Battle for Will

By Clive Mathieson

This article first appeared in The Weekend Australian Magazine, September 29-30

The Australian

After 11 hours in theatre, Will is alive – but the operation has not gone smoothly. Picture: Clive Mathieson

Will is now 16 months old and happy and healthy. Picture: Adam Knott 

CHRISTOPH Camphausen looks up from the ultrasound screen and across the body of my tiny, two-hour-old son. “We have a major problem,” he says. “A surgery kind of problem?” I ask, already starting to shake.

“Yes, let me finish this and I’ll come and talk to you.” The sight of the paediatric cardiologist at Will’s bedside as I walk back into the nursery after seeing his mum to the maternity ward conveys a strong hint of the kind of problem we are talking about. I can see the red and blue blood flows – arterial and venous blood – flashing across the screen as Camphausen moves an ultrasound around Will’s fragile little chest. I can hear the hushed conversations between him and another doctor. And, not for the last time in the coming six weeks, I stand there helplessly and think of the worst.

Twenty minutes pass before Camphausen, a softly spoken man with kind eyes and a reassuring manner, suggests we sit down in a “quiet room” off to the side of the nursery, the intensive care unit for newborns. I would come to dread invitations to quiet rooms, but it seems like a reasonable suggestion. He doesn’t sugarcoat it. Will – we’d settled on the name the moment we saw him – has “transposition of the great arteries”, or TGA for short. While I am still stuck on the marvellous Victorian-era name, Camphausen cuts to the chase. TGA, he says, is “not compatible with life”.

Our third child was unplanned and an utter shock – an embarrassing admission, perhaps, for an otherwise responsible, professional 30-something couple.

The pregnancy was met with the familiar surge of excitement but this time with a healthy amount of practical dread. My wife, Miranda Murphy (I’ll call her Murph because everyone does) and I were already parents to two fiery redheaded daughters, then aged four and two, and we were only just keeping up with the work/family juggle common to so many Australians.

As the otherwise uneventful pregnancy progressed, we came to terms with the wonderful chaos life was about to become and looked forward to meeting our newest family member. We learnt at the 18-week scan that we were having a boy. What we didn’t know – and what should have been picked up by the ultrasound operators – was that his body held a terrifying secret.

As Murph was wheeled into the operating theatre at the Royal Hospital for Women in Sydney’s eastern suburbs on May 6 last year for a caesarean, we expected the procedure to go pretty much the same way as the previous two. “A boy,” said the midwife, as Will was delivered an hour or so later by our trusted obstetrician, Alex Szirt. “Boys are trouble.”

The first thing we noticed about Will was that he was not scrawny like his sisters had been. Nearly full term, he was a healthy 3kg. He was alert, with dark blue eyes, good voice and, ironically in hindsight, he achieved a stellar nine out of 10 on the Apgar score that measures the health of newborns. He looked just like his sisters as we cuddled him. Only looking back at the photos in coming days would we notice the distinct shade of blue about his skin, particularly on his hands and feet. The nursing staff picked it up immediately. There are, generally, two reasons for blue babies: fluid on the lungs or heart defects. If the staff suspected heart trouble, they didn’t worry the new parents with their concerns. Will was encouraged to cough up any fluid, weighed, measured, pricked and bustled off to the nursery while his mum went into recovery. It was the same procedure for his sisters. He was given oxygen but there was no urgency or sense that anything was wrong.

Two hours later, as Murph rested in the maternity ward, Camphausen would deliver his devastating news. Using one of those pens with two colours I used to covet at school – handy for a cardiologist, with red for arterial blood and blue for venous – he explained transposition of the great arteries, which is one of the many congenital heart defects that together affect about one in 100 babies, or about six babies born in Australia every day. TGA means the aorta and the pulmonary artery are connected to the wrong sides of the heart, so blood that is pumped to the lungs to get its fill of oxygen never gets pumped around the body. Instead, it stays in a loop – into the right chamber of the heart, into the lungs and back into the right chamber. At the same time, de-oxygenated blood is pumped through the left chamber of the heart and back out into the body without ever going through the lungs for its oxygen hit. Round and round, losing oxygen “saturation” with every cycle.

Babies in the womb, who get their oxygen through the umbilical cord, have two natural holes in and around the heart that allow blood to mix between the chambers. But they quickly close once the baby is born and gets its oxygen by breathing.

