Now Onto Reality

Hello, again!!

Well, this past month has been an interesting one. As you all know, or should know from my previous posts, I did the 30 day Kuvan trial. I never thought it would entail so many things. I had to be very careful in every aspect of my life, which is not uncommon considering I have PKU.

It started on February 7th. I had to take a blood test and then write down everything I consumed for the day. Then at supper time I took the medication. The first dose was very hard to take because I wasn't expecting it to be so acidic and I got heartburn as soon as I swallowed it. The second day, I had to repeat day one, including the blood test. It was the same story for the third day. After that, they (being my dietitian in Portland, OR and the dietitian from Biomarin in San Francisco, CA) wanted me to cut back to only doing blood tests on Sundays, which I think anyone would be happy about.

I started on only 12 grams of natural protein daily, with 30 gram of medical protein. Well as soon as they figured out that Kuvan does work for me, they started putting together a "Booster Plan." This was to build up my natural protein intake steadily to see how much my body could handle with Kuvan. So far I have not heard back about any of these levels. But there plan was interesting.

On the first day of the "Booster Plan" I was to add Nonfat Dry Milk and chocolate milk to medical beverage. This mixture was surprisingly very tasty. In the next few days, that was not the case as they were adding more of each. A few days later we started adding one egg a day. I got up to adding 20 grams of natural protein to my existing 12g. Like I said, I haven't gotten any test results back from the time of my Booster Plan. But I am very hopeful for acceptable levels.

After my 30 day trail was over, it was then up to my insurance to decide what they were going to cover. Biomarin had a representative working with my insurance during the trial just in case it were to work for me. Well as you may know, I am prescribed eighteen pills a day, which equals 558 pills each month. Unfortunately, my insurance will only pay for just under 400 pills a month. That leaves a large expense to me, being that the pills are $37 each. I, also, have a 20% copay. That means my part, if my math is correct, is just over $9,000 per month.

That is a large bill for a single prescription for even the wealthiest family. There is, however, a foundation called NORD (National Organization of Rare Disorders), that I was unaware of until recently. They help those with rare disorders pay for medications that they cannot afford, like me! They do have certain requirements as far as financial caps. They determine the amount of help, if any, by your family size and your annual income. The basic standard is below national poverty level. I had no idea what the poverty level was until I applied for this assistance program. Apparently, my family income is in the poverty range. I thought we were doing well for our age and area. But I can't complain because I do qualify for the assistance. I am very thankful for that.

If you have any questions concerning NORD or would like more information please visit their website at  http://www.rarediseases.org/.

Also, if you have any question or comments for me you can visit my facebook page ( the link is below). You can also send me a message if you want it to be more private.

Until Next Time,
Phe

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Kuvan Thus Far!

Hello readers!

It has been an interesting past few weeks. As most of you know, I started my Kuvan trial!!

I started taking the pills on February 5th. I was told, by the Kuvan Metabolic Specialist, that the pills are very acidic. She wasn't kidding! The first day I took my dose with water. I put the water in my mouth first, nervous of what was coming next, and dropped the pills into my mouth. As soon as the pills touched the water it tasted as though I had squirted lemon juice in my mouth! It was so sour!

Enough about how it tasted, now onto the more interesting stuff. The day I started Kuvan, February 5th, my blood Phe level was 347. The next day, February 6th, my level went down to 200. I thought that was awesome!! My Metabolic Specialist was very pleased. But that's not all, on the third day, February 7th, my blood phe level dropped to 89!!

So, with that information, Dr. Huntington is wanting me to finish out the trial (of course) and then try adding more protein to my diet. She was even talking about an egg everyday or adding fat free dry milk to my formula. I am more excited about the possibility of getting to eat an egg, but I'll have to try the dry milk, too.

Anyways, that's all I have now. If you have any questions or comments don't be afraid to contact me on the Facebook page listed below.

Until next time,
Phe

Follow me on Facebook: http://www.facebook.com/#!/TheDailyStrugglesOfPKU

Follow me on Twitter: Phe_ThePKUGirl

Kuvan Meeting

Hello Readers!

Well I just got done with the meeting I had about my Kuvan Trial. I can't tell you how excited I am to start this. I am sitting at home waiting for my package from FedEx to arrive that contains my months supply of Kuvan.

As you may know, the Kuvan Trial is free for one month. Well during the first month one of the reps/ case managers from Biomarin (the company that makes Kuvan) will be working with my insurance to figure out how much they will cover. As of right now, I am not too worried about how much my insurance will cover. Right now, I am more worried about is if I will be a responder or not. But the company will be working on the insurance just in case I am a responder. That way I won't have a lapse in treatment (hopefully).

While Karen, the dietitian from Biomarin, was here she gave me the details of what this trial will include. It won't be as hard as I once thought. (Thank Goodness!) I was under the impression that I was to take my 18 pills a day first thing in the morning. Karen, however, told me that it is best to take them with the larger meal. So, since I don't really eat breakfast, the morning is not the best time for me to take them. But instead, take them with dinner.

Another thing that she cleared up for me, I thought I had to eat the same exact things everyday during the trial. That is not so, I can vary in the foods that I eat as long as I stay within the same range of protein a day. I was very happy to hear that. Also, she told me that I could either swallow the pills or dissolve them in a drink.

I also, got quite a few little gifts. She gave me a pill crusher, a food journal, a pill holder, a ton of pamphlets, lancets to test with, and a lancet disposal jar. Many more I am sure, I just can't recall them all.

AND, I came home last night to find a big box of medical foods on my doorstep that I didn't know was coming. So that was a nice surprise.

Well, that all I have for now. If you have any questions or comments you can comment below, or you can contact me on Facebook at the link below.

Until next time,
Phe

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Update

Hello, Everyone!

