Rachel Hill

It’s May, Cystic Fibrosis Awareness month, the time when we shout a little louder for a cure! It’s not always easy to share about our experiences with CF. It feels vulnerable and even exhausting, but I try to show up in the best ways that I can, that year and at that time in our journey. This is our eleventh year fundraising and walking for a cure for CF and you might think that I have said all I need to say already, but let me share a little about why that isn’t true.

Five years ago, my children’s health and outlook was so much different than it is today. Lucie and Xavier spent that school year alternating infections and admissions, and we were literally in the hospital every other month for a while. In May of that year, Lucie cultured a terrible pathogenic bacteria for the third time, the crushing culmination of two very extensive failed eradication attempts.

To be very blunt, we were confronted with the reality of our children’s mortality. I knew what could happen to Lucie’s health if—no, when, this bacteria became resistant in her lungs. I had discussions with our doctor about experimental phage treatments. I realized that a very, very small percentage of hospitals in the nation would consider a lung transplant for this particular strain (and no, the leading hospital we were at was not one of them). Our doctors were perplexed as to why our daughter who had “mild” mutations kept getting sick. But at our last appointment with our dear doctor that walked through this time with us, she offered a glimpse of HOPE. Even though our daughter was colonized with this bacteria, maybe, just maybe, the triple combination of modulator medicines that was still in trials could be the thing that could save Lucie.

I will be honest. It’s hard to hold on to hope sometimes. Hope means that you are opening your heart to be crushed again if it fails. Sometimes it just feels too hard to hope. But we tried.

Xavier started the triple combo Trikafta in November 2019 as soon as it was released for the 12+ age group and finally, Lucie started Trikafta in July 2021 when it was approved for ages 5-11. This drug has been close to a miracle for them. In the past, Lucie was hospitalized or on strong oral or inhaled antibiotics frequently after just picking up any cold or virus at school. But now, they have avoided hospital admissions for close to five years and we haven’t seen that bacteria again for several years either.

You might think we are content right now with how far we have come, but we aren’t there yet. Why not? My kids still spend hours a day doing treatments and deal with complications of CF frequently. We don’t know the long-term effects of their miracle medicine. And more significantly, ten percent of people with cystic fibrosis do not qualify for Trikafta because of their particular genetic mutations and have no access to any modulator medicines that treat CF at the cellular level. Trikafta is like any other medicine–it has side effects that have caused others to have to stop taking it, even if it was having positive effects on their pulmonary health. Crippling anxiety and depression, elevated liver enzymes, cataracts. Not necessarily common side effects, but heartbreaking for those who have lost access to this potentially life-changing medicine.

I try to put myself in the shoes of those who are still waiting for their highly effective modulator medicine. As a family and as a foundation, we are committed to continue in this fight until all have access to a modulator or a cure. UNTIL IT’S DONE.

Please stay tuned this month as I share a little more about our life with cystic fibrosis! We are eager to not just fundraise, but also to educate and advocate. Every dollar, every listening ear, every voice that speaks up in support–they are all so vital to the cause. Thank you from the bottom of our hearts!