That photo above is CF Fatboy. He weighed 135lbs in June or July 2010. This was on a California vacation. Here I am now. Shocking. 105lbs – keep in mind I’m 5’4,” though. That’s what CF is doing to my body at this time.

The long and short of this week has been that everyone in the house appears to be sick except for me. Boy brought something home over a week ago. I took him to Wednesday church, but that was the last day he was acceptable to be with other kids. I started massive toddler nose-wiping on Friday and moved the hand sanitizer next to me and the Kleenex box. By Sunday, he wanted to learn how to use the hand sanitizer. Rub, rub, rub!

We skipped Easter services – my wife went alone on Saturday evening and I pulled up the live service Sunday on my iPad and used AirPlay to watch on the TV. Unless there’s a hospitalization as a kid I’m not remembering, that was my first missed Easter in person.

Feels like I should have been getting new lungs for that personal record falling. #grin

Clinic this week

We head to clinic on Wednesday morning. They need to see me monthly now for lung function tests and a new tissue type blood sample. I’m sure I’ll cough up something that grows a need for more oral or IV meds. Ideally, I’d like to see a more or less stable situation with a 1-2% point drop in lung function, just to keep the need real.

New home concentrator

We’d noticed an issue since the second day we got an updated concentrator. We asked for a new one because the first one was too noisy and the beep it made when it turned on was like a shriek. The replacement wasn’t too much quieter but the beep was still loud but at least half the volume. Well, this one would start at 3L and drop to 2L within a couple of hours, and after a while, it just gave up and would be running 1.5L or less after 12 hours. I started getting headaches again and using tanks for couple of days.

We got a replacement that is the same model, but we can crank it all the way to 5L and it holds steady. The next day my headache was gone. I’ve since upped my home level to 4L and I run a pretty consistent 96%-98% saturation now. I run 3L when we’re out on a large tank and crank it to 5-6L while walking from place to place.

Exercising

Being listed for transplant, you sign a contract with your Center to be 100% compliant with your treatment plan. They told me to be sure I keep up with exercise because after surgery, you aren’t able to push yourself up from a chair with your arms – you have to do a seated squat to rise.

My legs, while they have lost mass, have not been my problem with my decline, as my recovery will show everyone, but I’m still compliant.

My wife walks two neighbors’ dogs for extra money, so our plan was for me to join her each day at 11:30 to do the 1/4 mile walk to our first client’s townhome & have me grab our mail from the community mailboxes and catch my breath.

Walking there was fine. We even talked halfway there on 5L/min (I can’t believe I’m saying this next part). Then I stop responding because it’s time to breathe for me to get there. Yeah, no talking and walking.

On the way back, I was already silent starting out. 2/3 of the way home, I felt my saturation levels drop, my legs got sluggish, and I looked for the closest spot and said, “Stop. Catch. My. Breath.”

“You can’t even walk home? Stop sucking,” she joked (yes, she’s allowed to joke this way without hurting my feelings because I’m putting strain on her).

After 60-90 seconds, I felt my head return to normal and we strolled along, hand-in-hand now.

In our driveway, I let her go ahead to open the door and I staggered and paused in the drive before deciding to go inside instead of taking another break outside with my tank.

So after 2-3 weeks of difficulty after my feeding tube surgery, I’m back in business and back doing business. It was a good week, especially since I didn’t get sick, even though I didn’t feel well enough to go to Easter services. I did make it on Wednesday night, which ended up being more walking than the previous Sunday morning.

Meta data and the incredible database

Warning: this is very geeky and technical, but I think I explained it well enough for anyone who is interested in the donation waiting list.

Because I’m such a geek, I wanted to spend some time illustrating how cool the waiting list database is. If you recall a couple of years ago when the whole Edward Snowden thing broke out, everyone was talking about meta data. People were saying things like, “Why do I care if they know call times, duration, incoming and outgoing numbers, and some GPS data? It’s all anonymous.” Yes, a lot of data is anonymous, but with enough data, you can create a fingerprint or portrait, no different than putting together a puzzle that is unrecognizable by looking at any single or small number of pieces.

But this isn’t about call monitoring, it’s about the date of listing, organs, transplant centers, blood type, age, LAS, disease diagnosis, etc. With enough of those data points and enough filtering, you can find specific people. Another set of data would be just as effective for me, such as: age, gender, marital status, car situation, home situation, city, and career industry (employer would be a VERY small set). I doubt there are any other 38yo married males with a 2000 Altima living in a townhome in Tampa who is a WordPress developer. Very unlikely.

