‘It’s like an exclusive club that no family wants to be part of’

For two weeks after Andrea and Padraic Smith brought their third child, Jake, home from the maternity unit of Cavan General Hospital, everything seemed perfect. He had passed his postnatal checks “with flying colours” before discharge and was settling into life at home with his two older siblings, Aoibhín and Joseph.

But then his parents noticed a small, soft lump around the back of the top of Jake’s head. A public health nurse suggested it was probably just a birthmark, but that didn’t stop Andrea worrying. “I just got paranoid about it,” she says. “I was really over-protective of him – I wouldn’t let anybody hold him, look at him. I really minded him.”

Not having been like this with her other two babies, she was told that it was more than likely due to postnatal depression.

To put Andrea’s mind at rest over that Christmas of 2014, Padraic suggested on December 23rd that they take Jake to the GP to have him checked. He thought this would enable her to relax, as everything else seemed fine with their four-week-old son, apart from her “paranoia”.

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The GP sent them on to Cavan hospital to get the lump scanned, just to be sure all was well. At that stage, on the afternoon of December 23rd, Andrea recalls how many of the non-essential staff were heading home for the festive break, and she and Padraic were “just concerned about getting the last of the Christmas shopping and maybe a bit of fresh fruit and veg in”. Little did they know such relatively trivial matters were about to be banished from their minds by devastating news.

‘On some level I probably knew – I don’t know if it was mother’s instinct or sixth sense’

The scan in Cavan indicated a “mass” on Jake’s brain and they were told he needed to be transferred immediately by ambulance to Temple Street Children’s Hospital in Dublin, where he was then scheduled for an MRI first thing the following morning. The results came through at lunchtime on Christmas Eve and showed Jake had a tumour on the brain, about the size of a man’s fist – roughly half the size of his brain. The couple were advised to go home with Jake, to try to enjoy Christmas, and he would be booked in for surgery on St Stephen’s Day if there was an ICU bed available – there wasn’t, as it turned out.

“Christmas Day went in a blur; Aoibhín was six and Joseph was four, we still had to carry on as normal,” says Andrea. They had Jake baptised on December 26th and that morning noticed his head had started to swell. He had surgery to remove the tumour on December 28th, once an ICU bed had become available. The couple were told it was a grade four brain cancer – glioblastoma – and the expected survival rate was less than a year.

Looking back now on her worries before the GP visit that led on to the diagnosis, Andrea says: “On some level I probably knew – I don’t know if it was mother’s instinct or sixth sense.”

‘Flat-lined’

Jake became very ill after the tumour was removed. “He kept seizing non-stop in the hospital. At one stage he ‘flatlined’ and they had to call an emergency. I will never forget there were that many doctors just running through the corridors.”

The fitting of a shunt in the brain helped to stabilise Jake and he was discharged on January 23rd, 2015. The family had about 10 days at home in Lavey, Co Cavan, to “regroup” before Jake’s chemotherapy treatment started. His parents had been told it was their choice if they wanted him to have active treatment or to go straight to palliative care, as the tumour was so aggressive.

“That was a very difficult decision as you’re told he probably won’t survive until he’s one. We decided to give him a fighting chance.”

On February 3rd that year, Jake started a very intensive routine at CHI Crumlin, with two to three days of chemotherapy administered intravenously and then seven days off before the next session. For the next nine months, Jake was either in CHI Crumlin or Cavan General. A bad reaction to one of the chemo drugs landed him in ICU during that time. A short time before his first birthday, an MRI showed the tumour had grown back.

When doctors operated on Jake again in January 2016 to remove the regrowth of the tumour, they were only able to get about 75 per cent of it out. Then he was put on oral chemotherapy, which the medical staff thought would slow the tumour growth, but it didn’t.

As Jake turned two, his parents were told about a clinical trial of a gene inhibitor that might help but it also came with high risks. He would be the first child in the world to try it, so they didn’t know what the side effects might be. In some adult cases, there had been excessive bleeding in the tumour site.

Jake was more well in himself on the gene inhibitor, says Andrea. “Although it is a type of chemotherapy, the side effects aren’t as severe.” She and Padraic could administer the medication at home, with some assistance from visiting nurses supplied by LauraLynn and Jack and Jill. It also meant that they felt Jake was stable enough for them to use hospice care at LauraLynn House in south Dublin, which “was just a godsend”, says Andrea, who recalls how nervous she was the first time.

“You spend your time assessing every micro-twitch or vomit or symptom; you are so used to being on hyper-alert for him that when somebody else actually takes over, it is so hard just to switch that off. Especially when it has been on for two-plus years. We had to learn how to step back a little, which was very scary but the staff were absolutely amazing. It was all at our own pace.”

Paralysed

Jake has severe neurological issues. After the first operation to remove the tumour, he was paralysed all down the right side; he has to be fed through a tube into the stomach. Therapy has helped him develop some mobility and he is able to walk now, says Andrea. Although he is non-verbal, he communicates in other ways.

“He loves watching TV, but if he is not happy with the programme, he will bring over the remote and that is your cue to change the station. Or if he wants to go outside, he will bring over his shoes or he will walk to the front door and bang the door.

“He is just so happy and pleasant and laughs all the time. He’s just a little monkey. He has definitely changed the house profoundly. His older brother and sister don’t really remember a time when he wasn’t here.”

A time when their parents weren’t constantly “a step away from red alert” to get out of the house with everybody’s bag packed and on the road to Cavan hospital within 15 minutes, dropping Jake’s siblings to nana on the way.

