5 Things You Didn't Know...

About CF.

There's really nothing exciting going on in our household right now. Just typical day-in day-out routine. So here are 5 things you probably didn't know about CF.

1. There's a third type of diabetes.
Did you know that? Yep. CF related diabetes. Until my son was diagnosed, I had NEVER heard of it.
50% of people diagnosed with CF end up with CFRD. Oh, and...unlike types 1 & 2...they can't just help it along with insulin.

2.  They are THISCLOSE to curing CF for double Delta F 508 mutations. That would lead to the cure for all the other mutations!
The basic defect in my son's mutations is that the salt receptors are MADE, they just don't get placed where they are supposed to be, thus CF. Kalydeco, and VX809, are both aimed toward correcting that salt receptor!!

3. Every day, someone is born with CF and someone dies from CF.
Do you see why we need the cure?!

4. Salty ocean air is supposed to be great for CFers. It helps them breathe better.
It makes sense, the defect is that salt doesn't pass through the cells like it should. Hypertonic Saline is salt water with lots of salt. Please though, tell me why I live in KANSAS?

and ...  I'm going to double this one up. Because they play together.

5. 1 in 400 couples, both people carry mutated genes and don't know it. 1 in 31 people carry cf, and don't know. For every couple in which both parents are carriers, there is a 25% possibility that EVERY child they have will have Cystic Fibrosis.

Now then, visit Corban's website to donate to the Cystic Fibrosis Foundation and help make more tomorrows everyday.

Oh the sounds... of music?

One of the things about Cystic Fibrosis that really sucks...is pancreatic insufficiency. Corban needs enzymes. Today..we had a catastrophe.
After church, was the girls' tea party. We had our niece and nephew, and my daughter's half sister. Corban and my nephew went with my mother after church. Now, generally we have a stash of enzymes at any place where Corban spends a good amount of time. For those...just in case moments. Mom did have a stash. However, said stash had been depleted more than we knew.
By the time that the tea party was over and I got out there to pick him up, he had announced he was starving and eaten all the fruit he could stand. (Enzymes not required) Well he apparently just could NOT wait to eat the chili and was served a bowl without enzymes.
Now - before you chastise me... yes. He's 5. Yes there were adults present, and yes..someone could have said okay we need to resolve this. That fact is behind us as it has already happened and nobody can change it.
Corban didn't eat all of the chili...and quickly made it known that he didn't have enzymes. Under normal circumstances where he'd gotten them he would of course get gas. The difference is...the smell.

I am surrounded by testosterone. There are 3 males in my home. Gas is not abnormal. Neither is hysterically laughing about said gas. Especially not with Corban. I promise you that it is not. The chili must have been fairly fat free because the ensuing tummy ache we just knew we'd deal with.. never happened. We did experience what Corban calls his "special music". Which is not very pretty. One started tooting... and the other followed suit. A couple hours later it is now a trio with my husband 'playing' along.
The thing about a CF toot...it STINKS. He has made the house smell horrible. And as I walk by his bedroom... wow. Plug your nose.
The lesson here...maybe Corban should carry a med-pack for those just in case moments, and perhaps an air freshener.

Just another log in the fire....

By now you all know that my son Corban has Cystic Fibrosis. We hate it. He hates it. Society hates it. (Especially when he's having tummy troubles haha.)

Well, today, a friend of mine on Facebook shared this photo. The issue I have with this is....nobody has ever said "enlarged heart or gallstones." Maybe I've been naive? I just don't remember it EVER coming up. My heart quite literally skipped a couple of beats when I saw this.
Just another reason for us to find that cure. I know it's out there...we just have to find it.

Which reminds me that it's been a while since I plugged THIS:
A Challenge that only you can help me conquer!
Go visit this guest post on my pal Non-Stop Mom's blog and read all the way to the bottom for how you can help!
Or you can visit our team page Corban's Crusaders to donate to my team! Help us Cure Cystic Fibrosis, and help ME beat Ames in this competition, so I can make her look SILLY, and y'all know there will be pics!!!

