Issue 145 Winter 2013/14

The Society reports from Antwerp on the European

conference

Inside this issue… Your letters E.S.PKU conference review Dietitian’s report PKU and me Perspectives: From the outside in

In touch Helpline 030 3040 1090

PO Box 3143, Purley CR8 9DD [email protected] www.nspku.org @NSPKU facebook.com/groups/nspku/

The Council of Management Dave Stening (Chair and MAP Chair) Merrywood, Green Road Wivelsfield Green West Sussex RH17 7QD T 01444 471270 F 0845 004 8339 E [email protected]

Pete Bramley (Editor: News & Views and Conference Co-ordinator) 51B High Street Watton at Stone Hertfordshire G14 3SX T 01920 830635 E [email protected]

Julia Bailey (Vice Chair) T 07790 572525 E [email protected]

Caroline Bridges (NSPKU Administrator and Editor: News & Views) PO Box 3143 Purley CR8 9DD T 020 3397 7320 E [email protected]

Paula Hallam (Society Dietitian) 65 Shortlands Road Kingston Surrey KT2 6HF T 020 3397 7494 E [email protected] Eric Lange (Secretary) 27 Western Road, Sutton Surrey SM1 2TE E [email protected] Vicki King (Bookkeeper) Tarkwa, 65 Cromwell Road Ribbleton, Preston PR2 6YD E [email protected]

Lisa Lee (Treasurer) 2 Brightstone Close Banks Southport PR9 8FH T 01704 226931 E [email protected] John Skidmore (Support Group Co-ordinator) 4 Hall Croft, off High Street Normanton West Yorkshire WF6 2DN T 01924 890991 E [email protected]

Iain Williamson (Teenage/Adult Co-ordinator) E [email protected] @pkuteens Julie Wilson 59 Coopers Green, Bicester Oxfordshire OX26 4XJ T 07830 452322 E [email protected] Duncan Noble-Nesbitt 3 Devonshire Street, Ambergate Belper, Derbyshire DE56 2GJ T 01773 853360 E [email protected] Paul Cardy (Webmaster) E [email protected] Niki MacLeod E [email protected] Peter Davies E [email protected]

Please address all correspondence to the Editor, News & Views 51B High Street Watton at Stone Hertfordshire SG14 3SX Email: [email protected] Tel: 01920 830635

Local contacts BRISTOL & BATH Katherine Senior 20 Eastfield Avenue, Weston Bath BA1 4HQ T 01225 469878 E [email protected]

PORTSMOUTH AND DISTRICT Nigel Martin 79 Strode Road, Stamshaw Portsmouth, Hants PO2 8PX T 01705 644341

SUSSEX Sue Greenhalgh 48 Pinehurst, Burgess Hill West Sussex RH15 0DQ T 01444 242788 E [email protected]

EAST ANGLIA Jo Savage 2 Snowdonia Way Huntingdon PE29 6XP T 01480 413221

READING AND DISTRICT Nicola Cragg 13 Blomfield Dale Binfield Bracknell, Berkshire RG42 1FY T 01344 305312

WESSEX Nicky Oliver 8 Badgers Walk, Ferndown Dorset BH22 9QF M 07971 293697 E [email protected]

SHEFFIELD Gillian Race (Liaison Nursing Officer – Health Visiting) Neonatal Screening Laboratory The Childrens Hospital Sheffield S10 2TH T 0114 271 7415

YORKSHIRE John Skidmore (Support Group Co-ordinator) 4 Hall Croft, off High Street Normanton, West Yorkshire WF6 2DN T 01924 890991 E [email protected]

LONDON & HOME COUNTIES Ruth Pannell T 07825 575826 E [email protected] NEWCASTLE Ken Dodsworth 7 Lingey Lane, Wardley Gateshead, Tyne & Wear NE10 8BR. T 0191 469 3424

SOUTH WALES Ifona Rogers 9 Pant Glas, Pentyrch Cardiff CF4 8TB T 02920 890065

While every care is taken in the preparation of News & Views, the Society cannot be held responsible for any information given or views expressed. All articles in News & Views are copyright of the Society, and must not be used without the Council of Management’s authority.

The National Society for Phenylketonuria (United Kingdom) Limited PO Box 3143, Purley CR8 9DD Registered in England Company No. 1256124 Charity No. 273670 Helpline: 030 3040 1090 Email: [email protected] Web Site: www.nspku.org Registered address: Richard House Winckley Square, Preston Lancashire PR1 3HP

Printed by Action Press 01444 236204

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News & Views | Issue 145 | Winter 2013/14

Contents 10

Issue 145 | Winter 2013/14

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21

Your letters

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Letters and donations from NSPKU members.

41st Annual Conference and AGM

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Dietitian’s report

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Awesome Augergine – Ideas and new products, plus delicious low protein recipes for all things aubergine!

Programme of events

PKU and Me

E.S.PKU report part 1

PKU and parties… through the ages – a light-hearted view from a PKU parent.

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The NSPKU visits the E.S.PKU – Dietitian Cerys Gingell reports from last autumn’s conference held in Antwerp.

E.S.PKU report part 2

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Perspectives 16

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From the outside in – Caitlin Breheny looks at the subject of PKU from a different viewpoint.

Duncan Noble-Nesbitt represented the NSPKU at the Executive Board meeting in Antwerp. Here he reports on the business end of the E.S.PKU.

Editors’ Reports In what we know is a small community of those with PKU and their carers – be they parents, grandparents or otherwise – we don’t very often hear about what others ‘on the outside’ think. We all hold strong beliefs on the subject and these are as individual as those with PKU. In this issue we have an article from Caitlin Breheny giving her views on PKU from the outside looking in to our community and what a complete mystery it was (and still is) to them. I think it’s interesting to discover what others think even when it doesn’t directly affect them. If you have a view on PKU, why not share it and write an article for News & Views. We often include articles from people with PKU but it is always good to hear from parents, carers, partners or peers on how they cope with the diet. We always welcome your input.

It’s conference time again. I think I have already told you this but my daughter Hannah once said that the best thing about having PKU is attending an NSPKU conference! I love it too – it’s the only time in your life when you can be in a room full of people who all totally understand PKU and the low protein diet. Another thing I love about the conference weekend is the carefree packing – train tickets, toothbrush, clothes and coolers is all that’s required. Not a cool bag or tupperware box in sight! Of course there’s a lot more to the conference than just the lovely low protein food – there are the inspirational talks, the knowledge you gain and of course you! See pages 8 and 9 for a preview of what’s in store. Looking forward to seeing you there.

I hope you enjoy this issue. Caroline Bridges Pete Bramley

News & Views | Issue 145 | Winter 2013/14

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Your letters and donations

Salter Scales Star Letter Thanks to Salter Scales who have very kindly donated a set of scales as a prize for the best letter in this issue. We hope to have more promotions soon for our star letters, so put pen to paper or fingers to keyboard and get writing! We look forward to receiving your letters. News & Views, 51B High Street, Watton at Stone, Hertfordshire SG14 3SX Email: [email protected]

Sta

Letter r Dear NSPKU Please find enclosed a cheque for £500. My husband and I have been doing some fundraising over the last few years and have finally reached our target! Special thanks to ‘The Star Wars Forum’ for their generous donations. Helen, Andy, Ellis and Eve Plant

Dear NSPKU

Dear NSPKU

Please find enclosed some cheques for the society. These were received from my parents’ good friends as a Ruby Wedding present. My mother and father had asked for donations as my 18 month old nephew, Henry, has PKU. I am happy to say that due to the dedication of his family he is doing wonderfully well and is a fabulous wee chap!

