After three major bleeds in nine months and six in six years I am trying a new technique recommended by GI docs familiar with VWD and Hemophilia. I self-inject subcutaneously, 3X a day, Somastatin (Octreotide), a drug which has numerous effects, one of which is to lower blood pressure difference in GI tract arteries and veins thus lessening chance of AVM popping in intestine. It also slows vein growth which slows AVM production. Wish me luck!!!
Home from the Hospital again!
John the elder- 76 years old very soon
John- good luck and let us know how it goes. We will be thinking of you!
My mom has a AVMs and she bleeds internally. It took the doctors a year to find out that she has VWD - type 2 they think. Her blood count hasn’t been much above 8.5 for the last three months. I have her on blended foods and soup for the most part but how long can you really stay on blended foods, ya know. So I thought I would check in to see how you were doing and to see if you have any insight or suggestions. I hope you’re doing well! Kate
I am glad to be able to respond to your note! I have been in the hospital four times since December with GI bleeds. Some interesting info I have gotten from doctors at Shands HTC in Gainsville, FL .
- As we age, and in the absence of von Willebrands protein, blood vessels in the GI tract keep growing and growing as VWF is a down regulator for vein growth- hence more opportunities for sites to break as new AVMs or dysplasias form. The endoscopy people won’t even attempt to find a bleeder as the process may cause new damage and bleeds.
- To control new vein growth I was given “octreotide”, a hormone, 3X/day subQ injection. That caused an edema behind the retina in one of my eyes and a little “gynecomastia”. So that was stopped.
- I was then given “thalidomide” tablets to achieve the same effect. It is a little risky and requires a monthly query to keep me reminded about the horror of thalidomide babies. Thalidomide also attacks B cells and reduces the inhibitors I have which destroy the VWF in factor if I do bleed.
- I now have a port and and a small refrigerator full of Wilate factor and a box full of IV catheters, flushes, sterile gloves and all to self-inject. I still need a large dose of IVIG to knock down the inhibitors but I can only get that in a hospital setting, Medicare rules.
- It has been six weeks since my last bleed and I am being very careful about any lifting of 30 pounds or more using abdominal muscles. I also avoid crowds and sick people as the thalidomide drops the white blood cell count.
- I am not sure whether the type 2 VWD also promotes vein growth (angiogenesis) but it is a good question for your hemoncs to pursue. Your Mom is probably beyond childbirth years so thalidomide isn’t a problem. Octreotide is possible but both drugs have side-effects.
So that is all the news from Lake Wobegone. My flower garden is beautiful by the way. Thanks again for the note, it has been a long winter.
John the elder
Hi John, thank you so much for responding, and I’m glad to hear you’re better. My mom is 67 years old - way beyond childbearing years. I’m sorry to hear you’ve been in the hospital four times.
I will bring up everything you’ve suggested on Tuesday when we visit my mom’s specialist. I think they actually want to scope my mom - her current blood count is 7.3. I’ve heard from many that you can’t find a bleed if your blood count is below 10, but the doctors do not agree with me. I don’t know what to do. The specialist wants my mom to try stimate on Tuesday - have you tried this? Do you go off food when you bleed or does food have nothing to do with it? Also, how do you get your blood count back up? Iron infusion? Sorry for all of the questions but I can’t thank you enough. Kate
Things I leaned fromGI and Hemo, people in the past few years:
- about 250 mL of whole blood is a “unit” of blood- a really black tarry stool is about 100 mL of blood. My bleeds have ranged from a unit a day to 0.2 units/day. The body can make some Hgb each day if iron is available.
- I get regular “iron panels” and iron infusions when the iron panels show low levels.
- PTT (APTT) test gives an indication of level of VWF effectiveness.
- Stimate causes an temporary increase in VWF in circulation but the VWD type two VWF is defective in function due to its size or shape. Type one and three have normal VWF in low or very low (zero) amounts.
- A Stimate challenge test measures PTT just before Stimate infusion and PTT after a set time. If Stimate will work, then PTT will drop into reference range- if not (my case), PTT will remain above reference range.
- A “mixing test” mixes patient blood with “standard blood” and the results will show whether the body is using antibodies against VWF- (My case- antibodies destroy VWF rapidly- factor gets destroyed almost immediately) I take immuno-suppressive drugs to keep antibodies in check.
- Red blood in stool is Colon bleed, black stool is small bowel bleed, vomiting “Coffee grounds” is stomach bleed,spitting up red blood is esophageal bleed. Ypu can buy Hemocult test kits on Amazon and test dark stool at home to see if it has Hgb in it.
- There are other conditions that can cause low Hgb besides bleeding and/or lack of iron.Some conditions clear Hgb too quickly and some conditions cause low production of Hgb precursors. A bone marrow biopsy and a look at the size distribution of red blood cells give hints.
John the elder
Thank you so much, John. I really appreciate this. I am so grateful for your knowledge. My mom had a bone marrow biopsy in January and it came back normal… But she has high LDH (lactic acid) now and she has MGUS… internal bleeding probably from the small bowl…And God knows what else. Does Vonvendi work for you? Also does the doctor scope you while you’re actively bleeding? They want to scope my mom, and The specialist mentioned that she would like to try Vonvendi. Really, can’t thank you enough.
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