Professor Sir Mark Pepys FRS FMedSci, Director of the
Wolfson Drug Discovery Unit, with the screen in the
background showing the appearance of amyloid deposits
viewed in a biopsy
NAC leads the way to improved amyloidosis
diagnosis in the UK
Early and correct diagnosis of amyloidosis is essential so that patients can
benefit from appropriate and timely treatment. As with any rare disease,
diagnosis first depends on physicians thinking of it, and then requires the
use of correct diagnostic techniques.
Doctors, scientists or technicians, who are expert at examining biopsy slides
under the microscope, are responsible for making the laboratory diagnosis
of amyloidosis. In order to detect and identify amyloid, biopsy slides must
be properly prepared, stained and examined. Each of these three stages
requires precise techniques, correct equipment and professional expertise,
or amyloid deposits may be missed (false negative result) or incorrectly
diagnosed when there is actually no amyloid present (false positive result).
The box on the next page lists the technical steps involved in histological
diagnosis of amyloid.
We see about 3,500 patients at the National Amyloidosis Centre every year,
including about 50% of all UK patients with systemic amyloidosis. Our
expert laboratory staff, led by Janet Gilbertson, also review hundreds of
biopsies annually in addition to our own patient throughput.
Professor Sir Mark Pepys began seeing patients with amyloidosis at the
Hammersmith Hospital in 1977. In 1986 Professor Philip Hawkins joined his
unit and together they soon provided the de facto national referral service
for amyloidosis in the UK. They subsequently established the UK National
Amyloidosis Centre at the Royal Free Hospital in 1999. Over the years the
expanding NAC team has been acutely aware of the worryingly high
frequency of both false positive and false negative histology reports from
elsewhere, based on incorrect use or interpretation of Congo red staining. A
recent audit by Janet Gilbertson found that both false positives and false
negatives in recent years, amongst biopsy specimens sent to her for review,
have run at about 8% in both directions. She was also surprised to discover
that about 20% of accredited histopathology laboratories do not even
possess polarising filters on their microscopes. Without these they cannot
possibly diagnose amyloid, as slides must be viewed through such filters in
order to make the diagnosis.
National
Amyloidosis
Centre
News
ISSUE 3: March 2014
IN THIS ISSUE
Improving amyloid
diagnosis in the UK
1
Patient story:
Dr David Webster
3
Focus on senile
Systemic amyloidosis
4
New equipment
purchased by the UCL
Amyloidosis Research
Fund
7
Fundraising news
8
Upcoming fundraising
challenge
10
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 3: March 2014
In mid-2013 Professor Sir Mark Pepys set out to address
the problem and to help patients by improving
amyloidosis diagnosis throughout the country. To pursue
this goal, he initiated collaboration between the NAC
laboratory team and the United Kingdom National
External Quality Assessment Service (UK NEQAS).
UK NEQAS comprises a network of quality assessment
and educational schemes and most histopathology
laboratories that wish to be accredited are signed up and
participate in these schemes. The objective of UK NEQAS
is to help ensure that the results of investigations are
reliable and comparable wherever they are produced. In
2014, Janet Gilbertson and Dr Glenys Tennent will be
actively engaged in the amyloid modules of the
UK NEQAS scheme. Dr Tennent is the Senior Lecturer in
the Wolfson Drug Discovery Unit and a world expert in
histological diagnosis and characterisation of amyloid
with over 30 years of experience.
• Written an article highlighting the reasons behind the
Centre’s involvement in the scheme, and detailing
best practice for amyloid diagnosis, for inclusion in
the next UK NEQAS newsletter which is distributed to
over 400 cell pathology laboratories nationwide.
Over the course of 2014, Dr Tennent will direct and
advise assessors and participants in several UK NEQAS
training activities. She will advise course leaders during
training courses, direct and advise assessors as they
review thousands of slides. She will also give a lecture
about amyloid diagnosis at the UK NEQAS national
meeting in autumn 2014.
In Professor Sir Mark Pepys’s words, “making the
diagnosis months or years earlier in even a few of the
many amyloidosis patients whose diagnosis is long
delayed by poor histopathology every year, will definitely
save lives and reduce suffering and costs”.
We hope to eventually ensure that every diagnostic
histopathology department in the UK knows how to stain
with Congo red so that the dye is as specific as possible
for amyloid deposits. Crucially, they also need to all be
equipped with polarising filters and to be sufficient
experienced to recognise and identify amyloid. We are
optimistic that the planned activities will help to bring us
closer to this goal, thereby improving standards of
amyloid diagnosis and benefitting many patients.
