in AUS than here in the USA? I was just curious because, as you said, your numbers don’t seem to make sense. Also, something else to ponder, I come from a lab background and trust me not all labs are created equal so there could have been a lab reporting error. I hope you get some clarification with your diagnoses. All the best!

We are frustrated too!

After trying to get a new referral to a different paediatric haematologist yesterday at our GP, we were told we would have to wait a year! There are only two paediatric haematologists in Perth and the other was no help at all. By the time we wait that long she will be almost 16 and can see a normal haematologist anyway!

Yesterday our GP (who knows my daughter has VWD) tried to tell me to give her Ibuprofen to help with her flu symptoms. Really?!!!!!

It is a sad state of affairs when the patient knows more about their illness than the doctor they are seeking help from. Our GP said he didn't think it was that important to find out my daughter's subtype, because they all get treated the same anyway. REALLY?!!!!!!

We don't want to give up seeking answers about her VWD... but it certainly is frustrating!

Hang in there! X

Serrum Iron for Women should be 50 to 170 μg/dL so my understanding is that this level for me is at 60

Ferritin for Women should be 18-160 ng/mL and I believe this is where the level of 3 is.

So if in fact the ferritin level is the level the GP said is at 3 then the lowest it has ever been before is 2. My GP wanted me in for an iron infusion however the Haematologist said there was no point as my levels appear normal. Going off what he said I would assume he would be more correct than my GP as he also ran a lot of other tests.

I can't get in to see the GP until next Tuesday as she is on holidays until then but that is fine because she wont get the report from the Haematologist until then anyway.

I know most people on this forum are in the USA. Perth is tiny compared to anywhere you have. Specialists are rare and extremely difficult to get in to when you need to. It took me 3mths just to get back in to see the Haematologist. Finding someone who can specialise in bleeding disorders in Perth and actually make you feel better is like finding a needle in the ocean! For us we go to the GP to get a referral for a specialist, then once we get in to see the specialist we go back to the GP for results. Basically the GP is used as a middle man and for me a complete lack of communication happens here and the GP isn't always great at interpreting the results from the specialist.

Di, have you called Dr Ward at Perth Haematology? He seems to look good and has a bit of info on the website which appears that he knows what he may be doing. http://www.perthhaematology.com.au/

I am so sorry to hear this and I feel the same frustration. All I can say is to keep on it and demand some clear answers. My 3 year old son got an abnormal blood count back and came back with vwd type 2 and left it at that. I am furious as to the lack of information. Maybe come up with a list of all the questions you can remember, make an appointment and ask away as much as you can.? I know the situation is frustrating but keep fighting for answers.