You Had A Bad Day

For all the days I'm here in which there's nothing to report, Tuesday was a doozy.

It started simply enough. I usually sleep on and off as the nursing staff, students, and residents buzz around taking care of business. In the midst of all this, my nurse tells me "they" are increasing my inotrophics. These are two meds (dopamine and dobutamine) on a drip that are administered the whole time I'm here. In fact, they are the main reason I'm here so often - UNOS (the transplant big shots) require that a transplant candidate spend a certain amount of time admitted to the hospital on inotrophic/IV drugs. So as long as I'm here, and as long as I'm receiving this treatment, I am gaining what's called "IA time" which in turn helps me to move up the transplant waiting list.

Ever present on Dr. Doodaa's hat, they look perfectly innocent but as I learned
Tuesday, can cause a world of hurt if you don't keep an eye on them.

I always want to know why something is being changed in my standing orders. I've had too many close-calls with the Grim Reaper in emergency rooms and hospitals not to. I advocate for myself so aggressively that it's probably offensive at times but not doing so is a quick way to get dead. And that involves a lot of paperwork, so I figure I'm helping everybody in the end.

The nurse didn't know why, but the doc was next door and would be stopping in on rounds to discuss it. Now my suspicion was that he himself didn't order it, but rather one of his residents - my usual doctor is out this week, so one of his colleagues is running the show here in the heart unit. He and I already have this understanding that I want to know what is happening at all times. The doc who's here this week apparently gives his students more free rein with patients. It's a teaching hospital, I get that. But I ran into a problem on Monday when a resident tried to stop my Heparin (blood thinner) drip in favor of Levanox - administered by shots in the stomach a few times a day. Um, no thank you Monty, I'm going to stay with whatever is behind Door #1. Sometimes doctors change orders without talking to or even looking at the patient, as was the case here. I don't tolerate it because it's dangerous and they know it's dangerous. Turns out, the resident ordered this change without talking to anyone. And since the Heparin drip isn't really necessary to being with, why change the blood thinner med to shot form? I get enough needles as is.

Re-enactment of my "Yo, residents be crazy" face upon learning that some moron
wanted to stab me in the stomach every eight hours. 

I suspected the inotrophic increase was a similar situation. Before I could ponder this further, I was overcome with a headache, cold sweats, and gut-wrenching nausea that left me dry-heaving about a dozen times into a bucket. I have a much milder but similar reaction when they start the drips the first time, but it passes quickly. The doctors explained that since the inotrophs were helping my heart function, giving me a bit more might help the diuretic to work more effectively, thus helping to shed some of the fluid I'm retaining in my abdomen. The nausea subsided, but within about thirty minutes it had returned with a vengeance. Horrible stomach cramps made it feel like fireworks were exploding in my kidneys. More dry heaving. The nurse was incredulous because it was such a small increase.

Actual bucket into which I dry heaved. E-bay listing to follow.

Thus began what I call the "Let's Fix That" cycle, in which you are administered a drug you don't need, spawning bizarre and painful side-effects, that must now be treated with further medications, causing side effects which must be treated with further medications, and so on. It seems to me doctors, especially younger ones, sometimes lose sight of how this cycle began, and become hellbent on seeing the "Let's Fix That" cycle through to the bitter end. And so the doctors began to surmise that the reason for my sudden onset of pain and misery was due to lack of bowel movement, too much sodium, too little this, too much that, no enough of the other, the pull of the moon on the tide, the changing weather, my astrological sign, etc. It takes quite a while sometimes to force them to admit that the only thing that has changed is the thing they just did to you.

In the meantime I couldn't sit or stand without excruciating pain in my lower back and abdomen. I was given Phenergan to relieve the stomach pain. If you've ever had this drug before, you know that it is most likely what the guys in Pink Floyd were passing around when they wrote "The Wall." My dreams consisted of marrying Etta James to conquering an evil corporation run by Will Smith (with my sidekick, a talking toaster oven. Who else?) all to a really terrible dubstep soundtrack. Trippy stuff and lots of it.

I slept from 1:30pm to 5pm only to awake and find that the pain was still there. I had to have a nurse help me from the bed to the chair because I was afraid I might fall. This is how the "Let's Fix That" cycle works. Now I was not only STILL in pain from the med increase, but hungover from the attempt to fix the side-effects from the med increase. A nurse finally convinced the doctor to decrease the med again.

By the time Christie and the boys arrived, I was feeling a bit better and as soon as the meds were put back to the original dose, surprise! Most of the pain went away. I'm such a lightweight when it comes to pain meds, so after a delicious meal of chicken enchiladas and a short visit, I went back to bed. Slept from 7pm to 1am, took some pain meds for my neck (it was stiff from sleeping so much) then slept again to 5:30am. I've been up for half an hour, sore in my abdomen, and shaky from the hangover. But breakfast, provided by my lovely wife, is restoring my strength a bit. If you called, texted or email Tuesday after about 11, it's unlikely I saw it or remembered it, so I'll try to catch up today. Nothing personal, I was just busy at a rave with Bert and Ernie after our water-skiing adventure.