The date is set. I am meeting with the Biomarin representative next week! She will be bringing the months supply of Kuvan. The trial is set to start on Thursday, I believe. I am a bit nervous but mostly excited to start the trial.
I am hoping this Kuvan brings down my levels, dramatically. I am hoping that this allows a lot more freedom with my diet. (It probably won't be that simple, but a girl can dream). Anyways, I just wanted to let you all know that the date is set.

Until next time,
Phe

Merry Christmas and a Happy New Year!!

 Well, hello again readers! Man, it has been a crazy month. I have been quite busy with work, lately.  I work in shipping so December is the craziest month for that, with Christmas. But it is almost over, and for that I am thankful.

I would like to reflect on this last year. One year ago today, I was not healthy at all. My low protein diet was practically nonexistent. My mind was foggy or cloudy more often than not, I was cranky or moody and just not me. Now, I have been on diet for the last 10 to 11 months and I feel as though I am a different person. I can think clear and my moods are those of a normal girl now. :)

In the next year, I hope to have my proteins in baby friendly range. My goal is to get as healthy as I possibly can be. And who knows, maybe God has it planned for my hubby and I to start a family.

That is all I have for  now. I wish you and your family a very Merry Christmas and a Happy New Year. May God bless your family in this new year.

Until next time,
Phe

Kuvan Soon??

Well hello, again, everyone! I know I promised to update you all on my last appointment, but since then I have not had access to the Internet. For that I am sorry. I have noticed a steady amount of page views everyday since my last post, and I am guessing that some of you are interested in how it went..... so here goes.

When I went up to my appointment ( I say went up because it is a 4 1/2 hour drive north) they had scheduled me for both a physical with the nurse practitioner and a regular appointment with my metabolic specialist. My physical went great, good blood pressure and everything. Of course they did blood work to test all my levels, but we will talk about that a little later.

In my appointment with the Metabolic Specialist we talked about Kuvan. She agreed that I am a good candidate for it. She had me fill out all the paperwork for a trial. We discussed my insurance because she was concerned about the copayment. I had no idea how outrageous the price of Kuvan was.

Kuvan is $37.00 per pill. That would already be expensive if I only had to take one a day... But I would have to take 18 pills a day!! That is $666.00 per day for me to take this medication!! Now if that isn't crazy I don't know what is.

Like I mentioned before, I filled out the paperwork for a trial of Kuvan. In studies they have done, the results show that not all candidates are responsive to the medication. They can't even say that a certain mutation of PKU will respond. Therefore the 4 week trial is how they determine whether or not you respond.

I got a call the other day from a representative of Biomarin (the company that makes Kuvan) to set up my free trial. They are so efficient that they want to send the representative (I cannot remember her name) up to see me before my trail. She is going to hand deliver the medication to me and help me set up a routine to take the medication and regulate everything else PKU related.

My husband and I have talked about it and we think it works best for us that I start this trial of Kuvan at the beginning of the year. I will keep you all informed when it gets closer.

Until Next Time,
Phe


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Losing Weight

Hello, readers! Once again it has been far too long since I have written. Since we last chatted I have started a new job and have been getting ready to move. My new job has kept me pretty busy with learning the tasks I am responsible for, as well as working the hours that are given to me. I must say I love my new job more than any other job I have ever had. That's besides the point though. I haven't been too busy with getting ready to move until the last few weeks. Anyways, I digress...

Lately, I have been thinking about weight loss and being healthy. I have been trying to lose weight lately. I haven't been comfortable with the way I look or feel for a long time now, so it is about time I do something about that. I never expected to feel the way I do now.

Having PKU and trying to lose weight is surprisingly easy and hard at the same time. Of course, having PKU, you have to cut out high protein foods. Then, trying to lose weight, you have to cut out high fats and carbohydrates. So basically, what I have been doing is drinking my formula and eating fruits and veggies. I haven't been too worried with cutting out bread because as of lately, I haven't been eating too much of it. I realized that is where a lot of my protein was coming from. I want to try to make low protein bread, but I just haven't gotten the mix for it yet.

On another note, I am scheduled to go see my Metabolic Specialist on the 30th. I am hoping that this appointment is the one where she puts me on Kuvan to try it out. I will update you all after the appointment.

Basic Bread Recipe
Makes 12 servings

• 1 box (500 g) Loprofin Mix
• 1 sachet dried yeast (supplied with mix)
• 2 tbsp vegetable oil
• 425 mL (about 14.5 fl oz) room temperature water
• 1 x (2 lb) bread loaf pan + 1 x (1 lb) loaf tin OR 3 x (1 lb) loaf tins
• Optional Flavors to be added at Step 4:
• Sweet breads: 250 g (8.5 oz) dried fruit of your choice, 2 tsp sweet spice and/or 2 tbsp molasses.
• Olive and Tomato Bread:1 tsp salt, 3 tbsp olives and tomato

Preparation:

1. Preheat the oven to 425°F.
2. Mix together the Loprofin Mix and the yeast into a large bowl, stir in the oil and water.
3. Beat the mixture for 1 minute, if using an electric hand mixer, or 2–3 minutes if using a wooden spoon, until the batter is smooth and glossy.
4. Stir in the flavorings if desired.
5. Divide the mixture between the prepared tins, smooth the surface and carefully brush with oil to seal the batter and prevent it drying out whilst rising.
6. Loosely cover the pans with cling wrap and put in a warm place to rise. This should take about 30 minutes, until the batter has doubled in size.
7. Remove the cling wrap and bake the bread in a preheated oven (425°F) for approximately 25 minutes, until golden brown.
8. Cool on a wire rack.

Nutrition Information (per serving):

Calories: 170
Protein 0.26 g
Phe: 10 mg

Anyways, that is all I have for now.

Until next time,
Phe


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