Living With CFTreatments

Trikafta, along with dogged perseverance, saved my life – by James Kain

Photo taken during a large blizzard which occurred on my birthday (January 24, 2016). I am with my Alaskan Husky named Lakota, whom I went snowshoeing with.

I was born in Washington, D.C. in 1985, and was raised in a nearby community known as Reston, Virginia. By the time I was born, my older sister had just recently passed away from Cystic Fibrosis at the all too innocent age of eight. To say that her passing was devastating would itself be an understatement, as most marriages rarely maintain themselves through such trauma. However, my sister’s wish for a baby brother was soon granted to my parents, and my own journey forward soon began.

Pulmozyme, known to many as DNAse, was in its experimental and not yet fully approved stages when my sister passed away. It remained a cruel irony that a potentially lifesaving treatment existed, yet it remained out of the grasp of critically ill patients. Such is the tragedy of medical developments, when a potential cure or likewise treatment can possibly appear a mere day after the passing of a life that it could have very well saved.

My younger sister was the first individual for whom the Cystic Fibrosis amniocentesis test was performed, and my entire family’s genetic data was stored as part of this process. Medical history was made, and another triumphant achievement was brought to fruition. Yet, how little could some people have completely realized the full-scale behind how such information would eventually become utilized in future treatments. Basic genetic data was acquired, studied, and understood, but no capability to manipulate it at the root level was available. 

Early stages of genetic treatments involved viral vector gene correction methods, some only requiring a once monthly dose. However, no truly integrative DNA-level correction has become available, because the process required to insert the correction simply isn’t precise enough to ensure positive and non-detrimental second and third order effects via this method. This form of a permanent cure may one day become a reality, in fact, it likely will become one, but until that day arrives, we once again find ourselves at the crossroads of what currently exists versus what may exist tomorrow or just beyond. For all of us currently living with Cystic Fibrosis, anything that resembles the closest achievement to a permanent cure at this very moment is itself momentous and gratifying. Speaking for myself, improvement equates to a cure in its own right, as it finally cured me of a crippling exacerbation which for the past three years had robbed me of a decent life.

From the time I was born, I faced a 50% chance of making it to my current age. Many of my friends who also knew my older sister are no longer with us, as Cystic Fibrosis claimed their lives before now. Dealing with these circumstances gave me a very solid idea of mortality, and a steadfast appreciation for life. I learned at a very young age to value life as an all-connective concept which binds all of us together, because it’s precious above all else and cannot be replaced once lost. There are people who learn this lesson much later in their own lives, and there are times when I wish that I had not learned it so young; but I I also realize that it’s granted me the gift of appreciation with plenty of years still ahead of me. Thus, I have been granted the opportunity to pay this appreciation back towards others, which stands as the prominent reason behind why I have decided upon going into the medical field myself.

Before now, I was always in great physical condition, remaining active in the outdoors ever since I was a child. I was a Boy Scout, a natural explorer, a lover of meteorology, astronomy, aerospace, and how we push the boundaries both here on Earth and across the universe. I was someone with great ambitions of pushing myself to my ultimate mental and physical potential which I possess. We all have our potential, and I was working extremely hard to discover mine. Achieving anything less than my full potential has always felt to be a complete waste of life, because, after all, life to me is precious and unpredictable. Life is something which many souls never have the opportunity to fully experience, for they are robbed of it before their time. Thus, I have always felt a deep responsibility to do my best at everything I set forth to accomplish.

Unfortunately, three years ago I was completely robbed of my capabilities when I came down with a fungal infection of the lungs. In another twist of cruel irony, I was partaking in long-distance swimming as a method for maintaining and improving my health, however, an improperly maintained pool gave me the worst infection I’ve had since childhood. For roughly three long years I battled multiple setbacks, including improvements which plateaued and then descended into an even lower bottomless pit of pain and hopelessness. I attempted utilizing every single medication in my arsenal, as well as new medications focused upon the fungus itself, all of which failed to bring me back to my state of health prior to this exacerbation. There were times when my parents felt they’d have to call the rescue squad for me, due to my prolonged fevers, muscle pulling coughs, cramps, chills, constant struggle to breathe, and 35 pounds of weight loss which I suffered no matter how much I tried to eat. I went through two solid episodes of severe sickness within the past three years, where my voice was lost, my stomach could not hold food, and I felt as if a ten-pound weight had been set upon my chest. Yet, through this all I still attempted to swim, ruck, and PT as much as possible, as I kept the faith that one day in the future I’d be able to once again live a normal thriving life. The problem is that I was losing hope, and the more time that went on, the quicker my hope began to fade.

