January Clinic Appointment

On Thursday, I went to my regularly scheduled Cystic Fibrosis clinic appointment. I knew that it was time for my annual blood work (plus genetic testing) and a chest x-ray, so I decided not to bring the kids with me. Anna had school and there's no way very-active Calvin would have been happy with all of the sitting/driving.

I decided to head up to Chicago earlier than my scheduled appointment to hopefully avoid the long wait in the lab and knock out any other tests before seeing the doctor. I arrived at the hospital around 9am and was able to get right into the lab to get my annual blood draw (7 tubes) and an extra tube for genetic testing. I believe that last time I had my genetics tested was about 10 years ago, and they were only able to identify one of my mutations - G551D. The other is still unknown. With all of the new medications in the pipeline, it's important to know exactly what my mutations are. As of now, I'm on the best medication for my mutation. Kalydeco is specifically for G551D, so the likelihood of me switching to a different medication any time soon is low, but learning my other mutation is still good information to have.

Jan 2019 - more areas of white
(plugging & mucus), especially
in the upper right lobe. Also a bit
more in my left lung.

April 2017

After my blood draw, I went downstairs for my chest x-ray. I actually requested getting the x-ray because I've been having such a rough time with coughing up blood lately - my latest episode was that morning when I woke up. I was so frustrated after seeing that, I emailed the nurse and asked her to put in the order. I wanted to see how/if my lungs have been affected by the sporadic hemoptysis episodes over the last five months or so.

After the chest x-ray, I waited to get a clinic room forever. As much as I love my doctor, I can't stand how long it takes. I feel like they're always running behind. Anyway, I did my PFTs, met with a new physical therapist (did a 6 minute walk test), saw the pharmacist, social worker and dietician before the doctor finally came in two hours after my scheduled appointment.

Thankfully my PFTs were pretty much unchanged from September's appointment. I was really glad and pleasantly surprised. But, that didn't explain what's going on with my lungs.

My doctor and I had an excellent, over an hour long, conversation. *That's what happens when I don't bring the kids* I showed her the calendar charting all of the dates that I've coughed up blood or had some streaks in my mucus. It seemed to be at least every two weeks, but without any kind of pattern or cause. It's happened when bending over, walking up stairs, just standing there, laying down, sitting up.... no pattern. Twice since August I've been on antibiotics - one round of Cipro in August and one round of Augmentin in November for a sinus infection. Even when on the oral antibiotics, I was having blood/streaking.

Then we finally got to the bottom of it - sort of. It's been months since I've been on inhaled antibiotics. I stopped taking Cayston in the summer or fall because I noticed I was coughing up blood more frequently while on it. I had tried a month of inhaled Tobi, but it made my lungs feel super tight, so I only managed about two weeks on it. So, since the fall, the pseudomonas (bugs in my mucus) in my lungs has been free to do whatever since it wasn't being knocked back by inhaled antibiotics. My doctor thinks this has caused a lot of inflammation in my lungs and irritation of my airways leading to all of the hemoptysis episodes. Thankfully, it hasn't made my lung function drop.

It made sense, and I'm really hoping she's right. So with the mucus sample I provided at the appointment, the doctor requested that the lab test the resistance of my mucus against the inhaled antibiotic called Colistin. As soon as we hear back, and are sure that my bugs are susceptible to Colistin, I'll hopefully be starting it. My doctor also wants me to go back on Azithromycin on Monday/Wednesday/Friday to help decrease the inflammation in my lungs. I originally stopped taking it about 5 years ago when we were trying to get pregnant for the first time. Its kind of a bummer to be put back on a medication that I've been off of for so long, but it's definitely needed right now. I feel like my list of non-IV medications is slowly dwindling, so I am really banking on the Colistin to help give my lungs the boost they desperately need.

I think we have a good plan in place and I'm trying to be optimistic. I know that 80% FEV1 isn't too far from my baseline of 85-88%, but I don't like seeing my numbers there. I hope that the medications will help my lungs get back in shape. I also need to really make an effort to add exercise into my routine to get my lungs working more. This is always the hardest part for me due to time constraints.

Right now my next appointment is scheduled for April, but I wouldn't be surprised if she wants to see me back sooner to see if the meds are working.

Next hurdle.... getting the meds approved my insurance and shipped out in a timely manner...

30 & 31 Weeks

Thursday, December 7th - 30 weeks
Something about getting to 30 weeks today really gets me excited and feels like an accomplishment. It's like we're in the home stretch! The longer you're in there, the less nervous I get about you being born early. I know that nothing is guaranteed, but I feel like as each week passes, your chance of survival just increases that much more.

Your movements have been getting so much more interesting. You're still sitting pretty low, but because you're getting bigger and running out of room, your movements are a lot more noticeable from the outside, especially when I'm reclining or laying. It's so fun to watch you jerk around and guess what we're seeing - a head, your back, legs, arms?! Sometimes it feels like you're doing the worm! You've had some really strong kicks/punches lately which always catch me off guard! But this is absolutely my favorite part about being pregnant. I love feeling you move all day long and I especially love watching you move in the evenings. Every little (or big) jab is an excellent reminder of how lucky we are that you're still doing well. Along with your movements, comes some tightness in my belly sometimes. I can't tell if these are Braxton Hicks or just the way you're positioned at the time. They don't hurt too bad, just a weird tight feeling and don't typically stick around for long. I know it's pretty normal to be having Braxton Hicks around this time (and I think I did with Anna). I'll have to run it by my nurse on Monday and see what she thinks.

Sunday, December 10 - 30w, 3d

Your room is painted! It was a big deal for me to get the room painted because it's one step closer to becoming your room and farther away from being the office. I love the blue we chose - it looks excellent on the wall and on your crib.

One wall is blue and the rest are gray

Your room is far from done and ready, but I feel like we made good progress this weekend. It's going to be tough getting anything done the next two weekends because of family Christmases, so I foresee Daddy working some evenings after work to keep going on some projects. We're quickly running out of time before you're here, but I don't care, I'm getting so excited!

Your little head or booty sticking up!

Monday, December 11 - 30w, 4d
I had an appointment today with the nurse practitioner at MFM and everything is going well. Sugars have been decent the last two weeks and your constant movements let us know that you're doing well, too. Your heart rate was 140s-150s today, my weight gain is good (about 13lbs so far) and that's about it. Nothing exciting, which is good. Even though we're within single-digit weeks of you being born, we need you to stay comfy for several more!

Thursday, December 14 - 31 weeks
We're two months (and one day) away from your official due date! I'm heading to CF clinic today, so I'm curious to see what my numbers look like. I started inhaled TOBI about a week ago and it slowly seems to be helping. I'm guessing (and hoping) I won't have to go back until after you're born. I am not looking forward to the long drive today!

