Pku postartum

After being in the hospital for five days. Safe to say Bentley doesn't have pku. Which is kinda a relief even though; it wasn't that big of a deal. I had to get an emergency C-section and, once he was born I wasn't able to really bond with him. because he start to have breathing problems. so they took him to the intensive Care unit, and I had no idea! so I woke up I had bentleys Dr come in and explain the situation. At first I beat myself up thinking my pku was responsible for this. because Its so easy just to blame myself! but then I was reminded by my husband that my levels were great up until that moment so It couldnt of been that. For three days Bentley stayed in intensive care. he was doing better every single day and surprising everybody with how strong he is!  I'm so happy that he's doing a lot better!! but at the time I was thinking of breastfeeding, little did I know you have to breastfeed about eight times a day and pump throughout the day. which is a lot to do especially for a woman with PKU. the biggest question was if Bentley had PKU!? that would determine whether I would or not but, since I got the test results he does not have it.  I still decide that the best thing for me to do was not to breastfeed since it's so involved and PKU is still involved. The best advice I was given when I was pregnant was make sure you take care of you so you can take of your family.  so I decided that it was best for me not breast feed. But to find a forumla that was the closest to breast forumla. Which at first I already felt like I was failing as a mom to make the decision I did. But then I realized that I'm not. That I try to be the best for Bentley. 

Taking Bentley home from the hospital was an emotional moment for both my husband and I. Because we just didn't know what would happen. But we're so happy things got better. I honestly thought pregnancy was hard and after wards won't be as hard. But I was wrong. Its hard. Especially if you just wanna continue good habits. Counting every thing you eat. Taking your protein drinks and taking your kuvan. Its overwhelming adding a new born into the picture. I just have to keep reminding my self take a deep breath it's a life style change!  

Pku around the world.

Its amazing to connect with people all over the world that have pku. It doesn't matter how old or what back ground you come from. Nine times out ten you always have an instant connection thanks to pku! I realize that even though we all live on different places we do have alot in common like some of the struggles we face. But I also realize that every country has there own issues with pku. Advocating is so important no matter where you are. Yeah we may be rare but it doesn't mean we aren't out there. It doesn't mean we need to be ingored of our existence. I encourage any one at any age or any where to raise awareness of pku 💙

Phe goals!

Every one with pku that is on the low protein diet has a phe goal/ tolerance! What is a phe goal? Its an amount that a pku person can consume a day. That is safe for the body to break down. And it won't be so much phe to hurt the brain! A dietation determines how much phe your body can have a day! Recently my tolerance changed to 900mg based off of my last blood spot results. I have to admit it's the highest I ever had. But that's because I'm in my third trimester. And luckily the baby has no problem helping me break down my protein. The biggest question is will he have pku? In my gut I have a feeling he won't. Because my phe intake had went up dramatically! And the lowest I ever had was 200mg a day. To people with out pku don't really understand why it's a big deal. But for anyone with pku it is! The more phe you can have the more easier life can be! And the options of different foods become available! Alot of people ask me all the time is will you ever grow out of it the answer is no. Its for life! Its not even remotely possible to grow out of it. The only way to get rid of is a cure. Which there's not one. There are just treatments to help make life alittle easier. But it's not the end of the world it's just a lifestyle that I've always had! 

Pku pregnancy (36 weeks!)

Tuesday was my high risk appointment. I'm so use to jumping back and fourth between obgyns. I was informed that it was my last visit that day. It blew my mind out of the water that we're almost to the finish line. That bentely will be here soon. The Dr had also mention he's currently weighing in at 5.4 pounds. Which is still normal. But the biggest question that everyone seems to wonder about is... Does he have pku? In my gut I have a feeling he doesn't comparing to my phe tolerance and levels have been. But wether he does or not my husband and I love him just the same! I'm not nervous about the possibility. Becaue I know that everything will be alright. But the Dr did inform me that it would take up to 24 to 48 hours just for the pku new born screening results would come back! But I'm ready for all the outcomes. The good, the bad,the scairy, and the ugly! Cause I know I'm strong enough to handle it. 💙

