new mum

I'm finding it hard to find time to do this. My mind isn't on VHL very much. Dad goes back into hospital next week for further tests. I guess that's why I'm on here again.
I'm very proud.
I'm very tired. 

She's here

She has arrived. Last week a perfect day in July at 2:45 pm our time.
7lb 4oz and she is wonderful I'm so happy.

I had an interesting stay in hospital.

I HATE IT

I hate it, I hate it I hate it. It creeps in and nibbles away at the perfectly good life you live. Why the rant well VHL does it’s bloody trick again.
I had an appointment with my prof and all was well. All of us in good spirits and off we went happy as the day was long. Then at about 8pm I suddenly experienced a strange change in my sight, a swirling kaleidoscope circle in my left field of vision. I carried on hoovering, naturally but when I’d finished I went into the kitchen to my partner who was finishing making dinner and I told him.

Nothing to say; perhaps a lead into a migraine. But I’ve never had one it just sounded like it might be. As I was eating my left hand filled with pins and needles. I sat up as instructed by my partner who said I may be leaning on something. My nose started to tingle too; like it does when I’ve had injections to numb it. It was now about 9pm and I was worried. I went up to the toilet and when I came back down the left side of my tongue was also tingling. We decided to go to A&E.

Now if it was just me I probably would have sat it out and stayed at home and seen what I felt like in the morning but it isn’t just me anymore. 37 weeks of a daughter is inside and we felt we should see a doctor. If I was ‘normal’ then I may well have gone to A&E because of the combination of symptoms and I’m not normal. And as all the symptoms faded I felt like such a fraud.

Even as I was taken very seriously and the baby was fine and the trace of her heart showed her to be completely unaffected and as they asked if I would agree to staying in over night and as I said no and as I said I’ll phone my prof in the morning and as the Doctor there did the neurological tests and said that it did sound like the symptoms of a stroke; which she said in such a blasé fashion I wondered if people had them all the time and even as my partners normally ever so calm face remained slightly sullen I did, I felt like I was a fraud and was wasting everyone’s time.

So now I’m sitting on my third day of maternity leave full of anxiety and typing away here to off load. Pissed off that the VHL monster has managed to take my calm and take my security. Again.

Vulnerable

He looked so vulnerable. He was at my gate, stick in had with a list to the right; his hat on his head and mum dashing around in the background sorting the dogs and the car out. He smiled and wobbled on in. Later that day I gave them both a lift to the tube as dad had his first check up since the op. The surgeon is very pleased with him. As we pulled up in a line of traffic I said they would be better off getting out there (nowhere to park) so mum got out. Dad tried, he tried to do the window, get his bag and stick out and open the door. All foiled by the seatbelt left around him still connected to the car. ‘Don’t worry about the window dad just get out’ I felt bad. He almost fell out of the car and a kind man steadied him as he tumbled towards the pavement. Mum looked flustered and cross; a common face these days. He seemed so disabled and I couldn’t stand it.
Since then I’ve seen many people who use sticks and wheelchairs and it seems to me that the problem with my dad is that he refuses to accept he has limitations so he’s not able to deal with them. He still insists on speaking at 90miles an hour and so it’s very hard to understand him and he doesn’t use his stick properly. E.g. as he gained his balance to walk towards the tube I saw him lift it up to point at something!
And then there is the usual fear; will that be me. Will it be her?
But then they came back from then check up and there was all the positive things. The gamma knife will work on another tumour that is looking dodgy and the ones on his spine are doable too. But the best news is they are waiting for a new, faby daby doesy thing that is coming to Britain soon that is better than a gamma knife that will get em. Not the most technical way of describing it but I wasn’t there and so I didn’t get to make my notes. But it sounds good doesn’t it?
I’m on maternity leave now. Two weeks on Wednesday for the c-section.
I will do pictures. Take care everyone

Mum to be

The best thing about having this blog is making contact with other people, well ‘Steven’s Journey’ really. But it makes me feel less isolated. In about 6 weeks my baby is going to actually make her presence know and I’ll see her. So today when I read the other blog I visit most often I thought about a day when I may have to have a conversation with an 18 year old. It may not happen; well at least she might not have VHL and I pray she doesn’t but we may talk about me or granddad. My niece and my dad sat on my sofa and he explained what the operation was for. She took in her nine year old stride. So will my little one.

