Embracing Changes in CF Management

In my last article, I discussed making accommodations for the changes CF imposes on me and many other patients. However, those aren’t the only developments we experience in life with CF. Living with CF means there is always the chance for things to get worse. Fortunately for many of us, managing CF is changing for the better recently. Advancements are being made every day in the realms of new CF treatments, medications, technology, and patient support. All these factors are leading to some significant improvements in managing CF patients’ health, some of which I’ll highlight below.

Coverage and support are improving

I’m not alone in shuddering when I hear the word “insurance.” It conjures memories of exhausting hours on phone calls arguing with reps about coverage or medication deliveries. Despite the inefficiencies within the healthcare industry, service and coverage have been trending upward.

While I can’t speak for many of the programs, I’ve worked with numerous private companies throughout my adult life. I’ve experienced the prior authorization processes streamlining to directly involve my care team. Insurance formularies add new medications more quickly. The formularies themselves continue to grow to include even more medications used in our specialized care. Systems are being implemented to improve medication shipping and delivery experiences. I certainly spend less time on phone calls with reps and departments than I previously did.

Improved affordability

Not only are the private insurance companies incrementally improving, but the affordability of our care is, too. Financial assistance programs are now the rule more often than the exception. This allows patients of more socioeconomic statuses access to life-altering treatments.

The programs provide additional benefits like nutritional supplements, support communities, and live advocates. We now have much more assistance in navigating insurance issues, as well as reminders for when to order meds. They also communicate tips on how to maintain health, nutrition, and mood throughout the year. With more resources becoming available to more of us daily, we can have additional time, money, and peace of mind while managing CF.

Increasing mobility and independence

The introduction of the first Vest was a significant improvement for me. Suddenly, I could be independent with my airway clearance therapy at home. My parents were able to free up their time and only resorted to the old patty babies on vacation.

New devices for CF treatment

The next iteration of the Vest was smaller and came with a carrying case. I could bring chest physical therapy wherever I went, making travel to and from college much easier than it otherwise would have been. Along the way, nebulizer compressors have been shrinking in size and weight while maintaining power and longevity. I got to a point where the equipment I had could give me comparable treatments at home and at a hotel for a weekend.

Recent devices like the Monarch and Vibralung are taking that change even further. Patients can now move freely around their environment without heavy equipment or being tethered to an electrical outlet.

Traveable and convenience

Every new generation of care device is also decreasing in size, making travel easier. I can now fit my portable nebulizer, antibiotics, Monarch, and inhaled meds all in the same overhead storage bag. I keep it pretty consistently packed with supplies as a “go” bag, ready for early morning travel or short-notice trips.

My Vibralung, meanwhile, has become my twice-daily commute buddy. Because of its small size and low power usage, I can operate it with no problem while traveling for an hour each way every day. My commute was simply lost time before, and now I've made it productive. I feel very excited about my growing arsenal of weapons in the fight against CF and how they help me maximize my time and effort in treatments.

Turning back the biological clock

Plenty of excitement surrounds new medications and research into CF treatment methods. Rightfully so, as the potential of treatments like gene editing and 3D printing organs would lead to significant improvement in, or elimination of, CF symptoms.

Personally, I try to resist researching too much about future treatments. That helps prevent unrealistic expectations regarding a treatment that may not even be available in my lifetime. As my grandfather would always say, “A watched pot never boils.” Plus it prevents me from too much unfounded excitement and speculation. That attitude has allowed me to avoid building up my expectations prior to beginning the newest CF breakthrough treatment: Trikafta.

Seeing improvements from CFTR modulators

I had been part of the clinical trial for Orkambi, an earlier modulator therapy, and had experienced modest improvements. I didn’t experience much change in my daily life, but my exacerbations reduced to almost none. Orkambi very much stabilized my health with regard to CF. Had I expected increased PFTs or other improvements, I may have been disappointed. I was instead able to appreciate the reduced need for antibiotics and the decelerated PFT decline.

With the advent of Trikafta, I’d heard teases from doctors and other patients about significant benefits that studies were seeing, but kept my expectations at their normal low levels. My first few weeks on the new medication blew those expectations out of the water.

Not only has Trikafta kept me stable, but my PFTs have improved and my hemoptysis has been reduced drastically. Now, even after being on it for a few months, I still wake up every morning shocked at how I feel 10+ years younger. Many other patients have been sharing similar experiences and reinforcing how significant the effects are. Hopefully, those that didn’t benefit from Trikafta will see improvements from the next wave of therapy innovations.

Positive change breeds hope

As we are all united by change, more and more of us patients can now be united by the positivity of all these and more improvements. My most recent visit with my care team had a new tone to it, a new atmosphere: excitement. Hope is something we always all have, but now that hope is being validated for many of us, including my care team. With the advent of all these new management methods, especially Trikafta, results and general wellbeing are improving for many, if not all, of us.

Are you on Trikafta? Share your experience with other CFers in the comments below!