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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. 

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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.

Types of Formula

Did you know that there are over 60 different PKU formula world-wide?

Whether you have been drinking the same formula and maintaining excellent dietary control or if you are reading this in a effort to learn more to return to the PKU diet plan, understanding the ‘Types’ of formulas is a great way to get started and stay in the loop of what formulas are available.

Types of PKU Medical Food

The Drink – PKU Formula in a powdered form that you mix with water. This type of medical food typically provides a balance of fat, carbohydrate, phenylalanine-free protein, vitamins and minerals. Often it contains a good amount of calories to aid satiety and help people with higher caloric needs. Examples from Nutricia include Periflex Junior, Periflex Advanced and PhenylAde Essential.

The Low Fat Formula – This type of formula is a growing segment of the PKU marketplace. Since PKU is diet for life, as people age their calorie needs sometimes lessen. Older people with PKU often have a wider array of low protein foods they will eat which makes them less reliant on formula for satiety. Low or reduced fat formulas typically have less calories and are lower in volume so you can drink less to meet your daily PHE-free protein needs, which can aid adherence and help keep levels in check. Examples from Nutricia include Xphe Maxamum, Lophlex Powders (also in Liquid) PhenylAde40, and PhenylAde60.

The Ready to Drink – PKU formula that is pre-mixed and ready to drink is in high demand. Everyone leads busy lives and don’t want their formula needs to slow them down. This segment is growing just as fast as you can drink one of these single serve units. The ready to drink formulas are often low in fat, calories and volume. For example, PKU Lophlex LQ provides 20 g of protein equivalent (PE) in only  4.2 fl oz. Ready-to-Drink (RTD) PKU formula are quick and easy to drink so you so you can get on with your day.

The Fortifier – Concentrated phenylalnine-free amino acid powder that can be added to any low protein food or drink you already enjoy. It can also be added to ‘Drink’ formula to increase protein content without added formula. Examples for Nutricia include PhenylAde Amino Acid Blend and PhenylAde MTE Amino Acid Blend (Minerals & Trace Elements)

The Tablets – Not everyone has an easy time drinking their formula. But the importance of consuming medical food (formula) and the consequences of not are is well known. For this reason there are formulas that are in tablet form. You would need to ingest quite a few to meet your full daily phe-free protein needs, but for some this is a better alternative than drinking formula.  You can choose to just replace 1 serving of liquid formula per day with tablets.  Example, just 12 tablets of Phlexy-10 Tablets replace 10 g PE of liquid formula. Talk to your metabolic professional if you are struggling with formula to see if a tablet or a combination of formula and tablets will work for you.


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 based on an excerpt from a book written by Sandy with the help of others titled: My PKU Toolkit: A Transition Guide to Adult PKU Management.