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.
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 Large intestine--leftover food is
are used to make new proteins or changed pushed through and water is
to other amino acids absorbed
Blood stream-- amino acids are carried Rectum, anus, and out--the body rids
by the blood to parts of the body that itself of waste products it cannot use
need them
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
- Show the picture of family on the Gene Scene handout.
- Explain that for every characteristic about them, everyone has two genes.
- This is true for PKU too.
- 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.
- 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.)
- 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.
- 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.
- 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.
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.
- Cut out the title on page one and place it on the table at the top of your playing area.
- Cut out the five columns of true/false statements (from pages 4-8) and “laminate” them with clear contact paper.
- Lay out the columns of true/false statements under the title.
- Cut out the 20 squares of paper using the grid provided on page one.
- 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.
The genotype of the non-PKU siblings is not known-they could be carriers or non-carriers 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.
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
v Invite the parents to join you if they have not done so for the game.
v Give PKU Facts for Now, Facts for the Future for later review.
v Give Maternal PKU Facts of Life---for parents of high school girls
v Give 15 Years on the Diet and Still Counting
v Give the Introductory Letter for Parents
v 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 6
Session D-1, Ages 15-18
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