This post was written by my niece Ashley.
OK, OK, technically she's not MY niece. She's Ex1's niece. So she's really my ex-niece. Or something like that. But you know, that's just semantics in the grand scheme of things.
Listen to what she has to say. It's important.
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Cystic Fibrosis is a life-threatening genetic disease. There is no cure. You cannot catch it. You are either born with it, or not. However, just being around someone with it or their cough, will NOT, I repeat, WILL NOT make you sick. CF causes mucus to build up and clog some of the organs in the body, most commonly the lungs and pancreas. Mucus building up and clogging the lungs cause difficulty breathing, and frequent infections since the thickened mucus is bacteria’s favorite place to take up house. This eventually leads to lung damage. Many patients with CF are diagnosed as Pancreatic Insufficient, and require replacement enzymes to digest their food, maintain a healthy weight, and receive appropriate nutrition. Statistics from the
Cystic Fibrosis Foundation indicate that approximately 30,000 adults and children are currently diagnosed with the disease, and that approximately 10 million more people are carriers! That breaks down to 1 in 31 Americans.
Let me introduce to you how that plays into our lives. In 2005, I met my husband. In 2007 we found out that we were 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 was, the excitement was tantamount to Christmas morning to a four year old. 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 7th day I’m thinking, whew, we really cut that one close. At 5:15pm 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.
On October 12, 2007, Corban made a sweeping appearance in the middle of the night. 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.
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| Digital Clubbing. Rounded fingers and wide fingertips. |
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.
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| Vesting |
How does this affect our family? 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.
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This is the machine that tests his FEV1 -Forced Expulsion Value based on 1%.
In other words...how much of the air in his lungs can he get out.
It tells what level his lungs are functioning at.
He has been as low as 70% - today he was at 98%! |
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Trip home. He puts out that much effort
on the machine. |
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| Corban's daily medication |
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. 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
this page to donate in my name...... or.......
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Here's where it gets fun. Ashley and I are both very competitive people. And we are evil. And we can really really torture each other.
So we have a little challenge going between us. You can either go
her page to donate to her, or you can go to
my page to donate to me. All of the money goes to the same place in the end.
Out of the two of us, whoever gets the most money donated gets to 'dress' the other person the morning of the walk. If I raise more money than she does, I get to make her wear whatever I want her to wear - clothes, hairstyle, shoes, makeup, accessories - you name it.
But, if she raises more money than I do, she gets to do it to me.
And we are evil. EVIL. Trust me, whoever wins this little challenge is going to TORTURE the other person. Just imagine - one of us is going to be dressed like an idiot while walking about 3 miles with over 1000 other people who are dressed normally.
Yes. There will be pictures. Lots of pictures. I can promise you that.
So will you please help us? Help Corban and the thousands of others who have this disease - with your help we can one day make CF stand for Cure Found!
You can also help by sharing this post as much as possible. Make THIS go viral. Make it knock that silly Tooth Fairy letter out of the bloggy world because this is way more important than that thing ever was. Corban thanks you.
