An account of my thoughts and feelings about having a genetic disease. Von Hippel Lindau disease, VHL. Not necessarily factual but real all the same.
There is a clear theme when you have a disease like VHL... waiting. Waiting for appointments, waiting for results, waiting rooms, waiting games.
I think most of us learn to forget the wait at times, it sits quietly in the back of our mind and surfaces most when someone else, who is waiting too, asks "do you know when..." or "have you heard?"
This week, an appointment I'd been waiting for arrived quickly and happily when I could go easily without a lot of rearranging and adjustment. And at the end of it I have a better idea of how long I need to wait for the next bit. I don't know exactly of course, that would be too easy, that would be too convenient. And so I give my news;
mid-December.
So I'll wait to find out the exact date and then I can plan more. It doesn't just impact me, it means my work, my family and my friends. It means cancelling things, probably, adapting things, likely and a period of recovery, definitely.
and then, of course, because it is VHL
wait for the next one.
8 years since that day that I didn't know would be a long month
you never know what's round the corner
What does soon mean then?
In Malawi if you say now, it's an unknown amount of time, if you say now now, it's now.
In the NHS when you say soon, it could be anything from a week to a month to more. An endless amount of obviously not now but when, running around my head.
I'd quite like a before or after Christmas answer.
My first brain tumour I was given the choice of before or after Christmas and I chose after so I had the operation in January 2000. As I sat on the sofa with my sister on the 31st of December 1999 we didn't feel like that was really the right choice. Party over, oops out of time. That's how it felt. It felt frightening. It felt unknown and it felt far away.
It always will when my first real experience of VHL was my brother's routine operation not going to plan.
This robot better be good.
Which one should I go for next?
so far in my various communications I have given the news in a range of ways
A cyst that is now more complex
A little bit of cancer
The tumour has grown
Good news - all basically the same - but I need a procedure
Luckily a robot will do it
Not as bad as last time
Will be able to get back to work faster
Cancer - they are getting rid of just the cyst, not the whole kidney
You can live off 10% of one kidney as long as that bit is working
Nope - I don't need yours, yet.
I'm thinking of - I've had kidney cancer for a few years now, the good kind but now it's looking like it might turn bad and they want to get it out, Please still give me a job.
and while this is my reality, while I'm taking this in I saw the scan and the size of the one in my spine. Ooof he's grown. It's a he. I might name him. He looked like a lozenge. Lonnie. You can stop showing off now Lonnie. You just stay right as you are. I wasn't ignoring you before it's just that your sister was doing such a good job of growing but quite as much as she could have. She's complicated your sister. She's moving out soon and I'll be all yours.
There is an oddity in all this.
"it's time for it to come out" and I had been anticipating it. I can track the growth and knew it would be within the next couple of years, but I had hoped for a bit more time.
But getting it out - something I wanted so much for the last one. I wanted to wait, I wanted to feel settled. I wanted to know I had a proper job. And I still don't know. I am now waiting to see the surgeon.
Strange too, to cry about getting rid of cancer. After this, it will be gone. After this I start that clock again. Because it doesn't end.
How you give the news matters, I have given my usual optimistic slant.
6 days to go. 6 days to wait and 6 to be uncertain. Although as you know well by now, it is a short lived sense of certainty.
The lack of stress of the last few years has meant that I dealt with it all so much better. I'm sure the sun helps too. I've been looking after myself in some important ways, I have cut down on my daily drinking massively and I sleep so much better for it. I have begun to take vitamin D supplements because I am not getting my daily dose of sun here. I'm trying to do weights a bit, I don't do a lot or often but I am doing some. I am thinking about how to get more fitness in. I eat well. I eat a varied and mostly vegetarian diet. I have never smoked. And despite this my body will do what it wants anyway. I'm aware these measures are, for me, much more about how to make my body recovery ready.
The news about a young girl having a successful stem cell transplant and kidney transplant made me smile. Not that I suspect that would be me one day, but more that we - the human race - keeps finding ways to make life last and last well.
I am expecting either, all stable let's scan in 6 months or, it's about time we thought about taking out those kidney tumours. I'm expecting a potential cerebellum tumour. I feel wobblier than I have before. I'm not expecting anything more than that. Ha - that's a VHLer - the good news of nothing is killing you right now. Come back in 2024 and we'll see how that year will be.
I am trying not to feel the stress of not having a job and the fear of unpaid sick leave. Can I sign on when I'm off for surgery?
