The ‘chronic sorrow’ of knowing your child will die

The recent birth of their second child was bittersweet for Deirdre and Tristan O’Gorman: while of course they were overjoyed, his safe arrival reinforced for them how unfortunate his big brother is.

Finn O’Gorman, now aged three, was born with a rare genetic condition that resulted in a “smooth brain” and severe neurological impairment. There had been concerns about him during the pregnancy, from the 20-week scan onwards, but subsequent scans and tests were inclusive and the couple’s consultant was “cautiously optimistic” that all might be well.

It was only two days after being delivered by Caesarean section at 37 weeks, weighing just 4lb 2oz, that Finn was diagnosed with the life-limiting condition of lissencephaly, along with microcephaly – a smaller than normal head size. His parents were told that life expectancy was about two years, maybe longer.

Sometimes we would fantasise that they had got things wrong because he was doing everything a normal baby should

“He won’t be able to walk or talk; may not be sociable; profound developmental delay; incurable epilepsy; failure to thrive; persistent chest infections; pneumonia” – Tristan recalls the distressing litany on the outlook for their first baby. “We were devastated to say the least, we didn’t know what to expect.”

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When they brought Finn home to Whitegate in Cork, after he had spent two weeks in the neo-natal unit, he seemed like any other baby. "Sometimes we would fantasise that they had got things wrong because he was doing everything a normal baby should, he was drinking his bottle and crying," says Tristan.

But at about three months, Finn started having seizures and there has been little normality since. He has had numerous hospital admissions, ranging from a few days to months at a time, and around-the-clock care is required for his multiple and complex medical needs.

Deirdre and Tristan made a conscious decision to try to keep a sense of positivity about their family life – “although it’s not always possible to adopt that attitude because you can get really mired down in the awfulness of the situation,” Tristan admits.

“If we were to focus on Finn’s condition, you would be in a constant state of depression. We try to be as normal as we can and not talk about him in those terms. It’s hard because his condition does subsume everything, depending on whether he’s well or not.”

Initially, Tristan didn’t want to know anything about his son’s condition and Deirdre was the opposite, researching the minutiae. “In our own ways, we were both obsessed with what he had,” Tristan says. “That wasn’t healthy and we had to take a step back and think of Finn in terms of day to day.”

Asleep, “he looks like a normal, handsome little boy”. But when they take him out of the house, with all his contraptions, “and you see people staring, that’s when it hits home”.

A happy child

Yet generally Finn is a happy child, always smiling, “especially when he is well. He is very expressive; he can’t talk but he lets us know what he’s thinking,” says Tristan. Their eldest son loves the social aspect of the LauraLynn hospice in Dublin, where the family go twice a year for a total of 15 nights’ respite.

"We know it's not just a break for us, it's a treat for him as well." Back home, Finn thrives on interaction with nurses from Jack & Jill and the HSE, along with other professionals, such as Cope Foundation therapists, who all contribute to trying to make the best of life for him and his parents.

Now there’s a sibling too. After more than two years of genetic testing, the couple were told that in all probability Finn’s condition was not hereditary but rather a random genetic mutation. While they were delighted to be given the go-ahead to have more children, it was a reminder of how Finn had been on the receiving end of what his father describes as “the worst lottery results ever”.

Finn is going to pass away one day and we feel guilty, almost, for putting that burden on Noah

Their second son, Noah, was born on September 22nd last, weighing eight pounds, and “straightaway we could see the difference”, says Tristan. Things that Finn was never able to do, Noah was doing already.

It has provoked a new wave of grief about what their first-born is missing out on. “Obviously, we couldn’t be more thrilled to have Noah and then you look at Finn, it doesn’t seem to be fair.”

They also worry about how their younger son will cope with bereavement, be it sooner or later, as new life has brought their first-born’s mortality into sharper focus.

“Finn is going to pass away one day and we feel guilty, almost, for putting that burden on Noah, especially if he is old enough to understand,” says Tristan who, like Deirdre, is originally from Thurles, Co Tipperary, so they have no family support nearby.

