Open Hearts E-Book

Kate Bull

• Herausgeber: Elliott & Thompson
• Kategorie: Fachliteratur
• Sprache: Englisch

Seitenzahl: 486

Veröffentlichungsjahr: 2016

Beschreibung

Nearly all of us will, at some point, know someone who was born with a heart defect. But, as the surgical scars so often remain hidden, we just might not realise it.Powerfully telling of the patients and their experiences, Open Hearts is a remarkable medical story: we are often so focused on 'extraordinary' people and their achievements, we forget just how incredible the 'ordinary' achievements of living can be.Until the 1960s 'blue babies' were a striking sight in our streets. Suffering from congenital heart disease offered a bleak outlook to young patients and a heartbreaking experience for parents. Very few would make it to adulthood; now, in the West at least, most have a much higher chance of survival.InOpen Hearts Kate Bull, formerly a cardiologist at Great Ormond Street Hospital, tells not just of the development of heart surgery in children, but of the patients, past and present, whose lives have been transformed. Besides the technology, the sociology of medicine has changed substantially since the 1950s - think of the atmosphere of children's wards. Other things have barely changed - consider the dread of kissing your child goodbye at the door of an operating theatre in any era.Children's heart surgery is often seen as a medical triumph; but, for all the successful operations completed, thousands of pioneering patients have gone before, perhaps facing their own uncertain futures. Today, we place great hope in the power of science. Many lives have been saved; but, sometimes, we ask medicine to do more than it can.By turns frightening, heart-wrenching and inspiring,Open Hearts is a powerful story of medical progress, hope and survival.

Leseprobe

Open Hearts

Thanks to Jane Somerville. We owe you.

CONTENTS

Introduction

1 Beginnings

2 Minnesota, Tuesday 31 August 1954

3 When nothing is done

4 Birth was almost the death of him

5 ‘Those children are my crossword puzzles’

6 When did you last see a blue baby?

7 Going into hospital

8 Magic sleep

9 Cold hearts

10 ‘It’s a very detailed and complex operation . . .’

11 This won’t hurt a bit: recovery

12 Finding a new normal: after surgery

13 Dirty washing

14 Flying solo

15 Grown up and trying to do normal things

16 Having a baby – what’s the big deal?

17 Rhythm problems and other woes: the rise of the expert patient

18 ‘Death alone is certain, the time of death is uncertain’

19 What’s new? (Besides innovation)

Appendix

Acknowledgements

References

Index

INTRODUCTION

I had a 1950s and 1960s childhood. Every summer we went to the same caravan park in the same sand dunes in Donegal and our first ritual on arrival was to take our pocket money to the shack-shop to buy a bar of Cadbury’s Mint Crisp, not sold in Belfast. The shop-family had two daughters, both older than I was, who never strayed from the step outside. Even the six-year-old me could see that they were ‘different’ and, knowing what I do now, I realize that both had Down’s syndrome. The sisters had the sameness of twins, but it was not their almond-shaped eyes or the way they held their tongues or their incurious manner that was so striking. They were definitely the wrong colour. Their lips were a dark, dark purple, their eyes were cerise and their complexions a weird blue. Children are told not to stare, but I promise that, even as an adult, you would have taken a second look. I referred my ‘why’ to my parents. Both doctors, they gave me my very first lesson about hearts and what you can deduce from appearances; for some years I became a proper little Sherlock Holmes. There came a year when only one girl was on the step.

Nowadays, if you ask people younger than a certain age when they last saw a ‘blue baby’, they often look a bit blank. With the march of progress, the phrase ‘blue baby’ has disappeared from the vernacular, along with children blue enough to stop us in the street. Before surgery became available, children born with badly organ-ized hearts lived out the ‘natural history’ of whatever defect they were born with, their bodies succumbing to the lethargy of living without an efficient circulation. Some would survive days, others to school age; very few became young adults who we might see out and about. Before the early 1960s, newborn babies were not rushed to specialist hospitals as they are today. The few surgeons of the time with any know-how would not have operated on them anyway; surgery was reserved for older and physically bigger children – and hardly any babies with critical heart disease would live to grow to their target size for having an operation.

The very first operation on a blue baby took place in the Johns Hopkins Hospital in Baltimore in late 1944. The surgeon was Alfred Blalock, who went on to visit London to perform the first operation in Europe in 1947. The variety of congenital heart disease is prodigious but his operation benefited a group of children who were blue because their lung-blood supply was restricted by some blockage inside their hearts. In the 1940s, the heart itself was still ‘off limits’ to surgeons, but Blalock’s operation redirected blood heading to the child’s left arm and diverted it into their lung arteries. These operations were palliative – the problems inside their hearts remained – but their effects were dramatic; the extra oxygen that the shunt afforded to the children’s bodies was life-changing. By the early 1950s, American surgeons were beginning to operate inside children’s hearts – accounts of their staggeringly low-tech equipment (ice baths, circuits mingling a parent’s blood with their child’s) will turn up in this book. These operations yielded the earliest survivors of ‘open-heart surgery’, and some recount their sides of the stories for us.

By the 1960s, confidence, competence and the availability of expertise led to an increase in the numbers arriving at hospitals in the hope of treatment, though the death rate for babies before, during and after surgery was still prohibitively high. The most straightforward defects were necessarily the first to be tackled, but over the next two decades major technological improvements – in diagnosis, anaesthesia and intensive care as well as surgery – meant that by the late 1980s the list of congenital heart problems that could not be alleviated at all had shrunk to almost nothing.

At the beginning, children had to be quite disabled by their hearts before surgeons could justify the risk of operating, so many of the early survivors remember their lives before, during and after their surgery. Some have offered to talk to a curious veteran from the other side of the doctor-patient relationship – for instance offering their perspective on the atmosphere of children’s wards. Some had been dragged from their parents on admission to hospital, nurses had sometimes been unsympathetic, stays had been long and parental visiting might only be allowed once a week. In such an environment, children look out for the good people, and there are also accounts of heart-stopping acts of kindness. The climate of children’s wards has changed but, related to the advances described in this book, most contemporary children born with heart problems have had their ‘main’ operation by the time they go to school (making most of them pink or nearly pink) and unlike their predecessors, most have no memory of their own life-or-death crises – ordeals that parents of every era remember only too well.

