National
Amyloidosis
Centre
News
Professor Philip Hawkins at the 7th Annual UKAN Meeting, presenting a summary of ongoing clinical trials at
the NAC
Introduction
On 23 February 2015 doctors from all over the UK gathered at the Royal Free
Hospital for the 7th Annual UK Amyloidosis Network meeting. Each year, the
meeting grows in popularity, with nearly 200 attending this year. After
spending a day hearing lectures from the NAC doctors and other specialists,
participants return to their hospitals all over the UK better informed about
amyloidosis diagnosis and treatment. We can see this reflected in the ever
increasing number of referrals to the NAC. In the special session held this year
called “Amyloidosis in the UK- A view from outside the NAC,” specialists from
Norwich, Bath, Liverpool and Birmingham reported on the advances they are
making in improving amyloidosis management in their hospitals. They all
emphasised that a multidisciplinary team ideally located in one clinic can
improve the patient journey and ongoing care. There was general agreement
that care should be delivered centrally (at the NAC) where necessary and
locally where possible.
In an update on AL amyloidosis, Dr Wechalekar from the NAC discussed the
gradual evolution of prescribing practices in the last few years. Bortezomib
(velcade) based chemotherapy regimes have become the most commonly
prescribed as evidence of efficacy has accumulated over the last few years.
Other subjects covered by NAC speakers included: light chain deposition
disease (an AL amyloidosis related condition); sleep apnoea, a newly identified
problem in AL amyloidosis; recent advances in ATTR amyloidosis and
challenges in cardiac amyloidosis. Guest lecturers from Greece and the US
gave their perspectives and the day concluded with an eagerly awaited update
on clinical trials from Professor Hawkins. He covered each of the ongoing
trials that are continuing to steadily add to our understanding of amyloidosis
(for more details, see www.ucl.ac.uk/amyloidosis/nac/clinical-research) and
conveyed cautious optimism about the future of the various new drugs that
are currently in development.
ISSUE 6: August 2015
IN THIS ISSUE
Introduction
1
Phase I trial of CPHPC
plus anti-SAP antibody
2
NAC online support forum
3
Local amyloidosis
support groups
3
Patient stories
Mark McConway
Ross Cunliffe
4
8
Fundraising news
Race4Ross
Holland in lycras!
10
11
Donations
12
National Amyloidosis Centre, UCL Division of Medicine, Royal Free Campus, Rowland Hill Street, London NW3 2PF, UK
www.ucl.ac.uk/amyloidosis
National Amyloidosis Centre News
2  Issue 6: August 2015
Phase I trial of CPHPC plus anti-SAP antibody
By Professor Sir Mark Pepys
The results of the initial part of the Phase I trial of the obligate therapeutic partnership of CPHPC plus anti-SAP
antibodies in patients with systemic amyloidosis were presented at the Annual Meeting of the Association of
Physicians on 27 March 2015, and have since been published in the prestigious New England Journal of Medicine
online on 15 July 2015.
This is the first clinical trial in patients of a first in class treatment that has never been used before. The main
aim was to show that the drug combination is safe and acceptably tolerated in patients.
Patients with clinical evidence of amyloid in the heart
were excluded from this first part of the trial for safety
reasons. We have substantial experience with CPHPC in
amyloid patients and it has always been safe but anti-SAP
antibodies have not previously been given. The initial
antibody doses were therefore very small but in the
absence of any ill effects, larger doses were given and
were generally well tolerated.
Molecular structure of the drug CPHPC in the complex with
SAP which is rapidly cleared, leading to removal of SAP from
the bloodstream
Most of the patients were selected to have amyloid in
the liver because there are several different powerful
methods for assessing both liver amyloid load and liver
function.
The important results of the trial so far are:
1.
The treatment is safe and well tolerated.
2.
After treatment with a sufficient antibody dose, there was evidence of reduction in amyloid load in almost
all subjects, associated with improved liver function in some.
