My Seester!

We are the world's okayest
sisters!

When you have a disease that takes up a lot of time and energy, it's easy to forget that you're not the only one the disease affects. My younger sister, Alaina, has always been by my side and one of my biggest supporters. I asked her a few questions about what it's like to have a CF sibling.

How do you think having a sibling that has a terminal illness affected you when you were growing up?

Ya know, I've thought about this before, and I kind of feel like when we were younger, it wasn't really a "big deal". I feel like Mom and dad always did a really good job of not highlighting the fact that you had a terminal illness (at least to me) It was just something you had to deal with a little differently than a "normal" kid. I don't ever remember feeling sad or scared about your CF until we got older and I had a better understanding of what it was. 

I’ve read articles about parents that are worried that their non-CF child will be jealous of the attention that the one with CF gets. Were you ever jealous or resentful of any additional attention that I might have gotten?

I'm sure there were a few times growing up where I was jealous of the extra attention that you got, but again, I think mom and dad did a pretty good job of making me feel equally as special. If anything, I think I maybe got away with more stuff than the average kid may have. I do remember plenty of times that you were angry or annoyed that I got all the same privileges as you even though I was 2 years younger. i.e. same curfew, same allowance, etc.

Both of us have made the decision to not have children. Did the fact that you may be a CF carrier play a part in your decision?

It did play into it a bit, but that wasn't the only deciding factor. If I had really wanted children, I would have definitely had myself tested for the CF gene prior to finalizing the decision to do so. 

Since I was pretty healthy growing up, we didn’t deal with a whole lot of CF stuff. I kind of feel like I missed a “CF 101” class along the way and still get caught a bit off guard when people ask specific questions about it. Do you feel like you could adequately explain what cystic fibrosis is to a stranger?

I feel pretty confident now while explaining what CF is when people ask me about it, but when we were younger, no way. I knew that whatever it was, it made you cough all the time, mom would always ask you about your poops, you had to take a buttload of pills with each meal/snack, and we had to take frequent trips down to Denver because of it. As I got older I realized that I hardly knew much about it and took it upon myself to research it more thoroughly. 

You are a massage therapist and yoga instructor/owner of Om On the Range Yoga & Massage. Do you think having to go with me to the hospital every 3 months for our whole childhood led you to a career in in the health industry?

I had never really thought about that. I imagine it probably had something to do with my interest and comfort level of being in hospitals and health centers. It did, however play a huge part in emphasizing the importance of breathing! I remember going along with you when you would do your lung function tests and having the docs give me a shot at it as well. I remember them teaching you how to take deep belly breaths and that has always stuck with me. In massage school and Yoga Teacher Training, they say how many folks never use even half of their lungs when they breath and I found it as shocking since taking big deep breaths was such a second nature thing in our household.

If you could give any advice to a kid that has a sibling with CF, what would it be?

Love them and treat them just like any other sibling. Don't treat them differently than you would just because they have CF (within reason. I realize that not all CF kids are as capable and healthy as my sister was, and may not be able to take part in all the "normal" kid activities). But play with them like they're your sibling, and fight with them like they're your sibling, because that gives them some normalcy in their lives. But, also, be curious about their illness and ask as many questions as possible, and be there for them when they need you. And just because they may be sick, doesn't mean that they can't be there for you when you need someone to talk to as well.

Follow Ups & Flu Shots

Yesterday was quite a busy day. I had a follow up clinic appointment at 11am and since I'm out of vacation days I worked from 5-9, so I was up quite early to get ready (I'll be spending the rest of the week working through lunch to make up the other 4 hours). We left town at 9 and made it to Seattle just in time to check in.

My appointment went pretty well. My lung functions are up 6% which makes me extremely happy and helps to make it feel like all of the extra treatments I do now are worth it. It's a huge relief to have my lung function be closer to 90% than to 80%!

My husband tried to get video of me doing my PFT, but the RT kept standing in his way. One of these days I'll be able to get a video so I can show you just how much fun blowing into a machine is. I didn't have to do much else at this appointment since it was just a follow up to see how the Pulmozyme is doing. Now I just have to wait for the results of my sputum culture to see if I grow Pseudomonas and have to repeat the treatment for that.

I also got my flu shot, so I'm going to add a little PSA here about how everyone should get a flu shot. You may not think you need it because you don't fit the stereotypical mold of being sick or elderly, but it helps protect everyone around you. I loved that last year we had them done at work, since I figured it would cause more people to get them instead of having to go on their own and therefore I would get sick less. Unfortunately they weren't offered this year, so I'm hoping it's a mild flu season.

