Download Session Guide D

Know to Grow
Session D1
Purpose:
1. Review PKU science including protein digestion, phe metabolism, genetics, and
maternal PKU using hands-on experiences.
2. Review need for formula, reasons for high blood phenylalanine levels, and risks of
high blood phenylalanine levels.
Supplies:
1. To review PKU science:
For protein digestion:
 Digestion Puzzle Frame, Handout D1-a
 Digestion Puzzle Pieces, Handout D1-b
 All of the Pieces in Place, Handout D1-c
 Scissors
 Restickable Adhesive Glue stick
 15-20 multicolored paper clips linked together
For phe metabolism:
 30 paper clips, medium sized in a single color
For genetics:
 The Gene Scene, Handout D1-d
 Post-it® PageMarkers (or other small Post-it®-type notes) in two colors
 Pen
For Maternal PKU
 Making Up Your Mind about Maternal PKU, Handout D1-e.
2. To review need for medical food, reasons for high blood phenylalanine levels, and
risks of high blood phenylalanine levels:
 Directions for Phe Facts Four-in-a-Row Game, Handout D1-f
 Phe Facts Four-in-a-Row Game Pieces, Handout D1-g (prepare game “board” in
advance, instructions are below)
 Clear Contact® Paper
 “overhead” or washable markers
 scissors
3. For Recap
 PKU: Facts for Now, Facts for the Future (booklet)
 Maternal PKU Facts of Life, Handout D1-h, Family Letter
 15 Years on the Diet and Still Counting, Handout D1-i, Family Letter
 Handout D1-j, Introductory Letter for Parents
Review PKU Science
What to Say
I’m going to put you to work today. And I’m not even going to pay you!
Would you please write “PKU” on six of these PageMarkers and “working” on six
PageMarkers in this other color?
I’ll get everything else set up.
Session Guide
Session D-1, Ages 15-18
1
Know to Grow
What to do (These topics have most likely been introduced to the youth at some time
previously. Move through these topics quickly and enthusiastically, stopping to review
or emphasize as necessary.)
Ahead of time, lay out handouts, scissors, restickable adhesive glue stick, and paperclips
linked together, in an organized way so you can move easily from one
activity/demonstration to the next.
If questions are asked about what the supplies are for be vague in your answer at this
point. Build some mystery. Appeal to the youth’s natural curiosity.
Protein digestion
Ask the youth to cut out the organs (or have them precut in the interest of time) on the
Digestion Puzzle Pieces handout. Apply restickable adhesive to the back of each organ
and ask the youth to place the organ in the spot on the Digestion Puzzle Frame handout
where the youth thinks it should go.
Correct the placement of organs as necessary referring to All of the Pieces in Place
handout.
Then, trace a path from mouth to esophagus to stomach to small intestines. Make a
“fork” in the road at intestines. With one arrow going to the liver and one going to the
large intestines, rectum, anus, exit (toilet if you want to be a little silly).
Explain that with PKU we are particularly interested in how the body uses one nutrient or
part of food---protein. A list of digestive organs and their function as it relates to protein
digestion follows.
Mouth
grind, moisten
Esophagus
pushes food down, helps keep it down
Stomach
mixes food with juices that break food into tiny, microscopic
pieces. It is here that protein, a long chain, gets broken into parts-amino acids
Small intestine
amino acids, including phe are absorbed into the body
Liver--amino acids, including phe
are used to make new proteins or changed
to other amino acids
Large intestine--leftover food is
pushed through and water is
absorbed
Blood stream-- amino acids are carried
by the blood to parts of the body that
need them
Rectum, anus, and out--the body rids
itself of waste products it cannot use
Session Guide
Session D-1, Ages 15-18
2
Know to Grow
Along with the Digestion Puzzle you could show a long chain of multicolored paper clips
which represent a long protein chain. Show where it is taken apart (mouth and stomach),
absorbed as pieces (small intestine), and then put back together in a new way (liver).
Phe metabolism
Use the 30 single colored paper clips for this activity.
Review that phe is changed to tyrosine by an enzyme.
The youth will be the enzyme and the paperclips will be the phe that will be changed to
tyrosine.
To demonstrate the efficiency of a working enzyme have the youth change the “phe”
paper clip to tyrosine by unbending the straight part of the paper clip using both hands.
As you feed lots of phe-paperclips to the enzyme-youth make the point that a working
enzyme can handle lots of phe. Someone who does not have PKU has lots of working
enzyme and does not need to limit phe.
Then demonstrate the effects of a non-working enzyme or limited amounts of working
enzyme by having the youth change the phe paperclip to tyrosine by unbending the
straight part of the paperclip using only one hand.
