Well, I'm really starting to feel better! On the last day of February 2012, I celebrated (perhaps 'marked' is more appropriate here), the 1st anniversary of being diagnosed with Amyloidosis. Contrary to expectation, the time has flown by and I have no real feeling of having lost a year out of my life. Of course, it's been a challenge physically but the biggest challenge has been avoiding the self-pity that comes from wondering why this happened to you in the first place.
Since finishing the chemo in the summer, I determined to get some level of fitness back as a route to normalisation. So, from not having the strength to propel my borrowed wheelchair - just 7 months ago - I've been able to cycle again, and it's been great for building my stamina and confidence, I guess.
Farm Food Aids My Recovery!
Fresh food has been great for my recovery and one of my highlights is taking my bike and riding to Wishart's Farm, just outside the village, for fresh winter vegetables. I turn up with a rucksack, fill it with stuff and I'm constantly amazed at how much you can get for a fiver!! At the moment, the spuds are amazing and Nancy at the farm has even introduced me to the superfood, kale. Here's a short clip to give you an idea of what your local (if you stay near Cairneyhill) farm shop sells at the moment.
Another wee treat that I've come to enjoy is visiting the Limekilns Hotel & Bistro for lunch with friends and business colleagues. The food is straightforward, tasty and reasonably priced and the place itself is lovely as a lunchtime venue or for an evening meal, tucked just off the main road with a view over the Forth.
Wow! Mark Swatton, Head of Clinical Governance at the National Waiting Times Centre at Golden Jubilee Hospital in Clydebank started the year as one of my clients. Little could he have known that a few months later, we'd be friends with a common interest in cycling and he would be picking up the challenge of raising awareness of this nasty, but rare disease that neither of us had heard of.
As you'll see from Mark's own blogs, his challenges are hard, taking part in cycling Etapes and Sportives in Scotland, over hundreds of miles in total and requiring many hours of training in between. He is not a club cyclist so this is a serious, one-man challenge and I am immensely impressed with the interest that he's shown in this cause and his desire to do something positive to help. According to the staff at the NAC, many people have probably died of Amyloidosis without ever having been diagnosed with it, such is the ignorance surrounding it. Raising awareness may be the thing that saves one person, simply by getting them to the attention of the right people, fast enough.
If you want to help, Mark's Justgiving site is the place to make a donation but read his Blog anyway to see how much he's done to help so far.
Part of what I do for work is building 'Cloud' databases for asset management, real-time information gathering etc so it seemed natural for me to build a system that could be used by researchers interested in assessing how Amyloid patients (to begin with) were responding to their chemotherapy drugs. As I have over 500 tablets to take in each 21 day cycle, I have to calendarise everything in a diary anyway, so adding the additional detail didn't take too long.
What you're about to see is my data, summarised as a report (my full system is much more detailed). It's unedited and perhaps a little graphic in places but it's just an honest record, put down dispassionately.
Amyloidosis. At 28th February 2011, this was simply a word that I had never heard of, let alone been aware of its meaning or darker implications. Why should I have known? I was 47, fit and healthy - didn't drink, didn't smoke and had a nice balanced diet - visiting the Doctor once in a blue moon. I was also an enthusiastic leisure cyclist, having started cycling at the age of 35 doing it almost every weekend since. (I'm also involved in running ACE, our brilliant little voluntary cycling project in Cairneyhill).
So, you see, probably just like you, I had no real reason to worry about my health.
But things change and sometimes the change is abrupt, unexpected and sets you on a new path, good or bad. Here's what happened to me...
In Nov 2010, I paid my GP a visit with suspected prostatitis and happened to mention that I was also feeling queasy most of the time and had a pain just below my right rib cage. Nothing alarming in itself but we'll deal with the prostate thing first and see if the nausea goes away.
By late January 2011, the prostate problem was away but the queasiness had developed into something more. I was starting to get sick, sometimes with blood, and with an intensity that I'd never in my life experienced before. On one occasion, I blew the blood vessels surrounding both my eyes - just from being sick. This left me black and blue, as if I'd been in a fight.
And the swelling! Within days, my feet, ankles and legs had swollen to about twice their normal girth and I couldn't easily fit my feet into shoes.
But still I tried to ignore it, forcing myself into cycle shoes and going out on bike rides in the belief that, whatever was going on, it would be temporary and easily dealt with after my next visit to the GP. I continued to work, meeting clients, delivering training seminars but becoming increasingly breathless and more dependent on water to get through the sessions. Something was up but no-one could tell me what!
My GP arranged an appointment for me to see a haematologist and get an ultrasound as she was starting to suspect that there was something happening in relation to my liver. There was also raised protein level in my urine, which suggested that there might be kidney involvement too.
I didn't make it as far as my next appointment. Instead, I presented at the A&E department of Queen Margaret Hospital, Dunfermline, knowing that I couldn't go on. I was admitted.
