Brass Knuckles

"Give thanks for what you are now, and keep fighting for what you want to be tomorrow." ~Fernanda Miramontes-Landeros

The last time I had intravenous antibiotics was in October of 2010. I'm almost at the one year mark of having no I.V. antibiotics. I try to go a year without needing the I.V. medication. Although, I started to wonder if waiting for the inevitable was really going to help me in the long run. No one has all the answers in the treatment of CF, it's not a black and white disease, it's complicated. I have no regrets in the health decisions I have made in my 26 years of living with Cystic Fibrosis and hopefully my tic-tac-toe decisions will pay off in the long run.

I had a doctors appointment on Tuesday and my PFT's were surprisingly better than my last PFT's! I was feeling a little junky so I expected the numbers to be in the low 40's, however, my FEV1 was 52% pre-RX and then after taking Albuterol via nebulizer my FEV1 was 57% post-RX. I was extremely excited to see those results. I have Asthma along with Cystic Fibrosis so my lungs seem to be happier after a bronchodilator. My x-ray showed a whole different view of my health. My x-ray looked like Casper the Ghost took a bath in my lungs, especially my right lung. A "normal" x-ray shows the lungs in black and the bones in white. This is what my x-ray showed:
The white color that is displayed in my lungs is all mucus. My lungs always have white in the x-ray because Cystic Fibrosis patients are always producing thick mucus in the lungs. When there is an increase in areas or clusters of white parts, I have to be more aggressive and make sure to knock CF and my goodies down a notch with I.V. antibiotics. An increase of white often means exacerbation or infection of the lungs.

I gave my consent to move forward with a bronchoscopy and I decided to show my lungs who's boss! :) I haven't been a fan of getting bronchoscopies because I know that the surgery is invasive. A scope is sent into the lungs to suck up the goodies and saline is also put in the lungs to flush, irrigate, as well as clean the goodies away from the bronchial walls. There are risks of puncturing the lung or the lung collapsing. This was my 2nd bronchoscopy and the surgery was performed on Thursday. I got an I.V. placed in my right hand. Then, I inhaled lidocaine via nebulizer to numb the back of my throat and lungs. I had planned to do the surgery by conscious sedation, but the plan changed because my doctor wanted me to be extra comfy. I was given the Michael Jackson drug, Propofol, through my I.V. and I was sleeping within 2 secs. The drug has an amnesia affect, so I don't remember too much. Hunter and my mom told me that I kept talking really loudly after the surgery, I was telling everyone how great they were, and I even fist bumped my doctor that performed the bronch. lol And I don't even remember talking to my doctor after the surgery.

After surgery, I coughed up blood for the first 2 days and then it went away. It was pinky mucus rather than full blood clots coming up. And I ran 101.4 fever which I was told can happen after a bronchoscopy. After a bronchoscopy the lungs are usually irritated and angry which causes inflammation, bleeding, and tenderness of the chest. I felt as though I had lifted heavy weights. My chest was tender and a little sore, but nothing too painful. I made sure to rest a lot to recoup from surgery.



If you have a weak stomach, don't view the pictures below. The pictures below were taken during my bronchoscopy. My doctor started with a picture of my vocal cords, then took another picture going down my larynx, which continues into my bronchial airways. Fortunately, he was able to get a lot of my everyday mucus up and send the goodies out to the lab to get cultured. I know the day will come where I don't cough, have thick mucus, and can breathe easy. That's my wish.
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*Graphic Pictures Below*

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Tools of the Trade

"If you don't like something change it; if you can't change it, change the way you think about it." ~Mary Engelbreit

If you were to tell me three years ago that I needed to get a port, I would have cried like a little baby. Today, I am actually excited that a port will be on the menu and I'm going to be fist-pumping all the way to surgery. I've decided to name the port Penelope and rock it out with pride! It is time to put on my big-girl panties and get it done!

