How Are You Doing? (Redux - The Morning Edition)

Much of my little corner of the web has been used to explain UNOS policies to the uninitiated. Patients like myself have a strong love/hate relationship with the United Network For Organ Sharing. On the one hand, they keep organ transplants and donations from devolving into an income based system whereby the wealthiest or most influential are able to buy organs out from under patients in greater need (with the exception of Dick Cheney who was able to procure the heart of Satan himself without contest, because no one else wanted it).

On the other hand, this leveling of the playing field, as it's viewed, works for most people, while leaving those with less common types of heart failure, such as HCM, on very uneven footing. Thier policies accommodate general congestive heart failure very well. But HCM patients in need of a heart are forced to undergo treatments and drug therapies that are not only un-needed, but often contra-indicated, so that they can meet the UNOS criteria for transplant. We've been jumping through those hoops for four years and this has been without question the single most challenging and frustrating part of this whole journey. It's forced our family to re-locate numerous times, not to mention forced us to spend time apart from one another - the last thing a family wants to do when there's a risky surgery on the horizon to begin with.

My current situation is a direct result of ill-fitting and short-sighted UNOS policies. I've been implanted with an artificial heart and placed on a portable driver for the express purpose of being able to live as normal a life as possible at home with my family while awaiting transplant. Yet, because of UNOS policies, my place on the waiting list drops down into the hundreds when I go home (what's currently known as a Status 2 listing). As long I stay admitted to the hospital, I am listed as a 1A patient, and my name rides the very top of the waiting list for O+ hearts. Due to my accumulated time here as a 1A for the last 2.5 years (spending 2 weeks at a time every 6 weeks with a swans cath on IV drugs I didn't need), my name shows up a lot when the facility gets offers - though so far, none of them have been good. So regardless of the fact that I would be safer from infection at home, as well as more closely watched and listened to (in case something went wrong with my heart driver), here I sit, an otherwise healthy, ambulatory, independent 45 year old with a family that can only be together about twice a week. The fact that I literally have no heart is irrelevant to UNOS. To their 1+1=3 way of thinking, I'm healthy enough to live at home, therefore, my need is less urgent than someone living in the hospital. Period. Though there is some recognition on the part of UNOS that this makes no sense, a forth-coming policy change from UNOS is about as likely as Sarah Palin being able to string two coherent sentences together in the English language. So it's about saying NO MORE because they don't want us to know with the people because hearts being needed for some who just can't handle the truth for all the sick, flick your bic, is that an icepick, dontcha know?

Huh?

It's really hard to answer the question, "So, how are you doing?" It's quite a loaded question. You can imagine the abiding frustration of the situation, just because of this whole UNOS inanity. But living in an ICU ward as an independent, functioning, technically "non-sick" person is enough to drive anyone mad after a time. I've tried to think of the best way to describe what it's like, but if you've never been hospitalized for any length of time, I imagine it's difficult to grasp. Let me relate this to normal life as much as possible, and maybe it will provide some insight.

It's imperative to me that my readers understand that I'm relaying all of the information on this blog as honestly as I can because I know that one of you might experience some of this in the future. That said, it reads like I'm whining. Which is the last thing I want to do, or be perceived as doing. The situation I'm in obviously elicits pity and sympathy from most people. I understand that. But in turn, please understand that I don't share these things for the sake of pity and sympathy. I'm a pragmatic person - pity and sympathy buy me nothing, and do nothing to change the situation. Therefore, I have no motive for complaining and whining to elicit these expressions of pity from anyone. I hope the relay of my experiences to the reader is received as intended - information, honest thoughts, reality.

Ready?

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You awake in your bed to find someone drawing blood from your left arm (now, I have a permanent line they can draw from, so I don't actually get a needle every morning). This person will be your escort today. You may not even know their name yet, maybe you've never seen them before in your life. You will interact with them roughly once per hour, and they will accompany you everywhere you go. You didn't pick them. They may not even be a nice or interesting person, certainly not someone you would seek a friendship with. Nevertheless, you're stuck with them  for the next 12 hours, at which point they will be replaced by someone else you may or may not know. Rinse and repeat.

As you come to your senses - possibly from REM sleep because you stayed up late the night before - another group of 4-6 people enter your bedroom, a mix of acquaintances and strangers. One carries a small netbook. One of them begins peppering you with questions - how are you feeling? What was your weight yesterday? Did you poop? You don't wear pajamas to sleep but try to keep yourself covered the best you can as this person squeezes your ankles and wants a detailed look at your stomach (where my drive lines are), all in view of the group. Any response you give is quickly recorded by the guy with the netbook. This is all WHILE you're still laying in bed. Before coffee. Before you've had a chance to pee. The only way around this is to put a sign on your bedroom door warning everyone not to disturb you before such-and-such o'clock, and hope they honor the request. Once they leave, your escort wheels a huge scale into your room. Don't like being in your underwear in front of strangers? Sorry - you need to step on with as little clothing as possible. You can buy your own scale for your room, but you still have to weigh on this one at least once a week. Because UNOS.

This scenario potentially happens every morning - 7 days a week, but here's the catch. Those acquaintances? You never really get to know them, and you never get used to it because it's a different group of people each morning, save one or two. Good morning, sunshine!

You pee in a jug that you'll keep for your escort because they need to measure how much you do that each day. So yeah, you'll be peeing in that same jug all day, every day, and they'll record it each time. Then you take a shower - wait no. You can't take a shower. Let's say your shower is broken indefinitely - you have to bathe and wash your hair using the sink (I haven't really showered in 9 months because I can't get the PIC line in my arm, or the drive lines in my stomach wet). This kind of works, but you still feel manky and try to deal with it using powder and lotion. You're not done yet -  take your own temperature and blood pressure, or let your escort do it. There are three numbers on the TAH read-out that have to be recorded. You'll be doing this roughly every four hours every day, whether you're in a meeting, the middle of a meal, television show, meal, phone conversation, while your eating, taking a nap, or mostly while you're trying to eat. Even though these numbers have been the same for four months, every single time, every four hours, for four months, you still have to do this. Because UNOS.

Time for first breakfast! A cocktail of nine pills, (most of them smell like roadkill) with water to drink. These will make you pee more, a real treat with the measuring jug (be sure to ask for hand sanitizer). Hungry yet? Yeah, you're starving. Time for second breakfast! You can have a tray delivered to your room. This morning it's scrambled liquid egg with a few chopped red peppers thrown in, diced potatoes, a fruit cup (like the kind you used to eat in third grade), a cardboard carton of skim milk (because whole milk apparently kills people instantly), and some unidentifiable liquid they claim is coffee. By the way, there is no seasoning on any of this - no salt, no pepper, no butter. You receive a small packet of pepper and Mrs. Dash. The meal smells like something the Donner party wouldn't touch. Be grateful - tomorrow is turkey bacon (last used to change the oil in someone's car) and microwave pancakes. This is breakfast. Every single morning. Unless you order something different. Kellogg's cereal? Fatty sausage patties with starchy biscuits? A fruit cup? (be sure to check each piece for bad spots, there will be many. And they're hard to see because by "fruit cup" I mean day old honeydew melon cubes).

But there's hope! Since you can walk, you could go down to your kitchen and choose your own breakfast. You're starving, but your escort has to repeat your morning ritual with one to two other people who can't move around as easily - they need help getting to the bathroom, and opening the little juice cups, cutting their pancakes, etc. So you wait. Sometimes you wait longer. Finally, your well-meaning and over-worked escort walks you down to your kitchen. You find pretty much the same options as offered on the tray. You're trying to eat as healthy as possible so if you manage to avoid the turkey bacon and greasy gravy and biscuits, you could have an omelet made from the chemical egg juice. Or maybe the yogurt. Yogurt's good for you, right? Which flavor would you like? Scooby-Doo Raspberry? Dulce de Leche with chocolate sprinkles? Ooo! How about Ghostbuster green apple! Where's the normal, healthy, unflavored yogurt, you ask. No one knows what you're talking about. There's desiccated fruit, or oatmeal. You could eat the oatmeal - that's good for you. Hope you like it A LOT because if you're going to eat healthy - it's oatmeal 7 days a week for you. Guess how quickly you'll get tired of oatmeal? I don't even want to type the word again.

