posterior cortical atrophy
PCA SUPPORT GROUP
www.pcasupport.ucl.ac.uk
newsletter
Welcome to the [PCA] Support Group
Welcome to this PCA Support Group
Newsletter. I hope that the content of the
newsletter is of interest to all who read
it; however, I am acutely aware of the
difficulties in producing information which
meets everyone’s needs and in a style
which they are comfortable to embrace.
Please do let me have any suggestions
regarding the format of the newsletter and
indeed any contributions you wish
to make.
This newsletter is available in a font and
format which people with PCA may find
easier to read. Please let Jill Walton know
if you would like to receive a copy. It will
also be available as an audio recording,
accessible via the website.
For those of you who receive the newsletter
as hard copy, we are trialling the ‘Articles
for the Blind – Free postage Scheme’ and
assume that newsletters are successfully
reaching the intended recipients!
Having access to this scheme makes a
considerable saving to the postage costs
incurred by the MEF, and may I please
remind you that if you are able to receive
the newsletter electronically, we are very
happy to provide it via this means.
Newsletter Issue 21, Jan 2015
Autumn 2014 was a busy time with
events culminating in a support group led
initiative to raise awareness of young onset
dementia, and PCA in particular, at an
event in the House of Lords on December
9th. Baroness Sally Greengross [crossbench
(independent) member of the House of Lords
and co-chair of the All-Party Parliamentary
Group on dementia] and Professor Nick Fox
(director of the Dementia Research Centre,
UCL) welcomed an invited list of over 130
guests on our behalf, including support
group members, MPs, Peers and a range
of professionals from across the social care
and charity sectors. The primary objective
of the event was to have key players leave
with an understanding that dementia is
not only about memory loss and that it
affects younger people too. Everybody
who attended the event was provided with
hard copy information about young onset
dementia and PCA specifically, as well as
being presented with data in the form of a
usb memory stick to take away and circulate
within their professional domain. This event
was a support group initiative and one
which I hope you regard as a platform from
which your views were represented. We
MEF MYRTLE ELLIS FUND
supporting rare dementia
The PCA Support Group is generously supported by the Myrtle Ellis Fund, as part of The National
Brain Appeal (Charity number 290173). For more information on the work of the Fund or to
make your own contribution to the running costs of the PCA Support Group, please contact the
Foundation on 020 3448 4724. Alternatively visit www.pcasupport.ucl.ac.uk or
www.justgiving.com/Myrtle-Ellis-Fund
1
intro cont’
are grateful to Alzheimer’s Research UK
(ARUK) for their assistance in some of the
administrative tasks involved with the event.
You can read Di Garfield’s summary of the
event in this newsletter and can visit
https://www.ucl.ac.uk/drc/support-groups/
house_of_lords for more information.
May I take this opportunity to invite you
to the forthcoming carers meeting on
Thursday 26 February 2015. The venue for
this meeting is Wilkins Front Quadrangle
Events Venue which is located just in front
of the usual Wilkins Building, UCL, Gower St,
London WC1E 6BT. The meeting is scheduled
from 11am -2pm, with coffee available
from 10.30am and lunch provided. We are
expecting 2 guest speakers at this meeting;
We were particularly grateful to Dr
Jonathon Schott who joined us at our
November meeting and explained very
clearly the advances that are being made
in our understanding of PCA, during a
talk entitled: “Using imaging and genetics
to understand PCA”. Audio and visual
recording of this presentation, and indeed
of other presentations made at the meeting
are available at http://www.ucl.ac.uk/
drc/pcasupport/meetings/meeting_
recordings/#Nov2014
Angela Sherman will be talking about
Continuing Health Care funding
applications. You can find out more about
the organisation she directs at www.
caretobedifferent.co.uk
Dr Eneida Mioshi: Department of Psychiatry,
University of Cambridge School of Clinical
Medicine, who will be talking about coping
strategies for carers.
Music provided by ‘The Cocklepickers’
was enjoyed at the close of the meeting,
with many of us taking the opportunity to
sing along with familiar songs, under the
direction of Chris and Innis, who kindly
volunteered their time and skills at no cost
to the group.
This meeting is a support group initiative
and is open to carers, family and friends of
people affected by the various diagnoses
that our support groups seek to reach. As
such we will be welcoming members from
the Primary Progressive Aphasia support
group, the Frontotemporal Dementia
support group, 2 support groups that are
dedicated to familial variants of dementia,
as well as the family and friends of people
affected by Posterior Cortical Atrophy at this
meeting.
The meeting is in addition to the next full
PCA support group meeting on March 27
2015.
I look forward to seeing you at one of
these meetings or indeed being in contact
with you in whatever capacity is most
appropriate.
Christopher Miller and Innis Bourne: ‘The
Cocklepickers’
Jill Walton
Regional meetings are now taking place
in Chichester, Oxfordshire, Berkshire,
Cambridgeshire and Hertfordshire. There
will also be an inaugural regional meeting
in Kent on Feb 6 2015. Details of forthcoming
meetings are listed overleaf and on
the website http://www.ucl.ac.uk/drc/
pcasupport/meetings and the minutes of
the UCL meetings are published at the back
of the newsletter.
2
future meetings
Future Meetings
Berkshire Regional Support Group Mtg:
Date to be confirmed. This meeting
will be held at Barkham COAMHS,
Wokingham Hospital. These meetings are
organised in conjunction with the ‘Young
People With Dementia’ Berkshire West
group.
Carers Meeting
Thursday 26 February 2015 Venue:
Wilkins Front Quadrangle Events Venue
which is located just in front of the usual
Wilkins Building, UCL, Gower St, London
WC1E 6BT. 11am -2pm.Coffee available
from 10.30am and lunch provided.
