"X" Marks The Spot

“Not all treasure is silver and gold, mate.” ~ Captain Jack Sparrow

"X" Marks The Spot

As an 80’s baby, I grew up with movies like “The Goonies” and “Hook.” If you’ve ever seen either of those movies, then the word, “pirates,” may have just come to mind. During my childhood, they were a recurring theme at birthday parties, and even in cartoons. Whimsical children’s stories about pirates oftentimes depicted them differently from one another – sometimes they were good, and sometimes evil – but the one thing they all had in common was their obsession with that big “X,” scrawled onto the tarnished paper of an old treasure map. Their journey to unearth that mythical treasure was usually dangerous, but the pirates believed the promise of reward was always worth the risk, so they forged onward and dug until they struck gold.

Like many children, I embodied the spirit of curiosity when I was younger – a curious, little pirate, as it were. While I was well aware, from a very young age, that I had Cystic Fibrosis, I was determined to not let the disease commandeer my ship. I recall sitting in the booth of a restaurant with my mom, eagerly anticipating the arrival of our food. Normally, I would wait for her to mix my pills (digestive enzymes) into my applesauce before eating a full meal. At the ripe age of three, however, I was ready to take the helm. I grabbed three of my enzyme capsules and swallowed them whole, much to my mom’s surprise. This was a turning point in my life - I was determined to be more self-sufficient, ready to take on anything, and forge onward with the wind in my sails. I wanted to live as freely as any other kid my age, with all of life’s adventures ahead of me, and the world at my fingertips. In order to achieve this goal, however, I would need to arm myself with the knowledge necessary to be hands-on in my own care. My mom was a fierce ally in my endeavor, as was my pediatric pulmonologist, who was a brilliant doctor. He always took the time to answer questions and ensure that my mom and I were as educated as possible about the disease, and that’s no simple task when dealing with a disease as complex as Cystic Fibrosis. Despite that, he firmly believed there would be a cure in my lifetime.

The symptoms and severity of Cystic Fibrosis can vary greatly depending on the combination of CF mutations one has, and there are hundreds of different mutations. I wanted to learn more about my Cystic Fibrosis mutations – to dig a little deeper. I was told by my doctors that I have a Delta-F508 mutation, which is rather common, as well as a W1282X mutation, which is surprisingly rare. The “X” in “W1282X” immediately grabbed my attention. “X marks the spot,” as they say. Would I one day be as fortunate as the sea-faring pirates from my childhood? Would scientists be able to find and unearth the defect in my cellular makeup? Would we be rewarded with a cure?

Ever since scientists first located the defective gene that causes Cystic Fibrosis, it’s felt like we’ve had the map that leads to that cure – the ultimate treasure trove. While it has yet to be found, other medical discoveries have been made which help correct certain combinations of CF mutations – medications like Kalydeco, Orkambi, and Symdeko are among these great discoveries - all of which were funded by people who support the Cystic Fibrosis Foundation. Many CF patients are now reaping the benefits of these medical breakthroughs, and I'm truly overjoyed for them! However, I'm currently shipwrecked on a smaller island with other CF patients who are still waiting for their CF mutations to be corrected, eagerly waiting to be saved. As you might suspect, it's been extra salty on this little island.

I was recently fortunate enough to participate in a clinical trial that was a game changer for me. The trial was a double-blind study, so I didn’t know if I had the real trial drug or a placebo. However, the feeling was uncanny. After a week of treatment, I felt like Super Mario after getting a hold of one of those blinking stars. My lung function increased, and I had more energy than I knew what to do with. I never wanted that feeling to leave. It reminded me of a time when I was just a teenager. Lying in bed one night, I took a deep, full breath of air, and told myself to remember what that felt like. I knew that someday, when the disease had progressed far enough, my lungs would forget the sensation. For a short time, however, I was able to unlock that magical feeling once more, thanks to the clinical trial.

Though the trial has since ended, more testing has to be done before a treatment for my W1282X mutation comes to market. In the meantime, the “X” has now been marked on my genetic map. "Not all treasure is silver and gold," as Captain Jack Sparrow famously said, and this quest has taught me that there is treasure in knowing that the disease has made me stronger, more patient, more compassionate, and more understanding. I do not take any breath for granted. Where there once was only ruin, there is now hope for treasure.

