It’s been a bit crazy since last week’s update. Sometimes it seems like everything is working against you, but those who know me know I don’t really think that, though it may be the case for brief periods. This may have been one of those brief periods. Stay along for the end…

The Weekend

We had a nice evening off last Saturday with the boys sent packing for Nana and Papa’s for the afternoon and night. I took a 3-4 hour nap and we were getting ready to go to Busch Gardens to see Daughtry play live. We’d been looking forward to it for weeks.

As the day proceeded, I started feeling really tight in the chest. At 2 hours, we were eating dinner and I was starting to gasp. As much as I hated to say it, I wasn’t going to make it for our date. So with the boys gone, we watched TWO shows instead of one before going to bed at 9pm! Imagine that! Two whole shows and neither of us fell asleep!

We decided to not set our alarms for church. Of course, I got up at something like 4:11 instead of 4:03. Kristin was up half the night with her sleep issues that defy logic or explanation. We decided to just go to class at 11:00. I got a pretty prime handicap spot that made me happy.

We’d planned on the annual foster picnic with the boys as very possibly our last pre-transplant outing as a family. It was the usual good BBQ chicken and hot dogs for the boys and they played for hours. We got in the car and my wife had already decided to drive us home…

I put the O₂ on 3L or 4L (double) and tried to relax. I couldn’t. By the time we hit the interstate, about halfway home, I was gasping, restless, and frankly darn near panic. It didn’t get any better all the way home. I apologized and just came in and laid down on the floor inside the door while Boy ran up to turn on my O₂ concentrator. After a while, I insisted on 5mg of Prednisone. Finally, after about half an hour, things opened up.

We both realized that was it for half-day family outings.

That night, while sleeping in bed with my CPAP, I woke up in a complete panic, unable to breathe. I realized my full-face mask was preventing me from sucking in enough air after a coughing fit at 11pm. I made a decision to come downstairs and sleep on tank air. I woke up when the tank ran out at 4am and I went upstairs to turn on the concentrator (it makes a very loud beep). No more messing around with no O₂ at any point in the day. I’m officially on O₂ 24/7.

The Week

On Monday, my wife told my long-time coordinator how the day before went and we asked to see the doctor at my next clinic or move it up. She said he wants to see me but we’d keep my appointment on the 16th. I got squeezed in, so “plan on staying a while.”

Monday was SUPER productive. I got real, concrete work done and was feeling very much my old work self. My brain was working, I was making for a good time on social media, and Nana brought over dinner, ate with us, and put the boys to bed on Monday.

Monday at dinner, the same symptoms from the day before started, but all I’d done was sit at my desk all day! Another Prednisone, then we ate (I ate a lot), and I laid down on the couch and very much passed out. I missed everything until Nana was getting ready to go. I slept on the couch on the concentrator air and got a very good night’s sleep. I even slept through our toddler waking up 4x, crying down here, my wife watching 2-3 hours of TV, Boy coming down to say he was thirsty, and all of them eating breakfast. Have I mentioned I’m a good sleeper?

Tuesday was the same thing, minus my mother-in-law helping after work, but she did take care of the boys in the morning so I could work during my wife’s doctor appointment. Productive, gasping before/after dinner, Prednisone, slept on the couch.

Wednesday was a sick day for Boy with a stomach bug or food poisoning, so scratch that for normalcy except I migrated a site, got paid, and then slept on the floor from 10p-5a.

This Brings us to Today

Around 9:30 I got a call from the pre-transplant coordinator. She said my coordinator and the doctor were talking about my clinic visit and he decided to present my case to the medical review board this morning and they approved me for listing now. I’m still functioning and “healthy” but my lungs are at that point. It’s time. She’s submitting my listing authorization to insurance and I’ll see the doctor at clinic.

I still needed to get a clean bill from the dermatologist because my scrape biopsy came back “inconclusive” and I still had a big lesion healing from the biopsy. So I scheduled a punch biopsy (basically to cookie-cut it out and stitch me up) an hour later. Yeah, I rushed into town. I got 6 or 7 stitches and it looks like a shark came after me.

