Monday

I had a really good blog written out, but then it got to this point where the next paragraph was either going to be super blunt, or super whiny. So away went the whole thing and now you get this:

When people are in need, and there is nothing you can do to truly help, the best thing you can do for them is pray. Whether or not they believe in the same God you do, or at all, sending "good vibes" doesn't mean anything, because generally, people don't even keep the person in their thoughts. Prayer is intentional. It is committing to sit down and think about and pray for this person in their trying time. Being intentional is a great sign of love for other people. And loving other people is one of our commandments as Christians.

God has brought me down a road I never in a million years saw coming. But through that, I have been able to bless more people more often- both in my works and my money. And I have learned, as I hope others do too, that God is doing far more behind the scenes than I even realize. I will never know the amount of fighting for me that has gone on, even when I don't feel like He remembers me at all.

I encourage each of you to find some way to help out, to volunteer time or money to a cause. It doesn't have to be a big production. It could simply be a friend who needs birthday or christmas gifts for their kids, someone who needs help with some groceries, a ride to the doctor's office, a cup of coffee and conversation, or even just a hug.

We never know what someone is going through until we reach out and ask. That person who seems so happy might be going through the hardest time of their life. And the hardship could be of their own doing, or someone else's against them, but no matter what- it is a hardship, and as fellow humans, friends, believers- we need to carry those burdens together, with each other and with Christ.

8/8

It's amazing how quickly one comment can make you reevaluate everything you thought to be right. But what's more amazing is how fickle the human mind is and can be. I will be the first to admit that I daily struggle with my faith, what I believe is right and wrong- not my moral compass, but how I don't see smoking or drinking as a sin necessarily (but when it becomes an addiction is a very real illness), or how I too think like so many millennials (and whatever my generation is called) and don't see it as a problem to not be completely involved in a church. And other questions of life.

So a couple weeks ago when a blast from my past said something along the lines of "maybe you need someone who sees the world a little differently" it really made me question everything. And this week, when a very close friend and great woman of God AGREED with that statement, and said that there's a little leeway for everything, my mind was all of a sudden blown.

I have been so focused on living this by the book life, but I have only gotten myself into situations and relationships that I don't love. I have prayed and trusted and felt led into these places, but maybe there was more to them that didn't get nurtured or understood.

I believe God created us to be people of wander. Of chances. Of second, third and fourth chances. Of Growth. Of change. Of doing. Of creating. Of life.

The definition of life is: The condition that distinguishes animals and plants from inorganic matter, including the capacity for growth, reproduction, functional activity, and continual change preceding death.

 So what do you do when the right situation comes along, but 2/8 of the things you thought should be one way, aren't? Do you overlook them? Do you compromise? Do you say "well, it's the perfect job in the worst town" or "Well, he's the unicorn I've wanted in every other way" or do you simply say "it's not the full 8/8."


 I have a vision for my life. A goal. I have things I want to accomplish, I have completely attainable dreams. But with those dreams, there have to be people in your life who will push you to accomplish those things. There has to be the job who is understanding and accommodating to the fact that you need to work, but also have a family, or other responsibilities, as well as a drive to better your life. There has to be those family members, significant others, friends, who stand behind you and encourage you every step of the way. I have these things, for the most part. But I also have better opportunities and relationships and friendships waiting out there in the world.

So when do you look at everything you thought to be right and true and good, and say it's time for change, and take the plunge? How much courage does it take? How do you know it's the "right" thing, or do you just sort of say "YOLO" and go for it? (I'm inclined to go the yolo* route these days).
 I don't know the right answer here, but I do know that life holds so much more than we think it does. There's an entire world out there that is ours for the discovering, if we'll only take the adventure.


*YOLO- You Only Live Once for those of you not hip to the lingo days**
** yolo itself is years old, that's where my knowledge ends. "It's been lit, fam" (I believe that's a legit sentence hahaha ;)

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!)