You'll repeat this scenario at every meal, but also be aware that you can only eat $10 worth of food at each meal. Ever eaten anywhere but McDonald's or Taco Bell for $10? Keep in mind that whatever you choose, your escort will take note, because they are charged with your overall well-being. You do the best you can, but it's far from ideal nutrition (more on this later). You have to take the food back to your bedroom to eat (let's say for the sake of the example you live in a large house). By the time you get back, the food is cold. You can microwave it in a room down the hall, but some of the other people living in your house don't want you going in there - you have to get your escort to do it for you. Hopefully she doesn't get called away while you're waiting. Getting breakfast, even if everything goes smoothly, takes about 15-20 minutes front to back. Hope you're not a person who wakes up hungry, like me. It's been over an hour since you were woken up. You reflect on how your life has come to a point where you can't even control when you eat your own meals.

You finally sit down with your food, and another group enters your bedroom. I deliberately use the word "entered" because they didn't knock, they just entered. They want to ask you questions about the quality of your room, the color of the drapes, the friendliness of the acquaintances who wake you up each morning, etc. Because you're fool enough to believe that describing your morning to them will somehow make a difference in mornings to come, you actually answer their questions. They don't write any of this down, as your food gets cold once again. They thank you for your feedback, leave the room, clock out, go home, and don't remember your name or anything you said by the next day.

But they'll want to come again - maybe next week, maybe the week after. They have to do this because it's their job. But they don't actually do anything to address the problems you have. In the end, it's really nothing more than an imposition and a waste of your time, but they must do it - if they don't, the property value of the house drops (Google: Healthcare Facilities re: Magnet Status). They need to keep that from happening, without really improving the quality or living conditions of the house. You're just a tool to help them do that. You can (as I have) put another sign on the door informing all visitors that they need permission from your escort before they can enter your room. You quickly become astounded at the illiteracy rate of the people living in your house. Now - make sure that each day, you point this sign out to your escort (because it may be a new person every day) so they will keep these intrusions to a minimum. But remember there will be times in the privacy of your bedroom when you're changing clothes, peeing in your jug, having a private or intense conversation with a loved one (you may die tomorrow, by the way, so understand my full-meaning when I use the adjective "intense"), or just having a good cry. If your escort is away helping one of their other charges, not watching your room, people will just (to be continued tomorrow)

The Story So Far

I know there are some new readers (HCMA Holla!) and there are a lot of old posts to wade through. It's probably an opportune moment for a timeline, while new readers catch up on old posts. When I say "timeline" it likely conjures up images a nice, tidy, short, linear graphic with three word phrases at various intervals to denote significant events. My timeline is more like a drawing scrawled in crayon by a blind meth-fiend struggling to find his seat-belt in the middle of an ill-advised roller coaster ride. Shall we begin?

May, 1990, Memphis, TN, 19 yrs. old - Struggling through a music performance degree, I awake in my dorm room one morning to intense chest pain, nausea, and disorientation. I had to skip my morning class, but knowing I was going to vomit, literally crawled down the long hallway to the dorm bathroom. I vomit twice on the way, and uncontrollably once I reach a toilet. I have no idea what's happening. My roommate all but carries me to the college clinic where a nurse (without a any testing) diagnoses me with mononucleosis. I don't leave the bed for three days except for the bathroom. My roommate graciously brings me soup from the cafeteria and I effectively manage to fail my most important class - music theory, by missing nearly two weeks of class including the review for the final. I know now that I had no signs of mono, but it was going around and the nurse made a lazy diagnosis. It was the first progression of my HCM, but with no family history, heart disease is the furthest thing from my 19 year old mind. The HCM goes back into hiding. I drop out of college and return home to Little Rock where I get married and spend the next eight years building a life and business, and playing music anywhere and anytime I can.

Spring 1998, Little Rock, AR, 27 yrs. old - After a nine month hell of exams, tests, and unnecessary medications administered by ENT's, Pulmonologists, Endocrinologists, Gastroenterologists, Cardiologists, and Neurologists, I been told it's possible I have chronic GURD (true), lung failure, HIV, stomach cancer, Gallstones, and finally "IIHS" - which I learn a few years later is actually called HCM. I'm told I have six months to live if the disease continues to progress. We sell our business at a loss (and subsequently become unemployed), lose our home, and move in with my in-laws, two hours away from all of our current friends and commitments while we circle the wagons and try to figure out how to pay $1100 a month for prescriptions. Being self-employed, our very expensive health insurance didn't cover pharmacy costs - we couldn't afford it. It took two years from this point to obtain Disability Compensation. Over the course of three years, we were able to recover some of our previous life, and though I felt sick most of the time, life returned to as normal as could be expected. By 2001 we'd had a son (after being told we couldn't - and knew that was probably best due to HCM being congenital).

May, 2003 - Little Rock, AR, 32 yrs. old - we are befriended by a young woman at church who it turns out has the same heart disease. "IIHS" "HOCM" we say. "HCM" she says. "Google it." We do. Boom! Google returns links for the HCMA, we meet Lisa Salberg, and eventually fly to the HCM clinic in Boston to meet with Dr. Maron and company. Medications are adjusted. HCM is dormant. Nothing to do now except hope it doesn't ever progress again.

September 2009, Bentonville, AR, 38 yrs. old -  My wife becomes an RN, largely so she can help to navigate this disease that no cardiologist in our part of the country seems to know anything about. Despite a healthy diet and low impact workouts, I start inexplicably gaining weight. By Christmas I'm a full 35 pounds heavier and feeling so awful I can barely get out of bed. By this time we've had our second son (again, after being told we couldn't conceive), who is now 4 yrs. old.

February 2010, Bentonville, AR -  39 yrs. old - My cardiologist recommends that I go back to the HCM clinic in Boston to see Dr. Maron. A test has shown a "shadow" on my heart and he doesn't know what it is. Or does, but doesn't want to tell me. He forwards the result of my recent MRI to the clinic at Tufts.

March 2010, Boston, MA - A  trip to Boston confirms without a doubt that my HCM has progressed aggressively in a matter of months. Dr Maron informs me I'll need a transplant, and worries that if the disease continues to progress at this rate, I'll be dead in six months. I've been unable to find an HCM specialist in Arkansas and trust Dr. Maron to keep me alive until I can get listed and transplanted. We return home, agonizing over how to break this news to parents, in-laws, and most of all our oldest son, Rich, who is only 9 at the time. We'd told them all before the trip that we planned to look at apartments in Boston but what was intended as a casual info gathering excursion suddenly turns into an exhausting and frantic two-day search for a place to live so we can relocate as soon as possible.

July 2010, Fayettville, AR - We move to a small apartment in view of having to downsize if we're to survive in the very expensive Boston economy. In the meantime, we find an HCM clinic in St. Louis that may save us from the move to Boston. Nursing wages are abysmal and cost of living is nearly twice our current expenses. Not sure what to do. Phone conversations with heart clinics in Memphis, Dallas, Houston, and Tulsa make it apparent that there is nowhere in reasonable driving distance where I can be successfully listed and eventually transplanted. I'm laughed at by several cardiologists for even suggesting that an HCM patient should be transplanted, despite the glaring evidence of the MRI comparisons and cardiac output numbers from the previous spring. Ignorant morons.

July- November 2010, Fayettville/St. Louis - Five trips to the supposed HCM clinic in St. Louis where the cardiologist tells me I'll never need a transplant, and refuses to even look at the MRI's. The transplant doctor, however, is willing to list me but as the months go by, it becomes clear that she has no plan or idea how to navigate the complicated and restrictive UNOS policies to actually list and transplant an HCM patient. A VO2 test says my heart will survive another five years, which is somewhat meaningless since HCM turns itself on and off of its own volition. Fortunately, mine stops progressing shortly after returning from Boston, buying us an unknown amount of time to figure out what to do. In the end we're out nearly $9,000 over the five months between hotels, food, gas, and lost wages, all for naught. I briefly consider a career in the meth/amphetamine industry after watching too many Breaking Bad episodes. Just kidding. Kind of.

December 2010, Fayetteville - Christie is unable to even apply for an RN job in Boston. There were record-breaking masses of RNs that graduated the previous spring thus flooding the job market. Even schools that had paid for student's education in exchange for 1-2 year employment commitments are now forced to release them from their contracts so they can seek employment in Maine and Rhode Island. Some find jobs, but many remain unemployed.

January 2011, Fayetteville - 40 yrs. old - We decide the only way Christie will ever land a job close enough to Boston is to take a travel position and request assignments in the Northeast U.S. corridor. I can be listed at Tufts if I live within four hours of the facility. There's more than one way to skin a UNOS policy.

