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Tag Archives: PKU

PKU Diet Adherence: Important, but not always easy

Many people hit a time in their life when their metabolic diet management may be… less than optimal.

It’s what you do next that is the key. Not losing hope, not saying “I’ll never be able to return to diet” is part of making a successful change. Keeping a positive outlook and setting small goals to get your PKU or other metabolic diet back on track is important to success.

For example, Katie, a second-grade teacher who recently became a mother, is committed to following her PKU diet.

However, it was not always that way for her.  As a teen, she was concerned about fitting in and that meant eating foods that were not part of a low protein diet.  She quickly found herself going off-diet. She knew that keeping her PHE levels in check was important to her future dreams of going to college.

Katie admitted that returning to diet and having to drink PKU formula again was something she was just not ready for but wanted to get her PHE levels in control once more.  She contacted her metabolic clinic and opted to begin her return to diet with PKU formula in a tablet form.  Tablets are discreet and can be taken anywhere.

Once she was ready, Katie returned to drinking formula and eating low pro. She was even featured in a low protein cooking video series sponsored by Nutricia.

Hear Katie’s Story

So, if you are looking to return to diet or find a new PKU formula, Nutricia is here to help.  We provide more than just formula and can help connect you with a local clinic and take that first step toward returning or improving your metabolic diet.

 

Contact us today!

DietforLife@Nutricia.com

 

 

Next Blog:   10 Reasons to Try Phlexy-10 Tablets

 

© 2019 Nutricia North America

Nutricia is a manufacturer of medical foods for the dietary management of inborn errors of metabolic.  All products must be used under medical supervision.

What is GMP?

You’ve likely seen ‘GMP’ on a Phenyketonuria (PKU) or Tyrosinemia formula label or heard about it through your dietitian. But what exactly is it and why is it getting so popular?GMP can be used as a protein source for PKU and Tyrosinemia formulas. GMP, short for glycomacropeptide, is a peptide – a type of short protein. Peptides typically consist of shorter chains of amino acids, whereas true proteins are larger and more complex structures.

GMP is a byproduct of the cheesemaking process. Cheese is made from milk and contains intact protein. Milk consists of two types of proteins: casein and whey. During the cheese making process, the whey is separated from the casein; the casein part is what is used for cheese, the whey part is used for other purposes. GMP is found in the whey portion and must be filtered out to be used as an ingredient in formulas.

Contrary to other proteins, pure GMP does not contain phenylalanine (PHE), tyrosine (TYR) and a couple of other amino acids. That’s why it is a suitable protein source for PKU and Tyrosinemia formulas. However, through the process of isolating GMP from the whey part of the milk, some very small amounts of PHE and TYR cannot be filtered from the actual GMP and are part of the GMP ingredient used in PKU and Tyrosinemia formulas.

GMP by itself lacks certain amino acids that are important for growth and development. These amino acids must be added to the formulas so that PKU and TYR formulas are appropriate for individuals with PKU or Tyrosinemia. That’s why you see GMP and free amino acids listed as ingredients on your PKU and Tyrosinemia formula label.

You may have noticed slight variations in the PHE content when comparing GMP formulas (for example, between 10‐18 mg PHE per 10 g protein equivalent). Healthcare professionals have told us, that the differences are so small that they are clinically not relevant, and studies support this. This means that if a GMP‐based product is appropriate for someone with PKU or Tyrosinemia, the slight differences in PHE content are not important.

GMP is still a growing concept, but several studies support that GMP‐based products are safe and appropriate for most individuals with PKU or Tyrosinemia. GMP‐based products have a milder taste, and that milder taste is often preferred over amino acid‐based products. Finding a formula you or your child like is very important as that can certainly help with diet adherence. In addition, a small number of studies also report that patients feel fuller when consuming a GMP‐based formula.

A small number of clinical studies are also looking into other benefits of GMP‐based products in metabolics. However, the results of these studies are inconclusive. More research is needed to provide definite answers.

For some people with PKU or Tyrosinemia, the amount of PHE in GMP‐based formulas may be too high. Rest assured that formulas that are based 100% on amino acids (with no GMP) provide you or your child with all the nutrients needed for proper growth and development, including important vitamins and minerals for bone health. But of course, your dietitian can advise you on which formula is best for you or your child.

Want to find a GMP‐based formula? You’ve come to the right place!

Check out the variety of GMP‐based formulas that Nutricia has to offer: click on a product above  and read our blog on GMP‐based products from Nutricia.  READ NOW

Always talk to your dietitian before making any diet changes – and contact us if you have any questions about metabolic formulas. Visit www.MedicalFood.com for more information.