For the avoidance of doubt, ‘blue babies’ are still out there – you simply have not noticed them. From a class photograph, I doubt you would pick out the child with only ‘half a heart’ who had already undergone an arduous series of operations before he was five years old – though you might spot him if you watched carefully on the sports field. Currently, in Britain alone, over 1,000 heart operations are performed every year on babies before they are even one month old. In the USA – thanks to childhood surgery – more than one million adults are now thought to be living with heart diseases that they were born with. Numerically, congenital heart disease is no longer primarily a problem of childhood – indeed, for the past twenty years, affected adults have outnumbered the children. With numbers like these, we have to wonder why we have not met one of the people this book is about. We probably have. Most patients keep their scars well hidden and many told me how ‘our’ many misconceptions make them cautious about who they tell.

The whole project of heart surgery on children has been a ‘gift that keeps on giving’ for the press, who love a triumphalist narrative of a surgeon meeting a challenge. This cliché is careless for several reasons. Firstly a child’s survival through surgery depends as much on the rest of their team’s competence in preoperative diagnosis, anaesthesia and post-operative intensive care; we hear how these evolved over the decades. Secondly, even now, few operations offer a ‘cure’. This means that many survivors are negotiating adult life with disabilities from incomplete repairs and some are squaring up to premature death. Finally and most unfairly, the part played by trailblazing patients is completely unacknowledged.

Blalock and many of the early ‘open heart’ surgeons became international celebrities, yet as accounts of burgeoning numbers of novel heart operations came to appear in the medical press, the patients’ identities were always redacted. But it is the patients’ stories that form the backbone of this book. No less than the surgeons, they were truly pioneers, yet no acknowledgement is ever made of how crucial they were to events. Until recently, most of them had never encountered anyone else who had had similar surgery. But now – like people who research their family history – patients use the internet to get in touch with others who share their diagnosis and the oldest patients are like the elders of a scattered tribe.

Almost all of my professional experience has been at the childhood end of congenital heart disease services, though I looked at my profession from another angle during the treatment of my own child for a queer cancer. That experience also taught me that – when a doctor takes their eyes off their ‘patients’ – they are not ‘patients’ at all, just kids trying to get on with their lives. This was the beginning of my interest in the stories of out-of-hospital lives.

Most of the ‘patients’ I talked to – in person, by Skype or over the telephone – I ‘met’ as strangers; it has been curious to have the doctor-patient relationship out of the way. To have an hour, sometimes two, occasionally a couple of days to spend with people is not something that ever happens in a hospital out-patient clinic. As doctors, we undoubtedly bear witness to what patients go through in hospital, but listening is another matter. Questions went back and forth in both directions; I think many of them enjoyed the sessions. They have been mainly adults – most children struggle one-to-one with an inquisitive stranger. We went through their life stories in much the same way that the book is constructed: birth, diagnosis, childhood, hospitals . . . Some had become parents themselves and had only then recognized what their own families had been through. A particular privilege for me were the pensive conversations with survivors of early operations for conditions that were edgy at the time but that we now think of as routine – Fallot, VSD (conditions that are explained more fully later). These were people of my own generation and it was poignant to hear them decades later describing their pin-bright recollections of fleeting childhood incidents in family life or the school playground. Memory is fickle; we forget what we want to remember and remember what we want to forget. But in the back of my mind I was constantly wondering how today’s children, born with the problems that are now on the margin of our capabilities – Hypoplastic Left Heart, complex pulmonary atresia, single ventricle – will look back on their childhoods in forty years’ time.

As may become evident if you read on, I have become a fan of what has come to be called the ‘Adult Congenital Heart’ community. Their very existence has been framed as a triumph of human progress over nature – but this tells only the ‘medical’ part of the story. The early patients were pathfinders; their survival launched them into uncharted waters. Incognito in books about the history of medicine – and often living ‘incognito’ among us – I believe they deserve more credit. Father Patrick (who you will first meet as a four-year-old learning to walk in a 1960s hospital ward) chides me for my ‘rose-tinted’ admiration of the ‘graduates’ of our surgery. He has good judgement, but I am not going to apologize for my admiration. To them I say: ‘Don’t underestimate what you have done.’

1

Beginnings

Making a blue child pink is one of the great stunts of modern medicine. Cosmetic surgery can change a face or a breast, but the first ‘reveal’ after these operations looks worse than before the surgeon started – stitching, bruising and distortion all take time to settle. But there is a moment in every operation on a blue baby when the scale of the transformation in the child’s appearance is revealed, the anaesthetist at the head of the table smiles, and an hour later the baby’s parents can hardly believe their eyes. The stereotypical surgeon is much harder to impress.

The year was 1947. Even Fellows of the Royal College of Surgeons of London start glancing at their watches, shifting in their seats and looking forward to a drink after two weighty lectures. The speakers were Drs Helen Taussig and Alfred Blalock who, in 1944, had accomplished the first ever operation on a ‘blue baby’ at the Johns Hopkins Hospital in Baltimore. The ‘blueness’ of their patients was plain for all to see. Born with a defect inside their hearts that blocked the way through to their lungs, their blood was short of oxygen, their energy levels pitiful and most would die after a short and sorry life.