Monday, December 18 - 31w, 4d
My clinic appointment went well on Thursday. My lung function remains at 85% and my small airways even increased slightly, so that's always good to hear. I'm not a big fan of the way TOBI is making me feel (scratchy cough, dry mouth), but it seems to be helping for now. Dr. Dowell did say that I am welcome to stop TOBI at any time and get back on Cayston. I'm coming up on two weeks on TOBI, so I'm hoping to stick it out the full four weeks to introduce my lung bacteria to a new med that it hasn't been exposed to in years.
The doctor wants me to come back one more time in January, but as long as I'm feeling well, I'm not planning on going. I do not have enough sick days to cover my maternity leave, so I don't want to use up one more before I have Brother.

We celebrated our first family Christmas on Saturday with Tim's whole family. It was a lot of fun hanging out with everyone! Anna had a good time with her cousins, even took a nap for a while in between all of her fun. It was all fine and dandy until Anna threw up four-five times on the way home. It's an hour drive between Grandma's and home, but Saturday night took us about two hours. The poor girl was complaining of a stomach ache about 20 minutes into our ride, so we figured she had to go to the bathroom and told her we'd stop at the next gas station....until we heard the all too familiar sound. :( We pulled over and got her cleaned up as best as we could. Thankfully she had received some new clothes and jammies that day, so we changed her into those. This happened three more times on the way home and we were quickly running out of things to clean her up with and things to change her into. It was a long, exhausting, late night.
It turns out, whatever she had eaten must not have agreed with her. Even though we all ate the same stuff, her body did not like something. She ended up puking up absolutely everything in her tummy, even after we got home. Finally, around 4am, she was done and was able to get some sleep. Thankfully, when she woke up yesterday, she was on the mend. She was able to keep down water, then some medicine, then apple sauce and toast. By early afternoon, I had to keep her from jumping on her bed - she was feeling back to normal! I'm really grateful that she didn't have a fever to go along with it and that it only lasted those 12ish hours. I'm also very glad that she is feeling back to herself and can go to school for these last few days before break, and that Tim and I didn't get sick right along with her.

Clinic Day

Just a quick post about my clinic appointment today.

I've been feeling really good, CF-wise, the last couple of months. A few weeks ago I was having some sinus issues, but I'm not sure if it was a cold or just allergies/change of weather. Whatever it was, thankfully it didn't stick around long and didn't get into my lungs. 

Today's appointment went well. As usual, I dreaded the three hour drive to Chicago (and would be okay never going to the city again if it wasn't for my amazing doctor). I made good time and was even called back right at my scheduled appointment time. I was a little nervous to do my PFTs today because when I was about this pregnant with the twins and did PFTs, I'm convinced it played a part in what set things in motion into their labor about a week or two later. Either way, this is a different pregnancy and I'm feeling completely different - in a good way. My lung function is stable at 85% today, which I was glad to see, but not surprised. My weight is slowly, steadily increasing. Doctor said I looked and sounded really good, and honestly, there wasn't really much to talk about this time around. I'll be going back in three months...and hopefully then again after I have Little Brother (even though I know she'll want to see me again). Overall, it was a successful clinic day and I'm glad that my body and lungs are cooperating with this pregnancy! 

Clinic

Thursday I had my routine clinic appointment in Chicago. Thankfully it wasn't until noon, and I was able to drop Anna off with a friend so I didn't have to take her with me - it's too much for her. I was looking forward to this appointment because I hadn't seen Dr. Dowell since we lost the twins, so I knew we'd need to talk about that. Of course when we did, we both got emotional - had a little cry and a hug. :) She told me, "You didn't have the rug pull out from under you, you had the whole floor!" And I completely agree. I love the relationship that I have with her and that we can talk so easily.

Health-wise, everything is looking good. My lung function is stable (up a tiny bit) and my weight is, too. Dr. was pleased with how I'm doing and won't see me back until September. It was a pretty non-eventful appointment, which is always a good thing. :)

Clinic Appointment Today

This picture basically sums up my appointment today! 

I was lucky enough to have my appointment moved up a couple weeks to today during my spring break so I didn't have to miss a day of work. It was wonderful because it was not a CF clinic day, meaning there weren't several other CF patients waiting and sharing germs in the lobby! It also meant that I got in right away! And, as a bonus, since things were going so smoothly, I decided to get in my chest x-ray today and that lady was super fast with it!

I'm currently nearing the end of my two-week Cipro cycle, but still not feeling 100% lung-wise yet. I'd say, I'm more like 85-90%. I'm still having a bit extra mucus than normal, and some at night which is not normal for me, so I don't feel like I'm quite ready to be done yet with the antibiotic. This is the first time I've ever asked about extending my antibiotic cycle, but I feel a few more days (up to a week) might be just what I need. The nurse practitioner agreed, even though my numbers looked good today. I appreciate that she respects what I have to say and trusts how I feel. The one good thing about my mail-order Cipro issues I had, is that now I have a ton extra on hand, so extending my meds another week isn't going to be an issue. I'm also going to order Cayston as soon as I can and start it a little bit earlier than scheduled.

One thing we did discuss today though, is that my lungs and pseudomonas (bacteria my lungs culture) are slowly becoming resistant to Cipro. I used to have a "moderate" resistance to Cipro, but according to my last culture in January, I'm now "intermediately" resistant. Unfortunately, Cipro is the only oral antibiotic that fights pseudomonas. Once I become completely resistant, that I'm left with IV antibiotics as my weapon against infections. I wasn't too happy to hear this, but I think (hope) I still have lots of time before I need to really consider that. Thankfully, I was able to make it almost seven months between needing Cipro, so hopefully I can make it another 7+ months before needing it again. The longer I can keep between cycles and the less often I need it, the slower I'll become completely resistant.

So, now for the numbers: My weight is stable at 132.7lbs. My lung function is up all around and closer to my baseline, at 85% today. Of course, I'm greedy and would like to see them closer to 90, but 85% is about right for what I'm feeling right now.

Today, I asked the RT to record me doing a PFT today. These are the tests that I do, at least three times, each clinic visit to determine my current lung function. For people without CF, "normal" lung function is between 80-100%, so technically I have "normal" lung function...just have to do lots of treatments to keep it that way. Anyway, I know CFers often put a lot of pressure on themselves during these tests because they tell us our next plan of action and overall health. Follow this link which will take you to my Colleen's Friends & Family facebook page where you can view the video. :)

18 & 19 Weeks

**I debated whether or not to share this post, since I never quite finished it, but I'm going to publish it so I have it for the memories.

January 24 - 18 Weeks today!
The title of the week on my app this morning was "The Smooth Second" -- I beg to differ! This last week has really thrown me off and I cannot wait to be feeling the "smooth" second trimester feelings. Although with twins, I feel like that may have been from about week 12-14, haha!

Yesterday, I woke up with the chills and took some Tylenol every six hours to get through work. When I got home (after a professional development meeting, of course - can't miss out on those hours!) was shaking & felt terrible. I took my temperature with Anna's little ear thermometer and it was 99.8*F. I don't know if that exactly counts as a fever, but I'd say it is based on the way I'm feeling. As soon as the Tylenol wears off, I'm shivering and shaking with a headache until I take it and it kicks in....then of course, I start sweating...

