Yesterday I broke out of jail. No, not really, I would never call the hospital jail... let me start again. Yesterday I came home from my amazing and health-restoring vacation. It was a wonderful two weeks, full of educating the uneducated and, well, coughing! I coughed so much more than any other patient anyone had seen. That's how I get better! At least it made them feel like they were making a difference. Some of the respiratory therapists who had me were shocked that I wanted CPT (chest physical therapy) longer than 10 minutes (or 1 minute per lobe/position)! I was shocked that no one had ever requested it or taught them differently. So what did I do? Educated everybody who handed me a nebulizer (except for the two wonderful ladies I met who could have written the book on being a good RT: Mary Catherine and Diana R. - you ladies rock!!) I was even asked one of the most ridiculous questions I've ever heard out of an RT's mouth: "Have you had CF since childhood, or did you acquire it later?" I know he didn't understand that CF is a genetic disease, because he didn't ask when I was diagnosed or even when I got sick. He asked if I "acquired" it. Now, I posed this issue to a lot of other CFers and many say it's common to get that question - and this alarmed me.
From a Respiratory Therapist, that question should be automatic grounds for re-enrollment in school. How can you take care of a CF patient properly if you don't know why they are there? The patient has lots of STICKY (more so than healthy people) mucus in the lungs. "But Justine" you may ask, "why is it stickier than healthy peoples' mucus?" Well I'm glad you asked, it's because of a defective mutation (actually two, one would still produce the phenotype of a healthy non-CFer) in the CFTR (Cystic fibrosis transmembrane conductance regulator) gene which causes chloride channels to trap chloride, and thus attract sodium to neutralize them both into - SALT!
Now I digress to ask you a question: What happens when you pour salt on a slug?.... Yes, it dries up.
Now back to my lungs: If chloride is trapped inside the excretory cells (lungs, sinuses etc), the salt forms and the water, instead of being freely excreted into the airways to form mucus, is not as free to flow because it is drawn to the salt in an attempt to create a homogenous salt water concentration on both sides of the cell wall. Result: stickier (drier) mucus.
End of science lesson.
If an RT doesn't know CF is genetic, they wouldn't know that we have sticky mucus, nor why we have persistent lung infections (bacteria thrive in moist, dark, undisturbed places). For all they know, we just have a bad cough, and they wouldn't be able to give us the kind of treatment we need, because they weren't aware of the root problem.
Please, the next time someone in the medical field doesn't know about CF, and should, educate them! Even if it's a simplified, two sentence explanation. It's really sad to think about how many people aren't getting the care they need! I know I am an overly involved patient, but you can't expect anyone else to be your (or your child's) advocate.
All in all, it was a great vacation. My PFTs yesterday morning were an FEV1(function) of 32% and an FVC(capacity) of 51%! Great, especially considering how sick I was when I went into the hospital. My port is healed and I was given thorough lessons on accessing and deaccessing, even doing it once myself. After two weeks it's healed very nicely, although it's still tender. I'm hoping that as it heals, the scar tissue will solidify it's placement. I took the advice of many other CFers with ports and cut a hole in a sponge to put over my port when using the vest. I tried it today, and it's still too tender for that, so I have been unhooking the shoulder velcro.