Sheree Kearns was just 20 weeks’ pregnant when doctors told her that her baby had a 50/50 chance of survival.
Subscribe now for unlimited access.
$0/
(min cost $0)
or signup to continue reading
Up until then, everything seemed to be going perfectly with the pregnancy, except for a bit of pelvic pain.
A scan to check on what was causing the pain instead picked up that something wasn’t right with the baby, but it wasn’t until they arrived home later that night that the doctor rang with the news.
“They thought it was congenital diaphragmatic hernia which is where there is hole in her diaphragm, and her organs that are meant to be in her abdominal cavity are actually in her chest cavity,” Sheree said.
Another scan the next day confirmed the diagnosis, and found Calla’s stomach and bowel was in her chest.
They later found that the left lobe of her liver was also squashed into her tiny chest, and they didn’t even know if Calla even had a left lung until she had surgery.
While she had a right lung, her heart was twisted and pushed over to the right side of her body.
“They gave her a 50 per cent chance of survival,” Sheree said.
Facing the news that their surprise honeymoon baby may not make it was the start of a rollercoaster ride of emotions for the couple. “That first week … we were just zombies. It was awful,” Sheree said.
By the next week the emotions flooded over, and then reality set in.
“They do give you the option of terminating because the baby may not survive anyway,” Sheree said.
“We were just too upset. We were still coming to terms with the diagnosis that we couldn’t consider it. We went to the women’s (hospital) the next week and they looked at her and told us she may be ‘compatible with life’ which gave us hope she’d be OK”.
Congenital diaphragmatic hernia affects one in 2500 babies worldwide, and while the chance of survival is 50 per cent they were told that at the Royal Children’s Hospital in Melbourne it is more like 80 per cent.
However, Sheree said they couldn’t promise that the odds would be any better than 50/50 until she was born.
In Victoria, 10 to 15 babies born are with the condition every year and while the condition can be genetic, in most instances the cause is unknown.
At 36 weeks, the couple had to relocate to Melbourne and wait. “It was too risky to stay here,” Sheree said.
But it wasn’t until 39 weeks and two days that Calla was born. “It was probably the worst part of the pregnancy. You were away from home, sitting and waiting not knowing if your baby was going to make it or not,” Sheree said.
In between the weekly scans, there was a lot time to kill for the Woolsthorpe couple. “I read five books and I’m not a reader,” husband Pat, a sheep and cattle farmer, said.
When the day finally came to be induced, Sheree ended up being sent home from hospital because the Neonatal Intensive Care Unit (NICU) at the Royal Children’s Hospital was full.
On February 23, Sheree was induced not knowing if she would even get to hold her baby before they whisked her off to the Royal Children’s Hospital down the road.
If the baby cried, she was allowed a cuddle and luckily Calla – who weighed a healthy 3.1kg - came out crying.
But it was just a very quick cuddle, and it would be 10 days before she could hold her baby again and three-and-a-half weeks before they could pick her up without the help of up to three nurses.
Sheree had to stay in hospital for another day, while Pat went with Calla in the Paediatric Infant Perinatal Emergency Retrieval ambulance for the one-kilometre journey to the children’s hospital. Five days later the doctors operated right there in the NICU.
“They don’t move them because it’s too much of a risk. They’re that fragile,” Sheree said. “It’s terrifying being kicked out of the room and being told just go wait for a few hours.
“The actual condition, the hole itself, is easy to fix. All they have to do is pull the organs through and then sew it up. It’s everything after that, her lungs and her heart.”
Baby Calla was heavily sedated and only came off sedation a few days before they were discharged from hospital almost six weeks later. “Withdrawal is one of the worst things for them because they are so fragile,” Sheree said. “They tried to wean her off and she went into hypertensive crisis which can kill them.
“We went for lunch and came back and if the nurse wasn’t there and she wasn’t hooked up to this equipment you would have thought she was dead. She was white. It was awful.”
That was when Pat finally cracked and the tears came. “He held strong until that day,” Sheree said. “That was really, really scary. The way all the nurses reacted and the doctors came in and you just panic.”
It happened a few more times before doctors worked out what was going on.
They had thought Calla’s pulmonary hypertension had resolved on its own after weaning her off nitric oxide, but discovered it hadn’t after the hypertensive crisis episodes. Then they were able to treat her and wean her off the heavy sedation drugs.
So to control the blood pressure in her heart and lungs until she outgrows it, Calla will need to take a low dose of sildenafil – commonly known as viagra, as the doctors and nurses loved to remind them.
“There’s apparently a valve in the heart that closes when babies are born, hers hadn’t, but that’s actually a good thing because she’s got that hypertension and it acts as a bit of blow-off valve with all the pressure.”
With just that initial first cuddle, the couple had to resist the urge to stroke their baby’s head or rub its hand during those first few weeks. “You’re only allowed to touch, you’re not allowed to rub,” Sheree said.
“They say that CDH babies don’t like being touched, they don’t like being moved. It can causes them to stress, and with all her hypertension that’s not good.”
Eventually Calla was able to breath on her own after coming off the ventilator. After six weeks in hospital, and countless hours of trying to fill in time, they were able to come home. But doctors will continue to monitor Calla, and hopefully her left lung, which was squashed, will continue to grow. “Last time they checked she was only using it for 10 per cent of her breathing, so she’s still relying heavily on her right lung,” Sheree said.
Calla is now part of the Royal Children’s Hospital’s research project into how babies with CDH, or who are premature, learn to use their lungs as they develop.
“She’s very, very susceptible to any respiratory illness, so if she gets a cold she may have to be hospitalised just because they need to make sure she’s able to breathe.
Pat said: “We were in the supermarket the other day and a woman coughed and I just bolted. I just thought ‘no I do not want to go back to hospital with her’.”
Calla has also been approved for the RSV immunisation which protects against the respiratory syncytial virus which can cause bronchiolitis.
“It’s not an immunisation as such, it’s an antibody and her body starts to reject it after four weeks and you need to go back,” Sheree said. “Not many babies get approved for it each year.”
At about six weeks old, the couple was able to bring baby Calla home. She still had a feeding tube in, but the next day she pulled it out. “Gee I panicked,” Sheree said.
Thankfully, Calla was able to feed normally and started to gain weight – something that didn’t seem possible just six weeks before when they weren’t even allowed to feed her. With her stomach squashed into the her chest, any expansion from feeding would put pressure on her already compromised lungs.
As the scar – which stretches about 8cm across her stomach – fades, like any new parents the couple have to work out what what is normal baby behaviour and what they should be concerned about.
When just days after arriving home from hospital Calla wouldn’t stop crying, the couple were just about to rush her to the hospital thinking she may have had a blocked bowel when she burped and she was fine. “Having been through what we’ve been through, we’re probably a bit more paranoid than most,” Sheree said.
“It seems so weird saying she’s fragile because she’s just a normal baby now with smaller lungs.”