Feed lots of phe paperclips to the youth and explain that when there is only a small
amount of working enzyme the phe builds up. That is what happens with PKU.
To make up for the enzyme not working, show what happens when only a small amount
of phe is fed to the enzyme-it doesn’t build up! That is why phe is limited in the diet for
PKU.
Genetics
1. Show the picture of family on the Gene Scene handout.
2. Explain that for every characteristic about them, everyone has two genes.
3. This is true for PKU too.
4. The labeled Post-it notes represent genes for PKU. The working gene makes the
enzyme that changes phe to tyrosine. The PKU gene does not.
5. The youth’s parents are carriers for PKU. Ask the youth to choose two genes for the
parents to show they are carries for PKU. (Each parent should receive one working
gene and one PKU gene.)
6. Ask the youth to show inheritance of two PKU genes, which results in a child with
PKU, by taking the PKU gene from each parent and pressing it onto one of the
pictures of a child.
7. If the youth has siblings who do not have PKU you can show the other possible
combinations for passing on the working and PKU gene from parents to children.
8. If the youth has questions about his/her future children show the possibilities. Give
the appropriate adult figure (based on the youth’s gender) 2 PKU genes. For the first
example give the spouse one working gene and one PKU gene to represent a carrier.
In this case the offspring have a 50-50 chance of either being a carrier or having
PKU. In the second example the spouse is a non-carrier for PKU and all of the
offspring will be carriers but none could have PKU.
Session Guide
Session D-1, Ages 15-18
3
Know to Grow
Maternal PKU
For girls only review Maternal PKU issues with them using the Making Up Your Mind
about Maternal PKU, Handout D1-e.
1. Highlight adverse affects of high blood phenylalanine levels during pregnancy (listed
in the lower left corner of the inside page) and the three spheres for decision-making:
sex, diet and pregnancy.
2. Emphasize making decisions before being forced to; e.g. deciding what to do about
sex before getting serious with someone, deciding about birth control before
becoming sexually active, deciding about diet before the crisis of an unplanned
pregnancy.
3. Do not read pamphlet word for word.
Review need for formula, reasons for high blood phenylalanine levels, and risks of high
blood phenylalanine levels
What to say
I’ve got a game to review what you know about PKU and the diet.
Would you like to play with just the two of us or should we ask your parents to join in
too?
What to do
To make the Game boards:
Use handout D1-f.
1. Cut out the title on page one and place it on the table at the top of your playing area.
2. Cut out the five columns of true/false statements (from pages 4-8) and “laminate”
them with clear contact paper.
3. Lay out the columns of true/false statements under the title.
4. Cut out the 20 squares of paper using the grid provided on page one.
5. Cover each statement box with a square of paper.
Play Phe Facts 4-in-a-Row by following the instructions below.
1. Give each player a wipe-off (overhead) marker of a different color.
2. Youngest player goes first.
3. First player chooses any square to uncover, reads the statement, and responds either
true or false. If the statement is false the player must explain what is false about it.
4. If the player is correct s/he makes an “X” over the statement with his color marker.
5. If the player is incorrect, place the square of paper back on the statement box.
6. Then the next player repeats the process.
7. Players attempt to get four “Xs” in a row by correctly stating true or false. The first
one to do so wins!
8. If there are many covered boxes at the end of the game change the position of the
columns and play again.
Answers to the true/false statements in Phe Facts Four in a Row:
Genetics
F
The siblings of someone with PKU are all carriers for PKU.
Session Guide
Session D-1, Ages 15-18
4
Know to Grow
The genotype of the non-PKU siblings is not known-they could be carriers or noncarriers of the PKU gene.
T
The parents of someone with PKU are both carriers.
F
The children of someone with PKU will all have PKU.
If the spouse of the person with PKU has PKU all of the children will have PKU.
If the spouse is a carrier for PKU then there is a 50/50 chance of the children
being carriers or having PKU.
If the spouse is a non-carrier (the most likely situation) then all of the children
will be carriers for PKU.
T
The children of someone with PKU will either be carriers or have PKU.
“Milk Madness”
F
PKU formulas contain lots of phe so kids with PKU will grow well.
PKU formulas contain no phe.
T
PKU formulas contain lots of vitamins and minerals.
F
PKU formula is all I ever need to eat or drink.
PKU formulas contain no phe and therefore are not nutritionally complete.
Natural food sources of phe are needed in the diet.
T
Skipping formula for a few days will probably make my phe level go up.
Why High?
T
Being sick may make my phe level go up.
F
Being very active and not getting enough sleep can make my phe level go up.
These activities have no known effect on blood phe levels.
T
Eating too many “yes” foods can make my phe level go up.
