Cristina Cadei is prepared for her baby to receive a blood transfusion in utero. Photo: Janie Barrett
Tiny Lucia Cadei is frozen. Her chest moves mechanically, up and down, but everywhere else she is still. Born at 32 weeks, she is not supposed to be in the world yet.
Her mother, Cristina, reaches out to touch her for the first time, and cries. Less than an hour ago, Lucia underwent a lifesaving procedure aimed at keeping her alive and growing inside her mother. It had seemed to go so well.
Lucia is one of a growing group of babies that develop complications in utero that in the past would almost certainly have led to a tragic end. But today these rare and complex cases are increasingly being picked up and treated early, before the baby is born.
Alec Welsh examines Cristina's ultrasound. Photo: Janie Barrett
Cristina Cadei had no idea her pregnancy was in trouble. A routine test changed everything for the mother-of-two, and her husband Giuseppe, when they discovered that Cristina and Lucia’s blood was incompatible.
Antibodies from Cristina had passed the placental barrier, attacking Lucia’s blood and making her anaemic.
“When I first found out, I was devastated,” Cristina says. “You think ‘why’ and is your baby going to be OK?”
Cristina and Giuseppe in the morning, before the procedure. Photo: Janie Barrett
The only treatment is for Lucia to be given regular blood transfusions before she is born. This is the fourth time the Canberra couple have made the trip to the Royal Hospital for Women in Randwick, where Professor Alec Welsh, the head of maternal fetal medicine at the NSW Fetal Therapy Centre, must painstakingly pierce a needle through Cristina’s abdomen and into Lucia, to give her enough blood to replenish the red cells that have been depleted.
Cristina and Giuseppe are nervous. “He gets a squashed hand during this,” Cristina says.
“I feel a bit nervous,” Giuseppe says. “But what the professor does, it’s like a miracle. If you don’t see it, you don’t believe it.”
Lucia Cadei, just after being born in an emergency c-section. “She’s 1850grams, with a hat and a nappy - and a tube," the nurses said. Photo: Janie Barrett
When Cristina is wheeled into the theatre nurses and doctors are busily setting up the room.
Then, the lights are turned out, and all eyes stare at a grainy ultrasound screen. The team are not happy with the baby’s position. She’s breech, and at the moment is lying curled up, blocking all access to the veins the team could use to deliver the blood.
They try to turn her, pushing from outside Cristina’s abdomen. She cries out in pain.
Lucia Cadei, just after being born. Photo: Janie Barrett
When they finally pierce her abdomen, she lets out a deep groan.
Through the needle they first give Lucia a paralysing medication, which will stop her moving during the delicate procedure. Because of her position the team’s only option is to try to deliver the blood through her umbilical cord, something made extremely difficult by the flat ultrasound image not clearly showing when the needle is at the right angle to pierce it.
Finally, success. She is slowly given the lifesaving blood, and even appears slightly less anaemic than they would have expected, meaning Cristina may have more chance of carrying her closer to her due date.
A few days after being born, Lucia is already trying to feed from Cristina, despite being only allowed 20 milligrams, every three hours through a feeding tube. Photo: Janie Barrett
“It went really well,” Welsh tells her.
You can almost feel the room breathe out. Giuseppe gently scratches Cristina's nose for her. The nurses begin packing things away.
As Welsh heads out, he glances almost imperceptibly at the monitors still connected to Cristina’s tummy, asking his obstetric registrar to check her heart beat one more time.
Cristina Cadei sees her baby Lucia for the first time. Photo: Janie Barrett
Suddenly she is moving the ultrasound probe around Cristina’s tummy, in quick, searching moves. Welsh is called back in.
The room shifts. They have seen what so far remains unspoken. The baby’s heart rate has dropped, and it is beating at only half the rate it should be, as it struggles to cope with the heavy load of blood.
As Welsh patiently continues watching the baby, the room seamlessly kicks into gear. Equipment that was being packed away is unpacked. The dark, surgical greens that designate sterile areas are rolled out, as it transforms around us into a fully-equipped operating theatre. Welsh checks, waits.
Her pulse is back up again, from 60 to 130.
“It’s OK, it’s getting better,” he reassures Cristina.
“Oh thank God, thank you doctor”, Cristina says, reaching to touch his hand.
But then it drops again, and stays down.