Between 1945 and 1947, youngsters had been arriving by the hundreds at Blalock’s hospital, blue and in wheelchairs, and the lucky ones had been trotting out pink and unaided a fortnight later. The success rate had improved and the operation standardized to the extent that Blalock could begin teaching it to other surgeons. His visit to Guy’s Hospital in London was his first trip outside the USA. There, with the local chief Russell Brock, he had spent a week operating on eight children. Before returning to the USA, Blalock was invited to address his eminent surgical audience. Realizing that history was being made, someone had recorded a flickering cine film showing the two surgeons at work; with 1947 film, lighting and lenses, it cannot have given much more than a record of the cast-list. The lights in the auditorium were dimmed for the cine and there was some shuffling on stage. Then, as Brock remembered:

a long searchlight beam traversed the whole length of the hall and unerringly picked out on the platform a Guy’s nursing sister in her attractive blue uniform, sitting on a chair and holding a small cherub-like girl of 2½years with a halo of blond curly hair and looking pink and well. It was a Madonna like tableau . . . no one there could possibly forget it.

Unbelievably, there was a standing ovation. Surgery for congenital heart disease, born in the USA, started in Europe that day.

Beginnings are exciting times and the legacies of Blalock and Brock are assured.* The operations that each championed are named after them and both published many medical papers. After his lecture, the Royal College of Surgeons festooned Blalock with bling and braid; later, Brock became an English lord. The first Baltimore operations have even been immortalized in a Hollywood film, Something the Lord Made (2004) – though this was a revisionist account and the title referred not to Blalock’s surgical skill but that of his African-American lab assistant.

Though we know that the very first baby to receive a Blalock shunt was called Eileen Saxon; she only survived for a few months. Nothing further is known of the little girl on the nurse’s lap. As she grew, the amount of blood going through her shunt would have gradually failed to meet her body’s oxygen needs and her energy levels must have declined as she became become bluer again. The radical solution of opening up her heart to deal with the problems inside it was more than a decade away in England. Again, the ‘first’ was to be in the USA.

_________

* Though as it turned out, neither man was temperamentally suited to the next challenge in congenital heart surgery: operating inside the heart itself required teamwork.

2

Minnesota, Tuesday 31 August 1954

Spectators looking down through the glass ceiling above a Minneapolis operating room witnessed a drama of first-night unpredictability playing out below them. The operating table looked like a kitchen table, neatly laid with a green cloth, and with shiny steel silverware arranged at one end. Androgynous figures, hatted and masked, indistinguishable in loose-fitting gowns, stooped close together, arms sometimes entwined; the usual conventions about personal space being suspended in an operating room. Within the central tableau, a bright spotlight drew attention to the compelling spectacle of a dark-red beating heart. Choreographed activities with an insistent momentum played out in the central huddle; esoteric sub-plots were discernible outside the spotlight. There were pauses with everyone standing stock-still as their attention converged. There was blood. All the drama somehow distracted from what was concealed under the green cloth.

Surgeons have often been painted as the main protagonists in the war against congenital heart disease. Portrayed as generals, some have been tacticians, others technicians, some prudent, others reckless. Some have rightly been decorated for their roles in the campaign, a few have become celebrities. But not one of these surgeons ever faced the prospect of dying on the battlefield. This book tells the story of the people underneath the green drapes, and in this particular operating theatre there were two.

* * *

In 1944, Mickey Shaw’s life had an inauspicious start. The nurses whose job it was to wrap up babies for their homeward journeys in a Minnesota February gave Mickey’s mother the wrong baby. Unpacking her bundle in her bedroom at home, there ensued one of those set-piece episodes that only seem funny in retrospect – a drive at breakneck speed, red-faced staff, two frantic mothers.

Young Mickey Shaw never really thrived; a puny baby, late to walk. He was the second of four sons but his little brother soon outgrew him. He walked exasperatingly slowly – half a block would leave him breathless. He would stop, squat and hug his knees to his chest, looking blue enough to alarm passers-by. By the time he was going to school, the whole town must have been aware of him being towed along by a reluctant brother on a wagon that the family used for groceries. ‘Heart problem’ was an easy call for the school doctor, who suggested that his mother take him to see a specialist in Minneapolis-St Paul which, although only sixty miles to the east, was a whole world away; locals called the twin cities ‘Sodom and Gomorrah’.

There, in 1950, Mickey had some tests, including a cardiac catheterization. The doctors opened a vein in the crook of his elbow and – watching on cine X-ray – ran a plastic tube towards and through his heart, taking blood samples on the way to track how the red and blue blood was streaming. By the end of a week, they had a name for Mickey’s condition. His blue colour was due to a shortage of oxygen in his blood and that in turn explained his lack of stamina. It is unlikely that the specialist conveyed much of the anatomy of Tetralogy of Fallot* to Mickey’s mother, but the implications were explicit. ‘Sadly’ he had a heart problem; ‘yes’ the problems were right inside his heart; ‘no’ there was nothing they could do and ‘yes’ he would die before he was sixteen. Mickey Shaw’s name, address and diagnosis was put on a card and added to a growing file of children with inoperable heart defects.

Mickey’s mother, Helen, was a more than decent woman: a good Catholic whose husband had abandoned her with no maintenance soon after the fourth boy was born. Rather than accept welfare, she did cleaning jobs on Saturday and Sunday afternoons and on weekdays worked in an industrial freezing unit, standing in an inch of water eviscerating turkeys. Her boys were lively but polite and well groomed. The family dressed neatly for Mass on Sundays and said grace before meals. Respecting her dignity, shopkeepers may have put a few dented cans her way and the nuns at school very probably looked out for Mickey. These were the days when pupils were taught how to ‘duck and cover’ in the event of nuclear war; a time of bikes and milkshakes and freeze-dried jeans and the Lone Ranger on the radio.

When the press began to publish news of heart operations on children in Baltimore, Helen wrote a pleading letter to the director of the hospital. In principle a Blalock shunt would have helped Mickey, but in practice, with no family money and no local expertise, there was no operation on offer. She became despondent – three boys boisterous and one gradually deteriorating. It was painful on some evenings for her loyal sons, each trying to help with paper rounds and lawn-mowing jobs, to overhear their uncomplaining mother crying in her room.

