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Cystic Fibrosis and Bi-Lateral Lung Transplants as Therapy

Donate LifeI’m pretty sure I’ve written about my views on CFers and lung transplants as the ultimate therapy/treatment nearly to the point of it being ad nauseum, except it may be continually overlooked since it’s often in combination with a manifesto about taking control of your life and doing everything you can do keep going on your original equipment as long as you can. Some of my comments about my view on transplantation on Facebook have shaken up some dust, gotten people upset, and encouraged/strengthened others. Since it’s such a mixed bag and Facebook is hardly the place to write 1,400 words on the topic, here goes!

Here, I am fighting to Change the Standard of Thinking of patients, family, friends, and even the medical community in several areas, and this is one of my most passionate, though likely not the most far-reaching and definitely my most controversial. The way I see it, if everyone thought they would eventually need a transplant no matter what they did regarding treatments and compliance, why the flip should anyone do any at all? They’ll just get new lungs earlier and go about their lives with digestive, sinus, vitamin and mineral absorption issues. I am not against transplants as a therapy or the people who get them. What I am against is the mindset that everyone with CF will need a transplant someday to avoid death or else live on life support in a hospital until they die once they can’t sustain themselves.

Keyword: Everyone

Why Fatboy is Pro-transplant and Organ Donation

Notice the banner for Angel Cove Organ Donation Awareness in my sidebar. It’s a shop and site run by a mutual acquaintance of a friend whom I met in Chicago at a blogging conference. Fast forward 3 years and I come to meet her after she used my business contact form after he referred her to me for some of my services. Three months later, she saw my tweets and Facebook stuff about this site and we were both floored by the “coincidence” that both her husband and I have CF. He just celebrated his 10th transplant anniversary this Fall. His story and watching this video where two of the most powerful things that made me set into my mind to never give up on my natural lungs, with those reasons following soon.

Organ donation saves lives. You can be in a slip and fall or a car accident (or a ton more freak scenarios that will get you on TLC or the Discovery Channel) that can cause brain or spinal trauma to the point where the body will shut down but clearly never be at all functional again. With good health, many people can be saved or rescued from a disability. With poor health even, many times some organs can still be sent off to someone in need.

Pros to Transplantation as a Therapy

  • Extended life past what is possible with original equipment.
  • Giving loved ones (hello, spouses) a new CFer and more time than otherwise.
  • Improved quality of life after life gets more normal after a few months.
  • Regained ability to exercise and do activities often absent for decades.
  • No need for supplemental oxygen.
  • After a while, no daily need for Vest or nebulizer treatments.
  • Easy weight gain if not experiencing common gastro issues after surgery.
  • No more wondering when these CF lungs will completely give out.

Cons to Transplantation as a Therapy

  • Could possibly not make it through surgery or the touch-and-go recovery period.
  • Living with the fear of rejection.
  • Living with the fear of infection with an immuno-suppressed body.
  • Severe initial demands of clinic visits, bronchs, and physiotherapy.
  • Restricted diet after a life of eating anything you want.
  • Relatively short-term data on success rates and skewed numbers make the decision difficult leading back to the last pro listed, only now with new lungs.

Now that it’s been summarily (and also once again) established that I am pro-transplantation what, exactly, are my feelings and thoughts on the matter. Forgive me if some of this is a re-run for you, but we’re joined by a bunch of new readers since my last run on the topic.

If my lungs gave out next month and I was on O2 and IVs didn’t give us any results, I’d be on “The List” in a heartbeat. We have saved for such a time, though it’s not at the level we would need to feel comfortable going forward because to do so is only prudent. We are not planning on a transplant yet still planning on the possibility of a transplant as wise financial and emotional planning. Some additional fund-raising would immediately begin and we are confident that we will be provided for in our time of need if that time should cause a need.

I was not always of this thinking because I was not always needed. It makes Beautiful very sad to think that there was a time in my life that I felt this way, but seriously, let’s dive into my head back in the day. One’s parents don’t need them, but they will always be liked and loved unless one’s parents are sick in the head. I had friends, but hindsight painfully shows that I was more interjecting myself into the lives of others because I was lonely, not that anyone anywhere needed me around. Any husband, wife, father, or mother knows of that difference that occurs in one’s psyche, their very soul, the moment they realize they are truly needed.

I was on track for a transplant pretty soon after our wedding on the course I was on the year leading up to it unless marriage “suited me well,” health-wise. Indeed it did. I went something like 13 or 16 months without IVs after the best day of my life. I knew she was on my team if that was what would be needed at any point to keep me around, but I was under strict notice while we were still engaged that I was not to let it happen before necessary due to any lack of effort on my part due to the very danger of the surgery.

