Vicky joins in

Hi everyone, Vicky here. Part of the reason for moving over to Blogspot was so that I could post too. Seeing as it may be some time before anything particularly dramatic happens on the transplant front, I thought we might share more of our day-to-day goings on. Not that our lives are terribly interesting, but it's either that or let things go awfully quiet for an unspecified period of time and you'll all lose interest.

You'll see from the photos Will added that we've had three lovely holidays. St Ives was mostly fish and pasty-based. Provence was hot, windy and beautiful and involved more patisserie. Aldeburgh brought a welcome return of fish but with a lot more ice cream (blame it on the children), an enormous house (ah, how we dream) and some great thunderstorms. There's something really special about the Suffolk coast and it was brilliant to see it through children's eyes. Yes, a boating pond and two small wooden sailing boats really can entertain them for hours.

Bliss, but back to work we go... And August 3rd rolls nearer...

Climbing on the shell sculpture near Aldeburgh.


A rather wet boat trip on the Alde in Suffolk. This is Julie (my mum), me, and then the Allen family: Billy, Tommy, Richard, Ellie (my sister) and Edie.









Chic coffee in chic Lourmarin, says Vicky.
In (En?) Provence. It's me with Vicky's mum Rosie and two sisters Maddy and Chloe, by the pool in Lourmarin.

We rented a lovely cottage overlooking the harbour, and this is the view at sunset. Photo by Vicky, annoyingly!

They say St. Ives has a "special light". Before we went we thought this was probably a load of..erm...cobblers, but as you can see it is quite nice. Photo by Vicky, annoyingly.

The holiday bonanza

I hear that people have checked back to see if I’ve written anything new and since I hadn’t were worried if I was ok. I’m fine and have just been having a month or two of holiday interspersed with a little work. In an effort to use up holiday opportunities before going on the list, as well as seeing family properly, we’ve been away quite a bit.

Problem is that I’ve got quite used to working in a week-on, week-off way and so the unbroken stretch between next week and Christmas is stressing us both out rather!

We went to St. Ives, enjoyed West Cornwall and thought about trying to walk the south west coast path when I’ve got breathing power. To Provence with Vicky’s mum and sisters. And are just back from Aldeburgh in Suffolk with my sister and her brood and my mum.

Waiting time

Lots of people have emailed their best wishes - thank you so much for all the support. Both Vicky and I will be calling on all the offers of support when the time comes! Talking of which, there was a critical piece of information I managed to miss out of the postings about the assessment - the likely waiting time.

I was told that for someone of my blood group (O+) and height the wait would be 6-8 months. However, it really is a one-on-one matching process so it all depends when the organs turn up. Being O+ is both good and bad. Good in that it is a common blood group so it is more likely any given donor will match. Bad in that it is a common blood group so more people on the list will also be O+ and might also be suitable for the lungs.

So, it could be a day or it could be a year, but it is most likely to be 6-8 months.

Singing

There was an interesting piece on PM on Radio 4 last night as I drove home from work about an experiment being done at Royal Brompton hospital in London, one of the main lung specialist hospitals. They deal with people with Asthma, CF, COPD, and so on and have introduced singing lessons and group singing on the wards. There were testimonials from a number of patients saying their ability to move around and get things done had increased since starting the singing. The doctor in charge said that simply teaching people breathing techniques can sometimes be detrimental as they become even more conscious of their breathing and this can lead to tension, but if you introduce the techniques through singing it works.

I have always maintained that singing has helped me fill my lungs and make the most of what capacity I have - nice to be vindicated and brilliant that more people are getting the opportunity and the benefits.

More here http://news.bbc.co.uk/1/hi/health/8085201.stm

Assessment day 3

After a welcome home-cooked dinner and a night in my own bed Vicky and I returned to Papworth for the third and final day of the assessment. On the programme for the day was an appointment with one of the transplant surgeons. The team would then have a meeting to discuss me and any other cases they were assessing and finally report back their recommendation to me.

We arrived and were met by one of the transplant coordinators, Ann. The coordinators are an amazingly bright and breezy bunch of people - I guess it is part of the job description - and they sort out all the arrangements before transplant: getting patients to the hospital, coordinating the delivery of organs from the donor and so on.

