Sept/Oct 2012

1

SPECTRUM Published by the Cystic FibrosisAssociation of Ireland

IN THIS ISSUE:

Issue 29: Sept/Oct 2012www.cfireland.ie

•Progress and Challenges in CF Centres•Kalydeco/Ivacaftor Update•New CFAI App Launched•A Neutron Walks Into a Bar...•Seasonal Flu Vaccine – Reminder• Launch of ‘Dinners to Make...Things to Bake’

Spectrum / Issue 29

2

Dear All,

Government must honour promise on free GP care for those with long-term illnessNotwithstanding the recent row and resignation of the Minister of State at the Department of Health, the Government has promised to extend access to GP services without fees to persons with those illnesses are prescribed in the Long-Term Illness Scheme, which includes cystic fibrosis. When this promise was made they indicated that primarylegislation would have to be brought in to effect this change.

The CFAI calls on the government to honour this commitment without further delay.

Domiciliary Care Allowance Many parents will have received letters on the government review of the vital Domiciliary CareAllowance. As cystic fibrosis is a genetically inherited, life-long disease and isprogressive by nature, and there are considerable additional costs for families arising from the care of PWCF. We have contended in a detailed submission, summarised in this issue of Spectrum, that the Domiciliary Care Allowance should be automatically granted to our members with no further assessments or follow-up reviews. We will be monitoring the work of the review committee closely to seek to ensure that this review is not aimed at providing rationale for cutbacks in this crucial allowance to our members.

CFAI Information Metings on New CF Therapies, including Vertex drugs The CFAI will shortly be announcing three information meetings around the country on new CF therapies and medicines. In the meantime, there is an update on Kalydeco/Ivacaftor in this issue of Spectrum.

CFAI at 50 – Events in 2013The CFAI was formed on a bleak November day in a small lecture theatre in Crumlin Children’s Hospital in 1963. The parents and doctors who were present at that ground-breaking meeting and the many people who have sustained the work of the CFAI in the intervening years can be proud of their work. To mark our 50th birthday and as a way of saying thanks to our members, CF Clinicians and staff and our supporters, we will be bringing out a book looking back over half a century, and we will be announcing a special event during 2013…watch out for more details.

Kind Regards,

Philip Watt (CEO) / Alica May (Editor)

Cover Photo of Kelli Maples (see PWCF Spotlight, page 20) courtesy of Tom Coakley Photography / www.tomcoakleyphotography.ie.

DISCLAIMER: The views of contributors, when expressed in this publication, do not necessarily reflect the

position or policy of the Cystic Fibrosis Associaiton of Ireland.

Sept/Oct 2012

1

Section 01:

LATEST NEWS : Page 2–10Progress and Challenges in CF CentresCFaces CampaignProgress on Kalydeco/IvacaftorNew CFAI App LaunchedA Neutron Walks Into a Bar... National Children of Courage Awards 2012OAK: Online Arts DocK for Teenagers with CFSeasonal Flu Vaccine – Reminder!Domiciliary Care Allowance Review – CFAI Submission- -

Section 02:

RESEARCH NEWS : Page 11–12Single high-dose oral vitamin D3 (stoss) therapy – A solution to vitamin D deficiency in children with cystic fibrosis?Successful treatment of cepacia syndrome with a combination of intravenous cyclosporin, antibiotics and oral corticosteroidsRisk factors for age at initial Pseudomonas acquisition in the cystic fibrosis epic observational cohort- -

Section 03:

ENTITLEMENTS : Page 13Disability Allowance – Your Questions Answered- -

Section 04:

PWCF SPOTLIGHT : Page 14–22I’ve lived a quality life through Sport and Exercise, By Paul MurphyThe Pursuit of a Dream, By Robert CookBeing a PWCF, By Kelli Tara Maples- -

Section 05:

FUNDRAISING : Page 23–3365 Roses National Awareness Week 2013Best Will in the World WeekChallenge and Community EventsLaunch of ‘Dinners to Make...Things to Bake’Mobile Phone Recycling for CFSoroptImist International IrelandThank You and much more inside...

- -

Spectrum / Issue 29

2

PROGRESS AND CHALLENGES IN CF CENTRES

News from Around the Country

St Vincent’s Update Since OpeningPhilip Watt, CEO of CFAI met with Professor Gallagher and visited the new CF Unit in Vincent’s shortly after opening and continues to liaise on a regular basis on progress in SVUH. Up to 34 patients can be accommodated in the Nutley Wing when required and this is copper-fastened in an agreement between the hospital, CFAI and the clinicians, which has been countersigned by the Minister for Helath, James Reilly, T.D.

The number of in-patients in the new wing has fluctuated between 23 and 28 since the unit has opened. This demonstrates that CFAI, clinicians and advocates were right to insist on 34 rooms being made available.

As might have been expected in a new unit, there have been a few glitches in some aspects which we hope will be ironed out as things progress, but overall the general sense from those who have been in the new unit has been very positive. We thank the team in SVUH for all their work in transferring patients over from the old St Christopher’s ward, in particular thanks to the CF specialist nurses in SVUH.

The protocol agreed between CFAI and the hospital will be closely monitored and we would ask patients in the Nutley Wing to continue to help the CFAI in this process; please give Helen Whitty a call to discuss at any point.

The CF services in the new wing comprise:1. One whole floor for CF Day Care services and offices/meeting room for the multi-disciplinary team and for family consultations. This floor includes 10 day care rooms.2. One Whole floor for in-patient beds with 20 rooms 3. The remaining 14 rooms are located on other floors of the Nutley Wing with special consideration given to the location of PWCF with Cepacia in line with good practice on infection control. BeaumontThe in-patient needs assessment was completed during the summer and hospital management endorsed the report that outlined the necessity for 13 in-patient beds for the hospital. A fundraising campaign is to be drawn up in partnership between CFAI and CF Hopesource. We estimate that we will need about €2.5 million to complete the project.

Drogheda Tenders for the new CF day care centre have just been issued. With the support from the Minister for Helath, James Reilly, T.D., €100,000 was secured from National Lottery by the CFAI National Office to further support this project and to add to local fundraising. We hope to start building just after Christmas. PS, many, many congratulations to Loretta Byrne of LM4CF on the birth of baby Caragh, born on 28 August weighing in at 6.13lbs.

LATEST NEWS SECTION 01 PAGE 2—10

Sept/Oct 2012

3

MWRH LimerickThere is a planned meeting with hospital management/TLC4CF/CFAI to further consider continued under-resourcing of Physiotherapy and Dietitian posts. The new TLC4CF/CFAI office has been opened now 7 months and is progressing very well, many thanks to Katie Murphy. The office officially opened in August with a very enjoyable fundraising barbeque.

CorkWith support from Southern Branch and the National Office, funding for major research projects is ongoing, including projects undertaken by Dr Barry Plant in CUH and Dr Eileen Savage in UCC.

MayoFundraising towards the €1m for the CF day care facility in Mayo General Hospital continues.

WaterfordA meeting took place with the hospital management in July regarding the development of three paediatric CF in-patient rooms. Progress includes plans drawn up. The estimated cost of this project is around €300,000 with the hospital seeking €250,000 support from CFAI.

A fundraising strategy with local branches and supporters was recently co-ordinated by Fundraising Manager Martin Cahill. A total of €40,000 has been raised already.

Cavan A needs assessment is currently being undertaken in partnership with the Cavan Branch. There are currently 25 paediatric patients attending Cavan Hospital. The needs assessment report will consider if Cavan should be formally recognised as a shared care centre.

Temple Street Children’s HospitalCFAI is making urgent submissions on the lack of replacement of a physiotherapist in Temple St Children’s Hospital.

CFACES CAMPAIGN

Raising Awareness of Cystic Fibrosis through Social Media

Share a story as part of the ‘CFaces Campaign’ which aims to promote people with Cystic Fibrosis and their families, building an online community of support and friendship. For more information email Ciara at cenright@cfireland.ie or to take part simply include image and story.

Saoirse Brophy, Age 4. Alex O’Herlihy Lilly Mae, age 2 and a half.

Spectrum / Issue 29

4

PROGRESS ON KALyDECO/IVACAFTOR

Latest News on Vertex Drug

What’s in a name?Confusingly there have been three names used for the same drug. These are Kalydeco, Ivacaftor or VX770. All these names relate to the same product.

When will Kalydeco/Ivacaftor become available in Ireland?We hope to know before the end of December 2012 if the Government is going to reimburse the pharmaceutical company, Vertex, for the costs of Kalydeco/Ivacaftor. The CFAI have written to the regulatory authorities in Ireland highlighting the importance of this drug to our members and asking the government to fast-track the assessment of this drug.

