It is absolutely and unequivocally clear :
“The PKU diet must include a medical product, usually consumed as a beverage. There are several brands available that are nutritionally suitable for adults. Without the PHE-free medical product, the diet is nutritionally inadequate.” –
Diet Intervention Guidelines For Adults With Untreated PKU
By Barbara E. Dolan, RN, MSN, Nurse Counselor for Genetics, Redwood Coast Regional Center, Ukiah, CA
Richard Koch, MD, Division of Medical Genetics, Children’s Hospital of Los Angeles and The Department of Pediatrics, University of Southern California, School of Medicine, Los Angeles, CA
Christina Bekins, MS, RD
Nutrition Consultant, Calistoga, CA
We KNOW that individuals born with an inborn error of metabolism will suffer serious consequences to their brain which can end in institutionalization. We KNOW they need to keep levels of PHE low in their diets and that they need all the other ESSENTIAL amino acids too, that is why they are called essential.
So how can an adult patient get a letter from a Federal Gov insurance plan that says the medical product is “experimental” in adults over age 22? Phenylketonuria (PKU) is a genetic defect- one cannot change an inborn error of metabolism by getting older.
It is always there (short of the development of gene therapy) and the consequences can be devastating: elevated PHE levels in the brain are neurotoxic.
So who is the insurance reviewer and what are their credentials to contradict volumes of medical literature on the topic?
Insurers want and need to keep costs down, and the producers of medical foods do not help, when they lobby to make everything they can think of a medical food, but lifetime provision of a medical, essential amnio acid rich formula keeps adults with PKU able to work and be contributing members of society.