bleeding disorders

My wife has mitral valve prolapse and severe mitral valve regurgitation, so her doctor has suggested mitral valve surgery. He suggests mechanical mitral valve because she is young (38). Unfortunately, my wife has von Willebrand's disease too. Is von Willebrand's disease contraindication for mechanical mitral valve?

Thank you for logging onto our web site.

Your is a most interesting question that I have not been posed in the past. I am also interested to see that our web site has reached as far as Croatia.

While I have had patients with bleeding disorders that required aspirin type agents, I personally have not had any patient who required a valve replacement. Therefore, I would be interested to hear if there is anyone else with this similar dilemma.

At your wife's age, a mechanical valve is her best option as it will last longer than a tissue valve. This, however would require the use of long term anticoagulation. Cardiologists, and some hematologists regularly use agents that cause platelets not to work well, such as happens in von Willebrand's disease, along with agents that interfere with clotting, such as heparin and coumadin. While there is an increased risk of bleeding in these patients, the benefits must be evaluated. Therefore, it is not an absolute contraindication, just a decision that must be made with careful thought and longterm care to prevent adverse bleeding with notification of your medical personnel at any sign of bleeding.

I would be most interested in hearing the outcome.

Thank you for logging onto our web site. Typically patients with von

Willebrand's disease or platelet function abnormalities do not have cerebral

bleeding such as your family member. Patients with very low levels of Factor

VIII as can occur with some forms of severe von Willebrand's disease, may

have bleeding complications that are more like hemophiliacs and include

serious bleeding including cerebral hemorrhage. There are also several other

factors that may contribute to cerebral hemorrhage that are more likely, such

as abnormal blood vessel formation called atrial venous malformation,

aneurysms, and trauma. These have nothing to do with a familial bleeding

tendency.

I am in the midst of being diagnosed with von willebrand's disease. I
have had bleeding problems after several surgeries, have heavy periods
etc... I am curious as to how my activities might be limited if I do
indeed have this disease? Heather

As for activities to avoid. By the time most people are diagnosed with this disorder, they are in their 30-40's so the question as to which activities to avoid may not be pertinent. This is a mild bleeding disorder which you have LIVED with for a while. The bleeding that occurs is usually not life threatening, therefore good common sense is what we recommend. I would not perform dangerous sports such as sky diving or soccer (without a helmet). However, I wouldn't allow my children to do these sports anyway as I would be concerned with damage occuring in any individual regardless of whether they have a bleeding disorder. Let your body be the judge and do not curtail much of your activities. Exercise is good for you and you should exercise regularly.

If you need to go to the dentist or for any procedure that involves needles or cutting, then pretreatment will be needed and should be coordinated with your hematologist.
It's important that you family members also be tested.

I was diagnosed with a very mild form of von Willebrand's disease approximately 3 years ago when I was pregnant with my second son. Since then I had a C-Section with some bleeding problems during the procedure and afterwards. I was treated with Premarin which seemed to help. I have not had any problems other than a very heavy monthly period since my C-Section. Until now, of course. On 2/6/02 I had my tubes tied and an endometrial ablation. I had some problems bleeding afterwards because of my activity but I eventually stopped, until 2 days ago. Now I am passing very large clots (child's fist size), and I have continuous bleeding. Bright red. I am going through a super plus tampon and a maxi pad every hour to 90 mins. I have called my doctor twice and he told me to keep my feet up and gave me a RX for medroxyprogesterone 10mg tabs, to be taken 2 pills once a day. My concern is that I am feeling very week and have not had any bloodwork done yet. I'm hot and flushed, sweaty and extremely dizzy when I am up.

In our experience, every uterine ablation that we have done in a patient with

von Willebrand's has failed. We are in the process of recording this

information and will hope to publish it. It would be nice to be able to

include your information in the article if there are details that you can

make available to us.

As for the bleeding, there are several options that may help. THe first is

Stimate nasal spray. Second, which may be used with the first, is Amicar.

THese work well to decrease bleeding complications with von Willebrand's

disease. (see bleeding disorders section for more information) If these do

not work, then Provera, progesterone may stop the bleeding. I would

recommend that a blood count and iron stores (ferritin) be obtained to make

sure that the weakness is not due to anemia that should be treated.

Best luck to you. I hope this has been helpful and will add your letter to

the web site board for others to benefit.

I have Von Willebrand's Disease and suffered a massive heart attack 9 months ago. I was put on Plavix. From the start I began having problems. I'm bruising more now than ever. I self inject medication 8 times a day. I bleed profusely from the sight I use to inject. A few times in the past 9 months my blood count was very low. I am also having the capillaries and veins in my leg coming out often. I will never wear a skirt or shorts again. Last night I suffered a subconjunctival hemorrhage in my right eye. The Dr at the emergency department told me to stop the Plavix at least for a few days. I'm concerned about going back on the drug. I don't want to have another heart attack but I also don't want to bleed to death. Could I have your opinion on this please. I am 50 yrs old.

Thank you for logging onto our web site.

Yours is a difficult problem. While we have had patients with bleeding disorders that suffer heart attacks and strokes, it is much less common than in the general population. Therefore, there is little information regarding the best management.
Patients even without bleeding disorders note increased bleeding with daily aspirin or Plavix. If your platelet count is low, then neither medication should be used. I do not know what you mean by your counts are low. We have been able to maintain patients who bleed on Plavix by altering the dosage slightly. We often hold it during their period to decrease the bleeding. Because it has a long half life, one tablet will stay in the system for quite a while. While the large studies in preventing heart attacks used daily dosages, I would try to stay on some form of anti platelet agent, either aspirin or Plavix, whichever seems to cause less bleeding.