There has never been any other treatment that directly targets systemic amyloid deposits or any intervention
which produces such rapid clearance of amyloid from the tissues.
The extent of amyloid clearance varied between individuals but seems to depend on the initial amyloid load and
the dose of antibody used. The results of ongoing studies in the trial continue to be encouraging. Plans for a
Phase II trial, aiming to confirm clinical efficacy and patient benefit, are in progress.
National Amyloidosis Centre News
Issue 6: August 2015  3
NAC Online Support Forum
The online National Amyloidosis Centre patient support forum has been up and running since the beginning of 2015. The
forum was the brainchild of John Plant, a patient with hereditary AFib amyloidosis, member of the UK Amyloidosis
Advisory Group and an avid cyclist. John’s experience as
administrator of his cycling group’s online forum led him to
suggest that a similar format could be of great value for NAC
patients. Sure enough the forum has been growing steadily in
popularity since its inception. New members join regularly,
some topics have already been viewed over 1,000 times and
we’ve received a lot of positive feedback from forum members.
The forum enables patients, family members and carers from all
over the country to meet and give each other support, practical
and emotional advice and encouragement. The feeling of
online community and mutual support is appreciated by many
people who had previously felt isolated coping with a rare
disease that no one they knew had even heard of. Everyone
finds it helpful to talk to people who understand what they’re
going through and benefit from others’ experiences. We hope
that the forum will continue to grow and to help more people to cope, communicate and help each other. Come and join
the conversations - http://amyloidosis.org.uk/forum.
Local Amyloidosis Support Groups
Some patients who attend the National Amyloidosis Centre are interested in meeting up in real life as well as on the online
forum. At this stage, small local support group meetings will be very informal – just a chance for patients, families and
carers to meet over a coffee, to get to know each other and to chat. The fledgling Scotland group had their first meeting in
April 2015 and their second in July. They plan on meeting again sometime in October. They hope to welcome more
members as word spreads.
Please contact the following organisers if you’re interested in hearing
more:
 Scotland
George  geordie_turner@hotmail.com
 Manchester
Lesley
 London
David
 lesley.toft@ntlworld.com
 davidgoldstone@btinternet.com
 Colchester, Essex
Sandra  sandrabl46@hotmail.com
The first meeting of the Scotland amyloidosis support
group: George Turner, Gail Hogg and Mark McConway,
founder members of the group
If you’d like to set up a group elsewhere, please contact
Miriam: miriam.pepys@ucl.ac.uk.
Myeloma UK groups – for patients with AL amyloidosis
The charity Myeloma UK also welcomes patients with AL amyloidosis at its support groups, which meet in around 60
locations around the UK. For more information see the Myeloma UK website: www.myeloma.org.uk.
National Amyloidosis Centre News
4  Issue 6: August 2015
Patient story: Mark McConway
Part I: From dialogue to diagnosis
“Tam, I can’t get my feet in my cycling shoes”
“Why not?”
“They’ve swollen up like balloons - and
I’m getting breathless cycling up the
wee hills.”

“What did the doctor say Mark?”
“Oh, I’ve got fluid retention - she gave
me Furosemide. I’ve got to have blood
tests. There was protein in my urine.”

“Sorry, why did you say I had to come
back in to the surgery?”
“Your blood haemolysed again before
it got to the lab. We need to do the
test again. This sort of thing happens
once in a blue moon but, in your case,
it’s happening every time we do it.
Might need to get you checked over
by a haematologist.”

“Dad, what’s wrong with your eyes?”
“What do you mean?”
“The whites of both of them are
completely filled with blood.”

“Hello Doctor, thanks for taking my call. My eyes are
bleeding.”
“What do you mean exactly - bleeding or bloodshot?”
“The whites are both red - almost completely.”
“That’s called a sub-conjunctival haemorrhage. You’ve
maybe coughed or sneezed too hard. By the way, could
you come back in to see me -your blood’s haemolysed
again.”
