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Metabolic Disorders – Talking to your young child

What to say to your child

While your child is not old enough to manage their metabolic disorder alone, it is valuable for them to begin to better understand their diet and treatment. When speaking with your child about their metabolic disorder, the following tips may be helpful:

  • Use simple examples to explain ideas
    For example, when explaining to your child why their metabolic diet is important, it may be helpful to relate the diet to that of a food allergy. Speak with your metabolic team who will also have information about books to read to your child to help him or her understand special diets.
  • Let your child know they can say “no”
    If you haven’t already, teach your child to ask you before eating unfamiliar foods, and that it is OK to say no to anyone who offers food that is unfamiliar or off limits.
  • Tell your child it isn’t his or her fault
    A child may not understand why he or she has metabolic disorder when others do not, and may think that he or she did something to ‘deserve’ it. Explain to your child that everyone is born with different qualities, such as hair and eye color, and a metabolic disorder is something that people are born with, not something that anyone causes. Reinforce to your child that he or she is special, and that this special way of eating is to keep him or her healthy.
  • Stay positive.
    Sending the right message about foods and treatment is important. It is better to talk about off-limit foods as “high-protein,” “no,” “red” or “stop” foods rather than “bad” or “naughty” foods. Help your child accept and manage their metabolic disorder as he or she grows. Never say anything negative about the food or formula to your child. This special way of eating is to keep him or her healthy.
  • You’re not alone
    Talk to your child about other people you know who are on a special diet, even if they are adults, so your child knows that he or she is not the only one on a special diet.   Get involved!   Go to a local metabolic or National event.

Click here to view upcoming events and metabolic support groups across the nation.

 

*Reference Source:Adapted from – My PKU Binder. National PKU Alliance. Chapter 5: Ages 3 to 6 Years, Page 41.

New Artificial Sweeter – OK for PKU?

FDA Approves New Artificial Sweetener

A new sugar substitute called advantame was approved on Monday by the U.S. Food and Drug Administration.  Advantame is chemically similar to aspartame (Equal), and certain people should avoid or limit their use of aspartame, the FDA noted.

These people have a genetic disorder called phenylketonuria (PKU), which makes it difficult for them to metabolize phenylalanine, a component of both aspartame and advantame. Food with aspartame must include label information warning people with PKU about the presence of phenylalanine.

The FDA evaluated whether advantame should also carry alerts for people with PKU. Because advantame is much sweeter than aspartame, only a small amount is needed to achieve the same level of sweetness.  As a result, foods that contain advantame do not need to include alerts for people with PKU, the FDA said.

Be sure to talk to your metabolic professional if they feel advantame is suitable for your diet.  Currently, the exact PHE content has not been reported.

SOURCE: U.S. Food and Drug Administration, news releases, May 19, 2014

At what age should I introduce solid foods?

At what age should I introduce solid foods?Feeding child

The American Academy of Pediatrics (AAP) currently recommends gradually introducing solid foods when a baby is about 6 months old.1

**Always consult your metabolic healthcare professional before introducing new foods or changing your child’s low protein diet.

How do I know if my baby is ready to eat solids?

The following tips may help2

  • Is your baby’s tongue-thrust reflex gone or diminished?
    This reflex prevents infants from choking on foreign objects, but also causes them to push food out of their mouths. Ask your pediatrician or metabolic dietitian.
  • Can your baby support his/her own head?
    To eat solid food, an infant needs good head and neck control and should be able to sit up unassisted in a high chair.
  • Is your baby interested in food?
    A 6-month-old baby who stares and grabs at your food at dinnertime is clearly ready for some variety.

What should I know about first foods?

Until now your baby is only used to liquids so it is essential that the first foods offered are a smooth, runny purée. First solids should be bland, easy to swallow and easy to digest. Your baby can progress to a thicker purée once they become used to the runny texture.

What types of food should I offer?

Check with your metabolic dietitian which foods are best for your baby.  Most parents begin with rice cereal mixed with formula and gradually introduce other foods. Homemade purées are inexpensive and easy to make. Fruit and vegetables can be cooked in a small amount of water until soft and then puréed using a hand blender or food processor. Ready-to-eat first foods and baby cereals are also available in the grocery store. Once you have introduced single purees to your baby you can try mixing different combinations for variety e.g. sweet potato and carrot, or apple and pear.

