Scroll to top

Tag Archives: pku formula

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!

mix-in-new

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*

    GMP_SAMPLE_button

So many choices, how will you mix it in?

mix-in-nutrition

*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

College & PKU: Getting Ready for the Next Chapter

PKU-Teen College Image
The completion of high school and the transition into the next chapter of education is a very exciting time. Applying to colleges, receiving the ‘fat envelope’, choosing classes, moving away from home, and living with a roommate for the first time, are all new and exciting experiences.

Arriving on campus and the first few weeks on campus will be exciting and full of change.  You will meet a variety of new people and have many new experiences.  The one thing that remains constant is adhering to your low protein diet and drinking your PKU formula daily.  Keeping your PHE levels in control when you don’t have your family watching over you may be more difficult than anticipated, but at the same time, more important than ever.

The key is to FOCUS and make a PLAN!  Commit yourself to your PKU diet.  Long lectures, late nights studying and preparing for exams will require you to be at your best and drinking your PKU formula daily, spread throughout the day will be one of your tickets to success.  As you know, elevated blood PHE levels are linked to reduced executive function skills such as memory, recall and concentration.

Write out your daily schedule and include dedicated time to mix and drink your PKU formula.  This will help make formula part of your day and reduce accidental skips.

Tips for Drinking PKU Formula on Campus

  • Add formula powder to an empty water bottle (or other reusable container) that you can fill with water later for formula anywhere, anytime.
  • Is your dorm far from campus? Rent a locker at the school gym or student union to keep your formula so you can refuel in between classes and don’t have to go all the way back to your room.
  • Choose a formula that is sold in single-serving, travel  friendly packaging.  Powders in little pouches can easily be added to store-bought beverages while on campus or out with friends.

Examples include:

Periflex® LQ  is a ready-to-drink formula that provides 15g PE in a highly portable and easy-to-drink container for long days on campus.Back-to-School with Periflex LQ - TEENS

 

 

 

PKU Lophlex® LQ is a ready-to-drink formula that provides 20g PE in only 4.2 fl oz.  Quick and easy to drink down and get back to the book.

PKU_Lophex_LQ_Group_2016

 

 

PhenylAde 60™  is a low volume formula that mixes with only 3 fl oz of water for 10g of PE or any protein-free beverage you enjoy.

Great for Adults & Teens. Try it Today!

Check out these simple on campus mixing ideas:

PhenylAde™ MTE Amino Acid Blend is a unique formula that mixes with anyPhenylAde MTE Amino Acid Blend for PKU protein-free beverage you enjoy, such as lemonade, sports drink or fruit punch.

 

 

Talk with your metabolic dietitian or clinic to find out which PKU formula from Nutricia may be right for you.

To learn more or REQUEST A FREE SAMPLE of a Campus Friendly PKU Formula visit MedicalFood.com today!

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.

PhenylAde™ GMP from Nutricia

A Whole New Way to Think About PKU Nutriton

PhenylAde™ GMP contains Glycomacropeptide (GMP), made from a whole protein source. It has a smooth, mild vanilla flavor AND is a GMP based PKU product with both DHA & Fiber.

 

PhenylAde GMP

CLICK HERE TO REQUEST A SAMPLE 

Talk to your metabolic dietitian or clinic about a PhenylAde GMP today!

DID YOU KNOW…

Nutricia has a team of Reimbursement Care Specialists to assist you in making a change to PhenylAde GMP or any other Nutricia product.  They can verify insurance benefits, help you find a supplier and more.

CLICK HERE TO LEARN MORE ABOUT OUR SERVICES

*This product is for use under medical supervision.  Please consult your metabolic healthcare professional prior to making any changes to your PKU diet plan.

 

Flying with Formula – Tips for your Next Trip

Flying with Formula – Tips for your Next Trip

airplane girl image

If you are planning a flying vacation, before you book, contact your airline’s customer service department to notify them of your travel requirements as soon as you can.  Airline policies regarding travelling with metabolic formula will differ so it is worth checking with a customer service representative before you arrive at the airport.

Airplane food is generally not PKU or low protein diet-friendly so you may need to pack (or purchase prior to boarding) any food or snacks you think you might need during the flight.

If you are booking an international flight you will need to order a special meal. Please note that even meals listed as low-protein or vegetarian/vegan may not be low enough in protein for the PKU diet, so explain your dietary requirements clearly.