February 2011, Hershey, PA - Our first of several three month assignments after tearful goodbyes, moving out of the apartment and storing our furniture during an epic snowstorm (Yes, I drove a 28 ft. U-haul truck in the ice. Yes I'm awesome) - we'd planned to transport it all back to Little Rock, three hours away, and store it near our parents, but the snow forced us into Plan B; store everything in Bentonville, and figure out the rest later. More tearful goodbyes and we were on the road to PA with only what we could fit in our Honda Odyssey. It feels like an adventure, but a forced one. (which, by the way, is also chronicled at our sister blog, The Gypsy Schoolhouse). The company provides a nice furnished apartment and we set about exploring the area, making friends in the local homeschool group, and planning our next move.

June 2011, Danville, PA - After several trips to Tufts in Boston we realize we'll have to move closer to begin the listing process. Travel contracts wax and wane and we're stuck with an assignment three hours north in a dying coal-mining town. The accommodations are miserable but the hospital is great and we can still drive back to Hershey to spend time with friends we've made.

July 2011, Danville, PA - My first experience with A-Fib. Returning home from a trip to Gettysburg, A-Fib hits me like a truck. Christie has already left for work (night shift) and I'm alone with the boys. Phone reception is non-existent in Danville, but luckily, I'm able to Skype my parents who just happen to be visiting on their way through to see friends in northern PA. They rush me to the hospital where I'm cardioverted and sent home the next morning, hoping it won't happen again. Fat chance.

August 2011, Little Rock, AR - A nice contract back near our hometowns where we get to spend the fall and the holidays with family and friends before leaving for the Northwest permanently. My family helps us move our furniture from the emergency storage three hours north in Bentonville, to my in-law's storage building in Searcy, AR, about an hour from Little Rock. It will stay there until we find a permanent home in the Northeast.

January 1st, 2012, Lebanon, NH - 41 yrs. old - After spending a few days with friends near Hershey, we arrive in New Hampshire, hopeful that we'll find a permanent home there. Christie is working on contract at Dartmouth, and Boston is an hour and a half away. Upon arrival we unpack that van and set out for dinner when A-Fib strikes again. We spend the night cramped and exhausted in my tiny ER room, Christie and kids sleeping in metal folding chairs. So far, the worst moment of my entire life. I'm cardioverted the following morning, stuck in a room on the heart ward and forgotten. After threatening to leave AMA, the doctor wants to aggressively diurese me (read: Kill me. Permanently.) because of all the fluid I'm retaining. I refuse and am discharged. I have A-fib three more times over the following weeks with the same results and misery. The next trip to Tufts I'm started on an Amiodarone regimen to control the A-Fib, but we all know it's a band-aid. My heart is failing. I complete the tests for being listed. Now we wait.

April 2012, Norwalk, CT - Having loved our time in NH (A-Fib aside) we take the next contract in CT. Dartmouth offers Christie a full-time job the day before we drive out, but she's already signed the next contract. Bummer. CT turns out to be the most miserable three months we've ever endured. Christie is working 12 hour day shifts for three months with no more than two days in a row off the entire time (we're used to four). We only have the one vehicle, effectively trapping the boys and myself in the apartment every day for three months. We manage a few day trips to New York and I make it back to Boston for a clinic visit, but we never really get to experience or see much in the area - the main thing making all this homelessness tolerable. Great restaurants though. My coordinator calls to inform me that I'm officially listed for transplant. Mixed feelings, unsettling dreams.

July 2012, Springfield, MA - Finally, a contract in Massachusetts, an hour and a half from Boston. Listed. We're not in love with the city, but the job is good and the nursing wage more than balances the increased cost of living. We decide to come off the road when Christie is offered a job at Bay State hospital in Springfield. She signs a year contract, we sign a one year lease on a house, and get the dog we've been promising the boys when we stopped travelling.

October 2012, Springfield, MA - It's nice to feel like we have a "home," though homeschooling is virtually non-existent (MA public schools are the best in the nation). Not making many friends but I join a progressive metal band as the vocalist, mainly for the challenge. We record a five song demo and gain interest from Gene Simmon's (KISS) regional record and distribution label, and get significant regional airplay Christie's parents load up our furniture and bring it to us from Arkansas so we can set up a real household. Still going back and forth to Boston for clinic checkups.

February 2013, Valentine's Day, 42 yrs. old - Springfield/Boston MA - after feeling somewhat sidelined due to my blood type and low priority on the waiting list, I'm informed by my transplant doctor at Tufts that there has been such an increase in need and decrease in donor organs that some people have been living in the heart failure unit for fourteen months or better, and the chances of me moving up the list before I'm in trouble are pretty much zero at this point. But it's okay, he tells us. Since Christie's a travel nurse, I can just get re-listed somewhere else with a sparser population, increasing my chances of getting a heart. I refrain from strangling the man, since we'd made commitments and made our home in Springfield to be near Tufts. He recommends North Carolina (where I wouldn't want to live were it literally the last place in the universe sustaining human life - all due respect to North Carolinians, but surely you've watched the news lately. And by "lately" I mean the last ten years), California, and a few other places where he doesn't know they won't list Hypertrophs (he's a transplant guy, not an HCM guy). We eat lunch at a nearby oyster bar, too stunned to taste the food, and once again wonder what to do. At home we methodically crunch data on the UNOS and OPTN website to find hospitals that have transplanted HCM patients successfully, how often, and how long the wait times average. The obvious choices are at the top of that data filtering - The Cleveland Clinic (owing to the famed Dr. Lever's excellent HCM work there), Tufts, of course, California, etc. About third on the list we're stunned to see Penn State Hershey Medical Center - the very same hospital Christie worked at on our first travel nursing contract. Though not listed with the HCMA, apparently a Dr. Popjes in their cardiac group specializes in Hypertrophy, and even has a family history of the disease himself. It was our last trip to Boston and we calculate that all told we'd spent roughly $20,000 in the preceding year on hotels near Tufts, gas, food, taxis, Charlie T-passes, outrageous parking fees, lost wages, and related expenses. Mostly for naught. Credit cards are maxed out and our youngest, Brennan falls apart when he learns we will have to move yet again.

March 2013, Springfield, MA/Hershey, PA - After some phone calls to our friend Lisa Salberg at the HCMA, and conversations with a few of the HCM patients who'd been treated and transplanted there, we made a trip back to PA to talk to the Dean of the cardiology school, Dr. Davis. He's the most brilliant man I've ever met. We leave the hospital confident that the program there will work because they have a very pro-active plan to allow Hypertrophs equal footing with other transplant candidates, as well as a solid history of treating them while waiting. Between March and July we make three more six hour trips to the Hershey area for doctor appointments, Christie's job interview, and house hunting. We want to rent but find it impossible to obtain something in our price range, close to the hospital that both meets my physical needs and allows the dog that we now have because we thought we were settled. We were able to stay with friends one of those times, but expenses, hotels, and lost wages hover somewhere in the  $3-4000 range. Load up the credit cards again.

Late May, 2013, Hershey, PA - On our second such trip, we find a home we love, with a few fixer-upper issues that we can deal with (Chip? Joanna? New Discover card?). It's perfect for our price range and our family. We make an offer through our realtor and head back to Springfield, confident we've found the right place and relieved that we'll have plenty of time to arrange the move.

Early June, 2013- Springfield, MA - Our realtor calls with bad news. The house, is incorrectly listed as being on the market, but is actually in short sale with a local bank. It could take years for anyone to consider our offer, and just as long to settle, since banks aren't typically in a hurry to sell re-possessed property. We're now in a very stressful position since a) Christie has accepted a job at Hershey Med that she MUST start on July 5th in order to orient on their schedule - or she can't begin until October, b) she's given her resignation at her current job effective on July 3rd, because she can't miss even a week of work - we're broke from all the trips back and forth and need our meager savings for a down payment on a house (which is why we wanted to rent!), and c) our landlord, having graciously released us from our lease agreement due to the circumstance, has a new tenant moving in on July 5th - two days after we're supposed to vacate. We frantically rush back to Hershey and decide to buy the very last house our realtor shows us, but can only spend fifteen minutes looking at it since we have to rush back so Christie doesn't miss work. Oh. And all these trips are happening with a dog who gets car sick just looking at a car. Fun times.

