CF Control, progression, and guilt

I have read this challenge on several blogs and decided to take the challenge myself. I had to go through several blogs to find the original poster bc I felt they deserved the credit. Anyways, http://amatteroflifeandbreath.blogspot.com was the cyster that posted the challenge.

1. Write a blog explaining your personal thoughts and experiences in dealing with CF control and progression. This could include your views on whether CF is in fact a "controllable" disease, your personal definition of compliance, your thoughts on whether (or how) someone with CF should be judged in terms of "good enough" self-care (what makes you feel judged? do you think those fears are justified? is judgment ever useful in this context?), your own struggles with control vs. unpredictability, and how you keep motivated in the face of so many questions. Or, you know, whatever you want to write about really. It's your blog.

2. Comment below with a link to your blog so that all of us can read your response. YOU DO NOT NEED TO LINK TO MY BLOG IN YOUR ANSWER. If you'd like to do so, please feel free, but this is about starting a discussion, not publicity.

3. Encourage your own readers to get in on the conversation by posting the same instructions on your blog. Remember, the more responses, the better the conversation. Let's see if we can get this one going as much as with past challenges.

4. If you don't have a personal blog (or just don't feel like going through steps 1-3), feel free to still make yourself heard by simply leaving a comment with your thoughts below.

5. Non-CFers are 100% welcome to participate, either by pulling from their own experiences or simply by offering their perspective as people, friends, and loved ones.


The problem with a progressive disease is that as we decline we have no idea if it was due to something we did or did not do OR if it is the natural way our lungs are declining. We are given tools and meds in order to increase our chances of keeping infections at bay and our lung functions as high as possible, but these tools do not guarantee success. In fact, even with all of these tools we will all most likely die of this disease. The gray area creates problems. Because no doc can say your decline was absolutely because you missed that dose of pulmozyme one too many times, or the decline was 100% unavoidable it leaves the patient to always wonder. What else could I have done? Could I have gone to the hospital sooner? Could I have worked out more? The list goes on and on. I wanted to explain my story of guilt and feeling like I wasn't a "good" enough CFer and what I feel about compliant and decline today.

I had an unusually late diagnosis at the age of 14. This created a few obvious problems considering I was a middle schooler and a teen. I was told by my doctors to take a few inhaled meds (puffers) and to use my flutter once a day. I look back and think how EASY I had it then, but in my illogical 14 year old brain I used to think, "I have lived 14 years without ANYTHING it is not a big deal if I skip my flutter." I HATED that thing and I HATED looking at my mucus. When I started my flutter I used to do it in the bathroom with the lights off so I wouldn't have to look at the disgusting slime coming out of my lungs.

As time went on I was instructed to do more to take care of my health, but I was so determined not to let it interfere with my life that my health took a back seat to having fun. I wanted to live in the dorms in college which meant late nights and MANY missed treatments. I wanted to be seen as normal so I hid my disease from roommates and boyfriends resulting in more missed treatments. I decided to go abroad for a summer and volunteer, but couldn't bring my vest. My health during this time slowly declined.

I am now 27 with lung function much lower than it used to be. Was this because of my spotty compliance? Was it natural progression? I don't know, but I cannot help feeling that I had a lot to do with where I am today, both good and bad.

I carried a lot of disappointment and hate towards my 14 year old self for feeling that this disease was not serious and therefore I did not need to be compliant. I carried a lot of shame and disgust for hiding my CF from others. I was putting what others thought of me OVER a long happy life? Really!? REALLY?! This guilt weighed heavily on me for a long time!

BUT I have to say that I have been able to let the feeling of guilt (mostly) go. I was a child and I made childish mistakes. As a teacher, I try to picture myself as one of my students and it puts it in perspective. I was LEARNING how to care for myself and this disease. I made mistakes and I made some good choices too. I think it is important to do your best and don't stress over the little mistakes, we are not perfect and we were never expected to be perfect.

