All posts by Nutricia Metabolics

Fun with Formula

Thank you NPKUA!

Leave a comment

Thank You to the National PKU Alliance for hosting a wonderful PKU conference in Indianapolis, IN on July 28-31 ,2016.Team at Booth (2)

Nutricia Metabolics was proud to be a GOLD sponsor for this event that brought together over 600 people from Family from Canadaacross the US and Canada.

The best part about attending events is meeting the families and children that are touched by PKU.

We took this opportunity to present our extensive PKU line of products in a simplified fashion.  We highlighted our ready-to-drink, lower volume and more flexible options with individualized  tasting stations.

Postcard Booth with Linda and Mallory

 

 

 

Conference attendees were also surprised with a sneak peak of the new PhenylAde GMP formulas coming out this fall.   PhenylAde GMP Original flavor (no flavor added) was a big hit, with many people requesting follow-up samples once it becomes available in November of this year.   More information about the launch of PhenylAde GMP Original will be available soon.

PhenylAde GMP

Available mid-November 2016!

 

 

Uncategorized

BIG WIN FOR MEDICAL FOOD COVERAGE

Leave a comment

Nutricia is pleased to share wonderful news regarding medical food coverage for families in the US military affected by a condition or metabolic disorders that requires the use of medical food.

On Tuesday, June 14th (also flag day), the US Senate in the National Defense Authorization Act  authorized medical foods coverage for Fiscal Year 2017 (S.2943).

Medical foods are defined by the FDA in section 5(b) of the Orphan Drug Act (21 U.S.C. 360ee (b) (3)), as “a food which is formulated to be consumed or administered enterally under the supervision of a physician and which is intended for the specific dietary management of a disease or condition for which distinctive nutritional requirements, based on recognized scientific principles, are established by medical evaluation.”  

Nutricia North America is dedicated to improving the quality of human life through medical nutrition and truly understands the impact of such a milestone in our country.  Those living with metabolic disorders such as Phenyketonuria (PKU), Maple Syrup Urines Disease (MSUD), Urea Cycle Disorders (UCD), as well as digestive disorders, such as Eosinophilic Esophagitis rely on medial food to support their health and well-being.  A lack of access to these medically necessary products can be catastrophic to many families both within and out of our military.

We are proud to innovate and manufacture medical foods and thank NORD and other organizations that work tirelessly to improve access to these important  resources .  This is a win for our military and a step forward for the future of medial food access for all.

Coverage Help Icon

Fun with Formula

Get Creative with PhenylAde™ 60

Leave a comment

You are busy, so don’t let your PKU formula slow you down.   Grab a store bought (protein-free) drink and add PhenylAde 60 to create a custom PKU formula you can enjoy anywhere, anytime!

Check out these great, 1-step recipes featuring PhenylAde 60.

ICED COFFEE

ICED TEA

ORANGE DRINK

REQUEST A SAMPLE NOW

1 serving (16.7 g) of PhenylAde 60:

  • Only 49 Calories

  • 10 g protein equivalent (PE)

  • Low Volume (mix with only 3 fl oz of cold water)

Try It Today!

 

 

 

 

Ask the Metabolic Expert

How do you manage PKU?

Leave a comment

Nutritional Management of PKU:

Since all foods with protein contain phenylalanine (PHE), a person with PKU must restrict the amount and types of food containing protein. For this reason, PKU is managed with a low-PHE diet consisting of foods low in protein and a PHE-free metabolic formula.

The main sources of foods with protein allowed in the low-PHE diet are fruits and vegetabiStock_000015639017Mediumles, and a limited amount of cereals and grains.

In addition to foods, individuals with PKU must drink a special PKU metabolic formula to supply the body with the necessary protein requirements the body needs for growth and maintenance, without the addition of the offending amino acid, PHE. There is also a wide range of commercially available low protein food products available for today’s PKU population. Click to View Nutricia’s Full Range of PKU products.

