Have You Heard About PKU (Phenylketonuria)?

Hi Guys!!
Well, let me start out by saying I’m not an expert on PKU. My 16 y/o son has it. I’m writing this post just to open the door for anyone who would like to discuss it (now, or through a search in the future), has an opinion about it, or would like to learn about it. I’m not going to go into great detail with a bunch of medical terms or scientific facts. I’m just writing what I have learned though our experience with it. I will link a couple sites at the bottom for anyone who would like more details.

PKU or Phenylketonuria is a metabolic disorder controlled by diet. It is inherited through genes. If both parents carry the recessive gene (like in my situation) all offspring have a 25% chance of having pku, 50% chance of carrying the gene, and a 25% chance of not having the gene at all.

When my son was born not all of the 50 states were testing newborns via a heel prick blood test. Thankfully ours did, and at 10 days old we were told our baby had pku. I think it’s like 1 in 10,000 have pku, and 1 in 50 carry the gene.

Here is how I simply explain it to people:

Practically everything we eat contains some kind of protein. Proteins contain many different acids; one of them is called phenylalanine (phe). Some foods have a lot of this “phe” like, meat, eggs, and milk. Some foods have a little like most fruits and vegetables. Our bodies create enzymes to break up these acids. People with pkus bodies don’t make a sufficient amount of the enzyme the body needs to do what it should with ‘phe’. Left untreated the phe builds up in the blood and deteriorates brain cells. Long ago babies with pku would become mentally retarded. Today through a very strictly calculated diet, a special drink, and a very new drug called Kuvan, kids with PKU do VERY well!!!

Ok, that’s all I have to say about that for now. Of course any questions or comments are welcome.

OH yes I wanted to say this. Seems like more and more people today know, or have heard about, PKU. Some of you here may have heard about it in discussions about Aspartame. Products that contain Aspartame sometimes have warning labels. Those foods are “never ever foods” for Pku people because (I Think) for some reason no one has a definitive answer to just how much phenylalanine is in it, therefore we can’t calculate it into the diet.


Thanks!!


newborn screening
Info
PKU news

Thank you for your VERY informative post.
I have seen the cautions on diet soda cans when I used to drink it and always wondered what it meant,now I know!

Best of luck to You and Yours

So what is a typical day of food look like for your son?

reply to post by smilesmcgee



Thanks Youjustneverknow!!

Smiles . Well the only thing he eats from the store are fruits, veggies, and a few other things like drinks and snacks made from fruit or veggie. The bulk of his diet we purchase from companies that make low-protein foods. We buy pasta, rice, bread, cakes, and many ingredients that we use to make things like pancakes, cakes, cookies, or breads. It's a very limited and boring diet, we are always looking for new ways to change things up. It dosen't help that he is picky and dosnt care for a lot of the things he can eat a lot of!!! LOL thanks for your question!

Originally posted by MidnightSunshine
When my son was born not all of the 50 states were testing newborns via a heel prick blood test.

But on ATS that just means another conspiracy theory...
DNA from millions of newborn babies is secretly stored
www.abovetopsecret.com...

reply to post by MidnightSunshine



hi,
I am curious, do you purchase your foods through Cambrooke foods?
I used to work in one of thier warehouses, and worked directly under the founders and owners. They started company because of the way the foods were for thier sons and now they have a great team researching products and coming up with new ideas and products all the time. They really have taken a passionate approach to issue and are very involved in that sector of the medical community.

You should look them up....Cambrooke foods, Ayer Mass.
GL

here is a link....
Cambrooke foods

some of the products they are making available now were only being developed years ago when i worked thier, good to see they are making progress.

reply to post by owtFsink


YES all the time, my son likes their food the best!! How cool, you worked there!! YES the defiantly are far more concerned with the look and taste of food for pku kids. Some places sell pasta that turns into a sticky starch ball, or falls apart. Some sell bread hard as a rock...Cambrooke is awesome!! Thanks Owt!

I worked at a Preschool in '92 and a little 3-yr-old girl had PKU. I was in that teacher's room one day when she was giving out the snack, and the little girl got out her few saltine crackers (I think that's what she ate.)

I had never heard of PKU before, and the teacher told me how serious it was, and how she had to watch like a hawk to make sure some well-intending child didn't try to share their snack with her.

My biggest question is: How do their bodies grow and develop without them eating protein? Isn't protein essential for cell growth?

reply to post by new_here


Good Question. It's in the special drink All of the nutrition from protien, (except for the phe acid, which they get in a calculated amount from food) is in the drink. I have a giant book with a list of every natural food, and manufactured food. It tells me how much phe is in everything.

PKU parents learn to poke the finger, blot the blood on a special paper that we send out in the mail to be tested. At first we do it about once a month to see fast the body is breaking up the phe. The dietitian then decides how much phe the child's body can handle in a day, we call them exchanges. My son was allotted 15 exchanges per day. Just for a reference, a piece of wonder bread has 7 exchanges of phe.

