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Presenters: Phyllis Acosta, DrPH, RD, & Steve Yannicelli, MMSc, RD
MSUD Parents: Anne Fredericks, Tish Fuller & Glenda Groff

Following are thoughts and information shared by the panel in answer to questions from the audience.

The idea of giving the medical food throughout the day was emphasized. It is very rapidly oxidized if taken all at once and is used for energy instead of growth. If you are giving the medical food in one feeding, your child will not be growing as he would if you were giving it in three or four feedings. It is not complete unless natural protein (from foods) is given with it. One mother feeds her child 1 to 2 oz. of medical food every hour day and night when he is sick as a preventative measure to keep him out of the hospital. This may not work for all children with MSUD.

Cardiomyopathy has been associated with selenium deficiency. In an area of China that is severely selenium deficient, children die of cardiomyopathy.

Carnitine by mouth can cause diarrhea, but otherwise large amounts are harmless. Pharmaceutical grade is very expensive and not likely to be given in large amounts. The percentage of carnitine in the cheaper variety available in the health food store varies considerable and it is not wise to buy it.

Selenium is toxic in large amounts. It can be tested by measuring either plasma selenium concentrations or the concentration of an enzyme in the red blood cells that requires selenium to function.

There was a discussion on dental problems for MSUD. Here are some of the conclusions:

  • When held in the mouth, formula (medical food) carries the same risk for dental caries (causing cavities) as milk.
  • It is important to brush teeth after drinking formula.
  • Some of the children actually have good teeth - flouride helps. Many have sensitive teeth for no known reason.
  • Families handled wisdom teeth extractions differently. Some teens with MSUD had anesthesia; others were given locals. One was on glucose IV's for one hour prior to anesthesia and extraction, during extractions, and one hour after surgery. She did very well. Some teens were put on sick day formula before extraction to prevent elevation of levels. No serious problems were reported.

Questioned about B vitamins in the formulas, parents were assured the Food and Drug Administration has an Infant Formula Act which specifies the range of levels of vitamins that have to be put in infant products. The MSUD formulas contain all the known required vitamins. (It is the amino acids and not the vitamins that make the formulas taste bad).

Some parents may not be giving their children enough fat. Your child needs some cholesterol. One doctor advised his parents to switch their children from margarine to butter. Over restriction of fat causes growth retardation. It is important to have a balance of protein, fats and energy.

When there are symptoms of an impending illness, increase carbohydrates (calories), decrease natural protein and continue the formula even if you need to freeze it and give it as ice cubes. Try to keep calories and fluid intake high to prevent catabolism (burning muscle protein). The child may need more sodium when sick, because the diet powder is low in sodium. Adding sodium to the formula can cause serious problems, so check with your doctor.

CHANGE THE LIVES OF MSUD ADULTS AND CHILDREN

The MSUD Family Support Group is currently funding several research projects and we are proactively looking for researchers interested in developing new treatments or finding a cure for MSUD. Significant funding is necessary if we are to accomplish this goal.
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