“The reward is in helping the children to live a good life, supporting them, and their families, while they are living. But it’s also ensuring that they have a good death, no matter where that occurs, whether it’s at home, in hospital or at a hospice,” according to Irene O’Brien, Clinical Nurse Coordinator for Children with Life-Limiting Conditions.
Speaking as the HSE recently launched My Story – a National Patient-held Folder for Children and Young People with Life-Limiting conditions, Irene reflected on the often challenging work she does and the many professionals with whom she co-ordinates services to ensure children and their families are fully supported during a difficult time.
Based in Drogheda, Irene coordinates care in Louth, Meath Monaghan and Cavan. She continues to explain that “these children have been diagnosed with conditions, often at birth, from which ultimately they will not survive.”
She explains that children linked with her service can be diagnosed with a life-limiting condition at any stage during their childhood years. The range of illnesses is vast but includes malignant and non-malignant conditions - a large number of cases involve children who have complex disabilities.
“Life-limiting essentially means that a child or young person is not expected to live beyond their teenage or early adult years. Many enjoy a good quality of life with the support of their primary care services and paediatric team who know them well.
“Some children we meet as new-borns sadly will live short lives and some children have rapidly deteriorating conditions. Other children can have a challenging start at diagnosis but then stabilise to live longer.
Irene clarifies that additional support from the CNC service is only considered when there are signs that a child’s clinical condition is starting to deteriorate, where their symptoms are becoming more challenging and the family could now do with more support: “Our governance lies within paediatric services so our direct link is with children’s services at CHI and local paediatric units, and along with our clinical experience this is generally welcome.”
One significant challenge is that “lifespan is often difficult to predict in a lot of these cases. Children can live for a number - and in some cases – many years. And of course, many developments in medicine and technical supports have extended the lifespan of children who would previously have died a lot younger.
“But in living longer, their life challenges and clinical support needs are greater, with often new or more troublesome symptoms. A ‘hospital at home’ environment is often referred to here – it generally involves children at home with high support needs who require nursing support, vast amounts of equipment and often respiratory supports like non-invasive ventilation.
“And yet quality of life is still achievable, family life is possible and with good co-ordination of care and collaborative teamwork, many of these children get to stay at home and when the time comes, have a good death at home with their family around them.”
With a background in paediatric nursing, Irene developed an interest in this area when she was working at Crumlin Hospital with neonatal babies with heart conditions and complex medical needs. After a return to college to complete a degree in palliative care, she was subsequently appointed as the first regional Clinical Nurse Coordinator for Children with Life-Limiting Conditions in the North East in 2011.
Her first patient, she explains, was “a little two year old boy who had recently been diagnosed with a life-limiting condition.” When Irene first met him, his parents were devastated - facing an uncertain life ahead. Over the next number of years the little boy faced many challenges including reduced respiratory function and spinal surgeries.
Following a long period on a ventilator for a severe infection, Irene explains that “it was considered that it wasn’t in the little boy’s best interests to keep him on the ventilator any longer. He was extubated with the expectation that he would live for a very short time afterwards.” Despite all expectations, the boy lived – for another seven years. However, from that time it was agreed with his parents that no further surgeries or resuscitative measures would be performed for him in the future.
Irene explains that although his symptoms progressed to “spinal complications in the absence of surgery, poor skin integrity and very difficult pain management - he had a great quality of life.
“He benefited from a significant nursing and carer support package and input from multiple community teams. Despite requiring 24-hour non-invasive ventilation and multiple medical challenges, his GP managed his medical needs largely at home with the support of the paediatrics and palliative care teams. He had minimal hospital admissions in those seven years and in the end had a very peaceful death at home with his parents.
“Even though that boy was the first patient I had, he only died a few months ago. I knew him for 12 of my 13 years in this job and he and his family required CNC support for all of that time. There were upsets and challenges along the way. It is important for others to know that children can be that vulnerable and that unwell but still have a good life at home - it can happen with adequate support.” His parents are very proud of the happy life he enjoyed in those last years, despite all the challenges, and are keen for Irene to talk about him and for others to learn from his story.
