Draft of "Help, doctor, my daughter has VWD" with content!

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.

This is wonderful! You have really captured many of our issues into one article!

The only other thing I could think of adding is about how travel or moving overseas also poses big problems as VWD is treated differently everywhere you go and medical treatments may not be available. Many of the medications people on this forum discuss are not available or have different names here in Australia and in other places around the world.

Or maybe that could be another article? :)

This is fresh in my mind now as we are moving to Malaysia at the end of this year and it is is proving difficult to find medical support there for my daughter with VWD. All her meds may have to change soon which is a very scary thought... it could be better for her or it could be worse...who knows?

Thank you so much for taking the time to write this article and advocate so well for those dealing with these issues. Much appreciated!

I admire your ambition in tackling this topic. Your article has covered most of the discussed topics that we typically encounter dealing with the physical aspects and treatment of VWD. If i could offer some minor constructive criticism, your first sentence starts with how lives are disruptive and depression has set in. Maybe you could elaborate a little more on the depression and mental health part of it? Young girls with VWD not only suffer from embarrassing issues asscociated with the physical aspects but there are other issues they must deal with it. It is hard enough dealing with changes of teenage-hood and VWD can make it more difficult. There is the feeling of isolation from friends because you can’t take part school trips, plays or extracurricular activities because you need to have immediate access to a restroom, sleep-over parties may be intimidating, joining sports teams impossible, and even going to school dances can cause stress. And then you have to deal with all of your other girl friends starting to date and you feel that you may never find someone who would date and accept you. Family vacations may need to be scheduled around you and you can start to feel like a burden to the rest of the family. As di ^^ said even the prospect of having to move can cause family stress. Is there an international network of resources to tap into when traveling? VWD can be a confidence and self esteem killer to young girls. A once outgoing young girl and suddenly become very introverted. Getting periods under control is much more favorable than having to take anti-depressants.

Thank-you for stepping up for all of us in the trenches! Hopefully your article will make a difference to a new generation of young girls with VWD and their families. Good luck!

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Yes. The depression. This is such a big part of VWD. One of my daughters doctors said she had depression “caused by medical trauma”. At first this surprised me. But, when you think about it. … how could anybody losing that much blood not feel low? The mood swings, isolation, missing out on things, stress and worry all lead to feeling low and like you are the only person on the planet dealing with this. That is why this site is so good. It brings people together who are going through the same thing. Thanks to all for sharing. X

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I gave my Hematologist a draft copy and he may sign on as a co-author which would give it some “gravitas”. He said send it straight to Journal of the American Medical Association (JAMA)

He agreed about the need for more awareness for this disease among ER and GP people. Especially about drug interactions and bleeding issues.

John the elder

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Great job! Can’t wait to see how far this rolling ball goes! Keep us updated.

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