June-July, 2013 - Springfield, MA - Our offer is accepted but our lender, FHA (administrated by Satan, apparently) asks for a long list of unnecessary, ridiculous "repairs" to the house. Between dealing with these and the incompetence of our lending officer, I spend (literally - not figuratively - literally) ten of the next fourteen days on the phone and filling out paperwork from about 9 A.M. to around 7 P.M. Every. Stinking. Day. Remember how stress is really bad for heart failure? Yeah. Me too. After the hell of negotiating "repair" expenses with the seller, scraping and clawing to make the closing and move-in dates work with our incredibly narrow window, and jumping through inane hoops with FHA, we finally get everything settled. Until a week before closing, when FHA announces that they want the entire exterior of the house re-painted. We're quickly approaching July 4th weekend. Another 2-3 days on the phone desperately trying to find a paint crew willing to work that fast and on a weekend. No dice. In much distress, we have to withdraw from the sale, but wait! The seller comes to the rescue, rounds up a bunch of friends and they get the painting done (with interior paint, as we learned after moving in). There were a hundred other twists and turns to this part of the story, but it's boring. I'll just say - live in a cardboard box before taking a home loan with FHA. They will steal your soul. Everything eventually works, we close on the 5th, Christie starts her job, and we hope this is the last time we have to move. We discover several problems with the house that prove extremely expensive to fix, but we're still kind of stuck with it until life returns to normal.

October 2013 - Hershey, PA, - My accumulated time on the list in the Boston zone is transferred to the PA zone. Dr. Popjes launches his plan to get me a fair shot on the waiting list. I'll be admitted for a swans cath and observation every 6 weeks. I'll stay on the HVIC unit for 1-2 weeks at a time, during which I'll be accumulating precious time as a 1A patient, and who knows, maybe something crazy will happen and a heart will become available while I'm there. Since I'm only a status 2 at home, this increases my chances of moving up the list.  I'll have to be placed on inotropic medications via IV - these drugs are contra-indicated for HCM patients, but UNOS will not honor my admission as 1A time unless I do this. It's one of the many nonsensical and dangerous boxes we have to check in order for me to qualify for transplant - but it's one of the things HCM patients have to do since the requirements only accommodate people with more general heart failure (meaning, most people who need transplant). The inotropes make me sick, as expected, and we all hate not being together, but it must be done. A few months later, we meet Perry Jenkins, an 18 year old guy, living in the HVIC with a Total Artificial Heart (TAH) because his HCM progressed to the point that his own heart had to be replaced. We hit it off, spend a lot of time in his room between my admissions cooking, eating, playing board games, watching movies, and so forth. He becomes like a big brother to the boys, and becomes my own personal Yoda.

Late June, 2015 - Hershey PA, 44 yrs. old - After about a year and a half of these hospital stays, my A-Fib returns, as we knew it would. I have two more cardio-versions and an ablation to burn out the nodes in my heart that are causing the electrical issues. It's the second most miserable experience of my life, as I spend the following two weeks sitting in a chair, grasping the arms with white knuckles, wondering if astral projection really works because I would do anything to escape the searing pain in my chest. No amount of pain medicine seems to help but it eventually goes away. During my next swans cath admission, Dr. Silber, one of my cardiologists expresses grave concern about my condition. According to my lab work, my liver and kidneys are on the verge of total failure, and it seems doubtful that the ablation fixed the A-Fib, which will likely pop up again in another spot. He recommends emplacement of a TAH, with transfer to the "Freedom Driver" - a portable pump that will allow me to go home when I'm stable. After long conversations with Perry, Dr. Maron at Tufts, Lisa Salberg at the HCMA (who in turn contacts the Cleveland Clinic), and much research, this seems like my best, and possibly only option to not end up with multiple organ failure and being dropped from the waiting list altogether. I schedule the procedure for August, after Rich's birthday. The deadline for Christie to start back to school for her BSN has arrived. Given the situation, she appeals to everyone possible for an extension until after my transplant. Hershey Med Center denies it and threatens termination if she doesn't honor the start date in her contract. I conclude that the Powers That Be are evil and vindictive and I want to burn the building down. But I have children to think of.

Early July, 2015 - Perry calls at 7 am to say he's getting a heart. We rush to the hospital to see him before the procedure, take the kids back home, and spend the rest of the day in the waiting room. The surgery starts late and goes on far longer than it should. The same surgeon who will be implanting my TAH comes out to inform us that they're still working on him and he is "very sick." Christie and I recognize this as surgeon code for "he may not survive the surgery." Hours later we learn that he's been moved to a room on the HVICU, but there's a team of docs and nurses still working on him. We have to go home, but this apparently goes on until the following day when we return to see the surgeon still making trips back and forth to his room. It seems that there was a very unusual and violent anti-body problem with the blood transfusions he received in surgery. He's in liver, kidney, and lung failure, and we can't learn anything about the health of the new heart, due to HIPPA privacy regulations. Eventually, we get permission from his family to see him, and learn from them that although the heart seems to be fine, he's in grave danger from the other issues. We try to prepare the boys since it's looking more and more like he may not survive. Nightmarish.

August - December, 2015 - Perry wakes up a few weeks before my surgery, but is extremely sick - yellow, atrophied, and fighting for his life. He can't talk much. I undergo heart removal and TAH emplacement, and see him a few times during my record six week recovery. I go home, Perry stays. I have some coagulation issues requiring me to be re-admitted for a week. Back home again, we start to grapple with the problem of being one of the only people in the nation living at home with a TAH, and discover that there's no system in place for such a thing. Christie spends a LOT of time figuring out how and where to do blood draws and dealing with the logistics of (surprise!) 3-4 trips a week to the hospital for coagulation checks, labs, etc. Insurance doesn't cover enough of home health care costs for us to use regularly. Sometimes she works 12 hours overnight, comes home, showers, loads all my backup equipment and drives the 25 minutes back to the hospital (TAH patients aren't allowed to drive). We normally get home around 1pm and she gets a 3-4 hour nap before waking up to eat and go back to work. This goes on for months. my aunt comes to stay for several weeks, but once she goes home, we need an adult in the house while Christie works. A few friends who were trained at the hospital on the pump, volunteer to spend those nights with me and the boys. The help is unbelievably generous and we are humbled. The loss of privacy is unbearable and we are unsure what to do. Conversations about the ramifications of leaving me home overnight with just the boys ensue - they're trained on the equipment, better than anyone, and pay very close attention. I trust them implicitly but lose many nights of sleep agonizing about the option of putting this burden on them. After many tears, much talking, and realizing our kids are way smarter and more practical than Christie and I combined, we decide to give it a shot. It works great - some degree of normalcy returns to the household, the boys and I get our time together back, and we also begin to set up rides to the hospital so Christie can get a reasonable amount of sleep. Perry meanwhile has finished rehab and gone home, only to return to the HVIC the day after Christmas because he feels sick and is coughing up blood.

January 16th, 2016 - 45 yrs. old - I feel great the rest of 2015. We want to move a little further east in the spring or fall, due to Christie possibly taking a much better paying, less stressful job at a hospital in that direction. We learn that Perry has been diagnosed with lymphoma, which spreads aggressively to his lungs and other organs. He's placed on a ventilator and is receiving round the clock blood transfusions.While painting some trim in our sun room the morning of the 16th, stuff happened (detailed in the last post). Two days after admission to the hospital, Christie receives a call offering her the job. She has to turn it down since it will put her nearly two hours from me. Perry finally passes away on January 31st. I think we've yet to fully process his absence. I end up in the room he spent many months in while living here on the HVIC. The lights flicker from time to time, and the motion sensor sink turns itself on occasionally. I think it's him, just messing with me, but by way of revenge I'm finishing off the huge box of Flavor Ice Popsicles he left in the patient freezer. I talk to him a lot some days. I miss him desperately; we all need him so much right now. I shake my fist at the sky.

February-March, 2016 - I didn't mention it in the last post but during my stay, Christie has completed a six week orientation course complete with bi-weekly discussions, papers, reading, etc. I could digress into a diatribe about how the glaring insecurity of nursing unions and organizations, along with the greed of corporate healthcare has culminated in a needy and ill-conceived plan to change society's perception of nursing as a just " a job" to that of a "professional" career field, thus forcing experienced nurses into expensive and unnecessary degree programs that do nothing to solve actual point-of care problems or provide hands-on experience, all for the purpose of obtaining a so called "Magnet" (or some similar) status for the facility in question, which ultimately means jack squat to the patient who just wants an experienced nurse that knows what she's doing regardless of her degree or test scores, instead of one with a doctorate who's too busy trying to finish homework and write papers to give said patient his/her undivided attention, which would actually contribute to greater patient satisfaction and outcomes as well as a more stable and content work environment, thus negating the need causing the insanity I've mentioned above. But I won't get into that here.

April, 2016 - Still waiting.

And that brings us to now. Or rather to the last post. So really, this post goes back while the last one moves forward. Maybe I should change the order. Is there even a way to do that? I'm so confused. Does anyone have a flux capacitor?

So A Few Things Have Happened...

I know I've not posted since Perry passed away. There are several reasons for this.

