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Celebrate Rare Disease Day

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

 

Ready-to-Drink PKU Formula

Busy Schedules.  Hectic Mornings.   After School Activities.  Road Trips.  Family Vacations.  Sleep Over at a Friends. 

Just a few things that may cause you to skip a serving of formula or slow you down on a busy day.  Ready-to-Drink PKU Formula can help you save time and drink on the go!

Check out this Video  – Request A Sample

View more PKU Videos from Nutricia

Nutricia’s Top 10 PKU Formula Options for Adults

PKU is Diet-for-Life. Get back on track and find a PKU Formula you can enjoy drinking. 

Nutricia Metabolics is proud to offer the largest selection of PKU formulas and flavors.  Nutricia also offer free samples* so you can see which product and flavor is best for you.

Top 10 PKU Formulas for Adults

Try 1, try them all!

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Request a FREE product sample direct from Nutricia at www.medicalfood.com

 

*All samples are verified with a metabolic healthcare professional prior to shipping.  All PKU formulas must be used under medical supervision.

NPKUA – 2016 PKU Hero Award Announement

The National PKU Alliance is proud to announce that Rani H. Singh, PhD, RD, will be the recipient of the 2016 PKU Hero Award.

2016 PKU Hero Award Winner - Dr. Rani Singh

2016 PKU Hero Award Winner – Dr. Rani Singh

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Dr. Rani Singh currently directs the Genetics Metabolic Nutrition program at the Emory University School of Medicine, Department of Human Genetics. The nutrition program is one of the nationally leading programs offering complete services to manage metabolic disorders.

Not only an established leader, Dr. Singh has been a trusted dietitian to PKU patients at Emory for over 25 years. In addition to caring for her patients, she has contributed to over 140 publications and initiated countless efforts to improve PKU outcomes.

Dr. Singh is the founder and prior president of Genetic Metabolic Dieticians International (GMDI), an organization whose mission is to provide standards of excellence and leadership in nutrition therapy for genetic metabolic disorders including PKU.

In 1994, she founded the Emory Metabolic Camp to help young women understand the importance of the PKU diet in preventing maternal PKU syndrome. Dr. Singh was the leading Co-Principal Investigator in the development of the evidence based Nutritional Management Guidelines for the treatment of patients with PKU to help ensure that people receive the highest standard quality of care in the management of their PKU.

Dr. Singh has been highly recommended as the 2016 PKU Hero Award recipient and is deserving of this recognition. “Dr. Singh’s work and values exemplify her belief in collaboration and commitment to maximizing the benefits of newborn screening,” says Kristen Vanags. Dr. Singh’s love for her patients and depth of expertise has improved countless lives, and her work will positively influence the PKU community for many year!

Rani H. Singh, PhD, RD will be officially recognized at the 2016 National PKU Alliance Conference during the PKU Hero Award Banquet on Saturday, July 30th in Indianapolis, Indiana.

Congratulations Dr. Singh!

Défi Faites des pompes pour la PCU

Nous sommes très heureux et fiers d’appuyer la National PKU Association (NPKUA) ainsi que PCU et maladies apparentées Canada Inc. (CanPKU) avec notre défi Faites Des Pompes Pour La PCU afin d’aider à accroître la sensibilisation et d’amasser des fonds pour la PCU.

Du 2 novembre jusqu’à la journée nationale de sensibilisation à la PCU, le 3 décembre 2015, Nutricia North America fera un don de 5 $ pour chaque vidéo montrant une personne ou un groupe de personnes faisant au moins 5 pompes en soutien à la PCU.

Tout le monde peut participer! Vous pouvez faire les pompes sur vos genoux, sur vos orteils ou même contre un mur. Les personnes à capacité restreinte peuvent soulever une boîte de préparation 5 fois ou trouver une autre façon créative de participer. Assurez-vous simplement d’inclure les mots-clics #NutriciaMetabolics et #Pushups4PKU afin que nous puissions répertorier l’ensemble des vidéos. Vous pouvez aussi afficher votre vidéo sur notre page Facebook ou Instagram.

Alors, rassemblez vos amis et filmez-vous en train de faire 5 pompes pour la PCU! En plus d’aider à accroître la sensibilisation et d’amasser des fonds pour une bonne cause, vous aurez l’occasion de rester en forme et en santé. N’oubliez pas de lancer le défi à vos amis et de passer le mot.

Pour donner le coup d’envoi au défi Faites des pompes pour la PCU, voici ma vidéo. Je VOUS lance maintenant un défi : Faites des pompes pour la PCU (#pushups4PKU)!

