CFAI Annual Report 2011

annUaL report 2011

Cystic Fibrosis association of ireland

www.cďŹ reland.ie

About the Cystic Fibrosis Association of Ireland (CFAI) The CFAI is a registered charity CHY 6350 that was set up by parents in 1963 to improve the treatment and facilities for people with CF in Ireland. It is a national organisation with many Branches around the country.

The CFAI is committed to working to improve CF services in Ireland and our recent progress includes: Lobbying to ensure that the new national adult CF centre in St Vincent’s University Hospital will be completed Providing funding towards new CF Units around the country including Crumlin, Drogheda, Galway, Mayo and Limerick Hospitals Funding research Campaigning to improve the rate of double lung transplantation in Ireland Providing advice and expertise

Contents Chairperson’s Foreword John Coleman, Chairperson CFAI. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 01

Section 1 Progress and Challenges in 2011 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 05

Section 2 Update on CF Centres in Ireland . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10

Section 3 Key Policy Developments in 2011. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21

Section 4 Support Services, Awareness and Fundraising. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 24

Section 5 Report of CFAI Annual Conference, 2011. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29

Annex National Executive Council and Staff 2011 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39

Information on Annual Report 2011, Progress & Plans This is the Annual Report of the Cystic Fibrosis Association of Ireland (CFAI) for 2011. The purpose of this summary is to provide an overview of the work of the CFAI, including progress in meeting our current programme of work.

The CFAI is a voluntary, non-profitable, charitable organisation. It was set up in 1963. The CFAI seeks to improve services, increase knowledge and awareness of Cystic Fibrosis (CF), and provide advice and support to people with CF and their families.

Charity Number: CHY6350

CFAI Annual Report 2011 Chairperson’s Foreword

Chairperson’s Foreword

page 1

Chairperson’s Foreword I am deeply honoured to be elected National Chairperson of the Cystic Fibrosis Association of Ireland. I would like to thank the Directors for their support and encouragement and trust in me to fulfil this very important role. I would also like to thank the many well-wishers who are too many to name, but who offered their support.

I would like to thank the outgoing Chairperson Paul Higgins on behalf of the Association for steering and directing the Cystic Fibrosis Association of Ireland to the position it is in today. I would also like to thank the Directors for their support and attendance at the NEC meetings and contribution to debates and discussions, and the many Branch Officers and Committee members whose tireless work is exceptional and also those many fundraisers who may organise or take part in an event or carry out collections.

Finally, I would like to thank my wife Lynda and children, and especially Christian who is the reason and motivator for my involvement, for allowing me the time to take this role on.

Now that our CEO Philip Watt has well and truly settled in the Association I am sure we will reap the benefits from his considerable experience and understanding of the voluntary sector. In my discussions with Philip, it is clear that his vision will provide innovative structures for the Association.

The National and Global economic crisis will no doubt provide stumbling blocks to the best laid plans, but I am sure that Philip’s diplomatic but firm approach will see us in good stead. Already Philip is forging links and alliances which will increase the profile and the prestige of the Association and keep us as the driver of innovation for patient care.

Subcommittees The subcommittees continue to be a success, allowing members to probe more deeply into the proposals put before it. In particular, the staff and finance committee have worked well. In addition to reviewing the monthly financial reports, they have helped to speed up decision making with regards to critical funding decisions. I hope to introduce more subcommittees to help push the boundaries and develop the Cystic Fibrosis Association of Ireland into a more transparent, dynamic, vibrant and professional Association.

I would urge members to get involved in these committees. I hope to continue holding Regional Meetings and, as far as practical, I would hope that at least one of the Officers of the Association will be present at these events to answer questions and queries of what is happening.

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CFAI Annual Report 2011 Chairperson’s Foreword

The CFAI continues to be a vocal advocate for the rights of People with Cystic Fibrosis (PWCF) and we are very fortunate to have PWCF who are willing to speak about the unjustness of aspects of the HSE system. We were very fortunate to have An Taoiseach Enda Kenny open our conference in Mayo this year – his speech and the time he spent with us indicates that Cystic Fibrosis is well and truly on the government agenda. Our challenge is to keep it there and indeed argue the case for moving it up to top priority.

The CEO and I have attended meetings with the organising committee for the European Conference to be held in Dublin in June 2012. This promises to be an exciting event and we will be calling our members to volunteer and assist for the occasion.

We also attended the Cystic Fibrosis Registry AGM in 2011. This AGM was tinged with sadness as we remembered the great work undertaken by Linda Foley RIP, the former Director of the CFRI. At this meeting I was appointed the Registry’s Treasurer.

We reviewed the latest statistics which demonstrated the improved prognosis for PWCF as survival rates and median age of death is cathing up with those of our neighbours.

The CF Consultants in attendance agreed that such reports were not only heartening to PWCF but would be helpful in arguing our case with the HSE. In September we met with the Minister for Health, Dr James O’Reilly TD, where there was a robust exchange of views. We emphasised the need for promised staffing levels to be immediately implemented and also to ensure that equipment should not be left idle for want of staffing. We would be naive to assume that it will be plain sailing in light of the economic climate we find our country in. It is clear from listening to the Minister that there will be huge pressures to restrict health expenditure in the short run and therefore it is important that we position ourselves to ensure that services to our members are not affected.

We are delighted that the building work on the new CF unit at St. Vincent’s University Hospital is making progress and will be completed in June 2012. The new four bed unit at Our Lady’s Hospital in Crumlin was officially opened by former President Mary McAleese on October 4th. This unit was directly funded by the CFAI including through the enormous support from the Shane Kinsella Foundation and the 1 in 1,000 initiative in 2010, and we are very grateful for their tremendous work.

The year, if not years ahead, will be full of financial challenges. It is important that the Association acts together and that Branches continue to support the Association as a whole.

We have seen how successful local fundraising has been with respect to TLC4F, Castlebar, Drogheda and Galway and the independent Build4Life initiatives, but it is important not to lose sight that the CFAI is an advocate for all our members and that in order to provide this support the National Office must be resourced properly.

Finally, let me sincerely thank the staff at CF House for their commitment and perseverance; their workload has increased dramatically and it never ceases to

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CFAI Annual Report 2011 Chairperson’s Foreword

amaze me how they remain so committed to our cause. They continually push the boundaries while at the same time keep a watchful eye on events.

I would like to thank the members of the Medical and Scientific Council who tirelessly support our cause and, most importantly, the cause of PWCF at an individual and local level.

Also to my fellow Officers and Directors, I look forward to working with you and sharing workloads in an effort to secure a better future for PWCF and their families.

John Coleman Chairperson

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seCtion 01

progress and Challenges in 2011

Section 01:

Progress and Challenges in 2011

Despite all the challenges, it has been another year of steady progress for the Cystic Fibrosis Association of Ireland (CFAI). Significant gains have been made in improving CF services and the public profile of issues related to CF and the CFAI remains high.

However, we are still playing ‘catch-up’ to meet the historic deficits in CF services in Ireland after years of underfunding. In addition, some of the general measures to save money in the health services, such as recruitment embargoes and cutbacks in disability and care supports, remain a major worry.

CFAI will celebrate its 50th anniversary in 2013. The CFAI is one of the longest established CF Associations in the world. We are one year older than our sister organisation in the UK and three years older than Belgium. The Canadian and the US Associations predate us only by a few years. The emergence and development of the CFAI is a tribute to those parents who had the vision to found the Association in 1963 and to all those who have been involved in the development of the CFAI. We will be organising events to mark this important anniversary and to acknowledge all those who have contributed to the development of the Association over the years.

The turning point for CF services in Ireland came about as the direct result of the CFAI’s ‘Pollock Report’ published in 2005 and largely endorsed in a subsequent report by the HSE in 2009. This report identified: The need for CF Centres to be developed in Ireland with adequate support services The centrality of the multi-disciplinary team approach The importance of cross-infection guidelines The need for specific programmes such as screening for CF at birth Despite the welcome investment in CF facilities staff by the Department of Health and Children resulting from the Pollock Report, there remain challenges.

