We (my husband & I) were recently told that the "acquired" version of Von Willebrand is even more rare than the version people have from birth. We'd like to find out if anyone on this site has the acquired version. My husband acquired Von Willebrand type 2a about 5 years ago. Before that he was normal. So it's been quite an adjustment. Now he is facing an angiogram on Thursday and while the hemotologist is great and we believe working well with the cardiologist it is still a very risky procedure. Anyway at this point, I'd just like to know if there are any members on this site who have the acquired version of Von Willebrand. Thanks so much for any input on this.
Hello JKR and spouse,
I have acquired VWD, type III, with "inhibitors" made by my immune system which attack VWF and destroy it. As a result, Factor VIII coagulation proteins are destroyed as the VWF wraps around the F VIII to protect it until it is needed. Consequently giving me Alphanate or Humate P doesn't work well and I need IGIV in large doses to trick my immune system into taking a vacation from making inhibitors. Then the Alphanate can work as it survives long enough to stop the bleeds. I have had numerous GI bleeds in the last few years. I was diagnosed about age 69,I am now 73.
In order to avoid a prostate biopsy which involves a dozen deep punctures with a #12 coring needle, I had to rely on blood tests(PSA), ultrasound and CT scan. I have had cardiac CT scan and ultrasound and was lucky not to have any severe calcium build-up in the cardiac arteries. A stent procedure would be a week of preparation with IGIV and Alphanate to bring my APTT down to the reference range. I currently run at 43 or more and that means I bleed extensively from any insult to my vascular system. I haven't had brain or joint bleeds yet but that is always lurking out there. I did lose about two liters of blood after a minor surgery (1 cm. cut, 0.5cm deep) due to the surgeon not following agreed protocol.
On the other hand.... I am leading an active life with lots of outdoor time kayaking, gardening and keeping up our one hectare mini-farm and forest. I do stay within fifty miles of my local hospital as they stock Alphanate for me and no other hospital for several hundred miles keeps that on hand. I have excellent specialists in GI tract and Hematology. I keep a notebook of blood tests and info about acquired VWD with me. I keep lots of bandage stuff in the kayak and our cars.
I wear heavy "farmer" boots in case I meet up with a dog in public areas and I have a wrist bracelet with VWD info on the back. I also practice keeping my anxiety levels down and I do take anxiety medicine as popping Arterio-venosal malformations, AVMs) is exacerbated by stress and that is the reason for my many GI bleeds. Everyone has a few AVMs and some people have many.
So..... my experience is enjoy life, listen to the docs you have trust in, verify what they say on NIH or Australian Health websites, As far as I know there are only a few hundred diagnosed "acquired VWD" cases and they all appear to involve stem cell (B cell) mutations in the bone marrow. My current Hgb is around 16 g/dL and I hope to keep it up there!!!!!
John the elder
I have acquired VWD. Are you the folks in the L.A. area?
John the Elder,
Do you have monoclonolgammopathy? Do they say it can lead to myeloma?
I wrote to you, but not sure it got sent. So I'll write again.
yes, we are in the LA area. Glad you are relatively close. What is your diagnosis? And when & how did you find out?
My husband was diagnosed 5 years ago with VWD after a unfortunate bout with a dental cleaning. He bled and bled, the hygienist couldn't stop it, and sent him across town to another dental doc. who was able to stop it. After that he saw the top hematologist at UCLA and got a diagnosis -- many vials of blood taken and he spent a full day there.
Munchie said:
I have acquired VWD. Are you the folks in the L.A. area?
JKR said:
I wrote to you, but not sure it got sent. So I'll write again.
yes, we are in the LA area. Glad you are relatively close. What is your diagnosis? And when & how did you find out?
My husband was diagnosed 5 years ago with VWD after a unfortunate bout with a dental cleaning. He bled and bled, the hygienist couldn't stop it, and sent him across town to another dental doc. who was able to stop it. After that he saw the top hematologist at UCLA and got a diagnosis -- many vials of blood taken and he spent a full day there.
Munchie said:I have acquired VWD. Are you the folks in the L.A. area?
JKR said:
I wrote to you, but not sure it got sent. So I'll write again.
yes, we are in the LA area. Glad you are relatively close. What is your diagnosis? And when & how did you find out?
My husband was diagnosed 5 years ago with VWD after a unfortunate bout with a dental cleaning. He bled and bled, the hygienist couldn't stop it, and sent him across town to another dental doc. who was able to stop it. After that he saw the top hematologist at UCLA and got a diagnosis -- many vials of blood taken and he spent a full day there.
Munchie said:I have acquired VWD. Are you the folks in the L.A. area?