I decided to stay home from work today so I could do extra treatments, although I'm not noticing much difference in my lungs; rest; stay hydrated and just relax. I've had a stressful week with the increasing pressure and bleeding, and now fevers. I'm over it! Thankfully I'm not vomiting, I'm able to keep food and water down just fine, not noticing any respiratory issues. Just headaches, chills/sweats.

I called MFM this morning to let them know about the fevers and they told me I'm allowed to take 2 extra strength Tylenol every six hours, so that's what I've been doing. I also just got off the phone with my CF doctor, who I was supposed to go see tomorrow for my three-month follow-up appointment. She said she doesn't want me driving that far by myself, so if I could get someone to come with me she'd still like to see me. If not, then she's okay with post-poning the visit unless I seem to get worse. She did tell me to have someone local see me and swab me for the flu, just in case. She said it may just be a viral thing that has to run it's coarse, but she wants me to get checked anyway. So as soon as I'm done with my treatments, I'm going to head to Express Care in town to see what they have to say.

January 25 - 18w, 1d

Good news: I don't have the flu! And my fevers are gone!!
I was able to go to my CF clinic appointment today which went pretty well. My lung function is down a tad, but that's to be expected with two growing little guys in there and a little extra mucus production. Lung-wise, I've been feeling great lately, so I'm not worried about the small decrease. I think once I start my Cayston back up in a week or two, it'll help get rid of some of the excess mucus I have going on.
My weight is still hanging at 138lbs which my CF doctor is complete fine with. I told her that I was worried I wasn't gaining enough weight with this pregnancy (only 4lbs so far) and she was worried one bit. She reminded me that I started out this pregnancy much higher than I did when I was pregnant with Anna, so that gives me a little "reserves" to use and means I don't have to gain as much as quickly this time. She thinks the further along I get, I'll eventually start putting on more weight, but she told me not to stress about it. I was able to talk to her and the dietitian today to get some more meal/snack ideas. They definitely don't want me cutting carbs because I need the nutrients and calories, but I just have to watch my sugars. My CF doctor is a bit more lax on what my fasting and two-hour numbers should be, which makes me feel better because I'd been having a hard time keeping them so low.

I feel like I'm finally starting to get better. I've had barely any fever-like symptoms today, only taken 3 Tylenol pills all day, although no naps, so I'm exhausted. I'm very happy that tomorrow is Friday so I can rest this weekend.

January 28 - 18w, 3d
I made it through work yesterday feeling pretty good. I did sit a lot which helps. Again, I'm just so surprised at how quickly I get worn out and tired. This last week has really put into perspective for me how much different and harder growing two humans is than one. I really had no clue!
I'm still having lingering headaches and some leaking/discharge, but I have a feeling those aren't going to go away any time soon. Oh the glamours of pregnancy... :)

I'm so anxious to start feeling the boys move!! Sometimes I can tell when they shift or are maybe sitting close to each other because I'll feel a lot of pressure in a specific area, but I still haven't felt any little flutters or jabs. I also can't wait for Anna to be able to feel them, too. She often gently pats my belly and says "Hi babies". This morning she laid in bed, patting my belly and singing "You Are My Sunshine" to them - it was adorable!!

January 30 - 18w, 5d
I can finally say I'm feeling better! I haven't had a big headache all day, I'm not super exhausted or in a lot of pain - yay!!! Even though I was feeling a lot better at work today, I still reminded myself to take it easy and slow. I sat when I could, walked slow when I had to walk and felt pretty good throughout the day. The boys still seem to be sitting fairly low and often put pressure on my lower uterus, but that's something I'm getting used to.

February 1 - 19 weeks
I think I fully slept through the night last night! I went to bed kind of early, and I don't remember rolling around uncomfortable or switching positions a million times. I actually woke up feeling rested this morning! Woo hoo!!

We're 19 weeks along today and you should be measuring about 6 inches long, each. You could be beginning to get little hairs on your head, your arms and legs are just about proportional to your body now, and your coordination is developing to control your arms and legs better.

I'm so excited for Monday's appointment to get your measurements for the first time. I'm also hoping we'll get lots of cute pictures and be able to see both of your faces. I can't wait to see every little inch of your bodies and make sure you're both healthy and developing as you should be.

CF Clinic

Here's a quick recap of my appointment yesterday.

I started feeling a cold, extra tingly cough coming on Monday that slowly increased throughout the week. I was worried about what that might show for my lung function because my lungs were getting tight. And now this morning I'm feeling worse than I did yesterday, and I'm really looking forward to the weekend to relax, shake extra, and maybe even get in a nap.

I got to my appointment about ten minutes early, but didn't get called back to a room until about half an hour after my appointment time. Oh well, I had time to scroll through FB and relax after the long drive. The took me back to get my weight (134.9lbs) and then to the room. The people who cleaned it were just walking out and the cleaner smell was SO strong. They asked if I wanted to wait in the waiting room for 5 more minutes while it aired out and I definitely said yes. So, five minutes later she brought me back to the room and it was much better. Someone came in to do my vitals (100% O2 level and blood pressure was fine). Then came the PFTs...

I always get a little bit nervous before doing them because it's basically the whole reason I'm there. It guides the rest of my appointment and tells how my lungs are holding up. My last visit was very disappointing when I had a huge drop and was down to 81%. I was hoping to be a few points higher than that today because I had been feeling excellent until this week, so I was worried that my tight lungs would skew the results. My first blow was pretty good, and when I looked at the screen I saw 86%! I said, "Yes!" and then coughed my brains out for a little bit. Once I got everything out, I did the test again and got an 83...not too bad, but I was getting tired and tight. I coughed a bit more until I felt clear again, then went for a third test. Same result - 83. I could tell the last two were lower because of the way I was feeling, so thankfully they took the first test as my best one. :)

So even with this cold beginning, my lung function has gone up at least 5% over the last couple months. I am very happy with that. I think the combination of being healthier, doing all of my treatments, and joining my running group has definitely contributed to that increase. I'm a little worried I'm going to lose a bit of it by the way I'm feeling this morning, but hopefully I'll fight this nasty bug off soon.

Thankfully, I'm at a very healthy weight so I have some back up pounds to help me get over this cold. As a person with CF, it's always been very hard for me to gain weight, and it's always come off very easily, which was not fun! My body is going to be working extra hard over the next few days to fight this cold, which burns more energy and calories. So I'm glad and lucky to have that extra weight, so that if I did lose a few pounds, I'm still within a healthy range. The unfortunate part of being at the heaviest weight I've ever been is my belly. Oh the dreaded CF belly.....I posted about it several years ago when I thought I was at my highest weight (about ten pounds lighter than I am today - thanks Kalydeco). It's very common in the CF community to have a belly. It's the way our bodies are built and I don't think people who are not familiar with CF understand it. Yes, I look pregnant, but it's all CF belly. That's where I gain my weight (and in my face) and I also have a distended belly because of my digestion issues. CF patients frequently have bowel/digestion issues, extra gas and bloating which all shows in that area....just an extra lovely perk of the disease. So for those of you who keep looking at me funny...please stop. :)

Overall, I had a great appointment. The doctor was very please with my increase in weight and lung function. She wants me to up my treatments to help kick this cold or whatever is developing. Hopefully it'll disappear by next weekend when I'm running in the Turkey Trot! I'll go back in January for a follow-up (she said unless the weather is bad, then we can wait til February).