Through our advocacy work, through our fundraising and through our support services we can report on some of the progress that has taken place over the past 12 months, which are further detailed in this report.

These include: The progress in building the new adult referral centre in St Vincent’s University Hospital Dublin to be completed in June 2012 and the commitment to 34 beds for CF patients.

The formal opening of the new 4 bed CF ward in Crumlin Children’s Hospital in October 2011 The progress made towards the forthcoming adult centre in Limerick by Branches in the region (TLC4CF) in partnership with the National Office Forthcoming developments in Castlebar and Drogheda developed through partnership with the Mayo Branch and Life Matters for CF (Louth and Meath Branches)

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CFAI Annual Report 2011 Section 01: Progress and Challenges in 2011

Funding for staff and/or equipment in Galway, Tallaght and Temple Street Hospitals The new out-patient facility in Cork, supported by the independent charity Build4Life and our members in the region The commencement of newborn screening for CF for the first time in Ireland in July 2011 The appointment of the first dedicated lung transplant surgeon in Ireland at the Mater Hospital in September 2011 Support from the CFAI and the HRB/MRCG for crucial research projects in Ireland Grant support for members including the exercise, fertility and transplant grants The CFAI has also contributed to broader policy developments that have the potential to impact on CF through our active involvement in national initiatives on the forthcoming Irish National Plan on Rare Diseases and the need for a National Strategy on Patient Registries in Ireland.

This work is in part motivated by our concern about the future of the Cystic Fibrosis Registry of Ireland (CFRI), which has had its funding slashed by 50%. The CFRI was established by the CFAI but is now independent in line with accepted good practice and the data it collects was crucial to the Pollock Report and how CF services should be continued to be targeted in Ireland.

We have many priorities for 2012 and beyond in addition to those already highlighted, in particular our ongoing support for in-patient and out-patient centres. One of the most important of these is the development of National Clinical Standards on Cystic Fibrosis in Ireland. The CFAI has been invited to participate in the development of such standards.

This builds on other policy initiatives including our active contribution to the HSE steering group on newborn screening for CF and our lobbying for the establishment of a National Organ Transplant and Donation office through our active involvement in the Irish Donor Network (IDN).

Welcome news was announced at the European Cystic Fibrosis Society (ECFS) Conference in Hamburg this year with further progress in the development of a new type of drug. Vertex Pharmaceuticals, an American company, is developing a CF drug called VX-770 (Kalydeco), which for the first time is designed to impact the underlying cause of CF – a faulty gene and its protein product called CFTR. It is important to emphasise once again that while VX-770 represents a significant advancement, it is not a ‘cure’. This drug could potentially impact up to 10% of the CF population in Ireland who have the so-called ‘Celtic gene’ alteration G551D. The work of Vertex in developing VX-809, which is designed to move defective CFTR to its proper place in the cell, is also highlighted in this report.

CFAI also welcomed the new Tobi®Podhaler that was introduced by Novartis in November 2011 which will simplify the process and reduce the time in taking antibiotics for CF patients.

The CFAI continues to support research in Ireland and we are pleased to confirm that two new research projects (see 1 and 2 below) will be funded over the next 3 years with 50% coming from the CFAI and 50% from the Health Research Board. A total of four research projects are therefore currently being funded by CFAI in conjunction with the HRB: Page 7

CFAI Annual Report 2011 Section 01: Progress and Challenges in 2011

1. The role of mucus and mucins in mediating Pseudomonas aeruginosa colonisation of the cystic fibrosis lung Principal Investigator: Dr. Marguerite Clyne, University College Dublin 2. National Prevalence of Depression and Anxiety in Patients with Cystic Fibrosis and Parents: Impact on Health and Quality of Life Principal Investigator: Prof Eileen Savage, University College Cork 3. Gender differences in muscle dysfunction and electrical muscle stimulation in cystic fibrosis.

Principal Investigator: Prof Charles Gallagher, St. Vincent’s University Hospital 4. Genetic Modifiers of the CF Phenotype Principal Investigator: Dr Ed McKone, St. Vincent’s University Hospital / University College Dublin We welcome Dr Barry Plant as the new Chair and Dr Ed McKone as Hon Secretary to the CFAI Medical and Scientific Council, which continues to meet on a quarterly basis.

The role of the Council remains of fundamental importance to the work of the CFAI and progress on CF in general in Ireland.

Ireland will be hosting the European Cystic Fibrosis Society (ECFS) Annual Conference in 2012. The conference will be an important opportunity to highlight the work and skills of clinicians and healthcare staff involved in our dedicated multidisciplinary teams.

The profile of CF and the CFAI remains high. Our national public awareness period ‘65 Roses Week’ in April 2011 was a great success. Highlights included: The Taoiseach’s, Enda Kenny T.D., speech at our Annual Conference in Westport The special edition of Prime Time on RTÉ presented by Orla Tinsley The significant increased sales of our ‘65 Roses Pin’ designed by the well known Irish artist Paul D’Arcy The many fundraising events that took place during the week at a local level The annual awards for support for CF presented this year to Professor Charles Gallagher, ‘1 in 1000’ Flora Women’s Mini-Marathon team and the Paddy Kieran’s Memorial Walk committee Thanks again to the wonderful commitment of our Branches, parents, CFAI staff and friends. The fantastic work of our Branches and the regional partnership of Branches in partnership with the National Office and our Board is the engine that drives the work of our Association.

On a personal note I would like to thank the members of the National Executive Committee of the CFAI (Annex), in particular the role of our present Chairperson John Coleman and outgoing Chairs, Seán O’Kennedy and Paul Higgins and the Officers of the Association including David Fitzgerald (Treasurer), Cyril Gillen (Vice Chairperson) and Patricia Duffy (Secretary).

Many thanks to our patron, Paul McGrath, the former Ireland footballer for his continuing support to CFAI and to Fuzion.

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CFAI Annual Report 2011 Section 01: Progress and Challenges in 2011

I would like to thank our excellent staff team (Annex), in particular our two senior staff members Helen Whitty and Martin Cahill. Once again, this report is very much the reflection of a team effort.

Philip Watt CEO, CFAI

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CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

seCtion 02

Update on CF Centres in ireland

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SeCtIon 02:

Update on CF Centres in Ireland

The following is an update on some of the main adult and paediatric CF Centres in Ireland over the past year, focusing on some of the most important developments during this time. adULt CF Centres St Vincent’s University Hospital, Dublin 2010 was the year that we finally saw commencement of building work at St Vincent’s University Hospital. On 14th October 2010, CFAI welcomed the final signing of the contract to build the new ward block at St Vincent’s University Hospital, part of which will form the basis of the new Cystic Fibrosis National Adult Referral Centre.

There are presently only eight en-suite isolation rooms plus two small single rooms in St Christopher’s ward at the hospital, which has resulted in many CF patients being accommodated in multi-bed wards. The cross-infection issues resulting from these unacceptable conditions were highlighted on many occasions in the media.

Construction of the new ward block commenced in November 2010 and the completion date is June 2012 (see details of the building programme below).

Activity

Planned Start

Planned Finish

Site set up/enabling works

1 November 2010

28 January 2011

31 January 2011

25 March 2011

Superstructure

14 March 2011

08 July 2011

Roofing

27 June 2011

12 August 2011

External envelope

16 May 2011

2 December 2012

Internal services and finishes

30 May 2011

20 January 2012

Delay thresholds

23 January 2012

16 March 2012

(road, bulk excavation)

Substructure (basement construction and foundations)

Project complete

June 2012

Construction was delayed in June 2010 when the preferred tenderer went into financial difficulties. A new construction company then had to be identified, which is John Paul Construction Ltd. The Government will reimburse the contractor on completion of the building project.

Artists Impression of the completed building at St Vincent’s.

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CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

Building work is ongoing at St Vincent’s University Hospital. Photo provided in September 2011.

The commitment of 34 in-patient beds for people with CF (PWCF) in this new ward block was approved by the Minister for Health, James Reilly T.D. When beds are not required for PWCF, they will be used for other in-patients with a clearly agreed protocol that there will always beds available for PWCF who require admission.

