Tis The Season

As we all know, it is flu season. We are all aware of the extra precautions to take in order to stay away from the tissues, cough medicine and all that yucky stuff. Well, normally, you would just wash your hands a lot more than normal and take extra vitamin C. It's a little more complicated for someone with PKU.

It's much harder to stay feeling healthy when you have PKU. Not only do you have to wash your hands regularly and take vitamin C, but you also have to take zinc. Protein has zinc (of course) and that's what helps your immune system stay healthy. You also have to make sure to eat right and follow the diet as closely as possible to make sure you are feeling healthy to begin with. If you aren't feeling well in the first place due to high protein levels, then the flu will knock you out.

I already had the flu this year and it was not fun. I had a flu shot, so luckily I didn't get it as bad as I could have. Like I have mentioned before, I have not been following the low protein diet closely in just over 4 years. So, I was hit hard with the flu this year. I am so glad I had gotten the flu shot, or I would have been in trouble.

It sounds really easy to stay on top of PKU. But there are a lot of things that you have to do and it takes a lot of money. The medication I mentioned in my last post, Kuvan, on average it costs $57,000 a year and for the maximum dose, $200,000 a year. You also have to take into consideration the traveling costs to see all your doctors. In my case, I have to drive 4 hours or 220 miles.

Not only is it the medications and the formulas expensive, but you have to think of the grocery bill. Someone with PKU eats very often. We can't have much protein, and protein is what makes someone feel full and stay full. Someone with PKU eats more often and more because we never actually feel full. So, it's only reasonable you have to buy more food. Well, that adds up.

Anyways, stay healthy everyone!!

Until next time,
Phe

A New Medication!

There is a medication for everything now days. All you have to do is go to your doctor and tell him or her what your symptoms. Well until now, I couldn't do that with my PKU. I was doing a little research and I found there is a medication to help make my life a little easier.

I was looking for a topic to write about tonight when I stumbled across a page that talked about a prescription. It's a medicine called, KUVAN. It acts with an enzyme in your body to reduce blood Phe levels. It would in a sense take the place of formula, but in a different way. Formula gives me the protein I need when it's already broken down. This medication would make it so I didn't need my formula. It would break down the protein for me.

I just found out about this medication threw my research. I haven't seen my doctor (for PKU) in almost 4 years. Guess it's time to make an appointment!! I will let you know how it goes!!

Until next time,
Phe

Something Fast...

It's been a rough few months for my husband and me. We moved 165 miles away from where we were living with less than a weeks notice. My husband's job went south (they wanted us to move over 1000 miles away than where we were with 2 weeks notice or find a different job). So, we found a different job. With moving with such short notice we didn't have a place to stay. My grandparents generously offered their travel trailer for us to stay in until we found a place.

It's not so easy to cook in a travel trailer, as you can imagine. So, we have been improvising. We both work and have to take lunches to work. It only takes a little while to get sick of sandwiches. So we started buying frozen meals, like TV dinners. Have you ever looked to see how much protein is in some of those??? I never thought to look (that was dumb of me).

I started not to feel so good and I couldn't figure it out. I was going grocery shopping when I decided to look at the protein content in everything. Well, most of the things I was buying had about just as much protein as I thought... that was until I got into the frozen food section.

Marie Callender's
Smokey Cheddar Mac                                15grams
Stuffed Pasta Medley                                  21 grams
Fettuccine Alfredo with Garlic Bread           24 grams
Pasta Al Dente Rigatoni Marinara Classico  19 grams 




I mean it makes sense why there is so much protein. It is a MEAL! I guess I didn't think about it. There aren't many frozen meals that people with PKU can have (without meat). But I guess, there is no such thing as a "fast meal."

Until next time,
Phe

It's Been Awhile!

Man, it seems like it has been forever since I have posted. I have been quite the busy bee lately. I got a new job that has kept me busy, plus looking for a new place, and of course taking care of my husband and the dog. It sure doesn't seem like much in comparison to the things some of you have to do on a daily basis, but it's a lot for me. Especially, since I went from taking care of the dog and my husband and nothing else.

Well, now that Thanksgiving has come and gone I want to share some of the things that I am thankful for this year. First of all, I am thankful for my wonderful husband who works so hard to take care of me and make sure I am happy and comfortable. Of course, I am thankful for all of my family. They are all so wonderful.

