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!

Review: Bahlsen Azora Cookies

I thought I would feature another wonderful snack from my childhood. Unfortunately, I know these are not marketed in the US- but I am curious if anyone has tried anything similar to them?

These cookies are produced by the German company Bahlsen. Bahlsen manufactures many different types of cookies, and I have seen some of them in the shelves of American grocery stores. However, I have never seen their "Azora" cookies state-side.

Azoras were a frequent snack of mine when I was younger because they are relatively low in phe. They have slightly over one gram of protein per fifteen (!) cookies. Granted, the cookies are relatively small, but I don't think I've ever eaten fifteen in one sitting.

What makes these "Azora" cookies so unique is that they are orange flavored! It is a very subtle flavor that blends with a more traditional vanilla taste. As corny as it may sound, I think they taste like summer. The flavor is fresh and the texture of the cookies is very light and crispy. It has the crunch of a waffle cookie without becoming splintery in your mouth.

These cookies travelled with us all the time. It was an easy snack to take on the road. And since they were considered a "normal" food, 5-year old me did not feel self conscious about having to eat them!

I have looked into ordering them online, and a website that my mother uses to buy German products online features them. At the very moment they are listed as out of stock- but hopefully they will come back soon!

Order at German Deli!

Every Rose Has Its Thorn: Drawing Blood

Part of life with PKU is having to take regular blood samples to test for phenylalanine levels, similar to diabetics drawing to test their blood sugar. Testing for phenylalanine, however, requires the blood to be sent to a lab for testing versus having an at home machine to do the reading for you.

Technologies for home blood tests have really improved in recent years to where pricking my finger is virtually painless. Nowadays, I use a small penlet that quickly pricks me with a sterile needle allowing for blood to be drawn. When I was younger, this process was much more complicated, and a little traumatic- for all parties involved!

Before the penlet, we were provided with sterile needles by the hospital (which resemble blades in my memory) that would have to be used by hand to break the skin and draw blood. My poor parents. One of them would have to hold me as the other held my hand and pricked me with the needle to do the monthly blood test. I didn't make it easy for them either!

I remember the testing supplies (and needles) were kept in a Lord and Taylor box- the ones that used to have the rose on them. I remember this so clearly because roses have thorns, and I associated that with having my blood drawn. Whenever I saw this box come out I would make a run for it and hide in the apartment. Under beds, behind the curtains- I think I exhausted every hiding place you could think of.

I can only imagine how hard this must have been for my parents. It was such a blessing when a friend of ours introduced us to the penlet. This device is roughly the size and shape of a thick sharpie marker and loads the sterile needle inside. There is a spring contraption which allows the needle to penetrate the skin quickly, reducing the sensation.

Needless to say, we made the switch to this new device! It was much quicker and less painful than before. As newer versions of the device have come out the pain threshold diminished as well. This technology also allowed me to start drawing my own blood as I got older. I could not imagine having to stick myself with a needle by hand.

Now if only someone could invent an at home testing machine, that would be great!

Review: Choquitos (Olé!)

For my next review, I thought I would feature a PKU food that I LOVED growing up! For those of you who don't know, I spent the first decade or so of my life in Europe. Now, Europe has some great products for a PKU friendly diet. Unfortunately, I have yet to find these treats stateside- kudos to anyone who may have some insight on how to get them sent to me in Cali!

Choquitos (manufactured by Taranis) look like rectangular chocolate truffles and come wrapped in festive metallic paper. As you can see above, they come in bags of twenty and are a great treat to keep in the cupboard! They are, however, not truffles- the center of the chocolate is filled with sweet, soft, and chewy nougat! They are specifically made for us who are following a low phe diet. Each nougat has about 11 mg of phe.

From a visual standpoint, these are great. The wrapping is fun and very easy to open- they twist just like a Jolly Rancher. The actual chocolate itself looks like a homemade truffle candy. If you ever go to a Kilwin's (anyone from the Midwest...?) these Choquitos look like they could be found on the shelves there. They are very transportable- perfect to stick in your kid's lunch!

