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Tag Archives: tyrosinemia

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. 

 

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 

New GMP-based option for Tyrosinemia!

TYR Lophlex GMP Mix-In is an unflavored product low in phenylalanine and tyrosine with glycomacropeptide (GMP). It is indicated for the dietary management of proven Tyrosinemia in individuals over 1 year of age.

TYR Lophlex® GMP Mix-In is your protein on-the-go:

  • Mixes easily with flavored drinks and blends easily into foods to add variety

  • Low calorie option with only 40 calories per 10 g sachet

  • Less than 0.5 g of sugar per pouch

  • Tastes great with no artificial flavors or colors

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

What is GMP?

Glycomacropeptide, or GMP for short, is derived from a whole protein source. It is naturally low in phenylalanine and tyrosine, and with the addition of certain amino acids, it makes a suitable source of protein for those with Tyrosinemia. Your dietitian will help determine how much to take.

What makes TYR Lophlex GMP Mix-In special?

TYR Lophlex GMP Mix-In tastes great and gives you the flexibility to boost your protein!

  • You can easily mix into foods, drinks or your current TYR formula

  • Each 10 g sachet is only 40 calories

Talk to your dietitian today and request a sample.

TYR Lophlex® GMP Mix-In is a medical food for the dietary management of proven Tyrosinemia and must be used under medical supervision.

©2019 Nutricia North America.

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

Metabolic Disorders – Talking to your young child

What to say to your child

While your child is not old enough to manage their metabolic disorder alone, it is valuable for them to begin to better understand their diet and treatment. When speaking with your child about their metabolic disorder, the following tips may be helpful:

  • Use simple examples to explain ideas
    For example, when explaining to your child why their metabolic diet is important, it may be helpful to relate the diet to that of a food allergy. Speak with your metabolic team who will also have information about books to read to your child to help him or her understand special diets.
  • Let your child know they can say “no”
    If you haven’t already, teach your child to ask you before eating unfamiliar foods, and that it is OK to say no to anyone who offers food that is unfamiliar or off limits.
  • Tell your child it isn’t his or her fault
    A child may not understand why he or she has metabolic disorder when others do not, and may think that he or she did something to ‘deserve’ it. Explain to your child that everyone is born with different qualities, such as hair and eye color, and a metabolic disorder is something that people are born with, not something that anyone causes. Reinforce to your child that he or she is special, and that this special way of eating is to keep him or her healthy.
  • Stay positive.
    Sending the right message about foods and treatment is important. It is better to talk about off-limit foods as “high-protein,” “no,” “red” or “stop” foods rather than “bad” or “naughty” foods. Help your child accept and manage their metabolic disorder as he or she grows. Never say anything negative about the food or formula to your child. This special way of eating is to keep him or her healthy.
  • You’re not alone
    Talk to your child about other people you know who are on a special diet, even if they are adults, so your child knows that he or she is not the only one on a special diet.   Get involved!   Go to a local metabolic or National event.

Click here to view upcoming events and metabolic support groups across the nation.

 

*Reference Source:Adapted from – My PKU Binder. National PKU Alliance. Chapter 5: Ages 3 to 6 Years, Page 41.