Special Preview - One of My Recent Projects!

Hi everyone!

Sorry, this post is not really related to PKU, but I want to share a recent project that I was a part of. It was a music video for Will Butler of Arcade Fire starring Emma Stone. We shot the video on the Queen Mary and it was choreographed by Ryan Heffington- who choreographed "Chandelier" by Sia.

I can safely say that this is my favorite project I have worked on so far. We all really want this video to do well and reach a lot of people, so please share this with all your family and friends!

The video officially launches on Wednesday October 14th- for now please enjoy the preview video!

Diet Expectations USA versus Europe

I spent the first decade of my life living in Europe, and was treated at clinics in France and England. From my experience, the PKU clinics in Europe are more strict about the diet and see it as something to follow for life. Similarly, Europe seems to have more accessibility to PKU foods and alternatives. I remember when we lived in France there was this specialty foods store in Paris that had a TON of PKU treats. I would always get my Choquitos when we visited! :)

My mother's family is German, but my father is American. Around the time I was finishing elementary school, my family decided to move to the United States to be closer to my dad's side of the family. This meant that my PKU would now be treated by an American clinic.

I remember one of the early visits to the American clinic, my doctor at the time told me, point blank, that I could go completely off the diet. I was 11 at the time, and this was the greatest news anyone could have ever told me! I would finally be able to eat like all my friends! My parents, however, were not so pleased.

Since birth, all my doctors had told my parents that the low phe diet was for life. I remember my dad saying that he was upset that the doctor made the announcement with me in the room. Having heard this news from my doctor, who I considered the ultimate authority on my PKU, I thought I had an all clear to eat whatever i wanted.

This doctor retired not long after, and the new doctor I started to see was more in line with what we had been told as I was growing up. The old way of thinking for PKUers was that the diet was only important to follow as the brain was still forming, until about age 12 or so. But the more up to date research has shown that the diet should be followed for life.

I am curious to know if any American readers had a similar situation where they were told they could go off diet, and are you back on the diet now?

I'd also love to know why the old methods in the USA were so different than the rest of the world... Maybe a future blog post?

My Professional Work w./ PKU

I wanted to make a quick post to talk a little bit more about myself and I what I do. I have realized that I have been pretty vague in saying that I work in entertainment and have not clarified what it is I do. I have seen some people reposting on Facebook saying that I am an actor with PKU... Well, sorry to burst your bubble but I am not an actor!

I work in production as a production manager. Most of my time is spent in the production office, putting together the logistics for a video shoot. During my time on a shoot, I work with the various departments to ensure that everyone is on the same page and all our needs are in place for the shoot day(s). I also manage the budget for a production. Unfortunately, not as glamorous as some of you may be thinking- but it is still a fun job!

Working freelance in this industry presents its challenges with managing a low phe diet. Often when you are on production, working lunches will be taken care of for you by the company. This can vary from a restaurant order for those in the office, to a catered meal while you are on set. This is the story of any PKUers life, being faced with all those foods you love but know you shouldn't eat. It is much easier to pack a lunch at home so I can more closely monitor what I am eating. I much prefer to prepare my lunch the night before so that I can just grab it and go in the morning.

What do you all do for low phe work lunches?

Now that I have cleared up a little bit about what exactly it is I do, I will leave you with some samples of what I have worked on since moving out to Los Angeles two years ago!

1. Iggy Azalea feat. Rita Ora - Black Widow

2. Jason Derulo feat. Snoop Dogg - Wiggle

3. Alesso feat. Tove Lo - Heroes

Poll: Where Do You Find Your Specialty Foods?

I would love to hear from some of you about where you get any specialty foods you use to manage your diet. This could include packaged prepared foods, low phe flours and mixes, formula- anything you use substitute for higher phe foods.

Where do you buy your specialty PKU foods?

I order them online via a specialty foods company (ie, Cambrooke)

I order them online via a non-specialty foods company (ie, Amazon)

I buy them in my local, chain grocery store

I buy them at a specialty foods store

I do not buy specialty PKU foods

pollcode.com free polls

PKU History: Robert Guthrie

I can't speak for everyone who has PKU, but as someone who has this condition I must admit that I know very little about its history. There are some names that have been thrown around that I remember from various doctor visits. I decided to do a little research on the history of PKU and its treatment, and I will being doing several "PKU History" posts as I learn more.

Phenylketonuria was first discovered in the 1930's by a doctor in Norway, Asbjorn Folling. He was studying the condition in two small children with severe mental retardation, and found high levels of phenylalanine in their urine. This lead him to conclude that they were not properly breaking down the amino acid.

