run down of PKU

So I haven't really written on Maggie's condition. So that is what we will learn about today.

Maggie was born with a rare metabolic disorder called Phenylketonuria, or PKU for short. The definition of PKU from the National PKU website is this: 

"Phenylketonuria (PKU) is a rare, inherited metabolic disorder that is characterized by the inability of the body to utilize the essential amino acid, phenylalanine (Phe). Amino acids, usually obtained from the food we eat, are the building blocks for body proteins. PKU is caused by a deficiency of the liver produced enzyme phenylalanine hydroxylase (PAH).

This enzyme normally converts Phe to another amino acid, tyrosine. Without this enzyme, Phe accumulates in the blood and body tissues. Excess Phe is toxic to the central nervous system and causes the severe problems normally associated with PKU. When left untreated, PKU patients who consume too much Phe are at risk of severe neurological complications, including IQ loss, memory loss, concentration problems, mood disorders, and in some cases, severe mental retardation.

Damage done is irreversible so early detection is crucial. When treatment is begun early (within the first few weeks of life) and rigorously adhered to, affected children can expect normal development and a normal life span. PKU can be treated by a diet low in phenylalanine and high in tyrosine. While there is no cure, in recent years a few drug products have become available that can be used in limited cases to mitigate the effects of the disorder. Other therapies currently under investigation include an injectable form of PAH and gene therapy."

People with PKU have to drink a daily formula that is the make up of protein, but doesn't have the amino acid phenylalanine. We call it her milk. She will have to drink it every day for the rest of her life. 4 cans (the size of regular Similac or Enfamil cans) cost about $700. She currently needs around 10 cans a month. The cost and the amount needed only increase as she gets older.  There are currently 16 states with little or no provisions for metabolic disorders, mainly PKU (I am currently putting a packet together to send to our congressmen to get this changed). Most states, including Mississippi (who has zero provisions for PKU), will allow coverage of formula through the state healthcare (medicaid) for children- if you fight with them enough. Most states give some sort of tax break or monetary help for the everyday foods. Most all of her foods are only available through online stores and some of the costs are as follows:

1 box of 30 individual snack bags tortilla chip bags: $30
8 oz Pea-not butter: $8.49
2lb bag of baking mix (for pancakes she loves): $12.79
(16) frozen veggie meatballs: $20.49
12 cranberry pumpkin cookies : $12.49
(8) mini plain bagels: $12.49
(4) Veggie burgers: $11.49
Pasta: $10/box (like we get for $1 at Wal-Mart)
Cereal: $7/box
Rice: $9/box
Cake mix: $6/box

She is allowed only 7g of protein a day. In comparison, 1/2 cup of peas has about 4g of protein. So even with her fruits and veggies there are some she can't have. 

So the question comes up "Why can't she just have a vegetarian/vegan diet?" Good question! Phenylalanine (PHE) is an amino acid that is contained within so many foods. Bananas have zero protein, but high PHE. High protein foods such as: meat, fish, poultry, eggs, cheese, milk, dried beans, and peas are avoided along with wheats and flours.  If she were to eat a normal diet that included these foods, severe and irreversible brain damage would be certain. 

"Phenylketonuria (PKU) is characterized by elevated blood levels of the amino acid phenylalanine (Phe), which is mainly obtained from eating proteins. The symptoms of PKU almost exclusively concern the brain, so how does an elevated blood Phe level affect the brain? The brain is protected by a surrounding layer called the blood brain barrier (BBB), which allows some material to cross while preventing others. The brain needs certain material to cross the BBB to allow for normal functioning. One of the raw materials the brain requires are amino acids including Phe for building brain proteins and making chemical messengers called neurotransmitters.
The way Phe and other amino acids get across the BBB and into your brain are by using transporters. This is similar to a bus (the transporter) carrying people (the amino acids) to a destination, each seat gets taken up by an amino acid and the next stop is the brain. This analogy provides a visual description of the main theory of how elevated blood Phe can affect normal functioning of the brain. Imagine that elevated blood Phe in PKU can lead to Phe taking up more seats on the bus, which may cause two things:
More Phe gets transported into the brain
Less of the other amino acids that share this bus get transported to the brain because Phe is taking up their spots!"

