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Final Nutriton Diet Presentation
1. The Phenylalanine restricted diet
for PKU
Presented by Madeline Caver, Laura
Collins, Myra McVay, and Laura Pagan
June 30, 2013
2. Literature Review Table
Team Member Database/
Journal Article
Citation
Summary
Statement
Website Summary
Statement
Madeline Caver Hoedt, A., Hollack, C., B
oelen, C., Wetering, N.,
Horst, N., Jonkers, C.,
Wijburg, F., Bosch, A.
(2011). "MY PKU":
increasing self-
management in
patients with
phenylketonuria. A
randomized controlled
trial. Orphanet Journal
of Rare Diseases
, volume 6.
Thirty-eight patients
were randomized into
a study group(1) or a
control group(2).
Group 2 continued
with their usual
procedure by
contacting a dietician
if deviant Phe levels
occurred. Group 1
was given a personal
"My PKU" web page
to increase self-
management of PKU.
All patients and/or
parents expressed a
high level of
satisfaction with the
new way of disease
management.
A secured personal
web page on a
special internet
site, named 'My
PKU‘ was given to
each patient.
This allowed a graph
of their recent and
previous Phe
values, general
information about
the dietary
treatment and
follow-up
guidelines, and a
message-box to
contact their
dietician. There was
no significant
difference in either
group.
This randomized
controlled trial is
the first to
demonstrate that
increased self-
management in
PKU patients
and/or their
parents by
providing Phe
values online is a
feasible, safe
method and is
highly appreciated.
All authors were in
agreement and
there were no
conflicts of interest.
4. Menu Analysis
Personal Diet List
Food Item
Number
of
Servings
Serving Size
Total
grams
SWEETS-FROZEN DESSERTS
-ICE CREAM-VANILLA
1 1 c
132.57
grams
WENDY'S SPICY CHICKEN SANDWICH 1
1
SANDWICH
213 grams
WENDY'S SOUR CREAM & CHIVES BAKED POTATO 1 1 POTATO 314 grams
WATER-BOTTLED-POLAND SPRING 20 1 oz 567 grams
CELERY-RAW 3 1 STALK 120 grams
DIP-FAT FREE; RANCH; KRAFT 4 1 floz 128 grams
VEGETABLES-MIXED-FRZ
-UNPREPARED
1 1 g 1 grams
SOUP-MINISTRONE-CANNED
-CHUNKY-READY-TO-SERVE
2 1 c 480 grams
BREAD-WHITE-COMMERCIALLY PREPARED
-TOASTED
1 1 SLICE 23 grams
COFFEE-INSTANT; ITALIAN CAPPUCCINO; PREPARED 2 1 c 480 grams
ORANGE JUICE-CHILLED
-INCLUDES FROM CONCENTRATE
2 1 c
497.6
grams
STRAWBERRIES-RAW 1 1 CUP 149 grams
PANCAKES-BUTTERMILK-PREPARED FROM RECIPE 4
1 IN
PANCAKE
38 grams
JELLO-READY TO EAT SNACKS; TROPICAL FRUIT PUNCH 1 1 SNACK 99 grams
By: Madeline Caver
Nutrient Total Rec. %Rec
Calories 2041.02 2200 92.77%
Pro (g) 73.36 48 152.83%
Fat (g) 52.23 73.33 71.23%
Carb (g) 332.41 --
Fiber (g) 21.91 30 73.03%
Cal (mg) 899.12 1000 89.91%
Iron (mg) 14.16 10 141.6%
Na (mg) 5067.34 2400 211.14%
Pot (mg) 3623.67 --
Phos (mg) 990.47 700 141.5%
Ash (g) 13.29 330 4.03%
vitA (IU) 11634 4000 290.85%
vitC (mg) 277.91 75 370.55%
Thia (mg) 0.98 1.1 89.09%
Ribo (mg) 1 1.1 90.91%
Nia (mg) 6.13 14 43.79%
H2O % 48.74 Female 19-30 --
satF (g) 19.39 24.44 79.34%
monoF (g) 7.69 24.44 31.46%
polyF (g) 3.63 24.44 14.85%
Chol (mg) 191.9 300 63.97%
5. Team Member Database/
Journal Article
Citation
Summary
Statement
Website Summary
Statement
Laura Pagan Gassio, R., J. Campistol,
M.A. Vilaseca, N
Lambruchinni, F.J
Cambra, and E. Fuste.
"Do adult patients with
phenylketonuria improve
their quality of life after
introduction/resumption
of a phenylalanine-
restricted diet?.." (2003):
n.pag. OVID. Web. 25
Jun 2013.
The index of dietary control was
poor in 8/15 patients, regular
(Phe: 514 micromol/L) in 4/15
and good (Phe: 354 micromol/L)
in 3/15 patients. Fifty-three
percent of patients reported that
their state of health was very
good, 47% described it as good,
40% felt that their present health
on-diet was better than it had
been off-diet; 53% believed that
they were calmer, quieter and
less easily upset and 40% were
more alert and were more able
to maintain attention while on-
diet. Thirty-three percent of the
patients felt happier, and 27%
felt more vital; 20% thought that
they were less impulsive and
aggressive, and that they were
now less argumentative than
before. Sixty percent of the
patients felt that their quality of
life had improved on-diet
compared with the situation off-
diet.
There was not a website
associated wit the study.
The fifteen participants
were given a twenty-four
question survey to
complete after being on
the restricted diet.
