Attitude · Blood Levels · Diet Compliance · Formula · Living with PKU · Moms · Promotion/Awareness · Science

My Passion

Diet compliance: How can we better empower people with PKU to follow the diet and reap the benefits?


PKUers Supporting Each Other

I think the true mark of accepting a challenge we’ve been given in life is turning around and trying to help others going through the same thing. Put differently, I think one of the best things we can do to deal with our own adversity to is look around for someone else to help.

I have seen so many of you in the “PKU world” as I call it, doing this very thing. We have a unique challenge in dealing with this disorder, and we long to connect with others and support each other in it.

It seems like because we all have different experiences and gifts, we all have a different niche where we try to make a difference. Some of you have Facebook pages and groups, some of us have blogs. Many people share low-protein recipes that have turned out well. People who have been off diet and returned offer support and encouragement to those embarking on that journey. Some people do the important work of advocating for PKU around the world – whether it’s for newborn screening, funding for medical foods, or simply awareness. This is not to mention all the scientists working on a cure and the medical professionals trying to serve our community. All of these things are so valuable and we all have a way we can make a difference!

My Passion: Diet Compliance

So what is my niche? We all have different areas that fit our personalities. I like having a blog where I can share positive things that have helped me in living with PKU. On the other hand, advocacy, for example, is totally out of my comfort zone. I am so glad there are people out there who are advocating PKU causes. But that is just not for me.

What is my passion? What I have come to realize is that the area of PKU I am most passionate about is diet compliance. That is, finding out why so many struggle with this and helping them make it happen. There are a few reasons for this:

1) I see it as an area that needs a lot of help.

2) I feel that it is an area that has great potential for improvement without requiring any major changes in government policy or huge amounts of funding.

3) My experience following the diet has totally blessed my life, and I want to see others experience that as well.

4) My background in Health Promotion gave me a little bit of knowledge on how to influence behavior change, and I would like to apply it here and see others do the same.

Let me expound.

Reason # 1

While PKU is a disorder with potentially serious consequences, it has fortunately been discovered and is completely treatable. Medical formula providing low-Phe protein, vitamins and minerals provides nutrients and calories for PKU patients. The low protein diet prevents neurological damage that would otherwise result from high blood Phe levels. Effective treatment is available and yet compliance to this diet and lifestyle is a huge problem among many people with PKU. This of course means many people are suffering from the troubling symptoms of high blood Phe levels.

J.H. Walter et al performed a study that showed that “adolescents and young adults generally do not comply with recommendations for the monitoring and control of phenylalanine concentrations”. In another study, Vernon et al noted that 75% of adults are essentially off-diet. Clearly, there is huge room for improvement.

Reason #2

While some people may feel it is important to raise money to discover a cure for PKU, I feel that because it is completely treatable, it would be a better use of time, energy and money to find out 1) Why people aren’t following the PKU diet and 2) What would help them follow it. Finding a cure may be way off in the future. Helping more people follow the diet, on the other hand, would improve the quality of life of thousands of people today.

How can we do this? I’m not exactly sure. I am hoping that by starting this conversation people will come to the table with ideas.

I have thought that it would be worthwhile for a university to conduct a major survey to try to isolate all the many factors that influence diet compliance and which ones cause the most trouble. This would give health professionals the information they need to develop better programs to help their PKU patients. In hopes of inspiring such research I have developed a simple, non-professional survey of my own that I plan on sending out soon in order to publish the results online and raise awareness.

Reason #3

Even without a cure, having PKU hasn’t stopped me from doing anything that I have wanted to do in life. Anything! But I realize that this is because I have followed the diet and drank my formula consistently. Doing this allowed me to continually have safe blood levels and suffer absolutely no damage to my brain. I have been given every opportunity I could have asked for in life. I think I’ve expressed that a lot on this blog so I will leave it at that. But this is what I wish everyone with PKU could say and that is why I am so passionate about encouraging others to completely commit to the diet and reap the benefits.

Reason #4

Like I said, everyone has different gifts and things to give. Doctors and dieticians for example have a very important role in treating PKU. At one point I actually thought I wanted to be a dietitian, by the way. I applied for the program at my university. I didn’t get in but soon discovered that there was a different field that was a better fit for me. I choose to study public health with an emphasis in health promotion. This field takes a more preventative approach to medicine. It explores how we can empower and enable people to make healthy choices for themselves. This philosophy really speaks to me.

In my classes I learned about the mind/body connection – how our emotions affect our physical health and vice versa; I learned about positive psychology and all the things we can do to prevent anxiety and depression and have a happy life despite any challenges that come our way. I feel this type of information is very relevant for the PKU community because of how easy it is to get down about this disorder and have negative thoughts. Also the fact that not following the diet brings on symptoms of anxiety and depression makes these findings especially valuable to our population. I aim to share these helpful lessons with others by posting about them on my blog and Facebook page. This way I hope to empower people with PKU to come to accept PKU, commit to the diet and have a positive outlook on life. I would like to see a more holistic approach in supporting people with PKU because after all, food isn’t just physical, it’s emotional!

In my major I also learned how to develop effective health programs. One really important facet of this is called conducting a “needs assessment.” The idea is, before you can help people change a health behavior, you have to know what is influencing that behavior so you know what to tackle. This requires studying the population and their needs. I just wonder how much of this has been done in the PKU community. Only if we know what things are affecting diet compliance can we have any influence on it. I would love to see more of this studied and applied to the area of PKU treatment.

Getting the Word Out

By posting this I just wanted to let the world know what I’m passionate about and find out if there is anyone else who feels this same way. What is already being done in this area? I want to know about it! Please contact me.

Also just wanted to put a bug in the ear of anyone who is willing to listen. Maybe people have ideas for how we can tackle this and we can start sharing. This movement could benefit from the combined efforts of doctors, dietitians, universities, public health professionals, social workers, psychologists, non-profits, public relations, journalists, advocates etc. I hope to share this with as many people as possible and hope all my readers will do the same.


Vernon, H. J., Koerner, C. B., Johnson, M. R., Bergner, A., & Hamosh, A. (2010). Introduction of sapropterin dihydrochloride as standard of care in patients with phenylketonuria. Molecular Genetics and Metabolism, 100(3), 229-233. doi:DOI: 10.1016/j.ymgme.2010.03.022

Walter, J. H., White, F. J., Hall, S. K., MacDonald, A., Rylance, G., Boneh, A., . . . Vail, A. (2002). How practical are recommendations for dietary control in phenylketonuria? Lancet, 360(9326), 55.


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