the nsw pku journal winter/spring 2012 - pkunsw€¦ · project with us over the last four years,...

Aaron Gambill Adrianna Campbell Andrew Pryor Angela Brischetto Antonello

Tilocca Antony Blanch Barker Constructions Pty Ltd (Travis & Sondra Barker)

Bob Beath Carmelo Previte Carol McGree Cathy Duncan Cheryl Rasmussen

Chris King Cleopatra's Temple - Spa & Beauty Centre WA D & K Wallace

Damian Cullen Nathan Cullen Daniel Roberts David Hodge Denise Drew

Dimity Curran Egidio & Maria Emmi Emily Cooper FM Gainsford G & J Ri-

dolfo Gemma Paul Georgina Fleming Gino & Rose Pruscino Giuseppe Angemi

Helen Cullen HMA Twomey Patterson Cootamundra HMA Twomey Patterson

Wagga James Martel James Robertson

Jenna Fallon Julie Smart Kerrie Walsh

Kim Mason Kylie Thompson Lindy

Chalmers Marc Dovico Marion

Finlay Matt Paterson Matt Woolnough

Matthew Chan Peacock Gardens Maureen & Terry Pankhurst Merralee Gordon

Micaela Hopkins Naomi Cooney Newhaven Funerals Paul Hopkins Pauline

Kanhalikhan Peter & Janice Volling Phoebe Payten Rachel Overton Rocco

Perna Rodney Buttriss Roshan Sahukar Rositano Quality Furniture Ruth An-

drews Salvatore Mameli Sarah Bennetts SJ Romanik Stevie Ann Petridis

Susan Leggio Tracey Curnoe V & D Currao Vicki Burgmann

THE CHALLENGE HONOUR ROLL Thank you to everyone who gave to the Challenge. Also to all those that

participated in fundraisers to raise funds for The Challenge

The NSW PKU Journal Winter/Spring 2012

Inside this issue:

Research Update

Low Protein Products

2

Presidents Piece


3

Gala Dinner Report

Golf Day Report

4

Gala Dinner Roll of Honour 5

Camp Is Important Stories 6

Upcoming Events

Contact Details

7

200,000 Cheque Presentation 8

To promote awareness of PKU:

- by providing information and support to families affected by PKU;

- by actively re-establishing contact with adults who have PKU and

do not attend the Adult Metabolic Clinic;

- by supporting research, including fundraising for the purpose of

research into the treatment of PKU; and

- by lobbying Government bodies where appropriate.

MISSION STATEMENT

ABN 78 410 895 701 The PKU Association of NSW Inc.

THANK YOU

Tax Receipts

Please contact Jason Russell, treasurer, ([email protected])

if you have not received a tax receipt for any donation made

during the course of the last financial year.

PKU Research at the Children’s Hospital at Westmead Update – August 2012

Prepared by: Professor John Christodoulou AM, Head, PKU Clinic

It has been a spectacular few months of fundraising by a small dedicated group of parents and friends,

and special vote of thanks must go to Mirella Nicomede for spearheading a team who staged a wonderful

gala dinner, which raised $140,000 on behalf of the PKU Association of NSW. On top of that, the unflap-

pable Maureen & Terry Pankhurst initiates the fundraising challenge raising another $50,000, all of

which has been tagged to support PKU research. We are humbled that so many friends should work so

tirelessly on our behalf, and of course the PKU research team at the Children's Hospital at Westmead is

spurred on by this faith in us to push our research activities to new levels.

The major focus of our research at present is the further development and validation of our genetically

modified probiotic to treat PKU. Our genetically engineered Lactococcus probiotic bug makes good

amounts of the enzyme, phenylalanine ammonia-lyase (PAL), and we have previously shown that in the

test tube we get good clearance of phenylalanine using this GM probiotic.

More recently, our Rotary PhD scholar, Naz Al-Hafid, has been testing the robustness of our GM probi-

otic, which was done by carrying out a series of experiments simulating the environment of the stomach

and small intestine, and has shown, at least in the test tube, that our GM probiotic can survive these

harsh conditions for the relative short duration of our testing time. Most exciting though was her first

experiments in the PKU mouse, where she showed when she feeds them our GM probiotic and labeled

phenylalanine, we see a marked reduction in the amount of phenylalanine being absorbed into the

mouse’s bloodstream. There are many questions though:

Will the GM probiotic break down phenylalanine “locked” in dietary protein in the gut? Our test tube

experiments suggest yes, but we need to do these experiments in the mouse too.

