Royal honour for Professor Anita MacDonald

36 Pages·2016·3.38 MB·English

Issue 151 Summer 2016
Inside this issue…
Your letters
NSPKU 2016
Conference review
Dietitian’s report
Prescription advice
A sister’s perspective
Royal honour
for Professor
Anita MacDonald
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K U 2 0 1 6w in sid e
N S P e vie
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In touch
Helpline 030 3040 1090
PO Box 3143, Purley CR8 9DD
The Council of Management
[email protected]
www.nspku.org
Eric Lange Caroline Bridges Iain Williamson
(Chair) (NSPKU Administrator (Secretary & Teenage/Adult
@NSPKU
27 Western Road, Sutton and Editor: News & Views) Co-ordinator)
Surrey SM1 2TE PO Box 3143 E [email protected]
facebook.com/groups/nspku/
E [email protected] Purley CR8 9DD E [email protected]
T 020 3397 7320 @pkuteens
Julia Bailey E [email protected]
(Vice Chair) Duncan Noble-Nesbitt
The treatment of phenylketonuria varies for
T 07790 572525 Lisa Lee (E.S.PKU Representative
each individual patient. No patient should alter
E [email protected] (Treasurer) & Advocacy)
their own treatment as a result of reading how
2 Brightstone Close 3 Devonshire Street
Paula Hallam Banks Ambergate, Belper another patient manages their diet without
(Society Dietitian) Southport PR9 8FH Derbyshire DE56 2GJ first consulting their doctor or dietitian.
65 Shortlands Road T 01704 226931 T 01773 853360
Kingston E [email protected] E [email protected] The NSPKU has received enquiries about
Surrey KT2 6HF
where it stands in relation to new treatments.
T 020 3397 7494 John Skidmore Paul Cardy
Below is the NSPKU’s policy statement which
E [email protected] (Support Group Co-ordinator) (Webmaster)
includes BH4:
4 Hall Croft, off High Street E [email protected]
Vicki King Normanton The NSPKU believes, without reservation, that
(Bookkeeper and West Yorkshire WF6 2DN Niki MacLeod people with Phenylketonuria (PKU) should have
2016 Spring Draw Officer) T 01924 890991 (2016 Conference Co-ordinator) access to clinically-proven treatments that help
Tarkwa, 65 Cromwell Road E [email protected] E [email protected] them cope with this hereditary condition.
Ribbleton, Preston PR2 6YD
E [email protected] Julie Cardy Peter Davies In the UK, people with PKU follow a strict ‘PKU
T 07830 452322 E [email protected] diet’. Whilst this works well for many families
E [email protected] and individuals, others have difficulties.
BH4 is a clinically-proven treatment for PKU,
Local contacts and support groups available in most European Union countries
but not in the UK. The NSPKU welcomes every
Support Group Co-ordinator: John Skidmore T 01924 890991 E [email protected] treatment with proven efficacy that may help
people with PKU and their families to manage
the condition and will advocate accordingly.
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News & Views
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While every care is taken in the preparation of
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NEWCASTLE SHEFFIELD Helen Scally of Management’s authority.
Ken Dodsworth Gillian Race 2 Berry View, Huddersfield
7 Lingey Lane, Wardley (Liaison Nursing Officer West Yorkshire HD4 6LQ
Gateshead – Health Visiting) T 07828 101191
Tyne & Wear NE10 8BR Neonatal Screening Laboratory E [email protected]
The National Society for Phenylketonuria
T 0191 469 3424 The Childrens Hospital
(United Kingdom) Limited
Sheffield S10 2TH
PO Box 3143,
T 0114 271 7415
Purley CR8 9DD
Registered in England
Medical Advisory Panel Company No. 1256124
Charity No. 273670
Heidi Chan Dr Alison Cozens Elaine Salmons Helpline: 030 3040 1090
Adult Metabolic Dietitian, Consultant in Inherited Metabolic Senior Specialist Nurse, Email: [email protected]
London Disorders, Edinburgh Birmingham Website: www.nspku.org
Registered address:
Dr Monique Cloherty Cerys Gingell Lucy White
Richard House
Clinical Neuropsychologist, Specialist Paediatric Dietitian, Metabolic Dietitian,
Winckley Square, Preston
London Nottingham Sheffield
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Dr Duncan Cole Dr Stewart Rust Dr Gisela Wilcox
Consultant in Medical Biochemistry Consultant Clinical Psychologist, Consultant in Metabolic Medicine,
& Metabolic Medicine, Cardiff Manchester Manchester Printed by Premier Print Group
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2 News & Views | Issue 151 | Summer 2016
Contents
Issue 151 | Summer 2016
10–11 26–29
32–33
14–17 34–35
Your letters 4 Editor’s Report
Letters and donations from NSPKU members.
