Double liver-kidney transplant for 16 year old twins
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A double liver-kidney transplant on the same day for two 16-year-old twin brothers suffering from a rare metabolic disease: a complex operation performed on Baby Jesus thanks to a compatible donor and the generosity of his family. Today the kids, who were forced into a strict diet to counteract the serious effects of their disease (methylmalonic acidemia), celebrate one year since the transplant with a quality of life more similar to that of their friends and schoolmates, also being able to indulge the taste of a pizza cooked by themselves in the catering institute they attend. On the eve of National Organ and Tissue Donation Day, which is celebrated on April 14, their story is an encouragement to all families going through similar difficulties.
What is methylmalonic acidemia
Methylmalonic acidemia is a rare disease that affects approximately 2 in 100,000 people. In the patients’ bodies there is a progressive accumulation of methylmalonic acid, a highly toxic and extremely harmful substance for various organs and systems such as the nervous system, kidneys, eyes and pancreas. From the first days of life, patients experience crises of metabolic intoxication, responsible for neurological disorders, neurocognitive deficit, growth retardation and renal failure.
“Until a few years ago – he explains Carlo Dionisi Viciresponsible for Metabolic Diseases and Hepatology of the Child Jesus – the only treatments available for this disease were based on a very restrictive diet with low protein content, the “precursors” of methylmalonic acid, which had to be maintained throughout life”. Despite the diet, which has a significant impact on the quality of life, patients still remain at risk for metabolic decompensation crises and for the development of serious complications related to the disease.
“Over the years – continues Dionisi Vici – an answer to these objective limitations of diet therapy has come from organ transplants: today liver transplants or combined liver-kidney transplants 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 arrival of the twins in hospital
The two twin brothers who underwent the double transplant arrived at Bambino Gesù in January 2021, referred by the doctors attending the Giovanni XXIII Hospital in Bari. Their story was characterized from the first months of life by frequent and prolonged hospitalizations for episodes of metabolic decompensation and, over the last few years, by progressive renal failure. In the Holy See Hospital they began the process of undergoing a combined liver and kidney transplant.
Both boys were followed from a clinical point of view and supported on an emotional-psychological profile by a dedicated multidisciplinary team. While they were on the waiting list for the transplant, in March 2023 the clinical condition of both entered a critical and delicate phase, characterized by a rapid clinical, neurological, cognitive worsening and with the need to start dialysis to treat the kidney failure. Luckily for them, a few days after having recorded these critical issues, the report of a compatible donor arrived and thanks to the generosity of the family who donated the organs, a double liver-kidney transplant was carried out on the same day for the two brothers.
Simultaneous liver-kidney transplant
“The simultaneous liver and kidney transplant in the twins, performed from a single deceased donor, was possible thanks to the application of a complex liver division technique, different from the one most often used, and the use of perfusion systems extracorporeal treatment of organs intended for transplantation – he explains Marco Spada, responsible for the Bambino Gesù Liver Transplant Program -. Our hospital possesses an exceptional sum of specialist and healthcare skills and uses all deceased donor and living donor transplant techniques to successfully transplant, as in the case of the twins, all patients on the waiting list, even those cases very complex that cannot be treated elsewhere. The difficulty of transplants is not only surgical and medical, but also organisational. In this sense, the Italian transplant network, managed by regional coordination and the National Transplant Center, is an example of effective integration between regions and national territory”.
A new life
One year after the transplant, the two brothers were able for the first time to start a life more similar to that of their companions and friends: they attend school lessons in the 2nd year of the Hotel Institute, actively participate in social events and make plans for the future. One of the twins’ dream is to become a chef: now he can cook for family and friends and finally also taste new foods, something that wasn’t possible before. The other twin is moving towards the profession of barman. The change in their living conditions and the new physical and psychological well-being affects the whole family.
“One year after the transplant, we see the doctors’ promise fulfilled: the boys are more serene and calm, with a new autonomy and maturity – explains the boys’ mother -. We parents too are experiencing the freedom of a lifetime for the first time “normal” with fewer worries about the management of their disease and their health. 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 live the same difficulty because the lives of their children and the whole family will change for the better.”
The search for the Baby Jesus
The story of the two brothers is also the fruit of the translational research activity carried out in recent years by the Hospital on hereditary metabolic diseases and liver transplantation, with the aim of improving knowledge on the natural history of diseases and offering new therapeutic approaches .”In particular, the extracorporeal perfusion system, today used to increase the availability of organs usable for transplantation and improve the outcome of the transplant, was applied by Bambino Gesù in an original and innovative way to the split liver and to the study of some rare metabolic diseases – explain the hospital leaders -. In some patients suffering from organic aciduria and defects in the urea cycle, the “diseased” liver – removed at the time of transplant and replaced with a “healthy” liver – was maintained in vital conditions with the instrumentation of extracorporeal perfusion, offering the possibility of studying metabolic disease in depth, making available an innovative means for the application of new pharmacological and gene therapies”.
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