X-Rays. Compliance. Kalydeco.

 

 

On the left: Chest x-ray from January 2013             On the right: Chest x-ray from January 2016

 

What's the first thing you notice? Be honest...

 

Fat! And that's a good thing for a CFer. Let me fill you in on the huge difference in these photos.

 

January 2013 - I was half way through my first year of teaching. I weighed 109lbs, although my lung function was 88% at the time, it wasn't consistently that high. I was showing signs of pre-diabetes, losing weight quickly and not compliant with my treatments. This x-ray is pretty good for a CF adult (24 then). Lungs are pretty clear with mild issues in the upper area of my right lower lobe.

 

January 2016 - I was into my fourth year of teaching, on Kalydeco for a little over two years, a mommy, 124lbs, and a steady lung function of 88%. I had a normal A1C (aka: no diabetes!) and had been compliant with my treatments for a couple years now. The lung damage was very similar, which is key. Typically, adults with CF slowly lose lung function over years, but that fact that my lung function is even better (now) is quite amazing.

 

 

 

There are two giant factors: Kalydeco and compliance.

 

When Tim and I got married and talked about starting a family, I became very serious about my health. I didn't want to sit back and let CF slowly destroy my body as it was. I finally took control and started being complaint with all of my medications and treatments. It was only fair for my husband, and then daughter. To this day, I remain complaint with my treatments because they're what keep me alive.

 

Looking back at blog posts from right before I began taking Kalydeco, I remember that I really didn't want to start it. I didn't want to rely on another drug to keep me healthy. But after realizing, 'what's another pill?!', I figured I'd give it a go. I started Kalydeco August 30, 2013. My lung function the month prior was 77% and I weighed 109lbs. Today, my lung function is 92% and my weight is typically between 124-127lbs. That means, since beginning Kalydeco 2 1/2 years ago my lung function has gone up about 15% and I've gained 15-20lbs! Now if that's not amazing, I don't know what is!! From two little blue pills twice a day!

 

I plan to continue taking those little blue pills and doing ALL of my treatments every single day...well, until there's a cure!

CF Clinic Appointment

Yesterday was CF clinic time, and I've been so busy with end of the school year craziness, it came upon me very fast. Over the last three months I've been feeling really good, no lung infections, no issues, so I figured my appointment would be fairly normal and smooth.

Then the weekend before my appointment, I started having this really high belly bloating/pain. At first it felt like bloating, but just higher up under my diaphragm and ribs, so I tried to cut back on my carbs for a few days, but the pain still lingered and felt more like pain instead of bloat. So, of course, I reached out to my CF family for their advice, and some said it sounded like a bowel blockage. Well, even though I'm pancreatic insufficient, I've never had too many GI issues, especially not constipation or a blockage, so I was really hoping that wasn't the issue, but I kept it in the back of my mind and added it to the list of things to discuss with my doctor.

I also hit another little bump in the road Tuesday evening, when I felt the all too familiar 'pop' and 'crackle' of hemoptysis. Ugh!! I hadn't had any episodes since my last appointment in January, and now two days before, it shows it's ugly face. Luckily it ended up being very small and went away quickly that evening. But Wednesday morning, while at school, I had another, even smaller, episode. Thankfully it was small enough for me to spit once into a tissue (the kids were not in the room at the time), take a drink and it was done. So I backed off my HTS Wednesday morning, and only did half a dose Thursday morning, and it seemed to be gone aside from tiny streaks.

Back to the appointment- it was at 11:30 which is probably the best time for a clinic appointment. Late enough to where I can leave my house at 8am and miss rush hour traffic on the way there and back! They got me in right away, weighed me (124.5 lbs), pulse ox (98%), blood pressure, etc...all normal.

Then come the PFTs! Basically the whole point of the visit to see how my lungs are doing after three months. The RT who does the tests is super friendly and I really like her, which always makes me feel better. I stood behind the computer on the first one so I couldn't see anything, closed my eyes, plugged my nose and gave it my biggest, hardest blow & breath as I could. When I peeked around the other side, I had to do a double take because I didn't see any 87, 88 or 89s. Now the screen is filled with a big grid with numbers everywhere, but I knew where to look. Then it hit me...it said 92!!! I said rather excitedly, "Does that say 92?!?!" The RT wanted to make sure I was looking at the right thing, so she doubled checked her notes, but while she was doing that, I just kept checking to see that I was looking in the right column. I was! My FEV1 was 92%!! So of course I got very excited and said, "Yes, it does! Oh man, I don't remember the last time I was in the 90s!" (And I may have thrown my arms up in the air in excitement, too...) Anyway, I did two more tests - next was 92 and the third was 90. I was so happy! I asked for a copy and she told me I got a gold star for the day. :-)

Needless to say, my doctor and nurse were very pleased with my results. My nurse thought it was funny how much of a reaction I was having because it's only a couple points higher than last time, and I've always been so close with 88 and 89 lately, but it's totally a mental thing for me. This is a milestone that I've wanted to reach since I started this blog five years ago, that I honestly thought was a bit unrealistic at the time, but it was a goal to keep me motivated. I honestly cannot tell you the last time my lung function was in the 90s. In high school I was in the 70s, so it had to be sometime in my childhood. Its amazing!

After the excitement died down a bit, we got back to business about the belly pain and hemoptysis. Doctor wasn't too concerned with the hemoptysis, but told me to let her know if it comes back. As for the belly pain, we figured out its most likely reflux/acid because I've been waiting on my Previcid to be refilled for over a week now. She told me to buy over the counter Previcid until I get my prescription in the mail. So I went straight to the store when I got back in town and too the meds in my car hoping this stupid pain would go away.

Needless to say, I had a wonderful appointment yesterday. I am so proud of myself for sticking with all of my treatments - it'll be five months of compliance on May 1st.

Compliance + Kalydeco = awesome lung function!

Imagine what it could be if I started exercising regularly again.....need to add that to my list of to-dos between now and my next appointment. I was 90s to be my new normal! :-)

Stay tuned, next blog post will look at my chest x-rays!

January Clinic Visit

Anna and I arrived home from a successful clinic day just a little while ago....but next time, remind me not to take a toddler on a six-hour round trip. :)

We started our day off around 5:15am, which is typical for us during the week. Tim accidently took the car to work because that's what he's used to, so Anna and I had to take the truck to Bloomington (luckily it's on the way) to switch it for the car. Poor Anna thought we had arrived at our destination and was not very happy with me when she went from one car seat into another. So for the next hour she was fussing and fighting sleep. Finally, she fell asleep and stayed asleep for almost another hour. We were on track to make it to clinic about ten minutes early, until we hit lovely Chicago traffic. Since we were completely stopped, I texted my doctor and let her know we'd be about a half hour late. The traffic ended up clearing quicker than I expected, so that gave us plenty of time to park and get in a diaper change/bathroom break without having to rush to check in.