I am, also, thankful for my church and salvation. I don't know about everyone who reads this, but I know I wouldn't be here today if it weren't for God's grace and mercy. I am thankful for the country I live in. It has the technology to detect and diagnose things like PKU that can be treated. I am thankful for so much more, but I don't want to take the time and list them all.

I hope you all had a wonderful Thanksgiving and are looking forward to Christmas as much as I am. Only 21 short days until Christmas.

Until next time,
Phe

Turkey Day!!

Thanksgiving is upon us. What is the first thing you think about when you year Thanksgiving Day? Some think Football, some think family. The most common response to that question is Turkey. Have you ever thought about having Thanksgiving dinner without turkey?

For people with PKU, it's a common thought. They hear Thanksgiving and the first thing they think of is NOT turkey. I think of the Macy's Thanksgiving Day Parade. Honestly, Thanksgiving dinner is just the same as any dinner, except there is more family around to share it with.

At my family dinner there is turkey, ham (sometimes), mashed potatoes, stuffing, green bean casserole, cranberry sauce, gravy, and an assortments of pies. So, of course no one goes hungry, but no turkey. Try having one plate at Thanksgiving dinner with no meat.

Until next time,
Phe

Low Protein Diet

Some people say the Low Protein Diet, for people with PKU, is a life long diet. The doctors and nutritionists recommend it as a life long diet as well. But is it really that important?? What would happen if someone with PKU went off the diet??

As a child, I grew up thinking that every kid my age were lucky to not have to drink the protein formula that I did. I wasn't envious by any means, but I did look forward to the day that I didn't have to drink it everyday. I mentioned it to one of my nutritionists at one of my appointments, and that was when the speech started. "Diet For Life!!"

Well in the past few years, I have not listened as well as I should have. I hate to admit that I am wrong, but.... I am. I had no idea of the problem that it could cause! I started developing a rash on my arms and neck. It wasn't until recently that I found out that high protein levels can cause eczema.

I, also, found that if someone with PKU were to have been off the diet for awhile, they could experience some hair loss. Nothing extreme, but hair would still be falling out, more than the normal shedding. I haven't experienced this one, if I have I didn't notice.

Some people have a hard time controlling emotions. Something that would normally be annoying could make someone with PKU want to scream in anger. I have noticed some irregular emotions, but nothing close to screaming in anger.

I'm sure there are a lot of other things that are caused by a high level of protein in the blood stream, but this is all I know of at the time. I will keep looking and post if I find any others.

That being said, I have to agree with the doctors and nutritionists. DIET FOR LIFE

Until next time,
Phe

There is a Time for Change

I have been telling you a lot of things about PKU lately. Basically, I am just informing you and not talking about the daily struggles. That is going to change a little bit. This blog is not only to inform and make you aware of PKU, but it is also to help those who have loved ones with it as well.

Having PKU is difficult, but not as difficult as living with someone with PKU. Going grocery shopping for a household that includes someone with PKU is hard when you yourself don't have PKU. You have to think about what is healthy, what everyone likes, and how much protein is in the food you are buying. You have to stay aware of how much food you are purchasing that the one with PKU can actually eat.

The "normal" person making a meal for the household needs to think the same way when cooking. "How much protein is in this?" "How much food did I make that _____ can eat?" It's not easy.

Now I wanted to start doing something new. I have been trying to keep count of my protein intake lately. I'm not doing very well by myself. So, I am going to start putting the amount of protein I ate/drank the day before I write a new blog.

Thank you for everyone reading. Today's blog has been a little scatter-brained.

Until next time,
Phe

Intake: 16 grams

How Often Does it Occur??

I have said before that PKU is not very common. That wasn't the complete truth. It depends on what part of the world you are in. In one country it could be more common due to intermarriage.

United States 1 in 25,000
Ireland 1 in 4,500
France 1 in 13,500
Turkey 1 in 2,600
Scotland 1 in 5,300
Iceland 1 in 8,400
United Kingdom 1 in 14,300
Norway 1 in 14,500

Denmark 1 in 12,000
China 1 in 17,000
Italy 1 in 17,000
Chile 1 in 18,916
Canada 1 in 20,000
Yugoslavia 1 in 25,042
Finland <1 in 100,000
Japan 1 in 125,000

As you can tell, it differs from country to country.  I find it interesting to me how it differs. Looking at all these numbers makes me feel extremely blessed to have been born in the United States, because it's one of the countries that have the low PKU birth rate. But I guess it really doesn't matter considering I was born with it. Anyways, I just wanted to clarify that statement.