To date, they have:
• Updated the UK NEQAS Amyloid Staining Assessment
Criteria.
• Provided standard amyloid biopsy material for
distribution to all UK diagnostic laboratories
participating in the scheme, for assessment of their
amyloid staining techniques.
Technical steps involved in the laboratory diagnosis of amyloid

1.
Biopsy or resected tissue specimen is brought to
the lab.
2.
The tissue is fixed and embedded in paraffin to
preserve it.
3.
Sections are cut from the fixed tissue – section
thickness must be correct.
4.
The sections are placed on a slide and stained
with Congo red dye – this must be done very
precisely to ensure reliable results.
5.
The stained sections are mounted in a
microscope and examined under strong cross
polarised light – this requires special polarising
filters in the microscope.
Bright green appearance of amyloid deposits when they
have been prepared, stained and viewed correctly
National Amyloidosis Centre News
Issue 3: March 2014  3
Patient story: Dr David Webster
The story starts with an abnormal routine
ECG
I had been a keen sportsman since my early school days,
focusing on rugby at university and medical school, and
then starting to play regularly again in my early thirties at
quite a high level and then in more social teams until I
was forty-eight. I regarded myself as physically fit during
my fifties although I developed arthritis in my right hip
that was surgically replaced, allowing me to continue an
active lifestyle with regular swimming, gym sessions and
winter skiing. By my mid-sixties the other hip had
deteriorated and I was heading for
more surgery in 2007 when my
‘amyloid’ story really started.
As a hospital consultant physician, I
immediately realised the potential
implications when a routine ECG
revealed a conduction defect (left
bundle branch block) in my heart
during a pre-operative assessment
for hip replacement. This was a big
surprise as I considered myself very
fit for my age. I had noticed some
recent slight shortness of breath on
reaching the top of the four flights
of stairs to my office over the
previous few months, but had put
this down to advancing age. My
surgery was postponed while a
series of investigations were done
over the next six months, undoubtedly speeded up by
initially seeing a cardiologist privately and having some
expensive tests done quickly.
My cardiologist, a
consultant colleague at the Royal Free Hospital where I
worked as a Professor of Immunology, initially thought
the cause was probably ‘heart strain’ due to high blood
pressure, but this was excluded after being fitted with a
24-hour monitoring system. I remember him mentioning
very rare causes of heart conduction defects, one of
which was amyloid, but he thought this extremely
unlikely as the rest of my body seemed in good working
order. However an echocardiogram was suggestive of
amyloidosis, as it showed a thickened heart wall with a
suspicion of muscle infiltration. A cardiac MRI scan was
then arranged at the Royal Brompton Hospital which I
gather was the only place in London at that time with a
suitable machine, and where research was being done to
find out if cardiac amyloid could be diagnosed using this
non-invasive procedure.
Confirming the diagnosis
Meanwhile it was considered safe to proceed with my
hip replacement. However, the day before surgery I had
a phone call from my surprised cardiologist who told me
that the MRI was typical of amyloidosis. Although
encouraged to proceed with the surgery I reckoned my
anaesthetist would ‘abandon ship’ when I told him I had
amyloid cardiomyopathy, so I urgently contacted
Professor Hawkins in the NAC for his opinion and was
advised to postpone surgery again until further
investigations were completed. An SAP scan at the NAC
showed I had no amyloid
infiltrating other organs so the
diagnosis was still in doubt and
could only be confirmed by a
cardiac biopsy. My doctors were a
little reticent in advising this
procedure because there was no
cure if the diagnosis was
confirmed.
However, I prefer
certainty to uncertainty so I went
to
the
Heart
Centre
at
Harefield Hospital where Dr Banner
and his impressive team took small
pieces of muscle from the left side
of my heart with a tiny ‘grab’ on
the end of a wire threaded through
an artery in my leg, all under local
anaesthetic. Special tests at the
NAC on the biopsies confirmed TTR
amyloid, but tests on my DNA found none of the rare
inherited gene defects known to cause this condition.
This left me with the official diagnosis of ‘senile systemic
amyloidosis’, a label that does not instil much confidence
in the future!