So about 14 days after I was listed, I ran a report on the database. It showed me that at Tampa, there were only 14 people waiting for lungs with my blood type. However, five were inactive, so that leaves nine. Four had a lower LAS than I do and I was grouped with four. One had a higher score category.

When you read down a little further in the report, you see them broken down by wait time. There was only one listed fewer than 30 days: ME. I ran this report with the LAS reporting so I could verify that the person in that time bracket had my score. Verified. You can click or tap the image to zoom in or get a full-size image.

Today is the day we’ve been waiting for ever since: the day I’d roll off the <30 bracket into 30-<90 days. You’ll notice in the first report that there was one person with an LAS of 30-<35. I played around with building the report different ways and found a nice, concise format.

Sorry for the size, but I wanted to preserve the query and date at the top instead of the most narrow view.

If you want that, here it is. Within the 30-<90 day range, there is one person, they have an LAS of 35-<40, and they have cystic fibrosis. Hmmm, that must be me since there is now NO LONGER anyone waiting for <30 days. You can also see that the 90 days – <6 months category is gone (because it’s empty) and the next bracket gained a person in the same LAS range as the one who left the 90d-<6m bracket  from the previous report period.

Unless someone is added to my blood type for lungs in the next 60 days, I’ll be alone in each bracket until the 6 months to a year bracket. I hope you enjoyed this data half as much as I do.

First of all, I wanted to thank you all for your incredible generosity to my medical leave fund. It’s been a really significant relief of several burdens, including stress from my to-do work list, earning enough for our house and business expenses, and guilt from napping when I’d normally be working. Interestingly, unlike our adoption donations, the vast majority of help has been $25-$50 donations with many, many people I don’t even know who they are. Simply many giving people from the WordPress community who are giving to a fellow contributor to our livelihoods.

At the time of this publishing, the count-up timer reads exactly 25 days, 11 minutes on the active list.

Despite all of that napping and resting, I finally finished (after more than two years of delays because of client projects) the first theme I started for sale. It’s actually the theme this site is running on, so if you run a self-hosted WordPress blog and like how this site looks, you can have the theme on your site, too. So for those family and friends who know me from years ago but are far away, that’s what I do now. I’ve been running my own WordPress digital agency for over 8 years. I’ve been spending the last 2-3 years transitioning away from client work to products and educational courses in long-term preparation for my transplant and the unpredictability of life without an immune system afterward.

Yes, I’ve been planning for this time for years… but life threw us so many curveballs (and blessings in the form of two happy and healthy boys) I just couldn’t get it all done in time.

Family and friends

We’ve had lots of help at home recently. Nana continues to come over to clean and cook us a meal or two per week, as well as periodic school runs, watching the boys, and staying until bedtime so I don’t have to baths or bedtime routines. They did some major de-cluttering of our one-car garage last week. I can now dump my wheelie O₂ tank out the passenger seat and pull it alongside the car to the door now.

The weekend before this, Nana and Papa got cedar flowerbed edging and put them in and re-did our flowerbeds. With Papa’s recent pressure washing of our walks and drive, we have curb appeal for the first time in our 9 years here. They’ve also deep cleaned both of our upstairs bathrooms with bleach and caulking so there is no possibility of mold to get to me.

This weekend my dad hung the entry light we had in our apartment for me. I gave it a shot a couple of years ago but it was a tough situation. It took both of our brains to figure it out because you’re working blind to get the screws into place, but he finally got them in and we love it. Then we did a Home Depot run for one more section of garage shelving to finish getting stuff off the floor and mini-fridge. He drilled all the holes I marked and sawed the boards because we got tired of waiting for the board ripper person to come help us at the store. My wife immediately filled the shelving up with the outdoor toys and beach toys.

Our church family keeps asking how to help but we’re truly at a loss pre-transplant. Our moments of greatest need are getting ready in the mornings and getting the boys fed and to bed, but everyone has kids to do that with their family, so we’ll probably be holding off on their help until after I get the call and the boys need meals. We’ve already got my wife’s meals planned for the hospital and Nana and her sister-in-law will probably be her main source of food while she’s still tending to me so much when I come home. Transplant experiences after discharge vary so greatly, we really don’t know what to expect as far as my abilities, restrictions for healing, or my pain/anxiety/hallucination levels.

