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”.