The new CF centre will meet both out-patient and in-patient requirements for CF patients and will be completed in June 2012.

Beaumont Hospital, Dublin Beaumont is one of the key adult CF centres in Ireland. In November 2010, a new outpatient unit opened that provides a significantly enhanced service to adult CF patients attending this hospital. The new unit facilitates the delivery of a comprehensive range of services by multidisciplinary teams to allow more CF patients to be seen during each day.

Services now available to PWCF include consultations with doctors and nurses, provision of treatments that might otherwise have required hospitalisation, assessment of pulmonary function, physiotherapy, dietetics and psychology.

The building includes consultation and therapy rooms with air filtration units which allow for twelve air changes per hour, approximately three times the average rate in a normal room. This has significant benefits in reducing the time needed between patients for infection control purposes, which is particularly important for PWCF. Along with priority access to a number of en-suite in-patient rooms, it means that CF patient’s admission as an in-patient via the Emergency Department in now a more rare occurrence.

The new out-patient unit at Beaumont Hospital opened in November 2010.

-Consultation and therapy rooms with air filtration units allow for 12 air changes per hour at the new unit in Beaumont.

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CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

The new 2,500 square foot unit has been built, at a cost of €3.5 million, with funds provided under a capital allocation from the HSE. CFAI would like to acknowledge the contribution that CF Hopesource Foundation, in particular, Paul Dempsey and colleagues in supporting the Beaumont development, which was identified as a key centre in the CFAI’s Pollock Report.

In autumn 2011, CFAI met with the CEO of Beaumont hospital, Liam Duffy, along with CF Consultants Prof. NG McElvaney and Dr. Cedric Gunaratnam and representatives from the CF Hopesource Foundation and the Eastern Branch of the CFAI, to discuss the lack of in-patient beds at Beaumont. Only four in-patient beds are made available for the 130 PWCF currently attending the hospital, despite the fact that six beds had been promised originally. This is clearly insufficient and CFAI, in conjunction with these groups, are currently liaising to find a solution to this problem.

PAEDIATRIC CF CENTRES Temple Street Children’s University Hospital, Dublin Temple Street Children’s University Hospital officially opened a brand new CF and respiratory out-patient unit on 25th March 2010. The total cost of this project was €3.2 million, over €2.5 million of which was raised through donor support and corporate partnerships.

The Fundraising Office partnered with Friends First and a charitable foundation run by its parent company, The Eureko Achmea Foundation, to fund the new respiratory laboratory, which is a vital component of the service.

The new CF and respiratory out-patient centre has made a significant difference to CF patients attending Temple Street. The centre will serve this function until the new National Children’s Hospital is built.

The CFAI contributed €15,000 towards equipment to Temple Street in 2011, which included funding from the Paul McGrath golf classic.

Our Lady’s Children’s Hospital Crumlin, Dublin The formal opening of the new 4-bed isolation, in-patient unit at Our Lady’s Children’s Hospital in Crumlin took place on 4th October 2011, with former President Mary McAleese and the Archbishop of Dublin Dr Diarmuid Martin.

Members of the 2010 1 in 1,000 committee at the official opening.

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CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

The CF team explaining the significance of the new unit.

-Former President McAleese meets a patient in the new unit.

The CFAI provided €750,000 towards the development of the new unit in 2009/10, the funds for which was drawn from a range of resources, particularly the Shane Kinsella Foundation and the 1 in 1,000 Mini-Marathon initiative in 2010.

The new CF unit comprises of four single en-suite rooms, two with isolation lobbies and full Hepa-Filtration, which will provide negative pressure and two without isolation lobbies. The unit area covers approx 200 sq metres designed to current best standards and finish. Each room has its own nurse call, monitoring system, a/c unit and television/ games console, pull-down bed for parent rooming-in, storage etc. A parent’s lounge with television, microwave, fridge and tea making facilities is included. The unit is connected back to the existing St Michael’s ward via a new link corridor with a nurse station and ancillary rooms including treatment room, minor procedures room at the axis. This part of the project was funded by the Children’s Medical Research Foundation of Crumlin Hospital.

These are the first dedicated isolation/en-suite rooms for children with CF in Ireland. They are state of the art and CFAI contends they should provide a template for children with CF who will be attending the future National Children’s Hospital.

Our Lady of Lourdes Hospital, Drogheda Drogheda is a shared care centre with Temple Street and Crumlin. The CFAI organised a meeting on 16th of February 2011 in Drogheda to discuss the ongoing unsatisfactory CF facilities for children in Drogheda Hospital for both parents and healthcare staff. Architect Manfredi Anello also spoke about how the out-patient facilities for children attending the hospital could be improved, if sufficient funding was secured and planning permission was granted.

Plans for the new out-patient facility on Windmill Road for children with CF attending Drogheda Hospital.

WINDMILL

ANNEVILLE HOUSE

HOSPITAL CAR PARK

n 21 JUNE

21 DEC

ANNEVILLE CRESCENT client

Cistic Fibrosis Association of Ireland

project Out Patients Facility at n°52 Windmill Road

Site Plan Scale 1:500

date

SITE OUTLINE IN RED ROAD ACCESS

Drogheda Co. Louth site plan

drawing

1028 ske

HOSPITAL ACCESS 16/3/11

page 14

description

issued to the HSE and clients

job ref.

1/200 scale

issue October 2010 date

0-001

series

n°

1028 ske CAD file name

anello architects 6-9 trinity street dublin 2 t/f:+353 1 617 7990/1 info@anelloarchitects.com

CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

Subsequent to this meeting, and with positive support from hospital management, an innovative solution came to fruition with the purchase of a building on Windmill Road facing onto the carpark of Our Lady of Lourdes Hospital, which will be the location of a dedicated out-patient facility for PWCF attending the hospital. Planning permission has been granted and a meeting with hospital management is forthcoming to progress these plans further.

These premises will be furnished to high specifications with isolated rooms and filtered air conditioning, thereby reducing the risks of cross infection. The CFAI National Office will also be making representations on behalf of members to have the appropriate level of specialised staff in place, including a permanent CF Consultant. A major focus of the CFAI over the next few years is to ensure that shared care centres, such as Drogheda, do not get left behind in relation to CF developments in Ireland.

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CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

CASESTUDY:

Focus on Life Matters 4 CF

Loretta Allen Byrne and Máire Gallagher at the launch of Life Matters 4 CF.

Iain, Cathal and Máire Gallagher with CF nurse specialist Mary Hanratty at the Lumpers Tain Trek

Life Matters 4 CF (LM4CF) is made up of a forum of people from Co. Louth and Co. Meath who are campaigning for a dedicated out-patient facility for the 25 children with cystic fibrosis from Meath and Louth attending Our Lady of Lourdes Hospital in Drogheda. This committee is made up of a dedicated team of parents/relatives of children with CF, and the Steering Group includes Máire Gallagher, Loretta Allen Byrne, Cyril Gillen, Gerard Fay, Philip Watt and Peter Hughes. This group aim is to improve services for these children to European standards, and they are working endlessly to fundraise and increase awareness for these improvements.

The official launch for Life Matters 4 CF was held on Saturday 23rd April in Kilmessan, Co. Meath where celebrity guest Jason Maguire, Aintree Grand National Winner, opened the event.

With the help of the CFAI National Office, a building on Windmill Road facing onto the car park of Our Lady of Lourdes Hospital has been secured and this, along with the positive support from hospital management, has enabled LM4CF to move forward with this project.

Planning permission has now been granted and renovations are set to begin shortly.

The Drogheda and Dundalk Branches of the CFAI have been fundraising for the last 4 years in anticipation for improvements of CF services and facilities in Drogheda. Gerard Fay, also known as ‘Marathon Man’ from the Drogheda Branch, has run countless marathons (Dublin 2013 will be his 100th!). Over 180 women ran or walked the Flora Dublin mini-marathon in 2011 and the Lumbers Tain trek organised by the Dundalk Branch are just some examples of the fundraising events supporting this initiative. A fundraiser for Life Matters 4 CF was also given by the renowned Irish folk group De Dannan in Ashbourne Co Meath during Christmas 2011, which proved to be a really special event for all involved.