So what are the next steps?From a reimbursement perspective we understand that Vertex, the company that produces Kalydeco/Ivacaftor, have just submitted final documentation last week to the National Centre for Pharmacoeconomics (NCPE). Kalydeco/Ivacaftor will then go through a Health Technology Assessment (HTA).

This may take up to three months to complete. In the meantime we understand that anyone who needs Kalydeco/Ivacaftor will continue to be prescribed this drug.

The National Centre for Pharmacoeconomics reviews the cost-effectiveness and budget impact of individual drugs in the Irish healthcare setting in response to requests from the HSE Corporate Pharmaceutical Unit (HSE-CPU).

Under an agreement with the pharmaceutical companies, according to the NCPE website, the ‘Health Services Executive reserves the right to assess new and existing technologies that may be high cost or have a significant budget impact’. All medicines will be subjected to a preliminary rapid review. This was completed by the NCPE in relation to Kalydeco/Ivacaftor and a HTA was recommended.

High cost products and those with significant budget impact will be subjected to formal pharmacoeconomic assessment, in accordance with existing Irish (HTA). Similarly, products where there is a query in relation to value for money will also be selected for formal pharmacoeconomic assessment.

Who is Kalydeco suitable for?● Kalydeco/Ivacaftor is indicated for the treatment of cystic fibrosis in people ages 6 years and older who have at least one copy of the G551D mutation in the CFTR gene.

● If a patient’s genotype is unknown, an accurate and validated genotyping method should be performed to confirm the presence of the G551D mutation in at least one allele of the CFTR gene before treatment with Kalydeco/Ivacaftor is initiated.

● In the Phase 3 clinical trials evaluating Kalydeco/Ivacaftor, adolescents and adults (age > 12 years) who enrolled in the trial had a predicted FEV1 (forced expiratory volume in one second) of 40% to 90% and children (age 6-11 years) had a predicted FEV1 of 40% to 105%.

Sept/Oct 2012

5

NEW CFAI APP LAUNCHED

Now Available on iTunes App Store

We are delighted to inform members that our new CFAI App went live a few weeks ago and is now available for download on the iTunes App store. The App was developed as a gesture of goodwill by a company called Kumquat and we would like to take this opportunity to thank them for developing this product for CFAI.

About the AppThe App will allow us to send you instant notifications about latest news updates, it will mean that we can let you know when grant schemes go live (e.g., for the exercise and fertility grants), and keep you up to date about major fundraising events taking place around the country.

We aim to bring you good news stories on building developments and bring more visibility to our younger CF members throught the CFaces campaign. We welcome members to rate the App and to leave comments on the iTunes page or email us with your feedback!

Requirements: Compatible with iPhone, iPod touch, and iPad. Requires iOS 5.1 or later. The App is not yet available for Android Operating Syemtems, but this is something we hope to look at developing in future. To download, all you need to do is search for ‘CFAI’ on the iTunes App Store. The App is free to download. To read more about this App, please click here: http://itunes.apple.com/us/app/cfai/id558551602?ls=1&mt=8 About KumquatKumquat is a software development project characterized by a high degree of flexibility. Available types of mobile applications that currently are and will be generated by Kumquat in the nearest future can satisfy needs of broad range of customers. Potential clients vary from individual/private users (like blog owners), and businesses who want to present their newsfeed or offer via mobile app to clients requiring more sophisticated functionalities such as higher level of application-user interaction (e.g. making bookings/orders) and e-commerce. All applications provided by Kumquat are characterized by high quality features (speed, unbeatable performance and high aesthetical standards) and very low maintenance costs. Market size is at present unlimited as smartphones became popular only few years ago and providing a mobile app for a running business is becoming a standard right now.

Please visit ther website www.kumquat.ie for more information or to get a quote.

Spectrum / Issue 29

6

A NEUTRON WALKS INTO A BAR...

Random Facts About Our Universe and Everything in It

Fun, quirky and informative, A Neutron Walks Into A Bar... is a collection of facts, definitions, explanations, biographies and jokes guaranteed to quench a thirst for knowledge, discovery and humour that’s out of the ordinary.

Did you know that water is the only thing found naturally on earth in each of the 3 states of matter?

This book began its life as Science140, an exciting social media project co-ordinated by four Irish science communicators and enthusiasts including Aoibhinn Ní Shúilleabháin presenter of RTE’s The Science Squad, Maria Delaney (PWCF), Humphrey Jones and Paul O’Dwyer.

Since its launch on Twitter in the spring of 2012, thousands of tweets have been collected, each explaining a scientific principle, fact, joke or biography in 140 characters or less. The tweets collected are on a range of topics from astronomy to zoology, and have been compiled by science enthusiasts, educators, members of the public and celebrities from all over the world.

CFAI was chosen as the charity partner for the Science140 project, and all proceeds of the book will be targeted towards cystic fibrosis research projects! A total of four research projects are currently being funded by CFAI in conjunction with the Health Research Board.

The book will available in shops across Ireland on 18th October, so please support this great cause!

Alternatively you can buy the book on Amazon by going to the following link: www.amazon.co.uk/Neutron-Bar-Random-Universe-Everything/dp/1444743732

NATIONAL CHILDREN OF COURAGE AWARDS 2012

Deadline is 16th November

Once again the Share A Dream Foundation will host the National Children of Courage Award Ceremony where eight children will be selected for their bravery and courage.

If you think you know a child with a life threatening illness, disability or chronic illness who have shown tremendous courage and bravery despite their suffering, then why not nominate them.

All 8 Award winners and their parents will be special guests at the Dream Ball & Awards on Sat the 8th December hosted by Miriam O’ Callaghan and Will Leahy. They will stay in the luxurious

‘A Neutron Walks into a Bar’ is now available on Amazon and will soon be selling in shops nationwide.

Sept/Oct 2012

7

Radisson Blu hotel and that evening receive their special award and a magical dream come true on the night.

The closing date is Friday the 16th of November.

For more information contact the Share A Dream Foundation t: 061 2000 80 e: dreams@shareadream.ie w: www.shareadream.ie

OAK: ONLINE ARTS DOCK

New Creative Website for Teenagers Living with Health Conditions

OAK, the Online Arts DocK, a private web-space where teenagers with health conditions can interact, share stories and artwork, and develop online arts projects in a safe and secure environment, was launched on September 24th 2012. OAK has been developed by Helium, the arts and health organisation for children and young people, and is designed by web developer and artist Clare Kelly.

The name OAK was inspired by a young creative writer with Cystic Fibrosis who came up with the title ‘Iridescent Oak’ because ‘we’ve come from one seed but go on to become individuals with different personalities – like a branch with leaves. I felt that this was what the site wanted to achieve – a place where we can show off our “leaves”!’

Members of OAK can start their own blogs; share stories, poems and other creative writing on the Files page; create galleries for their artwork and photos; and post topics and chat to fellow OAKers on the forum. Only members of OAK can view content on the website or chat on the forum and the site will be moderated by Helium to ensure that the space is enjoyed by those it is intended for.

OAK is also a space where you can learn how to develop creative projects online. Helium will be running a film and animation week from October 30th – November 3rd 2012 as part of the Two Suitcases Project and OAK members will have the opportunity to participate online and learn skills for developing future online projects. All the information will be posted on the Admin’s Blog and dates and times when you can participate will be posted in the Calendar.

If you would like to join OAK, you can register at www.onlineartsdock.com. Helium will send you a verification email to confirm where you heard about the website and that you are a teenager living with a health condition. You can also invite your friends to join by giving them your OAK profile name for verification.

For more information on OAK email Emma Eager communications@helium.ie. You can learn more about Helium and the Two Suitcases Project at www.helium.ie.

OAK provides a platform for teenager with CF to share their stories.

Spectrum / Issue 29

8

SEASONAL FLU VACCINE

Reminder to Members

Whatisinfluenza(flu)?Influenza is a highly infectious acute respiratory illness caused by the influenza virus. Influenza affects people of all ages. Outbreaks of influenza occur almost every year, usually in winter. This is why it is also known as seasonal flu.

Whoismostatriskfromflu?Anyone can get the flu but it is more severe in people aged 65 years and over and anyone with a chronic medical condition. Chronic medical conditions include chronic heart conditions, chronic respiratory disease (including Cystic Fibrosis), diabetes mellitus and immunosupression due to disease or treatment. Pregnant women have also been found to be at increased risk of the complications of flu.

Howcanflubeprevented?Flu can be prevented by vaccination. Flu vaccine is a safe, effective way to help prevent flu infection, avoiding hospitalisation, reducing flu related deaths and illnesses.