Today I am super compliant. I have made my health my one priority, but I am not perfect. With my imperfections I find the guilt still sneaks in. If I decided not to go to the gym in order to go to coffee with a friend I feel twinges of guilt. If I forget a dose of Cayston I feel guilt creeping into my thoughts. I have to constantly remind myself that I am doing my best while still have a life I enjoy! I also have to remember that we are not in control of this disease. We cannot stop decline or new bacteria from finding its way in our lungs, BUT we can do our best to help our bodies fight this never ending battle. I like to think it is a 20/80. 20% is controllable (although this may be generous) and 80% is nature/genes/whatever. I work as hard as I can to help out my 20%, but I also know when I get an infection that I can't shake or if my numbers drop it is not my fault. At that moment we can reassess our 20%. What else can I do to make myself healthier or feel better? But knowing that I am not the one in charge of when my lung function drops and infections arise sure makes breathing a little bit easier.

Back To Where I Started

So I have been on cipro and bactrim for a few days now and I feel almost back to baseline. I am still more tired than usual, but lung wise I feel good. In fact, my peak flow is back to my personal best! This may be the first time in my life that I caught an infection right away. Usually when I feel bad I give it a day or two because sometimes I just have a random off days. Then I usually try to do a few more treatments for a few days to see if that will clear it up and then usually I am super sick and need meds and am thinking, why oh why didn't I call sooner? I am no longer going to try to wait it out because it simply does not work.

Nausea is still involved in all aspects of my life. I have a feeling I am going to be losing weight because of these stupid meds. Last night I did manage to eat a bowl of cereal without wanting to die so that is a positive. Of course cereal for dinner would make my doctors frown and crinkle their eyebrows at me, but then again I could just throw up on them to show that this nausea means business. I am even disgusted by my Ensure which is usually my go to snack.

A little Fizzy NAC update: The flavor = eew. The ease of it = nice. The cost = sucks. The benefits of it = ???
I just can't tell due to being on meds and still a little under the weather. I am not sure what it is supposed to feel like, but apparently people say they feel much better on it. I will tell you if I feel anything in the upcoming weeks.

And So It Begins...

As I mentioned earlier, I am on the "good stuff" which comes with some not so good side effects. I love being on orals (vs PICC) because I can still swim and be active, but my body really hates them. I used to take them with no problems at all, but for the past year or two I get one particular nasty side effect! I have been on them for only a few days and it has already set in.

This morning I made a big breakfast with bacon and sausage (sometimes being a CFer has its perks)and eggs. The second I put down my fork after finishing off my meal the Bactrim hit hard! I looked at my husband and said, "I really feel like I need to throw up!" A wonderful thing to say to someone who is still eating, I know! Luckily, I never actually throw up, but I am tortured for a good while after every meal. I went and moaned on the couch...mainly for sympathy and over time it passed and life went on.

Fast forward to the early evening. I was hungry after yoga and because you can't eat junk food after yoga I decided to make a smoothie. I also find that liquids are less barf inducing. As I got everything ready I focused on how delicious it would be to distract myself from any oncoming nausea. The fruit was ripe and as I cut into each piece it gave off an amazing aroma. Everything seemed fine and no inkling of nausea. Pouring it in the glass, it looked perfect and I felt fine. I took a few sips and actually really enjoyed it. About 4 sips in, Bactrim took over and FAST. Ugh! I so want to barf right now. I really can't complain about it because people don't appreciate being told you want to blow chunks all the time. Something about it is a turn off. So here I am staring at a beautiful smoothie, listening to my stomach grumble from hunger, wishing I could just insert the smoothie in my stomach without actually drinking it.

Fizzy NAC

I have read SO much about fizzy NAC recently through different CF forums and I have been dying to try it. I have heard a LOT of good things and some people saying it did nothing. I so want to see if it does any good for me. It is really expensive at $25.00 a box w/taxes and one box will only get me through 10 days! Considering my husband and I don't make very much money and my health is already rather expensive, it is a little steep. The cost is why I did not order it sooner. I tried to get my doc to order mucomyst (which is the same as Fizzy NAC, but tastes much worse apparently) so insurance would cover it, but he refused. He then told me that if I went out and bought is myself (fizzy NAC) he couldn't stop me and would not think any differently towards me. He is SO conservative when it comes to meds. I am always trying to convince him to let me try new things and he always says no.

