I have been scoped, including enteroscopy (extended endoscopy) while bleeding and a few times an active or suspicious spot was cauterized. I also have had two pill endoscopies, the last one saw a bleeder near the duodenum but the rest of the GI tract was black with digested blood. Because my GI tract is a garden of AVMs there is a risk of collateral damage when scoping so that procedure is now not to be used. I found out the VWF is a natural down regulator of vein growth and since my inhibitors (antibodies) destroy my VWF I have prolific vein growth in the GI tract leading to the AVMs and other angiodysplasias causing the frequent bleeds. I have “spider” veins all over the GI system. To stop the vein multiplication I take the thalidomide which also is a down regulator of vein growth as well as an immune system down regulator- hence I wear a mask in stores and Doctor’s offices and avoid people who are sick. My white blood cell count is somewhat low as a result. Hopefully the reduced immune system activity will reduce the production of VWF antibodies and my natural level of VWF will increase from much less than 10% to something useful.
Vonvendi , itself, won’t help me without also getting a FVIII product as no VWF means no FVIII. The body naturally destroys any FVIII that is not protected by a “multimere” of VWF molecules. I have Wilate in a refrigerator and IV supplies to self-administer Wilate. To lower the level of VWF antibodies when bleeding I need a large dose of IVIG concurrent with Wilate but I have to get that at a hospital due to Medicare rules.
I also have an MGUS with an M spike that is present but not at myeloma levels. It would be interesting to know if your Mom has antibodies to VWF or FVIII. It is not impossible that she has symptoms of both type two and one of the other types at the same time. The other type being due to antibodies developing from Vonvendi or other previous treatments. My antibody production has increased since I developed the VWD seven years ago and have had PRBC blood transfusions as well as Alphanate, P-humate and Wilate treatments. Development of antibodies from treatments is a problem in the bleeding community.
I have taken advantage of using a Hemophilia Treatment Center in monitoring my condition and they work in conjunction with my local Hemonc.
Good luck and keep in contact.
John the elder