The family of a pair of twin brothers who underwent double liver-kidney transplant at Bambino Gesù Pediatric Hospital are celebrating one year since the successful procedures. The two 16-year-old brothers both needed the same surgery to treat their rare cases of methylmalonic acidemia, and the surgeries were performed on both boys on the same day, April 14, 2023, which happened to be the eve of National Organ and Tissue Donation Day.
The disease methylmalonic acidemia is estimated to affect only two in 100,000 people worldwide. The condition causes an accumulation of methylmalonic acid, a highly toxic substance that can interrupt the body’s functions. It especially causes problems in the nervous system, kidneys, eyes, and pancreas, but it can lead to other complications such as neurological disorders, neurocognitive deficit, growth retardation, and renal failure.
Carlo Dionisi Vici, head of Metabolic Diseases and Hepatology at Bambino Gesù, explained that until recently, the only treatment for methylmalonic acidemia was diet with high restrictions placed on the daily intake of proteins.
“Over the years,” Dionisi Vici said in a press release, “an answer to these objective limitations of diet therapy has come from organ transplantation: today liver transplantation or combined liver-kidney transplantation are increasingly used to improve the prognosis of patients with methylmalonic acidemia, to reduce the risk of complications and improve the quality of life.”
The twins had been waiting for transplants since January 2021 when in 2023 their conditions became critical. The boys required dialysis to treat kidney failure, while their neurological and cognitive functions worsened. Just days after their conditions turned critical, however, a compatible donor was identified and the boys underwent a double liver-kidney transplant on the same day, with each boy receiving a kidney and half a liver.
Now, one year after the surgery, the boys are happily living a lifestyle like that of their peers, attending high school and enjoying the freedom to eat what they like. Without the dietary restrictions, one of the twins has found a love of cooking and hopes to be a chef, while the other is interested in working in the bar industry. Of the transformative effects of the surgery on their quality of life, their mother commented:
“One year after the transplant we see the doctors' promise fulfilled: the boys are more serene and calm, with a new autonomy and maturity... It was a very difficult journey, in which we experienced a lot of fear and anxiety, but today I feel like recommending the choice of transplant to other parents who experience the same difficulty, because the lives of their children and the whole family will change for the better.”
Bambino Gesù credits the success of this difficult pair of surgeries to the expert multidisciplinary team of doctors and surgeons at the hospital, as well as advancements in technology that have increased the chances of a split liver being accepted by both patients. Furthermore, the hospital noted that it is utilizing “extracorporeal perfusion instrumentation” to keep the diseased livers taken from the two boys in vital condition so that they may be studied to learn more about metabolic disease.
