Saturday, 23 January 2010

3 months today!

And we are on the way to London on the train. Staying overnight at Will's friend Charlie's and we're travelling unfeasibly light. Freedom!!

Monday, 18 January 2010

Musings from an anxious wife

Many of you have been kind enough to ask how I am through all of this; some have noted my relative absence from the blog since the very early days. Will has been encouraging me to post, and I've been wanting to, but it's hard to know what to say when I honestly have no idea how I am or what I'm feeling. This transplant business is hard. I'll try to explain the worms in my head.

My main battle seems to be anxiety: there is always something to worry about. I feel like my anxiety pool is full up and it only takes a tiny thing to make it spill over, clenching my stomach so that I can't eat and tightening like an elastic band around my throat so that I can't breathe properly or even really speak. Anything will set me off: is that just a cough or is it the start of an infection? was that just a little bit of phlegm or was it a wheeze? is it completely reasonable that Will's tired this evening or is this a sign of rejection? is this just a muscular spasm or is his lung collapsing? was that cry from the other room Will dropping dead or did he just fly his plane into a mountain on Wii Sports Resort? I loved a recent entry on parttimeboddha's blog about the worry he felt when his girlfriend, who is two years post-tx, caught a cold. Clearly these fears don't go away.

Although things were hard before the transplant, day to day life was oddly stable and anxiety free. It can sometimes feel tough to have swapped that for this. We knew Will's poor gungy, crackly and exhausted lungs inside out. We knew the rhythm of the monthly nebuliser cycles; we knew good gunge from bad; we knew that when he was shattered and achy we could put him to bed and plug him into his Nippy ventilator and he'd be better in the morning; we knew when it was time to give in to Papworth and the IVs; we knew we had the hope of a transplant ahead of us. Post-transplant, we know nothing except that this is it - there'll be no more new lungs.

Rationally, I know things were going to get worse. Rationally, I know we are incredibly lucky. Unfairly lucky. Some of you read about Victoria Tremlett, who has been waiting for over two years for her new lungs. Or there's Jessica Wales, who went on the transplant list in 2005, when she was just 16. She battled for four and a half years before getting her new lungs just after Christmas, when she was in hospital literally fighting for every breath. She had had nine false alarms. Although things looked good to start with, it was too late for Jess and she died last week. But the unfair road is a psychological road to nowhere. Sometimes I catch myself flirting with it, wallowing in it a little, trying it out for size. After all, none of this is fair. CF isn't fair; it isn't fair that Will and Mitch and Jamie and Ashley and all the other post-transplant CFers out there had to make this choice; it isn't fair that medical science hasn't quite sorted lung transplantation yet. But then I pull myself together, remember that Life Isn't Fair, remember that we are lucky to have had the option of transplantation, remember that we're not in Haiti, remember that no one knows just what the future holds and remember that Will's new lungs are a gift. Sometimes these notes-to-self feel like horrific cliches and make me want to kick things, but mostly they make me resolve to try harder to stay positive and be happy and not worry and be a better transplant wife and be nicer to Will and not lose patience when he's down and to cook him nice meals and wash his socks and clear out the fridge more often so that the bugs don't bite.

And then sometimes, suddenly, it all seems completely and without reservation totally worth it. Like Saturday. We went and looked at a car, then we walked up to the cinema, then went for a burger, then walked home. Then, instead of collapsing in a massively exhausted and deoxygenated heap, Will voluntarily sat up and watched Bridget Jones' Diary through to the end without falling asleep on the sofa even once. And then we got up early on Sunday, packed the smallest overnight bag in our overnight bag history, drove to Will's sister's house, had a lovely countryside walk with wellies and mud and Will raced Billy UP THE HILL. We played with our delightful nephews and niece, ate an enormous meal and sat up until midnight listening to nostalgic music and drinking wine*. This might sound like a completely normal weekend to you. It does to me too and it is fabulous.

So how am I doing? Terrified. Is this the hardest thing I've ever experienced? Yes. Is it worth it? Yes. I'll leave you to judge yourselves with the following photos. This first is a rare photo of Will+oxygen, as delivered by The Monkey, our chugging portable oxygen concentrator. Will was determined to climb this hill in St Ives in Cornwall this summer and I managed to persuade him not to remove the tubes before I took the photo. The second photo freaked us out a little when we looked at it again the other day. It was taken on our holiday in Aldeburgh in July, just before Will went on the list. He really does look ill, doesn't he?! The third was taken over Christmas and is of Will RUNNING**.

* In moderation of course
**Just in case a recent post-txer reads this and feels inadequate, I'd like to make it clear that he didn't run for very long. And it hurt his chest.

