CF Happens

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

recent posts

  • Taboo subject…death and dying

    If the topic of death and dying is triggering for you, I suggest you don’t continue reading.

    A fellow CFer (in a CF women’s group I started) asked about end of life decisions as far as a living will and a DPOA (durable power of attorney) and what she should have in place. Her father is dying (not from CF) and as she’s by his side she realized she needs something in place for her wishes but didn’t know where to start.

    I wrote some thoughts in my notes app on my phone regarding death and dying a few days ago after watching a documentary on medical aid in dying (MAID). The point of this post is not the topic of medical aid in dying. I don’t feel the need to tell you my point of view on that subject because that is a personal thing and the way I feel is the way I feel. Anyway, that documentary got me thinking about end of life, in regard to my life with CF.

    My mom and I talked about end of life and death often because it’s my reality with an incurable genetic disease. Our family has never shied away from the topic. My mom had COPD. When she was visiting me in Tennessee the week before she passed away the topic of what she and I wanted at the end of our lives came up. Who would have thought that conversation would come into play a week later. She didn’t have anything in writing like a living will or a DPOA. My family listened to me when I shared what my mom told me her wishes were.

    I have a living will stating what my wishes are along with a DPOA stating who can make my healthcare decisions if I’m unable to make them. My CF hospital and my transplant hospital have a copy of both.

    POSSIBLY TRIGGERING PART: Now onto my feelings on dying in regard to my own life. Am I afraid of dying?? That’s a weighted question. Obviously I don’t want to leave this world where my people are and I don’t want them to have to live without me. I don’t want my loved ones to hurt when I die and they have to continue on without me.

    The fear comes in with not being able to breathe. I already get anxiety when I can’t catch my breath “easily.” It’s scary when I feel like I can’t get enough air in. This is the reason I take LOTS of breaks when doing basically anything physical. I usually push myself too far because I’m stubborn and don’t want to need breaks. When I have to catch my breath I concentrate on my breathing to focus on getting the air in slowly and not in a panic. This is my life with 30% lung function.

    I don’t swim anymore (aka go underwater). I either stand or sit on the stairs on the pool. haha The thought of being trapped” underwater and not being able to get to the top to breathe kinda freaks me out.

    Randomly I also have a little fear of throwing up and aspirating into my lungs (vomit going into my lungs). I feel like my body would try to instinctually breathe as I was throwing up because I am already hyper aware of my breathing. I’ve thrown up twice in my life that I can remember and that was when I was a kid. I hated every second of it! I will do everything in my power NOT to throw up. I lamaze breathe my way through feeling nauseous. I also have anti-nausea meds if I need them. I know that most people say “if I just throw up I know I’ll feel better,” I am NOT one of those people. haha

    I say all that to say, talking about death shouldn’t be a taboo subject. Talking about death doesn’t mean you are speaking it into existence. Death is going to happen to everyone. For those of us with chronic diseases, death due to complications from that disease is a reality. It’s not a fun reality but you can have a say how it goes. You can be in charge of it.

    I speak from experience when I say talking with your loved ones about how you want your end of life to look is worth it. Knowing what my mom wanted helped to make those decisions. If you have a hard time talking about your wishes with someone, you need to have a living will in place so you can put in writing what you want. Also you should have at least one person you can trust to fulfill your wishes if you are not able to with a DPOA.

    Losing loved ones isn’t fair. Living life without our loved ones isn’t fair. Death isn’t fair.

    Until next time…

  • CF Clinic…Nice and Early Today

    I had a lot to discuss at my appointment today, thanks to my body NEVER following the “normal” way of disease process. I’m good at making my doctors really use that degree of theirs.

    First stop was for pulmonary functions. For those not familiar with how lung functions work, here’s the process. When I do lung functions (just spirometry most times), I do 3 “tries/tests.” Usually my “tries/tests” get better with each one because my lungs open up more after forcing all the air in and blasting it out and another deep breath in multiple times. Although that’s not always the case. Today my best FEV1 was 0.81L/30%. My first try was 28% and my second try was 29%. My baseline (my normal) is around 32%. They were 30% in March and 32% in February.

    Next stop was clinic. My blood pressure has been running pretty high for maybe a year. It has definitely trended UP over the last yeah and a half. We have just been keeping an eye on it. Recently I’ve been having symptoms from the high BP for a 2-4 months now. I’ve always gotten headaches but my recent headaches have been lingering all day with nothing helping take them away. I’ll wake up to go to the bathroom in the middle of the night and have a headache and it will last all day long and not let up. They are not migraines, just a good ol’ annoying never ending headache. Another symptom has been increased shortness of breath, more than my normal, yay me. I’ve also been having more frequent acid reflux/blah stomach. The last symptom I’ve been having is blurry/unfocused vision randomly, not constant.

    The plan of action is starting a blood pressure medicine, lisinopril (ACE inhibitor) 10mg. Hopefully that will bring my BP down which in theory will make these headaches stop. I will be getting a MRI of my head to make sure we’re not missing anything with the headaches. We are also doing an overnight pulse oximetry to make sure my O2 sats aren’t dropping at night which could be a contributing factor to the headaches. That will be done through the home oxygen company I have used in the past. They will drop off a pulse ox and I’ll wear it overnight and it will record all night. We are also adding Pepcid 40mg (nighttime), on top of my daily Prilsoc 20mg (morning), to hopefully help the acid reflux to calm down and have no more blah belly.

    Now an update on my burning pain in my left thigh. The steroid shot in my left hip did nothing, as I expected because the pain is not in my hip. I follow up with ortho on Monday. I asked my primary doctor to refer me to neurology for an EMG (nerve conduction study) so that we can figure out if the pain is nerve related. I finally got that scheduled but it’s not until July 2nd. Ugh!

    I believe the timing of the onset of the burning pain and the reclast infusion is just plain coincidental and my luck. Also we found the labrum tear on “accident” with the MRI of my left hip and thigh. After thinking about it, the fall I had at the brewery in March may have been (probably is) when I tore the labrum even though I didn’t land on my hip. It may have just been the way I fell, trying to catch/protect myself. I didn’t really think about it being the case because the pain has always been in my thigh, not my hip. It will probably take up to 12 weeks for the tear to heal. So in my normal fashion, I can’t seem to do things “normally” when it comes to my health. Thankfully my doctors all know this is the case and they think outside the box when it comes to me.

    Lady parts update: I had a uterine biopsy at the beginning of April after my ultrasound showed a thick uterine lining while checking the size of my uterine fibroids. By the way, 10/10 DO NOT recommend having a uterine biopsy. That was maybe the worst thing/pain I’ve ever had. Luckily the pain didn’t last but the initial pain was HORRIBLE! The uterine fibroids are unchanged after being on the oral progesterone for 3 months, which was the goal. I’m continuing the progesterone. My gyno wanted to check the lining with it being thick. The biopsy was negative for any pre cancer/cancer. It did show “acute inflammation” and “necrotic debris” (dead tissue). My gyno called the pathologist to inquire about the “necrotic debris.” The pathologist said it was new to him. She told him I have CF and he said it shouldn’t have to do with me having CF. She is going to keep a close eye on me. I go back in 3 months. Once again, my body obviously does its own thing. haha I think that sums up everything.

    Until next time…