F
The best phe levels are between 6 and 14 mg/dl.
The optimal blood phe range is generally considered to be between 2-10 mg/dl.
This range may vary depending on the age of the patient and individual PKU clinic
policy.
Maternal PKU
F
An unborn baby will always die from high phe levels in a pregnant woman with
PKU.
Miscarriage can result, but typically high phe levels result in low birth weight,
poor growth, developmental delay and congenital heart defects.
F
High phe levels in the mother during pregnancy may cause the brain and head of
her baby to be small but this is OK.
High phe levels during pregnancy may cause microcephaly (small head size) in
the offspring, which results in developmental delay, a serious ongoing challenge. This is
not OK!
F
It’s OK to have high phe levels at the end of pregnancy.
High phe levels at any time in the pregnancy are toxic to the fetus.
T
The best phe levels for a pregnant woman with PKU are between 2 and 6 mg/dl.
Consequences
T
My brain will have abnormal changes if my blood phe levels are high.
Session Guide
Session D-1, Ages 15-18
5
Know to Grow
These changes are detected on MRIs.
F
Blood phe levels have no effect on how I do in school.
Elevated blood phe levels over time have resulted in poor school performance.
Short term memory, concentration, and the ability to organize and plan seem to
be affected.
T
I may feel irritable if my blood phe levels are high.
T
It’s harder to concentrate and pay attention for a long time if my phe levels are
high.
Recap
 Invite the parents to join you if they have not done so for the game.
 Give PKU Facts for Now, Facts for the Future for later review.
 Give Maternal PKU Facts of Life---for parents of high school girls
 Give 15 Years on the Diet and Still Counting
 Give the Introductory Letter for Parents
 Ask for questions.
Evaluation
Use the PHE-Quest cards for Session D1 to evaluate knowledge gained or use them at the
beginning of the next session to determine if review is needed.
Session Guide
Session D-1, Ages 15-18
6
Know to Grow
Session D2
Purpose:
1. Practice food preparation skills with a basic low protein food: pizza crust
2. Develop a plan for mixing formula
3. Practice meal planning including long-term planning and grocery lists
4. Incorporate variety into the diet
Note: this session is quite long and will probably take about one hour to get through
the materials. If you do not think the youth is up to that much education in a single
sitting either split the session or prioritize the topics and include only those of
greatest worth to the youth.
Supplies:
1. For making pizza crust:
 Recipe for Quick Pizza Crust, Handout D2-a
 Now We’re Cookin’, Handout D2-b
 Ingredients: Wel-plan Baking Mix, baking powder, salt, vegetable oil, yellow
food coloring, and pizza sauce (such as Pizza Quick).
 Note: Ask the youth to bring his/her favorite pizza topping to clinic.
 Equipment: gram scale, bowl, liquid measuring cup, 9-inch pie pan, spoon, fork,
knife, plates for serving and eating.
2. For making a mixing plan for formula:
 Giving Mixing a Second Thought, Handout D2-c
3. For meal planning
 Building a Meal, Handout D2-d (make three copies of this)
 The Quick Fix, Handout D2-e
 Bag of wrapped hard candies
4. For making a grocery list station:
 Medium sized notepad
 Sharp scissors
 Tape
 One piece of cardboard (the back of a legal pad will do just fine)
 Markers
 Flat magnets with adhesive on one side or double stick padded tape
5. For increasing variety
 Try It on Your Diet, Handout D2-f
 I Tried It!, Handout D2-g
 Low Protein Food List
 Low Protein Cookbook
6. For Recap
 Meal Planning a Parent’s Perspective, Handout D2-h, Family Letter
Session Guide
Session D-2, Ages 15-18
1
Know to Grow
Preparing a low protein food
What to say
Many kids with PKU eat a low protein food with every meal.
Low protein foods fill them up so they can save their exchanges (mg of phe) for bigger
portions of fruits and vegetables.
Today we’ll make low protein pizza crust that is quick, easy, and low in phe. You can
then experiment with different toppings.
What to do
Use the recipe (Handout D2-a) and Now We’re Cookin’ (Handout D2-b) to organize your
ingredients and equipment.
Prepare the pizza crust according to the recipe.
Top with 9 Tablespoons of Pizza Quick or Ragu Meatless Spaghetti sauce.
If the youth brought a topping add it and figure in the additional exchanges (mg of phe).
Mixing plan
What to say
While we’re waiting for the pizza to bake let’s take a few minutes to talk about the one
food that you eat that is high in protein, but low in phe---your “milk”.
What to do
Use the Giving Mixing a Second Thought handout to guide your discussion of mixing
medical food.