Nutrition management of PKU includes frequent monitoring of blood PHE levels and regular clinic visits. Blood PHE levels can be measured either at the clinic or hospital, or collected at home and mailed to the clinic for analysis. Managed blood PHE levels in persons with PKU are between 120-360 umol/L (2-6 mg/dL)1 .

Symptoms of Elevated Blood PHE Levels:

All patients with PKU, who are not started on diet immediately, will begin to build-up PHE in the blood and brain.

In an infant with classic PKU, poorly controlled PHE levels will result in irreversible brain damage. Therefore, it is essential that each infant who is diagnosed with PKU be started on a low-PHE diet to lower and maintain PHE levels within an appropriate range as soon after birth as possible.

If a child or adult previously diagnosed with PKU, and on diet, decides to discontinue the diet, he or she will begin to build up PHE in the blood, which may increase PHE levels in the brain. Over time, toxic levels of PHE in the brain can result in a change in their brain executive functions.

Executive function involves thinking skills, memory, and concentration, all of which can decline with high PHE levels. Also, an adult might feel shaky and clumsy.

If an adult is exposed to high brain PHE levels for a long enough time, IQ losses may occur. For Adult Imagethese reasons, it is important that persons with PKU stay on the low-PHE diet for life1,2.

 

REQUEST Book CoverA RETURN TO DIET GUIDE BOOK NOW

Tips for Making PKU Formula part of your life (1)

 

 

Know Your PKU Facts

 

 

 

 

To learn more about the PKU diet or for assistance returning to the PKU diet plan email DietforLife@nutricia.com or call 800-605-0410.

References:

1.. Singh RH, Rohr F, Frazier D, et al. Recommendations for the nutrition management of phenylalanine hydroxylase deficiency. Genet Med. 2014;16(2):121-31.

  1. Thompson AJ, Smith T, Brenton D et al.: Neurological deterioration in young adults with phenylketonuria. Lancet 1990; 336:602-605

Nutricia is a registered trademark of N.V. Nutricia.

Copyright 2015 Nutricia North America, Ic.  All Rights Reserved.

Diet-for-Life (Adult Corner)

Your Guide to Getting Back on the PKU Diet

Leave a comment

PKU is Diet-for-Life.  It’s Never Too Late to go back on diet.  

   Book Cover

Request a free copy of this helpful booklet “Your Guide to Getting Back on the PKU diet” and help yourself or someone you love get re-started on the PKU diet today!

This book contains:

  • Facts and Myths about the PKU and the diet

  • Tips for making formula part of your life again

  • Top 10 Nutricia PKU formulas for adults

  • Contact information to find a clinic and get re-started on diet

  • Inspiring quotes for PKU adults and more…

PKU Q&A
Tips for Making PKU Formula part of your life (1)

 

Top 10 PKU Formulas for Adults

REQUEST YOUR COPY

Ask the Metabolic Expert

What is PKU?

Leave a comment

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.

Low Protein Diet Tips, Parents Corner

Does your child love low protein pasta?

Leave a comment

My child would eat pasta every day, every meal if I let him. It is great he found a food he enjoys, but I want my son to eat more vegetables and other healthy foods that provide fiber and other nutrients.

Here are some ideas to feed your child’s low protein pasta cravings, but to make your low protein pasta last longer and provide more dietary variety.

Soup 

Dicing carrots and celery (the smaller the better for young kids) and simmering in a vegetable broth with low protein pasta added is a low pro and filling lunch or dinner.   311You can use Loprofin Animal Pasta, Rice, or broken Spaghetti to keep it new and exciting.   Fresh dill adds a great ‘soup’ flavor and expand your child’s taste  palate.

Veggie Noodles 

Bulk up your Low Protein Spaghetti by mixing 278with Zucchini or Yellow Squash noodles. With a spiral vegetable slicer this only takes minutes and can really satisfy a bigger appetite.

