I'm So Special, I Get To Be In Textbooks

I'm trying to look on the positive side right now, after my cardiology appointment, and the positive items I have to add to my list include:

• I'm such a special case that I got 20 minutes of free, extracurricular echocardigram imaging done (3D and all...the good stuff!) because they want to use my images in medical classes and in textbooks. I get it in my files, and yet I don't have to pay a cent. Aren't I lucky?
  
  
• I finally got my pneumonia vaccine I've been meaning to get.
  
  
• My cardiologist rocks. And is as willing to fight hard to keep me alive and functioning as long as possible. That may sound like a "normal" doctor but, trust me, I've been assigned cardiologists before her who wrote me off as a "soon to be dead girl" and wouldn't come up with treatment plans for me.
  
  
• I get to be at the head of the line for any/all flu vaccines in the future as I am in the super high risk group, along with the octagenarians, chronically ill, et al. There will be no vaccine shortages keeping me from getting vaccinated!
  
  
• I only have to wear a Holter monitor for 24 hours. Last time I had to wear it for 48 hours. It's itchy, somtimes painful, and you can't take a shower or bath when wearing it.

Umm, yeah, that's about it. Other than the positives I highlighted above, this morning's session at the cardiovascular center really sucked. What did I learn today?

• My heart function is worse. There are lots of people with worse heart functions than I, but I fell out of the range of "low end of normal" for my Ejection Fraction reading and now am in the level of "sick people" heart function level. That sucks.
  
  
• I can no longer be technically diagnosed as just Left Ventricular Noncompaction because it turns out that I have this problem in both the left and right ventricles. I guess that makes it Biventricular Noncompaction...I've read about it, but it's not as common as Left Ventricular Noncompaction (and that's really saying something, as LVNC is about as common as a pink spotted dinosaur). Like my left ventricular problems, the deep trabeculations in the right ventricle are particularly evident in the ventricle's apex. The left ventricle is still the star of this show of medical mayhem, as it's the one that is the wackiest and causing the most problems.
  
  
• To paraphrase my cardiologist: "we don't know much yet about noncompaction syndrome, but we have noticed that both people with ventricular noncompaction and other cardiomyopathies that have both arrythmias" (which I have) "and lowered ejection fraction" (which I now have) "are at a much higher risk."
  
  
• Basically, I am at a much higher risk of just plain old keeling over and dying from the heart shorting out and stopping now. How are we planning to handle this? My doctor is doing the following:
  
  
  
  • Adding another medication to my list. I am now on 4 daily heart-related meds, if you count the manditory 81mg of aspirin to reduce my "significantly higher than average" chance of blood clots, which may cause strokes. This new medication is the best possible chance to raise my ejection fraction. I hope it works.
    
    
  • She will be doing another echo to check out my heart function in November. As my health insurance only covers one per year, I will probably have to pay for it entirely out of pocket (it will cost approximately one week's take home pay for me).
    
    
  • She will be seeing me again in November as well. We might be switching over to a more frequent monitoring of my condition.
    
    
  • My doctor is doing a 24 hour holter monitor to get a good overview of my current frequency of tachycardia, extra heartbeats, irregular heartbeats, so she'll have a better idea of my level of risk, etc.
    
    
  • It is highly probable now that I will need to have a defibrillator installed at some point to regulate my wonky heartrate. At my last appointment this was merely in the realm of "possible", so this movement to now categorizing it as being in the range, instead, of "probable" and "something we'll need to consider seriously" is scary.
  
  
• Another point I have to make: my GP and cardiologist have been super happy the last couple of years that my readings have remained mostly pretty steady with little or no worsening of condition. Today's echo shows that my condition is definitely worsening, so now we're getting into a more scary area, a rather unknown area. No one knows how fast my condition will worsen, or if the new pills will help, or anything. We're in the big, fat "unknown" area of Noncompaction Syndrome. Most of the people being written about and studied with this fall into two categories: in really bad shape and close to dying, or technically have the condition but aren't manifesting many (if any) symptoms yet. Since I've been diagnosed I've always been in this weird middle ground of having symptoms and possible worrying signs, but having overall OK cardiac function. Now I'm slipping closer to the "sickos" group, and I hate that. And the medical folk are very, very interested in me, as I am in a weird class of noncompaction folk they never see. I might be able to provide some "missing link" clues between the two groups, especially if other patients are discovered and documented as going through similar stuff. I feel a little like a bug in a jar at times.
  
  
• I'm kinda pissed at myself because, between all the worrying about my lowered Ejection Fraction and the whole "probable defibrillator" talk, I forgot to ask what my pulmonary pressure was. As my Noncompaction syndrome caused a low level of secondary pulmonary hypertension, I like to keep track of my pulmonary blood pressure. It usually tends to run at the lowest levels for people with pulmonary hypertension, but it is still significantly higher than that of the "normal" population. I want to make sure it hasn't gone up, too. As my cardiologist only had a couple of minutes to quickly review the video of my echo (my echo was immediately before my appointment with her) and consult with the overseeing physican, I doubt she had time to look at all the measurements in great depth, as this is one number I specifically wanted to know. Now I will have to wait until the 2 page, typed report of my echo is mailed to me.
  

So, final result of all this? We have more signs that the body isn't doing well, and may be starting to fail me. I am in good shape overall for someone with congenital heart problems and secondary pulmonary hypertension because I eat well, exercise regularly, do all the right things I am supposed to, etc., but my efforts can only do so much, and my heart and lungs have been working so damn hard for so damn long just to do the normal "everyday" things like circulate blood and oxygen throughout my body. They're getting a bit tired right now, especially the heart, and that is to be expected....but we'd kinda been hoping the magical heart would keep plugging away at a better functioning rate for longer than it has. I was diagnosed with noncompaction syndrome exactly 2 years ago, and was told by the first cardiologist (the bad one!), that studies showed people usually live 5 to 10 years after thediagnosis. I was/am hoping for a helluva lot longer than that, but there's only so much the rest of me (i.e., the non-heart) can do towards extending my lifespan. The heart is the weakest, most messed up and overtaxed organ I have right now. I hope the new medication helps, but I am not going to pin too many hopes on it being the magical thing which will make things significantly better. At best it may help some, enough to put me back closer to the low end of "normal" again.

And, in other news, I promise to y'all that I won't go into my crying and kicking temper tantrum at the Meijer Pharmacy this morning in great detail. Let's just say that they royally screwed me over, pissed me off, charged 150% of the normal retail price for my new prescription, refused to give me my prescription back, refused to fill the prescription for the specified 90 days (despite the fact that my work's benefits specialist said the health care company would allow it, Meijer insists they do not) and, not only did they change my 90 day prescription to a 30 day one, they changed my prescription availability from 12 months worth of pills to 5 months worth. Entirely randomly and of their own volition. I hate it when pharmacy wonks think they have power over me and can screw with things they have no control over. Needless to say, my voice was raised, a door was kicked, there were tears and some sobbing and I was pretty pissed. I still am.

I will be transferring my prescriptions relatively soon, as I think this was the final straw for me. Bad customer service I can handle, but grossly overcharging for a prescription, refusing to fill it as written or give the prescription back to me so I could take it someplace which could, and randomly screwing with my prescription to greatly reduce the number of refills really pisses me off. Meijer Pharmacy has now lost me as a customer, and their retail division may loose my custom as well, as I am that angry.