final nutriton diet presentation
DESCRIPTION
Our slides discuss how to maintain a prpoer PKU diet. Members involved: Madeline Caver, Laura Pagan, Laura Collins, and Ashley McVay.TRANSCRIPT
The Phenylalanine restricted diet for PKU
Presented by Madeline Caver, Laura Collins, Myra McVay, and Laura Pagan
June 30, 2013
Literature Review TableTeam Member Database/
Journal Article Citation
Summary Statement
Website Summary Statement
Madeline Caver Hoedt, A., Hollack, C., Boelen, 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.
Choosing the right foods!
Menu AnalysisPersonal Diet List
Food ItemNumber
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%
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
Food Items Number of servings
Serving Size
Total Grams
ORANGE DRINK-BREAKFAST TYPE-W/ JUICE & PULP-PREP WITH WATER FROM FRZ 1 1 c 250.4
grams
FRENCH TOAST-PREPARED FROM 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-VEGETARIAN VEGETABLE-CND-PREP W/EQ VOL WATER
-COMMERCIAL8 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 11 CUP DICED PIECES
160 grams
GRAPE JUICE-CANNED OR BOTTLED-UNSWEETENED 1 1 c 252.8
grams
Analysis Results by Laura PaganNutrient 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%
Literature Review TableTeam Member Database/Journal
Article CitationSummary 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 631-5Lee 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.
Menu Analysis By: Laura Collins
Nutrient Total Rec. %Rec
Calories 2127.36 2200 96.7%
Pro (g) 51.3 48 106.88%
Fat (g) 55.35 73.33 75.48%
Carb (g) 372 --
Fiber (g) 19.07 30 63.57%
Cal (mg) 779.31 1000 77.93%
Iron (mg) 13.4 10 134%
Na (mg) 6519.97 2400 271.67%
Pot (mg) 3130.36 --
Phos (mg) 956.18 700 136.6%
Ash (g) 20.69 330 6.27%
vitA (IU) 4316.19 4000 107.90%
vitC (mg) 279.76 75 373.01%
Thia (mg) 1.89 1.1 171.82%
Ribo (mg) 1.81 1.1 164.55%
Nia (mg) 14.04 14 100.29%
H2O % 67.88 Female 19-30 --
satF (g) 21.13 24.44 86.46%
monoF (g) 11.31 24.44 46.28%
polyF (g) 5.18 24.44 21.19%
Chol (mg) 225.25 300 75.08%
Team Member Database/ Journal Article Citation
Summary Statement
Myra (Ashley) McVay Gale Health & Wellness Resource Center
Phenylketonuria: outcomes and treatmentPediatric Healthv2 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
Nutrient Total Rec. %RecCalories 584.97 2200 26.59%
Pro (g) 13.01 48 27.10%Fat (g) 5.89 73.33 8.03%
Carb (g) 127.56 --
Fiber (g) 8.38 30 27.93%
Cal (mg) 90.38 1000 9.04%
Iron (mg) 7.54 10 75.4%
Na (mg) 1100.91 2400 45.87%
Pot (mg) 2032.62 --
Phos (mg) 322.24 700 46.03%
Ash (g) 7.82 330 2.37%vitA (IU) 2477.13 4000 61.93%
vitC (mg) 142.21 75 189.61%
Thia (mg) 0.69 1.1 62.73%
Ribo (mg) 0.87 1.1 79.09%
Nia (mg) 12.33 14 88.07%
H2O % 58.74 Female 19-30 --
satF (g) 3.59 24.44 14.69%
monoF (g) 0.39 24.44 1.6%
polyF (g) 0.93 24.44 3.81%
Chol (mg) 0 300 0%
Food Item # of Servings
Serving Size
Total Grams
BROCCOLI-CKD-BOILED-DRAINED-W/SALT 0.5 1 CUP
CHOPPED 78 grams
MUSHROOMS-CKD-BOILED-DRAINED-W/SALT 0.5 1 CUP
PIECES 78 grams
POTATOES-BAKED-FLESH AND SKIN-WO/SALT 1 1 POTATO2-
1/3X4-3/4IN202
grams
BANANAS-RAW 11
FRUITWO/SKN&SEEDS
114 grams
SWEETS-CANDIES-JELLYBEANS 10 1 SM 11 grams
SOUP-TOMATO-CND-PREP W/EQ VOL WATER
-COMMERCIAL0.5 1 CUP (8 FL
OZ)122
grams
WATER-MUNICIPAL 1 1 c 682.67 grams
CREAM SUBSTITUTE-NONDAIRY-POWDERED 0.33 1 oz 9.3555
grams
CEREALS READY-TO-EAT-CORN FLAKES-KELLOGG'S
-(CORN)0.75 1 OZ BOX 21.3
grams
Analysis Review by Ashley McVay
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/DS00514/DSECTION=lifestyle-and-home-remedies
ConclusionExpense: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”.