What sustained me through this time-period above all else were my dogs, and my cat, as they frequently kept me moving ahead while constantly checking on me every time I coughed or twitched in pain. Over the years they began to pass away, a very unfortunate aspect of this precious life, but they inspired me through their own fight to survive while also being there for me in the most selfless of manners. The final of my original three passed away this past January 13th, 2022, as she fought through her own immense struggles in life to be there for me. She inspired me beyond all else, and for that I shall remain eternally grateful. It was during her remaining month with me that I discovered the other miracle in my life, this time one created my mankind in contast to God, although I still give credit to the latter for enabling the former to perform such amazing works of grace. December 3rd, 2021 is when my newfound life with Trikafta was first realized, and the difference in my life became immediate and drastic.

My mentioned dog pack: Lakota (the Alaskan Husky in the foreground), Maddie (the Jack Russell Terrier in the background to the right, she’s the founding member who raised the Husky), Breezie (the all-white JRT/Schnauzer mix who just passed away this past January 13th), Millie (the black Rottweiler/Hound mix), and Baxter (the fawn-colored American Dingo).

Trkafta is relatively new, but the process for this therapy has been going on for over a decade; and the time from grasping the genetics of Cystic Fibrosis to the point of now impacting the genetic chromosomal expression has been occurring since my birth. The work has been relentless, but the results are nothing short of miraculous. My pharmacist was the first person I contacted last November of 2021, when my most severe exacerbation failed to improve. The pharmacist immediately called me, and began discussing this new medication with me, before finally shipping me three boxes of the medication as quickly as possible. My first dose was during the early morning hours of December 3rd, with a high-fat breakfast consisting of poached eggs on English muffins (covered in cheese), and bacon. With my trusty pup nearby, I began taking my first dose and eating breakfast. Within 15 minutes, the cleansing process began.

My first experience with the cleanse was coughing up a handful of junk, and it happened out of nowhere. As this process progressed, I began to empty my lungs and sinuses of colors which I’ve never witnessed before, consisting of everything from red, to orange, brown, grey, green, and all colors in-between. At times, I wouldn’t even cough, and the congestion would come slipping out with none of the typical warning signs. Forget being self-conscience about any of this, I was elated! Within seven days I was healthy enough to attend a Santa Claus meeting with my pup, which only a week prior had been completely unthinkable. In fact, only one week prior I thought my life would soon be over, but here I was now having the best Christmas of our lives together! Soon, I began to regain my appetite, and my pup and I gorged together upon the greatest of Christmas feasts! Little did I know that only a few weeks later this would be the last Christmas with my pal, but thanks to this amazing medication I was able to enjoy it to its fullest! Thank to my friend, I was pushed through a torturous month and carried forward to the point where the medication could finally deliver me renewed hope.

84% Oxygen Saturation / 112BPM reading was during my severe illness episode this past November

88% Oxygen Saturation / 128BPM reading was my average for most of the past three years

Readings today! 99% Oxygen Saturation / 70BPM reading was just today

Since I’ve been in Trikafta, I have continued to improve. My appetite has once again increased, my food appears to better sustain me without as many wasted calories, my muscle mass has increased, my stamina has skyrocketed, and my heart flutters have been steadily decreasing during my workouts as my cardiovascular condition has continued to improve. My O2 level was steadily around 88% for the past three years, with a resting pulse of 110-130 BPM, and my O2 level went as low as 83%/84% last November, but now for the first time in my memorable life my O2 level is 99% with a resting pulse of 60-70 BPM! For the first time in years I have been able to scale the local hills with my ruck (filled with 80 pounds of weights), and not experience complete breathlessness and fatigue. For the first time in years, I have been able to mountain bike and run again, with my times already improving towards where they were three years ago. I am already back to swimming over a half-mile at a time, with only having gotten into the pool just this past week. My pullups, pushups, squats, and weightlifting have all experienced improvement, as did my performance of the Memorial Day Murph Challenge this year. So far I also appear to be tolerating the heat somewhat better than before, although the cold has never been a problem and I still enjoy it the best.

As I am currently writing this, I am now a student at the University of Alaska Fairbanks, where I am a pre-med major for veterinary medicine, and along the way I am also going to finalize my meteorology qualifications. This represent a 15 year dream of mine, a dream that was crushed three years ago, and completely unthinkable just last year. Because of this medication, along with my own dogged determination that was shaped by my closest friends and family, I have now overcome multiple close calls with death and prolonged hopelessness. I could have never done this alone, and my family, friends, and medical team allowed me to remain steadfast until a point in time when nothing short of a medical miracle gave me the chance to finally take-off and repay the favor. This is where my future is now headed, one of honoring those who kept me going, and in turn I hope that my renewed chance at life will afford me the opportunity to help make the lives of other amazing souls far more fulfilling and successful in their own right. I hope that I can help to save and prolong the lives of others, as they’ve done for me. 

My life has been renewed, and I pray that I can renew another life in return.

Thank you, and God Bless.

 

***Written by James Kain

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