Thursday, 14 January 2010

And the person going through to the next round is...

...Will Cramer!! Yes, it's an all clear for me on the 12-week bronch and biopsy. The results were 'completely normal' and there was 'no infection'. They seem to refer to infection as a flare up of a bacterial presence so I expect the pseudomonas is still there in some capacity, although I didn't actually ask, so don't know.

In the end they persuaded me to stay the night after the bronch as they wanted to do an X-ray the next day to check for any inadvertent damage to the lungs from poking around and taking biopsies. So I was installed in the RSSC (Respiratory Support and Sleep Centre), where they happened to have a spare bed as the usual Chest Medical wards were full. This is where I used to go for sleep studies when I used the NIPPV ventilator before the transplant, and I think I wrote about my fairly grim experience of one of those studies in the early stages of this blog. After spending lots of time in CF and Transplant wards and clinics, where everyone is doing their very best to look after themselves, it is striking to spend time in RSSC where the majority of patients have smoking-related emphysema or are desperately obese and so have problems breathing. There is a very different feel to the place - it's the 'other side' of NHS care, and feels a lot less positive.

I was second on the list so went down for the bronch around 3pm. I was mid-conversation with Jas about various things while Massoud (Little Mo) busily started injecting the sedative, so my conversational skills got a little hazy as I felt the now familiar numbness and tingling in my feet as the drug took hold. I was awake as they inserted the probe and it was very uncomfortable - I felt like I was choking and coughed a few times - I remember them encouraging me to breathe and heard Jas asking for another 2ml of the sedative. And that's the last thing I remember before being woken at the end of the procedure and getting onto the bed to be wheeled back to the ward. With all that sedative I was straight back to sleep and Vicky tells me I slept soundly for an hour before waking up around 5pm.

Not much else to tell really. I slept pretty well as I was in a side room so was spared the sound of all the other RSSC patients' ventilators whirring. Today was extremely dull. It was dreary and pissing with rain and I had nothing at all to do other than a 5 minute X-ray (Radiologist: "This is supposed to check for pneumothorax, but I think you'd already know if you had one of those"). I went for a walk in the rain, visited the duck pond up by Mallard ward for old times' sake, found it to be mostly frozen over, went back to the ward, messed up the Sudoku in the paper I'd bought, finished my book, watched rubbish daytime tv and waited for the docs to arrive with the results.

This 12-week point is a milestone, as I've pointed out several times, and the all clear result seems to have been a trigger for removal of a few meds. They've stopped Aciclovir (anti-herpes), Colomycin/salbutamol nebulisers (as the infection looks ok), Amiloride (one of the diuretics), Nystatin (anti-thrush). The immunosuppression is also starting to taper off. The prednisilone steroids are reduced to 12.5mg/day from 15mg, with the aim of getting down to 5mg. The Neoral is down to 175mg, 3 times a day, from 200mg, with the aim of reducing it as much as possible over time.

For my whole life pre-transplant I had to do physio in the morning to clear my lungs. 14 years ago I added to the regime of physio and pills my first nebuliser - DNase, a mucus-thinning drug started during my 4th year at University. From then on in it only got more and more onerous with additional nebs, right up to the point pre-transplant where the regime took around an hour. Now, for the first time ever I can just get up and go. Well...pop a few pills, weigh myself, take my temperature, do lung function and go. It's still completely brilliant!

Tuesday, 12 January 2010

It's Bronch time again!

Tomorrow is a fairly significant event - the 12 week bronchoscopy and biopsy. It seems this is the first major milestone on the standard recovery/rehab path where they check for infection and rejection, and if everything looks ok they start to taper off the steroids and maybe stop some of the other meds, although I'm not sure which ones - presumably some of the anti-fungal stuff can stop when the steroids go down as they are responsible for the risk of thrush.

The prospect of these bronchs tends to burrow subconsciously into your brain and gradually, over the days preceding them, my and Vicky's anxiety grows. It's certainly happened this time - little things like a bit of sputum or feeling slightly tight in the chest take on too much significance. Either one or the other of us is a bit ratty or quiet and down in the dumps. It hasn't been helped this time round by a couple of annoying/scary/odd episodes:

On Friday I was getting itchy all over, and I decided it was a reaction to one of the drugs - I had red slightly rashy legs and hot knees, weirdly, along with quite swollen ankles. The swollen ankles is a very common side effect of the amlodipine anti-blood pressure medication that I had started after the last clinic, and so I guessed the rash and itching was also to do with this drug, as it was the only thing that had been added into the regime. I rang Papworth and they said to hold off the drug and we'll discuss it at clinic tomorrow, and start me on an alternative medication. Gradually over the weekend and yesterday the symptoms have subsided and I'm much more comfortable now. These kind of side effects that are constant reminders of the fact you are loaded up with hundreds of pills can really get you down. I found myself feeling like it was all a bit out of control and somehow the whole thing was fake: The stability and feelings of being well I was experiencing were conjured up by the medication, without which the lungs would quickly stop working. The medication itself was then harming me causing me to take more medication that was having side effects. I almost found myself thinking I wanted to have my old, familiar lungs back, with the familiar regime of drugs. At least they were mine. As you can imagine, this kind of thought pattern is not very constructive or even rational, but I was stuck with it for a day.