Hertfordshire Regional Support Group
Mtg:
Date to be confirmed. The venue for
this meeting is The Point Meeting
Room, Christ Church, The Common,
Chorleywood, Hertfordshire,WD3 5SG.
We are expecting 2 guest speakers at this
meeting;
Angela Sherman will be talking about
Continuing Health Care funding
applications. You can find out more about
the organisation she directs at www.
caretobedifferent.co.uk
Kent Regional Support Group Meeting:
Friday February 6th 2015, 11.30AM –
1.30PM
Venue: The Elizabeth Raybould Centre’s
Lecture Theatre, Bow Arrow Lane,
Dartford DA2 6PJ
Dr Eneida Mioshi: Department of
Psychiatry, University of Cambridge School
of Clinical Medicine, who will be talking
about coping strategies for carers,
amongst other things
This meeting is in association with a
representative of Kent and Medway NHS
Social Care and Partnership Trust.
Full support group meetings
are scheduled for:
Please contact Jill Walton via jill.walton@
ftdsg.org or tel 07592 540 555 for more
information about any of these meetings.
Friday March 27 2015 Venue: Wilkins
Front Quadrangle Events Venue which is
located just in front of the usual Wilkins
Building, UCL, Gower St, London WC1E
6BT. 11am -2pm. Coffee available from
10.30am and lunch provided.
Chichester regional meetings:
If anyone is interested in attending these
meetings or wants more information,
please contact Fiona Chaabane on 07792
295711 or fchaabane@hotmail.co.uk
Friday June 19 2015 Venue: Wilkins
Haldane Room, Wilkins Building, UCL,
Gower St, London WC1E 6BT. 11am -2pm.
Coffee available from 10.30am and lunch
provided.
Meeting dates: Sunday 22nd February,
26th April , 28th June , 23rd August , 25th
October and 13th December.
Regional Meetings
The venue is Augusta Court Care Home,
Winterbourne Road, Chichester, West
Sussex PO19 6TT.
All meetings will take place from 2.30 – 4
p.m.
Regional meetings provide an informal
opportunity to meet other people
affected by PCA.
Disclaimer:
Please note that you assume full
responsibility and risk when attending
support group meetings, and also in
the use of the information contained on
our website, in our newsletters and at
support group meetings.
Central Regions PCA Support Group
Meeting:
The date for this 5th regional meeting
is Monday February 16 2015, from
11:00am -1:00 pm. The venue for this
meetings is at the home of Martina
Wise: in Blewbury, Oxfordshire, with
lunch provided. These are informal
social gatherings for people affected by
PCA. Please contact Martina by email or
mrsmartinawise@gmail.com or 07958
668 035 .
3
directions
Directions for forthcoming UCL meetings
Wilkins Main/Front Quadrangle, University College London, Gower Street, London WC1E 6BT
Underground
Services to these stops include route numbers:
10, 14, 24, 29, 73, 134, 390.
London Buses Infoline: 020 7222 1234
The closest underground stations to UCL are
Euston Square on the Circle, Metropolitan and
Hammersmith and City Lines, Goodge Street
on the Northern Line and Warren Street on the
Northern and Victoria Lines.
London Underground Infoline: 020 7222 1234.
Rail Travel
Disabled Persons Railcard scheme. A person
is eligible for the scheme either as a result of
being registered as having a visual impairment
or by virtue of being in receipt of attendance
allowance or disability living allowance/personal
independence payment. The railcard entitles
the holder and a friend to 1/3 off train fares.
Passenger assistance is also available to rail
users. Visit www.disabledpersons-railcard.co.uk
London Underground - Did you know that
you can ring London Underground Customer
Services on 0845 330 9880 the day before
you are due to use a service? They arrange
for someone to meet you in the entrance area
of the station at which your journey begins,
accompany you down to the platform and onto
your train. They then radio ahead to an official
at the relevant station to assist with any required
platform changes or take you up to ground level.
Parking
UCL Helpline 020 7974 4651 or 020 7974 4655
(Staffed Monday -Friday 9.00 am to 5.00pm)
We are very conscious that travelling to and
around London can seem a daunting prospect.
Be aware that pre-booked travel is generally
cheaper than tickets purchased on the day
and that the Myrtle Ellis Fund provides help
with travel costs where required. Contact Jill
for further details.
Buses
UCL’s Gower Street site is served by many
Transport for London bus routes. Buses travelling
from north to south stop in Gower Street,
immediately outside UCL’s main gate, while those
travelling from south to north stop outside Warren
Street station, about five minutes’ walk from UCL.
4
fuundraising
The issue of fundraising will continue to be
uppermost in our minds throughout 2015.
Around £20,000 per year is needed to keep
not only the PCA Support Group running, but
also three other rare dementia support groups
financed by the Myrtle Ellis Fund: Primary
Progressive Aphasia (PPA), familial Alzheimer’s
Disease (FAD) and familial Frontotemporal
Dementia (fFTD).
Donations can be made online www.justgiving.
com/Myrtle-Ellis-Fund, on your mobile by texting
TMEF50 £10 to 70070 or send a cheque for the
Myrtle Ellis Fund to the National Brain Appeal,
Box 123, Queen Square, London WC1N 3BG.
Details of some of the fundraising ventures that
have been initiated on behalf of the support
group can be found on the website at http://
www.ucl.ac.uk/drc/pcasupport/fundraising
Thank you to everyone who took part in the
Simultaneous Global Virtual Quiz in November
across twenty locations all over the UK and
even one family in Switzerland – we hope you
had fun. In the centre of this photo with the rest
of “The Oxheymorons” are Debbie and Aaron
Phethean who hosted the winning team in
Hertfordshire.