“OK. My legs are swollen now too - and I’m feeling sick all
the time.”

“Don’t worry, I’ll ask reception to postpone my next
appointment. This filling is going to take a bit longer than
I expected. For some reason, I’m finding it difficult to
stop your gums bleeding.”

“Are you okay in there? Are you still being sick? This
can’t go on! Shall I call a doctor?”

“Tell him he should just go to A & E. I used to be a nurse.
This is serious. He shouldn’t be vomiting blood like that.
Blood in his urine? Pains in the back? Swollen limbs? Are
you kidding?
“Oh my God, you look like you’ve
been in a fight. Your eyes are black
and blue. How did that happen?”
“I was being sick again.”

“Did you bang your face on the toilet
bowl?”
“No - being sick did this.”
“You must get to a hospital!”
“I can’t - I’ve got a meeting.”

“What did they say about your black
eyes at the meeting?”
“You should be in hospital!”
“They’re right, you know.”
“I know - we’ll go after dinner.”

“He’s not going home tonight. I think
you should arrange to bring some
clothes and things for him tomorrow.
A & E’s busy right now – so is the
entire hospital - so it might be a while
before we can find a bed.”

“Excuse me - nurse, can I go home please - I feel like a
fraud in here. These other people are really pretty sick.”
“You’re sick, Mark - we just don’t know why.”

Turns out I was the sickest of them all. Who knew?
Part II: The diagnosis
Amyloidosis. On 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. Living in a Fife village, close to
Dunfermline, I was also an enthusiastic leisure cyclist,
having started cycling at the age of 35 and doing it almost
every weekend since.
So, you see, probably just like you, I had no real reason to
worry about my health.
National Amyloidosis Centre News
Issue 6: August 2015  5
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 ...
decision was made to carry out a
kidney biopsy to get a tissue
sample analysed.
In November 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.”
This was the turning point.
By late January 2011, the prostate problem had gone
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 experienced in my life
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 became
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 a raised protein level
in my urine, which suggested 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
ankles/legs, etc), nothing was tying this up with the
enlarged liver, kidney problems, etc. Eventually, the
Biopsies and a diagnosis
The biopsy itself was relatively painless, if a little
uncomfortable. It was 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.
Returning 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 the doctor explained that this was a very rare illness,
and was potentially life-shortening, my horizons started
to cloud.
So I’m amongst 8 in a million – but does that
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, 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.
The haematologist explained that without knowing how
far along the disease 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 about 800 referrals each
6  Issue 6: August 2015
year. From across the UK and the US, they have 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 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 4½ hours. By sheer good fortune,
the Royal Free Hospital 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 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 5-10 years 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 insurance
policies that you never think you’ll ever look at ...
National Amyloidosis Centre News
The NAC is part of the Medical School of 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
calm on entering, rather than panic.
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 performs SAP scanning to
detect 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 radioactive 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.
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
imaging scan, through the donut shaped tunnel, etc. No
problems.
Then I met with Dr Julian Gillmore. 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 24 hour
urine sample taken on day 1 and, of course, the allimportant 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 systemic
AL amyloidosis. The ‘A’ just stands for amyloidosis, the ‘L’
refers to the fact that yours 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 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. Mmm, this is not looking good, I
thought. But would I be able to get 5-10 years out of it?
National Amyloidosis Centre News
Issue 6: August 2015  7
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 ...”
treated. If I hadn’t been so ill, I would have danced out
the room.
Me: “Sorry, reverse it?” (up until this point, no-one had
even mentioned the possibility).
Treatment for me involved swallowing a lot of tablets,
which took a bit of getting used to, especially as my fluid
intake was restricted to 1 litre per day. The principal
drugs
were
cyclophosphamide,
dexamethasone,
thalidomide and a fragmin injection once a day but I also
required frusemide and spironolactone to reduce the fluid
and domperidone for anti-sickness. In total, I would take
approximately 560 tablets every 21 day cycle for 5 cycles,
taking me up to mid-summer 2011.