The Life Matters 4 CF initiative is yet another example of how local Branches with a common objective can work together to help improve facilities for PWCF and their families at a local level. Open communication with the CFAI National Office and meetings with hospital management have been instrumental steps to improve services for PWCF in this region.

For more information on fundraising events being organised by LM4CF, or if you would like to find out more and/or get involved, please log on to the groups Facebook page: Life Matters 4 CF page 16

CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

ADULT & PAEDIATRIC CF CENTRES Cork University Hospital A new CF ambulatory day care unit in Cork University Hospital officially opened in 2011.

The unit comprises of five individual isolation rooms solely for CF patients, a dedicated gym, a multidisciplinary team room and consultation suites. Development of this unit will see a significant improvement in treatment facilities made available for PWCF from the Cork and Kerry area. The expansion also marks the first part of an ambitious plan to see the development of a 10 + 1 single en-suite, in-patient bedroom CF hospital ward.

The development of this new unit stemmed from a partnership between the HSE and local Munster independent CF charity, Build4Life, which raised more than €200,000 – members of the Kerry and the Southern Branch of CFAI were supportive of the fundraising effort.

Dr Barry Plant, programme lead for adult CF care at Cork University Hospital, commented that the new facility marks the start of an important phase in the hospital’s provision of care for CF patients. “CF patients are living longer into adult life – in the past, the majority of CF patients were children but now 56 per cent or so are adults. The majority of these are still young adults in their 20s and 30s, but we have patients in their 40s, some in their 50s, a few living into the 60s and we have one patient in their 70s, and it’s these patients living with CF who the ambulatory care centre will cater for,” he explained.

The National Office of the CFAI also provided €270,000 towards research and the employment of a Clinical Registrar in Cork University Hospital in recent years. The Southern Branch is playing a crucial role in supporting research in the hospital, providing €100,000 in 2011.

Mid-Western Regional Hospital, Limerick In January 2011, planning permission was granted for the new CF adult day care centre and in-patient unit at Mid-Western Regional Hospital (MWRH), Limerick. The funding for the new adult CF unit in Limerick has come from a range of sources including the JP McManus Pro Am tournament, TLC4CF and the National Office of the CFAI. It is a partnership project with the Parkinson’s Association and the Hospital Trust and is the largest capital project CFAI has been involved in.

The six-storey unit that will consist of two floors for CF patients shall provide a specialised CF out-patients unit with five treatment rooms and a dedicated in-patient unit with nine en-suite rooms. It is hoped building will commence in the first half of 2012.

The Tipperary, Limerick and Clare Branches of the CFAI (TLC4CF) have been instrumental in developing adult services for PWCF in the region. They have been the drivers of this project and demonstrate what can be achieved through a partnership approach.

Speaking at the press conference that announced details of the building works, 20-year old PWCF patient Katie Drennan from Ennis, Co. Clare said “The benefits of such a unit are many. It means we will not have to travel to other cystic fibrosis centres in Dublin to receive our treatment. We will not have to endure long hospital stays away from home. We will no longer fear the risk of cross-infection when we attend the hospital for treatments.

We all look forward to enjoying better facilities and a higher standard of care which will play a major role in improving the lives of so many people with Cystic Fibrosis.”

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CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

In November 2011, CFAI/TLC4CF met with the Regional Development Officer of the HSE to outline our concerns at the lack of a specialised CF physiotherapist for adults in the hospital and after a sustained period of lobbying, this gap was filled.

Katie Drennan, 20-year old PWCF, spoke at the press conference on the benefits of having the new unit.

-TLC4CF committee members (L-R) Liam O’Reilly, Caitríona Hayes, Linda Drennan, Marcella Clancy and Owen Kirby, have done tremendous work in developing services for PWCF in Limerick.

University Hospital Galway The Galway Branch of CFAI, led by Mary Lane Heneghan, remains very active in fundraising for improved facilities for CF patients and liaising with the hospital management. Key developments in recent years include fundraising for three en-suite isolation rooms and lung function equipment. The Galway Branch in partnership with the National Office has also made representations on the need for a CF full staff complement in Galway. In June 2011, Dr. Michael O’Mahony, Adult Consultant Respiratory Physician, joined the cystic fibrosis team at Galway University Hospital. Dr O’Mahony is the first physician appointment in the West of Ireland dedicated to the treatment of adults with CF. Dr O’Mahony was previously the attending Physician in the Department of Pulmonary and Critical Care Medicine Weill Cornell Medical College, New York.

Currently approximately 60 patients with CF from Galway, Mayo and Roscommon attend Galway and around half of these patients are adults.

“As CF has such a significant impact on PWCF and their families, it is important to continually strive to improve the services we provide and to support research initiatives. In this regard the CF Association of Ireland has been hugely supportive, providing funding for dedicated in-patient rooms, specialised equipment for CF patients and hiring of additional medical staff. Their support has also been instrumental in securing my appointment despite the very trying economic times we find ourselves in.”

The Cystic Fibrosis team at Galway University hospital welcoming Dr. Michael O’Mahony to his new post as Consultant Respiratory Physician.

Dr O’Mahony, Consultant Respiratory Physician, Galway University Hospital

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CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

Castlebar, Co Mayo Castlebar is one of the few CF centres north of the Galway/Dublin axis and provides important supports for CF patients in the greater Mayo area. The CFAI Mayo Branch and friends of CF have been active in fundraising for an ambulatory and day facility for all patients with CF (adults and children) so that they can access physiotherapy, dietetic and social work services in one dedicated area. It also provides a dedicated location where assessment of acute illness can take place and a meeting room will also be provided for parents and members of the CFAI. Mayo General Hospital

The new CF unit will be built on the grounds of Mayo General Hospital. This unit will have two rooms that will measure 25 square meters, have en-suite facilities and be large enough to have extra equipment placed in them for patient care. The ventilation system will be state of the art. Adjacent will be clinic rooms and a meeting room for the local branch.

The new development has been greatly welcomed by patients in the area.

Waterford Regional Hospital Following a meeting with the CFAI and members of the CF team in May 2010, a number of deficits in services made available to PWCF in this region were identified and the limitations in the current adult services at the hospital were recognised.

Staffing levels across multiple disciplines in adult CF care in Waterford Regional Hospital are far from adequate. For example, there is currently no designated Psychologist, Social Worker or dedicated Microbiology team member specific for CF care. Of vital need for the provision of adult care is the appointment of a Specialist Registrar. The lack of infrastructure (with particular reference to the absence of en-suite rooms and a dedicated day ward/drop-in ward) is also hindering the development of a safe and effective adult CF service. The CF team also highlighted the current deficiencies in paediatric CF services, particularly the lack of en-suite rooms and a dedicated examination room.

The key components identified for developing a limited adult service in the short-term are provision of a registrar and day ward/drop-in ward. Single en-suite rooms are needed both for adult and paediatric PWCF. The CFAI is committed to working closely with the CF team and hospital management in WRH to create more visibility to these issues and to develop a strategy to overcome the current limitations in services for PWCF in this region.

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CFAI Annual Report 2011 Section 02: Update on CF Centres in Ireland

Mater Hospital, Dublin Karen Redmond, the first dedicated lung transplant surgeon appointed in Ireland, commenced work in the Mater hospital in Dublin in September 2011. This is a position the CFAI has been advocating for over the past two years and is a very welcome development.

Karen is also a specialist in ex-vivo lung perfusion (EVLP), a new technique that allows time to accurately assess the lung and to optimise function of injured donor lungs so that they can be successfully transplanted into patients.

EVLP allows surgeons to “clean out’ otherwise unsuitable donor lungs by pumping a bloodless solution containing nutrients, steroids and antibiotics through them inside a protected dome-shaped chamber, outside the body.

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seCtion 03

Key policy developments in 2011

CFAI Annual Report 2011 Section 03: Key Policy Developments in 2011

Section 03:

Key Policy Developments in 2011

The most important policy development in 2011 was the introduction of newborn screening for CF, which has long been a goal of the CF Association.