Experimental treatment and then ‘heart
block’
As a specialist familiar with medical research on novel
diseases, I quickly grasped that I was one of the few
patients in the UK diagnosed while in relatively good
physical health, and that any attempts at treatment
would be experimental, with the side effects possibly
being worse than the disease. Furthermore nothing
much was known about the natural progression of
‘senile’ TTR amyloid, making it difficult to assess the
impact of any new treatments. This is one of those
situations where it is best not to be a medically qualified
patient as ‘ignorance can be bliss’! However, having
National Amyloidosis Centre News
4  Issue 3: March 2014
spent a career persuading patients to take part in various
drug trials, I saw the humorous side of having to step
forward myself! I started taking diflunisal, a drug
originally developed for arthritis but more recently found
to stabilise TTR and possibly prevent it from entering the
heart, with the immediate upside being an improvement
in my left hip which was still awaiting surgery. Nothing
much changed over the next year but then suddenly I
began to get pain in my abdomen after walking and my
ankles started swelling. After an urgent admission to
hospital complete ‘heart block’ was diagnosed, my pulse
having fallen to only 30 beats per minute.
A
pacemaker/defibrillator was inserted under the skin on
my chest and I was immediately back to ‘normal’. The
pacemaker also checked my heart activity and rhythm,
transmitting this information regularly to my
cardiologists through a monitor attached to my home
telephone. Having got over the initial sensation of being
‘controlled from outer space’ I felt more secure and less
likely to die suddenly from a cardiac event than others of
my age. This episode finally convinced my wife that
there really was something seriously wrong with my
heart!
I was now in a lot of pain from my hip so at last this was
successfully replaced in early 2011. About 18 months
later the cardiac monitor showed episodic atrial
fibrillation and I was advised to start on anti-coagulants;
this meant I had to stop the diflunisal which anyway
seemed to have had little or no impact on my disease. I
managed to persuade my doctors to prescribe one of the
new anticoagulants, dabigatran, which suited my busy
life-style better than the traditional warfarin which needs
regular monitoring of dose.
More information needed for doctors and
public
At my age you start to accumulate old friends with
cardiac pacemakers and abnormal heart rhythms, and I
often wonder whether their doctors have done the
appropriate tests for ‘senile’ TTR amyloid; I suspect not
but am too polite to enquire! My experience of major
and minor (a hernia repair) surgery over recent years is
that anaesthetists ‘run for cover’ when you tell them you
have cardiac amyloid. Although my hip surgery was
uneventful they did take fright and called in a specialist
cardiac anaesthetist, and kept me closely monitored in a
high dependency unit for 24 hours afterwards. This was
probably unnecessary and research is needed to assess
the surgical risks in my type of amyloid so that
anaesthetists can be more informed and confident.
Six years from diagnosis
I am now close to being seventy four, and six years from
the initial diagnosis of my cardiac amyloid. Despite some
deterioration in heart function requiring a pacemaker,
anti-coagulant pills, and a couple of other daily tablets to
stabilise and reduce the strain on my heart, my exercise
tolerance has not significantly changed and I have been
able to continue doing medical research. The only thing I
notice is a transient feeling of exhaustion and muscle
fatigue when I suddenly move from a sitting position to
walk or go up two flights of stairs, as if my heart is slow
to get into gear. Once I am moving I can walk or cycle for
miles at a steady pace. The specialists at the NAC tell me
that clinical trials on some exciting new drugs for TTR
amyloid will start soon, so perhaps I will be climbing
Everest at the age of eighty after all!
Focus on
senile systemic amyloidosis
In senile systemic amyloidosis (SSA) a normal blood
protein called transthyretin (TTR) is the amyloid
precursor protein that clumps together and forms ATTR
amyloid deposits, mainly in the heart and often also in
the part of the wrists called the carpal tunnel, causing
wrist pain and tingling called “carpal tunnel syndrome”.
Senile systemic amyloidosis is not an inherited condition
(does not run in families). Symptoms usually start over
the age of 65 and the disease usually progresses slowly.
It is far more common in men than in women and may
affect people from any ethnic background.
Other types of ATTR amyloidosis are inherited, affecting
people with genetic alterations (mutations) in the TTR
gene. People with these mutations have structurally
abnormal, amyloid-forming (amyloidogenic), “variant”
TTR in their blood.
People with senile systemic
amyloidosis have only the normal, “wild-type” TTR in
their blood, and do not have any abnormal “variant” TTR.
National Amyloidosis Centre News
Issue 3: March 2014  5
More common than we thought
Nobody knows how common senile systemic amyloidosis really is. Small amyloid deposits consisting of “wild-type” ATTR
amyloid are very common, found in the hearts and blood vessels of 1 in 4 people over the age of 80 in autopsy studies.