Please accept the cheque as our Christmas donation to the NSPKU. We have decided to donate to charity instead of sending Christmas cards. Our grandson Hugo James was born in March and has PKU. The information we receive from the society is invaluable John & June Fletcher

Many thanks, Lynsey Steele Dear News and Views Once again we are fortunate not to need our Senior Citizens’ winter fuel allowance and Christmas bonus this year. We would like to donate an equivalent sum to the NSPKU, and enclose a cheque for £220. Our Grandson with PKU is 18 now. The support, encouragement and inspiration we’ve had from the NSPKU over the years has been (and continues to be) invaluable. Thank you!

Dear NSPKU The enclosed cheque for £151 was raised by the committee and members of “The Time of Your Life” club, Droylsden. We held a Christmas Fair and members donated things to sell. Yours truly Mrs Joan Turton Treasurer for “The Time of Your Life”

With best wishes, Bevan and Barbara Broadbent

Dear NSPKU We have two delightful grandchildren, Sadie who is two and Stanley who is three, both have PKU. Their grandpa, Andy, had his 60th birthday in October and we had a lovely party!! He decided that instead of presents he would ask for donations to raise funds for the NSPKU. Please accept this donation of £125 for all the charity does for this special cause. Yours sincerely Patty & Andy Backler (Stanley & Sadie’s grandparents)

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News & Views | Issue 145 | Winter 2013/14

Dear NSPKU Please accept the enclosed cheques amounting to £230 raised at my husband’s recent 70th birthday party. Our grandson Christopher, aged 10, has PKU. Kind regards Mr and Mrs Evans

Dear News and Views Please accept a cheque for £164. We raised this money by selling a word quiz. Yours sincerely Donna Bruce

Dear NSPKU I enclose a cheque for £110 as a donation to the charity. My 6 month old granddaughter has PKU, hence my interest. I am an artist and last year I held a Christmas open day and I’m donating £65 from the sale of my paintings. Six friends have given £5 each instead of sending each other Christmas cards. The balance has come from other donations at my open day. Yours faithfully Mrs Pam Vardy PS A number of friends are making donations direct! www.pamvardyart.co.uk

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 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. Email: [email protected] News & Views, 51B High Street, Watton at Stone, Hertfordshire SG14 3SX

Donations over £30 received

Thank you to everyone who has donated

Mr and Mrs Evans

£230.00

John Whittle estate

Mrs Lynn Slack

£350.00

Mrs Pam Vardy

£110.00

Mrs Anne Pollock re Lynsey Steele

£250.00

The Chartered Institute of Waste Management

£3,801.60

£5,000.00

The Plant Family

£500.00

Mrs Rosemary Brown re Lynsey Steele

Patrick Minihane

£735.00

Mr and Mrs Steele re Lynsey Steele

£200.00

Patty and Andy Backler

£125.00

Allan and Donna Bruce

£164.00

Barbara Broadbent

£220.00

Mr Philip Taylor, Woodley Methodist Church Anne Marie Hemmer

£44.80 £200.00

Lady Teresa Carter

£40.00

The Time of Your Life Club, Droylsden John and June Fletcher

£151.00 £50.00

Lynn and Lee Slack

Dear NSPKU

£50.00

Greystones Ladies Group

£55.00

Hermes Parcelnet Ltd

£200.00

Lyn and Paul Houghton

£120.00

Joyce Jamieson

£80.00

GIFT AID: a message from the NSPKU Council of Management Last summer through the hard work and persistance of Rhodri and Katie Davies (below), the NSPKU became the chosen charity at the National Surveyor Rugby Sevens tournament (see

Please find enclosed a donation of £350, proceeds from my 60th birthday where I asked for donations instead of gifts. It is on behalf of our 4 year old grandson Ashton Lee Slack who has PKU.

N&V #143). This event raised £21,300 and with Gift Aid this total increased by £6,650 to a staggering £27,950! A big thank you to all involved, but especially to Rhodri and Katie. The NSPKU really appreciates every single penny raised by you for the Society. And this is a great example of how

Gift Aid really does make a big difference. If you are a UK taxpayer your amount can easily be increased by completing a Gift Aid form and sending it in with your donation cheque. You can download a form from our website www.nspku.org Thank you for your continued support.

Yours Lynn Slack

Dear News & Views We are a small ladies group of about 20 people in Sheffield who meet monthly. At our last meeting I gave a small talk about what I knew about PKU, as my grandson has the condition and they kindly had a collection for a donation of £55. Yours faithfully Mrs Kathleen Addis Secretary, Greystones Ladies Group

Dear News & Views Our grandson, George age 5 has PKU. At my Christmas fitness class the teacher Margaret McAllister donated her takings and raffle money to the NSPKU (£120). This is the second year she volunteered this – very generous of her. Kind regards Lyn & Paul Houghton

Dear NSPKU

Dear News & Views

Over a number of years my grandad and his friends collected and recycled aluminium drinks cans and foil and the monies raised was distributed to local and well known charities or my Church, Woodley Methodist Church. In 1996 the Church started a rebuilding scheme, we needed to raise huge sums of money. Upon completion of the work and then the passing of my Grandad I had to make the decision to stop collecting or to continue in his memory. My decision was to continue and my colleagues at work were and are still very supportive of this idea. One member of staff, who has a daughter with PKU, agreed to save cans for me and so in return I have agreed for a third time to make a donation to the work you undertake to help those living with the condition. Please therefore find a cheque for £44.80 that I hope you will be able to put to good use.

In the lead up to Christmas, Hermes Parcelnet Ltd ran a corporate social responsibility scheme for employees called ‘25 Days of Giving’. The scheme involves Hermes making donations on every day of advent to charities nominated by members of staff.

Yours sincerely Mr Philip G Taylor Woodley Methodist Church

I am delighted to inform you that your charity has been selected by one of our employees and we have enclosed a cheque for £200. We hope that this donation supports you in the year ahead. Yours faithfully The Human Resources Team Hermes Parcelnet Ltd

The treatment of phenylketonuria varies for each individual patient. No patient should alter their own treatment as a result of reading how another patient manages their diet without first consulting their doctor or dietitian.

News & Views | Issue 145 | Winter 2013/14

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NEW. EASY. TASTY. IT’S WHAT YOU’VE BEEN WAITING FOR. 6

News & Views | Issue 145 | Winter 2013/14

Mevalia Low Protein is a new range of foods created by Dr. Schär for people following a low protein diet. This tasty range is designed to replace traditional foods made from cereals and is suitable for those with inherited metabolic disorders including phenylketonuria, tyrosinaemia, maple syrup urine disease and homocystinuria. While developing Mevalia, we listened to people following low protein diets and created just what they wanted; great tasting, delicious looking and convenient foods. Our foods contain that all important ingredient that we believe has been missing from low protein products available in the UK until now…taste!

• • • •

LOW PROTEIN - LESS THAN 1G PER 100G LOW PHENYLALANINE CONTENT HIGH IN FIBRE CONVENIENT - NO NEED TO REFRESH!

REGISTER TODAY FOR YOUR FREE MEVALIA WELCOME PACK! Contact our Careline on 0800 988 2488 or email us at [email protected]

The proof is in our breads, pizza bases, biscuits and pasta.

