All posts by Nutricia Metabolics

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

Boo! A Simple Low Protein Halloween Treat

A not-so-spooky recipe for gatherings and parties this season

Free ground shipping on all orders over $30.

Happy October! Halloween will be here in a flash, and our team at Nutricia wants to share with you and your family a simple low protein Halloween snack: marshmallow squares made with our Loprofin Cereal Loops!

Nutricia Loprofin Cereal Loops are perfect for a satisfying low protein breakfast, as well as special treats like this! Available at MedicalFood.com.

With a delicious base recipe and endless decoration possibilities, this treat is ideal for Halloween gatherings and trick-or-treat parties where you and your family would like to include a low protein option.

Click here for our low protein cereal marshmallow treat recipe and nutrition information. These treats are frighteningly simple to make because the recipe calls for just 3 ingredients! As an added bonus, the recipe is ready in a matter of minutes.

With the base recipe complete, add some Halloween flair:

Fun candy eyes*:

*Candy eyes were purchased at a party store and contain 0 g protein per 1 tsp serving size, per Nutritional Facts label on the back of the package.

You can also insert Halloween cupcake toppers into the squares if you don’t want to add any additional food ingredients to the treats. Themed cupcake toppers can be found in party stores and even grocery stores during this season.

Lastly, adding simple paper decorations to your plate is another easy way to get into the Halloween spirit:

Enjoy, and Happy Halloween to you and yours from the Nutricia team!

Check out our Facebook page for more fun recipes and gathering ideas!

Loprofin brand cereal Loops 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.

© 2019 Nutricia North America

Proud Moments for the Mullen Family

Brady and Quinn drink PhenylAde® GMP Drink Mix and start owning their PKU diets

Do you remember the moment you got the call about your child having an inborn error of metabolism?

In this video, Allyson Mullen shares her heartfelt story about that moment. Her sons Brady and Quinn received classical Phenylketonuria (PKU) diagnoses, which scared Allyson at first. Allyson opens up about what she’s learned as a parent and how Brady and Quinn have started taking on responsibility for their PKU management.

Allyson also shares why the Mullen family made the move to PhenylAde GMP Drink Mix for the boys’ PKU formula. Spoiler Alert: Brady actually called this formula “delicious,” and mom Allyson loves it because the formula contains glycomacropeptide (GMP). GMP is made from a whole protein source, and Nutricia’s PhenylAde GMP Drink Mix has a creamy, mild vanilla flavor with a smooth drinkable texture. It is also available in an original plain flavor.

Watch now to hear more about Allyson Mullen’s proud moments and why her boys love PhenylAde GMP Drink Mix.

Brady and Quinn Mullen made the move to PhenylAde GMP Drink Mix for their PKU formula.

Will your family give it a try?

PhenylAde GMP Drink Mix is a medical food for the dietary management of proven Phenylketonuria (PKU) and must be used under medical supervision.

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

© 2019 Nutricia North America

Back to College Interview with James Fryer

Reflections from a rising sophomore managing PKU and a busy schedule

This time last year as James Fryer was beginning his first year of college at Willamette University, he knew he was heading into a big life transition. Some of the adjustments proved more stressful than he anticipated.

“Always remembering, on my own, to have an adequate supply of formula,” reflects James on one of his PKU management tips. His hometown and college are in different cities, meaning preparation is very important.

Good thing James is a planner. He thrives on a schedule, whether for formula supply or diet and wellness, and is a master at making them. “I look at my schedule to see where I have time to cook versus go for a run,” shares James. This is a prime example of James’ planning that he feels led him to be successful not only in the classroom but also on the soccer field, becoming part of Willamette’s club soccer team.

James tried PhenylAde GMP READY as part of his daily routine. Will you?

Another campus group important to James are the chefs who prepare James’ food. “Willamette has taught me new, interesting things about [cooking] vegetables,” says James.

He got close to one of the university chefs this past year who was dedicated to James’ diet. The chef introduced the freshman to new things, such as fried cauliflower “rice” and utilizing all types of spices like turmeric and paprika. It’s a great relationship that James hopes to continue.

“Perhaps the biggest mistake I made was not stocking enough prepared snacks around the dorm”

-James

Outside the dining hall and back in his dorm room, James learned a lesson he’s eager to share with other college-bound PKU’ers, “Perhaps the biggest mistake I made was not stocking enough prepared snacks around [the dorm].” By the end of the year, James had a nice stockpile of low protein snacks, including low protein cereal, his standby. And another favorite snack that he’ll be continuing to eat this year? James is ready with an answer: “Dried cranberries!”

For handy and filling dorm snack, try Loprofin Loops low protein cereal

Other “grab-and-go” food on hand included dried jackfruit, which his dad got for him, and which ended up being popular among his friends. Applesauce cups and low protein rice cereal treats also helped James stay full and fueled between meals.

