Hi, any input would be appreciated. My daughter was diagnosed with VWD type 1 after her delivery of twins using in vitro. She had two incidents, the first with internal bleeding after her eggs were harvested and then bleeding after the C-section delivery, and her OB recommended she have testing to see if she had a blood clotting disorder. So six months after delivery, she had the blood testing and was diagnosed with type 1 VWD.
After meeting with the hematologist, she said she would not undergo another in vitro procedure since the hematologist was clearly not in favor of her becoming pregnant again. However, she just called me today to say that it looks like she is pregnant again. She is very torn about what to do - she and her husband went through a lot before she was able to get pregnant from the in vitro fertilization and, as her husband says, it’s a miracle pregnancy because they were told that the test results indicated it would have been impossible to get pregnant naturally.
So, my concern is what are the risks of proceeding with this pregnancy given that she has VWD. She is finally slated for a trial of medication (I assume it’s the nasal spray) next week at the hematologist’s office and she said there were all kinds of problems arranging for this one-time trial - plus this trial is going to cost $100 out-of-pocket for this one time. As I understand it, you need to have the trial to see if it works and if it doesn’t, what other medication would be feasible. Also, she will be seeing her OB tomorrow and will ask for his opinion but since she had a C-section delivery with the twins, if she has another C-section, I am worried about bleeding again.
Any input you have is much appreciated. I did read the post of October 2013 which was very helpful and it did sound like this is do-able, at least medically.
As I understand type I VWD, there is a wide variation in severity and there is a wide variety of response to treatment. One of the important tests needed to make an informed decision is a “desmopressin effectiveness test”. I am a type III with “inhibitors” meaning that I have the characteristics of a type A hemophiliac.
I am a poor responder to “Stimate” nasal spray. It only increases my VWF a little bit. The little bit of response I get is decreased immediately by inhibitors I make that destroy the VWF. If my “Stimate” response was better, I would have an easy response to bleeding episodes. Many type I patients do quite well with Stimate (desmopressin) nasal spray. I don’t.
Your hematologist needs to know how the patient responds in order to give reasonable advice. I would personally try to avoid surgery with my situation as I am in a precarious position. Another person with a good response to treatment would easily undergo surgery. Knowing in advance what your response to treatment is likely to be your best friend. Get the tests done!
John the elder
I have a question about colonoscopy. I have acquired VWD type 3 moderately severe and it is time for a colonoscopy. I am petrified because I have had bleeding issues before and I am wondering if you have knowledge about this. I have checked with my doctor and it would be a diagnostic colonoscopy with no cutting if a polyp is found. I would have to go back another time and get my pre-treatment first. Just wondering if there could be any internal bleeding or complications. Any advice would be greatly appreciated. If any other VWD patients has any infomation for me I would so appeciate it. Thank you so much!!
You are right to be concerned. The GI people should be fully informed and you should stress the Type III/ Hemophilia A condition you have. My hospital keeps Alphanate factor in the pharmacy just for me. It has a long shelf life and the pharmacy only pays for it when I use it. It could be pre-ordered instead of depending on some outside source.
I have had multiple colonoscopies to stop arterio-venosal malformations that were causing GI bleeds. They never slipped with the equipment and cut my intestine. There is a cautery device on the colonoscopy tool that can stop a bleed, if one should start. Check with your doc to see that the cautery equipment is ready to go if needed.
The only complication with endoscopy that I have had was when I had an upper GI endoscopy and had hiccups. Since then I take a heavy tranquilizer (Thorazine) before cat scans and endoscopies. Nothing like psychosomatic attacks just because you are afraid of being injured!!!
Prior preparation prevents XXXX-poor performance when dealing with the medical profession- sad experience teaches me.
Assuming you can prep your caretakers, stride forth boldly. You did all you could. The chances of an incident are small. If the staff is prepared and takes your warning seriously all will be well. Remember to drop the Hemophilia A bomb on them. Almost all staff remember that. Almost none know of von Willebrands disease and even fewer know that type III is about the same as Hemophilia A in effects on the body, although the treatment is a little different.
Best of luck, Lyn and thanks for asking. I hope you are a little reassured!!!
By the way, Lyn, have you been tested for inhibitors? My acquired vWD apparently results from my immune system making antibodies that attack my vonWillebrands factor and/or factor VIII. In order to stop my immune system from killing me, the last time I had a GI bleed they gave me factor and IGIV (immunoglobulins by IV). The IGIV convinced my immune system to take a holiday long enough for the factor (Alphanate) to seal the leak. That is why I am able to write this!!!
Talk to your hematologist about that is my advice.
John the elder-- still having fun although I retired the gas chainsaws after the diagnosis!
Thank you so much John for the information. My last surgery we thought we had it under control with humate/alphanate but it did not work and I had major complications, my dr. is now saying if I have to have surgery He would try the IGIV first. Did your insurance company cover the IGIV I hear it is extremely costly.