Honeyed yogurt with blueberries and strawberries. Tons of potassium going down here. Yum!

What a day. This morning I have to discuss this whole goose chase with my doctor, who will be here). The students will probably leave convinced that poo-poo or the temperature in the room were to blame for the whole thing. Either way, I know for next time to refuse an increase unless I absolutely need it.

Christie met with the oncology surgeon today to find out more about her gallbladder issue. I was able to listen in on speakerphone and the doctor didn't seem to have any concerns about her long-term prognosis. The "polyph" actually turns out to be a thickening of the gallbladder wall, consistent with stones and cholesterol.  I'll update here if we get any new information. Thanks for reading and for all the encouraging comments, both here and on Facebook.

On The Inside

Being in the hospital for a week isn't all bad. I've been playing a lot of games, mixing some songs I've been working on at home, and catching up on my reading. Yesterday, my Valentine (Mark Foley) brought me sushi for lunch and hung out for a while until it started getting busy. We don't get to hang out just the two of us as much as we'd like, so it was really great to just chill out with him. I didn't take pictures because my phone keeps dying while looking for a cell signal and also sushi nom nom nom.

The best window view from my unit. The window has a nice, wide
windowsill and when it's not too cold, I like to sit up there and read.

On the other hand, this has been a really tough visit so far. Friday, Christie started having what we thought were back spasms. Turns out (after a visit to the ER later that night) she was passing a kidney stone. In the process of examining her, they found a polyp in her gallbladder, which means she needs to have the organ removed, posthaste. She's having an MRI today and meeting with a surgeon tomorrow to discuss biopsy. Hopefully it's just a run-of-the-mill gallbladder problem. We're not yet sure how it's going to work out if they want to do the surgery this week. Though it needs to be done quickly, with me being in the hospital, the scheduling could get dicey. To make matters worse, she finally has an interview this week on the cardio unit where I stay, after six months of toughing it out in a difficult assignment on a medical/surgical floor. Hopefully that won't be jepordized in the mix.

As always, people from our awesome homeschool group and other friends are helping with the kids, the dog, the snow shoveling, and someone even took Christie to the grocery store and back the other day. We're so glad that we're not going through this craziness in Springfield, MA where we barely knew anyone. In all the uncertainty, it's comforting to be surrounded by people who jump in and help where it's needed.

As for me, several people were taken a back by my recovery room picture, but I'm only like that for a few hours. Once I'm on the cardiology ward, I'm up and around, walking, scrounging for food, and making a general nuisance of myself.

Me and Dr. Doodaa. See? I'm not in a coma. He also got a new hat yesterday and thinks he looks very spiffy. 

I can't remember, but I may have mentioned the food here isn't the greatest. In fact, I'd rather eat feet.

This is the menu you receive each day so you can select
your meals. I will give you $100 right now if you
can find a single item pictured down there in that kitchen.

Great question. What IS on my plate? The CDC is supposed to be
getting back to me on that soon.

We decided after my last stay that we would freeze extra meals at home for my hospital stays. It relieves Christie of having to cook during such a busy week, and she brings me enough every few days that I'm not forced to eat whatever that is on my plate. I keep a little cooler in the room with real fruit drinks, yogurt and fruit, and some single serving meals to heat up.

Blue ice keeps everything cold and the nurses help me change it out every day or so.

The food situation poses other temptations that I'm slowly but surely giving into. I'm not allowed to leave the unit. That is a new rule instituted since some genius wandered down to the cafeteria and stepped on the cord to his swan catheter, effectively spurting blood all over the room and all the way back up to the unit. Tim Conway and Dan Akroyd would have been proud. I mean, the cafeteria probably has enough trouble keeping customers as it is without a Wes Craven floor show in the middle of lunch. 

Yet, the patients take hope in the whispered rumors of a mysterious room where untold culinary wonders are locked away from those who would seek to plunder its treasures. Though heavily guarded, I was able to sneak a picture of the supposed location of this room.

Forgive the blurriness, I had to act swiftly as the guards nurses were eying me suspiciously when I lingered.

Patients tell tales of ice cream, cold whole milk, endless cans of Ocean Spray juices and ginger ale, and sometimes, if the conditions are right, turkey sandwiches made from animals that were in fact actually turkeys to begin with. While the contents remain firmly in the realm of myth and legend, prophecies speak of One who will eventually breach the barrier and open the way for all to partake of the coveted bounty within. Until He comes, we wait.

Cabin fever sets in at times. Late at night I often open the door to the unit and stand in the hallway out of spite. It's empty - I obviously don't want to risk catching some nasty germ in a crowd and carrying it back into the cardio ward. But at night, it's quiet and peaceful out there and this small act of rebellion helps me make it through the week.

