Baby Alice's broken heart

Five-month old Alice Spang was diagnosed with dilated cardiomyopathy, and today (June 19) flies to Germany for a potentially life-saving operation. Tadhg Evans spoke to her dad, Jan, and her mom, Ballyduff woman Majella Spang

Alice with her mom, Majella, from Ballyduff, and her dad, Jan, who is from Holland
Alice with her mom, Majella, from Ballyduff, and her dad, Jan, who is from Holland

Ballyduff woman Majella Spang and her husband, Jan, looked forward to their daughter Alice's first trip abroad. It was meant to be joyous, to fly out and meet family. Instead, their five-month-old baby will today take her first flight - to University Hospital Frankfurt, Germany - in the company of her mother, a nurse, a doctor, and a medical engineer for a potentially life-saving operation.

Alice was born on January 4 last, and her Mallow-based parents thought their first daughter was in perfect health. But as Alice gained weight, her body became too much for her heart. She was just four weeks old when, one morning, her breathing and circulation had deteriorated badly.

"Nothing showed up in the scans before she was born," Majella says, "and it couldn't, because what she has is cardiomyopathy; she has a weak heart muscle.

"Almost every baby is born with a small hole in their heart. It acted like a vent in Alice's heart. It closed up, and her lungs built up with fluid…It was terrifying."

A GP was the first port of call, who sent them the family Paediatric Assessment Unit in CUH. Her heart was beating at close to 195 times a minute; her saturation was well below normal levels.

"The oxygen in her blood wasn't getting around," Majella says. "Her fingernails and toenails were blue. They did an x-ray and found her heart was enlarged; she was seriously ill.

"We were told a doctor with a special interest in cardiology in children was on the way to do an echo on her heart and that we might be sent to Dublin. That was the first inkling that something serious was wrong.

"She was in heart failure that day and still is. Technically she's in end-stage heart failure. She has dilated cardiomyopathy. Dilated means the heart is enlarged, as are the vessels. Cardiomyopathy means it's a weak muscle. There's nothing structurally wrong with the heart. It's just a very weak heart muscle; it can't cope with the body that it's in.

"They ventilated her in Cork, and a neonatal transport unit came from Crumlin to bring her to Dublin. She went to paediatric intensive care, and they tried to take pressure off the heart by doing as much as they could for her with medicine, with breathing. They fed her intravenously. Her digestive system was too much for her to handle. Every time they went to change her nappy, her heart rate flew up. They thought it might be a viral or bacterial infection, they didn't know."

Alice got worse during her first days in intensive care, and medics spoke about her potentially needing to go to Great Ormond Street Hospital, London, potentially needing to go on a transplant list, potentially needing support from an ECMO machine. Her heart was functioning at about eight per cent. A passport was prepared, and her photo is of her on a ventilator.

In February, staff at Crumlin contacted a German cardiologist, who recommended re-opening the hole in Alice's heart, which got her off her ventilator and out of ICU. He also suggested Pulmonary Artery Banding [PAB], the surgery Alice will undergo on Friday.

"The condition Alice has affects the left side of the heart," Jan explains. "They will put a band on the artery going from the right side of the heart to the lung. This, we hope, will increase the pressure on the right side, reducing pressure on the left, giving the heart a more normal shape. This could potentially allow the heart to pump better.

"Over time the hope is, because of the better shape, the heart will strengthen itself. We hope to increase heart function to more normal levels, buying her time or taking away the need for a transplant. That last part is theoretical because it hasn't been around for that long…But we hope it'll at least buy her time.

"The only treatment plan other than the surgery we're going for in Germany is a new heart for Alice," Majella says. "If it's not successful, she has to go on the transplant list, and the only thing that'll make her live longer is a new heart. That's the stark and painful truth for my baby.

"There are more toddler hearts available. If this gets her to that age, it might buy her time."

The operation has huge risk attached, but research suggests it can boost heart function by up to 60 per cent, Jan says, adding that any improvement is better than no improvement. The procedure will be carried out in one of two ways: percutaneously, involving a normal incision into the skin, the less risky option; and, if that doesn't work, open-heart surgery.

Alice isn't on the transplant list, and it's hard to tell how much her medical needs will cost. Her parents aren't sure how long the surgery will take. They'll be away for a week, best case scenario. It'll probably be two to three weeks. Financially, these have been difficult times; accommodation in Crumlin costs €30 a night. While Majella is in Dublin with Alice full-time, Jan, a medical scientist in Mallow General Hospital, had to return to working a three-day week.

Alice is on a huge amount of medication. Her parents hope that home nursing will be approved soon. She came home for seven weeks earlier this year, but feeding her was arduous. It took hours at a time, and she couldn't be left alone in case she became entangled in her feeding tube.

Just one in around six babies present to Crumlin annually with this illness.

"She's so content, has no idea that anything is wrong. She has a smile for every new nurse and doctor she meets. She's so strong that during a cardiac arrest a few weeks ago, she woke up," Majella says. "We see our little baby, and we feel robbed. Every hope and dream we had for her is gone.

"Babies aren't born with tears. Her first tear silently came out of her eyes while she was incubating in ICU. That broke me in half. Our hope now is to get her home and give her the best first birthday any baby has seen."

"You're expecting a child, dreaming of what their life will be like," Jan says. "You see other parents and think 'some day, that'll be us'. You think of their first day in school, first everything. Many of Alice's 'firsts' have happened in hospital. We're hoping the treatment will become available to others and will hopefully save lives. It isn't available in Dublin, but they would like to bring it here."

"If it's successful, it may open up a treatment to other babies with this," Majella says. "There are children, babies out there with serious diseases that aren't allowed on the transplant list. This could potentially prolong their heart and have ramifications for every baby in this country."

A GoFundMe page is live, and is titled 'Alice's broken heart'.