Then in March 1954 another letter arrived, inviting them back to Minneapolis, this time to meet a surgeon who offered some hope. He was prepared to try a completely new approach – to operate inside Mickey’s heart. In her straitened circumstances, money was the first problem that panicked Helen, but the surgeon promised that the research fund of the hospital would pay the medical bills, the family only needing to take responsibility for some other matters: the team would need four pints of blood for the operation to go ahead and mobilizing this was to be the family’s first obligation. Though in development, no reliable machine was yet ready to take over the work of a person’s heart and lungs. Instead, the family would also need to find an able-bodied adult with the same blood group who was willing to be a party to the operation and use his own body to support Mickey’s circulation while the surgeon was operating inside his heart. Problematically, Mickey’s blood group was AB negative – by some margin the rarest – and neither his parents nor any of his extended family had the same group.

But Litchfield, Minnesota epitomized ‘small town America’ and Litchfield looked after its own. There follows a story of a human-sized community playing to its strengths. Newsletters and dinners alerted people to Mickey’s situation. The Catholic Church worked through the Knights of Columbus, the veterans through the Veterans of Foreign Wars and the district Red Cross organized blood drives. A local man and father of three got a call out of the blue. The man’s dog-tag data was still on file after his service in the Second World War and on the telephone was an American Legion commander. The bizarre proposition was that the man submit to surgery so that his heart and lungs could support another man’s child while the boy’s heart was opened for a life-saving operation. After meeting Mickey’s family and the Minnesota surgeon, and after the risks and substantial uncertainties had been explained, Howard Holtz stepped up ‘because he hoped someone else would do the same for his kids if they were sick’. His brother Vernon also offered. Despite his restrictions, Mickey was a happy-go-lucky kid who would have learned little of the plans. His mother can only have been grateful; she had enough to do to keep the show on the road at home and organize friends and relatives to look after the other boys. A priest visited.

Underlining the solemnity of the journey, on 24 August 1954 his estranged father turned up to drive Mickey and his mother to the University of Minnesota Heart Hospital in Minneapolis. They would have been an eye-catching group. Mickey, aged eleven and a half but the size of an eight-year-old, wore jeans and check shirt, his parted hair stiff with Butch Wax. Even if trying not to stare, what nobody could quite ignore were his purplish-blue lips and improbably grey complexion, and the way he crouched on a seat with his knees up to his chin.

The hospital had opened only three years previously, long and low with sharp corners, a homage to red brick. Anyone who has themselves paused to take a deep breath on entering a major children’s hospital will recognize the inextricable emotions of hope and fear as you cross the threshold. Upstairs, a forty-bed ward with babies and children, some in for tests, others for surgery, heart problems, leukaemia and infections – a heavy workload with many children likely to die. Down the hall, a ward with thirty patients, some confined to iron lungs; this was the era of the last American polio epidemic. The nurses, who wore white caps on their heads, starched white dresses and thick-soled white shoes, were stern and terribly busy. Visiting hours were strictly enforced: 2–4 p.m. and 6–8 p.m. Toddlers feeling abandoned would be wailing, often tied by their legs to their cribs. Forlorn children would rock themselves to sleep. Offsetting this grim atmosphere was a playroom with a sand-table where children could make mud-pies.

Mickey spent a few days in hospital being checked out. He was weighed, had an electrocardiogram, X-rays, blood and urine tests. Already an object of fascination, many young doctors visited to examine the ‘Tet up for surgery’. Before the operation, one of the surgeons – a Dr Lillehei – came to greet him; other patients remember this man as the only doctor who took the trouble to talk to children directly. The AB-negative volunteer blood donors turned up at the hospital the night before the operation to give their pints of fresh blood, and Howard Holtz was admitted and ready.

On the morning of surgery, Mickey was trussed up in a cotton sheet and wheeled down long, bright corridors to a busy operating room. He was helped onto one of the operating tables – still vaguely awake – and a blood pressure cuff wound around his arm. Some local anaesthetic was injected around his inner ankle. A surgeon exposed the big vein that runs there and inserted a chunky metal needle. Because their oxygen levels are so precarious, putting blue children to sleep safely is quite a challenge, and the anaesthetist was loath to do this until a good drip was in place. But when all was set and after a few reassuring words, a mask went over Mickey’s face and he drifted off. The anaesthetist prised Mickey’s mouth open to insert an instrument to pull his tongue forward and slipped a tube right down his throat, past his vocal cords and down into his windpipe. Mickey breathed on, each breath shifting air through a black rubber bag next to the anaesthetist’s hand; now the operation could start.

Observers in the dome above saw Howard being moved onto another operating table and more surgeons arriving and scrubbing up. One team attended to Howard while the other painted Mickey’s chest with brown antiseptic and covered him head to toe in green sheets, leaving only a rectangular area framing his chest on which the spotlight was focused. An experienced surgical onlooker would have been struck by the curiosity of having two tables in a single overcrowded operating room and the uncommon spectacle of the two surgeons, Drs Varco and Lillehei, on either side of the main table, working without any sense of rank, each dissecting or stitching what was most readily accessible from his side of the table. One visitor remarked, ‘It was impossible to see who was doing what, as there were four hands working in unity. It was extremely interesting to watch.’ They proceeded to make the most immense incision – armpit to armpit – aiming between ribs and below the nipples, using a saw across the sternum. Like a needlewoman anticipating seams, one surgeon made little nicks in the skin to guide the matching-up of the edges at the end of the operation. After controlling bleeding vessels, the child’s chest was opened up like a clamshell to reveal heart and lungs; huge metal retractors were positioned to keep the field of view clear. With his lungs open to the air, Mickey could no longer breathe for himself and the anaesthetist took over this responsibility by squeezing the black rubber bag.