It's like a portal to another world. See the lights shining beyond? Can you hear the angelic music?

That's all for now. Much anxiety here about Christie's situation but hopefully we find something out tomorrow. I'll post here when we know.

Back Up A Minute

In my previous post I hinted at some circus-style craziness that took place in the recovery room Friday. Recovery rooms are interesting places because try as they might, the nurses there know they're only going to have you for a few hours. So they either care a lot about their job or they don't - the option of developing an endearing relationship with them isn't realistic because you're in and out so fast. You have to hope they take pride in their work because you're not attached to them in the same way as the nurses on your ward - that evolves into a relationship pretty quickly because how can you be formal with someone when you're passing your urine back and forth and talking about your gas-passing habits? Think there's no secrets between you and your priest? Spend a week in the hospital.

Recovery is also kind of awesome because although you've just had a wire stuck into your heart, you don't really feel it, and the anesthetic leaves you in a hazy euphoria for a bit afterwards. It's the one time I get a taste of this dream-like state so many waste their lives trying to achieve through illegal means. While I don't understand that mentality, I really do get why people who want to escape reality choose this method. Everything seems right with the world for a few precious hours. Usually.

Since there were considerable problems in the cath lab with getting my sheath inserted, all the joys of chemical la-la land had been driven away. By the time I got to recovery, I was feeling everything, and dreading returning to the care of Bill, the cro-magnum nurse-child. Communication is not fun when you're in pain. Imagine that the only person to whom you can communicate your needs answers every question with a grunted, "huh?" Then when you repeat the question, they repeat the question back to you in an incredulous tone that answers nothing.

Me: "Hey, when you get a chance, could I get some water?"
Bill: "......Huhmm?"
Me: "I was wondering if I could get a glass of water."
Bill: "..................................can you get a glass of water?"
Me: ".........umm.......I don't know.....that's why I was asking you."
Bill: "You're asking me?"

Every. Single. Exchange. When I'm in pain, my first choice of activity isn't a game of Who's On First? I haven't had anything to drink in almost ten hours. Wa-ter. Why do I feel like I'm being investigated for a murder? Can he read my mind?

Unless you've been in this type of dependent situation at length, you never realize how important certain trivialities become. Bed rails? If both sides are up, you can't get out. You're trapped. Call button? Great idea, but if it's not plugged in, you may as well be pushing your nose. Urinal? After you've been given a diuretic, the last thing you want to see is the urinal on the other side of the room, out of reach. I was hooked up to machines on my right side and didn't have much freedom to move around anyway. Then the nurse disappears for nearly thirty minutes. After desperately pounding the call button, then trying to use the Force, I began to debate the pros and cons of peeing in the bed verses trying to sit up and aim for the floor.

This is my urinal, whose name will not be mentioned here as I only use it when I accidentally drop him or knock him over. I needed him desperately in recovery, and he looked like this, only imagine a much smaller version because he's very far away.

This of course set off the bed alarm, causing a nearby nurse (presumably one of Bill's babysitters) to come in and yell at me for sitting up. When I explained that the call light wasn't plugged in, she informed me that I had unplugged it by raising the bed to sit up.

Okay then.

She left before I could ask for the urinal, and didn't ask why I was sitting in the bed. I sat up again to bring her back. The bed alarm went off and she arrived exasperated to find me suspended Mission Impossible style (except by my neck) trying to aim over the side of the bed. She hands me the urinal, sighs with the weight of the world upon her, and storms back out (poor girl, her neck must be hurting and she's probably distracted and anxious as she contemplates the frailty of life and her fate at the mercy of uncaring strangers. Oh wait, THAT'S ME).

Bill returns to ask if I'm okay to which I wish I'd had the presence of mind to answer: "Hmmph?"

Before I can answer, a race car pulls up to the door of the room. Like one of those kid's shopping carts, pin-striped, cherry red, number on the side. Someone steps into the room and says, "Okay! You ready to go?" But before I could decide if I was hallucinating or had been secretly drugged again, another nurse steps into the room to announce that I have visitors.

The Foley's have our kids, Christie's at home trying to make her way back to the hospital (another story for tomorrow)...who would be visiting me in recovery?

I know what you're thinking, and you're absolutely right. It was obviously a Barbershop Quartet, because that's what you expect to see in a recovery room. In fact, when I first come onto the ward, I ask where the Barbershop Quartet is. First thing that comes to mind.

Tuxedos with red lapels, red bow ties, holding a bag of Hershey Hugs and a black Valentines devil bear.

I have performed an experimental right heart cath on Devil Bear - you can see the wire in the above picture. It only made sense given that his heart is enlarged and sometimes common experience breeds true sympathy. What? Therapists use stuffed animals all the time. DON'T JUDGE ME.

"Do you know Mark Foley?"