Until recently it was believed that this type of amyloid deposit was hardly ever extensive enough to cause symptomatic
heart disease. But in recent years, new cardiac imaging techniques (DPD scans and cardiac MRI) have shown that, in fact,
“wild type” ATTR deposits causing senile systemic amyloidosis may be a far more common cause of heart disease than
anyone thought.
The graph here shows the diagnoses of new amyloidosis patients seen at the NAC each year since 2000. The overall
number of patients seen at the NAC has more than doubled in this time, with AL amyloidosis remaining the most common
diagnosis, and accounting for about half of
all patients diagnosed.
Relative
proportions of patients with most types of
amyloidosis have remained fairly steady.
But the rate of senile systemic amyloidosis
diagnosis has shot up dramatically, as
shown by the purple coloured area on the
graph.
A graph showing diagnoses of new amyloidosis patients seen at the NAC each year since 2000
In the year 2000, just one patient seen at
the NAC had senile systemic amyloidosis,
and in 2012, 62 patients were diagnosed
with this condition.
Nobody knows
whether this just represents the tip of the
iceberg. The coming years could see a
continuing increase in the frequency with
which senile systemic amyloidosis is
diagnosed.
Even though there is no specific treatment for senile systemic amyloidosis at present, patients benefit greatly from having
the correct diagnosis, as their doctors can then avoid prescribing unhelpful drug therapies that are used in “ordinary”
heart failure. There are also several specific treatments for senile systemic amyloidosis under development at present
(discussed below). It is hoped that patients will soon be able to benefit from these.
What
causes
amyloidosis?
senile
systemic
The cause of senile systemic amyloidosis is unknown –
we do not know why “wild-type” TTR, which is a normal
blood protein, forms amyloid deposits in some people
and not in others although advancing age is undoubtedly
a risk factor.
Symptoms
ATTR amyloid deposits in the heart muscle may cause no
symptoms at all if they are small. But when amyloid
deposits in the heart are large, they can lead to stiffening
of the heart muscle, called “restrictive cardiomyopathy”.
When the heart muscle is stiff, the heart is unable to
pump the blood around the body as efficiently as usual.
Symptoms of heart failure may then appear, including:
• breathlessness, sometimes just after mild exertion
• abnormal heart rhythms – most commonly atrial
•
•
•
•
•
•
•
fibrillation and atrial flutter
palpitations
swelling of the legs
weight loss
nausea
dizziness and collapse (syncope)
disrupted sleep
fatigue
Breathlessness may be worse during exercise or when
lying flat at night. Patients may feel more comfortable
propped up with several pillows.
At the time of diagnosis, heart failure symptoms are
usually less severe in patients with senile systemic
National Amyloidosis Centre News
6  Issue 3: March 2014
amyloidosis than in those with AL amyloid deposits in the
heart (AL amyloidosis is the other common type of
amyloidosis which may affect the heart). However,
abnormal heart rhythms are common in senile systemic
amyloidosis and some patients may need a pacemaker.
Fluid balance
Around 50% of patients with senile systemic amyloidosis
also experience carpal tunnel syndrome - tingling and
pain in the wrists, pins and needles in the hands. Carpal
tunnel syndrome often appears 3-20 years before the
symptoms of heart disease and in these individuals it is
caused by ATTR amyloid deposits in the wrists.
Fluid excess can be avoided by careful attention to the 3
Ds:
Nearly one in ten of the patients seen at the NAC
because of senile systemic amyloidosis have experienced
no symptoms but were found to have amyloid
incidentally on routine testing or at the time of an
operation for a different complaint.
Which tests can help to diagnose senile
systemic amyloidosis?

The most important principle of treatment for cardiac
amyloidosis is strict fluid balance control. Patients
should limit their fluid intake.
1.
2.
3.
Diet:
•
fluid intake should be steady and should usually
not exceed 1.5 litres per day
•
salt intake should be strictly limited
Diuretics:
•
diuretics (water tablets) such as furosemide
and spironolactone help the body to clear
excess salt and water via the urine
•
removal of excess fluid from the body leads to
reduced ankle swelling and breathlessness
Daily weights:
•
every litre of excess fluid weighs one kilogram
and several litres can accumulate in the body
without it being very noticeable
•
some patients should weigh themselves daily to
identify fluid gain early
ECG
Echocardiogram
Cardiac MRI (CMR) scan
Radionuclide imaging (DPD scan)
Blood tests: “cardiac biomarkers”
(NT-proBNP and troponin)
Genetic tests
Heart biopsy
Abdominal fat / rectal biopsy
For more details on each of these tests, see the patient
information leaflet on senile systemic amyloidosis,
available from the NAC or at
www.ucl.ac.uk/amyloidosis/nac
Some patients may benefit from other measures such as
support stockings for leg oedema (swelling),
anticoagulation for patients at risk of blood clots and
pacemakers for patients with slow or irregular heart
rhythm.