Whatistheseasonal(annual)fluvaccine?Each year the seasonal (annual) flu vaccine contains three common influenza virus strains. The flu virus changes each year this is why a new flu vaccine has to be given each year.

Howdoesseasonalfluvaccinework?Seasonal flu vaccine helps the person’s immune system to produce antibodies to the flu virus. When someone who has been vaccinated comes into contact with the virus these antibodies attack the virus.

Howeffectiveisseasonalfluvaccine?Seasonal flu vaccine prevents flu illness in approximately 70% – 90% of people. The effectiveness varies depending on the age and health of the person being vaccinated and the strains of flu virus that are circulating. Older persons and those with certain long term diseases have lower immune responses so the vaccine may not be as effective but it will still prevent severe illness and hospitalisation.

Whenshouldyougetthefluvaccine?Vaccination should take place in September and October.

How do I get vaccinated?● People aged 18 years or older may attend either their GP or Pharmacist.● People under 18 years of age should attend their GP for vaccination.

Please make an appointment now. The vaccine and consultation are free to those within the recommended groups who have a ‘Medical Card’ or ‘Doctor Only Card’. Family doctors and Pharmacists charge a consultation fee for seasonal flu vaccine to those who do not have a ‘Medical Card’ or ‘Doctor Only Card’.

Remember: you should also ask your doctor about the pneumococcal vaccine which protects against pneumonia, if you have not previously received it. For more information, log on to www.immunisation.ie or check out the information leaflet at the following link: www.immunisation.ie/en/Downloads/Flu2012/PDFFile_17069_en.pdf

Sept/Oct 2012

9

DOMICILIARy CARE ALLOWANCE REVIEW CFAI Submission

The Cystic Fibrosis Association or Ireland recently made a submission to the Domiciliary Care Allowance Review Group on behalf of our members, and many thanks to everyone who provided feedback to us for this purpose. The following are some of the issues we highlighted.

Ireland has the highest incidence of Cystic Fibrosis in the world with almost 7 in every 10,000 people with the disease. The incidence of Cystic Fibrosis in Ireland is almost 3 times the average rate in other EU countries and the United States.

Parents of children with Cystic Fibrosis use this scheme to pay for the costs associated with the additional care requirements of their children as a result of having this disability. It ensures that parents can provide their children with access to the services and supports they need in order to stay as well as possible. CFAI outlined these additional costs in detail, including loss of income/time off work, physiotherapy expenses, expense incurred from the requirement for a high calorie, high protein diet, travel expenses for hospital appointments and admissions, fuel expenses, GP visits and others.

Cystic Fibrosis is a progressive disease; as people with cystic fibrosis get older their symptoms become more acute and complex. CFAI also pointed out that the cost of caring for people with this disease, both in and out of hospital, will therefore increase as the person gets older. The point was also made that the most prevalent underlying mutation that causes this disease in Ireland is also synonymous with more severe disease outcomes.

As Cystic Fibrosis is a genetically inherited, life-long disease and is progressive by nature, the argument was made that the Domiciliary Care Allowance should be automatically granted to our members and no further assessments or follow-up reviews should be required to assess their eligibility to this scheme.

CF is recognised by the State as being a chronic, long-term condition since it is one of few illnesses automatically included under the Long-Term Illness Scheme. It is also regarded as a permanently incapacitating condition under the Incapacitated Child Tax Credit Scheme. There is no cure for CF.

We hope the information provided in the submission with help convey the complexities of Cystic Fibrosis to the Review Group, as well as highlighting the importance of the Domiciliary Care Allowance in supporting families affected by this illness.

If you would like to read the full submission, please log on to our website or contact the National Office on 01 4962433.

Spectrum / Issue 29

10

ExPERIENCES OF 10-17 yR OLD CHILDREN WITH CF

Researcher Breda Cotter Seeks Volunteers

Breda is still looking for volunteers for her study!

Breda has been employed by the Department of Education and Skills, Dublin, as a teacher and psychologist for over 30 years. She is currently undertaking a Doctorate in Educational Psychology at Newcastle University, under the supervision of Professor Liz Todd. As part of this degree, she is conducting a study of identity formation among school-going children with Cystic Fibrosis.

In order to progress her research, Breda needs to conduct face-to-face interviews with a small sample of children. They will be at critical transitional points in their schooling, i.e. in 5th or 6th class and transitioning to second level, or in the Leaving Certificate cycle and transitioning to third level.

If your child meets the above criteria and if you are interested in having him/her participate in this study, please contact Breda, as soon as possible, for further information. Also, please note that your child will not be identified in the study and will have the option of withdrawing at any time.

We hope that results from this study will lead to a greater understanding of both of the influences that shape identity formation and of the factors that contribute to a well-integrated sense of identity and, ultimately, to psychological well-being among children with CF.

For more information, please contact Breda Cotter Mobile: 087 9325634 / Email: breda.cotter@newcastle.ac.uk

TLC4CF UPDATE

By Kaite Murphy, Regional Development Officer, TLC4CF

The past two months have been extremely exciting for TLC4CF between visits from the Rose of Tralee 2012 participants (see Fundraising section for more information), major fundraising events, and the official opening of the TLC4CF regional office in Limerick.

Although the office has been in operation for almost 8 months, it was a wonderful opportunity to celebrate the recent developments at the Mid-Western Regional Hospital, Limerick. The evening was a great success with members from the three counties lending their support. We were also delighted to be joined by Philip Watt (CFAI) and Eammon Mellet, of the Pro-AM golf tournament organising committee.

TLC4CF made a presentation on the night to Mr Mellet (who was representing both the Pro-AM organising committee and the JP McManus family) as a sign of our unending appreciation for the enormous support shown to TLC4CF by JP McManus over the past three years.

Maeve Gaffrey (PWCF) pictured here with CF nurse specialist Noelle Power, giving the Roses some roses – which were hand made by some of the paediatric in-patients in the build-up to their visit.

Sept/Oct 2012

11

SINGLE HIGH-DOSE ORAL VITAMIN D3 (STOSS) THERAPy — A SOLUTION TO VITAMIN D DEFICIENCy IN CHILDREN WITH CF?

J Cyst Fibros. 2012 Sep 18.

Shepherd D, Belessis Y, Katz T, Morton J, Field P, Jaffe A.

ObjectivesTo determine the safety and efficacy of stoss therapy on vitamin D levels over a 12 month period in children with cystic fibrosis and vitamin D deficiency (<75nmol/L).

Study DesignRetrospective chart review of 142 paediatric CF patients from 2007 till 2011.

ResultsThirty eight children received stoss therapy and 37 children with vitamin D deficiency were not treated and served as a control group. The stoss treated group had a significant and sustained increase in 25-hydroxyvitamin D levels measured at 1, 3, 6 and 12 months post treatment compared to controls (94.82±41.0nmol/L, p=0.001; 81.54±24.6nmol/L, p=0.001; 92.18±36.5nmol/L, p=0.008 and 64.6±20.0nmol/L, p=0.006 respectively). At 12 months post intervention, the mean difference in vitamin D levels from baseline between the stoss treated group and controls was significant at 15nmol/L compared to 5nmol/L (p=0.038).

Conclusion: Stoss therapy effectively achieves and maintains levels of 25-hydroxyvitamin D greater than 75nmol/L over 12 months.

SUCCESSFUL TREATMENT OF CEPACIA SyNDROME WITH A COMBINATION OF INTRAVENOUS CyCLOSPORIN, ANTIBIOTICS AND ORAL CORTICOSTEROIDS

J Cyst Fibros. 2012 Sep;11(5):458-60

Gilchrist FJ, Webb AK, Bright-Thomas RJ, Jones AM.

Burkholderia cepacia complex (BCC) is a group of 17 closely related bacterial species that can cause pulmonary infection in patients with cystic fibrosis. The clinical manifestations of BCC infection are varied but can include cepacia syndrome, which is a rapidly progressing necrotising pneumonia with an almost universally fatal outcome.

We report the case of a 38 year old man, known to have chronic infection with the ET12 strain of Burkholderia cenocepacia who developed cepacia syndrome 26 years after initial infection.

RESEARCH NEWS SECTION 02 PAGE 11—12

Spectrum / Issue 29

12

Aggressive treatment with a combination of 4 intravenous antibiotics, oral corticosteroids and cyclosporin brought about clinical, radiological and biochemical resolution of his cepacia syndrome. This case highlights the possible role of cyclosporin in the treatment of cepacia syndrome.

RISK FACTORS FOR AGE AT INITIAL PSEUDOMONAS ACqUISITION IN THE CySTIC FIBROSIS EPIC OBSERVATIONAL COHORT

J Cyst Fibros. 2012 Sep;11(5):446-453

Rosenfeld M, Emerson J, McNamara S, Thompson V, Ramsey BW, Morgan W, Gibson RL; for the EPIC Study Group.