In clarifying the benefits and challenges of her role, Irene reflects that “parents’ support needs are huge and to live in this life of uncertainty is very difficult. Along with their emotional burden, they deal with multiple professional teams, nurses and carers, unending communication and frequently worry about doing their best for their child. Professional support needs are also huge – although it’s our area of care, many are not familiar with life-limiting conditions and even less comfortable with children with palliative and end of life support needs. Empowering and supporting professionals to be confident in their role with the family is really important.”
When she first started out Irene explains that there were not as many HSE and voluntary services as there are now, communication between services was poor and the need for care coordination was really evident.
Over the last number of years, support networks for families have improved greatly: “The approval process for primary care home care packages is now standardised, there has been an increase in specialist nursing roles in both the hospital and community services, and of course the development of LauraLynn Hospice services has been invaluable. As co-ordinators, we link in with all the teams and it is reassuring for families that there is one person who knows everyone. I would often say – every job is not my job but I will generally know whose job it is.”
The work Irene does is vital and with currently 19 Clinical Nurse Coordinators across the country, their work has a direct impact on lives and homes in every county. The role is described as really vast and the level of CNC input can vary greatly from case to case, guided by the children’s clinical needs but also by families’ wishes. “It is also okay in some cases to be the safety net in the background, to have met the family and professional teams, to have appropriate plans in place and then to allow the family to breathe and have some normality. They will know when they need support.”
Asked about how, in her experience, parents cope with the challenge of looking after a child with a life-limiting condition, Irene explains that “parents often talk of the ‘sink or swim’ scenario and that there is no choice but to swim, to find the strength to move forward, do their best for their child and family. Most parents would say they get up and they address the day as any other day, as in it’s their norm. Many have the mind-set of not thinking too far ahead, they feel that dealing with the day to day stuff is hard enough without spending time thinking of losing their child at some unknown time ahead – they just can’t go there.
“There are often other siblings to consider too - busy homes – so parents just get on with it. Life has its way of minding them too, it’s good to see laughter and humour surface. Even at sad times, there are still some giddy moments and days with some fun.”
Reflecting on the challenge of her role, Irene says it is “ultimately very fulfilling in the relationships that you build and the people that you help. You know what pathways to use and who to go to for various supports and needs. You can pull everything together and work as a team for the child and family, while at the same time respect everyone’s role. While helping to support and co-ordinate care at a child’s end of life is an important part of our role, the reward for me personally is helping the child and family to live a good life – supporting them while they are living.”
While their support role is significant and welcomed by most, Irene explains that the CNC service is not an emergency service, there is no out of hours or weekend cover. With that in mind, a significant part of supporting families is around educating and empowering parents in their carer role “to give them advice on what to do and when, who people are and what they do and who to contact when they need help, particularly out of hours and over the weekend.”
Although families get to know their CNC well, even over a short period of time, Irene stresses that they need to ensure that no family becomes solely dependent on them for support: “Things can’t fall askew because they are not around. Co-ordinated multidisciplinary team support is central to providing safe and quality care for these families and sharing of information is key to the continuity of that care.”
And it was that need that led to the development of the HSE My Story - The National Patient-held Folder for Children and Young People. “What we required was a nationally recognised communication resource to host important information in relation to the care of the child – for example prescriptions, medical updates, Emergency Care Plans and directives related to a child’s resuscitation status. It also required an ‘All About Me’ section that focused on the child’s story and specific care needs – the real ‘get to know me’ section of the folder.
“As some of the community organisations needed their own documentation in the child’s home, the challenge for us was to develop one central communication hub which was accessible to all involved in caring for the child. Promoted as the child’s ‘grab it and go’ folder, the key objective for My Story was for it to be the primary folder that families share with professionals at appointments, hospital admissions, during ambulance transfers, and with their community teams.
