Can I have this????

 On many, many occasions I see people posting in PKU groups asking if they can have an item, and then will see some people saying yes and others saying absolutely not, and the answer isn’t always that simple.  One reason the answer isn’t that simple is because PKU is not a one size fits all type of thing.  Not every person with PKU has the same protein/phe/exchange tolerance.  What might be acceptable in one person’s diet may be way out of range for another.  Not to mention there are different types of PKU with varying tolerances.  Over the years I have met people with Classical PKU (like my girls have) who have very low tolerance, and on the flip side I have met people who are Hyper-Phe who do not have to necessarily follow a diet or drink formula, but instead perhaps slightly modify what they eat.  Many years ago I met a family whose child could eat almost a normal diet.  This child could drink regular milk, and did not need formula.  The only thing the family needed to modify was the amount of meat in their child’s diet.  When this family would come to events people would be shocked that their daughter could eat such a lax diet.  Some parents would corner the mother and tell her how she shouldn’t let her child eat xyz when in reality her child didn’t have the same form of PKU, and she didn’t have the same tolerance.  Unfortunately this family stopped coming to PKU events rather quickly because they felt like they didn’t fit in which made me so sad because they deserved support too.

Secondly, when looking at an item it isn’t what is in the ingredients that matters, but instead how much phe/protein, etc it has and again whether it fits the diet.  Sometimes I see families who are counting grams of protein (like my girls) and who are on the simplified diet.....with the simplified diet we don’t count phe and are not as strict.  With the simplified diet we simply count grams of protein by reading labels and then there are items within the diet that are free.  So for example, an item can have gelatin in it (which is one I often see families asking about), but still be listed as zero grams of protein, and so for my girls we would not count that in their daily allowance.  We would count this as a free item.  When this occurs I often see parents saying “there is gelatin in it our kids can’t have that.”  Again, that depends.  Someone like my girls who are on Kuvan and doing the simplified diet can, but someone who has Classical PKU and can take in only 3 grams of protein (or 150 mg phe) might need to count that and look up the exact phe content.  

Another thing to remember is based upon where the particular ingredient is listed can determine how much of that item is in the product.  So when there is an ingredient listed first that means there is a larger quantity of that ingredient in the product.  If there are 20 different ingredients in something and the first ingredient is a higher protein item that would mean that item has a higher protein/phe content then something with 20 ingredients and the last one is the higher protein/phe item.  

Unfortunately there is not a one size fits all answer to the question (in most cases) when someone asks “can I have this?” Or “can I give this to my child?”  This is one of the reasons it is most important for parents (and later the kids/teens/adults with PKU) to be educated on the disorder and helped to understand it fully.  When they have gone through counseling on their disorder and had proper counseling on how to manage the diet they will be able to look at an item and decide whether it fits or doesn’t, and if they find an item that they do not know the phe/protein/exchange of then they should be contacting their medical providers to get the answers because they are the people who know the medical history of that individual person.  What do I mean by this?  Say for example, someone is counting mg phe and they find a new product in the store and they cannot find it listed in the How Much Phe list....then instead of asking on a facebook group and getting the “opinion” of a bunch of people who all manage the diet differently and all have different tolerances they should contact their medical providers because they would be the ones to better calculate out those numbers and say yes or no to the item.  

Another thing to consider is long term.....while something might fit in the diet when say a child is a toddler will that items still fit in the diet when that child’s appetite increases?  For example, when my girls were younger and not on Kuvan they were allowed 6 grams of protein or 300 mg/phe per day. Back then we counted mg/phe and they were not on the simplified diet.  When they were toddlers and their appetites were small, but we needed to get their phes in we could have given them something like regular bread (for example) to reach that 300 mg/phe, but we knew this wasn’t a good idea because as toddlers they might only eat a 1/4 of a sandwich which was 50 mg/phe or 1 gram of protein, but we knew that wouldn’t always be the case.  When you think about it most children/teens/adults don’t eat just a 1/4 of a sandwich but instead eat a full sandwich which would be 200 mg/phe or 4 grams of protein.  We knew when their appetites grew that this was not going to be feasible, and we didn’t want them to learn to love the taste and later struggle with taking it away and transitioning them to low protein bread....they do taste very different. I saw this recently in a group on facebook where a family was struggling to get in their toddlers allotment in for the day and the mother wanted to know if she could give her child a chicken nugget.  The problem with this is......who only eats one chicken nugget?  As a toddler one chicken nugget might be filling and give a large quantify of protein, but what happens when that toddler becomes a preschooler who now sees his/her friends eating 4, 5, or even 6 chicken nuggets and fries and the child can only have one nugget with nothing else.....that child is going to be hungry.  So, this is yet another thing to take into consideration. If a family is struggling with getting in the additional protein they could ask their healthcare providers how to deal with his.  Some might say add regular milk to the low protein formula (obviously weighing out the appropriate amounts).  We used powdered milk for our girls as it didn’t add more volume, but gave them the phes/protein they needed (again obviously this needed to be weighed out carefully and if someone wants to do this I highly suggest consulting their dietitian or clinic for specific instructions based upon their child’s tolerance).

Unfortunately PKU is not a one size fits all type of thing, and while we wish it was and that would make it easier that just isn’t the case.  It is very individualized and we need to think about using an individualized approach to diet management!