Quest to Kindergarten

 

In my quest to ensure that my baby boy goes to Kindergarten next year, I downloaded a few apps onto my iPod. I don't have an iPad but a friend suggested that I could use my iPod for the same thing. 

Corban is lacking the skills to move on to Kindergarten from Pre-Kindergarten in the fall. He will be 6 in October. Not being able to move on with the rest of his class mates could be quite the blow to his self esteem, which sometimes lacks as it is. He has to be able to rhyme, currently he can only guess a couple if he's lucky. He has to be able to name all the letters in the alphabet. He can't even name all of the letters in his first name.  He needs to understand alliteration which also falls into rhyming. Currently, he is unable to do that, as well.

One thing about CF is that it requires treatment time. That takes about 30-45 minutes for one session. So, I set him up to multitask. The downside? The iPod sounds like poo, since it was dropped and display fell apart. My husband had it fixed for me for Christmas, but the people didn't do it correctly, so the speakers sound muffled. Secondary to this...the vest is LOUD and you're running a nebulizer. I may need to invest in some headphones for him. But, long story short...I will send my little boy to kindergarten this year.

 

Corban is my first boy. When my daughter was in school, she flew through like a breeze, and she still does wonderfully. She reads almost 2 full grades above her current grade. She is picking up math skills, and just all around doing great. I guess I had built myself up to thinking that my son would be the same way. Unfortunately, I didn't take into consideration..not only is he a he; but he is my son....and his father's son..and that combination creates this wonderfully stubborn little boy, who has the ability to be as difficult as trying to squeeze toothpaste out of a tube that has had no cap for quite some time. This has definitely been something a little against us this year. 

With any luck, this will work. Otherwise, I may enlist other troops to get it done. I heard somewhere "raising a child takes a village." Raising a child with Cystic Fibrosis, and my genetics...is going to take an ARMY.

Cystic Fibrosis- Our Story

I originally wrote this as a guest post on my dear aunt's (Non-Stop Mom's) blog. However, I feel it would be a good story to share with you. I have edited to add things as well.

 

In 2004, I gave birth to the most beautiful little girl I've ever laid eyes on. Shortly after her first birthday, her dad split. In 2005, I met my now husband. He took her on as his own, loved her, cared for her, fought for her. For 2 1/2 years it was just the three of us. In February 2007 we find out we’re expecting. Things began rocky. I had what I believe to be the worst case of morning sickness known to man. If I was awake, I was nauseated. Exercising, eating, drinking, asleep…you guessed it. When we finally got to the point to where we could find out what the sex of the baby excitement was tantamount to Christmas morning to a four year old. We both had hoped for a girl, but I figured deep down my husband would want a boy. We hear, “you see that? Looks like a turtle? That’s the penis.” It’s a boy. Much to my surprise, my husband’s face dropped. However, by the time we got home that day he was excited. “I’m going to get to teach someone to wrench on things like I do. He’ll tear his bike apart and put it back together.” There was the excitement I’d dreamed of. Fast forward four weeks, to our next appointment. “The sonogram showed some fluid retention in the kidneys, which isn’t necessarily normal, but it’s not abnormal either. We’d like to do a second sonogram.” Okay, cool. We’ll get to see his chubby little cheeks and cute button nose again. Next sonogram the normal “drink until you think if you even inhale you’ll pee your pants” happens. They take a bunch of measurements, tell you what they’re looking at, we get a couple pictures, done.  The first appointment I attended alone, the OB was out of town.  Let me tell you, there are few things I thought would ever worry me. I never imagined that I would be 25-26 weeks pregnant and terrified. I see the PA and she says, “There were still bright spots on the bowel.” I’m sorry, what?!  This is the first I’ve heard of this. “Well, there are a few things it could be. It could be nothing, or there is a 1% possibility it’s Cystic Fibrosis.” They were going to play vampire that day anyway, so I told them to test it. Now, what they were looking for is whether I was a carrier or not. I was told 7 days for labs to come back and no news is good news. The 7^th day I’m thinking, whew, we really cut that one close. 5:15 pm my phone rings. “You ARE a carrier for Cystic Fibrosis.” Crap. Now we’d have to get my husband tested, but insurance refused to pay for it, and we were too broke to pay out of pocket. We had no choice, but to hope and pray.