BackgroundRisk factors for initial Pseudomonas aeruginosa (Pa) acquisition, particularly environmental exposures, are poorly understood. We aimed to identify such risk factors in order to inform prevention strategies and identify high-risk populations.

MethodsThe study cohort included all participants in the U.S. EPIC Observational Study who had no prior Pa-positive respiratory cultures (N=889). Cox proportional hazard models were used to test the effects of factors on age at first Pa-positive respiratory culture.

ResultsCystic fibrosis genotype functional class had an important effect on age at initial Pa acquisition (hazard ratio (HR) comparing minimal to residual CFTR function 2.87 (95% CI 1.88, 4.39)). None of the modifiable risk factors evaluated, including cigarette smoke, hot tub use, breastfeeding, or daycare, was associated with age at Pa acquisition. Similarly, newborn screening was not associated with age at Pa acquisition (HR 0.85, 95% CI 0.66, 1.09). Key associations were validated in a CF Foundation National Patient Registry replication cohort.

ConclusionsGiven the ubiquitous presence of Pa in the environment, it may be that many imposed lifestyle changes will have less impact on age at initial Pa acquisition than genetic determinants.

Sept/Oct 2012

13

ENTITLEMENTS SECTION 03 PAGE 13

DISABILITy ALLOWANCE

Your Questions Answered!

What age can I get DA?You can get Disability Allowance from 16 years of age. The age is currently under review and will be finalised in budget 2012.

Because I have CF do I qualify to get DA?You qualify to apply for DA but you are assessed as to your ability to work; you will need to show that you are “substantially restricted in undertaking work that would otherwise be suitable for a person of your age, experience and qualifications.”

Is DA means tested?Yes it is, any cash savings more than €50,000 will be assessed as well as any cash income from work or maintenance paid to you.

I am living with my parents is there income assessed for my DA?If you are living with a parent their income is not assessed as long as they are not providing you with maintenance payments. It should only be the PWCF means that is examined for DA. Cash income that you or your spouse, civil partner or cohabitant has may also be taken into account.

I am receiving DA and living with my boyfriend, we are getting married next year, can I still receive my DA once we get married?Once a PWCF gets married their DA income will be assessed as part of the family income and taken as one. If the family income (that is the DA payment plus any other income from work or maintenance will be assessed as one) is more than what is allowed your DA may be reduced or cut off.

I am on DA and am looking to do some part-time work; is this possible to do without affecting my DA?Yes, you can do rehabilitative work and earn up to €120 per week without your payment being affected. You must get permission from the Dept. Social Protection before you start work.

What happens if I earn more than €120 per week on rehabilitative work?If you earn more than €120 per week 50% of your earnings between €120 and €350 will not be taken into account in the Disability Allowance means test. Any earnings over €350 are fully assessed in the means test.

I am stating college this year; can I keep my DA in 3rd level education?Yes you can continue to receive your DA as a student in 3rd level education. There are also a number of other supports such as the back to education allowance that are available to persons on DA. For more information see the CFAI Guide to 3rd Level Education for PWCF, or call Tomas Thompson, CF Advocate on 087 9323 930.

Spectrum / Issue 29

14

I’VE LIVED A qUALITy LIFE THROUGH SPORT AND ExERCISE

By Paul Murphy, PWCF, Co Meath

My name is Paul Murphy and I am a 36 year old PWCF. I am one of five kids, but thankfully for my parents, the only one with CF.

All my life up until now I refused to write about myself in relation to CF, how well I have managed my life and more importantly my health. The reason for this is that I never wanted to upset any other PWCF that may not be doing as well as I am or maybe had more problems than I had. It was pointed out to me however, that a brief outline of my story may be of great encouragement to PWCF, or parents with newborns who have just been diagnosed with CF. It might well show them that there is light at the end of the tunnel. So here goes.

I was diagnosed with CF a couple of months after birth, like most people with CF. My parents were inevitably given the tragic news that my life would be very short. Although I may only have been a couple of months old, I must have been listening, because a decision was made there and then by my parents that I would spend my life proving people wrong.

My parents, from the off, decided they would change the rules by which a CF child was usually reared. Their view was that if I was here for a short time, then I was here to enjoy my life. They didn’t wrap me in cotton wool (though they did spoil me!). I was fed like a king, steak and chips most days for dinner while my siblings got whatever was going, but having said that, they never wanted anything but the best for me also. This was also played out when I was granted whatever sweets and toys I desired. Whatever kept me right they were one hundred percent behind it.

All the same, being a CF child my dietary requirements were watched carefully, but because my parents were both big into GAA and sport they had me running after a ball before I could walk after it. This meant that I gained an enormous appetite for both food and sport. And now I have touched on it, my main reason I believe I’ve lived a quality life is through SPORT and EXERCISE.

I started playing Under 12 football at just 6 years of age. I continued to play football and hurling up until this year, at 36 years of age. The love and determination for these games came directly from my wonderful parents encouragement to be the best I could be. I was slightly smaller than most

PWCF SPOTLIGHT SECTION 04 PAGE 14—22

A photo of a younger Paul Murphy on the field.

‘I believe a combination of sports and sticking to the daily routine of treatments has been a huge factor

in what has kept me so healthy.’

Sept/Oct 2012

15

kids because of CF but to balance this out I had free weights that I lifted from an early age to help me compete at the highest level.

Years later, the Physiotherapist in St Vincent’s told me that weights had been found to be great for PWCF, for strengthening the muscles around the lungs. So, once again I was ahead of the game.

While competing at hurling and football I wasn’t just filling my time. My team mates and I were the most successful group of underage players possibly in the history of Meath. We won Under 12,13,14,16,17,18 and 21 titles in football. Also in those years, we won Under 16,18 and 21 in hurling. All these titles were won at the highest grades.

I played no small part in these successes. At 18, our team having won the under 18 (minor) hurling County Championship, I was awarded the ‘Young Hurler of the Year’ in the club. At 20 after winning the Under 21 football Championship in Meath I was awarded Young Footballer of the Year. I was one of only three of my teammates to make the Senior Championship team at 18 years of age, the other lads didn’t advance until into their 20’s. Over the later years, I also collected Senior Hurler Of The Year and even ‘Junior Footballer Of The Year’, three years ago. So as you can see, I competed on equal terms with people that did not have CF and very much held my own.

Reading back over this it seems that I am just conveying how everything was ‘rosy’ in my life and I never encountered any problems. This is not so. Granted, I only first got admitted to hospital when I was 12 years old and not again until I was 22 when I had developed pneumonia. This was not just luck on my side. All my life when playing sport I had to work harder then my team mates by doing training at home on my own, coupled with all the medications that I needed to keep on top of.

Then as the years progressed, age and holding down a job comes into it. Thankfully I have been employed by AIB since I was 22 and they have always been very understanding of my illness. As I said earlier, I got pneumonia at 22 which put me through a lot of testing years. I spent more and more time being admitted, and as the CF nurses will verify, I wasn’t the easiest person to tell I’m being taken in to hospital.

Then when I hit about 28, it nearly all got too much for me. I found that I couldn’t reach the required levels to play Senior Football anymore. It depressed the hell out of me that my mates were still competing and I wasn’t part of that anymore. I decided that CF had finally caught me, so I packed in all sport and just continued to live my life without it; medications now and then and no sport led to my body failing more and more...which inevitably led to more and more infections. It wasn’t until I was 31 that I copped myself on. This really came about because age had caught my mates also and we were all back on a level playing field. They dragged me out of retirement and we all started to play junior football again. The bite was back. We even play 6 aside soccer now twice a week. The lads want to stay fit and I want to stay as healthy as possible. ‘Win win’ situation.

At 18, Paul won ‘young Hurler of the year’, at 20 ‘young Footballer of the year.’ In later years he also picked up awards for ‘Senior Hurler Of The year’ and even ‘Junior Footballer Of The year’.

Spectrum / Issue 29

16

Don’t get me wrong, I still party as good as the next man if not better, but every week I exercise more than most lads my age and I have my medications, nebs etc to fit in. Since 1999, I’ve also held down a permanent position in AIB.

By writing this article I’ve nothing to achieve but loads to share. I’m hoping that I’m some kind of light at the end of the tunnel for young PWCF and especially parents who have just had a child diagnosed with CF who undoubtedly think their world has come to an end. Really, the journey is just beginning. Embrace it and start with your child the way you mean to go on.

