Writing a will

I wrote a will when I was 17. 

It wasn’t a fully legal one with a solicitor or anything like that but I was thinking that now I’m a homeowner I should do a proper one. I’ve lost the other one, it was on the computer we had at my parent’s house. I didn’t have much to leave behind but I remember thinking about my diaries and any jewellery I liked. I think I was going to donate my violin to my old primary school. When I think about it now, I’d just have more stuff to give away and now it would go to my partner and then he’d sort out what to do. But that was the unimportant side of it, the important part was to help people who would be left know what my last wishes were.

When my brother died we didn’t know exactly what he would have wanted. ‘Not to be dead’ someone said at the time. I’m not sure that was completely true. If my brother got his way death is an adventure and he’s ‘still travelling’ as it says on his gravestone. What did he want? He mattered a bit at the time but I think we did him proud, as with many funerals of the too young the church was packed, standing room only and some people had to be outside. My uncle spoke about him and although I knew how great he was I don’t think everyone there quite realised just how great. He was cremated and some of the ashes were buried, some were scattered in other places. I want to be cremated and the ashes can be spread wherever you (loved ones left over) like. So what do I want? Well a packed church would be nice and my eulogy needs to sounds like the truth. And I want the truth to be that I have lived the life I wanted to and helped others while doing it. I’d like to think that I was a good enough teacher to warrant some of my students, past and present, turning up and I want to be missed. I’d like to think that I leave behind hope though. A grand idea I know but that’s something worth lasting.

Opticians

It isn’t all bad then, this disease of mine. I booked an opticians appointment at a well known chain of opticians, not that I think that’s a good thing, really I think we should support small businesses, but I haven’t because the money side of it swayed me, anyway. I haven’t been for a while, 3 years or so and as I get looked at at Moorefields every year and they know what they are doing as far as eyes are concerned. So the last time I went was in Barnet and this time I went to Woodgreen. They have a funny system of having to put you on their books, maybe it’s a franchise thing. The site test started as normal and then he asked ‘any problems with your eyes.’ ‘Well.’ I said and then I launched into… ‘I have a tumour on my optic nerve in the right eye and a small patch in that eye where I can’t see due to a crinkling of the maclia.’ The optician looked visibly happier at this news while trying o maintain his professional happy face, writing it all down in a box on his form that was far too small for all this. ‘I have Von hippel Lindau disease.’ I said smiling away as you do.

‘I don’t remember how to spell that.’ He said, his voice steadily controlling his excitement.

‘VHL will do.’ I suggested helpfully and he wrote it down. I smiled at him an ‘it’s alright to be excited smile.’

‘I used to work in a hospital and I saw all sorts there but not this, this is better than an average day isn’t it.’ He went about being very professional doing all the correct tests and I was pleased to hear that despite all that’s going on I actually have 20/20 vision. We talked about Moorefields and how when I go they often ask if the other people working there can have a look in my eye and I let them and so he tentatively asked if the other opticians could look, ‘of course.’ So for a brief time, my eye and my tumour had a slice of celebrity about them, we were now on first name terms and even though I hadn’t officially booked the contact lens appointment he saw me anyway so that I didn’t have to come back another day. Which was very kind of him (and I got that free which was even better) He discovered I had hayfever which was news to me and that I should change the type of contact lenses as I had a bit of an oxygen deficiency to my eyes. I have to say that I was very impressed by him. He told me off in a playful way when he heard that I don’t really check when I need to change the contact lenses each month. ‘with all that is happening with your eyes you should look after them.’ He’s right.

And so I left said chain opticians feeling special and rare and looked after and like I’d got a real bargain on my glasses (they did me a deal there too) Having a tumour on the optic nerve isn’t great but at least that day it felt better.

An email to people

Hi all

I thought you might like to know the latest on dad. He has spent the week in St Barts and has had lots of tests and scans, it’s mixed news really. Addenbrookes don’t appear to be able to locate his previous scans so they don’t know if any of his tumours are growing so he’s got to go back in 3 months to see if they are and then they’ll do a biopsy on one of the ones in his neck. It’s been a bit frustrating and dad feels a bit deflated as we all thought that they would be able to come up with a solution this time but we are still playing the waiting game.