Professor Anita MacDonald, OBE 10
It has been an absolute pleasure working with Paula over the last
News & Views is proud to celebrate Anita’s richly-deserved award.
three years. In that relatively short time Paula has made a lasting
NSPKU 2016 Conference review 14 impression on the NSPKU by bringing her kindness, knowledge,
experience and new ideas to the Society. Paula will still contribute
Sarah Ripley reviews the presentations and workshops from
this year’s conference at the Cheltenham Chase Hotel. to News & Views when she can and in this issue she brings us some
delicious salads and mouth-watering ways to liven up water as well
Prescriptions: How to get what you need, when you need it 22
as news from the supermarkets and low protein food manufacturers.
Advice from Barbara Cochrane to help overcome problems
that sometimes arise with the dispensing of low protein foods. We are fortunate to have so many dietitian’s out there working in
the field of PKU who have been very kind and supportive over the
Dietitian’s report: Salad days 26
past few months. Cerys Gingell and Anne Daly who organise and run
Great ideas for delicious summer dishes. Plus the latest news Edale each year, Sarah Ripley who organised this years conference
from manufacturers. and Barbara Cochrane who has provided a report for this issue on
prescriptions and who has been so brilliant with her support and
Perspectives: ‘My sister’s got this condition called PKU’ 32
advice to people who contact the NSPKU via email and the helpline.
Amber Oke has PKU. Her sister, Alicia, tells her story of living
with the condition from her point of view. Happy reading.
Take the high road 34 Caroline Bridges
Kylie Doonican travels from Australia to the 2015 conference
in Glasgow with her sons.
News & Views | Issue 151 | Summer 2016 3
Please send in your photos
with letters and donations
It’s always great to show pictures on these
pages, so please don’t forget to send in your
Your letters
photos. Digital images are preferable and as
long as they are large enough files we’ll do
our best to print them alongside your letters.
and donations Email: [email protected]
News & Views, PO Box 3143,
Purley, Surrey CR8 9DD
Dear NSPKU Dear NSPKU Dear NSPKU
Prior to Christmas, we asked our customers Please find enclosed a cheque for £130 for Please find enclosed a cheque in the sum of
to select a charity of their choice so we the Society. We are very happy to continue £33.04 for the NSPKU. This is a donation
could distribute an amount of money we our support for all your valuable work from the staff and parents at the nursery.
had to the charity they selected. We are connected with those who have experience Instead of sending each other Christmas
therefore delighted to make a donation of of PKU. I can confirm that this donation is cards, people were encouraged to write their
£100 in support of the great work you do. made within the Gift Aid provisions of the Christmas messages on our entrance notice
Treasury. board and leave a donation. We chose to
Best wishes,
donate the money to the NSPKU because we
With best wishes,
Victoria Bates, Axiom Engineering, currently have a child at the nursery who
Stockton-on-Tees John & Marina Darby, Edinburgh has PKU.
Yours faithfully,
Emma Wyer, Leapfrogs Nursery, Crediton
Dear NSPKU Dear News & Views
Thank you for the help and support We are delighted to enclose a cheque for
you have given our grandson Joe and £200. My daughter, Caoimhe, is four and
Dear NSPKU
his family over the first ten years of his has PKU. Her Uncle Shane and fiancée
life. We celebrated our Golden Wedding Fiona organised a table quiz and raffle in a We are delighted to enclose a cheque
Anniversary recently and suggested a local bar, Gribbens. I also donated the rest for £150 which once again is instead of
donation to the NSPKU instead of presents. of the money raised, £350.00, to our local sending Christmas cards. Our grandson
As a result I enclose a donation for £555. hospital, The Royal Victoria Hospital for Sick Hugo (now almost 3 years old) continues
Children, Belfast. to be a very lively and happy little boy
Keep up the good work.
and we are very grateful for the help and
Many thanks,
Many thanks, information from the society.
