A 47 year-old female presents for revision of total hip arthroplasty. Prior medical history is notable for von Willebrand’s Disease (vWD). When asked about her disease, she is unsure which type of von Willebrand’s Disease she has been diagnosed with. She does note that extremely heavy menses led to a hysterectomy at the age of 38, and that she bled for hours following a dental extraction 15 years ago. She also says she “nearly died from blood loss” during her first hip replacement after “that D-P medicine medicine which was supposed to help didn’t work”.
Questions for Discussion
1. What are the different types of von Willebrand’s Disease?
2. Would you offer this patient a neuraxial anesthetic? A para-neuraxial nerve block?
3. How would you treat her vWD perioperatively?
Related posts:
Hi I am a VWD patient. I had the same problem with DDAVP and had to use Humate P. I had a dental extraction in June performed in the hospital. I am about the same age as the woman and have had the same type of bleeding problems. When I was originally diagnosed there was only one VWD. In every day life I seem to be normal. I only have needed clotting assistance when I have had surgery.
First, we have to consult hematologist. Most likely it is type 3 and she needs replacement therapy with Humate P or other analogus concentrates( Crio or factor 7 if you deal with bad bleeding in the middle of the night and there is no much time and experts around…)But for elective case especially with this type of history, expert consult is the best. In my opinion: no RA for this patient, prior hematologist consult and some pre treatment.
Just want to add that it is getting more and more complicated as we learn more and more about VWD. This is the most common bleeding disorder up to 1% of population is afflicted but only 1% of those clinically gets in trouble. There are three types and type 2 has bunch of subtypes.There is even acquired form of VWD. There is no good test that correlate with the function.Some people have very mild disease( apparent only after significant challenge: surgery, asa..)Some have very bad disease…
What I think everyone needs to know is: 1)that VWF is important for a) primary hemostasis( platelets sticking to the endothelium and each other) and b) for fibrin formation( it carries factor 8 and without VWF factor 8 get easily destroyed- VWD used to be called “pseudohemophylia”).2)DDAVP 0.3 mcg/kg “squizes” endogenous VWF out of the vessels so it is helpfull in many situations, but not always… 3) if you get in trouble(big emergent case) and DDAVP did not help you have to provide VWF and factor 8 and on rare occasion even platelets. 4) VWF in reasonable concentration could be found in HumateP,Factor8 concentrate and other commerciall available concentrates-call pharmacy…5) one can also use Cryo( the last resort due to infection risk)…6) one can use “Novoseven” Factor 7 A – that bypass the need in factor 8 so it is a reasonable option, provided that platelets are OK. So in bad situation it may come down to “load the truck” eventually without going to much into the hemathology details.
When case is ellective- call for help(hemathologist). There is no way we can know all those details that are known now by experts…
Continious Femoral block(US guided) is the safest from all other blocks that we can offer for this case( maybe US guided fscia illiaca block, but I do nt have experience with this one) The rest of the blocks that we usually use for THA such as parasacral, lumbar paravertebral are risky for bleeding.( same with neuraxial techniques)
So the continuous femoral would be my best bet is anything at all.
The key point here is to find out the type and severity of disease which would determine whether replacement is needed. There was an article (metanalysis) recently in A and A which looked at neuraxial techniques in patients with bleeding disorders. 74 neuraxial procedures were performed in patients with vwf deficiency- 64 had normalization of factor levels spontaneously prior to needle placement. Key point here- 71 of them were of type 1 variety- the mildest form.
http://www.anesthesia-analgesia.org/cgi/content/abstract/109/2/648
There is a nice article on up to date on the treatment of von Willebrand disease. I think Barys has pretty much summarized von Willebrand disease, with its complicated classification system, very nicely. Most patients will have the mild type I VWD, which usually responds to ddavp, so since ddAVP did not work for our patient in this scenario, it seems like our patient has either a qualitative defect (type 2, one of the 4 subtypes) or severe quantitative defect (type 3). Requiring a hysterectomy for menstrual bleeding sounds quite severe.
I have to agree with Barys about the hematology consult. This is an elective surgery and we have the ability to further define hemostatic treatment for a REVISION total hip arthroplasty, and so we should consult the specialists. I think this patient is high risk for bleeding and would hesitate to perform lumbar paravertebral/parasacral for this patient unless I know the actual subtype and hematology recommendation.
And since our arthroplasties are performed at AGH, in an institution in which I am not too sure that Humate P or any other factor VIII concentrates or recombinant FVIIa is available, we should be extra prepared (contacting pharmacy for availability of products). As a last resort, of course cryoprecipitate and platelets could be given if none of the factor concentrates are available and the patient presents with excessive bleeding, but there will be the infectious risk of administering plasma products.
I think knowing the subtype will most optimally allow us to figure out what is the best product to give for this patient and we must confirm availability. It is, after all, an elective surgery and we should make sure that things are optimized prior to proceeding.