"Ummm....yes?"

"This is from him."

They launch into an amazing version of  Let Me Call You Sweetheart and Your Lovely Face. I didn't get a picture or video because electronics don't work in Acid Trip Land. But it was something like this:

Of course all the nurses and techs gathered around to listen. I now know that the quartet was actually a gift from our Foley friends, both Mark and Stacy, to Christie and I, in hopes they would catch her at the hospital. With it being Valentine's Day and me back and forth between rooms, they're lucky they found me at all. Of course everyone thought the songs were from my "friend" Mark to me alone. I didn't correct them. Mark and I have a kind of J.D./Turk thing going on (Scrubs), and are always joking about it, so it seemed like a great joke, given our on-going Bro-mance (though he was quick to assure me that it was Stacy's idea. Sure Mark. Whatever you say).

Once the they made their exit, the race car turned out to be the front end of an X-ray machine, borrowed from pediatrics. So I wasn't hallucinating. Good thing or bad thing?

I finally got up to my room to the welcome sight of Sara, my three-time, no-nonsense nurse who immediately got my pain situation under control and answered to my every beck and call (not really, but she's great).

So that was the recovery room. I'm still recovering from it.

All The Gory Details

Thus did my week at Penn State resort begin. Forgive me if I ramble a bit, I'm still pretty doped from the few drops of Delotid they gave me yesterday (I'm such a lightweight). Hopefully some of these details will help someone else headed down the same path at some point. And I don't know about you, but it helps me to actually see pictures when someone's going through a medical thing so I have an idea of what is happening. Nothing here is really gory (unless you count the shirtless pic of me further down).

Christie was up a 6 to rally the boys, pack bags, and dig a trench to the van. It snowed A LOT this last week (with more to come) so shoveling has been a daily chore. Nothing is melting so we've got an ice-under-snow situation. While I finished packing my stuff for the week, she and Rich went to work digging the van out.

 We have a garage, but it has snowed so much that the alley 

behind the house to which it's attached is a solid sheet of ice with mud beneath.

Rich really does take it upon himself as much as he can, for which we're so grateful.

  

And this:

Christie and Rich have also been clearing our elderly neighbor's walk and drive while she's in the hospital, and she generously offered for us to use her driveway until the alley and garage pad melted.

And the house looked like this:

 

Though you can see that once out of the driveway and our street, the roads were pretty clear to the hospital:

When we arrive, I check in while Christie parks. The boys know the drill - they head to the waiting room and log onto the Penn State wi-fi to play Minecraft with friends, or play DS, or read a book. I'm usually the first cath of the day so the place is empty most of the time. 

First things first, I need a port for anesthesia, labs, etc. For this part, you want Matt. You do not want Bill. Matt is a good stick and knows exactly what he's doing. Bill, on the other hand, learned to place ports at the Helen Keller school for Medical Hopefuls. And he was in the remedial class. Bill couldn't find a vein with a Garmin handheld, a flashing runway, and an arrow tattoo saying "INSERT NEEDLE HERE." Bill, in short, sucks not only as a nurse, but at life in general. Bill blows veins. Bill pierces veins. Bill has to have someone else do the stick for him because Bill is incompetent. Not to put too fine a point on it, but Matt = good, Bill = shouldn't even apply for a job sticking straws through the X's on the plastic lids for Styrofoam cups. 

But eventually one of his colleagues (read:babysitters) manages to get the port in. They wheel me into the cath lab and make me woozy with a mix of Phentenol and Versed. Once this kicks in, it is typically a painless procedure with a little pressure on your neck at times. Yesterday, the doctor inserting the cath met with scar tissue from the last 3-5 caths I've had, and was forced to push really hard to get the sheath and wire in. The procedure is two-fold: first, a short, rubber sheath is inserted as a guide for the wire (or "cath" that will run down the artery and into my heart). The sheath is pliable and therefore if it meets with any resistance has to be forced aggressively into place, which is what happened yesterday. This caused a considerable amount of unexpected pain. However, the cath went in easily after that and I was returned to recovery.

In recovery, Lucy in the Sky with Diamonds playing in my head. I think I also talked about how cool my hand looks.

As the anesthesia from the procedure wears off, it begins to dawn on me that I've been stabbed in the neck, albeit by a group of well-meaning professionals. Where there should be a standing order for pain meds, I inform Bill that I will need some Tylennol (at least) because of the normal pain, not to mention the now throbbing tissue surrounding the the cath site where the doc had to man-handle the sheath to get it in. Thus commenced a 45 minute dance routine known as the "Hem-Haw" while Bill tried to suss out how the phone works. Or whatever. Matt = pain meds in 15 minutes from first request. Bill = discussion about pain meds 45 minutes after first request accompanied by multiple asinine questions about what "number" the pain is at. After explaining that on a scale of 1 to 10 mine was around Get Me Drugs or I'm Going To Tear Out Your Spleen With This Urinal, he finally trotted off to call the doc for pain meds.

