One year ago today…
It has been 365 days since my life has been forever changed. I had just returned home from a week-long trip to Los Angeles (remember when we could travel freely) to promote The CF Warrior Project. I was very nervous about starting Trikafta, a drug that had been approved a month earlier by the FDA. I was very skeptical as I’d taken many drugs in my lifetime but none of them made the sort of difference that Trikafta was expected to make for me. This was the first breakthrough cystic fibrosis transmembrane conductance regulator (CFTR) modulator that I was able to take as I only have one copy of the Delta F508. My other mutation is a nonsense mutation called the W1282X, which is prevalent for Ashkenazi Jews.
Trikafta works this way. My chlorides all my life have not been able to come to the surface because they were blocked by my two gene mutations that screw up the salt and water content in my body. This causes the mucus to thicken in my lungs and other issues namely with my digestive system and sinuses. Trikafta allows the chlorides to come to the surface and virtually correct this. While this is not a cure, it’s certainly a difference-maker.
I took my first dose the morning of November 24, 2019 and joked that I could feel a difference immediately, but the truth was that I didn’t notice anything for about three days. It was around the third night that I noticed my cough was less of a congested cough but more of a productive cough. I could not stop coughing that night and was up until about two or three in the morning. The cough began filled with yellow and light green mucus which has been commonplace for me for years. Eventually it transformed into a clear liquid-like mucus which was quite unusual. This cough lasted for two to three days. The mucus has not turned back to yellow or green in the last year. Other side effects that I saw were severe stomachaches and diarrhea that lasted for a day. Then came the good side effects.
For the first time in my life, I was able to take deep breath after deep breath. I could run without coughing. I didn’t cough anymore when I laughed. I gained about seven pounds in the first week as I realized that I would now have to eat like a “normal” person instead of the garbage compactor I had become with cystic fibrosis as it was so difficult to absorb fats in the past. The biggest difference was seeing my pivotal pulmonary function results which went from the low 80 percent FEV1 (amount of air you can breathe out in the first second) to the low to mid 90 percent FEV1.
I have been doing blood tests every 3 to 6 months to make sure my liver function is cooperating with the drug as that is a major side effect and has forced others to take breaks from the drug. My function has gone up some but not enough to be considered dangerous.
As I sit here thinking about the past 365 days, I’m grateful to Vertex, the Cystic Fibrosis Foundation and everyone who worked so hard to get this drug to the market. I am also sad for all of those CF warriors who never were fortunate enough to benefit from this drug because they lost their lives to cystic fibrosis. I will continue to fight for those amazing fighters like Claire Wineland, Leann Ott and my own sister Wendy Lipman.
I also feel obligated to help those who cannot be on the drug either because they are too young, had a transplant, live in a country that access is not available or because their genotypes don’t allow for use of the drug. I will continue to help the CF Foundation and other CF based foundations around the world to help these people to see similar and even better results than I have had.
It has been a crazy year quarantining during a pandemic when for the first time I want to see the world from the perspective of someone who can finally breathe freely. I will do it again one day soon but until then I will do the “right” thing and mask up, practice social distancing and maintain good sanitary habits.
Stay positive everyone. Good things are still coming.
Live your dreams and love your life.
Best Wishes,
Andy