At the time, the published numbers were a 50/50 survival rate at 2 years. Now it’s 5 years. Both of those are skewed away from being true numbers for recently healthy CFers in their 20s and 30s because it includes all people for all reasons in any health condition at the time of transplant. Example: it’s like saying the divorce rate in the US is 50%, give or take. Yes, it is, until you take the following into account as positives that all decrease the “risk” of divorce: first marriage for both, have higher education, two sets of un-divorced parents, pre-marital counseling, dated longer than 6 months, earn more than $30k/yr, have a common belief system, and did not live together before marriage. From what I’ve gleaned from research and from seeing so many long-term survivors, the survival lengths of CFers are way beyond the medical community’s averages (and I like using median numbers better anyway).

What will get on my raw nerves very fast is the mindset of anyone (be it patient, family, friend, or doctor) who is counting on a transplant in the future “just because” it’s what is normal for CF. I’m not talking about those who decide to not get listed. I’m talking about those who were diagnosed late and have a more mild CF. Those who are seeing great results from their current therapy. Those who are so young they may get to benefit from a cure before they’ve even had half the number of PICC lines that I’ve had. Some day when the technology and techniques for a bi-lateral lung transplant are so refined after there is a cure, there may still be a day where natural aging would still continue to decrease my function and require a transplant – but I’m not going to revolve my life around the mis-guided “fact” that all CFers will die early from respiratory failure unless they choose and receive a transplant in time.

I will not discount that it is a very personal decision, as I know of CFers who have chosen not to go through with a transplant (though I don’t know of any married CFers who have chosen that) and a growing list of those who have been listed and successfully transplanted. Now that my decision is immaculately clear and why I would choose to get new lungs, let’s take a bow to those who’ve received new life and take a moment to remember those who didn’t.

Comments

  1. Excellent, as usual.

  2. Two thumbs up! Great job. You clearly make valid and well said points.Many CFers are living into their 40’s (and probably more) before needing to look into transplants. No one really knows what advances in CF (and Transplants) will be available in the next few years! ๐Ÿ™‚

    • Indeed. My CF center has several in their late 40s and even 50s who still aren’t on the list. I’m super glad you got yours when you needed them, b/c you REALLY needed them.

  3. A wonderful wonderful article. I hope that I did not offend with my FB comment the other day. I was agreeing with you but hurried and tired!

    Goodnight, fb!

  4. Thank you Jesse for including my Angel Cove Shop in your blog post. I am so honored to be your friend and we will most definitely *have to* get together one of these days real soon. Beautiful and I have a lot to talk about!

    One correction – Greg celebrated his 6th transplant anniversary this Fall. One day we will celebrate his 50th anniversary. ๐Ÿ˜‰

    Of course, I can’t just end there as I have some insight on the “Cons of Transplants”:

    1) When you get to the point of needing a transplant, there is no fear of making it through alive. You know the alternative is death so you openly choose life – no matter what happens.

    2) Fear of rejection will bring you rejection. Greg doesn’t think about his CF or his bilateral lung transplant. Yes, he takes pills but he only sees the doctor once a year and lives a normal life.

    3) Fear of infection…. see #2. ๐Ÿ™‚

    4) Yes, the clinic demands are high in the beginning but the feeling you get when you finally break free is like nothing else in this world. As Greg always said – you are just trading one disease for another (as far as clinics & pills) but this disease you have a chance at life.

    5) Restricted diet? Ha! Except for the sugar and sodium, Greg eats whatever he wants. Sure, he gains weight more easily now that his metabolism is lower (cause his lungs aren’t working as hard) but you should see the boy eat!

    6) Screw the statistics! When Greg got his lungs, they said 11 years life expectancy. I wrote this on my Angel Cove blog and was contacted by MANY double lung transplant recipients well over 11 years out. Greg was also told he would hit his peak lung function at 6-months out (which was 60%) and he is now at 94% 6 years later! And lastly, they told him he would definitely have some form of rejection in the first 6 months. Well, 6 years later and not a single hint of rejection or infection!

    It’s truly mind over matter. We listen to the doctors but never take what they say to heart. If we did, Greg would have died in 2002 when he was on life support for 9 months and his kidneys were failing. Doctors know nothing when it comes to the strength of a CFer. ๐Ÿ™‚

    Stay strong and let Beautiful know that if she ever has any questions or needs to talk, I’m just a Skype away.

