A word from our President . . . . . . . . . . 2 Latest News . . . . . . . . . . . . . . . . . . . . . . . . 3 Farewell and Thank You to Dr Beatrix Wonke . . . . . . . . . . . . . . . . . . 9 News from around the country . . . . . . 13 Medical News . . . . . . . . . . . . . . . . . . . . 14 Patient News. . . . . . . . . . . . . . . . . . . . . . 16 News from around the World . . . . . . . 21 Events . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 Office News . . . . . . . . . . . . . . . . . . . . . . . 23 Membership Application . . . . . . . . . . . 24 CONTENTS UNITED KINGDOM THALASSAEMIA SOCIETY A Charity Organisation Registration Number: 275107 19 The Broadway Southgate Circus, London N14 6PH Telephone: 0208 882 0011 • Fax: 0208 882 8618 Email: [email protected] • www.ukts.otg As hard as it seems to believe, Dr Beatrix Wonke, Consultant Haematologist and the vision behind the Thalassaemia Unit at the Whittington Hospital (North London) has finally retired, with effect from the end of April 2004. Internationally renowned for her pioneering work in the treatment of thalassaemia, Dr Wonke departs for her well-earned retirement also enjoying the unparalleled love and respect of her thalassaemia patients and their grateful families. Earlier this year Dr Wonke’s outstanding career in medicine was formally honoured when she was awarded the Order of the British Empire, presented by the Prince of Wales at Buckingham Palace. After 26 years at the Whittington Hospital, many of Dr Wonke’s patients know her almost as well as she knows them – and even though they are aware of her aversion to fuss and frills they could not let her go without having the opportunity to pay their tributes and show her how much she will always mean to them. They decided to arrange a surprise leaving party – and so began a stealth operation worthy of MI5. Letters were sent to all corners of the UK and some much further afield, inviting patients, their families and colleagues to the event, with strict injunctions not to breathe a word in case rumour should leak back to Dr Wonke herself. The venue (The Atrium Suite at the Royal Free Hospital), caterers and flowers were arranged, speeches were written and finally, on the evening designated with the connivance of Dr Wonke’s family (23rd April) all was ready. Almost unbelievably, Dr Wonke had no suspicion that when she walked into the Atrium she would be greeted and applauded by nearly 200 people including her children and grandchildren as well as her “thalassaemia family”. George Constantinou (acting as MC for the Dr Beatrix Wonke retires – farewell to a pioneer in the modern treatment of thalassaemia July 2004 ISSUE NUMBER 98 evening) made a short speech of welcome, to which an emotional Dr Wonke responded. Thereafter the guests enjoyed an excellent buffet supper before the commencement of the formal speeches, usually the least interesting part of any event but on this occasion the highlight of the evening. First was Emma Prescott, Thalassaemia Nurse Specialist at the Whittington Hospital and Dr Wonke’s close and trusted colleague of more than 12 years. Emma performed brilliantly, keeping the audience in gales of laughter with tales of her experiences with Dr Wonke, first describing her times as a junior sister in the thalassaemia clinic and, in later years, her international travels with Dr Wonke to conferences and clinics overseas. Emma bore up bravely until, finally describing how a huge clap of thunder sounded as Dr Wonke cleared her desk on the last day, she broke down, as did the rest of us, including Dr Wonke and her family! It was a wonderful tribute to a beloved colleague and mentor and we are proud to reproduce a transcript of Emma’s speech on page 9. There followed a number of speeches from long-standing patients of Dr Wonke’s, some of whom had first come to her as children and who now stood before us as adults, professionals and in some cases parents. Above all, their words brought home how Dr Wonke had inspired them Continues on page 3 ➡
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Dr Beatrix Wonke retires– farewell to a pioneer in the modern treatment of thalassaemia
July 2004 ISSUE NUMBER 98
evening) made a short speech of welcome,
to which an emotional Dr Wonke
responded. Thereafter the guests enjoyed
an excellent buffet supper before the
commencement of the formal speeches,
usually the least interesting part of any
event but on this occasion the highlight
of the evening. First was Emma Prescott,
Thalassaemia Nurse Specialist at the
Whittington Hospital and Dr Wonke’s close
and trusted colleague of more than 12
years. Emma performed brilliantly, keeping
the audience in gales of laughter with tales
of her experiences with Dr Wonke, first
describing her times as a junior sister in
the thalassaemia clinic and, in later years,
her international travels with Dr Wonke to
conferences and clinics overseas. Emma
bore up bravely until, finally describing
how a huge clap of thunder sounded as
Dr Wonke cleared her desk on the last
day, she broke down, as did the rest of
us, including Dr Wonke and her family!
It was a wonderful tribute to a beloved
colleague and mentor and we are proud
to reproduce a transcript of Emma’s speech
on page 9.
There followed a number of speeches
from long-standing patients of Dr Wonke’s,
some of whom had first come to her as
children and who now stood before us
as adults, professionals and in some cases
parents. Above all, their words brought
home how Dr Wonke had inspired them
Continues on page 3 ➡
www.ukts.org2
Mike Michael President
Costas Kountourou Vice-President
Maria Gavriel Secretary
Olga Demetriou A Secretary
Nina Demetriou Treasurer
George Constantinou A Treasurer
Avraam Demetriou Committee
Menuccia Tassone Committee
Andreas Yiannikou Committee
Dear Member
Welcome to the July issue of Thalassaemia Matters. This feature packed edition has the full story of Dr Wonke’s retirement party and an in depth interview with Dr Shah.
On the medical front we have a piece about the new portable MRI unit from the Royal Brompton Hospital bring the T2* sequence to the thalassaemics up and down the country. Just think a non-invasive test that shows iron loading in both the heart and liver.
Dr A. Mandeville, a clinical health psychologist working in the NHS provides us with an interesting report into the psychology of thalassaemia; this is still an area of thalassaemia that requires a great deal of research.
Thalassaemia awareness and the dissemination of information are some of the major concerns for any society and in this issue we have some reports from around the thalassaemia world both nationally and internationally:
Launch of The National Screening Programme
UKTS, on the nurses workshop held in May 2004
ABRASTA, the Brazilian Association of Thalassaemia, on their conference
To provide balance for all of this important medical information we have a selection of personal experiences and news from around the world.
Finally I would just like to remind thal’s traveling abroad during the holidays to remember the following:
Take all your medications;
Take a letter from your Dr to explain why you have these drugs;
Adequate travel insurance (see last issue, or phone the office);
Look after yourself; and most importantly,
ENJOY YOURSELVES
Until the next issue
M. Michael President
A word from our President
The UKTSManagementCommittee
Aims & Objectives
of UKTS
■ The relief of persons suffering from thalassaemia.
■ The promotion and co-ordination of research in connection with thalassaemia.
■ To educate people on the problems of thalassaemia.
■ To offer counselling to sufferers and carriers.
■ To bring together patients, their families and well-wishers to exchange ideas and information.
■ To raise by any legal means the funds required for the above activities.
Thalassaemia Matters ...continuing the fight against Thalassaemia 3
The NHS Sickle Cell & Thalassaemia
Screening Programme (formerly the
NHS Haemoglobinopathy Screening
Programme) has been established since
2001. The Screening Programme is
part of the wider UK National Screening
Committee. The overall aims are to
implement a linked antenatal and newborn
screening programmes for sickle cell and
thalassaemia. And raise awareness of these
disorders and improve understanding of
the value of the screening.
Newborn screening: The policy for newborn screening in
England is for the phased implementation
of a programme, which will allow the
offer of sickle cell screening to all infants
as an integral part of the newborn
bloodspot screening programme. The
technology currently used can also
detect � thalassaemia major, but cannot
reliably detect the other variants e.g. �
thalassaemia intermedia
Screening newborn babies for sickle
cell disorders is being integrated into the
established bloodspot or ‘heel prick’ test
taken at 5 to 8 days after birth.