I’ve spent time in and out of hospital, developed pneumonia, developed MRSA in the lungs, had the depressive thoughts and will have more, I’m sure, at different stages. It comes with the territory but I cannot and will not let this hold me back. In sport I’ve achieved a lot, I’ve held a permanent job in AIB since I was 22, I bought a house when I was 26, but my biggest achievement to date was my recent marriage to the beautiful Lisa. Between her and and my now stepson Dylan, they have given me my biggest reason in life to keep CF at a very far distance.

To PWCF and new Parents of PWCF I can’t emphasise enough the importance of exercise, you won’t get any medals or awards for walking to the shop, but do it enough times and you will get an extra couple of percent in your lung capacity.

I thank my family and friends for all pushing me in every walk of life.

Paul Murphy

Paul and Lisa on their wedding day.

‘My biggest achievement to date was my recent marriage to the beautiful Lisa. Between her and and my now stepson Dylan, they have given me my biggest reason in life to keep CF at a

very far distance.’

Sept/Oct 2012

17

THE PURSUIT OF A DREAM

By Robert Cook, PWCF, Co Clare

My name is Robert and I was born in 1984. At three months old I was diagnosed with cystic fibrosis.

I would class my CF as being mild as it has never stopped me from living a normal life. Like everyone, my dream was to grow up get married and have kids and enjoy life. I would have to say the beginning of this dream started when I met my wife Sandra back in 2004. We got on like a house on fire and we have stuck by each other through the good times and the bad. We eventually got married in 2010. Sandra and I always spoke about having kids and about the challenges we may face in achieving this dream. I think deep down I was hoping that I would be in the lucky 2% category where we would be able to conceive on our own; my CF was not that bad so I was hopeful, but I think the two of us knew the odds were against us.

After trying to conceive on our own with no success we made the decision to discuss the issue with the CF team at St.Vincent’s University Hospital. The CF nurse and my Consultant sat down and spoke to Sandra and I, and a decision was made to refer me to the Merrion Centre to have a semen analysis done. After waiting a few weeks the results were back and my fears were confirmed – the chance of conceiving on our own was slim to none. When the news was broke to us I had a terrible feeling in the pit of my stomach, but I pulled myself together pretty quickly and Sandra and I wanted to know where to go from there. To our delight an appointment was made for us to meet Dr. Mocanu at the HARI unit in Dublin. We were both very nervous about meeting Dr. Mocanu mainly because I was only 24 years old at the time and Sandra was 21. We were the youngest couple to have ever attended the clinic. Dr. Mocanu was excellent and we found him to be very kind and caring. He informed us of the road that was ahead of us. We were over the moon when Dr. Mocanu decided to help us and our IVF journey was under way.

First off Sandra had to have a blood test to check if she was a CF carrier; thankfully she wasn’t and I was scheduled in for a testicular biopsy to check if sperm was present and if so it would be extracted. The night before the biopsy we stayed in Dublin and went to the 02 to see boyzone. The concert was great but the biopsy was on the back of my mind. I wasn’t one bit worried about the procedure; all that was on my mind was if there would be a positive outcome. The next morning Sandra and I arrived at the HARI unit for 8am for me to have the biopsy carried out. I was awake for the biopsy but didn’t feel a thing; meanwhile Sandra was waiting patiently in the waiting room to find out how things went. Thankfully everything went well. Sandra then faced the process of egg collection. This was a tough process and involved Sandra having daily injections, one into her stomach and one into her leg. Personally I always wondered how Sandra would cope with the injections and regular blood tests as she hated the sight of needles, but during the IVF process it showed just how much she wanted a baby. She just soldiered on with it and never complained. Once Sandra’s ovaries were stimulated it was time for egg collection. Both of us were very nervous about this. I remember when they brought Sandra into theatre – I was a nervous wreck and all I could do was pray. I remember feeling very guilty over Sandra having to go through this, but shortly afterwards the nurse came out to tell me Sandra had done very well; she was fine and they hand managed to retrieve nine eggs. Over the next few hours the eggs were going to be injected with my sperm so we had to wait. We went back to our hotel where

‘Sandra and I always spoke about having kids and about the challenges we may face in

achieving this dream’

Spectrum / Issue 29

18

Sandra got some rest. The next morning we rang the HARI to see how things were going and were told that eight of the eggs had fertilized, so embryo transfer was scheduled. Sandra would have to use a hormone gel for the next 14 days and on day 14 we were to take a pregnancy test. 14 days felt like 14 years. We were very excited and couldn’t wait 14 days so we did a pregnancy test on day 7 which read positive. I thought I was seeing things, we couldn’t believe it. We bought another test on day 8 which also read positive – things were looking good. On day 9 we bought another test that also read positive so we decided not to buy anymore and wait until day 14. The big day arrived and Sandra was nervous. I wasn’t really nervous since we had three positive results already but the biggest shock of our lives happened on day 14 when we had a negative pregnancy test. Sandra broke down into tears, I held her and tried to comfort her and then I went back upstairs and cried my eyes out. I was convinced it had worked. That day we drove 100 miles for a cup of coffee, I don’t think we knew what we were doing. We found out later that the positive reading we had received were false readings as the hormones that Sandra had been taking were still in her system.

It was a year later when we were ready to try IVF for the second time. The procedure was a bit easier this time because we still had two frozen embryos that meant no biopsy for me and no injections or egg collection for Sandra. Day 14 we had a negative pregnancy test. This meant we had one frozen embryo left. It would be about another six months before we were ready to have another go.

When we reached our third attempt negativity started to set in a bit but my mother had raised me to always think positive and it was this positivity that gave Sandra and I the strength to carry on. We would always say to ourselves ‘this time things might be different’. We asked Dr. Mocanu, the head consultant at the clinic if he would carry out the embryo transfer for us. He was more than happy to. He came into work especially for us on a Sunday morning to carry out the transfer. Dr. Mocanu couldn’t understand why things were not working out for us, we were both very young and always had grade A embryos so in theory we should have a football team by now. But things were not to be and our third attempt at IVF had failed – you start to wonder will if it will ever work for you.

A full year had passed and we were ready to go back to the HARI unit for our fourth attempt. At this point we had been trying for three and a half years and it had taken its toll mentally, physically and financially on both of us. When I think back we would be driving the Dublin road at 5.30am most days, a 350 mile round trip outside of any medical procedures. Thank god for McDonald’s coffee!! You try to vision your future as a family and this drives you on – you will grab onto every bit of hope to keep going forward.

On this cycle of IVF we had no frozen embryos left so we had to start from scratch. During the 14 day wait we had to make our way to England for family reasons and this would mean doing the pregnancy test there. Sandra wanted to do a pregnancy test before we travelled. We did one on day 10 and being honest I didn’t want to do the test as I wasn’t sure how accurate it would be given what happened in the past. Sandra did the test on the morning we were to travel. The test was showing a slight positive result and as much as I wanted to believe in my heart that this reading was accurate my head was telling me otherwise. At this point Sandra was convinced she was pregnant. Being honest I think the test didn’t make me any wiser. So we set off on our journey to England. We travelled by ferry, we didn’t fly in case the altitude would affect our chances. Once again the big day arrived and it was time to do a pregnancy test. I remember when Sandra was doing the test I felt sick to my stomach. The two of us just hovered over it

‘You try to vision your future as a family and this drives you on – you will

grab onto every bit of hope to keep going forward.’

Sept/Oct 2012

19

waiting for a result. The test showed the strongest positive sign I had ever seen, we couldn’t believe it! I thought I was dreaming and we cried with joy – the dream was soon going to become a reality.

Three weeks passed, we were back in Ireland and on the road to Dublin. We had an appointment with the HARI unit to check for a heartbeat. Sandra and I had spotted the heartbeat before the nurse had pointed it out to us. We couldn’t take our eyes off the screen. Pretty soon we had reached the 12 week mark; the pregnancy was going perfect thank god. And on the 12th July 2012 our baby son Callum was born at 10.27pm, weighing 7lb9oz and healthy as can be, our bundle of joy. The dream has come true and life couldn’t be better. We call our little man the champ because his fists are always closed.

In conclusion, IVF is a rollercoaster but if you have a dream grab every opportunity with both hands to achieve it. You may not achieve it on your first attempt but if you want something that bad you will keep chasing it. When we see Callum smiling up at us there is no doubt that if we had to do it all over again in the morning we would.

Robert Cook

‘There is no doubt that if we had to do it all over again in the morning

we would.’

Robert and little Callum.