0800 988 2488 www.mevalia.com [email protected] facebook.com/mevaliauk

Taste, our secret ingredient. News & Views | Issue 145 | Winter 2013/14

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See you in Skipton… 41st Annual Conference and AGM 2014

The Rendezvous Hotel, Skipton 14th – 16th March 2014 The Conference Programme of Events* is shown on the right. As you’ll see it’s gearing up to be a great weekend packed full of fascinating talks, presentations, cookery demonstations, great food and evening entertainment. We’re looking forward to seeing you there…

AGM

DAY VISITORS: If you haven’t already booked for the weekend but would like to attend for the day on Saturday, Sunday or both, please contact the Conference Organiser for availability and rates: Tel 01920 830635 or email [email protected]

NOTICE OF ANNUAL GENERAL MEETING 2014 The Annual General Meeting of the National Society for Phenylketonuria (U.K.) Ltd will be held on Saturday 15th March at 5.45pm in the Wharfedale Suite, The Rendezvous Hotel, Skipton, North Yorkshire, England, BD23 2TA. All members are welcome to attend.

National Society for Phenylketonuria (United Kingdom) Limited

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News & Views | Issue 145 | Winter 2013/14

Company No:1256124

Charity No:273670

NSPKU 41ST ANNUAL CONFERENCE PROGRAMME OF EVENTS* DAY 1 – FRIDAY 14th MARCH TIMINGS

PROGRAMME CONTENT

LOCATION

14:00 – 18:00

REGISTRATION NSPKU registration desk opens

Main Reception

19:00 – 19:30

WELCOME DRINKS RECEPTION The Chairman of the Society welcomes delegates to the Conference

Wharfedale Suite

19:30 – 21:00

DINNER

Malham Suite

21:00 – 24:00

EVENING ENTERTAINMENT Disco, Presentations and Speeches

Malham Suite

DAY 2 – SATURDAY 15th MARCH 07:00 – 08:30

BREAKFAST

Malham Suite

08:30 – 12:45

Professional Nursery/Playgroup: (Infants & Children under 8 years of age). Children’s Outing (8 and Over) – Indoor Climbing Experience. Teenagers/Young Adults – Archery Experience.

Old Nursery

09:00 – 10:30

RESEARCH UPDATE Dr Anita MacDonald, Consultant Metabolic Dietitian, Birmingham Children’s Hospital Dietetic research update, including Kuvan. WORKSHOP/PANEL DISCUSSION Cerys Gingell, Specialist Metabolic Dietitian – Facilitating a group of PKU mums about their experiences.

Wharfedale Suite

10:30 – 11:00

Morning coffee/tea break and biscuits. PKU Food manufacturer exhibition stands will be open.

Winterburn Suite

11:00 – 12:30

TODDLER ISSUES AND POSSIBLE SOLUTIONS Rachel Skeath, Specialist Paediatric Dietitian – Feeding the PKU toddler. Dr Stewart Rust, Consultant Clinical Psychologist – TBA.

Wharfedale Suite

13:00 – 14:00

LUNCH

Malham Suite

14:15 – 16:00

GREAT BRITISH PKU BAKE OFF Cookery Demonstration – Eileen Green, Fate Foods.

Wharfedale Suite

16:00 – 16:15

Afternoon coffee/tea break and biscuits. PKU Food manufacturer exhibition stands will be open.

WinterburnSuite

16:15 – 17:30

COOKERY DEMONSTRATION – Nutricia

Wharfedale Suite

17:00 – 18:30

CHILDREN’S PARTY (note: a parent or carer must remain with their children). Infants/Children/PKU/Non-PKU welcome: Surprise Children’s Entertainer. The Party will be catered for (PKU and Non-PKU party food).

The Old Nursery

17:30 – 19:30

YOUNG ADULTS MEETING – Meet the manufacturers and new developments.

Webber Gallery

17:45 – 19:00

NSPKU ANNUAL GENERAL MEETING – Everybody welcome.

Wharfedale Suite

19:30 – 21:00

DINNER

Malham Suite

21:00 – 24:00

EVENING ENTERTAINMENT MAMA MIA – ABBA Tribute band. Presentations and Speeches.

Malham Suite

DAY 3 – SUNDAY 16th MARCH 07:00 – 08:30

BREAKFAST

Malham Suite

08:30 – 12:30

Professional Nursery/Playgroup: (Infants and children under 8 years of age). Children’s Outing (8 and over) – Bowling Tournament, Skipton.

Old Nursery

09:00 – 11:00

Jen Simpson – Mum of daughter with PKU and teacher/trainer – What the PKU is that? WORKSHOP FOR ADULTS WITH PKU Dr Elaine Murphy, Consultant in Adult IMD – Facilitating a panel discussion of adults on/off diet YOUNG PEOPLE’S EXPERIENCES Callum Graeme – My Experiences with PKU. Nicola Bramley – PKU and at University

Wharfedale Suite

11:00 – 11:30

Morning coffee/tea break and biscuits. PKU Food manufacturer exhibition stands will be open.

Winterburn Suite

11:30 – 12:30

PRESENTATION Dr Gisela Wilcox, Consultant in Adult Metabolic Medicine, Salford Royal Hospital – PKU and bone status

Wharfedale Suite

13:00 – 13:45

LUNCH

Malham Suite

CONFERENCE ENDS * This Programme of Events is correct at the time of going to press and is subject to change.

News & Views | Issue 145 | Winter 2013/14

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E.S.PKU Report

The NSPKU visits the Dietitian Cerys Gingell from Nottingham University Hospitals, parent Laura Sheridan and Heather Smith travelled to Antwerp, Belgium last October to attend the annual E.S.PKU conference. With a packed programme of specialist speakers, here is what they found out for us…

T

he 27th annual conference of the E.S.PKU was back in Belgium where the first conference was held in 1987. It was an opportunity for everyone with PKU, their parents, partners, delegates, scientists and professionals to meet others from all over the world, not just Europe, to learn about PKU. It was also an opportunity to socialise and meet with others who were involved with PKU and for the children to eat and have fun with other children with PKU. The children went on outings to the Zoo and ‘Aquatopia’ whilst also partying and enjoying lots of activities in the hotel. Our journey to Antwerp began at 8am on Thursday 31st October at Nottingham train station. With me were Laura Sheridan, a mum of two children with PKU and Heather Smith, one of our teenagers with PKU. We arrived in Antwerp six hours later having had our first experience on the Eurostar. Antwerp was indeed a beautiful, cultural city with amazing historical architecture, classic painters such as Rubens, excellent shopping facilities, delicious food, the fourth largest harbour in the world and a diamond district. On the Friday morning Eric Lange, president of the E.S.PKU, welcomed a total of 82 patients with PKU and 360 participants from 25 countries around the world. We were also welcomed by officials, including Prince Laurent of Belgium. A recommendation was made four years ago by ministers that there should be guidelines for rare diseases such as PKU. The aim of the guidelines is to have a consensus that treatment should not be compromised by the country in which you were born. The E.S.PKU is working collaboratively on European guidelines for PKU. This was a major topic of the conference with the aim that they will be completed in early 2015.