Newborn Screening Programme Aims:To achieve the lowest possible childhood
death rate and to minimise childhood
morbidity from sickle cell disorders.
Antenatal screeningThe policy for antenatal screening in
Antenatal Screening Programme Aims:To offer a sickle cell and thalassaemia
screening programme to all women and
couples in a timely manner in pregnancy to
facilitate informed decision making.
Progress of ImplementationThe implementation of the newborn
screening programme is underway, as of
June 2004, 56% of babies born in England
are being offered screening for sickle cell
disorders and the rollout of the newborn
screening programme should be complete
by March 2005.
The implementation of the antenatal
programme is also underway beginning
with the rollout of screening for sickle cell
and thalassaemia in high prevalence trusts
in the first instance. Further information is
available from the following web site:
http://www.kcl-phs.org.uk/
haemscreening/
Training & EducationAdequate training of health care
professionals is key to the successful
implementation of the Programme.
The Programme has commissioned the
Regional Antenatal Co-ordinators to
manage the training of Midwives and
Health visitors in their regions. The aim will
be easily accessible training, enabling the
Health care professionals (HCPs) to:
• Be aware of the screening
programmes polices and timetable for
implementation
• Have an understanding of what
haemoglobinopathies are and
pathophysiology of the disorders
• Have a basic understanding of the
genetics and how it is inherited
• Have an understanding of the screening
process
• Be aware of what treatments are
available and the impact of treatment
• Be aware of the prevalence in their areas
• Be aware of practical issues related to
taking blood spot samples for newborns
and the timing of for antenatal
screening
The Programme has also commissioned
the PEGASUS Network to develop
and deliver training for sickle cell and
thalassaemia screening for all relevant
Health Care Professionals. To develop
training for the professional functions
in relation to newborn and antenatal
screening for sickle cell and thalassaemia.
The overall aims are:
• To research, develop and facilitate
training that enables health care
professionals to respond proactively
and effectively to haemoglobinopathies
and those they affect, as part of the
implementation of national screening
for sickle cell and thalassaemia.
• To promote appropriate knowledge,
skills and attitudes, including effective
communication skills and responding to
diversity, among NHS professionals in
relation to haemoglobin disorders.
• To promulgate the mainstreaming
of genetic education in the NHS by
emphasizing generic and transferable
learning.
The PEGASUS Network plan to be up
and running by January 2005
Communication & Raising Awareness
The Programme has commissioned
Media Strategy Limited to work with
the key stakeholders to produce a public
awareness campaign and raise awareness
in healthcare professionals and users
about antenatal and newborn screening
programmes. The objectives are to:
• Improve the publics awareness of Sickle
Cell & Thalassaemia in order to improve
the acceptance of being screened in
communities at highest risk and within
the general population
• Improve the visibility of sickle cell and
thalasssaemia within communities at
highest risk and within the general
population
The Programme had its official launch
in Birmingham on 7th July 2004 linked to
the opening of the newborn laboratory
extension at Birmingham Children’s
Hospital and the Sickle Cell Awareness
Month. Ms Elaine Miller (Assistant Co-
ordinator UKTS) alongside representatives
from the Sickle Cell Society and health
care professionals have been media trained
and numerous spokespeople have been
involved in the awareness campaign so
far. The Screening Programme would like
to take this opportunity to thank all those
that have supported and participated in all
aspects of the implementation.
Community EngagementThe Programme has also commissioned
Dr Karl Atkin based at Leeds University
to start the process of community
engagement with the aim of exploring
how ideas about faith and religion mediate
South Asian and African-Caribbean
communities’ broad views of screening for
haemoglobinopathies and more specific
attitudes to pre-natal diagnosis and also
produce recommendations for the wider
public awareness campaign.
Evaluation ProcessThe Programme is also in the process of
commissioning organisations to evaluate
the communications strategy and to
make recommendations on improving
the process of targeting the stakeholder
groups. It will also advise on the approach
to evaluation and the impact of these
developments.
Sandra Anglin,
Programme Implementation Manager
July 2004
Continues from page 3
Thalassaemia Matters ...continuing the fight against Thalassaemia 5
latest news latest news
Please tell us something about your
background – eg where you were
born, educated etc. Do you have any
children?
I was born in Pakistan and came to
England as a baby. I grew up in London.
After I qualified as a doctor I did my basic
training in local London hospitals and
then went on to do my haematology
training on the University College London
Hospitals rotation. I have four children.
When did you know that you wanted
to study medicine? What made
you interested in working with
thalassaemia?
I have wanted to be a doctor ever since I
can remember.
When I worked at the Whittington
hospital in 2000 as part of my
haematology training I saw the way
Dr Wonke and Dr Parker looked after
their patients and became interested
in patients with Haemoglobinopathies.
They both actively encouraged me
to consider working in thalassaemia.
My pivotal moment really came in Dr
Wonke’s clinic when I saw a patient who
had not been transfused sufficiently
and had thalassaemic facies and growth
retardation. I then worked with Professor
John Porter to gain more experience in
thalassaemia and also to understand the
scientific concepts behind management
of thalassaemia.
What do you think are the most
important elements of a successful
thalassaemia unit?
The most important element that
underpins the provision of a good service
is the ‘patients’. Our job is to encourage
and support the people who live with
thalassaemia in all that they do, so they
can lead long and healthy lives due to
good adherence to treatment. From the
delivery of service perspective the things
that contribute greatly to a successful
unit is a combination of excellent
nursing, well informed doctors with an
interest in thalassaemia management,
good laboratory support and access
to professionals with expertise in
thalassaemia complications related to
their specialities.
Do you see thalassaemia treatment as
a co-operation between patients and
medical staff?
There is no doubt in my mind that people
who live with thalassaemia day in day out
all their lives are experts in the realities
of living with thalassaemia. The good
doctor is the one that encourages them
to lead productive lives and fulfil their
dreams but at the same time educates
and encourages adherence to treatment
so that the quality of life is good enough
for them to achieve their aims.
Which attributes do you think make a
good doctor?
Sound clinical knowledge and a never
ending thirst to find better ways of
treatment.
To have the courage to deal with
things that might be difficult.
The ability to listen to what the patient
is telling you, not just verbally but by
their actions as well.
Approachability (your patient
should have the confidence to tell you
everything and know that they will never
be judged by you but be supported and
guided instead)
How do you think you can make a
difference to the patients under your
care?
The Whittington thalassaemics have had
one of the most highly respected doctors
in the world looking after them for many
years.
I am a different person, younger and
with a different style. I will continue to
provide the Whittington thalassaemia
patients with the highest standard of care
that they have always received. I hope
that once everyone meets me we can
develop good working relationships.
With regards to ‘make a difference’
that will depend very much on each
individuals needs and requirements.
If you were not a doctor, what would
you be?
If you asked me this when I was younger
I would have said architect. Now I would
say gardener!
Introducing Dr Farrah Shah, New Consultant Haematologist at the Whittington Hospital
Continues on page 6 ➡
www.ukts.org6
latest news
On 5th May 2004 UKTS was delighted to
present the first national conference for
nurses and counsellors of thalassaemia
patients, at King’s Fund, Cavendish Square
in Central London. We at UKTS have been
contemplating holding such an event
ever since the first national thalassaemia
doctors’ conference in June 2002; and
feedback from our national conference
in November 2003 convinced us that
this was the time to go ahead. Work on
the event began in early February 2004.
Invitations and posters were sent to all
the Thalassaemia Centres and treating
hospitals. The Organising Committee
(see below) who assisted UKTS in putting
together the conference are all nurses/
counsellors and the theme of the day was
“Nurses talking to nurses”. Our primary
aim was to reach those hospitals with
only one or two thalassaemia patients, as
we particularly wanted to make contact
with the staff there to ensure that they
had the opportunity to hear information
on the latest treatment and network with
colleagues from the larger centres. Happily
we received an excellent response from the
nurses and counsellors and by 5th May all
available places were taken.