Next Week – Clinic and Feeding Tube

So the plan at clinic is to lay eyes on me, do a PFT to see where my FEV1 is at related to the 22%-24% I was at a month ago, and officially list me. I’m ready. I’ll probably be half-giddy by then after all of these weeks of leading up to this. We had a little denial, a big release to trust that’s where I am, then a push to delay it by gaining weight, and now the stark reality of what the situation is. I’m on a knife’s edge, plain and simple.

The next morning, I am finally following through with the doctor’s recommendation from his first meeting with me in November: get a feeding tube. I’ll be getting a G-J Tube. That’s a gastro-jejunal tube. It’s a tube that goes from the stomach to the first part of the small intestine with a small button access on the skin to be low-profile. This will allow me to get high-calorie drinks day or night without filling up my stomach and interfering with my eating that I do get done and enjoy. It will take all of the weight gain pressure off my brute force attempts and really pack on the pounds with Caesar dressing in addition to 6 or 8 360-calorie Boost Plus drinks going straight in every day. Do that math. 🙂

A fat me will be a successful transplant recovery mre. With listing now, I’m 100% going for the most successful situation we can muster, which leads into our last update.

Going Forward – Rest and Health

I wrote in last week’s update how difficult it was to get enough hours working. While it started to look like that was correcting itself this week, now with this news, family has told us it’s most important for Kristin to be in a good mental state of mind and me in the best rest and health state.

We’ll focus on learning the feeding tube situation to maximize my calories to get my weight up and thereby strengthen my lungs’ lives a little longer and get the rest of my body some reserves to pull from as my lungs decline more. In my limited work hours I am going to be releasing another theme (this theme) and expand a plugin I released last week.

My wife is focusing on doing things for the boys, staying healthy herself, and fixing high calorie meals and snacks for me. Some days, I’m just going to suck and fall asleep after dinner and she has to pick up the slack I create because I at least oversee picking up toys and the nighttime bottle time, which I do still enjoy that bonding time.

We could get the call days after listing or several months after. I’d like it to either be a couple of weeks or during early summer so my issues don’t affect school and create all the logistic items with getting to and from school when I’ll be admitted for so many weeks. It’ll be my longest stay in the hospital since before I was in kindergarten.

Thanks for reading this far.

If you already read the last actual medical update “Change of Plans, Again,” you know that my PFTs are still very low after finishing home IVs and that we are in the process of getting listed. I have already seen the dermatologist, dentist, and optometrist for their required parts. Having two fillings done today and am going to schedule a punch biopsy of the only spot the dermatologist found just to be sure and done with any possible skin cancer concerns.

Our script for getting Boost Plus was denied unless it’s provided as a medical benefit such as supplies for a feeding tube. I’ve been putting monumental efforts into gaining weight and am up 4-7 pounds by evening, but it’s exhausting me to do so… and we know Dr. Patel is talking more like gaining 30-35 pounds. So I’ve decided to swallow my pride and go ahead with a GJ Tube for night feedings and hopefully get 2200-3000 calories per night.

I’m a bit giddy thinking about gaining weight that easily, but I’m also very sad that my body has gotten to this point. I made this image back around 2010 at my highest weight and before kids. It’s laughable that I was stating that I was “easily tired – every day.” Ha! Insert note to future self there.

Eating has always produced results for me. Eating has more or less been enjoyable unless I was bulking. My appetite was never far away, and neither was my next chance to eat. That’s not how it works any more. When I’m stressed, I don’t want to eat. I don’t often have an appetite.

Ready

Once we finish our estate plan and see what my next set of vitals are at clinic, we’re ready to get listed.

I’m tired, exhausted, really. Never been so much in a state of perpetual napping in my life, even with all the newborns we’ve had. I’ve been getting a solid 8-9 hours of sleep per night and still sleeping anywhere from 1-3 hours during the workday.

Between my lower O2 saturation and all of the planning stress seeing all of my required doctor’s appointments and getting our personal and business affairs in order, I’m having extreme difficulty concentrating on work, let alone coding. I did successfully start coding yesterday. I wasted over an hour trying to sort out something of a junior front-end developer level and suddenly realized my error in using the wrong property… one that I’ve known for years but was glossing over.

