Chasing Dragons

Trying to keep up with each organ system effected by the progression of scleroderma is like chasing dragons.  I don’t know what to look for, so the dragons taunt me with a hint here, and a clue there.

I have pulmonary fibrosis.  It can exist alone or be part of an associated condition.  Pulmonary fibrosis is an associated condition of scleroderma for me. Parts of my lungs have scarred and become fibrotic.  Funny thing about that is the strain of my lungs working harder can also put a strain on my heart.  Or I could have pulmonary hypertension.

I know there may be friends and family recoiling in horror while reading this, (If this a surprise, I truly am sorry.  And if you are that far behind you really should follow my turtle page on Facebook.  Keeping up with this is exhausting.  I’ve had this diagnosis for a long time.  We all know where this is going in about 50 years.  I just might need a lung transplant on the way.  Again, if this is a surprise, we really need to connect on FB.)

I’m the protagonist in a dark comedy.  (Which could be a result of an over inflated opinion of myself, but that’s a whole other post.) Of all the medical names for things I have had bounced off of me, my internal voice sounds like Amy Poehler in a round of “Really?” with Seth Meyers.  The most exhausting thing about all of this, is having to fight for tests to get ahead of the mayhem of scleroderma and its associated conditions.


I Move Like Jagger, I Just Don’t Look As Cool

Mick Jagger’s is 69 and he can move around on stage like he does, because he stays in shape.  When I’m 69, I plan to move that way. Without all that head moving and hands-on-the-hips thing.  I’m cool, but not Mick Jagger-cool.  I said I can move like Jagger, I don’t have the moves like Jagger. 

If I stay in shape, I have a better chance of a favorable outcome when the disease progresses.

Wench!  Bring Me More Viagra! 

I am taking Viagra four times a day to keep blood flow to my fingers and toes, as well as normalizing the pressure in my lungs.  I know this, because if I skip a dose of Viagra, I know it.   I stay as active as possible.  I take long walks, go hiking and do martial arts.  If I don’t get some cardio, I feel it the next day.  In my head (And by in my head, I mean it’s what I think, but it’s probably not true.)  So, In my head, I believe I am somehow staying ahead of the progression, somehow breaking up the fibrosis with my magic exercise.  Abra-freakin-cadabra.  During my exercise I have no distress, except the occasional fall in Krav Maga (Israeli Martial Arts) which all students experience.

The Viagra makes it so I can do these things without causing damage.  Which is fine.  I’m okay with that, but like all treatments, they are no cure.  Eventually, I’m going to need more Viagra or a whole new drug.  But while I’m on Viagra, we should get to know my heart.  Because when lungs have difficulty that gets progressively worse, the heart can be damaged.

So Let’s Get a Baseline
Sounds so simple, doesn’t it?  We know enough about scleroderma and related conditions like pulmonary fibrosis, an echocardiogram  (1)”…estimate  PASP (Pulmonary Artery Systolic Pressure) in patients with advanced lung disease such as interstitial lung disease and chronic obstructive lung disease can be difficult to obtain”

Okay, so since we have pulmonary fibrosis with scleroderma and tricuspid regurgitation (That is asymptomatic for me.) there is more than one study that calls for a right heart catherization.  (It’s just a test, don’t freak out.)

So, as I mentioned earlier, getting a baseline seems simple.  We want one so that we know if a condition is progressing.  Let’s see what my normal is so that when “shit gets real”, we can quantify changes and treat effectively.  

Apparently, there are doctors, who believe that a baseline is not necessary, without a diagnosis of pulmonary hypertension.

It could be called a challenge.  I call it a day that ends in “Y”.
Challenges for tests are nothing new to me.  When this happens, someone is lacking information.  When this happens, it becomes my responsibility to get the practitioner the information.  I made a case to rheumatology, they concur and some department head will have a come to Jesus moment.  It’s all good.
When I’m told “no”, I find a way to get to yes.  And I have never even read that book.  I switch to sales mode.   What can I do do to get you in to this car, today? 

What’s with the dragons?
Today was just one of those days. This was the third follow up appointment about a right heart cath, because the Chief of Rheumatology had been out of the country. We needed some seniority to get this heart cath done.  I felt like I was chasing something during my appointment, then I thought:  hey, why not make it a dragon?  Then once we were all in agreement that my test would take place, I felt like I was ready to ride.  Because that’s really all this is.  You get the diagnostic tools, the results will dictate the next move.  The test is coming.  I’ve caught the dragon.  Why not go for a ride?  It will be bumpy and scary, but it’s just a dragon.   Maybe I should have used an airplane metaphor, because on planes they serve drinks, and on a metaphoric plane, I can drink.  Dragons sounded really good on my drive home from the VA Hospital today.  I’m too tired to redo this.

If You Skipped to the End:
When a doctor tells me a test I need can’t be done because the chief of that department where the test is needed from doesn’t want to play, that chief just needs more information.  I could get mad and pitch a fit, but I have found anger a waste of time.

If you find yourself fighting for tests, please don’t give up, keep fighting.  I know it sucks.  It won’t get easier, but eventually, you will get used to it.  Of course it sucks.  But life is what you make it and complaining without action will get you no where. You wil find ourself walking information to doctors.   There are a lot of crazy diseases no one has heard of, so it;s our job as our own best advocate to get info where needed.   Don’t hate the playa, hate the game.  Then, get to work.  

Get More Information

Join me with Treatment Diaries Tuesday, May 7, for a Tweet Chat!  Watch for announcement on Facebook.

(1) The Role of Echocardiography in the Diagnosis and Assessment of Pulmonary Hypertension

Right Heart Catherizaton, Johns Hopkins Medicine.

Scleroderma Research Foundation

The Scleroderma Foundation

Scleroderma Care Foundation

Pulmonary Hypertension Association

Pulmonary Fibrosis Foundation