May is very important to me

Yep may is very important to me. For two reasons. 1. its CF awareness month (break out the purple!) 2.its mental health awareness month. I'm going to focus this post on CF. I think mental health awareness is super important and I have talked about that before and will talk about that again BUT so many people don't even know what CF is!

CF is cystic fibrosis. A progressive, ultimately fatal genetic disorder that affects around 30,000 americans. To be born with cystic fibrosis both of your parents have to be the carriers of a recessive genetic mutation on their CFTR gene. This means they do NOT have CF but can pass this mutation on to their children. Everytime 2 people who are carriers of a mutated CF gene have a child that child has a 50% of also being a carrier, a 25% of having CF and a 25% of neither being a carrier or having CF. A little like playing the lottery. I know families who have no kids with it, some like mine who have 1, some who all the kids have it. There are literelly thousands of CFTR mutations...the deltaf508 is the most common especially in caucasions.

Okay so thats the technical stuff out the way. CF affects everyone differently. I showed signs very early but not severe enough to make any doctors say hey lets test for CF. It was brushed off as reflux, sinus problems, asthma, allergies, food allergies...you name it. Anything that was a "horse" and not a "zebra". I was a little peanut...even being overdue I was 6 pounds, 9 ounces and barely 19 inches long....always a little peanut..atleast until the steroids.  I coughed ALOT...coughed until I puked. I puked a lot. I was constantly sick. My stomach was constantly causing problems. I wheezed a lot. My sinuses were bad...one doctor when I was 10 or so said I had the worst sinuses he had seen on a child. And surprise...the second time I was allergy tested (the first I was only like 2 and was literally allergic to everything) I was allergic to NOTHING (except like a very minor reaction to dust which we were later told was so minor it didn't even really count). Nothing to explain my symptoms. After nothing was helping and I was getting worse and worse and had been on high dose (60-80mg) steroids for months and was about living in the hospital and ER one doctor finally got the bright idea to test me for CF. Yep...positive. Genetics(which back then they could test for 75 or so mutations) showed 1DF508, my sweat test was over 100 (normal is under 40, a definite positive is over 60) (the sweat test was and still is the golden test for CF because there is no way to test for all posible mutations)

I was 16. I will never forget my first CF clinic visit to MUSC. BEing told we can't predict how long you will live..some people are really sick when they are kids and then hold steady for years....some are healthy and then go downhill fast when they get older....there are no guarantees. All it takes is one bad bacteria, one bad case of the flu, one bad pneumonia. At that time the median age of survival was 32....so being told this is the age that people are living to. You have a 50/50 shot of making it there.  I was 16...thats middle aged! I know they were being realistic and wanted me to know what I was up against but its tough to hear at any age...let alone as a teenager. We were told good stuff too...about the progress that had been made etc but for some reason its the above that sticks with me

I just celebrated my 29th birthday last week. Its a day I sometimes thought I would never see. I know that sounds dramatic but its true.  As I have aged my lungs have not only taken a hit but my heart has as well. At one point my resting heart rate was nearing 175. Life is real fun when your life revolves around pulmonologists and cardiologists and all that good stuff. Thankfully I once again proved the doctors wrong. Still though...I do about 2 hours of breathing treatments a day and 1hr of something called the vest a day..on a good day. I take Symbicort which is an inhaler. I take creon, digestive enzyme pills before I eat...anything . Prilosec because my stomach acid is way too acidic thanks to the CF and my stomach PH is off plus I  have reflux. I take Cardizem to help my heart rate. I tire very easily....I always have...I can remember being 10, 11 years old and coming home from school and going to sleep because I was exhausted...I rarely make it through the day without a nap and its been like this as long as I can remember. I could keep going but I won't bore you :-)

I read a quote and I think it sums CF life up nicely... "a person with cystic fibrosis never merely lives but must be kept alive". It takes work to stay healthy, to stay alive.

I don't make myself out to be some kind of hero or anything like that...I'm a real person living with a real disease that sucks. I whine, I cry, I don't want to do my treatments etc.  I've argued with my doctors because I did not want to go in the hospital and was willing to do anything except that. I've laid in my hospital bed and cried many steroid induced tears. I've walked many a lap around a hospital hallway. I've complained about how unfair life is, I've questioned God, I've been angry, I've been sad, I've been scared. I've been so so bored. I've felt so bad and been so sick I wished I could just go ahead and die so the physical pain would end. I'm very human...as all who struggle with various illnesses are. I'm far from perfect. But at the same time...I do appreciate things in a different way. Birthdays are a little more special...hence my "birthday week" celebrations. I try to savor each experience...realizing tomorrow is not a promise. I get excited over little things...even at 29 I guess I'm somewhat childlike in that regard. Holidays still excite me..be it Christmas or the 4th of july or valentines day.  Family is special....I cherish every milestone I see my nephew make...every moment I'm there for.

I guess this ends volume 1....