With PKU there is only mild (HyperPHE) and high (classicPKU). Maggie girl is, thankfully, mild. This puts her as a prime candidate for a somewhat newly FDA approved therapy drug called Kuvan. Kuvan is a type of  Sapropterin pill. "Studies have shown that taking daily pills of sapropterin Iower and stabalize blood Phe level in some people with PKU. Sapropterin is available by prescription and is meant as an additional therapy to the Iow-Phe diet. To determine if sapropterin therapy will work, the physician puts the patient on the drug tor a trial period and evaluates its effectiveness." I am so excited to start this for her, and I hope she will be a responder. If she isn't, we will wait a couple years and try it again. It isn't covered by most insurances right now, but that is also something that can be fought for and sometimes approved. But since PKU is so rare (roughly 20,000 people in the US), it's not on the radar for most insurances and states.  But when not approved, BioMarin, the owners of Kuvan, have a fund set up and help for the families to pay for it. The PKU community is so amazing in that no one wants anyone to go without. It's truly a blessing that this community is so "all for all, no man left behind". 

We will be starting her trial on Kuvan in the next month or so. She will go from having one doctors appointment every other month, to 5 in one month to monitor if it is working for her or not. So, if you're the praying type- please pray with us that she responds, and if it works, she will be able to have much more protein in her diet! (yes & amen!)

It is finished

This past weekend I went on a much needed girl's trip with two of my best friends. We all three lead very different lives, but for the last 10 years we have stayed as close as possible. I don't know what the thread is that keeps us going, but if I didn't have these two God fearing women in my life, I don't know if I would be in the place I am now.

On Thursday afternoon we all met at the airport and boarded our first flight to San Francisco. We arrived at the SFO airport around midnight and drove to the hotel. The next morning we got up and took a bus tour of the city, visited Alcatraz, and had dinner at a wonderful restaurant on the beach. It's fun to say we did our touristy time in SF.

But we all had the most fun on the next leg of our trip. We drove up the Pacific Coast Highway, the long way up, just to see the scenery. It was absolutely breathtaking. We stopped at every stop we could and just got out and breathed in the air. The beautiful weather they had over the weekend really spoiled us. That evening we rode horses on the beach, and drove to a beautiful little spot in Albion called the Lord's land.

Let me tell you a bit about this place. We were looking for somewhere in between Humboldt Redwood Park, and SF. So I remembered that a dear old friend was currently at a YWAM (Youth With A Mission) base that way, so we looked it up and booked a cabin about 8 months ago.
As soon as we stepped foot on that campus, the bickering and bad attitudes between the three of us, for the most part, stopped. Which I can only attribute to the absolute peace that is felt as soon as you enter their grounds. Now, I am aware that nature is not everyone's cup of tea, and I even get super creeped out by the occasional bug or wild animal, but I could live at this spot forever. The welcoming beauty that greets you at every single turn is just so telling of what God had in mind for the property many years ago when it was first built. I have never loved sitting outside staring at trees so much in my life.

I know God plans each persons life individually, but I also believe that He is a big enough God to make some of those paths intersect at the exact moment that is needed for one or even sometimes both of those people. In this case, it was for me.

Had I not done a YWAM school around 10 years ago, I would not have met my friend J (fun fact, our parents did their (it's called DTS (discipleship training school)) school together). J is the reason I knew about the Lord's land retreat ywam base (whatever you want to call it) - and whether he knows it or not, God used his life to help mine, if that makes sense. Therefore the girls and I booked a cabin at this place, and thus completed the healing and renewal of my heart. From the second I stepped foot on the property, I just knew God was completing a work in my heart that I have just been praying, weeping, stressing, and waiting for.
On our last evening of our trip my friends and I met up with J and he showed us around the campus and we got to catch up for a little bit. As we were all talking-my friends said, and it came as a surprise to me, that in the last 3 weeks, I am like a whole new person. I knew that I felt different, happier than I have ever been...ever. But I didn't realize just how evident it was. J asked me some questions that sort of got me thinking and it was walking back to my cabin that night that I really thought about some things and really let go of things once and for all.

