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PKU Newsletter

Boston Children’s Hospital | Metabolism Clinic PKU News | For adults with PKU
October 2012 | childrenshospital.org/metabolism

Greetings from Boston Children’s Hospital Metabolism Clinic –

Our clinic continues to remain at the cutting edge of PKU treatment and research. We would like to let all adults with PKU know about some of the new developments in treatment for PKU and about opportunities to participate in research.

New Treatments for PKU

New and better tasting formulas and medical foods

  • Sports drinks, coolers and juice boxes providing the special PKU protein medical food (formula)
  • Glycomacropeptide (GMP) products (drinks, bars, puddings) that are based on a new type of whole protein that is low in phe
  • Large Neutral Amino Acids (LNAAs) that prevent the uptake of phe in the brain

Check out the following companies for more information on these new products:

Cambrooke Foods – cambrookefoods.com
Vitaflo USA – vitaflousa.com
Applied Nutrition – medicalfood.com
Nutricia North America – nutricia-na.com

Medication for PKU

Sapropterin dihydrochloride (BH4/Kuvan™)
Visit kuvan.com for more information

If you are interested in scheduling a clinic appointment to see if any of these new products or medications are right for you please contact Vera Anastasoaie:
Phone: 617-355-7345
Email: vera.anastasoaie@childrens.harvard.edu

Current Research Project

We want to see the differences between the normal PKU diet and a diet using new GMP products. This new study is called: Phase 2 crossover trial of glycomacropeptide (GMP) versus amino acid diet for management of PKU.

To learn more about our projects, contact Vera Anastasoaie:
Phone: 617 355-7345
Email at vera.anastasoaie@childrens.harvard.edu

Face Forward Summer 2012

The Face Forward Project stormed Ashland, Massachusetts on July 8th, 2012. For the five days 19 youth learned, laughed, danced, took risks and built a community designed to support one another. Face forward was launched in 2011 in collaboration with Next Step, an exciting transition program for adults with special health care needs. The week-long event was packed with workshops, discussions, and activities designed to push the participants to learn something new and grow from the experience. All of the people that participated reported having a wonderful time and most plan on returning next year. Since the Face Forward conference was such a success, it has taken the place of Boston Children’s Hospital’s weekend program, Teen Challenge. If you or someone you know is interested in learning more about Face Forward please visit the website at for more information!

http://www.nextstepnet.org/our-programs/face-forward

Or contact any of us at the Metabolism Clinic at Boston Children’s Hospital, 617-355-7346

Gerard Berry, MD, Director
Leah Hecht, RN, CPNP, MEd, Nurse Practitioner
Susan Waisbren, PhD, Psychologist
Harvey Levy, MD
David Harris, MD,
Philip James, MD, MPH
Edward Neilan, MD, PhD
Stephanie Newton, MA, Genetic Counselor
Frances Rohr & Ann Wessel Costas, Dieticians
Sheryl Hilliard, MSW, Social Worker

Stay Tuned!

  • Clinical trials are beginning for PEG-PAL. This trial involves injections of an enzyme that works on an alternative pathway for breaking down phenylalanine. If successful, this treatment would be the first enzyme replacement therapy for PKU and might permit people with PKU to eat a normal protein diet while maintaining blood phe levels in a safe range.
  • Neuroimaging (MRI) to visualize levels of phenylalanine, tyrosine and dopamine in the brain and associate these levels with neuropsychological functioning and EEG findings.
  • Results about depression, anxiety and emotional issues in PKU
  • Results from our long-term follow-up study of children born to mothers with PKU