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Saturday, September 3, 2011

Race to save Annie

Source: Herald Sun | Lucie Van Den Berg


HE LIES on a faded maroon couch, his brow wrinkled, deep in thought, eyes closed to the fluorescent lights of the tearoom. Grey stubble appears to be growing like time-lapse photography as a working day drags into night.
What is weighing on Professor Bob Jones's mind? Is he thinking about Annie?
Down the corridor, through a set of airlock doors, on a stainless steel table, lies a baby girl. Her eyes are shut, too, plastered down with clear tape. She is bloated and yellow -- a golden cherub.
Like Prof Jones, Annie is waiting. They are waiting for the same thing.
Downstairs, in the cafe, metal chairs screech. Business is closing for the day, though two customers remain. This is where Annie's parents Kerryn Kerr and Justin Billman wait. For two months they longed for this moment. Yet a note of desperation sneaks through Kerryn's normally bright front. ``We can't . . . '' she begins.
``We cannot lose another child.''
FOUR hours earlier, four floors above Kerryn and Justin, Prof Jones makes an incision that looks like a reverse L in Annie's chest.
Underneath the layers of blue cloth, her arms are spread out as though she is surrendering. A small section of her skin is exposed. It looks like processed cheese left in the sun to wrinkle.
Prof Jones has one knee raised and pressed into the side of the operating table. He's trying to get better access to his miniature work space.
Mum and Dad, Kerryn and Justin stick close to their precious Annie.
Just the previous week he was ``up to his armpits'' operating on media identity Derryn Hinch.
Patients tend to idolise Prof Jones. Some liken him to a rock star. He works on ``big kids'' at the Austin Hospital and ``little kids'' at the Royal Children's Hospital. He jokes that he is little more than an upmarket ``plumber''. Unlike most tradies, he has a soothing voice with a slight English lilt.
He can reduce something as complex as a liver transplant to a notepad sketch. Tonight he and liver surgeon Mr BZ Wang stand on opposite sides of the table. They peer down at Annie. Their patient is so small. It is like watching two men with tweezers moving checkers on a travellers' miniature board.
Prof Jones has done this operation 780 times, but on few patients as young or small. Annie is one of his tiniest liver transplant patients. She is only seven months old and weighs 6kg. She was just six weeks old when diagnosed with biliary atresia. The rare disease destroys the ducts that drain bile from the liver to the intestines. Without a liver transplant, Annie will die soon.
Kerryn and Justin have felt this pain before. A few years ago they terminated their unborn baby because he had extreme medical problems. ``It still hurts to this very day,'' Justin says. Annie's birth seemed set to change their fortunes.
Prof Jones
Surgeons Mr Wang and (background) Prof Jones snatch some rest.

Kerryn's cousin Rebecca Billman remembers: ``They finally got the child they always wanted.'' She was so precious, that they did not go faster than 10km/h on the drive home from the hospital after her birth. But Justin and Kerryn could do nothing to stop Annie getting sick. A pediatrician noticed something amiss with Annie's urine. He said something about tests, but Kerryn wasn't listening. ``Oh God, not again,'' she thought.

Annie's best hope, it transpired, would lie with Prof Jones and more than 100 members of the paediatric medical team. Her abdomen became so swollen that her dad says she looked like she was pregnant with quadruplets. It was 55cm, or two thirds the size of an average young woman's waist. The fluid build-up was making it hard for her to breathe. But just when her long-term Royal Children's Hospital gastroenterologist Dr George Alex thought she was big and strong enough for a transplant, she caught bronchiolitis. Annie was taken off the transplant waiting list for several weeks.

``It was touch and go,'' Kerryn recalls.

Annie's size was a major obstacle to a successful transplant, but there were some positives.

``Children are just incredibly robust and resilient,'' Prof Jones says. ``They haven't drunk, they haven't smoked and everything is in beautiful shape.''

The first part of the operation will cut through Annie's abdominal wall. A previous operation that joined the liver to the intestines has set like concrete. Prof Jones must delicately chip it apart again.
liver transplant
Professor Jones works on Annie.

He uses a tiny wand that emits radio waves and burns through the tissue. He powers it by pressing down on a foot pedal, which lets off a loud beep. A clear tube sucks up excess saline and blood from Annie's abdomen.

