Broader Shoulders

"I ask not for a lighter burden, but for broader shoulders." ~Jewish Proverb

Last Monday, I had a doctors appointment with Dr. Light. My FEV1 was 50%. When he first met me a good five months back, I was at my worst with FEV's in the 40's. This is a good sign that my numbers are higher, but of course not where I ultimately want to be number wise. We also discussed my sputum results which weren't good. MRSA has risen from the dead, pseudomonas aeruginosa was the life of the party as usual, and a newer bug that popped up was pseudomonas fluor-putida. Great, now the gang was all here, I thought to myself. I usually only culture pseaudomonas aeruginosa and the last time I had cultured MRSA was back in 2008. MRSA is sneaky though it can go to sleep and wake up whenever it wants. Keep in mind that these bugs are Cystic Fibrosis bugs only and no other "normal" person can catch what I have. Point blank, I'm not contagious. It's in my lungs and the bugs fancy my living quarters of CF lungs rather then clean fresh healthy lungs that "normal" people have. Just part of the joys of having Cystic Fibrosis, you never know what's cookin'.

Plan is to do Cayston via Altera nebulizer (3 times a day) for two months and then on the third month switch to Colistin via eflow nebulizer BID (twice a day) and continue that cycle. That will be my new nebulizer regimen as far as the nebulized antibiotic goes.

I started taking Doxycycline Hyclate 100 mg for the MRSA. One tablet twice a day. I did that for two days and for some reason it didn't go too well. This is the 2nd time that I have been on doxycycline and this time I was nauseous and throwing up both from the front and back ends. I know that oral antibiotics can be hard on the body, but to be in a fetal position all day was not a good response so I stopped taking it. Bactrim was ordered and I started the oral pill on Friday. I've been taking it twice a day and praying that the oral pill treats the goodies in my lungs. I don't want to have to resort to the intravenous way of treating the infections. Sometimes I don't have a say and my body is the one that has the last word. I've been going 5 months strong without needing a PICC line placed for intravenous antibiotics and would love to make it to the one year mark.

I had run high grade fevers last week ranging from 101.2, 102.5, to 103.7. Fevers often mean infection. Fevers are always uncomfortable because it keeps me from taking care of what I need to do most, which is my breathing treatments and airway clearance. When I have high fevers, it's like taking peanut butter, bacteria, and a 450 degree oven and baking for an hour here and there throughout the day. Basically a recipe for disaster! I called Dr. Light and he instructed me to switch every 4 hours between Tylenol and Ibuprofen which really helped. That and 2 cool showers every day seemed to do the trick. Luckily, I have been fever free as of yesterday, but I still know that I have an infection that needs to be treated.

Side note: I love that I am constantly learning from Dr. Light. A fun fact that he shared with me is that milia is common in those with Cystic Fibrosis. I started to recently notice a few small whitish bumps on my shoulders. He mentioned that because our skin is so salty it is common for milia to form on the skin. He told me to stay away from rich oily soaps. I've officially turned into a bar soap girl.

That is all for today. ::Splish...Splash::

Batter's Up

"I have heard there are troubles of more than one kind. Some come from ahead and some come from behind. But I've bought a big bat. I'm all ready you see. Now my troubles are going to have troubles with me!"
~Dr. Seuss

At times Cystic Fibrosis can be very dictating, but I've decided to make my own rules and show who's boss these days. There are different respiratory machines that are offered to help clear the secretions from our lungs, but throughout the years I have continued to drop in lung function despite having the Vest, Flutter, and Acapella on hand. Out of the three respiratory clearance devices that I own, I have found that I favor the flutter device because it's small and easy to travel with. Even though I show more favoritism towards the flutter, I have always continued to be compliant with my respiratory treatments and got to a point that even while doing different respiratory devices 4 to 5 times a day, I wasn't climbing higher as far as lung function goes. And like a rat in a maze, after running into the wall so many times, I started to look for a new path.

My last Pulmonary Function test showed my FEV1 in the 40's. When a Cystic Fibrosis patient drops to the 30's, a doctor proceeds to talk about going on the lung transplant list. And to me my lungs are my own and I can't give them up that easily especially not to Cystic Fibrosis. I believe that people come into our lives for a reason and I was blessed this year to have a respiratory therapist show me a respiratory device that could help get the mucus secretions up easier. I had thought that I knew every device that was out there for Cystic Fibrosis patients, but I had never known about the G5 Percussor! How did I not know about this!? This device provides vibration and percussion to create a powerful and comfortable percussion to get the goodies up. At first I felt mad that my past doctors had not told me about this machine because it really targets my problem areas which are in my upper airways. My upper airways in my right lung are really narrow from lung damage. I believe that if I would have known about this machine it could have possibly prevented some damage to my lungs that was caused by the disease. I realize that I can't focus on "what if" and I have to proceed with "right now."

I feel blessed to have this new device at my fingertips! My G5 Percussor:


For me respiratory treatments are like giving birth through the mouth haha. I do a lot of huffing, deep breathing, and blowing out to get the thick mucus up. After each respiratory session, I'm pooped. I'm happy to say though, having my G5 makes my secretions come up so much faster compared to the Vest, Flutter, and Acapella. Also, I get so many goodies up! I love it and have been feeling better since I started with the machine. Having tons of mucus in the body/lungs can feel like a huge weight and the goodies are better out then in! Hopefully having this new airway clearance device will help me out during my maze of life and I'll get my piece of cheese at the end.

Ferguson

What lies behind us and what lies before us are tiny matters compared to what lies within us. ~Ralph Waldo Emerson

It's amazing to me how sometimes the body has a way of healing itself.... if you just give it some time. Almost like a cactus in a way. A good 6 years ago, Hunter(my fiance), bought the perfect gift for me while I was on I.V. antibiotics at the hospital. Hunter had given me something unexpected and it was perfect. He arrived to the hospital with a cactus in hand. When my mom had asked him why a cactus and not flowers, he had responded that flowers die, a cactus won't. It was the most beautiful and thoughtful gift ever and we gave the cactus a name, Ferguson. Ferguson later became our love cactus and has been with us for a long time.

This past August, when we had moved, the Comcast guy had knocked him over and he had broke in half. I was in shock! Poor Ferguson! We needed to toothpick him, save him, glue him back together, or something. Comcast guy's attempt to kill Ferguson:
We continued to water Ferguson, watch him, and give him proper sunlight and he started to heal himself. He is still our amazing love cactus. He has grown more character from what he has been through and I think became stronger in a way, with his mickey mouse ears and all haha.


Ferguson healed! He just needed some time. :)

New Year's Eve(December 31st 2010), I started to go down hill. And after 2 and a half weeks of fighting a virus, I'm alive! Like Ferguson, I have healed! I had never experienced flu symptoms that severe in my whole life. During these past 2 and a half weeks, I had spent everyday running high grade fevers, having the chills, night sweats, not being able to keep down my food, not being able to go out and walk around, a whole bunch of mucus in my lung/sinus, and a constant cough(more then what I'm typically used to). The combination of my regular CF symptoms and having a virus was so intense it was as though someone had put a bag over my head and told me to lay down in ice water.

I knew I.V. antibiotics wouldn't help, that I had to just let the virus run it's course. At the same time I was worried because I needed to rest to fight the flu but I had to keep up with my daily health regimen and make sure that I didn't rest too much because I had to do respiratory therapy plus breathing machines to get the mucus out in order to prevent a severe lung infection. The virus and Cystic Fibrosis combination was so uncomfortable, I never thought that the feeling was going to end. I couldn't make it to Miami to see my doctors and it got so bad that my mom took me to a walk in clinic. I thought that I had mono I was so weak, sick, and exhausted. Luckily, I did not have mono and the doctor at the clinic diagnosed me with a bad virus. I started taking Tamiflu(1 pill, twice a day) and Cipro 750 mg(twice a day for 10 days). I also had on hand airborne tablets, iron 27 mg tablet, cf source vitamins, vitamin C 1000mg tablet, halls defense vitamin C drops, Nyquil cough syrup, Azithromycin 500 mg(M,W,F) and Tylenol extra strength because of the constant fevers. The constant song in my head during those days were, "If you're going through hell, keep on going don't slow down, if you're scared don't show it, you might get out before the devil even knows you're there." by Rodney Atkins. I'm just so glad I made it through the long haul, and the best part is I don't have to get I.V. antibiotics. Yay!

Release

In a way I've used my blog as a means to release emotion. And this seems like a good time to Release. I'm so excited for the New Year, I'm hoping 2011 will bring healthier moments. I'm starting off rocky on the health side because New Years eve I got hit with a nasty cold/flu and have been running 102.5 fever and coughing my brains out. I'm used to coughing on a typical day but at this point I'm so uncomfortable with snot and 10 times the amount of mucus due to the cold plus the combination of a sore throat that this situation isn't fun in the least. I'm surrounded by Nyquil, advil pm, cold-eeze, halls defense vitamin C, simply saline nose spray, zithromax and boxes of tissues and I'm hoping that one of these things will start having some beneficial affect. I hate when I get a cold because I spend most of my time praying that the sinus cold doesn't go and spread infection to my lungs. At that point it's game over and I.V. time. I'm going to the doctors tomorrow to see if a good oral antibiotic can kill whatever is starting to brew in my lungs.

Never in my life have I ever said "why me" when it comes to having Cystic Fibrosis. But lately I've been asking more questions trying to get down to the bottom of what the inside of my lungs look like. I feel as though if I had a chance to turn my body inside out for a day and really examine my lungs, I could have more of a game plan on what I'm truly up against. For the past 5 years I've had these crackles in my right upper lobes and I've just assumed that it was because I had a lot of mucus in my airways due to the disease. The crackling sounds in my lungs have become louder when I breathe and I started to wonder why I sounded like a bowl of rice crispies drenched in milk. I recently had a CT scan of my lungs and they found a lot of Bronchiectasis(damaged lung). A doctor had explained to me that the damaged lobe is floppy and its so damaged and narrow of a passageway that it makes that crackling sound. Basically it has no use, no airway function.

Bronchiectasis:

It's basically a breeding ground for infection. But I can't remove the damaged parts of my lungs because a CFer is told that we have to preserve as much of the lung as possible. And we can only go through the process of lung removal/transplant when it's basically a life or death situation and you need to have a lung transplant in order to live. Then comes a lot of anti-rejection medication and a whole other journey that I have yet to take and I'm pushing that door closed shut with all my might. My doctor believes that the removal of my right upper lobe wouldn't benefit me at this time and overall could cause more infection or more damage in the long run with the recovery being intense. So I just have to deal with the crackles for now and hope that I can maintain my lungs as long as possible.

A new chapter to 2011 is that I have stopped the PTC124/Alturen clinical trial that I was in. I had given one year of my life hoping that this drug would benefit me and really get into my system and help relieve some of my CF symptoms but it had done nothing. Then I signed up to do the extension of the clinical trial and after three months of being on "the real drug" my PFT's had dropped. I said sayonara and knew that it was time to take matters into my own hands. My old regimen consisted of pulmazyme, hypertonic saline 3%, and colistin(month on month off) and now my new daily regimen is pulmazyme, hypertonic saline solution 7%, and Cayston(month on month off). Hoping that switching up my health regimen and getting on some new medications could only help my lung function for the future. I'm hoping for a better year healthwise and hoping to breathe easier in 2011.

"Never let go of hope. One day you will see that it all has finally come together. What you have always wished for has finally come to be. You will look back and laugh at what has passed and you will ask yourself...'How did I get through all of that?"

Layers of Lasagna

My life right now has consisted of layers falling right into place, gradually making the perfect masterpiece.


The First Layer being the foundation. The part that balances all the ingredients and makes it sturdy. That being my fiance and the day that he proposed to me on 10/10/10. I felt like the luckiest girl in the whole wide world when he proposed to me at the Morikami Japanese Garden. I must have shook my head so many times it was though I was a little bobble head doll and of course said the magic word "YES" followed by times infinity. It was the most magical day of my life and it is forever stamped into my mind. I don't know how I could have been so lucky to find him. To me he is that four leaf clover that you only stumble upon once in a lifetime. I'm floating on engagement bliss right now!

The 2nd layer- The saucy, meaty, cheesy goodness was when I was told by my doctor that I was going to have a respiratory therapist come over to do chest physiotherapy on me everyday in the morning. I feel so spoiled because I've never had a specialist come to my home everyday before just to beat on my lungs. Mainly old school chest PT could only be done when I went into the hospital. As a child, my mom would clap/beat me on my chest to help get up the mucus but as I got older it became of course harder for her to do. The Vest or flutter became my form of chest physiotherapy but nothing compares to old school chest physiotherapy done by a professional. Before my respiratory therapist came into the picture my fiance would occasionally beat me and it was acceptable lol. But it's nice to give him a break and let a respiratory therapist do all the work. :) For 45 minutes every day in the morning my therapist beats me and I love it!

Third layer- After 6 weeks of intense I.V. therapy I had the line pulled! No more PICC line and I'm so happy to be free! It's not the PICC line part or the hospitalization part that I hate the most. It's more of the feeling like I'm on a leash and my CF is the thing that's holding me back. It's that reminder that as many times as I try to run free, it catches up to me. My FEV1 is back up to 51% and is staying stable. I'm just praying that I won't fall back again. I'm feeling amazing and free of infection. Although, I still have the abnormal production of mucus which is part of having Cystic Fibrosis. Unfortunately, the I.V. med's can't get rid of the mucus. The I.V. meds just clears the infection so that I can have the energy to work hard every day to clear the secretions out of my lungs and helps to create less mucus production. Creating a snowball affect of good times. A part of me did not want to get off of the I.V. meds because once getting a taste of having less peanut butter thick mucus production, clearer airways, less inflammation, breathing easier, more energy.... trust me you wouldn't want to give that up in a second! But I knew that I couldn't stay on the hardcore antibiotics forever because in the long run it's not good for the other organs in the body...ZONK!

Forth Layer- I have made a goal to run a half marathon in which I'm currently training for. I know you would think someone with 51% lung function(practically half a lung) running a half marathon is nuts. But it's just my can do attitude right now and I'm sticking to it. As I've been training my right lung has been giving me problems causing pain. My right wussy pants lung needs to learn that I'm going to accomplish this goal and needs to join the can do party already! I'm super excited and I can't wait to check it off my bucket list!

Fifth Layer- Sauce, Cheese...and more Cheese. I'm involved right now in an Ataluren/PTC124 clinical trial. This drug trial is for CFer's only with a rare mutation or properly defining it as a nonsense mutation for all you science nerds out there. :) I have one Deltaf508 mutation(90% of CFer's have this mutation) and one W1282X mutation(rare/nonsense mutation). There are many, many different mutations of Cystic Fibrosis. That is why every case of CF is different. In a short explanation, basically this drug will psyche the W1282X mutation that I have into believing it's "normal" and making it function properly causing less thick mucus production, easier breathing, and fewer hospitalizations. This is the scientists trying to get closer to a cure, getting down into the cellular composition of it all. If it works, it will be a huge breakthrough. In past phases there was an increase of lung function in patients. I have to take the powdered form of the drug, mix it in water, and drink it three times a day. I'm hoping for high numbers and no double heads! :)

Everything perfectly baked to perfection....

Golf Balls

I'm finally home. Happy. Stress-free. Surrounded by my two loves (my boyfriend and dog). I'm able to finish up my I.V. therapy at home and get the best sleep in my own bed. I couldn't ask for anything more.

A couple of days ago I read the best chain mail. I never really forward the mail out but I decided it was worthy enough to share.

The Mayonnaise Jar

When things in your life seem almost too much to handle,
When 24 hours in a day is not enough;
remember the mayonnaise jar and 2 cups of coffee.

A professor stood before his philosophy class
and had some items in front of him.

When the class began, wordlessly,
he picked up a very large and empty mayonnaise jar
and start to fill it with golf balls.

He then asked the students if the jar was full.
They agreed that it was.

The professor then picked up a box of pebbles and poured
it into the jar. He shook the jar lightly.
The pebbles rolled into the open areas between the golf balls.
He then asked the students again
if the jar was full. They agreed it was.

The professor next picked up a box of sand
and poured it into the jar. Of course, the sand filled up everything else
He asked once more if the jar was full. The students responded
With an unanimous 'yes.'

The professor then produced two cups of coffee from under the table
and poured the entire contents into the jar, effectively
filling the empty space between the sand.
The students laughed.

'Now,' said the professor, as the laughter subsided,
'I want you to recognize that this jar represents your life.

The golf balls are the important things - God, family,
children, health, friends, and favorite passions
Things that if everything else was lost
and only they remained, your life would still be full.

The pebbles are the things that matter like your job, house, and car.

The sand is everything else --
The small stuff.

'If you put the sand into the jar first,' he continued,
'there is no room for the pebbles or the golf balls.
The same goes for life.

If you spend all your time and energy on the small stuff,
You will never have room for the things that are
important to you.

So...

Pay attention to the things that are critical to your happiness.
Play with your children.
Take time to get medical checkups.
Take your partner out to dinner.

There will always be time
to clean the house and fix the dripping tap.

'Take care of the golf balls first --
The things that really matter.
Set your priorities. The rest is just sand.'

One of the students raised her hand
and inquired what the coffee represented.

The professor smiled.

'I'm glad you asked'.

It just goes to show you that no matter how full your life may seem,
there's always room for a couple of cups of coffee with a friend.'

Better Than This

As I think back 16 days ago, I realize that a lot has happened during my stay here at the University of Miami hospital. In a nutshell it has been constant work, work, work. My goal of trying to get my lung function back up has been a continuous fight while arm wrestling with the infection that has caused trouble in my lungs. My stay has consisted of hours of breathing treatments each day, chest physiotherapy(40 minutes of vest and an hour of manual chest PT each day), lab work, x-rays, Pulmonary Function Tests, and I.V. therapy. At this point I'm running on empty. But I know if I give up, I won't get anywhere.

As I rewind back to the first week I felt a little nervous because I was living in a new place here in Miami and had to find a new doctor. Fortunately, I found Dr. Light. His name had a nice ring to it and he had a British accent. I think he won me over with that haha. He informed me that he had been treating CF for 40 years and I could tell he was very smart. All I could think of was thank goodness he has been doing this for 40 years and I have been doing this for 25...we are going to be a great team! My first impression of him was great because of his positive demeanor. I explained to him that I'm no slacker when it comes to my health. I work really hard and when CF knocks me down, I need him to bring the big guns to help me get back up. I also loved during our first conversations he wanted me to set a goal. I've always wanted to run a marathon...well maybe start small and do a half marathon. :) And Dr. Light was positive that I could do it when my lung function got back up but it would take a lot of work and time. Everyone has challenges, it's how we deal with them that makes us either rise or fall.

They could not get a PICC line in my right arm which led to lots of holes and bruises:


Luckily success on my left arm...my Double Lumen Power PICC Solo, only requires saline flushes for long term I.V. use:


The first guns that Dr. Light pulled from his sleeve was Tobramycin(once a day) via I.V. and Zosyn every 6 hours via I.V. During that first week I felt like I was getting worse. And truly while on I.V. antibiotics my body always goes through the worst before it gets better. After the first week, I did PFT's and my lung function was down another two points at 44% FEV1. I told him that I wasn't discouraged and gave a BIG pft to my PFT's. My mind at this point was stronger then my body and I was holding on strongly to that. The stairs being my biggest barricade, I knew that if my lungs were going to become stronger I had to start with that. Putting one foot in front of the other I kept on telling myself it's only up from here and I can make it happen.

The second guns that Dr. Light pulled from his sleeve was Gentamycin(once a day) via I.V. and Imepenim(every 6 hours) via I.V. because he wanted something a little stronger after seeing my PFT results. That week was rough because I had noticed changes in my body feeling weaker then ever. Throwing up a lot from the I.V. meds going through my veins, weakness, and loss of appetite. I also noticed that when my boyfriend came to visit and we would go walking I felt like I couldn't breathe and I had to stop a few times to catch my breath. I felt like I was running in place and my heart was going to jump out of my chest but I wasn't running at all. We had just thought the medicine was much stronger this time around and that my body was getting hit hard. Little did we know that the reaction would intensify leading to two EKG's, a chest x-ray, not being able to breath on room air, being put on oxygen to breath, and having tachycardia. Unfortunately, during this time my doctor was out of town, and I had 3 brainless doctors working the floor during that week and they didn't know what was causing this reaction. Dur dur dur...if you google the medicine it shows that the antibiotics can cause adverse reactions such as not being able to breathe and tachycardia. I was in shock that the doctors did not have a clue and did not give a proper diagnosis. And it took my brilliant mom, me, and Hunter to figure it out. We knew immediately that it was the medicine and we had to tell the doctors to stop the I.V. medication going through my veins right away. When my doctor was informed he knew immediately as well that it was probably the imepenim that caused this type of reaction to occur and that my mom, me, and hunter did the right thing by telling the doctors to stop the I.V. meds.

Doctors FAIL diagnosis and FAILED words of wisdom that week:
1. "your lungs are filling up with water"...umm no that is mucus you see on my chest x-ray.
2. "you need to stop drinking so much water and be put on a diuretic"...ehhh no if I stop drinking water I will become dehydrated and hell no you are not putting me on a diuretic
3. chest x-ray is performed..."you need to have another chest x-ray two hours later"...umm no what will that prove and what is the logic behind that?
4. "you need to relax and take deep breaths and you will start to feel better"...umm really I'm very calm right now thank you very much even with the situation, it's not anxiety...seriously something is wrong here.
5. "You are walking too much you need to stop"..umm no exercise is good for the lungs and will help me to get better faster.
6. "This is normal it's your CF"...okay who are you and you can go sit in the corner...you will not pass GO or collect 200 dollars.

Overall, thank goodness I didn't listen to them because the situation could have gotten worse if I would have said yes to there stupid comments. I knew that this was not normal, it wasn't my CF causing the tachycardia and not being able to breath...it was an adverse affect from the I.V. medicine and my body just didn't like the drug and reacted in defense mode.

This week, week 3, my doctor is back in town what a relief. Lung function test down two more points to 42% FEV1. After looking at my chart and doctors notes he apologized for the stupid doctors that were on the floor. New guns and hopefully the final guns will be Ceftazadime(every 12 hours) via I.V. and Tobramycin(once a day) via I.V. I know that my body has been on a roller coaster ride and I've had a few minor set backs. Dr. Light knows that we still have a lot of work to do and I'm ready to put in the time if he is behind me. My mind continues to be strong focusing on the fact that things are going to get better and my body and lungs will eventually catch up. Because I have to believe that there is more than this...that I can be better than this.