October 10, is my husband's birthday, we had spent the day together, and generally just had fun. We weren't due until October 27th. Well, the night quickly turned into tomorrow, and around 4 AM on October 11, I KNEW I was in labor. When my daughter woke up that morning, she was sick. I had to get her to the doctor, her appointment wasn't until 9 am. Hubby was wrenching on his brother's truck, and a repair man was coming out to work on our stove. I was also to work that afternoon, my LAST day before taking maternity leave. We made through everything, and around 11 finally headed toward the hospital. When we got there, they tried to send us home. Now, we lived about an  hour away from the hospital, so I begged for them to let me wait at least another hour. You know how hospitals work.. 2 1/2 hours later they come check me. Now we're not leaving, active labor. Good job baby boy.

October 12, 2007, Corban made a sweeping appearance at 1:36 AM. Nothing like what labor was with my daughter. This was easy. He was 7lbs in all of his beautiful glory. He had passed meconium in utero, so we thought we were out of the woods. He nursed like a pro, except he seemed to always be hungry. He nursed 45 minutes, every 45 minutes. At 10 days, he developed a cough. It took some pushing, but I finally got them to test him. He was given a sweat test that afternoon. Now, typically they say they can’t get a newborn to sweat enough for good results. I reckon he didn’t need much sweat, because he was given a “suggestive positive” two days later. The phone call came while I was gone. I was eating lunch with my mother, and she had a phone call in the office. When she came back out she said “you need to go home right now. That was Matthew.” I knew. I hit the ground. All I could say was No repeatedly.  Suggestive positive meant he could still not have it. The only problem, his number was 112 mmol/L. Suggestive positive would mean he was below 60. At 60, it’s a diagnosis. We were referred to a specialist two weeks later. They did genetic work, and they found two genes. Official diagnosis.  Delta F 508, a very common mutation and Delta I 507, more rare. He was diagnosed as Pancreatic Insufficient in the office. He went on Creon (enzyme replacement) at that point, and will be on it for the rest of his life. Today, he is going on 6 years old. He has had pneumonia twice, tonsillectomy, adenoidectomy, polypectomy, diagnosed with Distal Intestinal Obstructive Syndrome (DIOS), at one point required a PICC line, and he has digital clubbing.

Treatments are a big deal in our lives. Corban takes 3 breathing treatments once a day, and two twice a day. During those treatments he uses a vest, which is a machine that basically shakes him and breaks up the mucus in his lungs. 28 pills on average per day, and a nasty nasal rinse twice a day. Every 3 months, we take a road trip 200 miles north to see the Pulmonologist, Dietitian, Social Worker, Gastroenterologist, and Respiratory Therapist. Despite all of this, he is your typical boy.

 

Corban at 5 years old

 

 This is the equivalent to a Peak Flow, however it is computerized. It tells what volume of air he can release from his lungs. We call it, FEV1. Because it's based on 1% This time, he blew a 98%. He's been as low as 70%.

 

This is an example of digital clubbing.These are his fingers.

 

Corban's Daily Med regimen, minus miralax, and vitamin D. 

All three crazy kids!

 

 

 

We now have another son, who was born September of 2010. He does NOT have CF, but could carry it. He is a definite rival to his brother, in all that they do. Height, weight, eating, playing, wrestling, you name, they argue over it.

 

Corban will live the rest of his life battling CF. I pray strongly each day that he will beat it. His odds are good. But it’s a downhill disease. Right now, the average life expectancy for a CFer is 37.

