Food Neophobia in Children with PKU Can Complicate Nutritional Status

Phenylketonuria is an uncommon but challenging genetic disorder which requires an extremely restrictive diet as part of its treatment plan. However, the issue can be even further complicated by the fact that children with PKU have a tendency towards behaviors like food neophobia, which even in absence of PKU can lead to poor food choices and compromised nutritional status. PKU is a genetic condition which occurs in 1 out of every 10,000 to 15,000 births; although it is rare, however, it can have devastating consequences for both babies and their families. Children born with this disease lack the ability to break down and digest an essential amino acid called phenylanine, which is found naturally in protein-containing foods as well as artificial sweeteners like aspartame. Even though it is a rare disorder, it is part of routine screening for newbo s, because if PKU babies are breastfed, exposure to the proteins in breast milk can result in seizures, tremors and permanent nervous system damage and mental retardation. But treatment can be difficult as well: lifelong adherence to a low-phenylanine diet. A low-phenylanine diet, however, is quite restrictive. In order to be compliant, patients must eat carefully measured portions of fruits, vegetables, and special breads, pastas and other grain products which are made using low-protein techniques. And because this diet does not provide all the nutrients needed for healthy growth and development, often a special supplement like Phenyl-Free, which contains vitamins, minerals and low-phenylanine proteins that are appropriate for a PKU diet. Thus, parents raising children with PKU face significant nutritional challenges to begin with. However, this issue can be exacerbated by the fact that, according to a 2015 study which looked at food acceptance in children with PKU showed a tendency towards food neophobia, or a fear or rejection of new foods. In the study, a group of children (half of whom had been diagnosed with PKU), were given a blindfolded taste test of a variety of pureed foods. While both groups of children showed a preference for sweet foods over ones that were bitter, sour or savory, the PKU group showed a significantly higher tendency towards food neophobia, or lack of acceptance of new kinds of foods. Food neophobia in and of itself is not considered to be abnormal behavior, and researchers on this issue note that it is usually part of a toddler's development and helps to protect them from potentially toxic or dangerous elements as they begin to explore the world around them. The behavior peaks between the ages of 2 and 6 but this study notes that there is a link between this phenomenon and poor food choices -- as well as being underweight or obese. This tendency, combined with the restrictions already inherent in PKU treatment, can add an extra layer of complexity to management of this disease. Authors of the neophobia study recommend that, in order to help combat this phenomenon that parents begin early to introduce their children to as wide a variety of foods as their diet will allow -- and to do this as soon the baby begins to be introduced to solid foods. They also recommend that doctors and nurses learn more about this issue in order to educate parents on overcoming this obstacle. In short, food neophobia can make the restrictive dietary management of PKU even more of a challenge. However, beginning early in a child's life to introduce them to a good variety of healthy foods can help ease this problem and enhance the status of a group of children who are already more vulnerable to problems of malnutrition.