What then happened was extremely odd. While making some coffee on Sunday morning I suddenly had double vision. My vision just split into two images at a vertical separation of what appeared to be about 5cm. However much I rubbed my eyes, blinked or tried to focus I couldn't get a single image. Needless to say, this was very scary. Covering up each eye one by one showed that the individual eyes were working, it was just that my brain couldn't resolve the two images into one. After 4 or 5 minutes the images started moving together and it sorted itself out. This isn't something I've experienced before, or since. I looked it up on the web and the NHS Choices website told me I'd had binocular double-vision and there were lots of potential causes, almost all of them terrifying, like stroke, bleeding behind the eyes, nerve damage, diabetes etc etc. However, since it went away on its own I'm hoping it was just a muscle spasm because I was a bit tired. I rang Papworth again and after speaking with Jas the nurse came back to me saying 'Jas isn't too concerned, so we'll just see you in clinic on Wednesday'. No explanation of why he wasn't concerned - whether it is a common thing and he's seen it before, that it's due to the drugs (Neoral lists 'visual disturbances' amongst its many many side effects), or just that since it sorted itself out he wasn't worried, or maybe he just doesn't know anything about eyes... I guess I'll ask and find out tomorrow.

So the rash and the double-vision left me feeling quite down on Sunday, but by yesterday I was feeling more positive again. Now I just need to keep my fingers crossed for tomorrow's procedure, and then suffer in anticipation through Thursday until the results call comes. At least they are letting me do the bronch as an outpatient again, so I can get home tomorrow night.

The rollercoaster continues...

Thursday, 7 January 2010

Drinking paint

Had the barium swallow yesterday and it wasn't all that bad - certainly not as awful as some people had said who'd had one. It is a mildly fruit flavoured white paint, served in a plastic cup. It seemed to come out of a drinks can as there was an empty one on the side. I had the procedure in the same room as the bronchoscopys, so now understand why the posh swively Xray machine is in there. I started standing on a little platform with a vertical plate /bed behind my back, holding the cup of liquid in my right hand as the radiographer manoevered the Xray in front of my throat. The Xray machine appears in front of you as a round plate, about the size of a car wheel, but with a flat gray plate on the front. It sits about 4 inches from your front. I was then asked to take a mouthful of the liquid and hold it in my mouth and then, on the radiographer's command, to swallow. The Xray then moved down my front taking a series of pictures, following the white liquid as it descended to my gullet and stomach. After a few mouthfuls with pictures at different angles they motorized the bed/platform to a horizontal position and I lay on my front and did another swallow, then lay on my back to see if any reflux occured.

The result of all this was that there was no reflux. There was also no hernia (where a little bit of stomach pokes up through the diaphragm. So both of those are good pieces of news. There was, however, a little bit of 'hold-up' where there was an accumulation of liquid in the gullet, just above the sphincter into the stomach - I was shown this on one of the shots on the monitor - and the radiographer explained that this could explain the symptoms I've had, and certainly is consistent with the feeling I had earlier in the recovery period where I couldn't eat fast at all as it felt like everything was backing up and wouldn't go down. It will be interesting to get Jas' reading of the results, but as far as I could see there was nothing structurally wrong so maybe I won't need the op. We'll see. It may just be a case of learning to eat a bit more slowly and keeping on taking the omeprazole anti-acid pill.

On another matter, I'm getting a little frustrated with the lung function testing. They give everyone a portable spirometer that you blow into to measure your FVC and FEV1 every day, as a 10% drop in FEV1 is a goood indicator of things going wrong. Problem is that using the thing is massively technique based. I get wildly variable results, and the other day randomly managed to get an FEV1 of 2.9L and simply cannot repeat it, however many times I try. The best I could get today was 2.44. Where has that half litre of air gone? The technique seems to require a weird combination of being relaxed and blowing out hard and I can't quite get it. I know the good results are out there/in my lungs somewhere... I guess eventually I'll learn. Of course, I made the mistake of giving Vicky a go and without any practice she blew my results out of the water straight away, first try. Let's just say she wasn't getting a second try... Something to do with a youth spent doing lots of swimming training.

