Story by Claire Elliot
BATTLING baby Evie Fraser has been hailed a “miracle” by doctors after she was born with her internal organs in the wrong place or back to front.
She has two right lungs, her heart is on the wrong side with four life-threatening defects, and her liver is in the middle of her abdomen, pushing her back-to-front stomach out of position.
Her intestines are also the wrong way around, she has no spleen and scans have yet to confirm if she has an appendix.
Doctors initially thought they would need to operate within the first month of her life, as she has no blood going to the left side of her heart.
Her worried parents Lyndsey Houston and Ruaridh Fraser, both 27, even had their daughter christened by the hospital chaplain as she battled for life in intensive care.
But as the bouncing baby approaches her first birthday on Sunday (February 17) - a day her parents feared she would never see - she has amazed everyone with her incredible resilience.
Proud Miss Houston, from Saline, Fife, said: “She really is my wee miracle.
“I can’t believe she’s doing so well without surgery. She’s amazing.
“When she was in intensive care one of the consultants said it was a miracle she even survived in the womb.
“And here she is, still thriving.
“You wouldn’t tell there is anything
wrong with here. She’s always
smiling and everyone who meets
her falls in love with her instantly.
I’ve never seen a baby as happy
Evie was born with right atrial
isomerism, a condition so rare
that there are thought to be
only two other cases in the UK.
As a result, Evie’s lungs are a
mirror image of each other,
with the left one formed as
if it was a right one.
Her heart is also on the right side instead of the left and has four major defects, including a large hole, no blood vessels connected to the body‘s main pumping chamber, and no blood being pumped to the left side of the organ, depriving it of oxygen-rich blood.
The fourth anomaly is a narrowing of the pulmonary artery, which pumps blood to the lungs - a defect which would normally be life-threatening but is helping to reduce the pressure and amount of blood going to Evie’s lungs.
Her liver also sits in the wrong place. Instead of being at the right side, it is in the middle and is pushing her stomach to the right side instead of the left.
And her stomach and intestines are back to front.
Her spleen is also missing, leaving her more susceptible to infection.
Miss Houston and her partner of 10 years, a sales advisor, know that Evie will need major surgery this year to prolong her life.
But the older their daughter is when she has the open-heart procedure, the better chance of success.
During the surgery, which could take up to 20 hours, Evie’s heart will be stopped and she will be placed on a heart and lung bypass machine, while doctors re-route the blood-flow around her heart.
Evie may also need a heart transplant in the future, but it is not clear if a normal heart could be used as her anatomy is so complex.
Miss Houston said: “We don’t know what the outlook will be. There is no cure for it and any surgery she does get will be palliative. All we can do is hope for technology to progress. But right now I am just enjoying every day with her.
“We just try to live day to day and not plan for the future. I don’t plan past cardiology appointments.
“We know she’ll need the surgery this year so since New Year that’s all I can think about.
“I’m dreading it. But she’s growing so well, they are going to do two open heart operations in one go.
“I’m so proud of her. Evie’s always put up a fighting spirit. She’s a fiery little red-head,” she added.
The family’s nightmare began just two hours after 8lb 13oz Evie was born at the Victoria Hospital in Kirkaldy on February 17 last year.
Miss Houston, who is also mum to Reiley, two, was waiting to go home with her new baby when the midwife noticed Evie’s skin was still blue.
Tests showed her oxygen levels were low and she was taken to the special care baby unit.
She was initially treated for a chest infection.
But when her condition failed to improve, that night she was transferred to Yorkhill Hospital in Glasgow, where doctors confirmed what was wrong.
Miss Houston said: “We were just devastated.
“I had a text-book delivery. Everything went so smoothly and in an instant it changed.
“I had her for two hours and I was told she was being discharged and then the midwife noticed her colour hadn’t improved.
“She was taken to special care baby unit and she had wires coming out of her arms and cannulas in her feet. Nothing prepares you for that. She was just a different baby to the one I had a few hours before.
“To go through nine months of pregnancy being told everything is healthy and then to be told that was horrendous.
“We didn’t know if we were going to lose her. I never got attached for the first few weeks. She needed an anaesthetic every time she got a CT scan and I just thought ‘how can her heart cope with that?’
“For days I just thought, this could be our last day with her. It was so touch and go.
“It was so hard to deal with it all. We just had to see how she was on a daily basis.”
The couple were dealt another agonising blow when Evie was struck down with a virus that went into her lungs at 12 days old and had to be ventilated for four days.
As she recovered in high dependency, Evie, who was also tube fed for several months, was christened by the hospital chaplain ahead of her planned operation at a month old.
But after five weeks in hospital the battling baby was allowed home to see how she coped without surgery.
Since then has continued to amaze doctors and her parents with her amazing fight for life.
Evie has been rushed back into hospital twice by ambulance with serious chest infections this winter.
But both times she has pulled through and on Sunday she will mark her first birthday, a day her parents thought she would never see.
Miss Houston said: “I still cry most days. The things you normally take for granted are such a big deal with Evie.
“Even putting on weight in the first few weeks was a massive milestone.
“The condition is so rare, not a lot is known about it. Her anatomy is very different. We don’t know where her appendix are, or if she had even got them. She’s very complex.
“But she’s remarkably well. She’s just started crawling and she dotes on her big brother. As long as he’s there, she’s happy.”
She even loves to play with his dinosaur toys and cars, as well as her own dolls.
And like most babies she loves being made a fuss over.
Miss Houston said: “It’s amazing she has come so far. She just makes me smile so much.
“I’m just so thankful every time she sits on my knee and cuddles right in.
“But I try and let her be as normal as she can. We just go with the flow and live life one day at a time.
“There are so many nights I’ve prayed and I feel like my prayers are being answered. She really is my wee miracle.”
Just 15 years ago the mortality rate for babies with the condition was 95% in the first year.
Doctors also told Miss Houston that had Evie been born just five years ago, there would have been no treatment available and she would have been sent home to die.
And with the help of family and friends, they have already collected more than £8,000 for the Yorkhill Children’s Foundation, as a thank you to staff for the care Evie has received at the hospital.
EVIE’S FOUR HEART DEFECTS
Right atrial isomerism is thought to affect only around four in one million babies worldwide. It is characterized by defects in the heart, spleen and paired organs such as the lungs and kidneys.
Evie has four major heart defects:
She was born with total anomalous pulmonary venous drainage, which means there is no blood going into the left side of her heart. Instead it is pumped back into to the right side.
She also has double outlet right ventricle, which means there are no blood vessels connected to the heart’s main pumping chamber to the body.
Her third defect is atrioventricular septal defect, leaving her with a large hole in the wall between the upper and lower chambers of the heart.
And she has sub pulmonary stenosis, a narrowing of the pulmonary artery, which pumps blood to the lungs.
Evie Fraser with her mum Lyndsey Houston
Evie Fraser aged two days old in intensive care