Introduction and Use of

PKU gelTM

GU

IDE

LIN

ES

1

Collaborators

Vitaflo dietitians in collaboration with Professor Anita MacDonald

(Birmingham Children’s Hospital), and Kathryn Moseley,

Assistant Professor of Pediatrics, USC/Keck School of Medicine.

Forward

Establishing good practices with medical food administration early in life

is essential. When choosing an appropriate medical food, it is important to

consider the age and development needs of the child. Feeding the young

child with PKU can be challenging. The child’s feeding skills and personality

are developing, and daily nutritional needs require attention. For optimal

growth and healthy weight gain, a child needs more phenylalanine-free

‘protein’ from medical foods and adequate energy. Finding a balance

between giving appropriate solid foods and maintaining sufficient intake

of phenylalanine-free medical food to meet protein requirements is also

challenging. As protein needs increase, a young child may struggle to drink

adequate volume in order to meet non-phenylalanine protein requirements.

Administering a concentrated second stage medical food from a spoon

is a good way to meet both the developmental needs of a young child,

and lower the volume of phenylalanine-free medical food needed to meet

protein requirements.

Parents need support with practical guidance and instructions to help with

the ‘day-to-day’ challenges of feeding. Following a systematic, stepwise

system for introducing PKU gel should help establish a good framework for

medical food intake in the young child, and build a good foundation for its

administration in future years.

Anita MacDonald

What is PKU gel? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Benefits of PKU gel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2

Why use PKU gel? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

Introducing PKU gel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4

Transition Plan for PKU gel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4

Preparation Guidelines . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6

Sample Meal Plan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8

Example Case Scenarios . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12

Appendix

Tips for Meeting Nutritional Needs

Tips for Introducing PKU gel at Home

PKU gel Preparation Guidelines

TABLE OF CONTENTS

Disclaimer

These guidelines should be read in conjunction with local guidelines for the dietary management of Phenylketonuria

(PKU). They are based on global best practices over the last 15 years for the introduction of solid foods and a more

concentrated second stage medical food (PKU gel).

They are for use by health care professionals working with young children.

The appendix contains information which may be shared with caregivers.

They are for general information only and must not be used as a substitute for professional medical advice or treatment.

The product information contained in these guidelines, although accurate at the time of publication, is subject to

change. The most current product information may be obtained by referring to product labels.

Use under medical supervision.

2

3

What is PKU gel?

PKU gel is for use in the dietary management of PKU from one year of age in North America. It is a concentrated, powdered, phenylalanine-free medical food, containing

essential and non-essential amino acids, carbohydrate and a complete range of

vitamins, minerals and trace elements.

PKU gel is designed to meet the nutritional and feeding needs of a developing

child with PKU, to ensure the goals of dietary management are met.

Benefits of PKU gel

Concentrated medical food

Mix to a low volume to help encourage a healthy appetite

for food and good feeding behavior

Encourages the transition from a bottle

Enables young children with PKU to developmentally progress

with less dependence on high volumes of medical food1

Similar consistency to other age appropriate foods

May aid acceptance particularly for the younger child

Flexible

Can be introduced and given as a spoonable semi-solid consistency

Can be given as a low volume drink

Is available in different flavors to meet individual taste preferences

Introducing PKU gel

Timing is important to the success of meeting milestones for accepting new

tastes and textures. Introducing a concentrated, second stage medical food,

while also maintaining metabolic control of blood phenylalanine levels within

treatment range are important.

Initially, PKU gel is introduced in small amounts. Over the age of one year, a

child can fully transition to PKU gel as their primary medical food. This transition

may encourage appetite for foods and aid metabolic control, while simplifying

the diet. The following chart illustrates how to introduce PKU gel in a stepwise

manner over time.

Why use PKU gel?

As protein needs increase and complementary feeding continues, meeting

daily nutritional requirements and achieving feeding milestones may become

challenging. Increasing protein needs require an increased volume of medical

food. The reliance on high volume bottle feedings leaves little room for an

appetite for complementary foods. Studies show that meeting protein needs

and adherence with protein substitutes is challenging, sub-optimal and/or

inconsistent.2–4 High volumes of medical food may delay the progression of

feeding development and adversely affect metabolic control.

PKU gel is designed to meet nutritional needs without adding volume to the

child’s diet. It is a concentrated, second stage medical food that can be given as

a spoonable semi-solid or low volume drink. This low volume decreases reliance

on high volume bottle feedings, promotes appetite for foods, and the semi-solid

consistency may better suit feeding development.

