An online ‘friend’ on Facebook who recently found my blog and then friended me there, introduced themselves to me by saying they were sorry to read about my health struggles. Without asking me anything about my health, and I realize in an effort to be positive and upbeat, this person said, “But smile! At least you don’t have cancer!”

Oh, how I would love to have cancer at this point. Cancer is one of those diseases that becomes a cause. Everyone knows what cancer is, but the thing is, there are some cancers that are highly treatable, some are even curable, and at least with cancer you have something to ‘fight’ and ‘beat’ and you can ‘win’ or ‘lose’ your battle. With me, there’s nothing to fight. I will just progressively get sicker and sicker until my disease, or some condition associated with my disease, kills me. I won’t ‘win’ the battle. The only way I can beat this condition is if I happen to die in an automobile accident or skydiving (like I can skydive with my oxygen tank!) before the disease kills me. Or kill myself, I guess, but that’s just not something I would be willing to entertain, thank you very much.

People know cancer is bad. People have sympathy when they hear ‘cancer’, because at some point in time everyone has been touched by cancer in some way. There are so many different types of cancer, and everyone knows someone who has it or has died from it or who beat it. My friend Rissa has had more problems with her health AFTER she’s been ‘cured’ from cancer than the cancer itself has caused. The treatment for the cancer has left her with problems she never imagined she’d have, evne though the lab tests say she’s ‘cancer free’. It’s a relentless disease with a relentless ‘cure’.

No one knows what CTEPH is. Most of the doctors I meet don’t even know what it is. I often have to explain to emergency room physicians what I have and why I can’t let them start a fluid IV on me. I frequently have to explain to nurses on my floor why they can’t add a saline bag to my potassium or magnesium drips, or why they can’t leave the IV running to the secondary line of saline after an IV. I have to be my own advocate, because no one even knows what it is I have.

And to make matters worse, because I have beaten all the statistical odds for this very rare condition, the few doctors who DO know what the condition is don’t even know how to treat me. I shouldn’t be alive, so they don’t really know what to do to make me feel better. I realize the fact I AM still alive and in fact doing relatively well all things considered should be a positive thing, but when I’m sitting here wanting to get BACK some of my life I’ve lost, I’ve got doctors who are treating me as though I should just be happy to be alive at all and quit trying to push to get better. I told my pulmonologist at my last visit with him a couple of weeks ago that I will NEVER give up on believing I can get better, no matter what he thinks or says. I simply won’t.

And the surgery that they keep calling a ‘cure’ isn’t really a cure. Most people who have it are still having residual PH after the surgery. Those who survive 10-12 years or so after surgery are seeing that there is vascular remodeling and it’s causing the condition to come back 10 years or so down the line. And this time, since it’s remodeling and scarring, there’s no surgery that can fix it. And for this type of surgery–where you are cut open, open heart, full bypass, in complete hypothermia for hours and hours, it’s extensive and painful and horrible to recover from–you expect to get more than 10 years out of it. But that just isn’t proving to be the case for most people.

Because the PTE surgery is so new, and the condition is so rare, only about 5000 of these surgeries have been performed world-wide. That means there’s just not a whole lot of data on how people survive and fare after the surgery, years down the road. It’s got me wondering now if having the surgery is even the right thing to do at all for me. I’m so confused.

So I’ve tried to explain my condition to people so they would understand, there should be awareness, but I doubt there ever will because it is so rare. Imagine, if you will, a fish, taken out of water, lying on the dock, flopping around and gasping for breath–this is me, when I simply get up and walk to and from the bathroom, and that’s WITH oxygen tubing up my nose. I sleep with an oxygen mask, and I wear a cannula all day long. I use a wheelchair when I go out, because, can you imagine gasping for breath and flopping around like that out in public? Sheesh. I’d never get anything done or go anywhere. Plus, once my oxygen saturation levels hit a certain point, I pass out anyway. That can be dangerous if you’re out in public to just pass out wherever you are. And if you stress the heart enough, you go into A-fib, or throw a clot, and you can die suddenly, in a condition called ‘sudden death’. How’s that for a name? Sudden death. Lovely. Lovely.

The stress of the lack of oxygen causes the heart to work harder to pump more oxygen rich blood to the body. This increase in blood flow creates more pulmonary pressure, which actually causes the opposite effect of what the body is trying to do, which is to increase oxygen, but instead, it decreases it, the heart has to work harder, the blood backs up into the heart, and we end up with congestive heart failure. Fluid retention and weakness, tiredness, lack of energy, low blood oxygenation, cold toes and fingers, so many other things. Eventually, over time, CTEPH causes the heart to wear out–and what most people end up dying from is the damage to the heart.

If there are no other co-morbidities, if it gets bad enough, and if the clotting disorder is properly treated, and if they can find a good match in time, some people can get a full heart/lung transplant–but they won’t do just one, because if they don’t fix the problem, one just causes the other to go bad again. That might give the person another 10-15 years of life, but they’ll be on anti-rejection meds for the rest of their lives, and have other complications that come from organ transplant, and transplanted organs rarely have a natural life expectancy.

So people with this condition stop counting their lives in decades left. Some even can’t count in years. The current research online (which is a little old, but is the most recent you can find) shows a life expectancy of only 6 years after diagnosis. I’m three years into that. The longest anyone was documented as living without the surgery after diagnosis was 12 years, and that was in Japan. Years… just a handful of years.

That’s where I am right now–at that point where I’m counting my life in the number of years, months, or days I have until I HAVE to have surgery in order to survive longer. Then it will be counting down again until the next surgery I will have to have to add a few more years. But eventually, the years run out or your health does, or the worst happens on the operating table, or or or…

…it’s a sucky way to live. And yes, sometimes I DO feel sorry for myself. But I WANT to live!

But most of the time, I do my best to just keep living my life and hope that they’ll find a cure before I’m too sick to benefit from it. Hang on one more day, one more month, one more surgery, one more hospital stay, one more treatment, one more, one more… until there are no more.

If I can get 20 years out of that, I’ll be happy. If I can get 30, so much the better. It’s doubtful, but no one is going to stop me from trying.

So yeah, I wish I had cancer. I wish I had something I could fight and beat. I wish I had a disease where people ‘understood’ as much as they THINK they can understand a disease they’ve never had. I wish I had something people recognized and fought causes for. I wish I did.

But I don’t. And maybe part of my job, my purpose, if you will, in this universe, is to help others understand that there are a lot more people like me out there, fighting mysterious diseases that will kill them, than there are cancer patients. Some of us even lose our hair from our treatments too (ask anyone woman who has taken certain blood thinners for long-term what happened to her hair) and we get sick and puke too and we get lots of medical treatments and procedures as well. Maybe I can bring about some awareness for others who suffer but don’t get the funding or the love or the attention or the care or the concern that cancer patients (as a whole) usually get.

I’m not meaning to take anything away from cancer patients either. It’s a disgustingly awful disease, and it’s ravaged friends of mine, some who survived and some who didn’t. But there are worse things out there than cancer, and some cancers are actually not that bad and easily treated. Pain and suffering can’t and shouldn’t be measured or compared.

Love everyone who needs love (which means loves everyone)… and be careful what you say to someone who is sick. No, you don’t understand. I promise you, no matter how much you think you do, even if you too have CTEPH, you really don’t understand. Our pain is unique. Our suffering is the only thing we can have that is truly ours and ours alone. So sit with me through it, but don’t try to share it with me. And when you need me, I’ll sit through your pain with you too.

Love and stuff,

Michy