Wednesday, March 23, 2011

The real story: Pulmonary Fibrosis and the Cleveland Clinic

I've been beating around the bush for a while with the back story of the situation behind a lot of the stress that we've been feeling. Well, here it is. Get comfortable.

My dad is sick. Not like 'I'm not feeling so well, so I think I'll stay home today' sick. Like, idiopathic pulmonary fibrosis sick. The short version of that is that his lung tissue has been turning into scar tissue over the course of the past couple of years which has made breathing and all of the activities associated with breathing (like continuing to breathe) much more difficult for him. Oh, and they have no idea what causes it, how to stop it, or how to treat and/or cure it.

When he got his initial diagnosis of this fine mess back in January 2009 we didn't quite know what to make of it. We knew that he was having a harder time doing the things that he used to enjoy, and over the past couple of years the list of activities that were being compromised grew and grew. After our fabulous vacation this summer (which we are soooo lucky to have been able to take), he had a lung biopsy in order to confirm the idiopathic nature of his disease. It was confirmed and no one had any damned clue why this was happening to him. He's never smoked, he's never abused drugs (or even taken any, to my knowledge), he's never worked in an industry where he would have been exposed to any fungi or other harmful inhaled things.

We knew, when we all started reading about this, that the only ultimate treatment option was a transplant. That seemed so science-fiction and surreal at the time. Two years ago we had no idea how quickly the disease would progress and how the rest of him would fare throughout this process. Every patient has a different experience. Some people decline, then plateau, and remain on that plateau for years and years and years. We're guessing that this was what happened with Dad, too, but he started falling off the end of that plateau in August.

We have been so fortunate and my dad has advocated for himself so well throughout this whole process, despite the fact that it has been so difficult. He tracked down the best pulmonologists in New Hampshire and a pulmonologist for himself at the Cleveland Clinic who is quite experienced with this disease and works with a team of lung transplant doctors. My uncle and cousin were instrumental in getting him an appointment with this doctor and his team, and my dad started visiting them over a year ago. He entered into the clinical trials that they asked him to be a part of and he's followed his doctors' orders to the letter.

Enter this past week. Tuesday, after visiting with my folks at their house, Matt and I went home and discussed that Dear Old Dad didn't seem to be doing so well. I cried. Finally. And a lot. Saturday morning, I got a tearful call from my mom saying that Dad wasn't feeling so well and wanted to go to the hospital. So, we called for an ambulance transport and he was taken to a local hospital where he was monitored and treated as best as they could treat him. When we checked in, the admitting doctor in the ER seemed to think that we were full of crap when we told him about Dad's disease and that he needed to be transferred to the Cleveland Clinic and to please call his doctors there. Of course it was the weekend, and things happen so slowly on the weekends.

So, they kept him for the weekend and treated him as best as they could. We said, on Sunday, that the best thing that could happen for him would be for him to be transferred to Cleveland on Monday, be qualified and on the list for a lung transplant by the end of the week, and [although we would feel terrible for the family who had tragically lost their otherwise healthy 40-something 6' something male in a car accident] he would have a new lung or two in a couple of weeks.

Monday morning, the people in New Hampshire started talking to the people in Cleveland, and by 1:00 that afternoon, his transfer had been approved. My mom decided that she would be more of a hindrance than a help in the private jet that they were sending, so she put me down as his travelling companion. I bolted to the hospital after school (with the clothes on my back, and the crap in my purse) for my journey to Cleveland while Matt picked up Joseph, packed for all 3 of us, picked up and delivered the dog and cat at my folks' house, and hopped in the car with my mom.

I can't even begin to express my awe and appreciation of these rock star EMTs. They rolled into this little hospital in NH in their big black 'Critical Care Transport: Cleveland Clinic' jumpsuits and their reflective armbands and all that stuff looking like they were ready to take over. Which is exactly what they did. They spent an hour stabilizing Dad on their machinery and loading him onto their equipment. We had an ambulance transport to the local airport where a private jet was waiting for us. They strapped him in there, and we were on our way. This was the smallest plane that I have ever been on or will probably ever fly on in my entire life. There were four seats. I took a picture while they were finegaling Dad into the plane. It's a crappy picture and it's on my phone, but it was an incredible process. Joe and Jonathan were our transport EMTs and they were absolutely amazing. I fell in love immediately. They watched him constantly and were tweaking this knob and fixing that dial throughout the entire hour and a half flight to make sure that Dad stayed comfortable and safe.

When we got to Cleveland, another ambulance was waiting for us, and he got admitted to the heart/lung transplant wing. This was midnight on Monday. After the transfer, we were all absolutely wiped, and his doctors decided that he needed to be monitored more closely to make sure that he could stabilize faster and more safely, so he was moved to the ICU. He landed in the ICU at about 2:30 Tuesday morning, and he's been there since then. He seems to be much more comfortable here, and it seems that he's bounced back from the stress and turmoil from the transfer. His color is back, his mood is great, and he's anxious to move on to the next step.

We've been chilling in the family waiting room for his group of ICU beds for the past three days while Dad gets poked and prodded and consulted and met. He's had a pretty consistent parade of doctors, nurses, therapists, social workers, and all kinds of people since he's been here. Our consistent goal has been to get him tested as quickly as possible so that his case can be reviewed by the lung transplant team and hopefully approved as soon as possible. We've learned that the team meets Monday mornings at 7am, and we despirately want him to be on the next meeting agenda. We've been interviewed by the social workers and she has filed her report, which we understand was favorable. They know that he has a strong support network that will help him as much as possible post-op. He's having a heart catheter this very moment, then the cardiologist will weigh in with his/her opinions about the strength of his heart to withstand the operation. Depending on how that procedure fares, he'll meet with either the cardio-thoracic surgeon, or the thoracic surgeon probably (hopefully) tomorrow. These are the last major hurdles for his eligibility.

We've all been kind of on edge the past few days, and are starting to realize the intensity of this marathon. We know that even if he qualifies, there is no guarantee that an appropriate match will be found. But, we've got to take things one step at a time, and we are truly doing everything that we can for him. I know that there are many more pieces to this puzzle, and I am happy to answer any questions that I can, so please, leave a question and ask. If we can help anyone else who may be going through this process also, we would be happy to do exactly that.

Don't forget to catch the rest of the story.


  1. Krones-
    Sorry to hear that you're going through all this crap. I'm hoping for the best possible outcome for your dad.

  2. I'm thinking of you and your entire family through this whole dang difficulty!

    Although not the same, I just remember how anxious I was when Jason was in the ICU for all that time he was sick and I know how incredibly difficult it can be! I understand the sitting and waiting and not knowing and the endless parade of doctors that you aren't quite sure what they're for!

    Seriously, email or FB me if you need to talk - no matter what time of day! Love you!

  3. Hey Allison, Matt, Joseph, Joann and of course Mark. Anything you guys need, please let us know, otherwise, prayers are offered up daily for all and that this transplant moves along sooner than later. Thanks for sharing your information with us, it helps it all to make a little more sense how quickly this has seemed to move along. Hang in there!!!

  4. I'm so sorry to hear about your dad, I hope things turn out well!

  5. Thanks for the detailed update I truly am praying for Mark, and all of you now. JoAnn said she needs all denominations so this crazy pagan is praying.
    I am so glad she has a daughter/friend like you now to walk with her through this time.
    Let us know at anytime if the MHS art team can do anything for you.

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