Weather the Storm with Joy

It has been a minute since I last blogged. Truth is, I’ve meant to. Truth also is, I’ve started three different ones yet cannot bring myself to finish them. Call it writers block or what you may, I just know I’ve struggled to set my words and feelings in stone for what is on my heart. As we begin each day, read the news, witness what is happening right now, it can be exhausting. We are all feeling it navigating this new “normal” way of life. Trying to keep our minds and bodies preoccupied while continuing to simply… exist. You may feel like the human connection is fading with every sunrise and sunset. I honestly feel we are growing closer together. We are living life in “social / physical distancing” and we have to. I hope everyone is doing their part and staying home as much as possible. Stay healthy. Stay at home. Please do your part in helping to flatten the curve. Never in my darkest of nightmares did I imagine a worldwide pandemic take place during my lifetime. I cannot wait to be able to look back at these days and breathe a sigh of relief for it’s ending.

For myself and others living with Cystic Fibrosis, or any chronic illness/condition, this isn’t uncharted territory. We are more than familiar with the terms “6 feet apart,” staying away from people when we are sick, when others are sick and distancing ourselves from social gatherings. We’ve been doing this most of our lives. We are experts in prevention. We know what being isolated and practicing physical distancing feels like. Imagine spending weeks in a hospital just to get your lungs back to “baseline,” or weeks to even months at home on IV antibiotics avoiding the public as much as possible. We understand it. We are in a way, more prepared for what is happening. Most of the world is being awakened to this isolation. Believe me, we are grieving just as much as everyone else. We miss our “normal” lives, seeing family and friends, giving hugs, all the human to human interactions and touch. It is worrisome.

Crazily, it has been a little over 5 months since I started Trikafta. I continue to feel good. (I better knock on wood and blow a kiss to the heavens). My lungs feel stronger, along with my voice. There is still an overall silence that accompanies me. My mornings remain effortlessly unreal. I have a new determination: to maintain half marathon distance / fitness level. This goal was one I was able to do in 2012-2013, but my lung function was higher than it currently is. My strength is found in setting this type of goal and to work hard to achieve it (until the rest of my body physically dictates different). Whereas in the last 4 years or so, it would have been much more difficult. It is also my way to escape CF, to escape everything, all while feeding my health and wellness. So, if you hear about me still running….you bet your life I am.

Not only am I experiencing all the emotions with being on Trikafta, my thoughts are all over the place given this bizarre and twilight zone haze we are all currently living in. However, I am also experiencing a heightened sense of unfamiliar comfort. I am honestly holding onto this with all my might. When I stop and think about timelines, it is incredibly ironic to me how this life altering genetic modulator was FDA approved 6 months earlier than anticipated. And now, we have this relentless virus COVID-19. If I wasn’t on Trikafta, my fears for my health and my anxieties would be on a higher level in the midst of this storm. The question “…how is this fair for me to feel this way?” consumes my mind as well. I can’t allow that question to control my every thought, but it creeps in. I feel so fortunate. I am beyond grateful. I am constantly clinging to the hope for all of us living with CF to have access to a genetic modulator. Sooner rather than later.

Despite this safety net contentment (if you will) of Trikafta, sometimes I wonder… when is this going to end? When will the jinx come? If I were to catch COVID-19 would I be prepared and have the strength to battle it? I don’t know. I recall days where I have fought and conquered numerous infections, even prayed for my life to be taken as I couldn’t handle the lung pain anymore…and yet, I am still here. Asking those ‘what if’ questions only brings me into the pit of despair. It gets me absolutely nowhere. I must focus on how far I have come, how far I’ve yet to go, and look at where I am presently. “You haven’t come this far to only come this far” echoes in my heart. Remaining in the present has never been more important to me as it is these days. Each day feels the same, yet they are not. Days run into each other, time even feels like a strange culmination of colors blending together on this chaotic canvas before me. Just focus on the here and now I tell myself. I eagerly look forward to seeing the “greener grass” when this is all over. It has to be there. When it reveals itself, I’m going to sprint to it…feel it between my toes as I glide across it with each step. With open arms I will run to give my parents the biggest hugs, to all my family, my friends and possibly never, ever let go.

As we live and breathe, try to remember to find joy in anything you can. Things that help me by providing peace and keeping my sanity in check: my forever therapy in running, (physically distancing myself safely) riding my bike on the trainer (for now), walking my dogs, eating all the foods, trying new recipes, cleaning/organizing, enjoying my first cup of coffee in the morning on the back patio, listening to the birds sing their melodies, buying yarn to finally learn how to crochet, a not-so new yet new and exciting journey in singing, (so much singing!!) and video chatting and/or phone calls with friends/family as often as possible. It may sound corny, but those who know me well know I strive to seek joy and all the goodness it brings to my heart.

In the middle of this pandemic, I encourage you to do the same. The other day, my joy was found in my dog being the biggest goof rolling around on the bed with her tongue sticking out. Another day it was a video of my dear friend’s 10 month old baby boy giggling. Yesterday, the joy revealed itself in a beautiful double rainbow after a magnificent thunderstorm. I do believe joy is always there, we just have to seek and find it. I am trying. We have to believe greener grass exists again on the other side of this. We just have to. On the other hand, remember this saying as well “The grass is also greener where you water it.” All my love to each of you now and always. Stay strong. Do get outside (safely) and enjoy the fresh air. Find the beauty in life. Lift each other up. We will get through this.

The Beauty of Silence

Who knew the impact this medication would have on me in not even 3 months? I remember the first dose, the purge of mucus from my lungs and sinuses the first 24 hours. My energy level feeling like I could climb a mountain effortlessly. The issues of wanting to eat everything I see in sight. It resembles that “hangry” feeling when on prednisone. That actually still holds true. I may or may not need to buy new jeans? We are working on that unimaginable adjustment. Being less salty after a workout or run. I miss my perfect line of salt crystals that used to form on my brow! The return capability of singing allegro vivace and not squeak. To feel the vibrato with my sound and with such clarity! I have missed being able to do that. Waking up and singing that one high, particular long note that used to fade in my voice when I gave it all I had. Waking up and not feeling congested! Do you have any idea how amazing that is for me? To dance between exercise reps, during meds, while cooking, while in the shower, in the car, in the yard (sorry neighbors) or with my dogs (they love it). To dance and sing and not feel winded. My laugh! To laugh without following a series of gasping-for-air-wheezy- moments that literally scare everyone who may witness, thinking you might die. Not a bad way to go, really. She laughed herself to death. Sorry, I can be morbid in my humor. Getting off topic. Laughing without coughing, laughing deeper and with more force as my airways finally feel this long awaited relief. Being able to run without coughing makes me cry quite often. I smile through the joyful waterfall that falls onto my path with each step. Sometimes, I just can’t help it.