Firstly, I needed a little time to process, and I was so frustrated that every time I tried to write something, it came out angry and sounding very woe-is-me. This being the antithesis of Perry's personality, I couldn't bring myself to follow up my last entry with such a post.

Secondly, as some of you know, I'm an aspiring writer/novelist (Isn't everyone these days?). In January I received serious interest in one of my manuscripts from a great New York literary agency. They ultimately passed due to timing conflicts with their 2016 list. They, along with another great agency in Conneticut, have asked to see one of my other manuscripts, pending a re-write to expand the length. For those of you who aren't familiar with the publishing world, the process goes like this: 1) spend a long time writing a book you're pretty sure no one will ever read, 2) get it perfect - manuscripts these days have to be near ready to publish when you submit them, 3) find a literary agent who agrees to represent you/your book to the publishing editors with whom they have an established relationship (book publishers/editors no longer accept unsolicited manuscripts - they MUST be submitted through a trusted lit agent) 4) re-write the book to the lit agent's standards, 5) hold your breath while they pitch the book to various publishers until someone buys it, 6) sit back, collect fat bank, and become the next Stephen King. Okay the last one is not true, but writers are usually dreamers as well. I've literally been working on this book pretty much all day every day since January, because as other writers know, once you get any kind of serious interest from a literary agent, you feel like you've won the lottery, and you want to make your book the best it can possibly be so you don't blow the opportunity. So I've purposely set all other projects and blog updates aside to get my book where it needs to be. What book? Which book? you may ask. I'm not telling. You'll have to click the website link in the sidebar and go to my new website to figure it out. No, I'm not above coercing people to create site traffic. Either way, I needed a break from the book today, so I'm composing several blog posts that I'll publish this week to catch everyone up on health stuff.

Thirdly. Health stuff - which will comprise the bulk of this post. Most of you are aware by now that I was admitted to the hospital on January 16th, the day before my birthday. I'd spent the morning touching up some of the paint trim in our sun room - this was not physically intensive labor, just standing, painting small strips of trim with an artist's brush. I'd not felt ill or bad, in fact, I've felt the best I have in years during the months between September and January when I went home with the TAH. As I was painting, the world started shaking up and down - like being on a trampoline. Everything was spinning and I couldn't take a step for fear of falling because the floor was shifting around. I was instantly sick to my stomach. Fortunately, there was a bench within reach so I sat down and yelled for Brennan to wake Christie. I began to dry heave uncontrollably as the room kept spinning. Closing my eyes heightened the sensation. Weird thing is, none of my heart pump alarms were going off - in fact, all the numbers were completely normal. I thought something had malfunctioned but the pump seemed fine. I was so dizzy, Christie had to call 911 for an ambulance while the boys hurriedly ran around collecting my spare bags and equipment.

The paramedics had to bodily lift me onto the stretcher. On the ambulance ride, Rich calmly fielded about two dozen questions from the paramedics, explaining the pump and device to them, answering questions about my medications and medicine allergies, and helping them establish a frame for my base vital numbers. I was blown away that he knew all this, but the kid pays attention to everything. I mean EVERYTHING. With Christie and Brennan following in the car, I was rushed to the ER while the nurses looked on, probably thinking, "Oh God, a TAH patient. I hope I don't get assigned to him." Even medical people are scared of the thing. After a volley of tests, blood cultures, x-rays, and CT scans, it was concluded that....nothing was wrong. This is particularly scary because I was obviously reacting to something and not knowing what leaves a looming question mark as to whether it will happen again, and how to prevent it. In retrospect, I was probably severely dehydrated, as I've has some smaller episodes of the same thing since I've been admitted, and it usually has to do with me being too dry.

The docs were willing to discharge me after a few nights of observation provided I remained stable. I did. But the conversations soon drifted to the possibility of me being able to simply be admitted indefinitely to the HVIC unit. Being in the hospital raises my status on the waiting list to the coveted 1A position. At this point, I've accumulated enough hospital time that I'm told my name is popping up every time there's an O+ heart available in our region - they've rejected the offers because of age, questionable drug history, etc. But when I'm in the hospital, I'm as close to transplant as a person can be. At home, the UNOS (Organ procurement organization) considers me to be a 1B status. At that status, my name drops down into the hundreds on the wait list. As an O+ blood type, the chances of me ever getting called up for transplant from home are about the same as a Beatles reunion - nill.

A hospital isn't a hotel. Insurance will not allow me to check in and out at will. But if I'm admitted for a legitimate reason, they will pay for me to stay indefinitely since I have an implanted device that needs to be monitored. As you can imagine, our family agonized for a few days over our options - go home as a 1B, enjoy life as much as possible, and put off transplant until it might possibly be too late. Or stay in the hospital as a 1A, sacrificing time together for months, and get a transplant sooner. The obvious choice is to get transplanted as soon as possible. But if you've been keeping up here on my blog, you know that I've reminded everyone many times that a heart transplant isn't the silver bullet. There is a real possibility that I would not survive the surgery. Or sustain a brain-damaging stroke while in surgery. Or reject the implanted organ for a number of reasons. I'm an otherwise healthy guy, in the demographic with the best survival rates, but those other factors weigh heavily on my mind when choosing to spend what could potentially be my last days on this earth separated from Christie, Rich, and Brennan. As usual, we decided to do the hard, but sensible thing, instead of the easy, emotionally driven thing, in all hope that this story has a happy ending.

It didn't help that Perry passed away after I'd been here for two weeks. Though his new heart was perfectly healthy, the immuno-suppressant drugs every transplant recipient has to take in the months following surgery, opened the gates for the cancer cells already lurking in his body to rear their ugly heads. The bad thing about the human body, is it's really hard to know what's lurking there until you suppress the immune system and invite everything out into the open. It really does make a person question the whole idea of Intelligent Design. Intelligent? Really? My own body wants to kill me 74 different ways. If this is the best version of an intelligent design, I'd hate to see what an Un-intelligent Design would look like! Perry didn't die from a heart problem - he fought through that and got it fixed. But the cancer was ultimately the result of the transplant. None of us needed to be witness to that when I'm on the same path, not to mention the horror of losing him in that way. It only amplified the perpetual state of stress and anxiety our family is fighting off daily.

This is getting long, so I'll write more later. Just so everyone knows, I've been living in the hospital since January 16th (over three months) and no one knows how long the wait will be. We plan for me to be here until this is over, one way or another. It's been 17 years since I was diagnosed, 6 since we started trying to get me on a transplant list, 4 on the list, and 2.5 years in and out of the hospital for swans caths, cardioversions, ablations, TAH impant, and now the wait. But at least it's coming to a head and there will hopefully be some type of resolution soon.

The Letter I Don't Know How To Write

Hey Perry,

I know this was an open ended conversation and we picked up the threads from time to time, but now I have to close it and I don't know how to. I talk more than you anyway so I guess it's only fair. But we had to talk about this, right? It's something that's really too hard to talk to your family or close friends about - not really talk. Too much baggage. It was easier when we didn't know each other so well at the beginning - it was a safe place to say things we wouldn't dare say to other people. Then it got harder because it's safe to talk about dying with a passing stranger. But once you know them, you don't want to think about it anymore. They go from being a statistic to being a friend, and it's impossible for us to imagine a friend becoming a statistic. It's easier to imagine it happening to ourselves, because we know in our hearts that we are not immortal. We know what it feels like for everything to spin out of control. But that friend? That's the guy who won't ever let it get him. I don't know how. Maybe because he's been through so much, that you come to suspect he's superhuman. So I have to confess, I simply overestimated your immortality. Everyone did. And that miscalculation shows on the faces of everyone here today. I hear the miscalculation in the quiet that has overtaken the HVIC unit today. The nurses are trying really hard to work, but we're all a bit lost. Krista's a mess. A few people won't make eye contact with me. Kat is putting on a brave face but I can hear it in her voice. Everyone is numb.

You were already out of it when they admitted me on the 16th. They moved me down to 1170, or as we call it here on the HVIC, "Perry's room." They didn't know whether to put me here or in 20, right next to where you've been since the day after Christmas. I think this room was the wiser course, because I would have likely tried to sleep in the chair next to you or refused to move from the hallway outside your door, if my IV cable and TAH hose would have reached that far. So here I am, sitting in your chair, using your TV and bathroom. Of course, I'm keeping it cleaner than you did because you're a slob, dude. Still, t's weird, your stuff not being here. Even weirder that you're not in here when I come back from a walk, cussing at Ryan or Tyler for creaming you in Madden. I feel like I'm violating your privacy somehow.