Do we need to give the GM probiotic every time the mouse feeds? If not, how many times a day will

we need to give the GM probiotic?

How long will the GM probiotic survive in the mouse gut?

How much GM probiotic will we need to give and how effective will it be in “real life”?

All of these experiments are planned and will be carried out over the next 12 months.

At the same time, Dr XingZhang Tong, a postdoctoral research scientist in our laboratory, who has been

working on producing the GM probiotic for about four years now, is working on making an even better

version, which will we hope will be even more resistant to the harsh conditions of the small intestine, and

which will enhance the functional capacity of the PAL enzyme. He is also planning to explore whether

Lactobacillus rather than Lactococcus may be a better way of delivering PAL to the gut.

Naz had an opportunity to present her research at the recent Human Genetics Society of Australasia

(HGSA) conference held in Canberra in July, which generated considerable interest indeed from those

involved in the management of children and adults with PKU. In fact, she won a prize for her work.

Well done Naz!

We’re also very pleased to report that Gladys Ho, who has been involved in a number of PKU research

project with us over the last four years, will very shortly be submitting her PhD thesis for examination.

She too presented her research at the HGSA conference, which was also very well received.


...KEEP AN EYE ON OUR WEBSITE FOR ALL OUR CURRENT FUNDRAISERS..... www.pkunsw.org.au


Freedom

Foods Berry

Good Morning

Cereal

6.5 protein/100g

Birds Instant

Custard

4.3 proetin /100g

1.1 protein/serve

Cheezly Mature

White Cheddar

Style Cheese

3.7 protein/100g

Gelativo Passsion-

fruit/ Mango &

Strawberry Sorbet

0.5 protein/100g

Presidents Piece Dear all,

Over the last few months the PKU Association of NSW has been incredibly active supporting numerous

fundraising initiatives as mentioned in the last newsletter. This surge in fundraising activity was initi-

ated by the realisation that funding for PKU research was running out. The threat to the cessation of

that research became very real and a call for support was warranted.

I am thrilled to say that our members, families and friends responded to the call and funds in excess of

$200,000 were collected. Such an overwhelming response confirms to us that the PKU community is fully

supportive of the Research undertaken by Professor Christodoulou and his research team at the Chil-

dren’s Hospital at Westmead.

Our first initiative, “The Challenge” was strongly supported by members, families and friends. Even as I

write, additional donations are being receipted. Our challenge of $25,000 by the 27th June was met with

close to $27,000 being raised. As pledged, Maureen and Terry Pankhurst provided us with a cheque for

$25,000, thereby ensuring $52,000 was available for research funding. This balance is likely to reach

$60,000 as corporate gift matching cheques are processed. On behalf of the PKU Association of NSW, I’d

like to thank all those who were in a position to help us with this challenge and to those who initiated

fundraising activities to support this cause.

Our second fundraiser was the annual Gala Dinner held at Castel D’oro on the 19th June. Close to 250

guests attended what was an enjoyable and from a fundraising perspective, thrilling evening. Many

thanks to Mirella and Mario Nicomede for hosting the evening and to Sandra Bellino, Maria Dovico,

Bridie Melham and Vanessa Towers, who were instrumental in ensuring the preparation and running of

the dinner on the evening, went according to plan. The results of the evening were well in excess of our

expectations with close to $140,000 being raised from raffle ticket sales, silent auctions and donation

pledges. More details of the event are included within the newsletter.

In recognition of these extraordinary events we have included an honour roll in this newsletter in appre-

ciation of all our donors generosity.

In addition to the fundraising activities, the Camp Committee has been busy planning this year’s 2012

PKU Youth Camp. We have 18 confirmed campers joining us in October. All activities and events have

been booked and Bradley Towers has been working with the Chef at Narrabeen and Sue Thompson at

CHW to ensure a five star menu is served up to our campers.

From an administrative perspective, our fundraising authority was successfully renewed for another five

years and all our insurances are up to date. Audited reports for the FY11 year are available for any mem-

ber wishing to inspect the Association’s financials.

