Chapter 7.5 Summer 2016

Alright Folks,

Here is the much anticipated Medical Update. (when I type that I hear the intro for “Weekend Update” from Saturday Night Live. If only I had Seth Myers and Kristin Wiig to read this to you.) It has taken so long to write this because we have been waiting for all for the test results to trickle in.

Since last Fall I have been experiencing a slow overall decline of my health. This was previously attributed to the fact that I had surgery three times last year, a Staph infection, a Norcardia infection, and a Pseudomonas infection in my sinuses all while desperately trying to finish up at the UO and working part time. I was tired and stressed out.

It turns out perhaps something more insidious was happening. Three weeks ago my FEV1 dropped dramatically and suddenly. My base line Total Lung Capacity (TLC) oscillates between 75% and 80%; almost overnight my TLC dropped to 38%. Not good.

(For the uninitiated: FEV1 = Forced Vital Capacity. This is one of the many numbers that is measured when you do Pulmonary Function Tests (PFTs), and is one of the key markers for monitoring signs of rejection in transplanted lungs. FVC = Forced Vital Capacity. Another number looked at on PFTs. FEV1/FVC = TLC).

My folks and I headed up to Seattle to my Transplant Center (from here on out Tx = Transplant. I get really tired of typing that over and over). I did three days worth of pulse dose steroids. This is where they blast you with 1000mg of IV methylprednisolone to beat your immune system into submission.

Let me put this is perspective; if you went to the ER with, say, a moderate to severe allergic reaction to poison oak they would give you about 40mg of oral prednisone. I was having 1grams worth injected directly into my veins. One of the many side affects of prednisone is insomnia; really what that means is being a cracked out jackrabbit. It’s like doing some meth and then adding a couple lines of cocaine just for fun. It also makes everything hurt. Fun times.

The IV pulse dose steroids are standard for when there is suspicion of rejection or inflammation in a Tx organ. After those three days you then continuous on an oral prednisone taper; decreasing from 1-5mg per day for a couple of weeks. (you have come off of prednisone slow from that high of a dose or else you will blow up your adrenal glands; mine are already fucked (I have Chronic Adrenal Insufficiency) so we don’t wont to destroy them twice over).

There are four main categories that doctors look at when dealing with an issue like this in a set of Tx lungs:

- Infection

- Rejection

- Gastrointestinal-Esophageal Reflux Disease (GERD) (a.k.a. Reflux)

- Post-nasal drip

What we have to do is go through these categories and try and pinpoint what is triggering this inflammation in my lungs.

I had a bronchoscopy up in Seattle and they took a bunch of samples and biopsies and tested me for everything under the sun. All viral, bacterial, and fungal panels, Tuberculosis, pneumococcal hemorrhagic fever something-or-other, aliens……

All viral, fungal, and bacterial panels were negative; so that means we can cross off infection.

INFECTION

The biopsies that took to test for rejection were also negative; so we can check of rejection.

REJECTION

That leaves us with SINUSES and REFLUX.

(Or some other mystery problem. I have habit of manifesting strange, unusual complications; I cannot tell you how many times I have had a doctor say “Well, I’ve never seen that before….”

Note: It is not a good sign when a doctor says this to you. That is the wrong kind of special).

We know I have sinus issues; I am having sinus surgery on August 30.

(All CF patients have sinus issues; just part of the package of CF). We also know that I have history of sever GERD. (Almost every Cystic fibrosis patient has GERD; again just part of package from the digestive disease of CF).

I initially responded to the IV prednisone great; after a few days my lung function began to go back up. I got all the way back up to TLC of 60% in four days. A significant improvement from 38%.

Besides from allowing me to feel better and not require as much oxygen use this response tells us a lot of important things:

1)   That type of response to prednisone is further evidence that I do not have an acute infection. (if you give someone with an infection that many steroids it will make them very ill).

2)   That quickness of a response indicates that inflammation is the primary issue in my lungs and this is easier to deal with than an infection or rejection.

The bad/interesting news is that as my prednisone dose got lower my lung function began to decrease. I had tapered all the way down to 20mg of prednisone and my TLC was at 40%. (down from the regained 60%). So we increased my prednisone back up to 40mg and I am beginning to show some improvement, although I am needing 3L of oxygen almost all the time still.

We are holding this dose of 40mg until tomorrow (Tuesday 8/23) when I will be repeating a full set PFTs and getting a chest x-ray. Depending on what those yield we will tweak the prednisone accordingly.

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The next step is pinpointing the source of the inflammation; which in all likelihood is sinuses or reflux or a combination thereof.

Tomorrow my dad and I will be heading up to Seattle for a week where I will have a weeks worth of testing before my surgery on 8/30. My mom will be joining us later in the week. We have a strange concept of “family time.”

The other half of Cystic fibrosis is digestive disease. Folks generally think of CF as a respiratory illness because that is most often the most severe set of symptoms and the part of the disease that kills you the fastest.

What happens in CF is that the pancreas is unable to make digestive enzymes which leads to our own special version of malnutrition due to the inability to digest our food properly and mal-absorption issues in the lower intestine. (meaning we cannot absorb the nutrients properly out of partially digested food).

This means I poop a lot. I don’t digest half the food I eat so it has to go somewhere! If you are ever hanging out with me and I disappear to bathroom for twenty minutes it’s because I am taking a colossal, and often very painful, CF bowel movement. CF poop smells particularly awful because of the lack of enzymes. I always apologize silently to the poor unfortunate soul who has to follow me in a public bathroom.

 I am also diabetic (which is a secondary complication from the pancreatitis from CF. I have Cystic Fibrosis Related Diabetes, it’s our own special version). The combination of CF gut disease and diabetes creates another problem called gastroparesis. This loosely translates from Latin to “slow stomach emptying.” This leads to a whole host of problems and causes a lot of dietary restrictions. (This is already a long ass email so I will forego the in depth gastroparesis explanation. If you are just dying for more medical mumbo jumbo let me know and I’ll give you the long version).

Since all of this began three weeks ago my gastroparesis has been really kicking up, and that is probably tied into the GERD and lack of oxygen. Shit just doesn’t work right when it doesn’t have enough air.

What I will be doing up UWMC this week is getting a complete GI work up. We are going back to the drawing board, if you will. to try and sort out what is causing what and how it is all tied together. We are dealing with a complicated chicken or the egg scenario.

I will be having:

- a 24h Ph study

-Mamometry

-Esophogram

- Bariun swallow

- Complete work up with the specialized GI team

- possibly and endoscopy

I am going to ask for a colonoscopy. (Because they are SO much fun!!! No, they are not. Actually colon and bowel cancer risks go way up in post Tx CF patients so I want to establish a baseline. The last thing I need is some version of ass cancer; I mean seriously I have enough problems).

It is going to be an exciting week.

Monday 8/29 I will have my pre-op with the ENT surgeon who will be doing my sinus surgery. This is planned as an outpatient procedure, but you never know. Since things have become so complicated they may want to admit me post-op but we don’t know yet.

I will keep y'all updated as we learn more. Thank you for all the love and support!