The goal of this activity is to have the youth think through and evaluate the process and
the result (the “milk”) of mixing.
Note: Much of the mixing procedure may be done out of habit (e.g. I always make it in
the morning) but the desired result may not be achieved (e.g. the youth likes his milk icy
cold so never drinks the milk till after school). This example shows that the habit
(mixing in the morning) is not contributing to easing adherence to the diet. The
following could result: “I don’t drink my milk until after school because it’s not cold
enough, but I’m really hungry in the morning and then I end up eating too much phe at
lunch.” The challenge for the clinician/educator is to remain non-judgmental while
reviewing medical food mixing schemes. Many variations can lead to a workable
situation for the individual and the family. Good luck!
Meal Planning
What to Say
Lots of thought goes into planning what to eat. For some meals (often breakfast) many
people eat pretty much the same thing every day.
Do you do this?
This makes it easy to know how many exchanges (mg of phe) you have used and how
many you have left.
Sometimes lunches, especially on school days end up being pretty similar.
Is this true for you?
It is the evening meal or weekend meals that tend to take the most planning.
Session Guide
Session D-2, Ages 15-18
2
Know to Grow
One reason for this is that the situation at suppertime and weekends can change a lot.
Are you eating alone or with others?
Did you use nearly all your exchanges (mg of phe) by supper? E.g. did you eat
lots of fries out for lunch at the mall?
Who’s doing the cooking?
What to do
Use Building a Meal, Handout D2-d to plan three suppers.
Planning the main meal depends on
1) how many exchanges (mg of phe) are available
This is the “foundation”
2) the low protein food on which the meal is based
which is portrayed
3) whether the youth will be eating with others and therefore
graphically on the handout.
incorporating items from their meal into his or hers or on his/her own
4) who is cooking.
Use the Building a Meal handout three times using the following scenarios.
You can assume for your discussion that a variety of low protein foods and ingredients
are available. First answer the foundation questions. Then plan, starting with the low
protein food, then add in leftovers (if desired) and continue filling in the circles in the
“plate” above the foundation as compliments the rest of the meal and as exchanges (mg
of phe/day) allow.
Scenarios:
1) The youth has 7 exchanges (105 mg phe), is eating alone, and cooking for him/herself.
The youth can choose the low protein food. (The youth can call the shots here. The
limiting factor in this scenario is the relatively small number of exchanges (mg of phe), so
the meal might best be built based on the choice of the low protein food.)
2) The youth has 10 exchanges (150 mg phe), is eating with the family, and a parent is
cooking hamburgers and zucchini on the grill and microwave fries. (Possibilities include:
grilling some low protein garlic bread too and eating the zucchini and fries, putting some
other vegetables on the grill, or making something independent of the family.)
3) The youth has 8 exchanges (120 mg phe), is eating alone after play practice, and there
are leftover stir-fry vegetables from the family meal. (Possibilities include: leftover
vegetables on low pro pasta, rolled in low pro tortilla with salsa, on low protein rice, or
skip the vegetables all together and have a sandwich, low pro pizza, a salad, etc.)
5) Or apply this exercise to a common situation at home.
Check on the pizza if you have not already done so, and if done, sit down and enjoy it.
Planning quick meals
What to say
Whether you or one of your parents is cooking the main meal there are times when it’s
really helpful to have some quick and easy meals up your sleeve.
Here’s my challenge to you:
In three minutes think of as many quick meals as you can that meet these two criteria:
1) it takes less than 20 minutes to prepare
Session Guide
Session D-2, Ages 15-18
3
Know to Grow
2) it incorporates a low protein food into the meal. Assume, if applicable, the
low protein food is premade (e.g. low protein pizza crust and tortillas are in
the freezer)
For each meal idea you come up with you get one piece of candy.
Take a minute to think.
Ready, Set, Go!
What to do
Use Quick Fix, Handout D2-e to record all of the ideas generated in the brainstorming
session. Do not veto any idea at this point.
Give a piece of candy for each idea as it is offered.
Some ideas to get the creative juices flowing:
Pasta with any topping-spaghetti sauce, grated low pro cheese, cream soups, vegetables
Sandwich on low pro bread
Pancakes
Soup, premade in the freezer
Burritos
Salad and low pro garlic bread
Low pro muffins and fruit salad
Low pro casseroles, premade in the freezer
Low pro pancakes and orange slices
Etc.
When three minutes are up go through the list and star any ideas which require prepreparation of certain foods to make it a quick meal.
Discuss a plan for preparing these foods ahead of time (i.e. pre-mixing ingredients for
pancakes).
List any special ingredients that are needed.
Making a grocery list station
What to say
Doing things on your own requires good communication, because you are still part of a
family.