I'm letting myself enjoy the happy moments in life, singing in the kitchen with my girl, eating great food and treating myself to new shoes. I think tomorrow I'll do a bit in the garden. I will possibly buy more shoes. You can't have too many.
I've got my clinic appointment through, at the end of September. I spent a few hungover moments this morning having a small panic about that and figuring out if I should or shouldn't tell any potential employees about my (currently fictional) operation.
I know that it would be classes as a protected characteristic so they can't not employ me because of it but if I tell them then they might and if I don't they might be really annoyed when I then tell them.
Remember that I don't even know if I do need surgery - I'm just having a planning ahead worry session.
I am being reunited with people who have kept me strong through hard times, I am enjoying the connection and closeness. I am also 'excited' to be reunited with my MRI machine tomorrow. I will get to lie in her cosy space and listen to her melody of bangs and clicks and I will then, in September know where we are. I will maybe get a sense of what the year ahead will really be about
I've been toying with some ideas around taking a year out, of spending it not doing very much at all. And I would simply put in the gap on my CV 'cancer recovery' would it put some off, no doubt but others - maybe would bump me up their pile. Maybe. This is just a small yet recurring idea. I still can't let go of the idea of going back to work, not least because we are not wealthy enough for me not to earn, my husband won't be getting a very well paid job any time soon. I can't imagine him suddenly needing or wanting to earn more. Would those who saw the gap think that I'm fully cured, all done, a once in a life time awful experience? Because I think a lot of people see cancer as a one off thing, one that can come back but often I've had cancer. Maybe not these days, maybe people know it isn't so simple. And that's partly because there are more people surviving the first one, maybe the second one too.
And here I am just assuming that I'm only worrying about my kidneys - and I am. But of course I know another little bit of me could be slowly fucking up, could have a new brain, spine, pancreas, spleen, liver and maybe even an ectopic one somewhere. Could.
I've been conscious too of the age of my body, hearing a pal support another woman, 50, who has just had surgery, the recovery is just harder the older you are. You don't bounce back in quite the same way, 6 weeks is not the whole story.
When we got back, less than 5 hours ago there were flowers and 2 letters in my familiar yet unfamiliar kitchen.
One was for my daughter and one was for me, And even though I'd asked my mum to send it I was still a little taken aback when I realised it was my scan letters.
I'm tired and grumpy and it's muggy. Thank god I could have a cup of tea.
This evening I set a fire and opened a bottle of wine that my yr 12 class bought me as part of a leaving gift. I danced in the front room and looked around at all I'm leaving and I felt a soft contentment.
Who knows what the next chapter will hold, I only know it will have ups and downs.
The comfort of going home and the people I will see and love is making these last few days so much easier. I have my worries, I have my mild anxiety but they are not overpowering.
Soon we go, go home and we'll find out about that when we get there.
I feel and affinity with a complete stranger, and have been happy to see her comment on my posts. She looks to be my age, think she has kidney issues too. She has a face not dissimilar to mine and hair of a equally enhanced shade of blonde. But more of course she speaks in a way I can connect to. Someone who gets it. Someone I hope I can support and share this all with.
Don't even know her name
I have been on a hen do, with some lovely women, each of us have been through trials and tribulations. None of us are coming out of this unscathed. As we get older the more events happen that shape us and more define moments too.
We talked a lot, us women, about the way marriage is a task. It's often hard work. I feel a little for the hen, but she took our wisdom in good grace, saw that each of us married ones have been through the ups and downs and she knows. She is not daft. There is not fairy-tale ending.
My husband has helped me with what I know is a life long affliction of being a worrier. As I woke next to a woman I have never spent an evening with let alone a bed with we talked about how we slept. Badly, of course, different bed and too much alcohol. I told her about my habit of worrying and it's mostly a voice that hits me hardest in the small hours of the morning.
As I explained this, the ways I deal with it, the self doubt, the nastiness my voice can be and how I have tamed her, I was able to see three things and slightly articulated them.
1. I've always done it as long as I can remember, my parents would try to help me, a worry book by my bed to write them in, the letters I would leave on their pillows so I could get it out of my head.
