TLC4CF|12

“I would like to wish Dr Mahony a well deserved retirement. He was so dedicated and kind to my late son Kevin Butler. We will always be so grateful to him. Kevin got on so well with Dr Mahony and he always treated Kevin with respect and explained everything to him so well”. (Pat and Patricia Butler, Tipperary)

I have a distinct recollection of the first time I met Dr Michael Mahony, as it coincides with the birth of our second daughter. It was not until some years later in 2008 that I began to realise how much of a part Michael would have in our lives and indeed in the lives of many people and families living with Cystic Fibrosis. It was not until a few years later again, through the course of working more directly with Michael in my capacity as Chairperson of TLC4CF, that I fully understood how passionate, resilient and dedicated he was to working for people with Cystic Fibrosis.

Any family attending what is now the University Hospital Limerick, back in the mid 2000’s, are all too aware of how limited the service was for people living with Cystic Fibrosis. It was a time when the country was witnessing unprecedented financial growth and thankfully Minister Mary Harney saw fit to ringfence some of that money to bolster services for people with CF. It was from this base that Michael took it on board as a personal challenge to improve services for all living with CF in Tipperary, Limerick, Clare and beyond.

The journey from Minister Harney making funds available to having nurses and doctors on the ground in the hospital was a complex and difficult task but one that Michael executed with consummate professionalism. It was his enthusiasm and dedication to do the right thing for his patients that was the catalyst for county branches of the CF organisation in Tipperary, Limerick and Clare to combine their efforts through TLC4CF and to work as a single unit to progress things for our members. From a personal point of view I am forever grateful to Michael on a number of fronts but also as his endeavors provided an environment for me to work with one of the best voluntary committees I have ever been part of. Linda Drennan (RIP) and Marcella Clancy in Clare, Caitriona Hayes in Tipperary and Liam O' Kelly in Limerick worked endlessly with Michael throughout 2008 to the opening of the Leben Building in 2016. Michael's support and encouragement to the committee during these times is a source of lasting inspiration.

It is true that it was Michael's job to provide a services to his patients, but it is how he has done this, that sets him apart. Few people go through their professional career and preside over and drive such significant changes. The development of services for people with CF in UHL will have Michael's name attributed to it, for some time to come. It’s a lasting legacy to his care for his patients, his resilience and determination. We wish his wife and Michael a long and happy retirement. I know from a recent call I had with him, that he has started that journey in good style. Thank you Michael. Owen Kirby, Chairperson TLC4CF

23 years ago my newly diagnosed son and I were very lucky to meet the wonderful Dr Mahony. At that time, he was the CF Consultant in Limerick along with the kind and caring CF Nurse Marion Lally. Back then the CF Paeds outpatient unit didn’t exist. Facilities for CF were poor. Dr Mahony rectified that and was the driving force in putting paeds CF care and facilities in place. He persisted until everything was up to his high standards. I am so grateful for his diligence and commitment to his CF patients. My boy ran in to trouble around age 10 with CF complications. Dr Mahony went above and beyond to source a treatment that would work for him to get him back to full health. He taught us well and due to his excellent care my son is living a healthy, happy life now. Dr Mahony also played a huge role in ensuring an urgent and very ambitious goal was fulfilled – the now state of the art adult CF inpatient and outpatient units. Even when his Pead patients had moved on to adult services he had their health and best interests at heart. Our family, and in particular my son are so very grateful to Dr Mahony. “Michael, we wish you and Kay a wonderful, happy and adventurous retirement, Thank You” The Clancy Family (Clare)

RESEARCH UPDATE

Cystic Fibrosis Ireland caught up with Jen Balfe, who is a researcher in the RCSI. She is also a Mam of two and a person with CF.

Project Title: Exploring the experiences of People with Cystic Fibrosis and the healthcare professionals involved in their care during the pre-conception to post-partum period.

In September 2020 I received a four-year scholarship from the Irish Research Council, under the Enterprise Partnership Scheme, which is co-funded by CF Ireland. The project aims to explore the experiences of people with Cystic Fibrosis (pwCF) and the healthcare professionals (HCPs) involved in their care, as they navigate fertility and pregnancy journeys in Ireland.

I personally would have loved a tangible booklet or companion guide while I was pregnant, to address the specific requirements and challenges involved in being pregnant with CF. This project was born out of the desire to create such a resource for pwCF who are thinking about or experiencing pregnancy in the future.

My supervisory team consists of Dr Aisling Walsh, a lecturer and researcher in the RCSI, Dr Jennifer Donnelly a consultant obstetrician in maternal and fetal medicine, who has experience providing care to pwCF during pregnancy in the Rotunda Hospital and Dr Sarah Tecklenborg, Senior Research and Policy Officer in CF Ireland. I feel incredibly lucky to have such a passionate and committed team behind me and I am already learning so much.

As an obstetrician who sees women with cystic fibrosis trying to become pregnant and negotiating the joys and challenges of pregnancy, I think this project will help improve the experience for women with CF, through better communication of needs and through clearer pathways of care.