Airline Tips

  • Always take a travel letter from your clinic explaining your medical condition, especially for international travel

  • Always pack extra PKU or metabolic formula in your carry-on bag in case of delays

  • Do not mix PKU or metabolic formula powders with liquid until you go past the security screening checkpoint

  • Keep your PKU or metabolic formula in its original sealed containers (packages, cans or sachets)

  • Take a copy of your child’s diet prescription with you

  • Take plenty of snacks for the flight

Useful PKU Traveler’s Tip

  • It may be useful to switch to a powdered PKU or metabolic formula when travelling abroad, to reduce your overall luggage weight. However, ready-to-drink pouches that don’t require mixing are also particularly convenient when traveling. Contact your dietitian for more information on these options

  • If you are traveling overseas, ask your metabolic healthcare professional team for information on where low-protein food supplies can be obtained in your destination country

  • If you are shipping your PKU or metabolic formula or food to a hotel prior to your arrival, be sure to label the box clearly with your name and arrival date on the package. Call and alert the hotel that a shipment will be arriving for you.

*Reference Source: My PKU Binder. National PKU Alliance. Chapter 11: Traveling, Page 88-89.

PKU Periflex Junior Plus – Tips for Transition

Nutricia recently launched PKU Periflex® Junior Plus, a new addition to the Periflex family, with the goal of providing the best in PKU nutrition to your child. Towards this goal they have improved Periflex® Junior based on latest medical and scientific knowledge.

PKU Periflex Junior Plus - PKU Formula for Children

Available in Berry, Orange, Plain and Vanilla

Learn more about PKU Periflex Junior Plus

PKU Periflex Junior Plus has been improved to now provide DHA and Fiber for your child

  • DHA is an omega-3 fatty acid found to be lacking in the typical PKU diet
  • Nutricia’s own patented Multi-Fiberblend was added to help support your child’s overall gut health.

DID YOU KNOW IT CAN TAKE SEVERAL ATTEMPTS FOR A CHILD TO GET USED TO A NEW FOOD OR PKU FORMULA?

For some kids, a change in formula can be challenging. Some kids may need to try new foods/formula many times before acquiring a taste for it. So please be patient if that is the case with your little one.

To help we have created the PKU Periflex Junior Plus Activity Book.

PKU Formula Activity Book

Help your child transition to PKU Periflex Junior Plus with this fun, interactive activity book

Call 800-605-0410 to request a FREE Copy

Be sure to talk to your dietitian or clinic about a transition plan. They can provide a plan that will guide you step by step to make a slow and stead move to the new product by mixing  the new product and current product together to aid your child in making a successful change to PKU Periflex Junior Plus.

Request a Sample Now

Written by Linda Leuffgen, RD – Medical Affairs Associate, Nutricia North America

Amino Acid Blends

Ever wish you could just make your own formula using your favorite great tasting drink?

Well, now you can with Nutricia’s Amino Acid Blends.

This type of formula is available for PKU (PhenylAde™ Amino Acid Blend), MSUD (Complex MSD Amino Acid Blend) and GA-1 (GlutarAde™Amino Acid Blend).

Create Your Own PKU Formula with PhenylAde MTE Amino Acid Blend

PhenylAde™ MTE Amino Acid Blend for PKU

It’s simple, this style of medical food is just the amino acid (protein) part of your formula . It is virtually tasteless and ordorless. You simply MIX-IT-IN to the drink of your choice.

Seriously, it is that easy. So, yes wishes can come true!

Example:

Love drinking sports drink while you exercise or play soccer?

Why not turn that sports drink into your PKU formula and get some post workout phenyalanine-free (for PKU) protein at the same time.

Other examples:

Mix  with lemonade, iced tea, fruit punch, orange drink or anything you think it would taste good with.

There are no rules, expect you want the drink you choose to be protein-free or very, very low in in protein. You can even mix Amino Acid Blend into ready to eat foods like applesauce and tomato sauce.

Mix-It-In---Convenience-Store

Lastly, if you or your child love your current formula but have a hard time with that last serving to meet your full daily diet prescription.

Talk to your metabolic healthcare professional about adding a scoop of Amino Acid Blend to increase the medical food protein content without adding volume.

For more information about PhenylAde Amino Acid Blend (for PKU), Complex MSD Amino Acid Blend (for MSUD) or GlutarAde GA-1 Amino Acid Blend (for GA-1) visit www.Medicalfood.com or 800-605-0410 to request a sample.

 

Please note:  PhenylAde Amino Acid Blend, Complex MSD Amino Acid Blend and GlutarAde GA-1Amino Acid Blend are medical foods intened for use under medical supervision.  Please consult a metabolic healthcare professional prior to making any changes to your metabolic diet. 
PhenylAde & GlutarAde are trademarks of Nutricia North America.

