Tina Priestley was six months pregnant with her fourth child when a routine scan showed that the baby’s head was much bigger than it should be.

The news got worse at each subsequent scan, and she and her husband, Derek, were told that the pregnancy might not go full term, as the baby had a rare chromosomal disorder, Tetrasomy 9P.

Tina was induced in the National Maternity Hospital (NMH) at 38 weeks and the couple opted for a "do not resuscitate" order for their baby.

“We felt if this little man was going to be here for only a few minutes, we would just cuddle him and love him for those few minutes, rather than have him whisked away and prodded and poked. So it was a calm birth in one sense but he was born alive and needed no intervention, just [lung] suction. His heart rate came right up and he was what they said, the ‘little miracle baby’.”

Three weeks later, Tina walked out of the NMH with baby Bobby, a photocopied piece of paper about infant resuscitation and a bag of feeding tubes. That was it, except for a follow-up, outpatient appointment in eight weeks’ time.

"It was daunting," she says. As an experienced mother she thought she would cope but, within hours of being back at home in Naas, Co Kildare, it was clear it was going to be extremely difficult.

“He was being tube fed, and it would take nearly an hour to feed him and he was being fed every three hours.” He also needed to be watched all the time.

“Pride gets in the way a little bit,” she admits. But luckily one of the nurses in the NMH neo-natal unit had given her the number of the Jack & Jill Children’s Foundation.

After 24 hours, Tina faced up to the fact that she had not only Bobby, but also three other children – then aged seven, six and four – a husband and herself to look after, and she needed help.

“If I had crumbled under the strain, there would have been a domino effect.”

She rang Jack & Jill, who sent a nurse manager to her the following day and two days later she had a paediatric nurse in the home helping her.

“It was more than respite; it was a feeling that we can get through this,” she says. In addition to the 40 nursing hours a month, it was simple things like advice on what tax form to fill in, what department to ring if they needed new feeding tubes and the sense that Jack & Jill was there for them.

That was 2008 and there has been significant progress in children’s palliative care since. A needs assessment had been done in 2005 but it took another five years before a national policy was created.

Tina, who is a parent representative on the national development committee of Paediatric Palliative Care, says she would like to think that any parent getting a “life-limiting” diagnosis for their child now would have a “road map” for what’s ahead.

It is estimated that there are up to 4,000 children living in Ireland with a life-limiting condition, which is defined as one for which there is currently no cure available and the likelihood is that the condition will lead to the child dying prematurely.

Affected children can range from a newborn with severe neurological disability or a child with a progressive disease such as Batten’s, to teenagers with cancer or heart failure. About 350 of these children will die each year.

Fledgling speciality

Children's palliative care is still a fledgling, medical speciality, says Mary Devins, the only consultant paediatrician in the Republic to have done further training in this area. People view it as end-of-life care but that's only a tiny bit of it, she explains.

Based in Our Lady’s Children’s Hospital, Crumlin, Devins is co-chairing a two-day international conference on children’s palliative care in Dublin next month. Entitled “Building Bridges – Home, Hospital and Hospice”, it is aimed at raising awareness of how seamless care between the three makes sense – medically, emotionally and financially.

It is also a chance to showcase the work that is being done in Ireland, says Devins. Various agencies, including the HSE, Jack & Jill, the Irish Hospice Foundation, LauraLynn, the Irish Association for Palliative Care, Mercy Hospital Fundraising, NUI Galway and Our Lady's Children's Hospital Crumlin, are collaborating to host the conference.

"Children's palliative care is really about treating symptoms so that the child can have great fun and be as well as they can possibly be," says conference co-chairwoman Claire Quinn. A lecturer in children's palliative care at NUI Galway and part-time head of research at LauraLynn, she has both personal and professional experience of what's involved.

Quinn was working in Limerick as a palliative care nurse when her first child, Olivia, was born in 2000 with an undiagnosed neurological condition and lived until she was five and a half years old.

“I was able to see firsthand all the challenges for parents,” she says. “I was expecting the system to be the same for children who were dying. But there was absolutely nothing for her, very little.”

During the last six months of Olivia’s life, she came across a lot of willing nurses but they were all unsure of what they were doing. “I used to think, ‘this wouldn’t be the same if she was a 70-year-old with lung cancer’.”

While Olivia had “a beautiful death” at home, Quinn, who has since had two more children, knew her daughter had been lucky that her mother was able to “fight her corner”.

This experience was “a big motivating factor” for her to specialise in the education of nurses in children’s palliative care at NUI Galway and she is delighted that a Masters course is being introduced there this September. “It is wonderful that we have got recognition that it is a growing speciality and that we need practitioners,” says Quinn.

National policy

Medicine has got very good at helping babies and children with very complex needs and so they are living longer, says Devins. But “sadly we are starting to see more children die in hospital”. It can be hard for doctors to tell whether an illness is just a “hump” the child will get over, she explains, or something that will prove fatal.