I spent two weeks in hospital, undergoing a variety of tests to establish what was wrong with me. Other than knowing that I was 'nephrotic' (in other words, swollen in ankles\legs etc), nothing was tying this up with the enlarged liver, kidney problems etc. Eventually, the decision was made to carry out a kidney biopsy to get a tissue sample analysed.
This was the turning point.
Biopsies and A Diagnosis
The biopsy itself was relatively painless, if a little uncomfortable. It's done under a local anaesthetic and accurately controlled with an ultrasound or CT scanner. As the risk of bleeding from the kidneys can be quite high, requiring intervention, it's important that they target the area accurately. My experience was fine in this respect.
Returned to the Renal Ward, I was shortly informed by my Renal Consultant that 'something' had shown up as a result of the biopsy and it wasn't good news. They had discovered the presence of Amyloid.
Now, having never heard the word mentioned before, I still had no idea what the implications of this were for me. Would I have to stay in hospital for a bit longer or would I be able to get some pills and go home? Slowly but surely as Doctor Alfonso explained that this was a very rare illness, and was potentially life-shortening, my horizons started to cloud.
So, What is Amyloid?
Rather than try to explain Amyloid within the confines of a Blog - when there are plenty of good articles on it (and I'll attach links to these), I'll try and summarise the main aspects:
Amyloidosis is a term that refers to a group of abnormally folded proteins that are produced in the bone marrow and can't leave the blood stream like normal waste products. In AL amyloidosis or light-chain amyloidosis, the type that I have, it is the build-up of these protein fibrils on the vital organs that reduces their function and stops them working properly. In most cases of AL, the main organs affected are kidneys, liver, heart and spleen. As it progresses, congestive heart failure, renal failure etc become the main risks and even with transplantation of new organs, they can be infected again if the production of amyloid hasn't been stopped in the bone marrow). In any event, it is pretty nasty and is treated with chemotherapy as a close cousin of Myeloma.
So, I'm Amongst 8 in A Million - But Does That Make Make Me Special?
Although they had detected Amyloid, the Doctors couldn't say which type it was, the extent of it and what my survival chances were. This would require a referral to the National Amyloidosis Centre at the Royal Free Hospital in London. First, we had to get a bone marrow biopsy organised with the Haematology Department.
This was organised immediately and I was introduced to my Haematology Consultant, Doctor McLintock, who carried out the procedure. Where the kidney biopsy was relatively painless, the bone marrow biopsy was very painful but for a small number of seconds.
In discussion with Doctor McLintock, she explained that survival rates for people with Amyloidosis were not very good at all. On average, life expectancy without treatment was 15 months; with treatment 5-10 years. Without knowing how far along the cycle I was, or the actual amyloid load in my body - and which organs had been damaged - it was too early to get a prognosis. This would only come after the visit to the NAC in London.
Apparently, Amyloidosis is very rare and approximately 8 people in a million are affected. In this country, the National Amyloidosis Centre gets a few hundred referrals each year, from across the UK and in the US, they pick up around 3000 per annum. Many people will have died without even knowing that they had it. No one knows how it's caused and, as yet, it is incurable. So, sometimes, you can feel special in belonging to a such an exclusive group of eight in a million people. At this time, I wanted to be with the majority...
Dealing With That News:
So, with this information, I phoned my wife, Audrey, and tried to give her the basic information that I'd just been given. Phones are not ideal in these circumstances but I knew that she was waiting to hear how the biopsy had gone. Inevitably, we were both shattered and struggling to cope with what we'd just been told.
I asked to be discharged from hospital there and then and went home, not really knowing how I was going to share this information with family and those close to me. For me, this was worse than hearing it for myself. Like everything else in life, if you are the person dealing with something, you know what's going on, every second of every day. For those who care about you, they can't know if you're suffering from one minute to the next (which isn't always the case) but they are forced to worry constantly. I hate being the author of that misery for anyone else.
The National Amyloidosis Centre - A Bridge Over Troubled Water
Within two weeks of leaving QM Hospital in Dunfermline, my son, Nathan and I were in London. We travelled by train, Inverkeithing to Kings Cross and it was a good, comfortable trip of only 4.5hrs. By sheer good fortune, the Royal Free Hosptial in Hampstead is located around 3 miles from the London branch of our family so we were fully supported by my cousins and Aunts whilst we were there. Although I was struggling with the symptoms of my illness - being sick, breathless on walking etc - I was still able to enjoy the hospitality and the fact that we weren't in a budget hotel made a huge difference psychologically.
However positive my attitude was - and I am, by nature, an extremely positive person, I would be lying to say that I wasn't scared of what I was going to find out. For two weeks, my thoughts had been see-sawing from bullish "get the 5-10years out of this and someone will cure it before then" through to "will Audrey and the kids be able to manage financially if I'm not around?" On the latter front, I wasn't sure and had to dig out the policies that you never think you'll ever look at...
The NAC is part of the Medical School of the University College London and Amyloidosis is what they deal with every day. Everyone, reception staff, nurses, clinical staff and consultants were all extremely pleasant, helpful and reassuring. Whatever the outcome was going to be, I felt calmed on entering, rather than panicked.