It's all hospitals

It was a hectic week but dad is home now. Although he seems a little depressed; I think he expected to be better than he is by now. His balance seems to be better than before the operation but is speech is effected more now. If he slows down he's much easier to understand and face to face I get almost everything he is saying. It's a different story when it come to over the phone though. But things are getting better everyday and I'm so relieved; a bit emotional as a result.

I've had an MRI to make sure my lumbar region of the spine is tumour free so that I can meet with the anaesthetist for my C section and hopefully be awake and not have to have a general anaesthetic. As a friend of mine put it yesterday, it's all about hospitals and tests at the moment.

Not long to go now

In and Out

Well not quite out yet but my dad has been in hospital and the tumour is out; all of it. He is sitting up this morning, has had breakfast and can speak, move fingers and toes and seems to be well on the road to recovery.
The road to recovery
we don't hurt until they fix us

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.

Email to people

Prayers and positive thoughts needed.

Hey hey

Some not so good news today. Dad has got a letter, it looks like it is a brain tumour in his cerebellum that is causing the balance problems. We thought as much. But that’s where you lot some in; go on get praying and thinking etc. We don’t know the plan of action yet; he’ll get an appointment soon and I’ll of course keep you posted.

My sister is on holiday at the moment and so she doesn’t know. So those of you who might have a slight chance of letting slip before dad gets to tell her shhhhhhhhhh. Very unlikely for most of you.

Sorry to say it via the wwb but you know me!

Take care ya’ll

Today I feel sad

My dad said ‘hello sweetheart, where are you?’

‘You’ve got some results haven’t you?’

‘Yes, are you with anyone?’

‘Yes.’

‘Right.’ He said in his matter a fact voice that I have come to know so well in these situations. I always know when it isn’t going to be great news, he always approaches it in this way. I love him so much.

The letter said that the tumour in his cerebellum has grown and is the most likely cause of the balance problem. He is to go and see the Neurologist on a Friday. It said A Friday, not a specific one. Odd. I cried quietly while he pretended to be fine about it all and mentioned that f they could solve the problem then he should be able to ride a bike again. We pretended to each other that we where fine and positive about it. We talked about mum and the fact that she has only just done a solid poo since she came back from Egypt. We laughed and we said we loved each other and I put the phone down and sobbed. I had a little bit of hope in me that it was going to be a medication problem. 

He can’t die before his granddaughter arrives and he must be able to hold her and hold me too.

I phoned him and mum this morning, they had been worrying all the night before and had somehow convinced themselves that the tumour the letter was referring to was the one on his brain stem. I hope I managed to convince them it isn’t; it’s one that was already in the cerebellum. They are different places in the brain; it would have made it clear that it was on the stem I’m sure. 

If it is that one then, well he’s fucked! Excuse my language but there it is. I feel sad and as I sat on the toilet and my baby girl kicked in my womb I said ‘sorry’ out loud. Sorry I’ve done this to her. Yes she wasn’t planned but who am I kidding we weren’t careful. I wanted to be a parent so much that I’ve been selfish. Please god don’t let her have it, please god don’t let her have to feel this pain. But she will won’t she. No matter what. I’m probably going to be phoning her and saying… ‘are you with anyone? Now sit down…’ And it won’t be one day it will more than likely be a few times.

How does he do it? He mustn’t die yet, he mustn’t be harmed again, he mustn’t be a memory I have to tell. We already have to do that with my brother for my nieces. Then I think of friends my age who don’t have one of their parents… for 28year of age I’m lucky and not so lucky. It could happen but I hope and pray it isn’t yet.

Today I feel sad.

Week 22

All is well as far as the baby is concerned. She has started to move about and I can feel her, and yes it is a She. I'm really enjoying being pregnant. I didn't let myself entertain the idea that I might be a mum that we could be parents and we are 22 weeks on our way to it coming true. It does feel like I’ve been pregnant for years not months but I’m not bored of it, I might get bored and I’ve been getting backache at the end of a day but I still love it. My baby.

