It's A Wonderful World

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Laura Spiegel

The words leaped off the screen like a lighthouse in the storm.“FDA approves Trikafta for children ages 6 through 11 with certain mutations.” For most, this was another headline to scroll past. For my family, it was a lifeline. An antidote. An answered prayer.

When my daughter was diagnosed with cystic fibrosis in 2013, her physician told us that she would lead a long and full life. It was her job as a physician, and ours as parents, to help make that happen. Daily therapy would be critical. Enzymes before every meal and snack, twice daily respiratory therapy, and limited contact with others who could transmit cold and flu germs. She also alluded to a promising drug pipeline. New therapies were under development to help correct the underlying abnormality in the CFTR protein that leads to many of CF’s pulmonary and digestive challenges. Target dates were tentative and ridden with asterisks. Vague beacons of hope for a future that would one day begin.

On Wednesday of this week, the future arrived. As I sat in the CF clinic and listened to the pulmonologist explain the new medication to my daughter, I had to laugh. My daughter was so goshdarn unimpressed. Another set of pills? Okay. They could help her lungs stay healthy? Yawn.

When would we be getting out of there? Could she get a stuffed animal at the gift shop? Her giant sequined cat, attained after her last blood draw, needed a twin. Meanwhile, I sat in a daze of paperwork and chagrin. So many emotions rolled through my mind. Disbelief that this was actually happening. Unbridled joy that the day had finally come. Hesitation lest the side effects be too heavy or the benefits less than expected. The urge to high-five the members of her care team. Restraint as high-fiving is still frowned upon given COVID. A feeling of loss because there are still so many children out there who aren’t eligible to benefit from this new therapy. And a gut check. This medication, while a beacon of hope, is not a cure. Medical and scientific research must continue to march on.

Throat culture swabbed, labs drawn, lung function tests completed, questions answered. We left the office in a whirlwind, eager to hit up the gift shop before it closed.

Negotiation of the toughest kind. Yes, you just got poked and prodded to no end, but no, you can’t have the biggest animal in the place. Too expensive. No room to put it. Fine, we’ll settle for the second biggest.

On the ride home, it started to rain. We looked for a rainbow, but no, that would have been too cliche. When we heard the opening lines to Louis Armstrong’s It’s A Wonderful World on Pandora, my daughter pressed skip before I could protest. “We’ve heard it too many times, Mom. We know it’s a wonderful world.” And it is.

 

 

 

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Laura Spiegel lives in Indianapolis with her husband and two children, one of whom has cystic fibrosis. She loves a good book, a great glass of wine, and connecting with others who nurture the blessings alongside the battles. Laura is the Founder and President of Paint Her in Color, a web site that offers emotional support to parents of children with special health care needs. She can be reached at https://www.paintherincolor.comlaura@paintherincolor.comhttps://www.facebook.com/paintherincolor, or https://twitter.com/her_color.

 

 

 

***Views expressed in the CFLF Blog are those of the bloggers themselves and not necessarily of the Cystic Fibrosis Lifestyle Foundation*** 

 

***Please speak with your physician before making any changes to your CF management***

 

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