Pushing Forward

I started this blog for two reasons:

1) To keep family and friends abreast of my ever changing medical situation
2) To give some insight to the mechanics and practical issues surrounding chronic disease and specifically HCM as it relates to heart transplants.

When nothing much was happening last year, the blog drifted into political/religious commentary, which I resolve to keep elsewhere from now on, in view of keeping things focused on the above.

The second reason is, I think, hugely important, because when I myself try to find such information online it is all but non-existent. It's such a rare thing to need transplantation for HCM that it's barely worth the time to write about it. Even the HCM forums only have a handful of members that post in the transplant section.

With that in mind, I need to catch everyone up on what is happening. We were in a holding pattern for quite a while an I haven't explained why or what is happening now. There's a lot happening now and I hope to chronicle as much as I can here over the next months and possibly years.

Boston:

Lots of confusion about why we're not in Boston, or at least in Massachusettss. Didn't we spend four years trying to get there? Isn't that where the best HCM doctor in the country practices? Don't they have the best seafood the world over?

The answer to all of those questions is yes, but we ran into a huge snafu this time last year (Valentine's Day of 2013, to be exact).

We'd found it curious that my HCM doc, Dr. Maron, had passed us over to Dr. Keirnan, a transplant surgeon over the course of 2012. He still consulted on my case, but we were seeing less and less of him. He had told us to expect this, but we weren't nearly as comfortable with the new guy as we wanted to be and we didn't trust him like we did Maron. But he was smart and confident, so we pressed on with him at the helm.

As 2012 passed, I began to notice a shift in priority. My calls, previously answered personally by Dr. Maron or Dr. Keirnan, were now being passed off to a Fellow or Resident who was not only less familiar with my unique case, but not as versed in HCM. Where I used to speak personally with my transplant coordinator (Heather Cote'), I found myself in an endless voice-mail cycle with one of her assistants. We weren't sure what to make of this at first, but it all became clear on Valentine's Day.

Christie and I sat in Dr. Keirnan's office at Tufts in Boston. We'd driven the hour and a half from Springfield and it was early in the day. The boys were on their laptops in the waiting room, under the watchful eye of Rhonda and Peg, the receptionists who'd offered to adopt them several times. This was a common scenario for us - we did it about every 5-6 weeks over the course of 2012.

"Here's where we're at," Dr. Keirnan said. I immediately knew something was wrong because he doesn't look me in the eye when delivering bad news. "The list here has really filled up. We have people with IA status (the highest priority) that have been living in the cardio ward for almost thirteen months, waiting on a heart. With your blood type and unique situation with HCM, it's going to be nearly impossible for you to move up the list any time soon."

We knew this would be the case no matter where I was listed. We had come to Tufts because I needed Dr. Maron to keep me alive until I could get a heart, and there are few docs in the country who have the experience with HCM to do that. It was the primary reason we left Arkansas and set out to find better medical care. Where was this going?

"You're a traveling nurse, right? You guys are still mobile?" he asked.

It was then I realized what was happening. He was going to tell us to go elsewhere, to get listed somewhere else. Suddenly, the lackluster communication made sense. They were so busy with all of these IA patients, there wasn't a lot that could be done to help someone who had no chance of getting heart in the foreseeable future. That may seem harsh but it's the practical side of transplant care.

I know the docs can't control what happens with the list, but at that moment I remember a sinking feeling of realization. Despite the knowledge that we had relocated to get as close to Boston as possible, no one really understood the magnitude of what we had done over the past three years. We had moved a total of seven times in that period, trying to find a job for Christie that would work with our limitations. Somewhere affordable, where the boys could be happy, despite having left all their friends and family back in Arkansas. I estimate that from the time we were told I needed a transplant, we have spent well over $30,000 on moving, travel, hospital, and food expenses alone. That's painful for a single income/disability household with prescription and co-pay expenses added. Christie's parents had just transported all of our household furniture and belongings to us from storage in Arkansas only three months prior. We put all our eggs into this basket called Boston, and now we were being turned away. I've never felt as exhausted as I did at that moment.

"The benefit of being here, with Dr. Maron for your HCM, is severaly diminished by the length of time you'll be waiting for a heart. He's of no use to you if you have to wait three years. You'd be better off with someone who knows less about HCM, but at a facility that has a significantly shorter wait time. Plus, your HCM isn't as big of a factor as we thought it might be back when it was progressing. Now your problem is more typical of heart failure and you'd do well to go where you can be transplanted quickly."

He recommended centers in North Carolina, Arizona, and California, but we weren't even ready to consider those yet. We ate lunch at a local oyster bar in a stupor.  On the ride home, it gave way to a sort of relief. We knew we'd never be able to afford to live in Boston. Springfield was too far away to work realistically for us when the time came for me to have extended hospital stays an hour and a half away. Dr. Keirnan noted all of this in our conversation. We only wish the conversation had taken place back in September, before Christie had signed a year long contract with her employer and me a year long lease with our landlord. Before we'd bought a dog (which makes finding rentals nearly impossible), and before the boys had gotten too involved in archery and fencing in Springfield. This was yet again another tearing away, the leaving behind of something familiar and secure. Brennan sobbed for a long while when we discussed the situation with the boys. He was so tired of moving around and not being able to make friends. Problem was, the boys hadn't made many friends in Springfield and neither had our family. We tried to hook up with some other homeschool families but they were boring and we didn't hit it off. The boys were spending the majority of their day playing Minecraft while I secluded myself away watching TV and writing. I sat in with a local band for a few months, but other than that, none of us had any social life to speak of. In retrospect, I think I was depressed, not liking our new surroundings very much, but resigned to cope and be happy because we really had no other choice. I have very few fond memories of Springfield.

The moment we got home, Christie (as always) jumped into action. She went to the UNOS (organ procurement) database online and started crunching numbers. After a few hours and a lot of re-checking, it became clear that the best move for us would be to go back to Hershey, PA, where our first travel contract had been. Penn State had, unbeknownst to us, transplanted nearly as many HCM patients over the last five years as Tufts. Their success rate was good, their wait list shorter, even for my blood type, and we already knew the area. We'd found kindred spirits in our friends The Foleys, and had remained active in the INCH homeschooling group even when we lived in other places. Hershey (and the small towns surrounding it - Lebanon, Anneville, Cleona, Palmyra) was like a second home to us and always our first choice for a weekend trip or getaway.

Over the course of the next few days I learned that Dr. Popjes at Penn State specialized in HCM and had family members with the disease. I was able to speak to him and one of his patients awaiting transplant at the time. Though Penn State isn't listed as an HCM center, my conversations convinced me that they should be.

February to July was spent house-hunting, first for rentals, then to buy. We made at least five trips to PA in that time, Christie took a position back at Penn State under the same manager she'd worked under in 2011. We found a rustic little Cape Cod style bungalow that fit us perfectly, and after struggling through the loan process, moved in on July 5th.

Now that I'm listed at Penn State, only thirty minutes away, I am admitted to the hospital for a week at a time every six weeks or so. During that time I have a swan catheter inserted into my neck and down into my heart for monitoring purposes, and am placed on inotropic drugs and IV diuretics. This allows me to accumulate time as a IA patient, but is also intended to help control the amount of fluid I'm retaining (thus sparing my lungs), and to increase my cardiac output. The first time (in November of 2013) it worked great. Not so much this last January. I go in again in this Friday and we'll see what happens.

In my next post I intend to give some details on the whole swan catheter procedure and what it's like to spend the week in the hospital with it sticking out of my neck. I'm constantly amazed at the great care I receive at Penn State, if not by the food. Hopefully it takes some of the mystery out of what's happening with me while I'm there.