Keeping up with each organ system effected by the progression of scleroderma is like chasing dragons. Sure, no one has ever seen an actual dragon, but something that "just" appears to be part of the progression, could be the start of a catastrophic condition that can be managed if caught in time. These dragons keep the villages of my body on high alert.
I have pulmonary fibrosis.
Pulmonary fibrosis can exist alone, or as part of an associated condition of a disease like scleroderma, which is the case for me. Parts of my lungs have scarred and become fibrotic. I function well, but without medication like Viagra to help my lungs work effectively, my heart could suffer damage and eventually fail. Another factor that has yet to be determined is whether or not I have pulmonary hypertension. Sure, Viagra may be helping the my lungs, but is it also helping to maintain a stable pressure in my pulmonary artery. I still may have undiagnosed pulmonary hypertension and it must be ruled out. If this is news to you:
I know there may be friends and family recoiling in horror while reading this, (If this a surprise, I truly am sorry, but it's your own fault for not paying attention. 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. (Probably a result of my own 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 by doctors and people who think they are doctors because they read Web MD, 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. The test I need to completely rule out pulmonary fibrosis is called a right heart caterization, called the Gold Standard of PH Diagnosis by the Pulmonary Hypertension Association.
Exercise helps me.
I move like Jagger, I just dont look as cool. Mick Jagger's is 69 and he can move around on stage like he does, because he has stayed in shape all these years. Do you really think he does his signature "chicken dance" because he thinks it looks cool? I don't, but he makes it look cool. Placing his hands on the back of his hops with his elbows behind him opens his chest to make room for his lungs to expand with even more air. (Look, I don't know if he starting doing this move on a dare, but technically, that's what it does, so I;m going to go with it. Mr. Jagger, your comments are certainly welcome.) 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.
Long story short, if I stay in shape, I have a better chance of a favorable outcome as the diseases progress.
Wench, Bring Me More Viagra Mead!
I take Viagra four times a day to keep blood flow to my fingers and toes, as well as to normalize the pressure in my lungs. I know this, because if I skip a dose of Viagra, I get tired faster, and my lungs feel like they aren't opening as much as they could. 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. Maybe it's all in my head, but it's working. 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.
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"
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. I would call them idiots, but the truth is, they don't know enough about pulmonary hypertension and variables that can provide a false negative with echo cardiograms.
Educating doctors 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, some department head will have a come to Jesus moment and maybe there's a hug at the end. (okay, the hug part never happens.)
When I'm told "no", I find a way to get to yes. And though I have never even read that book, I know how to get to yes. I switch to sales mode: What can I do do to get you in to this car, today?
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, some department head will have a come to Jesus moment and maybe there's a hug at the end. (okay, the hug part never happens.)
When I'm told "no", I find a way to get to yes. And though I have never even read that book, I know how to get to yes. 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 saddle up and 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 another department where the test is done 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.
When a doctor tells me a test I need can't be done because the chief of another department where the test is done 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 nowhere. 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
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Right Heart Catherizaton, Johns Hopkins Medicine.
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