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Cerys Gingell, Laura Sheridan and Heather Smith

E.S.PKU Our first presentation was on Maternal PKU (MPKU) by Professor Trefz from Germany. He highlighted a concern from Berlin that 67% of MPKU’s are late treated and pregnant without good control. He stressed the importance of good control in MPKU and careful monitoring to ensure healthy babies. MPKU will be included in the E.S.PKU guidelines and will include some of these preliminary recommendations: • Women with hyperphe ( Levels less than 360 µmol/l on normal diet) do not need to be treated • Recommended target range 120 µmol/l – 360 µmol/l • Levels should not be below 120 µmol/l to avoid low birth weight babies. Dr Schwartz gave an excellent summary of the challenges of treating PKU in Brazil. Brazil is the largest country in South America and holds a fifth of the world’s population. Screening began in 2001 and recorded a higher incidence of PKU compared with the UK – 1 in 7000. Adherence to treatment is only 35-70%. Poor compliance may be related to a lack of choice in protein substitutes, lack of health professionals, possible travel of 700km to clinic and poor access to more sophisticated equipment. Multi-disciplinary teams are working hard to improve compliance by organising cookery sessions, support groups, teenage groups, psychological support groups and home visits. Professor Van Spronsen gave us an update on the European guidelines. The work began in October 2012 with five working groups of health experts to produce guidelines on neurocognitive function, psychosocial, nutrition, adult and maternal PKU and medication. Professor Walter and Professor Anita MacDonald are representing the UK on the nutrition group. The remainder of the day was divided. I stayed to listen to the scientists and professional programme whilst Heather and Laura followed the parents and families programme. Dr Pascucci, a researcher from the Department of Psychology at the University of Rome, discussed

the usefulness of mice in understanding more about PKU with the hope that they may be able to help with treatments in the future. Professor Van der Zee described how mice could be used to understand brain dysfunction in more detail. Biochemically PKU mice show a strong increase in brain phenylalanine (phe) levels with high blood phe levels resembling the human situation. Dr Maillott from the University of Francois Rabelais in France, discussed PKU in adults who had been treated from birth. He believed that adult outcome is dependent on their care during childhood. It is documented that for every increase of 100 umol /l phe IQ decreases by 1.3 to 1.4. There are complications related to poor control in adulthood including depression, phobias, anxiety, poor memory and poor attention. There is evidence from a German paper to prove adults with PKU should be seen regularly in a specialist adult centre and the audience agreed with this. All adults with PKU, on or off diet, should have access to a specialist team of doctors, dietitians, specialist nurses, social workers and psychologists. The E.S.PKU guidelines will provide further information on how often phe levels should be checked and the recommended levels. He concluded: “There is now almost universal consensus amongst clinicians that diet for life is the best treatment. However, in many countries many have stopped a low phe diet. The metabolic team for adults has to deal with patients choice to a specific diet or not and to organise day to day care accordingly.” Dr Shakked from Israel presented a case study about a nine month old baby with PKU who had been on a protein substitute with no natural protein. As a result her blood phenalanine was very low and she suffered from a very bad skin rash, prolonged diarrhoea, hair loss and developmental delay. Fortunately, the symptoms were reversed when she started on some natural protein. Dr Feldman spoke about the shortfall in studies on adolescents and adults with PKU. They studied 57 patients with early treated classical PKU aged between 19 and 41. They were compared with controls of non PKU

t

Day one

News & Views | Issue 145 | Winter 2013/14 |

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E.S.PKU Report

Above: President of the E.S.PKU Eric Lange speaking at the conference Top right: Laura Sheridan with her children Faith and Blake

patients and were assessed for their intelligence, attention, information processing abilities. After five years they were reassessed and it was found that teenagers with high phe levels have lower IQ in adult years. He concluded: “Cognitive performance in adult patients with early treated PKU does not seem to be subject to deterioration observable in a five year interval. Neuropsychological assessment in adults with PKU revealed neurocognitive impairment, particularly in older adult patients. This seems to refer to an early relaxation of the diet that was recommended when the older patients were adolescents. Results indicate a benefit of dietary control in adolescence in PKU.” Professor Anita MacDonald discussed dietary adherence in PKU. Approximately 1/3 of patients have mean blood phe concentration outside the target treatment range, with control deteriorating with increased age. Overall outcome in maintaining good phe levels depends on parent/patient ability, their discipline, motivation, social and financial factors, organisational skills and overall coping ability to comply with dietary regimens. It is reported that the time burden associated with managing PKU is 1 hour 24 mins/day for caregivers and 30mins/day for adult patients. Many countries have difficulties in providing protein substitutes and the uptake is also poor. Anita reported on an American study where only 80 % of the 100% prescribed was collected and at the end of the study only 40% was collected. She concluded that health professionals need to understand the issues that influence adherence and to help the challenges they face. Mrs Van Vilest discussed single amino acid supplement in PKU. We know that phe is one of the building blocks of protein called amino acids. High blood phe levels will cause high concentration of phe in the brain and lower concentrations of other amino acids in the brain.

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She gave an interesting account of how certain amino acids can block other amino acids getting into the brain. Further research is needed as the studies are currently in animals only. Merve Vndriessche discussed PKU and school in Belgium, which starts when children are 2.5 years old. She discussed her aims as a dietitian in the metabolic team and her role in the school to arrange clear guidelines working as part of a team with the school and parents. A young Belgian man with PKU gave an inspiring talk. He talked about how he has not seen PKU as a problem. He feels he is very well and healthy and is never poorly because he knows he follows a good diet. He has been to university to study law. His advice to parents was to be honest with children, give them plenty opportunities, don’t overprotect them and empower your children to deal with their PKU. PKU shouldn’t restrict them. Maarten Criem is a young lady with PKU. She felt that books on PKU were limited. She met a lady called Mickle Vanpool at a painting class. She is a storywriter and between them they have written a book for teenagers with PKU to be published in 2014. I did ask if it could be translated to English. Dr Debray explained that Belgium has been running camps in Switzerland for 25 to 40 children with PKU between the age of 6 to 18 since 1986. The objective is to meet other children and young adults with PKU and to learn in a fun way and try new foods. An excellent idea they used was to give the children protein exchange money at the start of the day. They had to spend their money on protein exchanges by the end of the day.

Day two Saturday morning also started early. Dr Bosch from Amsterdam talked about the value of using Quality of Life (QOL) questionnaires in PKU. The E.S.PKU group agreed that a QOL questionnaire should be part of the PKU guidelines.

The NSPKU visits the E.S.PKU Professor Van Spronsen began his presentation by saying that the earlier the diagnosis and treatment of PKU, the higher the IQ. In adulthood stopping the diet can result in abnormal complex executive functions, behavioural issues, psychiatric problems with depression, anxiety, cerebral problems, eczema and obesity. As mentioned previously, the E.S.PKU guidelines will recommend diet for life. More information about PKU in adulthood including what levels we should be achieving is needed. Professor Gizewska’s presentation about deficiencies in macro and micronutrients started with the words ‘We are what we eat’. Our bodies need a combination of macro nutrients (fat, carbohydrate and protein) in addition to micronutrients (vitamins and minerals). We need a balance of all vitamins and minerals to avoid deficiency or toxicity. Deficiencies have been shown to cause hair loss and eczema. Mrs Van Wegber, a dietitian from Belgium, asked the patients and parents in the audience to participate in a multiple choice questionnaire. It was entertaining to see lots of different coloured cards held up (different colour corresponding to different answers). The results were counted. It gave those in the audience an insight into people’s thoughts to questions like; Do you think everyone should be followed up, even off diet?

