Good News, Bad News, Strange News

It’s a big long medical jargon phrase. Ready for it? Here goes: Chronic thromboembolic pulmonary hypertension

See, for the past year, we thought I had pulmonary hypertension. Some thought it might be pulmonary arterial hypertension, others thought just pulmonary hypertension, but nobody ever said the words Chronic thromboembolic pulmonary hypertension to me, or what is more commonly known as CTEPH. But it makes sense now that we’re getting to the bottom of what’s going on with me.

CTEPH is caused by a chronic thromboembolic disease, which basically is a fancy way of saying that someone has recurrent blood clots, usually from deep vein thrombisis–that means the clots move from the big veins, most often found in the legs or abdomen. These blood clots are usually caused by a medical condition, genetic disorder, and sometimes, though rarely, for no reason that anyone can ever find. For me, it’s likely from a chronic, undiagnoised inflammatory condition, auto-immune, and it’s looking more and more like my diagnoses from over seven years ago of Lupus was actually probably accurate. I am likely a serum negative lupus patient, and that would explain the inflammation, the immune issues, the pain, the butterfly rash everyone sees and comments on–but my ANAs are all negative. We are finding that from kidney issues to the blood clots, I’m having all the classic lupus complications, so really, at this point, ANAs seem a bit… well, useless.

Regardless, the blood clots traveled, unbeknownst to me, to my inferior vena cava, where they then made a straight shot to and through (thankfully), my heart, dilating one or two of the valves there, and stretching things out of shape for a while. This is not a huge deal, since the heart has an amazing capacity to heal itself, once you stop whatever is attacking it–in this case, stop the clots from going through the heart, or make them small enough they can’t damage the heart. They did that when they put me on blood thinners and inserted an IVC filter to ‘catch’ the bigger clots. Now, if there are clots–and there is proof there ARE still clots being thrown, even on anticoagulation therapy–they are small enough that it’s very unlikely they will cause the PE ‘sudden death’ condition. That’s good. Sudden death doesn’t sound like a lot of fun.

From the heart, the clots then traveled through the pulmonary artery, where they lodged in my lungs. That’s when I was hospitalized last year, almost exactly one year ago this month, with massive multiple bilateral pulmonary embolisms (or emboli, depending on how stodgy you want to be). That’s great in on respect, because slowly but surely, the anticoagulation therapy is helping to make those clots absorb back into my system, and eventually will clear out my lungs. I go in for a third VQ test (ventilation perfusion–it’s a cool nuclear exam that makes me radioactive for about three days!) as soon as we can afford to pay the $2300 that the test costs.

The IVC filter doesn’t prevent clots from forming or traveling to the lungs, but it does help ensure that any clot that breaks free from the filter is small enough it can’t kill me immediately–but that doesn’t stop new clots from landing in my lungs, and that means that my breathing will take a hit every time clots start filling up the lungs or attaching to the walls of the lungs. This explains why sometimes I breathe okay and sometimes I don’t breathe well at all. I never breathe good any more. Oxygen therapy is my friend. The clots hitting the lungs is ultimately a good thing, since it means they can be reabsorbed, and I’ll feel lousy for a while, but then it’ll all go away for a while.

But the bad news in all this is, not all of the clots make it all the way to the lungs. Some of them lodge in my pulmonary artery, and then calcification covers over them, making it nearly impossible for them to absorb. Eventually, they become calcified themselves, hard little lumps, that prevent the pulmonary artery from contracting and expanding properly. This causes the blood flowing through the artery to have a higher pressure to get past the rigidity of the artery, and that higher pressure is ultimately the problem. You might know the phrase hypertension to mean ‘high blood pressure’, and this is true, but I don’t have traditional high blood pressure. In fact, my normal blood pressure is often quite low, but usually normal. My high blood pressure is in the lungs and the pulmonary artery leading to the lungs.

This high blood pressure in the lungs and pulmonary artery eventually puts pressure back on the heart, and that causes congestive heart failure. Ultimately, most people with CTEPH will die from congestive heart failure, if left untreated. CTEPH is considered by most doctors as a terminal illness–that is, the patient is expected to die from this illness, provided they don’t get hit by a bus or something like that. One research study I read actually said those who have a mean pressure over 50, 97% of those patients were dead within three years of diagnosis.