I went ahead and forked over the $25.25 for fizzy NAC and just received it in the mail today where I promptly took the first dose. I am a little torn if I should be taking it. As we discovered in past posts, I am a horrible scientist. I know I cannot give NAC a fair assessment because I am sick and on orals. Will I feel better because of orals or because of NAC or both? Will I think NAC does nothing because I am already sick and therefore do not feel well? Will I think it works wonderfully because as I get over this infection I will naturally feel better? I will have no way of knowing which is precisely why I am a bad scientist. At the same time, I am sick and want to get better ASAP so I of course am going to try anything. I decided in order to give it a fair assessment I will continue to take fizzy NAC for a few months before deciding if I like it or not.

When your life and health and well being is on the line I think we are willing to try almost anything. I so desperately want to grow old with my husband. I realize we will never be the little old couple holding hands on the front porch swing, but I would love to be a CF old person holding my husbands not so old hand. I would love to find grey hairs and even a few crows feet. I would love to be able to order off the seniors menu at restaurant or get a senior discount. It is funny that the things most people dread would be an honor for me to experience. I guess when people complain about growing old they forget that the alternative would be much worse. I wonder how many 27 year olds have these worries.

I Got The Good Stuff

So I am back in home from vacation and my little visitor never went away. That's right, I am sick. Blah! I even had a low grade fever for most of the day. Luckily, I called my doc while out of town to get some orals. They were delivered today and so hopefully they will kick in soon. I slept most of the day and the fever is gone, but I have yet to see my appetite. I go to clinic next week to check my PFTs and if all is well i will continue with another week of orals, if not I will be sporting a PICC once again. I will keep you updated!

Who Invited You?!?

So a not so lovely surprise has been greeting me in the morning. My little morning cough is back. YUCK! I used to wake every morning with a little morning cough, but through my new psycho regiment I have evicted my morning cough for several months. I have also noticed the clear-away-before-speaking noise I make that has come back as well. I notice this mainly when I answer the phone. My clinic is only open on Mondays and I will be out of town on Monday. So I will have to wait until the following Monday to get in. I am going to see if I can get some meds before the appointment. Stupid CF..

Over The river and through the woods.

The husband and I went to take a walk through Muir Woods. I have been wanting to bring him since before we got married and I was excited that we finally made plans to go. A lot of the trees there are between 500-800 years old, but some are as old as 1000 years old! Could you imagine? There is a main path which is paved and easily accessible to all people. They also have paths that go off the main trail that very from a few to several miles long for those that like to hike.

Hubber and I decided to take a path that was called "Ocean View." We started the hike and realized that it was a steep incline because we had to get up and out of the forest in order to see the ocean. We started our hike and about 15 minutes in I told hubber I needed a break. I was kinda huffing and puffing and trying to catch my breath. Hubber and I were looking out at the beautiful scenery while I caught my breath. I then looked him up and down noticing his relaxed demeanor and easy breathing and asked, "Did that make you tired at all?" He looked at me and shrugged, "Nope." I was impressed, but also a little disappointed. I feel like despite my CF I am still pretty active, but comparing myself to a non CFer I realized how much it actually does interfere with my ability to be as active as I would like.

We continued on until a point came that I needed one more break, the last one of the hike. Hubber was a ball of energy and told me to jump on his back. Keep in mind that this hike was not simply a walk, but you are climbing UP a hill so it is a nice incline the whole time and here I am on Hubber's back as he climbs upward on the path. I am happy to say that when he finally put me down he was very out of breath and it made me feel better to know he is not invincible.

I have decided as hard as the hike was I would love to continue to hike. It was such a beautiful setting and a HUGE sense of accomplishment when I was done. Even though I needed a few breaks I was so unbelievably proud of myself when it was all said and done. Here are some pics from our hike.

A little snake we found along the way...

Here was the spectacular ocean view...under all those clouds :(

Confused?

I have two different insurance carriers due to a complicated reason that I won't get into on this blog. Anyways, I decided to use my work insurance to see another CF doctor in the area just because I figure getting a second opinion on everything (would pregnancy be a safe idea, is my medicine routine enough, is there anything else I should be doing health wise...) would only be beneficial. I currently go to an accredited CF center, but there is another local CF doctor that is not at an accredited clinic. BUT this doctor used to work for the clinic I currently go to. Is that confusing enough for you?