Seeker Of Truth

“I have been a seeker and still am, but I stopped asking the books and the stars. I started listening to the teaching of my soul.” ~Rumi

Never stop asking questions. Optimistically thinking, I never want to say that the intravenous antibiotics are not working. I’ve been waiting patiently for that moment where I wake up and feel better, but it just hasn’t happened yet. I’ve always had my own theory when it comes to superbugs in the lungs, that there is more than what meets the eye. And I’ve often used an analogy to explain my theory. You see, if you were to take a narrow flower pot, fill it with dirt, a little bit of water, and place the pot in a warm, dark area, what wouldn’t grow in the flower pot? I’ve always believed that CF patients grow more than what shows in the Petri dish. I stopped believing in Petri dishes a long time ago. The pseudomonas that is so prevalent in our lungs, forms a biofilm-like blanket that hinders the ability to see anything else that may be growing underneath all the mess. Scientists have started to dissect the lungs of patients with CF, after they have passed away, and are finding that they grew fungus, mold, staph, and other microorganisms that did not show in the Petri dish, which confirms my belief. The petri dish is little more than a GPS, in the grand scheme of things. It’s disheartening to know that these petri dishes can be deceiving - that there is more to find than what meets the eye. Especially, when these bugs cause irreversible lung damage and death. If only I could put a tent over my lungs and fumigate my bugs to rid the infectious chaos. I do hours and hours of chest physiotherapy and breathing treatments. However, if the infection still exists, the bugs will only grow bigger and meaner, which will cause more damage over time which will result in lung failure. If only we could get to the ROOT of the problem.

After being on intravenous Colistin and Minocycline every 12 hours as well as Cefepime every 8 hours for over a month, and not feeling better, I needed to know the new game plan. My doctors nurse explained that she was calling in to the pharmacy oral Minocycline and intravenous Ceftazidime. My gut feeling felt as though that wasn’t going to be enough to help me feel better. I knew I needed to be my own advocate and speak up. I didn’t want to take a step backwards and my gut feeling was that I needed to be on three antibiotics to kick this thing to the curb. Thank goodness I spoke up because when my doctor called me she pulled up my recent sputum culture and found the reason I wasn’t feeling better. I was growing a bug that did not show in the Petri dish before. I did in fact need to be on three different antibiotics to help to irradiate this bug. Ah-ha! This could be the needle that has been spoiling the haystack! I’m excited to start intravenous Zithromycin, Rifampin, and Ceftazidime. Plus, Ethambutol, an oral antibiotic for a tag team combo that could get me up and running again. Feeling hopeful and optimistic as always!

Always trust your intuition. Keep searching, keep asking questions, keep thinking and seeking your own truth. The doors are opening..and you will never be given more than you can handle. I decided years ago, that I have three choices in life. Give up, give in, or give it all I’ve got. I refuse to accept the reality I was given, I much rather create my own.  

Remember To Be A Coffee Bean

“Life is filled with unanswered questions, but it is the courage to seek those answers that continues to give meaning to life. You can spend your life wallowing in despair, wondering why you were the one who was led towards the road strewn with pain, or you can be grateful that you are strong enough to survive it.” ~J.D. Stroube 

 

The film on the left is my lung x-ray from a few years ago and the film on the right is my lung x-ray from August 2014.

 

X-ray taken 2 weeks ago after being on 4 weeks of intravenous antibiotics. Took an x-ray at Dr. Layish's office to see my progress which is shown below:

 

It has been 6 weeks of intravenous antibiotics, oral medications, and my usual CF bootcamp regimen. I have been fighting a bunch of really bad and deadly infections in my lungs.

The I.V. game plan:
Week 1,2,3: Aztreanam 2g q 8 and Ceftazadime 2g q 8, but I wasn't feeling any better. I still felt tight, like my airways were being squeezed.

My doctor in Palm Beach was very busy and did not have time to call me, he had been in meetings, out of town, and every patient that he had at this time was sick. I was told by his nurse, Lisa, that he was having a bad day. All I wanted was 20 minutes of his time, and he couldn't give it to me. The crazy thing is he has never been like this, he is always able to talk, always available. It was really upsetting because I needed my doctor more than anything at that time. I asked to look at my sensitivity and lab report. And was wondering why we weren't treating the stenotroph bug that I was growing in my lungs. His belief was that it was my pseudo bugs causing the ruckus in my lungs. I, on the other hand, know my body and did not think that the pseudo bugs were the culprit. My gut feeling was that the stenotroph bug or a fungal bug might have gotten into my lungs and was causing some damage. However, no one would listen.