Erin McKenna , County Tyrone
Jane & Mike Willis, Ipswich John and June Fletcher, Aberdeen
Callum Parkinson Dear NSPKU
running the Manchester
Marathon in April. I’m writing to let you know that our
friend, Callum Parkinson, ran the
Manchester marathon on 10th April 2016
to raise money for the NSPKU.
He did really well and ran it in 3:12:29
and raised £606 for the society!
www.justgiving.com/Callum-Parkinson
Many thanks
Kelly Lang, Manchester
4 News & Views | Issue 151 | Summer 2016
Donations over £30 received Thank you to everyone who has donated
NWF Fuels Ltd £200 Miss D Masters £30
Mandy Hamer: Cullingworth Bowling Team Raffle £80 In memory of Edward James Flynn £250
John & June Fletcher £150 Axiom Engineering Associates Ltd £100
Mr & Mrs A G Blowers £30 Dr & Mrs Noble-Nesbitt £80
R J Ollis £30 Kelly & Gareth: Lang calendar sales £700
St Nicholas’ Church, Warwick £200 Kelly & Gareth: Lang hair growing challenge £2,510
Erin & Paul McKenna £200 Callum Parkinson £606
John & Marina Darby £130 The Gala Group, Northampton £2,660
Mr & Mrs Evans: Diamond Wedding Anniversary £275 A M & R J Harker £50
Unimed Scientific Ltd £500 T & A L Sharman £60
Leapfrogs Nursery £33 A M Roberts £50
Victoria Stephenson via Workplace Giving UK £25 (monthly) A K Davies £50
Jane & Mike Willis (Golden Wedding Anniversary) £555 Vicky Ellis: proceeds of the charity tub at Cohens Chemist £43.52
Dear NSPKU
On the 27th December 2015 my wife
Kathleen and I celebrated our Diamond
Wedding. We had a celebratory dinner but
told our guests that we did not want any
presents. Instead, if they wished to mark
the occasion, we asked that they made a
donation to fund research into PKU. Our
grandson, Rhys Taylor in Bath, has the
condition so it is something of which we are
acutely aware.
I enclose cheques to the value of £275
money, which was given directly to my wife
and me. I know that several people sent
some amounts directly to you so we hope Gareth Lang, above and inset, shows
off his long-awaited hair cut having
that the Society will have benefitted from a
raised over £2,500 for the NSPKU.
fairly substantial sum of money.
Right: Freddie Lang
Yours faithfully
H D G Evans, Cheshire Dear NSPKU Dear NSPKU
I just wanted to let you know that my I have pleasure in enclosing a cheque for
husband, Gareth, finished his six month £80 which the Cullingworth Bowling
Hi NSPKU hair growing challenge, raising a grand Team raised from a raffle at my late father’s
total of £2,510! Hopefully this has already (Cameron Hamer’s grandfather) Memorial
Through an old work colleague I was made
reached the NSPKU. It looked ridiculous Cup Match.
aware of your charity as he was doing a
by the end (although not as ridiculous as
fund raising walk… Thank you very much.
the picture on the JustGiving page which
www.justgiving.com/Colin-Sutcliffe was actually a wig!) because his bald Mandy Hamer, Bradford
spot remained a bald spot! He was very
Persuaded after a couple of beers I added
relieved to have it neatened up on the 29th
your charity to one of my more popular
December (the day after Freddie’s 2nd THANK YOU TO ALL FUNDRAISERS
ebay shop listings.
birthday).
