Richard Guilliatt; 12/9/09
Cherie McFadden was just under six months pregnant and sitting at her desk in a high-rise office above Perth when she felt her body lurch suddenly into the early stages of labour. It was January 2007, and within an hour the 33-year-old was sit ting on a bus heading to King Edward Memorial Hospital for Women, crying and fearful, as her husband Paul drove madly down from RAAF Base Pearce on the city’s northern outskirts, where he worked as a pilot. The couple can still recall every detail of that grim afternoon in the hospital’s maternity ward, in particular the grave face of the obstetrician who advised them that a 22-week-old foetus was almost certain to die outside the womb. Its skin was so fragile that it could tear like wet paper; its lungs would struggle to process oxygen; its brain and other organs were still not fully formed. In theory, such a baby could be artificially resuscitated, the doctor said, but death or severe disability was almost inevitable.
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The McFaddens knew their baby was a girl, and they had already decided to name her Annie. Now they listened as the doctor explained how their first child would be delivered, would be laid on Cherie’s chest, would make several gasping attempts at breathing, and would die.
But that seeming inevitability never happened, because for the next week Cherie McFadden lay in a hospital bed fighting her body’s urge to give birth while her husband spent hours at home, hunched over his computer as he downloaded hundreds of pages of medical literature on premature birth.
In the course of that research Paul McFadden made a crucial discovery: in a few days’ time his wife’s pregnancy would enter an uncertain area of medical controversy known among neo-natologists as the “Grey Zone”.
The Grey Zone is a term coined to describe the three-week span in an unborn baby’s life, between 23 and 25 weeks’ gestation, when survival outside the womb is possible but outcomes are terribly uncertain.
Fifty per cent of the babies born at this gestation will die, and those who live can often be afflicted cerebral palsy, intellectual disability or blindness.
Some doctors refuse to resuscitate such fragile infants.
But the McFaddens happened to be in the Australian hospital which had the most aggressive policy of supporting “23-weekers” if their parents requested it. At Sam on January 23, only a few hours after Cherie’s pregnancy officially entered its 23rd week, the McFaddens made that request.
Annie McFadden was about the size of her father’s hand when she was delivered, weighing only 570 grams. “She looked like a baby bird that wouldn’t live,” remembers Paul. “Completely purple, almost black. Totally limp, way beyond anything you think could survive.” The pediatrician pressed a stethoscope to this tiny creature’s chest and announced, “She’s got a heartbeat”, then inserted a plastic breathing tithe down her throat.
Within minutes the McFaddens’ daughter was laid on a steel intensive care warmer and connected by a tangle of tubes and wires to banks of computerised life-support systems.
Aventilator delivered oxygen in carefully calibrated doses through her mouth; a catheter was inserted into the umbilical vein in her abdomen, and another pushed through to an artery near her heart to measure blood pressure; a tube was inserted in her stomach to drain off air pushed in by the ventilator, and a sensor attached to her foot measured oxygen saturation in her blood. Rushed to the Neonatal Intensive Care Unit, she was hooked up to a drip which fed glucose, amino acids and nutrients through a third catheter in her arm. It would be a month before Cherie McFadden was allowed to touch her.
With machines regulating her breathing, temperature, body fluids and nutrition levels, Annie spent the first two weeks hovering near death as she nearly succumbed to the shock of life outside the womb and a septic blood infection that required multiple transfusions.
Three weeks after delivery her bowel ruptured from a necrotising infection and she was transferred to Princess Margaret Hospital for Children, where surgeons cut open her abdomen from hip to hip and spent five hours mending and removing sections of her lower intestines which were barely wider than spaghetti.
A faulty valve near her lungs caused wild gyrations in her blood-oxygen levels, and at 3o weeks she developed a lung infection so dire it required the administration of four antibiotics.
In the second month following her birth she nearly died twice – first when a ventilation tube shifted in her airway, and shortly afterwards from severe pneumonia.
Antibiotics kept her alive, and steroids were given to promote her lung development. At 38 weeks severe gastro- enteritis nearly killed her, and a week later the retina in her right eye began detaching.