JG: “Yes. You see, your organs have been in pretty good
condition to date and nothing has failed so far. There are
no guarantees, but 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 you’re
undergoing this.”
Me: “How soon can I start the chemo? It’s Wednesday
today and I’m due back at the QM in Scotland 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. 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.”
Part III: The drugs, the database
and the bike
In order to keep track of the tablets I was taking - and my
outcomes each day - I built a ‘cloud’ database and
recorded everything as frankly as I could. In retrospect, it
makes gruesome reading in places but demonstrates a
clear and positive trajectory as far as being on treatment
is concerned.
My taste was inevitably affected and most food became
inedible to me for a while. The one notable exception
was a pleasurable (if ghastly to the observer!) addiction to
roll-mop herring, pickled onion and beetroot.
Although uncomfortable to admit now, I was inspired
greatly by (now disgraced) Tour de France winner,
Lance Armstrong’s book, ‘It’s Not About The Bike’, where
he recounted his journey back from testicular cancer and
on to winning the TdF. I reasoned that if I could get back
on my bike, it would act as a measure of how I was
progressing - and a good measure of success would be
cycling back to London one day for one of my
appointments at the NAC.
As my light chain levels came down towards the normal
range, I was still very weak, having lost nearly 10 kg in
weight I could ill-afford to shed (60 kg is my normal
weight).
Three months after finishing the chemotherapy, I did my
first bike ride of 5 miles and, going into 2012, did a little
more each time. By the end of 2012, I had cycled 1000
miles, most journeys being around 25 miles.
Me: “Thank you. This is amazing.”
Like most amyloidosis patients, I still have days where
exhaustion takes a hold and I have to sleep. These are
becoming less frequent but no less significant when they
arrive. The trick, I’ve learned, is to accept that this is the
way that things are now - at least until a cure is found.
A bridge over troubled water had been established and
there was now a real chance that I could be successfully
And, from what I understand, that day might not be too
far away now.
National Amyloidosis Centre News
8  Issue 6: August 2015
In April 2014, Mark cycled 500
miles from Dunfermline to
London, raising awareness as
well as over £2000 for the
Amyloidosis Research Fund. The
June 2014 NAC newsletter
included an article about this
amazing achievement, which
Mark dubbed the “Big McCycle”.
Mark has remained well since
then and has become an active
member of the NAC online
support forum and a founder
member of the new Scotland
patient support group.
Patient story: Ross Cunliffe
From stem cell transplant to snowboarding!
decided to visit my GP. He knows me well and knew how
In 2013 I was 55 years old, had a great career in IT, a
focussed I was on my fitness, which helped him take me
loving partner, Elaine (now wife), and two wonderful
seriously.
grown up sons, Ryan and Toby. Pretty much a perfect life.
I was a really keen athlete
We then started a whole batch
and had been all my life,
of tests to see if we could work
playing football and cricket,
out what was going wrong:
running
and
swimming,
anaemia,
diabetes,
lung
snowboarding in winter and,
problems,
etc.
I
also
had
a
scan
most important of all to me,
of my bowels, which didn’t
cycling. I have been riding all
show any major issues but the
my life but started to race
consultant noticed an artery
quite late and I combined this
was enlarged and decided to
with
my
running
and
ask the cardiologist to take a
swimming to compete in
look.
He did an echotriathlons at various levels,
cardiogram
and ECG and
including competing in the
Ross (right) snowboarding with his brother in January 2015, after a
realised
my
heart was very
Ironman event in Regensburg
stem cell transplant in September 2014
large. He initially put this down
in 2012 (swim 4 km, ride
to
the
very
high
volume
of
training I was doing (my resting
180 km and run a marathon).