How much should I give my baby to eat?

At first, only offer very small amounts (about 1-2 teaspoons at a single meal). Gradually more food can be offered. When your baby is taking a reasonable amount of solids at a single meal (about 6-10 teaspoons) you can introduce solids at a second meal in the day and then at a third.

Suitable Low Protein Baby Foods2

6-8 Months Old

  • Apple sauce
  • Very small pieces of soft fruit
  • Some cereals
  • Soft cooked vegetables such as carrots or squash

9-12 Months Old

FRUITS

Fresh

  • Wash thoroughly.
  • Remove skins and seeds.
  • Cut into small, bite-size pieces or thin sticks (i.e. quarter grapes).
  • At one year, include unpeeled ripe fruits, berries, pears and nectarines.

Frozen/Canned

  • Look for fruits packed in their own juices.
  • Avoid fruits canned in heavy syrup.
  • Frozen fruits are soothing to teething gums.

Dried

  • Pitted or seedless prunes, apples, apricots, peaches, and dates.
  • Avoid raisins, which can cause choking.

Examples: Apple, Apricot, Avocado, Banana, Berry Grape, Kiwi, Mango, Melon, Nectarine, Orange, Papaya, Peach, Pear, Plum.

VEGETABLES2

Vegetables can be served hot or cold, but all will need to be cooked.

Fresh

  • Wash thoroughly.
  • Raw vegetables are difficult to chew, swallow, and digest.
  • Cook until tender and easily pierced with a fork.
  • Cut into small pieces, long thin strips, or grate.

Canned or Jarred

  • Be careful of the amount of sodium, choose low sodium if possible.
  • Rinse the can or jar before opening.
  • Can be served directly from the can, cut to appropriate size.

Frozen

  • Must be cooked until tender and cut to appropriate size.

**Be sure to discuss PHE or protein tracking an your child’s daily allowance prior to proving any new foods.

References:
1. American Academy of Pediatrics Web Site. Web. 03 Mar. 2010. http://www.aap.org/
2. Eat Right Stay Bright. Guide for Hyperphenylalanemia. L Bernstein and C Freehauf. Chapter 1. Pages 56-58

Eating Out on a Low Protein Diet

Although it can be easier to prepare low protein meals at home, this can restrict your work and social activities. Fortunately, many eating places are beginning to realize that an increasing number of people follow “special diets”.

Many of the larger restaurant chains state that they will try and cater for customers on a special diet whenever possible. To get further information from a particular company contact their Customer Service helpline or check their website.

RESTAURANT TIPS

  • Try and give advanced notice to the restaurant whenever possible
  • When explaining your diet, try not to get caught up in a long list of “I can’t have” foods
  • Offer a few ideas of possible dishes you can eat and recipes if necessary
  • Ask if you can bring in your own low protein products such as pasta or pizza bases if this is suitable

EATING ON THE GO
1. Cafes/sandwich shops

Small cafes that make things up from scratch can prove useful (especially if they get to know you!).  Ask if nutritional information is available to find out ingredients /protein content of items.

Possible snack ideas

-Salad
-Fruit
-Tomatoes on toast*
-Chips*
-Jacket potato* and butter

2. Fast food outlets

Some larger, well known fast food chains, provide nutritional content leaflets  for customers in the shop or online access nutrition information.

Possible snack ideas

-Salad (if available)
-Chips*
-Onion rings*
-Hash browns*
– Most veggie burgers are NOT suitable, as they are high in protein.

3. Cafeterias at work or school

Some cafeterias can be quite flexible so it is worth asking if they can cook or re-heat some of your low protein foods. If the cafeteria food choices are limited it may be easier to take a packed lunch in.

Possible snack ideas

-Salad/vegetables
-Fruit
-Baked Potato (avoid mashed potato as it is likely to contain milk)
 *Weigh out as usual

Note: Each condition may vary in tolerance for specific foods that contain protein, even if low in protein. Always speak with your metabolic dietitian or healthcare provider before adding new foods or changing your metabolic diet in any way.

Meet Alicia – My PKU Story

Hi, my name is Alicia.  I am an only child who was born three weeks early in November of 1959.  At this time they had no Newborn Testing for PKU here in the United States.  However, soon after I was born my Alicia - Share My Storymother became worried.  She stayed in continuous contact with my Pediatrician.  He had just read an article concerning  the “Wet Diaper Test,” a way to test someone for PKU.