Written by: Ulrike Reichert, MS

Director Medical Affairs Keto/Metabolics Nutricia North America

 

 

PhenylAde GMP READY, PhenylAde GMP Drink Mix, and PhenylAde GMP Mix-In are medical foods for the dietary managment of proven Phenylketonuria (PKU) and must be used under medical supervision.  TYR Lophlex LQ is a medical food for the dietary management of proven Tyrosinemia  and must be used under medical supervision. 

 

Nutricia offers variety and choice in GMP‐based products:

At Nutricia, we are dedicated to providing more choices to more people. We have recently added new GMP‐based products to our portfolio. To read more about what GMP is, click HERE.

For those of you who want an overview of what is available in GMP‐based products from Nutricia, we made this quick reference guide. Get inspired to find your favorite one!

GMP‐based products for Phenylketonuria

(click the product box to learn more)

GMP‐based products for Tyrosinemia

Get READY for a Difference You Can Taste! No Mixing! GMP‐based powder to create the “Ultimate PKU Protein Shake” Virtually tasteless and odorless to mix in beverages or semi‐solid food Mild taste to mix in beverages or semi‐solid food

 

Always talk to your dietitian before making any diet changes – and contact us if you have any questions about metabolic formulas. Visit MedicalFood.com for more information.

Your Nutricia Metabolics Team

 

 

 

 

 

PhenylAde GMP READY, PhenylAde GMP Drink Mix, and PhenylAde GMP Mix-In are medical foods for the dietary managment of proven Phenylketonuria (PKU) and must be used under medical supervision.  TYR Lophlex LQ is a medical food for the dietary management of proven Tyrosinemia  and must be used under medical supervision. 

 

Are you READY?

Are you READY?

New at Nutricia is PhenylAde® GMP READY, a ready‐to‐ drink PKU formula, providing 10 g protein equivalent and 110 kcal in one drink box (8.5 fl oz). It is also the only GMP‐ based formula with both DHA and EPA, two important omega‐3 fatty acids missing in the PKU diet.

PhenylAde GMP READY:

  • Offers an enjoyable, neutral flavor with a smooth texture for easy drinking

  • Contains GMP, which is made from an intact protein source and is naturally low in phenylalanine.   Click HERE to learn more about GMP.

  • Provides an excellent source of both calcium and vitamin D, two nutrients that help build and maintain strong bones and teeth

A ready‐to‐drink formula can make your life so much easier: from grabbing it in the morning for your breakfast drink to throwing it in the lunch bag for school or work or when you travel. We all have busy lives, and PhenylAde GMP READY is here to provide great nutrition in a convenient drink box.

Take the taste challenge as some of your fellow PKUers have done and let us know your thoughts!

GET READY TODAY!

Always talk to your dietitian before making any diet changes – and contact us if you have any questions about metabolic formulas. Visit MedicalFood.com for more information.

Your Nutricia Metabolics Team

 

 

 

PhenylAde GMP READY is a medical food for the dietary management of proven Phenylketonuria (PKU) and must be used under medical supervision.

Loprofin Pasta and the delicious taste of Italy

Ever wonder where Loprofin Pasta is made?

Meet Lorenzo Mennucci. The Mennucci family began making Loprofin Pasta almost 50 years ago in Tuscany. It is now in its fifth generation and is managed by Lorenzo Mennucci. In 1877, Giuseppe Mennucci started making pasta in a small grocery store in the countryside of Tuscany. At that time, the process of making and drying the pasta was carried out in the courtyard under the sun. This all nearly ended during the Second World War, when the company was bombed and partially destroyed. After the bombing, the Mennucci family was quick to rebuild the factory. During the rebuilding process, all pasta machines that were not destroyed were moved into local homes near Tuscany to maintain the production of pasta.

The Mennucci family are very passionate about using the highest quality of ingredients which are mainly sourced from Italy. There are only four ingredients in low protein pasta which are mainly starches; corn, potato, rice and coloring (annatto). Lorenzo believes that when there are so few ingredients in a product it is important that you use the highest quality of ingredients.

The main difference in the production of low protein pasta and ordinary pasta is that gluten is the protein that binds the dough in ordinary pasta. For low protein pasta, steam is used instead to bind the starches together to make a dough. Low Protein Pasta is made using spring water that comes from the Tuscan mountains.

See for yourself where our Loprofin Pastas are made in this video  of the Mennucci family!

View recipes made with Loprofin Low Protein Pasta at MedicalFood.com/Recipes 

10 Low Protein Lunch Tips for Back-to-School

Back to School: Attention Moms & Dads

Written in collaboration with Katie Maguire, PKU Adult and 2nd grade school teacher

I don’t know about you, but it was stressful for my parents sending me off to school in September knowing that I would be making many food choices on my own without them present.

ALL children should be able to make healthy food choices. Children with PKU, MSUD, TYR or other metabolic disorders are no different.

