The protein-restricted diet is unique in many ways.  One thing that always has acquaintances shaking their heads is the freedom with which those with MSUD can eat fats and sugars.  When our children are little we struggle to get enough calories into them to prevent a breakdown of body tissue.  In this wacky turned-around world, soda is good, beans are bad.  French fries and potato chips are a go-to items when away from home.  My daughter used to eat the butter sitting on the table at restaurants, and still occasionally does when she needs extra calories.  While my other kids drink water or artificially sweetened drinks, she will have a Coke or Pepsi.

Metabolic formulas are the backbone of the MSUD diet.  As we clearly see from the stories we hear from around the world, access to metabolic formulas means the difference between life and death, life with normal physical and intellectual abilities and life with impairments.  But what about all that fat and sugar they’re loaded with to provide adequate calories?  Might the MSUD diet result in other problems such as obesity and chronic disease while allowing our kids to live “normal” lives?

A new paper published in the Journal of Human Nutrition and Dietetics reports on research addressing this issue in individuals living with PKU in Great Britain (Robertson 2013).  The researchers identified 236 individuals older than 16 years of age and obtained information on body mass index (BMI) and phenylalanine levels.  55% had a BMI indicating overweight or obesity.  While high, this was consistent with the rest of the British population.  The authors concluded that patients should be educated on a healthy low phenylalanine diet to prevent the development of chronic diseases.

This advice clearly applies to those following an MSUD diet as well.  Most fruits and vegetables are naturally low in protein and high in antioxidants and other phytochemicals associated with good health.  Vegetables prepared with olive oil will provide healthy fats for more calories.  Swap sodas and other highly sweetened drinks (teas, lemonade, punch) with water, flavored water or seltzer, or sports drinks such as Gatorade which have about half the sugar and calories of other sweetened drinks.   Let’s make sure that the MSUD diet provides the nutrients known to enhance health while limiting those that may compromise it.

Robertson L.V., McStravick N., Ripley S., Weetch E., Donald S., Adam S., Micciche A., Boocock S. & MacDonald A. (2013) Body mass index in adult patients with diet-treated phenylketonuria. J Hum Nutr Diet. 26 (Suppl. 1), 1–6 doi:10.1111/jhn.12054

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A Child's Life

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