Spectrum / Issue 29

20

BEING A PWCF

By Kelli Maples, PWCF, Co Dublin

My whole life I’d wondered would I be a Mammy, should I be a Mammy; I always pictured myself with a baby as a child. I have four sisters and two brothers – I’m the baby of seven (spoiled!). I wanted a job, someone to love me; a house, a family of my own; to me that was ‘normal’ and I just wanted to be normal. I was always worried growing up about whether someone would love me because I had CF, and all that comes with it including the nebs, physiotherapy, tablets, iv’s and hospital stays.

I was diagnosed at birth to a mother and father of 44 with 7 children in the family ranging from 6 to 22 years of age. My mother had 8 children, but Tracey who was born with a distended stomach and a mop of red curls died at just 4 days old. I am told I was the image of her so as you can imagine I was extra loved by everyone.

I spent my first two Christmas’s in Crumlin hospital and was in and out all my childhood life. My mam started to do home IVs when I was 6 years old. I spent my time under the care of Dr Gorman who was a wonderful doctor. I transferred to Beaumont when I was 16 and am now under the care of Prof McElvaney. The new out-patient unit is great and I don’t think I’ve been an in-patient for about 2 years now. I do my own home IVs now, Baxter is a godsend!

Growing up, I kept the fact that I had CF to myself. My close friends knew but I never really spoke about it unless I had to. I met Stephen through a school friend when I was just 16; I remember it was a Tuesday and I was waiting for a bed at the time. On the following Monday I got a bed so I had to tell him I was going into hospital. He said he would come and visit me but I honestly didn’t think I’d see him again. But he came on the Wednesday and every day until I got out three weeks later. He’d seen it all, the nebs, the IVs, all the tablets and even tube feeding, which I’d never been on before. I realised that my having CF didn’t bother him – I was just Kelli to him.

Later on in life, we decided we wanted to start a family. I tried to get pregnant naturally, but no such luck! We spoke with my CF Consultant in Beaumont and were referred to the Rotunda whereupon test after test was carried out. Eventually we made it to the HARI clinic and were advised to pursue three attempts of intrauterine insemination (IUI) and if that didn’t go well we were going to try in vitro fertilisation (IVF).

I was in good health at the time but had a set of IV’s just to prop myself up and in July 2010, at the age of 25, I started taking fertility injections and had my first attempt at IUI. You may leave your pride at the door, but don’t forget your wallet! If you have ever been through anything like this you know what I mean! I just kept reminding myself why I was there, but then I didn’t want to think that in case it didn’t work...talk about catch 22!

After the procedure we were told to wait 16 days before taking a pregnancy test. I spent this time crying, trying not to, and then crying again. Do I think it’s positive? Do I not? Eventually I just tried to blank it out. So day 16 arrived, I did the test and it was positive. I couldn’t believe my luck and we were over the moon – everyone was delighted for us.

At about 8 weeks I needed IV’s, I was so nervous going on them but I was assured it was safe.

‘ I wanted a job, someone to love me; a house, a family of my own; to me that was ‘normal’ and I just wanted to be normal.’

Sept/Oct 2012

21

The next two weeks I felt awful, but after the IV’s I went back to my constant state of bliss – will it be a boy or a girl...will it have red hair or black hair.

I was in work one Saturday (I work for MAC Cosmetics in BT2 in Blanchardstown) and I had a constant pain but I kept telling myself it was nothing to worry about. Later that evening I remember watching the X-Factor and I just knew something was wrong, I will never forget it. We drove in to the Rotunda and a scan confirmed I’d lost my baby at 11 weeks and 2 days, and all my hopes and dreams along with it.

Oh I wanted to never get out of bed...I just wanted the ground to swallow me up. All I’d been through was for nothing. All the staff were so lovely, telling me it happens to so many people and encouraging me not to lose hope. When I got back home with Stephen I’ll never forget my families poor faces, it was awful. Stephen was trying to put on a brave face but he was hurting for me, and for the baby he had just lost. The way everyone looked at me made me feel worse.It took me a long time to get my head together to be strong enough mentally to try IUI again. I told nobody this time; I was too afraid to even think about it myself. When I was told I had cysts on my ovaries I just wanted to give up and I was thinking ‘right Kelli, it just isn’t meant to be’. People can sympathise with you but unless they’ve had a longing to be a mother, to know what a kick feels like, to actually want morning sickness, then they will never know how you really feel. The only person I could really talk to was Stephen. It was hard on the two of us but it did bring us closer. Stephen didn’t want me to try IUI again, he said it was too much for me, but I felt strong enough to try again.

After the second attempt of IUI I didn’t even think about it for 16 days. It just so happens that day 16 was also my 26th birthday. I did the test but I couldn’t even look at it to see the result, my nerves were done! Was this going to be the worst birthday ever or possibly the best birthday ever!?

As soon as I saw his face I knew it was going to be the best, but I was afraid to get too excited. We kept it a secret until I reached 12 weeks and then we started to tell our families. It actually started to sink in that I was actually pregnant! My belly was growing by the minute and I loved every second of it. In work I wore things to hide my belly because I was so afraid to let people know in case I jinxed it.

At 15 weeks I needed IVs – talk about having a nervous breakdown! I couldn’t sleep without having nightmares about what the medications were doing to the baby. After two weeks and I don’t know how many scans I was fine and my baby was getting on just great in my big belly. I couldn’t have been happier; it felt like such an achievement. I got pregnancy diabetes and needed another set of IVs at 7 months, but all in all the pregnancy went well.

I was sectioned at 37 weeks. Aíbhlinn was a little pudding at 7lbs 14 oz. Now I can’t remember my life without her in it – she is my biggest achievement and I’m so proud of myself. Aíbhi is half of me and half of Stephen and we love her dearly.

Now it’s hard going, she is nearly 6 months. As a baby she had colic and really bad reflux so she spent a lot of her time crying – of course I got the blame for that; her Daddy says I spent most of the pregnancy crying but can you blame me!?

I’m so lucky to have Stephen and a big supportive family; I couldn’t have done it without them. My sister Susan is Aíbhi’s Godmother and second Mammy and she takes the baby every Thursday for a sleepover so I can catch up on some sleep. I’d be lost without my family and Aíbhi is very lucky to have so many people who love her – she is the baby of 17 grandchildren!

Spectrum / Issue 29

22

My only worry now is will I see her grow up? Well I’ll try my very best! I go to the gym at least three times a week, cycle my bike when the weather is fine and walk Aíbhi for miles. The whole time I’m sweating on the treadmill I’m thinking of my baby – she is my motivation, I have to stay as fit and healthy as I can for her!

And now I’m a Mammy I have to give my own Mammy some credit for all she has done and all she continues to do for me. She is extremely selfless and loving and I could never have done it without her. To my manager in work who was extremely supportive and understanding; without all the shift swaps Aíbhi might never have been here; poor Alica who lent her ear on many an occasion...thanks for listening, and my one and only Stephen because he bore the brunt of all my moments of madness!

The moral of my story and the reason I told it is because I know there are lots of people just like me, but just believe that God is Good and your day will come – mine did and I still pinch my baby every morning!

Kelli Tara Maples

Photographs courtesy of Tom Coakley Photography / www.tomcoakleyphotography.ie

KelliwithAíbhlinn.ThetwofeathersrepresentTraceyandthebabyKellilostatjust11weeksand2daysafterherfirstattemptof IUI.

Kelli and Stephen with their daughter Aíbhlinn, and Kelli’s parents.

Sept/Oct 2012

23

FUNDRAISING SECTION 05 PAGE 23—33

Once again, we take this opportunity to thank our volunteers and supporters for your Trojan efforts to fundraise on behalf of CFAI. Given the (now almost boring news) economic climate, we appreciate how difficult it is to maintain and increase income from community fundraising in particular. So thank you all very much.

65 ROSES NATIONAL AWARENESS WEEK 2013

Preparations are Underway!

We would like to thank all the volunteers who helped support our National Awareness Week in April – we would not be able to do this without you! If you would like to get involved in April 2013 please contact Martin or Rosie at 01 4962433 or email mcahill@cfireland.ie or rbegley@cfireland.ie.

1 IN 1,000

By Ciara Enright, 1 in 1000 Co-ordinator

Thank you for all the donations made so far for 1 in 1000. Here are the details for those yet to lodge their funds.

● Bank: AIBAccount Name: One in One ThousandSort Code: 93-10-71 (add name to narrative)

● Send a cheque to 1 in 1000, CFAI, 24 Lower Rathmines, Dublin 6 (payable to 1 in 1000)● Make an online donation at www.cfireland.ie (add 1 in 1000 to narrative)

For any queries, email Ciara at cenright@cfireland.ie or LoCall 1890 311 211

BEST WILL IN THE WORLD WEEK

22nd – 26th October

In the lead up to this week, www.mylegacy.ie will conduct a nationwide publicity campaign to highlight that leaving a gift in your will to your favourite charity is a simple, wonderfully fulfilling experience. It can benefit countless people and anyone can do it, but the first step has to be making a will or amending an existing will.