Having a high phe tolerance

I never had 900mg in my life. I always thought the more phe you have the easier life is for you! Its true to a certain extent. All my life I'm so use to eating low protein foods. And always use to running out of phe towards the end of the day or not being able to eat certain things like peanut butter. But since I'm in my third trimester my phe intake had went completely up! I thought to my self that I should enjoy the amount I'm aloud to have now because after bentely is born I go right back to a lower tolerance. Its almost like knowing what it's like having a liver that actually works. But I have to admit. Its been hard trying to eat 900mg. it's a learning process. And it's nice seeing the foods that are higher that I can finally have. But even though it won't be like this forever.  I'm ok with that. Because I know I'm already have conqured maternal pku. The one challenge every woman with pku faces. So any other struggles with pku that comes my way seem so minor to me! Because I know that I can handle it. 😀

Thoughts on Gene therapy

They say that Gene therapy could be the possible cure for pku.  It sounds like amazing idea. There are alot of risks. To the pku community minus all the sciencetist. Regular individuals like you and me. Don't know a whole lot about it. Alot of questions I wonder about is... What are the risks? They say it's a one time fix... But is that really the case? How would it work? Could your body reject over time? Is it even safe to get pregnant after taking it? So many unanswered questions. Makes me worried to even be willing to try it. But in cases like these with very little info to go off. You gotta keep your mind wide open. I really hope they find a cure some day. ❤️

Having a relationship while having pku

Having pku is apart of who you are. Its not something to be ashamed of. But you want to find that someone that understands your needs and your health. Alot of people like to judge. But it shouldnt be a subject you should avoid! I would tell the person your with when you feel comfortable. Its not something to hide forever. Believe me I have been in a relationship where the guy didn't understand pku. He actually thought I made it up! Even tho I had proved over and over that I did. I realized that he will never get it. And that is something that I find important in a relationship. The next time you go out on a date or get into a relationship. Find the time to tell that someone about pku! And remember don't be ashamed! Its just a life style not a life sentence. 

What is phe?

The one thing about pku that people don't understand much is what phe is. Phe is the type of protein we have to calculate. Phe is short for phenylanie. If someone with pku has to much phe during the day it raises there blood levels. On adverage someone with pku has to keep there phe levels (which is detected by a blood test) between 2 and 6. That is the healthy range to be in. It helps us function alot better. But higher then a 6 can be toxic to the brain. Which creates brain damage over course of time. The lower your levels are the more your taking care of your brain! When someone with pku has high levels especially for a long time. It will effect you long term. Some of the side effects are head aches, mood swings, depression, axienty, having troubles pay attention at school or work, troubles with relationships with others, socially akward. Some people don't experience everything. But for me. I've experienced most of these. Especially being off diet for so long! But everyone is different and so is there phe tolerance. Phe tolerance is the amount your body can break down a day. A metabolic dietation lets you know judging on your blood tests how much you can eat. Before I was pregnant I was at 345 mg of phe a day. Which is not that much. But now being 8 months pregnant I'm at 800mg. Which is a huge jump! I know you probably wonder why? Well because being pregnant the baby is taking more nutrient than I am of what ever I consume. He basically acts like the liver I never had. Once I got my phe levels lower he Continues to keep my levels low. After having the baby most likely I will have to cut my phe tolerance in half. Which will be hard. But I'm up for the challenge. 

Traveling with pku!

Traveling while having pku is kinda hard. It's all about planning each meal for the day. Making sure you stay within your phe goal range. I recently just got back from my trip from Gatlinburg. It wasn't just a regular vacataion. It was a trip that changed both my now husband and I. Because we had gotten married! Any ways packing for the trip was a challenge when it came to pku. I made sure I definitely had my forumla and my kuvan that lasted the three days we were gone! I also packed extra forumla just in case I needed it. Because when I ever I feel slightly sick I always turn to forumla to drink. Alot of the times it helps me feel better. As far as meals goes. I tried to figure out each day phe intake wise. Since I'm about eight months pregnant. I had a great amount of phe tolerance (800mg). Which wasn't to hard to plan meals. It's just making sure I had enough thru out the day was the hard part. During the trip I made sure to calculate everything I ate. I even brought my scale with me. As much as attention I got in public. I didn't care. I knew that it was better to use it.  Then let my my self and the baby have too much phe. Some mornings I made sure I had fruit. To help save phe to eat out later. The only foods around us that I was able to eat were potatoes and salads. Which I stuck to only that. I discovered the best place for salads is subway! They let you build your own salad. And everything is fresh. It's a good size salad. I would seriously reccomend it! If you have lower phe tolerance while traveling I reccomend to bring your own low protein food and make sure you eat plently of salads if you go out! 
But dont ever let pku stop you from traveling or living life. Remember it's a life style not a life sentence. I know sometimes it feels like its true. May the odds ever be in your favor if you do take vacation!