Finally, a reminder that tickets to this year’s Halloween Ball at the Epping Club are now on sale via our

website. Like previous Charity Balls, this looks set to be a great night of fun. Please contact Julie Pank-

hurst on 0403 331 360 for details and reservations.

Andrew

Winter/Spring 2012


Zehnder Bread

www.zehnder.com.au

Located at various IGA

Stores

Rice bread 2.7/100g

0.9/serve

Potato Bread 2.6/100g

0.8/serve Streets Blue Ribbon 3in1 fruit basket ice

cream

2.0 protein/100g

Oh What a Night – Close to $140,000 was raised for PKU Research

Our Annual Gala Dinner was held on Saturday 16th June at Castel D’oro Function Centre. It was a

great evening with 260 guests in attendance to raise money for PKU Research at The Children's Hospi-

tal at Westmead. Naz AL Hafid from the research team provided an informative update on the PKU

research. Without the generous donations from our sponsors, and the generosity of those who attended

on the evening, we would not have been able to raise $136,846 for PKU research. A special thank you to

our major sponsors Castel D’oro Function Centre, Onesteel, Coffee Warehouse Café & Deli and Daniele

Jewellers for their generosity and for making our Gala Dinner a night to remember. The logos of our

sponsors have been included in this newsletter and, whenever possible, we would encourage supporting

these businesses within our community. We would like to thank all the PKU families who attended on

the night and assisted in sourcing prizes for our raffle and auction items. A big thank you to Mirella &

Mario Nicomede, Sandra Bellino, Maria Dovico, Bridie Melham and Vanessa Towers for all their hard

work in making the evening such a success.


The 5th Rotary Club of Pennant Hills Annual Charity Golf Day was held at Muirfield Golf

Course on the 4th May in support of PKU Research underway at the Children's Hospital at Westmead.

Proceeds from the day go towards the funding of PhD student Naz Al Hafid, who is a key member of Pro-

fessor Christodoulou’s PKU Research team at the Children’s Hospital Westmead.

80 golfers enjoyed a clear autumn day on what was a beautiful playing course. The game was followed by

a splendid lunch and enthusiastic charity auction.

Congratulations to those who walked away with prizes and to those who were successful in the auction.

We are grateful to John Young, Terry Pankhurst and their Rotarian colleagues at the Rotary Club of

Pennant Hills, who continue to actively support Research into a cure for PKU.

Words of appreciation for Maureen and Julie Pankhurst, Christine Brennan and Theo

who ensured the running of the event on the day were a success.

I’d also like to take this opportunity to thank all our sponsors who continue to sup-

port us year after year ensuring the ongoing success of this event. Likewise, to the

generous families and Harvey Norman who provide the items for the charity auction.

Winter/Spring 2012

Gala Dinner Pledges Honour Roll Many thanks to all of the following the pledged

on the Gala Dinner evening

Beechwood Homes

BlueScope Steel

Business & Taxation Concepts

Casella Express

Danny Glavan

Dolcetti

Emilia Galtieri

Ferrari Industries (Franco & Robyn)

