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Category Archives: Diet-for-Life (Adult Corner)

PKU is Diet-for-Life. It’s Never too late to return to the PKU diet plan. Learn more about other PKU adults and how they returned or remained on the diet diet. Also learn about new PKU formulas and recipes to help keep the diet fun and interesting.

Sports Nutrition Basics for Active Individuals with Disorders of Amino Acid Metabolism – Part 2

We talked about general topics of sports nutrition in our last blog — let’s now discuss what is important BEFORE, DURING and AFTER exercise for people with an inborn error of amino acid metabolism such as Phenylketonuria (PKU), Maple Syrup Urine Disease (MSUD) or Homocystinuria (HCU).

Before getting started, it is important to know that you should always talk to your metabolic healthcare team before changing any exercise or diet routine. The information here is not meant to take the place of professional medical advice.

HYDRATION: Drink water before, during and after physical activity to stay hydrated

When our body temperature increases through exercise, we tend to sweat more and lose electrolytes. This can lead to dehydration and negative health effects.

Don’t just trust your thirst but have a regular schedule for drinking water. Start hydrating before exercise. The intensity of your exercise will determine how much you should drink before, during and after exercise. Your dietitian will guide you.

For those with PKU and Tyrosinemia (TYR), remember to choose drinks that are aspartame‐free as this artificial sweetener contains phenylalanine (PHE).

Nutrition BEFORE exercise: It’s all about the fuel

Nutricia makes Loprofin pastas that can be turned into tasty high-carbohydrate meals!

Start well‐fueled. For most athletes that means a carbohydrate‐containing meal and some protein (from metabolic formula and food) a few hours before exercise.

Not providing enough calories for sports can be problematic for people with a disorder of amino acid metabolism. The body could start breaking down body protein, and toxic substances might increase in the body.

Low protein pasta dishes are excellent choices for high‐carbohydrate meals; low protein baked goods are also great, delicious options. Check out our recipe ideas.

Metabolic formula provides a safe, appropriate source of protein, and a certain amount of formula (as recommended by your dietitian) should be used a few hours before exercise as part of a regular meal pattern. Think about making a smoothie with formula to give you some extra calories. Certain metabolic formulas also provide carbohydrates, so check with your dietitian about which options are best for you.

If a light snack is needed shortly before starting exercise, consider low protein crackers, fruits, a low protein snack bar or So Delicious® coconut milk yogurt alternative*.

Nutrition DURING exercise: Keep it going!

Hydration is key of course, and some people might also benefit from appropriate snacks with long‐term, high intensity exercise. Fruit juices or carb gels may be good options as they provide carbohydrates and some calories for energy. Your dietitian may also recommend a sports drink depending on your needs.

Nutrition AFTER exercise: And now about recovery

After exercise or sports, it is important to refuel—and that can be done with metabolic formula and food. Formula can provide an immediate boost of protein and other nutrients. Calories are needed to keep your body from breaking down too much body protein from muscles, which could result in an increase of toxic substances and be harmful to your health. For some people, it might be best to have a snack right after exercise.

Once you are home, have a proper meal such as: low protein pasta, a low protein sandwich, veggie chili or a fruit smoothie made with metabolic formula.

When it comes to metabolic formula, Nutricia offers a variety of options to choose from to meet your protein needs! Ready‐to‐drink products such as PhenylAde® GMP Ready and Periflex® LQ (for those with PKU) or Lophlex® LQ (available for those with PKU, HCU, TYR and MSUD) are great options to carry with you to practice or a game. Put them in a lunch bag to keep them cool if you like.

PKU PhenylAde GMP Mix‐In is a super‐convenient option to add straight into a sports drink after your game. Nutricia offers a Mix‐In product for the Tyrosinemia community as well called TYR Lophlex GMP Mix‐In.

We also offer formulas that provide more calories if that is what you need. Check out our options, and talk to your dietitian about which one is best for you.

Talk to your clinic about which Nutricia formulas may be suitable for you or your loved one’s sports and fitness needs.

We hope you or your loved one can find inspiration to explore more sports and fitness opportunities! Check out our Facebook page and share what exercise, sports, and activities you and your family do.

All products shown are medical foods for the dietary management of inborn errors of metabolism and must be used under medical supervision.

Always talk your metabolic healthcare professionals before making any changes to your activity or diet routine.