Ann took us in to meet the surgeon, Mr Tsui. Mr Tsui is the head of the transplant service at Papworth, so we were in the privileged position of meeting the boss! He was an impressive individual with a very measured manner and he slowly took us through the procedure for selection of donor lungs and the operation itself. What surprised me is how picky they are; how many people who on paper look to be reasonable candidates for donation are rejected. The donor is likely to be in another hospital and so either a team from Papworth or a team on site at the donor hospital will assess the organs. Of course there will be teams all taking their turns for the lungs, heart, liver, kidneys and anything else that might be required. The lung team will measure the gas exchange capability of the lungs, the size of the organs and will do a bronchoscopy where a camera is put inside the lungs to have a good look around and see if there are any tumours or other problems.

Matching of the lungs to the receipient (me!) will be through blood group and lung size. Mr Tsui said that lung size can be +/- 15% of my ideal size and still work ok.

Mr Tsui then moved on to the procedure itself. There are three possible operation types they can do. A vertical cut through the sternum, right down the middle of the chest; a cut “under the breasts”, the so-called clamshell procedure; and a cut round the back around the shoulder blade, which is more unusual I believe. Apparently the vertical cut is less painful post operation as there is less flesh down the middle and the sternum iteself can be immobilsed with wires and will heal quickly and well. However, there are a couple of issues. One is that access is restricted for releasing the existing lungs from the chest wall, which is often a problem with CF lungs where there is a lot of scarring from infection ‘welding’ the lungs in place. The second is that visually is it more difficult to get a good angle on aligning the two ends of various vessels that have to be joined that run horizontally across the chest. Because of this it is more likely that I would have the clamshell incision, which involves sawing through all the ribs (!!) and opening me up like a rack of lamb.

The old lungs are taken out one by one. I might ask them to keep them for me - I quite fancy them pickled in an old-style bell jar on a shelf in some future office…(am I being weird??). The new lungs only arrive at Papworth 10 to 15 minutes before they are put in. Sounds crazy (what if the ambulance bringing them crashes?) but that way they only have to keep them on ice for the shortest possible period. The new lungs are put in one by one, the windpipes joined and the vessels sewn up and then they close me up. Total time around 9 hours.

I go into intensive care and am kept sedated for 24 hours. I imagine this period of the operation and post-sedation will be totally hellish for Vicky.

Mr Tsui ended by talking again about survival and telling me everything that can go wrong, but pointed out I am a good candidate and some people can survive 20 years. I just need extreme quantities of luck to try and be one of those people.

Vicky and I went for lunch (lamb madras in the hospital canteen…mmm..) and then returned for a final chat with Ann, the coordinator, about what happens next. She reported that everyone thought I was a good candidate and it was the right time to go on the list. So we have agreed that I will be listed on 3rd August after I’ve had a couple of holidays in July and organised a few things, like signing my Will and so on.

Ann then told me about what needs to happen while I’m on the list, that I can be called at any time and must always be contactable. We left her with a slew of phone numbers for me, Vicky, my parents, her parents, our siblings - all the likely places we might be. I have to inform the transplant team every time I go more than 1 hour drive from Cambridge. I must stay in the UK, preferably England. I need to ring them monthly to give a basic update - what’s my weight, do I have any infections that might preclude transplant (such as random ear infections), am I well?

And that was about it. If you’ve managed to read this far, congratulations. I am feeling positive and in a way relieved that I’ve been offered the place on the list. It is terrifying and exciting. I’m looking forward to riding a bike, walking into town without feeling awful and without oxygen, climing a hill, going skiing, windsurfing again… I’ll update this blog with any significant developments as well as dropping in the odd post about what we’re up to. Finally, please do keep your fingers crossed for me!

Assessment day 2

Days 2 and 3 were all about information and talking. I got a talk from one of the nurses who look after continuing care for people post transplant, and she described all the drugs and the regime. It involves lots of pills - 3 basic anti-rejection (immunosupressant) medications and then a load of other stuff to counter the side effects of the immunosupressants, about 10 drugs in all. The side effects are everything from a higher chance of skin cancer and lymphoma to nausea. Hooray!

I can imagine for someone not used to dealing with a drug regime this could all sound pretty horrendous, but I counted up my current drugs from my repeat prescription:17. So I’ll lose a load of the CF drugs to do with the chest, keeping the ones to do with digestion, and take on a load more. I have systems….

