Surgeries

The incision on my stomach was pretty big!

My first surgery was at 2 weeks old. I had a pyloromyotomy (say that 3 times fast, I dare you) to widen the opening between my stomach and small intestine. The official term for what I had is Pyloric Stenosis, a blockage of the opening, due to a thickened pyloric muscle. Considering stomach/digestive issues associated with CF, it's surprising this is completely unrelated to it.

My parents were in Casper visiting family and showing off their adorable new baby, and I was having major issues with projectile vomiting. My mom took me to the doctor to have it checked out and found out that all 6 lbs of me would need emergency surgery. I do need to mention here that the doctor that diagnosed me with this is the same doctor that will 5 years later diagnose me with CF. Shout out to Dr. Green and my mom for essentially saving my life, not once, but twice!

Now I can't image that sending your new baby into surgery is a walk in the park and I was so small that they had to put the IV into my head. After surgery my mom was able to see me and the IV had slipped and started filling the space between my skull and scalp with fluid. She tells the story much better than I can, but needless to say she was a little shocked at my new alien-like appearance.

The surgeon wasn't exactly delicate with their incision or sutures so I still have a pretty distinct scar on my stomach. I'm told that it looked like a caterpillar because of the way the sutures stuck out of my stomach and to this day it still looks like a giant bug.

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Luckily I was able to make it several years without having to go into surgery again, but my second major one was just as unplanned. I was 25 and living on my own in Denver. I hadn't been feeling well and started to develop severe pain in my abdomen and wasn't able to keep much food down. At this point I hadn't really been seeing my CF clinic on a regular basis, so I went to an urgent care clinic. After a blood test (high white count), vaginal ultrasound (maybe it was ovarian cysts), and a CT scan, I was sent to see a surgeon to take out my appendix. They also saw a spot on my liver in the scan so I had a liver biopsy done at the same time.

A week after having my appendix out and still not feeling any better, I got a call from the surgeon that my tests came back negative for appendicitis, which was very frustrating to say the least. I called my CF clinic and explained everything to the nurse, and in a heartbeat she said that it was DIOS (an intestinal blockage) and made an appointment for me to get that treated. She was correct, and that was my hard-learned lesson to always check with my CF clinic first.

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My last major surgery was a few years later to have my sinuses cleaned out (scraped) along with a septoplasty and shaving down of my turbinates. Sinus issues are a big part of having CF and most CFers get this surgery every so often to clear things out. Incidentally my non-CF sister had to have this same surgery, so it's tough to say if mine is fully CF related, or if I'm just super lucky.

This surgery was quite possibly the best thing that I've ever done. I was having daily debilitating headaches by the time I had it, and though I still get sinus headaches, they're nothing like they were before. Every few years I get a CT scan done of my head to make sure my sinuses are still clear and luckily I can say that they are. Most of my sinus problems now are just due to swelling, which is why I use a nasal spray every day.

Interesting side note: I found out that I don't have a sinus cavity above my right eye at all, it just never formed (insert hard-headed jokes here). One way that chronic sinus issues can be treated is to obliterate the cavities, so I'm partway there naturally!

New Habits

How do you change 30+ years of habits (or lack thereof)? How do you deal when one day everything changes and you have to say to yourself “Ok, this is me now. This is my new reality.”?

I know I say this a lot, but I’m going to say it again. I’m really lucky with my CF. I know that I am and I can’t even express how deeply thankful I am for it. Most people with CF don’t get to live the essentially normal life that I’ve been able to lead up to this point. But right now I’m feeling like it is coming with it’s own set of drawbacks.

The Vest & nebulizer corner where I
spend time every morning & night.

If I had had to do chest therapy for my whole life I’d be used to it. It would be part of my daily routine that I wouldn’t think twice about. But now instead of coming home from a long day of work and crashing on the couch, I have to go put on my Vest and have my body pounded on for 30-45 minutes while I nebulize saline. Instead of hitting the snooze button a few times, I have to roll out of bed at the sound of an early alarm to do my nebulizer(s) and sinus rinse. Instead of doing whatever I want when I want, I have to make sure to plan around my treatments so I don’t miss any. Instead of taking days off when people come to town, I have to use half of my vacation days for doctor appointments.

Could things be worse? Yes, 100% they could. Do I feel grateful for the fact that I’m still relatively healthy? Yes, completely. But does that mean that I can’t grieve for the way of life that I’ve lost? I’m at the point where a lot of the novelty has worn off and I’m having to come to terms that this is how it will be forever. That more will be added down the road. I’m trying really hard to make all these new things a habit, but it’s hard. I’ll be the first to admit that I don’t have the greatest of will power. I have to lecture myself everyday about why I need to use the Vest. I’ve had my setbacks, missing a week or two by taking a day off and then repeatedly telling myself that I’ll start it back up again tomorrow. I’m having to learn that a life with CF means no days off from treatments and it’s a bit overwhelming.

