Tuesday, October 5, 2021

Brain on Fire!


My mysterious lost month of madness

I was a happy 24-year-old suddenly stricken by paranoia & seizures. Was I going crazy?

Teratoma: One Patient One Disease?

Hospital Medicine: Pride & World Class Costs!

Nov 29, 2012

·                                 By SUSANNAH CAHALAN
·                                 Last Updated: 6:01 AM, October 4, 2009
·                                 Posted: 3:49 AM, October 4, 2009

IT was a cold March day as I walked to work from my Hell's Kitchen studio. The weather was clear, people were out in their coats and scarves, but something did not feel right. The sky was so blue, it hurt my eyes. The billboards in Times Square assaulted me with violent reds, yellows and purples. It was like the world had become brighter, louder, more painful.
When I got to the Midtown newsroom of the New York Post, where I was a year into my first full-time reporting job, I asked a friend, "Have you ever not felt like yourself? Have you ever felt completely off?"
"Sure," she laughed. "Of course. I hardly ever feel like myself."
I laughed, too, but inside I worried. Lately, there had been more and more days like this. I had started to feel numb on the left side of my body. My arm and leg on that side tingled. Until recently, I had been a healthy 24-year-old, happily starting a career in the Big Apple with a new boyfriend. Now I spent sleepless hours wondering what was happening to my body and my mind.
Then, one Friday at my desk, I cracked.
I had just finished an interview, was starting to write, and for no reason I began crying hysterically. Co-workers gathered, wondering what was wrong. Moments later, I brushed it all off, and skipped down an office hall, giddy with excitement.
Later, at home, I panicked. Was I going mad? Was I mentally ill?
I contacted a neurologist. He sent me to a litany of tests, including two MRIs. Nothing.
"Maybe it's mono," he suggested. "Or a virus."
He promised to follow up with more tests. In the meantime, I curled up with my boyfriend to watch the PBS show "Spain -- On the Road Again." I remember thinking how much I disliked Gwyneth Paltrow. Then, he would tell me later, I started grinding my teeth, moaning and biting my tongue, until I finally passed out.
It was my first seizure. And the last thing I would remember for a month.
THROUGH interviews with family, my boyfriend and friends, I'm able to cobble together the darkest moments of my life. My father kept notes in a spiral notebook, details of what was happening that he could pass along to my mom as they took shifts caring for me.
From my boyfriend, I know that I awoke after the seizure at St. Luke's Hospital next to a puking man.
Doctors ordered a CAT scan that came up clean -- but my behavior was becoming increasingly bizarre.
"I'm dying of melanoma," I told my boyfriend. (I had been diagnosed with the skin cancer five years ago and was convinced that was the cause of my seizure.) I started ranting about suing the doctors and believed that they were out to get me.
But even after the seizure and the paranoid delusions, the neurologist didn't seem worried.
"It's probably alcohol withdrawal," he told my stunned mother and me. He was convinced I was an alcoholic; I barely drank.
My family packed up my clothes and took me to my mother's home in New Jersey, where I continued to deteriorate.
I stopped sleeping. Refused to eat. Paced the halls. Couldn't control my rambling thoughts, and ranted to my mother. I convinced myself that I was bipolar and that I was having a nervous breakdown.
Then I had a second seizure. Blood and foam spurted out of my mouth onto the Oriental rug in my family room.
Overwhelmed by my erratic behavior, my mom dropped me off at my dad's house in Brooklyn Heights for one night before my doctor's appointment. My paranoid delusions got worse. I refused to sleep and started banging on the locked door, screaming, "Get me out of here!" I imagined that my father had murdered his wife.
The next day, my mother, stepfather and boyfriend took me back to the neurologist for an EEG, which records the electrical activity of the brain. On the ride there, I opened up the car door and tried to jump out. My boyfriend held me down and my stepfather child-locked the car. I screamed hysterically until we got to the doctor.
Horrified by my increasing paranoia, and despite my neurologist's hesitations, my mom and stepfather took me immediately to the hospital. They demanded that I not be placed in a psych ward.
There I had my third grand mal seizure while waiting for a hospital bed, and was whisked up to the epilepsy floor of New York University's Medical Center.
For the first three days, I shared a room with three other people, mostly suffering from epileptic seizures.
Technicians glued EEG wires to my head, which snaked into a child's pink backpack on the side of my bed. I was monitored 24/7 by two video cameras mounted on the ceiling.
Two times, I successfully ripped the electrodes off my head, tugged at my IV as blood erupted from my veins, and ran shrieking down the halls, trying to find an exit. Nurses tackled me and stuck me with a sedative. An imposing Jamaican nurse even sat watch with me for 24 hours so that I couldn't escape again.
I had to wear an orange wristband that said "flight risk."
"The physical discomfort of not being able to shower or wash her hair coupled with the fact that she was tethered electronically to a monitor started to take its toll," my dad wrote in his notebook. "She told me she was dying a little bit every day. I told her to trust me and I would get her out, but we had to find out what was wrong with her. She said, no -- get me out now."
It's hard for me to hear about the things that I believed during my madness. When I turned on the TV, I imagined I was on the news and that satellite trucks from all the major networks were camped out outside of my hospital room waiting to interview me.
I imagined the woman beside me was tape-recording my conversations and talking about me in Spanish to her family. I was convinced that I could speak Spanish and interpret her words.
Multiple times a day, residents, doctors and nurses would ask me: "What day is it? Where are you? What's your name?" Though it would take me over a minute to answer, I did so correctly, albeit a little pissed off.
One night a nurse came by to do my vitals and ask the inane questions in the middle of the night. I had finally fallen asleep and was less than pleased. As she bent down to take my blood pressure -- which was spiking due to the illness -- I wound up and slapped her across her chest. She gasped in disbelief and fear.
