Surgeries

The incision on my stomach was pretty big!

My first surgery was at 2 weeks old. I had a pyloromyotomy (say that 3 times fast, I dare you) to widen the opening between my stomach and small intestine. The official term for what I had is Pyloric Stenosis, a blockage of the opening, due to a thickened pyloric muscle. Considering stomach/digestive issues associated with CF, it's surprising this is completely unrelated to it.

My parents were in Casper visiting family and showing off their adorable new baby, and I was having major issues with projectile vomiting. My mom took me to the doctor to have it checked out and found out that all 6 lbs of me would need emergency surgery. I do need to mention here that the doctor that diagnosed me with this is the same doctor that will 5 years later diagnose me with CF. Shout out to Dr. Green and my mom for essentially saving my life, not once, but twice!

Now I can't image that sending your new baby into surgery is a walk in the park and I was so small that they had to put the IV into my head. After surgery my mom was able to see me and the IV had slipped and started filling the space between my skull and scalp with fluid. She tells the story much better than I can, but needless to say she was a little shocked at my new alien-like appearance.

The surgeon wasn't exactly delicate with their incision or sutures so I still have a pretty distinct scar on my stomach. I'm told that it looked like a caterpillar because of the way the sutures stuck out of my stomach and to this day it still looks like a giant bug.

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Luckily I was able to make it several years without having to go into surgery again, but my second major one was just as unplanned. I was 25 and living on my own in Denver. I hadn't been feeling well and started to develop severe pain in my abdomen and wasn't able to keep much food down. At this point I hadn't really been seeing my CF clinic on a regular basis, so I went to an urgent care clinic. After a blood test (high white count), vaginal ultrasound (maybe it was ovarian cysts), and a CT scan, I was sent to see a surgeon to take out my appendix. They also saw a spot on my liver in the scan so I had a liver biopsy done at the same time.

A week after having my appendix out and still not feeling any better, I got a call from the surgeon that my tests came back negative for appendicitis, which was very frustrating to say the least. I called my CF clinic and explained everything to the nurse, and in a heartbeat she said that it was DIOS (an intestinal blockage) and made an appointment for me to get that treated. She was correct, and that was my hard-learned lesson to always check with my CF clinic first.

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My last major surgery was a few years later to have my sinuses cleaned out (scraped) along with a septoplasty and shaving down of my turbinates. Sinus issues are a big part of having CF and most CFers get this surgery every so often to clear things out. Incidentally my non-CF sister had to have this same surgery, so it's tough to say if mine is fully CF related, or if I'm just super lucky.

This surgery was quite possibly the best thing that I've ever done. I was having daily debilitating headaches by the time I had it, and though I still get sinus headaches, they're nothing like they were before. Every few years I get a CT scan done of my head to make sure my sinuses are still clear and luckily I can say that they are. Most of my sinus problems now are just due to swelling, which is why I use a nasal spray every day.

Interesting side note: I found out that I don't have a sinus cavity above my right eye at all, it just never formed (insert hard-headed jokes here). One way that chronic sinus issues can be treated is to obliterate the cavities, so I'm partway there naturally!

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.

New Habits

How do you change 30+ years of habits (or lack thereof)? How do you deal when one day everything changes and you have to say to yourself “Ok, this is me now. This is my new reality.”?

I know I say this a lot, but I’m going to say it again. I’m really lucky with my CF. I know that I am and I can’t even express how deeply thankful I am for it. Most people with CF don’t get to live the essentially normal life that I’ve been able to lead up to this point. But right now I’m feeling like it is coming with it’s own set of drawbacks.

The Vest & nebulizer corner where I
spend time every morning & night.

If I had had to do chest therapy for my whole life I’d be used to it. It would be part of my daily routine that I wouldn’t think twice about. But now instead of coming home from a long day of work and crashing on the couch, I have to go put on my Vest and have my body pounded on for 30-45 minutes while I nebulize saline. Instead of hitting the snooze button a few times, I have to roll out of bed at the sound of an early alarm to do my nebulizer(s) and sinus rinse. Instead of doing whatever I want when I want, I have to make sure to plan around my treatments so I don’t miss any. Instead of taking days off when people come to town, I have to use half of my vacation days for doctor appointments.