In order for you to be more independent in cooking you need to let the family shoppers
know what foods and ingredients you need.
If you see a new recipe or idea in the National PKU News (like the low pro cheese), you
need to let someone know that you need a certain ingredient before you can make
something with it (like a taco).
Who does the grocery shopping in your family?
A grocery list helps the shopper know what unusual or special foods you may need.
This is also helpful for letting the family shopper know if you have eaten the last of your
favorite fruit snacks (or whatever) and you need more.
A grocery list will not guarantee you will get the items you request--there is a family
budget to think about too--but it helps you work with your family to stay organized so
you get as many of the foods that you want and need as possible.
Session Guide
Session D-2, Ages 15-18
4
Know to Grow
cutting line
What to do
Have the supplies for making the grocery list station assembled.
Cut a horizontal slit, one third of the way down the piece of cardboard
that is slightly wider than the notepad.
Slide the back page or pages of the note pad through the slit.
Note
Tape the pages in place on the back of the cardboard.
pad
Ask the youth to decorate and label the board in any way they like.
This could be straightforward e.g. “grocery list” or free-spirited
e.g. “Yo! Write your groceries here!”
Planning for variety
What to say
Sometimes we all get in a rut eating similar things all of the time. That’s OK, but it
might lead to frustration and feelings of being deprived. That’s not OK.
This next activity will get us started thinking about food in new ways to give a little
variety to your life.
What to do
Work through the questions on the Try It on Your Diet handout to do some brainstorming
and decision-making about some new food combinations. Use the low protein food lists
or cookbooks for ideas.
Note: The point here is creative thinking--- not arm wrestling the youth to try a new food.
The goals are: 1) to do creative thinking 2) to consider options within some parameters
(in this case phe content and what they are willing to try). Perhaps the parents will be
stimulated by an idea that you generate in this exercise and follow through with it.
Possible ideas are as follows:
Topping
Serving size
mg phe/exchanges
Pizza crust: *Taco sauce
2 TB
10/1
with black olives
¼c
(34 g)
10/1
*Barbecue sauce
2 TB
10/1
with onions
4 TB (42 g)
10/1
and mushrooms
½c
(35 g)
28/2
*Pizza sauce
2 TB
20/1½
with low pro cheese
15 g
14/1
*Sautéed zucchini
¼c
(44 g)
17/1
and onions
4 TB (42 g)
10/1
and green peppers
2 TB (17 g)
5/½
*Ranch dressing (eat chilled)1 TB
free
with chopped green onions 2 TB (12 g)
8/ ½
and red peppers
¼c
(17 g)
10/1
and cucumbers
¼c
(36 g)
8/ ½
Bread:
*Mayonnaise
1 TB
8/ ½
or Miracle whip
2 TB
free
and cucumbers
¼c
8/ ½
Session Guide
Session D-2, Ages 15-18
5
Know to Grow
*Low pro peanut butter
2 TB (35 g)
and jelly grilled in a sandwich maker 2 TB
*Russian dressing
2 TB
with lettuce
¼c
(14 g)
and tomato
½c
(80 g)
*Turnip
½c
(65 g)
and mayonnaise
1 TB
Tortilla
*Shredded lettuce
¼c
(14 g)
with Catalina dressing
2 TB
*Low pro rice, cooked
½c
with taco seasoning
1 TB
*Stir-fried vegetables: pea pods 15 pods (45 g)
and onions
4 TB (42 g)
and mushrooms
½c
(78 g)
*Apple butter
2 TB
Pancake
*Fruit syrups
¼c
*Different pancake recipes
*Jelly
2 TB
*Honey and butter
1 TB each
*Chive cream cheese
1 TB (12 g)
(on a pancake that is less sweet)
Pasta
*Italian dressing
2 TB
*Catalina dressing
2 TB
and diced vegetables
varies
*Stir-fried vegetables
(see above)
*Olive oil
2 TB
and garlic salt
dash
Have fun in your creative thinking. No idea is too weird!
8/ ½
free
free
6/ ½
18/ 1
11/ 1
8/ ½
6/ ½
free
5/ ½
free
41/ 2½
10/1
28/ 2
free
free
free
free
50/3½
free
free
free
free
Recap
 Invite parents to join you.
 Give any leftover pizza to take home.
 Review conclusions generated from Giving Mixing a Second Thought.
 Show the three meals that you planned and the Quick Fix Ideas.
 Show the Grocery List Station and make a plan for where it will be hung at home.
 Show ideas generated from Try It On your Diet.
 Give I Tried It! and encourage the youth to send the note to the clinic with the next
blood sample.
