One, two, three.....

Social media is so amazing for so many things. Keeping in touch with friends and family, promoting your business, and making this big world feel a little smaller. 

When you're a stay at home mama of three little ones, one of which is homeschooled its especially awesome to say "World I'm here!" 

Before Facebook I didn't really "know" anyone with cystic fibrosis. It has been huge for me to connect with other parents and people living with CF. I learn things I didn't know, diagnose problems before doctors can at times through question and answer forums I'm fortunate enough to have access to. 

There is only ONE difficult part of being connected to this community. It is watching names of children you've prayed for, adults you've looked at and immortalized come across your feed and read of their passing. It's a reminder that CF doesn't care the age, how compliant you are or how amazing of a human being they are. It just simply takes. 

Yesterday THREE people passed away. It is crazy to me that with all of the advancements in our world people are still losing their lives. 

It's something that you don't really move past. I can't put it out of my mind like most of my friends or family. I can hide it away in a corner but it always makes the journey back to the forefront of my mind. It's a part of this I can't get around. 

With everything in life I supose that's the way it is, a little give and take, good with the bad, sacrifice for pleasure....

I've been chosen to walk this path, to know these amazing souls and to have to watch them leave this world. I try to not look too far ahead when it comes to Ayla, live in the now and do our best to stay well. There are so many uncertainties in her future, I guess that's the way all good things are. If it wasn't a little bit scary you wouldn't know how worth it -it was. 

Today's update

Ayla had a lung function test today. Her numbers were suprisingly good. Like better than they have been all year. Her dr is very pleased and is now looking at allergies and prescribing an inhaled steroid to add to her routine. This was an answer to prayer and a real shot in the arm for her weary mama. I went in expecting an admission and came out scratching my head. Thankful God keeps reminding me that no one (including me, in all of my triumphs and failures) is in control of Ayla's future but him.

A Bitter taste of truth....

Ayla had a check up today at CF clinic. Here's the 4-1-1

Despite all she has going on things went as well as can be expected. She had lost 2 pounds since Aug 4 (which brought her BMI down to 33%) in CF patients they want the BMI to be at least 50% since we know weight directly affects lung function). I'm not sure if it's increased activity or illness that's causing the loss, her appetite has been normal. 

She sounded pretty good when the doc listened to her take big breaths. Her lung function plummeted last fall when she was hospitalized for Mono and other infections and has never recovered. We talked about reasons for that, could have been permanent damage done to her lungs as an infant when she was FTT (failure to thrive) since we know

Weight affects lung function and she struggled for two months to put on ounces it could have affected "the generations" of airways that were being formed (as the Dr stated). Another reason could have been permanent damage from last falls illnesses. 

The Dr was on the fence about admitting her. None of us like the trend we are seeing with her and we need to stop this and gain some ground back. So she ordered a CT to compare it to last falls and go from there.

Shortly after the scan the Dr called to report to us. She said that it appeared "pretty good" some of the inflammation and issues that were present in 2013 had resolved themselves. BUT what she did see was 3-4 airways with bronchestatis.

http://en.m.wikipedia.org/wiki/Bronchiectasis

Bronchestasis is basically the scarring of airways, most people with CF end up with this but at Ayla's age it's "early" to show. CF is a progressive disease and there is no fixing bronchestasis. Once it starts it's likely to continue.

How do I feel about it?

Well pretty crappy. Ayla appears healthy and happy, exercise is good for her lungs but too much activity causes weight loss. I was praying not to get admitted but to be told there is nothing we can do if we admit her right now is more frightening for me. I don't get a clear picture of her health without traveling 4 hours for invasive tests. CF is peculiar, it hides and when you think you have a handle on it -it punches you in the gut. 

I can't control this, I'm at the mercy of god and his plan. A trip without a map makes it hard to travel. 

The last year we have been battling to get back what we lost and today we found that on top of being unsuccessful we have hit another dreaded CF milestone. 

I know that the impossible gives God the room to work and get the glory but I feel like I'm gambling with my kid. My most precious possession. I know that's what faith is, I'm always learning about faith. And sometimes I suck at it. I can say the right words but when it comes down to life or death for your kid you don't want "chance" you want "certainties."

I always felt like CF would be a part of her but not begin to affect her as early as it has. I don't know what the plan is God but I'm after quality of life and happiness for her. I know that he is too and in his own way he is working. Even though I can't feel it today his mercies are new every morning. 

I'm counting on that for September 3rd God. 

I am a "plan person" I need an attack plan and accepting this for Ayla is just foreign to me. Don't get me wrong, her Dr is amazing. And she is aggressive and "goes hard" against CF. She gives it to me straight and I trust her. This is just not something you can sugar coat, it's a bitter pill and it's not going down easily. 

Please pray that we find a way to reverse what's been done and to prevent this illness from taking away her spirit that I love so much. 

"I have CF, no biggie"

We recently moved to a new neighborhood. Ayla is so excited to meet new friends. Yesterday she and Larry were outside and so were our neighbors. They have two girls. They played and Ayla was so excited. She came inside and told me: "I told her mom what I have, but she didn't know what I was talking about."

Me: "what you have?"

Ayla: "you know cystic fibrosis?"

Me: "oh, yea? What did she say?"