Raising money for Research
into PCA
Included in the events listed at the above
site is a sponsored charity bike ride on the
beautiful island of Mallorca, taking place on
May 16th, 2015. This event is being organised
by Lisa Frankel Pollen and is to raise money for
research into PCA. Please see the website for
more information or contact lisafrankelpollen@
yahoo.co.uk directly for an application form
and details of how to make the £100 deposit
transfer, as well as more info about the route
and event.
The final total including donations from friends
and family who couldn’t make it on the night
itself was almost £2,700.
Another significant amount of money was raised
through the generosity of the Bulmer family.
Paul was diagnosed with PCA at the age of
53 and promptly took up painting, producing
beautiful artwork that was used to create a
print run of 4,000 Christmas cards sold in aid of
the Myrtle Ellis Fund. His wife Alison along with
fellow PCA carer, Christine Nancollas worked
tirelessly to organise the printing, package them
into plastic sleeves with envelopes and dispatch
them to waiting customers. The total paid into
the Fund came to almost £1800.
The goal for next year is to find a sponsor so
a larger print run can be undertaken along
with a longer lead time for a wider marketing
campaign. Any interested parties should send
an email to myrtleellisfund@outlook.com.
5
raising awareness
Raising awareness event at the House of Lords
The Myrtle Ellis Fund in conjunction with the UCL Dementia Research Centre
and the Frontotemporal Dementia Support Group, organised an event at
the House of Lords on 9 December 2014 with the support of Alzheimer’s
Research UK to which I was honoured to be invited.
By Diane Garfield
Diane Garfield with Baroness Knight
The event provided mutually
beneficial opportunity to collaborate
with ARUK
This event brought together MPs, peers, policy
makers, charity groups, researchers, clinicians,
industry stakeholders and people affected by
young-onset dementia to raise awareness and
discuss the key issues that need addressing to
improve the lives of people with dementia
under 65.
They spoke with love and humour about their
story; from pre-diagnosis when Paul’s condition
was beginning to cause issues at work, through
finally getting a diagnosis and now learning to
live and cope with PCA on a day-to-day basis,
both as a couple and a family.
The Bulmers have taken a proactive approach
to keeping Paul active by finding and - where
necessary - adapting pastimes and hobbies to
suit his changing needs. Of course this takes a
great deal of strength and concentration for both
of them so they find the ongoing support of the
in-person meetings and the online support of
the Facebook group, invaluable.
Speakers included Baroness Sally Greengross
(an independent member of the House of Lords
and chair of the All-Party Parliamentary Group
on dementia), Professor Nick Fox (director of the
Dementia Research Centre at University College
London), Malcolm Walker (CEO of Iceland Foods
which has raised more than £3m for youngonset dementia research) and a number of
people affected by young-onset dementia
reflecting on their experiences, including our
own Paul and Alison Bulmer.
The other PCA family in attendance was the
Womacks and whilst time did not permit a
personal presentation, their story was published
on a flyer given out as part of the welcome pack.
6
raising awareness cont’
Jan had written to her local MP asking him to
attend (which he did) and she spent time chatting
with Baroness Jill Knight whose son Roger has
PCA and grandchildren are members of the PCA
Awareness Facebook group that Jan runs.
Representatives of the Dementia Research
Centre familiar to our PCA Support group were
also there with Dr Seb Crutch giving a short
speech and Tim Shakespeare and Amelia Carton
also sharing their knowledge with medical
practitioners and fellow researchers during the
networking opportunities.
And last but by no means least, I was delighted
to have the company of Martina Wise; although
it’s been a few years since her cousin’s passing
from PCA, she continues to share her knowledge
and her heart with us all. She can be found
running the Oxfordshire branch of our regional
network, organising quarterly meetings at her
home and it is with this in mind that she made
excellent contacts with Young Dementia UK
representatives who provide one-to-one support
for families in that county.
Among a number of outcomes of this event has
been a generous donation to support group
funds and a commitment from the Myrtle Ellis
Fund to look further into advocacy opportunities.
If you have any thoughts on this or would like
more information on anything raised in this
article, please email me at: myrtleellisfund@
outlook.com
7
alzheimer’s society magazine
PCA featured in the Alzheimer’s Society Magazine
A family affected by PCA were interviewed by Fiona Madden in the
Alzheimer’s Society November 2014 publication of the ‘Living with
dementia’ magazine. Peter and Kath Bray are members of the support
group and have kindly given permission for us to reprint the article below.
http://www.alzheimers.org.uk/site/scripts/documents_info.php?documentID=2800
When Peter Bray’s wife, Kath, started to
experience problems with her vision six years
ago, neither of them associated these with any
kind of dementia.
‘About a month later I had a heart attack and I
couldn’t drive for a while after. She was having to
do more driving and we were together more so
I noticed a lot of things going on. We thought it
was a problem with her eyes.’
Kath BrayKath, now 63, and Peter, 67, were going
on holiday to Greece and arranged to stay at their
niece’s house the night before flying. During the
drive there Peter describes Kath as ‘coming over
all funny’ and starting to shake. They rushed to
hospital thinking that she may have had a stroke
or heart attack.
No improvement
Kath got contact lenses from one optician
but after a couple of months without any
improvement they visited a different chain.
They referred Kath to an NHS eye department
which she visited once every few weeks over
several months for an array of tests, including
another MMSE. Once again they were told that
her symptoms were a natural part of ageing.
They were told that Kath had probably had a
urinary tract infection and after being kept in for
observation for several hours she was given the
all-clear to go ahead with the holiday.
By 2011 Kath, still visiting the eye clinic regularly,
decided to stop driving altogether. She had an
operation to insert replacement natural lenses in
her eyes and although her eyesight was tested as
being 20/20 she was having recurring difficulties
with everyday activities as well as increased
confusion.
However after returning to Suffolk it was
evident that Kath, a hospital receptionist, was
experiencing new problems.