Could things be worse? Yes, 100% they could. Do I feel grateful for the fact that I’m still relatively healthy? Yes, completely. But does that mean that I can’t grieve for the way of life that I’ve lost? I’m at the point where a lot of the novelty has worn off and I’m having to come to terms that this is how it will be forever. That more will be added down the road. I’m trying really hard to make all these new things a habit, but it’s hard. I’ll be the first to admit that I don’t have the greatest of will power. I have to lecture myself everyday about why I need to use the Vest. I’ve had my setbacks, missing a week or two by taking a day off and then repeatedly telling myself that I’ll start it back up again tomorrow. I’m having to learn that a life with CF means no days off from treatments and it’s a bit overwhelming.

I’ll make it through this period of change, I know I will, there just might be a little kicking and screaming along the way.

*My parents did have do chest percussion on me when I was a kid, but at one point the doctors said that we didn’t need to and even later in my life when I got the Vest it was a “do it when you feel like you need it” kind of thing, not a strict regimen.

Fitting In

I just got done attending the first BreatheCon, a virtual conference for adults with CF. It was quite
something to get to meet so many other people with CF and hear their stories!

I have to say that I didn't say too much in each session beyond introducing myself. As I listened to some of the other people talk, I felt that I shouldn't share my story too much. Yes I have CF and that gives me several things in common with them, but I don't feel comfortable trying to get someone that's in their 20s with a 30% lung function to commiserate with me about just now having to start a "typical" CF protocol.

Attending BreatheCon 2016

At one session the discussion came around to some of these new CF drugs (that I don't qualify for because of my mutations) and how they've kept people out of the hospital and off of IV antibiotics for a year. How can I possibly come in and say that I've only ever been hospitalized and on IV antibiotics once in my whole life without sounding like a smug asshole? So I didn't say anything and just nodded in appreciation of how great these new drugs are for cystic fibrosis.

CF is already very isolating, but when you don't fit the "mold" of a person with CF it can be even more so. I walk this weird line every day, I'm not healthy because I do have CF, but I'm not sick enough to really be a part of the CF community and I'm not sure how to deal with that and I've honestly struggled with it for my whole life. I was really hoping to meet some other people in the same situation, but either they weren't at the conference or I just didn't end up in any sessions with them.

So I'm left feeling a little disappointed, but at the same time a little inspired by some of these amazing people that deal with so much on a daily basis and take it all with such grace. I can only hope that when it does come time for me to go through some of this I can handle it half as well as they do.

Clinic Visit, New Meds, and a Pity Party

I know they're for everyone's safety, but
I really hate these masks.

I had a clinic visit yesterday and had intended to write a full post about what is involved in them. Turns out my visit was a pretty simple one. I didn't have to do most of the normal tests or see most of the usual people, they didn't even to a blood draw. Way to ruin my intended blog post guys!

However, one test that never gets skipped is the PFT (pulmonary function test). This measures the capacity of my lungs to see disease progression. Growing up my FEV1 was always about 115%, but over the course of the last several years it has gone steadily down to about 83%. My functions have been stable for the last 18 months, which is good since I don't want to see the decrease keep going. But I had my hopes up (probably a little too much) that between running and doing my vest & hypertonic saline nebulizer on a consistent basis I would see an increase. I asked my doctor if it's even possible to see an increase or if this is just going to be my new baseline. We decided to try adding Pulmozyne to my routine to see if that will help bump my numbers up.

Pulmozyme is one of those medicines that most CFers get put on, kind of like digestive enzymes,but I've never needed it since my lungs were so good. I'm pretty bummed that I have to do this and that everything I've been trying isn't helping. I can be pretty competitive and controlling, so I'm feeling a little defeated that I need to start this new med. Not to mention the fact that I now have to get up about half an hour earlier in the morning to do it since it's a nebulized drug. I'm still waiting on insurance/specialty pharmacy to get it, but hopefully it will help and I'll see a rise in my number next time.

Technically I'm a Mutant

Cystic Fibrosis is caused by genetic mutations, so technically I'm a mutant, but sadlyI don't have any super powers (unless you count turning into a human salt lick after a work out or being able to swallow a handful of pills without choking).