HR was always very low) but then saw that my heart was
also very stiff. He set up for me to have an MRI done in
I probably started to notice things weren’t right in very
London to explore this further and what happened next,
early 2013. I was getting dropped on the Sunday Club
although a huge cliche, really did change my life. He
Run by guys I would normally cruise past and I was just
copied me in on the letter to the MRI team and asked
generally starting to struggle. This didn’t improve and I
National Amyloidosis Centre News
Issue 6: August 2015  9
them to firstly try to exclude amyloidosis. Having never
heard this term before I casually googled it (don’t we all
do this ...?) and landed on the NHS site which simply said
this rare condition has no cure and a median survival
time of 7 months. Literally in a matter of seconds I’d
gone from being one of the fittest people I knew, with a
fantastic life, to facing a condition which seemed likely to
kill me. So what did I do next? Took the dog for a walk of
course!! I needed to keep calm and think of ways to
explain all this to Elaine. She goes bonkers if I scratch my
elbow falling off the bike, this was really going to make
her angry ...!
The MRI results came back and I was referred to the
National Amyloidosis Centre to do all the tests needed to
confirm this was cardiac AL amyloidosis. Pretty quickly I
was assigned to Dr Wechalekar and his team who started
me on a velcade based chemo regime. Initially this was
as an outpatient but it soon became clear that my heart
condition was bad and that it would be much safer to be
monitored throughout the chemo sessions, so I
essentially moved into the Royal Free Hospital which
became my home for the next 9 months. At the start of
the treatment my light chain count was very high around 2,500. This is a measure of the amount of ‘bad
protein’ being created - with a healthy person having a
level around 30 ... - we knew we had a bit of a challenge.
I was by now finding it very difficult to walk far or climb
the stairs and it really started to hit home that I was
actually very ill and couldn’t just shrug this off like I’d
always done before. One day, early in the treatment and
whilst still an outpatient, I was at home and checked my
HR (this is a habit from years of fitness training) and saw
it was up around 120 bpm. It stayed there for a few
hours so I called Dr Wechalekar who told me to come to
the RFH immediately. I was admitted and underwent a
cardioversion, where my heart had an electric shock to
attempt, thankfully successfully, to put it back in normal
rhythm.
Soon after this, Dr Wechalekar and my cardiologist,
Dr Whelan, asked to see my wife and me and, in a very
caring but professional way, warned us to be ready for
the worst. I’m pretty sure I didn’t fully understand this at
the time but, having discussed this with the team since, I
now know they were doubtful I would survive.
Nevertheless, we continued the treatment and, despite a
few more hiccups (at one stage, due to my blood
pressure being very low, I stood up too quickly and
passed out, knocking my front teeth out and smashing
my face quite badly - luckily, as it was my face nobody
noticed ...), we finally started to see improvements in
May/June and I was allowed to go home and, at least for
the moment, get a rest from the chemo.
Things were all looking good and it was decided I was
strong enough to have a stem cell transplant. I
understand that I was the first patient with AL
amyloidosis and heart involvement to have this
treatment in the UK. I was admitted in September 2014
and spent nearly a month in the RFH again, getting the
cells transplanted back and going through the early
recovery stages. This treatment was my very best option
and, although it really is a bit unpleasant, the potential
outcomes are easily worthwhile. I was discharged in
October and went home to start the long recovery
process. Initially I felt pretty rotten, all my hair fell out
(... again, no one noticed!!) and I was pretty sick. I
steadily improved and by Christmas was feeling quite
chipper. I even felt able to go off snowboarding in
January (see pictures) and start riding my bike again,
albeit quite slowly. Since then I’ve managed to do more
and more. I sometimes get a bit frustrated thinking back
to ‘the old Ross’, but not too often.
So where am I now? My condition hasn’t worsened and,
as yet, my light chain count is still low. I fully realise that
the disease will come back but I’ve come to terms with
that and (cliche alert) am happy to feel okay day by day.