Very rarely, heart transplantation may be an option for a
patient who presents at an unusually young age (before
age 60). Most patients with senile systemic amyloidosis
are too elderly to undergo heart transplantation as the
risks of complications are high.
Outlook
Principles of treatment
Treatment of senile systemic amyloidosis is symptomatic
and supportive for most patients. Standard heart failure
medications are often not effective for patients with
amyloid deposits in the heart, but the following
measures can be very helpful:
Senile systemic amyloidosis progresses slowly. A recent
study at the NAC found that most patients with senile
systemic amyloidosis survived for over 6 years after
symptoms started.
Furthermore, a number of new drugs for ATTR
amyloidosis are in various stages of development. These
drugs are not yet available, but they do offer hope for
the future.
National Amyloidosis Centre News
New drugs in development for ATTR
amyloidosis
Diflunisal:
This is a “non-steroidal anti-inflammatory drug” (NSAID),
a drug class in common use as pain killers, for conditions
such as arthritis. Diflunisal is bound by TTR in the blood,
which may make TTR less amyloidogenic. However, this
drug may have serious side effects and diflunisal use for
ATTR amyloidosis is an ‘off-label’ indication so only
amyloidosis specialists should prescribe it.
Tafamadis:
Tafamidis was developed as a specific drug for ATTR
amyloidosis. It is bound by TTR in the blood, stabilising
the TTR and making it less amyloidogenic. Tafamadis has
been approved in Europe for polyneuropathy caused by
hereditary ATTR amyloid. But since the evidence that it
has a beneficial effect on polyneuropathy is not strong, it
has not been approved by the UK NHS or by the FDA in
the USA. Tafamadis has not been tested in senile
systemic amyloidosis and has not received approval for
this indication.
Genetic therapies:
Small interfering RNA and antisense oligonucleotides are
therapeutic approaches that aim to “switch off” the gene
for TTR in liver cells, so that TTR is simply not produced.
A drug called ALN-TTRsc, which belongs to the small
interfering RNA drug class, was shown to reduce blood
Issue 3: March 2014  7
levels of TTR by up to 94% in healthy volunteers.
ALN-TTRsc is currently undergoing preliminary clinical
trials in patients with ATTR cardiac amyloidosis, both
senile systemic amyloidosis and the inherited forms of
ATTR cardiac amyloidosis.
ISIS TTR Rx belongs to the antisense oligonucleotide drug
class. ISIS TTR Rx is currently undergoing trials in patients
with familial amyloid polyneuropathy, a hereditary type
of TTR amyloidosis. Current trials are only assessing the
impact of this drug on nerve damage caused by TTR
amyloid. It is not being tested at present in patients with
senile systemic amyloidosis.
Antibody mediated amyloid elimination:
Serum amyloid P component (SAP) is a normal blood
protein, present in everybody, which is always present in
amyloid deposits of all types because it binds strongly to
all amyloid fibrils. The Wolfson Drug Discovery Unit has
developed a drug called CPHPC, which clears all the SAP
from the blood but leaves some SAP bound to the
amyloid deposits. After CPHPC has been administered, it
is therefore safe and feasible to administer antibodies to
SAP which target the amyloid. In experimental models
these antibodies trigger the body’s normally very
efficient systems for removal of debris from tissues to act
on the amyloid.
This approach is currently being tested in patients with
amyloidosis, in collaboration with GlaxoSmithKline. If it
proves to be safe and effective in humans, it will be
applicable to patients with all types of amyloidosis
including senile systemic amyloidosis.
New equipment for studying protein folding purchased by
the UCL Amyloidosis Research Fund
Amyloidosis is caused by the formation of abnormal amyloid fibril deposits within the body. One
of the unsolved mysteries of amyloidosis is the question of how these fibrils all have similar
structures, regardless of which of the original, highly varied “precursor” proteins they are formed
from. The Wolfson Drug Discovery Unit has recently acquired a new, state-of-the-art, highly
sophisticated piece of equipment, an MOS-500 fast modular spectrometer/polarimeter, from BioLogic. Researchers believe this will help to improve our understanding of this conundrum.
The new spectrometer was purchased with funds from the UCL Amyloidosis Research Fund and it
is already proving invaluable in helping us unravel the processes whereby different “globular”
proteins unfold in the early stages of their journey to becoming amyloid fibrils.