Yes it did. Of course I am old and grey so I had Medicare and I have bluecross/blueshield. I hope it works out. The alphanate didn’t work for me until I had the IGIV. I could send you an excell spread sheet of my Hgb over a week while I was bleeding to death. Two units of apherized blood didn’t last. I had them use the apherized blood as packed-red-blood cells are full of Heparin which doesn’t help us out very much!!! The apherized blood has much less anti-coagulants in it. The IGIV is what saved me. The darn immune system really worked well but it had the wrong blueprints for making antibodies and the inhibitors were killing all the VWF mine, the blood transfusions and the alphanate. Let me know how it turns out, lyn.
John the elder
Thinking maybe I should have a virtual colonoscopy any thoughts on that? I am waiting to hear back from 2 doctors on what type of colonoscopy I should have. Crazy stress in my life due to VW sometimes it just gets so depressing what could happen to us!!!
You know, lyn, I have had this sword of Damocles hanging by a horsehair over my head for about six years. The constant presence of a major bleed possibility started to affect me subconsciously. Then my eldest son died unexpectedly, even to him. That was the the proximate cause of my first bad GI bleed. The bleed started in an AVM (arterial venosal malformation) and was fixed by an endoscopy that found the bleeder at the bottom of the stomach and cauterized it. Then there was another bleed. Then there was a bleed that was out of reach. It was either “double balloon” endoscopy of the small intestine which is really hard on the tissue, or hope it would stop. That’s when we tried the IGIV. It worked, immediately.
Since then I seized back my life with both hands. Knowing that there was a big history of depression in my family (and my wife’s), I started on Wellbutryn as it does not have the bleeding side effects of the other SSRI and SNRI drugs. I also take a really low dose of Xanax AM and PM. Because “inflammation” is an immune system problem and I suffer from a number of allergens- egg, mustard, dill, ragweed, mold spores etc. etc. I take a Loratidine (OTC) in the AM and a Levoceterizine in the PM (because it is a drowsy anti-histamine). As a proton blocker I take an OTC famotidine pill in the AM so my uper GI tract is calm. Some of this may be working on the “placebo” effect but I am not going to experiment. I really work on my mental and physical health. I put fun things first. I have non-blood related daydreams on the shelf to use when bad thoughts come up. That really works!
I started to learn the banjo. I have a large flower garden. I am active in my amateur radio hobby. We have four cats and a goose as well as a two person kayak and two tricycle recumbent bikes we keep two blocks from a bike trail.
Since then I have only had a nose bleed and several mouth bleeds. I get my teeth cleaned four times a year without deep scraping- that was learned early on after I looked like dracula after a big meal. I brush and floss after every meal. I keep stimate and Amicar in the fridge and take both to the dentist even though stimate is only partially effective. Each car, kayak and bike have large pressure bandage kits and “wound-care” type coagulators. I have good work gloves near all my tools and I really watch the safety procedures- no silly stuff.
Soooooooo… life is a gift, I have been close to the other side and choose to avoid that path. I sleep well and I have a very long bucket list…
Your GI people could choose the Barium X-ray colonoscopy substitute but it isn’t quite as definitive. My experience with colonoscopy people is that the procedure is not likely to cause a problem and I have had three or four with at least three different practitioners since I was diagnosed with acquired vWD. I would listen to the options vs-a-vi the outcomes. If they do see polyps you still have to have the procedure- I lost two sisters to colon or related cancers who didn’t have regular colonoscopies.
I hope you see my notes as positive. I shouldn’t be allowed near keyboards as I get carried away!
Love to all,
John the elder
Thank you John for all the information it is much appreciated!!
Take care of yourself!!
Hi, John -
Thanks for your response. Just wanted you to know that my daughter went in to see her OB and he said it would be best to terminate the pregnancy because of her VWD condition. However, when he did an ultrasound, he found no heartbeat so I guess this was just not meant to be. She did go in for the Stimate test and she said she had to go back as the test results were perplexing (not sure what happened but she’s not been feeling well so I didn’t press her on what exactly). But he did clear her for the D&C and it went well with no bleeding. So, we are all thankful that she didn’t have any problems.
Believe I have a “mild” form of VWD - never officially diagnosed since I had my symptoms in 1940’s thru 1960’s.
Had 8 miscarriages when my OB prescribed mega vitamin C.
My bruising and other symptoms went away in a week and I carried next child to term.
Here is a URL of a government study that did find links of Vit C to VWD. I still take 2 grams of C per day.
The bruises I mentioned would appear on my thighs after a good nights sleep and I would (laughingly) ask my husband if he had been kicking me. Also would have mosquito bites swell up to 4 inches and would take a tiny scratch from twigs (when hiking) an hour to stop bleeding.
Well…am going to have the blood tests for VWD - because I will be needing some kind of heart surgery in the not too distant future. The vitamin C 2 grams served me well with hip replacement (doctor OK’ed that - buy not sure if he believed me - or was humoring me). Heart is a different thing altogether - so will soon have evidence for … or against.