A letter to myself

Dear Vicky on 23rd March 2006,

You won’t believe it, but tonight you are going to meet your husband and your flirtation with internet dating as a way to entertain yourself and broaden your social circle will get you more than you bargained for. When you meet Will, he’s a little shorter than you expect. You'll note his not-entirely-blemish-free skin, his poo-brown t-shirt, a slight husk to his voice, a bit of a cough. He’s had a cold, he says. He apologises for yawning and you’ll decided not to be offended. When it’s time for that important second drink, you’ll realise you’re enjoying yourself; you’ll realise that this guy is easy to be with and interesting to talk to. You’ll get pulled in by the twinkle in his crinkly smiley eyes and the way that he seems entirely at ease with himself. You’ll agree to meet again and go home feeling cheerful.

In the middle of your second date you’ll decide you could fall in love with this person. I can still remember the happiness and peace of that exact moment. You’ll go on other dates and quickly begin to feel like a couple. It’s exciting, but occasionally a little puzzling. Sometimes it seems like Will holds back a little, and why isn’t he desperate to spend the night? You’ll hold him close, feel his heart beating fast and feel (wrongly) flattered. You’ll tell him he’s too good to be true and he won’t quite meet your gaze.

Three weeks in he’ll have a 4am coughing fit and you’ll ask where it came from. He’ll think about trying to gloss over it, but decide against it. Slowly, he’ll tell you that his lungs are buggered; that he has a genetic condition called cystic fibrosis. He’ll tell you it sounds scary when you don’t know anything about it. How I wish that were true, and that it didn’t get scarier the more you knew. You’ll think of programmes you’ve watched about little children with CF and assume out loud that he must have it mildly. “I’m doing ok”, he’ll say.

As the next days pass, slightly numb through lack of sleep and shock, you’ll realize that if you’re going to get out of this, you need to get out now. You’ll worry that staying would be weak – putting your short-term pleasure over your long-term future – but you can’t imagine walking away from Will. He already feels like part of you, part of your family, part of your past and present and future. You run with it; it’s not a choice as such, more of an instinct. You’ll be worried but you’ll also be the happiest you’ve ever been. You’ll introduce him to your family and friends (hoping for no errant coughing fits) and it will all be so easy.

As you get to know Will, and he shares more with you, you’ll get to know CF too. You’ll decide early on to learn through Will rather than the internet. So you’ll get used to the morning treatment regime, the creon at meal times (how did you not notice him taking them before?), the coughing and the evening tiredness. It’s not that he was lying about the cold on that first date, just that it was a handy excuse for things that are in fact always there. You’ll spend more and more time together and talk of living together. You’ll panic because you need to tell your parents about CF. Don’t worry – they turn out to be unquestioningly supportive and wonderful; they’ve got to know Will a little and they love him already. I remain overwhelmingly grateful to them for this reaction.

Your first year together will be pretty CF-free; it’s there but it’s not too worrying and there are no big infections. You'll climb hills together, fly kites, have some fabulous holidays and get used to Will needing to be in bed by 10pm. During the second year, now sharing a sweet little Victorian terraced house, he won’t seem quite so well. You’ll experience your first IV antibiotic round, then another and another. You’ll climb a hill together on a Greek Island and he will ask you to marry him. In a way, with all the excitement, you’ll not notice the subtle deterioration in his exercise tolerance and energy levels. You are so happy. Then comes the first hospital admission, which never quite delivers the lung function bounce you were expecting. Soon after, the doctors will want to introduce the overnight ventilator. After an initial panic you’ll decide to embrace this addition to your nights – a sensible decision – and you’ll soon be almost fond of it. It’s a hard few months, but it means you'll begin your marriage with your eyes as open as your heart.

So today, four years to the day after we met, I look back on that first date. I now know that Will’s quite tall for someone of his age with CF. Post-transplant, he skin is perfectly blemish free, the husk isn’t really there and neither is the cough, but he does still own (and wear) the poo-brown t-shirt. I wonder about that early instinct not to run for the hills. Knowing what I know now, I wouldn’t judge myself (or anyone else) for running. The fight against CF and the slow descent towards transplant is very very hard. My life has grown into something I could never have imagined; the fairy tales didn’t prepare me for it. But I equally never expected to love someone as completely as I do; to LIKE someone EVERYDAY as much as I do; to be as full-to-bursting proud of my husband as I am. Although I’d rather Will didn’t have CF, he wouldn’t be him without it. He wouldn’t be as open-minded and kind and honest and thoughtful and compassionate and humble and brave and determined and frankly bloody amazing, and so I cannot wish it away. The bottom line, in my mind, is that when you love someone, you just love them and you can only really get on with loving them, whatever direction that takes you. Walking away from love would be almost arrogant – to assume that something so precious would cross your path again. I’m not a saint, the worry gets me down and I am not always brave, but I am in love and I am happy and I cannot hope for more than that.