TWO weeks pass. Every day a new doctor comes to visit. There are internists, infec tious-disease doctors, immune-system specialists, psychiatrists, psychopharmacologists. They send me to MRIs, sonograms, X-rays, CAT scans, PET scans, multitudes of blood tests. All come back negative. No one could give my parents an answer.
My dad came in the mornings, sat with me and fed me breakfast. My mom would come in the afternoons, and my boyfriend came in the evenings and stayed with me until I fell asleep. They never missed a day.
Two comforts for me were apples -- which I inexplicably craved, eating three a day -- and my boyfriend's iPod, on which he made a mix of mostly Ryan Adams tunes, which helped me sleep through the night.
My father prayed. "I would walk across town on 33rd Street to catch the subway on Park Avenue South. There is a chapel between 1st and 2nd Avenue that is dedicated to Jesus and Mary. Each time I walked by I begged God to help Susannah," he wrote. "I even tried to make a deal. I asked God to take me right there on the spot or do anything he wanted with me if only Susannah could be helped."
The paranoid delusions started to wane as the medications, anti-anxiety drugs Geodon and Ativan, increased. But I was losing the battle. I could no longer read. My tongue curled, making it hard to speak. I had difficulty swallowing. It took me a whole minute to answer the simplest questions.
Day after day passed with no answer. I became more withdrawn. I found it hard to walk with my father and mother around the hospital floor. Close friends were alarmed at my passive and incoherent state. Doctors secretly feared that they were losing me.
The head doctors started speaking of a mythical miracle man named Dr. Souhel Najjar. "He would find out what it is," they would say. They even nicknamed him "Dr. House."
My mom requested that we meet this Najjar in person. And after two spinal taps came back with high white blood cell counts -- an abnormal occurrence that signifies brain swelling -- the case was officially passed on to Najjar, a Syrian-born neurologist, neuro-pathologist and epileptologist at NYU Medical Center.
When he came to speak with us, my family was buoyed by his confidence.
He grasped my hands and said, "I'm going to find out what this is and fix it."
Then he handed me a pencil and a piece of paper.
"Draw a clock," he said.
I grasped the pencil and made a circle. Feeling a little confused and put on the spot, I drew in the clock face.
My mother and father gasped.
All the numbers were written on the right side of the clock face, and no numbers were on the left side.
NAJJAR now had five clues as to what was overtaking me: the seizures, the catatonia, the high blood pressure, the high white blood cell count in my spinal taps and the bizarre clock. It was preliminary proof that the right side of my brain (which controls the left side of the body) was inflamed. He believed it was some sort of autoimmune encephalitis, or the swelling of the brain caused by an attack by rogue antibodies.
He decided to send my blood and spinal fluid to a well-respected neuro-oncologist from the University of Pennsylvania named Dr. Josep Dalmau to test to see if rare antibodies were present.
But we had to wait two weeks for the results.
In the meantime, a brain biopsy would be necessary, Najjar said. They would need to cut out a piece of my temporal lobe, my horrified parents learned.
"I can remember being alone in her room when she began to cry," my dad writes in his diary. "I went over to her on the bed and hugged her when I began to cry. Next thing I knew, Susannah was laughing. I asked why. She told me it was the first time she ever saw me cry and I must have looked pathetic."
The next day, I was taken to ICU for surgery. I remember opening my eyes as they sliced into my brain and saying something like, "I'm awake."
The anesthesiologist looked at me and said, "Oh, s- - -."
My mom thinks it was just another hallucination. It seemed so real to me, but I couldn't trust my own mind.
After the surgery, the blood work and spinal fluid came back positive for rare antibodies called anti-N-methyl-D-aspartic acid receptor, or anti-NMDAR encephalitis. The name signifies that the receptors in the frontal lobe, responsible for cognitive reasoning, and the limbic system, or the emotional center of the brain, are under assault by the immune system. My body was attacking my brain.
Penn's Dr. Dalmau had discovered these antibodies in 2003. Until then, people suffering from my madness were misdiagnosed, likely ending up in mental hospitals, if not dead. Experts aren't sure what causes it, though they believe it's genetic, not environmental.
According to his studies, the median age for the disease is 20. The youngest is 21 months. About 75 percent of those affected by it are women. All show forms of psychotic behavior, some show signs of catatonia. About 80 percent of patients have seizures and 70 percent of patients see psychiatrists before any other doctors, according to Najjar.
Najjar estimates that nearly 90 percent of those suffering from autoimmune encephalitis go undiagnosed.
"It's a death sentence when you're still alive," Najjar told me. "Many are wasting away in a psych ward or a nursing home."
I was the first person in NYU Medical Center's history to be diagnosed with NMDAR encephalitis.
Najjar wasted no time. He immediately hooked me up to an IV and started the treatments.
First came the intravenous immune globulin, which reduces inflammation of the brain. Then came high levels of steroids, which also reduce inflammation. And finally he hooked me up to a plasmapheresis machine, which flushes out the harmful antibodies from your system, through an IV in your neck.
The initial treatment took about a week, until I was well enough to be released from the hospital. Six months later, I'm still taking steroids, but I'm back at work, back at home, seeing colors clearly and not breaking down at my desk.
At a medical conference last week at NYU, Najjar presented my case and the wide spectrum of autoimmune encephalitis disorders, saying that I was back to "normal." A friend and co-worker laughed. "You're better, but you're not normal," she said.
My father reflected on my time in the hospital. "Najjar told me she could have easily ended up in a nursing home for the rest of her life. Najjar thought she was extremely lucky. He was saying, with time, she could get back 90 percent of her cognitive abilities," he wrote.
I'm happy to say that today I'm at 100 percent, and marveling at the lost month of my life, paging through my father's diary like I'm reading about a stranger.
"All I knew was that she was alive, and her spirit was intact," he wrote at the end, words that bring me to tears. "We had more hospital stays for treatments, doctor visits, and lots of medications to deal with, but my baby was on the way home."