That scares me. Scares the shit out of me, quite frankly.

Moving on…

There is no treatment for CTEPH. The doctor told me today that the condition I’m in right now, while it could improve a little bit more as we get all the clots out of my lungs, is likely not going to improve. In fact, it is chronic, it will get worse, and the biggest issue is keeping my currently mostly healthy heart as healthy as possible for as long as possible, by treating all the symptoms as they come up, immediately as they come up. Avoiding damage to the heart is a great thing, because that is what will make me live longer. As to how long before it kills me? Who knows. Could be a couple of years… could be 20 years. They have no way of knowing. The doctors were holding my hand, patting my leg, saying things like, “We’re looking to bring you hope. We want to manifest positive, put all our energy into getting you better…”

Why is it that when doctors say things like this, it doesn’t give me hope, but rather scares the hell out of me?

So the good news: We have a diagnosis and we know primarily what we’re fighting and we mostly know, as much as we’re ever probably going to know, how all of this came to be. The part that helped me the most was this: It’s not my fault. I didn’t DO or NOT DO anything that caused this. The factors are so variable, there’s no way to know what caused it all to begin with, but diet, obesity, activity level, habits, etc, none of those things caused this. That somehow helps, in a way–I didn’t do this to myself or through inaction caused this to happen. I can’t help but think if money hadn’t been an object, docs might have caught things sooner and prevented some of it, but we can’t go back now and fix what happened or didn’t happen. It just made me feel better to hear him say this wasn’t my fault.

The bad news: It’s a terminal illness. I am expected to die from this or complications associated with this. And it’s not treatable. Not really. There are things that can slightly improve my quality of life, things that might slightly extend the quantity of my life, but there is no real treatment for this other than alleviating symptoms with oxygen therapy and medication and attempting to avoid further damage by new clots embolizing and causing more damage so things worsen quicker.

The weird news: While there is no treatment, there IS a cure.

Wha–? Did you read that right? Yes, there IS a cure. A completely and total cure and it’s effective in about 85% of the people who are eligible and undergo the cure treatment. About 10% of the people who seek this cure actually end up with rapidly worsening PH and a poorer quality of life than before the cure. And about 5% of people who undergo this cure DIE from the cure itself or during the recovery period afterward.

The cure is a surgery called PTE, or Pulmonary Thromboendarterectomy. In this surgery, the patient (that would be me), is cut open, as they say, from stem to stern (or Median sternotomy). Well, in other words, they would cut my chest wide open, crack open my ribs, and then for about six hours, I would have hypothermia induced, while I go on cardiac (heart) and pulmonary (lung) bypass. They would dissect my pulmonary artery, go in there and clean out all the clots from the artery, and then sew it all back up and then try to restart my heart. Assuming the surgery goes well, I’ll only spend three weeks in the ICU, where they have to restrict fluids severely, and watch for blood clots and pneumonia and fluid in the peritoneal cavity, the chest cavity and all sorts of other fun stuff, all while my sats will be very, very low, while I’m on high-dose major diuretics, and thus trying to keep my blood pressure from tanking.

If I survive that, then I go into some long-term care facility and start what amounts to about a year of rehab therapy.

If I’m one of the lucky ones, this will be a complete and total cure. The surgery isn’t done just anywhere, so we’d have to go to a place where it is done, to see doctors who specialize in this procedure (the leader is currently the University of California at San Diego, of all places), and coming home until I’m completely stabilized is not even an option.

And that’s assuming that, even if they determine I’m a candidate for the surgery–which I might not be, and many people aren’t–that we could afford to have the surgery. This is a highly skilled procedure requiring specialists in anesthesia (because PAH and anesthesia don’t mix well) and cardiac specialists–not just one surgeon, but multiple ones, and a surgery that start to finish is about 12 hours long. This, plus travel, pre-op appointments, assessments, and other things, plus all the aftercare and rehabilitation–where do you come up with that kind of money? I don’t have insurance, and even if I did, this would likely cap that out.

There is one other potential option, but it’s more a treatment than a cure, and that is to wait until the CTEPH has damaged my heart enough that I would qualify for a heart/lung transplant, assuming I am eligible for one at that time.