The taste and texture of the Choquits are great as well. Again, they are not truffles, so they are soft and chewy- almost the texture of a caramel. It tastes sweet in the way that a marshmallow is sweet. Not sugary like a lollipop, but more understated. The chocolate shell is very thin and provides for a very light crack as you bite into the candy.

I can't begin to tell you how many happy memories I have of eating these as a kid. Inside the wrapper is a small paper comic, which I loved to look at as I ate my candy. This was one of those "special" PKU foods that I was happy to eat, and didn't mind other people watch me eat because I knew they were jealous. ;)

I can't figure out how these have not made their way stateside yet. If any of you reading this work for Cambrooke or Nutricia- *hint hint!* I think these would sell like hotcakes out here!

Thanks y'all for reading!

A Life of Alternatives

In my previous post I mentioned several foods that I never ate growing up, because of the high protein count. While people around me ate pizza and hamburgers, I had to eat some sort of low protein food that was often something completely different.

I remember one instance I was on vacation with my family, my entire family. My great aunt had (and still has) a cottage "up north" in Michigan. One summer, I must have been in late elementary school, we took a trip up with all my cousins. I mean all my cousins. There were probably about forty of us staying in the cottage that week. The first night we were at the cottage one of the cousins decided they would make sloppy joe's, since this was an easy way to feed such a large crowd.

Sloppy joe is definitely not a PKU-friendly food. Whenever it was possible, my mom would make me an alternative to what people were eating that resembled everyone else's food. This time, since the meal was sort of spur of the moment, there was no substitute for the sloppy joe meat. We would always take several packages of low protein pasta and other foods when we travelled to ensure I had something healthy to eat. Well, obviously, 11-year old me did not want to have pasta when everyone else was having sloppy joe. Of course I never had sloppy joe and probably would not have liked it, but the idea of having to have a special meal in front of all my cousins made me pout and refuse to eat. In retrospect, I probably called more attention to myself by refusing to eat than to have just eaten my pasta and shut up about it.

As I mentioned above, my mom would always try to replicate what everyone else was eating with my low protein foods. When the family had spaghetti for dinner, this was very easy because many diet food companies carry low protein pastas. The only major difference would be that I would have a meat free tomato sauce.

Meals such as this were easy, but my mom would get very creative when trying to replicate a meal where meat was the main dish. One example would be when she made schnitzel. For those of you who don't know, my mom is German, so this was a rather regular occurrence growing up. Schnitzel is traditionally made with pork, yet again not a very PKU friendly food. The sides however, usually some for of potatoes and salad, were fine. Rather than just eat the sides (and, heaven forbid, have a meal different from everyone else!) my mom would take a piece of my low protein bread and prepare like the meat. It wasn't an exact replica, but it looked similar to everyone else's plates- and I loved it!

I have very fond memories of these meals. A piece of bread that has been cooking in juices and gravy takes in so much more flavor than a piece of meat! As I try to get my low protein diet back to where it used to be, this is a method I really want to implement in my life again.

Review: Cambrooke Apple Breakfast Bars

So, one feature I have wanted to include on my blog is a review of PKU foods that I have tried. Sometimes they can be a little... interesting. But every now and then I come across some that I find delicious!

For my first review, I am going to feature the Apple Breakfast Bars made by the dietary food company Cambrooke Foods. I discovered these a few months ago, and I now I make sure I always have some in the freezer.

The breakfast bars come in packages of four, are individually wrapped and kept frozen. It is very simple to prepare one. You simply have to take it out of the package, put it on a microwave safe plate, and defrost for about a minute and a half. After it has cooled for about a minute, it is ready to eat!

The breakfast bar is essentially an apple turnover. It consists of a pastry-style shell which is dusted with cinnamon, and filled with an apple filling. The taste is very good! The warm apple is a really comforting taste, and the light cinnamon on top adds a great accent. 

The one down side of this food is the "pastry-style" shell. It's not quite as flaky as one would expect a turnover to be. And since I heat it up in the microwave, it rides the fine line between being soft and being chewy. 