Scientific America: https://www.scientificamerican.com/article/brain-on-fire-my-month-of/

Thursday, September 30, 2021

Boquete: Answer to Prayers!

According to old Chinese advice, it is wise never to discuss politics or religion even amongst best friends.  

Religious belief can often blur judgment in the wisest of people.

Thirty years ago, a patient of mine was unconscious for 23 days and it was mother's belief that it was through prayer that her daughter was saved. I did not argue with her then.

But perhaps God works through his people in his own way. Discoveries in Medicine should therefore enhance our faith rather than the other way round.

It took nearly 30 years for the real answer to her prayers to really emerge.

 ©2012 Am Ang Zhang
I was staying at our resort in Boquete and was having dinner with three friends all of them with medical connections. One was in hospital administration and one a nurse. The husband of the nurse was a pharmacist. Somehow the conversation drifted into medical topics and knowing that I am a Child Psychiatrist the pharmacist started talking about his nephew who was nearly sent to a mental institution as he suffers from catatonia and doctors eventually diagnosed schizophrenia and put him on antipsychotics. Luckily the catatonic symptom probably saved him as some bright young thing just read the book Brain On Fire and gave him the Clock Test. That led to the NMDAR antibody testing that proved positive. He responded well to the treatment regime that has been developed and is off all antipsychotic medication.

My Teratoma patient was lucky as she belong to that group that improved without further treatment once the Teratoma was removed. She eventually had a baby.

The Power of Prayers & Teratoma: Brain & NMDA!

Anti-NMDA Receptor Encephalitis

NEW ORLEANS — A mysterious, difficult-to-diagnose, and potentially deadly disease that was only recently discovered can be controlled most effectively if treatment is started within the first month that symptoms occur, according to a new report by researchers from the Perelman School of Medicine at the University of Pennsylvania. The researchers analyzed 565 cases of this recently discovered paraneoplastic condition, called Anti-NMDA Receptor Encephalitis, and determined that if initial treatments fail, second-line therapy significantly improves outcomes compared with repeating treatments or no additional treatments (76 percent versus 55 percent). The research is being presented at the American Academy of Neurology's 64th Annual Meeting in New Orleans.

565 cases! Not so rare!

The condition occurs most frequently in women (81 percent of cases), and predominately in younger people (36 percent of cases occurring in people under 18 years of age, the average age is 19). Symptoms range from psychiatric symptoms, memory issues, speech disorders, seizures, involuntary movements, to decreased levels of consciousness and breathing. Within the first month, movement disorders were more frequent in children, while memory problems and decreased breathing predominated in adults.

My patient was under 18 and presented with catatonia symptoms. She later lose consciousness and was ventilated.

"Our study establishes the first treatment guidelines for NMDA-receptor encephalitis, based on data from a large group of patients, experience using different types of treatment, and extensive long-term follow-up," said lead author Maarten TitulaerMD, PhD, clinical research fellow in Neuro-oncology and Immunology in the Perelman School of Medicine at the University of Pennsylvania. "In addition, the study provides an important update on the spectrum of symptoms, frequency of tumor association, and the need of prolonged rehabilitation in which multidisciplinary teams including neurologists, pediatricians, psychiatrists, behavioral rehabilitation, and others, should be involved."

The disease was first characterized by Penn's Josep Dalmau, MD, PhD, adjunct professor of Neurology, and David R. Lynch, MD, PhD, associate professor of Neurology and Pediatrics, in Annals of Neurology in 2007. One year later, the same investigators in collaboration with Rita Balice-Gordon, PhD, professor of Neuroscience, characterized the main syndrome and provided preliminary evidence that the antibodies have a pathogenic effect on the NR1 subunit of the NMDA receptor in the Lancet Neurology in December 2008. The disease can be diagnosed using a test developed at the University of Pennsylvania and currently available worldwide. With appropriate treatment, almost 80 percent of patients improve well and, with a recovery process that may take many months and years, can fully recover.

Teratoma: finally!

In earlier reports, 59 percent of patients had tumors, most commonly ovarian teratoma, but in the latest update, 54 percent of women over 12 years had tumors, and only six percent of girls under 12 years old had ovarian teratomas. In addition, relapses were noted in 13 percent of patients, 78 percent of the relapses occurred in patients without teratomas.
As Anti-NMDA Receptor Encephalitis, the most common and best characterized antibody-mediated encephalitis, becomes better understood, quicker diagnosis and early treatment can improve outcomes for this severe disease.
The study was presented in a plenary session on Wednesday, April 25, 2012 ET at 9:35 AM at the American Academy of Neurology's annual meeting.
[PL01.001] Clinical Features, Treatment, and Outcome of 500 Patients with Anti-NMDA Receptor Encephalitis

Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies

Of 100 patients with anti-NMDA-receptor encephalitis, a disorder that associates with antibodies against the NR1 subunit of the receptor, many were initially seen by psychiatrists or admitted to psychiatric centres but subsequently developed seizures, decline of consciousness, and complex symptoms requiring multidisciplinary care. While poorly responsive or in a catatonic-like state, 93 patients developed hypoventilation, autonomic imbalance, or abnormal movements, all overlapping in 52 patients. 59% of patients had a tumour, most commonly ovarian teratoma. Despite the severity of the disorder, 75 patients recovered and 25 had severe deficits or died.

Related paper:

Anti-NMDA Receptor Encephalitis: Diagnosis, Psychiatric Presentation, and Treatment

Chapter 29  The Power of Prayers

The following is extracted from The Cockroach Catcher: Chapter 29 The Power of Prayers.

According to old Chinese advice, it is wise never to discuss politics or religion even amongst best friends.  

         Religious belief can often blur judgement in the wisest of people. In psychiatry it is sometimes not easy. This is particularly true in cases of florid psychosis, which often presents with symptoms of hallucination, delusion and even vision.

         I remember my early days of psychiatry in a mental hospital in Hong Kong. Yes, it was the days of 2000-bed hospitals. Yes, it was the days of Medical Superintendents who had supreme power and all doctors of whatever rank and experience were Mental Health Officers with special authority to sign papers for compulsory admissions. The forensic unit was contained within the same complex.         Those were the days when we encountered psychosis in the raw so to speak. All the colony’s really mad people were admitted to this one place set in the furthest corner of the colony. In our year seven of us decided without much discussion that we all wanted to go into psychiatry. That was over 10% and all had quite idealistic reasons. It was perhaps a bit of a disappointment to our parents that we did not pursue a more conventional specialty that might provide us with more status and financial reward. Then there was the fear of contamination that somehow one might become mad too. Recent day medical students are said to shy away from psychiatry for these same reasons.         Education seems to have little effect on superstition.
                                     ......................................….read the full Chapter: HERE

Brain on Fire   

Scientific America: https://www.scientificamerican.com/article/brain-on-fire-my-month-of/

Sunday, September 26, 2021

International Daughter's Day: Stubborn Mother 2.