2. It makes sense to be a worrier when you know from before you even know it that your dad has a disease that gives him brain tumours (I was less aware of the others) and that you and your brother probably have that too. Having annual check ups, clinic appointments and then the scans, annual results. They did a good job of underplaying it, of making a family joke of the massive bottles of piss we collected for 24 hours once a year in our bathroom. The Dr that once checked if I had pubic hair by simply pulling my knickers down a bit while I lay on a bed. The change to a big hospital, Addenbrookes when a specialist was found. The eye scans of old, the yellow glow of skin and piss. The journey back through East Anglia, back to the coast with the words ringing in your head, angioma, pheochromocytoma, hemangioblastoma. Surgery, monitor, growth, tumour, cyst, partial nephrectomy and never really knowing the difference between them all until you then do.
3. My husband has helped me worry less, talking it through, refocusing, allowing me to fear the worst and then concentrate on the likely and the hopeful.
and as I reflect on that now I see a 4th.
4. I am a warrior
I had a fascinating chat with a colleague, we discussed all things kidney.
I will be pleased when I get back into the scan flow - I am pushing my luck.
But I'm glad I have, it was the right thing to do
My pal's brother has had to have emergency surgery - hemangioblastoma in his cerebellum. They have suggested he gets tested for VHL.
Small genetic world.
His father is unknown - he is probably the carrier- too long a go to have been my brother. My pal's half brother.
My brother was always told he couldn't be reckless.
He never was, we think.
Imagine if he was, a niece or nephew.
I didn't even know she existed until my brother stayed with her and my mum's cousin Tim. But I didn't register that, a couple of names of relative who lived in London.
Then as a 21 year old I was suddenly in need of a place to stay and not just for a night but for months and she and Tim said yes without any reservation. And how she looked after me, how we talked, Ros was such a generous person, with her care and love and often her clothes too, she was forever trying to give me items she had decided she wouldn't wear again.
She cooked the most fabulous and flavourful lamb shank I have ever eaten and her roast dinners were divine. I ate so much while living with them, I ended up the heaviest and plumpest I have ever been in my life and I include my time being pregnant in that calculation. I couldn't resist that early evening G&T and the expensive and quality wine while we ate.
If you haven't trained to be a teacher you won't fully appreciate how hard that first term is and She and Tim looked after me and made it a much easier experience. We stayed in touch over the 20 plus years that followed, they coming to us and being there not long after my little girl was born. We caught up whenever we could, at least once a year until COVID stopped so much of that and I so hoped that in July we would take the trip out to St Albans to see her and make each other laugh, I can hear it now, that slightly dirty chuckle of hers and how she would almost wink at me when my husband said something she thought was dirtier that it was!
I can leave Malawi content as I have harvested some passion fruit.
This little moment matters to me, not leaving something undone.
I don't really know why but it does.
Leaving behind a legacy of fresh, passion for others to enjoy.
I perhaps want that on my grave stone.
I am increasingly relying on fate and not sensible planning. I have applied for a maternity cover - the Headteacher was very nice but I got the feeling I'm not what he's looking for. I might be wrong. I've seen another role I think I'd love, but then I feel that clawing doubt that I won't get it, been out of it too long. The news schools might be shut here for longer so frustrating and unnecessary for us and other schools too. And with the devastation around me, the giving of stuff to those who now have nothing, and those who had almost nothing anyway it just doesn't feel important.
And yet it is and it is my life and I want to be happy.
With this sense I'm aware as well that I'm taking a gamble with my scan dates. I'm willing it all to be ok and waiting for July and not thinking about it, but of course that's a lie because I am thinking about it, often and I push it aside. My husband hugged me yesterday, he held me tight as he cried because he had seen a woman and a small child sat with almost nothing, a small cloth with what were probably all their belongings. He went to them and gave them the money he had in his pocket and some food he had bought for other people. As he held me I thought about my kidney! Imagine that, I thought about what is inside me and if it is growing and if it might kill me. The two things at the same time. Such is my privilege.
Today he has gone off to help again. We have a 4x4 and he's using it to help. I'm here, offering people our shower - people who are used to hot showers when they want them. Not much is it. I'm not sure how I feel. But I know I want to be happy.
I am home, in the Warm Heart of Africa. It certainly felt odd. My heart is split and that might never change.
I understand why I'm feeling this way, I don't feel like I am in either home properly, it's a strange transition and one that I don't want to waste on feeling befuddled and feeling sad about. This morning, having grabbed a grapefruit from my tree and had it for breakfast, reminded me to love each moment I can and take it in.
I've seen people who are leaving find fault and tell themselves it will be so much better somewhere else. It is different but not better. Some things are lovely to leave behind and some things are not.
