My CF Journey

I promised that I would further detail my CF mutations and journey - so here it is.

At birth, I had a few complications which led the doctors to test and ultimately diagnose me with cystic fibrosis. I am so blessed for this early diagnosis because it meant I was on medications and being watched from day one.

When I was first diagnosed, we were only aware of one mutation Delta508. The other, at that point, was unknown and therefore I was re-genotyped last year. We found out that my second mutation is also Delta508. Why it didn't pick it up the first time, we don't know, but ultimately what that means is that I have the most common gene mutation out there.

I was hopsitalized once at age 5 with a sinus infection but nothing lung related. Other than this minor hospitalization, I was a pretty normal kid for years. I ran outside and played endlessly, loved school, loved reading, loved learning (Ok, so maybe I was never normal). I did my nebulized treatments with few complaints until I reached the age of 14. Suddenly, high school hit and I didn't want to be "abnormal." So, the treatments were done sporadically - only when I felt I needed it or more realistically, when my parents made me do them. I firmly believe this is why, at 17, I found myself hospitalized with double pneumonia and bronchitis. I ended up in the hospital for weeks and came home on a PICC line for 2 more weeks.

You would think this would have been a huge wake-up call for me, right? Well, in some ways it was and I did start doing my treatments more often, but due to my desired invincibility, I still was not 100% compliant with them. At this time in my life, being 100% compliant meant doing my vest (a machine that helps loosen the mucous out of CF lungs by vibrating them) two times a day, nebulizing albuterol sulfate two times a day, and nebulizing pulmozyme one time a day. Total treatment time was an hour a day. I felt, at this young age, that was simply too much to do. And when my post-PICC line PFTs were back up over 100%, I saw no reason to be completely compliant.

By now, it should be clear that I've been blessed and God has been taking care of me day in and day out because I certainly wasn't taking care of myself. This struggle continued into college where I still just wanted to feel normal. My sophomore year of college, however, my relationship began with my now-husband, and suddenly everything changed. We had been friends from freshman year on, but as soon as we started dating, I felt as if there was a purpose to my life - a reason to live, if you will.

Instantly, I became more compliant. I wasn't 100% compliant from the first date, but it wasn't long after that. Now, I only miss a treatment if there is no choice (for example, if we are on the road for 12 hours at a time). In fact, now, I keep trying to add more treatments or additional exercise to keep myself as healthy as possible. It's a total shift from the way I was just six or seven years ago! Unfortunately, my treatments aren't just an hour long anymore, but it's worth it in the end.

In addition to my treatments, I also have to manage CFRD (cystic fibrosis related diabetes). I recently have started on insulin to try and put weight on me to prepare my body for a pregnancy. My FEV1 (the indicator number of how well us CFers are doing) is 81-84% It's far from the 100% I used to have when I was younger, but it is still really good. The only way to keep it up this high is to continue with all my treatments. With that being said, here is my typical day's schedule:

6:30 AM: Check sugars and eat high-calorie, high-fat breakfast (Can't just pour a bowl of cereal anymore...)
7:00 AM: Albuterol Sulfate nebulized, Hypertonic Saline nebulized, vest for 20 minutes
8:30 AM: Check sugars again and eat morning snack (which actually looks more like a lunch...)
9:00 AM: Do nasal irrigation rinse (These help tremendously with sinus pressure)
11:00 AM: Check sugars again and eat lunch
12:00 PM: Second treatment. This time, vest by itself for 20 minutes
2:00 PM: Albuterol inhaler or albuterol nebulizer (depending on how I'm feeling)
2:05 PM: Checking sugars and eat if sugars are low
2:30 PM: Exercise for 30 minutes
5:30 PM: Check sugars, take insulin, eat large dinner (usually 1000-1200 calories)
7:00 PM: Second nasal irrigation rinse
8:00 PM: Third treatment. This includes albuterol sulfate nebulized, pulmozyme nebulized, and vest for 30 minutes.

Every other month, I am on Cayston which is a nebulized treatement three times daily which increases treatment time only by a few minutes (it's an amazing nebulizer system).

It's a lot to get in to a single day and if I get a cold or any illness, I add in a few more vest treatments. While I do a lot of treatments, there are other CFers who do way more than I do every day. I am blessed!

1 comment:

  1. You really have come a long way. I remember how much you hated doing nebulizer treatments our freshman year. I still hate doing them, and mine are just sporadic ones for asthma lol.


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