So… these are my options, and we don’t even know if the cure/surgery even IS an option for me at this point or if I’d even be eligible for it.

Short of that, it doesn’t look so good to me… so overall, not a great bit of news today. Sure, they are hopeful, and I actually, statistically, have beaten a lot of the odds. The doctor today told me how proud he was of me for how good I’ve been taking care of myself. I’ve been trying hard. I have statistically beaten every odd out there… but this news does really make me a bit sad and scared.

I called my mom and talked to her about this today, before I posted anything here… and she said to me, “I don’t know if this is a good thing or a bad thing.” I told her I didn’t know either. I mean, a cure sure sounds like a good thing, no? A cure! I would love to be cured.

But…

Well, you read this. You get it. Right.

So that’s where Michy is today. I’m processing this information. I’m trying to decide how I feel about it. Right now, scared is about the only feeling I can come up with, and I’m really not even that scared, as much as I feel like I should be scared, if that makes any sense.

I’m sure I’ll be talking about this more to come… as I process things, I write about them, so you’ll get to listen to me mumble about things and such as we go through this. What else is new, right?

I’d love to hear what you guys and gals have to say here. Be honest with me. Don’t bullshit me. I don’t let the doctors do that, and I sure don’t want my friends to either. What do you think? What would you do? Do I even want to try to see if I’m eligible for this surgery, do I wait a while and see if I get better or worse, do I do I do I? What? What do I do?

The one bright light that might be ahead is they ARE doing research into a new technique for doing this CTEPH cure that uses sonic waves, medication and scopes, so the chest cracking might not be necessary. It’s a few years away, though, as it’s only recently been approved for test trials, and I’m pretty certain I wouldn’t currently qualify for a trial. But if I can hang in there a few years without any damage to my heart, maybe there will be advances that will make my decision easier.

In the meantime, I read, watch, wait… and think.

If you’ve made it this far, thanks for reading. I appreciate and love you for it.