‘So often they are stuck in the background and they never complain,’ Andrea says of Aoibhín and Joseph, now aged 14 and 12. ‘They have had to cancel so many things because Jake has been sick on the day’

The family can spend up to 15 nights a year in the LauraLynn hospice and booking short breaks there have enabled Andrea and Padraic to do “normal” things with their older two children, such as shopping for school uniforms, or First Communions/Confirmations and going to see Santa.

“So often they are stuck in the background and they never complain,” she says of Aoibhín and Joseph, now aged 14 and 12. “They have had to cancel so many things because Jake has been sick on the day. They get so used to everything revolving around Jake’s schedule.

“Although I do hate to say it, his needs come first because they are so intense and life-threatening. It is lovely to be able to put them first and dedicate 100 per cent of your time to them.”

Since Jake contracted acute hepatitis last November, causing liver and kidney failure, his clinical trial medication has been stopped. He was critically ill in ICU “but lucky enough, through typical Jake fashion, he fought back again against all the odds”.

Andrea thinks he might have picked the infection up on a family outing to the Wild Lights at Dublin Zoo, which they thought would be relatively safe, being outdoors. “You are caught between a rock and a hard place. You don’t want to cloister him too much, so that he doesn’t have any life experiences.”

Thousands of families

The Smiths are just one of several thousand families in Ireland who care around the clock at home for a child with a life-limiting condition. There is little recording of data here but, according to LauraLynn’s Model of Care (2019), extrapolations from UK figures suggest there are 3,840 children with life-limiting conditions in Ireland, about half of whom need palliative care. The organisation also estimates that at any one time, up to 720 children with life-limiting conditions would be classed as “unstable, deteriorating or at end of life”.

In this, Palliative Care Week (September 11th-17th), Liz O’Donoghue of LauraLynn explains how cancer accounts for just about 25 per cent of cases referred to the children’s hospice, which has supported children with 170 different diagnoses over the past 11 years. Having taken the job of referral development coordinator in June, she is looking at how more children with palliative care needs and their families might be able to access LauraLynn services, be that at home, in the hospice or at a hospital bedside.

“It’s like an exclusive club that nobody wants to be part of,” she says. “Across all conditions, there is that threshold of when do you make that referral to palliative care?” Yet, as a clinical nurse specialist with 20 years’ experience in children’s palliative care, on a two-year secondment from CHI Crumlin, she firmly believes that more families could benefit from earlier referrals to LauraLynn.

“The idea of bringing your child to a hospice is quite frightening,” she acknowledges. “For all these families, there is a high level of care that they deliver to their children on a daily basis and to pass that over to somebody else is quite daunting.”

Every stay in LauraLynn will have therapeutic benefits, says O’Donoghue, but she believes the “family camps”, where four or five families are matched for a programme of sessions and outings, bring “an extra charge of energy”. The peer connection, for parents, siblings and the affected child, is “massive”.

The Smiths attended a family camp in August and Andrea says it is “amazing” to be able to talk to other parents in similar situations. “When you have a child with a terminal illness or a child in palliative care, it is quite isolating.”

‘People have nothing to talk to you about because your whole life revolves around a sick child. Of course, people care, but you’ve no holidays to talk about; you don’t really have time to watch TV'

Before Jake was born, she worked full time for Dublin City Council, commuting up and down every day. “Then all of a sudden you’re not. You’re used to meeting with colleagues and going for lunch. You miss all that. You miss normal nights out with friends.

“Over seven years, you lose touch. People have nothing to talk to you about because your whole life revolves around a sick child. Of course, people care, but you’ve no holidays to talk about; you don’t really have time to watch TV. You kind of lose touch with the real world.”

‘We were exhausted’

Padraic’s business as a builder “went belly up”, she says, due to the demands of coping with Jake’s illness and long hospital stays, while raising two other children. She reckons that it was because the two of them were around that they managed without a HSE in-house care package until Jake was six. “I think they realised we needed help kind of badly at that stage. We were exhausted.” Now a night nurse comes in from 11pm to 7am to allow them to sleep.

An MRI last February showed that Jake’s tumour had grown and on the day we talk, Andrea has just been on to CHI Crumlin to see if there was any news yet of his latest scan a couple of weeks previously. “They call it ‘scanxiety’ – that waiting for results. People don’t realise how intense it is waiting and you have so many people asking, ‘any news?’”

Meanwhile, Jake is “happy out”, she reports, “enjoying the relief of not being in intensive treatment”. The minor side effects, the nausea, vomiting and exhaustion, which he has experienced continuously for the past seven years, have gone. “He has put on weight and developmentally come on a little bit better.

“We take it day by day,” she adds. “If you look too far into the future, it can be very depressing. It doesn’t put you in the best frame of mind to tackle day by day because you’re wondering, is he going to be here for his birthday (November 20th), is he going to be here for Christmas?”

All the family can do, to paraphrase LauraLynn’s motto, is to make the most of the short and precious life in their midst.

Children’s Hospice Care by numbers

• 3,800 children estimated to be living with life-limiting conditions in Ireland.
• 50 per cent of these are believed to have palliative care needs.
• 63 new families have been enrolled for LauraLynn hospice services so far this year.
• 232 families have received some level of its hospice care in 2022 to the end of August.
• 40 children who attended LauraLynn at some stage have died so far this year – at home, in the hospice or in hospital.