*So Delicious® coconut milk yogurt alternative is an affiliated Danone brand.
© Nutricia North America 2019

Written by: Ulrike Reichert, MS, Director Medical Affairs Keto/Metabolics Nutricia North America

Sports Nutrition Basics for Active Individuals with Disorders of Amino Acid Metabolism – Part 1

Participating in sports and fitness provides many benefits for health and wellbeing—and this holds true for many people with inherited metabolic disorders.

Plenty of members of the metabolic community run races, dance, and play team sports to stay fit. James, a college sophomore with Phenylketonuria (PKU), for example, plays club soccer. (Read our interview with him on getting back to college here). And Danae’, who has Homocystinuria (HCU) and leads the HCU Network America patient group, lifts weights regularly!

We put together a couple of blogs with some basic ideas of sports nutrition for those with amino acid disorders in hopes of inspiring you or your loved one to explore sports and fitness opportunities. Read on and be sure to check out our second blog in this series for more on nutrition before, during, and after exercise.

Huddle with your team (your metabolic healthcare team, that is!)

Before getting started, it is important to know that you should always talk to your metabolic healthcare team before changing any exercise or diet routine. The information here is not meant to take the place of professional medical advice.

Talk with your metabolic healthcare team about which sports and fitness activities may be good for you or your loved one. Your dietitian will take the type, intensity and duration of exercise into account and advise you on your needs.

Play at your best with the help of proper nutrition

Being at your best at your sport not only takes practice, but it starts at home at the table. What you eat affects your physical and mental performance. Being prepared before exercise is especially important for people with an amino acid metabolism disorder.

To play or exercise at your best, keep the following nutrition rules of thumb in mind:

  • A general healthy diet helps your fitness. That means limiting processed foods, avoiding added sugar and eating a variety of fruits and veggies.
  • Protein, coming mainly from the prescribed metabolic formula for most individuals with an amino acid disorder, is important for muscle building and repair.
  • Carbohydrates from special low protein foods, metabolic formula, and regular foods low in protein provide fuel so your body has enough for your sport; avoid sugary foods when possible.
  • Healthy fat sources such as sunflower or walnut oil give you essential fats and calories.
  • And as mentioned, fresh fruits and veggies provide nutrients such as fiber, vitamins, and minerals.

Remember: Always ask your metabolic healthcare professionals for advice.

Nutricia offers several different metabolic formulas to help you get your protein, including after sports. Check out ready‐to‐drink PhenylAde® GMP Ready, Lophlex® LQ and Periflex® LQ for those with PKU. Lophlex LQ is also available for those with HCU, Tyrosinemia (TYR), and Maple Syrup Urine Disease (MSUD). These products are super easy to carry with you in your sports bag.

Convenient options such as PhenylAde GMP Mix‐In that can be easily added to a sports drink, or even Phlexy‐10® Tablets, are other popular options for those with PKU.

Click here for more information and talk to your dietitian about which formula is right for you.

PhenylAde GMP READY, Periflex LQ, and PKU Lophlex LQ are easy to carry with you in your sports bag! Nutricia also makes ready-to-drink options for HCU, TYR, and MSUD.
Nutricia makes Loprofin pastas that can be turned into tasty high-carbohydrate meals!

And how about those carbs? When it comes to getting your carbs in before and after exercise, try our low protein pasta and recipes—made in Italy!

Let’s talk more about nutrition before, during, and after exercise in our next blog, Sports Nutrition Basics, Part 2

All products shown are medical foods for the dietary management of inborn errors of metabolism and must be used under medical supervision.

Always talk to your metabolic healthcare professionals before making any changes to your activity or diet routine.

© Nutricia North America 2019

Written by: Ulrike Reichert, MS, Director Medical Affairs Keto/Metabolics Nutricia North America

PKU & Pregnancy – 1st Trimester

Congratulations on your pregnancy! These are exciting times. Don’t forget: working closely with your clinic is important before, during and after pregnancy. Your clinic can provide the best guidance for you and your baby.

Once your blood phenylalanine (PHE) levels are well-controlled and you become pregnant you enter the first of three trimesters associated with pregnancy. Each trimester will have its own challenges and rewards.

In the 1st trimester, you will gain anywhere from 1-5 pounds. Your baby will grow from the size of a tiny poppy seed to about the size of lemon. Some women report feeling nausea during their first trimester and having difficulty keeping food down; especially in the morning hours.