Rachel could not get to school. She was having such bad back pain. Her family doctor wrote an urgent referral. As she would not see the psychologist at school, school was considering taking mother to court.
          There was a change in managing school refusal. Education Authorities suddenly turned trigger happy and all over the country parents were taken to court. I did wonder if this was due to a shortage of Educational Psychologists who were now too busy dealing with Formal Assessments as a result of the new Education Act, or whether it was due to years of public criticism of the inadequacy of the softly softly approach to the problem. There is some truth that there is a hard core of children whom no teacher really wants to see at school and the authorities are quite happy they are absent. These are children who are entitled to free meals and the hidden saving of them not attending school adds up to a pretty substantial sum. To assess them would take up precious Psychologist time and also may generate expenses in terms of ferrying these children by taxi to special tutorial units or schools.
          But Rachel came from a professional family. Mother was a lawyer and father an insurance executive commuting to London. Yes, Rachel had some problems a year earlier because of her height. She did stop attending school for a while, claiming she had pain in her back. She was way over the 98th percentile for height. Some strong pain killer prescribed by her doctor seemed to have done the trick and she had not been absent until the present attack of pain.
          Clinical judgment is indeed a kind of “profiling”. We judge our patients from a variety of information and we “profile” them. It may not be correct but we do.
          I had my suspicion that the Educational Psychologist never got to see her record to realise that she was not really the type anyone should ever dream of prosecuting.
          The family doctor thought that I should be given a shot before anyone should have a go. Mother was told in no uncertain term that she needed to get Rachel to see me.
          “But she was in such pain!” mother said.  She did protest but in the end succumbed. With the help of a neighbour, they managed to get her to the clinic and she was lying down in our waiting area.
          I had one look at Rachel, perhaps 6 ft tall, lying flat in the waiting area and asked my secretary to call an ambulance whilst I talked to the Radiology Consultant. An X-ray examination was ordered and if necessary an MRI scan.
          How could I come to such a decision without even spending half a minute with mother or the patient? Was I being over dramatic? Or was it what we have been trained for? Was it why psychiatrists are trained as doctors first?
          I could of course have been entirely wrong and the girl might really have been school phobic. Would I have subjected her to an unnecessary X-ray examination? Would my reputation suffer as a result?
          The ambulance came. The paramedics were excellent. They treated it as potential spinal injury and transported her that way. I accompanied her onto the ambulance. You had to see her face to know you were right. She was grateful someone believed her. For me it was worth all the drama. My only wish was we were not too late that she might not be able to walk.
          Mother too shook my hand as the ambulance got ready to go. I always told my juniors. “Trust them, most of the time.”
          I left a message for the radiologist to call me.
          The call came back from the radiologist. She had two collapsed vertebrae, a common condition among very tall children who have just had a growth spurt. The Orthopaedic Surgeon was preparing for an emergency operation.
          “Good work.” The radiologist said.
          I knew. He meant: “Good work for a psychiatrist, and a child psychiatrist at that.”
          Some time later mother arranged to see me to tell me in detail what was done.
          “She wants to thank you for believing her.”
          I was just doing my job.

International Daughter's Day: Stubborn Mother 1.

When the book by Stephen Westaby turned up for reasons too complex to explain, the Cockcroach Catcher somehow managed to read the most important chapter that was quoted by many reviewers.

When I turned back to the beginning, I have to disagree with Westaby’s proud claim that he lacked empathy from lack of sleep as a Junior Doctor and that was why he became a heart surgeon.

Brought me back to how one could help people who has lost their life saving during the financial crises and became depressed and suicidal. Is empathy going to help? What about someone giving him back all the money he has lost? Would he still kill himself?

Well the “empathy-free” Westaby arrived in Australia for a conference only to receive a call asking him to go back to England as a mother of a 6 month old girl would only have him operate on her daughter to save her life. So Westaby immediately arranged to fly back and planned his operation/s on the long flight back. I call that real "empathy"!

Unfortunately what was planned did not work and the parents were counseled to say farewell. Westaby did not join in with the “empathy” as he did not try to comfort the parents. It was left to the others.

No, he decided to try something that has never been tried. As the parents went in afterwards thinking that they were going to say goodbye, mother felt the girl's feet which was the warmest it has been, she realised that her faith in Westaby was totally justified.

The girl is still alive today. Westaby was threatened with the sack for doing something that has never been done before.

Well, that is my kind of “empathy”!

It was great to see him in person at Sage, Gateshead for the BBC Free Thinking event. The book is for all of you that loved the NHS, the NHS Original, the NHS born in the same month and year Westaby was born. We need to bring it back and we need to let good doctors do what they think is best, even if it is not NICE approved. Free thinking indeed!

Gateshead © Am Ang Zhang 2017

Westaby must have the last words:

Who would be a heart surgeon now? With long, taxing operations, anxious relatives, the nights and weekend on call. Worse still, a health system entrenched in nonsensical bureaucracy with the reward of public exposure for a run of bad luck. Already 60 per cent of our children’s heart surgeons are overseas graduates.

In the final analysis, a profession that dwells upon death is unlikely to prosper – undertakers and the military aside. As Dr Kirklin emphasised, some deaths in cardiac surgery are inevitable. When a surgeon is focused on helping as many patients as his ability will allow, some will die. But we should not accept inconsistent teams, substandard facilities or lack of vital equipment. Otherwise patients die needlessly.

The answer? Bury the blame-and-shame culture and give us the tools to do the job.

Fragile Lives: How Stephen Westaby's untried surgery saved ALCAPA baby Kirsty

Stephen Westaby

"We shall never surrender."

