Wednesday, August 31, 2011

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
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.

This is my current schedule.  In another 20 days, I'll be back on Cayston which adds an additional 3 nebulizers a day (granted, these only take 2 minutes each). 

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!

Tuesday, August 30, 2011

The biggest decision of our lives...

I remember when my now-husband and I first started dating.  I remember thinking that marriage was the biggest decision we were going to face and I remember it seemed terrifying at the time. My poor husband was more terrified of the actual engagement question and asking permission from my parents, I think, than a wedding.  In reality, marriage is a huge decision and it certainly should not be taken lightly, but compared to the decision we made a few months ago, it seems easy.

In fact, our engagement came and went and we were incredibly excited. Then, our wedding rolled around faster than I thought possible and we found ourselves staring at each other at 1:00 AM on our wedding night saying, "Wow. We just spent how much for one day?" Don't get me wrong, our wedding was beautiful and we were incredibly blessed with friends and family who traveled long distances to see us exchange our vows.  The actual day of the wedding, however, goes by so quickly that you feel like you blinked and it was done.  I wish there was a way to relive that day at any time. 

Going from being engaged to being married seemed hardly any different and we loved that. We enjoy being the type of couple that stays in on a Saturday evening to watch a movie together.  We enjoy laughing at each other's corny jokes and just sitting, doing nothing. We're boring and we love it! So now comes the twist: over the summer we officially decided we were ready to become parents. Throw boring, lazy, and doing nothing out the window! We just knew we were ready and the decision had ultimately been coming for a few months.  This decision is BIG for any couple to make, but I have cystic fibrosis which just adds another level of BIG.

More on my actual mutations and lung function later, but ultimately cystic fibrosis, or CF, is a challenging genetic disease which effects the lungs and digestive system.  Originally, I believed I would never be able to have children but (obviously) as far as my lungs go, my doctor is 100% on board with the idea.  From what I've read from other CFers (those with cystic fibrosis), getting pregnant with CF can be difficult..more so than for most women. With so little being written about this, I decided it may help someone else who is trying to come to this decision with her spouse and thus created the blog. Well, let the "difficulties" begin.  We are keeping our fingers, and toes, and hair crossed that it all goes smoothly and easily, but preparing ourselves for the alternative at the same time.  Here's wishing luck to all those going through the same process we are right now.  More to come soon...