Spring Forward & Bloom

"Flowers don't worry about how they're going to bloom. They just open up and turn toward the light and that makes them beautiful." ~Jim Carrey

46% FEV1 was the verdict, after I huffed my heart out during my pulmonary function test. Maybe I'm just too much of an overachiever at heart, but I always hope for higher numbers. Still, I felt mostly content with these results because I know that it can always be worse. My daily health regimen has been like a respiratory bootcamp, consisting of 2 hours of manual hand-clapping on my chest by a respiratory therapist, 5 breathing treatments, an hour of exercise with Aiden (my fuzzy son), 40 minutes of Vesting, and frequently using my G5 Percussor for airway clearance. One would hope that after constantly coughing and moving the goodies out, the mucus would eventually hold up the white flag and surrender. But, after 28 years of fighting Cystic Fibrosis, I've learned that my arch-nemesis is relentless!

Recently, I was honored and humbled by the opportunity to share my personal story living with Cystic Fibrosis at a Sequenom conference. During the Q&A session after my speech, an audience member asked, "Where do you find the time to do other activities besides your health?" I explained that it's a big challenge to find balance throughout the day. The doctors ask a lot from Cystic Fibrosis patients, and our daily health regimen is extremely involved as a result. Nevertheless, Cystic Fibrosis is what I have; it's not who I am.  

Another question that an audience member asked, "Do you think your lung function will ever improve?" My answer wasn't complicated, but is somewhat difficult to explain. I often find myself clinging to hope. I've been told that my lungs are damaged by mucus which continues to clog my airways, and that I cannot mend what the mucus has destroyed over the years. This mucus has managed to create holes in my lungs, destroying the cilia in my airways and causing my airflow to be diminished. The picture that has been painted in my mind is that my lungs are like a wasteland - damaged and scarred. This is a haunting image, to be sure, but I find comfort in knowing that, even in the darkest of places, light can exist. Even in the harshest environments, life can flourish. And even from the cracks in the pavement, a flower can bloom. I think it's important to always remember that.

   

Bungee Cord

“When you come to the edge of all the light you know, and are about to step off into the darkness of the unknown, faith is knowing one of two things will happen: there will be something solid to stand on or you will be taught how to fly.” ~Barbara J. Winter

In my last post, I wrote about my desire to find solid ground and above all, strength again. I wanted to update my blog to discuss what my journey has been like these past 6 months. Finally, I have found something solid to stand on, and I no longer feel like I'm bouncing up and down on a bungee cord. Let me explain: when Cystic Fibrosis is really kicking my booty, I sometimes feel helpless, like I'm suspended in mid-air by a bungee cord and plummeting toward the ground. In truth, I never really know if I'm going to hit the dirt, or bounce back and rise toward the sky again. It seems that each time I get sick and fall, the cord stretches a little more, and I'm left with that awful feeling of uncertainty. What if I hit the ground? What if this is it? Fortunately for me, I've always managed to bounce back after each drop.

I have been told many times over that exercise can be a huge benefit for the lungs of Cystic Fibrosis patients. Exercise helps to push air deep within the lungs, expanding them, and forcing mucus secretions out. Also, exercise can serve to strengthen the immune system. When I was feeling my worst and could barely breathe, my fiance helped me to get on the treadmill in our bedroom everyday. My lungs were on fire, burning with every step, but Hunter kept assuring me that it would get easier. He had enough faith for both of us. I'm not going to lie - just doing 2 minutes made my lungs hurt like hell, and exercising took a lot out of me. I threw up, I cried, but I made a promise to keep exercising a little more each day. In a way, maybe I did not want to let him down, and above all, myself. I became more focused, repeating the words over and over to myself, "it doesn't matter how fast you go, just keep going." With The Fray blasting on my MP3 player, my little 2-minute walk turned into a 5-minute walk, and in time, that 5-minute walk eventually became an hour-long cardio session. Months later, I found myself laughing through tears - not because of the pain - but because I was so relieved that I was able to do it. That slow, painful 2-minute walk that had me on the verge of tears was now a full-blown hour-long workout that left me feeling like Superwoman! I was ecstatic that I had come so far, and I never wanted to go back to feeling like I did just months prior. Hearing The Fray's song, "Run for Your Life," along with the words of my fiance, really helped light a fire inside me. I am so thankful to have Hunter's love, patience, and support. He makes me a better person everyday.