Will’s condition, Camphausen explained, was not compatible with life. The good news – and good news was in short supply on day one – was that Will had “simple” TGA; unlike a lot of other cases, there were no other visible problems with his heart or the vessels around it. Untreated, Will would probably last a few weeks or months at best, getting weaker and weaker until his body shut down. Until the 1950s, that was the fate of all babies born with TGA. Then doctors developed a couple of techniques that basically jerryrigged the heart and the surrounding vessels with shunts – or conduits – that allowed the oxygen-rich and de-oxygenated blood to mix. Survival rates were good but patients never had a “normal” heart.

From the mid-1970s, following advances in heart bypass surgery, doctors started performing an “arterial switch”, where the aorta and pulmonary artery are disconnected from the heart, swapped and reconnected.

The tiny coronary arteries, which feed oxygenated blood to the heart muscle itself, also have to be disconnected and re-attached to the new aorta.

All of this, Camphausen and one of the surgeons in training, Maqsood Elahi, explained, would be done on a heart the size of a walnut while Will’s blood was pumped through a bypass machine that acted for his heart and lungs. But that wouldn’t happen for a week.

First they wanted to fatten him up, get him feeding and as strong as possible before the operation. To buy time, they needed to perform minor surgery known as a balloon atrial septostomy almost immediately to open one of the small holes in his heart. With Will only a few hours old, they would knock him out and feed a tube through the umbilical vein from his belly button into his heart. There, a small balloon would be inflated, opening the existing hole a little wider and allowing more blood to mix. The prognosis after the major surgery was good: success rates were high; the chance of complications low; and, at the end of it, Will would have a “normal” heart. It was time for his mother to get the news – and to see her little boy for the first time since she left the delivery suite.

How do you tell a mother that the baby she has carried and just given birth to has a potentially fatal heart defect? How do you tell her that in a matter of hours they’ll be putting a catheter into his heart? And that next week, your six-day-old will be having open heart surgery? How do you break that gently? You don’t. You just try to remember what the doctors have told you. I can’t recall if there were tears but I can remember the numbness and disbelief. The last time I saw that face on my wife was several years earlier, when her brother rang to tell us her mother had cancer.

We wheeled Murph down to the neonatal intensive care unit in her bed, where she was able to hold Will’s little (blue) hand. By late afternoon, after the septostomy, we were back – this time staring at him through a sheet of clear plastic. He was still anaesthetised and, but for the rosier colour of his skin, he looked like a body in a morgue. He’d taken his first step to recovery but it was distressing. And far worse was to come.

The next five days had a steady rhythm to them. For Murph, it was about recovering from the caesarean, feeding and bonding with the little man. You can tell a child’s personality from the moment you hold them and Will had an extremely sweet nature and deep, knowing eyes. For me, it was also about caring for Will’s sisters, helped by a steady flow of mums and family from our home town, Adelaide. For Will, it was about staying alive. He fed well and regained his birth weight.

But every day was more of a struggle to get enough oxygen into his body. A normal baby has a blood oxygen saturation level – usually shortened in hospital to the “sats” – of 100 per cent. Initially, Will could manage moments in the 90s. Then the 80s. And then we were often dipping down into the 60s. By day three, he was regularly receiving oxygen through a tube under his nose and, with two days to go before the operation, the tube was in permanently.

Will was also getting tired, and would flag quickly during feeding. But put aside the tubes, the constant prodding and blood testing, and Will was like any other baby. We’d got to know him and our greatest fear as we prepared to send him off for surgery was that we wouldn’t get the same boy back.

The night before the surgery we met the man who would become a regular and reassuring presence in Will’s fight, anaesthetist John Awad. Will owes his life to so many doctors and nurses at the Royal Hospital for Women and the Sydney Children’s Hospital but Awad – solid-set, with long, wild, black hair pulled back in a ponytail – played a key role. That night, he explained how Will would be wheeled through the corridors from the RHW to the adjoining SCH. We’d say goodbye at 8am, leaving him in Awad’s arms ahead of an operation that should take five or six hours.

The handover was heart-wrenching. Every instinct screamed to hold onto him. “We’ll take good care of him,” Awad said. We choked out, “We know you will,” and turned away, walking back down the corridor leaving our baby behind.