For four weeks she underwent a series of operations in which eye surgeons attempted to reattach the retina by burning blood vessels around it with a laser, injecting a gas bubble into the centre of the eyeball, applying microdroplets of dry-ice and finally wrapping the entire eyeball with an elasticised “scleral buckle”.
The surgery saved 20 per cent of her vision in the eye, but two weeks later the entire retina in the other eye detached.
It wasn’t until June 27, five months after her birth, that the McFaddens were finally allowed to bring their baby home.
Since then the couple have had a son, Michael, whose birth last November was perfectly normal.
On a recent winter evening the family’s home in Perth’s southern suburbs is a hive of post-dinner activity as baby Michael is put to bed and Annie runs energetically around the lounge room in her pyjamas. Only at a second glance do you notice that Annie still does not speak, and that the story books littering the floor are written in Braille.
The McFaddens have thus become one of hundreds of families whose premature baby has miraculously survived, only to be significantly disabled. It’s an outcome that has led them to contemplate some big questions about the nature of life and the limits of medical intervention.
“The bad news doesn’t stop when you come home,” admits Paul. “One of the doctors we consulted compared it to the waves hitting the coast – it just keeps on coming. It might be five years before you discover whether your child is developmentally delayed.”
But as he looks at his daughter, his voice softens. “Whatever outcome Annie has, for her it will be normal. None of us are judged by what we don’t have. I don’t have Einstein’s intellect or Carl Lewis’s speed. Who makes the decision about what quality of life is acceptable?”
On the borderline of viability
The most important premature baby in history may well have been President John F. Kennedy’s son Patrick, who was born six weeks prematurely in August 1963 and died from respiratory failure less than two days later.
The outpouring of national grief that followed that death made neonatology a priority of US medical research funding.
It was only six years later that two New Zealand doctors, Graham Liggins and Ross Howie, used a Wellcome Trust grant to prove that steroids administered to pregnant women could dramatically improve a premature infant’s ability to breathe.
Liggins has since been knighted for his work, which would take more than 20 years to achieve widespread medical acceptance. But since the mid-’9os the use of steroids has become routine in premature births, along with improved ventilator equipment and newly developed drugs to help foetal lung development.
Whereas a baby such as Patrick Kennedy had only a 60 per cent chance of survival in 1963, it is rare today for babies of even 27 weeks’ gestation to die. At the same time, the number of premature births has risen dramatically as women opt for fertility treatments such as IVF, or wait until middle age to start their families.
More than 23,000 Australian babies were born prematurely in 2006, a prematurity rate roughly 5o per cent higher than 12 years earlier. One-tenth of those babies were born under 27 weeks’ gestation, and about 75o a year are born in the Grey Zone.
The media’s love affair with these modern “miracle babies” has focused on extraordinary examples such as Elora de Bondi, a 319g Melbourne infant born at 24 weeks in early 2007 and believed to be the smallest baby ever born in this country.
Only six months earlier, doctors in Miami reportedly “broke all records” by delivering a baby weighing barely 29og; Amillia Taylor was born at only 21 weeks and six days, and was so fragile that doctors accidentally tore off her left ear during the caesarean delivery.
What’s not nearly so interesting to the media is the long-term prognosis for such children, a
subject of contentious debate in medical circles.
In some countries, notably the Netherlands, babies under 25 weeks are not recommended for resuscitation because their prospects for quality of life are judged to be too poor.
Within Australia practices vary from state to state, and hospital to hospital.
The sometimes heated disagreements that result were on display at the University of Technology Sydney in June, when Dr Janet Green presented a lecture entitled Too Young To Live? A neonatal nurse who lectures in the university’s Faculty of Nursing, Midwifery and Health, Green has conducted extensive interviews with other neonatal nurses, compiling her findings into a 652-page doctoral thesis that paints a stark picture of life on the “miracle baby” ward.
The nurses Green interviewed spoke of their deep misgivings about inflicting repeated painful procedures on tiny infants who looked more like foetuses than babies, and in many cases were destined for a life of disability.
“We’re prolonging the suffering,” said one angry nurse. “We’re going to enable this child to survive so that it can sit in a beanbag for the rest of its life.”
