Guide To Treating Maple Syrup Urine Disease

Intravenous Treatment

A patient with maple syrup urine disease is constantly at risk of a metabolic crisis, even if they follow a strict diet. A metabolic crisis occurs when the amino acid levels in the blood get too high. Patients must be treated with emergency medical intervention to lower their amino acid levels, particularly their levels of leucine. Some medical professionals recommend aggressive medical therapy to protect against and during metabolic crises. The goal of this therapy is to reduce the factors causing the crisis. One way to do this is by increasing the calories an individual ingests. Any method of doing so is deemed valid. A patient may have intravenous treatment with glucose. If a doctor deems it necessary, there may also be a glucose-insulin drip added to the treatment plan.

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Liver Transplant

One treatment option a doctor may recommend is a liver transplant. Usually, this is done when the patient is still a child. There have been some reported cases where, following a transplant, previously afflicted children had their maple urine disease cured. Their metabolism corrected itself and began to break down the amino acids as it was meant to. A transplant is successful when it cures the disease and allows the child to eat an unrestricted diet. This is the only known cure for maple syrup urine disease. Transplants can sometimes be risky, so it's important for everyone involved to weigh the benefits and the risks. The biggest long-term concern for patients tends to be immunosuppression. It's also common for the body to reject a transplanted organ, so doctors and patients need to take great care following the surgery.

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