In order to inherit CF a person must get one defective (mutated) gene from each parent. A person with only one mutation is a 'carrier' of the CF gene. When two carriers have a child, there is a 25% chance the child will be born with CF, a 50% chance a child will be a carrier, and a 25% chance the child will not have CF and not be a carrier. Thank you high school biology!

There are roughly 1900 different mutations of the CFTR gene that can cause CF, which means that there are millions of different combinations of mutations. Since every mutation presents differently, you can see why CF is a difficult disease to treat. We're all super special little snowflakes that need individualized care to be at our best health.

My specific mutations are Delta f508 and G542X. Delta f508 is the most common one. About 90% of people with CF have this (or two of this) mutation. G542X is the second most common with about 5%. According to a chart I found published in 2012, there were about 2,100 CF patients with my same mutation combination, out of roughly 70,000 CF patients worldwide. So essentially I'm super duper special!

For more information on CF mutations or more specifics about the science behind CF please visit CFTR.info or cff.org.

The Diagnosis

Last week there was a story on the CF Foundation's facebook page about a woman that was diagnosed later in life, after a decade or so of not knowing what was causing her symptoms. Now days most people with CF are diagnosed either by a newborn screening (I think I heard that all states test for CF at birth now) or shortly into toddler-hood. Well it got me thinking about the story of my diagnosis.

I wasn't diagnosed with CF until I was 5. August 20, 1985 is the official diagnosis date according to my medical records, yep exactly 31 years ago today! Happy CFAnniversary to me ;)
I don't remember a whole lot about that time, so I asked my mom to tell the story:

My family circa 1982/83

"A friend had heard a radio announcement in 1982 about a "kiss your kids" campaign to detect a salty residue on your children to get them tested for cystic fibrosis. That is the first time I had ever heard of this disease. I took my beautiful little girl to the doctor to ask if he could run this test, because I had been detecting a salty taste on her face when giving her kisses. He said I was a neurotic first time mother and there was nothing wrong with my daughter. He was a doctor, I assumed he was right, and never gave in to my gut to force the issue. Jennifer continued to be the light of our lives and be our delightful baby girl, but she was sick frequently, had a hard time gaining weight, and always had"unique" bowel movements. We were blessed with another beautiful baby girl, and we grew as a family, moving a few times in those short years, as oilfield workers often did. I was taking Jennifer to the doctor for her check-up to get ready for kindergarten, and asked about her tummy......she always had a distended stomach on such a little body. He suggested we do a sweat test. I didn't know what that was, but said lets do it. My sweet neighbor, who worked in the hospital lab we went to, asked if I knew what that was for, and since I didn't, she informed me it was to test for cystic fibrosis. Well, I went to the library and looked up everything I could on CF.  Read everything I could get my hands on, and we cried and prayed more than I knew was possible. The tests came back positive, and our doctor sent us to the Children's Hospital in Denver, Colorado to the CF clinic they had there for further testing on her and her little sister.  Well, three years after my initial "neurotic" request, we found out my sweet little girl did have cystic fibrosis. I still kick myself every day that I didn't force the issue with my first doctor.....I let my daughter down, and I will always regret not standing up for her! I am so thankful for all the progress that has been made in the medical field. I'm so proud of my beautiful daughter for taking care of herself, and so thankful her husband is so caring and understanding and loving! I still pray for a cure and a miracle every day!" 

Me, Mom, and my Sister

My younger sister does not have CF, thank goodness. Neither of us can remember if she is a carrier, or if she was ever tested for that after the CF mutations were discovered in 1989.

As far as I know I am actually the only one in my extended family that has (or had) CF.

jigglyknits

Since my lung functions have started to go down and I spent all winter being sick, I decided that it was time to really take action and start using my vest for airway clearance. Up until this point my doctors have said that my vest is just 'use as needed'. Well as I was gathering up some knitting supplies so I had something to do while using my vest, my husband said "why don't you sell whatever you make and donate the money to CF research, you can call it jigglyknits". (we have always called my vest the jiggly vest, since, it shakes so much while its working)

I kind of brushed him off and went on with my treatment, but as I sat there the idea started to percolate and it sounded like a really good way to raise awareness and help raise some money for the CF Foundation. I started an etsy shop and now I put everything that I make while doing my vest treatments on there. I've sold a few items and I can say that it feels pretty good to send the profits to the CF Foundation!