So happy to be out of the car and in her "chair"

My appointment was pretty uneventful, which the doctor and I both agreed was a good thing. Thankfully, even though I'm not on Cayston right now, my lung function is pretty much the same as last time, at 88%. 😃 I've been lucky enough (so far) to not catch whatever Tim has and all the junk that's going around school. I hope it's able to stay that way these last couple months of winter, but that would be pretty much a miracle.

I talked to Dr. Dowell about getting an Afflo Vest, especially for travel, so she's going to see what she can find out for me before my next appointment. I'm also in the market for a travel nebulizer, one that can be plugged into the car. If anyone uses/knows a good brand, please let me know.

After seeing the doctor, we went to get my labs done and finally, a chest x-ray. I was concerned they wouldn't allow Anna in when I was getting the x-ray which would not allow me to get it, but they were very helpful. They x-ray tech just took her in the stroller behind where she stands and did both x-rays very quickly before Anna realized she couldn't see me. It worked out perfectly.

Anna really was a good sport during all of this at the hospital. She was talking to me more than I expected her to with people around, but of course, just stared at people when they talked to her. :)
Next time I take her though, it'll have to be a time where we come up the night before because that poor girl was so sick of sitting...who could blame her?! Every time we'd stop at a light she would say, "home" because she thought we'd pulled into the driveway; or while driving on the highway, many times she said, "Mommy, out!" As much as I know the doctors like to see her, and I like her to be comfortable with Mommy going to the doctor, I think next time she'll just stay with Grandma!

Kalydeco Is Not A Cure

Great, amazing, wonderful things are happening in the CF community, and have been for the last few years. The release of Kalydeco in January 2012, and now Orkambi in the beginning of July. They're making huge, positive impacts on a great deal of the CF population!
Unfortunately, I've heard these medications (more so, Kalydeco) referred to as a cure more times than I would like. They are NOT! They help fix the mutated gene, but do not completely correct/reverse it.

If Kalydeco was a cure:

• My lungs wouldn't be filled with bacteria-infested mucus
• I wouldn't still need to do 30 minutes of Vest twice a day (or more when sick)
• I wouldn't need at least 4 nebulizer treatments everyday
• I wouldn't need to swallow at least 15 digestive enzymes a day
• I wouldn't need several different vitamins and other various pills per day
• I wouldn't need to visit my CF clinic 3 hours away every three-four months
• I wouldn't have had an awful Spring with numerous episodes of hemoptysis
• I wouldn't need annual chest x-rays and blood work
• I wouldn't need a high-fat, high-calorie (watch the carbs & sugars though because you're borderline CF Related Diabetes) diet

I started Kalydeco on August 30, 2013. I expected immediate, dramatic changes when I first started the drug, but didn't notice too much of a change. Even though all of these things are true, I'm very glad that I had an excellent talk with my CF doctor (have I mentioned how much I love her?!) was

Dr. Dowell with Anna
from my clinic appt

reminded today how lucky I am to have Kalydeco and how much it really is positively impacting my life.

• Typically, CF patients exhibit a slow, steady decline in lung function over a few years time. My doctor kindly reminded me today that my lung function has been very stable over the last two years. Even when it declined during the beginning of pregnancy, it shot right back up at the end and has been stable since then.
• I have not had to increase my enzyme dosage which is also common for CF patients, which means my pancreatic function is stable.
• I've slowly and steadily been gaining weight over the last two years. This is one of the positives I have noticed over the last two years and have been very pleased with.
• I had a very easy, healthy pregnancy which may not have been the case without Kalydeco.

Despite not feeling immediate results, I am VERY lucky to be on Kalydeco, and now I can positively answer people when they ask how it's been working for me. I'm very happy to have 'normal' lung function, even if it means doing all of this work to keep it there! I hope it stays like this for a long time. :-)

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My Recent Health - The Good, The Bad & The Ugly

What a better way to kick off Cystic Fibrosis awareness month than a health update. Last time I posted about my health, I was doing very well! Unfortunately, just a few weeks after that post it started going downhill. I caught a cold which turned into a chest infection, so I emailed my doctor about getting on Cipro (an oral antibiotic). Well, it took much longer than usual for her to get back to me and fill the prescription, and in the mean time my lungs were feeling it. That was the first time in a very long time, if ever, that it just hurt to breathe. I was exhausted and felt like shit. My doctor was hesitant to give me the Cipro because its not breastfeeding friendly, but I didn't care. I had spent the last six months building a frozen stash of breast milk for this type of situation, so I wasn't too concerned.
We figured out a schedule that she and I were both comfortable with, then she called in a ten day supply of Cipro for me. I would nurse Anna first thing in the morning, then pump. Then I would take my Cipro and pump & dump the milk while at work while the Cipro was strongest in my system. By the time Tim and Anna came home in the evening, I was able to nurse her. After she went to bed, I would take my second dose so it was strongest while we were sleeping. The annoying part was pumping & dumping and bottle feeding on the weekend, but it was worth it to get my lungs back to normal.

Fast forward another few weeks to the middle of March. I was laying in bed one Saturday morning and I feel the 'pop' and gurgle...and I knew what was coming next - blood. I've had lung bleeds before, but not like this one. I tried to do a couple small little coughs, but I could tell this was straight blood and it was going to be a lot. I ran to the bathroom and just coughed it all into the toilet so I could monitor the color and consistency. Finally after a few minutes of straight blood, it started turning thicker, mucus-like consistency, but still bright red. I knew I had to contact my doctor abut this one, especially when it happened again that night and the following morning. This was unusual for me, so I was a little nervous, but I was also confused because I was feeling well prior to it and it seemed like it came out of no where.
My doctor started me on extra vitamin K (helps clot your blood) for a week or so, to see if it helped control the bleeding. After about a week, I didn't have anymore large bleeds and the streaking (mucus streaked with blood) slowed and finally disappeared. Sometimes bleeds happen when a mucus plug becomes loose, when an airway gets irritated, or at the sign of an infection. Since I felt relatively clear, we just assumed it was a fluke and my lungs just being annoying...

Until it happened again about a week after I stopped the vitamin K. This had me worried, but actually mostly annoyed! I was angry that my lungs were behaving this way and I was stumped because there didn't seem to be a pattern of when the blood was coming - it happened in the morning when I was laying down, at work once when I bent over, it happened after treatments... so this had me thinking there must have been more going on than just low vitamin k levels.
I decided to call my doctor and pick her brain, with the intention of getting on Cipro again. She was just as puzzled at what was going on and was hesitant to put me on Cipro again because the specific bacteria in my lungs was slowly growing resistant to it. She said she'd let me try the Cipro for ten days (the 10th day I had a scheduled clinic appt) and then we'd go from there. She said if my lung function was as beautiful as it was in January, then she'd let me stop the Cipro. If not, then we'd have to take a different route - possibly IV antibiotics. THIS FREAKED ME OUT!
I've never been on IVs before and I didn't want this to be the first time. I wasn't ready. What would I do about work? How long would I have to be in the hospital? Would I have to give up nursing - I don't think Anna or I are ready for that. It had me worried, but I tried not to dwell on it too much. I started my round of Cipro on a Monday, and by the following Monday I was feeling great. I was positive that I'd blow my typical numbers on my PFTs on Thursday...
Until Tuesday I woke up with post nasal drip and a sore throat...seriously?! 'Okay', I thought, 'this is just seasonal allergies, no big deal'. Then Wednesday rolls around with a lovely dry, itchy throat cough all day, UGH! I had a tight chest and I wasn't producing any mucus, which isn't typical for me, but I still wasn't feeling awful, but I was getting a little worried about my looming appointment the next day.