With love, thanks and respect,

Vicky on 23rd March 2010

p.s. There will be times when it will really help you to know that tonight, as I post this, Will is making pasta. I don't mean boiling water plus pan, I mean flour plus egg plus a load of kneading. Talk about energy levels.

Spring has sprung

The other evening I was wandering around Cambridge, waiting for Vicky to finish work so that we could meet for dinner, and I went to have a walk by the river and walked through my old college, Trinity. It was a beautiful evening, with the crocuses out on the avenue, and it reminded me again how it is so easy to forget what a stunning place Cambridge is and how we take living in this town for granted. I was struck by the peace of the place, especially when wandering through the courts of the college, and how it is just such a conducive atmosphere for thinking and study. Except, of course, when you are actually there you spend most of your time trying to avoid study, having too much fun or wrapped up in the petty worries of everyday life...

I snapped a few shots on my phone, so the quality isn't great, but I hope it shows spring has at last sprung in Cambridge.


Crocuses on the Avenue in Trinity College


Looking the other way, with the college library (Wren library) through the trees


Trinity college bridge over the river Cam. A classic view.

Learning to be normal

Well, all we really got from that clinic appointment was information we basically already knew and a big dose of reassurance, although that reassurance did have to be squeezed out of the doctor and nursing team with a series of questions and slight rants about our experience of the primary care NHS.


The thing is that in the past the most likely common illness I'd get would be a cold, and if I got a cold I'd be sure to get a nasty chest infection, and if I got a nasty chest infection I'd ring up Angie on the CF team at Papworth and she'd say 'come in and have IVs'. Simple. One person to call, immediate action; in hospital where you need to be, being pumped full of noxious antibiotics. Back then if I'd got a tummy bug I would have just waited for it to go away like everyone else. In the new post-tx situation if I get a cold I will more than likely be fine and won't get a chest infection, much like most people. It might take longer to fight off the cold, but I should be ok. If I get any other illness not directly connected with lungs the Papworth team really aren't interested - it's a job for the GP or local hospital, and that means the route we took this time - the out of hours GP service or A&E.


This is all in principle fine, but when you turn up and tell them you've had a bilateral lung transplant they, being intelligent doctors, get all interested and then, when they take a chest x-ray which makes it look like you have pneumonia, get worried and it is up to me and Vicky to feed them all the information. Of course they can phone a doctor at Papworth, but initially it is frustrating. They talk of administering antibiotics, but shouldn't we be checking with the Papworth team in case of allergies, or interfering somehow with the lungs?? I really wish they would hurry up with computerisation and networking of all your notes so docs have this info to hand. We got, as I expect a lot of you did, a thing through the post recently about the process of the NHS starting this with the summary record, but it would also be great if, for example, past X-rays were easily accessible by any doctor from any hospital.


Anyway, I'm getting off track. The upshot of clinic was that they did another ECG, it showed the same slight strangeness and even the cardiology consultant at Papworth couldn't work out what was going on. They don't seem too worried as the Echo was ok, so they will just monitor it. My lungs sounded ok and the X-ray from Saturday was ok (they got that sent over from Addies - so there is a system but you have to ring the radiology team and ask them to send it over, not possible at 3am on Saturday morning...) When I asked when they'd like to see me next they initially said 2-3 months and Vicky and I both exclaimed 'what??!' in unison, in comically high voices, since up to now we'd been on 3 weeks and I'd just been ill. It seems someone had written in last time's notes 3-4 months when they meant 3-4 weeks. So they then said 4-6 weeks. We went for a month to be safe.


So to the title of the post ' learning to be normal'. The general impression we got of the clinic was that their attitude was remarkably relaxed. "It doesn't matter if you miss the odd dose of immunosuppression (although we don't tell people that) - you aren't going to immediately start rejecting." "The first 3 months are the risky period, if the 3-month biopsy is clear of rejection it is much less likely things will start suddenly rejecting". Essentially they want you to start living your life and get on with things, and not have to be coming to Papworth all the time. If you get a common illness, you'll probably get better, and you can always call them, but go to the GP first as standard antibiotics will be fine. This is all terribly new, and a bit difficult to adjust to!

Part of the reason there was so much anxiety flowing around this time was that it was the first time I'd been ill post-tx. Next time we will know what to expect, what I can handle and how to work the GP/Addenbrookes system. Eventually we will learn to trust it will all be ok and learn to live life a bit more normally.

