Minutes of meeting 26th January 7.30pm at the CityWest Hotel

Meeting facilitator: PKU Association Chairman Fergus Woodcock
Attendees: Pauline Woodcock, Karen Willetts, Bernadette Gilroy, Karen Lavery, Deirdre Coll, Jennie Daly, Joanne Hughes, Karen Philips, Karen Moore, Goretti Gaule, Colin Donnelly, Cliona Donnelly, Nicola Routledge, Alex Markey, Damien Markey, John Markey, Deborah Markey, Sinead Kearns, Dereck O’Neill, Fiona Keane, Deirdre Coll, Susan Reddy, Louise Conlisk.

Agenda Item: Mevalia – Emma Coates from Mevalia presented many Mevalia products, giving out sample packs and explaining she has been working hard trying to get these products approved onto the LTI card. However she is having difficulty dealing with the HSE. In the meantime the products are available to purchase through the mevalia website. (UPDATE: purchasing through Mevalia website has been discontinued)

Actions to be taken: Please try the samples and email Emma (Emma.Coates@drschaer.com), The PKUAI (info@pku.ie) and the dietitians in Temple st (metabolicdietitians@cuh.ie) your thoughts on these products.

Agenda item: Kuvan – Louise Conlisk, Irish PKU mum in Australia whose 11mth old daughter is taking kuvan spoke about how this has greatly helped her baby. Karen advised that the HSE have still not let us know the results of their assessment. We need to continue applying pressure on the HSE for a response.

Karen has agreed to share her most recent correspondence with her local TD. This was written in response to the standard ‘HSE are still assessing kuvan’ letter which most of us have received from our TDs. We can use this letter as a starting point to help write our own letters. TDs are much more likely to help if the letters are personalised.

Actions to be taken: Karen will post on FB an abstract of Louise’s presentation (completed 27th Jan)
Everyone should write to their TDs. Karen Willetts will post on FB her TD response letter. (Completed 27th Jan)
Karen Willetts will provide a word version of the letter to anyone who asks.
(Update: HSE have asked BioMarin for more information which BioMarin are now compiling)

Agenda item: Update from recent meeting with BioMarin. Fergus spoke about the PKUAIs meeting on the 12th Jan. Nigel Nicholls started the meeting by explaining the history of Biomarin and the development of Kuvan. BioMarin are a company which specialises in the development of orphan drugs and they developed Kuvan. They then sold the rights to the drug to Merck Serono. BioMarin bought the licence for the drug back and they once again became the licence holder on the 8 January 2016. They intend to continue the process of the reapplication to the HSE for the drug to be included on the GMS list. They would welcome any support of the application that the PKU AI can give. The process is supposed to last 90 days but they had still not heard anything from the HSE. BioMarin intend to contact the HSE for an update and to confirm that they are taking over the application.

The reply can take one of 4 forms;
• They could ask for more information.
• They could refuse the application.
• They could accept the application.
• They could accept subject to a reduction in price.

(Update: HSE have asked BioMarin for more information which BioMarin are now compiling)

The PKU AI gave a brief description of their history and how they have supported the application and made a submission to NCPE when Merck Serono application was made. In addition Karen outlined the forthcoming article in the Times which raised awareness of PKU and also the issue of the availability of Kuvan. The PKU association would hope to continue to support the application to have Kuvan available in Ireland and would assist BioMarin where possible.

The PKU AI then outlined their plans and aims for 2016.
• Continue raising awareness Of PKU
• Raise awareness of new products for PKU to the PKU Community
• Develop website
• Implementation of European Guidelines
• Affiliate with one of the other rare disease organizations i.e. GORDO, IPOSSI
• Raise awareness and campaign for new dietary products
• Irish Times Article
• Address issues in Adult Metabolic Unit re transition.

Bernadette Gilroy and Dr Ronan Murphy gave an outline of Bernadette’s research proposal in relation to people with PKU and their diet and its long term effect on their weight, fitness and general wellbeing. They outlined the plan for how the research would be conducted and how DCU have various studies that could be adopted to look at the situation in relation to adults with PKU. There was also some discussion around the research proposal from Bernadette Gilroy and how BioMarin could help with this. This will be passed by Nigel Nicholls to the medical department for review. BioMarin have had a change of medical directors and Eleanor and Nigel would therefore have to make sure internally this was in front of the right person. Dr. Ronan Murphy from DCU, is Bernadette’s supervisor on the research, he works with Professor Niall Moynagh (of Operation Transformation fame). He also gave an insight into the type of cost annually this research would cost, he estimated anywhere from €15,000-20,000 per annum.

Agenda item: Fund raising, financing the PKUAI objectives – Vitaflo, Nutricia and Promin have been contacted by Karen Willetts and are very keen to discuss how they can help us financially. Niamh Keegan raised €150 for a Christmas Day swim!! Well done Niamh.

Actions to be taken: Vitaflo, Nutrica and Promin need to be followed up. This Item will be carried over.

Agenda item: The PKUAI website – hosting. It was agreed that developing a website is one of our top priorities. This will give us credibility and a platform to share information specifically for PKU in Ireland. We were advised that hosting cost should not be too high. Karen Willetts asked for help with this from anyone who had any experience.

Actions to be taken: Sinead Kearns and Susan Reddy said they will contact Karen to see what assistance they can provide. If anyone can help in any way please get in touch with Karen. (Update: Conor Byrne is helping with the website and we hope to launch soon)

Agenda Item: Low protein food manufacturers – Food products which are available in Europe but not Ireland. ie Taranis, Cambrookes as well as Mevalia are trying to make their products available on the LTI

Actions to be taken: Jennie Daly will contact Taranis and Cambrookes to see if they would like to present at one of our meetings similar to how Mevalia did at this meeting. (Completed)

Agenda Item: European Society for PKU conference. This conference cannot be recommended more highly. Seehttp://www.espku.org/conferences/e-s-pku-conference-2015/ for more details. Unfortunately the 2016 conference which was due to take place in Istanbul in October will have to be moved due to political unrest in Turkey. So they are looking for a new venue. This is a great opportunity to highlight PKU in Ireland and bring PKU key player from all over the world here! As Sheila said in her article in the Irish Times Jan 19th ‘although Ireland was once a world leader in the detection and treatment of PKU, this is no longer the case.’ Maybe through holding the ESPKU conference here we can help change that.

Actions to be taken: Karen Willetts will carry on looking for a hotel. If anyone would like to help please get in touch. (Completed)

Agenda Item: Nutricia’s annual conference. Fergus Woodcock will present at the LP wkend.

Actions to be taken: Fergus to prepare speech and share his presentation with Nutricia beforehand.

AOB: Susan Reddy advised that she was successful in her application for Domiciliary Care Allowance. It took a really long time, a couple of appeals and an oral hearing.

Actions to be taken:
Susan will share her experience and advise best she can to anyone who is considering applying for DCA. Please contact Susan at susan.reddy@hotmail.com
The PKUAI will discuss how we can further assist applications for DCA at our next meeting.

Our next meeting will take at the Low Protein weekend held by Nutricia in the Hodson Bay Hotel, Athlone, Saturday 27th February 4.30 pm