Despite these challenges it is extremely important to drink your formula as it provides PKU friendly protein and general nutrition, which is essential for you and your baby.

If nausea and vomiting strikes, here are some general tips to help:

  • Try eating just the foods you enjoy most
  • Cold foods are sometimes better tolerated than hot foods
  • Avoid taking any vitamin/mineral supplement on an empty stomach
  • Avoid an empty stomach with small frequent snacks every 2 hours during the day

If nausea and vomiting strikes, here are some tips if the volume of PKU formula is an issue:

  • Ask your metabolic dietitian if a lower volume PKU formula is appropriate for you
  • Divide your current PKU formula into small frequent portions throughout the day

If nausea or vomiting is triggered by formula smell, here are some tips:

  • Ask your metabolic dietitian if you can try a PKU formula with less of a smell
  • Drink your current formula through a straw from a container with a cover to avoid smelling the formula while drinking

Some women also report constipation in their 1st trimester. Here are some tips to help:

  • Focus on fruits and vegetables that are high in fiber and low in PHE
  • Be sure you are drinking enough fluid / water

Request a Nutricia Maternal PKU Starter Kit for more helpful tips and tools to get you ready for this next stage in life.

All products shown are medical foods for the dietary management of Phenylketonuria (PKU) and must be used under medical supervision. Please consult your metabolic healthcare professional prior to making any changes to your PKU diet.

© 2019 – Nutricia North America

Tips for a Low Pro Thanksgiving

The holidays can be an exciting time of year. However, they can be a bit challenging for someone on a restricted diet as most festivities center around food. This blog focuses on tips for managing a restricted diet during the holiday season.

The most important thing to do is plan ahead.  The more you know in advance, the better you can plan.

Here are some questions to ask yourself:

  • Where will I be spending the holidays, and how long will the trip be?
  • Do I have enough formula so I do not run out while away over the weekend?
  • Am I dining at home or at someone else’s home?
  • Will I be preparing the food, or will someone else be?

Once you know more about what your day/ weekend will look like, you can make a plan.

One key thing is to plan the menu in advance.  If you are not dining at home, but at a friend’s or relative’s home, it will be important to ask what is being served, then…

  • Ask if you can bring a side dish (one that is suitable for you, but enough for everyone to share).
  • Let them know about your dietary needs so they can omit or serve items on the side that are not suitable for a low protein diet, like cheese or bread crumbs.
  • See if you can access the kitchen to re-heat or make additional low protein items for the holiday meal.

Get Creative!

Many traditional holiday favorites can be modified to reduce the protein while still keeping the taste and texture you and others love.

Find our simple recipe for reduced protein mashed potatoes here – this dish is sure to become a family staple at your holiday meal.

And end your holiday meal on a sweet note with this low protein dessert.  This is a dish everyone at your holiday gathering is sure to enjoy!

Easy Pumpkin Pudding

Recipe developed by Sarah Marshall, classical Phenylketonuria (PKU)

Ingredients:

  • 1 fl oz water
  • 7 fl oz coconut milk (full fat, canned coconut milk required to get  pudding-like consistency)
  • 6 whole pistachios (for flavor, to be strained out later)
  • ½ Tbsp brown sugar
  • ¼ tsp pumpkin pie spice (or blend of cinnamon, ginger and nutmeg)
  • ½ tsp vanilla flavoring
  • ½ cup Loprofin Rice

Directions:

  1. In a small saucepan, add 1 oz water, 7 oz coconut milk, and 6 pistachios.
  2. Bring to a boil. Immediately when it begins boiling, add brown sugar, vanilla, and pumpkin pie spice. Stir.
  3. Add Loprofin Rice. Stir. Cover, and lower heat to simmer covered for 10 minutes.
  4. Once rice is cooked, remove pistachio pieces.
  5. Serve warm or cold.

Makes 2 servings

And most of all……

Holidays do not have to be all about food.  Make your holidays memorable with family traditions, such as a family football game or a checkers tournament.

Happy Thanksgiving to you and your family from Nutricia!

Loprofin brand Rice is a medical food for the dietary management of inherited metabolic disorders, renal or liver failure requiring a low protein diet, and other medical conditions where a low protein diet is indicated. Must be used under medical supervision.