“We were in our own little bubble and now that bubble has got a little bit bigger. It is forcing us to look at the outside world a little bit more. Everything we do is for Finn and most of the people that we interact with on a daily basis are involved in Finn and his care.

“Now with Noah, we are thinking for the first time about schools, taking him for his shots, things that normal families think about all the time – but then casting one eye back at Finn.”

It's the "chronic sorrow" that families such as the O'Gormans have to live with. That's a quote recalled by US consultant Dr Julie Hauer, who specialises in children's palliative care and who will be a keynote speaker at an upcoming interdisciplinary conference in Galway in November.

Serious brain injuries

She works at Boston Children's Hospital with patients with serious brain injuries or illness and she sees that while their parents have many joyous experiences, there is "chronic sorrow" when they see other, typical children reach milestones that they won't get a chance to celebrate with their son or daughter.

“It doesn’t mean the parent is sad every day,” she says, but “these life events are points in time that they get reminded of what their child won’t be.”

Dr Joanne Balfe, consultant paediatrician at Tallaght hospital and LauraLynn, has seen how the birth of a second child who is healthy highlights the impairment of the first child. The joy of having a new-born is "certainly bittersweet" for these families, she observes.

She is the co-chair of the Galway event – Ireland's fourth International Children's Palliative Care Conference (cpcconf.ie) with a focus on contemporary challenges. The care of today's children with complex medical needs is a new frontier, as they are surviving much longer and some will transfer into adult services.

It's not fair to look to parents to take the lead and make them feel responsible for decisions in a way that would be a burden

Their longer life-spans throws up new medical challenges, as well as ethical and emotional questions for health professionals and families alike around treatment plans and the prolonging of life. Shared decision-making between health staff and parents throughout is the optimum but, as Hauer says, “it’s complicated and emotionally laden”.

Balfe believes it’s the job of the healthcare team to determine the best course of action for a child and family by listening carefully to what’s really important for them. Most parents will say they want their child to live as long as possible, but also to be as well as possible.

“I always say we have to help parents choose the path of least regret,” she explains. “It’s not fair to look to parents to take the lead and make them feel responsible for decisions in a way that would be a burden.”

Hauer is assistant professor of paediatrics at Harvard Medical School and author of a guide for both professionals and parents entitled Caring for Children Who Have Severe Neurological Impairment: A Life with Grace. The most important chapter of that book for her, she says, was on decision-making.

For parents, who “are the child’s best experts”, it’s likely to be their first experience of paediatric palliative care. Professionals have to be sensitive in how they apply their knowledge and can’t rush families in coming to terms with the different stages.

Discussions are best focused on the parental perspective, Hauer suggests. It comes down to “are we processing information where their intellect and their emotions are at, at that point in time? As opposed to making assumptions where they’re at.”

Parents should never feel they are making the decision to let their child go

Everybody wants to be a good parent to their child, she points out, and this brings a different sense of responsibility. They are asking themselves: “How can I be a good parent to this child to ensure that I don’t give up on them but make sure that I don’t make decisions that contribute to how I wouldn’t want my child’s life to unfold?” Healthcare staff can use their expertise, she says, to guide parents through that.

Palliative care teams believe that “parents should never feel they are making the decision to let their child go”. Rather it is a case of “we have collectively agreed how we desire the child to live and there can be a point in time where that life is not what makes sense for that child”.

The O’Gormans are all too familiar with those very difficult discussions about ongoing care and interventions at times of crisis.

Early on, when Tyrone Horne, clinical nurse coordinator for children with life-limiting conditions at Cork University Hospital, first offered them home nursing hours from Jack & Jill and the HSE, they turned them down.

“We assumed we would be able to manage,” says Tristan. “As things became more and more difficult for Finn, they gently encouraged us to avail of them and we did.”

