The Good, The Bad, and The Ugly

I was off work yesterday, nevertheless, it was a busy day.  From 7:30 AM to 2:30 PM, my day was full of doctors, waiting, tests, clinic, waiting, labs, and more waiting.

The Good
CF Clinic was grand!  After dashing from St. Vincent's over to UAB, I leisurely strolled (editor's note: ran my ass off) trying to get to the 4th floor in time for my pulmonary function tests.  I made it with two minutes to spare - checking in at 9:28 AM for my 9:30 AM appointment.

The Stats

FEV1 = 1.58 liters (55%)    Woot!

O2 Saturation: 91% on room air

With vital signs and PFT's out of the way, I sat down to wait.  Luckily, I didn't have to wait long and I enjoyed a few minutes catching up with the two Ashley's.  A few minutes after 10 AM, I got called back to see the team.

First up was Karen, our new nurse practitioner.  I met Karen when I was in the hospital in January and I must say, I was very impressed.  She has not worked with CF patients in the past, but she is eager to learn and wants to get to know each patient individually.  This is important in any case, but particularly when you are dealing with a disease like CF that affects each person differently.

Next up to bat was Dr. Y, one of the best doctors in our group (and no, I'm not just saying that because I know he reads my blog) ... he really is that good.  After all, he listened to me babble on and on about the incessant itching that I've been fighting since last Friday.

By incessant I mean non-stop, never-ending, all over my body itching.  For reasons unknown.  I've ruled out all the obvious causes - dry skin (I've been drenching myself in Aveeno twice a day); changes in diet; new medicines; change in detergents; fleas (the kittehs are inside only so this was unlikely, but worth looking into).  I've re-washed all my bedding, bathed in oatmeal, taken Benadryl, and finally - out of sheer desperation - took 20 mg of Prednisone on Thursday night.  I think Dr. Y realized I was serious when I copped to taking the Pred - he knows how I detest that "evil little miracle."

After ruling out any likely reactions to my medicines, the next step is to see the Dermatologist.  We tried to setup an appointment for yesterday afternoon, but the earliest available was next Thursday.  Dr. Y took pity on me and prescribed me something stronger than the Benadryl to try and keep the itching at bay until then.  I took my first dose last night and was able to make it through the night without scratching my arm or leg off.  Sitting here typing this post working, the itching is itching to make a comeback, but it's tolerable.

The next topic of conversation was genotyping.  I've been genotyped before (DeltaF508 + unknown), but that was when they only tested for 32 mutations, not the 1000+ that are tested for today.

Dr. Carla submitted a pre-authorization request to BCBS while I was in the hospital, but the claim was denied.  The response that I received from the Medical Review Board stated that "the patient already has a diagnosis of Cystic Fibrosis" (really? you're kidding!).  We are going to file an appeal - two new treatments that are currently undergoing testing (VX809 and VX770) are genotype specific.  While I don't think I have the genotype necessary to be part of these studies, it will be helpful to know.

Last (but certainly not least) was the subject of my port. When I was in the hospital last month, I had developed cellulitis in the area around my port.  We decided to take a "wait and see" approach hoping that with the number of abx that I was on and with some "alone time" after I got de-accessed that it would heal on its own.  Happy to report that it did!  The infection, inflammation, and tenderness are all completely gone.  We were able to access my port and flush with zero problems and I had a beautiful, bright red blood return.

As you can tell, this appointment was a little more involved than the usual "How're you doing?" - "I'm fine." - "See ya in a month." conversation that I usually try to have.  Because of that, I was running a little late for my ENT appointment, which was scheduled at 11:15 AM.

The Bad
After finishing up in clinic, I high-tailed it upstairs to ENT clinic and checked in around 11:30 AM.

Where I proceeded to wait.  And wait.  And wait.

About 12:15 PM, the PCT called me back, took my vitals and set me up in Room 2.  Where I waited some more.  Finally around 12:30 PM, the nurse came in.  With a look of chagrin, she apologized profusely for the long wait. "No big deal," I said, "What happened?" "Well, umm ... you see ... mmmm, we kind of forgot you."  Wow.  OK.

Apparently word had not traveled from the front desk to the nurses that I arrived at 11:30 AM.  Dr. W. thought he'd seen everybody and went to lunch around noon.  She asked if I could wait til he returned at 1 PM and promised that I'd be the first patient he saw.  Even though I was mildly annoyed at being "forgotten," I know I'm not a VIP.  And I needed to have some lab work done, so I decided it was worth the wait.

Word to the wise - don't try to get labs drawn at Kirklin Clinic on your lunch hour.  Apparently, everybody and their brother had the same great idea.  I waited for about 25 minutes and called it quits.  I hopped on the next elevator up and headed back to ENT.  Fortunately, they didn't forget me this time.  =)

The Ugly
To be perfectly honest, ENT is never my favorite place to be.  Nobody in their right mind would enjoy having a 12-inch scope shoved up their nose.  I'd rather be strapped to a spinning wheel and have daggers thrown at me.  Fortunately, there's enough eye candy in the ENT office that the experience isn't a complete waste of time.

Even though my sinuses have been a little stuffy this past week, I honestly expected to hear the same pronouncement as last time -- "PERFECT."  But alas, it didn't happen quite the way I planned it.

My maxillary sinuses (the ones across your cheeks) still look great.  I flush my sinuses obsessively and it is well worth the time and effort.  He did clean out an inconsequential bit of gunk, but nothing to write home about.

And then he got to my frontal sinuses (the ones above your eyes).  Not so great.  Polyps and scar tissue that weren't present last time have decided to make themselves known.  GRRR.  It's frustrating because there is nothing I can do about it, it's "just a CF thing."

I'm going next week to have a repeat CT scan and then meeting with Dr. W. to discuss options.  I think the plan is to try and treat it with steroids (Pulmicort).  From what I understand, treating sinuses with Pulmicort is not an "approved" therapy (yet), so it has to be pre-authorized through Blue Cross before he can write me a script.

Just like that, my run of "perfect" sinuses was at an end.  With nothing more to be done (at least for now), my sinuses and I decided to head home after one more stop at the labs.  The crowd had finally thinned out and I was in and out in about five minutes.

Whew!  What a day.  I was glad to see it end (well, at least that part of it).  I was looking forward to the rest of my evening with family, friends and some excellent food.