Specialists come in and out of the theatre, but there is little chatter. When a saline bottle slips further into a bucket of melting ice, there is an audible crack. The surgeon's movements are recorded on a flat-screen TV. It flashes up objects unfamiliar to the untrained eye. Is that a lychee? Calamari?

Above the operating table, bags of clear and red liquid hang from a type of medical hat stand. One bag will collect Annie's blood, which will drain into a cell-saver machine. It will wash Annie's blood and feed it back to her during the operation. Every drop counts when your patient has high blood pressure and the blood doesn't clot.

Prof Jones knows there is a chance his patient may bleed to death.
liver transplant
Annie ready for the operation.

``Everyone can cope with success. You get a pat on the back, everyone feels great -- it can be embarrassing,'' he says. ``It's coping with failure that is a key part of surgery. It's coping with the disasters and being able to turn up and do the same again that's the stressful part.''

Finally, he mobilises the liver. It is still attached to Annie by several key arteries and veins. He needs to make sure he doesn't do anything irreversible. The new liver may not arrive. It may not be suitable. No more work can be done on Annie until they see the replacement organ.

SOMEWHERE in Australia someone has died. In the midst of their anguish, the family makes a selfless decision to donate their loved one's organs. So begins a complicated surgery that looks like any other, except this patient is brain-dead. Each organ is examined. It takes the surgical team, headed by Dr Michael Fink, several hours to split the liver inside the donor's body. The big right lobe will be given to an adult. The smaller section goes to Annie.

It is not simply a matter of cutting it in half. Each liver is different. And the portions must each have enough branches to adequately connect the organ to their new hosts. ``A lot of this we have to make up on the day,'' Prof Jones says. It's only when they decide exactly how to divide the liver that the anaesthetist stops the donor's heart.

Instantly, each organ is flushed with an icy-cold preservation solution. ``The trick is to get the organs as cool as possible, quickly,'' Prof Jones says.

The liver's temperature drops from 37C to 4C within an hour. Surgeons have less than 10 hours to get the organ portion to their new patient. Prof Jones and Mr Wang are scrubbing for the second half of the liver transplant operation when a blue Esky on wheels turns up. In it is Annie's new organ.

Mr Wang smashes ice with what looks like a meat cleaver, and places it in a silver bowl. The liver will sit in the bowl until it is placed inside Annie. ``OK,'' Prof Jones says, as he walks in to examine the organ. He peels back a plastic bag full of watery pale pink liquid and reveals the small section of liver. He is holding Annie's life in his hands.

``Good liver,'' Dr Wang declares. Now it is the liver's turn to wait.

`SCALPEL,'' Prof Jones says softly. Two big lights glare down on Annie, who looks like she has half a sewing kit poking out of her abdomen. Seven hours have passed. It's time to remove Annie's diseased liver. It's an odd marbling of grey flesh, like a lichen-covered rock smoothed by the sea. Annie's arteries and veins, which feed blood into the liver and drain it out, have been lassoed with thread, clamped and are now cut.

Prof Jones explains the extraordinary moment when a patient is ``liverless'': anaesthetists are keeping Annie alive. ``When you don't have a liver, you have got to have someone there turning the knobs,'' he says.

Annie's vitals are monitored minute by minute. The liver performs so many different jobs. One of them is to control the blood sugar and acid in the body.

``These are things that could kill you in a few hours,'' Prof Jones says. It's crucial to keep the liver-free phase as short as possible. He lifts out the liver. It looks like a big lump. Austin Hospital associate nurse unit manager Ann Heydon says: ``It's so hard, nothing would pass through that.''

Prof Jones hands her the organ and she pops it in a plastic bag. In fewer than 10 minutes the new liver is scooped up and placed inside Annie's belly.



It's time for some real plumbing. The liver has little branches that link it to other organs, such as the heart and intestines. ``It's like darning a sock,'' Ann Heydon says, as Prof Jones sews the branches together. ``He'd make a good seamstress.'' After 25 years of liver operations, Heydon seems to know what the surgeons need before they ask.

The reperfusion of a liver is a magical moment. The new liver is glistening, but it's still pale. No blood is running through it. Prof Jones attaches it to Annie's portal vein. The clamp is released. Blood rushes into the liver. For a couple of minutes, nothing much appears to happen. Then, slowly, the liver starts to blush a pale pink. Its colour will continue to deepen. Eventually, it will be red.