The morning of my clinic appointment, on the drive, I developed a mucus-producing cough so I tried to get out as much as possible. When Anna and I checked into the pulmonary section, we only had to wait a few minutes for a room which was pretty nice. We even got a room with a window which was excellent entertainment for a busy 8.5 month old baby. Soon after getting put in a room, the RT showed up with the lovely PFT machine...I did my first PFT and blew an FEV1 of 83% - thank goodness!! I was so worried they were going to be much lower. I did two more blows with the exact same result and felt a large weight lifted off my shoulders. Yes, my PFTs were down about 5%, but my weight was actually up about a half pound and my lung function didn't drop dramatically, so I felt that I was in the clear for IVs. Until...Anna and I were looking out the window, watching the traffic go by and I coughed up blood, right there in the doctor's office. Ahhhh, when was this ever going to end??? Thankfully it was a small bleed that turned into mixed blood and mucus fairly quickly, but I was so nervous to show the doctor. I thought for sure that she was going to recommend IVs now.

When she first came in the room, of course she had to say hi and talk with Anna before we discussed my health. :) She was pleased with my weight, but reminded me that my lung function was down. We discussed, in depth, everything that had been going on over the last couple months and brainstormed several ideas as to why it was happening. Thankfully, she's so wonderful and trusts me and how I tell her I'm feeling, and she was not going to put me on IVs right then and there, since I was feeling well and it seemed like I caught something within the last couple days. PHEW!
We decided to attack from all angles - I would continue my Cipro for another week (21 days total), adding Cayston (inhaled antibiotic) on top of it. We were hoping the overlap of Cipro and Cayston for about a week would really knock it out. I was also going to start Nasonex to attack whatever was going on in my sinuses that was not allergies. Then I'd come back in a month to see how everything worked and how I was feeling, and possibly do a round of Tobi (different inhaled antibiotic) just to keep things clear.

She was exhausted from my appointment, too!

So here I am, on day 20 of my Cipro and day six of my Cayston and I'm slowly starting to feel better. Whatever I caught right before I went to the doctor really hit me hard, and I'm glad we came up with this treatment plan because I have never coughed so much mucus out each day as I have for the last week. I think the combination of Cayston and Cipro really has been working. Fortunately, I haven't had any bleeding since the day at clinic, but I'm definitely not back to 100% yet and I'm really hoping the Cayston will help get me there by the end of the month. The talk of IVs and them being a possibility in the nearer future than I'd like still freaks me out a bit, but after conversations with Tim and several of my cyster friends, I am trying to remember that IVs are just another tool in the toolbox, not the end of the rope. I'm very fortunate to be this healthy at this age and that there are still so many antibiotic choices available to me.

Me, Myself & I (and a little about Anna)

My apologies for posts about Anna taking over the blog lately. As I have come to quickly realize, having a baby pretty much takes over your whole life (and I wouldn't have it any other way). Anna's doing very well, but I'm using this post to update about me.

I've been doing very well and enjoying motherhood so much! Of course it completely sucks working all day and not seeing Anna, but I know that she's in excellent hands. Before going back to work, I was worried about how my body and health would hold up with teaching, taking care of a baby and nursing. And I'll admit, although I feel its going pretty well, it's hard giving my 100% all day, every day at work because I want to be home with Anna. But I take pride in my job, and I want to do it well, and I hope that I'm getting back (even if its a bit slow) to being there for my students as much as they need....because they are extra needy this year.

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On the health side of things, I really couldn't be much happier. I just had my clinic visit on Thursday and it went very well. I'm up to 118.6lbs and my FEV1 was 88%!!! I haven't seen lung function numbers like that in two years and I haven't been at this weight (aside from pregnancy) in almost three years. Of course I'd love to be in the 90%s for my lung function (can't remember the last time I saw numbers like those), so that's my goal. But I am very proud of where I am now and still so amazed at what this "sick" body can do. :-)

I've started working out again, this time with several teachers after school doing PIYO work outs. We've been doing it for a few weeks now and they're great for strength exercises. To work on my cardio, I also began the Couch to 5K program yesterday, so I'm planning on balancing the two somehow.. I signed up to run a 5K in April, and I'll be running it for CF. I'll be on Team CF Superheroes and I'm really looking forward to it. I'm also planning on running Brett's Run again in June, which was the first race I did in 2012.

Overall, things are going wonderfully over here. Hopefully I'll keep finding some time to update on myself, but in the mean time, here's a super cute picture of Anna sporting her Rock CF gear at my clinic appointment on Thursday.

36 Weeks

Wednesday, July 23 - 36w, 5d
Friday, the day I hit 36 weeks, I had a CF clinic appointment. Prior to the appointment, I expected my lung function would probably be around 70% still just because I didn't feel completely different from my appointment six weeks before. To my surprise, my PFTs were no where near 70%...

That's right, an FEV1 of 83%!!!! I'm back to my pre-pregnancy baseline!! I was ecstatic! My first round, my FEV1 was 79%, the second is shown above, and the third one was around 80 or 81. I am so happy! It's a relief knowing that I'll be heading into labor & delivery with my pre-pregnancy lung function! I have to attribute this major increase to you dropping (didn't happen overnight and have a huge 'relief' feeling, but I can tell you're sitting lower) and me starting Cayston about a week before I did this test.
My doctor was pleased with the results, of course. We also discussed my lack of weight gain and thankfully, she's not as concerned as I thought she'd be. She said it's just going to have to be a threshold thing, see if I can tolerate the frequent bathroom trips and extra gas/bloating for another three-ish weeks. I told her I've gone this long, I can make it until you're born. Plus, there's not much else I can do...I've already increased my enzymes to the max amount and I'm taking two probiotics a day. They suggested adding Ensures back into my diet to try to add in extra calories, so we'll see if that helps at all.

After my appointment, I stayed with Auntie Catie for the weekend while Daddy was at Uncle Tony's

bachelor party. Catie and I caught up, pampered ourselves (she got a manicure, I got a pedicure) and visited with her family - my second family before moving away for college. It was a nice, relaxing weekend. Monday I spent a couple hours working in my classroom, and went back again on Tuesday for several more hours to meet with my sub to finalize things for the beginning of the year. My plan is to have everything completely ready to go for her (and my sanity) by the end of the day tomorrow. This way I can enjoy the wedding weekend and be prepared to have you any time after & not be stressed about what still needs to be done at work.

