Thursday, July 24, 2008

My personal CF journey.

How did I come to learn I have CF? Well we can all thank my brother for that. He was born with and obstructed bowel that actually ruptured. He was very near death and had 16 hours of surgery on the first day of his life. Mom didn't get to hold him until he was a week old. These issues are fairly common for CF babies so he was tested. This was before genetic testing, so they did the sweat chloride test (basically to measure the amount of salt in his sweat). It was positive.

At this point my parents decided not to rish having another child that could have CF.....little did they know that I was already on the way! My parents have never been sorry that I was born though. Mom says she knew I had CF before I was tested because of the stuff I would cough up, and sure enough my sweat chloride level was off the chart. She doesn't remember the exact number but it was very high.

As I kid I always knew I had CF, what that meant, and that it is fatal. That is probably the best thing my parents ever did for us, being honest. I remember telling kids at a very young age that I have a disease. Some of the kids would get scare, but I just told them that it wasn't contagious and we would continue with our game.

When I was young there was a regimine I had to follow every day, but I didn't feel like it made me a freak or anything. In the morning mom prepared out nebulizers (nebs) and then did therapy on us (chest physical therapy, CPT, to loosen the thick mucus). Then we went about our day, taking our enzymes with food. It was normal to me. I caught cold frequently as a kid and that was the worst, just being sick. But I never had to go to the hospital like most CF kids. We went to our CF clinic every six months (we lived in central Oregon then and clinic was in Portland so it was a 3 hour drive). And that was my life.

I went to CF camp 3 years, and I must say I didn't care for it too much. I missed my friends at home. But one year I got to help teach the kids how to use a PEP. It was a brand new device to replace CPT. How wonderful! Mom had taken us to Seattle to get it since they had it before the clinic in Portland did. We still did CPT when we were sick since it helped get out the extra thick stuff better.

When I was abot=ut 16, shortly after we moved to Washington, I discovered a web site for people with CF. This opened up a whole new world for me! To be able to talk to OTHER people with CF was fabulous. I learned a lot there and made many great friends. I have always been considered fairly "mild" so it was interesting to me to talk with people who had gone through more than me. Sort of like having a crystal ball.

I started realizing that my CF was getting worse when I was about 21 or 22. I would get short of breath just walking to the bus station which was only two blocks away. I asked my doctors to try pulmozyme. Its a nebulized treatment that breaks down the mucus in the lungs making it much thinner and easier to cough up. Unfortunately it didn't seem to help much. Then I participated in a research study that used hypertonic saline for sputum induction (in english: I inhaled salt water to thin out the mucus so they could get a good sample). I was horrified at the amount of stuff that came up! I had no idea that it was even there! I was on the phone instantly with my doctor getting a perscription. The HTS has done very well for me!

I kind of went along just fine for a few more years. I would have the occassional cold or sinus infection, even got the flu once, but still nothing serious enough to get me a trip to the hospital. Then in November 2007 I was exposed to mono and caught it. The doctor was worried about me getting a secondary infection and ordered me to stay home for 10 days. I got that infection anyway and went on oral antibiotics. Those seemed to help a lot and I was feeling pretty good when the Sunday after Thanksgiving I went to the emergency room with minor abdominal pain. I figured it was probably an ovarian cyst but wanted to be checked out since it was on the same side as my appendix. Well, it turned out to be both and I found myself being taken to surgery at 5 the next morning.

Going under general aneasthisia is hard on anyone's lungs, but for someone with CF still recovering from an infection its asking for trouble. I just didn't seem to bounce back. On Christmas day I coughed up sputum streaked with blood for the first time. That was the deal breaker. I contacted my CF clinic and told them that I thought it was time for my first IV antibiotics. They set me up with the PICC team (PICC=periferally inserted central catheter, and IV line that is inserted in the arm and runs up a vein to your heart) and the home health care company. Luckily many CF patients these days are able to administer their own IV meds at home thus saving a trip to the hospital. Insurance companies usually prefer this too, saves them money. Plus hospitals are filthy and CFers don't need that exposure.

The IVs worked very well for me and my only regret was having waited so long to do it. After this I decided it was time to get more aggresive. I asked for a vest, a CPT device that you strap on and it literally shakes the musuca loose. I asked to start using another nebulizer, Tobi, and antibiotic to help keep the bacteria in my lungs under control better.

I just finished a second round of IV antibiotics in preparation for shoulder surgery (the story behind that another time). I don't want to end up with another out of control infection from surgery again. These days my lung functions (PFTs) are about 46-50%. Not as good as I would like, but I should have been more agressive a long time ago. Now I tell everyone I can to not sit back and wait for your doctor to tell you, ask them! I am hoping to be able to quit working and go on disability by the end of the year, or begining of next. I need to be able to take more time to focus on my lungs and hopefully get them in better shape.

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