Until next time,
Phe

Scales Are Not Our Friends

Friday, I wrote about diets. I wanted to be clear that not all diets are unsafe for someone with PKU. Most of the very effective ones are not, I want to be clear that there are some diets that work great.

Grapefruit Diet
Eat half a grapefruit before each meal. Grapefruits have about 2 grams of protein in them, so it's safe. There are many variations of the diet but it is still safe.

If you think about the amount of diets that are available for someone with PKU, you can understand why the over 2/3 of all people with PKU are obese. Someone with PKU doesn't ingest enough protein to stay full for a long period of time. Potein is what makes you feel full. Since they can't have much protein they don't stay full for long periods of time.

The amount of fatty acids that are ingested by someone with PKU is a lot more than your average person. The PKU formula has a large amount of fatty amino acids which make up proteins. With ingesting that much fat and fatty amino acids, it would be hard to not struggle with your weight.

I can't wait for the day when my PKU won't cause weight and many many more issues.

Until next time,
Phe

Dieting Stinks!!

Have you ever had that moment where you are looking in the mirror and you notice your favorite outfit is fitting a bit snug. You think to yourself, "I need to go on a diet!" Dieting for some people is a very normal and easy (ish) thing. For someone with PKU it is a very hard thing to do.

Other than the low protein diet that all PKU patients should be on, there isn't a safe diet to go on. Most diets are based on PROTEIN, PROTEIN, PROTEIN!! Obviously, that is not an option for someone with PKU. Every diet has something that a person with PKU will have a problem with.

Here are a few examples...

Slim-Fast
Although their Shakes are delicious the contain 10 grams of protein each.
The Snack Bars aren't too bad, with 1 gram of protein, but they aren't really nutritious for you either.
The Meal Bars have 8 grams of protein.
The Shake Mixes are not much different than the shakes, coming in at 10 grams.

Atkins
Snack Bars come in at 8 grams of protein.
Breakfast Bars have 10 grams of protein.
Dessert Bars have 5 grams.
All Purpose Baking Mix has 20 grams.
Pasta has 11 grams.

South Beach Diet
Meal Bars: 12 grams of protein.
Snack Bars: 6 grams of protein.
Breakfast Bars: 9 grams of protein.

As you can see, all diets are based off of high protein levels. There are many more but I figured you wouldn't want to read more, you get the point.

Someone with PKU is already on what is known as the "Low protein diet." They get their protein from a formula, there are many different brands and flavors. They make shakes that have the daily amount of protein already broken down, or missing a piece known as phenylalanine.

There are no real diet options for someone with PKU, the only thing we can do is watch our portion sizes and exercise. That gets very difficult at times.

Until next time,
Phe

A Little More About Me

I haven't posted for a few days, I wasn't exactly sure what I wanted to write about. I realized in my first post I didn't tell you about me, I just told you about PKU. So here is a bit of my story...

My name is Laura, I am 20 years old. I live in North Bend, Or, with my husband of 8 months,Chipper, and our puppy, Peach. We just recently moved back here from Medford, Or. We are so happy to be back around family and friends.

Obviously from my previous posts, I have PKU. I have what's called hyper phenylketonuria, meaning it's the mildest version of PKU. Everything I have posted about affects me in one way or another.

Growing up people would ask me how I could stand not eating steak or hamburgers. They never understood that I didn't and still don't know what I am missing. I don't really care about eating meat, I am more concerned about the health and behavior issues that comes along with PKU. I want to not have to worry about getting pregnant unexpectedly and having to take extra precautions just in case I do.

The purpose for this blog is to spread awareness of PKU, help others who have PKU or knows someone who has it, and to keep track of myself. I want to feel that I am held accountable by my readers for my phe levels and my health.

If you have anything you want me to write about or questions you want me to answer please comment on the most recent blog post. Just let me know and I will do my best to write about it.

Until next time,
Phe

A Cure??

For as long as I can remember, the nutritionists I go to have been taking about a cure for PKU being only 2 or 3 years away. Well, that hasn't happened obviously. But it does give one hope to think about it.

The last time I went to my nutritionist's office (4 years ago), they told me that scientists were working on a pill or tablet that with subside the symptoms of PKU temporarily. I thought that would be great, but once you think about it... It "subsides the symptoms" well that couldn't do much good. That means I could eat all the protein I wanted and it would still affect me internally. The only difference there would be that I wouldn't feel depressed or aggressive.