I decided to call the appointment line to set an appointment with the new doctor. I explained that I had cystic fibrosis and was wondering if I needed a referral from my primary care doc to see a specialist or if I could bypass that process since I already have a confirmed diagnosis. This was the conversation that took place:

Lady: "Hmm, well I think you need to see your OBGYN about that before any referrals take place."
Me "Cystic Fibrosis is a lung disease"
Lady: "It is a lung disease?" (she sounded very confused)
Me: "Yes, so I would rather see a Pulmonary Dr."

Makes me a little concerned about the medical provider I am using.

Salty Air

I know most people in the US are unable to take a salty air day, but fortunately we do not live too far from the ocean. My sister and I headed up to the ocean for the day so I could have a relaxing day on the beach. I always feel so amazing on the beach, breathing in all the salt air.

A little story about salt air. While on my honeymoon I lost my flutter. This was my only form of airway clearance on the honeymoon because my Vest is about 40lbs (its the old school vest) and there was no way I was bringing that with me. I was basically freaking out and calling the hotel I got ready in for my wedding asking if they found it. Explaining what a flutter looks like it really hard to explain. Luckily, the hotel found it and my parents shipped it to our location. I still went several days without my Flutter and without any airway clearance. I usually think I am going to die if I miss one treatment and since my diagnosis I don't believe I have ever missed more than one treatment at a time. I was getting "pounded" by my husband, but it doesn't bring stuff up as much as my Flutter.

Long story short, despite missing several treatments and doing very little neds (horrible I know, but we were busy being in love and frolicking around the beach) I never once felt bad. I actually felt like I breath so much better! In fact, when I got back and went to clinic my numbers were higher than usual!

I now feel like every once in a while I need to take a beach vacation. Since I live close enough, we can make a day trip out of it which is much cheaper than sleeping over. Just breathing in the salty air seems to make me cough more and makes me feel amazing the next day!!

There have been studies about salt water and surfers which influenced the use of hypertonic saline. I wish I could live ON the beach, but since i am not a millionaire little day trips from time to time will have to do. And I could not think of a better place breath in salt air.

Here is an article about the saltwater and CF lung function.

http://articles.cnn.com/2006-01-18/health/cf.saltwater_1_cystic-fibrosis-mucus-lung-function?_s=PM:HEALTH

Clubbing Sans Music

CF is unique from many other diseases because to the outside world we often look healthy albeit a little skinny. People are often shocked when I tell them I have a chronic condition because they expect me to look sick or have an outwardly visible sign of my illness. In my teens years, and honestly even to this day, I am grateful that by appearance I am not labeled as "sick" or "different." With this being said, there is a small hint hidden among my body besides the subtle PICC scars that can clue one in that something is not quite right.

I was often teased for one thing in elementary school: my finger nails. It was only by a couple of kids, but for the life of me I could not figure out why. (I had a late diagnosis. Check out My Cf Story for more details)I would look at my nails completely baffled. What was so weird or different about them? I needed to know and because I was to mortified to ask my teasers, I made a conscious decision to start noticing other kids' nails. I quickly discovered that all nails look really different which left me perplexed. Some kids had nubby nails, others were chewed almost completely off, others had long nail beds, but mine were weird?. I couldn't figure it out. I went home and looked at my siblings nails. All 3 of us had very different looking nails. How did kids decide that out of all the different nails in the world mine were weird.

One summer, my aunt came to visit my family. We rarely saw her and we were so excited to see her again. As we were all gathered around breakfast my parents asked her to look at my nails. She was nurse and because at this time we still were trying to figure out why I coughed so much they thought she may have some insight. I know my family did not do this to make me feel bad, but I couldn't help feeling immense amounts of shame washing over my body. I was a freak and even my parents thought so! I honestly have no idea what she said. I am sure she knew it was clubbing and linked to lung disease, but I was melting away inside my own skin and did not hear a word anyone said.

Not long after this incident I was finally diagnosed with CF. The Dr explained the clubbing of my fingers and toes. (Here is some info if you don't know what it is. ) With the Drs explanation I realized why people said my nails were different; they were different. I was 14 and the fact that I was truly different (proven by a Dr) made me quickly decide that I never wanted to wear sandals that showed my toe nails and that I wanted to disguise my finger nails with polish. I become quiet the manicurist at that age.

