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.

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!

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.