My pulmonary function test went from 36% FEV1 to 57% FEV1 in April. I was super excited, and exercise has continued to be a part of my daily regimen since then. I have been feeling like a badass, inside and out!

Come April, I had completed 3 miles on my treadmill! :)

Also, I have been going to The Salt Room in Orlando twice a week. The Salt Room is a more holistic way of treating my CF lungs. Dry, pharmaceutical grade salt is pumped into a room during a 45-minute session. Salt naturally has antibacterial properties, and I'm able to get tons of secretions up during my sessions. The Salt Room is like a car wash for my lungs! In patients with Cystic Fibrosis, our salt channels are messed up, causing abnormally thick mucus to build up in the airways of our lungs. Scientifically speaking, Cystic Fibrosis (CF) is caused by the loss of functional CFTR Cl-channels on a cellular level. This defect disrupts salt and liquid movement in the airways of the lungs. The thick mucus makes it hard to breathe and leads to infections, lung damage, and clogged airways, as well as early death. The Salt Room has helped me to sleep better, get up more secretions easier, and has helped with my allergies as well. I'm hooked! :)

The room is covered in salt! Yum!

 

Finding Solid Ground

"A man who removes a mountain begins by carrying away small stones." ~Chinese Proverb

I was walking on unsteady ground last year, praying that 2012 would bring better days. Over the course of these last few months, I've undergone three surgeries, two trips to the E.R., three months of intravenous antibiotics, and two hospitalizations overall. But through it all, I think I've learned something important. When we find ourselves standing on unstable soil - as we all do from time to time - it's only natural that our first instinct is to try and find that familiar land that once lay firmly under our feet. So, what do we do? We dig. In search of solid ground, we dig frantically - sometimes using our hands, sometimes a shovel. And sometimes, we even resort to using the heavy machinery, like a bulldozer. But regardless of the tools we use, there are times when no matter how far we dig, we find that the soil is still unstable. Where are we then? Standing in a hole that we created all by ourselves. And unfortunately, climbing out is never as easy as it looks.

In November 2011, I thought all the bumps in my road were behind me, but they weren't. I've learned firsthand that complications and symptoms of Cystic Fibrosis can be unpredictable. There have been days where I have not been able to breathe on my own, and have had to rely on the use of oxygen, throughout the day and night. My days have been much like a roller coaster, both physically and mentally; feeling fine one day, and not able to crawl out of bed the next day. I've had days where I could hardly walk from my room to the front door, which left me feeling that I was no longer capable of fending off my disease. Was it winning? Was I reaching the end of my rope? Or did I need to remain vigilant and simply have more faith that my daily health regimen would prevail in time? My health had been number one on my "to do list," everyday, for a long time now - four breathing machines in the morning, two in the afternoon, three breathing machines at night, inhalers, countless pills each and every day, along with my G5 and Vest physiotherapy. I was working hard, and I was growing tired of all the roller coaster's loops, turns, and falls.

I tried to become friends with my new port in the months of September, October, and November, but the last straw was when my heart started to hurt. By the time December rolled around, I was ready to have this port taken out as soon as possible. A port might work for many people, but my body didn't have a great response to it. My favorite surgeon was booked, so I had the port taken out on December 9th, by a surgeon I did not know. I just knew that I wanted it out ASAP and I felt as though I could trust any surgeon to do a great job. Unfortunately, it was an awful experience. Many medications in surgery can affect the lungs in a negative way, causing even more complications. Also, I have a lot of contact allergies that all the nurses and doctors taking part in my procedures need to be made aware of beforehand. Therefore, I made it a point to tell them, so that things would go smoothly.