PKU is not like an allergy. If a child were to eat like lets say a peanut butter cracker one day at school, nothing would happen. The child might be a little hyper or crabby for a day but he would be fine. BUT if he were to eat a peanut butter cracker everyday, there would be problems. It takes a long time (months) of not following the diet before the effects of the cell deterioration are evident.

I saw what happened before they discovered this. When I worked at a facility for severely retarded men. One of them had this disorder. It was the only thing wrong with him, but it wasn't caught and his brain was destroyed. You're son is very lucky he was born after they found out about this.

reply to post by MidnightSunshine


It's great to hear that your son is doing well, OP. Same for others with their own or friends/family's children with the disorder.

I know of the disease mostly through a friend from college. He was in my major (philosophy) and we had many classes together and took a few philosophy club trips together. Anytime we had a meal for the department or club, he was the only one at the table with the vegetarian entree (at a rather conservative school with probably only a handful of other vegetarians). One day I asked him why he didn't eat meet, and he explained the disorder. It was obvious he had good care since childhood as he was extremely intelligent, and about as far from "mentally retarded" as one can get.

It's a sad thought to wonder how many children throughout the eons before modern medicine were unnecessarily affected by this disorder. Although it often gets flack from this community, kudos to modern medicine on this one.

Originally posted by MidnightSunshine
reply to post by owtFsink

 

YES all the time, my son likes their food the best!! How cool, you worked there!! YES the defiantly are far more concerned with the look and taste of food for pku kids. Some places sell pasta that turns into a sticky starch ball, or falls apart. Some sell bread hard as a rock...Cambrooke is awesome!! Thanks Owt!

YW, it was a great place to work, hard work but it gave me a different perspective on some things. It was sometimes "interesting" when the test foods were being prepared, they would send samples throughout the buidling and we would give them feedback on taste and texture and other things, it didnt always start out as the product they ended up with....
Things were hairy for a while there after the Earthquake/Tsunami in Japan as one of the main ingredients they used in thier rice and pasta's was shipped from a port not too far from the Fukashima area. IT took some time for that company to re-establish thier infrastructure and then when it finally got to us, the FDA had it under quarantine in the ports and then again in our warehouse. They had someone come and check it for radiation in our warehouse at least 3 times (over a few weeks) before they cleared it, the whole time the VP of operations, my boss, was more concerned about a new "safer" supply that would be better for the kids eating it....
Definitely a good company, in my opinion.

How about you elaborate and show what kind of diet your son is on? Very helpful for others i'm sure. Thanks for sharing this and the best of luck to you and yours.

Originally posted by MidnightSunshine
reply to post by smilesmcgee

 

Thanks Youjustneverknow!!

Smiles

. Well the only thing he eats from the store are fruits, veggies, and a few other things like drinks and snacks made from fruit or veggie. The bulk of his diet we purchase from companies that make low-protein foods. We buy pasta, rice, bread, cakes, and many ingredients that we use to make things like pancakes, cakes, cookies, or breads. It's a very limited and boring diet, we are always looking for new ways to change things up. It dosen't help that he is picky and dosnt care for a lot of the things he can eat a lot of!!! LOL thanks for your question!

reply to post by metodex


I kind of thought I did. I did forget to mention the "NEVER EVER FOODS"
No meat, fish, nuts, milk, eggs cheese....
Also didn't mention we can also buy low pro cheese, we do a LOT with that...grilled cheese, pizza(low pro crust) w/ veggies, mac n cheese, soups, toppings...
His favorite foods are mac n cheese, pickles, broccoli, french fries, and cola
Because my son is so limited in his diet, I let him get away with some things... Like drinking pop or energy drinks.

Things are different for him. Like this weekend, It's Halloween!!! He went trick or treating till about 10 years old, then he got tired of getting candy for me Kidding...But it did suck to have to dump out his pillow case every year and separate it. He can eat as much 'candy' (nerds, smarties, jollyrachers, peeps, starbursts...) as he wants, just no chocolate. He never really cared for candy though...He next to never eats it now. AND holidays are a little different for him at first, but we are lucky to have (for the most part) a pretty smart family, and my Mum and Aunts learned what to do for him, so his special diet to us was and is..Just the way it is.

I get A LOT of people who say things like "Oh my god that's awful,poor thing...or...I don't know how you do it...But when it's like that from day 1, It's all you know I didn't really realize just how much time we spend on his diet until 4 years later when i had a baby without pku.

HEY I JUST REMEMBERED SOMETHING IMPORTANT lol
He barely EVER gets sick. When he was a toddler he got sick a few times like any other kid, but I swear that kid never gets sick now. Seriously, since he was about 8 years old or so...No colds, No flu.... nothing! He gets an occasional head ache but that's it.

There were other posts above this one that I wanted to comment on and I will

Thank you all for your intrest and replies