Opinions needed

My daughter had an extended bleed at 9 months. Testing showed type 2a. Hematologist wanted a second round of testing to confirm. Second round resulted in normal bloodwork. She had another extended bleed at 14 months. They tested again… Normal.

Now the hematologist wants us to see someone at Texas children’s in Houston.

I’m concerned we are spinning our wheels and maybe this isn’t vwd?

Does anyone have a similar story or can offer more advice?

I have vWD type 2a. So does my youngest son.
Type 2A is difficult to diagnose. On one test, result is near normal. On another test it is not. What is important is that bleeding is real and life threatening.
I first took my son to a hematologist who read and went to seminars on vWD. That was not enough.
Hematology is a wide area. A bleeding disorder specialist is really needed. As for us, we went through DDAVP challenge tests. This is to confirm that Stimate won’t work. Only Immunate does.

But Immunate is not always available.

We spent so many trip to ER when our son turned 5 and started to have nose bleed. Now life is much better. No more ER.

Preventing and stoping bleeds are the keys. These are what we did.

Many antihistamines promote nose bleed as side effect. Talk to your doctor when your daughter has cold.
And some nasal spray decongestant causes nose bleed too.
Fever, high temp can cause nose bleed.
Check for allergy - prevent bleed.

After all that, the kid still bleed. then

Stoping bleed, out side is easy enough. Main trouble is the nose bleed.

Learn the usual location. It is usually not that deep. The wound is not that big. But in the beginning all must get used to the trauma of nose packing. Usually, hematologist are not trained for this. I recommend to find a really old ENT doctor. If your daughter has nose bleed go see this old doctor. Bleeding can be stop without specialized medicine.

For us, we take tranexamic acid to help with clotting.

To stop nose bleed, i use adrenaline soaked cotton strip.

Nose packing procedure-
Usually nose bleed happens during sleep. Pollen season and dry season.
First gently blow out the blood. Blood can pool in cavity near the forehead. The can make bleeding seemed worse than it is. And if the drug can’t reach the wound, it won’t work.

Gently feed the cotton strip in. You can do this much more gentle than a doctor would.

It can take one to eight strips. Depend how long your child had been bleeding before discovery.

Adrenaline is dangerous when injected. For topical application it is safe. I don’t know if a doctor would let you keep a stock of adrenaline at home.

The best way is to learn how how to treat your child’s bleeding.

I have vWD and am used to blood. Most people find the topic nauseating. My wife cried and cried when she learned that our baby bleeds.
After almost two years, we are taking things in stride. We get used to many things. And we took back control of our lives.
This is a rare disease. I a career, a doctor may not even see a vWD patient. And it turned out, you may not need a super specialist once you learn how to stop bleeding.

For me personally, i went through a few hematologists before my mother found a bleeding disorder hematologist.

Then a puzzle is solved.

There are certain positive side effects to vWD. Not better than a normal life. But there are consolation prizes.

  1. After extended bleed, the body adapt. Heart and lungs are stronger. Good for swimming.
  2. with special needs, vWD kids get attention form parents. Learn to be patient. Generally do well in school. (In my days, there was no iPhone games, i had to read while other kid played)

Dear Friends,

I have acquired vWD. It was first diagnosed as type one, then a couple of contrary VWF multimere tests at a major Hemophilia center, Then GI bleeds and a recent severe bleed after very minor surgery (I lost about two liters). VWF and Factor VIII now put me as type III vWD. Because the VWF is behaving so strangely and I am a non-responder to ddavp , I may have a combination of vWD types. Find a compassionate and empathetic Hematologist who really wants to establish the correct diagnosis so the cure will be the right one. Because type II can be several different forms and the cures are mutually exclusive, it is important to repeat the tests. This kind of testing is almost always sent out and the samples can degrade during transport leading to inconsistent results for the same test at different times. It is not your blood that is different, its the treatment of the blood after collection that differs and that is out of your control.

I have not had a nosebleed since a middle-aged ENT used silver nitrate swabs to cauterize a couple of Nasal veins. I was lucky.

Best of luck to all,

John the elder