I would recommend that you discuss this with both your hematologist and your cardiologist so that they may weigh the risks of your bleeding with the risk of another heart attack. Then they will be better able to help you decide.

Thank you for logging onto our web site. Does the au stand for Australia??

In response to your question, the first item is the iron deficiency and

anemia. It is important that your daughter is on appropriate dosages of

iron. The usual dosage is Ferrous Sulfate 325 mg three tablets daily. OR

one slow release iron tablet, slow Fe is equivalent.

If she is on this dosage of iron and there is no improvement in her blood

count, than she is unable to keep up with the amount of bleeding she has had.

In this situation, an iron infusion by vein may correct the iron deficiency
as we are able to administer several months of iron in one afternoon. It is
important that this be administered by a specialty center that is used to
monitor this therapy as there is a risk of allergic reactions. I would not
wait until April to correct the blood count, as your daughter will feel
better with a normal blood count and it would be best to correct the anemia
before a transfusion becomes necessary.
The von Willebrand's testing must be done by a specialty laboratory. It
should be interpreted with the blood type, as patients with blood type O have
lower levels of these factors and may be falsely diagnosed. Also, the levels
should be checked while OFF any hormones as hormones may elevate the levels
and the diagnosis may be missed. It is also often useful to check family
members as this is a familial disorder.

There is excellent treatment available, see bleeding disorders on the web

site.

My 8-year old daughter has just been diagnosed with type 1 von Willebrands. She suffers from frequent bloody noses, although they are not particularly heavy or long in duration. Now I am told to administer a spray each time she has a bloody nose and each time she bumps her head. She is a very active child, fearless in fact, so she bumps her head quite often. She always wears a helmet biking and roller blading, skiing and snowboarding. She is an avid ice skater, jumps and all. Do I have to curtail her activities? Do I have to worry about brain bleeding everytime she bumps head with another girl when playing tag? Also I am told that the spray can adversely affect her eleoctrolytes, so I wonder if the treatment is really necessary?
CCH in CT

The primary goal of treating persons with bleeding disorders is to improve their lives. This includes helping them to live NORMALLY for longer. Therefore, we encourage all our children to continue active lives. This includes sports, however we usually try to encourage them to NOT participate in contact sports such as hockey that are more likely to result in injury.

Persons with mild von Willebrand's disease do not usually suffer from head bleeds unless they have very low Factor VIII levels. Therefore, with minor bumps we would usually only watch them closely as you would any child. Recurrent nose bleeds are not life threatening, but may result in anemia and interfere with activity. Therefore, for recurrent nose bleeds we recommend the use of aminocaproic acid. For others, we use the nose spray you refer to. Usually it is just a matter of learning to live with this disorder to maintain health without interfering with normal life.

DDAVP nose spray if used more than three days in a row or in infants and babies may result in lowering of the sodium level in the blood. This usually does not occur, but the medication should not be overused.

I would encourage you to find others in your community with this disorder as speaking with them usually is helpful. If you prefer, I would be happy to help you contact one of our HART board members with similar problems and you could e mail or call them.

I'm 37, type O+, and I've been trying to get pregnant for about a year. After 2 very early miscarriages, the doctors ran a whole slew of blood tests. What came back initially pointed to a clotting disorder (compound heterozygous for the MTHFR gene mutations, and slightly elevated anticardiolipin Igm) . They started me on lovenox and baby aspirin. After I bruised so badly on it (although from everything I've read, most people bruise badly from the injections), they did more tests which came back pointing to von willebrand's and they took me right off the lovenox and aspirin. How can I have both of these problems at the same time? How could I treat one without exacerbating the other? The only symptom I've ever displayed for vWB is that I bruise easily. Normal periods, (short cycle though, but I don't think that's associated), and I've never had any clotting problems that would point to thrombophelia. The doc said that it's possible for the miscarriages to be caused by clotting, but it was so early there was no way to tell. So for months I've been concerned with making sure I don't clot during pregnancy and delivery, and now I'm worried that I might hemorrhage? Help....

Wendy

Whether the compound heterozygous mutation of the MTHFR without an elevated homocysteine is a risk factor for blood clots is controversial. If a risk factor, it is probably a very minor one. However, when you review the recent literature regarding problems with pregnancy, this may be of importance. Lovenox alone at a dosage of 40 mg daily may be adequate to ensure a healthy baby and the aspirin may increase bleeding risk in individuals with von Willebrand's disease.

In regard to the diagnosis of von Willebrand's disease, it is important that the labs be done in a specialized clotting laboratory. (Please see information under bleeding conditions). Typically people with this disorder do not have life threatening hemorrhage.

Both diagnosis are not uncommon in the general population so it is not unheard of that you have inherited both. They may balance each other actually. I would make certain that you actually have vW disease and then discuss the recent literature regarding lovenox alone with your physician.

Send mail to rshtcn@aol.com with questions or comments about this web site. Last modified: March 02, 2008

Send mail to rshtcn@aol.com with questions or comments about this web site.
Last modified: March 02, 2008