Y'all, God has such incredibly divine way of intervening in your life at the exact moment you need him to. He is so intricate in the woven design that is your life's movements and paths. He knows that standing in that parking lot, catching up with an old friend, mixing in my newer ones, completely removed from all those unnecessary stresses of my life, was exactly what He needed to make me listen and see exactly what He is doing for me and where He is directing me. I am living out an entirely new lease on life, and I have honestly never been happier than I am right now.

In the months leading up to this trip I was so incredibly depressed. I felt trapped on a road I did not want to go down, and I firmly believe God did not have in mind for me. I cried and cried daily, I didn't want to get out of bed, I couldn't bring myself to play with my kid, I was just not me. It was the hardest couple months I've had in a long time. But around a month ago I broke up with a really great guy. I did the breaking, I did the hurting, that was a weight I had to deal with. But as things came and got more serious with us, I just felt this huge weight on my shoulders, a seriously dark cloud looming over my head. I couldn't shake it. I tried for weeks to explain away the feeling of depression and doubt I had. I tried to pray it away, but it just got stronger. I thought I had heard God tell me this relationship was the right path for me, the right thing to do in my life- to marry this wonderful man of God. All of that came to a screeching halt when I cried out to God and just shut myself up and listened. God blaringly loud told me NO. I now believe that God allowed me to go through this hard time so that the depression would essentially force me to turn to Him, completely. I had nowhere else to turn. I believe God found me where I was, pulled me up, told me no to the relationship for a very specific reason. This guy is all the characteristics I would like in a man, but he is not the one I am supposed to be with. And I truly believe that with everything in me. It took me a couple weeks to stop feeling guilty and like a terrible person, but I believe I am following in the path God has for me- and I don't believe that that is a wrong choice. And I can only hope that other people too will understand that sometimes God tells us no, even if we don't like it.

I've had enough happen in my life before I even turned 28 to know when God is telling me no, to listen-100%. I am not looking to do life on my own, I need His guidance. And I know not everyone will agree with or understand that, but I can finally truly say that I am walking in His ways, and His guidance and His assurance. And I know that with Him and through Him, it is finished. And I will be just fine.

Let it go.

Have you ever been living in crisis mode for much longer than necessary? Ever thought "I should be strong enough by now, God, what's up"?

On my way into work this morning, I was listening to KLOVE, as I usually do on my morning commute, and Mandisa's "Stronger" came on. And for the first time I really listened to the lyrics instead of just singing along. The words hit home today- like every single word was me yesterday and even this morning. Anyways, here are the lyrics :

Hey, heard you were up all night
Thinking about how your world ain't right
And you wonder if things will ever get better
And you're asking why is it always raining on you
When all you want is just a little good news
Instead of standing there stuck out in the weather

Oh, don't hang your head
It's gonna end
God's right there
Even if it's hard to see Him
I promise you that He still cares

[Chorus:]
When the waves are taking you under
Hold on just a little bit longer
He knows that this is gonna make you stronger, stronger
The pain ain't gonna last forever
And things can only get better
Believe me
This is gonna make you stronger
Gonna make you stronger, stronger, stronger
Believe me, this is gonna make you Stronger

Try and do the best you can
Hold on and let Him hold your hand
And go on and fall into the arms of Jesus
Oh, lift your head it's gonna end
God's right there
Even when you just can't feel Him
I promise you that He still cares

[Chorus]

'Cause if He started this work in your life
He will be faithful to complete it
If only you believe it
He knows how much it hurts
And I'm sure that He's gonna help you get through this

When the waves are taking you under
Hold on just a little bit longer
He knows that this is gonna make you stronger, stronger
The pain ain't gonna last forever
In time it's gonna get better
Believe me
This is gonna make you stronger