MAKING room for Annie's liver has been like repacking a suitcase on the way back from a holiday. Finally, they find a way to squeeze it into Annie's chest. That's when the sewing starts. Every movement has a purpose. Prof Jones's white gloves are dancing above Annie's body like the hands of a mime artist. As he sews, the hole in Annie's abdomen is reduced to a small, fleshy wound. The nurses begin the equipment roll call to account for every pair of scissors, tweezers and swab. Eventually, there is a single red line, 6cm by 10cm, across Annie's abdomen. She is unwrapped.

Tape is gently teased off her skin until she is once again laying with her arms spread, still naked and still yellow. A big machine is wheeled into the theatre. Lights are dimmed. The radiologist with a Doppler ultrasound will tell surgeons if their plumbing job has become kinked. It lets them listen to the woosh of the blood rushing through the liver. It sounds like a mix between a wah-wah pedal and Rolf Harris's wobble board. So far, so good.

Mr Wang walks out of the theatre. He's been in there almost 11 hours. ``Little body, big job,'' he says.

A piece of plastic is wedged under Annie's body and used to slide her into a cot. She is wheeled down the corridor room to the Intensive Care Unit. ``Annie's been kind to us,'' Prof Jones says. She has made it through the operation.

FOR Kerryn, the hardest part is to come. The high of the operation soon dims to the reality of Annie's recovery.

``In my mind -- and I knew it wasn't right -- but I thought she would get the new liver and then I'd pick her up and walk out with her,'' she says.

Two days later fluid builds in Annie's lungs. She also develops an infection.

Yet, about the same time, she opens her eyes. Her tears are still yellow, but she is turning pink. The new liver is working. ``It was great to see her colour change from a deep jaundice to a normal baby,'' Royal Children's Hospital gastroenterologist Dr Jeremy Rosenbaum says.

For Kerryn and Justin, it signals a chance to feel like normal parents for the first time. Until now, Annie has been limited to a strange view of the world. She's used to lying on her back looking up at doctors. Her parents suspect she has learned to mimic their nodding. Now she can finally learn to roll and sit up -- like other babies of her age.

If Annie can make it through next year, there is every chance she will live.

GIVING ANNIE A NEW LIVER

The surgical team at the RCH anxiously await news of Annie's donor liver. Liver transplant surgeon professor Bob Jones needs to hear that the donor liver is suitable and that the operation to remove it has been successful.

Once he has received that call, he goes to work. Using a scalpel, he makes a cut into Annie's upper abdomen that looks like a reverse L.

Working carefully, his next task is to free Annie's diseased liver from her abdominal wall, the back of her stomach and her bowel. Then work stops as the surgical team wait for the donor liver to arrive.

When Prof Bob Jones returns to theatre, he has another job. He has to painstakingly undo the work from Annie's previous Kasai operation - which connected her liver to the small intestine.

He has to chip the liver away from the bowel - which he says has set like glue.

Annie's new liver arrives; it's been more than seven hours. It is removed from a plastic bag and placed into a silver bowl of ice so Prof Jones can examine it.

He likes it, so the operation continues. He disconnects Annie's liver from its blood supply by clamping the major veins and arteries. He then cuts each so he can remove Annie's diseased liver.

Only now does he place the donor liver into Annie's tiny abdomen. The donor liver is still a very pale pink color. He uses sutures to attach each vein and artery on the new liver to Annie's matching arteries and veins.

Now comes one of the most extraordinary things you will see in any operating theatre. Surgeons called it reperfusion. The clamps are removed from the major vessels and blood rushes through Annie's new liver. The liver changes colour to a deep maroon.

There is still one more thing that Annie needs - a bile duct. Prof Jones gives her one by trimming back a piece of bowel, shortening it and then attaching it to the donor's bile duct.

The last minutes of the operation are about stitching Annie's abdominal cavity closed.

She will have a 6cm by 10cm-long scar. Her mum hopes it will fade so one day she can wear a swimsuit.

After 10 hours and 21 minutes in surgery, Annie is taken to ICU. Prof Jones rings her parents to tell them the good news - Annie has a shiny new liver.





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