I’ve got more than enough work coming and working on some long-term support contracts that I can do without long sessions at a time at my desk. Still need to get the revenue going because the work is there just waiting to be done… before people get upset by delays just because I’m napping and can’t think.

That about sums it up.

Today was my clinic follow-up from the hospitalization. Pretty standard procedure: pulmonary function test, weight, vitals, and a basic check-in how I feel. Things didn’t start off very well.

I went back for my PFT straight from the waiting room because they didn’t have a room ready yet, which isn’t a good sign for getting out in a reasonable time. This got me craving sushi from the cafeteria since I won’t be allowed to eat raw fish after transplant.

As a CFer, and old CFer, I’ve probably done 300 PFTs. I’m as consistent as the Amazon River is long and can rely on my numbers being rock solid. If they’re off, it’s because it’s a bad lung day and I know it going in. Today had been a good lung day going in and even sitting there at every point since. When I blew a 24%, so soon after my 27% test in the hospital, we were shocked. We were expecting a 28% and hoping for a 30% and wanted to consider that my new baseline.

A 24% was pure demoralizing. I sunk into my seat. Every seat thereafter, I sunk into. We sat in the room and waited for someone to come in who had experience and a say in things. When my epic coordinator of over 15 years came in, I knew we’d get the real deal thrown at us. “Numbers aren’t everything,” is something we’ve heard before and we heard it again.

We reviewed the past two weeks. IVs ended on Saturday, I finish the oral Cipro that I started Friday night tomorrow morning. The symptoms I had that justified IVs (sweating, loss of appetite, increased cough, lots of gunk, and wheezing) were gone and I truly felt pretty good this morning. On 2L/min of O2, I feel pretty good. I recover from activity faster up to 98% and don’t drop below 94%.

So, the numbers suck but my quality of life is back… and vastly improved on O2, if you can consider being on home oxygen an improvement over not.

The nurse practitioner came in and said she’d look into getting me some better portable equipment so I can take the small tanks to Busch Gardens (with the passes we bought after New Year’s thinking I’d make it through the year without any health issues) to be social, make memories, and exercise. She was perplexed why my numbers during evaluation were high and was going to ask the pulmonologist.

We agreed that my declining weight was my biggest vital that is still going down at a startling rate. I asked if we could get a script for Boost Plus and she said that’s totally possible, so she coded me for CF malnutrition and got a script for a case per day (180/mo). We’ll see if insurance allows that. We know from my CF Fatboy experiment in 2010 that gaining weight improves lung function, so we’re going all out with calories!

After a brief discussion outside with the doctor, he came in and laid it down real. I’ll paraphrase and such in blockquotes.

Forget the percentage on the PFT, look at the raw numbers. You were 0.82L FEV1 before IVs. In evaluation, you were 1.02L. Now you’re 0.86L. When you have a dollar and we take 5 cents, you’re still rich. If you have a dime and we take 5 cents, you’re broke. You are playing with pennies now. 1.02L, 0.86L, it doesn’t matter, your lungs are done. We need to keep you healthy enough to stay listed, so you need to gain weight.

If we had said we had everything with the home and business in order, he would have met with the medical review board and activated me on the list now. The business isn’t ready yet. I still have some appointments to meet listing requirements. But other than that, if he ordered us to list today, we would have.

Instead, we have a 2-month plan. We’ll do the Boost Plus for a month and come back for weight, PFT, and vitals with a nurse practitioner visit. If I haven’t gained weight or increased lung function, we do one of two things: have a G-tube placed to do night feedings and see if that helps my weight, or list me then. If I don’t gain weight, after one month, we’ll list me then.

So I either have 1 or 2 months before we go active on the list.

My primary job is to gain weight. My secondary job is to make a killing with the business and prep it for someone to run at least email while I’m on leave. That person also needs to be able to access client sites and help out if it’s something that can’t wait for me to be on the laptop in a working capacity. I have to have my eyes checked, get all of my teeth filled, and see a dermatologist.

Our process of preparation has begun already. I’ll write about preparing our business separately, but I did start signing up new things and moving services with an end-game in mind. Be that a failed transplant, missed transplant, or as I call it, “got hit by a bus protocol” because it could be any of our days any time we get in the car. I felt like this was necessary so my family would have some income for a while as they figured out how to sell the business or clients to my friends.