So. I guess, to some people (I've actually been told), I have played the victim too long. I have mourned the loss of my marriage and my family for too long. Let's clear the air, let's fill in some blanks. 3 years ago to the day my husband text me saying he no longer wanted to be with me, but he was leaving me for my brother's wife. To which her and I were living in the same house. Cue move #1. I left my home and moved back to my parents in MS. They continued their affair through the summer. I moved back to PA (move #2) because he and I decided to work on things. I got pregnant, his affair continued behind my back. He left her, moved (#3) back in with me and 3 months later out again. I was 7 months pregnant. He left for a different woman at this point. (mourning of marriage loss part 2). My mom comes to live with me for 2 months. Maggie is born, Maggie is diagnosed with PKU- I am left to deal with this on my own (if anyone you know is ever diagnosed with PKU, I urge you, with all your might, to NOT look it up online, it is the scariest thing to read about, but in reality it is totally manageable and not scary at all). Move #4 happened when Maggie was 2 weeks old, I moved to his parents house for a year. That was hard in it's own right. He was finishing school on the other side of the country. I was a completely single mother for her first year. Around her 1st birthday I did my 5th and final move in 2 years back to my parents where we stay for now getting our feet back up on solid ground. July 2015 our divorce was finalized. And all the drama and sadness that could happen in between has happened. So, I feel, that in truth, this hasn't even been a full year since I had to give up all those hopes and dreams. I was waiting for and expecting a miracle that never came. That is also pretty devastating. So maybe it's just me, but I feel justified in still being sad over it from time to time. 

Until today.

Today as I drove into work, feeling bad about a recent heartbreak I caused, and feeling sad that the dreams I once had are no more, I actually Let it go.

And man, can I just breathe so much better?! It's truly like a weight has been lifted off my shoulders. Like a cloud has gone away and the sun has come out to play! The Lord has truly smiled on me and been so so gracious to deliver in his many promises. 

I believe that he is working hard on my behalf, on Maggie's behalf, to form and guide me to the right life path he has planned for us. I just need to remember that even when I'm not getting the answers I want, when he isn't moving the mountains that I want him to move, he has not forgotten about me, he is weaving together my perfect story. And I am excited to just let Him be in control, and see what adventures life takes me on.

God is good, all the time. All the time, God is good.

Selah.

Trust

            Trust 

noun \ˈtrəst\

Full Definition of trust

1.  Assured reliance on the character, ability, strength, or truth of someone or something

2.  One in which confidence is placed

3.  Dependence on something future or contingent :  hope

Webster's Dictionary defines trust as "Assured reliance on the CHARACTER, ABILITY, strength or TRUTH of SOMEONE or SOMETHING. 

One in which confidence is placed: HOPE.

This page contains 47 Bible verses about trust. 

We trust complete strangers, daily, to stay in their lane while driving on the highway, to not rob us when they come in to pay, to take care of our kids at school. We walk by over 100 strangers daily and trust that they aren't going to stick their foot out and trip us. We place daily, confident, trust in men. We trust our mechanics to fix our cars, our grocers to pick the best quality foods, our doctors to fix us, our bankers to take care of our money and our government to take care of us.

Why then, if we trust all these complete and total strangers, every single day of our lives, is it so hard to put trust in the one who created us? The one who loves us with no conditions, the one who has promised to provide for us and keep us alive and well? 

We establish trust with complete strangers in relationships. We start dating someone, we open up and tell them every little detail about our lives, we trust that they will be good people, we trust them to keep our secrets. We trust them with our hearts, we fall in love, we trust them to keep their promises, to keep our hearts. We trust the mess out of people. PEOPLE. humans. flawed beings. But we can't trust even for a full day that the GOD who CREATED US will bring us the right job, will help us figure out how to pay the bills, will bring us the right people, relationships, friends into our lives. We can't trust that the one who is Alpha and Omega, beginning and end, goes before us and behind us, knows all the hairs on our heads and every grain of sand in the ocean has the ability to provide every single need. He knows us better then we know ourselves. He knows what we need better then we do. Why? BECAUSE HE MADE US. He knows why he is taking me down this dreadful path of heartache I've been on. But he also knows I have stayed faithful in persecution, faithful in the bad. I have praised him in the storm.