The heart and the vessels coming in and out of it were exposed, preparing the field for the plumbing that would permit the surgeons to open Mickey’s heart without prejudicing the rest of his body. On the adjacent operating table, the other team was preparing the main vein and artery at the top of Howard Holtz’s right leg, slipping a plastic tube into each. The tube in the donor’s artery was connected to another and fed backwards into Mickey’s main artery; it would provide red, oxygenated blood to Mickey’s body when his own heart wasn’t pumping. Another tube joined Howard’s major vein to the main veins returning to Mickey’s heart. Before arrangements were quite complete, Mickey’s heart suddenly lost power. As if connecting jump-leads to recharge one car battery from another, the team quickly finalized the ‘cross-circulation’ plumbing so that Howard’s heart and lungs could take on the job of supporting Mickey’s brain and body. His heartbeat returned, confirming that the situation was no longer precarious. But this circuit only produced between a sixth and a quarter of Mickey’s normal heart output, so it could not be relied on to maintain stability for long. The clock started.

Fixing Fallot’s tetralogy has two main steps. The surgeons need to open the heart, find and then close the hole between its two pumping chambers; with the hole closed, the blue and red blood can no longer mix through the gap. They then need to relieve the blockage between the heart and the main lung artery.

With Mickey’s circulation served by Howard’s heart, immediately in front of the surgeons was Mickey’s right ventricle, the part of the heart that pumps blood into the lungs. The heart was still beating feebly when they incised it with a scalpel; as expected in Fallot’s tetralogy, the muscle was much thicker than normal. Sucking the blood out from the interior of the cavity exposed the anticipated hole between the pumping chambers; so far so good. The nurse at the table handed six curved needles threaded with heavy black silk, one after another, to the surgeons to stitch the hole closed. Before tying down the last stitch and closing off the deeper left ventricle from the outside air, they filled its cavity with salt solution to avoid the possibility of air being pumped around the circulation when the heart began to beat effectively. The team then needed to core out some muscle that was blocking the way through from the interior of the right ventricle to the main lung artery. They kept the bits to photograph for posterity, knowing that this operation was a first of its kind. The right ventricle was then also filled with saline, the initial incision was closed, the heart allowed to fill and accommodate to its new conditions. The heart took over confidently and the cross-circulation circuit connecting Mickey and Howard was closed off.

‘How long?’ The heart had been open for eleven and a half minutes. Relief was in the air, but there was still a lot to do. After his leg incisions were closed Howard was the first to wake up and he was wheeled back to his ward. Mickey’s chest had to be reconstituted, layer by layer, his skin finally stitched using the marking nicks to line things up. Just before the end of the operation the anaesthetic was lightened so that, when the green drapes came off and Mickey was stirring and gasping for breath, the tube was taken out of his throat, and his lungs and his repaired heart were really on their own. Before leaving the spotlight, the surgeons shook hands over the finished job and went off, each to tackle their next case – perhaps the removal of a gall bladder or a hernia repair.

Mickey woke in a weird environment. His bed was inside a clear plastic oxygen tent, its sides tucked under the mattress, its interior cool and damp. The oxygen was piped in from a tall cylinder by the bed and humidified with ice cubes. When the oxygen tent’s opening was unzipped, it usually meant an injection (intramuscular penicillin four times a day) or a blood test (needles blunt from use and reuse). His chest, which had been cranked apart, hurt. Moving hurt, breathing hurt, crying hurt, coughing was all but impossible; post-operative pain relief was not an advanced art. Mickey spent two days in a bay on his own with an experienced nurse all to himself; his parents could only peer in through a window. For two days they couldn’t get close enough to see how different he looked.

Back on the main ward, he was still in his tent but beginning to eat and drink. This was a lonely, alienated time as bedbound children were pretty much ignored, apart from doctors’ rounds or nurses’ checklists involving more needles and prodding. Mobile children would literally run away from nurses who pursued them with injections, immune to negotiations involving cookies or juice. Dressings were a misery. The bandages of the day were made of sticky crepe strapping, the stitches underneath snaggy and sore. But Mickey gradually started moving about, first into a wheelchair, then to the playroom; his wounds healed, he could cough (sort of) and when he could raise his arms almost to shoulder level, he was ready to go home. Before the operation, his dad had offered him 50 cents for each detested needle, but the bill got so great that Mickey accepted a new bike instead.

News was leaked to the press when – three weeks after he first went into hospital – Mickey returned to Litchfield, as pink as his brothers and ready to join the mainstream of life, a coast-to-coast celebrity. The achievement of the open-heart repair of a blue child was a realization of American virtues: the Minneapolis Heart Hospital itself had been built by the philanthropic efforts of the Variety Club and in 1952, President Truman had declared ‘war against heart disease’ and made federal funding available for heart research. Here was a dividend that would go down in world history.

But equally ‘it takes a village to raise a child’ and Litchfield was chuffed with what it had achieved; their town was in the national newspapers. Mickey’s celebrity was difficult for his brothers – there had never before been a new bike in the house, let alone a Labrador puppy ‘won’ in a rigged prize draw. They were alert to any whiff of favouritism from their mother. But over the winter, a new family normality descended. Mickey had missed a lot of school and with a bright younger brother snapping at his heels in the class below, he couldn’t afford to be put down a year. That was a struggle. But at twelve years of age, for the first time in his life his legs could run when he asked them to. After being excused for years, now he could be required to shovel snow.

The following year he was back in Minneapolis for a check-up. Contemporary surgeons, who do not expect to be in and out of the heart of a child with Fallot’s tetralogy within an hour, will not be surprised that tests showed that the hole in Mickey’s heart had not been quite closed nor the blockage under the lung artery quite relieved. But the imperfections balanced out and his progress testified to the operation’s success. His fame lasted for more than fifteen minutes. His mother mastered her stage-fright and she and Mickey did several ‘benefit gigs’ for the hospital. Somewhere in the family archive is a photograph of Mickey presenting a cheque for one million dollars; his operation had been a good investment for the research fund.