We followed the advice of clinical nurse consultant Catherine Reilly, and left the hospital rather than spend hours pacing a waiting room. It was a cold but sunny, clear autumn day. We’d moved away from Bondi a couple of years earlier but went looking for comfort and familiarity in our old suburb. We visited our favourite cafe, walked gingerly down the Hall Street strip to browse distractedly through a secondhand bookshop, then sat, hands clasped, on the grass near the beach and, again, thought of the worst.

After a few hours, we drove the short distance back to the hospital. We wanted to be there when Will came out of surgery. In the end, we needn’t have rushed. Six hours passed, then seven, as we waited in a state of unbelievable tension in a sparse family room. By mid-afternoon, we were still resisting calling for updates – and fighting growing panic. By 4pm, we finally cracked and called Reilly. The only update she could get out of the surgery team was that there had been “some kind of bleeding issue”. That didn’t help.

Just before 7pm, an agonising 11 hours after Will went into theatre, he was out. Reilly had prepared us for the sight – showing us photographs of other post-surgery babies – but this was something else.

Will was almost unrecognisable. Grossly red and swollen, on a ventilator, with 13 tubes going in and out of his prostrate body. Painkillers, oxygen, drains, dialysis lines, catheters, cannulas, monitors, heart pacing wires and, most distressing of all, his ribcage propped open, his heart fluttering visibly at more than 120 beats a minute beneath a thin strip of yellow Gore-Tex. He was marooned on an adult-sized bed that was needed to accommodate the defibrillator machine in case of emergency.

Will was alive, and a visibly weary surgeon Peter Grant was reassuring. But it was clear the operation had not gone smoothly. There had been low blood pressure, high blood pressure; by the morning they would volunteer the term “hypertensive crisis”, but at the time the surgeons were much more matter-offact.

There were two sessions on heart bypass and a bleed, somewhere behind the heart, that they had trouble stopping. There was no way they could release Will to the ICU if there was any blood loss.

Grant is the invisible hero of this story. We met him only two or three times but he is acknowledged as one of the nation’s best paediatric cardiac surgeons.

The surgery he performed on Will is high-stakes and enormously demanding in terms of the stamina, concentration and effort required. Despite all the setbacks to come, Will was given the chance to live because of his incredible skill.

Later that night, after the surgeons had gone, John Awad wandered up to Will’s bed. He looked shattered.

His face betrayed the struggle Will had experienced on the operating table and the extraordinary job the surgical team had done to bring him through.

The next 12 hours were critical, Awad explained. Will was now in the hands of the intensive care unit.

The ICU, for anyone who has been lucky enough never to visit one, is an extraordinary place, particularly in a children’s hospital. It is heart-breaking and inspiring in equal measure. There is constant motion day and night, with the dedicated nursing staff working 12-hour shifts, one-on-one with the most needy patients like Will. Surprisingly, at least to us, we were free to come and go as we pleased and bring visitors at designated times.

There are unwritten rules: in particular, don’t visit or stare at the other patients.

Each huddle of parents is going through their own pain. You pick up bits and pieces – the beds are close together – but you tend not to share much more than a nod and a weary greeting. There is a strange emotion as you see other children come and go, spending as little as a day in the ward while your son lies there for weeks.

Happy for them, envious perhaps, hopeful you’ll be the ones leaving soon.

There were other children who had been there for months, or in one case more than a year waiting for a new set of lungs.

On the first night, with Will fresh from surgery, the wonderful nurses Gerry and Carly fussed over him all night, monitoring him and the incredible bank of drug-delivery machines keeping him alive (morphine, adrenalin, sedatives, potassium, saline and diuretics), responding if his heartbeat, blood pressure or oxygen saturation moved outside normal range – if there is such a thing as normal in this situation. We had complete faith in the ability and judgment of the nurses and intensivists.

They were confident, reassuring and calming, even with the most anxious and difficult parents. All around were signs of the charities that support the hospital. Some of the machines next to Will’s bed were donated by the Humpty Dumpty Foundation, which some months earlier had encouraged me to put my hand in my pocket at a fund-raising event.

If everything went according to plan, Will would be out of ICU in a week or 10 days. Everything did not go to plan. The first setback was closing the wound in his chest and stitching up the ribcage, which had been propped open to allow his swollen heart to get back to its normal size. It is usually done within about two or three days, but Will was too swollen and it could not be carried out for five days. Finally, his chest was closed – a row of neat but raw stitches stretching half the length of his torso. By that time, however, a much more serious complication had arisen.