Freedom Concrete P/L

Handycrete

Hunter Pods P/L

J&H Automotive Services

Jessica Artuso

Joe & Rosetta Zumbo

Joseph and Roseanna D'Agostino

Josephine Ianni

Josie Cappetta

Ken & Kaye Towers

Lani & Saki Nioplias

Lifetime Wealth Planners

Linguagrossa P/L

LOTO Concretors

M&E Equipment

Margaret Nicomede

Matthew Ridolfo

Melissa Artuso

Michelle Ridolfo

Giuseppina & Joe Ridolfo

Mortgage Link

Mr & Mrs Vallario

N Distenfano

Nicson's Building Products

Nicsons Concrete

P&R Bellino

Pat Sergi

Rocco & Concetta Perna

Rockfort Group

SADAg Transport

Sam & Teresa Gallo

Scott Jacobson

Slon Electrical Services

Star Concrete (NSW) P/L

Superior Seals P/L

Syeno P/L

Vincenzo & Carmela Zumbo

Wetherill Park Industrial

A Special Thank you to all Sponsors for the Gala Dinner

3 For Fun La Piazza

Angelo’s on the Bay Lite n Easy

Barry Joseph Lorena DiStefano

Belinda Martino Marcia Cipri

Bella Spa Marconi Travel

Ben Woodward Metcash Bill McDeed

Castel D’oro Function Michael Daniele

Choices Gluten Free Mirella Perau

Cipri Italian Nati Bros

Coca Cola Amatil OneSteel Reinforcing Pty Ltd

Coffee Warehouse & Deli Paul & Maria Dovico

Crabtree & Evelyn Paul Melham

Crowd Goes Wild Fitness Pennas Chemist

Cue Clothing Richmond Skin Fitness

Diethnes Greek Restaurant Ridolfo Family

DiStefano Cafe Rip Curl

Dolcetti Pasticceria Sam & Teresa Gallo

Donna Hay Simpson Pharmacy

Ecco Ristorante Sydney Bridge Climb

Emilio Coiffure Tania Vallario

Eskal Taronga Zoo

Featherdale Wildlife Park Telstra

Ferrero Australia The Good Guys

Ferro Family The Steve Waugh Foundation

Guili Food Supplies Ticketek

Guy & Leanne Piccin Tony Trimboli

Icebergs Dining Room & Trims Fresh

J & D Providor Vince Hair Design

Jaycar Vitamin King

John Alten MC WDS

Julian & Franca Hutchen Zoya Kraus

Kmart

Joshua Mortimer - aged 10

Attending the camp is important to me because ... I learn new things. It's fun. Talent show is cool.

Sleepover with new friends. Cool activities like archery.

Lainey Holmes - aged 12

Attending the camp is important to me because ... you get to spend time with others with PKU. You can

learn from each other and share menu ideas. You always blend with the others for once making

everything easy to be yourself. You will never have to explain PKU to anybody. It's really great fun

especially when you can catch up with others from past camps. You do fun things like rock climbing,

archery, making damper, go swimming, go into our talent quest and heaps more. Last but not least

you'll get rid of your fears.


Molly Burley - aged 13

Attending the camp is important to me in many different ways. Firstly I love being surrounded with

many other PKU children just like me and going through the same things I deal with everyday, like

staying on my diet and doing a blood test every month. I like how I am not so different to everyone else

and I know the only thing that makes me different is the different characteristics that makes us all dif-

ferent, our personality and who we are. I also enjoy doing all the activities and I can join in that little

more because I have that weight off my shoulders, knowing that I have to watch everything I eat

whereas the foods at camp are PKU friendly and all I have to do is watch what goes into my mouth and

count my units. This way I get 5 days to me and not have to worry about my diet and also have a lot of

fun while I am there. Lastly I always find it hard to try new foods and make new friends but this is the

perfect opportunity for me to do this. I think it is a great idea to do little activities at night and like on

the last night we did a talent show so we had time to get to know everyone and come up with something

for the talent show. I like not also trying new foods but trying new activities and sport as well. Thank

you for reading my competition entry and to remember if you have PKU "You are just the same you just

don't like those foods" as I like to say or just say your on a diet. And not to mention we do not have to

worry about bringing food for all the days that we are there, just our supplement for the trip.

The PKU Camp is Important to me...

Jasmine Dawson - aged 8

Attending the camp in important to me because ... I want to meet other kids who have PKU. I want to

meet new friends. I want to learn more about PKU.

Tim Burgmann - aged 13

Attending the camp is important to me because ... its not very often I can go somewhere and everyone

has PKU. At camp we don't have to worry about finding food that is low enough because its all low pro-

tein food. Camp is great fun and somewhere to try new experiences such as the flying fox, sailing and

rock climbing. The night activities are fun too such as movies, the talent show and lots more. Hope to

see you there.

Elizabeth Burns - aged 13

Attending the camp is important to me because ... I am able to learn about PKU, meet people that have

PKU and spend a week doing fun and educational things. I get to spend time with friends that I have

made at the other camps and do enjoyable activities with them.

Ambrose Owens - aged 13

Attending the camp is important to me because ... I can be around other people with PKU that know

and understand all about it and are all the same. I can do heaps of cool activities

April Stephenson - aged 13

Attending the camp is important to me because ... its good to see my friends who have PKU again

Harry Owens - aged 10

Attending the camp is important to me because .. to meet other PKU kids because they know what PKU

is and they know all about it too. The are the same as me. To do cool activities and have fun with the

other kids.