The delegates were welcomed by
Mr Niall Dickson, Chief Executive of
King’s Fund and then followed the first
presentation from Mike Michael, our
President, on the subject of “Living with
thalassaemia”. As always, Mike’s laid-back,
self-deprecating style and humour were a
great hit with the audience as he described
how he faced the different stages and
experiences of life as a thalassaemia
patient. One of his concluding remarks
which really struck a chord however,
was; “I don’t live with thalassaemia
– thalassaemia lives with me.”
There followed presentations by Dr Anne
Yardumian (Overview of beta thalassaemia
major), Sr Andrea Hulston (Blood
transfusions, reactions & complications)
and Sr Lynne Mathers (The role of the
paediatric nurse in beta thalassaemia
major). After the lunch break the delegates
heard from Sr Emma Prescott (Adult
management, complications & endocrine
problems). Then followed an audience/
panel discussion session, chaired with
her usual flair and aplomb by Professor
Elizabeth Anionwu. This gave the delegates
the opportunity to put their questions to
the panel, mostly comprised of patient
representatives. The discussion was lively,
humourous and thought-provoking.
Although this was the longest session of
the day there was not nearly enough time
to address the many questions. The final
presentation of the day was “The latest
developments in chelation therapy” from
Dr Antonio Piga, who had travelled all the
way from Turin in Italy to be with us.
The delegates were asked to complete
evaluation forms so that we could get
their views of the day. We were naturally
delighted to discover that the response
was overwhelmingly enthusiastic and
very complimentary regarding the venue
and general level of organisation. The
Nurses Talking To NursesUKTS Host The First National Conference for Thalassaemia Nurses & Counsellors
What do you do to relax when you
are not working? Do you have any
hobbies?
Relax! Difficult to find the time! I do
like gardening however and when I
find things getting too much then a
couple of hours in the garden taking my
frustrations out on the weeds and pests
and nurturing my roses and lawn is time
well spent!
What is your most prized possession.
I prize no object but relationships with
people are very important to me. I never
choose to break off friendships and many
of the people I have looked after still
keep in touch with me.
What is your idea of perfect happiness
I am a realist, there is no such thing as
perfect happiness, but you can have a
good happy and productive life if you have
a good family life and excellent colleagues
such as Sister Emma Prescott and my
consultant colleagues to work with.
Continues from page 5
Nurses talking to nurses - no spare seats
Thalassaemia Matters ...continuing the fight against Thalassaemia 7
latest news latest news
comments section of the forms included
the following;
“Unbelievable to find an event of this
quality provided free of charge.”
“Fantastic – please make this an annual
event.”
“Well done UKTS – stimulating,
informative, great for networking - do it
again.”
“A motivating and inspiring day.
I would like to thank the patient
representatives for their openness and
honesty.”
“A well planned day from beginning
to end. This is the best conference I have
been to.”
Needless to say the response made the
hard work of planning the conference
worthwhile and rewarding. UKTS would
like to thank the members of the organising
committee for giving up their valuable
time to help us. We could never produce
events of this quality without their help
and expertise and we therefore gratefully
acknowledge the contributions of:
Joint chairs of the organising committee
Professor Elizabeth Anionwu CBE, &
George Constantinou of the UKTS
Management Committee
The organising committeeEmma Prescott, Lynne Mathers, Marjorie
Ferguson, Despina Karretti, Susie Davidson,
Christine Williams, Elaine Miller
Chairs, Presenters & Panel members (in alphabetical order)Prof Elizabeth Anionwu CBE, Head
of the Mary Seacole Centre for Nursing
Practice, Thames Valley University, Patron
of the Sickle & Thalassaemia Association of
Counsellors (STAC)
Mr George Constantinou, Asst
Treasurer, UK Thalassaemia Society, patient
representative, London
Dr Aamra Darr, Senior Research Fellow,
Centre for research in Primary Care,
University of Leeds
Ms Preeti Dhanak, State Registered
Nurse, patient representative
Ms Maria Gavriel, Secretary, UK
Thalassaemia Society, patient representative
Sr Andrea Hulston, Transfusion Liaison
Nurse, National Blood Service, Birmingham
Ms Maserat Lal, Leeds/Bradford
Thalassaemia Support Group, patient
representative
Sr Lynne Mathers, Paediatric
Thalassaemia Nurse Specialist, Birmingham
Children’s Hospital
Mr Mike Michael, President,
UK Thalassaemia Society, patient
representative, London
Dr Antonio Piga, Consultant Paediatric
Haematologist, University of Turin, Italy
Sr Emma Prescott, Thalassaemia Nurse
Specialist, Whittington Hospital
Dr Allison Streetly, National Director,
NHS Sickle Cell & Thalassaemia Screening
Programme
Ms Christine Williams, Chair of the
Sickle & Thalassaemia Association of
Counsellors (STAC)
Dr Anne Yardumian, Consultant
Haematologist, North Middlesex Hospitals
Prof Antonio Piga
Launch of New Mobile MRI ScannerFor a long time thalassaemia patients
throughout the UK have bemoaned the
fact that they had to travel to London
in order to undergo their heart scans.
However, a significant step towards
rectifying this situation has been taken with
the launch of a new mobile cardiovascular
magnetic resonance scanner. The scanner
was presented to the NHS Trust by CORDA
(The Coronary Artery Disease Research
Association), and we would like to
congratulate their magnificent fundraising
effort which made this wonderful donation
possible.
The scanner was launched by Her
Royal Highness Princess Alexandra at the
Royal Brompton Hospital on 15th April
2004. A reception was held afterwards,
during which Professor Dudley Pennell,
Professor of Cardiology and Director of
the Cardiovascular Magnetic Resonance
Unit gave an address thanking CORDA
and outlining the benefits of the scanner,
with particular reference to thalassaemia
patients. Professor Pennell gave the
encouraging news that over the past
four years, since cardiovascular scanning
has been available to patients, mortality
in thalassaemia has decreased by an
astounding 80% (ref. the Thalassaemia
Register). He also stated that the mobile
scanner has already made a trip to
Sardinia, where it was used on 300
thalassaemia patients. This enabled the
medical staff there to identify 5 cases
of previously undetected heart failure in
young people; of a severity which would
undoubtedly have led to death within the
year if left untreated. Happily, all 5 patients
are now being successfully treated and
Continues on page 8 ➡
www.ukts.org8
open letter to patients
Dear Patient,
As you are aware, Swedish Orphan
International are the distributors throughout
the UK, EU & several other countries of
Ferriprox (deferiprone) tablets.
Ferriprox tablets are manufactured
by Apotex of Canada. They have been
thoroughly researched for many years
& are now in fact licensed in over 40
countries worldwide for the treatment
of thalassaemia, with 9 other licence
applications pending. These tablets are
consequently freely available on standard
UK prescription from UK doctors.
As Ferriprox tablets have been licensed
within the UK & indeed throughout the EU
for over 3 years, we have been increasingly
disturbed in recent months to discover
that a significant number of UK & Swiss
thalassaemia patients have been receiving
a variety of unlicensed generic deferiprone
preparations. Such supply is completely
illegal when a licensed alternative is
available for physicians to prescribe.
Many (but by no means all) such patients
have been receiving these unlicensed tablets
under the auspices of “trials”, whereby they
are monitored as part of a “named-patient
program”. Such an arrangement basically
enables the treating hospital to receive as
many unlicensed deferiprone tablets as they
can acquire legally “within a trial protocol”.
The real motivation for doing so is clearly
the fact that the hospital obtains such
unlicensed tablets for free, as deferiprone as
a compound has already been extensively
researched for over 15 years, predominantly
in the form of branded Ferriprox.
