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!

The weird and the wonderful

No particular news at the moment (which is good news in itself I suppose), but here are a few anecdotes to keep you going:

1) The weird

A few nights ago our landline rang at 11.30pm. Will and I were well tucked up in bed. The short version of the story is that I eventually answered the phone - it was a slightly confused Will's mum - there was no drama and I went back to bed. The longer version emerged when I talked to Will about it in the morning and discovered we'd shared an identical thought pattern on hearing the phone ring:
- Oh my god, it's The Call, they must have lungs for Will.
- No, wait, Will already got his lungs.
- So are they giving him another pair?
- Nope, that doesn't happen.
- They must be ringing to tell us his lungs have stopped working.
- But why are they doing that in the middle of the night? And how do they know??
Took a while for the adrenalin to subside, I can tell you.

2) The wonderful

a) We realised that at Will's last clinic appointment, when they hooked him up to do his obs, we were so busy looking at the blood pressure numbers that we completely forgot to even look at his oxygen saturation. How times have changed.

b) On Saturday we went to a wedding and we cheesy-wedding-danced until the music stopped at midnight. The last time we made it to the end of a wedding was our own, 22 months ago.

Clinic update

A very quick post to say that I had my clinic appointment today and everything was fine. Jas was happy with my progress and is dropping the steroids down again a little bit, to 10mg/day - on the way to their eventual target of 5mg. The Neoral immunosuppressant levels are also a little high, so that's likely to drop too, and from previous experience even a small decrease in the Neoral dose makes me feel quite a lot better and more human, so that's good news.

I also took part in a clinical study today where they are using their brand spanking new posh and incredibly expensive looking CT scanner to take pictures of various patients at different stages of lung chronic rejection to see if CT scans could be used in future to diagnose early signs of rejection, cutting down on the number of bronchs that need to be done. I was injected with a marker that makes the blood vessels supplying the lungs show up very well under the CT scanner's X-rays and then rode the machine (it's one of those ones where you lie on a bed and it swooshes you back and forth through a do-nut ring shaped scanner). The marker injection makes you feel extremely weird - a hot flush all over and the sensation you have let go of all the bodily functions ...er... down below...

This week I also endured the oesophegal manometry and ph study which was very unpleasant. Having a well-lubed (KY jelly tastes bad!) flexible sensor about half the diameter of a pencil shoved up your nose and then 60cm down into your stomach is not great. Having it then taped to the side of your face and left there for 24h is close to maddening. Gives you a very sore throat. The results of all that will come in the next few weeks and that will tell them whether I need the other operation to sort out the reflux. Sounds fairly likely to me at this stage as there are real benefits to the long term health of the lungs.

Back to clinic in 3 weeks. I'm now on an 'every 3-weeks' regime until the 6-month point, which is the next major milestone.

Holidays, tests and work (?!)

Sorry it has been so long since I last posted anything, but life has been so normal that I haven't felt there is much to write about. I have sailed past the 100 day post op mark (today is 107), my PFTs (pumonary function tests) are gradually going up as I nail the technique of using the little machine and find it easier to push out all the air without it hurting my ribcage so much.

Vicky mentioned in her last post from the train down to London that we were packing mercifully light for a weekend away. It was just fantastic. This kind of thing was just competely impossible before - for one, I was pretty unable to do the required amount of walking to visit the big city (every walk to a tube train, every set of stairs in a station, was a real trial), and secondly we would have had to be carrying the NIPPV ventilator, the portable Oxygen concentrator, a nebuliser, a cool bag with ice blocks to keep the DNase and TOBI nebs cold, as well as the normal packing for a weekend away. Not very portable on public transport! This trip we each had our work bags and carried everything around with us all weekend as we saw various friends across the city. It was joyous!

We also spent a long weekend away in Aldeburgh on the Suffolk coast, as a trial first mini-holiday. We didn't want to go too far away from the hospital just in case, and it was great to go back to Aldeburgh after being there last summer, just before the transplant. Again the pre-post transplant transformation made us both very happy and we could indulge in long walks on the marshes and along the beach. One of the nicest things is being able to offer to be the one who walks to the other end of the village to get fish and chips for supper, where before I would have always been too tired and such jobs would default to Vicky. We had incredible bright sunshine, but absolutely freezing temperatures which made it simultaneously beautiful and painful as the windchill ripped into our faces on the walks. I don't have any photos to share with you as we forgot to take the camera - our drive for light packing obviously got the better of us!

On the recovery process, tests and monitoring, it has now been 3.5 weeks since the last bronchoscopy. As you may remember, after that bronch they lowered the dose of the steroids and the Neoral. They haven't checked the levels in my blood since, so I hope they are ok and they know what they are doing! I am going back to clinic next Thursday, which will be a month since the bronch. Hopefully all will be well and I'll be released for another month.

Before that, I have a Oesephegal Manometry and PH Study tomorrow. This is all related to the acid reflux problems I was having, especially just after the op. Things have improved a lot, but there is still a bit going on and, as I wrote before, breathing in the acidic air that comes up can be very damaging for transplanted lungs, so they like to get it sorted. I had the barium swallow a while ago which showed no physical problems with the gullet and no particular reflux, but they still want to study the acidity (PH) down there to make sure things are ok. If not ok, this could lead to a small operation where they tighten the sphincter that joins the gullet to the stomach, to stop the acid coming up. So, tomorrow morning I shall have a catheter (thin tube) stuffed up my nose and down my throat into my gullet, where it will stay for 24 hours. The PH readings are transmitted to a data recorder that sits on your belt. I am allowed to go home (but won't be showing myself in public!) and then have to return on Tuesday morning to have it removed. Hopefully it won't be too uncomfortable...

The possible return to the world of work is on the horizon, as my doctor's sick note runs out at the end of next week. I could renew it if I felt I needed to, but a big part of me would like to get back to work and engage my brain a bit. I've agreed with work that I can start gently, with a couple of days a week and then ramp up as I feel fit. I haven't actually asked the docs at Papworth yet, so will see what they say on Thursday and then take it from there.

It is very easy to feel like it is necessary to make some kind of massive life change after going through an experience like this. You feel you've been given a new lease of life and you need to make the most of it. You are only too aware of the survival statistics and the fact the good times won't last forever - the chronic rejection will start at some point, and it is hard to predict when. So do we go and live by the seaside, Vicky become the bread winner and I live some kind of idyllic life of leisure where I play the piano, compose a symphony, write a novel... It's easy to fall into these kind of reveries, but I reckon there is a big dose of 'grass is greener' syndrome here. I know from my experience of the last 3 months spent at home that I haven't played the piano as much as I would have liked, haven't composed music, haven't written much. Sure, I've been recovering and Vicky thinks I am far too hard on myself, but I suspect that given a life of leisure I would fritter it away with daytime TV, surfing the internet and playing video games rather than achieving much that would make me happy. So on balance I think the best thing is to try and return to the life I had before, enjoy being good at my job. At least give it a go, anyway. If I find it becomes soul destroying and I am constantly thinking 'why am I wasting so much of my days stuck in this office' then I can think again. I also realise now, more than I did at the time, that work was becoming increasingly untenable before the transplant. The very fact I managed to continue working 4 days/week right up to the day of the operation amazes me. Looking back I realised I was pretty useless at work and spent a good proportion of my time absolutely shattered, elbows on desk and head in hands feeling pretty deoxygenated and awful. So a return to the workplace with some energy to actually do the job well should be satisfying and fulfilling. Here's hoping!