Peter says,
‘When she went back to work she started noticing
trouble with her eyes with things like reading,
filing and computing. That year went on and she
started driving erratically.’
Peter says,
‘We’d just had a new grandson and she really
broke down in tears when she couldn’t read him
a bedtime story. She realised she couldn’t ride
her bike, had trouble dusting and polishing, and
I was trying to do more and that was upsetting
her too. Kath was crying knowing something was
wrong but couldn’t do anything about it.’
Vision problems
When the Brays, who were avid travellers, went
backpacking around Australia, New Zealand and
Fiji, Peter noticed that Kath was struggling with
her vision and experiencing confusion.
They decided to revisit their GP following the
trip and Kath was given the Mini Mental State
Examination (MMSE), a test used to detect
problems with mental processing and memory.
This came back with good results and the Brays
were told that Kath’s problems were a result of
ageing.
She was diagnosed with depression and put on
medication but she and Peter were frustrated at
not knowing the underlying cause.
Falling scores
Kath completed a few more MMSEs with falling
scores over the following months and was
referred to a neurologist. They were told that Kath
had most probably had a stroke on the drive to
their niece all those years ago.
Peter says,
‘She couldn’t drive at night and her work was
deteriorating. As the year carried on she gave up
work.
8
alzheimer’s society magazine
After the neurologist examined Kath’s history and
observed her having a problem sitting down and
with spatial awareness she said she thought Kath
had posterior cortical atrophy (PCA).
Peter is keen to raise awareness of PCA as
a rarer form of dementia. He is dismayed at
the lack of knowledge about it among health
professionals and believes that Kath could have
been diagnosed much earlier.
Peter says,
‘She said PCA was a rare form of dementia –
Kath burst into tears when we were told but at
least we knew, at least we had a diagnosis.’
Kath is now registered blind and her vision has
been coming and going without warning.
PCA can be difficult to identify and most cases
are caused by an unusual form of Alzheimer’s
disease. People with PCA tend to experience
problems with their vision initially and sometimes
with literacy and numeracy. They often develop
more typical symptoms of Alzheimer’s, such as
memory loss and confusion, as their condition
progresses.
‘Over the past year she can’t dress herself. She
can’t do anything on her own and is deteriorating.
Peter says,
‘We’ve been a lot happier as a married couple
since diagnosis. I cook and clean now but she
tries and gets frustrated still. She still speaks and
looks well.’
Getting the word out
Peter wants to continue raising awareness about
PCA and educating others about its early signs.
He is especially concerned for people living alone
with the condition.
Support group
The Brays were put in contact with the PCA
Support Group and Kath now visits the
neurologist every six months for a check-up.
He says,
Kath and Peter BrayThey are grateful for the
group’s support and meeting other people with
PCA through it. Kath has also been able to take
part in research to find out more about the
condition.
‘The more people who know about it the easier it
will be to spot. I think a lot of people could have it
but don’t know because they think it’s a problem
with their eyes. We need to get the word out
there.’
9
sunday times article
PCA featured in Sunday Times article
Valerie Blumenthal was interviewed about her diagnosis with PCA in the
November 1 2014 edition of the Sunday times magazine.
Valerie Blumenthal describes the
terrifying symptoms that led to
her diagnosis.
She ran down steps without a second thought,
sketched horses and played the piano. That
used to be me, before the PCA crept up,
amorphous, insidious. PCA stands for
Posterior Cortical Atrophy. An unusual form
of Alzheimer’s, it attacks the back part of
the brain, responsible for visual function,
orientation and spatial awareness, rather than
the front, which affects cognitive function. Trust
me to have something weird, which hardly
anyone has heard of, including many doctors.
I am in good company: another author, Terry
Pratchett, is a fellow sufferer.
I had never really considered my brain much;
other than as a gory slab, privy to my innermost
thoughts. Myself and it are supposedly one and
the same, yet currently my brain is out to thwart
me. But if my brain is what rules me, if it makes
me who I am, then, in fact, I rule it. But am I
really in charge?
Two years ago, when I finally got my diagnosis,
it felt like the missing part of the jigsaw. I tell
everyone what is happening to me. If I miss the
chair, or end up sitting on the floor of the train,
as I did recently to great embarrassment, I say,
“I’m sorry. I have a visual problem.”
Part of the trouble is, I look quite normal.
Not long ago, we were at a Michelin-starred
restaurant for my husband’s birthday.
Everything was perfect – the food, service,
ambience … Without a qualm, I walked up
the three wide, tartan-clad stairs to pay; then
turned to descend. The short run of stairs took
on the proportions of Kilimanjaro.
With a fixed smile, I placed one foot in front of
another – and fell. My landing was soft, but
was small consolation, as I lay, sprawling there.
It must have appeared comical to the dozen
onlookers, and I played up to that. Laughed as
I stood up, and offered an encore. It was not
the time or place to expound on the curious
affliction that is PCA.
Generally, PCA sufferers are in their early
fifties and sixties, much younger than those
with typical Alzheimer’s, which presents those
afflicted with memory problems. There are
reports of patients with symptom onset as early
as their forties.
Normally, I lie through my teeth about my age.
But in this instance it is important to be honest,
so, reluctantly, I must admit (deep breath)
to being a youthful sixtysomething. I am a
mother (to one daughter), grandmother (to one
granddaughter), wife. I have a large dog and
small cat.
In retrospect, I realise I began developing
symptoms of PCA five years ago. Driving, which
had always been a pleasure, increasingly
10
sunday times article cont’
was becoming a thing to dread. I could see
perfectly well, my driving glasses were the
correct prescription, yet I had problems judging
distances and where to position my car in
relation to the road; also where to pitch my line
of vision. Road signs leapt out at me, filling me
with panic. Busy junctions were my idea of hell.
