Triple transplant patient Iria, 10, leads 'normal life' after pioneering surgery

Iria, 10, was born with a gene mutation known as NEK8, which causes serious major organ developmental problems.

The condition affects the heart, liver and kidneys, causes fibrosis and sclerosis of the tissues, and affects the bones and the nervous system.

But Iria is quite possibly the only child to date with the condition who has lived beyond infancy.

Babies born with the NEK8 gene mutation rarely survive beyond being a foetus, and those who do normally die within months of birth, since the condition progresses rapidly and the only solution is multiple organ transplants.

Said to be one of just 20 living patients on earth with the NEK8 mutation, Iria was diagnosed with a hypertrophic myocardiopathy at five months of age, which, as the Vall d'Hebron explains, 'is incompatible with life'.

An immediate transplant was carried out as the only way to save her, but at this point she had not been formally diagnosed – time was not on medics' side as they opted to carry out the transplant and investigate the cause later.

Iria was the first NEK8 patient in the world to undergo a successful heart transplant, but the condition would persist and she was not expected to live beyond infancy.

At the end of 2019, however, she underwent a kidney and liver transplant.

Her liver was said to be too large for her age and was showing signs of fibrosis, and her kidneys had failed, meaning she needed to be on dialysis.

Surgeons said the double transplant was her only possible chance and there was no other way of treating her.

Following a 12-hour operation – making her the first case in the world with the NEK8 mutation to have received three new organs, Iria is now said to be 'free from disease'.

The latter two organs were implanted in the same operation, and although complications arose within the first 24 hours, she has now fully recovered.

Doctors say her prognosis in all areas is exactly the same as in any other patient who has undergone an organ transplant – meaning she is likely to live a normal life expectancy and the lifestyle of any other 10-year-old girl.

In fact, director-general of the Vall d'Hebron, Dr Antonio Román, says it is 'very important' that she does live a normal life for a child of her age.

Her mum, Judith, says Iria now has 'a permanent smile on her face', and that the family has been trying to treat her as 'normally' as possible over the last decade of her life.

And now, says Judith, “we don't want to treat her as though she were ill, because she's not, now.”

The Vall d'Hebron is the top transplant hospital in Europe, and one of the most pioneering.

Between 2016 and 2018 inclusive, the centre carried out 213 transplants, and another 43 were effected on child patients in 2019.

One in three paediatric transplant operations – including heart, kidneys, liver, lungs and bone marrow - is carried out in the Vall d'Hebron.