PKUAI meeting 6th March 2018 at 7.30pm at the Crowne Plaza Dublin Airport Hotel, Santry Dublin
Attendees: Fergus Woodcock, Karen Willetts, Karen Philips, Nicola Whelan, Elaine Hodges, Sarah Hodges, Niamh Farrell, Catherine Costello (Nutricia), Pauline Woodcock, Colm Galligan, Gary Conway, Ciara Cadwell, Karen Lavery, David Paolella (Cambrooke), Bernadette Gilroy
CAMBROOKE INTRO & UPDATE ON HSE APPLICATION
Cambrooke has been acquired by Ajinomoto, a Japanese company – Lynn & David now focused on end users of their products and are involved in molecular studies also.
David Paolella gave a brief overview of where applications were at since his last visit. HSE have been communicating regularly via Sabrina which is a plus, but they now require 30-day compliance study (compared to UK where only 7-day study is required). Their application to the HSE, for a number of glycatin products, has been left open for the meantime
David is communicating with Dr Pastores and says the Mater has agreed to run the study and will require at least 15 individuals to take part.
The hospitals ethics board is yet to approve this study but approval should only take a matter of weeks. The Mater is currently advertising for 2 dieticians and once that resource is in place it is hoped the study should commence if not before then.
If you are interested in taking part in the study keep an eye on the website and on our facebook group for notification on when the study is open for registration.
Following discussions with the HSE, David will limit the product range to 5 or 6 different products so he would like to hear which products pkuers are most interested in – he left a number of samples with the group – Again please contact David directly or the PKUAI if there is a product you would like to see on the application.
It is with great regret we hear that Una Hendroff is leaving the Mater Metabolic Unit – she has been a great supporter of the PKU community and will be missed.
NEW PRODUCTS UPDATE
Promin fresh bread now available on LTI card – contact your dietician and local pharmacy for more details.
Hooba foods available to buy privately on the web and reported to be very good with their products ranging from 1.5exchanges upwards – products are mushroom based – see https://hoobafoods.com/
Mevalia products available to buy privately through Amazon.co.uk using parcel motel/Address pal or a similar carrier into the north and then into the republic.
KUVAN UPDATE & PLAN NEXT STEPS
HSE still to issue a decision and it is hoped that will be soon – at the end of Quarter 1 2018 –
The result of the NCPE refusal back in October 2017 was that the process was shown not to be fit for purpose – The recent political pressure and media attention helped to highlight this also. The drugs group met the on January 18th of this year – but we do not know the outcome of this meeting or who makes up the group or how decisions are made. Also, a Rare Disease Committee to assess Rare disease treatments is being setup within the HSE
It is important that we keep in touch with local ministers for updates – to keep the matter on agendas.
MEETING WITH MINISTER FOR HEALTH SIMON HARRIS
Bernadette Gilroy met with Simon Harris recently thanks to TD John O Mahony creating the opportunity – Bernadette covered all the areas of concern for the PKU community –
Bernadette advised him of our support in retention of the newborn screening cards and discussed Assessment process for Kuvan / products available on GMS & LTI cards /adult care / lack of patient input / DCA /Budget spend – It was a very positive meeting which gave the minister a lot more insight into the complexities of living and managing PKU – Simon Harris to follow up on staffing issues within the adult clinic.
CHARITY STATUS UPDATE
Fergus reported on the Charity Status- we are in the final stages, with some small queries raised and our final submission due to be made by the end of this week.
The decision-making process could take another 6 to 9 months.
Once we become a registered charity we can join the Medical Research Charites group (MRCG). Joining the MRCG will give us opportunity to support research on PKU in Ireland
Bernadette Gilroy reported on the blood results. Method and how often is very important and we should agree on and push for best practice –
Temple street to follow the new EU guidelines which unfortunately reduces the number of bloods taken for those less than 6months from twice a week to once a week. There have been many lab issues over the years and issues with IT systems between the children and adult clinic. It is hoped the building of the new children’s hospital will eliminate a lot of discrepancies in service. It was suggested that Nutricia hold a workshop on bloods at the Low Protein Living weekend.
QUALITY ASSURANCE ON PKU SUPPLEMENT DRINKS
There have been many issues with the quality of certain drink batches over the years – with the smell, texture and consistency of the drinks and this directly effects adherence.
The PKUAI will draft a letter to the manufacturers for advice on how to report any bad batches received and to then issue guidelines to members on how to sample test each new batch received and how to report back the information effectively to manufacturers.
It was suggested to Catherine from Nutricia to include a quality assurance presentation and educational programme on this subject as part of the next Low protein living weekend event.
- Rare disease day 28 Feb coincided with our snow event! and the Rare Disease Symposium was postponed – This been rescheduled for 3rd May in UCD.
- NSPKU Event in the UK also took place during the snow and unfortunately Gwen Kennedy despite her efforts and camping out at the airport couldn’t make it due to flight cancellations.
- EURODIS have an event in May – no details to hand yet.
- NSPKU holding an event in Belfast on 28th April – no program issued yet.
- We are looking for ideas for the International PKU day on 28 June – please get in touch.
- NUTRICIA are holding a family event at Dublin Zoo on 9th June – visit their website or LP Connect for further details.
- National PKU Alliance are holding their yearly event in Atlanta USA in July – 2 of our members Bernadette Gilroy and Karen Willetts have obtained scholarships to attend – Gary Conway and Ciara Cadwell obtained sponsorship also to attend– we are delighted to have representation there this year.
- NUTRICIA are holding their PKU weekend event on 13th and 14th October at Trim Castle – more details to follow and they are interested in hearing what topics you would like covered.
- ESPKU dates yet to be announced will be held in Venice this year.
Next Meeting date agreed as Wednesday 2nd May 2018 –
VITAFLO to attend along with Chef Neil who will give a cookery demonstration 1 hour before scheduled meeting – Venue to be announced and more details to follow.
Meeting concluded 9:30pm – Thanks to all who attended /Skyped and contributed.