The Introduction of Newborn Screening for CF in Ireland Newborn bloodspot screening for Cystic Fibrosis commenced in Ireland on 1st July 2011. All newborn children in Ireland are now tested for CF as part of the existing national newborn bloodspot screening programme. Newborn bloodspot screening is usually known as the ‘heel-prick test’.

CF is the sixth rare condition that is now screened for through this test. The full list of conditions now screened for includes: Pheylketonuria Congenital Hypothyroidism Maple Syrup Urine Disease Classical Galactosaemia Homosystinuria Cystic Fibrosis The Reasons for Adding CF to the NNSP Before the introduction of CF in the newborn screening programme, the average age of diagnosis for children who presented with typical symptoms of CF was about 11 months.

The introduction of screening will result in earlier diagnosis.

The benefits of early detection and management of CF have been supported by international research and, more recently, through research in Ireland. The addition of CF to the newborn screening programme means that the condition can be identified early and the baby can be referred to the appropriate experts and be treated promptly. Early detection of CF in other countries has also been proven to reduce hospital admissions and the need for invasive therapy.

There is also ample evidence which illustrates that individuals diagnosed through newborn screening have significantly improved nutrition, with knock-on benefits to vitamin levels, cognitive functioning, improved lung function, less antibiotic use and admissions, and ultimately improved survival.

Role of CFAI The CFAI has played an active role in campaigning for the addition of CF to newborn screening including advocating for its commencement in the CFAI Pollock Report (2005) and contributing to its implementation on the HSE steering group. It was also reflected as a priority in the HSE CF Services Page 22

CFAI Annual Report 2011 Section 03: Key Policy Developments in 2011

Report (2009), which largely endorsed the Pollock report. It is estimated that three babies a week will be diagnosed with CF or will be diagnosed as being a healthy carrier of the altered gene that causes CF. The CFAI website has been updated with a new section containing information leaflets on newborn screening, the sweat test and what it means to be a carrier of the CF gene.

The HSE also have a good section in their website on newborn screening: www.hse.ie/eng/services/healthpromotion/newbornscreening

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CFAI Annual Report 2011 Section 04: Support Services, Awareness and Fundraising

seCtion 04

support services, awareness and Fundraising

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CFAI Annual Report 2011 Section 04: Support Services, Awareness and Fundraising

Section 04:

Support Services, Awareness and Fundraising

Support Services CFAI endeavours to ensure that support services are delivered effectively to the broadest possible range of members and information for members is a key role for CFAI. In particular, there is a strong need for quality information upon diagnosis and through life’s journey. A key focus for CFAI in 2011/12 (supporting the introduction of newborn screening) will be to develop a new parent information pack for parents of newly diagnosed children with CF. The CFAI website will also be revamped to provide more accessible and up to date information for members.

Cystic Fibrosis is a complex multi-system condition that is progressive and requires lifelong care that increases with age. Much of the daily burden of care is delivered by the parents of a child with CF and later by the patient. We aim to ensure that patients, parents and carers have access to the information they need to understand the condition and its complications, as well as the level of care they should expect to receive. In this way all those affected by CF are empowered to make fully informed decisions about their lives.

Advice and support for our members remains a key part of services provided by the CFAI. This is undertaken in a number of ways including: Direct advice and support from staff in CFAI to parents and PWCF, in particular, with respect to interfacing with CF centres and in seeking financial support Providing grants for exercise, fertility, transplant, counselling, financial distress and bereavement Analysing common issues raised by parents and PWCF to determine how these should shape future policy development In short, if there are a number of complaints about a particular problem, the CFAI shapes a strategy to try and address these issues.

Awareness and Fundraising Fundraising continues to be the main source of income for CFAI and remains a very active and busy element of our operations at the National Office.

Complemented by a significant increase in local fundraising initiatives at Branch level, heightened awareness levels and public support for CFAI campaigns during 2010/11 were evident. It is noteworthy that we have followed our success in winning a PR award in 2008 by winning again in 2010.

With such a wide variety of events held throughout the year, it is impossible to list each one. It is, however, appropriate to recognise a selection to provide an indication of what has benefited us, and what is viable in the current economic climate. Gala parties, fancy dress and masked ball events continue to be valuable fundraisers. Sporting challenges right across the spectrum from running, cycling, swimming, skydiving, hill-walking and mountaineering, golf and motorsport are regular features of our fundraising calendar.

We are extremely grateful to our volunteers who work so hard for CF in organising these events.

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CFAI Annual Report 2011 Section 04: Support Services, Awareness and Fundraising

We now take the opportunity to recognise some of the most significant fundraising events that took place over the past year.

National Awareness Week coinciding with our Annual Conference grows in popularity and raises considerable funds throughout the country, with many ‘side-events’ organised.

1 in 1,000 for Women’s Mini-marathon was inaugurated in 2010. Led and managed by a group of ladies under the stewardship of Gillian Scanlan, daughters Natasha and Zoe Woodward, with Lou Soden, Carol McCarthy and others, this was the biggest single event in terms of income, awareness and participation. The success was repeated in 2011 by the full staff team at CF House, no mean feat when keeping all other services running smoothly also.

Participants in this year’s 1 in 1000 mini-marathon event in Dublin.

-Zoe Woodward helps Clare Kambamettu, Rose of Tralee 2011, prepare for the race.

IPPA Charity of theYear – we were selected for Happy Faces Day 2010, which generated significant income and awareness nationally. We were subsequently selected for 2011 with similar success.

Westpark Fitness, Tallaght, hosts an Indoor Triathlon annually. Alison Kane is the organiser and runs a very tight ship. Alison and her fiancé James got married on Saturday 17th September and we wish them both well in their future together.

Pictured at the launch of IPPA Happy Faces 2011: (L-R) RTÉ weather presenter Nuala Carey; Martin Cahill of CFAI; IPPA President Gareth Byrne; Jonothan Sultan of Canon Ireland; Tiarnán (9) and Saoirse Ahearne (7).

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CFAI Annual Report 2011 Section 04: Support Services, Awareness and Fundraising

Paddy Kerins Memorial Walk took place in San Francisco in 2010 and in October 2011 the group headed for Istanbul in Turkey with a total of 42 walkers. Well done to the Committee of Julia, Bernie and Frank, and of course the solid back-up of Eufemia Solinas, Fundraising Co-ordinator at CF House.

The Paul McGrath Golf Classic took place at Arklow Golf Club in July 2010. Paul, who has become a patron of CFAI, has attended a number of functions on our behalf and has been an excellent supporter of the cause of CF. This year, he also made an album which was released on 23rd September. The publicity surrounding his new venture bodes well for awareness of CF nationally, and a percentage of the proceeds of sales will also be donated to CFAI. Below is a further list of major fundraising events that took place in 2011: Celebrity Table Quiz CF Lugathon Ballymurphy Truck Run 15 to 1 Motorcycle Run Barry Kelly Pitch and Putt Challenge Racecourse Challenge (Antony Lewis-Crosby) Carnvale Ball Midnight Shakers Society Masquerade Ball St. Gerard’s Bray Gala Ball Cycling4CF Gavin Egan Memorial Mondello Track event GSK Charity of the Year 2011 Experian Charity of the Year 2011 Lakes 10k Please see our fundraising Calendar at www.cfireland.ie for details on upcoming events and also to check out our online shop.

Many thanks to our volunteers, supporters and donors for your continued commitment to the cause of Cystic Fibrosis.

The Board of Directors (of The Cystic Fibrosis Association) have signed up to the Statement of Guiding Principles for Fundraising. This is a voluntary code specific to the third sector which is, in essence, self-regulation in place of statutory regulation by government. CFAI are to the forefront among charities signing up to this code, thereby promoting good fundraising practices in the public interest of openness, transparency and accountability.

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CFAI Annual Report 2011 Section 04: Support Services, Awareness and Fundraising

Cathal Phelan and Conor Doyle cycled around the coastline of Ireland for Cycling4CF in 2011.

-Over 500 runners took part in the Lakes 10k event in September.

Seån Kavanagh, PWCF, from Bray Co Wicklow, gives a helping hand to chief climber Cian O’Brolchain at the launch of Ireland To Everest.