When the port was placed in my chest in September, I was draped for surgery first, spoke with the surgeon who was performing the procedure, and then, when all was ready, I was sedated with Fentanyl. I was hoping that the same would happen when the port was removed, but unfortunately for me, this particular hospital's radiology team was a nightmare. The nurse drugged me without my consent. I was pleading with her to wait, at least until I was draped for surgery, but she refused. I remember lying there feeling helpless and uncomfortable, realizing that I didn't even have a say in my own health care at that moment. I wanted to run for the door, but I was hooked up to an I.V. on a gurney and couldn't move because the drug was already taking affect. At that point, no one was there in the room except for me, the nurse that drugged me without my consent, and a tech in the side-room who was still gathering materials for the surgery. It was truly a traumatizing experience that really left me not wanting to be put through another surgery anytime soon. I will never go back to that hospital again. Looking back, I wish I would have had my port removed at Winter Park's hospital, where I had it placed, because the radiology team there is the best. That chapter is closed, now on to the next.

The following day was my 27th Birthday Bash. My 27th Birthday Bash was a happy day for me. The theme was 50's housewives and I did not want to cancel the party because I needed to be around my favorites, I needed to laugh, and I needed to have a day to enjoy fully without complications. I am blessed to have the most amazing friends. Some of my best friends I grew up with, and others I met in college. It is funny how most of them became nurses, psychologists, and physical therapists....and I...I am Quasimodo, haha. They helped to make my Birthday celebration even more extra special.








The night before my birthday bash, I noticed that I had started forming a rash around the surgical sight. My allergic reactions never happen immediately. Most of my contact allergy reactions are gradual. The only reason for me to break out in a rash is if during surgery, someone accidentally uses something that I am allergic to. I had printed out a sheet stating all my contact allergies, including a wrist band with most of that information printed on it, but these idiots still managed to screw up. It was a localized rash that was only present on my chest at first, but eventually spread to my neck. I've had a reaction like this before, during my prior PICC line placements, and knew that it was only going to continue to get worse until I would have to take a trip to the E.R. I did not want to go to the hospital. That was the last place I wanted to be. I really wanted the Cortisone cream and Benadryl tablets to work, but as the hours passed and the clock turned to 3:00 am, it was a domino effect that would eventually lead to me spending 8 hours at the E.R.

The beginning of my gradual allergic reaction with little baby rashy bumps:

"Full blown, it's bad, time to go to the E.R." allergic reaction:

At the E.R. they made me do an x-ray, blood work, and pee in a cup. They pumped Benedryl, Steroids, and Pepcid through an I.V. The rash was so severe that it would not fully go away. When my I.V. vein blew, they decided to give me a shot of Benedryl in my arm. The medication going through the I.V. helped to relieve the feeling that I was rolling around in an ant pile, but the rash was still there. I came in specifically wanting to only treat my rash, and the doctors in the E.R. wanted to admit me into the hospital to start intravenous antibiotics immediately. Their reasoning was that I sounded congested in my chest. I couldn't believe what I was hearing. A CF patient with congestion? You don't say! I refused treatment of intravenous antibiotics and I just wanted to go home. I had spent 3 months on intravenous antibiotics by that time, and there was simply no way that I was going to start a whole new cycle of I.V. antibiotics. I felt as though I needed to consult with my Cystic Fibrosis doctor in Palm Beach before I signed up for any more surgeries or procedures. I was overcooked and DONE!

Exhausted at the E.R.:

Two days later, a nurse from my old doctor's office in Orlando called, wanting to inform me of my blood and urine results that were taken just recently in the E.R. My blood and urine is always normal so I was quite concerned why she felt the need to call me. She informed me that I had Staph in my blood and urine, and that I needed to come to the hospital immediately to be treated with Cubicin intravenously for 2 weeks. At that moment, I shut down. Staph in the blood can be fatal. Thank goodness for Hunter and my mom because I was completely frozen and numb. I needed to shut off my brain and they immediately took the role of investigators and problem solvers. I needed time to recharge and regain my composure.