On other news my dad hasn’t had any results as such but I always feel more hopeful when it takes a long time. It usually means that things aren’t a problem. But then they are, his balance isn’t any better and he keeps falling now. Injury list so far, finger and knee.

And my VHL health, well as you lot won’t tell; I’ve been getting some fluttery feelings around the left shoulder blade. It’s internal but just under the skin, a bit like pins and needles but not unpleasant. So I’m wondering if it’s got anything to do with the tumours on my spine. I mentioned it to my dad and he said it sounds like the sort of feeling he gets in his legs now the pain has been sorted through the medication. I’m not going to say anything to anyone else just yet. Even if it is the tumours there isn’t a problem, it’s not like they could operate while I’m pregnant. So apart from getting it out there in the open with you I’m not going to worry about it. Well not really.

Breast Feeding

As some of you avid readers may know, as I don’t have any adrenal glands I have to take steroids three times a day as replacement therapy (most of you produce it quite naturally). I, we were concerned that I may not be able to breast feed but hooray I’m allowed. I reserve the right to change my mind about being pleased when I give it a go and I have to put up with leaking boobs and feeling like a food factory.

Prof C also made it clear that he is being very cautious when insisting on a C-section for the birth but he doesn’t want any of my hemangeoblastomas bleeding under the pressure of pushing because the one woman he knew who that did happen to died. She had a much worse set of them than me and had had all sorts done to them like radiotherapy but I’m with him on the better be safe than sorry side of life.

So I’m enjoying being pregnant right now, I love the attention, at 19 weeks my bump is starting to look like a baby bump and not just a ‘who ate all the pies’ bump. I still want to tell everyone I meet but I’m not being that mad.

We saw the geneticist too and as testing/screening for tumours would start at 5years he suggested we wait till then to find out the child’s VHL status. I said I’d rather know as soon as possible because if I know it can be a natural part of who he/she is and if not wonderful. I’d just worry for 5 years if I didn’t know and my brilliant, better than I ever expected to get, partner agrees.

Something else that struck me was prof C’s comment that if our baby did have VHL he expected we would be devastated, you know I don’t think I will be, sad, yes, but I feel like - who better to be around if they do. My parents have never made me feel devastated about it, it’s part of me and it has made me who I am. And I may be repeating myself but what ever happens this child will have to cope with VHL because it’s mum and granddad will have it.

Really feeling upbeat today, I’ve got a smile on my face and it is there quite naturally.

So here’s hoping the next half of the pregnancy goes as well and then the rest of the little bundles life too.

e-mail about dad

hi all

just to let you know the latest, dad had an appointment with prof C tonight re balance and general wobbliness. The good news is prof C says that it should be something that can be solved. The two most likely reasons are either a tumour in the cerebellum or a problem with medication. To find out he is going to have a MIR scan and has had some blood taken. We are all pleased that he didn't say 'well there you go, bad luck old chap looks like you'll need two sticks or a wheelchair.' or words to that effect. I’ll keep you all posted of course. Thanks for positive thoughts prayers etc.
love

wobbly father

My parents came this weekend, it was great to see them. Each time my dad seems more wobbly. The radiotherapy took its toll and there isn't anything anyone can do. We talked about him getting a cane. My sister gets sad, she lives about 10mins from them and she gets to spend a lot more time with them. She said it makes her so sad when she is out with him and strangers assume he is drunk because of the way he weaves around.
As a family we laugh a bit at it now, dad does, making 'oops' and 'whoa, ooh' sounds when he's toppling towards the wall, door, floor, dog, grandchild...
At a New Years meal my mum screamed his name as he stumbled backwards from his chair.
'That'll help' I laughed as I saw my mum realise how angry she was getting at him for something he has no control over. It must be so much harder for her. I know they talk about when he may end up in a wheelchair and how the house would have to change. I don't want to think about that.

I'm not married but I want to be and my partner and I will one day but it occurred to me that I want my dad to walk me down the aisle, not me drag him down trying to avoid small children that he may go splat on. But then these are the moments that make the day, the funny stories you remember. I just pray that please god by the time we get round to it he is still with us. I'm so grateful that if all goes well he will meet his grandchild.