As teenage life supervened, Mickey turned into Mike, had a growth spurt, became a dedicated smoker, developed teen-idol looks, joined a band and found rock and roll. As the boys grew taller than her, their mother’s influence waned. Mike was up for an occasional fight, usually with a brother. Through high school he took on jobs that middle-sized kids could do – cleaning in the turkey plant, serving in a gas station, selling candy at baseball matches. He was glad to quit high school when he was eighteen. By that time, music was the constant in his life – he played bass guitar in the Nightbeats, the Embers, the Defiants and most successful of all Shaw-Allen-Shaw, the band that thawed the longstanding tension with his no-longer-little brother. They played Minnesota social halls almost every night for seven years and made two records that are still available on eBay. Mike had enough scars to avoid the Vietnam draft but in his fifties he needed coronary surgery. Over the years he never left the music business, working in music stores, as a booking agent or promoter or band manager. You can look him up in the Minnesota Music Hall of Fame. He married happily and his surgeon attended his twenty-fifth wedding anniversary. He had four kids – all musical – and six grandchildren, none of whom would exist but for his operation. His mother believed in miracles and her son was ‘the one’ who lived at just the right place at just the right time.

Howard Holtz, the modest man who supported Mike’s operation is still alive in his eighties. Sixty years after the operation, I could not persuade him to take any credit and had to turn to other local men for a ‘testimonial’. It turns out that what Howard Holtz had done for Mickey was completely in character; he had always ‘gone the extra mile’ for the citizens of his patch of the Forest City township where he maintained the roads and I had not been the only one to find him so self-effacing. Had there been complications, we trust that Litchfield would have looked after his wife and children. Although Dr Lillehei is often portrayed as the star of the cross-circulation operations, for me the outright hero of the first ever Fallot repair was the donor, Howard Holtz.

* * *

The repair of Mickey’s heart was unquestionably a ‘first’. But in the hothouse atmosphere of evolving cardiac surgery, ‘first’ could mean different things: the first floating of an idea, the first experiment, the first (failed) attempt, the first success, the first published record. In the story of the open-heart repair of ‘blue’ children this was a ‘first success’, but other children, adults and many, many animals had died on the way to its achievement.

Before Mickey’s operation, surgeons in Toronto had tried to repair the hearts of five children with Fallot’s tetralogy. While their hearts were open, their bodies were supported by a pump that circulated the patient’s blood through the lungs of four recently killed monkeys to oxygenate it. In London there were two rushed attempts without any machine or cross-circulation at all – just cooling the body in the hope of limiting brain damage. All seven children had died. There may well have been other attempts, but failure and shame were close cousins in the surgical community, so we cannot be certain that every death was made public. Using a parent to support their own sick child’s circulation, Lillehei had already had a few successes (and failures) in closing straightforward ‘holes in the heart’. But the Minneapolis operation using a donor and cross-circulation had provided the ‘first success’ in repairing the heart of a blue child.

The proposition that a parent uses their own body to maintain their child’s life while the child’s heart is opened up has an Old Testament feel about it, perhaps fitting for such a God-like endeavour. But striking though it sounds, cross-circulation was not a new idea in medicine. As soon as surgical techniques were up to the job, experimenters were joining together the circulations of two live animals to help answer questions about how blood carries chemical messages; hormones are an example – manufactured in one place, active in another. In a ‘proof of principle’ exercise, a surgeon at Johns Hopkins Medical School, Baltimore in 1929 established cross-circulation in over forty pairs of dogs by stitching a large artery of each of a pair of dogs to a large vein of the other. After the operations, the pairs of dogs were lashed together with sticky tape and allowed to wake up, move around, feed and sleep. Most died quickly, the suture lines tearing apart or blocking with a clot, or one dog simply bleeding into the other. But two pairs of animals padded around, their circulations joined, for six days. Pedestrians approaching the campus could not avoid knowing about the experiments; there were anti-vivisection posters in a shop window especially rented by the Society for the Prevention of Cruelty to Animals.

By the 1940s, medical experimenters had moved on to trying to use cross-circulation to help human patients. One option was to mingle the circulations of two patients who each had something to gain – patients with the same blood groups but with different illnesses. A woman who had sudden drug-related bone-marrow failure but whose kidneys were working was paired with a younger woman with kidney failure but good bone-marrow function. The blood tests of each improved, but keeping this up day after day was arduous. All parties decided to abandon the trial after two weeks and both women died.*

A second option was to pair patients, one of whom seemed to have something to gain and the other who seemed to have little to lose. In a Californian hospital in 1949 before the advent of chemotherapy, a three-year-old girl was dying of leukaemia; she was moribund, hard to rouse, on the brink. We know her initials were ‘J.S.’ Someone made an incision to find the big vein in her right groin and stuck in a fat metal cannula attached to a length of clear rubber tubing. Bumped up close in the adjacent bed was a thirty-two-year-old man, himself dying of lung cancer. His right groin was also exposed and cannulated. The tubings were joined together with a syringe at the junction and blood was manually sucked from the child and injected into the man, then vice versa, again and again. After two hours of cross-circulation, two-thirds of J.S.’s blood volume had been exchange-transfused and both patients were terribly uncomfortable, needles in their groins, blood seeping from their wounds. The child became a bit more alert and her numbers of leukaemia cells circulating had dropped modestly. Next time, the man in the next bed was a fifty-one-year-old with cancer ulcerating his lips, tongue and gums. Little J.S. passed away eleven days after a third exchange and both men died in their own time; autopsies showed neither had picked up her leukaemia. Each of the dying volunteers had signed a consent form confirming that they had understood that their own deaths might be accelerated or that they could acquire the child’s leukaemia. It is not recorded what form the dying children’s parents signed or even whether they observed her ordeal. Now that we understand more about leukaemia, we know that the child, her parents, the dying volunteers and the doctors were all embroiled in a hopeless hope.