The purpose of a clinical trial should
be purely to collate data regarding the
safety, efficacy or tolerability of a drug,
not to enable treating hospitals to obtain
cheap or free alternatives to established
licensed & researched products. Therefore,
the evident moral / ethical issue here is
should thalassaemia patients continue to
be enrolled into needless trials involving old
unlicensed preparations as a cost-saving
exercise, when no apparent outcomes are
being determined?
Throughout the UK of late, a number
of patients based in different parts of the
country have been receiving one particular
unlicensed preparation illegally. This
situation manifested itself late last year
after two particular patients experienced
serious side effects, since when more have
come to light. Clearly, neither Swedish
Orphan International nor Apotex ought
to be fielding calls from alarmed patients
& healthcare professionals when things
go badly wrong involving an unlicensed
deferiprone preparation not manufactured
by ourselves & one which is in fact distinctly
different in terms of both its physical
composition & its bioavailability within the
human body.
The Criminal Investigation Unit of the UK
Medical & Healthcare Products Regulatory
Authority is consequently investigating this
matter & assisting us to establish the full
extent of this illegal unlicensed deferiprone
usage.
In recent years Swedish Orphan
International have consistently supported
numerous initiatives & congresses held
both within the UK & in many countries
worldwide. More importantly, in-depth
clinical support & literature is also readily
available with regard to licensed Ferriprox.
By contrast, the 3 main suppliers of
unlicensed generic deferiprone provide
no financial support whatsoever to any
such events or initiatives & judging by the
number of enquiries this office receives with
respect to unlicensed alternatives outside
our responsibility, provide little if anything
with regard to clinical information to either
healthcare professionals or patients.
Thalassaemia patients should therefore
seriously consider (if they are presently
receiving any other form of tablet or
capsule than branded Ferriprox), why
they should be required to give written
consent to being unnecessarily “trialled”
on such drugs. They are perfectly entitled
to insist that they receive Ferriprox from
their physician, should they already have
been deemed suitable for any kind of oral
chelation. Furthermore, if no such consent
has been obtained from them, then an
even graver situation could exist.
In essence, Swedish Orphan International
wish to ensure that all thalassaemia
patients receive optimal treatment &
care appropriate to their individual needs
involving all available researched & licensed
chelators.
Paul Bellas.
Marketing Director Europe,
Swedish Orphan International
have an excellent prognosis. This clearly
demonstrates the life-saving capacity of
these wonderful machines.
Following the speeches, Princess
Alexandra unveiled a commemorative
plaque and was presented with a bouquet
by 11-year-old thalassaemia patient Shahi
Ghani (some of you may remember Shahi’s
mother, Mrs Fathima Ghani, who spoke
about her experiences as a parent at the
UKTS Compliance Workshop in 2002).
Shahi, who attends the Whittington
Hospital for his thalassaemia treatment,
is getting to be quite an old friend of the
royals, having previously met the Queen,
Princes William and Harry and the Duchess
of York! Three years ago Shahi became
the youngest ever patient to go through a
cardiovascular magnetic resonance scanner,
when he had his first scan at the age of
eight. Jennifer Jenks, Chief Executive of
CORDA, said “Shahi is originally from the
Maldives and his family moved over to
England so he could be treated, He is an
amazing boy who has shown immense
courage throughout his illness.”
Continues from page 7
Launch of New Mobile MRI Scanner
Thalassaemia Matters ...continuing the fight against Thalassaemia 9
open letter to patients farewell and thank you to Dr Beatrix Wonke
Most of us here tonight have no idea of the
life that has made you into the person that
we all know and love. So tonight Dr Beatrix
Wonke, this is your life.
Born in Budapest, at some point this
century, nobody is really quite sure when,
you had a very hard start, growing up under
the repressive communist regime governing
Hungary at that time. You were even denied
access to the education that you had always
craved. Your dream, from the age of six, to
be a doctor, was becoming evermore distant.
It was clear to you that if you were to remain
in Hungary, you would never fulfil this dream.
Following a short-lived and unsuccessful
revolution by the Hungarian people, you and
your brother embarked on a perilous journey,
to escape this oppressive regime. In order
to get there however, you had to negotiate
many obstacles, swimming across rivers in
the middle of the night, chased by patrol
dogs, sleeping rough during the day and
travelling under the cover of darkness, not
that dissimilar it seems to your time at the
Whittington Hospital. Nothing changes.
Once in Austria, separated from your
brother, you enrolled in high school and were
later offered a place in Switzerland to study
medicine. Apparently, in order to fund your
studies you waited on tables in a restaurant,
a restaurant where for some reason, no
one ever complained about the service. You
qualified as a doctor in 1963 and in the same
year moved to Highgate in London, where
you still live. A house, where your wonderful
children Andrea and Anthony grew up, and
where your beloved grandchildren now come
to play.
Having completed your post-graduate
training at the Royal Free Hospital in
1977, you were appointed Consultant
Haematologist at the Royal Northern
Hospital, a hospital that I know still holds very
fond memories for you. It was here that you
first developed your passion for thalassaemia,
something for which everyone here is
extremely grateful. Unfortunately, there aren’t
many colleagues of yours left from that era,
but there is however a formidable trio still
present, here tonight, namely: Theresa, Lynne
and Gareth. They have asked me to mention
just a few of the fond memories that you
all share. They are as follows: Gin kept in
a drawer, for times of extreme stress only.
Touting for cigarettes off somebody called
‘Butch’, for times of extreme stress only, a
‘mind blowing’ rendition of ‘O sole mio’,
and finally rushing into the lab with a blood
sample asking if someone could give you a
‘quickie’, a blood result that is. My favourite
however, is the story told to me yesterday,
of a time when a lymphoma patient of
yours asked you for a medical report for the
local court, stating that his chemotherapy
treatment had seriously affected his memory,
resulting in him forgetting to change his bald
tyres and renew his road tax. What we all
want to know is, did you write it?
In the mid 1980’s, you moved up to the
Whittington Hospital, which is where I first
met you 12 years ago, as an innocent and
naïve junior sister. I remember being called
to your office to introduce myself; and I can
safely say I speak on behalf of all the patients,
parents, doctors and nurses here tonight, you
will never have any idea what a daunting
experience that was. In your unique ‘Beatrix
Wonke’ style, you peered over your glasses
and said: ‘Well? What experience do you
have, what can you do for us?’ I remember
mumbling some sort of an answer, leaving
your office and praying to God that you
didn’t have many inpatients on Mercers
Ward.
But things changed over the years. You
introduced me to your thalassaemia family at
a very early stage, gradually bringing down
one patient at a time, thinking we wouldn’t
notice, very subtle. Then you opened your
own unit, a unit you fought for in your usual
tenacious and determined way. Next thing I
knew, I was sitting with you in your clinic at
9 o’clock at night, and I have never looked
back since.
The clinic: One of my first memories of this
clinic was when a teenager I had never met,
walked in looking very scared and very shifty.
Dr Wonke, you reminded me then as you
have done several times later, of my former
Latin teacher, another formidable woman
who could terrify you with one look. You
appeared to completely ignore this young
boy, your eyes pointedly scanning all his latest
test results. The room was deathly silent and
I sensed a feeling of doom in the air, just
as there had been that day I hadn’t done
my homework. Finally, you lifted your head
towards the boy, peered over the glasses, and
simply said; ‘Do you want to die? If you do
then please leave my clinic and do not waste
any more of my time.’ When the sobbing
boy left the room, you looked at me, winked,
and said; ‘Now he will do his treatment’.
I immediately thought back to my Latin
teacher and realised what you both have in
common and why I admired you both so
much, strong compassionate women, who
will defy all convention and stop at nothing
to get the very best out of your wards.