For me personally, it really is all about how you approach
life and cope with the bad stuff. The only part you can
really control is how you react. You can let the condition
rule you and define everything that happens, or you can
try to accept that your life has changed, but it’s still your
life. You decide how you feel and how you act, not the
disease.
National Amyloidosis Centre News
10  Issue 6: August 2015
I am one of the luckiest people around; I’ve got an
amazing family and fantastic friends. I know, without any
doubt, without these people I wouldn’t have been able to
adopt this approach. They have been my scaffolding and
their love and care has been more important than all the
drugs. I’m willing to admit that this positive approach
isn’t there every day - I certainly have my moments of
sitting in the corner of the bathroom at 3.00 am crying
and wishing my life was different - but it isn’t! This is
probably pretty healthy but it cannot be your ‘steady
state’ because you then miss all the bloody good stuff!
Finally, I have to talk about the team at the NAC.
Without exception, they have really cared for me
throughout this process. Not only treated me but
genuinely cared. The decisions made by Dr Wechalekar
and Dr Whelan have kept me alive and I can never repay
that.
So, today the sun is shining and I’m about to go back to
the NAC for my usual tests etc. I’m tired and have got a
nagging toothache but, on balance, that’s pretty
acceptable given the alternatives!!
Fundraising News
Race4Ross
By Mark Atkinson & Peter Coleman
Back in 2013 our friend, colleague, iron man, fitness
fanatic and cycling addict, Ross Cuncliffe, started to feel
tired and run down - completely out of character.
Extensive tests couldn’t work out why and the situation
continued to deteriorate, until one doctor suggested they
look to see if this was being caused by amyloidosis. For
Ross this resulted in a diagnosis of cardiac AL
amyloidosis. With 6 months of chemotherapy treatment
in the first half of 2014, followed by a stem cell transplant
in September, Ross has, like many others, come through
this period with a relentless passion for life and
determination to fight through this disease as best as he
possibly could.
Having seen and heard from Ross about the support he
was getting from everyone at the NAC, and
understanding more about this largely unknown disease,
we felt compelled to give something back. We managed
to convince a group of friends and colleagues to come
together and raise some much needed funds to help the
NAC.
Mark’s race:
Eight of us took on the challenge of the Richmond 10 km
or the half marathon. Given that the group was starting
either from the camp of ‘haven’t run for years, my knees
are gone’ or ‘the furthest I’ve run is 10 km’, there was
challenge enough for all in setting our goals and
objectives for race day. In the end five completed the
half marathon and three the 10 km. Ross contacted us
the day before the event to say he had a chest infection
and really couldn't make it. Even though he wasn’t going
to be there, the motivation did not subside and the team
ran on a beautiful day, down round Kew Gardens and
along the Thames path. As we neared the end there was
a familiar voice cheering us home. Initially thinking I
might be slightly delusional after 13 miles, I realised that
Ross had made the trip to see us all home - just a small
example of the man he is and why we all did this. The
generosity of friends, family and colleagues raised just
over £7,000 for the NAC which is a fantastic result. More
challenges are already being planned to help the great
people who support those afflicted with this disease.
National Amyloidosis Centre News
Peter’s race:
Our friend is a huge cycling fan so we felt it was
particularly appropriate to take on the challenge of the
Mallorca 312, a 193 mile (312 km) cycle sportive that
loops around the Island, including all the major mountain
climbs. With a total ascent of 14,110 ft (4003 m), even
for a family of fitness fanatics like ourselves this was
daunting!
We all had a great, if not long, day in the saddle and my
wife Tam and I managed to finish our prospective rides
very successfully. The icing on the cake was my daughter
Sophie who was the first woman home and finished 92
overall (including the men) out of a total of 3000 riders.
She has subsequently been picked up by the UK cycling
press and articles written about her race,
http://tinyurl.com/ndzs8d7 & http://tinyurl.com/qh75rza
which has hopefully helped amyloidosis get a bit more
exposure.
