A triple transplant saves the life of a 10 year old girl with a rare disease

Íria, a girl of 10 years old, was born with heart problems. A few months had to be submitted to a transplant of heart, with which he has been living since then.

A triple transplant saves the life of a 10 year old girl with a rare disease

Íria, a girl of 10 years old, was born with heart problems. A few months had to be submitted to a transplant of heart, with which he has been living since then. When they operated, the doctors suspected that I had a disease ultrarrara, one which affects just 10 children in the world, a mutation of the gene NEK8 causes a disease rapidly progressive —there are even fetuses with this condition are born dead— that causes the tissues to esclerosen and organs to stop working.

Your new heart took a few days to work well, but once it started it has done perfectly, something strange inside of this disease so rare: Íria is the patient longest in the world with it. His liver had some problems, but was controlled with medication. But eight failed kidneys, which suffered a rapid deterioration that forced him to undergo dialysis. At that time the specialists from the Hospital Vall d'hebron of Barcelona discovered that I had this disease and decided to do a double transplant, liver-kidney. Never before has a child in Spain had been subjected to a transplant of these three vital organs.

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dr. Ramón Chaco, head of the department of Surgery, Hepatobiliopancreática of the center, explains that the surgery was “complicated”, since that was “very unstable” because of the heart transplant he received when he was a baby. The operation took place last October and, although the girl remains at home to prevent infections, and will soon return to school and to “make a perfectly normal life,” says Chaco.

The transplants multiple in children are not uncommon, according to Elisabeth Coll, medical director of the National Organization of Transplants. But they tend to be visceral, including the abdominal organs. “What's extraordinary about the case is that they would have needed above a heart and, above all, the pathology, so well carried out that it has been for the girl to continue to live,” he says.

Chaco explains that though the child is healthy, both physically and intellectually, and presents only a small deterioration in the growth, it is possible that other organs are submitting this esclerosamiento. “But there are so few cases in the world that we cannot predict the evolution,” he explains.

Now Íria is in the house "happy", hoping to return to school, something that will be expected after the winter, when infections are more frequent, according to Judith, his mother. "With the trasnplantes is inmunosurpimida and there have to be more careful, especially with your little sister [two years] that is already going to school," she said.

Date Of Update: 04 January 2020, 03:00
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