Patient and families programme Professor Meirleir gave us an overview of advances in genetics and the importance of genetic research for genetic counselling and developing new treatments. Professor Eyskens told us that all EU countries screen for PKU apart from Finland and Malta. In many countries it is not compulsory. We all know, however, the importance of screening to diagnose PKU and prevent learning disabilities in our children. Dr Verellen explained that genetic counselling is where parents/patients are informed about the probability of inheriting a disease and the consequences of that disease. Parents have a choice if they want genetic counselling. Prenatal diagnosis can also be carried out by taking a sample during the pregnancy. This does carry a 1% chance of miscarriage and later in pregnancy amniocentesis carries a 1 in 200 chance of miscarriage.

The important thing to remember is that there are some ethical issues in that PKU is treatable and not life threatening despite the chronic treatment. We thoroughly enjoyed the next presentation by Mevr Diels, a dietitian, who outlined recommendations in maternal PKU. These included two tests per week with an aim of 120 -300 µmol/l for up to three months preconception and throughout pregnancy. During this time the challenges of sickness and ensuring sufficient energy intake was discussed. She encouraged healthy pregnancy, body weight and exercise. Unfortunately two out of three pregnancies are not planned and can have devastating effects on the baby. Two of her maternal PKU patients gave a very positive summary of their experiences and we met their healthy babies and children. Dr Delact updated us on the use of Kuvan. She said that trials have shown that 39.4% were responders. These had mild PKU. Another study showed there was no response when using Kuvan in classical PKU. Therefore it works better in mild patients. The audience highlighted that it is not widely available to patients and that although patients get a initial improvement, with some patients it might not be sustainable. Some audience members questioned if it should therefore be used. Merv Van Impe and K Bechx talked about the psychological aspects and encouraged us to have a holistic view of PKU to include family life, school and social life. She said that 80% of adolescents fail to comply with the diet. How the family copes will influence the PKU control. Positive parenting should empower the adolescent with good routine, support, clarity of diet, self-worth and positivity in how they are complying. It should also be empathetic and avoid fighting against resistance. Find a way to communicate with adolescents that works for you.

Summary What an amazing time we had. We received lots of new information, an update on the E.S.PKU guidelines, sampled some new low protein products and protein substitutes and made lots of new acquaintances from all over the world. We urge you to attend the next conference which is provisionally booked for October in Zagrab, Croatia. International PKU day is on 28th June, the birth date of Professor Robert Guthrie. Robert Guthrie M.D., Ph.D., (1916 – 1995) was an American microbiologist, best known for developing the bacterial inhibition assay used to screen infants for phenylketonuria at birth. There is a very interesting medical biography Robert Guthrie – The PKU Story written by Jean Holt Koch published by Hope Publishing House in the USA. Available via Amazon. We wanted to finish our article using the Governor of Antwerp’s opening remark: “The First Wealth is Health” n

Professor Anita MacDonald with the Nutricia team

News & Views | Issue 145 | Winter 2013/14 |

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E.S.PKU Report

Getting down to business NSPKU Council of Management member Duncan Noble-Nesbitt represented the NSPKU at the Executive Board meeting in Antwerp. Here he reports on the outcomes.

O

ne of the first things that I have been involved in since being back on the NSPKU Council of Management (COM) has been to attend the 27th Annual E.S.PKU conference in Antwerp, Belgium. The delegates and members of the Executive Board met at a general meeting on the 1st November 2013. The meeting is the business end of the E.S.PKU and was attended by up to two delegates from each legal member, which is similar to the setup of the last E.S.PKU meeting that I attended over a decade ago. The meeting had a full agenda for the timetabled 10 – 5.30 meeting, run concurrently with the family programme and the scientists and professional programmes. The meeting firstly summed up the successful 2012 E.S.PKU conference in Liverpool and the advances made in the E.S.PKU guidelines on PKU management, and the subsequent spring delegates meeting, which discussed various topics and included the identification of projects. Tobias Hagedon, the secretary, covered the status of the E.S.PKU consensus paper on PKU care from the patient’s perspective, which has since been published in the peer reviewed publication in the Orphanet Journal of Rare Diseases, entitled Requirements for a Minimum Standard of Care for Phenylketonuria, The patient’s

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perspective. He also summed up the work to date on the European Guidelines on the management of PKU which were also updated during the course of the scientific and professionals programme and summed up at the conference end with the agreements made during the weekend of a common optimal level for treatment. This kicked off the meeting with a noticeable increase in the level of input from the E.S.PKU from previous meetings that I have attended for NSPKU. Within the annual report other noted achievements in the year, under the presidency of Eric Lange, includes meetings with policy makers including the application of membership in Union Committee of Experts on Rare Diseases (EUCERD) representing patients organisations in the field of rare diseases. Italy, which is taking over the presidency of the European Council in 2014, is encouraging organisations such as E.S.PKU to put rare diseases on the agenda, as a follow on to the implantation report on rare diseases in 2013. Various meetings were reported on within member states raising the profile and addressing PKU issues at the European level within European Health politics. The meeting continued with a talk and session from Jacqueline Bowman-Busato from Eppossi who outlined the parameters for advocacy as a ten stage approach

Executive Board meeting

Left: The committee meeting in full swing. Right: Duncan signing a banner on behalf of the NSPKU promoting a specially commissioned song which aims to help raise the profile of the PKU story. Below: Delegates from the various member states holding the banner.

including structured interviews with stakeholders in developing neonatal screening programmes for PKU at European level, providing an insight into the theoretical aspects of putting these together with the various considerations required. One of the aims of the meeting was to consider the direction of E.S.PKU input, with the Guidelines underway, the attention was focussed to what next. Previously it has been agreed to collect data aimed at helping define strategies on how to implement the results of these guidelines, with two areas of data to collect. Firstly on new born screening provision throughout Europe and secondly the level of social support. A brainstorming session was set up to define each area and to identify key areas on which questions are to be based. A working party was set for each to consider the issues, with further work and consolidation at the next meeting to define the data collection parameters. The afternoon session followed included the presentations and application review of Les Feux Follets organisation from France, Fenilcentonurica and OTM Association of Galicia from Spain resulting in their successful addition as legal members to E.S.PKU at the AGM later. There were further presentations outlining the successful E.S.PKU adult meeting in 2013 and the Rare Disease Day 2014 from Paul Berkel due in March.

The idea of an international PKU day was discussed by Eric Lange and it was agreed that a suitable day would be 28th June, that being the birth date of Horst Bickel and Robert Guthrie – both pioneers of PKU treatment and management. Linked to this, consideration for a world PKU conference in 2016 was discussed and this will be discussed further at subsequent meetings. The summing up included expectations from E.S.PKU in 2014 with identification of the working groups that will feedback in the spring, 2014. The evening AGM was formal and efficient – the minutes to this will follow in due course – and ended a long but productive delegates’ meeting. I left this feeling enthused and genuinely impressed with the activity of the E.S.PKU and how the society has improved since the last meetings that I attended several years ago. Another thing that I was struck with was that half those on committee have PKU. I look forward to the next meetings and to being able to feedback on the good work being done n

News & Views | Issue 145 | Winter 2013/14 |

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News & Views | Issue 145 | Winter 2013/14

Fate Banana and Honey muffins

Hello. This recipe is great for using those over-ripe bananas left in the fruit bowl. They are really delicious anytime of day. They freeze well. We found it better to chop the banana into small pieces instead of mashing as it gives the muffin a better texture. Be aware – honey is unsuitable for children under 12 months. Ask your dietitian if you’re unsure about anything.

