By Janet Tappin Coelho in Brazil
A mother who has been living with the daily fear of her twin sons’ hearts stopping at any moment, revealed the emotional turmoil of watching one get better with a donor heart while the other remains chronically ill on the transplant waiting list.
Mila Costa’s identical two year old toddlers, Benjamin and Enzo, suffer from the same rare and deadly heart deformity, dilated cardiomyopathy (DCM), a progressive condition that causes the heart’s main pumping chamber, the left ventricle, to become enlarged, weakened and unable to push blood effectively around the body.
Doctors say it’s extremely rare for a baby to have the condition, and even rarer that both twins should suffer from it.
It’s the first case of its kind in Brazil and physicians at the National Institute of Cardiology (Incor) in Sao Paulo have been battling to keep the boys alive and stable with medication.
There is no cure to correct the defect. The only solution is for the tots to each receive a replacement heart.
And in a traumatic turn of events, the twin who had the greater need for the heart transplant had to wait while the other took his place and gained the new organ his brother should have had.
It’s left Mila and husband, David, both aged 35, devastated and ecstatic at the same time, as they watch one twin deteriorate while the other bounces back to life, full of the energy gained from the procedure.
Last Thursday 9 May, Enzo, who received the donor heart was discharged from hospital as doctors declared the transplant a huge success.
But it’s been hard for his brother, Benjaman. A family video made at the end of last month shows the touching moment, the adorable twins, who are too young to understand what has happened, were reunited after being separated for nearly two months following the critical surgery.
Recalling the emotional moment she received the news Mrs Costa said: “David was away visiting relatives when I got the surprise call late at night on March 7 from the hospital telling me there was a possible donor. I was told the child who died was roughly the same age, same height and had a very similar profile.
“I was immediately overwhelmed with a mixture of sadness, concern, worry and elation. There was absolute terror knowing this was a chance for one twin tinged with anxiety that we could lose him during the long and risky operation; fear that our other twin’s health would get far worse as he continued to suffer and wait for a donor; sadness that it had taken the life of another to give us so much hope and elation that we were being given this generous chance.
“As a mother, it would have been impossible for me to choose which one of my children should be given the heart.”
Thankfully, the doctors made the crucial decision for her the next day.
Benjamin, who was born first, was at the top of the waiting list with his heart failing fast and registering 11 per cent function. Enzo’s was at 18 per cent.
But on the day the donor heart became available, Benjamin fell sick with a viral infection which increased the risk of complications arising during surgery.
It meant Enzo, who was second in line, took his place.
The tots, who were born healthy in March 2017, were diagnosed with the killer disease at eight months old within days of each other.
Mrs Costa said: “It started with Benjamin getting tired really easily and struggling to breathe after playing. We thought he had the flu so we took him for a check-up.”
She was shocked by her GP’s reaction.
“He told me, ‘We need to get your child to a hospital immediately because he is dying’.”
The family put Enzo through the same tests and discovered, to their horror, that he had the same disorder.
The boys continued to be active and playful but their health rapidly deteriorated, displaying similar debilitating symptoms of shortness of breath, tiredness and difficulty in eating and gaining weight.
In desperation the family moved 1,450 miles from their home town Imperatriz in Maranhão, north east Brazil, to live closer to InCor hospital in the south east of the country.
In December 2017, the brothers were catapulted to the top of the waiting list for paediatric heart transplant, after doctors, carrying out a battery of tests to discover how they contracted the disease, found they also carried a de novo (new) gene mutation.
The genetic variation, apparently never detected before in Brazil’s population, is currently undergoing long term scientific research to discover what the variation and physiology means.
After waiting an agonising 15 months in the transplant queue, everything changed for one of the twins in March this year.
A tragic road accident in Goiania, some 600 miles from Sao Paulo, claimed the life of a 19 months old boy.
In the midst of their grief the bereaved family agreed to donate their baby’s organs, becoming one of the few in the country to do so.
The chances of finding a donor is especially acute for children in the South American country since organ size matters for liver, heart, and lung transplantation and there are not many deaths in younger children that are of the right match in size.
Mrs Costa said: “Neither of my sons are interchangeable so which ever one went first could only be seen as a blessing.
“Doctors said Enzo stood a better chance and I trusted their decision. I kissed and hugged him and told the medical team ‘take care of child, my joy, and don’t forget I’m going to have to go through this again’.
“I made the sign of the cross on his forehead and asked the doctors to return him to me alive”.
After an eight hour operation, surgeons reported that the transplanted heart was a success. It started beating on its own the moment they replaced Enzo’s sick and swollen one. And there was no need for artificial stimulation or medical intervention.
According to paediatric heart surgeon Luiz Fernando Caneo, the cardiopulmonary bypass pump (which temporarily takes over the function of the heart and lungs during surgery) was turned off and the new organ immediately generated enough pressure for the blood to circulate throughout the body.
“It was as if the heart was made especially for Enzo,” he said.
Although the child suffered a temporary setback last month when he contracted an infection, he was discharged last week once surgeons were satisfied the heart was not being rejected.
The grateful mum said: “Enzo’s body has adapted to his heart. He walks, plays and eats almost like a normal child. Before he used to be very pallid now he has a rosier colour. He’s put on some weight. It’s like he has been born again.”
Meanwhile Benjamin, who has been pining away at home for his brother, is still waiting for a compatible donor.
He is on medication to prevent his heart from suffering ventricular fibrillation – a life-threatening cardiac disturbance that causes fast chaotic heart rhythms.
The worried mum said: “Ben’s heart is very weak. A sudden fright or a strain on it from catching a cold or the flu could be fatal. He is only being kept alive by controlled doses of medication.
“But I believe that these crises in life are not sent to destroy us but to make us stronger and Ben’s chance of a new and healthier life will come in time,” she said with certainty.