All the little things, that many take for granted day to day, mean the most in how my life has changed since Trikafta. There is one colossal difference that adds to everything for my life. The sound of silence. I’ve never known how beautiful it can be until now. And that it prevails. All I’ve ever known is to cough. It’s defined me. It’s identified me. It has been the source of how I feel, the hint of possible infection, the definitive sign when getting sick, the overall factor in all my life. Now, I live in a silence. No coughing. At times I still cough, but it is honestly coughing to cough. Coughing to see if anything at all will come up. Doing huff coughs that simply lead to only expelling air out of my lungs. There are times I see color again. Usually after exercise. I get excited now if I see anything come up. For all my life, I have spent infinite hours of time and energy clearing the junk out of my lungs so I can live. Now, there is this silence upon me. The wheezes have also escaped (good riddance!) Allowing me to live and most of all breathe more freely. It is an indescribable, magnificent, most emotional heart explosion feeling I’ve possibly ever experienced. I never imagined this could exist for me.

The silence that now accompanies me is also displaying its beauty during my most recent pulmonary function test. My RT even noticed, from seeing me a month ago to now. By the way my friends, please give your CF care team love. They are left in awe of the things that are happening for us just as much we are. And equally sharing in our excitement. We are so lucky to have them. During my test, which was a complete PFT (pulmonary function) I didn’t cough once. This alone still gets to me. I didn’t experience any wheezing. If any, it was very tiny near the end. I did not feel exhausted. I felt amazing. This test is typically very exhausting, lasting for an hour, leaving you winded, dizzy, sweaty, shaky and ready to call it a day. Pre-Trikafta, I had declined lung function wise. Numbers do not do always reflect how I feel. They are the facts, but they do not define me. I began this medication with realistic expectations. I have not had a drastic increase in my fev1 outcomes, it has been gradual. I have 44 years of damage that cannot be reversed. My hope has been to gain back to being at what is considered my “baseline.” This week, that wonderful to me number came revealed itself again to me. I wanted to cry. I hugged my RT. I’ve gained 13% lung function while claiming my breaths again since starting Trikafta. I’ve finally reached my baseline. I haven’t been able to since last April. I am humbly thankful beyond words. With this, I am filled with a tenacious commitment and dedication to never stop, keep working hard and then work even harder for my lungs.

The next time you realize you are surrounded by silence, stop and soak it in for a moment. Take a deep breath. Smile. Give thanks for your life. Those of us living with CF, we live in the land of coughing. It is all we’ve known. Trikafa is giving us the beauty of quiet. It is a surreal feeling. It also is allowing us to hear more. To listen to life and all we’ve missed. I don’t know if I will ever get used to it. Will I possibly have a yucky cough again? Can I still get sick? Absolutely. Reminder, Trikafta is not a cure. But it’s giving us more hope, more strength in our lungs and airways, and improving, on numerous certain levels, our quality of life. Leaving me speechless and full of wonderment for what may come. This is why we cannot stop giving so research continues to happen. We need life changing genetic modulators to be available to everyone living with CF. With all my heart, I want all of my CF friends to reveal in the glorious joy of this silence.

Reclaiming My Breaths

I used to be lost. Lost in the daily grappling fear of will my lungs stay strong for me today? Will I spend today coughing, struggling to live all the while inhaling this beautiful life? Will I wake up tomorrow choking on mucus? Will each step and breath when I run burn within my lungs? I may appear strong, but the truth is, we all have fragile stages in life. There have been moments where I admittedly wanted to give up. Thankfully they were fleeting, but I have learned it is healthy to acknowledge they existed.

Before I begin, I wish to express to each of you reading I am not choosing to share this part of my story seeking sympathy or commendation in any form. My hope in sharing is for those of you also living with CF, or any other chronic illness, or going through struggles, to always know you are never alone. We all fight personal battles, health-wise, physically, mentally and beyond. Sharing our stories may offer peace, comfort, reassurance and encouragement to one another. I am eternally grateful for the tough times, in the end they make me stronger.

To paint you a small snip-it in the big picture of things, Nov 2017 my lung function was drastically higher than it is now. The following month, a sudden onset of pain in my lower left lung hit like a ton of bricks. A constant stabbing, tingling pain. The best explanation? It was as if many needles were playfully poking my left lung and rib. Taking in a deep breath was a struggle. After many years of dealing with all we encounter, and those unexpected encounters, I knew this was vastly different.

My lung function thankfully remained astonishing, which was a blessed mystery given how I felt. I, along with my doctor, hoped it was just an extensive muscle strain from coughing. I will not go into the details of all the back and forth Dr. visits, tests and such, but on my 42nd birthday I had the joy of a CT of my chest and abdomen, with and without contrast scheduled. I wanted detailed answers. Chest x-rays were not adequate enough. Five weeks of a continuous IV antibiotic shortly followed with the words “nodule” and “pneumonia” and “pockets of mucus all throughout your lungs.” As well as “give it time.” I was able to do this IV at home always hooked up with the medicine flowing. I honestly didn’t care, the IV didn’t bother me, I just wanted the pain to end. I cried myself to sleep many nights asking God to take it away or take me away. I felt the lowest I’d ever felt health-wise, defeated and after 3 weeks of this potent antibiotic, the pain remained. Why? I hated my left lung, hated Cystic Fibrosis and hated how the progression of it had caused multitude of damage to my airway passages. That is the reality of CF.

After 4 weeks, the pain lifted a bit. Not completely, but I finally had some relief that I was begging for. When physical pain causes suffering, it consumes you. Anyone who has dealt with any type of physical pain, be it from illness or injury, life as you know it drastically changes. You continue on, because you’ve been trained to do so, but you also wonder how much more of this can I take? Is this ever going to end? The saying “pain is temporary” kept playing like a broken record in my mind during those 5 weeks. I believe pain also nourishes courage. I remember specifically during a phone conversation with a dear friend of mine, (who also has CF) telling her “I feel like I must go through this suffering to get to whatever the other side of this is…things have to get the worst of the worst before I will feel any ounce of better.” I can’t explain why I felt this way, but it deemed true.