My doctor called my mother and asked her to bring me into his office because he had a suspicion of what the problem might be.  He used a stick (kind of like a popsicle stick) which was put into my diaper that would turn a green color if the test was positive for PKU.  The same afternoon of the test, the doctor called my mother to tell her that the test was positive for PKU and he was referring us to a hospital in Augusta, Georgia for further testing.  I was confirmed to have classical PKU when I was 4 months old.  There were very few infants diagnosed in the United States at that time. As you can imagine, my parents were devastated and had no clue as to what PKU was or what would happen in the future.

I was placed on a strict low protein diet and PKU formula.  I can still recall not liking the taste of the formula.   My formula was very Jerry Davis - babyexpensive, even back in those days.  My parents had insurance, but the insurance wouldn’t pay for my formula and at times my father would be working 2 jobs just to help pay for it. My mother managed everything to do with my diet from making my formula to measuring my food intake.  I seemed to be doing great over the next several years. Then, suddenly, when I was 5 years old we received a phone call from my clinic and they wanted to see me.  At this time some thought that diet was not for life and I was taken  off my diet.  I can vividly recall what I had to eat for my first meal off diet.  It was at lunchtime and they brought me some BAKED HAM!  Little did I know that this was the first bite of a lifelong struggle for me. Jerry David youngerI remained off diet until I was 44 years old (2004).  It was at this time that I decided to look into going back on diet due to some problems I had begun experiencing.  I had lost my last job due to emotional outbursts and I felt that I needed to see about trying to get some help.  I spoke with my mother and she called my old clinic on my behalf.  I can remember my mother telling me that the doctor said “Get her in here, I don’t care about the money issue.  She needs help to get back on diet.”  So within just a few days I was on my way to clinic again for help.  It was determined at that time the best course for me was to go back on the diet after having been off it for so long.  It was going to be a struggle, but for my health I had to do it. My doctor slowly took me off of all of the bad foods I had been eating for so long and got me started on Xphe Maxamum™ from Nutricia.

First I stopped eating meat and cheese over the next couple of months and then the t a little later on.  I’m currently still on diet, although I do sometimes struggle trying not to eat all the bad foods which I’ve had in past years. I know that I feel better when I’m on diet and have more energy, not to mention that my husband can put up with me so much easier. I am just so thankful for a persistent loving mother, wonderful pediatrician, great support, and a patient loving Husband. I’ve always said that, “God could have made me normal, but he chose to make me with PKU and now what can I do with that?”  I choose to help make the road a little easier for those coming behind me by doing whatever I can to spread the word about PKU. 

CLICK HERE TO SHARE YOUR STORY

Book CoverPKU is Diet-for-LIfe. It’s Never Too Late!

CLICK HERE TO REQUEST A FREE COPY OF “YOUR GUIDE TO GETTING BACK ON THE PKU DIET” TODAY.

5-A-Day the Low Protein Way

For those following a low protein diet for a medical reason, FRUITS and VEGETABLES are a very important part of the diet. The good news is fruits and veggies are good for you and it is recommended to have a least 5 servings a day. The best of all they are typically naturally low in protein and a good source of vitamins and antioxidants. Antioxidants are chemical compounds (natural) found in food that keep cells within your body strong and work to fight off illness.

Here are some quick tips for eating 5 A Day the Low Protein Way

  • Included a tossed green salad as part of your dinner and lunch
  • Keep celery and carrot sticks pre-cut so they are available for a quick snack
  • Add fresh berries to your low protein cereal (Shop Loprofin Low Protein Cereal Now)
  • Roast vegetables in advance for quick eating and reheating during the week
  • Don’t leave home without it! It = An apple!
  • Enjoy canned fruit cocktal (packed in juice) as an evening snack or when summer fruit is out of season
  • Try something tropical like guava, pineapple, mango or papaya diced on a salad
  • Add fruit to your formula and blend for a PKU formula (or any other metabolic condition) smoothie
  • Freeze grapes for a cold, bite size treat

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.

Exploring Exotic Fruits and Vegetables

Exploring Exotic Fruits and Vegetables

You can tell summer is approaching by the increased variety of fruit and produce at your local supermarket. Exploring new fruits and vegetables can add excitement and variety to a low protein diet. Most fruits and vegetables are naturally low in protein and can be included in meal plans that limit phenylalanine or other amino acids.