During ‘Best Will in the World Week’ solicitors in your local community will be offering will consultations for just €50 (including VAT). This fee will cover an initial consultation about your will if you have not yet made one or a consultation about your existing will. If there are any additional costs after the will consultation, your solicitor will discuss these with you separately. For more information please visit www.mylegacy.ie

Spectrum / Issue 29

24

CHALLENGE EVENTS

Fancy a Challenge?

65 Mile Kayak for CF The Waterford Emergency Services Kayak For Cystic Fibrosis unfortunately had to be postponed, “we had to abort after 10-15km for safety reasons. We will hopefully try again with 100% perfect weather conditions.”

If you would like to get involved in the next attempt, call Alan Magner on 087 9257105. Alan and friends have raised a considerable amount for CF so far, which is designated towards the refurbishment of four paediatric beds for CF in Waterford Regional Hospital.

Please support the guys in raising funds; their My Charity page is www.mycharity.ie/event/waterfordcysticfibrosis/

Joe Crowley Camino Walk – 24th AugustSupport Joe Crowley’s Camino walk of 800km for the Cystic Fibrosis Association of Ireland and the Pharmacy Benevolent Fund. 100% of the funds raised will go towards these two important causes and will be divided equally between the two charities. You can also support the Cystic Fibrosis Association of Ireland and the Pharmacy Benevolent Fund directly by sending a cheque to the ‘Joe Crowley Camino Walk c/o Cystic Fibrosis Association of Ireland, 24 Lower Rathmines Road, Dublin 6’.

You can donate online at www.cfireland.ie – please remember to mark your donation ‘Joe Crowley Camino Walk’.

The Paddy Kierans Memorial Walk – Poland, 23rd September – 4th October We would like to wish the best of luck to all the walkers who flew to Poland on 23rd September!If you would like to take part in 2013 please contact Rosie in CF House at 01 4962433 or one of the Committee members at the numbers below:

Bernie 087 2353319; Julia 087 9911331; Frank 086 6060261 or Tony 086 25112731

To Hell &Back 10k Challenge, 7th OctoberIreland’s toughest 10k challenge

What? Challenging 10k run that will test your boundariesWhen? Sunday 7th October 2011, start: 12.30pmWhere? Kilruddy Estate, Bray, Co. WicklowWho? Anyone who likes a challengeHow much? €45 (indiv.) €145 (team)How do I register? Log onto www.hellandback.ie

For sponsorship pack contact us at 01 4962433 or email Rosie at rbegley@cfireland.ie or Martin at mcahill@cfireland.ie

Limerick Women’s Mini-Marathon, 14th October For sponsorship pack contact us at 01 4962433 or email Rosie at rbegley@cfireland.ie or Martin at mcahill@cfireland.ie. Further information from www.limerickminimarathon.com

Sept/Oct 2012

25

Dublin Marathon – 29th OctoberThe Dublin Marathon is one of Ireland’s biggest and best marathons! Why not join our Dublin Marathon CFAI Team. How to join our team Enter the Dublin Marathon online at www.dublincitymarathon.ie or for a paper application form contact the Dublin Marathon office on 01 6232250.

Fundraising pack consists of:● CFAI running Jersey● Sponsorship cards ● Fundraising tips and ideas

For a sponsorship pack, please contact Rosie in the National Office t: 01 4962433 or e: rbegley@cfireland.ie, or Martin e: mcahill@cfireland.ie

New york Marathon 2012 – 4th NovemberThe very best of luck to everyone participating in the New York Marathon in November for CF – we will be cheering you all on!

If you would like to take part in November 2013, or know of any friend/s who may wish to do so on behalf of Cystic Fibrosis, please contact Rosie in the National Office t: 01 4962433 or e: rbegley@cfireland.ie, or Martin e: mcahill@cfireland.ie.

Further information from www.nycmarathon.org

CF Everest Base Camp Trek, 1st – 14th May 2013Are you up for a challenge?

Join the Irish Cystic Fibrosis Team to Nepal!

For sponsorship pack please contact Rosie in the National Office t: 01 4962433 or e: rbegley@cfireland.ie, or Martin e: mcahill@cfireland.ie

The Dublin City Marathon is on 29th October!

The CF Everest Base Camp Trek takes place in May, 2013.

The New york City Marathon is on 4th November!

Spectrum / Issue 29

26

COMMUNITy EVENTS

More Details Below

Irish Country Stars Fundraising Concert in aid of CF – 10th OctoberWhere: Hazel Hotel, Monasterevin, Co KildareTime: Doors open at 7.30 and concert starts at 9pmTickets: €20 eachContact: Mary Behan email: djmarybee@eircom.net

Irish Country Stars Calendar 2013 in Aid of CFPurchase an Irish Country Stars Calendar for just €10 (excluding post and packaging) with all proceeds going to CF.

Created by the purple Rose Bike Club to raise funds for the CFAI, the calendar features some of Ireland’s top Country Music Stars including Declan Nerney, Mick Flavin, Robert Mizzell, Patrick Feeney, Jimmy Buckley, Crystal Swing, Lousie Morrissey, Trudi Lalor, Derek Ryan, Nathan Carter, Johnny Brady & The Davitts Country Band on motorbikes!

If you would like to place an order please contact Martin or Rosie at 01 4962433 or email mcahill@cfireland.ie or rbegley@cfireland.ie or order online www.cfireland.ie

CD IN AID OF CF

Annette Andrews

Annette Andrews was a Senior Examiner at RIAM and a former student of the renowned pianist John O’Connor. In June 2011 she came out of retirement to give a recital at the Pavilion Theatre, Dun Laoghaire, in aid of the Cystic Fibrosis Association of Ireland. One of Annette’s grandchildren suffers from Cystic Fibrosis. This was a highly successful fundraiser.

The wonderfully varied programme showed Annette’s considerable talents as a performer and her sensitive interpretation of the Fauré and Chopin Nocturnes. This resulted in Annette being invited into the recording studio to record the repertoire with a view, of course, to further fundraising for this worthy cause.

Sept/Oct 2012

27

All costs associated with this recording and the production of the CD’s have been fully met by donors. Proceeds from the sale of the CD will be passed on in full to the Cystic Fibrosis Association of Ireland.

Contents1. Schubert Impromptu No. 2, Opus 90, in Eb Major 5:222. Schubert Impromptu No. 4, Opus 90, in Ab Major 7:293. Brahms Rhapsody No 2, Opus 79, in G Minor 6:264. RAVEL Minuet from Sonatine, in Db Major 3:395. Chopin Nocturne, (Posth.), in C# Minor 4:426. Fauré Nocturne No. 4, Opus 36, in Eb Major 7.20

The CD is now on sale at www.cfireland.ie and is priced at €10 (excluding post and packaging).

LAUNCH OF ‘DINNERS TO MAKE...THINGS TO BAKE’

18th October

The launch of ‘Dinners to Make...Things to Bake’ will take place at the Mc William Park Hotel Claremorris on Thursday 18th October at 8pm.

Marjorie Morrow Nolan will be one of the special guests and will be doing a cookery demonstration, Marjorie writes for the Ireland Own as well as doing a cookery slot on Midwest Radio.

There will also be food tasting and music by Gerry Carney. All the proceeds from this book will go to the Cystic Fibrosis Building Fund at Mayo General Hospital Castlebar Co Mayo.

Admission is complimentary, but if you would like to reserve a table please email philkilmaine@aol.com or phone Philomena on 087 3528953,

CHARITy OF THE yEAR

Royal College of Surgeons

The Royal College of Surgeons has kindly chosen us as their charity for 2013.

We look forward to reporting on their events throughout the year.

All proceeds of Annette Andrew’s CD will go to CFAI.

Spectrum / Issue 29

28

RECyCLE yOUR OLD MOBILE PHONE

With Fonebank

Through our partnership with the recycling experts at Fonebank, CFAI could earn around €5 for every mobile phone or iPod donated on our behalf.

Why recycle?As well as being good for the environment, recycling can help raise valuable funds for CFAI. Please encourage your friends, colleagues and family to donate their used mobile phones.

What can be recycled for CFAI?Mobile Phones (all models), Apple Ipads & Ipods.