-Participants of the CF Lugathon in October 2011

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CFAI Annual Report 2011 Section 05: Report of CFAI Annual Conference 2011

seCtion 05

report of CFai annual Conference 2011

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An Taoiseach, Enda Kenny TD

CFAI Annual Report 2011 Section 05: Report of CFAI Annual Conference 2011

Section 05:

Report of CFAI Annual Conference 2011

The CFAI Annual Conference 2011 took place in Knockranny House Hotel, Westport, Co Mayo from 8th–10th April. The theme of this year’s conference was ‘CF – The Challenges for Families, Healthcare staff and a New Government.’

The conference was one of the Association’s most successful to date, with over 250 members, friends and delegates coming from all corners of the country to listen to presentations, attend workshops and meet old friends and be introduced to new members.

Former Chairperson, Mr Seán O’Kennedy spoke at the opening reception on the Friday evening followed by warm welcomes from Cllr Michael McLoughlin, Leas Cathaoirleach, Westport Town Council, Eamonn Abberton, Mayo Branch Chairperson and Dr Michael O’Neill, CF Consultant, Mayo General Hospital.

An Taoiseach, Enda Kenny, T.D., is welcomed by Cllr. Michael Burke, Cathaoirleach, Mayo County Council and Cllr. Michael McLaughlin, Leas Cathaoirleach, Westport Town Council at the opening night of the Conference.

-An Taoiseach, Enda Kenny, T.D., being presented with a 65 Roses badge by the Rose of Tralee 2011, Ms Clare Kambamettu and Mr Philip Watt, CEO, CFAI on the opening night.

Our special guest of the evening was An Taoiseach, Enda Kenny, T.D. who followed suit by officially launching our 65 Roses National Awareness week for 2011. In his opening address, Mr Kenny assured that his government was very aware of the challenges that people with CF and their families face in managing their condition. The work of the CFAI was also acknowledged for its commitment to improving services for people with CF in Ireland.

‘‘For 48 years, the Cystic Fibrosis Association of Ireland has actively campaigned in the interests of people with cystic fibrosis and their families, increasing knowledge and awareness of the condition. It is as a result of their campaigning that the majority of services provided around the country are in existence.”

Building work for the new unit at St Vincent’s University Hospital was also addressed

by Mr Kenny, whereby he reassured members that the new unit ‘is on track, on time, on budget and it is my intention to make sure it remains that way.’ He continued by saying that ‘the new Programme for Government has committed to expediting its completion and commissioning...and I am confident of delivering upon this. You and your families have waited far too long for this project to open. It can never be forgotten that cystic fibrosis patients are fighting for their lives…you don’t have time for broken promises”.

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CFAI Annual Report 2011 Section 05: Report of CFAI Annual Conference 2011

An Taoiseach, Enda Kenny, T.D., speaking at the conference opening.

“This Government is very aware of the challenges that people with CF and their families face in managing their condition.

I would like to acknowledge the wonderful work carried out by the Cystic Fibrosis Association of Ireland and I want to reiterate my commitment to doing everything possible to secure improvements in services for people with cystic fibrosis. The current economic environment makes this all the more difficult, but I will continue in my efforts to make progress as quickly as possible.”

The full speech by An Taoiseach, Mr. Enda Kenny, T.D can be found online at the following link: www.merrionstreet.ie/index. php/2011/04/speech-by-the-taoiseach/

Special Recognition Awards The Rose of Tralee 2011, Clare Kambamettu, also attended the opening reception on the evening of Friday 8th April. Together with An Taoiseach, she presented awards to several members that have made outstanding contributions to the CFAI over the years.

A Special Recognition Award was presented to Prof Charles Gallagher, Adult CF Consultant at St Vincent’s University Hospital in Dublin. Zoe Woodward also accepted an award on behalf of the organisers of the 1 in 1,000 Dublin Mini-Marathon fundraising initiative in 2010 that raised much needed funds for a new unit in Our Lady’s Children’s Hospital, Crumlin, which opened on 4th October. Tribute was also paid to the Paddy Kieran’s Memorial Walk committee members, an initiative commenced by Paddy Kierans whose untimely death occurred in December 2006. Julia Kierans accepted this award on behalf of the committee, whose work has raised thousands of Euros for cystic fibrosis over the years.

Friday evening ended with a drinks reception and music entertainment, allowing members the opportunity to catch up and socialise before the next day.

Back L-R: Peter Carey, Shane Halligan, Dr. Michael O’Neill, CF Consultant, Mayo General Hospital, Mr. Philip Watt, CEO, CFAI, Vincent O’Malley, Tomas Thompson, David Spillane, Mark Campion and Michael Ring TD, Minister of State, Dept. Transport, Tourism and Sport. Front L-R: Patricia Duffy Barbar, Ita Minogue, Caroline Heffernan, An Taoiseach, Enda Kenny T.D., Debbie McCarthy, and Katie Moore.

-Dr Ed McKone, CF Consultant at St Vincent’s University Hospital, Mary McCarroll, Eastern Branch of the CFAI, and Tomás Thompson, CF Patient Advocate, CFAI.

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CFAI Annual Report 2011 Section 05: Report of CFAI Annual Conference 2011

Overview of Presentations

Dr. Kate Russo, Queen’s University, Belfast with Pádraig and Lorraine Burns, Mayo Branch.

A wide variety of presentations and workshops took place on Saturday 9th in line with the theme of this year’s conference, ‘CF – the Challenges for Families, Healthcare staff and a New Government.’

Morning Session Mr Seán O’Kennedy, former Chairperson of the CFAI, welcomed participants bright and early on the Saturday morning, while Dr Mary Herzig, CF Consultant, Galway University Hospital chaired the first session.

Members who could not attend the conference joined in via videolink. Professor John Moore, City Hospital, Belfast, kicked off proceedings with his presentation on ‘The Good, The Bad and The Ugly – What do CF Bugs mean for me?’

Lorraine O’Neill, Raymond Dunne, Rosaleen Cronin, Karen Keogan, Cavan Branch.

Eileen Lynch, Claire Toal and Maureen McKeown, Dundalk Branch.

Philip Watt, CEO of CFAI then addressed some of the challenges facing the new government in the context of the current economic climate. He provided examples. One Hospital was without a physiotherapist for 3 months with the result that many children with CF did not see a physiotherapist for this period. Another hospital has lung function equipment that cannot be used because they don’t have the resources to employ a technician.

Another hospital has a CF adult consultant who cannot see adults with CF as he does not have a proper consulting room.

Prof John Moore, ‘The Good, The Bad and The Ugly – What do CF Bugs mean for me?’

He further contended that new treatments for CF are becoming available – will these be funded by the Government?

Professor Jim Egan, who has recently been appointed as the new lead for the National Office for Organ Donation and Transplantation, spoke about lung transplantation in Ireland in 2011. Of the 32 lung transplants that have taken place in the Mater hospital to date, 5 have been for people with cystic fibrosis. Prof Egan also pointed out that the number of patients awaiting lung transplantation in Ireland has

Prof Jim Egan speaking on the subject of lung transplantation in Ireland in 2011, on Saturday.

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been increasing steadily since 2004. He also discussed rates of organ utilisation and legislation surrounding consent (opt in/opt out/mandatory).

Dr Kate Russo, Chartered Clinical Psychologist, Queen’s University Belfast finished the opening session with a discussion on the impact that living with CF can have on families, and how stress can influence your thoughts, emotions, physiological feelings and behaviours. She also suggested ways in which one can better manage these feelings by using certain coping strategies.

Following a short tea break, the parallel workshops began. This allowed people the opportunity to choose a themed seminar that most suited their individual interests and needs: Paediatric Services, including Newborn Screening; Adult CF services, including US Perspective; and CF and Mental Health.

1. Paediatric Services, including Newborn Screening Speaker: Dr Mary Herzig, CF Consultant Galway University Hospital Chair: John Coleman This was a very popular session with approx 30 conference attendees present. Dr. Herzig provided a very informative presentation, which detailed the development of the inclusion of CF testing in newborn screening internationally; the actual test itself and the screening process; the status of paediatric CF testing in Ireland at that time and the inclusion of the CF test in the newborn screening (through the “heel prick” test).