Once I completed the longer than I’ve ever been on IVs, IV course, I gradually began to feel more like myself. After many consultations and CT follow-ups, the main determination was with the significant scarring / scar tissue my lungs have accumulated from years of damage, and chronic inflammation along with having pneumonia, all contributed to inflicting the severe pain. But why other times when I had been sick, I did not experience pain like that? Just goes to you show you how CF is relentless and so unpredictable. It was also discovered I have knots between my ribs in that exact area. Physical therapy and massage provided some reprieve. I still am baffled by the magnitude of it when it hit. Will it happen again? Yes, and it has. And it will. Trikafta will not dissipate years of destruction CF has created. Now, when an infection feels it’s attacking my airways and the pain returns, it is a warning. Always trying to look on the bright side of things. Doesn’t mean it’s easy. Since this situation came to light, excruciating as it was, it has given me stronger determination to never stop, come hell or high water.

As I recall those times my lungs experienced sheer agony, times I was crying out in pain and wanting to tear my left lung out of my body, I must smile and return to the point of enigmatic gratitude. I smile with the realization of where I am now. Each day remains surreal. With each breath, I presently feel this sense of tangible certainty within my grasp along with the beauty of hope for aging while living with Cystic Fibrosis. I have found in this new “normal” life since Trikafta to continue humbly embracing the changes. Many things feel like a trade off. I’ll happily take a few belly aches here and there, my legs screaming “Woa there missy, wait up for me!” during a run and transformations of certain parts of my body (haha!) for how my lungs feel. My body now is trying to catch up to my breathing. How? This isn’t what I’m used to. But I love it. My heart and mind feel an irreplaceable serenity, in the physical sense. Anything can still happen in the blink of an eye. But oh how I’ve honestly speculated my outcomes were permanently unpleasantly designed for my days to come. Those cloudy thoughts are slowly fading, allowing some sunshine to come through.

In the midst of whatever may come, I choose to cling to the grace of it all. Since Trikafta, I continue to overall feel amazing. Lung pain will come and go but I want to thrive. When faced with unknowns, sickness, infections, misery and feelings of hopelessness, it is so tempting to give in and give up. I believe I am here for a reason. I’ve worked hard to be living, so I have to keep on reaching. Nothing in life is certain. We cannot predict our future. Little did I know back then I’d be on this life changing modulator now. Since Trikafta, I am finding myself again day to day. More alive. I want this for all of us living with CF! In all I do, I am reclaiming the many years of lost breaths from the past with the most passionate fervor I possibly can.

An unexpected gift of life

It’s been 2 months since I first took my dose of the triple combination genetic modulator drug, Trikafta. Two months of life changing feelings to process, emotional breakdowns, breathing deeper, sensing more life in all I do with an indescribable gratitude and newfound hope of what is to possibly come. Two months of navigating the outcomes, the affects, how I’m feeling, reaping the benefits thus far, along with repeated questioning times of what is happening to me? How is this real? Is this a dream? Sometimes in the quiet, peaceful moments when I am alone, I close my eyes, slowly inhale in this new life in my lungs…each breath is such a gift. As I slowly exhale, tears often find themselves trickling down my face, releasing a tension within my airways that once used to rob my lungs. My tears derive from various emotions, but mostly from a place of joyful bliss from the depths of my soul with the upmost, humbled thankfulness for life.

In the last week however, a different awareness has caused my tears to cascade like a waterfall. I wonderfully celebrated turning another year older. Birthdays are bittersweet for me. It can be frightening with a beautiful ribbon of hope wrapped around it. As we age, we know are closer to the inevitable. I find my heart often overcome with feelings of guilt. Survivors guilt. A deep sadness with happiness. I wonder at times, why am I still here? Why have others with CF gone before me? Have I done enough with my life?

Every morning I wake up, I think of three things I am thankful for. It is how I love to start my day. Truly my friends, I believe goodness is all around us. It may be hidden in the shadows. It may not reveal itself until a new chapter in your life is written. It may be in the smile you receive from a stranger. A hug from a loved one. It may be right in front of you. If we look hard enough, it is there. On my birthday this year, I began a larger list mentally of all the “good” things. My faith, my family, my friends and my loved ones. My two pups. The ability to exercise. My stamina. My healthy weight. My health. Which brings my focus to how my is health is now. For wonderfully reaching the age 44 and to amazingly feel the way I feel, it still encompasses an unreal serenity beyond belief. If I could write a letter of acknowledgement for what Trikafta has given me, it would go something like this:

Dear Trikafta,

You are a wonder drug. Perhaps the closest thing to a miracle. I know, you are not a cure. But you may be the closest thing to one in my lifetime and for so many of us. At this moment, you have given me new life. You are also life saving. I hardly cough anymore. I have little to zero sputum to cough up and out. Even during my therapies. Do you realize the impact that is alone? Thank you for easier mornings. Thank you. Thank you for deeper breaths. Thank you for laughter without coughing!!! Thank you for giving me stronger workouts as I am breathing easier. Physically new pains and aches befall me, but each confirms I am alive! I need the rest of my body to catch up now. I believe in time it will. Thank you for aiding in my lung function. If you create stability in my lungs for the rest of my life, I cannot ask for anything more. Consistency is vitality. Thank you for helping my body combat a cold/infection a few weeks ago. Normally when an infection is attacking my lungs, it develops into something worse. I know antibiotics are and still will be needed. With you though, I feel I have more of a fighting chance in…everything. To possibly live longer. In just these two short months, you have given me tremendous quality of life. Something I never expected now. Is this my new normal? I do not know. I feel a sense of reassurance. Thank you for being the greatest, unexpected gift as I turned another year older. I am indebted to be on this journey with you and eager (with honest caution) to see where you help guide me for the rest of my days. PS- My heart continually hopes you will become available for all, and a new formula for those who are waiting. I can attest, our lives depend on it. Thank you for all you’ve given me.

Life isn’t always easy, we all have trials to face. I certainly try to do all I can and give my all with each day I’m given. If statistics held true from the day my loving parents received news of my diagnosis, I am not supposed to still be living and breathing today. I’ve never believed in those statistics. Maybe that’s why I am still here? Who can put a number on the age you will live to be? I feel one cannot. I know that life is precious and never should be taken for granted. I’ve always believed that. Living with a chronic illness like cystic fibrosis, you learn to appreciate life with all the gusto you can. With each new year I live, the battles and victories, I am delighted for this life. For still being here. For how I feel now. For every single moment I am given. No matter where the path may lead.