 Give Meal Planning-A Parent’s Perspective
Evaluation
Use the PHE-Quest cards for Session D2 to evaluate knowledge gained or use them at the
beginning of the next session to determine if review is needed.
Session Guide
Session D-2, Ages 15-18
6
Know to Grow
Session D3
Purpose:
1. Prepare a basic low protein food: tortillas
2. Develop a monitoring plan for obtaining blood samples and recording intake
3. Plan for social eating including fast food and spontaneous food choices
Supplies:
1. For preparing low protein tortillas
 Recipe for Low Protein Tortillas, Handout D3-a
 Now We’re Cooking, Handout D3-b
 The Weigh to Way, Handout D3-c (have available if youth needs a review of the
use of the gram scale)
 Ingredients: Wel-plan Baking Mix, dry taco seasoning, salt, vegetable oil, yellow
food coloring, diet margarine (or any margarine with no phe), cinnamon and
sugar.
 Equipment: bowl, fork, liquid measuring cup, gram scale, wax paper, rolling pin,
sharp knife, skillet and stove or electric fry pan, foil, cookie sheet. If available, a
tortilla press is fun and results in a good product.
2. For developing a plan for obtaining blood samples
 What’s the Plan, Stan? Handout D3-d
 Stickers-small round “pricing” stickers
 Pen
3. For developing a plan for monitoring intake
 Phe Check-off, Handout D3-e
 A sheet of construction paper
 Clear Contact® paper
 Washable marker
4. For planning for fast food, spontaneous, social and travel eating
 Fast Food Frenzy, Handout D3-f
 Fending Off Vending Blunders, Handout D3-g
 Group Grub, Handout D3-h
 On the Road, Handout D3-i
5. For Recap
 Food on the Fly, Handout D3-j, Family Letter
Preparing low protein tortillas
What to say
To keep blood phe levels low, kids your age usually have to eat one or more special low
protein foods each day-often at every meal.
Which ones are your favorites?
A low protein food that you can eat in a lot of different ways is tortillas.
You can’t buy them premade so lets give them a try!
What to do
Session Guide
Session D-3, Ages 15-18
1
Know to Grow
Use the recipe (Handout D3-a) and Now We’re Cookin’ (Handout D3-b) to organize your
ingredients and equipment.
Prepare the tortillas according to the recipe. Make the dessert option at the bottom of the
page.
Use the gram scale to weigh out the baking mix and assess the youth’s skill.
If more practice is needed, use The Weigh to Weigh Handout D3-c to review use of the
gram scale.
Sit and enjoy the tortilla roll-up.
Developing a plan for monitoring blood phenylalanine levels
What to say
There is only one way to know if your diet is giving you the right amount of phe--taking
a blood sample.
Cooking special foods, keeping track of what you eat, drinking your “milk” will
contribute to safe levels, but they don’t tell us if the diet is right.
What was your last level?
How often do you take a sample?
How often are blood samples requested by the clinic?
What to do
Use What’s the Plan, Stan? Handout D3-d to make a plan for obtaining blood samples.
Decorate the garage sale stickers with slogans that will remind the youth to take a blood
sample. Examples: Blood today! POKE! Encourage the youth to put them on the family
calendar as a reminder to take blood.
Plan for monitoring phe intake
What to say
There are two pieces of information you provide to the PKU clinic staff that helps them,
help you, fine-tune your diet. They are: blood samples and diet records.
These two pieces of information go hand in hand.
If you send in a blood sample without a diet record there is no way for the doctor and
dietitian to give meaningful feedback if they don’t know what you ate. A diet record will
give information about how many exchanges (mg of phe) you ate, but it is just
information, not a tool, without a blood sample.
Keeping track of what you eat day to day and before blood samples can be a big job.
How do you keep track day to day?
How do you keep track before blood samples?
What to do
If the youth has no system in place for monitoring phe intake, and blood phe levels have
been high or inconsistent, the youth will benefit from a method of record keeping.
A systemized method is the Phe Check-off.
1) Have available the diet prescription and a three-day diet record (if possible).
2) Use typical foods listed on the three-day diet records to fill in the blanks under
Favorite Foods on the Phe Check-off. Or interview the youth for more ideas.
Session Guide
Session D-3, Ages 15-18
2
Know to Grow
3) Explain that the goal of the Check-off is to provide an easy means of recording
intake.
4) When the Check-off is completed, glue it to a piece of construction paper that is
slightly larger than the Check-off and cover the entire board with clear Contact®
paper.
5) The number of exchanges eaten at a meal can be tallied using a washable or overhead
marker. If the youth keeps track of phe intake by counting mg of phe, s/he can write
the total mg of phe eaten for a meal or snack under the appropriate heading.