Another thing I realised very early on was
that, like your patients, I could never hide
anything from you, whether it was trying to
A Tribute To Dr Wonke By Her Colleague Sr Emma Prescott
Continues on page 10 ➡
Our President Mike Michael presents a bouquet from UKTS
www.ukts.org10
farewell and thank you to Dr Beatrix Wonke
cover up something I had forgotten to do, or
whether something was troubling me in my
personal life. Within seconds you would pick
up that something was wrong. As with your
many patients, you were interested in me,
Emma the person, not just your colleague
‘the nurse’, or in your greatly appreciated
words, your ‘partner’. My family and friends
who have never met you, but have listened
to the many tales about you, feel as if they
know you and all of them share in our
sorrow that you have to leave us.
But it isn’t just your own patients at home
you care about, you have changed the lives
of people worldwide. One of my first visits
abroad with you was to Calcutta. You might
remember that the trip really didn’t get off to
a good start, as a result of the plane missing
its front windscreen. Not to be beaten by
this very small inconvenience, you tackled
the desk staff in your usual diplomatic
style, concerned that we would miss our
connecting flight. I remember a young
smiling British Airways girl cheerily offering
us a flight to Bombay instead. When you
asked her if she could get us from Bombay
to Calcutta, she politely told you ‘No’, but
cheerily pointed it out that it was closer
than London. It was at that very moment
that I genuinely feared for her young life.
Surprisingly unlike you, you lost your temper.
However, as always, your belief that nothing
is impossible paid off. A fellow passenger
having overheard this discussion intervened.
As luck would have it her husband happened
to be the pilot on our connecting flight.
Within minutes she had called him and
instructed him to delay his flight until we
arrived, which as you may remember resulted
in 600 passengers having to wait over 2
hours, on a plane on the tarmac, until we
were ready to board. To add insult to injury,
within minutes of take-off, a message came
over the tannoy asking if you would like to
use the fax machine in the cockpit just so
you could let Calcutta know you might be
a little late. If I remember rightly, we were
shortly after, invited to join club class for
our ‘convenience’. Needless to say, we left
Calcutta airport pretty quickly the following
morning. But once again, this showed me
that with you Dr Wonke, absolutely anything
is possible.
On a more serious note however, once we
had arrived in Calcutta I witnessed something
that will remain with me for the rest of my
life, and that is, what someone like you,
can really achieve in a situation, which to
most people would seem impossible. We
saw many children, all of whom seemed to
be in a hopeless state. You could not and
would not accept this. Through your sheer
determination, strength, compassion and
basic common sense you made realistic and
achievable plans for these children, many
of whom I was delighted to see two years
later, doing extremely well, a tribute to their
own doctors and most importantly to you Dr
Wonke.
The most amazing lesson you have taught
me, and many other of your colleagues, is to
continuously fight for our patients, regardless
of what others say or do, and to this end,
you have quite happily, fallen foul of many
a manager at our hospital. Tirelessly, even
to the end, you have persistently battled
with anyone and anything that has stood
in the way of your patients’ well being. Just
last week during your hospital leaving party,
you continued to fight, in fact for someone
here tonight. Your words were; ‘Nothing
is impossible if you plan, she is not just a
test-tube with a number on it, what if this
was your daughter, wouldn’t you fight for
her?’ As one colleague put it; ‘Dr Wonke
is like a tea bag, you’ll never appreciate
how strong she is until you put her in hot
water’. Another innate ability of yours is to
‘sniff’ out other experts who can help in
your quest for better treatments for your
patients, whether it be an initially cynical and
reluctant reproductive endocrinologist, who
now, proudly informs anyone who will listen
that he has yet again, made a thalassaemia
woman pregnant; or the dedicated cardiac
team, past and present, that is: Malcolm
Walker, Lisa Anderson, Mark Westward, Sally
Holden and more recently Mark Tanner, who
have all asked me to tell you that they will
remember you with such affection and not to
forget all the fun times we’ve together. Sister
Bernadette Clark has specifically asked me to
mention what an inspiration you have been
to her and her nurses on Mercers Ward, and
to express how your dedication has ‘rubbed
off’ on even the most ‘trickiest’ of her nurses.
The last few weeks have been very difficult
for all of us here, but most of all for you Dr
Wonke. I watched you earlier this week as
you cleared your office of all the things that
remind us of how special you really are. You
have for many years collected pictures of the
children, not thalassaemia children but sons
and daughters born to your thalassaemia
patients. A direct snub to all those who said
it was not possible and an inspiration to all
those parents and patients worldwide who
still believe it to be impossible. I watched
how you carefully and lovingly dusted the
frames before handing them over to me
so that we may proudly display them in
our unit, and I really couldn’t imagine what
thoughts must be flowing through your mind
and what memories must be flooding back. If
anyone was in North London, that day, they
will have heard a tremendous thunderbolt at
that precise time; I rest my case.
Finally, it breaks my heart to see you
leave but it also heartens me to wish you
all the happiness that you deserve in your
retirement, so that you may now be a little
less selfless and enjoy time with your precious
family. I very much doubt whether I will
ever meet someone again like you in my
professional or personal life, but regardless of
whether I do or do not, I will always have the
spirit and the drive that you have nurtured in
me and as your partner, I promise you that I
will do everything in my power to ensure that
your tremendous work will continue. I thank
you for everything and I love you from the
bottom of my heart.
Sr Emma Prescott
Continues from page 9
Thalassaemia Matters ...continuing the fight against Thalassaemia 11
farewell and thank you to Dr Beatrix Wonke farewell and thank you to Dr Beatrix Wonke
On Friday 23rd April 2004, Dr Wonke was given a surprise retirement party which had been organised by her patients as a token of their appreciation and love for her and all that she has done for them. This is the transcript of the speech written and delivered by Neelam Thapar who has seen Dr Wonke since 1977 (at the age of 9) when Dr Wonke first started working at the Whittington Hospital.
Dr Wonke, ladies and gentleman and
friends
I feel very honoured to have been asked to
say a few words to Dr Wonke, on behalf of
all her patients - her Thalassaemia family.
Dr Wonke, what I am about to say is an
attempt to reflect the feelings of your
patients, including those that were not
able to attend tonight, in honour of your
retirement.
You started at The Whittington Hospital
in 1977 with just 25 thalassaemic
patients - you now have over 120 at the
Whittington but we cannot even begin
to put a number on how so many more
you see from all over the UK and other
countries. Thalassaemia was a word that
meant nothing to people at that time.
There was no continuity in treatment, let
alone any significant research in to what
was becoming an increasing health issue.
You were just given a small group of
patients and told to do what you could
with them.
In an interview for UKTS in 1995 you
said that when you first started you found
Thalassaemia “fascinating and rewarding”.
This fascination has led to a glittering
working career where you have shown
us, your patients, extreme dedication in
innovative patient care improving and
extending the quality of our life , and
have at the same time also established an
international reputation in taking forward
research into Thalassaemia. We were so
proud to find out about the OBE because it
is excellent recognition for all that you have
been to us.
Words cannot really express or do justice
to what we really want to say to you. How
can we put into words the emotions we
feel and the respect we have for you - to
say thank you is not enough to someone
who:
• has been more than our doctor but
who has ultimately given us the gift of
life;
• someone who gave hope to our families
when all they felt was despair;
• someone who was passionate in
believing in us when we did not believe
in ourselves;
• someone who did not allow us to say
can’t or won’t but tried to encourage
us to think that we can do anything we
set our minds to providing we comply!!
• Someone who gave us confidence to
reach our own individual potential and
to try and begin to address that fear of
the unknown
Dr Wonke, your achievements are
numerous and there are so many things as
individuals we can say that you have done
for us. As a collective Thalassaemia group,
a few of the significant achievements you
have done to balance and improve the
quality of our life include:
• You have treated us with respect - you
were frustrated with us having to be
treated, when we were actually adults,
in the childrens’ ward and you took us
out of there by having a vision; a vision
that would give us the freedom, to
have our treatment at a time that suited
us. You fought to get our treatment
room on the adult Mercers Ward, and
you gave us Emma Prescott for whom
we are SO grateful. You have then
continued to have a vision to get us a
bigger better Unit which is being built
at the present time.
