Home Sweet Home

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.

Port

This morning I was awoken out of a dead sleep at 6 to my nurse flipping on the lights, "They're ready for you in 30 minutes."  It's port time!  I had them page respiratory so I could get an albuterol neb before they took me... gotta breathe, right?  The cute resident who came and talked to me yesterday was down in pre-op. :D He answered the rest of my million questions to the best of his ability - I'm a pretty involved patient (translation: control freak), so he wasn't really prepared to deal with me.  They wheeled me into the OR, I climbed onto the table and laid down.  They gave me some versed and started to wrap me up, "like a burrito".  The last thing I remember was muttering something about having a fallback career lined up at Chipotle.  When I woke up, I had some pain and a kickass incision near my shoulder:

 

A few hours later someone came to access it so I could start receiving my IV antibiotics through my brand new port instead of the peripheral IV they had in my arm (which are very painful for me!)

So there you have it! It was fairly simple and I can't believe I hadn't done it sooner.

I've fallen in love... with a new nebulizer!

A Breath Actuated Nebulizer... how cool is that?  There is no waste, whatsoever, and the treatment time is fast - faster than any neb I've ever had!  When you breathe in, the neb cup with the medicine pushes down on the air supply, nebulizing the medication, and when you breathe out, it lets up.  SO cool!  It's called the AeroEclipse and I'm going to get a script for them when I leave.  I have been doing research on nebs tonight and was wondering if anyone had any experience with using this neb with Tobi?

 

Also, I'm set for getting my port a cath in the morning, so I'll make sure to update, hopefully with a picture of it!

Tune up time

Went to the doctor yesterday, the PFTs were WAY down.  I think they are at my lowest, FEV1(function) at 23% and FVC(capacity) 32%.  I'm getting admitted to the hospital today.  While I'm here I'll be getting a port a cath so I won't have to get any more PICC lines (yay!).  I'll update more once I get checked in and settled. xoxo