Issue 6: August 2015  11
It’s interesting how getting fit for a challenge based
around raising money for amyloidosis research has ended
up with Sophie now cycle racing regularly in the UK.
We all really enjoyed this gruelling event and the fact we
were doing this for Ross made it inspirational. We all
knew that had he still been healthy he would have loved
to have done this challenge himself.
Holland in lycras!
By Julia Arrowsmith
Amyloidosis is not an everyday word. “Whatever is it?” is
the usual response when I explain that my brother has
what he calls the abominable twins, amyloidosis and
myeloma.
When Chris was diagnosed around this time last year, we
wondered what we could do to raise a worthwhile sum of
money and raise people's awareness of this little known
condition. We had reached retirement age without ever
tackling a sponsored event and, if there was a cause
crying out for us to do one, here it was.
Living in rural Shropshire, it was an easy decision not to
cycle from home - far too hilly for casual cyclists like us.
Sometimes the key to the future lies in the past and we
remembered the first holiday we had in our 1983
campervan and our bikes. We went to Friesland in North
Holland and pottered round lovely rural villages to our
hearts' content.
We would go again with two very dear friends, both of
whom had recovered from cancer. This time we would
photograph village signs beginning with the letters of the
word ‘amyloidosis’.
National Amyloidosis Centre News
12  Issue 6: August 2015
We started by arranging a couple of Dutch lessons with a
friend who, by an extraordinary coincidence, was born in
a village only five miles from where we were based. This
was surely a good omen!
Holland is a wonderful place to cycle. The country is so
cycle friendly - excellent cycle paths all marked clearly
on maps, cycle repair shops everywhere and, of course,
the land is so flat. It took us three days and just over a
hundred miles of gently cycling to take our eleven
photos. Some letters were a bit trickier to find but we
were helped by the fact that the signs are written in
both Friesian and Dutch.
On our return, it was so nice to be able to thank all the
good folk who had sponsored us - 94 at the last count.
We raised £3,550 for the Research Fund, alerted nearly
100 people to the condition and thoroughly enjoyed
every minute of it!
Donations
To ensure that your donation goes directly and exclusively to the NAC,
please contact Beth Jones on 020 7433 2802 or beth.jones@ucl.ac.uk, or send to the address below.
Research and development of new treatments for amyloidosis in
the Centre have made enormous contributions to understanding
of the disease. Availability of resources in the UCL Amyloidosis
Research Fund is of immense benefit for this research.
The resources provided by the UCL Amyloidosis Research Fund,
through the generosity of donors, undoubtedly makes major
contributions towards alleviating the suffering and saving the
lives of many thousands of people afflicted by this terrible
disease. Every penny is received with sincere gratitude and is
used specifically and in its entirety for amyloidosis research.
The UCL Amyloidosis Research Fund is a charitable fund within
University College London (UCL) which qualifies for Gift Aid. This
increases the amount donated by 25% without any extra cost to
the donor. It does so by allowing UCL to claim back the basic
rate tax paid by the donor. To qualify for Gift Aid the donor
must be a UK taxpayer and his/her combined income and capital
gains tax bill must equal or exceed the amount UCL claims back
on the gift. The gift can be for any amount and applies to
one-off gifts and regular gifts made over a number of years. For
UCL to claim the tax benefits the donor must complete a Gift Aid
form. To claim back the Gift Aid cheques should be made
payable to “UCL Development Fund”. This money is then
transferred to the Amyloidosis Research Fund, together with the
reclaimed tax. If a donor does not qualify for Gift Aid, or does
not wish to contribute in this way, cheques should be made
payable to: “UCL Amyloidosis Research Fund”.
Donations may be made online at:
http://www.ucl.ac.uk/amyloidosis/support-us
or by post. Gift Aid forms are also available online or from:
Beth Jones
National Amyloidosis Centre
Division of Medicine, Royal Free Campus
University College London
Rowland Hill Street
London NW3 2PF UK
 Newsletter funded by a bequest from Laura Lock 