Pre-narcotics

Post-narcotics. Have I mentioned how cool my hand looks?

You must know that Penn State is AWESOME. The doctors are brainiacs with incredible bedside manner, the nurses are sharp, pro-active, and compassionate, and even the people who clean the rooms are super-friendly. I love the place. Then there's Bill. But I digress. After a debacle involving a broken call button, a missing urinal, a sports car, and a barbershop quartet, I was taken up to my room (I'm making up zero of those things. Tune in next time for details).

Upon arrival I'm greeted by Sara, one of my favorite nurses on the IMC ward. Most of them know me by name now and she was on top of the pain meds and getting the room settled before Bill got the bed out of the room. It was so good to see her - recovery was not fun yesterday. Now she runs around for about an hour making sure my numbers look good, getting my med schedule fixed so it doesn't keep me up all night, deals with the room temp, food, drink, blood work, and about a dozen other things.

This is actually from day two - note my laptop setup so I can entertain myself for hours by trolling people on Facebook. I mean, by watching movies. I watch movies.

 At this point I'm usually awake enough to start contemplating the placement and configuration of the swan cath in my neck and whether or not the dressing is going to last the week (it wasn't, so Sara and Johanna, another great nurse replaces it).

What you see here is the yellow cap on the end of the catheter - that's where the long, plastic wrapped yellow wire is coming from. This line is used to draw blood, administer some medications, and since the other end of it is in my heart cavity, it can measure cardiac output. So I only get stuck twice - once for the anethesia for the procedure, and then this other time in the neck. After that everything goes in and out through this tube. The part that looks like a gold ball is where it's actually going into my neck. The bandage around it is simply to keep it sterile and isolated - the one on my shoulder is just to keep the tube itself from flopping around. You would think this would be incredibly painful, but once the initial inflammation calms down, I can generally go the rest of the week without any pain meds at all.

This tube branches off into 9-10 other tubes that are used to administer heparin and saline drips, dopamine and delbutamine (the inotropic drugs that I am required to take to meet UNOS transplant criteria for "1A" status time), as well as several ports used to draw blood for labs and inject Bumex (my diuretic). The rat's nest of tubes and connectors all lead to here:

This is Dr. DooDaa. He follows me wherever I go. Each of the little boxes regulates the frequency and speed of the drips from the bags hanging from his hat so I don't get too much too fast or too little too slow. Or vice-versa. He also has a love handle in the middle that holds my phone, urinal, and the index fingers of nurses who don't get the stick the first time. 

I'm also hooked up to a heart monitor which allows the nurses to see my cardiac activity from four different points in the hall as well as at their station.

Meet Boing. So named because if one of those leads come off or he gets unplugged, he relentlessly boings at you until you put him back together. Like a car alarm except this one brings half the nursing staff running with paddles and Epinephrine injections.

Meet Four-Eyed Bob. Bob lives above my bad. He isn't very talkative but since I tend to dominate the conversation anyway, it works well for both of us. Bob may look like a four-eyed simpleton, but he and I have had some very deep conversations about things like stem cell research, artificial hearts, John Boener's freakishly orange skin, various mystery meats (he doesn't know what animal the jellied, gray patties come from either), and the meaning of life. Bob also doesn't like Bill very much, so we have that in common.

The other adventure here is the food, as I have made well-known to any who will listen. I'm supposed to be on a "low-sodium" diet and the cafeteria is apparently on a "low-cost" budget. They've solved their problem by serving sodium-laden, super-processed, and often unidentifyable food-like substances, but only giving the patients 1/3rd of a serving of said substances. While it probably keeps them under-budget, the patients are typically grossed-out and hungry, and I'm ashamed to admit, often so hungry I just eat the stuff anyway. I've tried fasting, food-strikes, and leaving helpful notes for the "chef" (my apologies for using this term to describe the individual responsible for this. You short order guys at McDonald's are heroes. Carry on). The hunger pangs eventually wear you down and you find yourself trying to decide if the marbled yellow meat tastes more like raccoon or kangaroo. Probably whichever is cheaper.

In order of appearance from left to right: Orange flavored corn juice, warm milk, egg "product" with reconstituted potatoes and some green things trying very hard to resemble chopped bell-peppers. Cold coffee, corn syrup molded in the shape and color of a blueberry muffin. Fruit cup with actual slices of honeydew melon. Salsa with what I'm sure is tomato paste and powdered onion as its base (since when does salsa taste like unsalted marinara sauce?) Oh! And the package of low-sodium. low-fat, low-gluten, all natural organic Mrs. Dash containing exactly 4 specks of pepper, a flake of oregano, and 3,000 mg of MSG. It makes your food taste exactly like it did before you sprinkled it on in great hopes that somehow it would increase the quality and flavor, when in actuality it only increased your chance of developing stomach cancer. Hoho, that Mrs. Dash is such a hoot!