My life was turned upside down 3 years ago to the day. I trusted someone with every ounce of it I had. and that trust was shattered. Part of trust is forgiveness. But that's a post for a different day. As flawed beings, we have to learn to trust in God over man. It's hard. It's hard to not try to figure it all out on my own. It's hard to just hand it all over and say "OK God, it's in your hands." Some days I don't feel like he's there. But I am learning that just because He is quiet, doesn't mean He isn't there. He has provided my every single need the last 3 years. Maybe it's time I be quiet and just listen to what He has to say. Recently I had to make a very tough decision (And if not, he is still good.). But I genuinely believe, that once I just shut up and let God talk to me, that I knew what the right decision was, and I am confident in it. I have been trying to run my own life for far too long. My divorce should have been a turning point, and for a moment it was. But I started trying to figure everything out my own, in my own time, and lo and behold, that didn't work out for me, and left a lot of heartache in the wake. 

So, Trust I must learn, trust I must do.

“But blessed is the one who trusts in the Lord, whose confidence is in him. They will be like a tree planted by the water that sends out its roots by the stream. It does not fear when heat comes; its leaves are always green. It has no worries in a year of drought and never fails to bear fruit.” Jeremiah 17:7-8

"Commit to the Lord whatever you do, and he will establish your plans." Proverbs 16:3

"When I am afraid, I put my trust in you." Psalm 56:3 

"This is the confidence we have in approaching God: that if we ask anything according to his will, he hears us." 1 John 5:14

"But when you ask, you must believe and not doubt, because the one who doubts is like a wave of the sea, blown and tossed by the wind." James 1:6

"He will not let your foot slip— he who watches over you will not slumber." Psalm 121:3

"Look at the birds of the air; they do not sow or reap or store away in barns, and yet your heavenly Father feeds them. Are you not much more valuable than they?"Matthew 6:26 

"For we live by faith, not by sight." 2 Corinthians 5:7

"My salvation and my honor depend on God; he is my mighty rock, my refuge." Psalm 62:7

Well, you guys get the point. All my salvation and honor, depend on God. My life depends on God. Am I not more important that the birds?

To live by faith and not by sight. That is my goal. There is nothing I want more than to serve God with an open heart and an open mind- to go where he wants to send me, to stay when he wants me to stay.

But to do all of that, first I have to learn to trust.

today's thoughts.

Have you ever just been going about your daily life and been completely overwhelmed with how absolutely amazing God is? I honestly don't understand how people can deny his existence. I see it quite literally everywhere I look. People bring the science of the world at me, and then get a really great surprise when I tell them  I studied Earth Science in College. To me, it all adds up. The Big bang and the story of creation are the exact same story. 
However, you have to understand that the Earth was not created 6,000 years ago and our current 24-hour day was not the same 4.5 billion years ago. 
Here: http://www.rareuniverse.org/timeline.html
Is a pretty cool website that compares the two timelines : biblical and scientific


Anyways, God has been showing me so much lately and letting me figure out so much about myself that previously I just didn't see. God is on the move. And I can't even tell you how excited I am to see what he does from here. 

Overwhelmed- Big Daddy Weave

I see the work of Your Hands
Galaxies spin in a Heavenly dance oh God
All that You are is so overwhelming

I hear the sound of Your Voice
All at once it’s a gentle and thundering noise oh God
All that You are is so overwhelming

I delight myself in You
Captivated by Your beauty
I’m overwhelmed, I’m overwhelmed by You

God, I run into Your arms
Unashamed because of mercy
I’m overwhelmed, I’m overwhelmed by You

I know the power of Your Cross
Forgiven and free forever You’ll be my God

All that You’ve done is so overwhelming
I delight myself in You
In the Glory of Your Presence
I’m overwhelmed, I’m overwhelmed by You