Pregnancy-wise, I really can't complain. You're still moving a lot every day (not at night when I'm sleeping, thank you!) but your movements are different now. You like to shake your booty from side to side on the top of my uterus, right near my belly button. Sometimes, I'm guessing when you're stretching, you stick it out really far and my belly gets super hard in one spot & we can feel your butt! You also like to stick your little hands/elbows/knees/feet out on my left side. We're never exactly sure what we're feeling, but it's usually a little, hard, round thing poking out from time to time and it's so cute. My Braxton Hicks have noticeably picked up this week, so I'll make sure to let the doctor know tomorrow...hopefully that means my cervix is starting to ripen, even just a little. And thankfully, I've still been able to sleep great each night. Some women have trouble sleeping this far into pregnancy because they're uncomfortable and/or because their babies will move around a lot overnight. You don't move from the time I fall asleep to a little after I start waking up in the morning - let's hope that means you'll be a good sleeper! Occasionally I'll feel you shift when I'm in the process of rolling over for the 100th time, or when I lay down after getting up for the third time to pee, but you seem to fall right back asleep like mommy does. :)

Oh, and how could I forget, your cousin, Alayna, was born today!!! She's so cute, looks a little like Isaac and has lots of hair! I can't wait for you two to meet and to watch you grow up with all of your cousins!


Thursday, July 24 - 36w, 6d
I had another OB appointment today. They did the ultrasound first where you did everything you

Here's the back of your head & that white blob on the back, top
left side is your hair flowing in the fluid.  

needed to do so that I didn't have to get a non-stress test. Your heart rate was 153bpm, you moved quite a bit, practiced your breathing a little bit and were way head-down. After the ultrasound, I met with the doctor (weight: 129). She's extremely happy about my last CF appointment and there really wasn't much to talk about because everything's been going so smoothly. I asked her when they typically start checking my cervix, and she answered that usually they don't on most women because in their clinic many women don't make it to term. But she said she'd be happy to do it if I was curious, but she wouldn't change how she was going to treat the pregnancy and didn't think I'd be dilated yet. Of course I was curious, so I asked if she would check me anyway. My cervix is about one centimeter dilated and is about halfway thinned out, which the doctor was happy about because it showed that my body is slowly getting ready (I have been absolutely amazed at what my 'sick' body has been able to do this pregnancy!). She said she could also feel that your head was way down low which was a good sign because it indicated that you'll most likely fit through my birth canal, yay!

Clinic Appointment

Today's appointment was...okay. The doctor and nurse both commented on how 'cute' I look and that I'm all belly. I've gained a pound since my OB appointment last week, so I now weigh 131lbs! This means I've gained 17lbs so far this pregnancy which I'm pretty proud of. :) I explained to my doctor and nurse about my gas, bloating and extra bathroom trips and they suggested increasing my enzyme intake for a while, with all meals & snacks, to see if that helps.

As I mentioned in my post yesterday, I've been off of Cayston for a week now and have noticed an increase in my mucus production since stopping. When they listened to me today, they said I sounded great and I told them that I'm still feeling really well lung-wise, aside from my increased cough over the past few days. Before I did my PFTs (which took forever because the system quit working about an hour before I got there), we decided to start me back up on Tobi since my mucus and cough are increasing, so I'll be starting that probably next week.

Finally, they found one of the other CF doctors who was able to use the study PFT machine/cart, so he came in and did them with me. Apparently the system is a bit different and he had to click on things at a very specific time for it to register...so I ended up doing the test six times and only two of them registered. It was a great work out for my lungs and I was able to give my doctor a nice sputum sample afterward. My average FEV1 today was 70% and my FVC was 71%, which I was initially very bummed about! I was expecting them to be in the upper 70s with the way I've been feeling - not unchanged! My doctor assured me that she was very pleased with those results because I've been able to maintain my lung function during this portion of pregnancy. So overall I've gone down about 10% from my baseline during my entire pregnancy, and it was all lost in the first 16 weeks (well probably during weeks 14-16) when I was off Kalydeco and all antibiotics. The fact that I haven't lost any since then is apparently wonderful. Of course I always want to do better, but with this little lady slowly taking up more room, I guess I can settle for maintaining for now. I hope that the Tobi helps out and that I don't lose anymore of my ling function this pregnancy. I'll be going back to the CF doctor in just over a month (probably right as I wrap up my Tobi cycle) to make sure everything's still going well. :-)

CF Clinic Tomorrow

I finally have my next CF clinic appointment tomorrow afternoon. The last time I was there was in February, I was 16 weeks pregnant, had been off Kalydeco and any antibiotics for months, felt like crap, and blew my lowest FEV1 ever...clearly, not a great appointment.

Since that appointment I've started my Kalydeco back up (the night I got home from clinic), did a 28 day cycle of Tobi in March, a 28 day cycle of Cayston in May, have stuck to my treatments diligently, and am 30 weeks pregnant. At the end of my Cayston cycle, my mucus production was pretty much non-existent which was unusual, but a welcomed change for once. Since being off Cayston for just over a week, I've seen a slight increase in my mucus production, but I've still been able to take deep breaths and have been feeling generally well lung-wise.

Except, over the past couple of days, my mucus production has significantly increased, especially during the day, but I still don't feel CF-sick, or any kind of sick for that matter. I'm curious if this is pregnancy related or CF related, and I'm very curious to see what my FEV1 looks like tomorrow. A week ago, I would have bet money that it was back in the low 80s, but now I'm not sure where all of this extra mucus is coming from and if it's going to effect my results tomorrow....as long as they're up! I know that one PFT doesn't determine too much as far as how I'm feeling, but like I said, a week ago I felt like my lungs were so clear and now they're filling up out of no where....I guess that Cayston really did something.

I've also been feeling extremely gassy and bloated again this week, but I think this is pregnancy related somehow. I had these same symptoms at the beginning of May, around my 25th week and they lasted a couple weeks. Then I thankfully had a couple weeks of relief until it started again at the beginning of this week.

Don't really have a point to this post, other than documenting how I'm feeling before my appointment tomorrow because I really have no idea what to expect. I'm assuming I'll have to go on at least one more round of inhaled antibiotics before delivery so that my lungs are in the best shape possible when Baby Girl arrives. Stay tuned tomorrow evening to see how it all goes down. :-)

Clinic & 15 Week Update

Today I had my first CF clinic appointment since becoming pregnant. And to be completely honest, I've been feeling like total crap the past few weeks. Thanks to my stupid insurance change, I was supposed to start Tobi a week or two ago, but it's still being approved, ugh! So since I haven't started the antibiotics yet, I knew my PFTs were going to be pretty low today.