It was disconcerting, to say the least. I would
arrive at my destination a wreck. I was always
clipping the curb or, worse, the wing mirror of
an oncoming car.
“You again! What’s the excuse this time?” the
garage man would comment.
As my driving grew worse, I used landmarks,
counting them as I passed them unscathed. I
removed the radio so I would not be distracted,
and talked aloud to myself: “Concentrate …
You love driving; it equals freedom … This is all
in your head.”
I promised my husband I would take a driving
course, but chickened out, fearing I would be
told I shouldn’t be on the road. Maybe this
phase would pass? It didn’t. Reversing down
our own driveway became a hit-and-miss
exercise that left zigzagging tyre imprints on
the lawn. In order to park, I would drive my
small hatchback round and round, searching
for a space big enough for a stretch limo.
Then there was the day I was about to park in
an empty space; except it wasn’t empty. The
vacant slot was next to it. For several minutes I
felt sick.
Over the last two years that I drove – until the
diagnosis – I rarely had a passenger, other
than my German shepherd, and even he
made it clear he didn’t like my driving. Friends
and family knew about my nervousness, but
nobody knew the full extent: that I regarded
my car as a killing machine and had to brace
myself every time I climbed into it. Or that I
dreaded being responsible for a fatal
accident.
Around the same time, I developed an
inexplicable fear of steps and stairs: winding
ones, glass ones, sloping ones, crumbling
ones, short ones your feet hang over. I could
climb them with comparative ease, but
descending was another matter. As they
swayed before me I morphed into a scared old
woman, clinging on to the railings for dear life.
The stairs existed solely for me to break my
neck.
As for escalators, I confronted these with
horror. I could not get the timing right and
lurched onto them, getting my feet sandwiched
between the treads. Nowadays, when there is
no option other than to use one, I enlist help;
prior to my diagnosis, it was important to me
to face my fears. I used frequently to travel to
London and brave the escalator at Marylebone
station, waiting for a gap between the endless
hoards streaming past me. Who would have
guessed at my hammering heart?
It’s ironic, because I’ve always had a strong
spatial awareness. For four years I taught
creative writing in a men’s maximum security
prison, a job I was very proud of. They gave
me my own set of keys and I would wander
confidently through miles of corridor, unlocking
doors, without any sense of panic. What had
happened to that woman?
Practical chores became ever more difficult, but
I continued to bluff. I could confide in nobody.
PCA affects motor skills and literacy; it almost
makes you illiterate. Newspapers were the next
thing to go. If I read an entire article, it was an
accomplishment. Fancy fonts confused me, and
finding my way round the columns was akin to
searching for the exit in a maze.
About three years ago, shortly before my
mother died, I offered to read to her from a
novel of my own. It was her favourite of my
books, and I noticed a spark of interest come
into her eyes. Sitting close, I began to read
aloud. Stumbled. Stopped. Started again. The
same thing happened. I could not comprehend
it. I continued for a few sentences, from
memory, and ad-libbed, but had to abandon
the idea. I put the book, which had once meant
so much to us both, back on its shelf.
In fact my mother, a hugely intelligent woman
who developed Parkinson’s and then severe
dementia in her eighties, seemed to sense
what was happening to me. After I’d done
something slightly batty, she looked at me
acutely, saying, “Darling, I hope you’re not
getting what I’ve got.” It was very shrewd of her
to pick it up so instinctively. Perhaps that’s why
my diagnosis didn’t come as such a shock in
the end.
11
sunday times article cont’
Cheques became another nightmare. I couldn’t
keep to the lines, and there seemed to be
insufficient space. Completing another form,
I could not keep to the boxes and had to
start afresh. Finally, in barely legible writing, I
succeeded. I felt foolish and inadequate. And
something else besides: I felt the prickling of
concern.
The months progressed. My husband
commented I hardly read any more. I made
some excuse. He did not know that reading
had ceased to be enjoyable for me and had
become a strain. I did not tell him that the
letters and words danced in front of my eyes
and I would lose my place or read the wrong
line. I went on buying books but, unread, they
taunted me with their pristine promise.
Then I discovered that I could no longer play
the piano. It seemed to have gone from me
overnight. In fact, there had been an interlude
of a few years, but I assumed that, as with
riding a bike, it was something I would always
retain. Full of anticipation I positioned the
Mozart sonata on the piano, and leant forward
to remind myself of the opening chords.
Crotchets and quavers were in a jumble,
meaningless. I was unable to read a note.
And I used to like playing backgammon. Just
setting up the board was part of the pleasure
for me. I played fast and furiously, but then
that, too, changed. I’m getting the knack of this,
gloated my husband, as he won three nights in
a row and I fumbled around the board, taking
for ever, picking up the wrong pieces, counting
laboriously and incorrectly. I could see them
clearly, yet when I went to move them, the
crowded board merged into a homogenous
blur. I remember thinking it was as though my
brain was unable to coordinate with my eyes. I
became tearful.
You’re tired, that’s all, my husband, the kindest
man on the planet, tried to mollify me. I knew
it was more than that. But we stopped playing
backgammon. Scrabble met the same fate.
And then I started losing my way round the
computer. What was happening to me? I
could see no correlation between the strange,
disparate symptoms I was experiencing. So,
a couple of years ago, I paid a visit to my GP. I
told him I feared I had some form of dementia.
He tried to reassure me; it was far more likely
I was suffering from anxiety or depression,
or both – understandable, given certain
circumstances, he said. I was not reassured. I
had – and have – immense respect for him, but
I knew I was neither depressed nor anxious. I
declined the offer of pills and muddled on.