6) Emphasize that it is the youth’s responsibility to fill in the check-off for all meals.
Use the Phe Check-off and the list of favorite foods to plan a menu for a day that fits the
youth’s diet plan (prescription) or use 300-350 mg phe (20-25 exchanges) for the purpose
of the activity.
Explain that the goal of the Check-off is not to have the youth eat the same thing every
day, but give a convenient, easy means of recording intake.
Planning for fast food, spontaneous eating and travel
What to say
You may have a typical way of eating that works well for you during the week.
But what about weekend, travel, or when you are just hanging out with friends and you
all get hungry.
What to do
Use the Fast Food Frenzy handout and the Low Protein Food Lists for planning a meal
and a snack at each of the types of fast food restaurants listed on the sheet.
If the youth is not acquainted with the food lists, describe how they are organized (by
food groups) and review the column headings.
The fast foods are listed under the Convenience Foods heading.
If the youth has not looked at the fast food category s/he might enjoy seeing what options
are available.
Preparing for spontaneous eating opportunities
What to say
Imagine this: you are riding your bike (or in the car) with your friends and they suggest
stopping at a convenience store (like 7-11) for a snack (ask if the youth has a place in
his/her neighborhood or town where kids often drop in).
You’ve got 2 dollars in your pocket. Your friends are all getting chips or candy bars.
What will you do? (ask rhetorically)
It helps to be prepared and know what foods to choose ahead of time.
The last thing you want to do is un-do your hard work and preparation by eating just
anything at a time when you didn’t plan on eating.
There will be more and more times that the chance to eat something you have not planned
will come up, and often there will be a friend innocently encouraging you to go ahead.
What to do
To help consider your options, walk over to a vending area or gift shop near the clinic.
Session Guide
Session D-3, Ages 15-18
3
Know to Grow
Use Fending Off the Vending Blunders to prepare the youth for unplanned eating.
At the vending area have the youth write in those foods he thinks are free foods and “yes”
foods. Back at the clinic look up the foods the youth wrote down and determine amounts
and exchanges (mg of phe) using a low protein food list.
Consider good choices for the future.
Reinforce the need to be ready to make free-food-only choices if s/he had a high
phenylalanine breakfast or lunch or if plans for a higher phe dinner were already made.
Group activities
What to say
You are now equipped to make some good choices when you are out and can buy a quick
snack. The situation changes when you are at someone’s home or in a big group.
The food offered may not be predictable (it is fairly predictable at a store or vending
machine).
Be prepared to choose from the options you have.
What to do
Review Group Grub, Handout D3-h. Emphasize options.
If time permits and/or this seems to be an especially relevant topic for the youth, use the
scenarios from on What Would You Do?, Handout C5-d.
Emphasize: Ask questions assertively (that is, get the information you need while
practicing politeness and respect).
Travel Ideas
What to say
Over the years you’ve had to figure out how to work the diet into a variety of situations
away from home. Will you go down memory lane with me and tell me some things that
have worked for you?
What to do
Work through On the Road, Handout D3-i.
Recap
 Invite the parents to join you.
 Wrap up any leftover tortillas to take home.
 Give the stickers for reminding to send a blood sample.
 If a Phe Check-Off was completed show the parents and review its use.
 Show ideas generated by Fast Food Frenzy, Fending Off Vending Blunders, and On
the Road.
 Give Food on the Fly.
Evaluation
Use the PHE-Quest cards for Session D3 to evaluate knowledge gained or use them at the
beginning of the next session to determine if review is needed.
Session Guide
Session D-3, Ages 15-18
4
Know to Grow
Session Guide
Session D-3, Ages 15-18
5
Know to Grow
Session D4
Purpose:
1. Apply problem-solving strategies
2. Practice rehearsed responses to questions assertively
3. Review diet discontinuation issues
Supplies:
1. For problem solving
 You Solve It!, Handout D4-a
 Questions to Ask, Handout D4-b
 Your Problem---Your Fix, Handout D4-c
 Problem Free Press, Handout D4-d
2. For practicing rehearsed responses
 On the Tip of Your Tongue, Handout D4-e
 Explain Game, Handout D4-f
 Scissors
 Brass fastener
3. For reviewing diet discontinuation issues
 Diet for Life, Handout D4-g
4. For Recap
 You’ve Come a Long Way Baby!, Handout D4-h, Family Letter
 Certificate of Completion, Handout D4-i
For problem-solving
What to say
One of the challenges of following the diet for PKU is that you have to adjust things in
your life all of the time.
You have to figure out different ways of doing things, especially with food, that many of
your friends don’t have to think about at all.