Jeanine

 Jeanine Kendzejeski Metabolics Sales Director Nutricia North America

Jeanine Kendzejeski
Metabolics Sales Director
Nutricia North America

Jeanine Kendzejeski, directrice des ventes, Produits nutritionnels pour maladies métaboliques

N.B. Pour en connaître davantage sur la PCU, veuillez lire l’excellent billet

What is PKU (disponible en anglais seulement) publié sur le blogue LowProliving.com (lien vers http://www.lowproliving.com/what-is-pku-2/ ).

Nutricia ne prend aucun engagement et, par la présente, décline expressément toute responsabilité envers toute personne physique ou morale quant à des réclamations, des dommages, des dettes ou d’autres pertes relativement au présent concours. Nutricia ne pourra, en aucune circonstance, être tenue responsable envers toute personne physique ou morale quant à des dommages directs, particuliers, exemplaires, indirects, accessoires, consécutifs ou punitifs, quel qu’en soit le type ou la nature (y compris, sans restriction, toute responsabilité quant aux blessures ou autres dommages liés, d’une quelconque façon, à la participation directe ou indirecte au présent concours), qu’il s’agisse d’une poursuite fondée sur un contrat, une garantie, la responsabilité civile absolue, un délit civil (y compris la négligence, sans s’y limiter) ou autre, même si Nutricia a été informée préalablement de la possibilité de tels dommages ou si Nutricia aurait pu raisonnablement prévoir de tels dommages. Nutricia fera un don pour chaque vidéo reçu pendant cette période, jusqu’à concurrence de 400 vidéos.

 

How does PhenylAde GMP taste? Ask David.

 

My name is David Harris. I’m 40 years old and I was diagnosed with classical PKU.

When I tasted the PhenylAde GMP product, it was very easy to drink, it was very smooth, and in comparison to other products in the market, I actually preferred it.

Formula taste is key. It’s very important, because without a good taste, and a good consistency; it’s very difficult to get down.

If it tastes good, then I’ll drink it.


Curious to try PhenylAde GMP?

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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

Low Protein Food – Buy Direct from Nutricia!

There are a growing number of options when it comes to low protein food.  One of your options includes buying direct from the company, which could add up to a cost savings for you.

For example, Loprofin brand pasta by Nutricia is available online in more than one place, but buying direct from Nutricia’s website www.medicalfood.com provides up to a $2 per box savings per box compared to other online low protein food shops when purchased by the case.*  This can up to BIG savings when buying 6, 12 or 24 boxes in one order.

Loprofin_Pastas

Quantity per unit is also another important item to explore.  Some products may appear similar in nature and have the same cost per unit.  But with a closer look you may see that one product unit may only provide 10 oz  while the other supplies 16 oz.  You want to compare you prices by ounce or gram to determine the best value for your money.

Next time you are ready to place a low protein food order, compare the volume and cost.   Also consider buying direct from the company and registering on their webistes for special offers and promotions.

 

Written by Sandy Simons, MA, RD, CHES

Sandy works for Nutricia North America.

*based on prices published online by PKU Perspectives as of September 22, 2014.  Varies based on product.

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.

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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.

Do I really need to follow the expiration date?

by Linda Leuffgen, RD

It’s now 2015 and you see those expiration dates creeping up.

“Is expired product still safe to use?”

We get this question a lot. Our advice is not to use a medical food or low protein food product past its’ date of expiration. Let me explain why as a lot of factors go into determining an expiration date for all food; not just for Nutricia products.

First is the reliability of the packaging materials. We need to make sure the product inside that packaging is safe until the expiration date. Second is the stability of the ingredients in the product. For example, some fats go bad faster than others. We want to ensure the product you consume is fresh which is indicated by the use by date.

Another determination for expiration dates is to ensure the nutrients in the product are true to the levels marked on their label. Nutrients, especially vitamins, have a tendency to naturally break down over time; some faster than others. It is extremely important that the right amount of each nutrient is in the product since these formulas tend to make up more than 50% of your nutritional needs. We conduct extensive testing under a number of conditions to ensure that the nutrients hold up until the date of expiration.

Nutricia’s goal is to bring you the best nutritional products possible. The intent of an expiration date is to keep our consumers safe and ensure they receive the nutrition they need. Again, we cannot recommend using any of our products past their date of expiration. We suggest discussing this further with your healthcare professional if you have expired product at home and require further questions.