I donate to several other charities this way
We also produced some novelty calendars We know that many people do wonderful
and although the amounts are small it will
using the Manchester City and Manchester fundraising events for the NSPKU via
raise £1.50 – £3 per day which adds up over
United mascots, and sold these to family and organisations such as JustGiving, Virgin
a year.
friends for £8 each. We sold 150 of them Money Giving and Charities Trust that don’t
All the best and raised £700. always get a mention. We would like to say
a BIG THANK YOU to all of you. And if you’d
Quentin Turner-Zealey Best wishes
like to tell us your story, please do write in!
www.tzlaces.com
Kelly Lang, Manchester
News & Views | Issue 151 | Summer 2016 5
NSPKU NEWS
A message from the Chair
I
t is with regret that we announce the After a busy conference in Cheltenham
resignation of our Society Dietitian, and an excellent exchange of views after
Paula Hallam, due to a serious illness the AGM, we have digested what was said
in her family. We would like to thank her and now we need to implement what our
for the tremendous work she has done in members have asked us.
her time as dietitian for the NSPKU; all the
We are pleased to announce that we are
families and individuals she has helped;
going to lobby Parliament on 28th June
all the publications she has worked on
2017 – International PKU Day. We – with
– and those she has proof-read; all the
your help – are going to raise awareness
conferences she has attended on behalf
of PKU amongst politicians, NHS
of the Society and much more. We wish
administrators and the general public.
her and her family all the very best for the
future. If we don’t shout about it nobody will
do it for us! Gathering further evidence
Ordinarily we would be looking for one
of the problems you face over the next
dietitian to replace her. However, our
twelve months will enable us to prepare
helpline has highlighted increasing issues
thoroughly.
within the PKU community. These appear
to be growing. Consequently we shall be
advertising for a paediatric dietitian and Eric Lange, Chair, NSPKU
an adult dietitian.
A message from
NSPKU Charity Tubs
Paula Hallam
Would you like to promote awareness of PKU and
Dear NSPKU members,
raise money for the NSPKU at the same time?
It is with great sadness that I need
to inform you that I will be stepping
down from my role as the NSPKU
Dietitian, due to an illness in my Our recently upgraded charity
family.
donation tubs are now available.
Thank you all for your support over
The new, eye-catching design
the years, since I took over the role
from Eleanor in 2013. will make PKU a real talking point
I still hope to be involved with the at your workplace, local shop or
NSPKU in a small way, perhaps
fundraising event.
writing for News & Views, but I will
not be able to manage the full role
anymore.
Available now. Please contact the
I am very sad to say goodbye to my
role as the NSPKU Dietitian, but I NSPKU by email: [email protected]
know that you will all understand
or telephone: [phone]
my position.
With very best wishes,
Paula x
6 News & Views | Issue 151 | Summer 2016
NSPKU NEWS
Looking for adult (18+) patients
with PKU to participate in an
Investing online survey
in the Future
My name is Denise Hofman.
I’m a Dutch PKU patient,
The Society continues to
currently doing a PhD at
benefit from your excellent
the University of Leeds. My
support which is now enabling
research focusses on the
us to invest in a stronger
dietary management and
Society to deliver increased
impact of PKU in adulthood.
benefits.
Denise Hofman speaking at the NSPKU Conference 2016 in Cheltenham
The Council of Management (COM),
after careful contemplation and
financial planning, has decided to We all know that adhering to the strict PKU diet and protein substitutes
can be hard. Various metabolic centres have reported decreased
increase capacity and resource in:
adherence to dietary recommendations when patients grow older.
Adherence to both the diet and protein substitutes are important for
• An additional Society Dietitian
both physical and mental health and low adherence has been shown
We will have two dietitians working
to lead to deficiencies in vitamins and minerals as well as anxiety,
together and supporting each other
depression and difficulties with cognitive functions like memory or
– one focussed on support for concentration.
children and the other focussed
With this survey, I hope to contribute to a better understanding of
on support for adults
(1) factors leading to low adherence to the PKU diet and protein
substitutes and (2) the cognitive functioning in daily life as well as quality
• A Conference Organiser
of life impacts experienced by adult PKU patients as a result of different
• Communications levels of adherence. My ultimate aim is to help improve the treatment
Website and social media and quality of life of adult PKU patients.
So far, 65 patients from all over the world have completed the survey.
This helps us build on the excellent
I’m looking for more patients both on and off diet (and anywhere in
work to-date, but develop new between!) to participate in my study.
opportunities and offer enhanced
A major thank you to everyone who has participated to date!
support in the future.