One big plus for this, however, is how incredibly convenient it is. Preparation takes about two minutes, and the bars can be eaten on the go. Since they come frozen, they keep a very long time as well. This is the type of food that can be taken to work, traveling, and virtually anywhere you can find a microwave.

Overall, I would highly recommend this breakfast bar. The taste of the turnover is excellent, and the convenience of it far outweighs the less-than-perfect "pastry" shell.

Rainbow Bread

Hi everyone! My name is Christoph, I live in Los Angeles, and I lead a pretty typical life, except for one small detail... I have a genetic condition known as phenylketonuria.

You most likely don't remember, but as a baby chances are that you underwent a heel prick shortly after birth, as a part of newborn screening. For most families this is a small detail that you never look back on. For my family, however, this changed the course of our lives forever.

Phenylketonuria is caused by a defective enzyme called phenylalanine hydroxylase. Without a properly functioning enzyme, phenylalanine from protein can build up in the blood and hinder brain development and function. In other words, I should not be eating protein rich foods, such as meat, fish, and dairy. Although this is a condition I will have for the rest of my life, the formative years of the brain are a crucial time to be following a strict low protein diet.

This is where my mom comes in. When I was a small child through the time I moved away from home, she would make special food for me. Growing up with PKU I couldn't eat regular bread or pasta, couldn't drink regular milk... the list goes on and on. Looking back on it now, I appreciate so much what she did for me and I know I was not always appreciative back then. As a child, all I wanted to do was fit in with everyone else. Having separate meals from all your friends may seem like it would make you feel special, but it makes you feel more like a spectacle.

My mom would make low protein bread from special mixes we got to manage my diet. To make it special she would sometimes put different colored food dye into it to make the bread rainbow when it came out of the oven- I thought that was so cool! I remember I was in first grade, at lunch, and my mom packed me a sandwich. I was sitting at the table with some of my friends, and some second and third graders (very intimidating to a first grader). I unwrapped the aluminum foil from my sandwich to find that she had made it with the rainbow bread. Someone sitting next to me saw it and got the attention of the whole table. An older kid sitting at the head of the table yelled "look, he's got rainbow bread!" I can clearly remember him and all the kids at other tables turn their heads to look at my special sandwich. I had never wanted Wonderbread and bologna more in my whole life.

This is the first time I remember feeling that I was different from most kids my age because of PKU, and that feeling never really went away. I admit that I have not been managing my diet as well recently as I should be, and that is part of why I am starting this blog. When I first moved away from home to go to college was the first time I had to manage my low protein diet on my own. Being on a college campus, providing for yourself for the first time is very different from living at home. I started to give in to the food, ready for taking, at the cafeteria and started to loosen on my diet. Again, I just wanted to be like everyone else.

As a part of my treatment for PKU, I have to drink a formula every day. Basically, it replenishes my body with what it looses by having the defective enzyme. Luckily for me I love the taste of the formula and have never had a problem drinking it. College was the first time I really drank the formula around anyone except my family. Naturally, I wanted to hide it because I thought people would find it weird or different. I drink my formula throughout the day, so I will usually have a bottle of it sitting out. So it was only a matter of time before people started to see it.

Of course people thought it was "different," but not in a weird way. As more of my friends learned about my PKU and what I was drinking they seemed interested in it since they were unfamiliar. Over time, it just became a fact of life that I had PKU and had to drink this strange orange mixture.

There are definitely struggles living with such a condition. It is hard to grow up having to be different from others. It is frustrating to know that the reason I don't feel as focused, or am more irritable, is because I ate too much protein. All I wanted was to eat a burger! Is that too much to ask!?

With this blog, I will share anecdotes of my life growing up with PKU and dealing with PKU as an adult. Hopefully I can get myself back on a better track with managing my low protein diet this way, and I also hope that families who are raising a child with PKU can take comfort hearing from someone who has been through the struggles.

I'm looking forward to me next post already! :)

- Christoph

That is me on the left, with my fiance Molly. We're getting married this summer!