So life carries on, but there are still some difficulties. I still sleep badly - I get little chunks, no decent stretches it seems. I regularly wake up three or four times a night and have very weird, vivid and often disturbing dreams. Last night I dreamt I was driving and had awful pain in my shoulders so had to pull over, and woke with terrible pain in my shoulders and rib cage, surprise surprise. I have got this a few times, generally related to bad posture in bed, driving or slouching on a sofa and it is a quite extraordinary pain. It is as if my whole back and rib cage go into spasm all at once. The first time it happened I almost called 999, but now recognise it is muscular and not the lungs falling over. It is pretty much the only pain I've experienced that is bad enough to make me involuntarily sick. If I walk around and do stretches it does go away, but is unpleasant. I seem also to get more little pains in random places like joints of my big toe, or my calves - enough to wake me sometimes. Of course this could just be because I am doing more exercise than ever before.

I also had a horrible dream last night that I was coughing up CF-style sputum again - the big sticky green globs that used to be a daily familiarity to me. In the dream there was obviously something terribly wrong and I needed to call the doctors - the lungs were failing already.

As the concerns of the transplant and recovery recede and stability and normality return other things start to crowd into the spare worry space and the major one currently is how best to deal with my mother's needs as Alzheimer's begins to have a really debilitating effect on her. She lives in an isolated location and is getting to the point where she needs increasing care. Of course many people have to deal with these choices and difficult decisions, but we didn't expect to have to do it so soon - mum is only 67 and is already fairly bad. It is so very hard to know if you are doing the right thing when it is almost impossible to work out what she really wants - is it most important to stay in her own home and have care brought in, remaining in an isolated place and not seeing her kids that much, or should we encourage her to move into a home nearer to either me or my sister? How do we finance the care? Sell her house, equity release? And so on and so on. Once you start looking into these issues you realise it requires a great deal of research and effort and I have the conflict between caring for myself in the period I am off work and using the time to make some headway into dealing with these issues. My sister is always very busy with 3 kids under 6 and working 2 days a week, and she puts in a great deal of time and effort into helping mum already. I need to start to help out more.

Ah well, sorry to grumble. It is approaching 4pm Neoral time, so I'll stop now, take some pills and then walk through the ice and snow into Cambridge to spend an HMV token I got for Christmas (on some silly game for the Wii I expect) and then meet Vicky after work for some dinner.

Monday, 4 January 2010

A New Year update

Happy New Year everyone and I'm very sorry for not writing anything over the festive period. Just so darned festive, you see. Something to do with that anyway, combined with a generous dose of laziness and far too much chocolate. One of the disadvantages of my situation is having to weigh myself every morning - let's just say that I almost had to call Papworth a couple of times as I was close to exceeding the 2kg in 2 days weight gain limit! Seems to have settled now at what must be my 'natural' weight.

So, where to start? After a few more shopping trips we packed Vicky's Clio and endured a very busy and frozen journey out to my mum's in North Norfolk. We were lucky that East Anglia was busily starting to thaw when other parts of the country were getting much worse. We arrived to a VERY cold farmhouse and made ourselves busy getting wood and lighting the wood burner to try and warm up the 'big room'. Once this room gets warm it helps raise the temperature of the whole house.

The view from the Big Room when we arrived in Norfolk

We helped mum decorate the tree and made the old place as merry as we could with good chocolate, booze and springs of holly on the beams. On Christmas Eve we went for an extremely bracing walk on the beach at a very desolate Walcott and had excellent fish and chips for lunch in the cafe. For Christmas we met up with my sister, her family and inlaws and then on Boxing Day they all came to mum's house. We went for a walk down the field and were pleased to see the trees we'd planted 20 years ago had finally provided enough cover for the brambles to be supressed and a proper little wood has appeared.

We also managed to catch on camera the moment when my elder nephew attempted to murder my younger nephew:

On Tuesday 29th we left Norfolk and made our way back to Cambridge for the night, as I was due in clinic at Papworth on Wednesday morning. We arrived early at clinic to try and beat the first come, first served system. Arriving at 8:30am got me at number 2 on the Lung List, but we rapidly realised that there were very few people there anyway - I think 4 in total - so we need not have bothered to get up early. My blood pressure came in at 160/100, which is high. Jas said that 96.4% of people on Cyclosporin (Neoral) got high blood pressure and he didn't believe the other 3.6%. He thought we should treat it to make sure my heart isn't working too hard, so I am now on 5mg of Amlodipine a day. The most common side effect is swollen ankles, so I'm waiting to see if they appear. Apparently they can treat that with another drug....wheels within wheels...