In terms of the set-up there, it is highly professional with what appears to be the best of equipment. The NAC is the only centre in the UK that possesses a full-body SAP Scanner, which is the scanner that detects how much amyloid is in your body and exactly where it's been distributed.
My visit, which is pretty typical for everyone who goes there, was split over 2 days, but only involved a few hours each. In addition to similar blood and urine tests that I'd had in QM, I was also injected with radio-active dye on Day 1 that would be used on Day 2 when I would have the SAP Scan. After the SAP scan, I would meet with the Consultant and get the verdict.
Lying in bed that night, I was strangely calm but curious to know how I would react to the news if it was really bad. Nathan would be the first person beyond myself to hear the information, even before his Mother. It occurred to me that he had shown remarkable courage in agreeing to escort me to London, knowing that this is what we were there for. At 19, I'm not sure if I would have been quite as calm and mature and reassuring to my Father as he was to me in those circumstances.
Day 2 arrived and we went back to the NAC. Again, I was well received, and wasn't kept waiting. My SAP scan took around half an hour and was pretty much like a typical MRI scan, through the donut shaped tunnel etc. No problems.
Then the meeting with Dr Julian Gilmore, who I found immediately to be a man with a passion for taking this disease on with his colleagues, with a view to nailing it completely. (I am convinced that they will do it and that that day is not too far away.) His manner was professional and friendly in equal measure and, whatever the news, I felt I was in the right place -with the right person - to hear it. Within 20 minutes of my scan, he had all the information from everywhere (previous test results from QM in Dunfermline, the latest blood and 24hr urine sample taken on Day1 and, of course, the all-important SAP Scan) available on his desk and PC.
So, the dialogue went something like this.
JG " So, you've probably done your own research on Amyloidosis, since getting the news at QM. As you know, there are different types. Yours is what we call AL Amyloidosis or systemic. The 'A' just stands for Amyloidosis, the 'L' refers to the fact that yours is systemic, being produced in the Bone Marrow is of the 'Light Chain' type.
ME: Nodding only. I had read up on all of this but, as I didn't know what I had, I didn't pay it too much attention.
JG: "What we know now is that you have a heavy load of Amyloid distributed throughout your body. So far, the damage is affecting your liver, kidneys, spleen and there are early indications that your heart is also involved."
ME: Another nod. Shit, this is not looking good, I thought. But would I be able to get 5-10years out of it?
JG: "You've probably been reading that, as Amyloidosis is an incurable illness at this time, the prospects are not very good. (This I had read - too many times!) However, in your particular case, I'm pretty confident that we can reverse this in you...
ME: "Sorry, reverse it?" (Up until this point, no-one had even mentioned the possibility.)
JG: "Yes. You see, your organs have been in pretty good condition to date and nothing has failed so far. If we can attack the production of Amyloid in your bone marrow using Chemotherapy, we can turn the 'tap' off and allow the existing Amyloid to eventually escape your body. We use an analogy of a sink to describe this process. If you imagine that you are a sink with a plug hole that has no plug, it is allowing some Amyloid to escape your body. However, at the top of the sink, a tap is running that is producing lots more Amyloid and filling up the sink faster than it can drain away. If the sink fills up, the vital organs will progressively fail and the condition inevitably becomes fatal for a lot of people. "
ME: "So, when the tap is turned off, how long does it take for the remainder to drain away?"
JG: "No-one can know for sure because everyone's 'drain-hole' is different. I would think, in your case, you would be getting back to fitness in about 2 years, fairly close to where you were before you got the diagnosis. The key thing is to get the Chemotherapy started as soon as possible. It will probably be the case that you will feel a lot worse before you start to feel better as your undergoing this."
ME: "How soon can I start the Chemo. It's Wednesday today and I'm due back at the QM on Friday to see the Haematologist. Could I start on Friday?" (To myself, "My God, My God, My God. A 'get out of jail card' - can this really be true? Weeping buckets inside - even writing about that moment now still stirs a similar emotion in me, such was its impact).
JG: "It'll all depend if they have the drugs available but I'll send an email today. It would be fantastic if you could start as soon as that. We will manage your treatment from here, in conjunction with your Haematologist\Oncologist. We'd like to see you back here in 3 months, to monitor the progress of the chemotherapy drugs and see if we need to adjust the mix to get the best results."
ME: "Thank you. This is amazing. "
A bridge over troubled water had been established and there was now a real chance that I would recover. If I hadn't been so ill, I would have danced out the room.
At this point, it would be a good idea for you to have a look at Dr Gilmore's video, explaining what Amyloidosis is and what hope is now there for people who may be coming to terms with news such as mine.
Since finishing the chemo in the summer, I determined to get some level of fitness back as a route to normalisation. So, from not having the strength to propel my borrowed wheelchair - just 7 months ago - I've been able to cycle again, and it's been great for building my stamina and confidence, I guess.