I was struck by the huge difference in my friendships between the two places are very different. It was lovely to see everyone here but really only one person has a piece of my heart. But in the UK I have a bundle of people with whom my heart has grown.
My heart is my little family - my little girl who is so tall. And so I'm here with her, my heart.
The last few days I've seen posts from people who have not had the miracle cure from the drug that they and the rest of us were expecting. Tumours re-growing once the medication stopped, having to still have the kidney removed despite the months of side effects.
It's making me feel a bit sad and I begin to spiral into the unknown future.
I need to focus on something else
When the fates allow it I will look back and use these last few weeks as a lesson in keeping on. I feel so utterly supported by so many people in my life and I'm sure if it was based on that alone I would have smashed it by now.
My husband doesn't take things personally which I admire and try to emulate. He has a good philosophy on this. I wasn't the only one disappointed - only one person was happy.
So now to revision on the next thing...
Maybe this is the one
I've been very tearful today and finally had a good cry. Simply put, although I know it's the right thing to do for so many reasons, I don't really want to be doing this. It isn't helping that I miss my family loads and that they are in the sun and I'm in a jumper.
I was just, essentially, coached by my clever and logical husband and decided to put my energy into the job I want more. I have no idea if that's the right choice. If I could I would fly back now and just accept I don't have a job. I want so much to hold my baby girl and be held by my man.
It's hard to maintain the joy of London when I want to be sharing it with them.
Tonight I want to sleep well and prepare and feel as ready as I can.
I want to be able to do my best.
I want to be appreciated for all I can offer.
I want to want the job.
I wish I was better at finding out what I want.
Humph
I need more than I brought with me and it's very annoying. In Malawi I can just go and buy it. I don't think this is necessary, in the grand scheme of things, a good thing, but my goodness I wish I could go and get some that way now.
I've considered halving my dose so it will just about last. That, of course is not a good idea.
Humph
There are moments in your life that hit harder than others and then, like any other kind of grief, it tends to fade or at least hits less often.
This week I again talked to a A-level biology class about my life with VHL. The starter for this lesson is essentially a similar genetic profile to me and the students are looking at the ethics of genetics. I've talked about it before, but the teacher and I have this routine down now. We are a great team. I'm also surprised that 4 years on and several versions of this lesson and they are still always so surprised when I reveal I have VHL. I'm the person they advised that couple not to try for. the disease sounds so awful. And that the girl they know, my daughter was a surprise and 'happiest day of my life wasn't when she was born but when we found out she didn't have VHL'
Classes obviously don't really talk about it out of the room. But I know from those students who I talked to later on it had a significant impact. One boy telling his mum (who then told me) he's going to go into genetic to find a cure.
And thanks to a scan they also get to look inside my body. They have hard copies of my abdominal MRI.
I am a teaching tool.
Why moments hit hard is that of late I've been thinking about that nasty brain tumour, it hit hard. It was the worst and I stood in front of them realising I am past the main fear. I'm coping well and I am more optimistic. Yes today when I had a sharp pain in my head I suddenly let myself quietly freak out. I was doing a poo. My dad was diagnosed with one of his brain tumours because he reported pain when did did a poo. And just for a moment I drifted back to the room and the weeks of fear and utter hideous dizziness.
someone I know and respect has begun to read this blog. Exciting and a little unnerving. In response to his comment via email I thought, well I should read those ones again as, understandably I can't remember what I wrote. And I kept reading, enjoying the memories and reflecting on them and then I see one about my dad and his wobbliness.
At the time I had simply written that he probably had another 15 years in him. I was kinda spot on. 15 years. good guess hey.
My little girl is 15 and a half. How many years do I predict for myself? Not my life expectancy. How many does she need? I know that we are making this move, partly to give her a step towards better independence. This week she asked if I would sleep in her bed with her. She didn't want to be alone. I obliged. I know what it means to be able to be there for her, emotionally and physically.
we have a couple of secret messages we share, I of course won't say what they are but they are proof that we are who we are. No alien invasion. I have the same with my mum. we used it often. She would ask me them when I awoke from an operation. Imagine that, my mum checking I hadn't been replaced by a science experiment.
I know that's not what that was. I have passed this onto my one. I'll be me always, we have our code. if there is an after life and I get shunted back to her I will be able to prove I'm me. A bit like 'ditto' in the film Ghost. It is ours.
nope - not telling