Dr Herzig set out the key benefits and advantages of including the CF test in the newborn screening process: It saves money Less invasive for children The test was being done anyways, so why not include it?

She listed the key barrier (in Ireland) as being the HSE re-organisation and current financial restraints. However, the set-up or initial cost is approx €1m; with a recurring or annual cost after that of approx €400,000 – in relative terms this is quite low.

After the presentation, there was a very engaging question and answer session and discussion, in which several parents spoke of their experiences. The key points arising for this session were as follows: As the CF test is rolled out in the newborn screening programme, cascade screening on direct siblings and then wider family will follow as CF cases are identified. However, the genetic counselling provision in Ireland at the moment is inadequate, and there needs to be greater access to such counselling for families of newly diagnosed PWCF’s.

Parents should have access to the genetic information collected through testing – i.e., knowing if they have children who are carrying CF gene. During the discussion it became clear that some parents had been told that they could not have that information, whilst others were told the results. This was reported to have happened even within the same hospital.

Arising from this discussion, another key point that came from this session was that pre-natal screening is something that should be investigated and debated further in

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an evolving Ireland. Religious imperatives are becoming less and less significant for many people. For some parents and potential parents this is a choice they should have access to, but such tests are not available in Ireland. They are available in other European countries, but at quite a significant cost.

There needs to be universal protocols in place across all CF centres to ensure equal standards of care.

2. Adult CF Services, including US Perspective Chair: Caitríona Hayes, Tipperary Branch, CFAI Speaker: Dr Michael O’Mahony, CF Consultant, Galway University Hospital Dr O’Mahony started the workshop by providing some background history on CF. In 1938, Dorothy Andersen, was the first person to provide a clear detailed clinical and pathological description on cystic fibrosis. During a heat wave in New York City in 1953, Paul di Sant’Agnese, M.D. and others connected the extra loss of salt by people with CF to the disease’s underlying cellular problem. Dr Carolyn Denning was the first woman to chair the National Cystic Fibrosis Foundation’s Medical Advisory Council and was one of the first to organise and initiate a multidisciplinary team approach to management of the disease.

Born in the 1950s, the CF Foundation has grown into a 600-employee, 250,000-volunteer, and $250 million per year enterprise that supports basic science, drug discovery, drug development, clinical care, patient education and advocacy. The Foundation funds and accredits more than 110 cystic fibrosis care centres nationwide. Each centre undergoes thorough review by the CF Foundation’s Centre Committee before it receives accreditation and funding. This review for accreditation occurs every year.

Dr O’Mahony then discussed how in 2007 the CF population in Ireland changed and the adult segment became greater in proportion than the children’s group. This trend will continue into the future so we can now describe the CF population in Ireland as being predominantly adult in nature. Higher intensity of services may be needed due to the complexity of medical, social and psychological issues, and there is a consensus that multidisciplinary teams with training and experience in adult CF care should oversee the care of adults with CF. Adult-specific issues were addressed including, transition from paediatric to adult CF care centres, family planning, fertility treatments and pregnancy care, bone and joint disease, education and training after secondary school and career planning. Some of the challenges facing adolescents and young adults with CF were also highlighted, for example, delayed physical growth, dependence on care givers and development of relationships. CF patients face few absolute restrictions on choice of career and Dr O’Mahony pointed out that education and career choices should be based on an individual’s intellect, ability, interests and life goals.

Finally, members were encouraged to engage with other CF families and health care providers through mechanisms like the CFAI to continually improve care.

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3. CF and Mental Health Chair: Pádraig Burns, Mayo Branch, CFAI Speakers: Dr. Kate Russo, Psychologist, Royal Belfast Hospital for Sick Children, Caroline Heffernan, Patient Advocate (PWCF), CFAI This workshop was mainly attended by parents of PWCF and complimented Dr. Russo’s earlier morning presentation on “Impact of Living with CF on Families and Coping Strategies”. Dr. Russo commenced the workshop by encouraging a very interactive and open session.

One parent noted that her child had always been very positive around CF but had recently become more negative and unenthusiastic around her daily treatments and CF care. Dr. Russo acknowledged this process by saying PWCF will go through different stages and experiences with their care and treatments as they grow and develop. They experience various different stages and expectations, stages of trust and transition and often it is finding their way through this, negotiating between transition, parents and their CF. Understandably, it is difficult for parents to let go but it is important to be aware of this and learn how to manage it. Dr. Russo emphasised the importance of positive praise and reinforcement with the PWCF. Dr. Russo discussed the role of “choices” for the PWCF – sometimes the PWCF has to learn for themselves, they will see the consequences of their behaviour. For example, if a PWCF stops taking their medication, he/she will experience the physical effects of that and probably won’t do it again. It is ok to be angry and frustrated, it is difficult and sometimes it is harder to do medications when you are feeling well and are a healthier PWCF. Try to manage the stress and anxiety by moving the negative experience of taking medication to a positive; for example, if I take my medications and keep well, I can go travel and do the things I want to do.

Another parent asked if it is appropriate to raise/discuss the seriousness of CF with her 12 year old daughter? Dr. Russo emphasised the importance of being honest and open, as at certain ages your children know about certain things and probably know a lot more than you think they do. It’s about finding the right balance, for example, certain issues such as fertility issues are normally managed through the CF team but it’s ok to talk about that too if you feel you and your child/PWCF are ready to do so.

Caroline noted that PWCF will experience normal teenage anger along with their CF and its finding out what makes them angry and supporting them with that. The concern for parents is are they doing it right or wrong, but there is no “right way”. It is about focusing on the here and now, keeping extremely positive and learning coping techniques to overcome obstacles that will come your way.

Another parent discussed his and his partner’s personal experience with CF since it entered their lives 12 years ago when their daughter was diagnosed. They acknowledged that there is so much negativity thrown at them, and that that they have to try and be positive. There are everyday treatments that need to be done and you can’t take a day off. He gave the analogy of the “Myth of Sisyphus” figure of Greek mythology who was condemned to repeat forever the same meaningless task of pushing a boulder up a mountain, only to see it roll down again and Sisyphus has to start over. He concluded

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CFAI Annual Report 2011 Section 05: Report of CFAI Annual Conference 2011

saying CF is always there, an ever presence in your life and it is about managing that as parents; the practicalities, the grieving process, the sadness and the challenges.

Dr. Russo reiterated that there is no right or wrong way to look after your child, every way you look after them is right. Life is good, life is full, and life is fun. Having great memories, that’s what matters. “Realise that this very body with its aches and it’s pleasures… is exactly what we need to be fully human, fully awake, fully alive” – Pema Chodron (quotation take from Dr. Russo’s presentation).

Afternoon Session The afternoon session of the conference was chaired by Tomás Thompson, CFAI Patient Advocate, and members of the PWCF group first took to the stage with a presentation on ‘PWCF Communication, Past, Present & Future’. Caroline Heffernan, CFAI Patient Advocate, discussed the physical and mental impact of having CF, and how the issue of cross-infection has hampered physical contact and communication between PWCF. Maria Delaney (PWCF) also joined this session via videolink. Modern technology has a big role to play in overcoming the isolation many PWCF may feel. The CFAI Forum, PWCF Adult Group and social networking sites were mentioned as supports to overcome such isolation.

Dr Ed McKone, CF Consultant, St Vincent’s University Hospital, subsequently provided an overview of genetics and CF. Over 60% of Irish CF patients have two copies of the F508del mutation, which causes severe or “classic” CF. In Ireland, seven mutations account for over 80% of CF, with the G551D mutation being the second most common, found in 11% of Irish patients. Dr McKone also mentioned how different CFTR mutations can be grouped together according to function. The implications of CF mutations on prognosis, treatment, and new therapies that target the CF gene were also discussed – the most promising is VX770 which is effective in G551D and has important implications for Irish CF patients.

