Goodbye For Now

I am having a hard time finding the words to say for this post, but here we go.

 

I am not going to be posting on this blog page for a while, possibly ever again. I'm not sure what will happen but I have decided to start a new blog page.

 

As I go back and read my previous posts, I realize that this blog is a real downer. Even in the title, it shows a pessimistic view on PKU as a whole. While having PKU is no walk in the park, it's not all bad. I have joined many groups on Facebook that consist of people around the globe who have PKU. Since joining these groups I have learned many things, but the most prominent:

                        EVERYONE THINKS OF THEMSELVES POORLY BECAUSE OF PKU

              EVERYONE BLAMES THE BAD THINGS THAT HAPPEN IN THEIR LIVES ON PKU

                        NO ONE IS THANKFUL FOR THEIR PKU

 

I know that last one sounds weird. But honestly, out of everything that God could have given you He chose PKU for a reason. He could have given you a much worse disease or condition. But He didn't. It's time to be thankful for what you have and what you don't.

 

"In every thing give thanks: for this is the will of God in Christ Jesus concerning you."

I Thessalonians 5:18

Baby Time!!

As you may know, due to my last post, I was finally able to get pregnant... Well, I would like to introduce to you my sweet daughter, Elaina!

 

She was born on March 12th after 22 hours of labor (I was induced on the morning of the 11th). She weighed in at 6lbs 7.2oz and was 20.75in long!! She did have a moderate case of Jaundice when she arrived and had excess Biliruben (not sure if I spelt that correctly). But after some photo therapy and 4 days in the hospital (starting the day she was born), she is doing great!! Like every other baby born at the hospital she had her 24 hour screening test, and it was rushed to the lab. We haven't heard anything back from that yet, so her pediatrician is hoping no news is good news. On Monday we went in for her 2 week well baby check up and did the next and final phase of the newborn screening test. Her doctor says she usually hears back within 2 days (which is today) if something is abnormal and if she doesn't hear anything today she will be calling the lab on Friday to make sure. OF course she doesn't do this with every newborn, but since I have PKU and Elaina has a 25% chance (if daddy is not a carrier) of having PKU as well.

 

We are anxiously awaiting to hear back from her doctor, hoping she doesn't have PKU, and enjoying life with our new little addition to the family.

 

Until next time,

PHE

 

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Surprise!!

Hello everyone!

          It's been a whole year since the last post, and for that I am sorry. This last year (more or less) there have been quite a few changes in my life. Not only with my PKU.
           First with the Kuvan trial. I believe you all know that I did the Kuvan trial last year and discovered that I am a responder. I went from being able to eat 12 grams of natural protein with levels in the 400s ml/dl... to being able to eat 30 grams of natural protein with levels in the 120s. That was quite a shock to me and my lifestyle!
           Second of all, which I didn't announce on here to all my readers... I got PREGNANT!!! My husband and I found out in the beginning of July. Shortly after that, we had our first appointment with my midwife. She did an ultrasound to make sure I didn't have a false positive. We discovered I was indeed pregnant and at the time of the appointment I was 6 weeks along! Both my husband and I were over the moon!!
          I was beyond nervous to be pregnant. I was always worried about my levels spiking without my knowledge and I would miscarry. I had to keep in mind that I was being watched very carefully by my specialist. I was doing blood spots twice a week. As I got farther along my body decided to handle more protein!!
         We found out in early November that we were expecting a little girl!! We had been told 6 weeks earlier that there was a 70% chance that we were having a boy. We were told that it was still VERY early to tell for sure (so I couldn't go shopping yet). My husband and I didn't care what we had. We kept saying whatever God decided to give us was wonderful. I wanted to know as soon as possible because being the girl that I am.... I WANTED TO GO SHOPPING!!!!
         Now, I am currently 39 weeks along. Our little girl is due to be born soon! I can't wait to see what she looks like, how much she weighs, how tall she is, and if she has PKU or not. And of course I will post all of the details.

Until next time,
Phe

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

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

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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

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

Follow me on Twitter: Phe_ThePKUGirl

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