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Dr. Andrew Rynne

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Can 1/3 medium eggs be added to increase protein intake for a patient with PKU?

Answered by
Kathy
Kathy Shattler

Dietitian & Nutritionist

Practicing since :1985

Answered : 872 Questions

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Posted on Sat, 2 Jun 2018 in Diet Plans
Question: I cook for a man with pku. I really don't know the difference between phenalaladine and protein. the man does get his protein shake everyday. His proteins should be between 8 and 12 grams a day. Can I add 1/3 of a medium egg ( 2grams of protein) but high in phenalaladine to his ) to his 0 gram egg substitute. Also can I add 1/6 cup of cheese( 3.5 grams of protein, but high in phenalaladine) to his 0 gram egg substitute.?
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Answered by Kathy Shattler 1 hour later
Brief Answer:
Hello and Welcome to HealthcareMagic

Detailed Answer:
Hello dear, a low phenylalanine diet sheet should help guide you. You see, there are several amino acids in a protein. Phenylalanine is just one amino acid from a protein chain. It is also one of the 9 essential amino acids so it is present in all complete proteins.
Know first:
Foods not allowed in phenylalanine (PHE or PKU restricted)-restricted meals include the following:
Cheese and dairy
Nuts, seeds, dried beans and peas, peanut butter
Eggs, poultry, meat, fish and other seafood

Foods allowed in phenylalanine (PHE)-restricted meals—but that need to be monitored or measured and weighed according to the individual’s diet prescription (Shuett, 2011)—include the following:
Bread, crackers, potato chips, popcorn
Fruit, vegetables, and fruit and vegetable juices
Special low-protein foods, low-protein cereals

You cannot use egg whites as they are high in phenylalanine, so no, you cannot add a medium egg or egg white to egg substitute, unfortunately. Even egg substitutes should be avoided unless they state "no phenylalanine" We are looking at mg in the 100's for phenylalanine. The best thing to do is to get a food chart of foods with phenylalanine content in them and focus on meeting protein needs by using the special medical foods that need to be purchased (possibly his PKU shake he drinks)?

The PKU diet is complicated and best learned if you sit down with a dietitian and review the special foods used and the special nutrient needs of the PKU patient.

The bulk of the diet should be fruits and vegetables. You say he needs 8-12 grams proteins per day. Total proteins should be similar as for a normal individual with restriction of the phenylalanine amino acid only to less than 10 grams daily, so it is his phenylalanine that is restricted to 8-12 g/daily. Medical food will be required to meet his protein needs while restricting the phenylalanine amino acid and increasing the tyrosine amino acid which is part of the treatment for PKU.

Suggestions for formulas to use are Phenyl-Free 2 by Mead XXXXXXX or Phenex 2 by Abbot Labs. Need to use low protein cereals. Without an adequate intake of phenylalanine-free formula or supplemental calcium, adults with PKU are at increased risk for decreased bone density. His total protein intake is closer to that of a normal person without PKU, so individual amino acids/peptides should be distributed evenly through the day avoiding all protein sources with phenylalanine except for the amount indicated per day.

This is a complicated diet so I would focus on feeding your client the diet the family gives you and be creative with the foods only on the "allowed list" I gave you here emphasizing fruits and vegetables to fill him up and spice up his palate.

Good luck and I did answer your questions, but feel free to pursue clarification if you need additional understanding. I wish you the best. Respectfully, Kathy J Shattler, MS, RDN Clinical Nutritionist
Above answer was peer-reviewed by : Dr. Yogesh D
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Follow up: Kathy Shattler 36 hours later
What factors would cause a PKU person's phe levels to fluctuate
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Answered by Kathy Shattler 21 minutes later
Brief Answer:
Welcome back

Detailed Answer:
PKU levels fluctuate in response to phenylalanine contents in food, medications or hidden sources of phenylalanine you may not be aware of. Keeping a list of foods and their phenylalanine contents would be most helpful.

To completely answer your question, however,in patients with PKU, phenylalanine concentrations are highest in the morning. Factors that can affect phenylalanine fluctuations include age, diet, timing and dosing of protein substitute and energy intake, dietary adherence, phenylalanine hydroxylase genotype, changes in dietary phenylalanine intake and protein metabolism, illness, and growth rate. Even distribution of phenylalanine-free protein substitute intake throughout 24 h may reduce blood phenylalanine fluctuations. I think I mentioned distributing amino acids evenly throughout the day in my first answer and this may help stabilize levels.

A study done in 2013 showed that a medication called sapropterin may help with stabilization and this can be discussed with treating physician if matter is of great concern and fluctuations are wide.

Realize that daily blood Phe levels vary by ≤ 50% in health but can be much higher in PKU.

An increase in blood phenylalanine concentration may result in increased brain and cerebrospinal fluid phenylalanine concentrations within hours. Although some evidence suggests that stabilization of blood phenylalanine concentrations may have benefits in patients with PKU, more studies are needed to distinguish the effects of blood phenylalanine fluctuations from those of poor metabolic control.

If he were my patient and the fluctuations were wide, I would attempt to stabilize them through the even distribution of amino acids, looking at labels for hidden sources of Phe and discussing your concern/medication options with treating physician.

There is a resource online you may be interested in. Check out:
https://npkua.org/portals/0/pdfs/pkubinder/PKU%20Binder%202011.pdf

I am unable to paste tables in this window without losing formatting, but if you do a search online for Phe content of foods you should be able to find some useful resources. You know to stay away from aspartame, correct? Sugar sweetener made from Phe?

I hope I have thoroughly covered your query and I most certainly wish you the best of luck with your client. If more clarification is needed please feel free to use your remaining query to ask (after 5 pm today as I will be away from desk). If satisfied, kindly rate my answer as this helps me improve my online consultations.