Ingredients

Method

200g ripe Bananas, cut into small pieces

Pre-heat oven Gas 6, 200°C, 400°F.

300g Fate Low Protein All-Purpose Mix 120g soft light brown sugar 1tsp baking powder ½ tsp cinnamon ¼ tsp nutmeg 120g soft margarine 3tsp runny honey, I used Gale’s brand 200mls unsweetened pure apple juice Little Demerara sugar to sprinkle

Place the Fate All-Purpose mix into a mixing bowl. Add the light brown sugar, baking powder, cinnamon and nutmeg. Stir. Add the margarine and honey. Pour in the apple juice and using a wire whisk, mix quickly for about a minute until well blended and smooth. Stir in the chopped bananas. Divide the mixture between 12 large muffin paper cases set inside a muffin tin. Sprinkle some Demerara sugar on to each muffin before baking in a pre-heated oven for 25-30 mins until risen and golden brown. Place on a cooling rack to cool.

Eileen Green © Fate Special Foods

News & Views | Issue 145 | Winter 2013/14

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PKU – NOT

PKU – NotEdale Activity Weekend A light-hearted of a parent 4th – 6thview July, 2014 bringing upBookings a PKU child are now being taken for this great, fun-

Travelling with PKU filled weekend in Derbyshire. You can learn lots of

new skills such as abseiling, canoeing, raft building,

That’s another cramming exercise too. If you are It doesn’t matter how far you go with a PKU child and weighing Andyou you get to stuff to goingexchanges! by car with a baby have enough or as someone with PKU. It couldcaving be to TESCO. It of new takefriends! anyhow, without trying to get a Pharmacy in could be to the Far East. Doesn’tmake matter. loads Only one too – I felt like one of those guards on the Japanese thing is for certain PKU comes nailed on. The gets weekend is open tounderground boys and girls with PKU aged 8 tointo 11 ayears stuffing commuters train. You can be 1 or 31 but as the world smaller (still in Junior school). through the benefit of air travel (notwithstanding VENUEdollar Edale that YHA Activity Centre, Hope Valley,being Derbyshire Foreign travel despite loadedS33 up 7ZH is no volcanic ash) you can bet your bottom www.but yha.org.ukproblem though. You are on holiday and there’s a you may be in Row 38 Seat B on See the at aircraft little bit of you that wants a break from the diet in Row 38 Seat C is your mate PKU a shed DATEand Friday 4th July 1.30pm to Sunday 6th July 1pm – we all do. And that’s why God invented CHIPS. load of Pasta/Analogue/Tablets etc etc. And how COST £85Charge (This is actually half tell the your true cost as thetho’ NSPKU Don’t Dietitian – it’ssubsidises naughty. about short-haul? Ryan-Air case in point. the we’ll weekend for bags! That’s us out then because haveby to 50%)* But don’t let PKUtime holdwill youbeback from get the Ferry to Ireland if it doesn’t sink under the to attend *Children wishing for a second subject totravelling anywhere. We’ve out at to full the price Far East weight of dietary paraphernalia. availability and, unfortunately, have to bebeen charged to and enable us to support first-timers. came back and she got on fine – plenty of veg and a much healthier PKU diet than the UK eating I always remember when my little one was really All the low protein meals and instruction by qualified YHA staff is establishments provide – did godietitians to McDonalds little. When she was swamped byincluded a car seatand andthe children are helped bycan several metabolic a couple of times I admit. However as they cook having to stop on a long road tripexperienced every so often in PKU treatment. food from fresh in the Far East you can have what to feed her. I remember having to mix Analog at you want, just ask – and they smile at you too – all the side of the road off the M6 and marvelling at theweekend time, no I mean all the time. the way that the powder and the Don’t water could stay delay, this fun is popular. apart for so long whilst my child shattered the car Book now by contacting Paula Hallam, Society Dietitian, It’s not as easy travelling with PKU, but it’s not windows with shrill screaming in demand for her by email: [email protected] phone: hard enough toor stop you. 020 3397 7494 fill – deep joy. Christopher Columbus - Hertfordshire.

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News & Views | Issue 145 | Winter 2013/14 News & Views

Issue 139

Summer 2012

Dietitian’s report: Paula Hallam

Awesome Aubergines Hello everyone! With all this awful, wet weather we are having, I am certainly looking forward to spring and the NSPKU Conference! We are all busy with final preparations for the conference and we have a fantastic programme that we hope everyone will enjoy. I look forward to seeing many of you there.

This issue I have chosen aubergine as the feature vegetable… it is such a versatile vegetable as you will see from the recipes I have included for your to try. To quote the lovely Eileen Green: “I think aubergines are one of those vegetables that are really versatile; they can be used in so many ways… they have a delicious flavour and texture on their own but will take on many other flavours too”. Also in this issue we also have a clever idea of using cauliflower as a rice-substitute from Heather PaineCardy, some savoury snack ideas and some new vegan products that are low enough in protein to include in the PKU diet.

News & Views | Issue 145 | Winter 2013/14

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Dietitian’s report: Paula Hallam

Aubergine Melts

Recipes

This recipe has been adapted from BBC Good Food website – www.bbcgoodfood.com. They have lots of great recipes and this one is very simple and easy to make. Method 1. Pre-heat the oven to 200°C/fan 180°C/ gas 6. 2. Drizzle the oil over the aubergine and bake in the oven for 30-40mins until softened.

Ingredients 3 Tablespoons olive oil 2 aubergines, halved lengthways 4 tomatoes, sliced Cheezly mozzarella-style cheese (20g = 1 exchange) or Mozzarisella* Handful fresh basil leaves * Mozzarisella = 0.7g protein per 100g – You won’t need to count it if only use 20g

3. Meanwhile, slice the tomatoes and Cheezly mozzarella (20g = 1 exchange) or Mozzarisella* 4. Arrange the tomatoes and Cheezly/ Mozzarisella on top of the aubergines. 5. Return to the oven for another 5-10 mins or until the cheese has melted. 6. Remove from the oven and scatter over some basil leaves. 7. Serve with low protein couscous and a green salad.

Aubergine and Coriander Dip This recipe has been adapted from the www.bbcgoodfood.com website. It can be used as a dip with some low protein crisps or free vegetables such as carrots, celery, peppers or cucumber. Or it can be used as a paté spread on some homemade low protein bread. Ingredients

Method

4 tablespoons olive oil

1. Pre-heat the oven to 200°C/fan 180°C/ gas 6.

4 large aubergines 4 tablespoons Coyo natural coconut yoghurt (45g = 1 exchange) Large bunch coriander, leaves only, finely chopped 1-2 garlic cloves, crushed Juice of 1 lemon or lime juice Crushed red chillies (optional) Salt and pepper to season

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2. Drizzle some olive oil over the aubergine and roast in the oven for 30-40mins until very soft. 3. Scoop out the insides of the aubergine and transfer to a bowl, then mash with a fork or blitz with a hand blender, but don’t make it completely smooth. 4. Stir in the natural coconut yogurt, the rest of the olive oil and a pinch of salt, then add coriander, garlic and lemon or lime juice. Taste and add more salt or lemon juice if necessary and the chilli if would like a bit of “heat”!