Remember that time you put the Ghost Pepper sauce in Adam's drink? He was sick for a whole day, but we were laughing about it the other night. It was a good prank. Sean's not here anymore, but I'm sure he'd laugh about the apple incident too. Cindy went back to the float pool but every time I see her she wants to do a skin assessment. Glad you got such a kick out of my victimization. Jerk. A lot of people are gone now, which is pretty much your fault. They can't come in this room anymore, and they can't focus without the thump of your Freedom Driver as you try to dictate which nurse you'll have, or bugging them to walk over to the Slushie machine in the Children's hospital with you. They've either left or moved to other units because they screwed up and got emotionally involved. Impossible not to. You kind of sucked people in that way.

It just seems like this whole thing should have ended right, ya know? The story of a young guy who struggled with HCM his whole childhood, implanted with a TAH at 18, going on to transplant and having a long, happy life. Because for some reason, those are the only stories people want to hear. They don't want to hear about brain bleeds, bone marrow cancer, ECKMO machines, ventilators, bleeding out, brain damage, transfusions, and then at the end...the good guy loses. You gotta admit, it makes for a really crappy story.

But it's our story, right? The one about private battles, fought in P.E. class in jr. high when you couldn't run the laps like everyone else. Hiding out to escape the inevitable pain that will plague you the rest of the day if you do the push-ups. A-fib spinning the room around, and that frantic moment when it feels like someone just dropped a truck on your throat. Waking up in the middle of the night and it really hits you that your life is at the mercy of a machine you don't understand, or even trust. And trying to breathe through the panic so the alarm doesn't go off. Holding at bay the worry that you could buy it at any time. Watching friends fall away as the drama burns itself out when they realize there may be no end to this. It's okay that they can't finish the story with you, but it's another battle to let them go and not be angry. About making plans for a food truck and an apartment, soccer with the kids, and drum sets, and fantasizing about a normal life on the other side - one you know is unlikely to ever exist because of rejection and biopsies and immuo-drugs and possibly dying from the stupid common cold.

But you talk about it anyway, and you make plans because it helps you, just for a moment, to believe that there really is life beyond these small rooms filled with needles and tubes and finger pricks and painful dressing changes and PICC line changes. In the end, you fall asleep knowing that you probably shouldn't have talked about it at all, because it just makes you want it more, and there's nothing you can do to make it happen. If there's one thing that HCM transplants like us know, it's to not get your hopes too high. They've been smashed so many times that you don't have the energy to pick up the pieces anymore. It's not a story for TV. But it's our story and it's important because some of the time, we actually won those small battles. And we're the only ones who know how much strength and soul it sucked out of us. And I think it's okay to be proud of surviving the things we did, even it's just one more measly blood draw.

Remember how on the last episode of Scrubs, J.D. walks out of the hospital doors that last time and has a vision of all the things that might be? Friendships, and family, and happily ever after? And he's at such peace because even though he can't really see into the future, he sees a possible future, and that's enough?

I want that moment. Where I see you living in our spare room, just like we planned, being my trained volunteer in case the TAH malfunctions. And Rich and Brennan not having to carry that weight. Where I see you in your food truck, knocking out that fantastic Lasagna and all the fusion foods we worked on during my last SWANS cath. I'm working the window between taking orders and there's a big heart painted on the outside of the van. And everyone knows our story - that we fought a war together, and came out the other side with all the scars and the lessons that we learned. That you're older and I am wiser. And then we go to the apartment you had your eye on and your girlfriend and Christie meet us there with the boys and you beat us badly at Settlers of Catan and then gloat about it. You're a horrible winner, did I ever tell you that? And the kids pig out on Warheads and Troll Bites because you keep way too much candy around (if they end up with diabetes, I'm totally blaming you). I want that moment, in the middle of normal life, when no one else notices the look we exchange that says we know, between the two of us - we won the war.

But now I'm forced to let go of that moment, and remember this part, with all the tubes and machines and blood, and more needles, and remember every time I look up from my laptop how I came to be in your room. I'm forced to admit that for all the bloody battles we won, that the war was lost. It's not your fault. It's just biology, and sooner or later it gets the better of us all. It's stupid to say it's not fair that it got you sooner - that's obvious. The hardest part for me will be holding myself back from trying to make sense of it. Because no one can, and anyone who thinks they can is a damned liar. We talked a lot about that too.

I can say with a lot of conviction that I'm going to win my war. Maybe in that way, it'll feel a little like we both won, somehow. Probably not. Then again, we both know that no matter how strong our will is, and how righteous our determination, we are at the mercy of biology. And it's nothing personal - it's just doing the job Mother Nature gave it to do. There are hopes and prayers, but we also both know that in this environment, there are way more tragedies than miracles. We've seen enough of both. I allowed myself to hope for a miracle, though I've never seen anything to make me believe in such a phenomenon. I'd hoped the third-hand anecdotes were true, that maybe I would have one of those unbelievable stories about you. But we don't get to pick our stories.

I'll stay away from the fish in the cafeteria, like you said, and use the TV for my Xbox whether they like it or not. I'll check in on Jamie every day for you (I've been doing it the last two weeks if that makes you feel any better) and even try to get her to eat real food when I can. Tommy came by today to check in on you like he always does, and to say goodbye. Nearly everyone did. I'm giving Adam a lot of crap on your behalf and when I see Sean, I'll tell him you said hello. I'll stay in touch with Mudge and Terri too. But I don't promise too stay here long. Eventually the HVIC will be absent of me and Jamie as well, one way or another, and life will move on until most of us forget each other. If I do come out of this alive, and get to tell my story, it will be partly because, despite your age, you made me face reality at some pivotal moments when I almost blew it. And because even though it was a risk, you determined to finish the war, win or lose. Sometimes it's just the fighting that counts and you should get a medal for that. I'm glad we got to fight a little bit together. I expect the air is easier to breathe now, and there are no more machines to keep you from flying.

Clear skies to you, my brother.

Dave

The Five Man Electrical Band Was Right

"Signs, signs, everywhere a sign, blocking up the scenery, breaking my mind, do this/don't do that, can't you read the signs?" (Five Man Electrical Band)

If you've had any interaction whatsoever with the medical, pharmaceutical, or insurance industry, you've no doubt encountered rules. They have rules for everything: how long you can stay in the hospital, what constitutes an "emergency," orders for filing claims, time windows for prescription refills, who can prescribe what, referrals, percentage caps on co-pays and coverage, on and on and on.

While I still have to deal with most of that stuff, it's been a little unnerving how mushy some of the rules are for the TAH itself, not to mention some of the gaps in my practical care. The problem is that this whole notion of sending a patient home to live with an artificial heart is so new, there aren't a lot of protocols in place. There are some hard fast rules about how often to change out the machine (120 days), when to change the filter (once a week), the range of your PT/INR (blood coagulation - and this is a biggie), blood sugar levels, etc. But in terms of day to day issues, there's a lot of uncharted territory. And we've encountered a lot of unexplained things.

We underwent a 30-something odd hour training class in which we handled the equipment, tested rigorously, demonstrated proficiency, participated in trial runs away from the comfort of the HVIC unit, and signed agreements that we would care for the equipment and such. We learned what each beep and boop meant, what the alarm button colors meant, and how to methodically assess the problem when alarms sounded.

When we got home, the machine started doing weird things. Things no one had told us about. For instance, when unplugged, the machine emits a series of beeps at about 20 minute intervals. We finally realized that the beeps corresponded to each 20% mark on the battery life indicators. It's great because it helps me keep track of how much battery life I have left without having to look at the machine itself. Problem is, this is mentioned not a single time in the literature, nor was it mentioned in the class, nor has our device coordinator ever heard of this before. I got a new machine last week and it does the same thing, only with both drivers, they didn't do it all the time. Sometimes I would hear nothing for 3 hours, then a low battery low alarm sounds off. No warning. The new driver emits the same beeping sound intermittently even when I'm plugged in. No one knows why. We think it's probably beeping to let us know when each battery is fully charged after plugging in, but that doesn't seem consistent either. The machine functions properly, and has been rigorously tested by the Syncardia company, and two separate bio-engineering teams at Penn State.  But there's no explanation for these inconsistent alarms. I try not to let it unnerve me but it's a little more than a distraction or curiosity when the thing is controlling your heartbeat.