Winston Churchill, during the Battle of Britain, 1940

Monday 15 February 1999, 3.45am. No one calls with good news at night. I'd been in Australia for just thirteen hours after a twenty-hour flight. In pitch darkness I scrambled across my hotel bed and knocked the receiver to the floor. The call was lost. I swiftly slipped back into sleep, courtesy of melatonin tablets and the bottle of Merlot I'd drunk at dinner. Ten minutes later the phone rang again. This time I fielded it successfully, but I was irritated.

Professor Stephen Westaby, a British heart surgeon at John Radcliffe Hospital. Supplied

"Westaby? This is Archer. Where are you?"

"Nick, you know I'm damned well in Australia. It's the middle of the f***ing night – what's the problem?"


I didn't want to hear the answer.

"Steve, I'm sorry, but we need you to come back. We have a sick baby with ALCAPA. The parents know you and want you to do the surgery."

Oh joy. "When?"

"As soon as possible."

I really had no other choice.

Kirsty was a beautiful six-month-old baby girl in whom fate had installed a lethal self-destruct mechanism, a miserable detail that seemed destined to end her life before her first birthday. ALCAPA is an abbreviation for Anomalous Left Coronary Artery from the Pulmonary Artery, an isolated and exceptionally rare congenital anomaly in the overall complexity of human anatomy.

Simply put, it's bad wiring. Both coronary arteries should rise out of the aorta and supply the heart muscle with well-oxygenated blood under high pressure. They should never be attached to the pulmonary artery, as this has both low pressure and poor oxygen content. Early survival with ALCAPA therefore depends on the development of new "collateral" blood vessels between the normal right coronary artery and the misplaced left coronary artery. But these eventually are insufficient to sustain blood flow to the main pumping chamber.

Muscle cells deprived of oxygen die and are replaced by scar tissue, leaving baby to suffer what are in effect repeated, painful heart attacks. Scar tissue stretches, causing the left ventricle to dilate, then the heart progressively fails and the lungs become congested with blood, leading to breathlessness and exhaustion. Even during feeding.

Kirsty Collier with her mum Becky before her operation. After having a third of her heart removed in pioneering surgery Kirsty astounded medical experts by making a ''miracle'' recovery.  SWNS.com

But because ALCAPA is exceedingly rare, the diagnosis is seldom made until the infant is terminally ill. Fortunately, her parents were intelligent, had recognised that there was a serious problem and were persistent in finding help for her.

Kirsty's story was particularly harrowing. Her mother Becky already had a three-year-old son and was an experienced and responsible mum. All antenatal checks and ultra-sound scans had appeared normal. Kirsty was born on August 21, 1998, by elective Caesarean section with a spinal anaesthetic and at first everything seemed normal. But not for long.

In the womb, the pressures and oxygen content of the aorta and pulmonary artery are the same, so Kirsty's tiny heart was safe. After birth, the circulations to the body and newly expanded lungs separate, and both pressure and oxygen content in the pulmonary artery fall. So, in cases of ALCAPA, both blood flow and oxygen content in that critically important left coronary artery fall precipitously, too.

Kirsty was grunting even during the first attempt to breastfeed in the hospital, and Becky noticed beads of sweat trickling from the bridge of her baby's nose. The effort of feeding repeatedly made her fractious and distressed.

Kirsty Collier two months after her operation in February 1999. Kirsty had a third of her heart removed when she was just 4 months old.  SWNS.com

This was distinctly different from how her son had been, so Becky asked for a paediatrician to review Kirsty. She was told that there was absolutely nothing to worry about but the truth was that no one had bothered to find out what was wrong.At this stage, Becky had little choice but to take her irritable but precious little bundle home.

Within weeks Becky was certain that something was seriously amiss, because during every feed there was sweating and vomiting. Kirsty struggled for breath, clenched her little fists and screamed until she was puce in the face. Together they made many visits to the GP, sometimes as many as three times a week, but they always received the same non-committal reassurance – tense, unpleasant encounters, as Becky was deemed neurotic and unable to cope.

Despite Kirsty's rapid breathing she had no temperature. Chest infections were ruled out, and her belly was soft, with no signs of stomach or intestinal blockage. All of the common paediatric problems were excluded. Becky became more and more anxious. Kirsty wasn't gaining weight. She had a pasty, washed-out look and a cough like a dog's bark.

In reality this baby was suffering repeated small heart attacks with excruciating chest pain that she could neither communicate nor understand. The human body can be outlandishly cruel.

Kirsty as a 10-year-old with her mother Becky Collier.  SWNS.com

Eventually, after a meltdown in the GP's surgery, Becky insisted that Kirsty should be referred to the local hospital. Twice she had chest X-rays, only to be diagnosed with bronchiolitis – inflamed breathing tubes – on both occasions. Then one day during her afternoon nap Kirsty turned a terrible slate-grey colour. She was barely rousable and limp. Becky snatched her up and rushed to the surgery. But by the time they presented themselves at the receptionist the baby was awake and pink again.

Becky was told to stop fussing and that there were children to be seen who were actually sick. On this occasion mother and child were acrimoniously dispatched, once more with a prescription for antibiotics. Kirsty's enormous heart remained undetected.

This time, Becky drove directly to the accident and emergency department of her small local hospital. They were seen by a sympathetic female doctor who had children of her own. Recognising that a mother's instinct was usually correct, she referred them on to a larger city hospital for review by the on-call paediatrician.

It was a bitterly cold, frosty night and they were left sitting in an unheated hospital corridor for several hours. Becky frantically struggled to keep Kirsty warm but she became progressively more limp and grey. Eventually, late into the night, they were seen. The first junior doctor suggested bronchiolitis and intended to dismiss them without investigation.

Beckyrefused to leave without a chest X-ray. She was told off for her unreasonable attitude – how thoughtless to inconvenience the hard-pressed duty radiographer at that time of night. So the sad pair were dispatched unescorted down poorly lit corridors and icy outside walkways to find their own way to the X-ray department. It was well past midnight when they returned, clutching the tell-tale picture, which Becky presented to a nurse. They were parked once more.

Another thirty minutes passed, then a dramatic shift in attitude from the hospital staff. Now there were hushed voices, grave expressions, and nurses bringing drips and drugs. The previously mean, now embarrassed nurse took Becky aside to explain that Kirsty was being transferred to the specialist children's heart unit in Oxford. By ambulance this time.