Sometimes the adjustment you make will be easy --like hot lunch at school left you
hungry so you bring your own.
But other situations are tougher.
It helps to have a strategy for confronting and solving problems that come your way.
What to do
Show the You Solve It handout in which the problem to be solved is inconsistent blood
phe levels.
Use the seven-step outline for problem solving to work out a solution.
For step 2 (Get information) use Questions to Ask, Handout D4-b to help you think up
options.
Then use Your Problem—Your fix, Handout D4-c, on which the youth writes in his/her
own situation that needs a solution.
Start the problem solving process together and go through step 4 (think of the pros and
cons of your options).
Encourage the youth to choose the best solution on his/her own.
Session Guide
Session D-4, Ages 15-18
1
Know to Grow
Give Problem Free Press. This will serve as a summary of your problem solving activity.
For practicing rehearsed responses
What to say
What question do most people ask when they find out you have PKU?
What do you say in response?
Having a ready answer can really help you be assertive.
It helps the other person understand what you need.
It also meets your need to not tell your life story while still sticking with your diet.
You can learn the skills to help you balance your needs and the needs of others.
Two of those skills are assertiveness and rehearsed responses.
Assertiveness means you have the confidence to let others know what you need in a
polite and respectful way.
Rehearsed responses can give you the words to use when you need to be assertive.
Just because you rehearse or practice the responses does not mean they are phony or fake.
It means you are ready and prepared to use them in any situation that comes up--they are
at the tip of your tongue!
What to do
Practice rehearsed responses from the On the Tip of Your Tongue handout.
First, have the youth ask you the questions and you respond. Then reverse roles.
Line up the two-page game board so that “You’re at the Top of Your Game” is at the top.
Then play the Explain Game to put the rehearsed responses into practice.
Possible answers to the questions in the Explain Game are as follows.
What is PKU?
PKU is a genetic disorder in which the person cannot use phe. The
phe builds up and is harmful to the brain. A special diet is needed
to treat it.
PKU?
Phenylketonuria
How do you get it? PKU is inherited from your parents.
2 carriers for PKU? The youth’s mom and dad.
Why weigh?
Foods that contain phe are weighed so that too much phe is not
eaten.
Why are you eating that? How about a burger?
The youth can use one of the rehearsed responses:
No thanks, I’m not hungry, It’s not on my diet, or I’m a vegetarian.
Why a special diet? You don’t need to lose weight!
Again a rehearsed response is appropriate such as:
I have PKU. It’s no big deal--it’s just how I eat.
How does phe build up in your blood?
Phe comes from foods you eat. The enzyme that changes phe is
not working. If too much phe is eaten, it cannot be changed and
builds up in the blood.
Reasons for “milk”: 1) Protein without phe, 2) fills you up, 3) gives nutrients for
growth including vitamins, minerals, CHO, and fat, and 4) helps
keep levels low
How does phe get into your body?
Session Guide
Session D-4, Ages 15-18
2
Know to Grow
Phe comes from protein in food. When you eat, you break apart
the protein into small pieces (amino acids). Phe is one of the
amino acids. It is absorbed into the body through the small
intestine.
Is that all you are having?
Yeah, I’m not hungry.
Thanks, I’m fine.
Try one of my famous brownies!
No thank you. Thanks, but I can’t eat them.
Fast Food
fries, sodas, taco chips, cookies, slurpies
Review issues of diet discontinuation
What to say
Following the diet has not always been easy.
When you were young your parents took all the responsibility for you and the diet.
There are hassles that go along with following it as well as great benefits to you.
As you approach adulthood you will have to make the decision to follow through with the
diet yourself and then assume all responsibility for it.
We urge you to stick with it.
What to do
Show Diet for Life, Handout D4-g.
Highlight the benefits to following the diet.
Discuss the “costs” of following the diet and jot those on the side of the handout.
Emphasize that there are two factors to consider: 1) the consequences of high levels and
2) the medical problems associated with going off the diet but not eating a nutritious
mixed diet. The later include nutrient deficiencies and subsequent neurological
problems.
Give appropriate support and praise for diet adherence to date.
Commit to support the youth into adulthood regardless of decision to continue diet or not.
Recap
 Invite parents to join you.
 Review problem solving steps.
 Review Rehearsed Responses and encourage the family to practice any especially
relevant ones.
 Review consequences of high levels and going off diet but not eating a nutritious
mixed diet.
 Give You’ve Come a Long Way Baby!
 Encourage for the future.
Evaluation
Use the PHE-Quest cards for Session D4 to determine if review is needed in the future.
Graduation
Give Certificate of completion with significant pomp and praise.
Session Guide
Session D-4, Ages 15-18
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