During illness or after an injury, the body increases the breakdown of protein from muscles and organs which increases blood levels of the branched chained amino acids leucine, isoleucine, and valine. If unchecked, this can lead to serious complications. Early signs of dangerously high levels include vomiting, excessive sleepiness, coordination or balance problems and/or changes in mental status. These signs are more apparent in children then adults.
Watch for these early signs of dangerously high leucine levels:
During any illness, it is very important to notify your metabolic clinic immediately. Often, the diet is adjusted to decrease or eliminate leucine intake, increase calories, and increase isoleucine and valine supplements. These changes help slow the breakdown of protein from the body.
For MSUD, the amount of special medical food (supplement devoid of the branched-chain amino acids) is typically increased to 110% to 150% of the usual prescription and additional isoleucine and valine is provided. If any table foods are taken, they should contain minimal or no leucine so leucine intake is lowered to near zero. The goal is to keep the caloric intake high enough to prevent the breakdown of body proteins.
Anti-nausea medicines can be used to prevent vomiting, especially if the vomiting is related to a gastroenteritis (stomach flu). Medicines may also be prescribed for diarrhea. Check with your doctor for appropriate medicines and doses for the age of the child. Also check about any other cold remedies or medications.
As the individual improves, they may return to the usual daily formula, and slowly increase the leucine from natural foods over a few days.
Frequent communication with your metabolic staff is essential to determine if home care is sufficient or if hospitalizing the ill individual with MSUD is necessary.
Notify your metabolic team immediately at the first sign of illness. They will provide specific guidance for your situation.
Increase medical formula to 110-150% of normal, reduce or eliminate leucine intake, and increase isoleucine and valine supplements as directed by your metabolic team.
Be prepared to go to the hospital if symptoms worsen or if directed by your metabolic team. Have your emergency protocol letter ready.
This protocol is only a guideline and should NOT be used for definitive treatment without metabolic consultation. Always contact your metabolic team for personalized guidance during illness.