The back surgeon doesn’t think his pain is being caused by the tumour on his lower spine but would operate to remove it if dad wants him to. I won’t go into the remarks on all of his tumours as I’ll be here all night.

He has been started on testosterone gel, weird and he can’t get it near women or children with it unless he’s covered up!

Any way

I’ll let you all know more when we do

I wish

I wish someone could fix my dad

20th May 2006

An odd week coming up, my dad is in hospital all week and I’m going to visit him everyday.

Tonight I walked onto the ward. 

You know the expression ‘face lit up’ well that happened, it was so strange. I know my dad loves me but I think I know him well enough now to see when he is scared. 

He is scared, he won’t admit it, anxious or nervous or apprehensive but he doesn’t say scared. I am. More afraid of the inevitable and as selfish as it sounds I always expect what ever happens to him to happen to me but earlier. When I’m feeling positive I think that it won’t ever be me that I’ve had my last tumour.

When I was at university I have a strong memory of sitting in the auditorium, listening to a lecture and feeling my arm go heavy. I could lift it, use it but some how it felt heavy and I knew that feeling, my other hand and arm had been doing it for months before I told anyone and then I had a scan and then I had a brain tumour. Of course I had the tumour while my arm was going heavy but it felt like it didn’t appear until I told anyone. I sat wriggling my fingers, pinching away at my arm and then I started to cry. My lecturer notice and she looked really pissed off. At the end of the lecture she asked me what was wrong in a cross way. She knew about the previous surgery and I told her about my arm, her face changed. We talked and she asked what would make me feel better. Imagine.

'A scan everyday.' That was it, I wanted to know every second what was going on inside me. Impossible.

I didn’t get there on Thursday by the way.

You don’t always get what you want

MARCH

Every one of these changes has left its mark

This time of year is bizarre. Ten years ago my brother died. Ten years ago I notice how warm the sun was on my face and I was glad I was alive. He wasn’t but I was. I took my life and I decided to make it worth living. I didn’t go to Disney land, I didn’t think about that side of life ten years ago. I listened to my uncle speak about my brother at his funeral and I knew that I wanted to have someone say things about me if I died. I didn’t want to be dead and have no epitaph so I set about living a bit more. You might not recognise it as such but that’s what this is. Take every moment and live it, even if it is crossing the street, cleaning the toilet, enjoying the touch of a hand, flirting, coughing, sleeping. As long as I’m not bored. I realised that I was bored and I don’t ever want to experience that again. Now if I feel boredom creeping in I get up and I do something, anything. 

I loved my brother and I remember him with love and fondness and I want him to still be here. I don’t want to be dead.

I still love him. When I’m at my most optimistic I think that he is still capable of loving me. I’m pretty sure he is the one who stops me having car accidents.

It is three minutes past ten

Can't sleep

I should be asleep but I can’t, I’m thinking of my non child, she is just like me but better, she has all the best bits of me and all the best bits of him. She doesn’t get upset or cross and she is bright and beautiful.

24th February 2006

Apparently we are young to adopt.

I want to take my own risk; I want to have a baby. Partly because the new child laws make it almost impossible to adopt without having the fear of god shoved at you, with all of the statistics and the worries who would. It makes having children a very harsh reality.

Everyone I know seems to be getting pregnant, older people and people at work – and even on TV.