Learning healthy choices can begin at a young age when you grocery shop and prep food in the kitchen together.

10 Low Protein Lunch Tips for Back-to-School

 

  • Pack lunch in fun, creative containers in all different sizes.

  • Use an insulated lunch tote with ice packs to keep food and formula cool.

  • Make lunch colorful with a small amount of different veggies, such as baby carrots, cherry tomatoes, cucumbers, yellow peppers, etc.

  • Prepare, weigh and measure food ahead of time to speed up mornings.

  • Give your child a low protein “surprise” snack like homemade cookies or a cupcake.

  • Write a special ‘good job’ note or include a sticker 2-3 times a week.

  • Send formula in a fun re-usable drink container or sports bottle.

  • Plan ahead with a 5-day menu to ease grocery shopping and food prep.

  • Try a ready-to-drink formula if unable to mix formula at school.

  • Involve your child in shopping, prepping and weighing their own lunch.


Ask your clinic about Ready-to-Drink Formula for school children

 

Periflex® LQ is a great tasting, ready-to-drink   that provides 15 g of protein equivalents with 0 mg of PHE.  (For PKU Only). Great for school lunch!

 


Nutricia also offers the Lophlex® LQ line of Ready-to-Drink formula options for Maple Syrup Urine Disease (MSUD), Tyrosinemia (TYR), Homocystinuria (HCU) and Phenylketonuria (PKU).

 

 

Lophlex LQ is a low volume, quick and easy way to get 20 g of protein equivalents.

As always, check with your dietitian if these products are appropriate for your child prior to making any changes to their diet.

Ask your clinic about Periflex LQ or Lophlex LQ Today!

 


Periflex LQ is a medical food for proven Phenylketonuria (PKU). Lophlex LQ is a medical food for proven Maple Syrup Urine Disease (MSUD), Tyrosinemia (TYR), Homocystinuria (HCU) or Phenylketonuria (PKU). Must be used under medical supervision. Please consult a metabolichealthcare professional prior to making any changes in your child’s diet.

©2018 Nutricia North America

Help your child’s teacher better understand PKU

It’s Back-to-School Time! 

The return to school is an exciting time of year.  But for a parent of a child with a metabolic disorder, such as Phenylketonuria (PKU), there are also certain concerns and stresses that comes with the start of school. 

Each year you may be faced with educating a new teacher or school nurse about PKU.

What is PKU?  

How it is Treated?  

What that means to the teacher and fellow students…

Nutricia Metabolics is proud to share an educational handout to help you inform your child’s new teacher and/or school nurse about PKU.

It includes information about What PKU is, What happens, How it is treated with additional classroom tips for the teacher.

Click to Download:  Guide to PKU – Teachers & School Nurses

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Click to Download

 

Nutricia also provides information and education available about other rare metabolic disorders such as Maple Syrup Urine Disease (MSUD), Tyrosinemia (TYR), Glutaric Aciduria Type-1 (GA-1), Homocystinuria (HCU) and more available online at medicalfood.com/connect

 

Drink It How You Like It

Turn What You Like Into PKU Formula with PhenylAde® GMP Mix-In

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6300143Virtually Tasteless and Odorless

#DrinkItHowYouLikeIt

PhenylAde GMP Mix-In is a medical food for the dietary management of proven Phenylketonuria (PKU) and must be used under medical supervision. Always consult your metabolic healthcare professional prior to making any changes in your PKU diet plan.

 

Make PKU Formula Part of Your Daily Routine

If you have Phenylketonuria (PKU), then you know….PKU formula is one of the most important parts of your diet.

Since PKU is generally managed with a diet low in whole protein, you need to get additional, PKU-appropriate protein from a different source.

PKU formula is your main source of protein and is essential for your health and well-being. Taking your full prescription of formula every day will provide your body with the protein it needs.

 

Here are some tips to help make PKU formula part of your daily routine
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Learn more about Why you need PKU Formula with these tips for Making PKU Formula Part of your Routine 

Download Now

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Create a Custom GMP-based PKU formula!

Have you wish you could turn your favorite drink to a great tasting PKU formula?

NOW YOU CAN!

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Introducing…PhenylAde® GMP Mix-In 

 

  • An easy way to add Glycomacropeptide (GMP), made from a whole protein source, to your PKU diet

  • Simply mix with just 6 fl oz of water or any flavored drink for added variety

  • Great as a once daily or as your main PKU formula*

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So many choices, how will you mix it in?

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*Must be combined with an appropriate vitamin and mineral supplement when taken as the sole source of PKU medical food.   Be sure to consult your metabolic healthcare professional prior to making any changes to your PKU diet plan.

Available is US only.

Request A Sample