First Post-Kalydeco Clinic

Today I had my regular CF clinic appointment, and tomorrow marks the 8th week I've been on Kalydeco. Of course I've been looking forward to this appointment pretty much since I started on Kalydeco just because I wanted to see (in numbers) the change that was happening with my weight and lung function. 

Overall, I haven't noticed a huge transformation in my lungs, but I have noticed that I'm starting to get my CF belly back a little bit, as well as some "love handles" - but then again I thought I was noticing these things the before my last appointment, in July, and I ended up losing a half pound, so I wasn't getting my hopes up today. Over the last eight weeks, I've coughed up a ton of mucus. There are days where I feel it's endless, but then there are days where I barely notice that I'm coughing at all. One thing that I have noticed though, is that when I am coughing this stuff out, it's from deep down in my lungs. I've coughed up some funky colored stuff over the past couple months, which I'm guessing was just old, deep mucus. 

As far as my weight and eating habits, I've started drinking two Ensure Plus's per day - one with breakfast (and my first K of the day) and one with lunch at work to replace those calories I'm burning while working. If I have both per day, that's an extra 700 calories and 24 grams of fat added to my already high-calorie and peanut butter-filled diet. Speaking of peanut butter, I've been on this crazy PB & J kick lately and I eat at least one or two pb&j sandwiches (or on toast) per day. 

So anyway, back to my appointment. Due to the changes I mentioned above, I was hoping that my weight would be at least 111 lbs (3 more pounds than what I weighed in July), and my FEV1 somewhere in the 80s. I knew that I shouldn't set my expectations too high 1)because I've only been on K for two months and 2) because I don't want to be disappointed in my body if I don't get those numbers because I've been feeling great and doing everything I'm supposed to do.

Of course I got to clinic early, and it was packed. At one point, there were at least 4 other CF patients in the waiting room -which is totally against proper infection control...but that's for another blog- (they weren't 'labeled', I just think that I have pretty good CF radar), and I was the only one wearing a mask! Either way, it was packed. So I kept myself occupied with my phone and my book for the hour or so that I waited to be called back to my own room. The first thing they do (usually) at my clinic is weight, vitals, PFTs, then I usually sit forever until a doctor or nurse comes in. Today, she weighed me first and I was a whopping 114.8!!! I totally said, "YES!" when I saw that on the scale, haha! That's up SIX pounds since July and I'm very comfortable with that. I think this is a good, healthy weight for me, but I'm not going to stop what I'm doing with the Ensures & pb&j's. A little extra weight wouldn't be a bad thing for me.

After being weighed, I went into my room and had my O2, blood pressure and temperature checked. My O2 was 97%, blood pressure was a little elevated and my temp was 99.3. She asked me if I was nervous, and I said no, I was just anxious to get my PFTs done and that I was a little hot (I was sweating!). Then I sat in the room, for probably 10-15 minutes before my new nurse came in. She introduced herslef and we chatted for a little bit. Then she asked me about my lung function, she sounded just as anxious about my results as I was, and I told her that I was still waiting to get my PFTs done. She was like, Oh man, let's get going on that! How exciting! Oh, but no pressure! Haha!

Finally the RT (the good one, Patti!) came in with the PFT machine. I pulled my hair back and stood up anxiously while she got the machine ready. She, too, was so excited for me to do my test because she wanted to see what my numbers looked like after being on Kalydeco. I did the first test, with her wonderful encouragement, and blew an FEV1 of 82% (5% higher than July!). Honestly, I was a little disappointed with that number, but I thought to myself at least it went up & it's in the 80s. So I did two more tests - the second test was 82 again, and the last was 80. So, my overall lung function as increased by 5% since July. Who can complain about that?? The best part - my small airway function...this is something that my doctor looked at a couple years ago and decided to put me on Advair to try to increase. And after being on Advair, my small airway function went up to 96% in August 2011. The over the next couple years, it followed this trend = 85, 88, 86, 78, and finally 72 in July. Today, my small airway function was 90%! That's an 18% increase!!! And I can feel that! When I take deep breaths, I'm not as wheezing and crackly as I've been in the past! :-)