Others argued that some of the treatments amounted to medical experimentation. When Green presented this latter finding in her lecture, neo-natal specialists in the audience were outraged. One of them, Dr Kei Lui of the Royal Hospital for Women in Randwick, complained to the head of Green’s faculty.
Privately, however, even some neo-natologists admit to being troubled by the changes technology has wrought. “It horrifies us sometimes when we see a 400-grammer who needs surgery,” says one. “But the reality is that there is no going back now. It’s just a matter of trying to do it better.”
Four years ago, Dr Lui convened a “consensus conference” at the Royal Hospital for Women designed to iron out some of the philosophical disputes that swirl around the Grey Zone. Doctors, nurses and a handful of parents gathered in a theatrette and voted on various clinical scenarios using an electronic blind-voting system.
The consensus that emerged was that babies under 24 weeks’ gestation should not be resuscitated.
But one dissident in the gathering was Dr Noel French, an English-born neo–natologist who has been practising at King Edward Memorial Hospital for Women in Perth – where Annie McFadden was born – since the late ’7os.
Unlike many of his counterparts in the eastern states, French’s team has for many years offered support to babies born at 23 weeks if the parents request it.
As a result, King Edward Memorial has delivered more of these infants than any other hospital in Australia.
And because he runs the only neonatal intensive care unit in the state, French has been able to follow the progress of virtually every Grey Zone child born in Western Australia in the past 3o years.
It’s a position that has given him a unique and perhaps unexpected outlook.
“The consensus conference basically said: Don’t support these infants,” French notes. “Our philosophy is that this is not an issue that should be decided by doctors talking among themselves.”
Doctors, he says, are frequently far more pessimistic in their attitude to disabled children than parents. He recalls presenting a paper at a US medical conference seven years ago which included a poster showing photos of some of the children who, as pre-24-week babies, his team had cared for.
As doctors crowded around these pictures of smiling kids playing with blocks and colouring books, many remarked on how happy they looked, and French realised that his peers often have little notion of the lives led by these babies after they leave their wards.
“We’re dealing with an area where disability and complex learning disorders are extremely common – but should we be saying we shouldn’t support life because of a complex learning difficulty?” French continues.
“If we look at the average child with Down syndrome, they have a level of intellectual incapacity we would rate as severe. But the community out there has become completely used to the idea of children with Down syndrome. No one would choose to have a child with Down syndrome but when it happens we are prepared to philosophically say, ‘OK, we’ll deal with it.’
These premature kids are, for the most, going to be a whole lot better in terms of severity than the average kid with Down syndrome.”
As French acknowledges, however, this debate is complicated by other issues, not least the cost of treatment – to the health system and to the babies themselves. It is widely accepted that high-level neo-natal intensive care beds represent a cost of $1 million each annually to the health system; in 2006 the single most expensive patient in the Australian private health system was a premature infant whose treatment incurred medical bills of $276,722 over six months.
And the treatments themselves – from massive antibiotic doses to painful invasive
procedures and multiple surgeries requiring general anaesthetics – may have adverse effects that are impossible to gauge.
The quiet hum of machinery
On a recent day at King Edward Memorial, French’s nenatal intensive care unit is hosting 67 babies from all points of the state. Thirty-four of the most at-risk babies, requiring respiratory support, are in the Level Three nursery. The ward is a bright, open-plan room containing rows of incubators in which the dark, tiny forms of babies lie silently like little aliens inside perspex space capsules.
Each bed is surrounded by about $100,000 worth of ventilators, drips, monitors and other life-support systems.
One sound you rarely hear in a Level Three neonatal intensive care unit is a baby’s cry, for most of these children have a ventilator pushing a carefully calibrated amount of air into their lungs through a breathing tube.
The quiet hum and bleep of machinery is deceptive, for emergencies erupt almost daily and deaths are not uncommon.
The day I arrive, a young couple, Misha and Damien Chalk, are taking their son, Levi, home five months after his birth at 23 weeks’ gestation. In that time he has survived a burst appendix, hernia surgery, eye surgery and a staph infection. Other babies around him died as the Chalks kept a daily vigil.