    • Six years?! MY bad. Well, I’m sure it seems like 10 years for you sometimes, since it doesn’t feel like I graduated this year. Re: #1 – Yeah, I’m sure you get to that point. We had a huge hurdle just having my sinus surgery, since anything is risky. I didn’t want to kick off just because of my sinuses, but being on life support pretty much takes care of the fear, no doubt! For me, the restricted diet will be hard, since I really like my salty foods, unless the cravings fade without such a need for salt.

      I’ll be sure to bring Beautiful when I make my trip up to WI to visit Phil and a few people in the area and down in Chicago. I have enough clients up there to make a trip worthwhile, plus my best friend from high school is a lawyer in Milwaukee.

      Thanks for reading, and being a friend, as always!

  5. I’m sure you remember it Jesse, because I told you at least 15 years ago and a few times since, but I don’t think many of your readers have heard it. You still might outlive your parents. You just never know.

    And while parents don’t “need” their kids the way they need each other, Rachel’s death left a hole that I still feel and I think your mom feels it even more. Next summer will be 30 years ago. But you’re right that it doesn’t compare to how bad it would feel to be widowed.

    Good job on explaining your position.

    • I’ve seen the hole on Facebook with several people this year, while tragic, I have also seen the widows – there is a difference, but it can be hard to put your finger on it.

  6. I am so glad I have found you and your thinking. Unfortunetly there is so much negative out there and I was “sucked in” to that negative thinking and was honestly juts waiting for Kaleb to die and or need a transplant. There is a fine line between denial and hope and for a while when Kaleb was first diagnosed I was in denial. I would ignore reality and not think about the disease and all that it entails, I then swung to the other side of the spectrum where I was bascially holding my breath and my thinking was “what’s the point, he is going to die and or need a transplant in a few years anyways”. In steps FB and other awesome CF mentors in the community who were LIVING with CF and sharing positive ways to cope and live. With my change in thinking came Kaleb’s change in thinking. I didn’t realize how much my attitude and thinking had rubbed off on Kaleb and we both now have a new perspective. Thank you for your role in that.

    • I never thought about dying as kid his age, but in high school, it was on to college, and career. Real life with the degradation of the things I used to be able to do and the bouts of hemoptysis left me thinking pretty frequently that the end was around the corner.

      I’m glad your new attitude is rubbing off on him. The more I read about Kaleb, the more he reminds me of myself at his age – the blonde hair sure helps with that, too. I hope he’s having a fantastic 10th birthday today.

  7. I saw your little exchange on FB yesterday regarding this topic and I was there with you in spirit. I’ve had less that cordial exchanges with some of our fellow fibros and cysters out there regarding this topic and have decided that I will venture down that road less often. People (including me) tend to mold arguments and “reasons” to fit nicely into the life choices they have made for themselves and…actually, I’m going down that road again, I’ll stop. Point is, I know what you’re saying and I agree with you 100%. Yes, a lung transplant is an option, but it’s absolutely not an option for me, ever (that of course is only my mindset and does not reflect my feelings if I were actually faced with that decision). I will always choose life. I choose life everyday that I wake up, do my treatments, run, exercise and put my family and health first.

    • Yeah, I think I’ll venture down that road less often now, but I also have this post to always refer people to if I start off on a rant or manifesto. I’m glad you were with me in spirit, fibro!

  8. Queenbillabong says:

    I appreciate you taking the time to clear up how you feel on this issue.

    One of the creeds I’ve held since I was a child was “never let what other people tell you lock you into believing it’s true”.

