At three weeks of age, Reuben and Joceline discovered that their little one, Adrianna, had a congenital heart condition. They share their journey through six open-heart surgeries over five years, each one carrying the risk of death.
WORDS CHRISTEL GERALYN GOMES
Adrianna Delilah Tupaz, Reuben and Joceline’s second daughter, was born on 24 October 2008 and discharged, a healthy baby girl, after a smooth pregnancy. Coming home, Adrianna soon showed signs of distress. Crying, she would take deep, ragged breaths and arch backwards. Adrianna wasn’t feeding well and couldn’t drink and breathe at the same time, but drank in short bursts instead, tiring quickly.
“It was a very difficult time,” said Reuben. “We tried everything, but she kept crying and arching backwards.”
A Mother’s Intuition
A week after birth, their paediatrician diagnosed it as colic. Joceline however, wasn’t satisfied. Instinct told her there was something else wrong and they sought a second opinion. They were soon told an entire chamber of Adrianna’s heart was missing. Adrianna was referred to the cardiac team at the hospital.
Reuben recalls that strangely enough, once the diagnosis was made, Adrianna stopped struggling. He explains, “[Before the diagnosis] every mealtime was a battle to hold her steady, to keep her calm… But after the doctor told us what the problem was, that same day, she stopped crying, stopped arching… it was as if she was trying to tell us that she had a problem, and once we found out, she became very calm.”
“Yeah, it was so weird! Now she would drink her milk slowly, but she would drink it,” Joceline chimes in.
Multiple Open-Heart Surgeries
The couple was soon told that Adrianna had hypoplastic left heart syndrome, a rare birth defect which meant there was a hole between the left and right upper chambers, such that the heart couldn’t pump oxygen-rich blood properly. There was the risk of lung collapse from the elevated pressure. Adrianna’s heart was also developed backwards, complicating matters further.
Surgery 1: Banding
Her first surgery was scheduled quickly for third December. Adrianna was only over a month old. The aim was to narrow her vessels to stabilise her condition till she was older and stronger.
Knowing the risk of death was high, Reuben and Joceline did a private emergency baptism the day before the surgery.
Thankfully, the banding went as planned. “But traveling home with the c-pap machine attached to Adrianna was not easy. Adrianna really did not like the tubes in her nose and velcro on her cheeks. We were told not to let our baby go without the c-pap for more than an hour. You could cut the tension at home with a knife,” said Reuben.
Adrianna did not talk for two years.
Surgery 2: Arterial rerouting
The next operation was scheduled for when Adrianna was two, to reroute her arteries. Again, Reuben and Joceline put on a brave front. “I was fighting back tears as I walked to the waiting area,” Reuben recounts.
After the surgery, infinitely relieved that she had made it through again, Reuben and Joceline took shifts by her bed for a month.
Surgery 3: Complications
17 days after her operation, a CT scan found fluid build-up behind her chest bones. Unsure of whether it was blood clots or fluid from pus, the doctors, suspecting it as the reason for her continuous fever, subjected Adrianna again to a knife through her chest bones.
The stress was beginning to show; Joceline found that Adrianna was losing hair and saw that her legs had also become skinny and weak from laying down. Reuben wanted nothing more than to take Adrianna home to her big sister Angelia, who missed her very much. Thankfully, her doctors soon agreed.
Surgery 4: Fontan
Adrianna’s final surgery was scheduled for when she was four. Reuben and Joceline hoped it would be the last. This time, doctors would widen the hole in the dividing wall of her heart permanently to equalise the pressure in her heart. They would also reroute the upper arteries.
Reuben remembers the walk to the operating theatre vividly; “As we entered the OT, Adrianna whispered, “Daddy, I am scared”, so I carried her and whispered back that Mummy and I love her and will be waiting for her. I sat her on my lap and she was calm and as they placed the gas mask on her, she did not struggle or cry and seemed almost happy. She was so brave. Slowly, as I cradled her, she fell asleep. I had been through this moment with her three times already and each time, it gets harder and harder to leave her in the OT and walk away…”
Two months after her discharge, Brave Heart found that she could run without being tired – their jubilance was inexpressible.
Unexpected Surgeries 5 and 6: The Closing Hole
Another unexpected blow came a year later – the hole that was widened “permanently” was closing, and only another surgery would fix it.
It was decided that a new procedure would be tried out, one that would be performed for the first time in Singapore, by a specialist who had done the procedure abroad. They would insert a bovine transplant valve as an emergency pressure exit.
“The fifth and sixth surgeries were the most stressful,” Reuben tells us. “This was the first time we felt like she might not make it”.
When it was time for the surgery, to their shock, their surgeon was replaced by someone else. Uncomfortable, all they could do was wait.
Although they were told that the surgery had gone fine, the entire surgical team filled the ICU in fervent discussion the morning after. The tension was palpable.
The electrical wiring connecting Adrianna’s heart to her brain was hit during surgery. Another open-heart surgery was needed, this time, to be done by their original surgeon, a mere five days after. Adrianna would need to be fitted with a pacemaker because of her now damaged nerves and weakened heartbeat. Joceline could see her surgeon was exhausted. “I’ll do it fast”, he promised.
It was a relief all around when their surgeon emerged with a big smile on his face. “I’m happy with how it went”, he said.
Today, Adrianna jumps about carefree. Although she will have to be on Wafarin – a blood thinner – for the rest of her life, Adrianna lives normally. She bruises more easily, bleeds longer, and will never be able to do competitive sports or climb mountains, but is otherwise healthy.
When Adrianna becomes a young adult, she will have a heart transplant.
Family is Everything
When asked how they did it, the couple said it was truly friends and family, camped out at the hospital with them, who kept them going. Reuben, who co-founded with Mark The Amazing Toybox, which performs music for children, said Mark “held the fort” when he needed time off. Joceline’s sister was also a reassuring presence, carefully timing her trips back from Australia to coincide with the operations.
They are especially thankful for Marjorie, their helper who went above and beyond the call of duty and never once complained. Marjorie often slept by Adrianna’s bedside from 7pm to 8am in the hospital. She is also Adrianna’s “best friend”.
Reuben started the facebook page, “Adrianna Brave Heart and Angelia Brownie Bear”, in the hope
of creating a space to connect with other families going through similar situations, especially since he found no support group in Singapore.