Dr Patrizia Mangione, working in Professor Vittorio Bellotti’s Protein Misfolding Group, is shown
on the left using this new instrument. Until recently she had to travel to Pavia in Italy to have
access to this technology. Patrizia says that the new insights into amyloid fibril formation gained
from our new equipment will help to bring us a step closer to understanding how amyloid fibrils
form, and thus help us in developing new medicines to treat our patients with amyloidosis.
National Amyloidosis Centre News
8  Issue 3: March 2014
Fundraising News
Father and daughter’s sky-dive in aid of the UCL Amyloidosis Research Fund
By Pat Pinchin [based on information provided by Tina Smith]
The Smith family from Barbaraville, Easter Ross in the Scottish Highlands, were so devastated by the death from AL
amyloidosis of father and grandfather Kenneth Rudkin, that they set about
organising a charity event to raise money for the Amyloidosis Research Fund.
Kenneth, shown in the picture on the left before he became ill, was father to
Tina Smith and grandfather to her daughters.
Tina recalls her father as a very kind person who would always do anything for
anyone. He loved reading war or spy books and she kept some of his books so
that her daughters could enjoy reading them.
Kenneth had been unwell for about 10 years with symptoms that had mystified
his doctors but had not been diagnosed as amyloidosis. He was uncomplaining
and reluctant to seek medical advice, but eventually a frightened Tina
intervened. His frequent bouts of uncontrollable diarrhoea, tiredness and
breathlessness led him to various consultants at the local hospital in Inverness
but it took until November 2012 before suspicion of amyloidosis was raised.
The consultant explained that amyloidosis was an illness he knew little about and referred Kenneth to the NAC. None of
the family were able to accompany Kenneth on the flight to London. In the company of a carer he went as an emergency
patient to the NAC for tests and the special SAP scan. Amyloidosis was confirmed but sadly by this stage, the disease was
advanced and Kenneth was too weak for treatment. He was flown back to Scotland and died in Edinburgh Royal Infirmary
on 2 January 2013.
Kenneth’s family were in shock at the death of their much loved father and
grandfather from a rare and incurable disease they had never heard of. They
learned that their experience was not uncommon because of the relative
rarity of the disease. When patients present with the range of sometimes
vague symptoms that amyloidosis can cause, doctors often do not think of
amyloidosis. The family promptly decided to do something positive to raise
money for the Amyloidosis Research Fund. Their aim was to support the
research into new treatments for the disease but also to help promote
awareness so as to lessen the frequency of similar late diagnosis experiences
for others.
One of Kenneth’s granddaughters, Alex, aged 18, is a keen member of Air
Cadet Squadron 378, Easter Ross, based in Alness where she lives. Alex’s Dad
Paul decided to join her in a 10,000 foot sponsored jump from an aeroplane.
Their courageous tandem jump took place at St Andrews in Glenrothes, with
full support from Alex’s squadron. The jump was an exhilarating experience
for both Alex and Paul and a very proud one for Mum Tina. A total of
£600.00 was raised for the Amyloidosis Research Fund. Now Tina is
considering an adventurous challenge for herself to continue fund raising for
amyloidosis.
After the event, Paul said: “The skydive was an amazing experience. It is something I have always wanted to do. As my
father-in-law died of amyloidosis earlier this year, the sky-dive was a great opportunity to promote the awareness of the
disease and to ask friends, family and colleagues for donations to the Amyloidosis Research Fund." Alex said: “I've always
wanted to do a skydive, so I did by raising awareness and money for amyloidosis in memory of my Grandad”.
National Amyloidosis Centre News
Issue 3: March 2014  9
Fundraising walk in Durham
By Malcolm Glass
My wife Janice and I were on holiday around 6 years ago
when she began struggling with her breathing on a short
uphill walk. After number of hospital visits and tests she
was diagnosed with “AL Amyloidosis” which had affected
her heart. Janice was eventually referred to The National
Amyloidosis Centre (NAC) in London.
finished 10 hours 18 minutes later, starting and finishing at
Wolsingham, a small village in Weardale. I was pleased to
receive a certificate and medal for my achievement, not to
mention the welcome pie and peas after a very wet day.
The terrain is beautiful countryside including open moors
and forests. Walkers use public rights of way, and land
where access has been given for the event. It rained most
of the day and conditions were not great, but everyone
enjoyed it and the spirit of the walkers and mutual
encouragement was terrific on the day.
Drinks and help were offered at various points, and we
were pleased to be given delicious cakes and sandwiches
half way around. It was a hard day but very rewarding
especially when doing it for such a good cause - helping
Janice and other sufferers with this cruel disease.
