Elevated Von Willebrand risto factor [Archive] - The Lupus Site Message Boards

Clare.T

08-27-2007, 01:23 PM

Hello Elena

I wish we could sit down together over a cup of coffee and talk about things face to face - tone of voice, facial expression body language make such a difference.
You've been doing a fantastic job over many years getting help for your daughter. It's hard to imagine what a struggle you have been through, what anxiety you feel and, from what you say, all without any support from your husband, her father. That puts an extra burden on yourself, on your couple and on your daughter and the rest of the family, even if she has no siblings. If she has no siblings the family dynamics could be even worse in intensity

It sounds very much from what you have said and from the accounts of the latest consultations and doctors' comments, that she does have an autoimmune connective tissue disease, so let's assume that is the case.

What happens next ? You will have succeeded in the aim of making sure your child gets the best care possible so she can have the best life possible. Nothing will be undone as a result of you not following through your worries. ( If you were reassured that there is no sign of chronic disease you would also have achieved that aim.)
At this particular stage I suggest you leave the interpretation of the tests to the doctors - it sounds as if you have now gone beyond the need to know what tests should be done and what they are for ("the homework" ) and have gone on to trying to interpret them, or diagnose, at least to foresee diagnosis.
I suggest it's waste of time and your very valuable energy that could be put to better use in considering your probable future role as the parent of a sick child with a chronic disease, in what ways can you continue to ensure the best for her. This is a whole other topic.

About the test results.

As of the letter they were still waiting for the antiphospholipid to come back(trying to remember spelling).

The tests for the anti phospholid antibodies, lupus anticoagulant and anticardiolipins are important for diagnosis. Abnormal results might indicate an increased risk of clotting episodes and could account for any number of other symptoms such as headaches. From what I have read, the anti beta2- 1 glycoprotein test is important in establishing the cause of abnormal test results as being an autoimmune process, rather than an infectious disease, like Lyme Disease, or congenital / acquired blood clotting disorders like von Willebrand.

One thing I did notice, was that her Von Willebrand factor came back high (140-160 was listed on result sheet as normal) Rachel's was 170. There was also another Von Willebrand type test where same 140-160 was normal and her's was a 160.

Only the doctor can decide if out of range results are significant, taking into account all other evidence. Blood test results can vary from day to day

or Of course I had to look it up. I was able to understand that that a Low Von willebrand factor could/does mean blood clotting problem. (anemia) Also called Hughe's syndrome, a high result could mean too much clotting "sticky blood" as it is called.

This all sounds very muddled to me, not that I really know what I am talking about.
Hughes' Syndrome is another term for anti phospholipid syndrome, APS, sometimes referred to as 'sticky blood'. It isn't another name for v Willebrand's disease. As far as I know, neither can be described as anemia.

I have no idea what relationship if any there might be between the two.
After a certain point, it's all way beyond my understanding and I dont need to addle my brains more than they already are !

Rheumy did mention potential MCTD

MCTD is a term usually used as a label for a particular set of symptoms some of which are often found together. Usually this diagnosis isn't made unless there is a high level of anti U1 RNP antibodies which is generally regarded as a defining characteristic, one of the criteria for this diagnosis.
The symptoms are a mix of aspects of lupus, polymyositis and scleroderma plus other features like 'sausage' fingers and almost always Raynaud's.

There's not much point getting too hung up on the actual diagnosis. Although we do like to know 'exact' diagnoses, it's arguable that there is no such thing. There's some controversy over the usefulness of this label, so who knows they might abandon it tomorrow. There will still be patients with these sort of symptoms who need treating. Disease modifying treatment is the same as the major connective tissue diseases plus treatment for the various symptoms as required

Rheumy felt that the test results of the blood work were "encouraging".

That sounds good even though I'm not sure what encouraging means. It's possibly good that anti ds DNA isn't present. Kidney function needs checking with urine and blood tests. Absence of anti ds DNA doesn't mean she doesn't have lupus.

Rheumy upon examining Rachel did mention shiny tight skin on fingers, some "drop off" when using that eye thingy when looking at her nail beds. Does "drop off" mean that the blood vessels end in the fingers before they should?

I have no idea what 'drop off' might mean. The shiny tight skin might be sclerodactyly, fairly often found in connective tissue diseases. Again its diagnostic significance depends on many other factors.

I also came across some info that mention that more than 90% of patients with Scleroderma had Raynaud's years before a diagnosis.

Looking at statistics can be comforting but they often prey on the mind and be uselessly harmful. The obvious come- back to that one, is how many people have Raynauds and never get scleroderma, or anything else

My advice is to let the research be for now. Switch off the PC and focus on building up your relationships with daughter and husband. In his case essential bridge building. It's rather bad if he has taken to hiding test results from you - that's a factual comment not saying who's right or wrong.
I strongly advise against getting test results before you have seen the doctor, unless you are already very knowledgable and experienced and can roll with any blows. It always leads to increase levels of anxiety not to say anguish.

Find some enjoyable activities together, you all, and you and your husband.
If you can access professional help in dealing with emotional and psychological issues, do so. If you really can't stop worrying about your daughter's health consider anti depressant and anti anxiety medication as well.

If you can resist other topics, you might find useful online resources on dealing with chronic illness in the young.
Have a look at the Paediatric section here to which I will copy this in the hope that other parents might be able to offer their experienced advice or you can make contacts

Please keep in touch and let us know how things go.

Many strengthening hugs

Clare