I created a facebook page & Instagram account to help advertise jigglyknits, which has then led to writing this blog. I never used to really talk about my CF as I was able to hide it pretty well from anyone that I wasn't super close to, but doing all of this over the last 6 months has led me to realized that if I don't talk about it, then I'm really hurting everyone that has CF. There are only so many of us, so the more that it's talked about and the more people are aware of it, the better chance that someday there will be a cure.

To own a jigglyknits creation of your own, you can visit my etsy shop or you can donate directly to the Cystic Fibrosis Foundation to help fund research to cure CF.

It's been a pretty crazy summer with travel and visitors, and I actually haven't really been doing too much of my vest. It's still sort of habit to not have to do it. I was moving all of my equipment around the house depending on which room we had guests in which made it easier to talk myself out of using it, so this weekend I organized the spare bedroom/craftroom/office to have a dedicated jigglyknits corner. I'm pretty excited about it and am ready to be able to add more to my shop!

Getting to Know Other CFers

Cystic Fibrosis can be a very isolating disease. Infections can be passed pretty easily between CFers, causing it to be dangerous to be together, so there is a rule that we aren't supposed to be within 6 feet of each other (the exclusion to this is siblings, since they are around each other all the time and most likely have the same bacteria). Because of this we can't have really support groups or friends that know what you're going through. At clinic visits they hurry us through the corridors and all patient rooms are kept closed. The only way you can even tell that other CFers are there is by the sound of coughing coming through the walls.

Instagram posts - I like to keep it a mix of
day to day life and my jigglyknits items.
You can follow me @jigglyknits

It is a lot better now with the internet and social media, us CFers can chat, commiserate, and offer advice. A group is starting a YouTube channel where they'll post videos about living with CF and another group is putting on BreatheCon this year (all going to be done digitally of course). I share pictures of my everyday life with CF and my jigglyknits creations on Instagram and follow several others with CF. I have to say I get a bit excited with every new follow.

While it's great to be able to meet and get to know other CFers there is a down side. Before, when I had never met anyone else with CF, I only knew my story and my experiences living with CF, which I can't stress enough are not that bad. Now, I see all of these amazing people that are fighting so hard and have low lung functions, and are in and out of the hospital constantly, and it's painful to see what is probably my fate. It's hard to say because I have been so healthy and only hospitalized once, but I do know that it's a real possibility as my lung functions have started to decrease over the last few years. It can be extremely depressing, but it can also be very inspiring. It makes me want to run every day and eat right and take care of myself to stay healthy for as long as possible. It makes me want to live each day to the fullest and not let my CF get in the way. So thank you to all of the other CFers out there that inspire me everyday to be better in every way!

Pills, Pills, and More Pills (plus a bunch of other stuff)

 This photo represents the typical amount of pills that I take in a day. Included are my vitamins, acid reducer, and pancreatic enzymes. I also threw in something for headaches since I end up taking that on most days :/

My enzymes (specifically Zenpep 15 for any curious fellow CFers) are the bulk of this since I have to take them whenever I eat. How many I take depends on how much fat is in what I'm eating, but on average I do 2-4 with a meal or snack. The enzymes have improved a lot since I was a kid, at one point I was taking 7 with every meal. My acid reducer also helps increase the functionality of the enzymes as well as let me get through the day without crazy heartburn. TUMS were my best friends for several years, now omeprazole is.

Since my pancreas doesn't work properly to pull the nutrients out of my food I need to take additional vitamins so I can make sure that I'm getting enough. Low vitamin levels are pretty common in CF. There are some vitamin brands out there like AquADEK, that combine a bunch of them into one pill, but I've found that those come back to haunt me (and anyone around me) all day long so I prefer to take them individually.