After I was released, we treated ourselves to some lunch and shopping to make a day of it in the city. Got back to town in time to get dinner and catch up on The Walking Dead.
**We didn't get to watch it on Sunday night because we were busy watching the Sounders smash Dallas in their playoff game;)

Lungball

I know by now I've mentioned a couple of times that I've only ever been hospitalized once (I'm not counting surgeries, that's another post). Well let me tell you the story about that one time, cause it's a good one. It was about 9 years ago and probably the longest and scariest 10 days I've ever experienced...

My husband and I (not married at the time) were back in our hometown for our friends' wedding. I don't really remember feeling bad, but I was freezing and could not get warm. It was the middle of July in Wyoming so there's no reason I should have felt this way. I distinctly remember borrowing a hoodie from my sister and sitting on my parent's front porch in the sun for a long time and still not being able to feel warm. But we had things to do and people to see, so I went on with business as usual. The night of the rehearsal dinner I started noticing that I didn't feel well. When I'd take a breath it felt like my lung was going to pop. I tried going to the bathroom to see if maybe I just had a gas bubble, and my husband tried popping my back to see if something was just out of whack, but neither thing worked. As the pain intensified and I could barely take a breath in, I decided it was time to call the hospital and see what I should do. They told me that I could have a pneumothorax (air between lung and chest wall) and to go to the ER right away.

So scared, crying, and in pain I headed to the hospital. Luckily it was late at night and not very busy so I got in pretty quickly. I had x-rays done and blood drawn. The nurse that did my blood draw was more interested in flirting with my husband and ended up blowing a vein in my arm (thanks a lot lady). An ER doctor came in and told me that I had a cyst in my lung and I would need to be admitted. I panicked. I had no idea what a cyst in my lung could mean and I remember asking if it was something that they could fix. He kind of chuckled and said yes, like I was being over dramatic. Doctors just assume that when you have CF, you're used to things like this happening. I also was running a pretty high grade fever (hence the freezing) and my brain wasn't thinking super clearly.

I was checked into the hospital and had more tests done. Needless to say I missed the wedding and after two days in that hospital we decided that it would be better if I went back to Denver and saw my CF doctors. I won't even go into how horrible my experience at this hospital was, but if I'm ever back in my hometown and have an emergency, I'm screwed. I checked out AMA (against medical advice) and we started the journey back to Colorado.

The x-ray from after my lungball
had been popped. It was in my upper
left lung.

It turned out that the 'cyst' was really a MRSA infection abscess. The x-rays were actually kind of amusing, I wish I still had a copy. It looked like I had a rubber bouncy ball lodged in my lung, like the kind you find in the quarter machines at grocery stores (why we called it my lungball). I was outfitted with a PICC line (an IV line inserted into my upper arm that ran to just above my heart), since I would be on antibiotics for an extended period of time. They also had me start using the Vest (first time I'd ever heard of the jiggly vest) to try and pop the abscess so the antibiotics could reach it. This whole time my lung functions were above 100% and my O2 saturation was at 100%, the doctors found it very odd and I even had a medical student write a paper about me, neato!

Well nothing worked to burst my bubble, so they ended up having to do a chest tube to pop it manually. Not really an experience I wish to ever have again. Luckily it wasn't an emergency so I was under the influence of some heavy pain meds, which helped. After 10 days of total hospital stay I was going a little bit crazy and managed to cry my way out of there. Hospital stays are no fun. You barely get to sleep since someone comes to check on you every hour and draw blood. Side note: thank the heavens for PICC lines because then you don't have to get stuck every time they draw blood. The second night I was in the hospital in Wyoming I had to beg the phlebotomist to draw out of my other arm, because they kept using the same one and it was so raw and sore. She got super huffy about it because my IV was in the other arm so she had to have a nurse stop it. Cow.

Once I was released I had to go back every morning and evening to get my hard core IV antibiotics. This meant driving across the city of Denver (quite literally) and back twice a day and going to work in between IVs, the fact that I was totally okay with doing this is how much I wanted to get out of the hospital. Eventually I was placed on some lower dose antibiotics that my husband could administer from home. He had to learn how to flush out my PICC line and setup the IV. He was amazing through the whole thing, he slept at the hospital for several nights, but since we lived across the city and he had to work I talked him into sleeping at home and just coming by after work. Once the home IVs were done and all of my follow up tests came back clear I got my PICC line out, which was just a nurse saying "take a deep breath" and pulling 3-4 feet of IV tubing right out of my arm. Yep, that is as fun as it sounds.

I had a lot of firsts during this time that are old hat to most CFers, so it was quite a learning experience for me. It was all very scary and exhausting, but luckily I came out the other side of it okay, if a little bit (a lot bit) paranoid. I called into the CF clinic for every little twinge for a few months and probably drove the nurses a bit crazy. I haven't tested positive for MRSA since then which is fabulous. I really don't want a repeat of that experience again.