Always consult a metabolic healthcare professional prior to making any diet changes.

© 2019 Nutricia North America

PKU & Pregnancy – Preconception

The journey to motherhood, when you have Phenylketonuria (PKU), should begin with your metabolic clinic. It is essential to work with your clinic before, during and even after pregnancy. Only your clinic can provide the best guidance to ensure the best outcome for you and your baby. They will work with you to ensure well-controlled blood phenylalanine (PHE) levels prior to pregnancy and throughout your pregnancy to prevent the negative effects of elevated PHE.

Studies in maternal PKU (MPKU) have shown uncontrolled blood PHE levels during pregnancy can cause the following outcomes for the baby:

  • Microcephaly (small head size)
  • Intellectual disability
  • Behavioral issues
  • Congenital heart defects (CHD)
  • Low birth weight and reduced length for age

Keeping your PHE levels within recommended range will help protect your baby from these risks.

Steps to getting PHE levels in the recommended range:

Find low-PHE recipes at medicalfood.com/recipes

Recommit yourself to the low PHE diet

  • Explore vegan recipes with the plant-based protein ingredient omitted (such as nuts, soy, tofu, etc.)
  • Change regular bread, rice and pasta to low protein versions, such as Nutricia’s Loprofin low protein food line

Find a PKU formula you enjoy

  • Nutricia offers the widest range of PKU formula options. You are sure to find one that meets your personal taste and needs. Check out Nutricia’s formula options here.
  • Create a plan and stick to it – this will help to make sure you take all your recommended formula each day at scheduled times.

Make a new routine

  • Regular blood samples (blood spots) will let you know when your PHE levels are in the ‘safe-range’ for pregnancy. Set a reminder on your calendar or cellphone to send your blood spots to your clinic on a regular basis (as instructed by your clinic).
  • Meal planning in advance will help make food shopping and new dining habits easier.

Get connected

For more helpful tips and tools to help get you ready for this next stage of life, request a Nutricia Maternal PKU Starter Kit.

All products shown are medical foods are for the dietary management of Phenylketonuria (PKU) and must be used under medical supervision. Please consult your metabolic healthcare professional prior to making any changes to your PKU diet.

© 2019 – Nutricia North America

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.

10 Reasons to Try Phlexy-10 Tablets

Did you know… Drinking PKU formula isn’t your only option?

There is another option to help get the daily PKU formula you need.*   Nutricia is proud to offer the option of PKU formula in tablet form. 

  • Phlexy-10 Tablets are a discreet and convenient way to take your formula. 

  • Use in addition to your current formula to help reduce the amount you need to drink. 12 Tablets = 10 g of Protein Equivalents

  • May be used as your main PKU formula instead of drinking PKU formula


 10 reasons to use Phlexy-10® Tablets for PKU

  1. Help meet your total daily formula intake, by taking tablets at night to replace that last serving of formula that you may be already skipping

  2. Convenient to take when drinking formula is not, like before sports practice or other after school activities

  3. Tablets won’t cause your locker or work area to smell like formula

  4. Lightweight source of protein (PHE-free) when hiking or camping

  5. A way to take in PKU formula when you just don’t want to drink it

  6. Prevents having to explain about your “special drink” in social settings

  7. Keep in your purse or back-pack for times you forget your formula at home

  8. Easy to take on a plane, train or other times when you will be away from home for long periods of time

  9. No messy liquid spills in your bag, purse or locker

  10. A quick and easy source of phenylalanine-free protein you can take just about ANYWHERE!


What is your top reason to try PKU Formula in Tablet Form?  Do you have another reason not listed?    Let us know!

 DietForLife@Nutricia.com

 

*Always talk to your metabolic healthcare professional prior to making any changes to your PKU diet.


Phlexy-10 Tablets are a phenylalanine-free medical food in tablet form, containing a mix of other essential and non-essential amino acids and small amounts of carbohydrate and fat. For the dietary management of proven Phenylketonuria (PKU), suitable for individuals over 8 years of age, including pregnant women and women of childbearing age. Must be used under medical supervision. Does not contain vitamins and minerals; must be combined with an appropriate vitamin and mineral supplement when taken as primary source of PKU medical food.

© 2019 Nutricia North America

Happy New Year!

Happy New Year!

The start of a new year is a great time to re-commit to better diet management.