In addition to pills I also have to use a nasal spray to help keep the inflammation in my sinuses down, a sinus rinse to keep them gunk free, and a saline solution that I inhale using a nebulizer to help loosen the mucus in my lungs. Oh and I almost forgot about my bronchodilator inhaler, I use this prior to running/exercise to help open up my airways (or if I'm just having a bad, wheezy, lung day).

I'm no stranger to antibiotics either, as soon as I know a respiratory infection has started I'm on the phone with my doc to get a round to help clear it up. I don't love the idea of taking antibiotics so often, but I do love being able to breathe.

I know this seems like a lot and slightly overwhelming, but keep in mind that I am on very few medications compared to most CFers out there. Maybe one day I'll have to give up an entire closet to my medicine cabinet, but for now a couple of baskets stashed in the kitchen and bathroom will do.


**I have tried using essential oils instead of antibiotics (my last employer sold them, so I got well versed in all that I needed to try), but I found that they didn't quite work like the miracle cure that they were being sold as. I do still use them, but more as a supplement to what I already do. 

Let's Talk About Mucus

Boogers. Snot. Phlegm. Mucus. Spit.
Whatever you want to call it, someone with CF has a lot of it. Without getting too sciencey, salt doesn't stay in our bodies like a "normal" person (that's why our skin is super salty, but more on that another time), which causes our mucus to be thicker and stickier.

This thick, sticky mucus is harder to clear out of the lungs and is a great place for bacteria to grow, causing infections, that leads to scar tissue. All of this causes our lung function to decrease and it gets harder and harder to breathe as time goes on.

Mucus is found in many places in the body; pancreas, sinuses, cervix, etc. Anywhere that it's found, it will be worse in someone with CF. That's why many of us have to take supplemental enzymes when we eat. Our pancreas doesn't work properly due to the increased mucus and doesn't digest our food. Woe to us when we forget to, or just don't, take them when we eat something (again, that's a whole story for another time, yay, a poop post to look forward to!). Women with CF tend to have a harder time getting pregnant due to mucus plugs in their cervix, and most men with CF are infertile.
Chronic sinus infections (sinusitis) are also a common occurrence with CF.

Growing up, I didn't have any lung issues at all, but have definitely had my share of sinus issues. I had surgery in 2009 to open up my sinuses and get out all of the gunk that was jammed in them, they were totally blocked. They've stayed pretty clear since, other than major inflammation, but it's not uncommon for a CFer to have a sinus scraping surgery on a regular basis. And I've heard that sometimes the sinus cavities are 'obliterated' to close them off to future infection. I actually didn't even develop the one above my right eye, so lucky me, my sinus headaches are concentrated on my left side for the most part.

With CF, you learn to not be too squeamish about what comes out of your nose or mouth, as a single cough or sneeze can get you a sleeveful of goo. You also learn to inspect it for color as that is an indicator of an infection (yellow = bad, green = good). I can get up close and personal with mine til the cows come home, but if I even see someone else spit a glob out from a distance my gag reflex goes crazy.

Just wait...

Just wait.

I heard these two words so much when I was young. Not from my parents telling me that I wasn't old enough to do something, but from my doctors.

See I have Cystic Fibrosis, which is a genetic disease that causes my body to create thick sticky mucus. This mucus builds up in the lungs and pancreas (and anywhere else mucus is located in a body). In the lungs it can cause serious, repetitive infections and scarring until the lungs no longer function. My pancreas doesn't digest my food properly so I can't get the nourishment from it. I take enzymes (pills) every time I eat to help with the digestion process, and I have trouble gaining weight. I also catch colds a lot easier, that pretty much instantly turn into an upper respiratory infection.

Luckily my lungs were never really bad growing up. My lung function tests were always 100% or higher (so better than a 'normal' person). Which is great. It really is and I'm not complaining, but it left me in a sort of limbo. I wasn't healthy, I still had to go to my quarterly checkups, but the doctors didn't really have that much to do for me. So my family would take time off of work and school and we'd drive to Denver (4 hours away) every 3 months to go to Children's Hospital. We'd check in, get escorted to a room and wait all day to see a doctor. The doctor would come in, look me over tell me that while I'm healthy now, just wait, one day the lung disease would catch up with me and I'd start to get sick. Really sick.

Me as a baby, at 17, and  now at 36