If you are an adult (18+) PKU patient and
Many of you have been telling us
about the opportunities to improve are interested in completing the survey,
our services and so we are delighted please contact me via [email protected]
and excited to announce our plans
and I will send you more information.
to increase our capacity in these
important areas.
Denise Hofman | Postgraduate Research Student | Human Appetite Research Unit
School of Psychology Faculty of Medicine and Health | University of Leeds
We will be advertising for these
roles on our website and elsewhere This survey has been
so we can secure the best possible granted ethical approval by
the School of Psychology
outcomes from this new additional
Ethical Review Committee,
investment.
University of Leeds,
United Kingdom.
www.nspku.org Reference number:
15-0398 (07/01/2016)
News & Views | Issue 151 | Summer 2016 7
8 News & Views | Issue 151 | Summer 2016
Fate Mexican Cheesy Bites
This recipe is so quick and easy to
do. It’s a basic recipe that you can
change to suit your taste, simply
by changing the seasonings. I use
fajita spice in the recipe which really
goes with the ‘cheese’ flavour,
but you can use whatever other
suitable spice or herb mixture that
you fancy. Even dried onion or garlic
granules are good. You could also
add different veggies to the mixture;
red pepper and spring onion is good,
as well as sweetcorn if you have the
exchanges!
This recipe makes about 12 bites.
Ingredients Method
Makes 12 bites Place the Fate Low Protein All Purpose Mix into Leave to cook for 3-4 minutes until the
a bowl. Stir in the yeast, salt and fajita spice. underside is browned. Turn over and cook for
½ tsp yeast
Place the browning and water into a jug, add a further 3-4 minutes till cooked through.
¼ tsp salt
the 2 tsp oil. Pour onto the dry mixture in the
2 tsp fajita spice, (more or less to taste) They are lovely served with a suitable fresh
bowl and stir well to make a smooth batter.
2 tsp oil, preferably olive salad and a squeeze of lime.
Stir in the cheese pieces.
100mls warm water
They are also good served with a suitable
Then, place a frying pan onto heat, lightly
A few drops browning tomato based sauce or dip.
grease it with a little spray oil. Place
5 slices/100g Violife protein free cheese,
dessertspoons of the cheesy mixture into Fate Mexican Cheesy Bites can be frozen,
any flavour, cut into small pieces.
the pan just a few centimetres apart. Use a however they are best eaten fresh
Spray oil for greasing
spoon dipped in water to level the top slightly.

Sprinkle with a little extra spice if liked.
See this recipe step by step on youtube
www.youtube.com/user/fatespecialfoods
Fate Special Foods cookery videos – now on YouTube
Step-by-step recipes, helping you make great low protein food.
Eileen Green © Fate Special Foods
News & Views | Issue 151 | Summer 2016 9
Congratulations
Anita MacDonald, OBE
Anita MacDonald (right), On 4th February this year Professor Anita MacDonald received her OBE
with colleague Anne Daly,
at Buckinham Palace from HRH The Prince of Wales, which was awarded for her services to
dietetics. The NSPKU is so proud of Anita and thankful for her years of
dedication to PKU.
A
nita wanted to be a PKU dietitian from the That little baby is now 29 years old! Anita is also his
time of being a student, particularly after she godmother and he regularly pops in to say hello to her
spent time doing some practical training at at her clinics.
Alder Hey Children’s Hospital. She was truly inspired
Anita announced her semi retirement this year but
by Christine Clothier, a great practical metabolic
she still intends to keep very active with PKU. She is
dietitian.
still caring for her patients with PKU and intends to
Anita saw her first patients with PKU in 1979. do more research. Officially she is working three days
Initially she only saw a small number of patients a week – but in theory only. She intends to keep the
with this condition and she had to wait patiently for a ‘BCH Metabolics’ Twitter account very active and
metabolic dietetic post to come up in Birmingham in watch out for her new PKU blog. The only reason for
1987. Within a week or two of starting her new role retirement is so she could have a little more sleep!
her first Birmingham baby with PKU was diagnosed.
10 News & Views | Issue 151 | Summer 2016