Improving your metabolic diet is different for everyone. It can range from calling your clinic for the first time in years to making simple improvements such as:

 

  • Eating more fresh fruits and vegetables

  • Drinking the full amount of formula

  • Finding a formula you enjoy drinking

  • Sending in blood spots more frequently

  • Trying new low protein recipes

Nutricia is proud to offer support services to help you achieve some of your dietary goals.

 Our Nutricia Connect™ program is here to provide support at various stages of life’s journey.  We offer an array of low protein recipes, educational tips and resources and a team of professionals who can assist you or your loved one to get back on-diet.

Additionally, Nutricia’s team of formula coverage specialists can review your insurance and answer any questions you may have about formula coverage.

Connect with them today by emailing Coverage@Nutricia.com or by calling 1-800-605-0410.

Improve Your Metabolic Diet Today


About Us:

Nutricia Metabolics provides a variety of formula options for inborn errors of metabolism, including Phenylketonuria (PKU), Maple Syrup Urine Disease (MSUD), Tyrosinemia (TYR), Homocystinuria (HCU), Urea Cycle Disorders (UCD), Glutaric Aciduria Type-1 (GA-1), Isovaleric Acidemia (IVA), Propionic Acidemia (PA), Methylmalonic Acidemia (MMA) and Fatty Acid Oxidation Disorders (FAODs). Learn more at www.MedicalFood.com

 

 

NOTE: All Nutricia Metabolics products are medical foods and must be used under medical supervision. Always consult your metabolic healthcare professional prior to making any changes in your metabolic diet plan.

Get Ready for Summer with PKU Lophlex LQ!

Summer is one of the best season!  Long days, fun adventures, amusement parks and the warm sun are just a few reasons summer is such a great time of the year.

When you have PKU, you also need to think about how you will transport and drink your PKU formula when out for a full day.

Imagine you could have a great tasting PKU formula that you could easily drink when:

– Mountain Biking
– Canoeing with Friends
– At an Amusement Park
– Hiking or Camping
– Vising Family or Friends
– Hanging Out by a Lake

 

Don’t imagine it any longer!

Try PKU Lophlex LQ, a great PKU formula for summer. Each ready-to-drink pouch is protein packed with 20 g of protein equivalent and only 120 calories. Lophlex LQ are low volume and ready-to-drink which is great for life on-the-go.

Plus, it is the only low volume, ready-to-drink PKU formula made with real fruit juice* from concentrate for a refreshing taste on a hot summer day.

The only question is …

Post your on-the-go PKU Lophlex LQ photo on Instagram!  #PKU Summer

 


Try PKU Lophlex LQ Today!

✓ Low Volume
✓ Only 120 Calories
✓ Quick & Easy to Drink

      REQUEST A SAMPLE 3-PACK

Available in Mixed Berry, Juicy Orange and Juicy Tropical


*Juicy Orange contains 45.5% juice. Mixed Berry Blast contains 44.1% juice. Juicy Tropical contains 28.4% juice.

PKU Lophlex LQ is a medical food for the dietary management of proven Phenylketonuria (PKU) in individuals over 4 years of age. Must be used under medical supervision. Consult your metabolic clinic prior to making any changes to your metabolic diet.

Copyright 2018 Nutricia North America

PKU is Diet-for-Life

 

Now is a great time to re-commit to better PKU diet management. Nutricia has a team of professionals who can assist you, or your loved one, get back on-diet.

Our help includes personalized support ranging from finding the right PKU formula, to resetting your low protein kitchen, and more. Our Diet-For-Life Care Coordinator can also lend an extra helping hand.

In addition, Nutricia can review your insurance and answer any questions you may have about formula coverage. Schedule a one-on-one insurance review today. Coverage@Nutricia.com

You can do it. You are not alone!

You can do it – and Nutricia is here to help. 

Nutricia can:

• Help you learn about new PKU formula options
• Provide more information about PKU and returning to diet
• Connect you with a metabolic clinic or a return to diet specialist
• Review your insurance plan for PKU formula coverage
• Share recipes and low protein cooking tips

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Don’t Wait Any Longer!

DietforLife@Nutricia.com or call 800-605-0410

NOTE: All PKU formulas must be used under medical supervision. Always consult your metabolic healthcare professional prior to making any changes in your PKU diet plan.


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