After years of numerous PICC lines for intravenous antibiotics, my right-side vein that goes to my heart has been scarred. This has led to a narrowing of the vein that leads to the heart, which in turn makes the catheter hard to thread and eventually gets kinked around the armpit area. Also, when it comes to my left side, the main vein that leads to the heart is very small, which makes it difficult for the PICC line team or radiologist to deal with. Basically, no good veins = time for a port.

Also, I have become allergic to a lot of the surgical material used in the prepping of intravenous antibiotics. I am allergic to tegaderm, chloroprep, betadine (under dressing), and surgical sticky tape. The only I.V. dressings that work for me are the I.V. 3000 dressing and Opsite dressing, and then rubbing alcohol to clean the area. I know that PICC lines are already hard for radiologists to do, but to prevent allergic reactions on top of the surgical procedure can become a little much at times. Keep in mind I'm not getting the port for them, but for me. I think the port will reduce the tedium of getting intravenous antibiotics - it simplifies the whole process. All I have to do is access the port when needed and flush once a month when I am not using it. Easy as pie!

I have decided to make the decision to move forward and reach for higher health goals. I'm hoping the port is going to make my life easier and make my lung function better as a result. I've been thinking about my port, not as a negative stepping stone, but as an accomplishment in my CF life that I have come this far.

My port will be used as another tool in my arsenal. After reading and educating myself on ports, I found that I can either get an arm port or a chest port. I'm leaning towards getting a Navilyst Xcela chest port. Almost like a car, I want it to be a long-lasting and reliable addition to my life. I also have an option to get a Groshong port, but I've heard some complications have been seen with blood return. I loved my Groshong PICC lines, but I am thinking I want an open ended catheter for my port instead of the side slits that are displayed in a Groshong type of catheter. I want a port that won't clot, won't cause complications, get infected, or float. Seems like simple requests to me. ;)

This is the Navilyst Xcela chest port that has an open end (which requires Heparin flushes) and I liked the fact that it can be sewn down in the pocket stitch that the radiologist makes. This one is pretty! This port is the one that I'm currently leaning towards getting. :)



And this is the Groshong chest port that does not require Heparin:

And PURPLE! Weeeeeeeeeeeeee!

I'm finally ready for this adventure. :)

Breakfast of Champions

"To succeed in life, you need three things: a wishbone, a backbone and a funnybone." ~Reba McEntire

After fasting from midnight until ten in the morning, the only thing that I could have for breakfast was a bottle of orange liquid that a nurse handed me to drink. The sugar coated mix of glucose vaguely resembled the citrus beverage Orangina in a way, but ten times sweeter. This breakfast of champions concoction was meant to test my glucose tolerance. And so the glucose challenge began....

The reason I was having this test done is because it is common in those with Cystic Fibrosis to develop Cystic Fibrosis Related Diabetes(CFRD). Cystic Fibrosis Related Diabetes is a unique type of diabetes and is not the same as diabetes in people without CF. The two types of diabetes in a non-CF population are Type I diabetes(insulin dependent) and Type II diabetes(known as non-insulin dependent). CFRD has some features of both types of diabetes. I have been more fatigued and my lung function has been low which are common symptoms of Cystic Fibrosis Related Diabetes. I was hoping deep down inside that I wouldn't have to add CFRD to my CF résumé. CFRD develops in CF patients because our pancreas becomes scarred causing the body to not properly produce insulin.


Blood was drawn before as well as after I drank the orange goop. The results came back that my fasting level was 88 and 2 hours after I drank the orange glucose my level was 172. My fasting level was normal which was great to hear and the 2 hours after showed impaired glucose tolerance(borderline diabetic), but was not high enough to declare that I have Cystic Fibrosis Related Diabetes. I was happy to hear those results! I don't mind staying borderline and would hope to remain that way for years to come(my optimistic side coming out). After the blood draws and fasting were done, I immediately ate a plethora of food, and enjoyed each scrumptious bite!

Dakota

"Spread the diaper in the position of the diamond with you at bat. Then fold second base down to home and set the baby on the pitcher's mound. Put first base and third together, bring up home plate and pin the three together. Of course, in case of rain, you gotta call the game and start all over again."~Jimmy Piersal

When your little and the teacher asks you, "what are you going to be when you grow up?", many young girls answer, "I'm going to be a mommy." I, on the other hand, had a different response believing that I was going to be a singer. But, it's funny how we all go from being in a preschool class to eventually growing up on different paths with different life goals.

My friend Danielle is pregnant and about to become a mommy. Danielle and her husband, Brandon, will be having a beautiful baby girl in August. I am so excited for them, I know they will be great parents! Yesterday was the baby shower for her baby, Dakota. Danielle was beautiful and glowing. She had her close friends and family there to celebrate her exciting new venture into mommyhood and we all brought gifts to spoil the baby that is on the way. We played the "guess the baby animal name" game, "guess the measurement of Danielle's tummy" game, and "how many diapers are in the diaper cake" game. All of Dakota's gifts were so tiny and cute. When I first found out that Danielle was pregnant, I was so excited to start shopping for tiny baby items. I found myself getting distracted and making a detour to the baby section every time that I was at a store because everything was just so darn cute! Basically, I had a new addiction, it was called shopping for Danielle and Brandon's unborn baby!

Hunter, Me, Danielle, and Brandon at the Baby Shower:

I guess I'm at that age where many of my friends are getting married or having children. I grew up thinking that is what people do, they get married and have kids. But that is not always the case. I've always been fond of kids, thinking they were really cute, but somewhere down the line I came to a fork in the road and started to ask myself, "is this what I truly want in life?" I remember a doctor telling me that it would be really hard for me to carry a baby. When a baby is growing inside a womb, the baby will start to press on the lungs and other organs making the experience uncomfortable. Also, for a woman with Cystic Fibrosis, I've heard that when the baby pushes on the lungs, it makes it even more difficult to breathe. I already have compromised lungs from the disease and I wouldn't want to put myself at risk by trying to carry a baby for 9 months as well as give birth. I sometimes get uncomfortable when I eat too much, I couldn't imagine having a watermelon inside of me and feeling that I just had to take a poop and couldn't haha.

I know that I can always choose the path of adoption. I believe adoption is a beautiful route to take. If Hunter and I, down the line, ever have the urge to have a child and decide to take that route, we know that adoption is a great option. Hunter has told me that if a baby is going to jeopardize my health, he would pick me over having a baby, hands down. Thankfully, I have an amazing man in my life, that is supportive, and has the same views as me when it comes to a family. For now, we are happy being proud parents to Aiden, our fuzzy border collie. And I love our little family. :)

Just A Number

"Adapt or die. As many times as we've heard it, the lesson doesn't get easier. The problem is we're human. We want more than just to survive. We want love. We want success. We want to be the best that we can be. So, we fight like hell to get those things." ~Grey's Anatomy

When did life become a number game? Age is just a number, weight is just a number, and my pulmonary function test results were just a number today. I've never been that great with math, but in my life where numbers are so important, I put a lot of chips on the number game hoping for the best luck.

Today, was a regular check up day with my CF doctor, Dr. Light. I laid in bed the night before after hours and hours of doing chest physiotherapy and breathing treatments, wondering about my appointment and how it was going to play out. I knew that I had to do a PFT(pulmonary function test) to see the progress of my disease and I've gotten to a point in my life where just thinking about the test makes me a little shaky in the knees. My disease is progressing and I'm aware of the situation. When I go to the doctors, I feel like I am under a microscope as I talk about my medications, health regimen, and daily obstacles with Cystic Fibrosis. By the time I'm done with seeing the nurse, the psychologist, the nutritionist, and doctor plus a resident to examine me, I'm exhausted. At this point, I just want someone to come in and say your perfect, but I know even if I put on make-up and dress up, I still can't hide the fact that my organs are failing on me. My PFT results were 46% FEV1, they had dropped. As I stared down at the results on paper, I couldn't hold back the emotion I was feeling, even though I was telling myself it's okay, it's okay. I allowed myself to release knowing I needed that moment to cry. At that moment my smile was stolen. Why is the number so low? Why can't it be better?

The doctors, Dr. Avacelis and Dr. Light, who were both in the room said, that a number is just a number and it's more important how I feel. So numbers aside, I don't feel super great health wise, but I don't feel crappy enough that it is hospital time either. I just feel like I'm coasting. I feel that people adapt, adaptation is a part of life. Living with Cystic Fibrosis, my body has learned to adapt. I have learned to keep going and keep pushing, regardless of my low lung function. From what I have read and learned throughout the years from reading blogs and talking with other people with Cystic Fibrosis is that even a person going through transplant or chronic rejection after transplant will have the will to adapt, keep on going til there is no more sand in the hourglass, and on the outside you wouldn't even know how hard the person is truly working. It's how the body is made. I explained to the doctors that I'm keeping up with my breathing treatments, chest physiotherapy, I take all my oral medications, and inhalers, my CF digestive issues are good(I eat like a cow), and I have my workout buddy, Aiden(my fuzzy son), who is my outdoor trainer. I'm doing everything that is expected of me. Doctors orders are to keep on doing what I'm doing and increase the exercise. Also, they want to run a glucose tolerance test again to see if I have Cystic Fibrosis related diabetes(CFRD) which is common in Cystic Fibrosis. There was one tweak in my regimen and that is to not do Colistin via nebulizer anymore because that antibiotic does not seem to prevent infection as well as it used to in the past. I will be going back to using Tobramyciin via nebulizer twice a day on my months off of Cayston. We either adapt, fight, or flee.

Bucket List

"If you want your life to be a magnificent story, then begin by realizing that you are the author and everyday you have the opportunity to write a new page." ~Mark Houlahan

I haven't written in awhile and I decided to come here and let me thoughts wander as usual. Since I last wrote, I was able to prevent the infectious fire from spreading in my lungs, avoiding the hospital at all costs. Thankfully, the oral antibiotics did the trick with the help of the magical nebulized Cayston antibiotic. Being that I can not see what is going on inside my lungs, I often use analogies and imagery to paint a picture. When I start becoming more congested with an infection, this causes my airways to tighten, and I become more crackly compared to my regular CF mucus days, I try to jump on it as fast as possible. I almost think of it as a fire inside, if I'm able to catch it early then I can prevent the fast spread, damage, and disaster to come. With my pulmazyme, hypertonic saline solution, and Cayston trying to be the extinguisher. Everyday it's like a constant flame burning in my lungs, the disease being chronic, causing damage over time. Making me realize that time is a very precious thing I have right now.

I have never really sat down and written a bucket list on a piece of paper, but inside my head I have my list of things I want to accomplish in life. I was able to cross out one of the activities on my bucket list recently. I went to PARIS! I had the most amazing time with my fiancee, my best friend Christina, and her fiancee Jake. I loved every moment. It was definitely an adventure for me and my taste buds! I tried so many french dishes, I was open to everything including escargot which I enjoyed. We were able to see the top tourist sites including, The Eiffel Tower, Arc de Triomphe, Notre Dame, shopping on the Champs-Elysees, Sacre Coeur, The Louvre, and we saw the Moulin Rouge. The best part is that my best friend asked me to be her bridesmaid on the very top of the Eiffel Tower! I was shocked, I had no idea she was going to pop the question haha. There were two moments during the trip where I had thought I can't believe I am doing this, as I put one foot in front of the other. I was able to climb to the top of the Notre Dame and the top of the Arc de Triomphe. My lungs aren't in the best shape right now and I'm lacking a normal oxygen level on room air. After hearing that my lungs are damaged due to Cystic Fibrosis a billion times has made me look at climbing a billion stairs as a feat in itself. I definitely spoke some lovely french words on the Notre Dame climb and the Arc de Triomphe climb! Even though I probably looked like Quasimodo when I got to the top, I felt like a superstar. There was an indescribable feeling that came over me, and even though I did not make it known to my fiancee, Christina, or Jake, I was silently dancing inside. Overall, it was an amazing trip with amazing people!

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::