• You encouraged our families to allow
us to lead a normal life and not be
over protective - your words were often
Dr Wonke with Emma Prescott and patients from the Whittington Hospital
Continues on page 12 ➡
www.ukts.org12
“you have to be cruel to be kind”
when talking about giving the injections
• You promoted our use of the Baxter
pumps to increase the volume of
iron chelation we could do in a more
accessible way to help us continue with
our day to day life
• You were willing to research and
support the use of the tablet for those
of us who could not comply
• You referred us to wherever was
necessary, whenever was necessary
- if we had any additional difficulty you
always knew someone that could help!!
• You revolutionised our medical care by
helping to develop and sending us for
the special MRI scans. We know how
difficult it must have been for you, to
see the very people who had grown
up before your eyes, struggle with the
news that they had iron in their heart
and liver and then you had to develop
care plans to reduce that iron overload.
• You developed and implemented
strategies that involved us - You always
tried to find a way around the system to
benefit us.
But that’s what you have done throughout
your career - you have not allowed
beaurocracy to get in the way of putting
our needs first. One of the things about
family is that members give unconditional
love and that is what you have always
given us.
We know how much pain you must
have felt when you lost a patient - where
we could spend time grieving for a friend
or relative, you often had to go straight to
a clinic to see more patients and put on
a brave face. But all the losses seemed to
make you more determined to fight for the
cause and help us help ourselves.
We have shared with you our times
of happiness, sadness, anger and pain.
Sometimes we have been too confused to
even begin to express what we felt and at
times you have had to be very strict, hard
and even nag us!!!!
How many times have you had to say
these words to us;
“Use your Pump, take your L1, why
are you not complying!!!!!, “you look
black as soot”
What ever you have said to us, you
have done with a great passion for
wanting us to lead “normal” lives. You
have never been frightened to be truthful
and tell us exactly what would happen if
we did not comply. Often you vocalised the
things that we knew but were too scared
to confront. It has to be said that for
those of us that have left school, receiving
a letter from you at home still feels like
opening an exam result!!
However, you have tried to make sure
that we do not see ourselves as “ill”
people but people that can move forward
to achieve our goals and aspirations and
look at what we have as evidence of that:
Amongst your Thalassaemia family seen
at the Whittington we have:
• children who now have a treatment
regime which leaves no room for any
doubt that they will be able to have the
very fun that perhaps some of the older
ones did not experience in the very early
days.
• People who are in their forties and even
fifties
• people who are blessed with children of
their own and even a grandmother
• people who have achieved certificates
and letters behind their name or are
working towards that
• working professionals with a range of
careers
• people who have devoted time in
voluntary work to help others in the
Thalassaemia world
All this progress we owe to you. You
have been an inspiration to those here
and overseas. Your contribution through
TIF and those that you have kept alive
internationally goes without saying. Your
capacity to embrace the cultural diversity of
the Thalassaemia world is a credit to you.
When I asked some patients what words
they would use to describe you, these were
some of things said:
“mother”, “my rock” “guardian
angel” “wonderful” “dedicated”,
“gave me love “, “empowered me”
I think they speak volumes about the
love we have for you inside and the impact
that you have had on our lives.
Dr Wonke, you have never let any
problem serve as an excuse but have
always tried to see it as an opportunity. In
1995 you also said that you did not like
people not realising their potential and not
even bothering to try - nearly ten years on
from that interview, we are testimony to
the fact that you have helped us realise our
own potential by keeping us alive.
We shall miss your compassion, your
strength and your motivation - however,
because of your direct openness with us,
you have made each of us experts in our
own treatment to be able to carry on and
make you truly proud.
As sad and emotional as we are, we
know that you now deserve to have time
to yourself and your immediate family. We
hope that you will enjoy your holidays,
going to museums and galleries, listening
to music as well as your favourite gin and
tonic!
We know that you enjoy gardening and
rumour has it that unlike us, your flowers
have never been anaemic, but we know
they will be the best ones in North London
if they have any of the love and attention
you have given us helping us grow.
Dr Wonke , your extended Thalassaemia
family thank you for all your dedication,
encouragement and support . We wish
you much health and happiness for the
future and we love you very much.
Thank you
Neelam Thapar
April 2004
farewell and thank you to Dr Beatrix Wonke
Continues from page 11
Thalassaemia Matters ...continuing the fight against Thalassaemia 13
farewell and thank you to Dr Beatrix Wonke news from around the country
The Psychological Integration of Thalassaemics in the
21st Century
Thalassaemia Matters ...continuing the fight against Thalassaemia 15
medical news
of ‘trust’ in the doctor, which is arguably
extremely poignant when one has no
choice but to rely on the expertise of
another person to maintain one’s health.
Our research also suggested that patterns
of adherence were better in people who
believed strongly in their own capacity
to be an effective person in the face of
difficult tasks generally, than in people who
were less confident.
We also did several in depth interviews
about processes involved in adherence.
This research gave us some important
additional information. It suggested
that fully accepting the disease may be
important in helping to take treatment
consistently, as well as being able to
value ones health and take responsibility
for treatment. Interviewees said that
complications can actually be useful
in terms of acting as a ‘wake-up’ call,
helping people to fully take on board
the consequences of not adhering to
treatments. This may be particularly
important as the people we interviewed
commented that unlike illnesses such as
diabetes there is no immediate biological
‘feedback’ from missing treatment.
Interviewees also described a struggle
with processes such as ‘denial’. One person
expressed this by saying, ‘I think since I
was very little I always knew I would have
(Thalassaemia) for life. So it was a big time
span in my mind where I thought ‘if I miss
out on the odd day I can catch up’. It’s a
bit like a diet that you say you will start
tomorrow. But tomorrow never comes’.
Self consciousness may sometimes lead
to being selective about social activities
which may be exposing, such as going to
the beach and there was a general tension
about whether or not to keep the disease
‘secret’ from other people. Adolescence
was mentioned as a particularly difficult
time, whilst competing demands from
social and work commitments were a
theme in adulthood.
My own opinion of what is involved in
coping with the demands of Thalassaemia
comes from my experience of trying to
offer support to adults who are facing
particularly difficult challenges. I believe
coping with Thalasseamia involves a
complex interplay of many factors, which
actually start from a young age. As is true
for all of us our early experience tends
to form the ‘blue-print’ of our sense of
self and this can be particularly delicate
for young children trying to cope with
the ramifications of an illness such as
Thalassaemia. We can all relate to the
lasting effect of comments of bullies in the
playground in making us feel like we don’t
belong and that we are ‘different’ from
others and this can have a damaging effect
on self esteem. The presence of desferral
pumps, frequent visits to hospital or
missing time off school leave Thalassaemic
children particularly vulnerable. The way
teachers and parents help children with
Thalassameia cope can have a substantial
positive or negative impact in later life.
These types of things can still affect
confidence, even in adulthood.
If we are going through very difficult
periods in our lives such as bereavement
, separation, family or work difficulties
doubts about ourselves can be amplified,
perhaps ultimately leading to feelings of
depression and hopelessness about the
future. For people with Thalassameia the
demands of the illness and treatment can
seem absolutely overwhelming at times
of additional stress and a lot of courage is
needed to persist with life.
At such times the team must respond to
the needs of patients by taking the time to
really understand individuals’ circumstances
and emotions. In doing this the team is
then in a position to plan the best way to
help. Examples of this may include taking
decisions to defer non essential treatments
(such Hep-C therapy) at such times or
in extreme cases consider portacaths for
those who are just not doing treatment at
all. A psychologist can play an important
part in gaining a deeper understanding of
a person’s difficulties and helping the team
to plan the best response. In some cases it
has helped to provide individual psychology
sessions to look more closely at people’s
thoughts and feelings. My experience has
been that this can be helpful in allowing
people to express their true feelings
without fear of ‘burdening’ those around
them. This process may lead to people
seeing new ways of dealing with difficulties
or in helping them to reconnect to their
courage and passions which give them a
reason to carry on contending with the
challenges Thalassamia inevitably brings.