A bit of a blip

...and hopefully just a blip. I awoke on Friday morning with stomach ache which, after breakfast, developed very quickly into what they call in the trade 'D&V', that is diarrhoea and vomiting. It very quickly knocked me completely for six and I was stuck in bed needing all my willpower to haul myself to the toilet every 20 minutes or so. My temperature by the afternoon was running at 38.8deg C (101.8 F) and both I and especially poor old Vicky were starting to get quite worried. Vicky rang the Papworth team and they said they wouldn't usually do anything until 24 hours had passed - they are not especially keen to have anyone admitted with D&V unless absolutely necessary as they don't want to introduce these kind of infections into the hospital if they can avoid it. Vicky ended up ringing twice and the second time spoke to the transplant doctor on call who basically just said if it is still bad after 24 hours, or if you feel you need to, go to A&E at Addenbrookes. Great, we thought, I can barely move and the prospect of waiting for hours in A&E on a Friday night with the usual selection of drunks doesn't sound great.

A bit later, Vicky called my sister, Ellie, who is a GP and she recommended going to the out of hours GP service as they should be able to fast track you through A&E if we needed to go. So we managed, just, to get out to Chesterton on the edge of Cambridge and wait to see a very nice GP. She was just about to send us back home, since the vomiting had stopped and the trips to the toilet were getting less frequent, but then took my blood pressure which was low at 95/60. This was likely a combination of dehydration and the ramipril anti high blood pressure pills I take. So she recommended we go to Addenbrookes A&E after all and get some IV fluids into me.

So to Addies (as we know it). By the time we saw a doctor in A&E it was 1:30 in the morning. They did a load of bloods, an X-ray of chest and stomach, an ECG of the heart and a physical examination. At one point we overheard the doctors discussing my X-ray and one saying it looked like I had pneumonia, but the other saying that he didn't hear anything in my chest when he had a listen. This confusion was cleared up when I explained I had the fluid perfusion around the right lung post-op that was still hanging around, so that would explain the shadow on the X-ray. They put a cannula in and got me on IV fluids, and decided to admit me to a ward for the night, which took a bit of time as they needed to find a side-room so I was isolated and could be barrier nursed because of the D&V.

By 3am we were installed on ward M4 in quite a nice new big room with bathroom and a big window over the back of the site, out over fields. A great deal better than that awful side room in Papworth you may remember me describing a few months ago! Vicky went home at 4am and I tried to get some sleep.

A doctor who looked classically doctor-y came by in the morning, Professor Carmichael, wearing a bow tie (surely only doctors can continue to get away with this sartorially challenging move?). He had concerns about my ECG which showed some oddities. So he wanted that repeated, as well as a stool sample done. Once those were in the bag (as it were) he came back and had an extended and extremely careful listen to my heart, thought he could hear some slight 'rubbing' and arranged for an echo to be done, which is an ultrasound of the heart. He himself pushed me in a wheelchair the not inconsiderable distance to the Echo room where he left me with a young cardiologist to do the scan. The cardiologist said it was the first time he had met the legendary and 'incredibly intelligent' Prof. Carmichael and sounded slightly in awe of him. This probably explains why he pushed me to the echo room - once you get to a certain level of importance you feel able to do that without the insecurity that it would somehow jeopardise your authority...

The echo was a little uncomfortable as the probe seemed to have to be positioned right on my scar most of the time and pressed quite hard. Still, the cardiologist said he couldn't see anything untoward and couldn't really explain the odd ECG traces. There might be some slight thickening of the right ventricle (right ventricular hypertrophy) but that's really to be expected from a heart chamber that has spent years trying to pump blood into heavily diseased lungs. The poor old heart had to work really hard for a number of years.

Eventually then, at 8pm on Saturday evening, I was discharged and we came home. My CRP from the blood tests was high, and higher than normal for a viral infection, so the docs suspected a bacterial infection. However, I remember the Papworth crew saying before that the immunosuppression can raise the CRP, so it may still have been viral. They will only know for sure when the stool sample results come back and that takes a few days. Now I am a lot better, the bowels are still pretty dodgy and my appetite isn't great, but I think I'm getting there. I do feel that my chest is a tiny bit rattly when I breathe in fast, and that's a bit worrying, but I have my Papworth clinic appointment tomorrow, so I should be able to discuss all that's happened with Jas. They'll get another X-ray and have a listen to my chest and I guess if it seems there is something awry they might admit me for some IV antibiotics. Also I hope they will do another ECG and get the Echo results from Addies and make sense of all this heart stuff, which never would have turned up if it wasn't for the fact that A&E routinely do ECGs on people since I don't normally have them at Papworth.