Many fruits and vegetables we consume today were once considered exotic, but are now readily available. By exploring new items, you can open the door to new low protein foods, recipes and improved nutrition. Fruits and vegetables are a good source of fiber and other nutrients needed to maintain a healthy diet. Below are some of our favorite fruits and vegetables that can spark your culinary imagination, increase fiber intake and add a new twist to an ordinary low protein meal.

Star Fruit (Carambola) 1 medium (91g) Protein 0.9g PHE 33.7mg LEU 70mg Fiber 2.5g Kcal 28
Star Fruit, also known as Carambola, is a juicy tropical fruit grown in Thailand and throughout Southeast Asia. It is also grown domestically in Hawaii and Florida, so it is readily available at your local grocery store. This exotic fruit is both fun and nutritious. When you slice through the yellow shiny skin, it resembles a 5 pointed star and is packed with fiber and vitamin C. Add it to a fruit salad or enjoy it sliced for a sweet, crisp, and refreshing low protein snack.

Figs. 1 medium (50g) Protein 0.4g PHE 9mg LEU 17mg Fiber 1.4g Kcal 39
Figs are a great way to add flavor and fiber to a meal. Figs are sweet in taste and can be diced and tossed into a salad or made into a spread to add flavor and excitement to low protein bread or scones. Besides the delicious, sweet taste, one medium fig contains 1.4 grams of fiber, an abundance of minerals and only 9mg of PHE. They can be found fresh in season or dried all year round.

Jicama. 1 cup (130g) Protein 0.9g PHE 20mg LEU 33mg Fiber 6g Kcal 49
Jicama is an often forgotten low protein food. It is a root vegetable that is native to Mexico and Central America. It has a crisp texture and when sliced open resembles a raw potato, but with much less PHE. Complete a summer meal with sliced Jicama, chili powder and a splash of lime juice for a crunchy side dish at your next barbeque. Check out Celebrity Chef, Bobby Flay’s recipe for Jicama Slaw that can be found on the food network website at www.foodnetwork.com.

Kiwi Fruit. 1 medium (76g) Protein 0.8g PHE 21mg LEU 43mg Fiber 2.6g Kcal 46
Kiwi Fruit is a good example of an exotic fruit that has become more available in local food markets. Also known as Chinese Gooseberry, once native to China, Kiwi is now grown in New Zealand, Israel, Italy and domestically in California. This little green fruit makes a great addition to fruit salads and can be diced and tossed over greens to add flavor to a salad.

Mango. 1/2 cup (83g) Protein 0.4g PHE 14mg LEU 26 Fiber 1.5g Kcal 54
Some call mango the king of tropical fruits. We call it a great low protein snack. Mango can range in colors but all have a sweet, soft texture when ripe. You can be creative with this fruit and add to low protein rice, splash with spicy seasonings or chop into a salad.

The following recipe was created by Chef Birch DeVault, MEd, Department Chair of Culinary Arts at Johnson & Wales University, Denver, CO

JICAMA AND MANGO SALAD

NUTRITION:
Per Serving: 1/8th recipe Protein: 1.4g PHE: 37mg LEU: 53mg Kcal: 125

Ingredients:

  • 2 small (730g) jicama, peeled, cut into julienne strips
  • 3 cups (495g) mango, peeled, sliced
  • 1 each (14g) jalapeno, seeded, diced fine
  • 1 each (80g) red onion, peeled, minced
  • 1 clove (3g) garlic, minced
  • 1.5 fl oz orange juice
  • 1 fl oz lemon juice
  • 2 each (152g) kiwi, peeled, sliced
  • 2 tablespoons (27g) olive oil
  • To Taste salt and pepper
  • 1/2 cup (8g) cilantro – chopped

Method of Preparation:

  1. Mix garlic, orange and lemon juice, cilantro and olive oil. Season with salt and pepper.
  2. Add remaining ingredients, toss lightly.
  3. Divide in 8 servings and Enjoy!

Click here to view More Low Protein Recipes

Nutrition information obtained from the following sources: USDA nutrient database; Low Protein Food List for PKU by Virginia Schuett; The Food Processor, ESHA Research; MSUD Foodlist, Emory University; Manufacturer’s packaging. Household measurements are approximate, for greater accuracy use a gram scale.

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.