So what did the X-ray show to trigger this frenzy of activity? Kirsty had a massive heart. Her problem was immediately obvious on the X-ray film. When the staff were pressed about her previous X-rays at the same hospital, Becky was told that the heart shadow had been misinterpreted as fluid. Some mistake!

In Oxford, the paediatric cardiology registrar met the ambulance and took them directly to a ward packed with children with serious heart problems and beeping monitors – a hive of activity in the depths of night.

Kirsty with her parents before her school prom. Kirsty made a full recovery and aside from a feint scar on her chest has little to show for the surgery. Supplied

Nick Archer arrived at 3am. An ECG and blood tests were done quickly, then the echo machine was brought in to image Kirsty's heart chambers. First it seemed like good news. All four chambers were there, with no holes between them. But worryingly the left atrium and ventricle were both enlarged, the ventricle dramatically so. This explained the heart failure and accounted for the striking chest X-ray.

In little more than an hour, the cardiology team established that Kirsty had severe heart failure from multiple heart attacks. Parts of the left ventricular wall now consisted of thin scar tissue interspersed with poorly contracting muscle, a rare condition in an infant but one that provided the likely diagnosis. One further test was needed. A cardiac catheter would confirm the diagnosis but would require a general anaesthetic, so she'd have to be in much better condition before proceeding.

It was a full five weeks before Becky's baby was considered fit enough for the cardiac catheter. The evening before the operation, the anaesthetist came round to talk. Normally a jolly and optimistic character, on this occasion he didn't have much to smile about. That night, Kirsty was christened in her cot by the hospital chaplain, the doctors, nurses and other families gathering around the bed to support them.

That morning, Becky dressed her baby in her best pyjamas and tied a bow in her hair for her trip to the catheterisation laboratory. Ironically it was Valentine's Day. As Becky put it to me later, "A girl has to look her best, even when she's poorly.":

Once I was on the plane home I started to sketch the anatomy of Kirsty's aorta, pulmonary artery and abnormal left coronary artery. I knew that the current techniques in operations for ALCAPA had limitations and a substantial failure rate, so I used my time during the flight to work out an alternative. By the time we were cruising high above Java, I'd designed my new operation.

I called my colleague Takahiro Katsumata when I got back to Oxford and asked him to bring Kirsty's parents to my office with a consent form. The cardiac catheter had shown precisely what Archer had suspected. Kirsty needed surgery as soon as possible.

Becky looked tired and drawn when I saw her.

I don't do transmitted anxiety if I can help it. But it's much more difficult for my anaesthetist colleagues who have to deal with the agonising separation when the patient is given over to them.

FRAGILE LIVES Illustrations labeling and depicting elements of the heart and cardiovascular systems. Heart Surgery Prodger, Isabel

I described my planned operation to the team and explained why I felt it would be an improvement on existing techniques. The new left coronary artery would be constructed with a flap of aortic wall that would sit below a corresponding pulmonary flap to make a tube, the latter containing the misplaced origin of the left coronary artery at its apex. The product would be a new coronary artery delivering high-pressure, well-oxygenated blood directly from the aorta, where it should have come from in the first place. Blood fully saturated with oxygen would then supply the failing heart muscle and prevent further heart attacks. Katsumata was intrigued and excited by my proposed approach, so much so that he rushed off to call the hospital's film crew.

The risks of the operation were substantial. Becky's shaky hand signed the consent form and I walked back with them to the children's ward. When we reached the cot, Kirsty's heart failure was worse than I imagined. She was emaciated, her heaving ribs and rapid breathing a consequence of her congested lungs, and her abdomen swollen with fluid. Without immediate surgery she'd be dead within days. Although a voice in my head screamed, "Oh shit," my mouth correctly said, "I'll go to theatre now."

The anaesthetist knew the score, having already anaesthetised Kirsty for the cardiac catheterisation, and some of the monitoring lines were still in place.

"Do you really think you can get this baby through?" was his opening line.

I didn't reply, bidding a cheery "Good morning" to the nurses and perfusion team in the operating theatre, then went straight to the coffee room. I wanted to avoid seeing Becky leave her baby with strangers, always an excruciating event.

When I returned, Kirsty was already on the operating table, covered in green drapes held in place with an adherent plastic drape. All that was visible were her bony little chest and swollen abdomen. Heart surgery needs to be an impersonal, technical exercise.

I joined Katsumata and my six-foot, six-inch Australian colleague Matthew at the scrub sink. While we scrubbed in silence, the film camera was carefully positioned next to the operating lights. There was a palpable buzz of excitement. We were about to do something novel, esoteric and risky.

There was no bleeding as I drew the scalpel blade along the skin over Kirsty's breast bone. In shock, her skin capillaries had shut down to divert blood to vital organs. Next, the electrocauter cut through the thin layer of fat onto the bone, producing its characteristic buzzing noise accompanied by a whiff of burning as the current cauterised the oozing blood vessels, although this time there were few of these. Then the electric saw cut through the length of her sternum, exposing bright red bone marrow.

FRAGILE LIVES Illustrations labeling and depicting elements of the heart and cardiovascular systems. Heart Surgery Prodger, Isabel

We used a small metal retractor to crank open her tiny chest, bending and stretching the joints between the ribs and the spinal column. In babies, the fleshy thymus gland lies between the sternum and the fibrous sac around the heart, but by now it had done its work producing antibodies for the foetus so we removed it.

The electrocauter continued its messy but vital work, cutting through the fibrous pericardial sac to expose the heart, straw-coloured fluid pouring out and being drawn away by the sucker. Meanwhile the other members of the team worked on silently. The anaesthetist gave heparin to stop Kirsty's blood from clotting in the heart–lung machine, the perfusion team set up the complex array of tubing, pumps and oxygenating equipment to keep Kirsty's body alive when her heart was stopped, and the scrub nurse concentrated on having the correct surgical instruments ready to slap into my palm. I rarely had to ask for anything.

