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.