Continue to progress textures, introduce new foods

and variety within the diet

Encourage self feeding to develop independence

Encourage drinking from a cup to help reduce

reliance on high volume bottle feedings

Introduce low-phenylalanine foods from an

early age to aid future acceptance of a wide

variety of foods

Introduce PKU gel as a concentrated, second

stage medical food

Measuring out protein-containing foods/

phe-exchanges

General feeding considerations for the young child Additional considerations for PKU

One 24 g packet of PKU gel = 10 g Protein Equivalent (PE)

PKU gel is reconstituted with a small amount of water (30 ml) to make a

smooth, semi-solid consistency (i.e. similar to pudding) that can be taken

from a spoon. PKU gel can also be mixed with at least 80 ml of water to

make a low volume drink.

Available in unflavored, orange or raspberry

Presentation

4 5

n

Transition Plan for PKU gel

Introduce PKU gel Child is familiar with…

The amino acid taste

from other medical food

Eating from a spoon

Drinking from a sippy cup

or other cup

Begin to introduce

small amountsa

of PKU gel at the

same time every

day before foods

or other medical

food. Mix same way

each time and serve

immediately.b

Continue to offer a

variety of foods at each meal.

Continue to

monitor phe

intake.

Continue to

encourage

self feeding and

drinking from

a sippy cup or

other cup.

Offer water throughout the day.

Increase the amount of PKU gel given systematically

and as quickly as

the young child

accepts. Increasing

on a weekly basis is a

good place to begin.

Offer water after

PKU gel to ensure

adequate fluid

intake.

Adjust the volume of other medical food according

to weight, protein requirements

and total volume of PKU gel

consumed.

Continue to monitor

growth, weight gain

and assess intake.

Since PKU gel does not contain fat,

incorporate a source of long chain poly unsaturated fatty acids(LCPs).c

As the volume of

other medical food

decreases, the child’s

need for high energy

foods will increase.

Include additional low

protein foods into the

diet if necessary.

Begin to fully

replace other

medical food

with PKU gel.d

Total protein requirements can be achieved

through PKU gel

and other protein-

containing foods.

Divide dose of

PKU gel evenly

over three meals.

Progression from other medical food to PKU gel Progression of complimentary foods a This could be 1 teaspoon to half a packet depending on what is age appropriate.

b For detailed instructions on mixing PKU gel refer to the PKU gel Preparation Guidelines in the Appendix.

c For food sources of LCPs refer to Tips for Meeting Nutritional Needs in the Appendix.

d Aim for full transition over the age of one year.

76

3 Mix until smooth with a small spoon.

Full packet (24 g of powder) = 10 g PE

1 Empty full contents of PKU gel powder into a small feeding container.

Half packet (12 g of powder) = 5 g PE

3 Mix until smooth with a small spoon.

1 Measure out 12 g of PKU gel powder with a gram scale.

Preparation Guidelines

1 Measure out 5 g of PKU gel powder with a gram scale.

3 Mix until smooth with a small spoon.

2 Add 5 ml of cold water.

2 Add 1 tablespoon (15 ml) of cold water.

2 Add 2 tablespoons (30 ml) of cold water.

1 full teaspoon (5 g of powder) = 2 g PE

How to prepare PKU gel powder as a low volume drink:

2 Add at least 80 ml of cold water, secure lid and shake well until powder is dissolved.

Full packet (24 g of powder) = 10 g PE

1 Empty contents of PKU gel powder into a cup with a lid.

3 Drink immediately.

How to prepare PKU gel powder as a spoonable semi-solid:

T I P S When introducing PKU gel as a smooth semi-solid consistency it is best to reconstitute with the same amount of water each time. This will aid in acceptance as the child becomes familiar with the consistency. For ease and convenience it may be best to measure out PKU gel powder into a small feeding container so it is ready to feed when reconstituted, especially when starting off with small amounts at one time (i.e. less than one packet).

It is recommended that gram scales be used to accurately make up small quantities of PKU gel.

8 9

z

Food Intake: 400 kcal3.43 g intact protein 192 mg phe 14 g fat

Other Medical Food: 600 ml 420 kcal 13.5 g PE 20.4 g fat

PKU gel: 3 tsp 51 kcal 6.25 g PE

The patient weighs 7.5 kilograms (kg) and plots appropriately on the growth chart.

He takes a medical food mixture of standard infant formula and phe-free infant

formula. Phe levels are well controlled and within the accepted reference range.

His parents will be introducing solid foods soon and start the transition to PKU gel

with the guidance of their metabolic dietitian.

Current dietary regimen: The patient takes 30 ounces (900 ml) of medical food

mixture daily. The parents and the metabolic dietitian have agreed that cutting

back on some formula volume would allow for more successful solid food intake.