My best, Kathy J. Shattler, MS, RDN

Above answer was peer-reviewed by : Dr. Prasad
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Follow up: Kathy Shattler 27 hours later
Dear XXXXXXX Thanks so much for your input. Unfortunately I was not able to open up any links in the e-mails I got from two different replies. My client does get his "PKU med bev". We get it from a metabolic clinic. I think the State of Oregon pays for it, as it is pretty progressive regarding PKU. His med powder is I
1/3 of something, and 2/3 of another powder, for a total of 99 grams. His "used to be" contract nurse once said "he gets everything his body needs in the med bev", so I asked , "So his food is just to add a few extra calories, and to give him the experience of eating?" She seemed to agree with this. As you say 8 to 12 grams of protein are a lot. Especially since they took fruit and Cool Whip off the list.
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Answered by Kathy Shattler 14 hours later
Brief Answer:
Sorry for the delay

Detailed Answer:
I checked with administration and, apparently, we cannot give links on this website. I can tell you that if you have access to a computer you can locate these resources by searching for "pku food tables" or "pku resources" to obtain the PDF copies of the booklets and tables.The one link came from the National PKU Alliance, which may assist you also in locating these references.

Not to be disrespectful, but a nurse is not a dietitian. The drink does give your patient most of his essential needs but calories need to be watched. In the elderly, a BMI of less than 23 is underweight and constitutes a need for referral to the PKU dietitian. Rapid weight loss also means he is not getting enough calories and should be referred back to the PKU dietitian. A weight loss of greater than 5% in one month, 7 % in 3 months or 10% in six months are all valid malnutrition indicators for referrals.

Yes, this man should be able to enjoy chewing food and the flavor of taste to add to the quality of his life. He can live without the cool-whip but I question why the fruit was taken off, of course not knowing the entire case. This does not leave him much to eat. There is virtually no protein in fruit, so taking it completely off his diet list is my confusion. Carbohydrates such as breads/pastas have protein, vegetables have a little protein, but fruit has little to worry about. Have you tried dividing his drink up into 3 separate servings throughout the day to spread out his proteins throughout the day so his Phe levels don't fluctuate so much?

Again, I do hope all this discussion on a complicated metabolic patient has assisted your care in this gentleman. Kindly rate our answers to the extent you feel each of us have helped you on an individual basis as this is important to me and to my employers.

My best regards to you in the coming New Year and if you ever need to get in touch with me again, my clinic is listed on my profile and I have a private consultation area here at https://www.bit.ly/askkathyshattler.

Kathy J. Shattler, BS, MS, RDN, Clinical Nutritionist


Above answer was peer-reviewed by : Dr. Vaishalee Punj
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Follow up: Kathy Shattler 4 hours later
Good morning XXXXXXX Well, I am have really learned a lot I will look up the web pages you suggested. Thank you. I do not make his med bev. I work graveyard, and when his "one on one" comes in at 7am , it has always been the history (for over 12 years) that she will give him the med bev, all of it in the in the morning . Mostly because it is not an easy task to give it to him, he resists, and will gag even though we put in 0 gram strawberry syrup in it to make it more palatable It's just something you have to do, and want to get it over with as soon as possible. I did not mean that he cannot have fruit, but now they are considered a 0 gram food. So it makes me have to scramble that much harder to give him his 8 to 12 grams. I could give him 8 or 9, but then for some reason the house manager seems to think that the closer I get to 12 grams the better job I am doing. She is not anyone I can talk to.
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Follow up: Kathy Shattler 13 minutes later
Also, another thing XXXXXXX once his PHE levels went up, and it was determined by the dietician it was because he was getting oatmeal in his diet. The amount of oatmeal was within the range of his protein allowance (enough oatmeal to account for 3 grams of protein) is the protein in oatmeal extremely high in pheyketalalyne? Thanks for answering this Eva
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Answered by Kathy Shattler 0 minute later
Brief Answer:
Hi again

Detailed Answer:
I am sorry to hear that you are in such a difficult situation. It seems like the easiest thing to do would be to try to incorporate a special medical food, one more than his drink such as a lo protein cereal or lo protein pancake mix made specifically for PKU patients to boost those couple of grams your manager wants. Gee, it is so hard to make everyone happy, isn't it?

Just to give you an example, if you could obtain the lo Phe pancake mix, you would add a mere 3 gm. of Phe per 3 (3in.wide) pancakes with 0 syrup (2 TB). That would get you up to the manager's expectations of 12 g Phe. You would not want to give that close to the time he gets his drink but at a later time. Who says you can't have pancakes for supper??

Hope this idea helps a little! Cheers! Kathy
Above answer was peer-reviewed by : Dr. Prasad
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Follow up: Kathy Shattler 48 minutes later
HI XXXXXXX You did not answer my oatmeal question, so I will just ask it again. One time my client's phe levels went up, and the dietician determined it was because it was because he was eating oatmeal I gave him a scant amount of oatmeal (3grams worth). So I am asking, is the phenylaladine in oatmeal quite high?
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Answered by Kathy Shattler 15 minutes later
Brief Answer:
Hello

Detailed Answer:
I went back to your original question and see no mention of oatmeal, but do see it mentioned later. Apologies. Yes, 3 grams of oatmeal would have about 25 g of Phe in it according to the Phe table I looked at.

This is why it is important to have access to a table of Phe content of foods, so you can meet his dietary needs and since I can't post a link and there are many foods, I will do the best I can to direct you to a resource.

Search for "Phenylalanine content in foods" and you should see a selection of tables. Choose the easiest one for you to use or access in your location of work.

Sorry about that! Kathy
Above answer was peer-reviewed by : Dr. Prasad
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