How long can I sustain myself on saltines and Hershey kisses?

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.

Why My Facebook Movie Sucked

I avoided Facebook for a few years when it first became a craze. Frankly, there are people who I didn't want to connect with, and I certainly didn't care to tell the world What Was On My Mind because I was not in a good place.

Like most people, my relationship is love/hate. I love the way I can interact with distant cousins and internet friends who I would have otherwise lost track of because, let's face it, I'm not big on phone calls and its nice to be able to drop them a PM occasionally without having a two hour conversation. That may sound like a hollow relationship but it's better than no relationship at all and it often does lead to two hour phone conversations or longer emails.

I began to hate the way Facebook prompted me to share my every thought and life event with all those people. I'm increasingly uncomfortable sharing my life one out of context picture at a time to be seen by whoever wanders on for a moment that day, only to have it buried under commentary about Obama or Justin Bieber the next. Maybe it's my growing health problem, but I've come to value some of these candid moments in a way that makes me want to hoard them for myself.

Which leaves me without much to say on FB unless I engage in political and religious debate, and that is a temptation I am seldom able to resist. My views on those topics have come after many long and difficult years of testing them out, revising them, and even suffering for them. So I feel strongly. Conversely I am given to snarkiness and satire, which makes my posts about these topics seem flippant and reactionary when I intend them to be anything but. Since most FB readers have a short attention span, I'm forced to cram what should be a 30 minute discussion with context into a two sentence comment. It's a bad combination which has painted me as a grumpy, loud mouthed jerk who speaks before he thinks. If you know me in real life, I only tend toward that occasionally (I hope). I think about things deeply but am inclined to do so out loud when I haven't quite got it figured out. This is a formula for hurt feelings and arguments on Facebook.

So in trying to avoid all of the above I'm left with commenting on geek culture and short updates about my health. Boring.

So my Facebook Movie was put together last week, I presume, by the good folks who run the site. But when I watched it, it didn't look like a movie of my life at all. There were none of the heart wrenching failures and losses, none of the elated victories and sense of accomplishment that has characterized the last four years of my life. No mention of finishing The Phantom Tollbooth with two delighted boys. No coming to an understanding with a new dog that I struggled to accept because our previous dog left an aching hole in our lives that will never be filled. No memories of my boys tousled hair when they slept in too late, their joys and sorrows. No coming to terms with my anger about old grievances and finding peace. Nothing about the ongoing kindness of strangers in our lives. No victory lap for my wife and I as we conquered obstacles that would have landed most couples in divorce court. None of that was there. And that has been my life over four years.

Facebook got it wrong because I refused to share myself with it on any meaningful level. I wonder how true that is for my friends and family who are often put off by my FB comments that come from years of examination of what I believe to be true and real in this life, and far more experience than any 43 year old should rightfully have. There is no way to convey all of that in three sentences and a picture and is therefore easy to dismiss as ranting or emotion. Either way, that stuff isn't my life. It may define me to a point, but my life is so much more than anything I can fit on FB and more than I can express in the time most people give a typical FB post. It is more than a funny meme or a passing quiz to find out which Star Wars character I am (Anakin).

I guess that's why I have a blog. Maybe I should start using it more instead of trying to make empty points that don't come off as I intend in the first place. That's not a resolution but a realization that Facebook is what it is. And for all its immediacy and cleverness it still doesn't know me, and it's impossible for anyone else to know me through it. That job is still mine and I have to do better than memes and cheap shot political commentary. I promise I'll try.

P.S. Also that's not the music of my life's movie. How about In My Life by The Beatles or Right Now by Van Halen? Or even Live Forever by Queen, or Die With Your Boots On by Maiden? Wrong, wrong, wrong.

Final Destination

(Cross posted from Gypsyschoolhouse in case anyone missed it over there). 
 
It's been a turbulent few months in the Johnson household, to say the least. We've picked everything up and moved once again, one final time, to Lebanon, Pennsylvania, where the weather is mild, the trees are green, and the dairy is fresh. I won't torture you with the tale of woe concerning the buying of houses, the changing of jobs, the unreliability of moving companies, and so on. I would rather talk about how well the boys are doing, how generous our friends have been, and how we finally feel like we've found our home after nearly eight years of bouncing around, wondering where we were supposed to be.

When we left the ministry in 2006, we felt a bit lost, but we also had a new purpose in getting Christie through nursing school. When she graduated in 2009 we toyed with the idea of traveling, simply to see if there was a place out there that fit our family better than Arkansas. As much as we love the Ozarks, especially Bentonville and Rogers, we felt like we didn't fit in. We had made good friends and found a healthy homeschool community, but we were restless. If you've followed this blog, you know we hit the road in early 2011 to see what the world had to offer.

