The Last Few Years

It feels funny to write that title. I can hardly believe that it has been years since I have been on my blog. This used to be a refuge for me, and I took comfort in the ability to write about my illness, infertility, and life in general. Then, my daughter was born, my sister passed away, and life became hard, busy, and sometimes quite depressing. 

Over the last few years, I've struggled with such varying feelings. I have felt the very lows of depression and anxiety where I have been unable to leave the house, and I have felt the immense highs that occur as you watch your beautiful child accomplish something new or give you the biggest hug as she tells you she loves you more than anything. In truth, even with all of the high moments my daughter has given me, the last few years have been downright hard. 

My sister lost her battle with cancer at the end of December in 2014. It has now been three years since I last held her hand, hugged her, or heard her contagious laughter as it filled a room. The first two years, I felt like a zombie moving through the motions as I learned how to somehow cope with her absence in my life. Over the last few months, I've finally just now begun to really feel like my "old self" again. I still have my days when I feel the sadness of loss overwhelm me, but they are few and farther between than they have been, and while a part of me feels angry that I'm allowing myself to feel happiness, I also know that my sister wouldn't want me to live my life sad. So I've been pushing forward, and the one thing that has kept me grounded is my beautiful little girl.

My daughter, Sydney, is now almost 5. She'll turn 5 next month, and she is beyond excited to celebrate her birthday with a My Little Pony theme. She is so intelligent, one of the kindest kids I've ever met, and so very sweet to others. She's also quite smart, and has a great sense of humor. I know I'm biased, but she's pretty darn amazing. She's currently in her second year of preschool, and her teachers have said that they will be so sad to see her go to kindergarten next year. She's ready though. She knows all her letters, and she is even reading and writing some words. I'm amazed by how much she has learned. She is the light of my life, and I'm so blessed to have her.

Two years ago now, we were actually pregnant with a second baby. We were both so excited and we couldn't wait to have a sibling for our little girl, but sadly I had a miscarriage just shy of twelve weeks. It was another blow that led me into a worsened state of depression and anxiety as I learned how to accept the loss of our baby and move on again. We had everything set up to try and carry another child yet again, but sadly, in November of last year I wound up hospitalized, and it changed the game for us. 

Last year, Sydney brought home every germ imaginable as she triumphed through her first year of preschool. Everything she brought home, I managed to catch, and before I knew it, I was really struggling. My CF team and I made the decision together that it was time to call in reinforcements and I received my first hospitalization in 12 years and my first round of IV antibiotics in 8 years. It was hard for me to mentally accept, but I knew I was doing what was best for my health.

During my hospitalization, I wound up doing 3 different IV meds. At first it was Merepenum, Tobi, and Vanco. I then ended up culturing C. Diff. while in hospital, and the IV meds were changed from Merepenum to Zosyn. It did help my lung function and my energy as these drugs kicked in and took care of the high levels of pseudo and MRSA I was carrying, and before too long I was sent home on a PICC with a home health care nurse coming out to help me.

The very first night I was home, the home health care nurse came and set up a schedule for me to do my drugs. She then gave me IV Tobi, even though I had already had my one dose for the day, 10 hours prior. Shortly after, I started feeling very odd and different. I called my team and they suggested I might be dehydrated so I chugged gatorade and rested, only to feel worse a little later. They then suggested I head into the hospital again to be checked out. As blood tests came back, we found out I was in kidney failure. I had been given two doses of Tobi in one day, when I should have been given one, and it was too much for my body. I was then readmitted to the hospital to sit and wait until my kidney levels bounced back. The doctors were shocked, as it took nearly a week before we saw them start to drop. 

I made it out of the hospital just before Thanksgiving, but the doctors told me that it was no longer wise for me to carry another child. With the CF, the diabetes, and now damaged kidneys (they did bounce back, but pregnancy is hard on the kidneys and mine have taken a big hit now), it was simply too risky. Obviously, I have a beautiful daughter, and I can't risk not being here for her to try and have another child. This also was a huge hit to my mental health as I struggled with the depression of never carrying another child again. 

The last year, thankfully, has been a much better year. While we did manage to pass around EVERY sickness again this year, including Influenza A (it was TERRIBLE), I managed to stay healthy and out of the hospital. I haven't even had a course of oral antibiotics this year (knock on wood). I would say that the biggest reason for this has to be my improvement when I started the new CF drug, Orkambi. Once we knew that I couldn't carry a child anymore, we immediately started me on Orkambi as we had held off only due to pregnancy. It was a game changer for me. I handled illnesses so much better, and I saw a 6% increase in PFTs. My last FEV1 was 82%. Unfortunately, I did have a lot of side effects from Orkambi. Higher blood pressure, higher blood sugars, increased depression and anxiety, horrific GI issues where I'd be in the bathroom 8-10 times a day, and issues with ovarian cysts (I have to have surgery this coming summer to get rid of them). It was worth ALL of those issues to be able to breathe better and handle illnesses better, but it was a host of rough side effects. 

A week ago, I made the transition from Orkambi to Symdeko. I'm now 8 days in, and while the first week was quite rough, I'm doing better now. At first, I experienced a lot of shortness of breath and a horrible headache that lasted around the clock for days, but now I'm feeling really pretty good. I have more energy than I did on Orkambi, and for the first time in a year, I'm only going to the bathroom 1-2 times a day! I'm hoping the positives of this drug keep up. We'll see what happens as it continues, and I'm curious to see how it impacts my lung function, but I'm hopeful. 

<3 

My Scope and My Sister's Appointment

Wow. What a BIG week for my family.

Last Thursday, I attended my CF clinic while my sister met with her surgical oncologist. It was a stressful day, to say the least. I was a nervous wreck as I did my PFTs (Pulmonary Function Test), anxious to hear what the surgeon was telling my sister at a nearby hospital in Chicago.  I was anxious that my lung function would go down from the stress of the last few months, I was anxious about getting the approval to go ahead with infertility treatments next month, and I was especially anxious about whether or not my sister was all set for surgery to remove the cancer. I also had nothing to eat that day as it was the day before my colonoscopy and I was only allowed to have clear liquids.

For starters, my body is stronger than I previously thought. Even with the stress of my sister's diagnosis, my father-in-law's passing, and my great-aunt's passing, my lung function stayed stable. I was 85% this time (86% last time, which is considered unchanged).  I was STUNNED. I never expected to be this high, but I was grateful. My oxygen was also at 100% on room air, and I sounded nice and clear. In addition, my doctor didn't even hesitate when she told us to go ahead and start infertility treatments next month. Needless to say, it was a good appointment. My mom and I left thinking, one down, one to go.

We headed back home to change really quickly and then headed to the local hospital. I was to be admitted for my scope prep due to the fact that I have a prolonged QT interval, and the doctors felt it would be safer for me to be on a heart monitor while doing the prep. I wasn't thrilled about being admitted, but I went along with it. This was my first time overnight in a hospital since I was 17.

I checked myself in, and I prepared for a long night of prep.  Basically, I had to drink two 16 oz glasses of a strong laxative, and then follow that with 64 ounces of water. The prep tastes horrible, and the laxative effects aren't fun, but it's well worth it to get checked out when you have a family history of colon cancer. The first half of the prep didn't go so well for me as I didn't take anti-nausea meds prior to starting it. Once the nausea meds were on board, I was fine to finish it. The second half of the prep went just fine since we let the meds kick in an hour before starting the prep.  I didn't feel queasy once, and I was able to keep it all down without an issue.

The scope showed that I had a polyp which was removed during the procedure.  I wonder how long it would have been inside of me if it hadn't been for my sister's diagnosis. I had no pain, no issues, and no reason for a scope aside from my family history.  The problem with polyps is that they have a tendency to become cancerous. This is why they are instantly removed when they are found during a colonoscopy.  I'm so grateful that it was found and removed!! Due to the finding of the polyp, I will have to repeat my scope in three years to make sure I am still doing ok.

As for my sister's news. . . .we actually found out about my sister's appointment about four hours into my hospital stay. The surgeon said that he wants to go ahead with surgery in early June!! We're so happy that they are setting up for surgery and will remove the tumor from the colon!  The plan is to cut out half of the liver, and remove a good portion of the colon. It will be a big surgery, but my sister is a fighter and she will come through it with flying colors! The other half of my sister's liver is in FANTASTIC condition, which is great because it gives it a higher chance of regenerating once the surgery is complete. The only problem is that in order to prepare for surgery, my sister has to stop chemo. The surgeon said that they will do a scope and a PET scan the day of surgery, but if anything has spread the surgery will be cancelled and she will have to go back to chemo. He called this a "leap of faith." As we await the beginning of June, please keep my sister in your prayers and thoughts. We're praying that the cancer does not spread anywhere else so that the operation can take place!

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.  

Things I've noticed about CF women

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?

Suffering from infertility, 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).  

One Step Forward . . . Two Steps Back

Sometimes, when you really want something, it seems like progress almost moves backward before you start to see any results.

After posting yesterday I got the news from my doctor that as far as my lungs are concerned, there is no reason not to go through with IVF.  I was and still am elated and overjoyed about this factor.  She said that there are risks with IVF, but it shouldn't be any more prevalent just because of my cystic fibrosis.  Fantastic news!! 

Then she dropped the other shoe.  

The last time I was up at clinic my liver enzymes were much higher than normal.  I run somewhat high liver enzymes always due to the CF, but these were about 2 times as high as normal amounts.  She said that some IVF drugs are rough on the liver and therefore I need to see a liver specialist prior to doing my IVF treatments.  Surprisingly, I was ok with this information when she gave it to me.  I took down the specialists number and realized that this was just another step in the process.  

After hanging up with my doctor, I immediately called the scheduling department for the liver specialist.  Scheduling informed me that the earliest appointment is the last week of January/first week of February 2012.  This part...I'm NOT ok with.  

There's no real reason this should bother me as I realize that seeing specialty doctors often comes with a long wait, but when I was told my first cycle of IVF would be in January/February I was excited, and now I'm being told we have to wait even longer.  Yes, I know it's only another couple of months.  Yes, I realize that the ultimate goal is for me to be healthy to carry a baby. Yes, I realize that God has a right course for me and that it will work out the way it's supposed to.  No, I'm still not ok.

Ultimately, this is just a bump in the road, but once my husband and I made the decision to start a family every  month without a positive pregnancy test has been torture.  I'm so ready to be a mommy and being told that your dream has been delayed even more is hard to hear sometimes.  I'm also worried about the liver enzyme numbers.  I'm seriously hoping that nothing is wrong with my liver outside of normal CF inflammation and such, and I have to wait til next YEAR to find out. 

Until then, I'll just cross my fingers that all is well.  Is 2012 here yet? Please?

With that said, I think it's appropriate to be thankful for fresh starts.  I love the new year because it always seems like a new beginning and that anything is possible. There is no logical reasoning why problems should stop at the end of one calendar year, but sometimes it certainly seems to work out that way.  I love being able to make resolutions (and usually break them), and I love reflecting on the past year and creating a plan to make the upcoming year even better.  It seems like a second chance and it happens every twelve months!  I can't wait for New Years Eve this year!!

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.

David and I are really coming to terms with the infertility issues we are facing.  I even went to a baby shower yesterday and was able to get through it without any feelings of resentment (and only a twinge of sadness).  David also wanted to tell his parents so we told them over the course of the weekend as well. They are both very supportive and keeping us in their prayers.  At this point, we need to schedule David's semen analysis and then just wait for my period to continue.  Technically, I'm still not sure if my doctor has given the green light for the treatment, but I don't want to bother her again (it's only been a week).

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 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 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.  

5 ways cystic fibrosis has positively impacted my life

Blessed with eternal optimism, I felt a desire to write about the ways that CF impacts my life positively.  I believe I often get bogged down by the weight of the disease and forget to look at any good it has caused.  Although I would never wish cystic fibrosis on my worst enemy, I do believe that it has changed my life in some ways for the better. Below are five of the ways it has changed me or impacted me in a positive way.

5. CF causes me to exercise

As stated in a previous post, I despise exercise.  I honestly believe that if it wasn’t for cystic fibrosis being a critical part of my life, I would be sitting on the couch eating bon-bons and not worrying about exercise in the slightest.  Instead, I am forcing myself to walk and run as much as I can.  Exercise is not just beneficial for my CF lungs but also for the rest of my body!

4. CF allows me to explore new worlds through reading

This may be a stretch since I have always enjoyed reading, but having CF means being blessed with hours of reading time every day.  During my treatment time, I have been able to explore various parts of the world, solve exciting murder mysteries, and imagine myself at Hogwarts.  Even with an inherent passion for reading, I doubt I would finish as many novels if I weren’t confined to a chair with a vibrating vest on me. 

3. Having CF has given me a caring and supportive virtual family

Due to my quest to feel normal despite this disease, I have ended up meeting so many wonderful people online.  I have formed and am continuing to form real relationships with cysters (female CF patients) and fibros (male CF patients) from around the world.  We share in each other’s struggles, root for each other’s achievements, and celebrate life together.  The CF community, on the whole, is a very positive and uplifting community.  It is a family of people triumphing together over this disease and I’m blessed enough to be a part of it.

2. CF has led me to value the time I have on this Earth

I’m certainly not planning on going anywhere soon, but CF has made me realize time is precious.  I have written about this one a few times now, but I have to reiterate it due to its significance.  None of us have a guarantee on the amount of time they will have on this Earth, but many of us don’t realize this until it is too late.  I started realizing it in my teens and it has only become more apparent as I watch major milestones in my life pass by me.  I don’t believe many people in their 20s value time as much as they should.  If they did, I personally doubt they would spend so much of the time they’ve been given on a bathroom floor, drunk and puking.

1. CF has helped me to love as unconditionally as is humanly possible

Tying in with #2, CF has made me realize how important it is to love as fully as possible and to show my love as often as possible.  Since I do have a chronic condition, it is important that I make sure my loved ones know how I feel about them.  I forgive very quickly, and do not hold grudges.  I say “I love you” a lot and I mean it each time.  I try to spend time with friends and family as frequently as possible, and I thoroughly enjoy the time we spend together.  I love creating and capturing memories, and I try my hardest to end every conversation or experience on a positive note.  This is not saying I don’t fight, because I do.  I get mad, bossy, am sometimes controlling, and probably very hard to be around some days, but I try to fix those situations as quickly as possible.  I’m the queen of, “I’m sorry,” always said sincerely and honestly.  Apologizing when I’m wrong and loving each person for his or her personality and strengths is important – CF has helped me realize just how important it really is.

So there it is – 5 ways that cystic fibrosis has made a positive impact in my life.  I’ve wondered on numerous occasions how different I would be if it weren’t for my disease.  Although it’s impossible to be certain, I doubt I would realize or experience the five statements above if it wasn’t for CF. 

Now the question is passed on to you – in what ways have a disease or disability (it could be your own or a family member’s) changed your life for the better?  Think about it and you may be surprised at the answers.

Antibiotic Appetite

My “Antibiotic Appetite”

I’m not sure if other cysters and fibros go through this, but I rarely want to eat whenever I’m on antibiotics.

Over the past week or two, I have been craving Thanksgiving dinner with all the trimmings.  So, when I saw turkey on sale last week I decided to pick it up.  Yesterday, I proceeded to make roast turkey with stuffing, creamed corn casserole, green bean casserole, and gravy to go over everything.  I was so proud of myself, because this meal is such a high-fat, high-calorie meal that can now be eaten as leftovers for days!! 

It had been two years since I last made turkey with sides – I’ve been relying on my parents and mother-in-law for Thanksgiving dinner for the last few years.  Two years is a long amount of time, and I definitely forgot how to prepare a turkey.  I immediately became best friends with Youtube, and the smiling turkey woman in the video walked me through the prep work pretty well.  Sadly, this turned into one of those meals where you end up at the store a dozen times before it’s done.  I was a bit scatterbrained yesterday and kept forgetting items for the sides.  I also had my meat thermometer break when the turkey needed to be taken out of the oven. Needless to say, my local grocery store loved me and my wallet yesterday!

After all this hard work and numerous trips to the store, it was quite disappointing to finally get the food on the plates and not want a single bite of it.  I call this problem, my “antibiotic appetite,” and I did not expect it to hit this early into my course of Levaquin.  To be fair, it doesn’t matter what antibiotic I’m on, as long as it’s strong enough to kill the bugs in my body, it leaves me with no appetite.  So I stared at the food, pushed it around with my fork a bit, considered giving it to the dog, and ultimately forced myself to eat it.

When I woke up this morning, the smell of roast turkey still lingered in the air and made me queasy.  I ate a single waffle for breakfast and forced it down with a glass of milk.  I know I will have no desire to eat lunch or dinner, but I’ll get to make myself do it anyway.  It’s one of the things I hate about CF.  It doesn’t matter if I don’t want to eat, I have to in order to keep my weight up (lack of desire to eat happens even without antibiotics, but the antibiotics make it worse).  There are also times when I would much rather have a nice lean salad and a piece of fruit, but instead I wind up choking down high-fat, high-calorie, and high-protein foods.  I sometimes find myself feeling like a human waste receptacle who wears a sign stating, "I’ll take all the fatty stuff no one else dares to eat."

So for the next 12 days, I will find myself forcing food into my stomach with no desire to eat.  I’m probably going to lose the weight gain I have managed over the past few months, and I’m going to have to work extra hard to put it back on two weeks from now.  Oh, antibiotic appetite, how I loathe you.

Advice for the younger CF generation

Advice for the younger CF generation

Although my blog is mostly adult content and will hardly ever fall upon the eyes of the younger CF generation, I was inspired by another blogger (CF Fatboy and his post which inspired me to write this) to impart advice I have learned and share my struggles of growing up with CF.  These are the things I wish someone had told me when I was younger (not that I would have listened).  None of this is to be taken as medical advice. Please consult your doctor before trying any new treatments or exercise program. I am NOT a medical specialist!

1.       You’re going to feel out of place.

This is true of everyone human being on Earth, even if it doesn’t appear to be the case.  At some point in your life, you will feel awkward and as if you don’t belong.  This just seems to happen more often if you have a chronic illness.  When it happens, try to remember all your great strengths and assets.  Remember that other people feel this way too and that thousands of other cysters and fibros have gone through similar feelings as well.  You are not alone.  If someone calls you out on why you do something different, think of it as an opportunity to raise awareness for CF.  I know this is easier said than done. I went through high school feeling left out and alone a lot of the time.  I shied away from my disease and never talked about it, but I regret that now.

2.       Compliance is Key – You are NOT Invincible.

This goes hand in hand with #1.  If you already feel out of place, then taking time to do breathing treatments is hardly going to make you feel more “normal.”  However, it is easier to feel normal by keeping up with treatments every day than ending up in the hospital multiple times a school year because you refused to do the treatments.  I experienced this first hand.  I skimmed through high school doing treatments here and there but never fully committing to them.  It didn’t matter, I had a mild case of CF and didn’t need to do the treatments.  Then, one February night, I found myself in the E.R. with MRSA, P.A., and bronchitis. My sats were in the 80s and I had an extremely high fever.  I didn’t get to go back to school for an entire month.  Instead, I spent my days in a hospital with a PICC line, trying to recover – try feeling normal after explaining that to your fellow classmates. 

3.       Every case of CF is different.

This is one that I am still trying to learn myself. Cystic Fibrosis is one of those diseases where everybody is affected differently by it. There are some things that are common place, but as far as infections, PICC lines, surgeries, and pregnancy go – everyone is very different.  When I was 17, just getting out of the hospital, I met my first fellow fibro.  He was 23 and had just undergone a double lung and heart transplant and we talked online all the time for months. Then suddenly, he wasn’t online as much anymore and I soon learned that he passed away.  This hit me hard – harder than I ever expected.  I was convinced that I also would pass away at the age of 23 (like he did) and that my CF was just like his case.  Well, as my 23^rd year passed, I began to realize I didn’t have the ability to see the future.  I then started seeking out other cysters and fibros so that I could share my journey with someone yet again.  If you become part of the CF virtual family, you will unfortunately meet other cysters and fibros who pass away much earlier than they should.  You will lose some of your friends, but you will also gain a deeper love and understanding for others dealing with our disease.  You will also learn things that may downright shock you or encourage you to do everything you can to kick CF booty.  For example, it was through the CF community that I learned of Hal Soloff, who is now in his 80s and fighting CF every step of the way.  So stay encouraged, stay positive, and stay compliant.

4.       There is never enough time so live fully, laugh often, and love unconditionally.

This one has been drilled into my head more and more in recent weeks.  As stated in #2, you are not invincible.  You may live to be 102 or you may have a rogue piece of satellite come hurtling at you from the sky tomorrow – you NEVER know.  Having CF, I think we often get pulled into the mindset that we will die before our family and friends.  I don’t like getting into this mindset for a variety of reasons, but most of all it ends up creating a feeling that there will always be a tomorrow and that words/actions don’t hurt others.  I have recently (in the past few years) decided to live my life as if there is no tomorrow for any of us.  I’m not always successful, but I try really hard to abide by this philosophy. I don’t end conversations with a harsh word – EVER.  I always say I love you. I give way too many hugs/kisses to the people I love.  I thank everyone for all that they do on a regular basis.  I tell people how much I appreciate a kind word or action they have done. The list goes on – I think you get the point.  If something happens, I want those around me to know that I 100% loved them and appreciated them.  I never want that to be a doubt in their minds. Also, if something happens to someone I love, I want to know that the last thing we were able to say to each other was positive.  This is so important to me and it has made me a happier person.

5.       Listen to your doctors AND don’t stop exercising.

When I was younger, I believed I knew what was best for my body.  In some regards this is still true.  However, I am much more likely to listen to my CF doctors than ever before. If they want to me to increase my treatments or go on an antibiotic – I don’t fight them. I also don’t tell them I will do it and then never actually go through with it (ahhh my teenage years...how stupid of me).  I believe that CF care centers work very hard to provide us with the most accurate and up-to-date information they can. They are doing the best they can to keep us as healthy as possible for as long as possible.  I didn’t get the medical degree (though some days I feel like it) and therefore I listen to what they have to say.  If I disagree, I discuss it openly with them prior to leaving clinic and we reach a mutual understanding.  

Recently, their biggest push is for me to exercise.  Exercise has been cited again and again as one of the best ways to keep lung function up.  I loathe running and despise sweating, but I’m doing it for my health.  I’m not 100% compliant with this yet, but I’m working toward it.  My advice is to stay active your entire life.  Don’t let the early twenties slip you by as you head into college and lounge around in the dorms way too much.  If you never stop exercising, you won’t have the issue of trying to start it back up again and build up your tolerance. 

Our clock in our kitchen.  I love it - ties into my philosophy beautifully!

Well that’s it for now.  This blog has gotten way too long, but these are 5 (technically 6) things I firmly believe in. I wish these had been shared with me by a fellow cyster or fibro at a younger age.  Now the challenge goes out to all my fellow adult cysters and fibros out there: What advice would you impart on our younger generation?

My CF Journey

I promised that I would further detail my CF mutations and journey - so here it is.

At birth, I had a few complications which led the doctors to test and ultimately diagnose me with cystic fibrosis.  I am so blessed for this early diagnosis because it meant I was on medications and being watched from day one. 

When I was first diagnosed, we were only aware of one mutation Delta508.  The other, at that point, was unknown and therefore I was re-genotyped last year.  We found out that my second mutation is also Delta508.  Why it didn't pick it up the first time, we don't know, but ultimately what that means is that I have the most common gene mutation out there.

I was hopsitalized once at age 5 with a sinus infection but nothing lung related.  Other than this minor hospitalization, I was a pretty normal kid for years.  I ran outside and played endlessly, loved school, loved reading, loved learning (Ok, so maybe I was never normal).  I did my nebulized treatments with few complaints until I reached the age of 14.  Suddenly, high school hit and I didn't want to be "abnormal."  So, the treatments were done sporadically - only when I felt I needed it or more realistically, when my parents made me do them. I firmly believe this is why, at 17, I found myself hospitalized with double pneumonia and bronchitis.  I ended up in the hospital for weeks and came home on a PICC line for 2 more weeks. 

You would think this would have been a huge wake-up call for me, right?  Well, in some ways it was and I did start doing my treatments more often, but due to my desired invincibility, I still was not 100% compliant with them.  At this time in my life, being 100% compliant meant doing my vest (a machine that helps loosen the mucous out of CF lungs by vibrating them) two times a day, nebulizing albuterol sulfate two times a day, and nebulizing pulmozyme one time a day.  Total treatment time was an hour a day.  I felt, at this young age, that was simply too much to do.  And when my post-PICC line PFTs were back up over 100%, I saw no reason to be completely compliant. 

By now, it should be clear that I've been blessed and God has been taking care of me day in and day out because I certainly wasn't taking care of myself.  This struggle continued into college where I still just wanted to feel normal.  My sophomore year of college, however, my relationship began with my now-husband, and suddenly everything changed.  We had been friends from freshman year on, but as soon as we started dating, I felt as if there was a purpose to my life - a reason to live, if you will. 

Instantly, I became more compliant.  I wasn't 100% compliant from the first date, but it wasn't long after that.  Now, I only miss a treatment if there is no choice (for example, if we are on the road for 12 hours at a time).  In fact, now, I keep trying to add more treatments or additional exercise to keep myself as healthy as possible.  It's a total shift from the way I was just six or seven years ago!  Unfortunately, my treatments aren't just an hour long anymore, but it's worth it in the end. 

In addition to my treatments, I also have to manage CFRD (cystic fibrosis related diabetes).  I recently have started on insulin to try and put weight on me to prepare my body for a pregnancy. My FEV1 (the indicator number of how well us CFers are doing) is 81-84% It's far from the 100% I used to have when I was younger, but it is still really good. The only way to keep it up this high is to continue with all my treatments.  With that being said, here is my typical day's schedule:

6:30 AM: Check sugars and eat high-calorie, high-fat breakfast (Can't just pour a bowl of cereal anymore...)
7:00 AM: Albuterol Sulfate nebulized, Hypertonic Saline nebulized, vest for 20 minutes
8:30 AM: Check sugars again and eat morning snack (which actually looks more like a lunch...)
9:00 AM: Do nasal irrigation rinse (These help tremendously with sinus pressure)
11:00 AM: Check sugars again and eat lunch
12:00 PM: Second treatment.  This time, vest by itself for 20 minutes
2:00 PM: Albuterol inhaler
2:05 PM: Checking sugars and eat if sugars are low
2:30 PM: Exercise for 30 minutes
5:30 PM: Check sugars, take insulin, eat large dinner (usually 1000-1200 calories)
7:00 PM: Second nasal irrigation rinse
8:00 PM: Third treatment.  This includes, albuterol sulfate nebulized, pulmozyme nebulized, and vest for 30 minutes.

This is my current schedule.  In another 20 days, I'll be back on Cayston which adds an additional 3 nebulizers a day (granted, these only take 2 minutes each). 

It's a lot to get in to a single day and if I get a cold or any illness, I add in a few more vest treatments.  While I do a lot of treatments, there are other CFers who do way more than I do every day.  I am blessed!