One study by Laerum (1987) found that
people with chronic illness may place more
value on close relationships, give greater
meaning to everyday activities and show
greater compassion towards people with
similar problems. I have certainly witnessed
this in the people with whom I have
worked who have continued to touch and
inspire me with their resourcefulness and
ability to ‘keep going’.
It goes without saying that access to
the best medical expertise and treatment
methods are pre-requisites to quality
of life and must form the backbone of
Thalassaemia services. Flexibility of clinic
and transfusion appointments to take
account of busy lives also seem to be
valued by our patients at UCLH. However,
there is much work still to be done to
increase our understanding of how we
respond to patients’ psychological and
social needs and how we can best support
them to maintain as much good health as
possible. More formal research is urgently
needed to help us explore and evaluate the
most important areas to target for further
intervention.
Dr Anna Mandeville
Consultant Clinical Health
Psychologist
Haemaglobinopathy Service
UCLH
medical news
www.ukts.org16
patient news
A parent’s experience(continued from Issue 97)
By Aggie Michael, President of the Thalassaemia Society of Singapore & mother of Valerie Kum, a 15 year old thalassaemia major patient.
The Cyprus conference in September
2002 was a workshop for the societies
representing Thalassaemics from around
the world. The representatives from most
European and American societies are
Thalassaemics themselves. Sadly, such a
grand, positive outlook has yet to reach the
Asian side of the world. Yet, to the handful
of Thalassaemics from India, Pakistan,
Taiwan and Singapore, the two days were
a moving experience. It was there that I
understand the true meaning of “leading a
life just like everyone else.”
During the tea breaks, we would mingle,
sit down with a cup of tea or coffee
and chat about work, family and kids.
There I realized that there is life beyond
blood transfusions, Desferal injections
and medication for Thalassaemics. There
is a whole world of friends and places
of interest out there beyond homes and
hospitals. There is so much to learn about
peace and chaos, control and freedom.
There is no limit to knowledge and
education. As we learn about treatments
of Thalassaemia from the doctors and
nurses, they in turn learn from the
Thalassaemics. It was an afternoon tea that
changed my life. It was at that afternoon
tea that I got to know Mike, my future
husband and President of UKTS.
There is something about Mike that is
silently reassuring. He projects the feeling
that he has all the time in the world for
anyone who needs a listening ear or a
shoulder to cry on. Like all successful
adult Thalassaemics, he has never
allowed Thalassaemia treatments to rule
his life, instead, he leads his life around
the treatments. The blood transfusions,
hospital visits and treatments required to
maintain his health have never been a
hindrance to how he lives his life. Mike
even travelled to Thailand and stayed for
a month there nine years ago. He had a
“full to the rim” blood transfusion just
before and right after the trip, and toured
the sights with disposable infusion sets
(balloons) attached for many hours a day.
At the Cyprus workshop, he presented
the objectives and roles UKTS played. As
Professor Bernadette Modell mentioned in
her speech during the opening ceremony
of TIF’s 2001 Conference, UKTS was
one of the world’s pioneer societies for
Thalassaemia. It funded the prototype of
the world’s first portable infusion pump,
and it also funded the research for oral
chelation, amongst many other ground-
breaking treatments for Thalassaemia.
One of the many friends I made at the
2003 conference in Palermo, Sicily, was
Costas Kountourou of UKTS. I remember
him constantly amongst Thalassaemics
from developing countries. He would
distribute donated Desferal-filled disposable
infusion sets (balloons) and ‘thumb-tacks’,
patiently explaining the functions and
usage, and guiding the patients into doing
the injections properly. Some of them
were so overloaded with iron that even
walking was painful. They did not have
proper education about treatments, even
Desferal was a luxury item to them and it
could be ages before treatments in their
countries are able to match those available
in the UK. But for those two days at the
workshop, they had a glimpse of hope.
Upon returning from Palermo, the
Committee and Sub-committee members
of the UKTS braced themselves to the
task of putting their 2003 UK Conference
together. Meeting once every week after
work, there were times when it was easier
to just call in pleading unable to attend.
They don’t get paid for their efforts, only
reimbursements for expenses incurred
related to the projects backed by receipts.
The amount of time spent travelling to
and from Nottingham to check the hotel
and its facilities. The communications with
guest speakers to ensure their attendance
and that their abstracts arrived in time for
putting together in the folders. The visits
to hospitals to make sure the posters were
being put up. The hunt for a reasonably
priced and fast turnaround printing
company. The nights of putting all the
material together in the conference bag.
Everything was being executed by the
small, tight team of about 10 volunteers
and one and a half paid employees. I am
amazed at the attitude of the handful of
volunteers. It’s a thankless job, putting
together something of this magnitude
which involved so much of their free
time, when they could have been out
doing other things to keep themselves
entertained, or just resting at home.
They do not behave the way the
Thalassaemics in my country do. I felt as
if I was amongst my peers or directors
in the office where I work when we are
organising an event. They brain-stormed,
gave ideas, argued and worked things
out. Thanks to the UKTS, the healthcare
for Thalassaemics in UK is one of the
best in the world. I’ve heard praises being
sung from fellow Thalassaemics and their
parents who have the resources to travel
to London for a consultation with the
specialists or a visit to the Thalassaemia
Units here.
UKTS was formed in 1976 by a
group of parents with the objective of a
Thalassaemia-free future for their affected
children. In the years that have passed,
their children have grown up, and though
they still have Thalassaemia, long gone are
the days of uncertainty. Almost all UKTS
committee members are Thalassaemics
themselves, having blood transfusions,
Thalassaemia Matters ...continuing the fight against Thalassaemia 17
patient news
nightly Desferal injections, daily drug
supplements and scheduled check-ups
as part of a routine. Today, they look at
Thalassaemia as a condition, just as some
people have sensitive skin or allergies.
They have gone through the normal
growth process just like everyone else,
and are individuals with mature mindsets.
Around the conference table they decide
whether or not to; fund research proposals
from professionals, answer requests
for assistance, release related medical
information, meet with hospital personnel
to improve Thalassaemia treatments and
organize awareness campaigns.
A parent myself, I was used to taking
charge, deciding what’s good or bad for
Valerie. As she grows older, I’ve learnt
to let her take over the responsibilities
of her treatment. If she delays her
blood transfusion, she will suffer the
consequences of anaemia which will
hinder her active lifestyle. If she skips
her Desferal injections, she will feel the
impact of overloaded iron in her body.
Thalassaemics have a very strong life force.
The more I expose Valerie to the world, the
stronger will to survive she develops. I can
see Valerie growing into an independent
adult, capable of making decisions, just like
any other adults we meet in our everyday
lives.
The forefathers (and mothers) of UKTS
have worked hard to make sure that their
children grow just as I do. Now is the time
to let our children to take over the rein.
We may not agree with everything our
children do, but it’s their time now. They
have survived researches being guinea pigs
themselves. They are more involved in the
treatments than we can ever imagine as
parents. They have experienced successes
and failures. They know what they want.
We should have faith in them to take
Thalassaemia to another level where we
have not been before, knowing it’s only for
the best.
patient news
My name is Michael, I am a 27 year old
Thalassaemia patient from Bristol. I thought
I would write some information on my
experience with Thalassaemia.
I’m not too sure exactly what to write
but hopefully any information I do will be
of some use.
I was diagnosed with the condition at
around 13months old when my parents
took me to the doctors as I had been ill
for a few months. I am now 27yrs old and
you can probably imagine that 26 yrs ago
the knowledge of Thalassaemia wasn’t too
great.