A very stressful few days, especially for Vicky, so if any of her friends or family read this please do give her a call as she is so rubbish at asking for support when she needs it. I guess we should admit to ourselves that episodes like this are bound to happen however hard you try to avoid them, especially when your immune system is suppressed. It's interesting to note that Vicky, with a fully functioning immune system, thought she maybe had a very slight bit of diarrhoea yesterday and that might have been the sum total of her version of the symptoms.

I'll report on developments after clinic tomorrow.

Are they just having fun with words?

Sometimes you wonder if the medical profession has a serious dose of tongue in cheek when they name things. Both drug names and, it seems, surgical procedures often have ridiculous names. Perhaps it is to maintain a sense of mystique and exclusivity, to allow doctors to talk to each other without the patient understanding, or maybe it's just a laugh...

For example, the official name for the operation I am due to have is a Laproscopic Nissen Fundoplication. Fundoplication?? What? That's so much a made-up silly word.

For those of you interested in what it involves I found a video of the procedure online: Here

Looks to be...er...fun! Nice stomach wrapping technique.

It has left me in a bit of a slough of despond for the last few days anticipating this procedure. The things that I found hardest post transplant were the pain and discomfort, but also the sickness and problems swallowing. To be faced with another operation that will give me some pain (but obviously a lot less and less long lasting) but then a big dose of sickness and problems swallowing is just such a depressing prospect. I know that it is for the best in the long term and it will keep me alive for longer but I feel so settled and normal now and don't want that taken away.

Ah well. I shall await the letters from Addenbrookes - initially it will be an endoscopy to have a good look around (within March) and then the op in a 2-3 month time frame.

On a happier note we had a great weekend in Oxfordshire, visiting Vicky's mum and sister. Sunday was a glorious, sunny day and we went for a walk in the Chiltern Hills for about an hour and a half - I expect Vicky will upload some photos sometime.

A tightening of the sphincter...

...no, not that one...the one at the top of the stomach.

It's been confirmed today, at the second clinic appointment in two days, that I do need the operation to tighten up the sphincter/valve arrangement at the bottom of the oesophagus/top of the stomach. You'll remember that I had an oesophageal manometry and Ph study a while ago, and the results are in. They score the whole study using a number of factors, where anything less than about 14 is normal, they worry if the score gets to 20, and mine is 41. So yesterday at my scheduled clinic appointment Jas said that I should have the op, and could I come back today for a joint clinic with the gastro doctor from Addenbrookes (big hospital in Cambridge). By the way, my PFTs were further improved at yesterday's clinic which is great. Things are looking good with the lungs so far.

So Vicky and I trogged back to Papworth this morning and met with a room full of doctors and nurses. The consultant from Addenbrookes led the discussion and outlined the results of my ph study in more detail. They test for the sequential contraction of the muscles lining the oesophagus, to check the swallowing thing is working well, and that was ok for me. They then test the strength of the sphincter muscles, and mine was on the weak side of normal. The acid readings, however, were way up and there was lots of reflux recorded. I take omeprazole to control the production of stomach acid so generally this is kept under control. The test is done without omeprazole to help show up the reflux, but they say that even with the omeprazole the reflux will still be happening, even though you may not notice it. The acid and enzymes that come up are then breathed into the lungs in vapour form and do their damage. There is strong evidence that patients with reflux correlate with those who have the early onset of OB (bronchiolitis obliterans), which is the main symptom of chronic rejection.

The doctor said that I ticked all the boxes for the op. People who respond well to omeprazole (like me) tend to respond better to the operation. The swallowing muscles need to work well to push the food through the tightened sphincter, and mine are ok. The downsides of the operation are that for 4-6 weeks post op the diet is 'slops' - so lots of soups and easy to swallow foods. Things get better as the inflammation from the operation subsides. (Side note: Anyone with inventive and tasty recipes for sloppy foods please let me know!). As a result of the tightening it is basically impossible to vomit and very difficult to burp. This means a fizzy drink ban (to a Coke lover like myself this is particularly harsh!) and an increased propensity to pass wind as you can't burp the gas up...

So, some down sides but these have to be balanced with the long-term survival benefit. Basically it is a no-brainer when you think - just a real pain to have to go through another procedure just when I feel like I'm on an even keel and Vicky and I are starting to enjoy life. I guess it will be a lot less traumatic than the last operation - it is just keyhole surgery and a couple of days in hospital after all. The tightening is done by wrapping a bit of the stomach around the bottom of the oesophagus.

Other than the anticipation of all that, everything is going very well. I am feeling fitter every day and have started doing a few days a week at work. Work feels a lot easier than it did pre-op and I'm looking forward to being able to do the job more effectively and being able to maintain more enthusiasm. Wishful thinking? We'll see!