Recipes

Aubergine Rolls This recipe is from Eileen Green’s recipe book ‘FATE Special Recipes’. It is great as a main meal served with warm low protein bread and a crisp green salad. Ingredients

Method

1 large aubergine

1. Pre-heat the oven to gas mark 6/200°C/400°F

200g roughly sliced onions 1 clove garlic, chopped 150g chopped red pepper 200g chopped mushrooms Salt and pepper to season Handful of fresh basil leaves or 1tsp dried basil 15g butter 50g FATE Low Protein All Purpose Mix For the sauce 350g Passata (chopped and sieved tomatoes) 2 cloves garlic, chopped Salt and pepper to season

2. Cut the aubergine lengthways into thin slices – you should be able to cut about 6 slices. Place them onto a large baking tray and put in the oven for about 10 mins to soften. 3. Meanwhile, heat a frying pan over a high heat and add the sliced onions. Cook for a couple of minutes and add the garlic, red peppers and mushrooms. Cook for about 5 minutes, until lightly browned. 4. Season well with salt and pepper and add the basil. 5. Add the butter and when it is melted, turn off the heat and add the all purpose mix and stir well. 6. Take the aubergine slices from the oven and place one on the work surface. Take a large spoonful of the filling mixture and put it in the centre of the slice and roll up as tight as you can without squeezing the filling out! 7. Place into an ovenproof dish and continue with the rest of the aubergine slices. 8. In a separate bowl, add the passata, season with salt and pepper and add the garlic. Spoon this sauce on top of the aubergine slices. 9. Place in the oven and cook for 30-40 minutes until cooked, hot and bubbling! Eileen says that the ‘Aubergine Rolls’ freeze well so that they can be made in batches and taken out the freezer for a quick meal to reheat.

News & Views | Issue 145 | Winter 2013/14

23

Dietitian’s report: Paula Hallam

New products and ideas Savoury snack and lunch box ideas

• Dr Schar Gluten Free Pretzels – contain 10mg phe per bag – FREE • D  S Herb and Onion Crackers – FREE (0.18g protein/cracker)

Try these savoury crackers with the Aubergine Pate’ recipe as a delicious snack or in a lunch box. You will find these crackers in the ‘free from’ aisle of large supermarkets.

•  DS Rice Crackers – ½ exchange/ cracker •  Clearspring Organic Corn Cakes (gluten free) – ½ exchange per corn cake •  Clearspring Japanese Rice Cakes (black sesame) – ½ exchange per rice cake •  Rude Health Corn Thins and Brown Rice Thins – ½ exchange per cracker •  Nairns Gluten Free Oatcakes (Original and Herb & Seed) – 1 exchange per oatcake

Low protein prescribable crackers: • Loprofin Crackers and Herb Crackers – FREE (0 exchanges) • Vitaflo new Mini Crackers – FREE (0 exchanges) •  Mevalia Crostini low protein crisp bread – FREE (0 exchanges)

Cauliflower rice Heather Paine-Cardy sent in this clever idea to me. It requires very little preparation and is very quick and easy to make! Remember that cauliflower is in the ‘once per day’ list which means that it does not need to be measured but you should only have one portion per day. The recipe comes from the Daily Mail (Mail Online) newspaper. All you have to do is grate or finely chop

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News & Views | Issue 145 | Winter 2013/14

some cauliflower florets until they are about the size of rice grains. You can also use a food processor if you have one. Then cook on full power in the microwave for about two minutes in a covered microwaveable dish. You do not need to add any water, as there is enough water in the cauliflower itself. And that is it! Serve with your choice of meal, such as a vegetable curry.

News

NEW ! Mozzarisella and SmokyRisella Mozzarisella and SmokyRisella are new vegan cheeses made from Italian brown rice, coconut oil, carob flour and apple vinegar, as well as xantham gum and agar-agar. See the website for more details: www.mozzarisella.co.uk/what-is/ mozzarisella I am considering this as an item for amino acid analysis as the phenylalanine content is likely to be very low. Protein = 0.7g per 100g (140g = 1 exchange)

Xotic Desserts These are a new group of desserts by a company called Global Bounty – www. globalbounty.com – and made from coconut cream with fruit flavours. They are available to buy online from The Alternative Stores – www.alternativestores.com The desserts are available in four flavours:

Moo Free dairy free chocolates These are new vegan mini “chocolate” bars called ‘Mini Moos’. They come in an original flavour, mint flavour, a Bunny bar (for Easter) and Bunnycomb (honeycomb flavour). They are ½ exchange per bar. See www.moofreechocolates.com for more information.

Jelly Eggs from the Co-operative These are little bags of assorted fruit flavour jelly beans – a nice idea as an alternative to chocolates at Easter time. They are completely FREE (they contain only a trace of protein), but remember they are only a treat item!

Ask your Dietitian H Have you got a question about the PKU diet? H Is there a particular food you have found in the supermarket that you are unsure about? H Is there an unusual ingredient you have found on packaging? H Confused about food labels? H Would you like to know the latest about PKU research? If you answered YES to one of these questions, then now is your opportunity to send in a question to the NSPKU Dietitian and we will print your question along with an answer from the Dietitian. Email: [email protected] Please note: The NSPKU Dietitian cannot give individualised dietary advice. Please speak with your own Dietitian first, as they will be able to advise you.

• Berry • Mango and Passion Fruit • Hint of Lime • Hint of Lemon Each pot is 125g and the protein content is from 0.6g per 100g, except for the Berry flavour, which is 0.5g per 100g. So I would count them as 1 exchange per pot, except for Berry flavour – ½ exchange per pot. I am also considering adding these to the amino acid analysis list, as they are likely to be very low in phenylalanine due to the coconut cream (we know that coconut milk is very low in phenylalanine).

News & Views | Issue 145 | Winter 2013/14

25

A range of convenient, low protein foods. More choice, more freedom

Protein free alternative to milk

A low protein savoury,

Low protein chocolate flavoured bars.

to make your life easier every day!

herb flavoured snack.

Use in cooking recipes or make into

Use in sweet and savoury recipes.

novelties such as chocolate buttons, lollipops or Easter eggs.

Recipes available upon request

*

tabite Easter B t for Vi unnie * u o k s o ava Lo ila ble

! on

To make life simpler the Vitaflo Choices® range

so

is available on our home delivery service. If you wish to be registered on our free home delivery service, please contact your dietitian.

www.vitafloweb.com ® Reg. Trademarks of Société des Produits Nestlé S.A., Vevey, Switzerland. A Nestlé Health Science Company

*Please contact your dietitian.

26 PROZERO™, News & Views | Issue 145 |&Winter 2013/14 Mini Crackers VitaBite™ are foods for special medical purposes and should be used under strict medical supervision.

NV0114 Jan 14

Blancmange Easter Bunnies Blancmange is a pudding good for all ages. It is a kind of set custard that is quick ‘n’ easy to make and eat. Equipment needed

Ingredients

• Small bowl

• 1 sachet of blancmange powder#, vanilla, raspberry or strawberry

• Small saucepan

• 500mls ProZero™

• 500ml/pint heat proof ‘bunny’ shaped mould or individual moulds

• 35g granulated sugar (more or less to taste)

Instructions 1. Place the blancmange powder into a small bowl or basin, add the sugar. 2. Stir in about 30mls of

ProZero™.

Mix until smooth.