The medical advances made over the
years have been really significant and the
standard of treatment that patients receive
nowadays is first class.
I have always been positive in connection
with receiving my treatment and now think
nothing of it when visiting the hospital for
blood transfusions. I like to think of it as a
day off work!
The frequency of my visits to the
Children’s ward of Bristol’s Southmead
hospital varied from anything between 3
wkly to 6 wkly depending on haemoglobin
levels.
During my school years I would
sometimes stay overnight, as long as I
survived the hospital dinners!, then go to
school the next morning. I know it was
probably hard for my parents to have to
take me to the hospital all the time and
sometimes have to leave me there for
treatment but I’m sure they look back at
it now and fully understand it was for the
best, sometimes they would be able to stay
the night with me which would kind of
ease the stress.
I don’t know much about bone marrow
transplant procedures nowadays but by
the time it was mentioned to my family I
had been given alot of blood transfusions
and the risk was not thought worthwhile.
I know it would have been a chance at life
without Thalassaemia and all it in tails but
I know that having this disorder has made
me strong and made me the person I am
today.
As I got older and received more
transfusions I needed additional treatment
to overcome the excess iron I was receiving
from the blood. This was done by way of
desferal injections at home during the night
over an 8-10 hr period. I currently do this
for 5 nights a week but it varies dependent
how much iron is in the body. I remember
my mum practicing with a butterfly needle
on an orange in the early years as she was
the lucky one who had to administer it for
me. When I was old enough to do it myself
I was obviously scared but the more I had
to do it the easier it became. As long as
you are disciplined with this aspect of the
treatment, it can only help in keeping a
low iron/ferritin level, major problems with
hopefully be avoided. Remember the pump
is your friend.
I have coped quite well with the blood
transfusion sessions at the hospital and
also with the nightly injections, I put this
down to the great loving support of my
family and friends, including all the doctors
and nurses and associated staff I have met
during my time and from my girlfriend over
the last 4 yrs.
However I do remember a couple of
instances when it could be quite depressing
if I thought about it too much. Mainly
thinking why me? Why do I have to do
these injections all the time? Why couldn’t
it be someone else or not exist at all? But I
also used to think that there are alot worse
things that can happen to people and that I
had to be brave and get on with life.
Of course the waiting around in
outpatients for hours to see a doctor can
be boring and tiresome but it is good to
Continues on page 18 ➡
A patient’s experience
www.ukts.org18
patient news
Employment and Career Mattersby Neelam Thapar(Neelam is a Careers Adviser at London Metropolitan University and also a thalassaemia major patient at Whittington Hospital, London)
4th International Conference In BrazilTo coincide with International Thalassaemia
Day May the 8th, ABRASTA (the Brazilian
Association of Thalassaemia) held their
fourth international conference from
30.4.04 – 2.5.04 at the Costa do Sauipe
resort. The aim of the conference was
to update all physicians involved in
thalassaemia treatment in Brazil; and, in
particular, to focus on the diagnosis and
treatment of cardiac complications. Guest
presenters included; Dr Beatrix Wonke
(Health care for thalassaemic patients), Dr
John Wood (Pathophysiology of iron in the
myocardium), Dr Renzo Galanello (Treating
iron overload myocardiopathies), Dr Nelson
Hamerschlak (MRI cardiac iron assessment)
and Dr Sandra Almeida (Psychosocial
aspects of thalassaemia). Dr Wonke, Dr
Wood, Dr Galanello and Dr Fernando
Tricta jointly presented a proposal for
individualized chelation therapy based on
the T2* (MRI).
Thalassaemia Matters ...continuing the fight against Thalassaemia 21
news from around the world events
Congratulations to Michael and AggieWe are sure all our readers will join us in congratulating our President Mike Michael and his bride Aggie Tan, who were married on 12th June at Enfield Registry Office, North London
THE UK THALASSAEMIA
SOCIETY ANNUAL DINNER
& DANCE
will be held on
Saturday 27th November 2004
at
The Brewery Chiswell Street
London EC1Y 4SD
TICKETS £40 Available from the
UKTS office
N.B. For security reasons all tickets must be paid for in
advance.No places will be reserved unless payment has been
received.
UKTS at the Cyprus Wine Festival
The 2004 Cyprus Wine Festival was held at
Alexandra Palace on 26th-27th June 2004
and UKTS were out in force. Our stand
was manned throughout the two days
and we gave out leaflets and spoke to
hundreds of people on the importance of
blood screening for thalassaemia carriers.
We also held a raffle which raised £900 in
aid of Thalassaemia and we would like to
express our gratitude to HELIOS AIRWAYS
who kindly donated the prize of an airline
ticket to Cyprus. Our heartfelt thanks
to the many volunteers who assisted us
throughout the weekend.
www.ukts.org22
events
May 2nd (Bank Holiday Sunday) saw the
second “Greek Night” organised by a few
of our talented socialites (Maria Gavriel,
Olga Demetriou & Angelica Gavriel).
This year’s venue was the swanky KO
Club in Wood Green. After weeks of
organising, phone calls and flyer mail outs
by the ladies, this year’s club night was
another overwhelming success!
Special guest DJ Polis Polycarpou (LGR
Radio) with resident DJ Mikey Mike wooed
the crowd with their mixing of old and
new Greek tunes as well as adding in some
up to the minute UK chart toppers.
Many volunteers helped on the night at
the ticket booth and ensured the smooth
entry for the streams of enthusiastic
punters. However by 1am their services
were no longer needed as the club was at
capacity and there was no more room on
the already packed dance floor!
The crowd had a fabulous night, so
much so that KO management have
invited the organisers to hold another
‘club night’. A date has not yet been
confirmed however for those of you who
unfortunately missed out this time round
or who are looking forward to coming
to another be sure that we will keep you
posted on the next big ‘Greek Night’ out.
The UKTS would like to thank the
organisers above, volunteers and all those
who attended in raising over £3,700 on
the night.
Greek NightGreek Night
This year’s Flora London Marathon was
held on 18th April. We would like to thank
and congratulate our stalwart runners,
who performed brilliantly in spite of less-
than-perfect weather conditions. They are
(in alphabetical order)
Mrs Jackie Bevan
Mr Martyn Bevan
Mr Geoff Burrows
Dr Stephen Cray
Mr Darren Evans
Ms Amanda Fetti
Mr Tadgh Flanagan
Mr Richard Seabrook
So far almost £5,000 has been raised in
support of thalassaemia and some of the
runners are still collecting. We are more
than grateful to them, not only for their
performance on the day but for all the
long, hard hours of training and equally
long, hard hours of finding sponsors and
collecting the money! It is not an easy
task to find people to donate money to
a cause that few of them have heard of,
as we at UKTS are only too well aware.
Many thanks to the runners from all our
members.
Congratulations to our Marathon runners
Thalassaemia Matters ...continuing the fight against Thalassaemia 23
events office news
Changes in the UKTS OfficeAs our regular readers will be aware our
long-standing Co-ordinator Costas Paul
retired in December 2003. His assistant
Elaine Miller has now been promoted
to Co-ordinator and we are pleased to
welcome new part-time staff member Pat
Hayward who will assist Elaine in running
the office. Congratulations to both the
ladies on their new positions and thank
you for your continued hard work and
dedication!
The UKTS Personal Organiser
GIVE US YOURGOOD NEWS
Are you a thalssaemia patient (or a
parent) who has some good news to
tell us? At UKTS we are always keen to
hear of any achievements and success
stories from our patient members and
we are therefore thinking of including
a new “Congratulations” feature in
Thalassaemia Matters. The good news
can be anything at all, as all success is
relative and what would be routine for
one person can be a great achievement
for another! So please ask yourself if there
is anything you would like to share with
our readers – every achievement we can
print will be a source of encouragement
to other thalassaemia patients and their
families. Accompanying photographs
will also be very welcome.
Many thanks – we look forward to hearing from you.