On our last visit to the NAC for Janice’s check-up, I was
inspired by reading notices on the wall there about the
various fundraising events that were taking place and
decided to do something to raise funds for the Amyloidosis
Research Fund.
I am keen on a variety of sporting activities so I signed up
for the June 2013 Durham Dales Challenge, an annual
challenge walk which takes place in Weardale in County
Durham. The walk is organised by members of the Durham
branch of the long distance walkers association (LDWA
http://www.ldwa.org.uk/challenge_events/show_event.ph
p?event_id=11128), and walkers check in at each
checkpoint along the way. There is a choice between
walking 15 or 30 miles within 12 hours. Around 300 of us
set out on 22 June 2013. We started at 9.00 am and I
I managed to raise £500 for the Amyloidosis Research Fund.
This was mainly from family and work colleagues. There are
often charity events taking place at work so asking for more
is always difficult. My daughter Michelle set up my fund
raising page for me through www.justgiving.com which is a
very easy way to generate voluntary contributions. I
received a lot of encouragement and support from my
family, particularly my two daughters, Heather and
Michelle, who follow the progress of the work being carried
out by the NAC and the many fundraisers. For me, this
event was a start of what I hope will be more fundraising
events for the NAC. I am certainly keen to do more and
urge others to feel inspired to take part in charity
fundraising events for the Amyloidosis Research Fund to
help the scientists and doctors discover new treatments to
improve the lives of sufferers.
For more information on the LDWA (Long Distance Walkers
Association) see the website : www.ldwa.org.uk.
“Cornwall Tor” charity ride
By Pat Pinchin
“With a bit of luck someone will have nicked me bike” said
one competitor to Miles and Patrick Pinchin before the start
of the North Cornwall Tor. Patricia, wife to Patrick and
Mum to Miles and Chloe suffers from AL amyloidosis.
According to the Met office, it was the wettest day of the
year! Just 367 of the 1000 entrants started, and Miles and
Patrick were among the hardy who set off. For safety
reasons the organisers shortened the 75 mile course and
Miles and Patrick completed approximately 52 miles.
Unable to take advantage of the greasy downhill stretches
to give them momentum on the climbs, they managed to
keep pedalling upwards past those who had succumbed to
walking. In spite of being well insulated with warm
clothing, on reaching Tintagel with temperatures of
5/6 degrees, but which felt more like below freezing, they
National Amyloidosis Centre News
10  Issue 3: March 2014
were so cold and wet that they downed a welcome hot
chocolate break in a cafe. Many were suffering from
hypothermia. When a minibus driver entered the cafe and
called out, “I've got room for 15”, he was almost knocked
to the ground in the stampede! Some riders called up
taxis on their mobile phones. With dogged determination,
Miles and Patrick reached the wilds of Bodmin Moor. The
north-easterly gale proved a welcome tail wind as they
“flew” along the homeward stretch to the finish. “In 50
years of cycling” said Patrick, “this is one of the hardest
rides I've ever done; on a par with the fearsome massed
start races in Scotland I rode in my 20s!” This is from a
seasoned rider who has spent a life-time of racing in time
trials from 10 miles to 12 hours!
So - well done to father and son duo Patrick and Miles, for
rising to the challenge and finishing comfortably in the
bronze medal category. With tired legs, feeling “stuffed”,
sodden clothes and shoes in bin bags but none the worse
for wear, they arrived home ready for a soothing hot
shower. They raised £1,335.00 for the Amyloidosis
Research Fund.
An upcoming fundraising challenge:
LEJoG or End to End
The End to End (Land’s End to John O’Groats) is one of the classic long distance cycle challenges. It is called LEJoG if
ridden North, and JoGLE if ridden South. There are many routes and it has been cycled, walked, hopped, roller skated
and even driven on lawn mowers!
In July 2014 a team from the National Amyloidosis Centre will be riding the LEJoG. At the moment the team comprises
Thirusha Lane (Lead Nurse) and John Plant (a patient with AFib amyloidosis), shown in the picture above. It is hoped that
other members of staff, patients or relatives might also join. A few have already committed to ride with the team for a
day or so. The route they will take will be over 1,000 miles, and they will climb nearly 16,000 meters (almost twice the
height of Mount Everest). Each day they will cycle an average of 71 miles with the longest day at least 83 miles. They
will start riding at Land’s End on the 6 July and will finish at John O’Groats on the 19 July.