Recently, I stumbled onto this article online that was dated in 2005. It talked about shots in the liver might cure PKU. The scientists, Dr. Woo and Li Chen, first started with mice that had PKU. They developed a shot that was administered to the liver of each of the mice.


The scientists were able to cure PKU in mice with just three injections. The levels of phenylalanine in the treated mice dropped to a normal range and remained stable thereafter. Their fur color changed from grey to black, that means that the body was producing normal levels of melanin, or pigment, which is under produced in mice and humans with PKU.

This is promising to me, and all others who are affected by PKU. I pray that it is able to be given to humans very very soon.

Until next time,
Phe


http://www.outsourcing-pharma.com/Preclinical-Research/New-gene-therapy-technique-cures-PKU

I Can't Blame PMS

Have you ever been around someone who was angry or sad/down and they couldn't tell you why? That's because it doesn't happen that often. For someone with PKU it could happen quite often, if they aren't careful.

I started doing some research last night. While researching, I found some interesting and helpful information. There has been a study done recently that links aggression and depression to PKU patients. When a person with PKU goes off their low protein diet it can cause them to be more aggressive and have a short temper. This is because the phe levels are too high and causes excessive amount of phes in the brain at any given time.

That is useful for me because I have been off the diet for 4 or so years. I have been noticing myself to be more aggressive and easily upset. I get annoyed very easily, which is not normal for me. That tells me my protein levels are too high. I have been cutting down my protein intake and I have noticed a difference. The problem hasn’t gone away completely, but that’s because I’m not on the formula as well as the low protein diet.

Not only can protein levels make someone aggressive, they can also cause depression. There are several reports in the medical literature that suggest a relationship between high phe levels and depression. Sometimes when the phe levels are too high the aggressive behavior and depression can alternate.

I have never experienced the depression side of it, but it’s different for every PKU patient. Like I said in my last post, maybe they will find a cure for PKU in the near future so every person with PKU can rest easy and not have to be on their toes looking for warning signs all the time. I know that would make my life a lot easier.
Until next time,
Phe

Maternal PKU

Every little girl plays with baby dolls. They have dreams of growing up and becoming a mommy. Some women have very strong maternal instincts, they want and feel like they should have children. Women with PKU have to look at pregnancy and having children differently.When a woman wants a baby they make the decision and starts trying. It's different for a woman with PKU. There is a lot of planning that goes into it.

First, the woman with PKU decides she wants a baby. The man she wants to have the baby with (hopefully her husband) needs to have a DNA analysis done. She needs to find out if he is a carrier of the mutated gene that causes PKU. If he is then there is a 25% chance that they will have a baby with PKU. If not, then the baby will be a carrier.

Second, she needs to see her PKU nutritionist and dietitian. She needs to go back on their low protein diet or go on a stricter diet. The woman has to keep the Phe (or protein in the blood and brain) from 2 to 6 mg/dl (milligrams per deciliter). If the woman is not on her diet at all, it takes about 2 months on the diet to get the protein levels down to the healthy levels. If she is on her diet, she needs to get use to her stricter diet. I am guessing that could take anywhere from a week to 2 months, but it's different for every person.

Next, she has to get pregnant and schedule appointments. Unlike normal women, she will have to make an appointment with an OBGYN, a nutritionist, and a dietitian. Usually they have to go see all those doctors more frequently than the normal pregnant woman visits her OBGYN.

Finally, the woman with PKU has to stay in the hospital longer than the average woman after the baby is born. The baby needs to be monitored closely and longer than the normal newborn because some of the side effects the baby could have if the low protein diet wasn't followed as close as it should be.

If a woman with PKU doesn't follow the low protein diet the baby could suffer greatly. The baby could be mentally challenged due to raw protein in the baby's body while the brain is developing. The protein kills the brain cells as fast as they are developing. The baby could also have frequent seizures due to protein killing brain cells in a certain part of the brain, only. The baby could, also, have heart problems and/or body deformities.

Until recent years, doctors didn't even think it was possible for a PKU patient to have a healthy baby or carry a baby full term. They only started saying otherwise in the early to mid 90's.