We all made it to both hours of church yesterday. I learned walking in that 3L/min is NOT enough for walking that distance any longer. I used 4L to walk to class for second hour. Still not enough. Walking out, I bumped it to 6L and that worked, but it’s really blasting up my nose at that level and feels like a hot poker. I suppose it’s something to get used to. We took a walk today and I used 6L again and did pretty okay for my half-mile.

Oxygen, antibiotics, and feeding tube

In all of this going on, I’m pretty at ease with the vast majority of it. That’s definitely an advantage to be had from having so much advance knowledge this was the ultimate “treatment” for my cystic fibrosis. That said, some things are getting old quickly or are some level of frustrating and discouraging. I need a little rant, partly because my family is done hearing it and because it shows that I’m okay with the big issues and that it’s the little things now that are getting to me.

First to resolve is getting off this 10-day round of antibiotics. I’m getting up at least 3x per night and not getting enough sleep as a result. It’s really messing with my night feedings.

We ramped up to full volume and rate on my feeding last night and it’s really not comfortable. It ran from 8p until 4:20a and I felt uncomfortable and was burping intestines for an hour after waking up. I can’t adequately convey how much I hate that. That didn’t happen with 2 cans of formula and it doesn’t happen with 3 cans at full rate so it finishes early enough to keep sleeping after I disconnect and flush the button. My most recent frustration was a discovery in the car on Friday. I can’t cross my arms while relaxing with the feeding tube button placement. Unless it’s completely touchable and movable at some point, it’s out of the question.

Our home concentrator runs 24/7 while I’m home. It’s supposed to be rated to 5L/min. If we leave and come back, turned on all the way, it gets to 4L for a while, but slowly creeps down to 2L, which is too low for me now. I’ve been waking up with headaches again. We last turned it on after church yesterday and It’s now right at 2L. So we’ve got to call and see to getting a new unit. It’s silly to mention this with the litany of real issues, but we’re all the way done with this 50′ hose. It snags on corners, stools, pillows on the floor, kids’ feet, gets pinched in the baby gate, has to be maneuvered to go up or down the stairs, and is an extra tube at night with the tube feeding. The call can’t come soon enough.

It’s now been almost 3 weeks since my feeding tube surgery and I’m almost used to it now. My pulled rib muscles have eased and the nerve pain is gone. Now it’s strictly the passage (stoma) itself that gets a little irritated with jostling or big coughing fits… yeah, stomach acid burns. I even made it to Boy’s baseball game on Saturday. Wore my backpack oxygen instead of dragging my tank on tiny wheels! We swung by Redbox on the way home and I got Dr. Strange for my first viewing of it and my wife went to a baby shower and hung out with her sister until late while Nana and Papa had the boys until half an hour after she got home. I watched the whole movie and all the extras and really recharged my geek batteries.

When clinic called

I was having a rough energy day on Monday that prompted me to step away from my desk before lunch and grab a few winks. Then I repeated around when school was letting out. Right before everyone came home from school, my phone rang. It was the clinic number. Of course, now this incites much involuntary excitement but I’m pretty sure it won’t be a clinic number calling – hospital prefix, maybe, but not clinic.

It was my new awesome pre-transplant coordinator saying that I cultured strep and they wanted to put me on a round of oral antibiotics. I hadn’t been feeling the best for the past 3-4 days but I’d attributed it to pushing it at my desk too hard. I guess we can see now how a raging lung infection affects me with 26% lung function. It doesn’t feel great, but they clearly caught it super early since I didn’t call them yet. This made me feel super safe with them going forward post-transplant.

Now I’ve had 4 doses and am feeling considerably better (which is odd to say since I’m still on O₂) than I did on Saturday and Monday.

Making new friends

I’ve made quite a few new friends on Facebook since I re-joined to make our secret page for family and friends. I joined a global lung transplant group and one that’s just for my hospital. Having a lung transplant is starting to feel like an initiation into a super cool club. I guess the evaluation and waiting on the list is their idea of hazing. 😀

While I felt really grateful to everyone in the support group we visited recently (I forgot to mention that), they were all seniors and none of them had CF. Their knowledge and care is evident and I’ll be sure to tap it as long as I can and still live my life with our family, but there is something missing in camaraderie when there’s a 30-year age difference and they were faced with sudden transplants because they hadn’t lived with CF their whole life. Well, then I found one.

In support group they kept mentioning one young guy who came, got his new lungs, and stopped coming to go live his life and I found him when his mom answered a question for me. I read his whole story from listing to now, at my hospital, living life in the Tampa area. I can’t put my finger on why, but reading his story and seeing his photos at the actual hospital I’d be at and dealing with CF after transplant really put things at ease. He just celebrated his 1yr 2nd birthday, as we call transplant day, on March 31st.

