First draft for journal article on VWD
Help! Doctor, my fourteen old daughter and I are totally depressed and our lives destroyed by von Willebrand’s disease. We have been to Hematologists, Emergency Room physicians and clinics but nothing seems to work. Nobody seems to have experience with VWD and we get conflicting information and treatment plans that don’t work. Help us! There must be a way to comprehensively solve all the problems we are having with treatment protocols, school issues, diet, diagnosis and complications.
It started before she started having periods. She had nosebleeds and dental issues usually involved lots of bleeding but nobody thought to test for VWD. When her periods began our lives went off the cliff. She bleeds for weeks at a time. Most periods last for close to three weeks and involve massive amounts of blood which are beyond control with pads. She can bleed through her clothes despite changing the pads regularly. In school, her most personal body issues become a public issue because the teachers do not handle the bathroom pass issue professionally. She has missed so much school that the authorities are harassing us. She is constantly tired and seems anemic despite our diet of iron-rich foods. Her treatment doesn’t schedule “Iron panels” on a regular basis so we never know when her levels are low. She has had reactions to some Iron IV preparations but alternative Iron IV products haven’t been tried. Iron pills don’t seem to work and her stool turns black and gives a positive Hemocult test which causes even more stress.
She has had a number of PRBC infusions but we found out that PRBC is full of Heparin and she loses the blood as fast as it comes in. Apherised blood has less Heparin preservative but the Professionals don’t think along those lines. What do you think? She also may have developed immune system reactions to the blood transfusions as her APTT results are still way out of normal reference range and her bleeding seems to be getting worse with time. We heard there was a “Mixing Test” to check for VWF inhibitors but nobody has called for it. Concurrently, Desmopressin doesn’t work as well as it used to work and a Desmopressin Response test showed that APTT didn’t drop very much two hours after IV Desmopressin. A friend with type III VWD has inhibitors and had to be given IVIG to suppress the immune system during a GI bleed. When is IVIG called for???
During some periods, she suffers immense pain, but, of course, she can’t use NSAIDS because of the side effects on clotting. One Hematologist said large clots were backed up in the cervix but what can be done for the pain issue without worsening the bleeding issue? Likewise, most anti-depressants in the SNRI, SSRI families increase bleeding so they are out of the picture to treat the depression. Wellbutryn type anti-depressants seem to have less bleeding issues. What are the choices for clinical depression treatment with this type of coagulation issue?
Birth control pills have been tried but we have had to try several different types and the bleeding issues still continue. What can be pursue on this approach?
One Doctor told us she has two types of VWD, Type I and 2N. Another Doctor said she only had one type as a Blood Smear test came back normal. We found out that most labs have difficulties with this test as the blood sample can degrade before the test is done. How can we get a second opinion?? Apparently Heterozygous VWD is not uncommon. Having two different types requires two different treatment plans doesn’t it?
Can you help us deal with the School Issues. Is there someone who can help us navigate the Educational Complex in such a way as to get results? Having to tell the teacher you need to leave the classroom, again, because your pad is soaked through, is not a workable solution for a fourteen year old. Life is hard enough for a teen-ager. Having to publically acknowledge menstrual issues in front of male teachers and classmates is unacceptable. Help Us!!!
This article was composed by a “Moderator” for “Living With VWD”, a web community for VWD patients. The contents are a compilation of comments made by many mothers of teen girls who are facing this horrible condition and its medical and social issues at a very difficult time of passage. Most of the Moms give the Medical Profession a less than average rating in seeing the problem as a family disease, (Yes, we know it is inherited), affecting the whole family and the society where the family lives.
The author is 74 years old and is now diagnosed with VWD type III – acquired. A very rare condition due to an immune system malfunction. He, also, has had some very bad, life-threatening, experiences with Hospital care and Emergency Room procedures due precisely to the poor familiarity of the modern medical community with VWD and its various forms.