Initially a small amount (1 teaspoon) of PKU gel powder will be mixed with water

to make a spoonable semi-solid. PKU gel will be given once a day at meal time

before solid foods or other medical food are offered. The amount of PKU gel

powder and frequency will gradually increase over time until the patient reaches

the goal intake of 1 full packet of PKU gel per day. Throughout this period the

metabolic dietitian may adjust the medical food mixture recipe to ensure the

child’s protein needs are adequately met.

New dietary regimen: The patient now takes 1 packet of PKU gel powder mixed

with water to make a spoonable semi-solid throughout the day. The patient also

continues to take a mixture of his old medical food throughout the day. The

patient has done well with solid food introduction as PKU gel has allowed lower

liquid medical food volume intake, promoting his appetite for foods.

Scenario 1

1

EX

AM

PL

E C

AS

E S

CE

NA

RIO

S

Protein/g or PE/g Phe/mg kcal

Wake-up 200 ml medical food 4.5 140

Breakfast 1 tsp (5g) PKU gel 2 17

2 oz whole milk 2 100 40

¼ cup low protein cereal 1 54

4 large strawberries 0.32 12 24

Lunch 1 tsp (5 g) PKU gel 2 17

1 slice low protein toast 0.1 5 100

1 tbsp pureed avocado 0.29 14 23

½ cup cooked carrots 0.59 39 27

Afternoon 200 ml medical food 4.5 140 ¼ cup sliced pears 0.13 4 20

Dinner 1 tsp (5 g) PKU gel 2 17 Water – – ¼ cup cooked low protein pasta 3 37 1 tsp olive oil – 40

¼ cup sautéed, 0.28 14 35 chopped red bell peppers

Bedtime 200 ml medical food 4.5 140

Totals: 23.2 192 871

34.4 g fat (36%)

Sample Meal Plan

Estimated Requirements Actual Intake

Total kcals/d

Total Protein/d

Natural Protein Rx/d

T I P S

Consider incorporating food sources of LCPs in the child’s diet during the transition for adequate fat and energy intake.

For example: Avocado

Olive oil

Walnut oil720–918 (80–102 kcal/kg) 871 kcal

18.9–30 g/day (2.1–3.3 g/kg) 23.2 g

4 g (200 mg Phe) 192 mg

9 kg boy(25–50th percentile for The World Health Organization (WHO) Child Growth Standards)

Has just recently started PKU gelsee page 10 for full transition to goal medical food plan

Goal medical food plan

Wake up 200 ml of medical food

Breakfast 2 tsp PKU gel

Low phe foods

Lunch 1 tsp PKU gel

200 ml of medical food

Low phe foods

Afternoon Low phe foods

Dinner 2 tsp PKU gel + low phe foods

Bedtime 200 ml of medical food

10 11

The patient weighs 9 kg and plots appropriately on the growth chart. He takes

a medical food mixture daily. He has completed the transition to single and

combination pureed foods. His parents would like to encourage the progression to

more textures and gradually reduce reliance on bottle feeds. To aid in this transition

PKU gel powder will also be introduced.

Current dietary regimen: The patient drinks 25 ounces (750 ml) of medical food

daily. The patient also takes 100 mg phe of age-appropriate foods. Initially a

small amount (1 teaspoon) of PKU gel powder will be mixed with water to make a

spoonable semi-solid and given once a day at meal time before solid foods or other

medical food are offered. When the child accepts 1 full teaspoon (2 g PE) PKU gel

the metabolic dietitian may decrease the amount of the medical food mixture by

2 g PE. Each increase of 1 teaspoon PKU gel is matched by a 2 g PE decrease in the

medical food mixture until the patient has reached the goal of 1.5 full packets of

PKU gel each day.

New dietary regimen: Now the patient takes 1.5 packets of PKU gel powder with

age-appropriate solid foods throughout the day. The patient also enjoys PKU gel

powder mixed to a low volume drink. He also continues to drink 10 ounces

(300 ml) of medical food mixture, a much lower volume, allowing good intake of

a variety of age-appropriate solid foods.

Scenario 2

2

Scenario 3

3

The patient weighs 16 kg and plots appropriately on the growth chart. He takes

medical food daily. He has some intake of low protein and moderate protein foods

but seems to get too full from the volume of medical food he consumes throughout

the day. The parents would like to add more variety in his diet. The metabolic

dietitian has suggested the family gradually transition the patient from a high

volume medical food to PKU gel, to help encourage a healthy appetite for food.