God, I run into Your arms
Unashamed because of mercy
I’m overwhelmed, I’m overwhelmed by You

You are Beautiful, You are Beautiful
Oh God, there is no one more Beautiful
You are Beautiful, God you are the most Beautiful

You are Wonderful, You are Wonderful
Oh God, there is no one more Wonderful
You are Wonderful, God You are the most Wonderful

You are Glorious, You are Glorious
Oh God, there is no one more Glorious
You are Glorious, God you are the most Glorious

stuff

Maggie Jean's PKU fund
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Please consider clicking on the link above and donating to our funds! I wish I alone was able to give my little girl everything, but I am learning that I can't do it all alone. And even with her dad's monthly child support, the cost is still higher than I can foot alone. I've said it before, it's a very embarrassing turned humbling experience to ask for help. But it is not for me, it is for Maggie. And she deserves to be able to live the healthiest life she can. We appreciate all the help so far, more than words can say.




Next-
If you are going through a hard time, whatever it might be, let this song encourage you. Pass it on to someone who needs to keep going. God is always good. We may not always understand, but if we listen, and we trust and believe in Him, and his character, we can know, without a doubt, that He will take care of us, and will make things good for us as his will leads.

a little bit of it all: revised

Oh, Shonda. DISCLAIMER: if you aren't caught up on Grey's, I suggest not reading this as it's a big spoiler.

I have been watching last week's episode of Grey's Anatomy for 3 days. It has taken me that long to watch it. Every time I start it something comes up. But I believe that was for the best. The emotion heavy episode of April and Jackson's divorce just seemed to hit a little too close to home for me this week.

It has been one year since I was seemingly forced into filing for divorce. And I am happy to say I have finally moved on. 3 years of separation total. But that scene where April asks Jackson if this is really what he wants was what got me thinking. Just like she did, while fighting to save the marriage, and hoping and doing everything she could, she was putting him first. I'm only talking about after she came home and they decided to work on things. SHE asks HIM if it's what HE wants. And he says yes. She says "okay" and signs the papers. I had that exact conversation, word for word, look for look, tear for tear. We were still putting the other person's feelings first, above our own. Giving them what they wanted.

I hope eventually I won't be angry over my divorce anymore. I know that our marriage was not good anymore. That my ex-husband had checked out years before we divorced. He was never interested in trying. But like April, I found myself pregnant as well. I found myself married and divorced to a man who never had any intention of believing in the same things I do. I guess their relationship has always had a soft spot in my heart because in so many ways it mirrored mine and my Ex's.

Today I can say that the very same God he doesn't want to believe in, is the one who kept me from depression, took away my bitterness, and let me lean on Him to walk me through the worst time of my life. At 25 years old, my husband's affair with someone who had been family for 10 years, rocked my world, his family's world, all our friend's comfort zones and left my family completely shattered in so many ways. I cannot even begin to explain the amount of bitterness, anger, jealousy and even rage that flowed through my body. But can I tell you what is even stronger than all of that? God's grace. God's love. God's hope.

The promises on which I stand, on which my family will be built, are unlike anything any man can offer. The peace of knowing that I have a Father who will carry me and Maggie through this life with his blessings, with his constant care and understanding- the feeling is like no other. The grace on which I stand, that I have been blessed to be offered, is the only thing that has made this situation better. No person, no words, no smiles from my sweet little girl. Grace. Peace. Love. Patience. Kindness and Self Control. I probably missed one. but you get the point. GOD- He is it. He is all.

I am no longer angry or bitter at David. I am hurt, and I imagine I always will be. He is finding his happiness with a new family, but that does not mean he doesn't remember HIS daughter. He loves her well, like any father loves their child. And that is what it's all about. I am sad that it didn't work out with the father of my child. But God will bring us a man who is truly after His heart, His ways, and has no intentions of ever straying from the covenant and promise that we will make to each other and to God.

So, here are my thoughts all splattered out here for you to read.
If I can leave you with anything, it's this: God is good, all the time. And all the time, God is good.


Shalom.