As we pulled up the edges of the pericardial membrane to display the heart, Katsumata – in a moment of wry Japanese humour in times of stress – involuntarily uttered what now had become our surgical catchphrase, "Oh shit, don't mention the war." Back from his first cigarette, the anaesthetist popped his head over the drapes in response to Katsumata's comment. Others could see it all on the video screen.

What should have been a walnut-sized heart was revealed to be the size of a lemon. The enlarged right coronary artery was obvious, its many inflated branches crossing over towards the left ventricle. While the right side of the heart pumped vigorously against raised pressure in the lungs, the left ventricle was hugely dilated and barely moved.

Batches of newly necrotic muscle merged with areas of white, fibrous scar tissue, the result of the many small, painful heart attacks endured by Kirsty during her first six months of life. But Kirsty had survived to this point and it was our job to keep it that way.

Having exposed this heart I began to question the wisdom of attempting such a complex operation having come straight from a day-long flight.

But urgent heart transplants were virtually impossible in babies; this replumbing of her heart's blood supply was her only chance of life.

Tiny pipes were inserted to connect her to the heart–lung machine, and I then gave the signal to continue. The perfusion technician turned on the roller pump and Kirsty's heart gradually emptied. At this point, technology had taken over, diverting blood away from her lungs and into the synthetic oxygenator.

FRAGILE LIVES Illustrations labeling and depicting elements of the heart and cardiovascular systems. Heart Surgery Prodger, Isabel

With her empty heart still beating I cut through the pulmonary artery above the origin of the anomalous coronary, which sat there like the pearl in an oyster. Now we had to connect it without tension to the high-pressure aorta that lay an inch away. The conventional method was simply to trying to stretch and re-implant the origin of the vessel into the side of the aorta. But this could result in thrombosis and blockage, so I pressed on with my new technique.

This delicate exercise could only be achieved by clamping the aorta and temporarily stopping all blood flow to the heart. We'd protect the muscle by infusing cardioplegia fluid directly into both coronary arteries, flushing all the blood out and collapsing the ventricle like a punctured football. This induced state of inactivity, common in heart surgery, is reversed simply by removing the clamp on the aorta, which allows blood from the heart–lung machine to flow back into the coronary arteries.

For the reconstruction of this tiny vessel, the stitching had to be precise, accurate and watertight. The procedure went well. Just thirty minutes after the heart was stopped, the conjoined flaps restored Kirsty's coronary artery anatomy to what it should have been.

As the clamp was removed, bright red oxygenated blood – rather than de-oxygenated blue blood – flooded the left ventricular muscle. Her heart changed from a pale pink colour to deep purple, then became almost black in parts. Before re-constructing the pulmonary artery we checked that there was no bleeding from the lines of stitching behind it. Soon the electro-cardiogram showed uncoordinated electrical activity, and the heart stiffened with renewed muscle tone.

Unusually for a child, her reperfused heart kept writhing and squirming in ventricular fibrillation. We used an electric shock directly through the muscle to restore normal rhythm. Ten volts – zap! The heart defibrillated and stopped wriggling. It was now motionless but we expected a normal rhythm to resume at any moment.

But it didn't. The purple ball fibrillated and squirmed again, and the anaesthetist's head popped over the drapes to request the obvious – "Shock it again!" We did and the same thing happened. It wasn't coming back.

This was serious electrical instability caused by the scar tissue, so we gave the appropriate drugs to stabilise the muscle cell membranes.

"Let's give it more reperfusion time," I told the anaesthetist.

"OK, I'll go out for a fag then," he said.

Twenty minutes later we tried again. Twenty volts – zap! This time her whole little body levitated from the operating table and her heart defibrillated. Although it slowly began to beat, it was barely more than a flicker. Ominous, but we had drugs in reserve to make it pump harder.

I asked the anaesthetist to start an adrenaline infusion and told the perfusionist to cut back on pump flow to leave some blood in the heart. This was operating theatre protocol, and it's just like the military. You make a request to a medical colleague but give orders to the technical staff. If you start giving orders to an anaesthetist they'll tell you to piss off, and will go off and do something different.

My gaze remained fixed on Kirsty's pathetic little heart. The new coronary artery was fine – there was no kink in the tube and no bleeding. For the first time, the left ventricle was receiving well-oxygenated blood at the same pressure as the rest of the body. But her heart still looked like an overripe plum and was barely beating at all. What I was thinking was we're stuffed, this heart's had it; great operation – dead baby.

Of course, I didn't let the others know my thoughts but I was starting to fade. I suggested that the cameraman should stop filming for a while because nothing was going to change and asked Katsumata to come to my side of the operating table while I took a break.

I removed my gown and gloves, and went to make a call in the anaesthetic room. Mike followed.

"Can you repair it?" he asked me.

"Don't think so."

"I'll get Archer to warn the parents, then."

I slumped on a stool and picked up the phone. One of the lovely nurses put a coffee and doughnut in front of me.

In five minutes, Archer was down from his outpatient clinic at the theatre door. He didn't need to ask.

My bladder was full and I wandered off to the loo. When I got back, my brain had regained control without that distraction and I now really needed to focus. What, if anything, could I do to make things better? I was running out of ideas.

The left ventricle was scarred, dilated and now globular – not the normal elliptical shape. This distortion had pulled open the mitral valve and prevented it from closing. As the left ventricle tried to pump blood around the body as much as half of it flowed backwards to the lungs. Heart function is always temporarily worsened during surgery, but in Kirsty's case it seemed terminal. I'd hoped that resting the heart on the bypass machine would help it to recover. It hadn't.

I went back to the operating theatre, scrubbed up again and switched with Katsumata. He said nothing, but looked crestfallen – a clear message. I asked the anaesthetist to start ventilating the lungs and told the perfusionist to prepare to slowly ease off the machine. At this point, Kirsty's heart needed to take over and support the circulation, otherwise she'd die on the operating table. We all stared at the traces on the screen, hoping to see her blood pressure rise. It briefly reached half normal, but then fell away rapidly as the pump was switched off.

"Shall we go back on?" asked Katsumata.

Watching the left ventricle flicker on the echo, the perfusionist questioned whether it was worth it.