The wonder of it all…

As I sit here and reflect on how this past month on Trikafta has been for me, the most gorgeous, breath-taking snowflakes are falling steadily in their own peaceful rhythm. A soft snowfall. Serenity. All things are made new. It sparks a deep perspective within my soul. In all we are given..the joys, sorrow, struggles, confusion, frustrations, clarity.. life is so beautiful. All my troubles disappear. My heart is rejoicing. Everything is covered in the awe-inspiring magic that is snow.

Growing up, my family went on ski trips every February. As a kid, snow feels even more enchanting. We would leave our state, enter another and soon witness all things covered in a glorious white blanket. Add the peace of the mountains in view and there lies my true contentment with and in everything. The bite in the cold air with each breath, seeing your breath! Hearing the crunch in each step. The pine trees veiled in white. The entire earth glistening like diamonds when the sun came out. Excitement would consume me. I remember how seeing the first sight of snow would create my heart to skip a beat! Once we arrived to our destination, got to our lodge, figured out sleeping arrangements, etc., it was usually bedtime. All the fun awaited for us come morning.

I have such fond memories from these ski trips. I remember distinctively being scared in the beginning learning how to ski. When I was 5, I actually broke my leg on a bunny slope. If even that, I think it was just a small little snow hill. My Dad started teaching me at an early age, to share his love of skiing with me and my brother. I used to ski between his legs as he would hold me giving verbal cues as to how to move my skis. I had many fun times getting accustomed to the ski lift. I remember telling my Mom or Dad to hold me as the chair came around so I wouldn’t fall. Getting on and off I had my share of falling! I had to conquer that fear and just do it! Silly now that I think about it. I was fairly tiny and seeing that big chair come around the corner at me at what I felt was light speed, was a bit nerve-wracking.

Over time, my Dad would hold a ski pole out to the side for me to grab onto with both my hands and ski with him. Observe his skis. Watch and learn. Feel the snow hitting my skis and all the sounds. To turn, to snow plow, etc., all the basic ski techniques. I loved every minute of it. I felt so important, wanted to make my Dad proud as I listened to him guide me. Gradually he’d encourage me to let go and ski solo. He would continue to hold his ski pole out for me if I needed. I had the best teacher. I remember skiing by myself for the first time. Freedom and thrill! As the years went by I was given my own ski poles, bigger skis and even goggles. Like the grown-ups. Time for me to do this thing on my own. I was ready, nervous and ecstatic. I still wanted my Dad by my side though.

One particular time I recall like it was yesterday. My Dad and I had a day of it on the slopes. Everything felt so perfect. I was skiing on my own next to my Dad. We would always take the blue (beginner) runs. This particular path we were on was smooth sailing. We reached an open meadow. So peaceful. Next was a breath-taking path surrounded by trees. We came upon some little snow moguls. I absolutely loved skiing on them! I wanted to do this path again and again. Somehow though, along the way, we took a wrong turn. I don’t know how. We hit a black (expert) run and as we turned, we realized we were at the top of the face of the mountain. I sensed immediately this wasn’t good because there weren’t many other skiers around. Fear overtook me. There was no turning back.

My stomach dropped as I looked down. I felt sick. The look on my Dad’s face was not comforting. Even in his Stetson Billy the Kid hat that distinguished him from all the other skiers, his face displayed uneasiness. When I saw his reaction, I wanted to cry. He knew I was scared. We stood there for what seemed forever. He comforted and encouraged me “We can do this Sis. We are going to take it slow. Just listen to everything I say and do as I do.” I tried to remain calm but every time I looked down all I could think of was “I can’t do this.” My Dad’s strength in that moment carried me. He couldn’t hold onto me. I had to face this obstacle on my own. It was icy in spots. If either of us fell or lost control, it was a very long, steep drop.

We began moving laterally and side stepping in our skis, very slowly, leaning into the mountain as we did. I was so frightened. The wind picked up and pierced my skin. My Dad was trying to get us to a point where we could possibly ski downhill again. It wasn’t happening. We would take breaks, standing there on what felt like a cliff, my Dad yelling “Don’t look down, just look at your skis and focus”. So I did. We continued on, it felt like the longest journey on snow and ice. After awhile, knowing that it wasn’t getting any easier, and tears starting to fall from my eyes, my Dad said to me “Sit. Just lean to one side, skis in front, and slowly go down.” Maybe that wasn’t the smartest but I obeyed my Dad. He held out one of his ski poles. I grabbed it. We then scooted very carefully the remaining way down. He never left my side.

Once we made it to the bottom, relief reigned over us. My muscles hurt. Every inch of my body was tense. My mind was tired. I know my Dad felt all these things as well. I also felt so accomplished. My Dad said to me “Sis, look at what we just did…” he pointed behind us as my eyes wandered up the mountain face. We did it. We made it to the bottom in one piece. I don’t recall how long it took us, but it felt like an eternity. My Mom finally saw us and came over to us quickly. Concerned about where we’d been and the amount of time gone, my Dad looked at her and said “Your girl just tackled that” pointing back again. I said “Dad, WE tackled it together.”

We called it a day. We spent the evening out enjoying a meal as a family at one of my favorite restaurants, walked up and down the strip taking in the adorable little shops, grabbing some hot cocoa, and taking in the lights. On several rare occasions, we witnessed night skiers. Glancing up the mountain, they gracefully were making their way down with lights. They may have been holding flares. I don’t know how they did it without falling. They were the experts. They were on the face. The face we had just conquered. What another fascinating, entrancing view to witness.

Thinking about how nervous I felt in that moment with my Dad, not knowing the outcome, unsure how we were going to navigate our path, in some ways, mirrored how I felt starting Trikafta. Honestly, I did not know what to expect. Would it work for me? Would it create unwanted side affects? Any difficulties and I could possibly have to discontinue taking it. If so, I’ll have to face those unwanted changes. That is still a concern down the road.

I completed one month post Trikafta follow up labs. Thankfully, (oh so thankfully) results revealed my liver enzyme levels are normal, as well as kidneys and everything else they checked. I have Budd-Chiari Syndrome. It is a rare disorder characterized by narrowing and obstruction (occlusion) of the veins of the liver (hepatic veins). As a result, I am on a blood thinner indefinitely. This places me at higher risks for liver complications. I was relieved all is well so far. Hoping and praying it stays this way.

I continue to feel… inexplicably incredible. Hardly coughing. Minuscule to nonexistent mucus. If any, it’s clear to pale yellow. Mind-blowing. I brought up some junk along with a little blood the other day. At first I panicked. Then instant relief came over me with the reminder “Better out than in!” and “Oh hey remember me? It’s been awhile! I’m still here.” That small amount (maybe the size of a pea) and color was the most I’ve coughed up since the first week of Trikafta. Unreal. I’ve had two nothing short of amazing runs (for me) this week. The new life I’m feeling and breathing in my lungs with each step is something else. My workouts do not consist of spitting up green junk (marking my path with my mucus) and stopping to catch my breath. I didn’t realize until the other day how winded I used to get when running with my two pups. I’m not experiencing that hardly at all anymore. I think my dogs are just as happy as I am with these changes within me when we go on adventures together. I think they can sense something is different. I still have more energy. I am noticing gains, in so many ways. I am starting my days earlier. More productivity. Which I thrive on.

Where will Trikafta take me? Only time will tell. Like freshly fallen snowflakes blanketing the earth, making all things new, Trikafta continues to reveal this newness in me. As I watch the snow covering the ground, trees, all of nature…I am left in amazement. Which brings forth peace and reflection for how immensely grateful I am for this past month, for how I feel, and how my life is different thus far. My mindset is joyous…I feel a tangible hope in my soul from three is exciting! I am engulfed in astonishment every morning. This isn’t a cure, some things will obviously remain. But for now, right here in this moment, I will hold onto all that is happening. I give so many thanks above for this medication. I feel like that little girl I once was in the snow and in her skis…being captivated by the beauty and wonder of it all. And someday, I plan to find myself on the slopes again, breathing in the smell of the snow and pine, permitting the crisp mountain air to delightfully claim my new-life-in-each-breath lungs. Pure elation. Who is with me?

Life to my days

It’s time. I’ve stretched. I’ve rolled out every inch of my being on the foam roller. I’ve hydrated. I’ve eaten. I’ve got anything else I need ready. Here begins one of the best parts of my day. Time to lace up the two most beautiful running shoes before me that help carry my entire body. They feel every movement, every pause, every tempo and every pain that flows through my body with each step. I grab my phone, my watch, my cycling jacket, my buff and ball cap. As I begin, I take in the deepest breath. It’s as if I can feel my breath go to the center of my soul now. I exhale slowly. ‘Thank you for this life, for every step and for every breath I am given’ I whisper under my breath. I soon find my rhythm. I find my peace. I find exactly where I want to be. As I begin another journey, I find my lips forming into the biggest smile as joy fills my heart. Let’s do this.

A dear friend the other day referred to me as a “runner.” It’s quite funny. I never thought I’d ever hear that word be associated with me, let alone consider myself one. Growing up, I didn’t exercise. I didn’t participate in sports. I hated PE class with a passion. I was slow, always coughing, embarrassed for my cough, and known as “little Michelle.” I was teased at times by classmates for my size and lack of physical strength. Nobody wanted me on their team and honestly I didn’t blame them. I remember my freshman year in high school having to complete a mile for our final. I thought I might die. I didn’t want to do it. I finished last. I loathed every moment of that mile. I despised anything exercise related. I just wanted to play piano, read books, write stories, sing in the church choir and be with my friends and family. In college, the trend continued. I focused on piano, the organ, studied for my classes, my major, sang in Concert Choir and prayed I stayed healthy enough to graduate, earn my degree and enter adulthood.

A few years after finishing college (1999) I joined a small, local gym in the town I lived in at the time. I tried running for the first “actual” time. I still felt resentment towards it. We were not going to be friends. I discovered yoga and weights, and loved both. Soon I began riding a mountain/hybrid bike. So much easier than running! That became my focus: ride my bike, yoga, and weights. I remember completing a 25 mile ride. It felt so good! My lungs and body awakened. I wanted more of that feeling. I was 24 years old and little did I know this awakening was soon to be a huge part of my life that was missing.

Jump to 2011. Social media. I actually joined that world in 2008. I became friends with others who lived with CF doing all these amazing fitness-related accomplishments. I was in awe! My constant inspirations. Runners, riders, power lifters, yoga teachers, triathletes. I wanted to jump on the band wagon. I tried running again with a new determination. Walking and running intervals. I kept putting one foot in front of the other and a willingness to keep going. My motivation: if my friends can do this while also living with CF, so can I. A beloved friend of mine challenged me to start running 100 miles a month. She and I did this, miles away from each other, for 6 months. She also had CF. She was my biggest cheerleader to run for these lungs. We encouraged each other daily. I miss her dearly. I carry her in my heart every day.

It wasn’t a stroll in the park when I began. I wanted to give up so many times when my lungs were screaming at me to stop. But with each step, I discovered what I needed. A sense of normalcy. To breathe a bit easier. To feel strong! It became this magnificent escape allowing me to forget in those minutes and miles that I live with cystic fibrosis. With each cough and spit, I would smile and think to myself “Yeah, that’s right… you don’t live in my lungs anymore you bastard. Take that CF!” As I endured the pain, the courage, the powerful relief it provided me, I knew I didn’t want to stop. Who had I become? I just wanted to keep going. It’s never been about losing weight, winning that race, or to impress anyone. I aim only to impress my lungs. I wanted to make them as tough as possible. With each step and breath, I wanted to add life to my days.

Present day. I love running. It is forever my outlet. I love how freeing it is for my mind, body, lungs (literally!) and heart. It is pure therapy for my soul. I’ve found myself running longer distances. Distances I never ever imagined I was capable of. I find refreshment in how far I can push my body, my lungs and my legs. I love a challenge. Challenges bring self satisfaction in overcoming obstacles and digging deep within yourself as your eyes remain on your goal. I had a short episode where I didn’t run, I took a break. I permitted life to take over. Mental health took a dive. Lung function took a dive. I paid for it with the ramifications of my inactivity. I refuse to allow that to happen ever again. My life depends on it. My lungs depend on it. The overall benefits of running are limitless. It helps fight heart disease and diabetes, maintain proper blood pressure, boost confidence, fight depression, strengthen your core, reduce bone/ muscle deterioration, and overall keep a person healthy. I know it has contributed to me still being alive in my 40’s, living with Cystic Fibrosis and having my original lungs.