My doctor is really looking forward to see what my cultures look like to see if the K is affecting that. She also mentioned that she's looking forward to seeing what happens at my next appointment and that maybe we can talk about backing off of anything....that would be fantastic, but we'll see - not getting my hopes up on that one. And finally, in another month, I'll get my liver enzymes checked to make sure that's reacting appropriately with the Kalydeco, too. Needless to say, everyone was very pleased with my results, including myself! For only being on Kalydeco for two months and to notice these changes already, really makes me happy! I'm going to keep doing what I'm doing, plus I'm going to try to increase my exercise (I've been completely slacking) since that made a great impact on my lung function...it can only go up from here, right?! :-)

CF CLIMB

Call us crazy, but Tim and I just signed up (and paid) to climb 58 flights of stairs on a Sunday morning! 

Why, you might ask?? Well to support the Cystic Fibrosis Foundation, of course!!! I received an email from the Foundation the other day informing me about this event and it piqued my interest. It's downtown Chicago, so I thought I'd get more of my family to sign up (not one yet....they must be too scared ;) ) and I've been exploring lots of Great Strides events this year, what's one more fundraiser for the year? Plus, they were having a promotion where it was only $10 to sign up instead of the typical $45 - can't pass up a deal like that! It's going to be a physical challenge, but I won't let me CF stop me on this one.

And with the new CF regulations & guidelines, more than one CF patient can attend indoor events sponsored by the CF Foundation at their own risk. Here's the disclosure from the event's website:

B. CEPACIA AND INFECTION CONTROL POLICYBecause of risks to people with cystic fibrosis (CF), only one person with CF may be invited. Also,individuals who have ever had a confirmed positive sputum culture for Burkholderia cepacia (B. cepacia) complex shall not attend any Foundation events/meetings. These requirements are because CF germs can be passed between individuals who have CF. Some germs in a person with CF can cause serious respiratory illness and, in some patients, may lead to death. CF germs are not a risk for otherwise healthy individuals.

Despite this policy, individuals with CF might choose to attend events or meetings without informing the CF Foundation or without the Foundation's knowledge. If so, they do so at their own risk. The Foundation accepts no responsibility for any risk to health involved in attendance, or in any social contact between persons with CF. For more information, please visit www.cff.org.

I'm glad that they're not enforcing the strict regulations of only one CFer attending an indoor event. I feel like a lot of people in the CF community were really turned off by this, and I think it's fair that we're able to make our own decisions on things like this. I understand they're doing it to keep us safe, and also to cover themselves, but I think that CF adults should be responsible and mature enough to make their own decisions. With that said, I am aware that at least one other CF patient will be there, so I will wear my pin proudly to identify myself and be sure to keep my distance.

Finally, my goal for this event is to raise at least $200. If you'd like to donate to my CF Climb, please let me know. My page hasn't been set up yet on the CFF website, but as soon as it is, I'll post it on here. Right now, I'm just collecting any cash or check (made out to the Cystic Fibrosis Foundation) donations to submit on the morning of the Climb.....which is only three weeks away! I better get these legs into shape!!!!!

Positive Thoughts Thursday: Quotes

I have a board on Pinterest called "Quotes & Cute Things", which is where these all came from. I enjoy going through that board occasionally and reading these happy thoughts, so I wanted to share them with you.

Positive Thoughts Thursday: Health & Fall Fun

I've been totally slacking on blog posts lately, but honestly there hasn't been much going on. I'm glad that several of my other blogger friends do their "Thankful Thursday" posts each week because it reminds be to get back on here and appreciate the little (and big) things in life. :)