    I have the double delta F508 mutation and was born with meconium ileus too. I spent the first three months of my life in PICU getting fed through tubes in my head, and I’ve had similar issues with my sinuses etc. I’ve had 7 surgeries on my nose throughout the course of my life! I was diagnosed with a virulent strain of Cepacia when I was 14 and told that I’d be dead within 18 months. I refused to believe it, and managed to live for another 13 years culturing a Cepacia that doctors couldn’t believe hadn’t killed me yet.
    I grew up swimming competitively, dancing for several hours a day, doing sports and running my own design business from the age of 15. I never listened to what the status quo was, and always decided that I would decide how my life would turn out.
    In fact, I went to university and did a degree in Opera Performance and have sung in many huge venues in front of massive audiences, with famous opera singers. Everyone around me always expressed amazement that I had these horrible lungs (with a major asthma component and recurring hemoptysis as well) and that I could sing massive opera arias. I never thought anything was abnormal about it because that’s just how I am.
    I live my life without limits.
    While I was doing this I was training for and running in marathons and triathlons (which made keeping my weight up additionally difficult). I was a huge proponent of exercise as the most effective form of therapy for CF (and still am). I was 22 when I met my husband, and meeting him was the best thing that could have happened to me. I gained 15lbs just in the first six months of being with him, and when we got married it was the same as your situation. I just did so very well, and he’s truly the best husband a CFer could have. He saw firsthand what having CF might do to a person as he witnessed the death of my 18-year-old brother from CF, and he still decided he wanted to spend his life with me.
    My downturn started when my cepacia became too difficult to control, and one bad flu and a few broken ribs was enough to start the dominos going. I found myself on 5L of oxygen, unable to do the things I loved or even take care of myself anymore, which was a massive, massive blow to my very being. I tried and tried to recover – I did everything I possibly could – and felt that I was making progress, but then a huge bleed nearly did me in.
    I never treated transplant as a therapeutic option because I’d always been told that a transplant was even possible for me because my cepacia. In Canada they only do cepacia transplants in Toronto, and not usually with my strain. When the team approached me offering to transplant me in Vancouver where I lived, I said yes. It was not a difficult choice. It was time. I had a husband and five pets, and a huge family, and hundreds of amazing friends to stick around for.

    I was on the list for 9 months, as they were waiting for the best lungs for me. They were amazed that I could hold out so long, but I was praying for the perfect set. I finally got the call and my surgery lasted 13 hours because there was so much horrible scarring. I had complications after my surgery that led to all of my organs shutting down, and my cepacia had gone systemic.
    There was no possible way I should have survived that. My life was on a dime edge, I was in critical condition for 2 weeks, yet somehow, my innate will to live got me through. I was in the ICU for 6 weeks, and in the hospital for 3 months recovering.

    Now, it will be 2 years out on January 23rd, and I haven’t had a single infection, a single fever, no rejection, no problems with anything at all. I’ve gained 30lbs, my pre-diabetes is gone, my sinuses are fine, my gut is almost normal. The only thing I can’t eat is grapefruit, and I see my clinic three times a year. Nothing else has changed except that I sleep soundly all night, I never, ever cough, I have more energy than ever before, and I look not only like a normal healthy person, but a strong, fit, vibrant healthy person.

    Now, my approach to transplant is the same as to my CF, even though I’ve traded diseases. I refuse to allow statistics to guide my life. I live now as though this is just the beginning of a looooong life. I have no fear of anything anymore, because I made it through something far worse. Even though it’s with transplant now instead of CF, I believe, like you do, that every person is different, that every case is unique and that there is no reason to assume a pre-set way of thinking about anything.

    Sorry for the essay here but I wanted to give you my point of view. I’m glad we see eye to eye and that you accepted my apology. I think you kick ass, brother.

    • Wow! One miracle after another. You remind me quite a bit of Jesse. Thanks for sharing your story.

    • Awesomeeeee… I’ve heard the trading diseases analogy from several people. I’m glad you got help despite the Cepacia b/c I hear there are only a couple of places here that will consider that, too.

  9. I understand where you are coming from Jesse and totally agree with you 100%. It has been nearly 20 years that we have been living with CF in our lives and we have seen so many improvements with the medicines and techniques that are used to treat CF, and year on year we have also seen because of these improvements, the average life expectancy increase. I think in all that time we as a family have tried to be as positive as we can with the girls and be compliant with treatments.

    This brings me to your fight to Change The Standard Of Thinking, if parents of new born/younger CFers put this into practice then surely that generation will grow up with the right positive mental attitude towards dealing with CF and keeping as healthy as they can. I support your fight and campaign 100%. Through the contact we have had through the internet with so many older CFers, like yourself, ๐Ÿ™‚ it has certainly helped me as a parent to come to terms with and understand the next stage in Lauren’s life, and in turn that will also help us to help Robyn when she reaches adulthood.

    • Thanks. Your support rocks my socks. It is disturbing that so many people with young kids or babies on Facebook are freaking out instead of getting educated. I’d bet that their doctors gave them grim news and they often get that in mind before going to a CF center and getting the real story, which may still be very tainted, depending on who is educating them.

  10. I think part of the problem, is that – at least for me – when I approached getting on the list, and then when I had to get on it, after 29 years of an active life, I felt like a failure. So, sometimes I think people read their own issues into articles, and take what you’re saying about yourself, to apply to themselves, and then they get sad which turns into angry.

    • Great insight. I know I’d have felt like a failure being on the list after
      the wedding. Now I know I’m doing everything and can’t look back on being a
      idiot before.