It brought us first to Harrisburg/Hershey, Pennsylvania where we immediately found friends in the homeschool community, and developed a lasting friendship with the Foley family. We hated to leave, but we felt that our adventure must continue, plus we needed to be closer to Boston. Two years later, we find ourselves back in Pennsylvania, five minutes from the park where we first got to know so many wonderful people, and eight minutes from the Foleys. This also puts us in proximity to a ton of farmer's markets, local dairies, great restaurants, Penn State (where Christie now works full-time and I will eventually have my heart transplant), libraries, shopping, bike trails, parks and so many other great things I can't list them all here.

Not our house - the view from the front porch nearly every day

As soon as we get a few more boxes unpacked, I'll put up pictures of the actual house. We're taking our time because there's a lot of furniture to buy, but one of the first things we did was paint the boy's room. I figured they've barely been able to hang a poster on the wall in years, so even though there are more pressing things to be done, I was anxious to finish this. Here's a small teaser, more to come:

Brennan wanted penguins and Rich wanted turtles, so we're making that work together, sort of...

More than the house, there's a sense of belonging, and permanency that we haven't had in so long that Brennan doesn't even remember it. Now he's immersed in a world of friends, playing, and a place he can call his own. At 8, it's about time.

Brennan with his buddy, Sean

It was clear that we belonged on moving day. Jilted by our movers, nearly 21 different people showed up to help us unload our van, including two neighbors whom we'd never even met!

Kirsten Waldron in typical non-plussed mode. Ron Ratcliffe considers stealing Dave's CD's, and Avery and Kate goof off for the camera.

Kristie Waldron, Stacy Foley, and Jen McCurdy. Triple Trouble.

Jake Waldron mugging.

Kirstin and Kate realize there are more of these shelves than any sensible person could ever use...

Mark is probably sick of Dave's books at this point, but soldiered on valiantly in the 97 degree heat.

The best thing is that we feel like we're finding our feet again as a family. The boys are happier than I've ever seen them. We've spent a lot of time playing games, at home and at the mall where a local bookstore sponsors a game night once a week. The boys even won some prizes there by participating in a Where's Waldo scavenger hunt that spanned the whole Lebanon county area.

Brennan wipes out Kate and the Ratcliffe girls at Snake Oil

Maxim teaching Rick to play Magic: The Gathering

At Infinitos at Rich's early birthday party

Brennan loves Eleminis

Rich killed everyone at Flame Wars

They found Waldo! Rich, Brenna, Kate & Maxim. Brennan won a $50 gift certificate to the game store, and Rich won a basket full of pet goodies for Blitz.

We've spent time in the park, read together at night, had game night, devoured the new season of Master Chef, eaten at our favorite restaurants a few times, and spent time with friends. It's the way life is supposed to be. The Foley's housewarming gift sums it up:

 

It goes to show that life never turns out how you expected. Here we are in Pennsylvania, in a beautiful old house that's a bit like us - a little beat up from the adventure, but still strong at the core. A little older, hopefully a little wiser, certainly richer for the experiences we've had. The hills of Pennsylvania, downtown Little Rock, a ski resort town in northern New Hampshire, a stone's throw from Long Island Sound in Norwalk, Connecticut. The metro area of Springfield, Massachusetts. And all the places we explored in-between. The coal-mine towns of Danville and Sunberry, the Poconos, Dallas, St. Louis, Raliegh North Carolina, the visits to Delaware, Baltimore, NYC, Hartford and of course, our beloved Boston. I am hard pressed to not cheer for the Red Sox and compare all seafood to Boston cuisine. Boston, small and over-crowded, opened its arms to us so many times when we were worried and despairing. Our first experience there involved a woman we'd never met giving us her phone number and address if we needed a place to stay. Boston opened its doors and its heart to us and will forever be "our" city. It's where our kids learned that people are the same, no matter what they look like or what language they speak. It's where they learned to explore food. It's where they rode a subway and a taxi for the first time. It's where we all learned that the world is so much bigger and wondrous than we'd ever thought. The doctors, transplant coordinators, and nurses that held our hands, patiently took our phone calls, and loved and embraced our boys call Boston home. It's because of their concern for us that we are where we need to be now. And of course, the friends and family along the way that have encouraged us so much. The Johnsons, Bostics, Foleys, our incredible cousins, friends like Lisa and Smitty who quietly pray and hope for us. For all this we are truly thankful. 

The fall brings new things - an attempt to cyber-school, new clubs and activities for the boys and our family, new friendships, and inevitably new food. These things that are the essence of life, more so than careers or money. We've believed that for a long time, but were often forced to re-arrange our priorities to get to this place in time. Now, it's time to live. And hopefully to laugh a lot more.

Here, There, & Everywhere

Hello all. Big News here, even for gypsies like us.