Every day, the “brain gremlins”, as I call them,
plotted a different prank to play on me. They
had a warped sense of humour. I struggled to
wrap presents and became entangled with the
Sellotape. I struggled to lay the table or read the
time on the kitchen clock. I found it hard to walk
down the street without weaving drunkenly,
and had to make a conscious effort not to do
so. I felt unsteady on my feet, and took extra
care crossing roads, as I could no longer judge
distance. I felt vulnerable when out of my own
environment. Crowds bothered me.
At the supermarket, the tension built in me. I
would fail to see the item I wanted, although
it would be under my nose. When I asked,
sometimes the assistants were impatient,
because I did not look old, infirm or crazy.
When it came to paying, I trembled as I
rummaged in my bag for my wallet, then held
up everyone as I attempted to fit my card into
the slot; I could feel waves of irritation from the
person behind. And their supercilious pity –
even if I imagined it.
The pretence went on as I floundered with daily
life. My confidence ebbed. Quite simply, I felt as
though I were losing my mind. I’ve always been
seen as slightly unusual, eccentric even, by
friends, but this was different.
Ten months ago, I booked to see my optician
(interestingly, many people with PCA are picked
up by a concerned optician) and explained the
situation to him. He decided to test for macular
degeneration. I knew he would find nothing
wrong with my eyes. Tests confirmed this, and
he agreed with me that the brain didn’t seem to
be processing signals. Finally, I was referred to
a neurologist, and after extensive psychological
tests, had a brain scan. It showed substantial
atrophy.
It may sound strange, but I cannot express the
relief I felt upon learning that diagnosis. I could
stop pretending. Yes, I have had the odd cry,
but I am under a fantastic team at the John
Radcliffe Hospital in Oxford who monitor my
progress and help to treat my symptoms. I am
also helping out with a research project on PCA
being run by my neurologist, Dr Chris Butler,
12
sunday times article cont’
and neuropsychologist, Dr Samrah Ahmed.
Viewed objectively, PCA is a fascinating illness.
No one knows how many people have been
diagnosed: there are no consistent diagnostic
criteria and a general lack of understanding
and recognition of the condition among health
professionals. As a rough guide, however, a
study that looked at 523 cases of patients who
were diagnosed with Alzheimer’s found that
5 per cent of these cases fulfilled criteria for
PCA. No clear association between PCA and a
positive family history of the condition has been
found. However, research is still in its infancy.
I’m no do-gooding saint, but I’d be thrilled if
others could learn from my situation. Just writing
about it feels therapeutic. Since diagnosis, the
stress has been much reduced. My friends all
know. My very supportive husband is no longer
impatient when I fail to see what is right in front
of me, and shepherds me in unfamiliar places,
as I lose my bearings. I can, to some extent,
capitulate to the illness; that said, there are
moments when I let myself down.
And although I am typing this with painful
slowness as the letters dart about and elude
me, I try to focus on what I can do rather than
what I can’t. I cannot draw beautiful horses any
more, but I can paint wild abstracts full of colour.
I can look after my four-year-old granddaughter.
I am absolutely capable in my own environment;
I just get jittery when I go out into the world.
I am in the middle of writing a new novel – very
slowly. Ironically, although I started it before I got
my diagnosis, the mother of the book’s narrator
has Alzheimer’s and makes the decision to go
to Dignitas in Switzerland. I don’t fear death.
Or rather, to quote Woody Allen, “I’m not afraid
to die; I just don’t want to be there when it
happens.”
It did come as a shock when I read about
the typical life span of someone with PCA.
It’s a progressive syndrome, like Alzheimer’s,
meaning that cognition will decline over time, so
patients may only have 10-15 years.
Dr Ahmed, the neuropsychologist running the
PCA project at Oxford University, tells me that
every individual patient will have their own
specific pattern of deficits: “If the patient has
experienced mild changes in symptoms over a
number of years, we would expect that future
changes would proceed like this. Other patients
report a rather more rapid decline, experiencing
visual problems early on and then very soon
afterwards changes in language function and
their ability to manage physically.”
Some PCA patients report feeling that their
memory is worse than it used to be, while others
feel they are doing quite well. Symptoms such
as depression and anxiety, sleep or behavioural
disturbances can have an adverse impact on
cognition, increase burden for caregivers and
increase incidence of institutionalisation.
Dr Ahmed says Terry Pratchett’s diagnosis has
definitely helped to increase awareness of
the existence of variants of Alzheimer’s, and
how these can affect people at a relatively
younger age. Having said that, PCA is still little
understood and is also given relatively less
attention by researchers, which is why their work
is so valuable.
Sometimes I forget there is anything wrong with
me, and think the whole thing is a mistake. What
I wish, more than anything, is that every GP
could be made aware of this wretched illness.
Knowledge of it could prevent much anguish for
the sufferer.
As for the future, who knows? None of us ever
knows. I am lucky in love. I am not worried for
myself, but for family. I will have to teach my
husband how to put on my lipstick.
If you have been affected by PCA and would like
to be part of the Oxford University study, contact
Dr Samrah Ahmed (samrah.ahmed@ndcn.
ox.ac.uk) or Dr Christopher Butler (chris.butler@
ndcn.ox.ac.uk). The project is being funded by
Alzheimer’s Research UK
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alzheimer’s accountability act
The recent news below from the Alzheimer’s
Association represents a significant step
forward and is good news for US families
affected by these diagnoses as well as the
research communities affiliated to them. In the
longer term, we all stand to gain from these
developments.
The US-based Alzheimer’s Association have
written to members recently to highlight
the signing into law of a significant piece
of US legislation to support future research
in to Alzheimer’s disease and other causes
of dementia. Congress previously passed
the National Alzheimer’s Project Act (NAPA),
requiring a plan. The Alzheimer’s Association
team then worked with government leaders to
create this plan with a core objective to prevent
and effectively treat Alzheimer’s by 2025.