 Spicy Vegetables over Low Pro Rice Pasta

You can do so many different meals with fresh or frozen cauliflower and carrots, which are Vegetable Curryboth naturally quite low in protein. Often, people on a low protein diet enjoy spice. Check out this recipe for Indian Curry with Loprofin Rice.

 Dish it out slow 

I often serve my son a small amount of pasta, which he gobbles up. Then ask him to eat 3-4 bites of the vegetables on his plate. Once done I serve another small portion of pasta and repeat. All children are motivated differently, but that love of pasta usually is an incentive to eat more veggies at meal time.

 Roll it up 

Kids love to eat with their hands (or at least my son does). Use Loprofin Lasagna to make a salad roll-up or other roll-up filled with veggies. Try filling a pasta roll-up with a blend of mashed potato and mashed cauliflower for a yummy kid friendly dinner entree.

Loprofin_Pastas

 

Order Loprofin Low Protein Pasta online at medicalfood.com

 

Written by Sandy Simons, MA, RD, CHES

Diet-for-Life (Adult Corner), Parents Corner, Tips for Teens

PhenylAde® GMP Drink Mix from Nutricia

Leave a comment

A Whole New Way to Think About PKU Nutriton

PhenylAde® GMP Drink Mix 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.

 

Diet-for-Life (Adult Corner), Low Protein Diet Tips

Flying with Formula – Tips for your Next Trip

Leave a comment

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.

Uncategorized

Celebrate Rare Disease Day

Leave a comment

February 29th is Rare Disease Day!Rare Disease Day

Leap day is rare day that happens only once every 4 years, but many people live with a rare disease every day of the year.  Nutricia Metabolics is proud to help support those living with a rare metabolic condition.

Rare Metabolic Disorder List

 

Learn more about these Rare Metabolic Conditions – CLICK the letters to learn more:

HCU

 

Homocystinuria is an inherited, genetic disorder. “Classic” homocystinuria occurs because of a deficiency of the enzyme cystathionine betasynthase (CBS).  A deficiency in CBS interferes with the body’s ability to fully process an essential amino acid called methionine. If left untreated, this can cause serious health problems.

IVA

 

Isovaleric acidemia (IVA) is an inherited, genetic disorder. IVA occurs when there is a defect in an enzyme that helps break down protein from food. The name of the enzyme is isovaleryl-CoA dehydrogenase (IVD).

GA-1

 

Glutaric Aciduria Type 1 (GA-1) is an inherited, genetic disorder.  GA-1 occurs when there is a defect in an enzyme that helps break down protein from food. The name of the enzyme is glutaryl-CoA dehydrogenase (GCDH).

MMAPPA

 

 

 

Propionic acidemia (PA) and methylmalonic acidemia (MMA) are inherited, genetic disorders. Both are caused by defects in enzymes that are needed to break down protein.  In PA and MMA, four of the amino acids cannot be broken down but instead produce toxic acids in the body. These amino acids are methionine, isoleucine, threonine and valine, which are called “propiogenic amino acids.”

MSUD

Maple Syrup Urine Disease, or MSUD, is an inherited, genetic disorder caused by a defect in three enzymes that help break down protein from food. The official name for MSUD is Branched- Chain Alpha-Ketoacid Dehydrogenase Deficiency, but it is called MSUD because the urine from untreated infants with this disorder can smell sweet like maple syrup.

UCD

 

Urea cycle disorders (UCDs) are a group of inherited, genetic disorders. The urea cycle is a chain of reactions using a group of enzymes in the liver. These enzymes are important in removing unneeded protein byproducts (nitrogen) from the blood.

FAOD

Long-chain fatty acid oxidation disorders (LC-FAODs) are a group of inherited, genetic disorders. LC-FAODS occur when there is a defect in one or more enzymes that help break down fat from food for energy. LC-FAODs prevent the body from using fats normally.

LEARN MORE ABOUT OUR FULL LINE OF METABOLIC FORMULAS FOR THE DIETARY MANANGEMENT OF THESE RARE DISORDERS