My mom called my Cystic Fibrosis doctor in Palm Beach to inform him of what had recently occurred and to get him in the loop. He requested that I visit my local Quest Diagnostics lab as soon as possible to get blood work and a urine test done to confirm the Orlando hospital's findings. He believed that those results might have been false-positives because I showed no actual symptoms of a Staph infection. Still, we needed to be certain. Ultimately, the results showed that no Staph was present in my blood or urine. I was extremely relieved! How could someone make that error?

At the end of December, I started to feel congested again and I ran a fever on New Year's of 2012. The last couple of New Years I've been sick, so I knew that it was going to be a television and cuddle type of night with the love of my life. I am so lucky to have Hunter as my fiance. He is the best thing that has ever happened to me. We have been through everything together and he truly is my sanctuary.

In the midst of everything, I noticed that my port incision was not quite healing properly. When the port was put in, the incision looked beautiful. However, when the port was taken out the incision healing process was not looking so good.

Dissolvable stitches coming out of the incision:

There was no way I was going back to the hospital that removed my port, so I made the decision to go to Winter Park, in hopes that their radiology team could better treat my wound. I thought that it might be gauze stuck in the wound, peaking out of the incision. The radiology team there knows me well, and paged my favorite surgeon, Dr. Mancilla, to look at it. I was so relieved that he was there because he normally travels from hospital to hospital. Thank goodness! I needed luck on my side, and I definitely took it as a sign that other good things were coming my way. He explained that the white part that I was seeing was one of the stitches surfacing. I did not understand why that would happen because I thought dissolvable stitches were used. When I first had my port placed, the stitches dissolved on their own, and I wondered why the stitches used for the port removal procedure had not dissolved. Dr. Mancilla explained that the resident that had performed my port removal surgery made the stitches too superficial and they ended up being pushed out by the body. He removed the stitches and made the incision beautiful and smooth. My path has been shaky, cracked, and rough, but like my scar, I want things to be smoother for me.

My smooth scar after Dr. Mancilla pulled the stitches out:

I've now been battling a lung infection since New Year's 2012. I'm on oral Bactrim and inhaled Tobramycin, and I am slowly trying to find something solid to stand on again. I don't want more intravenous antibiotics or hospitalizations any time soon. My body needs to realize that it can be strong on its own, like it once was. Without the use of toxic antibiotics streaming through my veins, I need it to be strong like the ground where I once stood so firmly.

"Fall seven times and stand up eight." ~Japanese Proverb

Thankful

"Be thankful for what you have; you'll end up having more. If you concentrate on what you don't have, you will never, ever have enough." ~Oprah Winfrey

Monday, November 14th, I was released from the hospital. My FEV1 was sitting stable at 48% and I was grateful to see those results after my body had been turned upside down for months. I got the go-ahead from the doctors to leave and finish the rest of the intravenous antibiotics at home. Hunter rescued me from the hospital; he picked me up and I was relieved to be going home. As I looked into his warm, loving eyes, his hand in mine was all I needed during the 3-hour car ride home.

My last day of intravenous antibiotics was November 18th. I pulled the 20-gauge X 3/4 inch needle out of my port and I was a free woman, at last! Typically, I would use a 20 gauge X 1 inch port needle, but the hospital had recently used a shorter needle on me, and it worked perfectly. I will have to access my port every 30 days when not being used, in order to maintain and keep the port functional. I'm going to have to take a needle and push it into my chest, and I'm hoping for a smooth experience. It's amazing what a person can endure when the future is in full focus.

I have learned that life moves in a circle. There are moments of greatness and moments that make you feel rough around the edges. The highs and lows have taught me so much about life, and I've learned to really appreciate every second. I was thankful to have an amazing Thanksgiving with my mom, Hunter and his family. I was basking in the day, thankful for those little moments of greatness.

My honey and I on Thanksgiving Day

Delicious! I made a spaghetti squash and zucchini creation on the bottom right!

Excitement

"Get excited and enthusiastic about your own dream. This excitement is like a forest fire - you can smell it, taste it, and see it from a mile away." ~Denis Waitley

It's the final countdown...do do do do...do do do do doooo....