Ayla: "she just said what's that? And I said oh you know I do my jacket and take enzymes and stuff"

Me: "yea we will have to tell her about it sometime, huh?"

I wanted to cry for a second and I'm not sure why. I don't know if i could have been more proud of my kid than that very moment. 

To me Cystic Fibrosis runs my life at times and feels like it's all consuming. But to her she's like: "oh yea you know it's this thing I deal with but I'm still awesome"

I'll gladly take the heat for you babe, as long as you'll let me! 

Want to learn more about CF? Visit www.cff.org and see how awesome she is!

A rock and a hard place....

I have felt something was "off" with Ayla for quite sometime now. As we're driving home today thoughts are flooding my mind:

-Can we make it through another 2 week admit?

-I can't not be there for Autumn and Amelia.

-This is the third time in 11 months

-she's only 7, what does 8,9,10.....look like?

-we will adjust

-she still hasn't mentally recovered from the last year

-will she become depressed?

-will I become depressed?

-is there something more I should be doing?

-colonized??

We put Ayla's health at the forefront of our minds, every decision is filtered through CF.  We are compliant, we do what we're told...and still CF is winning. 

If we are admitted how hard that will be? If we are not admitted does that mean we accept this cough and this decline in overall health as our "baseline?" NO!

I pray every night for all my family, my children especially. This is so much harder than I expected, although I never expected any of this. 

Today while we were in the pre-op area a little boy was there, couldn't have been but 3 or 4. He was a few beds down, with his sweet little face he gave us a big smile on the way up. He had lost his hair and it was clear his fight was cancer. Things like this are so senseless to me. Although I can't see what's going on in his life, who his persevering smile is affecting. His parents filed in with a big "hope binder" carefully reading his chart and doing everything they can to just get him better. He had an iv placed and his cries were haunting, Ayla was affected. She knew his pain and covered her ears. Next to him lay an inmate with shackles on his feet and his only visitors were correction officers. We were all there together, the innocent and the guilty. It didn't matter who was who, disease doesn't care. I couldn't help but say like I have so many times. "Is this really happening?" 

We then talked to Ayla about how lucky we are to have this medical care when we need it. It's so hard at times to see the positives and have to point then out and sell it to your little ones.

Ayla wasn't feeling well on the way home, I could see behind those eyes. She was afraid. She was upset "to be there again" and I am too. 

Your grateful but also scared, a definite rock and a hard place....

Where does my hope come from?

Over the last few days there has been negative press regarding the corrector-combo drug we have all hung our hats on for the last 7 years. This is not a cure but if successful could extend Ayla's life. 

Each time I see a new article or see the stock owned by Vertex Pharmaceuticals drop or being sold off in large quantities my heart drops and my anxieties rise. 

I am reminded to ask myself, "Where does my hope come from?" Is it in man or in God?

I know I have faith and hope in God. I know what I believe to be true for Ayla's future.....but I also know the lives that are being lost daily....and all of us in the CF community are fighting tooth and nail to keep our kids healthy and preserve lung function while in waiting...and now...we wait some more...

So today I'm mad. Tomorrow I'll have faith for the next day....and keep waiting....

The worst kind of heartache....

Tonight seemed like any other night, Ayla and I had just made "Not Yo Mamas Banana Pudding" together and it was time for treatments. She walked over slowly....and said she wanted to be normal and not have CF. Those words always catch me off guard and take my breath away. I told her I understand but what's normal anyhow!? She dragged herself over to her treatment center and I could feel her welling up inside. I put down the dishes and went over to her and got to her just before she lost it. I got down on the floor with her and that's where we stayed. She was hurting so....."why don't I have any cf friends?" "Why am I always sick?" "I don't wanna drive hundreds of miles to the dr" "I don't want to go in the hospital again" "I don't want to do this jacket all the time" 

My mind was racing, what do you say to a child who is different to make them feel like they are not? How do justify the need for dr's, treatments and hospitals without being insensitive to the fact that is SUcKs! 

I felt myself pray, and say "I can't do this god" 

We prayed, we cried, we read her life verse in the bible (Isaiah 61) I asked if she wanted to write in her "angry journal" she said she did. So we started the treatment and she began....I sat across from her trying to compose myself. 

I was just watching her write throigh tear stained pages, I've never really seen her upset for this amount of time. I went back over to her....I explained that so many have struggles, we have our own...she is normal she can do anything she wants. Talked to her about fighting for a cure and what that could mean for her. Talked about hope and finding it always. She desperately wants a friend to talk with..I told her how many people in the world have cf. She can now understand numbers and place value do she seemed to be pacified by that. Ofcourse she apoligized for making me cry, she is such an angel. 

I never imagined I would be living the life I am. Never imagined I'd be fighting for my child's life. Never imagined her pain would be from my genetic makeup. I had never heard of cf when I had her. Never knew a soul who dealt with it. And as the years went by I became familiarized with the medical side (which is no walk in the park) but the layers that are being revealed to me as we age are often more than I can bear. I read a blog today about giving it to god. I could never bear it on my own. I pray for stength bc I am weary lord. I know he helps me to say the right things because she often feels better after we have it out. But I am left feeling like I battled it out and lost all my company-men and am left alone to deal and pick up the peices. I know it's not true, I have so many to help me through. But just as Ayla feels no one really gets it. I am with her...this is our bond, the one we never asked for but as special as they come.....