I had just pulled into the airport parking lot when I got the call. There was a national conference on some ground-breaking issue in some big city that I needed to attend as part of my new job as Deputy Commissioner of Something Very Important. Ten years later, I can’t remember the issue, the city or my precise title, but I remember exactly what my mother said on the phone:
“They’ve found a pair of lungs that match. She’s getting prepped for surgery right now.”
I went inside the terminal and canceled my flight.
My niece Cecelia is one of the 70,000 people on this planet who have cystic fibrosis. You have to beat the odds to get it. You need not just one, but two parents who, probably unbeknownst to them, carry the defective CF gene. Even then, your chances are good you won’t inherit the double-whammy of that defective gene from both parents; only 1 in 4 children of CF carriers do. My niece was that fourth child.
Three of four children are diagnosed with CF by the age of 2. My niece beat those odds, too. She was almost 7 when her parents concluded that there had to be something more to her constant infections and persistent coughing. In 1992 they had her tested and we all began learning about cystic fibrosis.
Here’s what the Cystic Fibrosis Foundation tells you:[The] defective gene causes a thick, sticky buildup of mucus in the lungs, pancreas, and other organs. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage, and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients.
For Cece, the primary impact was on her lungs. The infections and coughing continued throughout her childhood. Most days began with her mother or father pounding on her back to loosen up the mucus so she could breathe more easily. By the time she was in middle school, a mechanical “vest” had been developed to relieve parents of the awful duty of beating their child every morning, but the vest wasn’t covered by her insurance policy. It was viewed as a “cosmetic” treatment, a non-essential expense.
Not infrequently, the infections worsened, requiring prolonged hospital stays. Uncommonly bright, Cece wiled away the hospital hours studying and lying in bed beating the pants off the contestants on Jeopardy and Wheel of Fortune.
In high school, sports were out for her, but she still followed them avidly. She had already become a die-hard New Orleans Saints fan. She and her brother began a feud with my son Paul that continues to this day over the relative merits of Duke sports. Paul lived and breathed Blue Devil basketball. Cece loathed all things Duke. This evidence of bad judgment notwithstanding, she excelled in her high school honors coursework and was admitted into the National Honors Society her junior year. Her senior year, she was healthy enough to go to Prom. Her oxygen tank went with her.
After high school, she enrolled in the honors college at the University of Montana. She and Paul shared an apartment for most of those college years. Other than one spat over Duke, the explosiveness of which took them both by surprise, they got on famously. Cece continued to excel academically, but her lungs were failing. The oxygen tank was never far away, and it could only help so much. She couldn’t sleep at night because she couldn’t lie flat. Functionality meant sitting upright, her tiny frame curled around her lungs, coughing. Although she graduated with high honors in 2008, she didn’t attend commencement. She wouldn’t have been able to make it from her seat to the stage to accept her diploma.
Her parents were desperate to get the double-lung transplant that could save her life, but the obstacles were daunting. In spite of an expertise that qualified her for jobs that would pay far more, Cece’s mother had stayed in the government job she held when Cece was first diagnosed. Why? Because leaving that job would mean applying for insurance with a child who had a very expensive pre-existing condition. Cece’s mom had to stay put. Now, with Cece’s legal independence fast approaching, soon Cece would “age off” her parents’ policy, becoming for all practical purposes uninsurable.
That wasn’t the only challenge. Lung transplant facilities were few and far between; they had to be picky about their patients. Cece was not a good risk. The damage to her lungs was extensive, and the years of expensive care had put her perilously close to exceeding the lifetime cap on her insurance policy. Anticipating setbacks beyond the surgery, many university hospitals required $1,000,000 coverage. Cece’s policy only provided $500,000. Stanford turned her down. The University of Washington did too. Just when they had begun to accept the inevitable, a university finally said yes. It was Duke.
In June 2008, Cece and her parents moved to North Carolina to race time. It typically takes a tragic accident to get a lung donation – not something one wishes for. And yet … in mid-July, an accident occurred; a set of lungs became available. Parents, relatives, and friends held their breath as Cece waited for the lungs to arrive and the difficult surgery to begin. We all then exhaled sadly: The lungs were not a match. But on August 3, 2008, the second donation came. My mother called me with the news on my way to the airport.