Why me? I really don’t get why him, a choice made once. I wanted to be married, I wanted that, I wanted the dress and the day and the ring and the silly surname vain – but once upon a time he said he wanted me. It wasn’t in a chapel, church, there was no priest or rabbi there was only us and an empty house and door between us and my tears falling down a sink.
As I remember it we had to talk, after all sorts of not really talking about it we sat on the shared sofa happy that we were going to live together. We had of course been living together but hadn’t been sharing rent, we were of course deeply in a relationship but I was in Barnet and he was in Bounds Green. His flat mates had agreed and so had we, we were going to live together but we had avoided the big conversation. Children.
Not a simple conversation when one of you is a genetic defect with a 50/50 chance of passing it on made more complicated still by the almost genetic fear of eternal damnation verse atheism. If I could have a baby and then terminate, if I was capable of knowing I had a life in side me and then having it tested and knowing it was the same as me and my brother and my dad and then killing it, then the conversation would have been quite a bit easier. But I am not a simple person; I am a non-practising catholic with all the guilt that goes with it (if I was still practising the guilt would be less) There we were having the hard moment deciding if we thought we should still bury our heads in the sand and forget that we would not be able to make the easy decisions in life ‘leave the condom off baby let;s make a baby’ you know the kind. If you do know the kind then you are not me.
That only happens on the very tail of my period when we are both, ex catholic and atheist, hoping for a miracle. Funny isn’t it I spent a heavy part of my young sex life in perpetual fear of getting pregnant and now I spend my adult hood in fear that I will never be a mother, ever.
I digress, so there we are talking about our non-existent baby, our non-existent future and we are deciding on it. I cry, I get frightened and I need to make a choice. It turns out this is my decision. He is very clear about his philosophy on life and when it begins. I worry that I don’t know if I can love him as much as I should and I worry that I love him too much and I worry that if I was with a less intelligent man I might be getting pregnant there and then which I don’t think is financially a good plan.
I cry more, he doesn’t and through it all it gets very intense, I don’t remember much of it because I feel that at some point I have to black out. I do remember saying ‘ I need the toilet’ on the pretence of blowing my nose and actually have a pee. I cry, I ask god for help and I cry and I realise that I need to decide between the man I’m fairly sure I love and the baby I will not be able to have with him because either we will never have one or if he got his way I would have to terminate, abort, cut up, destroy, kill one that had a genetic defect, my, my dads and my brother genetic defect.
Nice.

I hold on to the sink with my hands as I sink down, beneath it, the light isn’t on I don’t want to see my reflection and I sob. I hear him at the door. He is telling me he loves me and I cry and I tell him I have to choose my baby. I have to do that. He asks, begs me to open the door and I say a prayer and I tell him again that I can’t do it. He sounds like he is crying and he tells me that he wants to be with me.
I wait for a sign, it doesn’t come and so I open the door.
He holds me while I tell him I can’t give up my child, he says he knows and that’s something he is willing to accept. Stupid bastard. In hindsight he really should have broken it off with me and then he would be free from this. He is too clever and although he says there is no such thing as being IN love, it feels like if there was such a thing he definitely feels it for me.
Idiot. I wouldn’t choose me.

And that was that, there it was, the moment we joined, no cake, or bridesmaids, no photographer just a moment when we became us. Since then we have been close to taking it all back and then we don’t. Love is a funny thing.

We talked today about adoption, again and we are young to adopt. I know we will be great parents but this is fucking scary, I haven’t spoken to anyone with any adoption experience who say, oh yes do it, the best thing that ever happened to us. Adoption where both of you have no genetic connection is hard. Children are hard work. I know I’m a teacher. Some of my pupils are wonderful, I love them already but some wind me up.
I love my niece, right now she is 8 but she can be a pain in the ass but I love her, can I love a child that I don’t know? Can you fall in love with a child, should you? ARRRRRRGHHHH

We could be great parents.
50/50

The tick list is numbing. You get a tick list, it’s numbing. How do you know if you can handle any of these things? Disabled, stupid, mentally disabled, HIV positive, handicapped, potential killer, parents were drug addicts, under 11, over 5. If it is of your own womb it is your own fault, if it is from someone else’s it isn’t. You aren’t thinking about a human, your thinking value for money, traits, nature verse nurture. I say all this and I don’t know because I’ve never been a mother and at this rate I never will be.

Adoption is a difficult choice, it is not an easy alternative, no wonder so many 40 something potentials go for I.V.F and that emotional angst or if they are really rich they buy a ‘foreign ‘baby. We are not even 30. ‘Young’ like the woman said.

I want to give love and help a child, or children, be wonderful people, I want them to play music, like art and enjoy my company. I want to give advice, watch them grow and help them fulfil their dreams. I want to be proud of them. Is this too much about what I want? Really, if you think about it having children is the most selfish thing to do.

I’m a teacher. I want. I’m scared.

I’m not a mother. Should I be?

It is ten to ten

19th February 2006

I’ve been on holiday (half term) and I’m feeling refreshed. Being at home brings back memories of my brother. He would have been 32 yesterday.

This time of year is hard, it is made difficult this year because of waiting for test results. Been here recently, and before.

