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How long between 1st and 2nd VHL related illness

KateInUK
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I had a HAB on the cerebellum 12 years ago and then told it could be VHL and should be screened. I was screened for a while. The DNA test has twice come out in the "don't know" and so have the tests on my parents. Now the doctors have dropped my screening - except the opthamologist who is still screening me annually.

I have recently had a run of headaches (although not as bad as fisrt time around), nausea, dizziness and general feeling ill.

My question is, if I have VHL, can the second occurence of a HAB or other cyst be 12 years apart - or is it unlikely due to time lapse.

The GP has referred me to the neurologist, waiting for appointment, but I can't stop worrying, which in itself causes headaches. Also I now have 3 children and it worries me how a diagnosis of VHL will affect them.

Explore topics in this discussion:

Anxiety Dizziness Astrocytoma Vertigo Hemangioblastoma

16 replies

SHAWN1

Kate,
I totally understand how you are felling. I have VHL and had a hemangioblastoma in my right cerebellum removed in 1993. In the past fifteen years I have seen my brother go through countless surgeries to remove tumors while I have not had any problems. I just had an MRI last week, the first in probably 8 years. Though I feel fine, my report said that I have two hemangioblastomas. I have an appt next week to see what my options are.
I read something on the internet and I may be able to forward you the pag. My understanding of it was that through genectic testing that, doctors/researchers, can tell how bad, for lack of a better word, the strain of VHL a person has which is useful for future treatment.
In the US, there is the NIH which has specialized resources in dealing with VHL, including genetic testing.
Take care.

KateInUK

Thankyou Shawn1 - I think I should stop feeling guilty about worrying and feeling a hypocondriac - I need to get this checked out and find out what is needed for my children.

SHAWN1

You are welcome! I hope everything works out for you. You should also check out www.vhl.org as it has a tremendous amount of info that will be helpful to both you and your doctor.

Also, I think on inspire you can search for people in UK that have VHL. You may be able to contact them as far as what resources are available to you there.

Take care,
Shawn

SHAWN1

Kate,
I did a search under "meet friends" in the UK. One of the results was Maryweetman. Try to contact her as she heads the VHL Alliance in the UK.

Good luck!

KateInUK

Shawn,

Thankyou for your time and effort - I hope your doc has some positive news for you - I will keep you in my prayers.

Kate

TinaDiane

Hi Kate,
I don't think you are a hypochondriac! I would feel awful if the DNA test came back "not sure". When I was tested here, in the US, the genetic testing went to Boston University School of Medicine Center for Human Genetics , (617) 638-7083. The director is
Aubrey Milunsky, MD, DSc. I found out I have VHL mutation R167Q, which is type 2 b. This is very helpful as it indicates which types of tumors I have the most chance for growing. I am 50 years old and had 2 surgeries (adrenal pheochromocytomas and pancreatic islet cells tumors removed) 22 years ago and 5 years ago. I now have 8 hemangioblastomas on my spine that have been there for 2 years (at least because my first MRI was 2 years ago) and they have not changed. I encourage you to try to have genetic testing again, maybe they can send your blood to Boston. I have a large family, none of my brothers or sisters or Dad have it. My mom died 40 years ago of a brain tumor but it was an astrocytoma and I have no way of knowing if she had it or not.
Good luck and God Bless,
Tina

Crazydog

Hi Kate

It is now 20 years since I had 2 brain tumours removed from the cerebellum and about 19 since I had a large part of my right kidney removed. I was diagnosed about 5 years ago with another brain tumour that has so far not grown but last week my abdomen scan revealed I had another kidney tumour. I consider myself very lucky with this time scale as my son is alot worse especially his eyes. So some VHL is slow to show itself while my son had a tumour removed from the brain then 6 months later has another plus a spinal one.
I don't know what part of the uk you live in but we are looked after by Oxford hospitals and they are very good and their genetic nurse is brilliant. We love him to pieces!!
I would also like to say my daughter was tested and is clear of VHL which I will admit was a big relief.
I personally think you need to know if any of your children have VHL as at least then if they have any symptoms you will be prepared. My son was diagnosed at 7, has grown up with it and apart from when he was a moody teen has coped very well as VHL has been a big part of his life.
Keep your chin up and fingers x that all is ok
Vicky (crazydog)

joyceg98

In the UK, most if not all VHL testing is done in Birmingham by the laboratory of Dr. Eamonn Maher. Mary Weetman is a great resource for finding help in the UK.