“We call him the Marathon Man,” says Misha, who has just spent her first five minutes alone with her son in a private change room.
Beneath their excitement at leaving the hospital is an undertow of uncertainty; it will be at least a year before they learn whether or not Levi is in the minority of unlucky babies who has suffered some severe outcome such as cerebral palsy. “It might not be us; it might be us,” says Misha.
“We may in the future get some shocking information. But we did some research, we found out what the long-term outlook was and we made decisions based on that. We live in the moment right now, and he’s doing all the right things.”
The statistics compiled by French’s team over a 10-year period show that, of the 96 infants who survived at the hospital after delivery at 24 weeks, only five per cent suffered severe disabilities – defined as blindness, wheelchair-dependent cerebral palsy or major intellectual impairment. The figure rises to 18 per cent for the 48 infants who survived at 23 weeks.
Those numbers are substantially lower than statistics compiled in NSW and the ACT, which show “severe functional disability” in 19 per cent of the first group and 33 per cent of the second, although French cautions that state-by-state comparisons are difficult because of differences in the way each hospital system works.
Overseas studies that have tracked Grey Zone babies into adulthood have shown they suffer twice the normal rate of psychiatric hospitalisations and are six times more likely to have behavioural or educational difficulties.
“There may be a perception that I have observer bias,” French acknowledges. “But our hospital follows virtually every survivor in Western Australia, which does give us a perspective
not many others have – we see the whole range, from the severely disabled children to the very good ones. You or I might look at a particular family and say, ‘Gosh, that outcome really is severe – surely they must have misgivings about going that route.’ But it would be rare to find a family who regret their decision. There is nothing you wouldn’t do for your own child – that’s how families are.”
What is a full life?
Watching his daughter hurtle around the lounge room in her pyjamas, navigating by touch and the partial vision that remains in her right eye, Paul McFadden says pointedly: “If Annie had been born in Sydney, she would be dead. Having been there, I do believe a baby should be assisted on its merits.”
Sooner or later, any discussion about the ethics of saving premature babies veers into the same philosophical terrain as the abortion and euthanasia debates.
The Victorian government passed a law last year allowing abortions at up to 24 weeks, making it theoretically easier to end a baby’s life at 23 weeks than save it. Dr Lui at Sydney’s Royal Hospital For Women found himself in a vexed confrontation with a deeply religious family several years ago after he recommended they remove life support for their baby at 26 weeks’ gestation because of a chronic infection; Lui ended up speaking on the phone to a rabbi in Jerusalem who also insisted on the sanctity of the baby’s life.
The baby died several hours later.
Conversely, some hospitals now insist on resuscitating a baby once it is older than 25 weeks’ gestation, irrespective of the parents’ wishes and regardless of the fact that the child will have a heightened chance of developmental problems. “We would have difficulty not supporting a 25-weeker who in all other respects is perfectly well,” says Noel French. “We would not be willing to accede to parents’ wishes if it came to that. And this gets into heavy territory. But it’s a very rare situation for a parent of an otherwise normal 25-week gestation baby not to want it.”
Two and a half years after Annie’s birth, the McFaddens are still not entirely clear what the future holds for their daughter. Her mother has started taking her to classes at the Association for the Blind.
Her speech is still stuck at the level of a nine-month-old, but whether that is a by-product of blindness or some deeper disability is unclear. Her comprehension is good, however, and she is already beginning to read Braille.
“In a world where people are customising their babies to have blond hair or blue eyes, I understand that some people are not going to cope with having a ‘flawed’ child,” says Cherie McFadden.
“But I don’t look at Annie and think, ‘How is she going to live her life?’ I see these people every week at the Blind Association.”
“People don’t question cancer treatment,” adds Paul, “but with neonatal intensive care they’ll say: ‘These children are blind, they’re disabled, they’re a burden on society and they don’t have a full life.’ What they’re really saying is that that’s not a life they would want for themselves.
But when I come home in my flying suit, Annie rushes to the door and she’s so excited to see me that she’s crying. To me, she’s perfect the way she is. She experiences wonder, she feels the wind on her face. Who are we to deny her that?”