Family

The current status of the household is still simmering somewhere below freak-out. My wife is still a bit freaking out about me and the boys, I’m freaking out about the business, and the boys are freaking out about things feeling different. The good news is that our families have really come together to support us even while we adjust to the news and I adjust to life on oxygen.

For example, my sisters-in-law took the boys to the park Saturday. I could tell my wife was still stressing about the state of the house’s cleanliness after so many days of me in the hospital, so I sat on the bathroom floors and cleaned the toilets and sucked on O2 and listened to podcasts… and she felt much better about the house. Just as we finished, the girls texted and asked if we had dinner plans. We were going to have our bacon and orange slices night, which must have sounded pitiful (but we really do like that dinner) so they went to the store, fixed us dinner, and cleaned up and put the boys to bed.

Can we just say how awesome that is? Yes… it was awesome.

De-stressing

This week, our moms are here helping with the baby and making a family master list of things that will take stress and to-dos off our plates. My mother-in-law is our “agent” for the process and is spearheading the distribution of tasks to interested parties who can help. Her primary goal is for my wife to be of sound mind through the whole thing (since she is her mom’s daughter) and for me to be at my peak health possible (awesome that she cares so much). No wearing me out living our normal life allowed while I’m at such a physical disadvantage. I am to ask for help… or else. 🙂

She brought us dinner to fix last night and it was delicious. I was asked what my mental health days would look like. For me, that’d be putting a LEGO set together, going to see a Marvel movie if one is out or Redbox if it’s old, napping, and maybe coding for fun. My wife would have her mental health day on the same day and everyone would bring the boys back at night.

Then there are date days and sleepovers for the boys. Also tagging along for family days, like Busch Gardens, will help ensure nothing stresses us out and I can take as many breaks as I need. Doing things will become harder, but the boys need normalcy and having aunts, uncles, and grandparents along makes it more fun!

Shocked

We’ve had a very stressful past three years, but it seems that those were just warm-ups for life’s big trial. A few things have shocked me so far that I didn’t anticipate with my years of mentally preparing myself for lung transplant. I’ve known it was coming since 2006 when I dipped into the <30% lung function range after a drug trial placebo left me hanging. That’s a long time to prepare, but still…

1. I didn’t expect to see such clear damage to my lungs – visually – with a CT scan. I’m looking forward to seeing my old lungs in the lab after surgery, but to see their insides with cysts and mucus was a shock but made how they physically feel in my chest a much more real and visceral status going forward.
2. When we read an article about the new transplant pulmonologist arriving at Tampa General/USF, one thing caught us off-guard. In order to be considered for transplant, you’re not expected to live more than one or two years without this life-saving surgery. Yeah. No one said that in so many words to me before. “You’d be dead in two years if you weren’t moving forward.” My mind and heart wasn’t ready for that.
3. I didn’t expect to feel so discombobulated and wrecked. I’m strong. I’ve prepared for this. I’ve fantasized about it since at least mid-2010. I want it so much… so why am I stressed? Thinking it may have to do with the first two points, but I’m not sure.

Plans/to-dos

We’ve made plans for who can be here with the boys when we get “the call,” who will stay/transport them so my wife can be with me 24/7 for the first few days, and how people can help when I come home.

There are a lot of meals to prepare and freeze for her, a bag to pack and leave in the car, ensure our laptops always have some charge on them, ensure the cars don’t get below 1/4 tank, extra phone cables, and a hand-off plan for my business items.

Just inside the door, we set up a sanitizing station with masks and a hand sanitizer and I got a washable 1-micron mask for pre-transplant. Amazon has the post-transplant mask I want, but we didn’t go that extreme yet. Then there is pressure-washing the mold and mildew on the sidewalks and driveway and top-to-bottom cleaning the house so while I’m recovering it doesn’t take as long cleaning before I come home. I’ve got part numbers of air filters to replace.

Some people go through this with a significant other and maybe a parent or two, but we have eight family members ready to step in and a church group asking how they can help. With so much family, we’re actually thinking of gaps that our plan has to give them an opportunity to help.

It’s good to be so well loved.