Some interesting facts for the readers here:

• His lungs were put in from the back. It’s called “getting your wings” among lung transplantees.
• He gained 23 pounds in 2 months and was blowing a 94% lung function.
• 33lbs ater 5 months and 102% lung function.
• The docs discharged him after only 11 days after transplant!
• He felt so good, he went to a Lightning hockey game a couple of days after coming home, but that didn’t end well, so I’ll learn from his example.

New stuff

You may notice that I put a ticker in the sidebar set to the exact time we got an email from the coordinator that I was live on the transplant list.

I found myself using meta data and searching the national organ list database. I’m the only person at my hospital listed in the last 30 days, so it was pretty easy. Yes, I’m that geeky.

I’ve created a new segment in my CF Fatboy MailChimp account so you can subscribe by email for updates to this site apart from Facebook. I don’t believe you can get multiple emails per day if I post more than once per day. I’m going to set them to go out at 11am every morning to hopefully ensure the doctors have done their rounds and I’ve done any morning stuff the nurses need me to do. The form is in the sidebar.

These posts will almost all be from me, but my good friend Ben in North Carolina may do daily updates here based on the Facebook statuses and keeping in contact with my wife. I don’t want her feeling like she has to post to too many places because I’m in infoholic. As soon as I’m able to post (and not use the force to close windows on my MacBook) I will be. No multi-day lapses here, folks! I’m ensuring full coverage, as it’s very important to me to document this for me and for anyone else coming along after me to know the whole deal.

I leave you with a video of me trying to use The Force while on muscle relaxants and pain killers after my feeding tube surgery.

It’s taken two full weeks, but I’m more or less back to the baseline I was at when I got listed… the day before I got the feeding tube. While I can’t say I’m pain-free, the nerve in the ab muscle that they desecrated with the tube has died off and isn’t sending me over the edge without massive drugs. Still tender and the hole/tunnel itself is very tender, but it’s massive progress that’s unmeasurable compared to when I came out of anesthesia.

After our last steak night I posted about, we ate the two aged bacon-wrapped filet mignon we bought the same day. Oh my! IF that was my last rare steak ever, I’ll be a very happy man it was that steak. I got my wife’s to a perfect mid-well to well-done, just how she likes and mine was delectable. Shoot, I was going to make it the featured image but that’d look weird on the homepage, so enjoy these.

Tube feeding

As a result of learning our lesson with the heavy meal and then tube feeding making me feel awful before we went to feeding tube clinic, we skipped feeding for the steak and for a couple of days while I ensured that Cipro was not an influence on anything leaving my body. We doubled my super pro-biotics that really made a difference for me when we switched brands and I was beyond normal within 48 hours!

Two nights ago, we resumed feeding with 2 bottles instead of 4 (500mL) AND decreased the flow from 120mL/hr to 60mL/hr. I didn’t get more than a couple of winks of sleep but I was only mildly uncomfortable, though my burps still felt like they were coming from well past my stomach. I also felt like the formula was going to come right back out as it went in, but I was wrong, so it was a big win! I was seriously happy.

My biggest issue with the night was a result of the equipment we have. The food bag has a very short tube – like 5 feet long. Once up on the pole and connected to the pump, I have about 18″ of play when I sit in the leather chair in the living room. I didn’t even feel like it was enough to be safe if I rolled over if we pushed the coffee table out of the way to sleep on the leather couch. However, the leather is the other issue: I’ve been a nuclear body heat sleeper my whole life and I just sweat like crazy any time I nap on our furniture.

Before we loaded up the food last night, we took a moment to lower the pole so the pump is <2 feet off the floor so I could sleep on the floor on a blanket and use my fluffy pillow. I slept almost all night! We used 2 bottles again but set it at 90mL/hr so it didn’t finish after 5am. It woke me up at 3:00am beeping that it was done. I flushed my access tube, closed the button, took more enzymes, and slept until the baby woke us up at 5:45am. I didn’t have any nasty burping or feeling like it was running through me, so I’m greatly encouraged to continue night feedings.

Work and business

It was a good and bad week. Lost a client whose site I was working on since January right before my hospital stay that got my evaluation process started. He didn’t want to “get mixed up in my personal business.” I was absolutely floored by this reaction since he was about the 10th client I’d told.