The PKU Youth Camp will be held on the 1st to the 5th of October. Readers can follow the campers

experiences on our Facebook page

European Society For PKU Allied Disorders Conference 2012

18th October to 21st October, Adelphi Hotel Liverpool, United Kingdom

The European Society are funning there annual conference in Liverpool, UK in October. For further information visit the ESPKU

website. www.espku.org.au

Committee Meeting 20th October North Ryde RSL from 2pm. Come along and contribute to the committee.

Rotary Charity Halloween Ball

27th October 2012, Tickets are $75. Limited spaces left. RSVP 20th October

The Rotary Charity Halloween Ball will be held at the prestigious Epping Club, Epping. The Halloween themed eve-

ning will comprise of a three course meal, beer, wine & soft drinks—and by great demand, the return of the Legendary

Von Trapp Singers. Contact Julie Pankhurst on 0403 331 360 for further information.

Italian Chamber of Commerce Golf Day & Golf Dinner 22nd November from 11am

Macquarie Links Golf Club

Tickets for the golf event are $120, which includes lunch, cart hire & buffet dinner for one

Tickets for the buffet dinner $55.00

Contact Maria for further details, 0419 628 861 or [email protected]

Ladies Charity Golf Day

23rd November Bonnie Doon Golf Club

Contact Maria for further details, 0419 628 861 or [email protected]

End of Year Picnic Sunday 25th November Blenheim Road Park, North Ryde

Come along to Blenheim Road Park from 12 noon and join in a picnic provided by your committee. For catering purposes please

RSVP [email protected]

FOLLOW US ON FACEBOOK FOR UP TO DATE INFORMATION ON YOUR ASSOCIATIONS ACTIVITIES

Upcoming Events

Winter/Spring 2012

Contact Details

President: Andrew McDonald Phone: 0421 708 657

Email: [email protected]

Public Officer: Debbie Colyer Phone: (02) 9498 6549

Mobile: 0420 978 872


Treasurer: Jason Russell Email: [email protected]

Postal Address:

PKU Association of NSW Inc.

PO Box 274

West Pennant Hills NSW 2115

Website: www.pkunsw.org.au


Secretary: Maria Dovico Phone: 02 9702 5448

Mobile: 0419 628 861


Editor: Vanessa Towers Phone: 0407 264 332


Committee Members;

Sub Committee Members:

Correspondence Representative Michaela Hopkins

Adults & Teens Jill Butler, Debbie Colyer

Events & Fundraising Bridie Melham, Maria Dovico

National Youth Camp 2012

Jason Russell, Paul Melham, Bradley Towers,

Andrew McDonald

Website Development

Geoff Mortimer, Remona Coleman

The PKU Committee warmly welcomes members and friends stories, events or PKU news as

contributions to the Newsletters.

Please feel free to contact us at [email protected]


On the 6th August, the Research Institute at the Children's Hospital at Westmead opened its doors to

the PKU Association of NSW for the 2nd time this year.

The event was hosted by Professor John Christodoulou, who with his research team, have been pro-

gressing with their unique research into alternative PKU treatments.

Following an introduction to the Research Institute by Murray Stone, the tour group was divided into

two groups.

The first group visited the research laboratory, where an orientation of the scientists work environ-

ment was provided, together with demonstrations of the powerful microscope and scanning equip-

ment used by the researchers. Meanwhile, the second group toured the mice husbandry facilities

where they got to see up close our marvellous PKU mice colony.

After a quick break, the groups swapped guides ensuring that all participants toured both facilities.

Upon completion of the tour, Professor Christodoulou answered questions from the group about what

they had seen on the tour.

During afternoon tea Andrew McDonald, President of the PKU Association of NSW, took the opportu-

nity to thank Professor Christodoulou and the research team for all their effort in researching alter-

native treatments for PKU. The PKU Association of NSW took the opportunity to present to Professor

Christodoulou a Platypus Painting done by artist Zoya Kraus that had been donated at the recent

Gala Dinner. We hope the painting can be displayed in the Westmead Metabolic clinic.

The Association also took the opportunity for a retrospective photo opportunity recording the presen-

tation of the $200,000 research cheque.

the nsw pku journal winter/spring 2012 - pkunsw€¦ · project with us over the last four years,...

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