I am happy to say that I no longer feel ashamed of my nails. Once my peers become less critical of others and their own self hate disappeared the teasing stopped and was often replaced with compliments. I have had several people ask me if my nails are fake when they are polished. When I explain, "No, they really are my natural nails" they often look at me like I am a liar. In fact, last year before my wedding I was getting a manicure and the manicurist was raving over my amazing nails beds. With all that being said just yesterday some man was intently staring at my feel in flip flops. I couldn't help, but feel a blush of red creep up my face and I wiggled uncomfortably going right back to that place in junior high where my nails brought me so much shame. Maybe he was daydreaming and my feet were where his eyes rested, maybe he liked my flip flops or maybe his daughter had CF and he recognized a stranger's foot. I will never know, but frankly I really don't care. I still love my nails despite their "flaws."

(Polished toe nails on my honeymoon)

(Polished fingernails. I rarely take pics of my fingers so I don't have a non-polished pic)


Click here to see a great link about why clubbing happens.

Very Stubborn..Kinda Like Me

This plug will NOT move for the life of me. I am using albuterol and Mortin every 4 hours, vesting (in all different positions) for an hour at a time 3x/day, manual CPT 2x/day, using a vial and 1/2 of hypertonic saline instead of one vial, plus all my regular stuff and the thing is still stuck!! If anyone has advice, PLEASE share it!! I am desperate!

In other news, I was able to swim yesterday and it caused almost no pain. This was probably because I was all drugged up on Mortin, but I was happy nonetheless. The other great news is that I had a post swim coughing attack on my way home that was so bad that I had to pull over into a parking lot because I couldn't drive. Yes, I know this doesn't sound like great news, but it was. I really thought the plug was coming out because I was coughing so hard I couldn't breath or see which rarely happens. I did not move the plug (dammit), but I did start coughing out some very old disgusting dark green mucus. This continued all evening. It was awesome because my mucus lately is always very light yellow and super thin so this nasty stuff must have been from way deep down. It is amazing that thick nasty mucus can make my ecstatic when "normal" people would probably throw up with disgust.

Last bit of news, the other day hubber and I got to play with his 1 and something month old cousin. It was so fun! When we arrived the baby squealed and came running/wobbiling over to hubber full blast and attempted to climb up his legs. Hubber picked him up and the baby nuzzled hubber so hard, I thought the baby was trying to nuzzle right through hubber's skin. The baby loved my husband so much that although I usually have the magic touch with kids (That is my job after all) the baby only had eyes for hubber. If fact, when my husband went to the restroom the baby fell on the floor and cried hysterically until he came back. I love seeing that my husband is so good with kids and that they love him. It really melts my heart, BUT I am now concerned that if we become parents I will NOT be the favorite parent. Sigh.

Pain In The Lung *Update*

So the Dr called and I have good news and I have bad news. I will present the bad news first because it is always good to get the worst part over with.

Bad News:
My lung hurts and there is little I can do about it besides take Motrin which would be fine if it actually worked well. As a result of the lung pain I am still trying to avoid sneezing (which has yet to become an issue again), coughing (big issue), and breathing too much (Ginormous issue). That wasn't too bad now was it? On to the good news...

Good News:
The Dr said that he thinks because I am being such a good and overly compliant patient, I am moving jumbo old plugs around in my lungs. He thinks it is only a plug and nothing more due to several reasons. I did not possess and other symptoms such as fever, shortness of breath, or fatigue. Also, it started soon after I started swimming again and also that I coughed out a plug not long before the pain began is another clue. He thinks swimming and acting like a crazy person doing treatments all day started to rattle things loose. He said a big ol' plug probably started to work its way out, but then got trapped. This part of the lung that is not used to being irritated got really inflamed which then caused pain. He said that I can do a few things to help speed up the process of getting this nasty plug out. He said I can drink a lot of fluid. Yes, this is doable. I can take Motrin. I will get right on that one! He also said I need to continue to be aggressive with my treatments and continue to swim. Uhh, do I have to? Because it kinda hurts a lot! I am doing my treatments as always despite the pain, but I skipped swimming today because I am hoping by tomorrow my lung pain will be less severe. I will let you all know when/if it comes out and I will be excited to see how big it actually is and it better be big after all the trouble it put me through. <---That is totally gross, but oh so true!

