Presented by Neil Buist, M.D. - Portland, Oregon

There are 130 metabolic diseases treated in Oregon. Oregon was the first State to set up screening for MSUD. Dr. Buist showed slides including the first infant with MSUD to be identified through state screening (Monte Brubacher, son of Wayne and Joyce, 3/12/65-12/4/74).

Certain amino acids, vitamins and trace elements cause the bad taste and smell in most formulas. Dr. Buist and the Oregon Clinic developed a good tasting MSUD formula that Dr. Buist brought along to the Symposium. In it they eliminated some nonessential amino acids and adjusted the amounts of others. It is similar to the new PKU formula Periflex, developed at the Clinic and distributed by S H S North America (new name for Scientific Hospital Supplies). The adults and children sampled the new MSUD formula.*

There is the possibility of using the amino acids to make foods that look and taste like their counterparts but do not contain the BCAA. However, amino acids cannot be baked. They are best used in spreads. There is research being done on a protein free cheese. Dr. Buist's dream is to make a BCAA-free hot dog someday!

Massachusetts has a law that requires manufacturers to add a medical label to medical foods. This makes it easier to get insurance to pay for the foods.

Phenylalanine is a certain percentage of protein but leucine amounts vary in different foods. A very general rule for figuring leucine is to double the phenylalanine amount in a food.

Questions from the audience

Q. Is MSUD included in the Oregon health plan?
A. MSUD is very well treated in the health plan, since there is a treatment available, and death is the result if it is not treated

Q. What about the National health care plan?
A. Dr. Buist fears that the metabolic diseases will be discriminated against, because they are of a minority group. (Ted Kennedy's health care plan includes language for coverage of medical foods.)

* I was skeptical when Dr. Buist handed me a cup of liquid and said, "Taste it." Hm-m-m, it looked good and yes, it even smelled good. I took a tiny sip and immediately took another drink. To me it tasted like orange sherbet. And believe it or not√∑no aftertaste! All of the other formulas make me gag at the smell and the taste is worse than the smell. This new formula enthused the parents but not all the children.

Many children with MSUD are skeptical of anything that looks a little different or is "good for them." Each child was served a cup of the formula during one of their break periods in the activity room. They were not told it was formula and their reactions were video taped and shown later to the parents.

It was quite amusing to see them look at this strange looking drink. Some drank it down without the least suspicion√∑especially the siblings of the children with MSUD. Some would hesitate, take a small sip, set it down and act so undecided. Others with MSUD refused to touch it. The teenagers and young adults liked it. Shayla thinks it would be great.

One girl with MSUD wouldn't taste the drink, but her sister asked for refills during the children's break and later was filling her cup from the pitcher in the conference room. I think the new formula would be great for those who dislike their current formula and for the newly diagnosed.

Dr. Buist says it should be no more expensive than the current formulas. It is not the ingredients that are expensive, but the processing. It may be more expensive if the whole family wants to drink it! Imagine having formula in the refrigerator that doesn't create a catastrophe if a friend begins to drink it by mistake. Hurry Dr. Buist, hurry, get it on the market.


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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