I wasn't yet ready to call it. Failure would mean death for the little girl and a life of torment for the parents.

"Let's go back on, give it another half hour."

This in itself was problematic, as a long bypass time always lessened the chance of recovery.

Kirsty's parents were waiting in the children's ward – Archer had gone to warn them. When we called him back, Becky insisted on coming to the doors of the operating theatre complex with him. Should I tell her that the heart was too badly damaged, that the diagnosis should have been made months ago and that Kirsty had been let down by an overburdened system?

On the very rare occasions that a child died on the operating table I always talked to the parents myself. It was something I dreaded, the very worst part of my job.

The entrance to the theatre complex opened automatically onto the hospital corridor. I was immediately confronted by eyes full of grief and desperation. I remember Becky saying, "Please save my little girl." I was pole-axed. I turned back to the sombre theatre, put on a new mask and scrubbed up again.

The anaesthetist had finished yet another cigarette, and said, "Things are no better. Can we turn the pump off?"

"No, I'm going to try one more thing. Turn the lungs off. Run the camera again."

This was my last-gasp attempt. It was something that could only be justified by invoking the laws of physics and had never been done before in a child. The tension on the wall of Kirsty's scarred left ventricle was elevated because of the size of the cavity. From a recent conference, I knew that a Brazilian surgeon had made a series of failing adult hearts smaller when a tropical infection, Chagas disease, had weakened the muscle. The operation had been attempted for other types of heart failure patients in North America but was quickly discredited and abandoned. In my view, this bold approach was Kirsty's last hope.

I was not going to risk stopping the heart again, so I took a glistening new scalpel and cut the beating left ventricle wide open from apex to base, just like unzipping a sleeping bag. I began in an area of scar, carefully avoiding the muscles that support the mitral valve, and the filleted heart immediately fibrillated in response to cutting. This was fine because there was no risk of it pumping air.

Frankly, I was stunned by the unexpected appearance of the inner lining of the heart. It was covered in thick, white scar tissue. To reduce the diameter of the ventricle I cut away the scar tissue on either side of the incision until I reached bleeding muscle and then removed one third of the circumference of the chamber.

In an attempt to stop the mitral valve from leaking I sewed the central point of its two leaflets together, turning it from an oval to a double orifice structure resembling a pair of spectacles. Then I simply sewed the muscle edges together with a double row of stitches to close the heart. In the end, this much smaller heart looked like a quivering black banana. Not for a moment did I think it would ever start again – and nor did my colleagues. Most of them thought I was crazy.

Word of the bizarre operation in Theatre 5 soon spread. The curious gathered to watch and the camera kept on filming. We had to ensure that all air was removed from the heart, otherwise it could be ejected into the blood vessels of the brain and cause a stroke. After that, all that remained was to defibrillate and try to restore normal heart rhythm.

"We're done," I announced. "Try 20 volts."

Zap! The heart stopped quivering, and for what seemed like an age there was no spontaneous electrical activity. I poked the muscle with forceps and it contracted in response. This time there was a flicker on the blood pressure trace. Miraculously, the black banana had ejected blood into the aorta.

The anaesthetist looked again at the echo. "It certainly looks different. Shall we try using the pacemaker?"

I was already sewing the fine pacing wires into place. We arbitrarily set the pacing box rate at 100 beats per minute and switched it on. I told the perfusionist to cut back on the pump flow rate and leave blood in the heart to see if it would eject consistently. It did. What's more, the echo showed that the mitral valve no longer leaked. At this point I felt that we were in with a chance. Life really does depend upon physics and geometry.

It was now after midday. Kirsty had been on the bypass machine for more than three hours and we needed to get her off it. As if timed to perfection, her own heart rhythm suddenly broke through the pacing. Coordinated natural heart rhythm is much more efficient than electrical pacing, providing much better blood flow and pressure.

It was like switching on a light in the operating theatre. Gloom changed to elation, adrenaline kicked in and my fatigue suddenly lifted. We gave Kirsty an infusion of adrenaline to help her heart take over from the bypass machine. Finally I gave the instruction to"'come off slowly". We still expected her blood pressure to fade, yet the curiously reconfigured little heart kept on pumping.

I remained silent, but I looked over my mask at Katsumata. He knew that I'd had enough by now. "Let me finish," he said.

I took a last, disbelieving glance at the little black banana pumping away, then turned to the echo screen, where the incomprehensible flashes of white, blue and yellow were also reassuring, like a blazing fire. We could see blood streaming through the new left coronary and a double jet entering the left ventricle through the mitral valve – a curiously reconfigured baby's heart that finally worked.

Katsumata made certain that there was no bleeding, then meticulously closed the chest.

"Never been done before," he said, looking over at me. Soon afterwards Becky came down to the theatre in shock. After the encounter at the theatre door, she believed Kirsty had died. She put her hand on her baby's little foot and exclaimed, "It's warm. It's never been warm." When she started to cry I left. It had been a long day.

We followed Kirsty carefully over the following ten years, using echocardiography to watch her heart develop. She was a perfect little girl, happy, outgoing and energetic, the only clue to her extraordinary internal metamorphosis being the faint stripe up the middle of her chest.

When we felt she was mature enough to discuss it, we asked for her permission to carry out a magnetic resonance imaging scan to show us how the remodelled heart had developed. What we found was quite extraordinary. Apart from the double-orifice mitral valve her heart appeared normal, as did her new left coronary artery. Only the thin nest of scars showed the position of the line of stitches up the heart. Remarkably, all other scar tissue had disappeared. The whole inner lining of her left ventricle had been pure white scar tissue – now all gone.

This provided some of the first evidence that an infant's own cardiac stem cells can regenerate heart muscle and actually remove fibrous tissue. Adult hearts cannot recover in the same way. But what if we could identify and culture stem cells that could do just this for an adult heart? One day I'd find out.

Now aged eighteen, Kirsty is a vivacious and athletic teenager. Had she died we'd never have known about this exciting possibility for heart regeneration.

This is an edited extract from Fragile Lives by Stephen Westaby, published by HarperCollins.