Random side note, but my Dr is in love with CFers that swim. He begs me all time time to start swimming again. In fact, he told me that he would give me 20 bucks if I started swimming again. Yeah, we have a weird Dr/patient relationship. He likes when I run or do yoga or bike, but he always wants me to swim. I think because my lung function was amazing when I was a regular swimmer. So if you live by a pool or have a gym membership you may want to give it a go. Be warned though because it may move mucus halfway out of your lungs and then you have to work your butt off getting it the REST of the way out.

Pain In The Lung

So two days ago I rid my lungs of a nice big plug. Later that day and everyday since then I have had a pain in the lung which to be honest is also a big pain in my ass! So basically, I have this sharp pain on my left side right where an under wire of a bra sits. For my male readers that is about midway. The thing that really stinks about it, besides the pain, is that it makes treatments a little more difficult because it hurts when I cough! My treatment times have been less than productive (pun intended) due to my unwillingness to cough too hard. I also had the pleasure of sneezing the other day, which I rarely ever do, and needless to say I will NOT sneeze again until this pain goes away no matter the cost. I have never tried to derail a sneeze before, but trust me I will find a way!

So here is the weird thing. I have this book that my dad created of all my medical info. Whenever I went to the Dr my dad would bring this book and scribble everything the Dr said down. O2 levels, heart rate, advice, meds, how I am feeling, you get the gist. Can you say scientist much? Despite my rolling of my eyes (I was 14 when I was diagnosed so my dad could not be any nerdier taking notes at the Dr) we still very much use the book. I have to say it had been such a life saver and great for piece of mind. So I dug through the book and realized 2 years ago almost to the DAY I had the same problem on my right side. Weird! The Dr at the time said the pain could be due to pleurisy, a collapsed lung (but I was still breathing fine so that was ruled out), a fatty plug, or something else. Helpful right? They ended up just giving me meds, a cat scan, and mortin around the clock. The cat scan showed plugging and some scarring, but other than that nothing unusual and it went away.

I looked up pleurisy and everything says that with pleurisy there is usually a fever (nope-I checked) or shortness of breath (nope), dry cough (no), loss of appetite (uhh no!), or Chest pain when you inhale and exhale, but between breaths, you feel almost no pain. Well, that is a big fat yes. So I called the doc and he has yet to call me back, typical. The nurse called and said she paged him, but he hasn't gotten back to her and she would let me know when he did.

My guess as of now out of the possibilities is a big fat plug or the "something else." I will let you know as soon as I find out. So for now I will try not to cough, sneeze or really breath for that matter.

Psycho CFer

I have turned into an obsessive psycho CFer in hopes of improving my health. I have to explain that despite my Drs disapproving looks and sighs, I work at a school. Yes! I know children are slimy balls of jumping germs. I know they will gladly sneeze or cough right in your face; I have had several colds due to this coughing in face act. I know they carry chicken pox, the flu, the common cold and oh so much more. BUT, by working at a school a CFer has 2 FULL months to work obsessively on their health. What other jobs allows you to do this? Uh, none that I know of. And even if there is another job that does, you probably don't get hugged 32 times a day and told, "I love you" by your co-workers.

Anyways, back to my point. I am off for the summer, but the hubbers and all my friends still have regular jobs so....I occupy my time with tons of treatments. Just the other day, my husband came home and looked at me and said, "Wow, seems like all you do is treatments!" Yup, it's true! And guess what? I coughed up 2, yes, I said 2 plugs in ONE cough!!! I know! Amazing right? And one was super fatty and big. Gross, but so cool. I had to inspect it for an hour thinking how gross it is that these are ALL over my lungs.

Now that I am doing crazy amounts of lung therapy and exercise, I can feel where my plugs are. It is weird, like all the junk cleared out around the plugs and I am super aware of where they are hanging out. I should say I know where some of the plugs are because I am sure I have dozens. Well, my mission this summer is to get as many out as possible, but as any CFer knows plugs are hard to move. The other problem is one of my problem spots is right behind my lady friend (I feel weird saying boob on my blog). This sucks because if it was anywhere else I could have hubbers pound away with chest percussion, but no can do thanks to the lady friend. Sigh.

I have an appointment in August so my goal is to be in the high 50s (wishful thinking??) by my next appointment. My (very) recent all time high was 54% which is still crazy because my norm had become 46-49% Okay, now I am rambling...