National Amyloidosis Centre News
Issue 3: March 2014  11
Why are we doing this?
The ride has a number of objectives including: raising money for the
Amyloidosis Research Fund through sponsorship, providing an opportunity
for patients and relatives to meet up while supporting the team, and
importantly, raising amyloidosis awareness across the UK. We hope to use
local media in the main towns and cities that the route takes to create a
“ribbon of awareness” that stretches from one end of the UK to the other.
How can you help?
1.
Would you like to ride with the team? If so, make contact with
John Plant (john.h.plant@gmail.com) and he can explain what is
involved. This route is suitable for someone with a good level of fitness
and who wants a challenge. If you do plenty of training beforehand you
should have no problem completing the ride.
2.
Would you like to join for part of the ride? Having extra people along to
lend support would be great. Again, if you are interested please contact
John Plant. You don’t need a carbon fibre racing bike to do this - many
have ridden successfully and comfortably on light hybrid bicycles.
3.
Would you be prepared to meet the team as it comes through major towns on route? This is where we really need
the help of patients and relatives. By having a story to tell with local interest the team will contact local media
(newspapers, radio, and television) and will arrange to meet groups of supporters to spread news about amyloidosis.
These events will also give patients and relatives an opportunity to meet one another. If you are able to meet the
team as they ride through a town near you, and if you would be happy to share your story, please get in touch. The
more media coverage we can get the more people will become aware of amyloidosis and the work of the NAC.
4.
Perhaps you could help by raising sponsorship? We will set up a website and a JustGiving Page. The website
http://rideonforamyloidosis.blogspot.co.uk will keep everyone up to date with news of plans, preparations and
progress. It will also be a great way to share information with supporters. JustGiving provides a simple and effective
method to manage sponsorship.
Where will the team be?
Day
1
2
3
4
5
6
7
8
9
10
11
12
13
14
/
Date
Start
6 July
7 July
8 July
9 July
10 July
11 July
12 July
13 July
14 July
15 July
16 July
17 July
18 July
19 July
Land’s End
Fowey
Morton Hampstead
Glastonbury
Monmouth
Clun
Runcorn
Conder Green
Keswick
Moffat
Balloch
Glencoe
Inverness
Lairg / The Crask
Passes
Penzance
Plymouth
Exeter
Wells / Chepstow
Hereford
Shrewsbury
Warrington
Lancaster / Ambleside
Carlisle
Glasgow
Crianlarich
Spean Bridge
Bonar Bridges
Altnaharra
Ends
St Austell
Taunton
Bristol / Tintern
Chester
Bolton
Windermere
Gretna
Fort William
Thurso
Fowey
Morton Hampstead
Glastonbury
Monmouth
Clun
Runcorn
Conder Green
Keswick
Moffat
Balloch
Glencoe
Inverness
Lairg / The Crask
John O’Groats
National Amyloidosis Centre News
12  Issue 3: March 2014
Times at each location will depend upon progress; head winds in particular can slow things down, but the website will
have a link to allow you to track progress every 10 minutes or so making it easy for people to meet the team along the
way, especially at any pre-arranged venues where we will be able to work with the media to get publicity.
What next?
In future newsletters we will tell you more about this exciting challenge, and by that time others may have agreed to help
in some of the ways that we have suggested. If you have any ideas of your own that will add to the success of this
challenge, especially ones that will create greater awareness, please let us know.
Donations
To ensure that your donations go directly and exclusively to the NAC,
please send directly to us or contact Beth Jones on 020 7433 2802 or beth.jones@ucl.ac.uk.
All university based medical research depends on funds that are
fought for in open competition (grants) from the
Government-funded Medical Research Council and other
charitable bodies. Their renewal depends on a successful
research programme and the NAC has an excellent track record
in this respect. However, there are constant shortfalls and every
penny from other sources is received with sincere gratitude, and
is used specifically and in its entirety for amyloidosis research.
You can make an online donation at:
http://www.ucl.ac.uk/amyloidosis/support-us
or make a donation by post. A gift aid form is included here and
is 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
UCL Medical School is part of University College London (UCL)
which is a registered charity. Due to changes in the Budget
nearly all gifts given to UCL now qualify for the Gift Aid
Scheme. This increases the amount of the gift 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 make a "Gift Aid Declaration". One
declaration will cover all future gifts and may be cancelled at
any time. Cheques should be drawn in favour of "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 the Gift Aid Scheme or does not
wish to contribute in this way, cheques should be made
payable to: "UCL Amyloidosis Research Fund".
 Newsletter funded by a bequest from Laura Lock 