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Tag Archives: What is PKU

Making the Most of Your Meal

Satisfaction from food or a meal is something everyone wants. Although many foods may be restricted or limited on a low protein diet, you do not have to compromise taste. Selecting the  foods you eat involves a variety of factors. We choose foods based on taste and smell, in addition to texture, sight and culture.

To make the most of your meal, begin by identifying what you crave, like spicy, sweet, crunchy or salty. This will make your meal more satisfying.Here are some tips to help you make the most of your meal.

  • Spicy: Add seasonings that can add a kick. For example, add cracked pepper, crushed red pepper or hot sauce to steamed vegetables to give a familiar item a new twist.

  • Sweet & Tangy:  Satisfy your sweet and tangy craving with grilled pineapple topped with BBQ sauce on a low protein bun. You can also add mandarin oranges and Italian dressing to a garden salad for added flavor and tang.

  • Crunchy: With a little creativity you can add a snap to any dish. Make your own salad iStock_000015639017Mediumcroutons with low protein bread. Season them in a variety of ways by using garlic powder, cayenne pepper or Italian herbs depending on your personal desire for savory or spice. Another great way to add crunch is to include raw vegetables, like cucumbers or carrots slices to a salad or sandwich.

  • Salty: Salt is among the most popular taste craving. Although highly desired, salt is an ingredient to be used in moderation. A little bit goes a long way. To satisfy your craving for salt, try slicing fresh potatoes very thin and sprinkling with garlic and a touch of sea salt. Bake them until crisp for a satisfying addition to your meal or snack.  

  • Smooth and Creamy: Create your own creamy low protein dip with your favorite seasonings for a flavorful way to enjoy Low Protein Crackers or fresh cut vegetables. You can also include a sweet treat at the end of your meal to help satisfy your need for something smooth and creamy.

What is PKU?

What is PKU?

PKU is short for Phenyketonuria. PKU is a genetic disorder that a child inherits from both parents. For a child to be born with PKU both parents must either be carriers of PKU or have PKU, which means they carry at least one PKU gene.

PKU occurs due to a deficiency or absence of the enzyme phenylalanine hydroxylase (PAH), which interferes with a person’s ability to metabolize or process the amino acid phenylalanine (PHE) into tyrosine, another amino acid. Amino acids, like PHE, are the building blocks of protein found in food and the human body. Consuming too much PHE from food can lead to a toxic build-up of PHE in the blood and brain of a person with PKU. Without treatment, this buildup of PHE will cause irreversible brain damage.

Presently, there is no cure for PKU, but it can be managed effectively with proper diet and special nutritional formulas designed for persons with PKU.  View products.

Incidence of PKUAutosomalRecessiveInheritance

In order for an individual to have PKU, both parents must pass along their PKU gene to their child. When both parents are carriers of PKU, there is 1 in 4 chance with each pregnancy that the child will have PKU, 1 in 2 chance of the child being a carrier, and 1 in 4 chance that the child will neither have PKU nor be a carrier.

It is estimated that one out of 10,000 live births has decreased PAH activity(1). Autosomal Recessive Inheritance Normal Gene Carrier Non Carrier Carrier Carrier PKU Carrier PKU Gene Phenylketonuria (PKU) *Phenotype- observable traits, outward appearances, development or behavior.

Newborn Screening for PKU

Newborn screening for PKU began in the US and Canada in the 1960s. It is important that an infant is diagnosed and managed with diet within days after birth to prevent permanent negative effects from PKU.

Nutricia is proud to offer the largest variety of great tasting formula options for people with PKU.  

pku-by-brand

 

 

 

Learn more online at MedicalFood.com or contact us at (800) 605-0410 for free product samples.

Your metabolic dietitian will help you decide which products are right for you.  PKU formula must be used under medical supervision.

Nutricia is a proud supporter of PKU Awareness Month and the National PKU Alliance

Nutricia is a proud supporter of PKU Awareness Month and the National PKU Alliance

References
1. Vockley J, Andersson HC, Antshel KM, et al. Phenylalanine hydroxylase deficiency: diagnosis and management guideline. Genet Med. 2014;16(2):188-200.

What is PKU?

What is PKU?

If you have PKU or your child with phenylketonuria is older, you may think this is a silly question, but even though you are living with and managing this metabolic condition on a daily basis, you may not completely understand what PKU is or be able to explain it to others.

There are some terms that you may or may not be familiar with. Learning these key terms will help you better understand or better explain why you follow a special low protein diet.

Art: In “medical terms’ Phenyketonuria (PKU) is an autosomal recessive genetic disorder that results in incomplete phenylalanine metabolism.

But in “REAL TERMS” what does this mean?

Let’s break it down:
Autosomal: a chromosome other than an X or Y sex chromosome
Recessive: a trait that appears only when a gene has been inherited from both parents
Genetic: hereditary characteristic that you get from your parents
Disorder: an abnormal condition
Phenylalanine: an essential amino acid (must be consumed, the body doesn’t make it) commonly referred to as “PHE”
Metabolism: the process in which your body breaks down particular substances

So in REAL TERMS:
PKU is an inherited genetic disorder that prevents the full breakdown of phenylalaine.

Did you know: (image) When phenylalanine builds up in the blood it is excreted in the urine as phenyketones. That is how this condition became known as PhenyKetonUria or PKU for short.


Posted by: Sandy Simons, MA, RD, CHES

Sandy is a Registered Dietitian and Certified Health Education Specialist. She received her graduate training at Columbia University’s Teacher College in New York. She has been working on the industry side of metabolic nutrition for the past 11 years and is often seen at patient events around the country. This post is also found in a book written by Sandy with the help of others titled: My PKU Toolkit: A Transition Guide to Adult PKU Management.