An excess accumulation of the amino acid phenylalanine in the blood can cause a genetically inherited birth defect known as phenylketonuria or PKU. The accumulation occurs due to the absence/deficiency of the enzyme responsible for converting one amino acid (phenylalanine) to another amino acid (tyrosine). Phenylalanine that accumulates in the blood proves to be toxic to brain tissues.
Infants Born with PKU
Screening for blood test shows the presence of PKU and treatment should be started immediately to avoid adverse side effects such as declining intelligence, neurological deterioration, stunted growth, hyperactivity and seizures. The treatment team consists of a metabolic dietitian, physician, geneticist and psychologist to monitor the different phases of growth and development of an infant from childhood into adulthood.
Imposing a strict diet limited in phenylalanine is the primary treatment option for PKU. Phenylalanine is present predominantly in protein-containing foods such as eggs, nuts, milk, chicken, fish, beans, beef, pork, soybeans and cheese. Infants with PKU must solely be fed with formula that is phenylalanine-free for the first year of life. Breast milk can be fed to the infant sometimes in limited quantities.
Creating Unique Meal Plans
Once your infant grows and becomes ready for solids, a registered dietitian nutritionist with expert knowledge in PKU will create a customized diet plan for the kid that fulfills calorie, nutrient, vitamin and mineral needs. As many foods are restricted for a person with PKU, the meal plan would definitely include special formulas that are often the primary source of calories and essential nutrients. Even special phenylalanine-free foods are available for children and teens with PKU. The metabolic dietitian would alter the meal plan as the child grows, to fulfill all the nutrient requirements.
The special diet needs to be followed throughout the life of the person to maintain serum phenylalanine levels which can be checked through blood tests. High serum phenylalanine levels can cause lack of concentration, shortened attention span and memory difficulties. Along with blood monitoring, the dietitian will also keep an eye on diet records and growth charts.
Parents of children with PKU need to use their creativity to cook food and seek the help of an RDN to plan their meals. Such parents should also plan ahead for eating out, school lunches, potlucks, trips and vacations and other special events. Try to make activities and other events a priority in your child’s life over food. Also, involve your child in managing his/her diet from the minimal age possible.
If you are looking out for a registered dietitian nutritionist to plan a phenylalanine-free diet for your child with PKU, please visit the website www.firsteatright.com.