Keeping an Off-Diet Food Diary during a PKU Kuvan Responder Trial

Off-Diet PKU Kuvan Responder Trial Food DiaryWhen BioMarin’s Kuvan was approved by the FDA in 2007 as the first prescription drug to treat phenylketonuria (PKU), directions for taking the prescription drug stipulated that a low-protein diet must also be followed. However, when I first discussed a Kuvan responder trial with my clinic in the fall of 2010, I was advised that I could remain on a non-restricted diet as long as I consume close to the same amount of protein each day. I suspect that this relaxation was granted with the understanding that if I do in fact respond to the drug, then lower phe levels are better than completely unbridled protein intake. They key; however, to making sure that you consume roughly the same amount of protein each day means keeping a very detailed food diary.

Initially, I found it tricky to figure out a good method for capturing my daily protein intake. I explored the possibility of using Cambrooke’s DietWell app—a fabulous tool for the PKU community—but the more I experimented with it, the more I realized that the items that are truly high in protein are absent from its database.

So now that I’m nearly a week into my Kuvan responder trial, just how have I managed to keep track of my protein consumption? Well, here are the three “secrets” to my success:

  1. Take advantage of publicly available information—Obviously, checking a food product’s nutrition label is a great place to start when keeping a food diary. Be sure that the first thing you look at is the serving size because many products you buy in the grocery store will contain multiple servings per container. Use a weighted food scale, measuring cups or measuring spoons to gauge you portions. For times when not eating at home, try to plan ahead. Many restaurants list nutritional information on their Websites, have apps you can download to your mobile device or sometimes you can even ask your server for a printed brochure of nutritional information.
  2. Men’s Health—Whether it’s the New York Times Bestselling “Abs Diet” or the popular “Eat this, Not that” series, Men’s Health does an excellent job of providing all the information you ever possibly want on a food item. I especially like their recipes where we can cook healthy, in the comfort of our own home, and know exactly how much protein there is in a serving size. I’m sure other recipes, whether in a magazine or posted on a popular cooking website, will provide the same type of useful information. The Men’s Health product line has worked very well for me though.iPhone notepad app screen shot Kuvan Responder Trial PKU
  3. iPhone Notepad App—Lastly, because I am getting my protein equivalent information from a variety of sources, I decided to use my iPhone’s notepad application to track my meals. Even though this notepad app came standard on my iPhone, it has the mobile convenience of the DietWell app, but the flexibility of a blank page. I’m able to enter whatever food product I ate, total the daily protein amount, and when I’m done, forward the finished document directly to my email.



Filed under Kuvan

10 responses to “Keeping an Off-Diet Food Diary during a PKU Kuvan Responder Trial

  1. Mandy

    You’re doing great! If anyone has the detail oriented mentality to do this, it’s you.

  2. Erica Stelten

    Wow, Nicole! I am very imporessed by this! I would love to talk to you more about your experience. Darla mentioned that you were keeping a blog. Will you be able to make it to the event at the UT Culinary Institute on 9/17? I hope to see you there!
    Erica Stelten, RD

  3. Darla Smith, RD, MPH, LDN

    Dear Nicole: I know the trial was a mixed experience for you, but I do think we can try again when things are less busy for you and perhaps we can keep your diet more consistent from day to day so there are not so many variables to consider. Also, I am going to enter some of the foods you consumed to the Metabolic Pro database to perhaps get a more accurate reading of your food intake. That will help the analysis. Good luck w working on your graduate degree and I look forward to trying again….take care!

  4. Came across your blog from Cambrooke via Facebook. It’s great to read something that is based on PKU life from my stage of life (married and thinking about kids) rather than raising a child with PKU. I read a bit already and relate a lot. I am a KUVAN responder and I can’t tell you how amazing it has been for travel especially. But it is almost impossible to keep actual PHE mg on diet records though and the blood levels I get back on some things vary a lot even though its the same protein. It’s been an adventure of learning which items seem to have more PHE in the protein I actually had to do the trial twice as the first results were confusing and I gave up until about 6 months later. My protein wasn’t exactly the same every for each week, but close. And the 3 days leading up to the bloodwork, I ate the same set of meals each week (in different order or days sometimes). Boring, but it worked. Hopefully, as more people respond to KUVAN they will come up with more PHE information for “real” food as I am not on any low-protein foods anymore other than the formula.

    • Hi Erin – it is so good to hear from you! I wanted so much to be able to connect to other PKU adults through this blog so I’m happy you took the time to comment. You may have noticed that I haven’t created a blog entry on the results of my Kuvan trial yet. Apparently the results were inconclusive and I’m considering a second attempt…very similar to what you commented above. It sounds as though I may have to eat the same thing over and over too. I’d love to keep in touch – if you’re interested. If so, feel free to email me at



  5. Hey Nicole – Check out it has a platform for tracking PHE and links to the USDA database that includes analysis of PHE in addition to other amino acids, vitamins, minerals, etc for anything you would find on the USDA database. You can track daily intake or you can put in recipes. I think this would be great for you if you eat higher protein foods than are available on DietWell (which also pulls from the USDA database, but as you mentioned not all high protein foods are included)

    I also have a website/blog with some PKU info on it! I have some recipes and things that are lower in PHE so may not be useful to you now, but if you ever are pursuing a maternal pku diet they might be helpful! There are some resources on there as well (many of which you listed here…) I also write up some blog posts for CamBrooke at their blog at – check them out!

    Great to see another PKU name in the social media universe!

    Best of Luck! Feel free to contact me : )

    Hunter Rametta
    25, PKU

  6. Sarah LeNoue

    Hi Nicole!

    I am the mother of a 4 year old with PKU. We just did his one month trial on Kuvan and they were confused at his results. I keep an exact journal of what he eats, down to the exact mg of protein in his diet and they were still unsure! However, we did a second attempt and they saw that he was a responder in 24 hours! We had to give him the EXACT amount ot PHE each day to know for sure if he was a responder. It was tough, but very worth it. We are waiting on his supply of Kuvan now. I highly recommend doing a second atttempt and keeping exact records. It was worth it for us, even if it was a pain!

    Please keep writing on your blog. I just discovered you and I’m really enjoying reading!

    Sarah (Mother to Grant-4 years with CPKU)

    • Hi Sarah –

      Thanks so much for taking the time to post a comment on PKU Parlor! I’m very excited to hear that your 4-year-old was determined to be a responder on the second attempt. I’m considering a second Kuvan trial since my results were determined inconclusive. Gotta get through grad school first. Once I do, I’m sure I can tackle head-on the challenge of eating & recording the exact PHE each day of the trial.


  7. Pingback: Kuvan Responder Trial: Prepping for a Second Attempt | PKU Parlor

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