It was January, 1998 when Rebecca told me she had cystic fibrosis. She told me right away and I pretended to know what it was. Clearly I wasn’t very convincing because she promptly gave me an 8-page technical document describing it at both a clinical and molecular level. I felt confident I understood what it was and then a little over a year later she got pneumonia. It was one thing to read about it but another thing to see it really impact her. As I’d assume with many other CF spouses and significant others, it scared me but didn’t scare me away.
It was 2003 when we got married…or from Rebecca’s perspective “finally got married.” According to her schedule, I should have proposed much earlier, like perhaps when her 25th birthday came along. In retrospect, I shouldn’t have been surprised when she wasn’t overly thrilled about that sweet mountain bike I bought her. She was expecting something much smaller. In any case, about two years after we got married we decided to start a family but it turned out to be a false start. We tried everything from IUI to IVF. In the end it just wasn’t in the cards for us.
Rebecca continued to decline a little bit each year. She’d require tune-ups more frequently. Then she needed oxygen when she slept. In 2014 she declined sharply and needed O2 around the clock. I remember her calling me from downstairs one night because she was too weak and out of breath to make it up the stairs on her own. Then on New Year’s Eve she went into respiratory failure and was placed on a ventilator in a chemically-induced coma. The pulmonary team set her odds of survival at days to weeks. As pro-active as we thought we had been, we were unprepared. She was not yet listed for transplant and now she was too sick to be considered.
She remained in the coma until February and then woke up on a ventilator with a partially collapsed lung. She couldn’t walk, talk, or breathe on her own but there was a silver lining: She was awake and regardless of what was to come, we had that day. Then we had another day…and another. That feeling that each day might be the last was both terrifying and motivating at the same time. We played music, got take-out, and worked together on her physical therapy. Fortunately, Rebecca focused on the ‘here and now’ leaving her more concentrated on her pillows and the temperature in the room than her chances at transplant. I was happy to help her with this small stuff as I did my best to find a transplant center that would accept her. I think that’s what made it work for us, the fact that she is so tough and that I’m a bit of a strategist.
With the ventilator attached to a trach below her vocal cords, I had to read her lips. We actually had some good laughs during that time about how she would say everything fast except for the things she REALLY cared about…t_a_p_i_o_c_a pudding and cheeeeeese Danishes. She also had a neighbor that had a lot to say and often chose to spend some time yelling loudly about her butt. From doing apple-presses for physical therapy to playing “Eye of the Tiger” before her walks, we tried to have fun.
She beat the odds surviving 171 days on a ventilator. During that time she dealt with pneumonia, a collapsed lung, aspiration, severe muscle atrophy, sepsis, ICU delirium, panic attacks, and ECMO to name a few things. But she was lucky. She had (what she describes as) an army of guardian angels. Then on June 18, 2015, she received a life-saving transplant in the nick of time. Through it all, she was a fighter and I was proud to be there in her corner.
When she was admitted to the hospital in December 2014, we had no idea that she wouldn’t get out until 219 days later. Sometimes looking back, the whole journey seems surreal. Our focus since then has been to share her story widely because we know it would have helped to hear a similar story as we were going through those trying times. More than anything, it gives me empathy for people at all steps of their journey. I hope that one day others won’t need to look to Rebecca’s story for inspiration because CF will be a thing of
the past. Until then, we’ll keep doing those apple presses.