A last option was to pair a sick person with a fit volunteer, putting a healthy body at the service of the ill and dying by joining up their circulations – just as the cardiac surgeons were to do later. Parents, in particular, were willing to take considerable risks to help their children: in Italy, mothers shared their circulations with daughters in an effort to relieve the ‘persistent vomiting’ of pregnancy. In the USA, an attempt was made to save a son whose kidneys had stopped working by using his father’s kidneys. But when the father later died as a result of complications arising from this procedure, this foreshadowed the criticism that the Minneapolis team later faced when they publicized their open-heart methods; these were interventions that could produce a 200 per cent mortality.*

Thus already by the early 1950s it had become clear that the impending death of a child can make people – parents, doctors and dying patients – take some very extraordinary decisions. Also, there was a modest practical experience of cross-circulation to draw from. In therapeutic terms, the interventions had been largely ineffective but a lot had been learned about how to do it; a ‘blood transfer apparatus’ had even been patented. But matters of optimal blood-flow rates and oxygen transfer had not been addressed and for this the Minnesota surgeons went back to the animal lab to refine the cross-circulation technique so that one set of heart and lungs could serve two bodies.

In the lab, dogs were chosen because they were cheap and available in a range of sizes. There the Minneapolis surgeons sorted out the best tubing (clear hose used for siphoning beer), a method for matching the flows in and out of the two animals (a pump sold for use in the milking industry) and a regime for preventing clotting in the plumbing. Then they would stop one of the dog’s hearts so that it depended on the other dog for a short period. When the assembly was almost perfected, they were impressed that the dogs would wake up promptly and seem to behave normally. But they wanted to be as sure as possible that brain damage was not a side effect of the set-up. A local cardiologist who trained golden retrievers for hunting offered some of his dogs as subjects; after the cross-circulation experiments, they seemed to remember all their commands. The team also realized that any ‘donor’ would need to be much larger than the ‘recipient’ if the donor’s lungs were to provide enough oxygen for both bodies while the recipient’s heart was open and not working. So once again, sick children were to be the subject of the first human experiments.

The first Minneapolis cross-circulation operation took place in February 1954 and the forty-fifth and last in July 1955. After that, acceptable heart-lung machines had been developed. Forty-three of the operations involved a parent as the blood-group-matched donor. Apart from Mickey Shaw and Howard Holtz, the only other exception was the pairing of a three-year-old boy with a woman prisoner from a local penitentiary.*

Sixteen of the forty-five children died in hospital. One operation, in which a mother was sharing her circulation with her daughter, was halted when horrified spectators in the dome above the operating table saw what nobody on the operating-room floor below had noticed – a column of air being pumped into the mother’s circulation through the clear tubing. There was no audio connection between upstairs and downstairs. The mother’s heart stopped as the air was pumped through her circulation and the operating room descended into commotion; though she survived, the bubbles that had blocked vessels in her brain left her more childlike than her children.

The audacity and technical achievement of operating inside a child’s heart was a godsend to the world of photo-journalism. Sandwiched between an advertisement for cake-mix and another for Fitch’s Hair Tonic, Life magazine of November 1954 has a photo-montage of the whole glorious story: a sick baby, a ‘living heart cut open’, a husband bending over his wife after her return from the surgery and a final photo of the mother’s reward as she cradles her post-operative infant.

When we learn that the daughter of that brain-damaged mother survived to be operated on using a heart-lung machine six years later, we have to wonder if some of the other patients who had died post-operatively might not have been better advised to wait. In principle, there had even been another option for Mickey Shaw. A Blalock shunt operation would at least have made him pinker and given him more energy; such surgery had already been available for almost ten years. This ‘shunt’ option had been mentioned, but in terms of the research fund financing Mickey’s surgery, it was the open heart offer or nothing. For Mickey’s parents, their lack of money meant lack of choice.

_________

* Tetralogy of Fallot a.k.a Fallot’s Tetralogy, ‘Fallot’ or ‘Tetralogy’ or ‘Tet’.

* Not before the woman with marrow failure’s kidney was transplanted post-mortem into the renal failure patient. The kidney functioned for a while until she too died from an abscess at the suture line.

* Robert Liston, a surgeon in pre-anaesthesia, pre-surgical-hygiene London was later described as ‘the fastest knife in the West End’; he held the knife between his teeth to be better able to use a saw. Unfortunately, during one rushed amputation he accidentally also amputated the fingers of his assistant and slashed the coat of a spectator. Patient and assistant subsequently died of gangrene and the spectator, terrified that it was his own blood on the floor, died of a heart attack on the spot: an operation with a 300 per cent mortality.

* In that era 40 per cent of blood donations in America came from people in correctional facilities: the Minnesota men’s prison had a club called the ‘Leaky Arm’ club.

3

When nothing is done

Before the 1940s, there was no treatment for children born with heart defects and, because their private lives were undocumented, it is difficult to illustrate their destinies. Yet in the first half of the last century there are two people born with heart conditions whose life-stories help us to understand how heart disease curtails the existence of people left unoperated. The only reason we know about them at all is through their biographers – because they became famous before they died. One of these was Dick Ket, later a celebrated artist, born in 1902 with what was almost certainly tetralogy of Fallot, though this was not diagnosed in his lifetime.

Ket was born in Den Helder, a Dutch coastal town that had been going downhill since the eighteenth century. The family later moved to The Hague with his father’s job as an army pharmacy assistant. Ket was an only child with a protective mother. He was bullied at school. Delivering medicines around town to help his father, he was slow, puffing and stooping as shoppers overtook him.

He went to art school in Arnhem at the age of twenty and there met teachers who introduced him to the worlds of theosophy and mysticism. These ideas became important to him: perhaps he sensed that his life would be short and could end at any moment. There are many competent sketches of landscapes and townscapes in Dutch collections from his art-school era, along with the first of his self-portraits: one showing a larky young man dressed in a spivvy suit and another, from 1927, in which he is a little more dishevelled but still very much the student.