The first diet for children diagnosed with PKU comes around in England in the 1950's. The basic idea was to use a reduced phe milk to feed to the babies during the first six months. This showed great improvements in the mental function of PKU babies versus those who did not receive this treatment. At this point in history, the condition was found by testing the urine of newborns (the "wet diaper test") for high levels of phe, an indication of phenylketonuria. However, this test is not very accurate.

Enter Dr. Robert Guthrie, an American doctor whose fifteen month old niece was diagnosed with PKU. Around this time, PKU was being treated by diets low in phe, thanks to the work of Horst Bickel. The problem was that methods of identifying PKU at this time were not effective in catching the condition early enough.

Dr. Guthrie pioneered what would become known as the Guthrie test. Using blood samples and inhibiting bacteria growth using a phenylalanine antagonist, his test would allow the bacteria to grow if high levels of phenylalanine were present in the blood.

Initially, the tests were done on patients with known PKU as well as some who were undiagnosed. Once the tests were shown to be successful, they started implementing them in new born babies. In two years 400,000 babies were tested in the United States and 39 were diagnosed with PKU. Today, the test is used all over the world to screen newborns, with many countries requiring it by law.

Back on the Bandwagon

Hello again! Remember me?

I can't believe I let this blog fall to the wayside for so long. I was really enjoying posting, and have gotten some really great responses from people. I was recently having a conversation with someone about PKU, which reinvigorated me to continue my blog.

It has been a busy six months (or so) since I have posted. I have worked on several cool music video projects, done some traveling... and I got married!

My wife and I tied the knot on August 9th, back home in Michigan, overlooking Lake St. Clair. We had an incredible day with our friends and family. After the wedding we celebrated our honeymoon in Cabo San Lucas! We stayed at an all-inclusive resort, relaxed by the beach and pool, and went on a sailing/snorkeling cruise!

The resort had a ton of great food options, but unfortunately so many temptations high in phe! If anyone reading this has travelled to an all-inclusive, I would love to hear how you managed your diet while staying there.

I will be sure to post again soon! Just wanted to send a quick update and let you know to keep an eye out for more soon!

Best,
Christoph

Diet Formula

An integral part of managing PKU is to take an amino acid formula daily. Essentially, this formula replaces what your body misses out on by not being able to break down phenylalanine. There are many, many different formulas available and they come in various powders, mixtures, bottles, and pre-packaged "Capri Sun" pouches.

I have been on several throughout my life, but have been taking Maxamum XP since for about fifteen years now. Maxamum is an orange flavored powder that I mix with water and shake up in a sports bottle. I love the taste of it. The other people in my life are not so keen on it. It does have a very pungent smell- both in powder form and on my breath. I won't lie, this can be quite fun for me since I have become completely immune to the smell!

In order to get the appropriate nutrition I need to take in 180 grams of the powder each day. How I mix it with water is up to me. The downside is that Maxamum is pretty high in calories, especially since it is considered to be a dietary supplement and not recommended as a sole source of nutrition. According to the label on the can, in order to take in the correct amount of formula daily, I also take in over 500 additional calories.

Since moving to California about a year-and-a-half ago I have gotten in much better shape than I was. When I first met with my PKU doctor and dietician in LA, I was significantly heavier than I am now.

It is so easy to stay fit in Los Angeles. For one thing, Californians are much more conscious about what they put in their bodies than people in many other places I have lived. Also, people are more active here than other places. One of my favorite activities in my down time is to go hiking. I live about a mile and a half from one of LA's most popular hiking trails- Runyon Canyon. It is about a five mile journey roundtrip, and the views from the top of the canyon are spectacular on a clear day!

The second time I went to see the specialists at my PKU clinic, they commented that I seemed to have lost some weight. It felt good to hear them say that because I wasn't making any huge changes in my life, but was adapting to the lifestyle out here. During this meeting, they brought up the subject of Maxamum being rather high in calories and asked if I wanted to try some different formulas with a lower calorie count to help with my weight loss.

Over the next few months my dietician sent me samples of various formulas that were new to me. Some were similar to my Maxamum and required me to mix them with water, others came premade- but none of them were quite the same as my Maxamum. The flavors were off, or the texture was gritty- I had a hard time finding something I would want to replace my Maxamum with.

I had another appointment at the clinic recently. We discussed the formulas I had tried and that the flavors were too different for me to want to replace my current formula. My dietician then recommended I try a flavorless formula that I could mix with my current formula.

It arrived in the mail a few days ago, and I just tried it for the first time mixed with my current formula. I have found that even when something is called "flavorless" there is still a strange undertone to the taste. In this case, mixing it with my current formula, I do not taste anything strange. It only tastes a little watered down from what it normally is like.

Hopefully this time we have found something that can help me cut the calories I take in on a daily basis, but allow me to stick with the formula I have grown to love. I will continue to post as I try this new mixture and update on my weight loss goals!