Chylothorax is the leaking of fluid from the body’s lymphatic system into the gap between the lungs and the ribcage. As the cavity around the lungs fill up with this fluid, called chyle, the lungs cannot fully inflate, robbing the body of its ability to get enough oxygen.

In Will, it started on the left side. A couple of days later, the right side joined in. The fluid had to be drained with tubes inserted between his ribs on each side of his body. Nobody really knows what causes chylothorax and there is no surefire way to stop it. It can last for days, weeks or, in some awful cases, months. They do know that fatty substances can make it worse, so the first thing they did was take Will off his mother’s milk, which had been fed into his stomach with a nasal feeding tube. He was put instead on a fat-free diet of “total parenteral nutrition” – an intravenous delivery of a formula containing only the basic building blocks of nutrition.

For a week, Will’s recovery was achingly slow. The chest was closed, he could open his eyes – he looked, frankly, bewildered – but the chyle showed no sign of slowing. Still, he was recovering from surgery. There was little I could do except sit by his bedside, and Murph seemed to be doing a perfectly good job at that, so I went back to work on May 23 to pick up my new job, editor of this newspaper, which I had been given just a week before Will’s birth.

Later that morning, Murph was driving to the airport to pick up her sister when the call came from the ICU duty doctor. Will had suffered “focal” seizures.

After prodding, he agreed it could be a sign of a stroke. Murph rang me (I hadn’t lasted one morning at work), picked up her sister, dropped her off with the girls and sped back to the hospital.

We met at the hospital and watched as our otherwise sleeping baby had another seizure, his thin little arm and shoulder twitching crazily. Initial ultrasounds showed a spot on his brain. It appeared to be a stroke – an occasional but rare risk with heart bypass surgery – but only an MRI scan would tell for sure what we were dealing with. Will was placed on anti-seizure medication and prepared for the MRI late that afternoon.

Before being fed into the scanner he would need to be fully sedated again and all his tubes and wires replaced with much longer ones. He would also get extremely cold during the hour-long procedure. It was a risky step that required the utmost care.

As we waited for the results later that evening, we wandered down to a restaurant in nearby Randwick for dinner. I remember telling Murph that Will’s luck – our luck – was about to change. The results would show no major damage, I assured her. “Don’t worry, we will get our boy back.” Then we got the call to return to the ICU for a briefing. I was hopelessly and horribly wrong.

I still feel sorry for Kevin Swil, who was running the ICU that night. But he will have our respect forever.

Swil had never met us; he wasn’t one of the regular doctors, nurses or intensivists we’d dealt with over the previous couple of weeks. He apologised for this as he sat us down in one of those quiet rooms. Will had indeed suffered a stroke, he said, but that was not the worst of it. The MRI had showed up a cerebral venous sinus thrombosis – a blood clot in the veins that drain blood from the brain. It was big, running most of the way down the back of his head. And it was definitely life-threatening. As he was telling us the news, I started to shake again. Murph and I held each other’s hands so tightly it hurt.

I googled a lot of things during Will’s stay in hos pital, never to question the doctors but to look up medical terms. Cerebral venous sinus thrombosis is one I wish I’d left alone. The prognosis was not great. Swil had already worked the phones, calling neurologists, haematologists and clot experts here and overseas for options. Sitting in front of two distraught parents he’d never met, the poor bugger laid them out: use a clot-busting drug to break it up (too dangerous); do nothing (not really an option); put him on bloodthinner and hope for the best. Option C, thank you.

A lot of euphemisms are used in hospitals and ICU wards; the one you don’t want to hear is “catastrophic event”. But the one we heard often was “disappointing development”. This is how Andrew Numa, who was running the ICU unit the next morning, described our news. “But let’s not panic just yet,” he said. “We may still get out of this.” I found this strangely encouraging. Will’s mother didn’t.

Neurologist Annie Bye talked optimistically about babies’ “plastic” brains and their ability to find new pathways to cope with events such as strokes and clots.

But our greatest fear looked like being realised: we would not be getting our Will back, the one we had met before the operation. There was little to do except let the blood-thinner, administered in two injections each day, do its work and spend as much time as possible with Will. The hard truth was that the only way we could tell if the medication was having an effect would be if he had no more strokes. A few days earlier, workers from the HeartKids Australia charity had been past. Murph, in a lighter moment, wondered if Will was still a Heart Kid or now a Brain Kid.