I reckon he's got a good 15 years in him yet. I hope and pray I'm right.

Another Day Curve

Not much to report, feeling healthy but tired. Just making sure I keep you posted, the loyal few that is.
Everything is going according to plan.

a few days at hospital

Just to say that next week I'm in twice for baby things. I'll let you know how it goes.

Day Curve

I had to get up very early, it should have been my first day back at school but I had a Day Curve. That’s when you have your blood taken through the day to see how much cortisone you have in your system. I was due for one anyway but we need to see if my baby is taking it and leaving me with too little. I haven’t had one done at Barts before but they are very thorough. As I was laying on the bed, (something to do with blood pressure I think) another person came in and ear-wigging I realised that he was having a day curve too. Now I’m having it because I don’t have any adrenal glands, but some women can have conditions where their adrenal glands don’t work, like Addisons. Anyway. He was a he so I started to wonder. We always used to wonder, my family and I, if people in waiting rooms had VHL but we never asked. My mum never wanted me to join one of those support groups because she didn’t want me to fall in love with someone with VHL and have a real problem having babies. I think that she also worried that we would hear horror stories or that our problems would upset others. Back to the day curve. So you have blood taken then you’re free for a period of time, sometimes just 30mins sometimes 2hours. There is a day room to go to and I took a good book and some school work and a deck of cards, hopeful that my partner would leave work early and come and entertain me. (He did) So after my third lot of blood I went into the day room and there was the other day curver. We exchanged hellos and then my curiosity took over. ‘You having a cortisone day curve?’ ‘Yes, you?’ ‘Yes.’ Pause. ‘Are you under Prof C?’ ‘Yep.’ ‘Me too.’ Then the moment of truth. ‘Have you got VHL?’ ‘Yes.’ ‘Me too, rare bunch aren’t we?’ And we got to talking. It was good. By the end of the day I felt a bit like I knew loads about him. We talked about our operations, our families, the losses the way we found out. We discussed our levels, I told him I was pregnant and he seemed really pleased, which was lovely because as odd as it sounds I thought he might say, that’s stupid why have you done that don’t you know the risks, but he didn’t. In fact when my partner turned up we talked about our knowledge of thinking about having a baby as a couple and he seemed to find this, well comforting, maybe.
There was a connection, almost like a distant family member. It’s good to meet others, to know we exist and we are strong and alive. It’s sad to hear of those who have died but I was proud to say my dad is on his way to 60 and still working. It gives hope. Too British to really make a leap into the unknown and offer a phone number, so I left a little card with my email address on it on his bag when he was having his penultimate blood done as we went home. Who knows if he will get in touch, I’ll let you know.

Eye Eye

I had my annual review for my eyes at the end of December. It was a warm day for December. My partner came with me even though I could have gone alone. I really like it when he comes because feel, well supported. Not to say that he isn’t wonderful and the number of appointments I have he couldn’t make all of them without using all his annual leave. So I saw a Miss and told them I was pregnant so they only gave me one eye drop. I had my eye, the one with the tumour on the optic nerve photographed and then scanned because of the maclia issue. I asked if I could talk to the main man. Mr Webster. I’m happy to name him because I think he is brilliant, they all are there actually. As my partner pointed out, they speak to you with respect and don’t patronise. They use medical language and make it clear. So the crunch, when Mr Webster spoke to me he said the tumour had grown. It’s been the same size for years now, but u have noticed a slight change in my vision in that eye so I wasn’t totally surprised. But he did say that there is anecdotal evidence that pregnancy can make tumours grow. So previously id been told the only treatment is laser and that would damage my sight because of the position of the tumour. The good news is that in America they had been working on a medication that will sort the tumour out and make it go away. ‘Is that oral medication?’ I asked, really what was I thinking?
‘No, that will be an injection in the eye.’ Right! I didn’t ask anymore, I can’t have it till after the baby is born so I’m happy to wait until nearer the time to ask if I’m awake when they do it, I bet you have to be.

Happy New Year by the way.