‘It’s like a comfort blanket knowing they are there all the time’
For parents of a child with Aicardi syndrome, palliative care works wonders
Sam and Francisco Villena with their daughter Isabella (9) and son Alex (12). Photograph: Nick Bradshaw
“A comfort blanket over the whole family.”
“We savoured every moment.”
“No area of medicine in which you can make a bigger difference.”
Those seemingly unrelated phrases are three people’s perspectives on different experiences of the one thing – palliative care. They are far from the frightening sense of doom that those two words often conjure up.
That’s why Sam Villena, whose nine-year-old daughter Isabella is receiving palliative care, thinks the term needs to be rebranded for children. “A lot of parents hear ‘palliative care’ and just panic, ‘Oh my god, you’re talking about the end’.”
She and her husband Francisco would have been the same before Isabella was registered with the LauraLynn children’s hospice in south Dublin when she was 18 months old. They have since learnt palliative care is about enabling their child to live a better life.
“Once she’s happy and getting to enjoy life, that’s all we really want for her,” says Sam of their youngest child, who was diagnosed shortly after birth with a very rare life-limiting condition, Aicardi syndrome, a characteristic of which is recurrent seizures. She spent much of her first year in hospital when her seizures were “relentless – every hour on the hour for 20 minutes. It was awful she was on so much medication.”
The couple, who have one older child, Alex (12), were having to cope on their own with her at home in Palmerstown, Dublin, until she was linked in with LauraLynn. “Up to then we had been running on empty and adrenaline as well, we just knew we needed some help, that specialised care,” says Sam. Having that support and the availability of respite “was such a break”, knowing that Isabella would be well-looked after.
“We could just switch off; I could go to bed and sleep all night, or we could go for a meal, or sleep on the couch all day if I wanted, or do things with Alex. They took over all the medical stuff. I always say, Isabella’s a pleasure and it is all the medical stuff that is the hard work. Everything is on a timetable; everything has to be done.”
A typical day with Isabella could start at 4am, as her sleep is very erratic. Due to constant seizure activity, she has to be sedated to sleep, but “she never sleeps all night” – she needs medication, she could have a seizure, she needs to be repositioned and she needs to be changed.
Sam rattles off her daughter’s daily medication times: “7am, 9am, 10am, 2pm, 5pm, 7.30pm and 8.30pm” and she needs to be tube-fed four times a day, a process that take half an hour to an hour each time.
“Your day just goes between feeding, medication and then there is all her personal care, to get her into equipment – she has a standing frame, a wheelchair and you are trying to do physio with her. She does keep you very busy.”
The family had no help during the pandemic lockdown, but, since July, they have had a nurse coming in five nights a week from 11pm to 7am “just so we can get sleep – there’s no daytime help or weekend help”. But it’s a marked improvement on the 12 nights-a-month cover they were receiving before lockdown.
However, the palliative care team at the hospice are always at the end of the phone, to advise on management of symptoms or pain. “It’s like a comfort blanket knowing that they are there all the time,” says Sam. “It’s not just about the time she is in the hospice.”
LauraLynn’s services also include memory-making as a family, couple counselling and sibling camps for Alex. “Without them, we wouldn’t be able to manage as people either.”
Consultant paediatrician Joanne Balfe, who works with LauraLynn and CHI@Tallaght, defines palliative care as “holistic care of a child and family” and meeting all their needs – medical, psycho-social and spiritual. She stresses the importance of its “active management” aspect. “People sometimes think palliative care is giving up – ‘there’s nothing more they can do’,” she says ahead of Palliative Care Week, September 13th-19th, facilitated by the All Ireland Institute of Hospice and Palliative Care to raise awareness about the difference this approach can make.
“In fact, you are actively managing somebody until the very last breath of their life – and even after that, you are actively managing their family. You are always doing something, which may be just being with somebody.”
Isabella is very typical of the cohort of children who are born with life-limiting conditions and have a prolonged period of palliative care, Balfe says. With severe disability and medical complexities, there are “multiple losses for the family after the birth, as they grieve the future milestones that will never be. They need a lot of psycho-social support, which they don’t always get that in the very early period. Then there are all the disability needs.”
These children are straddling the worlds of disability and acute hospitals and sometimes don’t fit within either, she suggests.
Balfe also works at the hospice with children who have developed a terminal condition and she agrees their families’ experiences are different.
They see their child growing up and “then they develop something, which is often initially classed as life-threatening. The vast majority of children will get treatment aimed at cure and understandably there’s a big focus on pursing that treatment and aiming for cure. Then there’s that change and shift into a more palliative approach.”
However, palliative care and care with an aim to cure somebody are not exclusive, she says. You can be pursuing treatment with an aim for cure, such as managing a bout of pneumonia in a child with severe disability. The same applies to children with cancer going on to experimental treatment, or continuing chemotherapy while in palliative care. “People can be hoping, wishing or praying for a miracle right up to the last moment, while also planning [for death]. They are not mutually exclusive, but palliative care becomes more of a feature closer to end of life.”
Balfe acknowledges that her work is “sometimes incredibly sad”, but “I don’t think there is an area of medicine where you can make a bigger difference”, she continues. “When your child is dying it is really important that you feel you are safe – that you have somebody to turn to if you have a question and you know that question will be answered.”
Parents must be able to be parents, she stresses, rather than the de facto doctors, nurses and physios they have had to become, with no training. The biggest tragedy is always that a child is dying but Balfe believes there is a difference if it is a good death.
“A good death is where parents feel that their child was comfortable and that their child felt safe and the family felt supported.” The manner of death will deeply affect the rest of the family, as they will relive the experiences of the last few days again and again through their lives, she points out.
Some of the tragedies that came out of Covid-19 “remind us what is so important about human life, which is relationships and family and love. All of us shared universal distress about people dying without being surrounded by the people they love; that reminds us what is so important about palliative care.”
The Villena family have had “those conversations” with the palliative care team about their wishes for Isabella if things take a turn for the worse. Sam knows events can be unpredictable but “at least everybody knows what we want and it is all about her”. While it is upsetting to have those kinds of discussions, it is so much better to do it when there isn’t a crisis, “and you can actually think more clearly”.
As a paediatrician, says Balfe, you can anticipate a crossroads looming and talk families through the “what ifs” beforehand, offering options and helping them in the decision-making. “You don’t want to come to that crossroads at three o’clock in the morning with someone.”
A common scenario is whether parents would pursue an option for the child to go into intensive care or would they choose a situation more focused on comfort, that might allow a child to be at home rather than a hospital.
Sam and Francisco have always told their son Alex the truth about Isabella. “They do have a lovely relationship and he is very comfortable with what is going on with her,” says Sam. “He would help out, but I try not to make him do anything as I just think he’s a child and they should be just brother and sister.”
Alex is due to start secondary school on September 10th and his parents will wait to see him settled in before thinking about sending Isabella back to her special school in Stewart’s Care in Palmerstown.
“I think all parents like us are very nervous,” adds Sam, whose family have limited their movements throughout the pandemic so far to keep Isabella as safe as possible. “Nobody can tell us what the right thing is to do, it has got to be our own personal decision.”