DNA answers are not always clear, for no fault of the lab. Some of us may even be "mosaic" for VHL (there's a discussion dedicated to this). If your parents have VHL, then you are not mosaic. But in a situation like this, where your own diagnosis is unclear, one answer might be mosaicism.

To answer your original question, there is no way to predict when you might get another VHL-related issue. As Shawn described, it varies widely from one person to another, even in the same family, with the exact same alteration in the gene.

All we can do is continue to be vigilant. Yes, you need to get these symptoms checked out, if only to allay your worries. Worry alone with give you a headache, so let's get that out of the way and get some DATA that show whether or not you need to worry.

With an unclear diagnosis of VHL, it would be smart to continue VHL screening as if you had a diagnosis of VHL, just to make sure that nothing gets ahead of you. If nothing more shows up on those scans in the next five years, you might lengthen the intervals. But since you are having symptoms, you should check this out as if it were a suspected VHL issue.

All best wishes,
Joyce

MARYWEETMAN

Hi Kate.

I was sorry to hear of all the anxiety you are feeling in regard to your health at this time. This is not unusual or irrational and I am sure that most of us would react in the same way.

Your doctor has done the right thing in refering you to a neurologist, and hopefully this will give you reassurance on the VHL front. Your symptoms are the same as in other conditions, so are not nessesarily related to VHL.

We have a group in the UK called the Von HIppel Lindau Contact group, which exists to give help and support to people with VHL and those at risk of the condition. Whilst you do not fall into this category I would be only too pleased to give you any support you need, as the issue of VHL has been raised in the past.

The issue of your blood test results is clearly problematic, as ambiguous results neither confirm nor dismiss your fears. This happens very occasionally and you have been really unlucky to be in this situation.

It is a good idea to keep seeing your opthalmologist, as they are skilled at detecting the manifestations of VHL in the eye.

Please feel free to contact me at any time by e-mail maryweetman@waitrose.com or by phone on 01204 886112.

Let me know how you get on following your consultion with the neurologist.

Best wishes

Mary.

diaryofageneticdefec

by now you have heard that time between tumours, cysts etc can be maddening. I remember growing up thinking that VHL wasn't so bad because i was either too young or not even born when my dad had surgeries. Then at about 13 he had a brain op, that's when it became a reality for me and then finding out I was high risk of the gene was obviously rubbish news.
I respond to every twinge, each and every tingle and have worried so much about headaches i've given myself a headache. The best advice i can give is to have regular scans, make sure you contact your consultant is you are worried about anything. Keep positive and don;t beat yourself up about your worried and fears. VHL can make you mad sometimes, but get mad at it back

KateInUK

Thank you everyone - its good to know other people feel the same.

I have had my blood tested twice by Professor Maher - but I do wonder if my case is just not being managed by my local geneticist because they don't know where to get the funding from for this sort of disorder. I was seeing someone who either covered paedeatric genetics or oncology - I must try and change this.

I have got a neuro appointment next week - luckily not with the consultant who 12 years ago put all my symptons down to migrane and vertigo!
Until I went private and he agreed to do a scan and I then had to be operated on on the same day - what state is the British NHS in! See - I am getting a bit mad.

Mary - I may contact you separately for advice on screening programs for me and my children.

Thanks all.

SHAWN1

Kate,
Best of luck with everything as we are all here for you!

Shawn

joyceg98

It's not just the UK -- this kind of thing happens everywhere. I'm just glad that you are now on a good path.

Best wishes,
Joyce

KateInUK

Saw the neuro guy yesterday and he has booked me in for an MRI to find out what's happening at the moment. He also is lining me up for annual renal ultrasound and pheo testing. He will do MRI any time I have any worrying symptoms. He does not think it necessary to screen my children yet - I think I need to take advice on that because the VHL Handbook suggests screening from the age of 1.

Although I'm not feeling well - I do feel positive that something is being done. The MRI will either confirm something or rule it out and then I can move on.

MARYWEETMAN

Kate,

I hope you have now received my e-mail reply to your questions. If not pleased contact me again by e-mail, maryweetman@waitrose.com or by phone 01204 886112.

Mary.

KateInUK

Great news - MRI clear. Just got to sort out why I am feeling ill? Back to GP.

At least this scare has got me back into a screening program.

Thanks to all your support.

Kate

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