Yesterday’s Clinic Visit

I am so lucky.

Lucky, lucky, lucky. I’m not sure how I’ve lived this long. Better people haven’t.

My clinic appointment went pretty well. It’s funny now my blood pressure goes up during the visit, especially if they take it before I do my PFTs, which make me nervous as I can’t tell which way they’re going to go. They end in happiness or dejection. No in-between for me.

When I was in the hospital for my two embolizations and 20 straight days of partying, my PFTs when down to level unseen since another doctor ruled the clinic years ago. In the span of my hospital stay, I lost over 20% of my lung function. The good news is – and why I am lucky – most of my lung function returned. I worked my ass off trying to get it back over the past weeks, even succumbing to twice a day pulmozyme, a drug has may cause me to bleed and one which my doctor and I discussed for 10 minutes. (Does twice a day make a difference? He says it’s only a 1 to 2% gain twice a day and wants me to take it once a day.) But I was desperate to use any tool in the tool box to get my numbers up. I’ve been doing my treatments three times a day, as always, with more vest time.  Flutter, of course. And I used the big gun of Cayston the minute I got out of the hospital.

Lucky me.

I also had a great visit because the doctor is top notch – outstanding from a medical standpoint – and human, caring and understanding.  It wasn’t a busy day and he listened to me vent about the last hospital stay and we came up with ways that future stays for all CFers can be improved – measured cups for hemoptysis volumes to reduce guessing; faster decisions to embolize. I’ve never met a doctor with a better understanding of what it’s like to stay in the hospital. He’s awesome. Bromance in the air? Kidding.

I’ve avoided a oral glucose test for the past three years. I’ve never had the time to go to the lab for two hours, or I haven’t wanted to take the time to do it. They’ve written me a dozen Rx’s for the test and it’s kind of a joke when I ask them for a new one each time. Tired of that game, they made me do the test at clinic and skip my morning McGriddle, which made me McMad. Not sure how I did, but I know I’ll find out soon. Scary waiting for results for that test, which is why I’ve avoided it.

I hate questionnaires – especially CF-related questionnaires. I’m at the point in my life that if I don’t like a question, I don’t answer it. When they asked me about sexual intercourse and my family history, I said “next, please.” Let’s concentrate on the questions that have some chance of making me well, people. I don’t give a sh** about zoo experiment statistics that you discuss over lunch and have no bearing on my health. Some of what I go through is none of your business. And the less I talk about certain members of my family, the happier I am.

The social worker visit? Hi. Nothing to say. Bye. Don’t take it personally. (They are my dark thoughts. I don’t need to share them with the world. Does a blog count?)

Here’s a mini-story about irony. The clinic visit, which was my “annual” visit even though feel like I live there, went fast. Usually they go slow and I’m there three hours. I could have been out  in 90 minutes if not for the glucose test. ARGH. And I promised to complete a six-minute walking test for a study they were doing, which also included a questionnaire. And I completed that questionnaire while the nurse was asking me questions from another questionnaire. Two birds, baby. I’m the zen master of multitasking questions. Ask away. I won’t answer, but you can ask.

I completed the six-minute walking test, back and forth in the waiting area. 9 complete laps and some extra feet for good measure. A month ago in the hospital my results were not good and I “desatted.” Not this time because I am full of luck to the point it’s falling out of my pants. And I’ll feel that way until the next time I cough up blood on a Saturday afternoon or have to be admitted. But until then, it’s all gravy, baby. Gravy.

Renaming My CF Drugs

It started with a puppy

Hello, Cystic Gal

When our then five-year-old daughter wanted to name our new lab puppy, we created a naming rule to weed out the names we couldn’t live with or inflict on the pup.

The naming rule: If we couldn’t stand at our front door and scream the name into the neighborhood without feeling embarrassed, the name was scratched.

Examples of names that didn’t make the cut: Cupcake! Creamsickle! Vanilla Latte! Banana Cream Pie! We laughed hard testing them, confirming to neighbors that we’re nutty, or least that I am.

Which brings me to CF drugs. Who names this stuff? Someone in Marketing obviously doesn’t understand what it’s like to take a medicine every day or every other month of your life. Or, at the very least, they don’t understand the psychology of the disease.

So, I’ve decided that a little renaming is in order.  Here we go.

Giving CF drug names some teeth
My wife once asked what drug I was doing and I answered, “I’m doing TOBI.” A small body shiver hit me as I realized how that might sound to the untrained ear. Not something I would want to yell from my front door.  Not to mention that our friend’s yellow lab was named Toby, and he couldn’t do much harm to bacteria.

We need drug names that sound tough. When I inhale or swallow a medicine, I expect it to go into my body and crush the last breath out of the bacteria. No more taking drugs with names created by nuns moonlighting as scientists. How about some muscle and weaponry?

Let the hellfire rain down on the cockroach Pa

Old name: TOBI. A name for a yellow lab.