Love and stuff,
Michy

 

~~~ ***~~~

UPDATE:

After the bad news, my test results came in tonight on MyChart–I love MyChart. I’m seeing my test results before the docs even get to see them tonight. Anyway, they auto-posted at 8:45. My B-Type Natriuretic Peptide test, the one that was nearly 7000 when admitted to the hospital last year, was almost 600 just a few months ago… People with congestive heart failure, like I

have, are usually over 100. Normal is 50 or less. My test today was 53! THIS made me cry. CTEPH is terminal, because it damages the heart and people who don’t get transplants die from heart failure–and my heart is actually currently NOT in the heart failure range! This is good news! It doesn’t change my diagnosis–but it improves the outlook of my prognosis. The longer we keep my heart healthy, the longer I live and the better the quality of my life! 53 is fantastic!
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15 comments to Good News, Bad News, Strange News

  • Jeff  says:

    Michy, no matter what happens, no matter what the out come of your decision is we love you and appreciate all you’ve done for us over the years. Thank you for sharing all this.

    Our prayers, thoughts and hopes are with you as you face this long uncertain road ahead.

  • Cindy  says:

    Okay, so here goes. Congrats on finding out (finally!) what is happening. It sucks that it is a diagnosis that will likely kill you. After you get done be glad that you’ve beaten the odds so far and that your heart is basically healthy, go ahead and get pissed. Get pissed at God, the Universe, Fate, whatever for allowing this into you life. You need to and deserve to.

    I understand the joy/frustration of knowing nothing you did or didn’t do could change this. That’s how it is with Thor’s heart. It’s congenital and so no one even talks about how to prevent further damage just how bad the damage has to get before we talk valve replacement. It fucking sucks. There should be something that we can do; the same with you. There should be something you can do.

    That said, I say check into the surgery. Instead of worrying about how to pay for it, focus on whether you are a good candidate for it. Once you know if it’s an option, you can go from there. Until then, worrying about the potential cost is just wasting good energy.

    In the meantime, channel the passion for life that you have and live every moment of it. Make the life you have one that you enjoy living. That, in my opinion, is how we defeat the sometimes shitty hands life gives us. And, you know the odds, but sometimes, just sometimes, you can win the hand even if you started with crap.

    Hugs, healing energy, and a bit of pissed off sent with my deepest understanding,

    Cin

  • Cyndee  says:

    Michy, I don’t know what to say yet. Please know that you are loved by many. If we could take away THIS…Wow, I don’t even know what to call it without cursing! I have been through many struggles, as you know, but none such as this. I am not the preachy sort, but this passage has helped me through many of those struggles. I even had multiple copies of it taped around the house during the most difficult times.

    “Trust in the LORD with all your heart, And lean not on your own understanding; In all your ways acknowledge Him, And He shall direct your paths.” (Proverbs 3:5-6)

    Until I can come up with something more helpful to say, I hope this will inspire you toward whatever decisions you need to make.

    Cyn

  • Melissa Bisbee  says:

    I would first file for disability. Then find out if I was a candidate for surgery. But that is just me. I am glad that they finally put all the pieces together though! Call me if ya need to chat! Love ya bunches!

    Melissa

  • John Morrison  says:

    Let me second the filing for disability, and immediately. With that, comes immediate Medicare. Add Medicaid to that, if you should qualify, and poof… no more cost concern.

    But just look at you!.. right now, tonight:

    Worst news in the world, and you’re doing what you do – editorializing! You’re great at it because it’s what you do, and from that processing, no one can say what’s in a future that doesn’t even exist. Keep chewing up the fodder ‘now’ hands you, and spitting what you mold of it. The rest of things will take care of themselves.

    Keep it up. Keep doing what you do.

    John

  • Angela Parson Myers  says:

    If you can qualify for the trials of the new procedures, some or most of the cost might be covered. I’d guess the smartest thing to do would be to start all three processes at once: 1. Apply for disability 2. Find out if you qualify for surgery 3. Find out if you qualify for trials. All will likely take long enough for you to find out how you respond to present treatment before they come through. Then if and when they do come through, you’ll be in a better position to make decisions. To do anything else would be to burn your bridges before you even reach them.

    Prayers and good thoughts. If anyone can do this, you can.

  • Michy Devon  says:

    Hey, gang… I’ll comment to you all individually later, but for right now, I’ve already applied for and been approved for Social Security Disability. My payments aren’t a lot, since I’ve been self employed a large number of years during my work career, so I had no employer paying into my SS, and I also worked for the government for a few years, where SS wasn’t paid in either… there’s a retirement thing for me when I’m able to retire, but nothing to help me out now. My SSDI payments are less than $900 per month. My medical bills are currently $1120 per month, minimum, so SSDI helps, but it’s not enough.

    As for Medicare, John, et al, I can’t get Medicare until I’ve been receiving SSDI for 24 months. My first payment wasn’t sent to me until February of this year, so I haven’t even made it half that time yet.

    I don’t qualify for Medicaid, because the SSDI alone excludes me, plus there’s others’ incomes to consider in my family. I no longer have any minor children in my home, and in the State of Texas, there is no Medicaid for persons who are disabled, unless you have a minor child or receive SSI (the disability welfare program), not SSDI, the social security disability program. I don’t qualify for SSI; I do for SSDI, so there is no Medicaid for which I am eligible.