3. Put the remaining ProZero™ into a saucepan and heat gently until it begins to simmer, pour a little onto the blancmange mixture. Stir well, and then pour in the remainder. Stir well as it begins to thicken.

Top Tip: Rinsing the mould with cold water before filling will make it easier to tip out once cold. Serving suggestions: Fresh fruit is a great accompaniment to blancmange.

4. Pour the mixture back into the saucepan and place over a gentle heat. Simmer for about 1 minute. 5. Taste and add more sugar to sweeten if desired. 6. Pour into a 500ml/pint heat proof mould/individual dishes/moulds. 7. Leave to cool, then place in the fridge for 3-4 hours to chill completely. 8. Turn out of the mould.

#

Special Note! Check with your di etitian for a suitable brand of blancmange. News & Views | Issue 145 | Winter 2013/14

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PKU and me

A light-hearted view of a PKU parent

PKU and parties… through the ages N

ow this might sound like an historical piece from the title but it’s not. If I look back at my daughter’s life with PKU as the main narrative, I can see huge milestones in her PKU career. These being parties according to her age (hence the title). Now when she was conceived, all we saw was a Beautiful – capital B – little baby all shiny new and perfect, bit like getting one of those new frost free fridges from Curry’s. Big on Kudos. For six weeks we thought that until the idiot doctor called came around to tell us that she would grow up to be brain damaged. Tell that to her 2nd year tutor at Leeds University. Idiot. And from that point she grew into a party animal at about six months of age. Our first party with our new daughter… well, scary. If I look back, most social functions were a bit of a nightmare. In point of fact at the parties we did go to, my wife and I became the PKU police without any shred of congratulatory compassion towards the other little party goers or the recipient of presents. It was all about the paranoia surrounding the diet. Party sausages were stomped on. Cheese and pineapple on a stick, burned at the stick and non-pku pizzas slung like Frisbees over the heads of little ones at the inevitable inflatable bouncy castle. These were the early years. Credit where credit is due, the hosts became fantastic at catering for our little party hound, possibly to appease us, her parents, but partly to make sure we didn’t poke and prod their party food offerings. Fast forward to terrible twos, threes fours, fives, etc to the age of about 10. Fortunately for us we got the diet right, unfortunately for us this made our daughter understand that parties were for partying at, and at parties there are no holds barred. This would set the scene right throughout the teenage years. Bear with, bear with.

At the age of 10 and the associated parties, came the screaming years. What I found out is tha PKU becomes irrelevant when you put 20 ten year old girls in one room and tell them that it’s a party. I’d rather stick my head in a 747 jet engine and have a good listen rather than be subjected to the colossal, high-pitched, glass shattering explosion of sound that comes nailed on to one of these gigs. I have a theory that this is subterfuge. Bear with, again. This so when the din has us (parents) bleeding from the ears, the PKU child robs a sausage or other exchange ridden no-nos. To me a party epitomises a life of PKU polarised into a couple of frantic, mayhem-ridden hours. Hours filled with temptation and then denial. At the age of 10 this works fine as the parents hover like big brother in the background ensuring that there are no PKU improprieties. Then they go to parties on their own! How flippin dare they! And so it begins. The metamorphosis from a likkle baby party puppy into the fully armed, sharped toothed, gun totin’ party animal. Locked and loaded and lookin’ for exchanges. This transformation changes the PKU child from a forager, foraging for any scrap of exchange they can forage into “look at me I’m so freeeeee” kind of blood phe level busting blunder beast. This will not stop until they are 28 or in the case of a PKU lady, they wish to conceive. Consult a dietitian… And I write all of this with bitter irony, as this was me many moons ago and I’m just jealous that this husk, mere shell of a body is unable to party any more. My idea of excitement is opening the car window at 60mph on the motorway and watching the Daily Mail blow out of my wife’s hands into the kids faces in the back. My partying days are over and my PKU child is having a massive blast. For those who remember the party seven manufactured by Watneys, I salute you, and those PKUs who have it all ahead of you. Go party. If you’re allowed. And stick to the flippin’ diet. Withered of Herfordshire

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Perspectives

Most contributors to News & Views are deeply involved within the world of PKU and its everyday dietary treatment. Aspiring journalist Caitlin Breheny had little knowledge of the condition and no link to the PKU community, but she wanted to find out more. Here she writes on the subject from a different viewpoint…

From the outside in H

Payments (PIPs). This may or may not be supportive i there, I’ll start by quickly introducing and sensitive to the needs of adults and children who myself. My name is Caitlin Breheny and have adjusted their lifestyle to live with PKU. This I want to give my perspective on PKU as remains to be seen. somebody looking at the disorder from the outside. So, for me, the issues are coming to light very In doing so, I hope to give some insight into what quickly. How is society meant to adapt in order to I feel about PKU as someone who is detached from offer the best care it can for people with PKU if the PKU community. nobody knows anything about it and how do So, the obligatory background summary first. you secure a future for your Society? Is there an I’m a twenty year-old History graduate from Leeds underlying assumption perhaps that people like me University, bit of a yoga fanatic, vegetarian (this is can’t understand what it must be like to be affected where our dietary paths cross to a lesser extent!), by PKU and therefore there’s no need for us to be aspiring journalist, pro-baker (...ok, so the last one interested or at all involved in the individual or group might be slightly twisting the truth). Basically, I don’t activities of PKU sufferers? have PKU, and neither do any of my family or friends. But it’s my belief that it’s not just a small Before I was introduced to the disorder, my knowledge community’s problem. It’s not just something which of what it is was very limited to say the least. Quickly people like me shouldn’t surveying those around me care about if it doesn’t affect in my immediate social circle “I was only one of many millions them personally. In so far of friends and family also who had no concept of PKU. as I understand it, PKU is confirmed to me that no one I do now…” a very successfully treated had much more than heard inherited disorder and you of PKU before. all should embrace the idea of promoting this success A quick Google search brought up some general and the notion of getting other people to understand websites about the disorder and many advertising just how hard the diet sometimes becomes. products for people on a PKU diet. Vague memories of Raising awareness and gaining support is still biology lessons from several years ago about recessive work in progress and long may it continue in greater and dominant genes in genetic disorders were leaps and bounds. Positive steps are being made, and brought back to the surface of my mind, but besides many more are there to be taken if we simply forge that, my entire knowledge and connection with any the links we need to so that PKU is widely recognised of what I was reading was just beginning. and accounted for. My first read of an issue of the NSPKU News & I was only one of many millions who had no Views told a widely different story from what was concept of PKU. I do now and when I think of newavailable on the internet. Turning the pages and born screening, I will be more understanding of the learning of the experiences of those who have dealt condition and its consequences n with PKU and had to adjust to living with it was like unlocking a new door. One that had said ‘PKU only’ Caitlin Breheny – non PKU on it. It wasn’t made for me, but I was intrigued and inspired by everything I discovered. Recent changes to the qualifications for a person in the UK to gain a Disability Living Allowance mean The charity Contact a Family has up-to-date information that many people in the UK living with disabilities regarding DLA/PIP on its website: www.cafamily.org.uk will be required to undergo ‘re-assessments’ to see Click on ‘Know Your Rights’ where you’ll find it under the that they qualify for new Personal Independence ‘Benefits and Tax Credits’ heading.

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News & Views | Issue 145 | Winter 2013/14

News & Views | Issue 145 | Winter 2013/14

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