Current dietary regimen: The patient takes 16 ounces (480 ml) of medical food

and 10 g of dietary protein from food daily. Initially half a packet of PKU gel powder

(5 g PE) will be mixed with at least 40 ml of water to make a low volume drink given

once a day at meal time before solid foods or other medical food are offered. When

the child accepts half a packet of PKU gel the metabolic dietitian may decrease the

amount of the medical food mixture by 5 g PE. Each increase of half packet of PKU

gel is matched by a 5 g PE decrease in the medical food mixture until the patient

has reached the goal of 2 full packets of PKU gel each day.

New dietary regimen: Now the patient takes 2 packets of PKU gel per day mixed

to a low volume drink. The switch from a higher volume medical food to PKU gel has

allowed for greater freedom with his food intake and less stress for the parents.

T I P S

Encourage adequate

fluid intake throughout

the day.

T I P S

PKU gel can be prepared and mixed into age and diet-appropriate foods.

For example: Unflavored PKU gel can be mixed with:

phe-free lemon pudding

applesauce

coconut milk yogurt

Goal medical food plan

Wake up 200 ml of medical food

Breakfast 2 tsp PKU gel

Low phe foods

Mid-morning Low phe foods

Lunch 2.5 tsp PKU gel

Low phe foods

Afternoon Low phe foods

Dinner 3 tsp PKU gel + low phe foods

Bedtime 100 ml of medical food

Goal medical food plan

Breakfast 1 packet PKU gel

Low phe foods

Lunch 1/2 packet PKU gel

Low phe foods

Afternoon Low phe foods

Dinner 1/2 packet PKU gel

Low phe foods

Guideline References:1 MacDonald A, Evans S, Cochrane B, Wildgoose J. Weaning infants with phenylketonuria:

a review. J Hum Nutr Diet. 2012;25(2):103–110.

2 Schulz B, Bremer HJ. Nutrient intake and food consumption of adolescents and young adults with phenylketonuria. Acta Paediatr. 1995;84(7):743–748.

3 MacDonald A, Harris G, Rylance G, Asplin D, Booth IW. Abnormal feeding behaviour in PKU. J Hum Nutr Diet. 1997;10:163–170.

4 Prince AP, McMurray MP, Buist NR. Treatment products and approaches for phenylketonuria: improved palatability and flexibility demonstrate safety, efficacy and acceptance in US clinical trials. JIMD. 1997;20(4):486–498.

Meal Plan References:1 Bunting KD, Mills J, Ramsey E, Rich S, Trout S. Texas Children’s Hospital-Pediatric Nutrition

Reference Guide. 10th ed. Houston, TX: Texas Children’s Hospital; 2013.

2 Sample Menu for a 1-year-old. Healthy Children from the American Academy of Pediatrics website. https://www.healthychildren.org/English/ages-stages/baby/feeding-nutrition/Pages/Sample-One-Day-Menu-for-a-One-Year-Old.aspx Last update: December 21, 2015. Accessed: January 21, 2016.

3 Singh RS, Rohr F, Frazier D, Cunningham A, Mofidi S, Ogata B, Splett PL, Moseley K, Huntington K, Acosta PB, Vockley J, Van Calcar SC. Recommendations for the nutrition management of phenylalanine hydroxylase deficiency. Genet Med. 2014;16:121–131.

4 Singh RS, Rohr F, Frazier D, Cunningham A, Mofidi S, Ogata B, Splett PL, Moseley K, Huntington K, Acosta PB, Vockley J, Van Calcar SC. Table 3: Recommended intakes of PHE, TYR and Protein for PKU. In PKU Nutrition Management Guidelines. 1st ed. Southeast Newborn Screening and Genetics Collaboration (SERC) and the Genetic Metabolic Dietitians International (GMDI). https://southeastgenetics.org/ngp/guidelines.php/87/tbls/0/0/PKU%20Nutrition%20Guidelines/ Version%201.11/List%20of%20Tables#tid_173 Published: March 2015. Last update: January 2016. Accessed: January 21, 2016.

5 National Nutrient Database for Standard Reference. Agricultural Research Service. United States Department of Agriculture website. https://ndb.nal.usda.gov/ Last update: November 30, 2015. Accessed: March 16, 2016

Appendix: Tips for Meeting Nutritional Needs References:1 Recipes. Cristine M. Trahms Program for Phenylketonuria from the University of Washington

website: http://depts.washington.edu/pku/recipes/ Accessed: March 15, 2016.

2 Low protein recipes for the PKU community. Cook for Love website: http://cookforlove.org/ Accessed: March 15, 2016.

3 PKU Recipes! New England Consortium of Metabolic Programs website: http://newengland consortium.org/for-families/phenylketonuria-pku/pku-nutrition/pku-recipes/ Last update: September 18, 2015. Accessed: March 16, 2016.1212

References

® Reg. Trademark of Société des Produits Neststlé S.A.