For those of us who were hungry enough for more information and discussion, the afternoon workshops offered members a choice from the following: Microbiology of the CF Lung; Focus on Patient Interaction/Fundraising with CF centres; and A Profile of CF in Ireland and Genetics.

1. Microbiology of the CF Lung Chair: Helen Whitty, CFAI Speaker: Professor John Moore, City Hospital, Belfast In preparation for the above session, Prof Moore asked members to list the five most important aspects of microbiology/infection for them.

This was a very interactive session, with parents asking specific questions in relation to their child’s infections. Prof Moore articulated that the aspiration of the microbiologist is eradication of infection. Good laboratory methods are required to identify and treat infections. Chronic infections generally involve microorganisms that are more difficult to eradicate.

One member questioned what the long-term effects of MRSA/Pseudomonas infection on the lungs were – the answer: tissue damage and a decrease in FEV1 levels if chronic infection continued.

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Members at the seminar also wanted to know how often PWCF should have their sputum tested. Prof Moore recommended that testing should take place at every exacerbation, at annual review and in times when an individual is feeling particularly unwell or if new symptoms develop that hadn’t been present before. People were also anxious to find out why it took so long to get results back from sputum samples. Prof Moore pointed out that testing sputum for different bugs requires specialised pieces of diagnostic equipment and media, which may not necessarily be available in every hospital, so oftentimes the sample must be sent to the National Reference Laboratory in Tallaght or elsewhere. In addition, the amount of time is takes to culture or grow the bugs may differ substantially.

For example, Pseudomonas can grow overnight, but Mycobacteria will take up to 6 weeks to grow. Time is therefore a factor. Efficacy would also determine how each centre manages the testing process; communication, management and availability of resources also comes into play.

One parent also asked what happens if a child was too young to get a good sputum sample to which Prof Moore responded that a throat swab would be better than nothing at all. Induced sputum or bronchial lavage would only be considered if there were serious concerns (e.g., big drop in FEV1 levels) – the clinician wouldn’t put the child under stress unnecessarily.

The prevalence of MRSA in the home was also discussed. A lot is known about MRSA in the hospital setting, but very little is known about the dynamics of MRSA in the home environment. MRSA doesn’t exist on its’ own; it must exist in a biological matrix.

2. Focus on Patient Interaction/Fundraising with CF centres Chair: Philip Watt, CEO, CFAI Speakers: Mary Lane Heneghan, Galway Branch, CFAI & Marcella Clancy, Clare Branch, CFAI Mary Lane Heneghan and Marcella Clancy provided overviews of the developments in Galway and Limerick in recent years focussing on the need for fundraising to go hand in hand with interacting with the management of local hospitals. In Galway, the Branch had focussed on fundraising for three isolation rooms and lung function equipment. The Branch office has met with staff on a regular basis and developed a strong basis of trust, even though exchanges can at times be robust.

Marcella outlined the genesis of TLC4CF and how this had facilitated the Tipperary, Limerick and Clare Branches to come together. This had resulted in the hospital taking CF services more seriously than before. The focus of the work of TLC4CF is presently on a new two floor adult CF unit which will cost over €4m.

Both presentations highlighted: The focus on a hospital and identification of key fundraising objectives is an effective way of mobilising Branches.

The importance of Branches working in partnership with the National Office and within the structures of the CFAI was a real benefit in ‘getting things done’.

Fundraising alone will not solve all the issues. There is a need for strong co-operation between the CFAI, healthcare staff and hospital management to resolve issues.

The cutbacks in health were having a serious impact on front line services.

The participation of people with CF is very important to the process – it is their voice which will be picked up by the media.

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CFAI Annual Report 2011 Section 05: Report of CFAI Annual Conference 2011

3. A Profile of CF in Ireland and Genetics Chair: Dr Ed McKone, CF Consultant, St Vincent’s University Hospital Speakers: Dr Abaigeal Jackson, Research Fellow, Cystic Fibrosis Registry of Ireland & Dr Ed McKone Dr Jackson provided an overview of the CFRI to workshop attendees. Established in 2002, the CFRI is an independent organisation that is located in UCD Belfield and funded by the HSE. The CFRI operate a web-based registry with advanced encryption technology that is compliant with the Research Ethics & Data Protection Act (patient-informed consent is required to allow CFRI collect data and patient data remains confidential with advanced encryption technology.

So what is the role of the CFRI? It provides information, carries out research, publishes reports, and assists in planning and management of CF health services. In 2009, 89.2% of PWCF had enrolled with the registry. By enrolling, people with CF are helping us to better understand CF in Ireland, enable us to compare treatment and management, help us to identify areas for improvement in CF care and improve service planning.

Dr Jackson also highlighted that in 2009 the percentage of paediatrics (<18) with CF in Ireland was 48.3% compared with 51.7% adults. CF survival in the Republic of Ireland has improved. Dr Jackson wrapped up the workshop by concluding that differences in US and ROI survival estimates may have existed, though pointed out that gap has now narrowed. She also noted that further research using registry data is needed to examine health care service use in Ireland and compare CF health service delivery internationally.

The plenary session finally wrapped up proceedings for the Saturday whereby rapporteurs presented five key points from each of the parallel seminars to the rest of the conference participants.

After a few hours of rest and relaxation, attendees congregated for a drinks reception before the gala dinner dance. A raffle was held and prizes included a laptop, camera, Raddisson hotel voucher and cash. A great night was had by all, and thanks were, and continue to be, extended to the Mayo Branch for their support in organising this yearâ&#x20AC;&#x2122;s conference!

Note: Webcasts from the conference are available for viewing on the CFAI website at the following link: www.cfireland.ie/articles.php/articles.php/annual_conference2011/_ conference_clips

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annex:

National Executive Committee (CFAI Board)

John Coleman

David Fitzgerald

Patricia Duffy Barber Brendan Lonergan

Mary Lane Heneghan

Cyril Gillen

Louise Harty

Esther L’Estrange

Seán O’Kennedy Vice Chairperson

Karen Dowling Prendergast

Kenneth Flanagan

Ian Duffy

Tess Brady

Catríona Hayes

Mary McCarroll

Isabella Higgins

Carmel Delaney

Ger Menihane

Chairperson

Drogheda/Vice Chairperson

Treasurer

Secretary

National Executive Council members not present in above photographs: Tracy O’Kennedy, Denise Taylor, Chistine Drummond and Prof. Charles Gallagher.

(Observers: Fiona Bodels and Dr Barry Plant)

annex:

CFAI Staff 2011 Philip Watt

Chief Executive Officer

Helen Whitty

Operations Manager

Martin Cahill

Fundraising Manager

Eufemia Solinas

Fundraising Co-ordinator

Ciara Enright

Fundraising Co-ordinator (1 in 1000)

From December 2011

Maria Caldwell

1 in 1000 Co-ordinator

From Feb – Oct 2011

Kathy Baker

1 in 1000 Assistant

From Feb – Jul 2011

Agata Adamaszek

Finance Officer

On maternity leave

Jolyn Mulvey

Finance Officer

Maternity leave Cover

Alica May

Services and Information Officer

Tomás Thompson

Advocacy Officer (part time)

Caroline Heffernan

Advocacy Officer (part time)

Suzanne Brock

Office Administrator

Catherine Cronogue

Intern (voluntary)

About the Cystic Fibrosis Association of Ireland (CFAI) The CFAI is a registered charity CHY 6350 that was set up by parents in 1963 to improve the treatment and facilities for people with CF in Ireland. It is a national organisation with many Branches around the country.

The CFAI is committed to working to improve CF services in Ireland and our recent progress includes: Lobbying to ensure that the new national adult CF centre in St Vincentâ&#x20AC;&#x2122;s University Hospital will be completed Providing funding towards new CF Units around the country including Crumlin, Drogheda, Galway, Mayo and Limerick Hospitals Funding research Campaigning to improve the rate of double lung transplantation in Ireland Providing advice and expertise

Cystic Fibrosis Association of Ireland CF House 24 Lower Rathmines Road Dublin 6 Ireland

t: +353 1 496 2433 f: +353 1 496 2201 e: info@cfireland.ie w: www.cfireland.ie Charity: CHY6350 Company Reg: 449954