• Today I'm thankful for good health. I've been on Kalydeco now for six weeks. And just now, over the past couple days, I feel like I'm finally slowing down with my mucus movement. Over the last six weeks, I experienced some serious mucus movement in my lungs, especially deep down in there. Things started moving around the first few weeks I was on Kalydeco, then I got a cold which tightened my lungs for about a week. And then after that cold, I felt like I was constantly coughing my brains out! I'm pretty sure I've coughed up every color of the rainbow mucus (except maybe blue/purple...) and I've gotten a LOT of stuff out. Over the past couple days, I feel like I've finally been able to take a good, nice deep breath without coughing up a chunk of my lung. 
• Related to the above post - I'm really looking forward to my CF appointment coming up soon. I'm very anxious to know what my FEV1 is, especially now since I feel like my mucus production is slowing down. I'm also curious to see if my weight has gone up. Since starting Kalydeco, I've been having at least one, usually two, Ensure Plus's each day which is an extra 350 calories each. I hope K really helps me in the weight-gain department.
• Fall weather! I am not a fan of the heat. I enjoy sunny & 75 (Joe Nichols, anyone?). It's so much more comfortable to teach in the cooler weather. I love having the windows open all day and getting fresh air in the house. And of course, all the fall fun & fall food (aka: apple donuts!)! This weekend we're hanging out with Tim's aunt & uncle, and his sister, her husband and their two little guys! We're going to the pumpkin patch/apple orchard on Saturday -- always a good time! :)  
• Last weekend, Tim and I walked in (my third) our second Great Strides of the year. I was asked to speak at this one which I enjoy doing. I like to share my story with others, especially parents of little ones with CF to give them hope about their child's future. I was also able to meet one of my blogging cysters, Cheriz - who's now in the hospital for a tune-up- hope you start feeling better soon!! Here are some pictures from the event.

• Oh, and how could I forget?! Yesterday, Tim and I celebrated our third wedding anniversary! :-) Today marks our 7th anniversary of being together, total - seven years ago today, Tim asked me to be his girlfriend while eating lunch in the "Dog-Pound Deli". This year, not only were we husband and wife, but we also became "homeowners"! I'm looking forward to what this next year (and MANY more) has in store for us! I love you, Tim!!!! 

I'm Still Here!

I'm sorry for the lack of posts lately. The day I was planning on typing up something, I turned my laptop on to a very dark screen and a high pitch squealing noise. Of course, I texted our friend Chris, who's an HP rep and asked him what I could do, and he just suggested turning it off and that there could be several things going on...great. Thank goodness I recently backed everything up onto an external hard drive! I'm now using a desktop computer that we bought several years ago and then never ended up using for whatever reason. (I used to always give Tim crap about still having it, telling him we should have sold it....good thing we didn't!)

Right now I'm finishing up with my first cold of the season...already! It started last Monday as this awful stuffy nose and sneezing all day, so I thought at first it could just be allergies...of course not. It slowly moved into my chest, but it was different this time. I had a tight chest for a couple days and I was only coughing up yellow or clear mucus (when I was able to get anything up). I was so excited because I thought I was going to have a normal, "non-CFer" cold. Well then Sunday morning I woke up and my chest was much more open than the previous days. Thanks to good ol' hypertonic saline (the best, right, John?!), that morning I was stirring things up deep down in my lungs. So for the past couple days I've been working really hard to get all of this really deep, thick stuff that had been stuck down in my lungs when they were too tight to get up anything. Other than a rattly cough, I've been feeling fine. I still have my appetite and energy, so I think in a couple more days I'll be back to normal.

In other news, Tim and I will be walking in another Great Strides this Saturday. I don't have a big team signed up because I haven't promoted it too much, just because it's not super close and I just wanted to check it out. I think its fun to go to other Great Strides walks to get ideas and see what they do. This one on Saturday should be fun because I might be able to actually meet one of my blogger friends, Cheriz!

Oh, I started my second bottle of Kalydeco this week! I've now been on it for a month and one day. I can't say that I have been noticing any major changes to my body, but I can tell you that I feel like I've been eating more. I have to take the Kalydeco with fat, so typically I take it with my breakfast and dinner, but sometimes that just doesn't happen, so I have to take it with a fatty snack. I've also been having two Ensure Plus's a day, one with breakfast (and my morning K dose) and one with lunch everyday at work - that's an extra 700 calories per day. I'm really hoping that's been helping with weight-gain. I can't tell right now if I've gained anything, and we don't own a scale because I'd weigh myself all the time, so I'll just have to wait and see at my next clinic appointment which is this month. I'm really hoping to see an increase in lung function, of course, but I'm actually looking forward to seeing my weight, too.