We've been semi-settled in Springfield, Mass since about October of last year. We like it here for the most part, but there is very little in the way of homschooling groups. The boys are involved in archery and fencing, but they spend most of their time on Skype with various friends from Pennsylvania, especially The Foleys (whom are featured in nearly every post on this blog starting in March of 2011). But honestly, it hasn't felt like home. Note the lack of blog posts. We've all been a little "blah" about the whole thing, but resolved to staying here because it's close to my doctors in Boston. This has been the goal from the beginning, due simply to the fact that my heart disease is rare and his case of the disease is unique in itself. We found no doctors elsewhere that were as knowledgeable about it than the Boston/Tufts Cardiology group.

At an appointment last Thursday, my cardiologist had a serious conversation with us about the road to a transplant, and several things had changed since our last visit.

Firstly, the team there has discussed the fact that as my heart failure progresses, I'm having little to no trouble because of the HCM. But because of the damage the HCM has done to my heart over the years, I am having very typical heart failure symptoms for someone in need of a transplant. In other words, the HCM aspect of my care isn't nearly as important as it was a year ago, though still an important issue in his care up until a transplant. I've been primarily seeing the transplant doctor at Tufts, while the HCM doctor is only consulted occasionally.

Secondly, the waiting time for people in even worse shape than me has risen dramatically in this region, and at Tufts, over the last six months. Without getting into the gory details, they informed us that people in my condition aren't even getting accepted to move up the list because there are more urgent cases that have been waiting, living in the hospital for almost a year now. Too many sick people, not enough hearts. Check that donor box on your driver's license, people. It's rough out here.

This puts us in a difficult position. We expected a lengthy hospital stay, but not 10-12 months. We live an hour and a half from Tufts, where I would be. It's almost impossible to imagine how that would work, not to mention the expense of the rest of the family travelling back and forth, hotels, food, missed work, etc. Our goal was to get closer to Boston, but honestly, we're as close as we can get before cost of living outpaces nurses salary. We love Boston, LOVE IT. But it's expensive.

We started exploring alternative transplant centers when we got home, looking specifically at Hershey, PA because of Penn State Med Center where Christie took her first travel contract. Again, if you're a regular here, you know we have a special place in our hearts for the Hershey/Harrisburg area because of the great experiences there, and mostly because of the wonderful homeschool group (INCH) and our friends, the Foleys.

Penn State in Hershey has a shorter average waiting time for people in my situation 3-4 months. This is not to say I need to be transplanted soon - I am still a "status II" and have to move up two more rankings on the waiting list to even be considered for a heart. I'm not sick enough yet. But when that time comes, the prospect of living at the hospital for 4 months instead of 12 is very appealing. There is a good transplant team there with a fairly strong background in HCM, according to the HCM patient advocate organization, my doctor at Tufts, and an HCM patient currently living there, whom I was able to track down. It isn't Tufts, the centrifuge of HCM study and treatment in the country, but it's good enough for what I need now. Better to have a good doctor who can get you through a 3-4 month wait, than the best doctor who has to get you through a 10-12 month wait. Plus the longer I'm in that condition, the more danger of problems with other organs.

So by moving to the Hershey area, we gain many things:

1) A shorter wait time once I get sicker
2) A big support system with the homeschool group that we still feel very much a part of
3) Being near our best friends
4) Christie being able to work at the hospital where I'll receive care, which should take the sting out of some medical costs
5) The ability to live within 20-30 minutes of the hospital
6) Closer to Philly, D.C. Baltimore, and North Carolina and Arkansas (just by a few hours) where our families live.
7) A slower paced lifestyle in a quaint little Pennsylvania town chock full of farmer's markets, old bookstores, community theaters, mom and pop businesses, great food...all nestled into the beautiful PA countryside dotted with dairy farms and creameries.
8) Cheaper cost of living, less driving to the doctor, etc.

Christie is assured of several jobs or contracts with a 30 day notice. Our landlord here has agreed to terminate our lease as soon as he finds new tenants. So we're about to enter that chaotic time of trips to PA to house hunt, packing up here, applying for jobs, and trying to replant ourselves. We feel confidant that this will be the last time. We can foresee being completely relocated by the end of May, depending on the lease situation here.

Life is funny. We've had a great time moving around, but the stress of my medical situation has made it difficult. We feel like a huge weight has been lifted from our shoulders in so many ways. It's scary to leave the shelter of Tufts because they have been an oasis for us after so many years of bad medical treatment. But overall this is for the best. At the cost of sounding whiny, we feel like we deserve a break. If there was one thing we could have (besides a cure) in this situation, it would be the go ahead to move back to PA where we have so many friends. We know Arkansas isn't an option because of the medical care, though we truly miss all our friends and family there. Honestly, PA has become our new home in a sense, the place we go for vacation, where we celebrate birthdays, and where we intended to settle post-transplant. We've been dealt a really crappy card of late, and it's like we were just handed an Ace.


You can read the Foley's perspective on this HERE. We're not sure who is more excited.