“If you’re constantly hungry,” James recommends to other PKU college students, “consider talking [to your metabolic healthcare professional] about adjusting formula or food, versus going off diet.”

With a new school year on the horizon, James is looking to stay on track diet-wise to help him succeed at new heights on the field and in the classroom. In fact, he’s hoping to declare a major this year, potentially math. With club soccer, he shares that he’s interested in building a soccer community around him, which ties into another goal of establishing relationships that go deeper than the differences in his diet.

Good luck with sophomore year, James! And be sure to pack plenty of dried cranberries!

PhenylAde GMP READY is a medical food for the dietary management of proven Phenylketonuria (PKU) and must be used under medical supervision.

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

© 2019 Nutricia North America

Back to School Interview with Heather Bomar

Heather, right, with daughter Ellis

Tips and wisdom from a parent of a PKU 7th grader

“I feel like I’m pulled in four different directions all summer!” says Heather Bomar of Brentwood, Tennessee, in describing her busy but fun summer season. Her four children, including 12-year-old Ellis who has classical Phenylketonuria (PKU), do a variety of activities over the summer. This means Heather spends much of the warmer months making sure they get where they need to go. 

In thinking about the back-to-school season, Heather reflects that the new year brings a new routine:

“The kids actually love the new schedule and seeing who’s in their classes,” shares Heather. The new routine also helps keep Ellis’ phenylalanine (PHE) levels in check, since the school year means set meal times and fewer impromptu gatherings that may or may not have low protein food options.

With Ellis heading into 7th grade, Heather is very familiar with back-to-school planning that works well for both Ellis and the family:

Download our Teachers and School Nurses PKU Education Guide to hand to your child’s teachers or use during parent-teacher meetings! Check out other PKU educational materials on the Nutricia website.

First, Heather meets with Ellis’ new teacher at the beginning of each school year to run through the basics of Ellis’ condition and diet. Second, she recommends co-creating a plan with the teacher, if necessary, to provide the best chance of success for a student with PKU, such as the student sitting in one of the least distracting places in the classroom. 

One of the most top-of-mind topics for parents of children with PKU is school lunch. The Bomars send Ellis to school with lunches packed at home. It’s a system that works for Ellis and the family. For her PKU formula, Ellis drinks PhenylAde® GMP Mix-In formula at school. When asked about snacks and lunch items, Heather responds without hesitation that Ellis’ favorite is “anything that she can dip in Ranch dressing!” Examples include celery, carrots, and peppers.

Ellis drinks PhenylAde GMP Mix-In formula at school. Will your family give it a try this year?

Other lunch favorites of Ellis’ include dried fruit, especially store-bought freeze-dried strawberries and apples. After school or at home on the weekends, Ellis enjoys low protein crackers smeared with butter and salt, a snack that Heather sometimes shares with her daughter as they catch up and talk about Ellis’ middle school, which Heather also attended!

Outside of lunchtime and PKU management, Ellis’ 7th grade year will revolve around establishing her sense of self: 

“Ellis loves school and has a good group of friends,” reflects Heather, “and she’s focused on finding her identity in things outside of PKU.”

As advice to other parents raising children with PKU, Heather takes a step beyond day-to-day food prep and considers the full parent-child relationship: “Stick with them through the highs and lows,” says Heather, “Keep up open conversations.”

… And don’t forget the Ranch dressing!

Read on for 10 More Low Protein Lunch Tips for School:   http://www.lowproliving.com/lunchtips/

PhenylAde GMP Mix-In is a medical food for the dietary management of proven Phenylketonuria (PKU) and must be used under medical supervision.

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

© 2019 Nutricia North America

Shake-Up Your Day with PhenylAde® GMP Drink Mix

Here are some fun recipes using PhenylAde GMP Drink Mix to help you ‘Shake-Up Your Day’.

If you are looking for a great tasting PKU formula that also provides a source of Glycomacropeptide (GMP), then you found it.

PhenylAde GMP Drink Mix contains GMP, made from an intact protein source.

PKU formula with GMP is known for having a mild taste, which can help make drinking formula more enjoyable. Another way to help keep PKU formula fun and interesting is to create a custom shake to provide additional variety.

**Adding flavoring to PhenylAde GMP Drink Mix may change the PHE/Protein content. Be sure to read labels and account for any added PHE or Protein

Request a Sample Today!

Download the full PhenylAde GMP Recipe Book Now

© 2019 Nutricia North America

PhenylAde®  GMP Drink Mix is a medical food for the dietary management of proven Phenylketonuria  (PKU) and must be used under medical supervision.   Always consult your metabolic clinic prior to making any changes to your PKU diet.