Navigating CF parenthood during a new era of the disease

CF life expectancy has improved greatly, but nothing is ever guaranteed

Jennifer Chamberlain avatar

by Jennifer Chamberlain |

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“It’s a great time to be born with cystic fibrosis.”

I remember hearing this sentence from the head nurse during the first clinic of our daughter, Claire. At the time, it felt like they were just words meant to comfort a new cystic fibrosis (CF) mom. Maybe I was jaded because I’d already done a significant amount of research about the disease. That research had given me hope, but it’d also given me a realistic perspective on what we faced.

The reality of CF and life expectancy

While most parents don’t have to live with the nagging thought of their child’s mortality, that’s exactly what CF parents do.

Historically, CF parents received a bleak prognosis along with their child’s diagnosis. Just decades ago, in 1978, a child with CF had a median survival age of 11. And while we were given a far better prediction for life expectancy, there was no avoiding the stark reality of those numbers.

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A banner illustration depicting a woman holding her baby daughter at a beach for Jennifer Chamberlain's column

How I found my voice as a cystic fibrosis parent

However, recent advancements in therapeutic treatments have evolved tremendously, even since Claire’s birth just over four years ago. It seems like there’s positive news daily surrounding drug development for cystic fibrosis.

While CF patients born today are expected to live into their 50s, just last month Cystic Fibrosis News Today reported on a study that showed people with CF who begin the newest generation of medications in adolescence may live nearly normal life spans.

This progress is largely due to unprecedented medical breakthroughs and the introduction of highly effective modulators. Modulators are medications that help to increase functionality of the CFTR protein, which is affected by CF-causing mutations in the CFTR gene. To put it simply, these drugs treat CF on a cellular level to mitigate symptoms of the disease.

Modulators have greatly improved the lives of many CF patients. But they’re not a cure, and not everyone with CF has access to them.

The modulator generation

I’ve heard my daughter’s age group referred to as the “modulator generation.” Claire and her peers who are eligible to take a modulator are the children benefiting from these drugs from infancy and toddlerhood. I’m grateful that our daughter has the mutations and resources to access a modulator at such a young age.

But at a time when there’s so much hope for the CF community, I also feel the need to have realistic expectations for Claire’s life. As a lawyer, I analyze risks and liabilities in my professional life daily. For that reason, I consider all the objective data around CF drug developments in the context of how my daughter’s disease is presenting.

It’s hard to know exactly what the outcome will be for those of us with children who started a modulator at a young age. We’re living in unprecedented times with groundbreaking drugs. The generations of people with cystic fibrosis before us didn’t have access to these advancements at such an early stage in life.

But no one truly knows what will happen long term. And those life expectancy numbers aren’t one-size-fits-all. There are still people with CF passing away far too young.

Most days, I don’t think about Claire’s life expectancy. I choose to focus on the positives regarding her future. We have been the fortunate recipients of these modulators that patients, parents, doctors, and scientists worked tirelessly for. Not everyone lived to see these therapeutic drugs come to fruition. But it’s not always an easy choice to focus on the positives when faced with the realities of the disease. There are no guarantees with CF, or with life in general.

Note: Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Cystic Fibrosis News Today or its parent company, BioNews, and are intended to spark discussion about issues pertaining to cystic fibrosis.


Sarah K avatar

Sarah K

This. Exactly this. My oldest daughter has CF and is 15. We've witnessed the development of each modulator and the hope that comes along with them. When my first was born, life expectancy was 37. Along with the news of her diagnosis, I was told to plan on her future - plan for her to go to college, plan for her to get married... plan for her to live. So, I pray, and I plan. I'm hopeful and I'm terrified. I grieve over the CF friends we have lost along the way, and I hold onto my daughter a little tighter. I cherish every moment, and I'm so thankful for the true gift that my daughter is. I'm also so thankful that the scientific community is not satisfied with the (amazing) modulators that we have and are continuing to search for a cure. I pray that one day CF = Cure Found.

Jennifer Chamberlain avatar

Jennifer Chamberlain

I agree its a cautious optimism perspective. I try to do the same :)

Gisele F Lapointe avatar

Gisele F Lapointe

I shared your enthusiasm and hope that these new modulators will finally vanquish this awful disease. Boy, how things have changed since my daugther passed away, only 20 years ago, at age 43, after a two-love implant. I keep telling myself that if only she had been born 10, 20 years later, she would have lived to the age of at least 70. Things are looking up, don't give up hoping.

Jennifer Chamberlain avatar

Jennifer Chamberlain

I am so sorry to hear about your daughter. I can only imagine how difficult it must be to see these drugs are developed. I am very grateful my daughter is a part of the modulator generation but feel for you CF parents that came before us.


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