New name: Armageddon Hellfire Mist. This drug name should be the equivalent of spraying RAID on a cockroach, then backing your monster truck over its carcass four or five times. If I’m going to invest 20 minutes twice a day breathing this stuff, I want it to be the end of existence for the cockroach known as Pseudomonas. I want this bacteria to face its worst nightmare – Armageddon – because this is a battle of good vs evil.

Old name: Colistin. A name a celebrity would give their kid.

New name: Lung-Jax. There’s no substitute for the scrubbing power of Ajax. I want my lungs shining like my sink and toilet do when my maid hasn’t been on a three-day bender of Mojitos and Manhattans. So shiny and new, the bacteria can see their reflection as they melt like the wicked witch in the Wizard of Oz.

Old name: Hypertonic Saline. This isn’t the worst name I’ve heard. I like the “hypertonic” part. Saline, not as much.

Love this stuff! Blast the mucus away

And Hyper-Sal? “I’m doing Hyper-Sal,” sounds like something a mafia wife might tell her friend over lunch.

New name: Hypertonic Jet Wash. Mythbusters placed an old school bus behind a 747 with its jet engines blasting. The “wash” from the engines blew the bus away like it was made of paper. That is what HJW does. Load up that old bus with bacteria and give them a ride they’ll never forget – right into a trash can on the wing of a paper towel.

Old name: Cayston. I want to tread lightly here because when I tested this drug I thought the nurse told me its name had a special meaning. Still, the name is a little too “prep school” for my taste. Let’s give Cayston a pet.

New name: CRR (Cayston’s Rabid Rottweiler) Unleash the attack dog with this drug. It chases down Pseudomonas aeruginosa and bites it in the ass.  “Oh, it’s just a doggy bite,” the bacterium says in its fake British accent, until the rabies start kicking in. No laughing then. And, no shot clinics in the lungs, either. Whose mouth is foaming now, bacteria scum.

Old name: Pulmozyme. I get it. The drug thins and loosens the mucus. Nice. I can work with that.

Clean-up hitter.

New name: Lobe Lube. This is like taking your 67 Mustang into Jiffy Lube for an oil change. Out with the crud, in with the new slippery oil.  Man, this baby purrs now. Not sure I’d take my new car here, but my beater, yes.

Old name: Xopenex: This sounds like something my crazy aunt calls her ex-husband when she’s pissed and lubed, which is most of the time.

Wedge those lungs open

New name: Crowbar. This has a double meaning. First, I want this drug to go in and wedge open my lungs, making way for the other drugs that follow.  Second, a crowbar is what my aunt used on her ex-husband to open his skull to the tune of 40 stitches when she caught him with Hyper-Sal’s wife. Good enough for her, good enough for me.

I’m feeling better already. Think of the marketing potential and packaging possibilities. Nice, huh?

Well, it’s time for me to go Crowbar my lungs open, flush them with a some Hypertonic Jet Wash, coat them with Lung Lube, then inhale a little Armageddon Hellfire Mist to kill the cockroaches that have been living large in my lungs.

I’ll get to the pills another day. I’m thinking Al Pacino in Scarface Say “Hello” to my little friends, Cipro and Z-Pack.


The Ultimate Cage Match? Fighting cystic fibrosis

Have you ever watched Ultimate Fighting?  It’s a simple concept: two fighters enter a caged ring and beat the living daylights out of each other until one of them is knocked out, gives up, or time runs out. It’s pretty brutal and blood is often spilled. I watch 30 seconds before I switch channels.

What strikes me about the cage match is how similar it feels to being boxed in or trapped by cystic fibrosis. Years ago, the cage was much larger and my opponent, cf, much weaker and inexperienced.  It was a mental game.  But my opponent has grown stronger and learned to punch and cheat and kick me in the groin.  The cage walls have moved closer together, too

Over the years, some great weapons have been tossed in the cage: TOBI, Pulmozyme, Hypertonic Saline, AZLI.  With them I have been able to give the hulking mass of bacteria a good beating.  Or, perhaps, at least moved it back to its corner to regain its strength.

But the cage feels tiny now and my opponent stronger and craftier.  The marks of two collapsed lungs scar my right chest, but most of the damage inflicted has been internal, though I’ve seen plenty of the blood.  And yet, I am so lucky, as it has hit others much harder and earlier in life.  But I still feel trapped by it, as it affects each decision and limits my choices in life. Just the stress it causes alone, is suffocating some days.

But I am lucky, very lucky.  I have my family, the CFF, my Twitter friends, my CF team, and  I know that soon another weapon will be thrown in the ring.  And better yet, one day someone will throw in the medieval battle mace of cf weapons.

With my spiked weapon in hand, I am going to beat the living shit out of this terrible disease for all the lives it has taken and the suffering it has caused. And then I’m going to beat it some more.  No horror movie ending here where the bad guy gets up again and again.  I’ll make sure of that.

Then I’m going to open the cage door and walk out, bloodied but still standing, the enemy defeated. What a day that will be.