I'm excited! I have a Pulmonary Function Test (PFT) tomorrow morning. I have been persistent, diligent, determined, and working hard. My regimen in the hospital has included around the clock nebulizers, chest physiotherapy, intravenous antibiotics, as well as 5 hours of sleep every night (due to my vigorous respiratory schedule). Rarely, do I have any time to rest during my stay at the hospital and I am looking forward to peaceful sleep without any disturbances when I get home. I have been experiencing Respiratory Boot Camp here at the hospital and no boogers will get out alive! ;)

In September when I started I.V. therapy my FEV1 was 57%. While I was on intravenous antibiotics my FEV1 fluctuated down to 45%. Then, to 29% FEV1 (lung transplant list status) because I was not getting the proper treatment in Orlando. Now, that I have been seeing the doctor here at the Miami hospital, I'm feeling amazing! On November 4th, my intravenous antibiotics were changed to Zosyn (3.375 gram) every 6 hours and Tobramycin (470 mg) every 24 hours. This final antibiotic duo ended up doing the trick! I am so happy! I feel like grabbing all the nurses and jumping up and down with them in excitement! I feel like skipping down the halls. To be able to breathe without the feeling of an elephant sitting on my chest is blissful! I am so grateful for how I am feeling at this moment. My doctor informed me that the game plan for tomorrow is PFT's, a lung x-ray, and if all looks great, then home sweet home! I am excited to see what tomorrow brings! Wish me luck! Yayyyyy!

In the video below I'm showing off my new handy dandy vest wrap and getting prepared for tomorrow! :)

Hanging On

"When you reach the end of your rope, tie a knot in it and hang on." ~Thomas Jefferson

A lot has happened since my last post, and I have no idea where to start. It's as though I was a hostage in the months of September and October, and I've just wanted to rewind time and go back to August. Life isn't like that though, there is no going back...just forward.

In September, when my intravenous antibiotics were switched to Merropenem and Colistin, I started to feel different. My O2 had dropped from a 98% to 93% pulse oxygen rate since the bronchoscopy and port was placed, but I had thought it would be temporary and that it would come back if I just gave my body some time to fight infection and recover. As days went by, I was experiencing high tachycardia (increased heart rate) and my O2 started dropping to the 80's on room air. I wasn't able to breath on my own without the help of oxygen. My doctor ordered a CT scan of my lungs with contrast. When the contrast was pumped into my heart, I had my eyes closed. I saw an orange flash and I felt a warm sensation in my chest. My O2 dropped to 70 and I was rushed to the E.R. I was so glad to have my friend Paula there because this was not the norm for me and I was scared.

I knew that the reason why my oxygen dropped to 70% was from the reaction to the contrast. But what was causing my O2 to be in the 80's before that was the big question. A part of me wanted to put all the blame on the port because of the fact that it was a new, unfamiliar, foreign object in my body. I wondered if my body was rejecting it and if my sudden symptoms could easily be fixed by taking the thing out. Then, I wondered if it could be a reaction to the toxic intravenous medications that were being pumped into my heart. And my final guess was simply Cystic Fibrosis, the culprit that I fight so hard every day to just keep jailed up.

I know that when living with Cystic Fibrosis, life can change in an instant. I'm not oblivious to what the disease can do. I have heard stories of those who have lost their battle with Cystic Fibrosis. The cause of death in a Cystic Fibrosis patient is usually respiratory tract infections, respiratory distress, transplant rejection, or complications from Cystic Fibrosis. Though, I want my story to be different - I want to somehow change my story. That is why I fight so hard everyday to defeat Cystic Fibrosis, the disease that tries to rob me, hold me back, and steal everything away.