It is now more that 6 weeks since the fluid drain so I did my first official lung function test, and got an FVC (Full vital capacity) of 3.5ish and an FEV1 (volume exhaled in the first 1 second) of 2.43 litres. This puts me at about 70% predicted for my age, sex and height. Jas said this was 'good', and I have read in other places that these numbers can improve over time (someone had 50% at 3 months and 80% at 6 months), so I am staying positive. I also remember that during the assessment Jas had said it is wildly variable with some people having 120% lung function and others with 30%, but both reporting 100% oxygenation. My saturations were 99% and seem, from the odd measurement I've done at home, to be very stable in the high 90s, so that is very positive.

We discussed acid reflux which I still suffer from periodically. Jas said that in CF-ers the stomach produces lots of acid to try and compensate for the lack of digestive enzymes - attempting to break down the food more in the stomach. Also, CF folk can have weak diaphragms due to the years of shallow breathing, and this, combined with the trauma of the operation can mean a lack of support of the osoephagus at the sphincter at the top of the stomach. This weakening of the sphincter, combined with the high acid level in the stomach, leads to reflux. This in turn has been stongly shown to be bad news for the transplanted lungs as you are constantly burping up acid air and then breathing it back down into the lungs. Nasty. There are two ways to combat this. The first is omeprazole which inhibits the production of stomach acid (which I am already taking) and the second is to do an operation to give more support to the osoephagus and shpincter by wrapping a bit of stomach around it, or something - frankly I didn't quite get all the details... The operation is done by keyhole surgery, would involve a 3-day stay in Addenbrookes hospital in Cambridge, and would require a 'mushy diet' for 4-6 weeks afterwards. I know which bit would piss me off the most already. To decide whether the operation is necessary they will first do a Barium Swallow, where you drink a fluid that reflects X-rays and so shows up where stuff goes when you drink/eat - this checks for any physical abnormalities. The second test is a 24h monitoring of stomach ph, which involves having a tube up your nose and down into the stomach, and carrying around a little data recorder on your belt.

The Barium swallow is scheduled for Wednesday this week. Apparently it is pretty grim - doesn't taste of much but is very much like swallowing emulsion paint. Great. The ph monitoring isn't yet scheduled, but I guess will be in the next few weeks.

The final bit of info Jas had was that in my last bronch the samples of sputum they took grew pseudomonas aeruginosa, my old CF lungs bacterium of choice. Not a lot, but it is there. This is extremely common as I'm sure I've already mentioned because it lives not only in the CF lungs but also in the upper airway and sinuses which, of course, were not transplanted. The new lungs should be much better at dealing with it, and so far I'm not feeling anything other than a tiny bit of sputum from time to time. I'm still doing the nebulised antibiotics, which will help to keep it at bay, but I'm not sure if this means I'll continue to need to do them ad infinitum.

Jas wants to do another bronchoscopy and biopsy on Wednesday next week, a week after the Barium Swallow. That will be nominally the 3-month post-op point (in reality 2 days early) and so is a big milestone. If there is still no rejection they will start to very gradually reduce the steroid dose.

A mixed bag of a clinic then, but largely positive, and we left feeling pretty happy and got in the car to drive directly to Vicky's mum's house in Oxfordshire. After a fairly dire session on the M25 we arrived to another set of Christmas present giving and receiving and had a very relaxing time. I was sneezing quite a lot by this point - it seemed to be a combination of a slight cold I must have picked up from one of those snotty nephews and a fairly severe allergy to Vicky's mum's 2 dogs and her sister's cat. It seems that immunosuppressants don't interfere with histamine reactions - must be a different bit of the immune system entirely. I ended up on the first night in a bit of a sneezy, blocked up, snotty mess and then woke up at 1:30am with itchy legs and a rash. I ended up calling Papworth and asking them if it was ok for me to take antihisthamines - I have to check every drug carefully just in case. They said yes and so I managed the next few days with plenty of pills and staying away from the dogs as much as possible. New Years Eve was very quiet - for some reason I was utterly shattered so ended up in bed at 10pm, so nothing to report there.

On New Years Day we went to see Vicky's Dad, had dinner, a most fabulous firework display and yet more presents (endless Christmas was the theme this year). On the way we had stopped off for a sunset walk up a small hill - Wittenham Clumps - and so I will leave you with this particularly flattering shot of two sisters. My darling wife on the right and her younger sister Chloe. If you got this far, congratulations and Happy New Year again!

That's too mean isn't it? How about this one instead. Much better: