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My Ever-Changing Definition of Cystic Fibrosis

** I apologize in advance for the length of this blog post. If you do not make it to the end, I will definitely not be offended.  Some of my posts are written more for myself, my CF community, and my future child/children, rather than just the public.  This is one of those posts, and I felt I needed to provide a lot of explanation throughout.**

Inspired by fellow blogger and fibro, Ronnie Sharpe, I felt it was about time I wrote about my views of cystic fibrosis and what it has meant to me throughout the years.

Those of us who have cystic fibrosis all look at it slightly differently.  Some see it as a burden, others as a constant fear of death, others as just an inconvenience, and still others even view it as a blessing.  For some, the definition of cystic fibrosis is a combination of all of the above terms and more.  Understandably, my definition of cystic fibrosis has changed throughout the years as I’ve matured.

When I was little having CF meant nothing to me. I ran, jumped, played hard, swam, and did everything normal kids do.  I had to do my treatments, which I hated, but that was all that was different.  This idea of CF stayed with me until my senior year of high school.  I often skipped treatments as I got older, but I could do the same things every other teen could do, so I didn’t view it as much of a problem. If anything, I enjoyed getting out of school for CF clinic days!  At this point, CF, to me, was an undefined “illness” that I had to deal with, but rarely affected me.

During my senior year, my definition of CF changed drastically.  I got sick. Really sick.  I entered the hospital with my O2 sats being quite low, and I was told by a doctor that if I continued this way I’d be looking at a double lung and heart transplant in a few years (talk about scary for a 17 year old). Because of this flare-up, I had my first PICC line and was home from school for a long time.  I also began connecting with my first fellow CFer, Jeremy.  Jeremy lived in the same town as me and had been reported in the local newspaper following his successful double lung transplant.  He was 22, not much older than me, and I felt a strong need to talk to him.  I honestly can not remember how I found him, but we formed a quick friendship where he encouraged me to become more compliant.  We never met in person, but we talked for hours on AOL instant messenger.  At this point, my CF was beginning to scare me.  Due to my first hospitalization and my first PICC line, I realized what it meant to be sick because of cystic fibrosis.  Jeremy helped to calm me down, but at the same time I looked at him (22 years old and having a transplant), and I wrongly figured that would be me as well.  I thought I had a good 5 years left, and CF suddenly meant a shortened life expectancy which invariably led to an early transplant and daily worrying.

That fear multiplied enormously when Jeremy began getting sick.  A few months post-transplant, and he was sick with a lung infection.  He struggled off and on for a few more months before unfortunately passing away.  He was the first fibro I had grown close to, and this was the first time I realized that CF was unfair and painful.  I attended his funeral, unable to keep myself composed, and then I became angry and scared.  I feared death, a lot.  I was angry at CF for taking Jeremy at such a young age.  I hated it all, and I saw CF as an evil, unjust monster lurking behind me with every step I took.

Soon after his passing, I looked for CF support groups to help me heal.  To show me that there was more than just an early death with CF.  At the time (around 8 years ago), I found complete negativity. I couldn’t find a single positive story, and my findings only reinforced my fear and anger about the disease.  I ended up at a therapist’s office and that finally helped me to deal with the feelings I had.   I was working on my fears and pushing the fears I couldn’t deal with to the side.  I began forming a closer relationship with God, and I felt better about my life expectancy, my future goals, and basically life in general.  I also realized after talking with my therapist and my doctors that CF affects every single person in a different way.  This comforted me tremendously, and I began to realize that CF could not be clearly labeled and had to be dealt with in different ways for different people.

As I went off to college, I let my fears mostly subside.  I did my treatments, though nowhere near 100% of the time.  I enjoyed my evenings, I went to my first parties, I enjoyed a drink or two, and I fell deeply in love with David.  Between David and my wonderful roommate, Lindzi, I was encouraged to keep doing my treatments.  I had a handful of people (David, Lindzi, Anna, a childhood friend of mine, and a few others) who truly accepted me for who I was, despite the fact that I took countless pills and did nebulizing treatments.  They pushed me to keep myself healthy, and I truly owe them a lot.  Because of this love and support, I became less fearful of my disease at this time.  I sadly, however, hid my disease from everybody I could.  I had done this all of my life, and I still worried about people accepting me.  CF, to me, was something to be kept in a closet, hidden, although worked on privately, daily.

As college came to a close, I found myself engaged and planning a wedding, graduating after completing a double major with honors, and happier than I ever imagined.  By this point, I was starting to do my treatments all the time.  I would occasionally miss one treatment here or there, but I would say I was 90% compliant.  David and I moved in together in May 2009, and even though I was being mostly compliant, my fears came back full-force.  This was the first time I really was fully taking care of myself without help from my parents, and I was worried whenever my cough sounded different or I had a cold.  It took me a good year and a half to get over this new-found fear, but I attribute this period of insecurity to the fact that my life had dramatically changed (in regards to living away from home), rather than because of my cystic fibrosis.  I can still say, however, that CF became a fearful uncertainty, where I always wondered if a cold or cough would land me in the hospital with another PICC line.

Now, in 2012, I honestly view CF a lot differently than I did during late high school and throughout college.  I have been accepted warmly into my CF family, which has helped me maintain a positive attitude.  I have been told by my doctors that my CF won’t be what takes me out, and I really try to look at it just this way.  I firmly believe that CF will be a part of my life I deal with every single day, but it’s not a curse to me. It’s a burden some days, especially during vacations, when I have to take time out of my day for breathing treatments.  It causes fear at times, when my breathing is difficult for a day or when I get sick.  CF makes me sad sometimes as well, especially when I lose a fellow cyster or fibro.  BUT, overall, I look at CF positively.  I see it as an ever-changing disease.  When I was born, average life expectancy was late teens, early twenties.  Now, it’s almost 40.  There are new drugs being developed all the time for this disease, and I am so grateful for that.  Because of these factors, I truly see myself as a grey-haired old lady, holding her grandkids and complaining about these young whipper-snappers who plainly don’t realize how lucky they have it. I firmly plan on embellishing all the “rough-patches” of my childhood in the stories I tell them.

CF may be a part of my daily life, but I’m working as hard as possible to ensure it doesn’t take me out of this world.  I focus on the positives, and I fight every single day to maintain 100% compliance with my treatments and good health.  Today, CF is as much a part of me as my brown hair and brown eyes, but it is certainly not an all-encompassing label which defines me.  It simply is. And I accept it, work to better myself because of it, and live every day to its fullest, a life motto cystic fibrosis reminds me of daily.  

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CF women are different. . .

When I began blogging, I also began seeking out fellow cysters (CF women) to talk to, ask questions to, and just to have someone to relate to.  Over the last few months, I’ve discovered that a CF woman is, in general, a totally different kind of woman. I’m not saying that we are better or worse than the normal woman, but I am saying that we are totally different. Let me explain (I will refer to CF women collectively as “we” for the remainder of the blog):

We have, in general, managed to find the really good guys to marry.  I have read through stories, blog posts, or individual conversations about many of the husbands of CFers.  I have found that they are, more often than not, really special, caring, genuinely nice, and dedicated husbands.  In addition to this, many of us are married by our early twenties and already looking to start a family. I think this deals with the fact that we are forced to grow up earlier.
We tend to be, on the whole, really happy people.  The vast majority of us don’t sit and complain about having CF. We might have a day or two of moaning to each other about it, but then we get over it and move on.  We tend to be really optimistic and supportive of each other.  We tend to share ways that we are seizing life’s golden opportunities.  We truly love to hear when a fellow cyster’s lung function is up or that she manages to get pregnant.  We root for each other when we’re in the hospital.  And this is all within our group.  Outside of it, we tend to live for the moment.  We often realize the blessings we have and we enjoy them as fully as possible!
We understand just how necessary patience is.  With the groundbreaking FDA approval of Kalydeco, I was reminded of just how true this statement is. We wait years to get a medicine which will help improve our lung function or quality of lives.  We wait hours each time we have CF clinic days.  We often wait months to years for a transplant when we have to have one.  We also often wait years to get pregnant. Many CFers have fertility issues which makes getting pregnant very difficult.  And, most importantly, we are still waiting for a cure.  If we didn’t have patience, we wouldn’t make it through.
We are no strangers to hospitals. Whether it’s due to the clinic appointments every 3 months, numerous blood draws, additional doctor appointments (for example, I have had or still have to visit an ENT, endocrinologist, cardiologist, geneticist, and liver specialist), or the unfortunate but necessary hospital stays, we have all seen our fair share of hospitals.  I personally visit the University of Chicago, and I have now officially been to every single floor of that building to see a different doctor! I probably know that hospital better than some interns!
We have thought about our mortality, usually extensively. This just goes with the territory when you have CF.  Having a disease where the average life expectancy is only 38, means that you think about death a lot. We wonder which cold we won’t be able to bounce back from, when we’ll have to have a transplant, when we will become resistant to all current antibiotics, and more morbid topics that I won’t go into.  That said, we often tend to focus on our mortality a bit too much.  I remember being sure I was going to die early, but then asked my doctors who in turn said not to worry about my life expectancy so much. They told me I was healthy, compliant, and positive. Those three things will take me far.
And the final way (though I am sure there are many other ways) we are different is . . . .
We talk about anything and everything without getting grossed out.  Now, don’t get me wrong. I have female friends and we talk about a  lot of stuff, but my cysters and I just take it to the next level.  So what do we talk about that normal women either have no reason to talk about or are too embarrassed to talk about?  Mucus? Yep! Blood? Yep! Comparing scars and surgical procedures? Yep! Boogers? Yep! Ovulation? Yep! Life expectancy? Yep! Bathroom issues? Yep! With all of those topics on the table, we even share embarrassing stories which usually entail one of the above topics, if not more than one!
So overall, I have to say that CF women are different, but it’s certainly not a bad thing.

“Wow! When are you Due?” And Other Hurtful Judgments

I am the kind of person who hates to judge others.  There are a few times where I, against my own desire, find myself coming up with preconceptions for certain people I see on the streets, but I always try to come back to the fact that I do not know their story, and I have not walked even a foot in their shoes.  I have no right to judge anyone.

This has been bothering me recently.  I think that judging others is a condition of our society despite the fact that it’s not nice, fair, or religiously acceptable.  Regardless, I think we all need to work hard at not judging those around us.  Due to my CF, I think I work extra hard at not judging others, because I know what it means to be judged.

Here are the judgments that have been made about me by various people.  These were just the people that were audacious enough to publicly share their judgments:

1)      You don’t look like you have CF.

There are so many reasons I have trouble with this one.  What does it mean to look like you have CF? It is quite rare that I run into a CFer who wears oxygen all the time, can’t walk on their own, or has “I have CF” tattooed on their forehead.  In fact, my fellow cysters and fibros look VERY normal.  You wouldn’t be able to discern us from the general population, unless we got into a coughing fit.  We might be tinier than those around us, but that’s not even true for all CF patients. Those who are pancreatic sufficient do not struggle with gaining weight much.  That said, please don’t say I don’t look like I have CF.  I often want to say, “You look like you have a brain, but I guess first impressions aren’t always correct.” I never do, but I’ve thought about it.

2)      You don’t look/act sick.

Very similar to the first one, but said to me equally as often, if not more so.  No, I do not look sick, but I work really hard at not looking “sick.”  There are days when my breathing is very tight, where I have to do 4 breathing treatments to feel better, where I have to force myself to eat over 3,000 calories despite the fact that I feel nauseous.  There are times when I’m suffering from intense abdominal pain because I failed to take enough enzymes to digest my food.  There are times when I’ve gotten only a few hours of sleep because I’ve been up half the night coughing.  I may not look “sick,” but I work my butt off to keep it that way.  I also know that many of my fellow CFers don’t look or act sick either.  If we’re suffering from a lung infection or on IV antibiotics, we might look sick.  Otherwise, we look healthy but most don’t see the work that goes into that.

3)      Wow! When are you due?

This is my least favorite.  It amazes me that people still ask pure strangers when their due date is.  While gaining weight, my CF belly has become quite obvious.  While my belly has grown, my legs and arms have stayed stick thin. On a normal day, you could say I look like I’m in my first trimester.  On a bad day, I look a good 5 months pregnant, no joke.  My CF belly bloats like crazy, and I get a belly that no shirt can fully hide.  While I understand why people ask me this, I still don’t think it’s polite.  Again, don’t assume someone is pregnant just because they have a protruding belly – it’s not always the case.  While many pregnant women can’t wait to be asked for their due date, I hate having to answer that I’m not pregnant at all.  I often brush this one off by saying, “I wish I was, but nope – not yet!” Usually the person who asked feels embarrassed, and I feel embarrassed. Overall, it’s just NOT a good question to ask.

These three judgments are ones that personally affect me because of my CF.  Having had a mother with MS, I have seen others pass judgment on her as well.  The theme of this post is that you can’t possibly know what someone goes through on a daily basis, so stop and rethink the labels you put on people before you know them.  Labels hurt – think before you speak.

Amazing Thanksgiving Weekend!

I’ve been so busy that I haven’t had much time to write over the past few days.  I feel like so much has happened this weekend, and I needed a day to take it all in.

Thanksgiving was incredible.  Wednesday evening was spent with my parents, my sister, her fiance, and his two little girls.  They are adorable and we had fun doing crafts, playing Christmas bingo, and being entertained by all the wonderfully humorous musings of children.  We stayed entirely too late and I ended up getting just a few hours of sleep before we had to head off on Thanksgiving morning to my mother-in-law’s house.

Our second Thanksgiving was just as enjoyable, if not more so, than our first.  We all had a fantastic time, ate delicious food, and David and I got to spend time with our adorable niece (almost 2) and nephew (1.5). We also played UNO for a few hours, set up the Christmas tree, and listened to my favorite kind of music . . . Christmas music!!

David’s brother also helped us set up a more realistic and easier budget which showed us the importance of me getting a job next year as our insurance copays and medication costs will be doubling and we will be losing money each month.  We were really trying to keep me from working as it limits my exposure and keeps me healthier, but we can’t afford to do it this coming year.

I had been looking into jobs, but the budget made me realize that we had NO choice.  Surprisingly, I ended up getting an interview on Monday for a caregiver position for a 2.5 year old little girl.  I figure it will limit my exposure to just one child which is better than subbing for me.  I ended up getting the job and will only be working Thursdays for now.  In the spring it will more than likely turn into 4 days, 5 hours each day which will give us more than enough to keep us afloat.

There have been so many blessings that it’s hard to count them this past week.  I am so grateful for the chance to work again and be able to help our family financially.  I also am grateful for my husband and the hard work he continuously does to ensure I stay healthy and happy.  I couldn’t have married a kinder, more caring man that’s for sure.

Unfortunately tonight I had more blood in my mucus.  I have been doing really well, but tonight after clearing my throat and then coughing I brought up a nickel sized amount.  It worried me, but I’m also wondering if it’s from my nose (draining down my throat) as it has been cold and dry here.  If it happens once more, I’m calling the doctor that’s for sure.  I think it’s so difficult for CFers to walk the line between being too cautious and not cautious enough.  It’s a constant juggling act.

I hope you all had a fantastic weekend, and I also wish you all a blessed and happy week ahead.

Preparing for Thanksgiving and Junky Lungs

Well! It’s that time of year! Thanksgiving is now less than a week away, and the black Friday ads are popping up everywhere.  Turkey Day is truly one of my favorite holidays (and I would imagine many other cysters and fibros might agree with me).  Any holiday which encourages high fat, high calorie, super-filling meals, which I don’t have to prepare, is perfection!  I can’t wait to sit down to a nice huge plate of turkey smothered in gravy, smooth whipped potatoes bathed in butter and gravy, creamy green bean casserole topped with crunchy fried onions, sweet potato casserole, corn casserole, cranberries, Jello fluff, and pumpkin pie. Anybody else hungry yet?

This year we will be celebrating with my parents and sister the day before Thanksgiving, and then will celebrate with David’s family on Thanksgiving day.  I’m planning on making something for each get together, though I have no idea what to bring. I’m just glad we will all be together as a family and able to enjoy a filling and delicious meal!

Until then, I will continue to worry about my lungs a bit.  Last week I had blood in my mucus which quit after laying off of the Cayston and hypertonic saline for a day or two.  Yesterday, I coughed up more mucus with blood in it.  I really really hate seeing that. I’m grateful that it’s not pure blood, but it’s still scary, that’s for sure.  Today I’ve been extremely exhausted and my lungs are sore so I’m hoping it’s not a sign of a bad lung infection coming on.  I have been coughing way more than normal the past few days so that could easily be the reason for both of those feelings.

I did get in touch with my CF nurse practitioner, Penny.  She was the one who suggested laying off of the hypertonic and Cayston to begin with which did help.  I found out I have 3 different strains of MRSA in my lungs and I’m allergic to one of the best drugs to fight off MRSA.  This means if I have to do a PICC line it will be massive..huge…last-resort kind of antibiotics.  That might be why coughing up bloody sputum scares me so much.  So, hopefully as the week continues my lungs will heal up and start feeling better!!

Today I am thankful for clean drinking water.  I know this seems odd but I really think that too many of us take this for granted.  I was putting a new filter into my PUR water pitcher and I realized that we are so blessed to have an abundance of clean, delicious, water.  There is nothing that tastes better than ice-cold water when you’re thirsty (at least in my opinion).

A Writing Rut

Despite the fact that dear hubby and I had a wonderful weekend, I am in a bit of a writing rut. I feel desire to write, but everything that seems to come out is incoherent trash. Therefore this is going to a very brief update.

My lungs have been a bit junky over the past week, but it seems like they are getting better on their own. Last week I did have some blood in my sputum, but I think it came from my sinuses/throat. I did, however, back off of hyper-sal and Cayston for a day or two to let whatever was open heal. Hopefully everything stays healed and no more blood presents itself.