Even though I’m soon to start month #2 on Trikafta, my exercise regime continues. I am feeling a different kind of energy and vigor during my workouts. I truly hope for long-term stability in where I am. I will certainly take it and my gratitude cup will continue to overflow. I’ve had many emotional break downs. All from the fact that since Trikafta, running feels easier. Working out feels easier. I am breathing easier. It’s amazing. I just want to run faster, workout harder, jump higher, climb mountains and throw big objects. Not necessarily in that order. My legs and body are having difficulty keeping up with my lungs. How is this happening, again? Is this maybe my new normal? I learned this past week I’ve to pace myself. I over-stressed my sacrum (where I have degenerative arthritis). It’s vital I pace myself in everything, to slow down a bit. Wait, what? This leaves me baffled because for me, my “pace” has always been mediocre to slow to just finishing even if I have to crawl. Finishing is winning, by the way. No matter what the pace.

All I’ve ever known is what I’ve only known. I’ve kept going despite the decline in my lung function, pain in my left lower lung from scar tissue and just a few months ago needing 3 weeks of IV antibiotics for an exacerbation. Pain nourishes courage. Never forget that. During the toughest times, my desire to never stop only multiplies. I know I will still feel pain. I will still struggle. And I will keep going. This is my health, my life. With each run, ride, training…I find myself again. I find a purpose, an inner peace with each breath, with every drop of sweat….I find what life means, what life is, and that you can do anything you put your mind to.

It is a thrilling thought to see where Trikafta may take me. I’m trying to not be overly optimistic. It’s still overwhelming emotionally at times to accurately articulate the feelings I feel when it comes to all of this. Registered running events are already written in stone and on the tablet of my heart for 2020. No matter where I find myself, running / exercise is my constant source of life affirmation and contentment. As long I have breath in my lungs, I will keep going. I feel a renewed spirit since Trikafta. I want to shout from the mountain tops my joy and gratitude. And please, will someone come along and pinch me?

Here is a little something I wish to share. I wrote this many months ago (pre-Trikafta) immediately following a run. When feelings flood my soul, I must write.

When nothing makes sense, I run. 

When things are crumbling around me, I run. 

When I can’t stand the noise of life anymore, I run. 

When anger enters my mind, I run. 

When fuck yous are everywhere, I run.

When sadness fills my soul, I run. 

When I want to feel normal, I run. 
I run to release.

I run to feel free.

I run to believe.

I run to be me. 

I run to be strong.

I run to replace wrong.

I run for my lungs.

 I run for my life. 

I run. 

The path turns to gold with every step. 

No more noise.

Only joy in my breath. 

My heart races.

My soul smiles.

I soak it in.

All is well again.

I am alive. 

“Don’t stop anything”

This past Wednesday I had CF clinic. It was my first appointment since starting Trikafta. I don’t know if this is the beginning of repeated check-ups like this one. If so, I may just be a sobbing-able-to-breathe-deeper-laughing-longer-without-coughing-no-longer-wheezy-singing mess for the rest of my life. All went..incredibly well. Typing now, I forever struggle to express the thankfulness in my heart. My only concerns were: a) follow-up labs one month after starting Trikafta to check liver enzymes; b) check sinuses to confirm if any issues as I’ve had more drainage / post nasal drip; c) I need new bras (added bonus? what.. how..?)

I gained 6 lbs in a month! I was shocked. Living with CF, being at a healthy weight is important. We need that reserve for when and if we become sick. Luckily, I’ve maintained a healthy weight for several years now. It’s not easy. As I’ve so wonderfully aged, I have noticed my metabolism has slowed down. Now that I’m taking Trikafta, my GI tract is likely going to be working more “normal” and therefore weight gain may happen easier. Which is good! And yet, I will be keeping an eye on it. I will monitor how the next several months go. My doc discussed with me a test down the road to see how pancreatic insufficient or if become sufficient. If enzyme dosage adjustments are necessary. That is wild…just mind-boggling!

A month ago, my FEV1 (the amount of air you can force from your lungs in one second) had revealed a decline again. On Wednesday, results revealed an increase! I’ll take any. It wasn’t the number I secretly hoped to see, but it is a few points shy from being back to my baseline. My Dr. is confident I will hit that number and possibly keep going. What encouragement and light to my heart and mind to hear him say that. There have been many times my FEV1 does not mirror how I feel. How I feel is what matters the most. The most wonderful part of my pulmonary function test (PFT) was how my lungs felt during. I could force the air out in that one second with more power. I didn’t sound like the penguin from Toy Story as the air was leaving my lungs. I didn’t feel winded at the end when normally I was certain I might pass out. I was able to take that quick deep inhale at the end comfortably. For me, this means everything. I thought to myself, “alright numbers, whatever you show it’s going to be just fine.” I felt relieved. Peaceful. This year, PFTs have given me elevated anxiety. Anxiety, if you allow it to, can eat you alive. I’ve dreaded doing them. I could tell my lungs were struggling with every attempt. It’s such a fearful mind thief. Hence hello uninvited anxiety. Perhaps now, hello peace of mind. Oh how I’ve missed you.

We discussed in detail all things regarding how I’ve been feeling since Trikafta. I shared with him that I hardly cough anymore. I purged within the first 24 hours of that first dose, a bit the second day. As each day has passed, now starting week 4, my cough has left me like a candle in the wind. This alone makes me smile and cry big, fat happy tears. When I do cough, it’s because I’m huffing to huff and coughing to cough to bring forth what I keep thinking will be more gunk. Just how is this? Over time, as Trikafta continues to filter all the tracts- lungs, sinuses, GI, kidneys, liver, etc…the tougher spots where mucus gets trapped (areas of scar tissue build up) should eventually clear out. My doc stated to not panic or be surprised if I have sudden bouts of more production as the medication is continuing to do it’s job. Yes please and thank you Trikafta.