I also detailed in the last post that I essentially have two wound sites - the canula site where the driver tubes enter the body, and the Picc line. I guess "wound" isn't the right word but they have to be treated with extreme care to prevent infection - it's not like they can just remove the canulas because the site got infected. There's no backup for that. The Picc line is important because of weekly blood draws, and the immediate access it gives nurses if I need emergency meds. After having blood drawn conventionally for labs about 6-8 times a month starting back in 2013 (and 3-4 times a week in 2015), my veins are trashed. Even the instructor in the Penn State lab confided that they would soon have to start using veins in my feet and legs if my arms didn't get a rest. So the Picc line is my friend.

Upon leaving the hospital, Christie was "trained" to clean the canula site and change the dressing. She's been doing this for patients for years, not to mention much more complicated wound care, so that was no problem. Then came time for the Picc line dressing to be changed, and blood to be drawn for labs. She wasn't allowed to do it - we were given a dozen reasons why (all asinine) - if it gets infected, insurance won't know who to blame and whether to cover the following treatment - only RN's employed on the HVIC are "covered" to draw blood through a Picc line. This couldn't be done in the general lab since lab draws MUST be done by RN's and the lab does not employ one. The cancer infusion center draws blood from Picc lines, but I'm not a cancer patient. Though Christie does blood draws for labs dozens of times per week, and a home health RN is allowed to do it, AND she's changing the dressing on a much more dangerous open wound (the canula site), she was instructed unequivocally not to do anything with the Picc site.

So where was I supposed to get labs drawn? Home health costs $27 a pop, $54 a week. That adds up fast when you're already going broke paying medication co-pays and the primary breadwinner is missing work because of all the doctor appointments. The spouse (in my case the RN) changing the canula dressing can't do it. The lab can't do it. So what do I do? We posed this question to three different device coordinators, three intensivists,  a cardiologist, two nurse practitioners, and every HVIC nurse we could talk to. No one had a definitive answer. There was no protocol for this because I was the very first patient sent home on a TAH, needing bi-weekly blood draws, and needing them done via a Picc line. It started to seem like all involved wanted Christie to do the blood draws - despite their official instructions not to, wink wink. If an infection actually did occur, she'd be left holding the bag, whether it was her fault or not.

Because she works at Penn State, she was able to circumvent the insanity and get an order for me to have labs drawn in the cancer infusion center. What about patients whose spouse doesn't work for Penn State? What do they do? There is no answer.

PT/INR checks are part of this mess as well. I need them three times a week to ensure my blood isn't thickening, putting me at risk for stroke. They cannot be skipped. Again, a home health nurse can do it for $27 a pop. Or we travel the hour round-trip to the INR clinic so they can prick my finger, put a drop of blood on a slide, and feed it to a handheld machine. Don't they have these for home use? Why yes, they do. But insurance will only pay for it to be used once per week. That helps, but what about the other two times? Why won't they cover three times per week since I'm at a higher risk than most, due to the TAH? What's a TAH? You've never heard of a TAH? Oh, you don't have a box to check for "TAH" on your claim paperwork? You have to file it under LVAD? Then what happens? Insurance will cover one stick per week, like they do for LVAD patients? But I don't have an LVAD, I have a TAH. Remember? I was just telling you about the TAH? You don't have a box for that?

You get the idea.

Speaking of no box to check, this is especially interesting when it comes to OPTN and UNOS, the organizations that essentially oversee the waiting list for transplants. They also have no box to check. I'm sure I've posted info on how this works before, but as a quick refresher, the list works like this:

Status 1A: the most urgent, will-die-very-soon-without-organ patients, who are likely living in the hospital hooked up to life-sustaining devices.
Status 1B: patient needs a transplant asap, but isn't at death's door - possibly spends time in the hospital as a 1A patient, but mostly lives at home with some type of device or intravenous drug dependency.
Status 2: patient who eventually needs a transplant, but doesn't need any type of life-sustaining devices and is being managed with oral drugs.
Status 7: patient who could have been any of the above but is currently sick, away from the transplant facility, or for some temporary reason unable to undergo surgery at the present time (usually sick with flu, cold, etc.)

I was Status 2 before the TAH when I was at home. When I would spend a couple of weeks in the hospital, with a Swans cath and intravenous drugs, my status was elevated to 1A. When I went home, back to Status 2.

Most people on LVADs are considered 1B's because they don't have to live in the hospital, and the device is sustaining them at home. In most cases, should the LVAD stop doing the trick, they would be hospitalized and become 1A, probably dependent on bypass machines, intravenous drugs, etc. until they can get a heart.

TAH's are classified as LVADs because UNOS and OPTN have yet to seriously evaluate exactly how TAH patients should be treated in relation to the waiting list. The problem is, a TAH is NOT the same thing as an LVAD. An LVAD patient has several options left if their device starts to fail, or their condition worsens while on the device. TAH patients have already exhausted every other option. There is no further treatment if the TAH fails. At that point, they are in as much if not more trouble then a 1A patient because there's no way to "treat" an artificial heart. You can't coax a few more days or weeks from it. It's a machine. For now, TAH patients are 2's unless admitted to the hospital, or they can get an exception in which case they are elevated to 1B's.

So it would seem like the sensible thing to do would be to stay admitted to the hospital as a 1A, which would increase the odds of getting a heart sooner, right? We had long, long, long conversations between ourselves about whether I should come home or stay in the hospital post-transplant, because we thought it was an option. Brennan, sensibly, argued that I'd have to wait several months to be re-listed anyway, so I might as well be home during that time, then get re-admitted when I was re-listed. That was the plan (though, to our surprise, I was re-listed within weeks of the surgery). Makes sense, except according to insurance, as long as my PT/INR is reasonably stable and there are no other complications, there is no medical reason for a TAH patient to be admitted to the hospital. They won't pay for it. But the reason they take this stance is because by their rules, TAH's are the same thing as LVADs. 

Obviously TAH's need their own classification with OPTN and the insurance industry. But think about this for a moment: I am insured by the very facility that implanted the TAH device, yet they have no separate classification for it. That's how new this is.

The medical/science aspect of the TAH is wonderful. I'm lucky to be alive, and so grateful that this technology even exists. But it comes with many challenges that are frustrating, maddening at times. Five years from now, most of these issues will be resolved, but they are definitely things for the future TAH patient to be aware of. We weren't. Hopefully, our experience helps to prepare those of you considering a TAH down the road. All of this is workable, but it takes work, a lot of time on the phone, pushing, prodding, and arguing at this point in time. The great hope is that the medical and insurance industry will catch up with the technology soon, and none of it will be an issue anymore. Until then, there are a lot of Signs.

Someone Broke My Ribs...

So this post is more for the benefit of those with TAH's in their future, or for those who are morbidly fascinated with open wounds, blood, bile, and the gamut of aches and pains. Honestly, this is the kind of post I've been putting off, because it's probably a little more personal than I'm comfortable with. But when I was preparing myself for surgery, I asked Perry a million questions. I wanted to see everything. I even wanted to touch everything. You probably do too.

Fair warning: I'm going to include pictures and a few videos in this post, and some of them are not for the squeamish. But if you're about to undergo this procedure, there's nowhere else on the web to get a first hand look at the process. The Syncardia website makes the TAH implant look like a day at the carnival. The doctors don't tell you all the things the nurses will have to do post-op, or really what to expect physically once you're home. These are all things I wish I'd known ahead of time, because in all seriousness, if Christie were not a nurse, I'm not sure how I would have handled all this. Not to mention that she works at the hospital where the procedure was done and knows how the system works, and how to get things done.

There are two major issues I want to address over the next few days: 
1) The actual physicality of the procedure, along with post-op and daily living challenges, and 
2) Navigating the murky waters of hospital protocols once you're home.

Let's start with #1

The Procedure

Before surgery, a nurse or aide will shave everything from your nostrils to your nethers and in the words of Perry - "...and I mean everything." Haircuts are not uncommon. Surgeons don't like hair in their OR's and you shouldn't either. This is significant because about 2-3 days later, it starts to itch. This is particularly a problem because you are covered in wound dressings, taped up in about five different places, and possibly taking pain meds that make you itch even more. Lotions are prohibited because of healing surgical sites and the risk of infection. The solution for this? Suffer. It will be over in a day or two and hopefully the pain meds take the edge off. You'll be asleep most of the time anyway.

In the OR, you will have an "A line" placed. Basically, an IV line with a needle the size of a pencil inserted into the very most tender, mommy-loving, part of your wrist - that part that you check to see if the bottled milk is too hot for baby because it's the most sensitive part? Yeah, right there. For me, this was the absolute worst part of the whole ordeal, and it only lasted about four seconds. I'm just being straight here because you need to be mentally prepared. I have no words to describe the excruciating, mind-numbing, primal pain of the A-line. It was all the pain in the universe packed into a tight four seconds, but then it was over. They actually strap your arm down and give you 4-5 local anesthetic shots in your palm and arm to lessen the pain of the IV - probably because people would pass out without that mercy. I let out a blood-curdling, I'm-being-murdered-slowly-with-an-axe- scream - and the docs said it was pretty run-of-the-mill for an A-line placement. I don't remember much else after that.

