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

Melvin Carruth

This letter is a tribute to my brother Melvin Carruth! We believe he is one of the oldest living African Americans with Maple Syrup Urine Disease.

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NBS-MSUD Connect: Your One-Stop Shop For MSUD Resources

NBS-MSUD Connect was launched as part of the Newborn Screening Connect patient registry (NBS Connect) in 2013 through a partnership between the Department of Human Genetics at Emory University, the Maple Syrup Urine Disease (MSUD) Family Support Group and other key stakeholders.

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Cambrooke Therapeutics

Cambrooke Therapeutics continues to expand its line of delicious and nutritious low protein foods to help improve the lives of individuals with Inborn Errors of Metabolism such as MSUD.

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Updates To Nutrition Management Guidelines

The Nutrition Management Guideline for MSUD was first published in 2014. Since that time, there have been reports of new research and experiences that have prompted updates of the guideline.

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Emory Metabolic Camp 2018 Announcement

Join us June 18-23, 2018 for the 24th Annual Metabolic Camp at Emory University in Atlanta, GA!

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From The Chairman’s Desk

As I sit here reflecting on how much the care and treatment of MSUD has changed over the years, my mind goes back to 1978 when our son Keith was born.

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MSUD Advocacy Report

Medical Nutrition Equity Act

The Medical Nutrition Equity Act (MNEA) would require all private insurance plans (state regulated or self-insured/self-funded) and federal health programs, including Children’s Health Insurance Program, Tricare, Medicaid, Medicare, and Federal Employee Health Benefit Plans, to provide coverage for formula and low-protein foods for all children and adults with MSUD.

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The MSUD Family Support Group Is Excited To Announce Their Participation In The Million Dollar Bike Ride

The MSUD Family Support Group is excited to announce their participation in The Million Dollar Bike Ride

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19th Biennial MSUD Symposium

I can’t believe that almost two years have passed and it is time for another MSUD Symposium! I am especially excited about this conference because I’ve built in extra time for social interaction.

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A Child's Life

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