A surreal encounter during my appt was my doctor’s reaction when listening to my lungs. I’ll never forget that moment. He chuckled and said “This is the clearest I’ve ever heard them. No crackles, wheezes, nothing.” Followed immediately with “Don’t stop anything. Keep doing all of your therapies, exercise, all.” The thought had crossed my mind, however I had not mentioned this to him. He informed me that during Trikafta clinical trials, those who participated were at maximal levels with therapies while taking the modulator. Therefore this reflected their outcomes. I want to have the best possible outcome. Nothing less. In addition, the years of damage to my lungs will not be reversed. Therefore, I plan to resume all therapies while taking Trikafta. I will do everything in my power to remain stable, to hopefully have less lung infections and less use of antibiotics. I have quite a resistance presently to many of them, which is frightening. I’m hopeful Trikafta will give my body a fighting chance without needing antibiotics as often.

Overall, I had a fantastic appointment. I am so grateful beyond words for a great clinic. My doc said come back in 3 months! I’ve been seeing him every 1-2 months since February this year. I wonder how things will be in a few months? Not only for myself but for all of us on this modulator. Time will tell. My spirit continues to feel renewed. I work hard for my lungs. Every day. I know my hard work all these years has paid off. Why do I feel that I don’t deserve this…change? I think this sometimes since that first dose. Those of us living with CF know that we must do all we can to stay alive. The progression of cystic fibrosis is inevitable. But now…Trikafta. It isn’t a cure. But oh how I want this for all of us. I won’t be stopping a thing. With each breath I take, I give my all in this and in everything. Life is so precious! If I am lucky to receive added years of living along with improved quality of life, that is the most wondrous gift I could ever be given.

Give me all the air

I’m on day 19 of Trikafta. I remain speechless so many times, (yet I also have been informed I am more talkative sorry not sorry), have had a few emotional break downs, and unending gratitude in my heart for what is happening. Trikafta continues to baffle me, leaving me thinking repeatedly “how is this real life?” My days no longer feel as numbered. My soul feels awakened with the hope of what is to come. I didn’t think I could feel this way. It is a magnitude to take in.

My body is changing. How you ask? My skin appears healthier, more visible color. When I sweat, however, I stink. I mean, REALLY stink. An odor is emitting from my pores that leaves me laughing and gagging. I invested in a clinical strength deodorant this week! We shall see if it works, haha! I am no longer losing what always appeared to be enough salt to fill a shaker after a workout. When it comes to salt, it’s been a part of me that I have identified with. Salty. I thought perhaps the salt loss was all together gone and my heart sank. I have some issues, I know. The other day after a run, I saw a few salt crystals on my ankles and I breathed a sigh of relief. But I am not seeing it around my temples anymore, on my face…where I typically sweat the most and that is just not the usual. And good. So good. This means I am no longer losing sodium like I did typically during exercise. Which means more energy. Which means better workouts. Which means overall goodness. I am seeing this, loving it and so thankful.

I continue to feel rejuvenated throughout my day. More energy. Laughing longer. Not coughing during my laugh! Singing louder and hitting high notes that before my voice would become raspy and give up. I wake up and sing! I’m not short of breath. And I dance. I love to make up songs as I sing, which is annoying to some. I am testing my voice and I love it. My voice is stronger. I’m talking more. There is more air entering and exiting my lungs so therefore, if you don’t wish to hear me, well…you’re just out of luck. 😉

I’ve been going to a chiropractor since 2013 for my posture, degenerative arthritis and for my overall well being. Furthermore to benefit my health and body for exercise. My beloved chiropractor, Dr. Jayme Gawith at Wellness One, has been amazed at the changes within my structure since I began Trikafta. He is noticing during adjustments that there is less tension in my body. My ribs are no longer needing adjusted! He informed me today he no longer has to apply such strong pressure. He has been speechless, and sharing in my emotions at how this medication is working. Not only is it affecting my lungs, it is impacting my joints and structure in a remarkable way. It is …crazy amazing.

I stated earlier I’ve had a few emotional break downs this past week. These are happy tears. My lung function this year alone has been nothing but a roller coaster. Added new inhaled antibiotic in March. Trying to target particular bugs in my lungs. I saw some numbers this year that scared me. I began having anxiety before and during a pulmonary function test. This doesn’t help and can contribute to the outcome of the test. My doc has even been astonished at times as to why I declined. He also assured me by looking at the history of my pulmonary function tests over the past decade. It revealed that overall, I have remained stable. Added affirmation that exercise is vital and crucial for my lungs and health. As is doing all of your medications, treatments and therapies.

Today I have a CF clinic appt. It is a follow up previously scheduled. I am nervous, excited and will be feeling many emotions. The last time I had a pulmonary function test at clinic it had dwindled down again to that number that I didn’t want to see. I am anxious to see how all things go and discuss with my doctor the results. Even if I maintain, I’ll be on cloud nine. That is my hope. We will see.

Returning to my emotional moments. I’m currently training to run another half marathon early Spring 2020. I’ve been so excited about this particular race. Since Trikafta, I’m able to take deeper breaths, and not feeling as short of breath during a run. I am able to run a bit faster. I feel like my legs cannot keep up with my breathing! I am not having to stop to cough, work to clear my lungs, catch my breath, and then when I’m ready, continue on my way.

On Friday last week, it was cold, drizzly. Perfect day for a run. I am wanting to run in the cold. I love it. I didn’t used to love it. Now, my lungs are able to manage the cold with more ease. I completed my 5 miles that day. I returned home, grabbed a hot beverage and soaked in the tub. Then, it hit me like a freight train. I didn’t cough. Not one time. How? It was cold. I breathed harder. I felt so alive. But I should have coughed. For the first time ever in my life, I had just ran without coughing. The tears came. I let them fall. Tears triggered by an experience in my life I never would have imagined. All of my emotions in that instant came to the surface. This drug. I didn’t cough. I just ran. I didn’t even cough afterwards. What does this mean for my life? This cannot be. My lungs feel stronger. I’m breathing easier. My workouts are different. I want to go faster. Push myself more. See how far my limits can exceed in a way I cannot explain. What does this mean for me, for my fitness goals? I don’t know. But my heart is so happy. My quality of breathing feels vastly enriched. I want this so much for all of us living with CF!! If my lungs could smile, they would. Thank you Vertex pharmaceuticals and all the research funding made possible for this life changing modulator. Thank you Trikafta for giving me all the air. I can’t wait for new adventures ahead.