Now, Congress has passed the Alzheimer’s
not enough, it is 1/6 of the money added to the
whole National Institute of Health budget in this
bill and AAA creates the mechanism to raise it
higher in future years. There is much more to
do in the years ahead to realize the potential
of what has just been accomplished, but the
Alzheimer’s Association are hugely proud of and
grateful for your contribution to this outcome (as
many of you have kindly participated in research
presented in the Association’s journal or at the
annual international conference) and appreciate
their champions in the Congress who worked
across the aisle to make this possible.
Accountability Act (AAA, or Triple A), requiring
National Institute of Health scientists to tell the
Congress what it will cost each year until 2025
to succeed at meeting this goal. The Congress
also added $25 million to the base of the budget
for Alzheimer’s research. While this is certainly
14
your contributions
Cycle eyes campaign
Celia Heath responded to an article published
in the Times on August 27 2014; “Blind people
terrorised by cyclists on pavements”.
Cyclists also need to be sensitive to deaf
disabled walking on the flat paths in London’s
parks, which have been designated as cycle
tracks and are hence shared with pedestrians.
They may be the only place where carers
and the disabled can enjoy the green space
together. They need to stay together and may
need to stop to let you past.
She wrote: As a carer to my disabled husband
I am all too aware of the dangers for the
deaf when cyclists share pavements with
disabled pedestrians. In my husband’s case,
a degenerative brain disease is damaging his
sight and sense of balance. The deaf do not
carry a white stick and may be startled by an
unexpected bell, so their reaction may be late,
unpredictable and may be even in the wrong
direction! Bells sound aggressive on pavements
- riders do need to slow down, if they see a
person with a stick: voice contact is friendlier
and more effective.
Jill has made enquiries and is investigating
how we can support the Cycle Eyes campaign,
and whether they might be available to visit a
future PCA support group meeting.
Pam O’Donell updates us on PCA links she has
come across
I found a nice story about Liz who has PCA and
lives in Belfast. I contacted the Author and she
sent a PDF of the story and said I could share the
article. I then, emailed Liz and she said please
share as she is doing interviews in order to
increase awareness of younger onset dementia.
This is an other story on Liz from a BBC interview
last week http://www.bbc.com/news/uknorthern-ireland-30540576
I found a story out of Scotland about a man with
PCA training a group at RNIB about Dementia.
http://ssscnews.uk.com/2014/12/22/rnibbecoming-dementia-informed/
In the article it mentions a film of Stewart Burrows
and his partner Grace teaching about PCA.
Paul and his partner Penny did a TV program
last week for Text Santa http://www.itv.com/
lorraine/hot-topics/alzheimers-changed-ourlives
I messaged Paul and he is trying to get the
interview on YouTube so people outside
the UK can watch the entire interview. This
was the interview I found on twitter https://
www.youtube.com/watch?feature=youtube_
gdata&v=pkGqr5Eoqes&app=desktop
Dementia Engagement and Empowerment Project
(DEEP)
The support group has recently agreed to join
the DEEP network. The Dementia Engagement
& Empowerment Project (DEEP) brings together
groups of people with dementia from across
the UK and is a network that is growing and
becoming more influential.
We look forward to hearing more about the DEEP
network at a future support group meeting. In
the meantime:
• Watch a short film about DEEP on the Mental
Health Foundation website
• Read the report from the first phase of DEEP
on the Joseph Rowntree Foundation website
DEEP supports these groups to try to change
services and policies that affect the lives of
people with dementia.
• Go to the DEEP project website: www.
dementiavoices.org.uk
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young dementia uk
YoungDementia UK Steering Committee
Jill is delighted to have been asked to represent
the support groups on a new steering committee
created by the charitable organisation
YoungDementia UK.
The committee aims to create a national highly
visible identity/focus for young onset dementia
(YOD) , since there is currently none in place. Our
purpose will be to improve the lives of people
with young onset dementia and their families
by harnessing passion, energy, experience and
knowledge.
16
With this momentum, we will create effective
connections and use these connections to exert
a powerful influence over key areas that present
a barrier to living fully and well with dementia as
a younger person.
By acting with strength and determination, we
intend to bring about significant changes that
will have a lasting benefit for people living with
young onset dementia.
Jill will keep you updated….watch this space!
minutes
PCA Support Group Minutes – November 2014
Venue and Attendees
4. Key research questions.
Our thanks once again to UCL for their
hospitality. The sideways layout of the room
meant that it was easy for all to see and
hear the speakers – and later on the band!
Jill welcomed 49 attendees including 12 new
members from across the country – and beyond,
via Skype.
4.1. Why do some people and not others
develop PCA?
PCA News. Baroness Greengross will be hosting
a support group event in the House of Lords
on Dec. 9th: hopefully this will mean a general
awareness raising, i.e. that dementias are not
simply about loss of memory in the elderly but
that they can also strike younger people during
their working lives.
It is progressive, though progress is usually
slow. PCA has specific cognitive problems:
the back of the brain is affected, as
increasingly sensitive and accurate scans
of the brain’s 100 billion cells demonstrate.
4.4. Can PCA research provide insights into AD
in general?
5. Why does PCA develop?
5.1. Both genetics and the individual’s
environment may together influence the
likelihood of someone developing PCA.
It has been known for some time that an
abnormal build-up of ß amyloid (proteins)
causes Alzheimer’s disease (the disease
which most commonly causes the PCA
syndrome).
to understand PCA. “
Key defining features of PCA.
4.3. Why are the symptoms so different from
typical AD?
4.5. How best to help people cope with PCA?
Dr Jon Schott’s talk “Using Imaging and Genetics
1. 4.2. Why are PCA patients younger than typical
AD sufferers?
