CF Happens

Sometimes in life CF (Cystic Fibrosis) happens…pun intended

recent posts

  • Tezspire…Ummmm….What the heck is that??

    My CF doctor, along with the CF clinic pharmacist, have been wracking their brains on what to do with me…in terms of my severe asthma that is. My severe asthma has been the main culprit to most of my issues in the last…hmmmm…well, forever…but a lot of this calendar year.

    First, let me explain what makes my asthma in the “severe” category. The type of asthma I have is eosinophilic asthma. This type of asthma is characterized by elevated levels of eosinophils (a type of white blood cell) in the airways, often associated with severe asthma. This is not your “run of the mill” allergic asthma, seasonal asthma or exercise induced asthma. The three types of asthma I just listed are the most common types of asthma, per se. Allergic asthma and seasonal asthma are triggered by allergens like pollen, different trees, grass, weeds, dust mites, animal dander, mold, seasonal changes, etc. Exercise induced asthma is just that, your airways and lungs get irritated and tighten and get inflamed as you exercise.

    When you throw CF on top of my severe asthma, along with the state of my CF progression AND the fact that I’m working with anywhere between 25%-30% lung function, it’s not the greatest combo to treat.

    SIDE NOTE: I AM allergic to animal dander, dust, mold and seasonal changes do affect me BUT that is not necessarily what triggers my asthma in every instance. Once again, this is what this ol’ body of mine likes to do…which is NOT what it “should” do. haha

    Ok, now onto the purpose of this update. I’m going to talk about asthma biologic medicines. They are an injection by a pen. They look just like what an EpiPen or an insulin pen looks like. Asthma biologics are a class of medicines used to treat moderate to severe uncontrolled asthma. They work by targeting specific inflammatory pathways in the airways, reducing inflammation and improving asthma control. Or, that’s the hope anyway.

    Many people have heard of, or seen commercials for Xolair. Xolair is one of, what I would call the “most common” asthma biologics. It is to treat moderate to severe asthma, more of the allergic asthma side of things. Theoretically, this particular biologic would NOT be one that would/should work for my type of asthma. I have NOT taken Xolair.

    The next one I’m going to mention is, Dupixent. Dupixent is to treat moderate to severe eosinophilic asthma as well as if you are dependent on oral steroids like prednisone. Meaning that you need prednisone to help your asthma be more under control. I’ve been on A LOT of prednisone over the last 6-9 months. I was on Dupixent for 1 year with no improvements or noticeable changes in my asthma and asthma symptoms.

    Next up is Fasenra. Fasenra is to treat severe eosinophilic asthma. I have been on Fasenra for the last year with no improvement or change in my asthma symptoms. If anything, my asthma has been more uncontrolled.

    So here we are with the meaning behind the title of this blog post. My CF doctor and the CF clinic pharmacist put their big educated brains together and decided the next step to try is the asthma biologic, Tezspire. Tezspire is to treat severe asthma. It is a newer biologic medicine. Tezspire targets a broader range of asthma types to include eosinophilic and non-eosinophilic asthma. Tezspire works by blocking TSLP (thyme stroll lymphopoietin) which is a key inflammatory chemical, to reduce airway inflammation in the broader asthma pathway. Ummm yeah, that was a lot of medical jargon, that I don’t even really know what those words all mean. haha. Basically, Tezspire is supposed to block the chemical that causes the inflammation in the airways/lungs in hopes that it will improve my asthma symptoms. Which would mean needing less to no prednisone tapers, improve my tightness, wheezing and excessive shortness of breath.

    I had my first injection today with no problems or side effects. This injection is every 4 weeks. I will get my second dose at my next CF clinic appointment on October 2nd. Then I will be doing the injections at home every for 4 weeks. The goal is to try the new biologic (Tezspire) for at least a year to give it the good ol’ college try. Fingers crossed this one helps me. If there’s one thing I would LOVE, it is for my lungs to not be so freaking tight and to not get so short of breath doing very little. I want to be the super woman that everyone thinks I am when it comes to getting house chores done or even running errands…WITHOUT having to take a million breaks to catch my breath. One can dream, right?

    Until next time…

  • Today Was A LONG Day Follow Up Appointments (Neurosurgeon/Back Surgery Doc & CF Clinic)

    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…