Humbled Breaths

For breath is life, so if you breathe well you will live long on earth ~ Sanskrit Proverb

Tomorrow is Thanksgiving. A day we typically reflect on all we are grateful for. I give thanks every day for every breath, each step and for every moment, big or small, I am given. Right now though, it is difficult for me to adequately express the level of gratitude I feel presently in my heart. As I sit here, taking in the deepest breath, filling my little lungs with all the beautiful air I can, I then hold it steady. A smile spreads widely on my lips. I then slowly exhale…emptying my lungs slowly. I didn’t cough. I didn’t struggle. I didn’t feel the itchy, wheezy gasps that usually consume my lungs and throat. I am left speechless in this moment.

How is this happening? There is a new strength in my airways. In my workouts. In my voice. In my laugh. All I’ve ever known as “normal” is to cough, to constantly work hard to clear my lungs, to struggle to breathe, to feel wheezy at times accompanied by all this uninvited mucus just stealing my air. With my goal being to get it all out as much as I can. All the time. 24/7. Mornings have always been my most difficult. All that thick junk building up in my sleep and come morning, a fight waiting to get out all that I could. Even with all of my breathing treatments/ chest physiotherapy. Relentlessly time consuming and exhausting. Not anymore. I am so humbled as I write and share this with you.

I have cystic fibrosis (CF). That would have been perhaps helpful to have mentioned at the start. If you google CF, one definition you’ll find states “Cystic fibrosis is an inherited life- threatening disease that damages the lungs and digestive system. CF affects the cells that produce mucus, sweat and digestive juices. (yummy) It causes these fluids to become thick and sticky. ” This leads to severe respiratory and digestive issues, infections and sometimes, diabetes. Good times.

Now that you have read that lovely piece, I want to introduce to you Trikafta. Again, thank you google because sometimes it’s just easier. “Trikafta is a combination of three drugs that target the defective CFTR protein. It helps the protein made by the CFTR gene mutation function more effectively.” Trikafta is “for the treatment of CF in patients 12 years and older who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene”. If you wish, research this even further for the medically technical scientific fun factor. Trikafta is, for the first time in my life, a triple combination genetic modulator that my specific mutations qualified for. I have been so anxiously (to put it lightly) awaiting for this drug! Not just for myself. For all of my friends living with CF who are eligible to be on it. The FDA approved it 6 months earlier than anticipated. It is an exciting and hopeful time in the CF community!

I am asking myself every day “How is this real life…is this a new life?” Nov 16, 2019. I took my first dose of Trikafta. In 12 days my life has drastically changed. Days 1-2 I purged. Immediately upon dosage. Dark green, to gray, to old blood, to thick mucus…all coming up. Disgusting junk that I didn’t know existed in my lungs. I had a runny nose, drainage in my sinuses and throat. Sore throat briefly. Some nausea. Soon what I coughed up went from dark green, to light green, to faded yellow, to white foamy, to clear. With hardly any effort, it came up in big amounts. Again, this occurred for me during the first two days. Suddenly, I could breath easier. I could feel air moving in my lungs in a way I have never felt. And I feel this now.

As each day goes by my cough has become less and less. Nonexistent at times. Where is the mucus I’ve known forever? How can this be? My morning routine changed. I no longer wake up congested. Even during my therapies very little, if anything, is productive. I have tons of energy. My workouts feel at a new level. Easier. Stronger. I will write about those another time and try to hold back the tears. I want to sing, whistle, hum all the time! There is more pep in my step. I am breathing easier. I am breathing easier. I hardly have a cough. Those five words! I can breathe deep and not feel restricted as I once did. Someone just pinch me. It is unreal. Yet, it IS real.

Emotions have taken over several times as this is a lot to process. When I was approved for Trikafta after rounds of prior authorization denials from insurance, I cried. My heart leaped for joy followed by a sick feeling in the pit of my stomach. Guilt swept over me. Why guilt? I couldn’t help but think of my dear friends who have gone before me and will never experience this. Survivors guilt. Guilt for those who do not have access to this medication yet and need it now. It’s hard to NOT feel this way sometimes. I want this for all of my CF friends, and for those still waiting for a genetic modulator…we all deserve this. Each and every one of us.

Please understand. It’s not a cure. It will not reverse the years of damage to my lungs. It’s another form of treatment. I resume all of my current medical regime and exercise in addition to taking Trikafta. It has honestly increased my motivation to exercise. I have worked hard for 43 years to still have my original lungs. Now that I am fortunate beyond words to have this medication, I want to work ever harder to maintain and keep my lungs as strong as possible. I can still get sick easily, get infections, be on antibiotics as needed…CF doesn’t go away. But I feel now, for the first time, less fear for my future. I feel now a new beginning to the course of my life that I didn’t think was possible. I do not know how to comprehend this and what the outcome will be for me. But with each breath, my heart is overflowing with joy and gratitude. I give thanks above. My breaths are humbled with the hope of more days ahead, more time with my loved ones and more time to live and love with my entire being. Thank you, Trikafta. I am excited for this journey with you.

Maybe I’ll live past 50…

Hi, my name is Michelle (you may call me Chelle) and welcome to my page! I’ve always loved to write. Ever since I was a little girl. I even enjoy sitting down, grabbing pen/paper and writing down all of my thoughts. Reflecting my thoughts in a journal. It’s healing. It’s releasing. It’s a way to remember life. I also love to write letters. Growing up, I wrote letters to both of my grandmothers and my cousin. As a kid, it is so exciting to get something in the mail. For me, when I would write to them, address and place the stamp on the envelope, I was more anxious and happy for them to receive my letter! Writing has always brought me joy and comfort.

So, why am I doing this blog? What do I want to achieve? There are no achievements determined, only my deepest thanks to each of you for taking the time to read my ramblings and follow my adventures. I decided to blog to share my life experiences, the ups and downs and everything in between. I also want to share my journey since beginning the medication Trikafta. I have Cystic Fibrosis. I am 43 years old, and for the first time in my life, I am able to take a triple combination genetic modulator. It isn’t a cure. My lungs still are damaged after years of infections, coughing, mucus build-up. etc., with scarring and scar tissue. Trikafta will not reverse the damage. I am hoping for stability, maintaining and overall my quality of life improving. Maybe I will live past 50..? I’ve never felt this hopeful. Trikafta may be life changing for me. For all of us who can take it. I cannot wait for each of us living with CF to experience these new therapies and modulators. We all deserve it.

Thank you again for visiting my page. I hope wherever this finds you in life, you are happy, content and receiving the love you so deserve. All my love to you.