It started a few weeks ago, when I felt like my lungs became flooded with mucus over night. It came on so suddenly and not like a typical CF chest infection, that I was thrown off. I had an increased cough and increased mucus production, but it wasn't really a different color, just so much more. Of course I tried to fight it myself, but it got to the point where it was interrupting my sleep and making me completely exhausted, so I called in the "big guns" aka: Tobi. Now, I haven't been on a full month of Tobi since August, so I really thought it would help clean me out... but insurance decided to get in the way of that...thanks insurance! Then on top of it, I started developing some crazy sinus infection which has clogged my sinuses so bad that it started coming out of my eyes yesterday morning, I know, lovely! So I've been doing two sinus rinses a day for about a week which temporarily help, but it wasn't enough. All I have to say is... thank goodness I had my appointment today!

Anyway, so my PFTs were down, as I expected. My FEV1 was 69% - I've never seen numbers that low, but I can't say that I was surprised. My doctor didn't seem as concerned as I thought she would and she said that my PFT graph looks symmetrical and looks much better than how I'm feeling, so that relieved me a bit. She also wasn't too concerned because thankfully, I have plenty of options right now. I'm starting my Kalydeco back up tonight after being off of it for nine weeks - I'm glad that I was able to stay off of it during the critical development period of my pregnancy. I'm also going to start Tobi whenever my insurance decides to cooperate. As for my sinuses, I'm going to continue my sinus rinses followed up with some Nasonex. I'm hoping that the combination of these things will get my lungs back to normal soon before Baby starts squishing them.

As far as my pregnancy goes, everything is going very well, today is the last day of my fifteenth week. As I said before, I'm proud of myself for not exposing Baby to Kalydeco, or any antibiotics, during its first trimester. This week I've been pretty exhausted...I feel like I'm feeling more like I should have first trimester now in my second... but I think I'm finally on the right path to feeling better. This week, the baby was the size of a naval orange and could sense light shined into the belly even though its eyes are still fused shut, and can now move all of its limbs and joints now. I can't wait to feel Baby move soon!

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?! :-)

Clinic Day & Positive Thoughts Thursday

I had my CF clinic appointment today and number-wise it wasn't the best, but feeling-wise it went well. Everything's pretty much the same as my last appointment - my lung function is still hovering in the high 70s which I really don't like to see, but I've been feeling really great lately. My weight is about the same - today it was 108.5. Thankfully I have a wonderful relationship with my doctor and I was able to explain to her how I was feeling and she understands that sometimes the numbers don't reflect that. She just told me not to worry or focus on the numbers which made me feel better...as long as things don't keep going down, that's what matters. Of course I'm still trying to increase my weight and lung function (always!), but for some reason it's just sticking where it is right now, so I think I'm going to really have to take advantage of the next month and really kick my butt into gear. I need to make sure that I'm consistently exercising and consuming at least 2,500 calories per day, especially before I head back to work.

We did discuss the possibility of adding Kalydeco into my life if I can't seem to get out of this rut which really makes me excited. When Kalydeco first came out I was honestly a little hesitant about going on it. In a weird way, I felt like if I started taking Kalydeco, then I was "giving up" on fighting CF myself. After typing that out, it sounds totally strange, but that's the way I felt. I feel like I've been doing a really great job keeping myself healthy and I didn't want to....I don't even know how to word it... I was also concerned that 'What if I was taking it for several months, saw a great increase in lung function, and then for some reason I had to stop taking it and then my lung function would go back down (to the 80s which is still 'normal') and I would feel like complete crap.' I was nervous that once I started it, it would be something that I'd never be able to live without, something that I'd rely on forever.
But after a great discussion with my doctor today, she put it in perspective for me. Everything that I'm doing now -Vest twice a day, six-eight nebulizers a day, countless amounts of pills, etc -I'm going to rely on those forever to keep me healthy, so what's another pill? Especially if it's going to change things for the better, increase my quality of life, and possibly reduce the amount of medication/treatments I do now...duh! What was I thinking?! So the plan is to see how the next few months go and at my next clinic appointment in October we'll make the decision on whether to start the approval paperwork, or to keep it on the back burner.

And finally, I've totally been slacking on my positive thoughts Thursday posts over the past few months, honestly, because we've been completely stressed...which is probably when I should be doing it, but either way here's some recent positive thoughts:

• We are 15 days away from our scheduled closing date on our first home! As long as everything keeps going according to plan we should be moved in before August! Yay!
• The possibility of Kalydeco entering my life is starting to get me really excited it. Of course it may not be until the end of this year, it's something that I'm looking forward to. :)
• I love summer break! Not a huge fan of summer weather, but the time off is amazing! I've been able to do way more this summer than I even thought I was going to do and there are still several more fun things planned...next one on the list: Country Thunder! One week from today! Oh yeah!
• Our new little guy, Ozzie, is adjusting very well to life in our home. He has an enormous amount of energy and he's hilarious to watch when he's running around! Archie is also doing a wonderful job being patient with him and playing (usually) gently with him. 

Can you tell who's who?! Haha...Archie's (4 yrs old) a big hefalump, but
Tim thinks Ozzie (3 months) might get to be about his size...we'll see. :) 

Holiday Weekend

We had such a fun and busy 4th of July weekend! We spent Thursday during the day hanging out with all of Tim's family and then that night we enjoyed fireworks together. I have a soft spot for the fourth of July because that was the night that Tim proposed to me, four years ago. :-)

The following day, my 25th birthday, a bunch of us went together to see Despicable Me 2. It was so cute, during the show, our three year old nephew, Cole, would tap us on the shoulder and say, "Tim, Tim,(or Coyeen, Coyeen) shhhhh!" when we weren't even talking, haha. :) That night they also surprised me with a delicious Reese's ice cream cake!

On Saturday, Tim's other sister, her husband & their son came up for the day to visit. We hung out at Tim's dad's house where some went swimming while I was on baby Liam watch. :) Then we visited Tim's mom so she could see all of the boys. Finally, on Sunday, I drove up for my friend Catie's bridal shower while Tim stayed to hang out with his sister, her kids, and his grandma.

When I came back from the bridal shower, we packed up all of our stuff from the weekend, headed out to Tim's dad's house to pick up our kitty, Ozzie (which we finally agreed on the day before we got him) and finally bring him home! He is so adorable and FULL of energy! I've never had a kitten before so this is a totally new experience for me. Archie is being very patient with him and they even were playing together for a little bit today without Archie being totally overbearing -- he means well, he's just super friendly...which is kind of scary to a tiny kitten. It's been an interesting (and exhausting!)
couple of days having him home, but I love it. It's going to be so fun when him and Archie are comfortable with each other because I think they're going to get along very well.

Next on my summer 'to-do' list is my CF clinic appointment which is tomorrow. I'm planning on heading up to stay with my parents this evening to make the drive more bearable tomorrow morning. I'm hoping for a slight increase in my weight and lung function. I've been trying to do my medicine ball workout video a couple times a week and I'm at 131 days of 100% compliance with all of my treatments. I've been feeling good, coughing out a lot of nasty mucus (but not anything more than usual, which is good), so I'm hoping that shows tomorrow. If not, at least I know that I'm feeling well and that's what matters.

And hopefully in the next week or two I'll finally be able to share with you something that I'm totally proud of...as for now though, my lips are sealed. ;) Stay tuned....