Dad is almost definitely going to have to have another operation or two. I love my dad, he is gentle and kind, he doesn’t make this disease a big deal.

It is.

On TV is ‘Honey We’re killing the kids.’ The irony isn’t lost on me. 
I was looking at adoption information today on the internet. It didn’t make it any clearer; the fact that adoption is complicated and fraught with pain and angst doesn’t make me feel any better about it. The fact that we are only likely to be able to have a child with previous history of serious trauma makes me wonder if VHL is really that bad. Oh bollocks. 
Please just let me get pregnant by accident and it not be a choice, just let it be.

50/50

It's a 50% chance of passing on my disease to any child I/we have

Would you take the chance?

A history lesson

It occurred to me that a context may be needed and although I don’t intend to put my name on here if you do know me well then you’ll figure out who I am through details. What’s happened to me over the past 27 years is rare.

Here goes

My genetic Gran (on my dad’s side) died of a brain tumour 50 odd years ago, when they did an autopsy they found an adrenal gland tumour too.

As a young boy my dad kept fainting and they took him in and cut him open and found an adrenal gland tumour the size of a grapefruit. (no beta blockers or anything)

After I was born they found he had a brain tumour, which was removed. Then some clever Dr asked some questions and suggested that he might have Von Hippel Lindau disease. The clever Dr was right.

My parents had had 3 children. Dad got more tumours!

Ten or so years later they did a high risk/low risk test for the children. My brother and I were high risk.

My brother and I and my dad were screened annually for various tumours.

In 1996 they discovered a tumour on one of my brothers adrenal glands. They removed it. The operation was successful but then something else (they don’t know what) went wrong. His brain swelled and crushed inside his skull. He was brain dead. He was kept ‘alive’ for three days then they turned off the machines and he died.

The following year they found a tumour on one of my adrenal glands. It was removed. I didn’t die.

In 1999 I had my second adrenal gland removed

In 2000 I had a brain tumour removed

In 2001 I was told I had a tumour on my optic nerve

In 2003 (I think) they found that I had a crinkling of the maclia (I don’t think I’ve spelt that right)

In 2005 I was told I had a small tumour on my spine, later I was told there were 2.

I’d tell you all of my dad’s tumours but it’ll take too long.

Positivity

For the record I am a positive person and I feel that on balance I am incredibly blessed, I have so many wonderful things in my life, I am, in theory, a very healthy person and I live a full active and enjoyable life.
I like my job and I have a great family and superb friends, and I don’t use that word lightly. I just feel like I want to reflect on the part of my life that isn’t great so overall this blog may seem down or depressing. I can assure you that for the vast majority of my life I’m neither down or depressed. When I am I’m most likely going to post it here. Please bare that in mind if you are a regular reader (do you see my optimism – I expect regular readers!)

Diary of a Genetic defect

The story so far

I’m creating this blog because when I was sitting next to my dad a few months ago now in St Barts Hospital, he was having some blood taken, a slightly batty woman, who was a nurse I believe, saw me laughing at my dad who had to lie down and he was getting grumpy about it as he ‘needed a pee’ as he put it. The woman seemed cross that I should be laughing at my dad’s misfortune and commented on the fact that I wouldn't like it if it happened to me. To which my dad simply said. ‘She’s got it too.’ We looked at each other and smiled, he rolled his eyes and the batty nurse launched into a list of questions. She asked if I belonged to any help groups, which I don’t but I did say that I’m on the email list of the VHL alliance which is based in America.

‘Oh,’ she said, ‘you should write about it, it would help so many people.’ Now she didn’t know me at all, all she knew was I had a genetic disease and I was over 20. But it made me think, maybe I should. A long time has passed and I’m going to write about it.

The disease I have is rare so there won’t be many people who have it or who want to read about my experience of it but maybe other people who have genetic diseases or are friends or family if those who do might find it interesting. Even if you don’t I find it calming to write about it and have done in diary format for sometime.

I’ll start from now but I might have to fill you in on the past from time to time.

I am female, I am currently 27, I live in north London, I am a full time teacher. I have a mortgage with my long time partner.

The disease is Von Hippel Lindau Disease or VHL if you are in the know.

http://www.vhl.org - if you want to know more.