I’ll make it through this period of change, I know I will, there just might be a little kicking and screaming along the way.

*My parents did have do chest percussion on me when I was a kid, but at one point the doctors said that we didn’t need to and even later in my life when I got the Vest it was a “do it when you feel like you need it” kind of thing, not a strict regimen.

Pills, Pills, and More Pills (plus a bunch of other stuff)

 This photo represents the typical amount of pills that I take in a day. Included are my vitamins, acid reducer, and pancreatic enzymes. I also threw in something for headaches since I end up taking that on most days :/

My enzymes (specifically Zenpep 15 for any curious fellow CFers) are the bulk of this since I have to take them whenever I eat. How many I take depends on how much fat is in what I'm eating, but on average I do 2-4 with a meal or snack. The enzymes have improved a lot since I was a kid, at one point I was taking 7 with every meal. My acid reducer also helps increase the functionality of the enzymes as well as let me get through the day without crazy heartburn. TUMS were my best friends for several years, now omeprazole is.

Since my pancreas doesn't work properly to pull the nutrients out of my food I need to take additional vitamins so I can make sure that I'm getting enough. Low vitamin levels are pretty common in CF. There are some vitamin brands out there like AquADEK, that combine a bunch of them into one pill, but I've found that those come back to haunt me (and anyone around me) all day long so I prefer to take them individually.

In addition to pills I also have to use a nasal spray to help keep the inflammation in my sinuses down, a sinus rinse to keep them gunk free, and a saline solution that I inhale using a nebulizer to help loosen the mucus in my lungs. Oh and I almost forgot about my bronchodilator inhaler, I use this prior to running/exercise to help open up my airways (or if I'm just having a bad, wheezy, lung day).

I'm no stranger to antibiotics either, as soon as I know a respiratory infection has started I'm on the phone with my doc to get a round to help clear it up. I don't love the idea of taking antibiotics so often, but I do love being able to breathe.

I know this seems like a lot and slightly overwhelming, but keep in mind that I am on very few medications compared to most CFers out there. Maybe one day I'll have to give up an entire closet to my medicine cabinet, but for now a couple of baskets stashed in the kitchen and bathroom will do.


**I have tried using essential oils instead of antibiotics (my last employer sold them, so I got well versed in all that I needed to try), but I found that they didn't quite work like the miracle cure that they were being sold as. I do still use them, but more as a supplement to what I already do. 

Let's Talk About Mucus

Boogers. Snot. Phlegm. Mucus. Spit.
Whatever you want to call it, someone with CF has a lot of it. Without getting too sciencey, salt doesn't stay in our bodies like a "normal" person (that's why our skin is super salty, but more on that another time), which causes our mucus to be thicker and stickier.

This thick, sticky mucus is harder to clear out of the lungs and is a great place for bacteria to grow, causing infections, that leads to scar tissue. All of this causes our lung function to decrease and it gets harder and harder to breathe as time goes on.

Mucus is found in many places in the body; pancreas, sinuses, cervix, etc. Anywhere that it's found, it will be worse in someone with CF. That's why many of us have to take supplemental enzymes when we eat. Our pancreas doesn't work properly due to the increased mucus and doesn't digest our food. Woe to us when we forget to, or just don't, take them when we eat something (again, that's a whole story for another time, yay, a poop post to look forward to!). Women with CF tend to have a harder time getting pregnant due to mucus plugs in their cervix, and most men with CF are infertile.
Chronic sinus infections (sinusitis) are also a common occurrence with CF.

Growing up, I didn't have any lung issues at all, but have definitely had my share of sinus issues. I had surgery in 2009 to open up my sinuses and get out all of the gunk that was jammed in them, they were totally blocked. They've stayed pretty clear since, other than major inflammation, but it's not uncommon for a CFer to have a sinus scraping surgery on a regular basis. And I've heard that sometimes the sinus cavities are 'obliterated' to close them off to future infection. I actually didn't even develop the one above my right eye, so lucky me, my sinus headaches are concentrated on my left side for the most part.

With CF, you learn to not be too squeamish about what comes out of your nose or mouth, as a single cough or sneeze can get you a sleeveful of goo. You also learn to inspect it for color as that is an indicator of an infection (yellow = bad, green = good). I can get up close and personal with mine til the cows come home, but if I even see someone else spit a glob out from a distance my gag reflex goes crazy.