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 12 years and is often seen at patient events around the country.

Why can’t I just follow a low protein diet?

The simplest answer is because you need protein.  Following a low protein diet without formula could lead to protein deficiency. You may also lack energy and develop an array of secondary health problems.

PKU formula  provides phenylalanine-free protein.  Since all natural food, with the exception of pure fat and sugar, contain some PHE, you must watch your total food intake. If you only eat foods that are low in protein you body may not get enought daily protein.  In addition, eating larger portions of foods that are ‘lower’ in protein can still add up to up more phenylalanine than you can tolerate in a day, resulting in high blood PHE levels.

Formula allows you to take in PHE-free protein and calories to help you meet your daily needs.   PKU formula can also help you feel less hungry.   Controling hunger is important becasue despite your best efforts to only eat low protein, excess hunger may lead you to consume some foods that are higher in protein or larger portions.

Don’t forget – drinking PKU formula also provides a balance of all the other amino acids (building blocks of protein) you need plus tyrosine which is an essential amino acid (needed from food) for those with PKU.

If you are currently not drinking formula and only watching what you eat, assess the reasons why you are not going ‘all in’ on your PKU diet.

  • Are you uncertain if your insurance covers formula or have you been denied in the past?
  • Do you recall from childhood hating your formula and not wanting to drink it?
  • Just stopped re-ordering for no good reason at all?

If any of these ring a bell, let Nutricia help you.

We offer a complimentary samples and a staff of trained coverage specialist that can help navigate your insurance to see if you have coverage and help find a local supplier.

Don’t delay – getting back on track and including PKU formula in your diet will be one decision you will not regret.

Request a free PKU product sample at www.medicalfood.com


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 based on an excerpt from My PKU Toolkit: A Transition Guide to Adult PKU Management.

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.

Types of Formula

Did you know that there are over 60 different PKU formula world-wide?

Whether you have been drinking the same formula and maintaining excellent dietary control or if you are reading this in a effort to learn more to return to the PKU diet plan, understanding the ‘Types’ of formulas is a great way to get started and stay in the loop of what formulas are available.

Types of PKU Medical Food

The Drink – PKU Formula in a powdered form that you mix with water. This type of medical food typically provides a balance of fat, carbohydrate, phenylalanine-free protein, vitamins and minerals. Often it contains a good amount of calories to aid satiety and help people with higher caloric needs. Examples from Nutricia include Periflex Junior, Periflex Advanced and PhenylAde Essential.

The Low Fat Formula – This type of formula is a growing segment of the PKU marketplace. Since PKU is diet for life, as people age their calorie needs sometimes lessen. Older people with PKU often have a wider array of low protein foods they will eat which makes them less reliant on formula for satiety. Low or reduced fat formulas typically have less calories and are lower in volume so you can drink less to meet your daily PHE-free protein needs, which can aid adherence and help keep levels in check. Examples from Nutricia include Xphe Maxamum, Lophlex Powders (also in Liquid) PhenylAde40, and PhenylAde60.

The Ready to Drink – PKU formula that is pre-mixed and ready to drink is in high demand. Everyone leads busy lives and don’t want their formula needs to slow them down. This segment is growing just as fast as you can drink one of these single serve units. The ready to drink formulas are often low in fat, calories and volume. For example, PKU Lophlex LQ provides 20 g of protein equivalent (PE) in only  4.2 fl oz. Ready-to-Drink (RTD) PKU formula are quick and easy to drink so you so you can get on with your day.

The Fortifier – Concentrated phenylalnine-free amino acid powder that can be added to any low protein food or drink you already enjoy. It can also be added to ‘Drink’ formula to increase protein content without added formula. Examples for Nutricia include PhenylAde Amino Acid Blend and PhenylAde MTE Amino Acid Blend (Minerals & Trace Elements)

The Tablets – Not everyone has an easy time drinking their formula. But the importance of consuming medical food (formula) and the consequences of not are is well known. For this reason there are formulas that are in tablet form. You would need to ingest quite a few to meet your full daily phe-free protein needs, but for some this is a better alternative than drinking formula.  You can choose to just replace 1 serving of liquid formula per day with tablets.  Example, just 12 tablets of Phlexy-10 Tablets replace 10 g PE of liquid formula. Talk to your metabolic professional if you are struggling with formula to see if a tablet or a combination of formula and tablets will work for you.


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 based on an excerpt from a book written by Sandy with the help of others titled: My PKU Toolkit: A Transition Guide to Adult PKU Management.