The Phenylalanine restricted diet
for PKU
Presented by Madeline Caver, Laura
Collins, Myra McVay, and Laura Pagan
June 30,...
Literature Review Table
Team Member Database/
Journal Article
Citation
Summary
Statement
Website Summary
Statement
Madelin...
Choosing the right foods!
Menu Analysis
Personal Diet List
Food Item
Number
of
Servings
Serving Size
Total
grams
SWEETS-FROZEN DESSERTS
-ICE CREAM-V...
Team Member Database/
Journal Article
Citation
Summary
Statement
Website Summary
Statement
Laura Pagan Gassio, R., J. Camp...
Food Items Number
of
servings
Servin
g Size
Total
Gram
s
ORANGE DRINK-BREAKFAST TYPE
-W/ JUICE & PULP-PREP WITH WATER FROM...
Literature Review Table
Team Member Database/Journal
Article Citation
Summary Statement
Laura Collins PubMed
Adults with l...
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...
Team Member Database/ Journal Article
Citation
Summary Statement
Myra (Ashley) McVay Gale Health & Wellness
Resource Cente...
Nutrient Total Rec. %Rec
Calories 584.97 2200 26.59%
Pro (g) 13.01 48 27.10%
Fat (g) 5.89 73.33 8.03%
Carb (g) 127.56 --
F...
Explanation of Diet
• The previously shown meal plan hinges on supplementation
of a PKU Formula taken 3 times a day. The f...
Conclusion
Expense:
A phenylalanine restricted diet can be
expensive. If a milk replacement such as
Phenyl-Free is needed,...
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Final Nutriton Diet Presentation

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Our slides discuss how to maintain a prpoer PKU diet. Members involved: Madeline Caver, Laura Pagan, Laura Collins, and Ashley McVay.

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Final Nutriton Diet Presentation

  1. 1. The Phenylalanine restricted diet for PKU Presented by Madeline Caver, Laura Collins, Myra McVay, and Laura Pagan June 30, 2013
  2. 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.
  3. 3. Choosing the right foods!
  4. 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. 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. 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. 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.
  8. 8. 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%
  9. 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
  10. 10. Nutrient Total Rec. %Rec Calories 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- 1/3X4-3/4IN 202 grams BANANAS-RAW 1 1 FRUITWO/S KN&SEEDS 114 grams SWEETS-CANDIES-JELLYBEANS 10 1 SM 11 grams SOUP-TOMATO-CND-PREP W/EQ VOL WATER -COMMERCIAL 0.5 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
  11. 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. 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”.

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