CF Happens

The first appointment of the day was a follow up with my neurosurgeon who did my back surgery in January. I had a MRI last Thursday to check for any residual tumor (tumor left behind after surgery) or any new tumors in my lower back. Nothing was left behind AND nothing new has formed! My surgeon said my MRI looked great. We will do another MRI in 2 years to check for any new tumors. Then as long as that MRI is all clear like it is now…there will be NO MORE MRIs…at least for this issue!! haha

He did say there weren’t not too many surgeries that he remembers for the simple fact that he has done so many over his many years of practicing. Well guess what? My surgery is one that he remembers. I said, “well I am memorable.” Or as my CF doctor would say, “it’s just Shannon being Shannon. (regarding my body not playing by the rules and not going by the text books…AT ALL!) haha. He said the reason he remembers it so well is because once he got into my back where the tumor was, it was much more involved than he anticipated. The tumor had grown through/towards the spinal canal (I can’t remember the exact words he used). Basically, the MRI was a little deceiving as far as where all the tumor had invaded and even its size. I told him I named the tumor, “Pete” because according to the dimensions on the MRI report it was about 20mm which on the tumor diagram is the size of peanut. He quickly said, “oh no, it was much bigger than a peanut. It was upwards of 2 inches.” Now that I’m home I just looked up different dimensions of tumor sizes. A peanut is about 20mm according to the information I found. An inch is 25.4mm and 2 inches is 50.8mm. So I looked at a few different things to give a visual because I’m visual person when it comes to this kind of stuff. A walnut is around 30mm and a lime is around 50mm. So needless to say, I’m very happy that the tumor is gone!!

He did say that had I left it alone and not pursued surgery I would have started to have some neurological symptoms with its size and the way it was intertwined in there depending on how fast it was growing. Well, good thing I trusted my gut and the ridiculous amount of sciatic pain and thigh numbness I was having. I knew something wasn’t right. It only took a year to figure out what was wrong!

Now onto the next set of appointments. Let me preface the next set of appointment updates with….I haven’t been feeling the greatest for the past week to a week and a half. I’ve been very tight, wheezy and short of breath when doing very little.

First stop of the afternoon was for lung functions. They take my height and weight every time I get lung functions. Side note: as the RT is getting my height and weight, she said, “wow, you are wheezing really bad today!” Yes, I am, thanks for pointing out the obvious. I try to ignore it, every day of my life. haha

There are 5 things taken into account when calculating lung functions…age, gender, height, ethnicity and weight. Taller individuals tend to have larger lung volumes (more room in their chest for their lungs). Weight/BMI is a factor because being overweight can restrict chest wall movement and reduce lung capacity. With all that said…I have come to terms that I have officially shrunk. I am no longer 5 foot 2.5 inches tall. I’m 5 foot 1.5 inches tall. Ugh. This is BS! haha. Oh and remember with the last few posts, I mentioned that I had lost a decent amount of weight from being sick and when I had my bad asthma attack and was inpatient for 8 days, etc. Well, let’s just say the weight I “lost” has been “found” and is back on my body…blah!

Ok, now to the point of the lung function part of this update…My first try was 27%. My second and third try were 30%. EDUCATION PORTION OF POST ALERT: Those percentages are for the FEV1 (Forced Expiratory Volume in 1 second), which is the measurement of quickly and forcefully I can exhale after taking a really deep breath and pushing the air out in that first second. We also look at the actual liters (of the FEV1) compared to what the predicted amount is based on the above 5 factors I listed above. Using the percentage is easier to tell people to help them understand where I’m at as far as how much of my lungs are really working in my favor (so to speak). We also look at the FVC (Forced Vital Capacity) which is the total amount of air I can forcefully and completely exhale from my lungs after taking the deepest breath possible. Basically if the FVC value in liters is decreased is a sign of a lung condition…hello Captain Obvious, my FVC is NEVER normal. haha.

Next up was the actual CF clinic appointment with my doctor. We both agreed that the increase in wheezing, tightness and shortness of breath is more of my really bad asthma and being off of prednisone for the last 2 weeks, than CF stuff at the moment. So we agreed on starting a low dose prednisone course. I will do 20mg for 3 days, then go down to 10mg for 10 days, then go down to 5mg and stay on that until I see them again in about a month. I have my pre-transplant yearly check up in about 2.5 weeks. My doctor wants to see what my transplant doctor has to say about my asthma being a big culprit to a lot of my crappy breathing issues in the last…well, what seems like forever.

People may be wondering why I’m not starting IV antibiotics. Well, considering I’m not coughing up anything more than usual (which I don’t cough much at all unless I force it out for a sputum culture), I’m not running any fevers and I’m showing any signs of a typical CF lung exacerbation…antibiotics would do nothing except for build up a resistance to that drug and we would no longer be able to use it in the future. With being very limited on what antibiotics I can take, we can’t take that risk.

We did talk about the newer CF drug (modulator) Alyftrek. We talked about what my thoughts were on it and what his thoughts were on it, for me in particular. It’s only been “on the market” for 6 months or so. I told him I’m not really sold on switching to it from Trikafta, for the simple fact that I didn’t gain any lung function when I started Trikafta. That is most likely due to the progression of CF in my lungs. My lungs are very damaged due to disease progression. Which is just how life with CF goes. I said it’s like the old saying, “if it ain’t broke, don’t try to fix it.” So as for right now I am not switching. I have many CF friends that have made the switch. It’s just not for me right now or maybe ever. My doctors and I have to make the decision that is best for me and right now that’s the decision we are making.

I think that about sums the day full of “fun” at the hospital. I will be updating after my pre-transplants appointments next month.

Until next time…