More thoughts

It can bring you closer. My sister and I have had an interesting relationship; we love each other but recently we have become closer. It was a change in her I think. She realised that she had been holding me at arms length; too afraid to love me completely because somewhere inside it would mean that she wouldn’t hurt so much if I died like our brother. But that was hurting her and with some honesty and perhaps me being a bit more grown up we have become who we are now. Sisters.

Today we talked about VHL. She realised that as her husband who was asking lots of questions about the disease, asked away she felt that she didn’t know anything. She felt like she had heard so much but taken in nothing. Perhaps, but as I said I’ve had to repeat my information so many times I suspect it’s just there.

Dad Update - email to people

Hello everyone.

Not the best news but the expected news nonetheless. Dad met with Mr S and Prof C on Friday and the results of the scan show that he does need to have surgery. The tumour in question has grown quickly and is what is causing the balance and speech problems. It was 10mm a year ago and now it is 2.7cm; it’s solid and it needs to come out. There was talk about waiting till after the baby is born but after a family conflab it was agreed that the little bugger should come out as soon as possible which is what Prof C says is the best course of action. Dad is going to contact them tomorrow to tell them this and I suspect that dad will be admitted quite soon.

Obviously there are risks with the surgery and they all sound horrendous but there is the chance that he’ll be fixed and as good as new. So once more, please put your positive thoughts; prayers; ask the universe… what ever it is you do... (probably best to avoid any pacts with the devil) to ask that all’s well that ends well.

Love to you all

Hatfield-McCoy Feud caused by Von Hippel-Lindau - my arse

There has been coverage of this story for a few days now. I'm really surprised by the number pf people who are interested in the story. I don't know why but it annoyed me. I think because I don't want this disease labelled a 'rage disease' I mean, really!

So I’ve commented on a few blogs about it. I saw on one that the VHL alliance have done there bit too see this

Good Old Joyce said “VHL does not cause rage, or even hormonal issues. It may lead to one of six different kinds of tumours, only one of which causes hormonal issues. A "pheo", a tumour of the adrenal gland, can cause surges of adrenaline, that fight-or-flight response that when you need it is great. It gives you extra speed and strength in an emergency. But injected into your body at random intervals, it can feel like a panic attack or palpitations. Very rarely is it interpreted as rage.

Might it have been a factor in the feud? Possibly. But don't forget that the Hatfields do not get pheos and they participated just as wholeheartedly in the feuding. There's a very strong component of culture and environment operating here in addition to the undiagnosed medical issue.

Having a medical problem is NOT an excuse for bad behavior. Everyone occasionally has bad days, or feelings of rage. Fortunately, few of us act on them. We are all still responsible for our actions.

Best wishes,
Joyce Graff
Executive Director
VHL Family Alliance
800-767-4845 or www.vhl.org

Bit maybe all publicity is a good thing.

Genetic testing

You know, it is an odd phenomenon. I was thinking about the implication. We said no to the idea of screening for VHL on our little girl and if you’ve read my whole blog you’ll know we made that decision long long before this little one came into being. But what does it mean in a wider context? It has implications for everyone. So many diseases and syndromes have genetic implications; could it get to a time when if you refuse to have a general genetic text then your children could be refused health care? Could it be the case that if you make the choice to go au natural then your child would be disadvantaged by your stance. And then I wonder to myself. If someone offered to engineer this little person growing inside me a way of ensuring she were VHL free would I take it? If they could of it to me would I? Ummm yes. I think so. I’m not 100% sure. Just something on my mind. Seems lots of people are thinking about it.

The Real VHL

For my partner this is the first real journey into the reality of VHL. We have been together 6 years and he has heard the word tumour so may times and he knows I have them, he knows my dad has them but so far no need for treatment. And so here we are slap back bang in the bit that makes it as bad as it is. It’s been a long time. Long in my sense of it. And I found myself thinking, ‘thank god I got pregnant before he found out the truth.’ So yesterday I felt guilty and today grateful. He would have probably run off and got a vasectomy if he knew. It’s a rough time ahead. I’m wondering if having been through it a few times now if it will be any easier. I may be being a bit premature, as we don’t know if they are going to operate yet. I don’t feel quite so sad today but I do feel anxious. I’ve started tidying, my coping mechanism. When I can’t control the health of my family I start controlling the environment around me.

I have a haunting memory of saying goodbye to my dad the last time he has a brain operation. He walked down to the exit of the hospital with us and I held him tight. The ‘I love yous’ flowed but the tears didn’t; we had hope in our hearts and minds and at the same time I felt deeply fearful. I felt full of sadness and I was a very scared little girl. I was about 13. I didn’t know if I would see this version of my father ever again. I did, as you know. My family have said goodbye to me; three times. My partner doesn’t know that feeling; but he will.

In a bizarre way I suspect this will help his understanding of my family and my ways. It could be a very important development and make him a better father to our little girl. He doesn’t need it to be an amazing father but maybe, just maybe this needs to happen. My eldest niece will find this very hard; she is nine. She is a drama queen and she’ll find it very hard to reconcile her real emotions and her nature to blow them out of proportion. I know this because that’s me too. I’ve had to learn to not be hard on myself when I indulged the fear and grief that comes with it but more importantly I’ve learnt how to lift myself up and get on. She is learning these lessons at a very young age. She already understands grief because of her parents splitting up but this is a different flavour of it.

So my partner and my niece will be doing this for the first time together.