    Additionally, even if I could qualify for some type of assistance here in Texas, the surgery as far as I can see right now isn’t able to be done here, assuming I even qualify. The top place is San Diego–there’re a few other places and some on the east coast too, but not many. Only about 3500 of these surgeries are done per year, so it’s not like it’s something you can pop in anywhere and schedule. It’s a three-week, partially in-patient setup just to see if I qualify for the surgery. Any government assistance in Texas won’t be useable in San Diego or anywhere other than Texas, even if I did qualify.

  • Beverly Bright  says:

    Even though I no longer write, I have been following your progress. The news you have is just that…news. Thankfully they have a diagnosis. From this point, just run the ‘fire drills’. Take each step slowly and by all means live life to the fullest as you walk this path. I believe in a God that supplies all our needs, yours included. I pray for you and your family for all that ya’ll have been through. My heart goes out to your need and your decisions.

    There are so many people depending on, praying for you. I am only one. May God bless you and yours and hold you up in your decisions.

    Very, very best regards,

  • Michy Devon  says:

    Just an update, and I put it in the post too:

    After the bad news (see my previous post), my test results came in tonight on MyChart–I love MyChart. I’m seeing my test results before the docs even get to see them tonight. Anyway, they auto-posted at 8:45. My B-Type Natriuretic Peptide test, the one that was nearly 7000 when admitted to the hospital last year, was almost 600 just a few months ago… People with congestive heart failure, like I
    have, are usually over 100. Normal is 50 or less. My test today was 53! THIS made me cry. CTEPH is terminal, because it damages the heart and people who don’t get transplants die from heart failure–and my heart is actually currently NOT in the heart failure range! This is good news! It doesn’t change my diagnosis–but it improves the outlook of my prognosis. The longer we keep my heart healthy, the longer I live and the better the quality of my life! 53 is fantastic!

  • cathy  says:

    All I can tell you is, if my doctors told me I could be cured with a surgery (and a year in Hell) I’d ask where to sign. And I get the feeling that’s how you’re leaning. I can’t give you any advice – I know you. I know you’ll examine this up one side and down the other. You’ll know more about the whole process than the doctors, by the time you’ve decided…

    Know (of course you do!) that you’re always in my prayers. I hate that this is happening to you. Why is it, I can be all philosophical about it when I’m sick, but damn, it is hard to watch someone else go through it.

  • Jo Brielyn  says:

    Oh Michy. You’ve waited a long time for a diagnosis, now this one. I could fill this post with platitudes or promises I can’t deliver but neither of us “does” them. What I will say is I love ya, lady, and I will be here anyway I can. Always. <3

  • Kathy  says:

    So, the first thing I thought of when I read about this was “Her heart?! Wtf…she has one of the biggest hearts I know of!” I cried for a bit and realized that’s exactly why you will pull through. Your heart doesn’t know how to say “no” or “stop”, no matter the pain. Of course, the money hating hippy in me in is infuriated that money is the only thing standing in your way, but we’ll see what we can do about that.

    You are loved by many. You do so much for so many. That will serve you well as you know about the power of positive thinking and love. I’m rambling I know, but I’m hating this for you and hating that there is no quick fix. Much love Michy. May the white light shroud you always.

  • Linda  says:

    I’m so glad you finally have the diagnosis. I know that empowers you. I’m so sorry it’s such a shitty one. I wish there was more I could do for you. I wish I had the money to contribute to your surgery. I sense you are leaning that way. I think I would do the same. I don’t mean to sound morbid, but if I died on the table when they were doing the surgery, that’s not such a bad way to go. And if I lived, I’d fight like hell to keep on living. You are one of the strongest people I know. None of us understand why God lets things like this happen, but there is a reason for it, and I can only think God has amazing things in store for you. Hang in there, my friend.

    • Michy Devon  says:

      Linda, as morbid as it sounds, you’re right–dying on an operating table, while you’re completely knocked out and can’t suffer or feel pain — if you have to die, it’s one of the better ways to do it, I guess.

      The part that I worry about is, I CAN live like this indefinitely–as long as my heart stays healthy (and I have a little bit of control over that)–so is the ‘cure’ with the risk? And that’s where we take other things into consideration.

      But it’s moot at this point. My doctor is not even talking to me about the surgery at this point… he’s not even considering it. We have time, don’t know how much, but time.

      So for now, I stay as healthy as I can, try to prevent damage to my heart, lose fluid, get healthier/healthy as possible, lose some weight, and wait and watch.

      Then, when the symptoms are bad enough or my health takes a hit that would make the surgery more viable or when I get healthy enough that the risk of surgery is completely minimal, then we’ll move forward.

      That gives us time to save and put aside money and maybe invest or go to vegas or win the lottery or something.

      But yes–I do think that if it comes down to it and they eventually do say I’m eligible for the surgery–that is likely what I will do.

      I just can’t help but hope that by the time they say there’s a green light on it, there will be an easier, better surgery for me!

  • What do you do when they tell you…  says:

    […] for the terminal illness part, I’ve learned some more since my last blog post. First, terminal doesn’t mean tomorrow. In fact, to be precise, terminal illness simply means […]

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