As you can tell, it's not the same for women with PKU to have healthy babies. I always think it would be nice for doctors and scientists to find a cure for PKU so that we wouldn't have to worry so much about it. Maybe in the near future they will.

Until next time,
Phe

Cheese Junkie

PKU is kind of like diabetes in the sense that not all cases are the same. I was blessed enough to have a very mild case. Many patients are only allowed to have 5 grams of protein a day. I am allowed a bit more than that. I don't have an exact amount that I am allowed (only because I haven't seen my nutritionist in a few years). When I was 16, I was allowed 16 to 18 grams a day.

One of my favorite foods is CHEESE. Even though I am only allotted a small portion a day, I love it. In fact a lot of my favorite foods have quite a bit of protein. Have you ever really looked at packaging labels and noticed the amounts of protein in things?

Dairy & Eggs
Soft cheeses (Mozzarella, Brie, Camembert) – 6 grams per oz
Medium cheeses (Cheddar, Swiss) – 7 or 8 grams per oz
Hard cheeses (Parmesan) – 10 grams per oz
Egg, large - 6 grams protein
Milk, 1 cup - 8 grams
Cottage cheese, ½ cup - 15 grams
Yogurt, 1 cup – usually 8-12 grams

Meat
Hamburger patty, 4 oz – 28 grams protein
Steak, 6 oz – 42 grams
Chicken breast, 3.5 oz - 30 grams protein
Chicken thigh – 10 grams (for average size)
Drumstick – 11 grams
Wing – 6 grams
Tuna, 6 oz can - 40 grams of protein
Pork chop, average - 22 grams protein
Pork loin or tenderloin, 4 oz – 29 grams
Ham, 3 oz serving – 19 grams
Ground pork, 1 oz raw – 5 grams; 3 oz cooked – 22 grams
Bacon, 1 slice – 3 grams




As you can tell from the list above, every main dish has a large amount of protein (as one would expect). But who would think about protein in fruits and vegetables?

Vegetables
Alfalfa, sprouted, 1 cup raw- 1.32 grams
Artichoke, medium - 3.47 grams
Asparagus, 1/2 cup - 2.16 grams
Broccoli, 1/2 cup - 1.86 grams
Brussels Sprouts, 1 cup - 3.98 grams
Carrots, 1/2 cup -0.59 grams
Cauliflower, 1/2 cup - 1.14 grams
Corn, 1 ear yellow - 4.02 grams
Lima Beans, 1 cup large - 14.66 grams


Fruits
Apple, 1 medium - 0.47 grams
Avocado, 1 medium - 4.02 grams
Banana, 1 medium - 1.29 grams
Blackberries, 1 cup- 2 grams
Blueberries, 1 cup - 1.1 grams
Grapefruit, 1 cup - 1.45 grams
Grapes, 1 cup - 1.09 grams
Passion fruit, 1 cup - 5.19 grams
Peach, 1 medium - 1.36 grams
Pomegranate, 1 - 4.71 grams

You get the point, EVERYTHING has protein. I am very blessed to be able to choose from so many foods because of my mild case. You can see how someone with a severe case would have a very difficult time with their diet.

Because I can't have a lot of main dishes I make my meals out of side dishes. One can imagine how boring and repetitive that would get, that's why I am always looking for new recipes and ways to prepare the food I love and can have.

Until next time,
Phe

Introduction of Me

Hello!

I am a young, married woman who has PKU or Phenylketonuria. Many mothers are aware of such a thing but not many people know exactly what it is.
PKU is an inherited disorder of body chemistry that, if untreated, causes mental retardation. Someone with PKU has a missing enzyme, that breaks down protein into usable amino acids. It is allows raw protein to enter the blood stream. The brain is very sensitive to raw protein, it kills brain cells that are not replenish able.

Through routine newborn screening, almost all affected newborns are diagnosed and treated early, allowing them to grow up with normal intelligence. I was diagnosed with PKU when I was 2 weeks old. At that time, my parents had no idea what that was. It was very hard for them to receive any usable information, although my nutritionists were able to explain it in scientific terms, that was not a big help to my parents.

I thought I was a normal kid until I got to public school lunch time. I was asked over and over where my meat sandwich was. When I went to friends' houses and their moms made dinner, I couldn't eat the same thing as everyone else. It didn't affect my psychologically, I knew nothing was wrong with me.


I want to help people to become more aware of what PKU is and how it affects everyday life. That's my goal with my blog.

Until next time,
Phe