As you age, time goes by faster. It seems like only a year or two, not ten, since I left that airport and went over to wait and worry with my folks. I haven’t been Deputy Commissioner of Something Very Important for eight years. My mom and dad are both gone now.
But Cece isn’t. She made it through that surgery. When she came to, her family was there to greet her. A friend from school made a special trip to see her. But in a few days, he had to go back home. During the next month, her brothers and sister had to return to college and jobs. The month after that, her mom had to get back to work. Through the summer and all through the fall, her father stayed, first visiting the hospital to see her and then staying with her in an apartment nearby as she continued the visits and tests and therapies involved in adjusting to the two foreign but vital organs now implanted in her body.
That was a dark, difficult time. They say that when you get an organ transplant, you’re just trading one set of terrible symptoms for another. I can’t pretend to grasp it. I just know that Cece was weak, sick, and struggling for months in North Carolina and would suffer for months more when she came home.
When you’re young and ill, time goes by slowly. In that little apartment in Durham as winter approached, Cece didn’t know what lay ahead. She didn’t know that the charismatic Chicago lawyer who just got elected president would make sure she could stay on her parents’ policy for a few more years. She didn’t know that he would make sure that lifetime caps on insurance policies would be eliminated. She didn’t know that by the time she recovered fully, she could get a job and change jobs – even with her pre-existing condition.
She didn’t know that the young man who had come to her bedside so soon after the surgery would become her husband in 4 years. She didn’t imagine that every year from then till now he would photograph the young woman who couldn’t climb the stairs to get her diploma hiking to the summit of this mountain or that one on her “lungiversary.” She didn’t dream that in 10 years – this week – brothers and sisters and uncles and aunts and cousins and nieces and nephews and friends from far and near would gather to toast the girl who always, somehow, beats the odds.
When you have good health, you don’t know how difficult even a simple thing like breathing can be. I was reminded of that fact yesterday as I listened to the recording of Cece testifying against the “Obamacare nullification” bill introduced in the 2011 Montana legislative session. For over an hour in the jam-packed committee room, Tea Party “Obamacare” opponents laid out all the reasons the republic would soon collapse under the weight of the Affordable Care Act: the demise of the 10thAmendment, forced vaccinations of mercury, the new slavery of socialism, the gutting of the commerce clause, the ignored lessons of Exodus.
Then Cece stood up, the only private citizen there to support the ACA. In a voice trembling with nervousness, in words punctuated by that telltale cough, she explained why. No scripture. No ideology. Just the story I told you here.
In the presence of such an authentic account of how the ACA was saving lives like hers, making lives like hers livable, you’d think the sponsor would have quietly withdrawn his bill and slunk away. But no. As we have learned in the last decade, ideology trumps reality. The sponsor closed the hearing by recalling the good old days. The days when your mom took care of what ailed you “unless she couldn’t stop the bleeding.” When neighbors stepped up to help, bringing firewood and such to the infirm. When personal responsibility kept you from going to the emergency room “every time you got a runny nose.”
Egad. That a gentle, well-meaning man could be so cavalier! A testament to the ignorant bliss of good health.
When Cece was born, the life expectancy for someone with CF was less than 20 years. Cece is 32 now. She has a new set of lungs that differ genetically from the rest of her body, but she still has cystic fibrosis. She will always be at risk of infection and will always take antibiotics and immunosuppressants to keep infections at bay.
One of the drawbacks of having such a rare disease, a disease that kills people too young to have made a mark on the world, is that it’s harder to raise the level of awareness and concern that generates funding for research. It took researchers 20 years from the time the AIDS virus was identified to develop the autoretroviral therapeutics that now allow people with AIDS to live long and stable lives. The first scientific paper on CF came out in 1938; it wasn’t until this last decade that CFTR was developed, a modular therapy that shows great promise for correcting the defect at the genetic level. However, it works only on a few specific mutations; the CF gene has over 1,300.
Nonetheless, Cece remains optimistic about the development of drug therapies that will successfully target all 1,300 mutations. “I think it’s just a matter of time,” she told me, and I believe her. This amazing creature is never wrong. Well, maybe just that once. She really blew it on Duke.
Mary Sheehy Moe retired from a career in education in 2010. She lives in Great Falls.