By the time he was twenty-six, his disability was becoming very limiting; the family had moved and the commute to Arnhem had become intolerable for him. A house was built to his specifi-cations with a ground-floor bedroom and studio, and after 1930 he rarely left home. Largely isolated from the art world, his artistic style matured. In 1932 he painted his girlfriend, Nel Schilt, and they were briefly engaged; although she married someone else a couple of years later, they kept in touch by letter to the end of his life.* He had a one-man exhibition in 1933, which he did not have the strength to attend, and in 1935 won the Queen’s gold medal for a self-portrait. But within a year he was struggling even to finish his canvases. He was sometimes in bed for weeks.

Tetralogy is a common diagnosis that sometimes allows blue babies to survive through childhood; it is the condition shared by Mickey Shaw and many of Blalock’s patients. Every cell of our bodies needs oxygen, and these patients’ cells don’t get enough. The less oxygen that is carried in the blood, the bluer, more limited and fatigued the patient will be. Severe lack of oxygen will gradually ruin every organ and tissue: brain, muscle, heart, skin.

As Ket’s horizons narrowed, he started to paint the self-portraits for which he became best known. These serve as his completely unsentimental, almost forensic record of how his heart disease was wrecking his body. In his studio with its gloomy, prison-like background – no window, no cosiness – he documented his blue-grey complexion, his suffused eyes and his rotten gums in meticulous detail. It is as if in his studio mirror we can see his inner state, a sort of anti-hero wedged inside his crumbling physique. He died at home in 1940 at the age of thirty-six – four years before Blalock performed the first shunt operation in North America.

Exhibitions in prestigious museums have been mounted since Ket’s death. Most catalogues claim that he would not venture out in his final years because of agoraphobia and paranoia about visitors, but it is at least as plausible that, for the last years of his life, he simply could neither walk beyond his garden gate nor bear to be viewed by sightseers from the land of the well.

* * *

Before the 1940s the brief lives and inevitable deaths of children were isolated, family affairs. Yet if we want to convey in cold numbers – rather than in stories – what happens to people born with congenital heart disease when nothing is done, we need to look at what happened in a society that kept a careful count of all of its citizens; a society that was stable over many years with little immigration or emigration; a society in which every child’s death was fully documented by doctors competent to discriminate the many forms of congenital heart disease – yet one in which no surgery was available that might alter the ‘natural history’ of the disease.

Such a society existed in Central Bohemia when Czechoslovakia was part of the Eastern Bloc (this area is now in the Czech Republic and is very well served for the treatment of congenital heart disease). In the years from 1952 to 1979, 468,733 babies were born in Central Bohemia. A full autopsy, supervised by a pathologist who specialized in heart malformations, was performed on every child who died before the age of fifteen. This provided 946 well-documented reports, including a reliable diagnosis and the age at which the child died. From this data it is possible to see the typical age at which a person born with each of the common congenital heart diseases would die. It is hard to see how such a study could ever be repeated.

In the Bohemian study, children with heart problems were at the greatest risk of dying soon after birth: 10 per cent of all deaths in childhood from heart disease happened in the very first week of life. After one month, 30 per cent of babies with Transposition of the Great Arteries had died, along with 80 per cent of those with Hypoplastic Left Heart Syndrome, 50 per cent of those with Total Anomalous Pulmonary Venous Drainage and 40 per cent of babies with pulmonary atresia. (Sorry for the jargon here, but we will meet and understand more about the particular problems later; they represent varieties of ‘critical’ congenital heart disease.) All these diagnoses involve some abnormality that makes the transition at birth to lung-breathing life very problematic. No baby in any of these groups survived to the age of two.

In the report, 60 per cent of babies born with Fallot’s tetralogy – Dick Ket’s disease – lived to their first birthday, 23 per cent to their tenth and only 4 per cent to their fifteenth; he was a rare survivor into adulthood.

The commonest of all anomalies among the Bohemian babies was a hole between the pumping chambers of the heart – Ventricular Septal Defect (VSD). One in five babies with a significant VSD died in the first year of life, but the others survived childhood. Unfortunately this did not necessarily mean that they survived unscathed. As we’ve seen, Ket’s health inexorably deteriorated throughout his life. Children with a VSD take a different course; if they survive babyhood, some will be tolerably well for many years but may yet run into trouble.

To illustrate, we know of another famous but fated victim of untreated congenital heart disease: the legendary Indian film actress Madhubala. She is to Bollywood what Marilyn Monroe is to Hollywood, an icon who died young. In her time, even an American film magazine called her ‘the biggest star in the world’.*

Born in 1933, as a child Madhubala† was a cute singer and dancer, and her family gravitated towards Bombay, famous for its film industry. When she was only nine years old, her pushy father landed her a small part in a Bombay ‘talkies’ movie. From that debut to her death, she brought in most of the family’s income. Her first big break came in Neel Kamal when the director’s wife, who was scheduled to play the lead role, died after filming had started. Madhubala knew the dialogue and stepped into the part, becoming a Bollywood heroine at the age of thirteen. At sixteen she was dominating the film posters for a reincarnation thriller, Mahal, which was a huge box-office hit that made her a superstar. She was bright and bubbly, and if she knew she was ill she was telling nobody.

During a shoot in 1954, when she was twenty, she coughed up some blood; she went straight back to filming. In 1957, she fainted on set. Nobody had previously recognized that she had a VSD. Her heart and lungs had accommodated the situation for twenty-four years, but a tipping point had been reached. By now she was going visibly blue, at first intermittently, later constantly. For the first time, a doctor diagnosed her underlying problem and knew that things were about to go downhill badly. He could suggest no treatment other than bed rest, but Madhubala was not ready to believe that she was sick and again resumed work. The movies had gone into colour, but lipstick and nail varnish were sufficient disguise.

Her fans were unaware of her medical problems, privacy being