“Key to the success of the My Story was always going to be user engagement and with that in mind, the front of the folder was designed to clearly define its purpose to users, ‘Me, My Family and My Professional Team, Sharing What Matters to Me.’ In the child’s voice - it’s me, it’s my family and it’s my professional team’s responsibility to share everything about my condition and what matters to me, right now.
“And of course, children and young people are encouraged to contribute to the folder when they can, to include personal information, photos and their likes and dislikes so it is truly their story. Ensuring the information contained within the folder is up to date remains one of the biggest challenges for all involved.
“The anticipated benefits of the HSE national patient-held folder are many and in just a short time the positive feedback we have received is very encouraging. The ease of access to patient information at point of contact is proving invaluable. As a notable example, the phrase ‘mammy knows everything’ is often proclaimed, but what if mammy is not there?
“My Story now provides reassurance to daddy, granny, older siblings or to any carer that they have that everything at hand or that they can simply just pass the folder over to professionals if required. This has also provided an additional benefit, for some, of increasing the net of family and friends who now feel more confident and safer in caring for these children and subsequently allowing some respite time for parents.
“We have also been fortunate to have early examples of the value of My Story for families transitioning from children’s to adult healthcare services. The anticipation of this transition process causes enormous stress and anxiety for both young people and their parents. For parents of children with disabilities, the complexity of their child’s care needs are difficult to communicate and often challenging for professionals to understand.
“For competent young people, the change from their parents steering their healthcare journey and decision making to now, as a young adult, being responsible for all of their health information is really daunting. We had recent example of how the MY STORY was introduced to a very shy 16 year old girl who always looked to her mother to speak and answer for her when professionals were present. She was encouraged to fill the folder with information and resources to suit her needs and, with time, had the confidence to attend her first adult appointment with her My Story as a prop and without mammy.”
Outlining how invaluable the folder was in an acute emergency case, Irene shares the story of a teenage boy who was still linked with Children’s Services at Crumlin Hospital. However, when he subsequently deteriorated at home he was over 16 years and was taken to his nearest adult hospital: “Although the team there had never met or treated him before, his family had his My Story folder with them which contained everything necessary and relevant to him - his medication scripts, resuscitation status and all his care plans. He was really ill and sadly only had days left to live. However, having his My Story folder enabled swift attention from the medical team, appropriate family support and the provision of quality end of life care for the young boy. And that’s exactly where My Story really came into its own.”
The My Story project has been jointly led by HSE Primary Care and Palliative Care divisions by assigning joint project leads – Margaret Rafferty, Project Manager Primary Care, along with Irene: “While the two of us co-chaired the project, there was an established working group and the project involved significant consultation and collaboration with the key stakeholders nationally. The folder was designed to ensure its suitability for use by all paediatric specialities for their cohort of patients.”
While it is a physical folder for now, Irene explains that at some stage in the future consideration on “how to transform it into an electronic or digital resource will be necessary. There are positive developments to in relation to the new HSE app. However, at this stage there is no facility to download patient documents or prescriptions to the app to provide up to date direction for professionals, one of the key functions of MY STORY. The new CHI hospital will be fully digital, however this will be confined to CHI services. It will be some time yet before all national paediatrics services convert to a digital system.
“Our primary focus for now is on promoting education and awareness of the hard copy folder. Margaret and I, along with the CNC group, have been engaged in both virtual and in person education sessions with the professional teams and organisations to whom parents or young people will be presenting their folder to. We need to progress now to MY STORY being universally known, understood and used to maintain current and quality patient information at point of contact, with the ultimate goal of ensuring safe and appropriate care provision for the children and young people involved.”
The folders are now in circulation, have been given to many families and are in use nationally. MY STORY is incorporated in the National Communication Pathway of Children’s DNACPR’s to professionals working with the National Ambulance Service and Dublin Fire Brigade. It also is a significant communication tool in the national discharge and care pathways for children with life-limiting conditions in the community, developed by the HSE in 2020. The project received a commendation under the Excellence in Quality and Patient Safety category of the HSE Excellence Awards 2024. The printable format of the My Story is now available along with a comprehensive educational presentation on www.hse.ie/mystory