Dr. Layish (my Orlando doctor) did not have any answers. After 3 weeks of intravenous antibiotics, the Merrepenem seemed like the culprit and I was taken off of the antibiotics. My O2 was sitting stable at 92% in the hospital, so I got the go-ahead to be released. I was off intravenous antibiotics for a week to try to see if that would make everything better. I know it might sound crazy, but I decided to go to New York with my mom for four days. It was a mother/daughter vacation that we had always wanted to go on. I did not want my Cystic Fibrosis to steal that away from me, and I thought to myself, as they say on broadway: the show must go on! It was an amazing four days with my mom. I made sure to have oxygen there when I arrived so that if my symptoms were to occur again, I would have backup. And I made sure to sleep with the oxygen at night as well. On the airplane ride back, my lips and fingernails suddenly turned blue, which had never happened to me before. The stewardess grabbed a tank of oxygen to help me breathe. I was scared. My body was giving me clues that something was wrong.

My oxygen was constantly fluctuating. One moment I would be fine at 92%, and the next, I would be needing oxygen to breathe. My doctor ordered a bag pack and small oxygen tanks for me to carry with me during the day. He did not seem to have any answers. In fact, he actually asked ME what I wanted HIM to do. I was in shock! Isn't he supposed to be the doctor? Dr. Layish always sends me to an infectious disease doctor when it is time for I.V.'s, and even though I am not contagious, he feels that she is the best person to decide which intravenous medications should be used to treat my infections. Usually, a good Cystic Fibrosis doctor knows everything there is to know about Cystic Fibrosis, especially when it comes to treating infections with intravenous antibiotics. This goes to show that he truly doesn't have a clue. Dr. Curbelo, my infectious disease doctor, decided more intravenous antibiotics were necessary, and that the cause of all this commotion was not the port, but rather my Cystic Fibrosis. I started back on intravenous antibiotics on October 11th.

Colistin (every 12 hrs) and Cefepime (every 8 hrs)...only to find out one week later that I wasn't getting any better, due to the fact that I was resistant to the Cefepime.
My antibiotics were then changed to Colistin (every 12 hours) and Azactam (every 8 hrs), on October 17th, and I have remained on these drugs since then.

I have finally decided that it is time for a change. Enough is enough. Time for a new CF doctor. My best friend, Christina, is amazing! She drove me down to Palm Beach to help me find answers. She was my old college roommate and has been through it all with me. I have heard many great things about Dr. Salathe, and I decided after we met that I wanted him to be my new doctor. When I lived in Miami last year, Dr. Light was my Cystic Fibrosis doctor. When I told him that I was moving back to Orlando, he informed me that he was retiring and that I could always drive to Palm Beach to see Dr. Salathe, but I felt that the drive was just too far. For the sake of convenience, I told myself that my old Orlando doctor would do fine, but who am I kidding? Cystic Fibrosis doctors in Miami are simply the best in Florida. And if I want to get the best treatment, I need to make the 3-hour drive for it. I think it will be worth it. Dr. Salathe wants to start from scratch and reevaluate my overall condition. Tomorrow morning, Hunter is taking me to the hospital in Miami. Dr. Salathe's game plan for me includes vigorous chest physiotherapy, prolonged intravenous antibiotics, around the clock inhaled medications, and generally kicking some CF butt!

I'm aware that I have holes in my lungs from the disease, and mucus galore blocking my airways, but it's like I said - I want my story to be different. I want a doctor that will also be my cheerleader. I don't want to be a statistic. I want someone that will fight as hard as me to keep me going. At times, I think my mind has gotten me this far. The constant push to survive; the will to beat the odds. September and October might have been stolen from me, but I want to enjoy my November and, more importantly, my 27th birthday in December.

Beauty

“I don’t like standard beauty – there is no beauty without strangeness.” ~Karl Lagerfeld

As a makeup artist, I've learned that we are our biggest critics when it comes to beauty, but, in my opinion anyway, true beauty can be found in a person's attitude. Scars and wrinkles should not be viewed as flaws, but rather as beauty marks or stories that make each person unique. Each marking on my body illustrates a different chapter of my life. I would not be where I am today without my scars, freckles, "lines of intelligence" (as Bobbi Brown would say), or anything else you may find on my body. My port is my newest beauty mark to add to the collection, and it is healing nicely.

My beautiful booboo is healing. :)