5.2. 1 in 3 elderly people start to accumulate
these proteins.
5.3. Brain cells start to die: networks between
the cerebral lobes are disrupted and
connections stop working.
2. Review of brain functions.
2.1. The frontal lobes of the brain deal with
planning, interacting, and the navigation of
the memory.
2.2. Occipital lobes monitor vision.
2.3. Parietal lobes control calculation, spelling
and the ability to perform complex actions.
2.4. Temporal lobesare key to day-to-day
memory functions.
5.4. A small number of genes have been found
to cause the very rare familial (directly
inherited) form of typical AD. However we
are yet to see any convincing evidence
ofPCA running in families. More broadly,
we currently know of about 20 other
genes which confer slightly greater risk
of developing AD (though which do not
guarantee you will get the disease).
5.5. One of these risk genes (Apolipoprotein
E or ApoE) is of particular interest in PCA.
At UCL, a pilot study of 10 PCA patients
aged 48-60, (i.e. with young onset
dementia), suggested that people with
PCA were actually less likely than those
with typical AD to have the form of ApoE
that confers the greatest risk for AD. This
has led to UCL setting up an international
consortium on genetic research involving
8 different countries. 302 samples have
3. Characteristics of PCA vision impairment.
3.1. PCA affects vision adversely in locating
and manipulating objects, in reading
and spelling, in coping with stairs and
escalators, in day to day orientation via the
memory.
3.2. PCA patients are relatively young.
3.3. PCA is most commonly caused by
Alzheimer’s disease (AD).
17
minutes cont’
been collected from patients aged 50 – 60
years, and we are in the process of testing
whether the known risk genes confer the
same risk for PCA as they do for typical AD.
6. Progress to date.
6.1. A study inspecting 21 known risk factors is
almost complete.
6.2. New genes are being examined across the
whole genetic code.
6.3. Fundraising is ongoing.
6.4. We need to collect more samples.
7. Imaging.
7.1. MRI scans show brain structure and
shrinkage.
controlled. At present, we are only taking
samples from people being seen by us or
one of our collaborators.
3. An article on PCA in the Times
Colour Supplement for Nov 2nd was
recommended from the floor.This is the
Valerie Blumenthal article referred to
earlier in this newsletter.
8. Di Garfield , founder member of the MEF
provided an update report:
8.1. Jan Womack’s Face Book page is now
global.
8.2. Via connections with Terry Pratchet, a grant
of £24,000 has been received, which will
run the dementia groups for 18 months.
7.3. PCA shows considerably more shrinkage
at the back of the brain than in AD.
8.3. It is vital to have 3 year’s money in
the bank. Suggested fundraisers are
sponsorship. The Internet Supper Quiz due
on Nov 11th. Alison Bulmer is selling her
husband, Paul’s art Christmas cards (Paul
has PCA) at a cost of £4 a pack.
7.4. Though the disposition of amyloid is similar
to AD, there are different patterns of brain
cell loss and energy usage.
9. Dr. Seb Crutch’s update on Progress at UCL
(Queen Square)
7.2. PET scans are highly detailed and provide
a picture of brain metabolism and energy
use plus brain amyloid (Key AD protein).
7.5. Combined with genetic research and new
and improved scanning techniques, there
is an improved ability to explore factors
influencing PCA.
Questions.
1. Can Lewy body dementia cause PCA?
PCA can be caused by AD, LB and other
degenerative diseases.
PCA is under researched when it comes to
data analysis. Psychiatrists, Opticians and
GP’s are largely unaware of its existence.
PCA is so rare that GPs may come across
PCA only once in a lifetime. There is a
need to target those practitioners who are
likely to see a person at their first visit to
a specialist i.e. Optometrists, opticians,
neurologists.
2.
PCA genetic research: Can one request to
donate a blood sample for such research
purposes?And must one come to UCL in
person to do so?
The taking of blood samples and any
subsequent DNA analysis is strictly
9.1. A communications network to standardise
consensus criteria internationally will be up
and running by the end of the year.
9.2. The Stages of PCA document which
was piloted at the carers’ meetings has
reached its final draft and will be on
the web site shortly. It will be subject
to ongoing modification as research
progresses.
9.3. The case study of Dennis and Jenny.
Jenny was a research phlebotomist and
first noticed problems (after early quirky
problems with perceptual oddities), when
she became unable to calculate fasting
times. Her ward boss sent her for tests.
She was OK for 10 years, though it was a
fight to stay on top of PCA as she became
less able to cope with change and verbal
expression slowed. Her physical health
was OK but the final decline was rapid.
9.4. Art and Neurology: An exhibition at The
Rag Factory in Shoreditch is showing the
first images based on what PCA people
actually see.
18
minutes cont’
9.5. An interesting video of PCA perceptions of
objects near Brighton Pier was shown: it
demonstrated how sight becomes a puzzle
with disassociated pieces. A red life-ring
stand, seen sideways, was mistaken for a
large lollypop.
9.6. Human beings have a natural tendency to
make sense of shapes – even black and
white test shapes! PCA people are left with
the first interpretation and cannot see the
context quickly enough to further interpret it.
9.7. A comment from the floor; “When drawing
with my grandchild, I drew arms coming
from the head!”
Kangaroo dow, Sport” etc. and soon got
everyone singing, people with PCA , family
and friends alike.
Celia Heath, July 2014
DISCLAIMER: The information contained on
our website, in our newsletters and at support
group meetings is for information purposes only.
You assume full responsibility and risk for the
appropriate use of the information contained
herein and attendance at any support group
meetings.
Our meeting concluded with The Cockle
Pickers Ukulele Band. Chris and Innes
entertained us with old favorites, e.g. “Bye
Bye Love”, “King of the Road”, “Tie me
19