The side effects are obviously pretty scary, but people can and do live pretty normal lives after transplant, so it must be manageable. The other thing you have to do daily is weigh yourself and do lung function tests, as both of these are indicators of rejection starting. The drug regime is all about damping down the immune system so the lungs don’t reject, but leaving enough immune system to fight infection and viruses - so colds don’t kill you. It is a delicate balancing act and sometimes the rejection starts and they have to bang up the immunosuppressants for a while to get it under control, generally steroids, and then tail them off again once things are ok. The nurse also mentioned OB - Obstructive Bronchiolitis, which is the gradual scarring and decline of the transplanted lungs due to the chronic rejection of your body’s immune system constantly attacking the lungs at a low level, and one of the achilles heels of lung transplantation. Everyone points out that this procedure is all about quality of life, not necessarily longeivity (sp?). If left without transplant I might, with a lot of luck, just about make it for another 5 years, but those would be pretty miserable years with an increased reliance on oxygen, more hospital stays and reduced mobility. If I do the transplant, the median survival is 5-6 years, but during those years I could ride bike, climb hills, etc etc. Of course I’m hoping for more than 5 years, but better not to wish too much to early - I am being cautiously optimistic about getting through the operation, and that’s enough for now.

After seeing the nurse I met Jas Palmer, the consultant chest pysician on the transplant team, who I remember being one of the doctors with the CF team about 10 years ago. He reiterated much of what we’d discussed in our previous meeting 4 weeks before, checked there were no counter-indications in my test results and basically said that it is about the right time to go on the list. “If you were transplanted tomorrow I wouldn’t say that was a bad thing”, as he put it. He suggested I go home to Cambridge for the night and come back for the final round of chats the following day, so after convincing the nurses over in the chest medical unit, where my bed was, that I could discharge myself and go home, I did just that.

Assessment day 1

Transplant assessment is all about trying to get as much information as possible to allow the doctors to make the most informed recommendation to me that they can. The problem is that there is a lot of uncertainty, both in the amount of time I’ll have to wait for organs and in what happens to me while I’m waiting - how well I can stay. So they try to assess my ‘trajectory’ of decline, and judge whether it is speeding up or is gradual and constant. They also determine my blood typing (group and antibodies) and the physical size of my chest cavity (through a CT Scan). This will tell them which unfortunate donors will be able to offer me their lungs.

…and that’s the thing that must be remembered through this whole process, that a new set of lungs for me represents a tragedy for a family somewhere. It may have been head injury, road traffic accident, stroke, cardiac arrest (with no lung damage) - all sorts of possibilities.

So anyway, the assessment itself took three days between Monday and Wednesday this week. Day 1 was all about tests. I did lung function tests that measure your lung capacity (mine: 2.45L, normal: 4-6L), the amount you can blow out in 1 second (mine: 25% capacity, normal: 90%), how well the gas transfer is working across the lungs (I heard in the past mine was about 6% of normal…). They also make you do a 6 minute walk to look at pulse rate and oxygen saturation in your blood. Normal folk breathe a bit harder when exercising and the saturation stays at 98-100%. My rest saturation without Oxygen is ~88% if I do nothing, and drops rapidly as soon as I start moving around. I did the walk with 4 Litres/min Oxygen (fairly high flow) and dropped to around 83% walking at a moderate pace.

In other words, the lungs are rubbish. This is Type II Lung Failure - where the scarring and inflamation from 34 years of infection has meant the tiny tubes at the bottom of the lungs, next to where the gas transfer takes place, have blocked up.

On day 1 I also had an echocardiogram, which is a detailed Ultrasound of the heart to look at whether there are any heart problems such as pulmonary hypertension, which can happen when the lungs are compromised and the heart has to work very hard. Luckily my heart is fine.

After all the tests I spent the night in a cubby hole style room in Papworth. Luckily I managed to escape hospital dinner (the food is truly disasterous in the evening, lunch is just about bearable). Vicky and I drove out to a nearby village pub and tried to finish some unfeasibly large portions of unreconstructed pub food. Nice.

Well, here we go...

As some of you know, and some don’t, I’m at that stage of Cystic Fibrosis (CF) where my lungs are starting to give up the ghost and really aren’t working too well any more. The only option left open to me is lung transplantation and for the last 4 years the doctors at Papworth Hospital have been mentioning it more and more, getting me used to the idea.

After a couple of nasty chest infections earlier this year that landed me on IVs (intravenous antibiotics) in hospital I have dipped in my ability to exercise and use my lungs, so the docs and I agreed it was time to get properly assessed for transplant, to see if I’d be a good candidate for the surgery and the life after transplant.

I plan to post updates on here from time to time as I journey down the road to transplant, partly as a kind of catharsis for me, and partly to let everyone know what I’m up to and how I’m getting on.