Will was now in quite a fight. His grip was firm on both sides and through the fog of morphine he seemed to focus on sights, sounds and his parents. But he was clearly getting weaker. The fluid continued to drain and his condition deteriorated. He developed pressure sores on the back of his head, and no amount of turning by nurses seemed to relieve it. Then the stitches holding his ribcage together popped through his paper-thin skin. Even the nurses had never seen that.

“Baby Will is three weeks old today,” Murph wrote on Facebook. “He has survived open heart surgery, bypass, two more heart procedures, a stroke, a separate clot in the brain, fluid leaking into both lungs requiring drains, plus countless injections, cannulas, infusions, X-rays, ultrasounds, MRIs and blood tests. He is the toughest person we know.”

As I drove back through Sydney’s inner west one night that week, I let the thought that he wouldn’t make it sink in. I choked up and thought of the effect it would have on his mother and sisters. It was too much for his little body. It couldn’t take the multiple assaults.

Maybe that was a better outcome than permanent brain damage or disability. No, don’t think like that.

Not for the first time, I thought about praying.

Although baptised and confirmed in the Anglican church, I’d pretty much drifted towards atheism, occasionally putting in a good word upstairs for a friend or family member who’d died. But the sickness of a child brings out the belief in people. Colleagues and friends who’d never flaunted their religion were now praying for Will. We were in people’s prayers.

The only thing that stopped me praying was the acknowledgement that I would be a complete hypocrite.

So, now that you need God, you suddenly believe in him? And what if Will recovered? Could I still put that down to medical science and Will’s tenacity, or would I want to give the credit to God?

In the end, I only wanted to help Will, so I prayed. I didn’t ask God to make him better; I asked only that, whatever the outcome, he didn’t suffer.

When things were looking the bleakest, John Awad reappeared in our lives, starting a seven-day shift as head of the ICU. Awad figured the intravenous diet was doing nothing to stop the chylothorax. All it seemed to do was make Will too weak to fight. So he decided to introduce Monogen, a low-fat formula, fed into his stomach through the nasal tube. Quite a few staff had said that when babies start to get better, the turnaround happens quickly. And almost at that moment, Will’s fortunes turned. Within days, the fluid began to dry up. By the end, his tiny body would have produced more than two litres. Even better news came on May 31, when a follow-up MRI revealed the clot had not advanced and may have shrunk. The following day, he came off the ventilator for the first time since the operation three weeks earlier. “Can see actual baby again,” his mother texted to friends and family.

“He’s coping well and we are having a cuddle.”

Two days later, his chest drains were gone, he was dressed in the first baby clothes of his life and he was moved out of ICU to the cardiac ward. Will still had to gradually withdraw from painkillers and it was traumatic to watch. And he had to learn how to feed again, this time from a bottle containing the unappetising Monogen to stave off a recurrence of chylothorax.

On June 14, nearly six weeks after he was born, we loaded Will into a baby capsule, took him out of the hospital for the first time in his short life, and drove him home, where his sisters were waiting.

Today, Will shows no signs of his ordeal other than multiple scars on his body. He is a happy and healthy 16-month-old. Astonishingly, an MRI revealed the clot has effectively vanished and what we feared was a stroke may have been little more than bruising of the brain. Time will tell if there are complications in future years – and he will be a regular visitor to cardiologists and other specialists for the rest of his life. But at his last check-up Christoph Camphausen didn’t see any further issues. “He has a normal heart,” he said.

Why publish Will’s story? Thankfully for us, more than a year later, that whole terrifying episode seems a world away and the puffy, punctured baby in the pictures does not seem like our little man. But the perpetual traffic through the children’s ICU and the photos on the hospital walls tell of so many like him, the lucky, and also the devastating.

In the middle of Will’s struggle, former Olympian Alisa Camplin told the heart-breaking story of her son, Finnan, to 60 Minutes. I watched it at home alone as our son fought for his life. Finnan was born with congenital heart disease and, despite the efforts of doctors, it proved too much for his tiny body. We can’t pretend to comprehend their pain. Camplin and her husband now raise money for The Royal Children’s Hospital, Melbourne, through Finnan’s Gift.

We also hope to help draw attention to congenital heart disease and the exceptional professionals who treat and save children. Hopefully, our story can also help other parents going through similar ordeals.

Sydney Children’s Hospital Foundation
The Royal Children’s Hospital Foundation
Humpty Dumpty Foundation
Royal Hospital for Women Foundation

Heart Kids | Australia