Week 4: Zosyn 3.375 mg q 6 was ordered and I'm allergic to penicillin, and I felt that it only made things worse. I couldn't breathe, my lungs felt so tight as though someone put a plastic bag over my head. Dr. Salathe was out of town for a couple of days. His nurse tried to get me a hospital bed, so that I could be admitted and treated at UM, but they were full with no beds available. I was packed, ready to go.... and then, I was told to go to the ER because there were no beds and wait there. The ER and I do not get along, I don't want any other bugs, infection control is key to survival, and the ER is the least clean place to be. Even if my eyeball was falling out, I would tell Hunter to put a Band-Aid on it.

Week 5 and 6: I needed help and I needed help NOW! I made an emergency visit to my old doctor, Dr. Layish, who was 20 min away compared to a 3 hour drive to see Dr. Salathe. I started taking Septra 320 mg q 8 and steroids. Plus, a new addition, Voriconazole 200 mg tablets BID which is a first. Dr. Layish was wonderful. We did a CT scan of my lungs, sputum test, x-ray, and he even spent extra time going over everything with me because he knows that I want to learn as much as possible about this disease in order to win. I was so fortunate that he was able to listen to me... really listen to me. I am highly allergic to mold and fungus, it grows rapid in the summer time here in Florida. I wanted him to test for ABPA. Luckily for me, he trusts that I know what I'm talking about and ordered it. Dr. Curbelo, the infectious disease doctor, that he partners with took a chance and ordered antibiotics to treat ABPA. And it's working!!! :)
For me, living with CF is like a constant board game or an episode of House, you never know what might happen, but you always want to figure it out or win! Luckily, the game plan is working and I can finally hear air, rather than crackles when I breathe! Septra, Voriconazole, and the steroids are taking the bad guys out! :) Hopefully, the PICC line will be pulled out on Thursday during my follow up with Dr. Layish and I'll be free. In life, I don't want to just exist, I want to truly live. I have things to do and people to see!

Side note: Whenever I go through challenges, or hiccups as I like to call them, I always look at Carrots, Eggs, and Coffee. The story reminds me to try to be a coffee bean no matter what. Read below.

Carrots, Eggs, and Coffee

A carrot, an egg, and a cup of coffee.....You will never look at a cup of coffee the same way again.
A young woman went to her mother and told her about her life and how things were so hard for her. She did not know how she was going to make it and wanted to give up, She was tired of fighting and struggling. It seemed as one problem was solved, a new one arose.
Her mother took her to the kitchen. She filled three pots with water and placed each on a high fire. Soon the pots came to boil. In the first she placed carrots, in the second she placed eggs, and in the last she placed ground coffee beans. She let them sit and boil; without saying a word.

In about twenty minutes she turned off the burners. She fished the carrots out and placed them in a bowl. She pulled the eggs out and placed them in a bowl. Then she ladled the coffee out and placed it in a bowl. Turning to her daughter, she asked, ' Tell me what you see.'

'Carrots, eggs, and coffee,' she replied.

Her mother brought her closer and asked her to feel the carrots. She did and noted that they were soft. The mother then asked the daughter to take an egg and break it. After pulling off the shell, she observed the hard boiled egg.
Finally, the mother asked the daughter to sip the coffee.
The daughter smiled as she tasted its rich aroma.
The daughter then asked, 'What does it mean, mother?'

Her mother explained that each of these objects had faced the same adversity: boiling water. Each reacted differently.
The carrot went in strong, hard, and unrelenting. However, after being subjected to the boiling water, it softened and became weak.
The egg had been fragile. Its thin outer shell had protected its liquid interior, but after sitting through the boiling water, its inside became hardened.
The ground coffee beans were unique, however. After they were in the boiling water, they had changed the water..
'Which are you?' she asked her daughter. 'When adversity knocks on your door, how do you respond? Are you a carrot, an egg or a coffee bean?

Think of this: Which am I? Am I the carrot that seems strong, but with pain and adversity do I wilt and become soft and lose my strength?
Am I the egg that starts with a malleable heart, but changes with the heat? Did I have a fluid spirit, but after a death, a breakup, a financial hardship or some other trial, have I become hardened and stiff? Does my shell look the same, but on the inside am I bitter and tough with a stiff spirit and hardened heart?
Or am I like the coffee bean? The bean actually changes the hot water, the very circumstance that brings the pain. When the water gets hot, it releases the fragrance and flavor. If you are like the bean, when things are at their worst, you get better and change the situation around you.
When the hour is the darkest and trials are their greatest do you elevate yourself to another level? How do you handle adversity? Are you a carrot, an egg or a coffee bean?

May you have enough happiness to make you sweet, enough trials to make you strong, enough sorrow to keep you human and enough hope to make you happy.
The happiest of people don't necessarily have the best of everything; they just make the most of everything that comes along their way. The brightest future will always be based on a forgotten past; you can't go forward in life until you let go of your past failures and heartaches.

When you were born, you were crying and everyone around you was smiling.

Live your life so at the end, you're the one who is smiling and everyone around you is crying.
You may want to share this message with those who mean something to you; to those who have touched your life in one way or another; to those who make you smile when you really need it; to those who make you see the brighter side of things when you are really down; to those whose friendship you appreciate; to those who are so meaningful in your life.

May we all be COFFEE!!!!!!

Kickin' Grass

"Learn a lesson from your dog- no matter what life brings you, kick some grass over that shit and move on." ~ Unknown

Imagine that you have an infection, and the only oral medications on the market that can help you fight off that infection are Levofloxacin and Ciprofloxacin. Now, imagine being told that those medications will not work for you. How do you feel?

That is what my doctor's nurse told me on the phone today. She mentioned that the next time I get hit with a Cystic Fibrosis lung infection, the only thing that could help me are a combination of several intravenous medications. Not what I wanted to hear while this cold of mine is working hard to spread to my lungs.

I've had many peripheral lines put in my arm, a port, and hospitalizations; it doesn't get any easier over time. I never want to be the "sick girl." I feel that I'm capable of much more than a title like that implies. A feeling that "I'm bigger than my body gives me credit for."

Unfortunately, I'm not feeling my best today. My body aches, I feel hot/cold/feverish, and I can't stop coughing. All the coughing is causing me to throw up frequently, which is great fun. At this time, all I keep thinking is that it's not time for my body to just surrender - to go on IV antibiotics, or spend time in the hospital.

Cystic Fibrosis is like living with the flu every day. What did you feel like the last time you had a really bad flu? That is my everyday. After living with me for several years, my old college roommate and best friend, Christina, used that flu analogy after seeing what my everyday health regimen truly looked like. I've often used that analogy when speaking publicly about Cystic Fibrosis. The bare definition of the disease is sometimes insufficient to explain what living with the disease is like, and just how aggressive CF is in its quest to slowly destroy the body from the inside. Constant congestion, coughing, tissues, fevers, throwing up, digestive issues, body aches, and the inability to breathe clearly are only the basic symptoms.

I have so much that I want to do in the coming months, and I don't want to sit on the sidelines. I have the Great Strides walk that I so desperately want to attend, a public speaking engagement to spread awareness about CF, yoga classes to enjoy, makeup lessons that I want to teach, getting together with friends, and so much more. They sound simple enough, these little moments that I don't want CF to take away from me. So, it's hard to hear when a nurse tells you that no oral antibiotics will help you. There are no easy answers to Cystic Fibrosis - I get it. I'm trying my hardest to fight this with the limited resources I have. I am surrounded by lots of water, peppermint tea, vitamins, tissues, enzymes, and my daily medications, all while attempting hardcore airway clearance. Wishing the mucus waterfall would end. Just waiting for that magic pill.

Rooted, Strong, and Growing

"Meditate. Live purely. Be quiet. Do your work with mastery. Like the moon, come out from behind the clouds! Shine." ~Buddha

I have become very open minded over the years when it comes to my health while trying to combat Cystic Fibrosis. I tend to mix a little of Western medicine, Eastern medicine, and holistic treatments to my daily health regimen in hopes for the best health outcome possible.

During my yoga practice, I've learned about Chakras being centers of energy which are located on the midline of the body. Within the body exist a series of minor and major energy centers called chakras. The word chakra is Sanskrit for "wheel" or "disk". Each of the seven major chakras has its own distinct character and relates to a unique aspect of our being. The chakras correlate to levels of consciousness, body functions, colors, elements, sounds, and much more. The blockage or energetic dysfunction in the chakras is believed to give rise to physiological, psychological, emotional, and spiritual disorders. During my yoga practice, I try to align my chakras and balance my body through different yoga poses.

While this may sound a little outside of the box, I'm willing to try anything to improve my health and strengthen my lungs in order to help me breathe easier. With the knowledge of chakra colors on the brain this morning, I picked out a bright red shirt to wear at my doctors appointment. Red symbolizes the Root Chaka. I knew I wanted to be grounded while being present in the here and now. I wanted to feel confident when doing my lung function test, to know  that I had the ability to succeed. I was hoping that the red would boost my energy center which, in turn, would help me have better lung function results.

I love my lungs and was thankful for my 55% FEV1 lung function results today. I am aware that normal is 100%, but I am grateful that my lungs are still working and are still remaining unstoppable. At my last doctor's appointment, I was at 52% FEV1 lung function and I was elated to hear that my lung function had improved since my last visit. I believe that yoga, pole fitness, and weight training each day is paying off!

I choose to be unstoppable. I am bigger than my concerns and worries. The strength of others inspires me daily. I will continue to focus on my goals because I believe I am capable of much more! As I continue to breathe, I continue to hope.

 

  

Lotus Flower

"Start by doing what’s necessary; then do what’s possible; and suddenly you are doing the impossible." ~Saint Francis of Assisi

I've learned that lotus flowers are symbolic, inspiring people to continue striving through difficulties and to show their best part to the outside world, no matter how bad the circumstances may be. The lotus flower grows from the murky darkness at the bottom of the pond, emerging pure and clean. It is said to symbolize beauty and light. The plant's stalk is easy to bend in two, but it is very hard to break. Resilient in its own unique way. When I think of the lotus flower, I imagine perseverance. The lotus flower reminds me that life is fragile, everyone has their own obstacles in life and can find light even after the darkness has faded. I believe that it is important to always remember that.

We are continuously driven to edit and revise our daily lives. We make sure to prepare for the day, and revise as we see fit, each day leading to new growth and development. I woke up craving some positive juju, so I've decided to take an Aroma Yoga class today, I know it will be just what my body needs.

Pump You Up!

"Life is not a dress rehearsal. Stop practicing what you're going to do and just go do it. In one bold stroke you can transform today." ~Marilyn Grey

At my last doctors appointment, my FEV1 was at 52%. My doctor had suggested that if I increase my BMI, it could help increase my lung function. I have to admit, I've lost a lot of muscle and I'm often fatigued by the lack of oxygen going through my bloodstream. However, if I'm going to succeed, I have to push myself. Arnold Schwarzenegger's got nothing on me!

I'm proud to say that I've been working out 4 days a week since January. My exercise schedule has consisted of weight training, pole dancing, and yoga. Odd combination for sure, but I'm enjoying the diversity during the week! Yoga has continued to be my favorite form of exercise. As cheesy as it sounds, when I'm on my green yoga mat, the world just melts away and any stress that I may have in the day just disappears. Yoga is often portrayed as a Hindu spiritual and ascetic discipline, a part of which, including breath control, simple meditation, and the adoption of specific bodily postures, is widely practiced for health and relaxation. Exercise is the focal point in my daily regimen, I am determined to strengthen my lungs and body, improve my breathing capacity, achieve a sense of calm, a state of relaxation - overall, to enhance my quality of life. I believe that yoga combined with other forms of exercise will help me accomplish just that.

To increase my muscle mass, I've gone back to the basics, like in Child's Pose during a Yoga class. This is symbolic in a way because like a child, I have to start from scratch, break down my muscle, and watch it grow slowly over time.  Even though I'm not seeing results yet, I feel stronger.  

Child's Pose: Start on your hands and knees. Sit your tailbone back towards your heels, coming to sit on your calves. Bend your upper body forward, resting your chest on your thighs, your forehead on the floor, and your arms outstretched forward. Child's Pose is in fact a restorative yoga position that induces deep relaxation and even stimulates the pituitary gland. It's true. As soon as my forehead hits the mat, I feel centered, and experience a sense of peace. It's no wonder why a child resorts to this position after throwing a temper tantrum.

According to Sri Dharma Mittra, there are at least 1,300 total variations of yoga positions. Only 1,299 more positions to master as I build my breath and strength.