Other than that, I’m just getting ready for Thanksgiving (a week and a half away!!!) and Christmas. I’m ready to decorate the house, and I think I may start hauling boxes downstairs as early as tomorrow. Decorating usually takes me a full week for Christmas. I can’t wait to share pictures of the house and how cute it looks when I’m done this year. Each year, we add to our Christmas decorations…I wonder what our new addition will be this year??

To get me into the Christmas mood, I’ve been blaring Christmas music (yes, I’m one of those people). I have been listening to it since November 1..possibly a bit earlier? Our one radio station here changed over to 24/7 Christmas music Wednesday of last week which has just caused me to get even MORE excited. I’m sorry to those who hate Christmas music and early Christmas decorating. I know I’m a bit eccentric, but I love it :)

As we head into another full week, I am grateful for family dinners. My parents had us over last Friday and it was such a nice time. We had pot roast with potatoes, carrots, and garlic bread. Both David’s family and mine really enjoy family dinners together. We are very blessed to live so close to our families and to be able to share a nice meal with them on a regular basis.

What are YOU grateful for today?

How Would my Life be Different?

Have you ever had one of those moments where you look back at your life and you realize that hundreds/thousands of individual events have all come together to form your life the way it is now?  I’ve had these moments throughout my life, and I realize how blessed I am for the way things have turned out in my life so far.  I was thinking about how blessed I am with my PFTs and my life right now, and I was thinking about how it would all be different if certain events had never happened.

First, I was entirely blessed that my dad finished his nursing degree right at the time I was born.  He knew the medical terminology, knew various medical issues that were hot topics in the medical community, and he was able to do the feeding tubes I needed without problem.  In addition, he pushed to find a CF center that was not jumping on antibiotics every time a child got sick.  He felt that resistance would be a future issue, and he wanted to let my immune system fight what it could on its own.  Wow, am I grateful for that.  At this point in my life, I’m resistant to a few antibiotics, but thankfully not too many.  Because of this push, I have been at University of Chicago since I was born.  I had one of the best pediatric doctors in the world, Dr. Lester, and she was wonderful.

Tying in with the above, I am so fortunate that I live close to Chicago.  I can’t imagine how different it had been if I had grown up in a state that didn’t have the massive teaching centers that Chicago offers.  Chicago has numerous teaching centers and I think the standard of care provided at University of Chicago is incredible. Had I been in a state without an established and well-known CF clinic, then my life might be totally different right now.  I have been blessed with an amazing, caring team of doctors who are entirely focused on my health.  In 2008, I had sinus surgery and cultured both MRSA and P.A. in my sinus cavities.  I was told that they had numerous doctors together discussing my case to figure out the best way to treat me.  When it comes to my health, I won’t lie to you, I love being talked about J.

As stated initially, my dad’s career choice was a blessing in my life.  I also, however, was blessed to have the parents I do.  My parents treated me like a normal child from day one. CF was not a disease I was going to die from. They encouraged me to be just like every other kid.  When I was little, I was outside running, biking, rollerblading, and swimming from sun up to sun down.  I came in for lunch and water breaks, but I was allowed to be a kid.  I wonder how it would have been different if my parents hadn’t let me get all that wonderful exercise throughout my childhood. I also wonder, if I had been born in today’s day and age, would I have been playing video games instead of playing outside?

I was also born at the right time.  When I came into this world, CF was advancing and research was being completed.  I was fortunate enough to start on pulmozyme when I was very young, and I was lucky that the vest came out when I was 8 or 9.  I was one of the first people to have it and it helped tremendously as I was getting too big to lay on my parents legs while they beat on me.  I have seen so many medications come into the CF pipeline, and the way CF is treated has completely changed. I am so blessed to have seen all of these medical advancements, and I can not wait to see the other advancements still to come (like the vertex drug that I am very excited about).

Even heading to the college I did and meeting my now-husband has greatly impacted my CF life.  I am so grateful that he has such a caring heart, and a dedication to helping me fight this disease.  He works to provide us with income and insurance. He makes me compliant with my treatments, and he puts my health first all of the time.  He happened to get a job close to both of our families, and still close to Chicago. Had he chosen a job somewhere else, I would be at a different CF center.  Had he not graduated a year early, he may not have gotten a teaching job, and we may not have been blessed with the wonderful insurance we have now.  I feel like I can never thank him enough for his continuous work and dedication to ensure my health. He is a blessing, hands down.

Needless to say, I have been extremely blessed in my life.  This blog could go on and on, but these are some of the top things I think about on a regular basis. If even one of these factors had changed in my life, I don’t know that my lung function would nearly be as high as it is. I am so grateful for my parents, my CF center, and my husband. They are all incredible allies against this disease, and they have helped me more than I will ever be able to realize/thank them for.