Very bad period

When Finn was going through a very bad period with his epilepsy, before doctors found the right combination of medication to control the 70-80 seizures he was having a day, staff from Marymount Hospice stepped in quietly. At first, the couple were alarmed that a “palliative” service would be involved, having as it does connotations with “end of life”, but “we soon became aware that it had wider meanings”, says Tristan.

As Horne, who is the other co-chair of the palliative care conference, explains to families, “palliative” is derived from the Latin word “pallium”, which means cloak, and so interventions should be seen as “cloaking” the symptoms.

“Finn is not end-of-life,” says Tristan, “but he certainly does have palliative requirements.” But there have been three occasions so far when Tristan and Deirdre were warned he might not pull through: firstly, during the period of uncontrollable epilepsy; the second time he had septicaemia and the third time the flu.

The couple found it extremely tough discussing the question of a Do Not Resuscitate (DNR) order. “While we are not extremely religious any more, we are both from a Catholic background and always told you should prolong life as long as possible.”

It's one thing when he's fine to say 'do this . . .' but it's quite another when it is almost upon you

You would presume everything would be done to keep Finn alive as long as possible, continues Tristan, “but when you are in a situation such as ours, what exactly that means was one of the most difficult conversations Deirdre and I had”. They decided the medical team were better experienced and to go along with their guidance, “assuming that they would do everything to keep Finn alive – the caveat being that would be a life worth living to some extent”.

Their son does have a DNR order but they were assured they could change their mind at any time and it has been brought up a couple of times since.

“We didn’t change our mind but we took the option of discussing it alone again to make sure of what we really want,” says Tristan. “It’s one thing when he’s fine to say ‘do this . . .’ but it’s quite another when it is almost upon you and you realise the repercussions of that decision one way or the other.”

Hauer acknowledges that families can be “intellectually prepared but they don’t know how their emotions are going to respond in the moment”. Expression of raw emotion can be difficult for medical staff, particularly those who are inexperienced or don’t know a family well, as they may misinterpret it as a request to change course.

For her, the “new frontier” in paediatric palliative care is getting the balance right between using interventions to enhance health and thereby the quality of children’s days, and the point where these measures are life-sustaining beyond the benefits to the person, “by allowing them to live longer than the body can enjoy and maintain comfort”.

Hasten death

Society can fear that treating symptoms may hasten death but the end-of-life process is occurring because of the underlying condition, she stresses, not the medication.

Once there is a recognition that interventions to prolong life are not benefitting the person, the full goal in a person’s life’s trajectory becomes comfort. People should not get caught up in the worry that maybe a medication will hasten death, she says, as “it’s not relevant because the goal isn’t to make death happen sooner, it is to ensure comfort throughout life”.

The O’Gormans don’t know how or when the end will come, “although we have talked about it more than we would like”, says Tristan.

You're walking along that cliff edge and you know that one day it is going to give way and Finn is going to fall and not be with us

One thing they are sure of is that they want Finn to be at home and they are confident that will be possible with the support of voluntary organisations such as Jack & Jill and Marymount Hospice, working alongside the HSE. Currently, with Deirdre recovering from her Caesarean section, they have a nurse coming in five hours a day. Night nurses come twice a week – the other nights the couple take turns to sleep in with Finn.

“Even when he is well, he still needs to be turned and suctioned,” says Tristan, who jokes that he goes to work, in software, for a rest.

“As a family with Finn, you’re walking along that cliff edge and you know that one day it is going to give way and Finn is going to fall and not be with us. With a service like that you have a guardrail, holding your hand a little bit, it does help.”

Meanwhile, they take each day as it comes. “That is the only way you can do it,” he adds, “otherwise you will go crazy.”

Palliative care in Ireland in numbers

- 3,840 children, approximately, live with life-limiting conditions*
- 370 children, approximately, die each year
- 150-170 of these deaths are in children with pre-existing, life-limiting conditions
*Extrapolated from UK data in Children with life-limiting conditions: establishing accurate prevalence figures, IMJ 2015, Ling J, O'Reilly M, Balfe J, Quinn C and Devins M