JKR,
I've had it somewhere around 10 years, was diagnosed finally by the V.A., after a deep dental cleaning. Same thing, they couldn't stop the bleeding. I oozed for days. It was a process to accurately diagnose the type & treatment. 2 years ago I spent 5 days in the Hospital bleeding after a gum surgery,(under doctors care). They gave me Desmopressin prior to the surgery. I just got the precise diagnosis & treatment a few months ago at Loma Linda. My treatment of choice is IVIG.
My test for monoclonalgammmopathy was negative. Boy! that is a tongue twister!
I get the feeling that we "acquired VWD" people could have gotten any of the platelet/ IG protein disorders and we just happened to have a stem cell decide to make inhibitor antibodies to VWF molecules. I tell people I got hit by a bad cosmic ray in the bone marrow. That makes about as much sense as a random DNA malformation that changed a harmless protein into a VWF killer which is the way my system appears to behave. DDAVP does practically nothing for me as the inhibitors must be numerous and effective. I bless the Hematologist who heard about me in the Hospital and went out of his way to come in and suggest, strongly, the IGIV treatment in conjunction with Alphanate. My Hematologist is going to write a note for me to carry along on trips that tells ER people the best approach to my bleeding issues.
By the way, I have worked with some interesting chemicals in my day as well as intense, low frequency (10 kHz) fields. some of the chemicals were Nd, Er, V, Y and Ir. The Vanadium would be a strong suspect except I was very careful with my exposure to it and wore a lot of personal protection when dealing with it.
So I will research the monoclonalgammopathy. I have always wondered why I got VWD and not one of the other platelet disorders.
Munchie said:
John the Elder,
Do you have monoclonolgammopathy? Do they say it can lead to myeloma?
Munchie said:
JKR,
I've had it somewhere around 10 years, was diagnosed finally by the V.A., after a deep dental cleaning. Same thing, they couldn't stop the bleeding. I oozed for days. It was a process to accurately diagnose the type & treatment. 2 years ago I spent 5 days in the Hospital bleeding after a gum surgery,(under doctors care). They gave me Desmopressin prior to the surgery. I just got the precise diagnosis & treatment a few months ago at Loma Linda. My treatment of choice is IVIG.
JKR said: I'm not sure how this process of replying works. I'd like to reply to Munchie's last comment about diagnosis etc. My main question is what is IVIG? And does DDAVP work for you (Munchie)? . DDAVP definitely works for my husband.
But since a stent has been ruled out due to the requirement of plavix & aspirin, turns out the recommendation is that my
husband have open heart surgery. This of course gives us both chills. Currently his hematologist who seems to be a great doc, has said he probably needs Humate P for several days (during surgery & after). I am wondering why not DDAVP for the surgery and then Humate P. (Apparently DDAVP is not good for long term use according to this hematologist.
Munchie said:JKR,
I've had it somewhere around 10 years, was diagnosed finally by the V.A., after a deep dental cleaning. Same thing, they couldn't stop the bleeding. I oozed for days. It was a process to accurately diagnose the type & treatment. 2 years ago I spent 5 days in the Hospital bleeding after a gum surgery,(under doctors care). They gave me Desmopressin prior to the surgery. I just got the precise diagnosis & treatment a few months ago at Loma Linda. My treatment of choice is IVIG.
Dear JKR and others,
Regarding DDAVP and IVIG - what I have been told but verify---
DDAVP causes, among other things, little cellular containers of von Willebrands Factor to break open and get into the blood stream. The cells that have these little "granulocytes" are the ones that make up veins, capillaries and arteries. Normally if you cut yourself, the granulocytes get cut open when the capillaries get cut. DDAVP just forces the issue, you don't need to cut the blood vessels to increase the VWF clotting factor. The reason DDAVP doesn't work well after a few days is that the available granulocytes have all been emptied and it takes time to make new VWF. Continued use of DDAVP may also cause the body to accumulate too much water as DDAVP depresses the urge to urinate and water builds up. That condition is hyponatremia (too much water or not enough sodium) I always eat saltines or salted peanuts when I use stimate.
IVIG is intravenous (IV) infusion of immunoglobulin (IG) proteins. Since I and others have "inhibitor" proteins that attack factor VIII or VWF proteins and these are IG family of proteins produced by the immune system, the infusion of large amounts of IG proteins into the body tricks the immune system into taking a vacation from making inhibitor proteins thus allowing Humate P, Alphanate or your own VWF to survive long enough to form a clot where the bleeding is taking place.It saved my life when I was bleeding to death, seriously, from a broken arterio-venosal malformation in my small intestine beyond the easy reach of GI doctors. The bleeding stopped within hours of IVIG and my Hgb went from less than 8 g/dL to 16 g/dL-- a very nice number for a bleeder!!!!!
John the elder who loves doctor google