Here’s where you come in. Each year Great Strides puts on walks all over America. Our Local walk is May 4^th. This is our chance to raise money to fund the research for the cure, as the Foundation runs solely on donation. Nearly 90 cents of every dollar raised goes directly to research.  Just visit www.cff.org/great_strides/Hollenbeck to donate on behalf of Corban’s Crusaders.

Revamping

Anyone who has followed me long enough, has probably noticed posts disappearing and the general look reforming.
I want to take this moment to let you in on my secret. I have decided to switch gears. This blog has always been more about my selfish rants, than about anything in particular. I could be doing so much more. So I'm going to gear this blog from now on to a collection of life as a Mother. A mom of 3, who battles not only 3 kids, but CF and a spouse who battles his own addiction.

With that being said...I'd like to reach out a little bit. I would like to do a couple of things.

• A Q&A, with you asking, and me answering.
• Tell me something you would like to know about Cystic Fibrosis
• Anything else you'd like to see me write about.

Ready. Set. GO!

Stress eats away at.....

What? It certainly doesn't eat away my fat cells. Though, I wish it did.

Tonight was Parent Teacher Conferences. I knew my daughter would do great. She always does. She's almost a straight A kid. She needs to work on her listening and direction following skills, as well as her fiddling habits. Sad to say, she gets that from me. I fiddle when I listen. I cannot sit still, and it helps to draw pictures.

My eldest son on the other hand...he's a handful. Stubborn mister Corban. He's doing well, but I know he could do better, and if he doesn't...well he may not make it to Kindergarten this year. His teacher is amazing. I have the utmost respect for her, and great faith in her abilities. It's mine I question. He doesn't quite grasp rhyming. He can only name visually a handful of letters in the alphabet. When he should be able to name them all, and if not...then at least all of the ones in his first name, right? Nope. Corban has his mind set in his ways and honestly, that's my fault. Let me explain why.

The day Corban was diagnosed with Cystic Fibrosis, I was enveloped with a large feeling of guilt. In my mind, it was kind of my fault that he has it. I carried it. So that began a vicious cycle. He was taken from sleeping in his own bed until he was ill, to sleeping in our bed every night. I mean he had a bed, and he slept in it maybe 13 days until he was 3. To this day I struggle to keep him in his bed. He has a temper beyond belief. For years, I have blamed this on the medication. A prime example of this is this evening. His sister and I were working on Rhyming words with him, and he got upset because bed and baseball didn't rhyme. He repeatedly punched the couch and screamed quite literally as high pitched and loud as he could. It's so frustrating for me, since I don't know how to reprimand it the appropriate way...I don't necessarily let it go, but I can't really do anything about it without teaching him it's okay. So again, he basically gets away with it.

I know my guilt is something that I have to put aside. I have to accept that this is what God has handed me. It's really hard to do, even 5 years later.

We definitely have our work cut out for us with him..and his younger brother is watching with a learning eye.

Cystic Fibrosis and How YOU can help!

Hey y'all. Great news, our clinic visit on Friday went well. Corban is up 3 lbs, and grown an inch. I got the call this morning that he grew Staph. This is normal for the last year. If he has an exacerbation, we'll get antibiotics, but he's doing GREAT!
So, that being said...not everyone is as lucky as we are. There are parents out there waiting for new lungs, waiting for test results, sitting by their child's bedside as they lose this battle. The ONLY way to stop this is with YOUR HELP!
Oh, and you can prevent me from looking like an idiot at the walk on May 4th. Click here to donate to our team, and prevent glittery hair, moomoos, pig tails, or that horrendous rainbow mohawk!!!
Non-Stop Mom: Do you really want me to look like an idiot? Here's your chance.

My Current Challenge - I need YOUR help.

Hey everyone!! i wish i were as successful in bloggyland as my favorite blogger Ames over at Non-Stop Mom. but since I'm not...go check out my guest post for her about a subject that IS my life. And as is par for Ames and myself, we upped the anty with a challenge. Spread the word!

Non-Stop Mom: Do you really want me to look like an idiot? Here's your chance.