Lone Cyprus

Lone Cyprus

Wednesday, October 10, 2012

Now what?

This is what I'd like to do: I'd like to just present the issue and have everyone I know, well or otherwise, vote yes or no. Just make up my mind. Choose for me. You guys can do that, right? Make just this one decision for me. No biggie. Easy. Well, you know, except for the part where my life depends on it. I know, I know, not so easy anymore. Tell me about it.

I have pulmonary fibrosis and pulmonary hypertension secondary to scleroderma. If you're reading this and you don't know what that means, I have to assume you have access to google. So there's no cure for this stuff. I'm terminal. In the past 2 years, I've gone through the evaluation process at both Vanderbilt University and Cleveland Clinic to be listed for a double lung transplant. Both times I've been rejected. Neither were willing and/or able to take on the risk of a patient with my disease coupled with the fact that I have an esophagus that doesn't work. Since January I've been working to adjust to and accept the fact that I'm out of options. There's no fixing it or putting it off. The time I have left, is the time I have left. Period.

Except two months ago I met with my pulmonologist at Vanderbilt for a regular checkup and he told me maybe that's not quite the case. The University of Pittsburgh Medical Center has begun doing some transplants for patients like me. My doctor gave me some information and said to go home, think about it and let him know what I want to do. Obviously, that's not been an easy decision for me. Two months have already passed and I'm no closer to deciding. I know it sounds crazy. If I have a chance to live longer, why in the world wouldn't I take it? Why am I not running to Pittsburgh?

It's just not that easy. There are so many catches to this it's crazy. First of all, the evaluation process alone for this is the most intensive week of hell you can possibly imagine. I'm willingly signing up for something I swore 9 months ago I would never do again. Blood draws, IVs, scans, xrays, ultrasounds, freezing cold temps, etc. AND you want me to pay you $25,000 to let you put a tube up my nose and down my throat while I'm awake?? All that to tell me that MAYBE I can be listed for a transplant. No promise of how long I'd wait for lungs. No promise that I would survive the surgery. No promise of how long I would live afterward.

We're talking about one of the most painful, intense surgery recoveries possible. Assuming I live through the surgery, I'd wake up on a ventilator with chest tubes, IVs in my neck, a catheter, an incision that spans from armpit to armpit and a feeding tube. No eating food for like 3 months? Whaaaa?

Let's say I put away the fear of pain and just think about what I'd be gaining. Here's the truth: The average life of transplanted lungs is 3-5 years. About 80% of recipients live 1 year, with most of that first year being rehab. Right at 50% of patients live 3 years after transplant. Only 50%. That's not a lot for only 3 years. And with one year being recovery and rehab, what will the quality of life be like?

Then there's the financial cost. A couple hundred thousand for the surgery. The constant cost of drugs, doctor visits, travel to Pittsburgh & other medical supplies needed for the rest of my life. Yes, I have Medicare but it's not free and it doesn't pay for everything. Not even close. I have the most generous parents in the world, but there's a limit to their resources too. The weight of the financial stress doesn't even compare to the emotional stress to consider. I'm quite familiar with the building of hope. And the crashing as well. If this only affected me, I could make this decision. But it doesn't. It includes and affects every person that loves me. Not to brag but I have a heck of a lot of people who love me. I deserve none of it, but I have it.

I think about all of that. And I think, yeah Beth, just let go. It's so much. It's too much. BUT what if I could breathe again? Just breathe freely. What price, financial or emotional, do I put on that?

How much of this is bravery for daring to hope and how much is actually my courage failing me? Is it refusal to give up or a fear of accepting the end? Is the pay off worth the sacrifice? Being sick isn't fair but what about my responsibility to what's fair both emotionally and financially for my friends and family?

I don't expect anyone to have the answers for this. This is just what's happening in my head. A little glimpse into how this is all going down for me. I really don't know what I'm doing. I'll holla.


  1. Hello Beth,
    I am a fellow Scleroderma patient just like you with pumonary hypertension and fibrosis. I read your post with great interest, and we are almost on the same path. I have been turned down by Loyola University Medical Center for lung transplant due to my esophagus issues - no motility at all, and I will soon be travelling to the Cleveland Clinic for an evaluation. I am curious about your experiene there. My doc wanted me to go to UPMC, but my insuance will not allow me to go there. If you would like to email me privately you can at stborowiak at yahoo dot com. Thank you!

    1. Sue, Cleveland went well. Everyone was kind & helpful. I was told, however that the problem was my immotility. I don't know what they will say to you, but they would not accept me. UPMC is apparently the only place taking the risk, because they are avoiding aspiration after surgery by using a feeding tube instead of allowing you to eat. I haven't been there yet to get all the details, as there are insurance & financial concerns for me as well. I wish you the best of luck & if I can help more feel free to email me on yahoo at bethann914.

  2. An exceptionally hard decision to make. No simple list of pros and cons can work this out. The financial cost can seem overwhelming but it's able to be estimated; the physical and emotional cost for yourself and your loved ones is a much harder thing to predict and prepare for. To have the hope of time and the freedom to breathe are things nearly everyone is able to take for granted, but they're priceless when you don't have them. However, after many years of fighting for hope and health, doubts arise about whether to venture forward and risk the unknown. I deeply sympathize with how hard this decision is.

    For what it's worth, I don't think you're losing your courage. I don't think you're giving up. I think you're counting the cost, for yourself and those you love. That's is part of this godawful process, and you're handling it as honestly as you can. I think you're assessing what your limits are and what kind of quality of life you want. I think you're doing the impossible task of trying to guess at what kind of pain your loved ones can or would rather bear. I think you're exploring what hope means for you and if it can take on different meanings. I think you're trying to figure out where you can find a measure of peace for yourself. This. Is. Hard. All of it.

    A couple questions I would have if I were someone close to you:

    1) Along with quality of life, what is the predicted outcome for if/when the lung transplant fails, either in the best case (5 years) or worst case (<1 year)? To put it very crudely, what does that death look like? What do your doctors say that can look and feel like? Is it it comparable to your current prognosis? Which endgame do you have a greater sense of peace with?
    2) Is there greater peace of mind for you in knowing every possible avenue for remission has been acted on, or are there other sets of goals that might bring equal or greater peace? Does pursuing a lung transplant give you a goal to work toward that is valuable for you, regardless of success? I know that for me, when faced with life-altering decisions, the hardest and scariest part can often be not having a goal or plan to act on, no matter how hard that plan may be. Which set of goals or course of action would give you the greatest sense of control?

    I sincerely apologize if those are hurtful, simplistic, or offensive questions, and please don't feel the need to respond if you don't have the desire to do so. I want you to know that you're being heard, and you're not crazy or depressing to be thinking and feeling these things.