You awake with a tube down your throat and not much sensation anywhere else. That part wasn't as bad as I imagined - just relax and breathe with it. When I first came to, I listened for the familiar sound of the TAH that I knew from being around Perry. The thumping is a pretty good sign that everything went well. Fog for about a few hours, then the realization that you're rigged up much like Neo in The Matrix. The sooner you start breathing on your own the sooner the tube comes out. The nurses will ask you to cough and take a few deep breaths with it. Once you can, out it comes. A little burning, but not bad.

Five chest tubes protruding from the lower edge of the rib cage, two tubes feeding air to the artificial heart at on the left side of the abdomen, the afore-mentioned A-line, and if you're very lucky, a Picc line in your right bicep that was placed with little ceremony or pain prior to the surgery. More on the 

wonders of the Picc line in a the next post.

About 2-3 hours post-op

Pic line. I still have this today, though most people can't keep them sterile for that long and they have to be removed after about a month. My veins are trashed from continuous lab draws pre-surgery, so the nurses can now draw blood or administer meds through this. Not sure how long I'll be able to keep it clean and safe, but it's already saved me nearly 50 needle sticks (probably more, since no one can really get a vein on the first or second try)

I could actually feel the contours of the chest tubes coiled inside my chest cavity - there being lots of empty space now where my over-sized heart had been. They didn't hurt, they were just uncomfortable, making it difficult to draw deep breaths. This was disturbing because breathing deep is one of the things I looked forward to the most after the hellish shortness of breath over the last few years. All was well by the third day and out they came:

This only burned a little, but the pain was immediately rewarded by glorious oxygen - deep, full breaths. I could feel the coils unwinding as they came out - a little odd feeling, but not very painful at all. Deep breathing made me suddenly aware that someone had broken my ribs. Bone pain is awful, and Christie made sure the nurses stayed on top of my pain meds accordingly. The incision in my chest was healing nicely, but the skin is incredibly tight, and breathing deep definitely makes you aware of it. Nevertheless, being free of the chest tubes allowed me to start moving from the bed to the chair and back with assistance, and trying to use the bathroom. It's all important to get the digestive system moving - a sure sign that the body is recovering nicely and the plumbing is working. This took quite a few days for me as anesthetic tends to slow things down. Trips to the chair, bed, or bathroom are tedious - I was connected by the tubes in my abdomen to the Companion 2. This is the machine that accompanied me everywhere I went. The bathroom, walks around the halls, etc.

        

I had to be stable on the Companion 2 before I could be switched over the Freedom Driver:

(For videos on how the Freedom Driver works, go to Syncardia.com )

People will want to visit you during these first few days, but the trouble for me was that it was an incredible effort just to stay awake. Even talking was exhausting, but your family and friends will be excited to see you as soon as they can. It's probably a good idea to discuss this before hand with everyone and have a plan - I wasn't truly up to company until about 2-3 weeks post-op because every action is a huge expense of energy, there's a lot of digestive uncertainty, and privacy goes a long way toward helping you recover at a good pace. A trip from the bed to the chair, or the bathroom and back required a 3-4 hour nap to recover. It took me about 4-5 weeks to be independent and strong enough to consider switching to the freedom driver, and by then, I was more than happy to see friends and have people in the room. Obviously there are people you'll want to see as soon as possible, just be warned that you will be way more knocked out than you expect, and keeping you eyes open for any length of time requires a supreme effort those first few weeks.

Once I was stable on the Freedom Driver, and family and friends had undergone the week of training, I was discharged. Though I had a little scare and had to be re-admitted a week later, I only stayed another six days. Then the real fun started. 

The immediate challenge was (and is) making sure the canula dressing stays nice and clean. This means weekly dressing changes, and again, I'm so fortunate that Christie has experience with these. I generally "shower" once a week, followed by a canula dressing change, and hopefully, a pic line dressing change. These don't always line up due to life and Christie's ever-changing schedule, but we get pretty close. 

Showers - most people take sink baths and use lots of powder and lotion. We've actually devised a way of waterproofing the canula and pic line dressing with water shields and plastic bags. This allows me to at least stand in the shower and use the spray shower head to wash off. I don't stand directly underneath the water, but I can at least get wet, use a real washcloth and soap, and lean over far enough to wash my hair. Note that this whole time, the actual backpack with the pump sits on a chair outside the shower. It makes a bit of a wet mess, but we're getting better at it. This generally takes about 30 minutes. Then I dry off quickly and move to the bedroom where I lay down on the bed for the dressing changes. Rich and Christie work together to create a sterile space, then prep the various tools and materials for the change. They gingerly remove the dressing, because if too much hair has grown under the bandages, it becomes a very unprofessional wax job. They use chloro-prep applicators to remove any bile build-up, sanitize the area around and under the tubes, then carefully shave the whole area. This can be a bit tricky because there are raw spots and stitches to avoid. It often burns and it definitely freaks me out to have everything exposed like that for half an hour. When everything is clean, they use a skin prep solution to coat the area where the bandages will go. I'm violently allergic to adhesive (as it turns out) and without this barrier in place, I develop nasty, oozing blisters that get infected, raise my white blood cell count, and potentially landing me in the hospital. Once it dries, the bandages are carefully applied, followed by an abdominal binder as an added layer of protection. The same procedure is used to replace the Picc line dressing, though I sometimes have that done by the nurse at the hospital after my Wednesday lab draws. This all takes anywhere from an thirty to forty-five minutes, and creates quite a mess.

So much bio waste, so little time

Not as painful as it looks, just always in the way. But a clean dressing makes you feel a little more human again.

It holds me for the week, but I have to be careful not to get too sweaty or overheated between shower days or everything can get really manky.

Beyond this are the meds. Be prepared to spend around $200 a month, even with good insurance. This is for meds, supplies, binders, and might cost more depending on your coverage and need for a home health nurse. We used one for a few weks but it became cost prohibitive, so now we make a trip or two a week to the anti-coagulation clinic for my blood checks. Again - be prepared to have someone drive you if you don't use home health.

Every two weeks I'll load these guys up - too many pills to remember, so I have a list. Anyone going into TAH or transplant surgery MUST have something like this that is pedantically organized down to the hour so you don't miss meds. It could literally be a life or death issue. Spend the $10 and get good med organizer. 

Speaking of driving, you can't. Period. I know some of my readers are HCMers like I was (WAS...feels so good to use that tense) and are used to doing small things like light trips to the grocery store, or taking the kids places, even running up the street to grab take-out. That's over. Not only can you not go anywhere by yourself, you can't be left alone at home either - meaning that the other members of the household can't go anywhere without you. One of the ways I used to conserve energy was staying home for things I didn't need to attend: dropping the kids off at book club, dropping off a package, a quick vet appointment. I'm in attendance for all of those things now because if they're going, I have to go. This isn't so much a burden on me, but someone has to haul the equipment to the van, load it, cart it around, unload it and haul it back to the house when we get home. Sometimes you need to run out and back, then out again soon after - the equipment goes every time. It's not uncommon for Christie or the boys to load and re-load my bags 5-8 times a day. We can't just leave them in the van if we're going back out because if the machine faults, the bags need to be within arms reach.

Sleeping can be difficult at first. I put the backpack on a chair by the bed to give myself the a generous stretch of tubing. I slept on my back the first two months, but if I wear the binder at night, I can sleep pretty comfortably on my side now. The machine tends to alarm if I stop breathing momentarily because of a dream, or hold my breath while stretching my muscles (which no one really notices themselves doing until an alarm tells you so).

I'm sure I'll think of more things in the future. Next post I'll discuss the obstacles involving insurance, hospital protocols, and the importance of a device coordinator who gives a rip.

This all sounds scary but I wish someone had prepared us for it all beforehand. It's both an emotional and psychological adjustment. Your life is literally at the mercy of this impersonal machine, and it takes a lot of effort on the part of loved ones and friends to keep everything moving smoothly. It's easy to get down in the dumps - while the Freedom Driver takes away all of the horrific sickness from before, it also chains you to itself and forces you into a life of even greater dependence on others. It's important to sleep well, eat well, and focus on the things you can do. Hopefully some of these details help someone to get straight with it all beforehand. I feel like we're still playing catch up.