I moved on to the people who do love my work and quickly got their stuff back in motion. I may have overdone it because I felt like I was about to lay down sick at the end of the day yesterday, but I got enough sleep to fight it off. This morning I finished a large set of code blocks and image editing for my favorite client and I feel very accomplished with that feat… so I’m watching movies at my desk now.

To end my week, I got a big lift in spirits. My hosting company, WP Engine, sent me a gift basket from Harry & David yesterday. To receive such a thoughtful gift from the founder, CEO, support lead, and marketing lead, who are all dear friends since 2013… it almost made me cry.

Still waiting

So with everything basically where it was 2 weeks ago, plus 1,000 calories at night and a lot of care to protect my belly from the boys, life’s moving on and we’re just waiting for the call. Every phone ring brings excitement and disappointment. I feel like I’m missing out on everything (like Boy’s first baseball game of the year and I still haven’t seen Logan yet), but there will be plenty of living large soon enough.

It will be best for everyone involved if this happens in April, so let’s do this. I promise to not do an April Fool’s about the call. If we say it, it’s actually happened.

It’s been a rather miserable week that just started to get better today, so for that, YAY! Small victories. Yesterday, we really “embraced the suck.”

The house was a mess because it was Wednesday night church and Boy needed to get out and do something and everyone came home exhausted. There was so much to have done before school and the boys both got up at 5:30am. I was useless. Kristin was just taking a beating from taking care of me, me not helping (lousy loser I am), and taking care of both boys. So I went behind her back and called in the cavalry and Nana was over here whipping the kitchen into shape before we even left for my dermatology appointment. When we got back, the house was clean and I napped and they got stuff done, she did school pick-up, fed the boys dinner, and I made us a nice steak dinner while the boys were upstairs.

I was so darn proud of myself taking care of Kristin just a tiny bit, I confessed before bed that I went behind her back. “Thank you.”

Thank you, Nana. It made all the difference.

“The Tube”

So many of you know from Facebook that the pain from my procedure has been unreasonable. Nurse friends and CFers who have feeding tubes were concerned, so we went to interventional radiology (IR) clinic this morning for them to put eyes on the issue and consult us what they thought the pain issue was… but then last night was horrible.

As part of having an invasive procedure done, I was put on Cipro. We knew before taking my first dose that it does not sit well with my stomach, so I started the anti-nausea meds with the first dose. Amazingly, with my round of IVs before getting listed, I got through all of them with my digestive tract intact. ONE day of Cipro (and the next 2 weeks) ruined me for over a month. I don’t like it, Sam I am.

So that’s where we were the last two nights with Cipro… and we’d had a steak dinner and I was feeling full. All. Night. Long. I started to feel sick to my stomach. I lost so much water overnight from Cipro, I decided to start chugging Arizona fruit punch, then apple juice, then sweet tea. By the time everyone was awake, I was sitting very still so I didn’t lose it. Nothing was emptying out of my stomach and I was filled to the brim with dinner and drink. It wasn’t a fun ride to downtown but I made the most of it with as much hilarity as I could muster.

We walked in the clinic waiting room and I told them my last name. I got a warm greeting by memory and she said she’d go get them to bring me back. We only sat for about 60 seconds in a room of waiting people. We had no idea what they were going to say at IR – “you’re a wimp, this is a painless procedure,” “you’re infected,” “what is CF?” A surgeon in scrubs walked in within about 10 minutes…

Instead, the surgeon agreed the suture was buried in my ab muscle and digging the t-bar into it and causing a lot of pain. “So we’ll cut it today.” There was bruising. There was some tenderness but it wasn’t infected and was healing, though slowly. Then he let us ask him 20-30 minutes of questions about what was going on with the healing, the issues this week, the issues overnight, and future care.

In the end, he suspected that the procedure needle and coring (to get it larger) pierced a nerve in the skin. It can also go through a vessel and take weeks to heal. It’s a crap shoot, plain and simple. I think I also lost the bet on one of the sutures, too. We also learned that the J-tube leading from the button to the jejunum is 45cm long and is the diameter of my oxygen hose: 18fr or 6mm. I thought it was tiny like the pH probe I had for a couple of days to monitor for GERD. We also learned that they replace the whole get-up every 2-3 months, but it just takes a few minutes. Hopefully I only need it replaced 2-4 times before I’m up in weight and have healed from transplant and won’t need it any longer.

After we explained we had the heavy steak dinner because I won’t be able to have undercooked meat (I eat my steak mooing), he said he’d do the same thing. “‘Merica!” I said. He nodded, “‘Merica,” and smiled. We liked him a lot.