Devins’s post at Crumlin, which she took up in May 2011, was created on recommendation of the national policy on children’s palliative care. For the first three and a half years her salary was paid for by the Irish Hospice Foundation but the HSE is now funding it.

Before Devins started, there were about 50 new referrals a year to the palliative care team in Crumlin. Last year this had risen to 112 and they were “directly involved in 67 deaths”, the vast majority of which were at home.

There are eight outreach nurses working in children's palliative care, operating from hospitals in Dublin, Waterford, Mullingar, Cork, Limerick, Galway and Drogheda.

Appointment of a further two is imminent, with one planned for Letterkenny and another needed in Dublin. They are the link between the hospital and the community and help to arrange home nursing.

“The HSE has got much better at approving [home care] packages but one of the sad things is that everybody is really stuck for paediatric nurses,” says Devins.

Adult palliative teams do get involved in end-of-life care of children and, while there is a certain amount of commonality, says Devins, there are also differences. A large part of her job is phone support for these care teams around the country.

She is also involved with any child attending Crumlin with a life-limiting condition, supporting the family and trying to get the child home. In addition she does one session a week at the Coombe maternity hospital, where she may see newborns, or women ante-natally whose babies have been diagnosed through scans.

Quality of life

Devins wants to make sure the focus of care is on quality of life and in keeping with the family’s wishes as much as possible.

“Our main responsibility is to the child and to make sure that everything is done with the child’s benefit at heart. But as a child, how your parents and your siblings are feeling actually affects how you feel. If you have a very upset parent, it can affect how a child perceives pain or symptoms.”

This whole-family approach attracted Devins to paediatrics but the seed for her later specialisation in palliative care was planted when she was a junior doctor in Sligo.

She recalls how the surgeon called in a member of a recently established palliative team to see a woman who had horrendous pain with an advanced pancreatic tumour.

“He sat and talked to her for a long time and prescribed medication and it was like somebody had flicked a switch. The next day it was a completely different woman and I thought he performed magic.”

Working in paediatrics, she came across a lot of children like that. “We can’t change the awful thing that is happening but you can do something, even small, that will make a huge difference to that child and that family. It is so satisfying,” explains Devins who, since becoming a mother three years ago, admits it has become harder – “but hopefully it has made me better”.

National access

The dream, she adds, “would be that children’s palliative care would be the same no matter where you are in the country. If you’re a little baby at the very tip of the Beara peninsula, on Dursey Island, you can have the same palliative care as somebody who lives next door to Crumlin.”

While she concedes there is still some way to go, she believes “the will is there”.

The Priestley family made the most of the 13 months they had with Bobby, including taking him away on a memorable summer holiday to France.

“He was a very tactile little fella. He was partially sighted and almost completely deaf, so he spent most of his time up in one of our arms. He slept in the bed between the two of us; there was usually one of the other kids in there as well.

“That was the nice thing; when Bobby was in hospital you weren’t able to cuddle him,” she says.

Bobby had severe hydrocephalus and, while he had had a shunt fitted, doctors identified an over-sized nodule in the back of his brain producing the excess fluid. Just before Christmas 2008, they tried a pioneering procedure to “zap” it.

“The irony is that the procedure actually worked but there was a risk of stroke and, unfortunately, he suffered a major stroke,” Tina says.

It was then they were given a palliative diagnosis and, when Bobby returned home from hospital, what Tina describes as “the swat team” went into action, co-ordinated by Crumlin.

The middle of February proved to be a tragic time for the family as, on the 14th, Derek’s father, a fit and healthy man, died in a fall down the stairs. Two days after his funeral, it was Bobby’s time to go.

That Saturday morning the other children had gone for a walk with friends of the family and it was just Tina and Derek at home with Bobby, along with the Jack & Jill nurse.

“He was in my husband’s arms, sitting on the sofa,” Tina recalls, when the nurse said she thought death was imminent and talked them through what to expect.

Bobby always used to love when Tina sang to him, “so I put my cheek right up beside him when he was passing away and I sang ‘Edelweiss’ to him and the breathing stopped – it was very serene”.

“When the kids came back from their walk, he was in our arms and they had a little cuddle and gradually word went out and people came to the house and they had a little cuddle. It was as nice as it could be. Whereas, if we had been in a hospital, he would have been whisked off to a mortuary and we would be looking at him on a table in a room.”

This is why Tina plays her part in raising awareness and serves on the “hard-working” national palliative care committee to give a parent’s perspective.

She will also speak at the upcoming conference and jokes about the daunting prospect of addressing an audience of high-powered health professionals: “The only letters I have after my name are MUM!”

The Second International Children’s Palliative Care conference takes place in Farmleigh, Phoenix Park, Dublin on April 22nd and 23rd. For more details see cpcconf.ie

swayman@irishtimes.com