Donate in 3 easy steps:

1. Place phone(s) in a suitably sized Jiffy bag and seal securely with your order details inside. If you do not have a jiffy bag wrap the phone(s) in bubble wrap or tissue paper and place in well taped envelope or box. Please note that we do not require chargers or boxes but do require batteries

2. We recommend that you use registered post as you can insure your phones with An Post

3. Please state on the envelopes that you are recycling for CFAI

FonebankUnit C2 Airside Enterprise CentreAirside, Swords, Co. Dublin Or at greater risk by FREEPOST to:

FonebankUnit C2 Airside Enterprise CentreAirside, FREEPOST S4728Swords, Co. Dublin

For more information, contact Fonebank by email: info@fonebank.ie or call: 01 4229004

If you’d like to hold a collection in your school or community centre, please get in touch with the CFAI Fundraising team at LoCall 1890 311211 or email info@cfireland.ie

Sept/Oct 2012

29

SOROPTIMIST INTERNATIONAL IRELAND

National Project for CF

The Soroptimist International, Ireland has recently written to the CFAI to let us know how they have distributed the funding raised for CF-related projects in Ireland through their national project.

Once again, we would like to acknowledge and extend our gratitude to Soroptimist International, Ireland for their very supportive and important fundraising efforts, which we have previously highlighted in Spectrum and on our website. Their total contribution for the support of CF related projects is €102,676. Soroptimist International, Ireland have provided the following breakdown of funding allocation:

Allocation FundingBuilding Fund Mayo General Hospital €5,085CFAI Local Associations (affiliated to CFAI) €20,973Build4Life Cork University Hospital €5,650CF Unit Crumlin Hospital €4,122MWRH Limerick (some forthcoming) €66,846 for CF Equipment

Please note that Build4life is a separate CF charity that is not connected to CFAI, which undertakes fundraising for Cork University Hospital.

CHEqUE PRESENTATIONS

See Below

Lakes 10k 2011Due to the illness of one of the organisers of the above event, the presentation of this cheque was delayed from early 2012.

CFAI have been beneficiaries of the proceeds of this event for a three year period and to date €55,000 has been donated and thank you to Lorna, Shane, Siobhan and crew for your unmitigated support over the years.

Paul Plunkett, Managing Director of Ace Autobody, has been the title sponsor of the event since it’s inception in 2009 and we thank you, Paul, for your generous support.

L-R: Martin Cahill, Lorna Brennan, Organiser, Jordan Kidd (PWCF), Ann-Marie Kidd, and Paul Plunkett, MD of Ace Autobody.

Spectrum / Issue 29

30

THANK yOU

See Below

CF LugathonA big THANK YOU to the CF Lugathon team who braved the mist and cold on Lugnaquilla Mountain on 15th September and raised a fantastic €4,570 with more funds to come in!

A special word of thanks to the Garda Síochana Mountaineering Club for providing leaders, marshals, medical cover and guides for the day. Your support was brilliant and unobtrusive, and contributed to the safety and enjoyment of all participants on the day. Robin Faughnan and Aidan McGuinness deserve special mention for organising the team. Thanks also to Tim Clifford and Rory Tallon.

Carlow Credit Union’s youth Advisory CommitteeMany thanks to Sarah Nolan, Niamh Dundon, Kayleigh Elgar and Catriona Farrell for selling ribbons in their school.

They raised the sum of €380 for selling the ribbons in St. Leo’s College, CBS College and Carlow Credit Union.

Green Day for CFAIA ‘Green Day’ was supported by staff in both the Department of Public Expenditure & Reform and the Department of Finance recently. A total of €1,200 was raised for CFAI – thank you to all involved

Charity Truck Show Thank you to Harte Peat Ltd who organised a Charity Truck Show in Clones Co Monaghan on Saturday 8th September and raised a fantastic €3,800 for Cystic Fibrosis.

The CF Lugathon team in 2012.

L-R: Sarah Nolan, Niamh Dundon, Kayleigh Elgar and Catriona Farrell.

The Charity Truck Show raised €3,800 for CFAI.

‘Green Day’ raised €1,200 for CFAI.

Sept/Oct 2012

31

Rose of Thurles Gala BallWe are extremely grateful to Evelyn Roberts and her colleagues who are organising this event to raise funds for our recently commenced Waterford Hospital Project.

It promises to be a great night in the Ragg in Thurles, with interval entertainment, judging of Roses, Raffle and Auction. And of course, it’s keenly priced for a great night’s entertainment.

The gala ball will take place on October 13th and tickets cost €25.

So hurry up and call the ladies on numbers on the poster and reserve your place!

ROSE OF TRALEE

Journey with CFAI

We have been one of the chosen charities for the Rose Of Tralee for 2011 and 2012.

This has been a wonderful journey for CFAI with huge awareness gained form the great ambassadorial roles played by both Clare Kambamettu in 2011 and Tara Talbot throughout 2012. Given the huge commitments both ladies had in their roles, they were both very generous to us in attending events during their tenure, helping us to gain much needed publicity for the cause of CF in Ireland.

Also Oliver Hurley and Valerie Kerins and all the team in the Rose of Tralee office were most helpful at all times. A big thank you to all.

Roses of Tralee 2012 pay a visit to the CF unit at the Mid-Western Regional Hospital putting a smile on all of the children on the wards.

Spectrum / Issue 29

32

Antony Lewis-Crosby carries the Olympic TorchAntony Lewis-Crosby, CF parent from UK, was chosen to carry the Olympic Torch through Penge to mark his excellent work and dedication to the cause of Cystic Fibrosis both in the UK and Ireland. His challenge event, over an estimated 5 years period, has been a great success to date.

Antony’s unique ‘Racecourse Challenge’ which he financed himself, has raised a whopping €11,084.97 for CF in Ireland, and many CF volunteers will attest to his unassuming and gentlemanly determined nature, while themselves enjoying the day at the races also.

In 2009, Antony set out on a unique Racecourse Challenge event the basis of which was to visit every one of the 87 racecourses in the UK and Ireland in alphabetical order to raise money for Cystic Fibrosis in UK and Ireland.

The challenge is split into two parts which will run simultaneously, one in the UK and one in Ireland. The whole project will take approximately 5 years to complete. In the UK, where money raised will go to the Cystic Fibrosis Trust, there are 62 racecourses, with a further 27 racecourses in Ireland, a total of 89 venues. However, at the commencement of the project, both Balinrobe and Clonmel were ‘rained off’.

Money raised in Ireland has been allocated to the provision of adult CF services in the Republic’s hospitals. Funds are raised through betting on every race on the day and also where possible through collections among the crowd.

CF supporters are invited to join Antony and his family at the meetings and you should contact CFAI to find out about future events. Antony is determined that everyone has a good day out as well as raising money for such an important cause.

We know of few more deserving people to receive this signal honour, and we congratulate Antony and thank him profusely on behalf of CFAI for his fundraising efforts to date.

NATIONAL PLOUGHING CHAMPIONSHIPS

September 2012

CFAI & TLC4CF headed to the National Ploughing Championships (NPC) to raise funds and awareness for Cystic Fibrosis this September.

On arrival CFAI and well known band Crystal Swing launched the Irish Country Stars Calendar 2013 in aid of Cystic Fibrosis.

The concept and delivery of the calendar came from Mary Behan and Billy Bresnan, CF parent. Congratulations to both on the launch.

Anthony carrying the Olympic Torch through Penge.

Sept/Oct 2012

33

Andfinally...WatchoutfornewandexcitingeventsinournextSpectrum

Take part in exciting events and activities to help us raise awareness of Cystic Fibrosis and to raise much need funds for services and facilities for the benefit of our CF Community.

If you would like to organise your own event please contact the Fundraising Department on LoCall: 1890 311 211 or e: info@cfireland.ie

TLC4CF were selling their ‘Food for Thought’ cook book by Breda O’Shaughnessy which was a hit with the public at the NPC, with delicious recipes.

Well done to Ciara Enright, Fundraising and PR Co-ordinator, CFAI, who capably managed the CFAI Stand for the 3-day period, ensuring that CF Awareness was the overall priority of CFAI presence.

To purchase the calendar go to www.cfireland.ie or for more information on the cook books visit www.tlc4cf.com

Crystal Swing launched the Irish Country Stars Calendar at the event.

Spectrum / Issue 29

34

ISSN 2009-4132ISSN 2009-4132

Cystic Fibrosis Association of Ireland t: +353 1 496 2433 CF House f: +353 1 496 2201 24 Lower Rathmines Road e: info@cfireland.ie Dublin 6 w: www.cfireland.ie Ireland Company Reg: 449954 Charity: CHY6350

ISSN 2009-4132

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 was be completed●Providing funding towards new CF Units around the country including Crumlin, Drogheda, Galway, Mayo and Limerick Hospitals●Funding research in Cork University Hospital, St Vincent’s University Hospital and University College Dublin●Campaigning to improve the rate of double lung transplantation in Ireland●Providing advice and expertise