We all know how important protein is for our bodies. It provides energy and helps us grow, breaks down food, and repairs body tissue, among other critical functions.\n\nAmino acids\u00a0are organic compounds that combine to create protein. So what happens when the body is unable to process some of these amino acids?\n\nTo meet some of the children on the liver transplant waiting list and to learn how you can help, visit\u00a0UPMC.com\/BeADonor.\nThe answer is\u00a0maple syrup urine disease (MSUD), a genetic disorder in which the body cannot break down certain amino acids, leaving toxic chemicals in the blood. This condition is found most often in babies and children.\nSigns and Symptoms of Maple Syrup Urine Disease\nThe symptoms and severity of MSUD vary from child to child. The most noticeable symptom is the strong and sweet odor similar to maple syrup that develops in the urine.\u00a0Other common symptoms of MSUD include:\n\nPoor appetite\nFatigue\nSeizures\nVomiting\n\nTreatment for Maple Syrup Urine Disease\nWhen\u00a0MSUD is detected during a newborn\u2019s screening, treatment must start right away. For some children, the disease can be controlled with a very strict diet. But a liver transplant is increasingly considered the best treatment option.\nDiet for Children with MSUD\nSince a child with MSUD is unable to process amino acids, a protein-restricted diet is very important. Even when the strictest diet is followed, complications can occur. Certain illnesses or situations can raise levels of amino acids, leading to:\n\nBrain damage\nComa\nMental disability\nDeath\n\nLiver Transplant for Maple Syrup Urine Disease\nIn addition to a strict diet, a\u00a0liver transplant\u00a0can help manage and possibly cure MSUD.\nThe new liver helps supply the body with the enzymes needed to break down amino acids and process protein normally. Many children with MSUD who receive a liver transplant can live a normal, healthy life\u2014 and may even be able to eat protein-rich foods.\nWhile a liver transplant can be life changing for a child with MSUD, it can be a very long and difficult process. Due to the shortage of organs, the wait for a liver to become available can take months to years.\nHow Can You Help?\nYou can give hope to children with MSUD and others who are waiting for a\u00a0liver transplant\u00a0by registering as an\u00a0organ donor\u00a0or becoming a living donor.