“You think ‘I have to call at some stage that this needs a caesarean’,” Welsh explains later. “It just crossed that line.”
Only a few minutes later, she is born.
“That’s when the neonatologist cursed me for giving the medication [to paralyse] her,” Welsh says. Vital for the transfusion, it means Lucia cannot breathe. “We had to get oxygen into her straight away - it’s a question of being able to survive.”
Outside, the nervous wait continues.
Susan Walker, another of Australia’s leading maternal fetal medicine experts, says more women are needing help for their babies in pregnancy not only for conditions such as Cristina’s, which is extremely rare, but also because of their increasing risk profile.
“When it comes to women getting pregnant in 2014, compared to 1984, they are having babies a bit later, they are a bit heavier, and sometimes that means they have a bit more trouble getting pregnant, and so we are seeing this slightly increased risk,” she says.
The chairwoman of the Royal Australian and New Zealand College of Obstetricians and Gynaecologists maternal-fetal medicine arm says in some cases treatments are revolutionising babies’ chances of survival.
In conditions such as twin to twin transfusion syndrome - where an unequal sharing of the placenta means one twin does not get enough nutrients - if it is left untreated the vast majority of babies will die. But if caught early one or both babies will survive in about 85 per cent of cases.
It’s an unusual situation for a doctor, having one patient tucked away inside another - a woman who in many cases is otherwise completely healthy.
Walker says advances in treatment can bring ethical questions when procedures risk the mother for the benefit of her baby.
“The benefits to the fetus have to justify whatever risks might go to her.”
At the Royal Hospital for Women, Alec Welsh makes those decisions every day. He believes part of the solution is to involve a wide team of midwives and specialists to ensure that the mother has the support she needs.
While midwife Sandy Emerson is clearly in charge of the theatre, organising instruments and people in easy succession, she also advocates for Cristina.
“Making sure that Cristina is aware of what’s happening and everything is explained to her, and also Giuseppe, we have to remember dad,” she says.
Welsh believes it’s equally important for women with complicated pregnancies to have the opportunity to give birth as normally as possible.
“I don’t believe we can classify and identify women as being high, low, or medium risk, I believe people are made up of a multitude of components, and one of those may be high risk,” he says.
“She may go through her entire pregnancy physically completely normal... Once the baby is born there is going to be a focus on neonatal surgery, or paediatric neonatal care… but she is still going to go into labour just like everyone else”.
Back in the neonatal critical care unit, when Cristina sees Lucia for the first time, she is still paralysed. “She’s 1850grams, with a hat, and a nappy - and a tube," the nurses say. Around her, machines beep ominously, and the crying babies sound like tiny mewing kittens.
She cannot hold her, reassure her, protect her.
“I felt I was robbed, that I couldn’t have her with me,” she says.
But now, a month later, Lucia has been discharged from hospital in Canberra, and life is getting back to normal.
“I feel very, very blessed to have had the procedure,” Cristina says. “I know I could have lost her without it - she really is my miracle baby”.
Call for national register, funding
Doctors are calling for a national register and funding for medical procedures performed on babies before they are born, to ensure all children are given the best possible treatment.
The last two decades have seen growth in the procedures doctors can perform on babies that might not otherwise survive, but they are still so rare that there is little formal assessment of their success rates, and some invasive surgeries that received early hype have later been abandoned when it became clear they could do more harm than good.
Associate Professor Daniel Challis, a staff specialist in maternal-fetal medicine at the Royal Hospital for Women in Randwick said there had never been any federal research money allocated to the procedures.
“It means it might take longer to instigate these therapies because no-one is funding them, it might mean there is less ethical oversight, and to a certain extent individual clinicians can go ahead and do procedures they think are justified [without knowing how their outcomes compare]," he said.
Overseas research indicates different surgeons have different success rates. Mortality rates can be up to ten times higher in hospitals that perform surgeries less regularly.
The chair of the Royal Australian and New Zealand College of Obstetricians and Gynaecologists maternal-fetal medicine sub-specialty committee, Susan Walker, said Australia was in a particularly difficult position because of its geography and the rarity of cases among a small population.
“In [some of these cases], if someone just looked at the number of births they would see it would make more sense if they were just done in one place… but we are all so far away from each other, and some of these women are not in a position where they would be able to fly,” she said.