Dr Jennifer Donnelly

The research will involve different stages and processes. To date I have developed a protocol for a systematic review of the literature in this area and we are currently developing a protocol for the study which will begin in 2022. This will involve surveys and interviews with pwCF and the HCPs involved in their care. We will also investigate how pregnancy is experienced by people in other patient populations, with a particular focus on people with epilepsy. This will be done with a view to learning from areas of crossover in managing care for pwCF.

In order to reflect the voices of pwCF, we will be establishing a PPI panel to help guide the study.

The panel will be open to women with CF from Ireland, who have accessed or utilised fertility or maternity services. Panellists will be over 18 years of age and will be willing to provide feedback and guidance on the research project, using their experience to enhance the research study.

Despite my personal experience as a pwCF, I am acutely aware that it is an experience which is unique to me. Given the diversity of experience in Cystic Fibrosis, I want to learn from as many different people with CF to ensure that a collective and representative voice is present in the study. The voices of people with CF and the healthcare professionals involved in their care are vital as they are the ones that can help shed a light on the experience of fertility and pregnancy in Ireland.

It is a really exciting time to be working in the field of CF research in Ireland. The landscape of health in CF has changed dramatically for many, with the use of CFTR modulator therapies and improved treatments. As a result of this changed landscape, more pwCF are becoming parents and raising families. Finding out the priorities and needs of pwCF and their HCPs during this important period, and reflecting them so that they can inform policy and planning is something that drives me.

In the future I hope to continue researching questions that are important to pwCF by listening to others in the CF community and learning from their experiences.

“I am delighted to be part of this exciting and important study, which will make a positive contribution to the experience of people with Cystic Fibrosis during the pre-conception to the postpartum period. Not only will the results be beneficial to women and families with CF, it will also assist health professionals as they deliver health services to women with CF during this period.”

Dr Aisling Walsh

A doggy tale

How vaccination for your dog may benefit you.

Professor John E. Moore, Dr Jacqueline C. Rendall and Professor Beverley C. Millar

Northern Ireland Adult Cystic Fibrosis Centre

One vaccine that your vet may suggest that your dog should get is the vaccine for Kennel Cough. The common cause of Kennel Cough is the bacterium called, Bordetella bronchiseptica, which may cause respiratory disease in small companion animals, as well as in certain human vulnerable groups, including those with CF. The good news for PwCF is that these infections in dogs can be prevented through routine vaccination against Kennel Cough, which PwCF should discuss with their vet. Also, infection from Bordetella bronchiseptica in PwCF is rare and if present, can be treated with appropriate antibiotics.

Licenced veterinary bacterial vaccines for Bordetella bronchiseptica (Kennel Cough) are widely available for dogs, which utilise live attenuated bacteria, which are usually administered to the dog via their nose. The presence of these live vaccine bacteria, now residing in the dog’s upper respiratry tract, is detected by the dog’s immune system, which then produces antibodies against the live vaccine bacteria and so the dog becomes immune to the vaccine strain, which also protects against Kennel Cough bacteria found in other non-vaccinated dogs. The live vaccine bacteria can live in the vaccinated dog’s nose for anywhere between 35 days up to 11 weeks,2 and in certain circumstances in the dog’s poo for up to 70 days.2 During this shedding period, these bacteria may be excreted from the vaccinated dog’s nasal and upper respiratory tract secretions, to other dogs, their owners, fomites and the environment. The close social bond between puppy and child with CF makes zoonotic transmission more likely from a vaccinated animal, which is shedding live vaccine bacteria.

Recently, a new dead vaccine for Kennel Cough (Nobivac Respira Bb) in dogs has now become available for use with vets in Ireland, which utilises a different vaccine strategy, not requiring the use of live vaccine bacteria, but alternatively uses bacterial proteins, which are dead and without risk of infection to dog or owner.3 This vaccine is administered subcutaneously, which is better tolerated by the dog than administration up the nose and can be given to puppies from the age of six weeks onwards. Given that there are no live bacteria involved with this vaccine, the dog does not shed any such bacteria and hence there is no potential infection risk from the vaccine to the PwCF owner. The advantage of this vaccine is that there is no need for social distancing/exclusion during the shedding period between PwCF owner and dog because of vacinne-related issues.

PwCF should understand the small but potential infection risk from Bordetella bronchiseptica to themselves of using the live vaccines for Kennel Cough in their dogs and should therefore consider to have their dog vaccinated for Kennel Cough with the new dead vaccine, which negates the risk of infection. Where a live Kennel Cough vaccine has recently been adminsitered to their dog, then the PwCF should check with their vet with regard to the time of shedding of the vaccine bacteria in their dog and follow good hygiene precautions during this period to minimise potential transmission from vaccinated dog to PwCF. This may be mitigated through (i) social distancing with the dog, (ii) avoidance of letting the dog lick the face and (iii) good hand and canine respiratory hygiene, in all dog-related