More than half of the
patients believed that
their quality of life
improved with a Phe-
restricted diet; they
reported feeling calmer,
quieter, and less easily
upset. Only 47% attained
regular to good dietary
control.
Literature Review Table
6. Food Items Number
of
servings
Servin
g Size
Total
Gram
s
ORANGE DRINK-BREAKFAST TYPE
-W/ JUICE & PULP-PREP WITH WATER FROM FRZ
1 1 c
250.4
grams
FRENCH TOAST-PREPAREDFROM RECIPE
-MADE WITH LOWFAT (2%) MILK
2 1 oz
56.7
grams
SNACKS-TRAIL MIX-REGULAR
-UNSALTED
1 1 CUP
150
grams
CELERY-RAW 0.5
1 CUP
DICES
60
grams
SOUP-VEGETARIANVEGETABLE
-CND-PREP W/EQ VOL WATER
-COMMERCIAL
8 1 oz
226.8
grams
APPLESAUCE-CANNED-SWEETENED
-WO/SALT
1 1 c
256
grams
VEGGIE BURGER-HARVEST BURGER; ORIGINAL 1 1 PATTIE
90
grams
WATERMELON-RAW 1
1 CUP
DICED
PIECES
160
grams
GRAPE JUICE-CANNED OR BOTTLED
-UNSWEETENED
1 1 c
252.8
grams
Analysis Results by Laura Pagan
Nutrient Total Rec. %Rec
Calories 1551.73 2200 70.53%
Pro (g) 48.81 48 101.69%
Fat (g) 57.49 73.33 78.4%
Carb (g) 231.76 --
Fiber (g) 10.59 30 35.3%
Cal (mg) 636.79 1000 63.68%
Iron (mg) 11.78 10 117.8%
Na (mg) 1525.31 2400 63.55%
Pot (mg) 2916.07 --
Phos (mg) 859.3 700 122.76%
Ash (g) 10.17 330 3.08%
vitA (IU) 3859.03 4000 96.48%
vitC (mg) 165.76 75 221.01%
Thia (mg) 1.69 1.1 153.64%
Ribo (mg) 3.8 1.1 345.45%
Nia (mg) 15.14 14 108.14%
H2O % 65.9 Female 19-30 --
satF (g) 11.81 24.44 48.32%
monoF (g) 22.18 24.44 90.75%
polyF (g) 17.35 24.44 70.99%
Chol (mg) 65.77 300 21.92%
7. Literature Review Table
Team Member Database/Journal
Article Citation
Summary Statement
Laura Collins PubMed
Adults with late diagnosed PKU and
severe challenging behavior: a
randomized placebo-controlled trial
of a phenylalanine-restricted diet.
80(6):pp 631-5
Lee PJ, Amos A, Robertson L,
Fitzgerald B, Hoskin R, Lilburn M,
Weetch E, Murphy G.
2009
A group of 34 adults,
ages 21-61, who were
diagnosed late with PKU
and have intellectual
disabilities and behavior
problems were studied
and put on a
phenylalanine-restricted
diet. Only 17 people
completed the 60 diet.
Results were that it is
difficult to study
intellectual disability
people but that
phenylalanine-restricted
diet could help correct
the behavior problems.
9. Team Member Database/ Journal Article
Citation
Summary Statement
Myra (Ashley) McVay Gale Health & Wellness
Resource Center
Phenylketonuria: outcomes
and treatment
Pediatric Health
v2 i2; p225(10)
Fickie, M.R.,
Gentile, J.,
Waisbren, S.
2008
PKU is a genetic disorder
affecting nearly 1 in every
10,000 babies born in the US
and Europe. It occurs when
the liver can not metabolize
phenylalanine. Unless
treated early, a buildup of
phenylalanine in the body
can be toxic, resulting in
severe mental retardation,
seizures, and eczema,
among other things.
Prognosis is relatively good
with this disorder providing
that the patient follows a
life-long strict dietary
regiment.
Literature Review Table
11. Explanation of Diet
• The previously shown meal plan hinges on supplementation
of a PKU Formula taken 3 times a day. The formula used in my
calculations is Phenex-2.
• Based on the low-protein diet and supplementation of the
formula, I have compiled a revised summary of some basic
nutrients.
• Total Calories: 1814.97 Protein (g): 103.01
• Fat (g): 47.89 Sodium (mg): 3740
• A complete list of nutrition facts for Phenex-2 can be found at
http://abbottnutrition.com/brands/products/phenex-2
• The basis for my diet plan came from
http://www.mayoclinic.com/health/phenylketonuria/DS0051
4/DSECTION=lifestyle-and-home-remedies
12. Conclusion
Expense:
A phenylalanine restricted diet can be
expensive. If a milk replacement such as
Phenyl-Free is needed, they can cost
around $55 per can.
Balance:
The diet can be complicated. It requires a
lot of planning, buying and preparing
foods that are different from what the
rest of the family eats. The diet requires
careful measurement, calculation, and
record keeping. There has to be a good
balance of nutrients. This can be hard
because certain foods cannot be
consumed. These foods include high
protein foods such as fish, chicken, eggs,
milk, cheese, dried beans and tofu.
Time: (Lifelong)
The Phenylalanine restricted diet has to
be followed for the rest of a person’s life.
Going off of the diet can lead to a
buildup of phenylalanine in the blood.
The increase in the blood level can cause
brain function problems, irritability, slow
response times and feeling “foggy”.
