Guide to PKU - Mead Johnson Nutrition

careful not to center their attentionon the child with PKU, particularlyat mealtimes. Older brothers andsisters may try to “mother”the child with PKU, and siblingsmay even taunt the child withfoods he cannot eat. Parents willhave to make it very clear thatsuch behavior is not acceptable.What is the PKU diet?It is a diet low in phenylalanine,which is the amino acid that thechild’s body cannot use properly.The specific diet for your child willbe specially designed by your metabolicphysician and nutritionist.The diet is designed to meet yourchild’s individual needs for growthand development. The dietincludes a protein substitute, suchas Phenyl-Free ® 1 or Phenyl-Free 2dietary powder, which provides themissing protein but has little or nophenylalanine. In addition to theseproducts, patients are given a prescribedspecific amount of phenylalaninefrom breast milk, regularinfant formula, or solid foods toprovide enough phenylalanine forgrowth. Measured amounts offruits, vegetables, cereals, lowproteinbreads, pasta, and specialdesserts are added to the diet as thechild gets older.Is the diet expensive?The formula costs more thanregular infant formula because itis specially designed for medicalpurposes. However, because of theimportance of formula for yourchild’s mental and physical development,most health insuranceplans pay for it. In some states, thespecial medical formula is providedby the State Department of Health.In other states, these products areprovided on a sliding fee scale,based on ability to pay. The socialworker, nutritionist, or nurse atthe PKU clinic can usually helpidentify a payment method.Can I breastfeed mybaby with PKU?Yes. Breast milk is lower inphenylalanine than either infantformulas or cow’s milk, and it canprovide the supplemental amountsof phenylalanine required for yourbaby’s growth when used with aspecial phenylalanine-free formulasuch as Phenyl-Free 1. Many newbornbabies with PKU can be fedwith breast milk and phenylalanine-freeformulas and still keeptheir blood levels in an acceptablerange. All babies with PKU musthave their diet intake and bloodphenylalanine levels monitoredclosely. You’ll want to discuss yourdecision to breastfeed your babywith PKU with your baby’sdoctor and dietitian.Why are diet diaries kept?Diet diaries are records of foodintake which serve as guides for theclinic team when adjusting yourchild’s diet. These diaries are anecessary management tool. It isthe only way for the nutritionist toknow exactly what your child hasbeen eating or drinking so thatappropriate changes can be madeto assure the proper levels ofblood phenylalanine.How long will my child withPKU be on the diet?Medical experts state thatmaintaining the restricted dietthroughout life is preferable. Datafrom the National CollaborativeStudy of Children Treated for PKUshow that intellectual ability andacademic achievement in schoolare negatively affected when bloodlevels are persistently high beforethe age of six years. Other studieshave shown that children whomaintained acceptable levels ofblood phenylalanine through age10 had higher IQ scores thanchildren who didn’t have low Phelevels. A “diet for life” approach iscurrently recommended.17