Last year, my daughter graduated from college. She recently started her first full-time job and is apartment hunting with her best friend. These milestones, bittersweet for most parents, feel monumental to me. As my daughter steps into her future, our family of three stands on the precipice of a life we didn’t dare contemplate before now. Until recently, I still believed I would outlive her.
My daughter was almost 2 when we learned she had cystic fibrosis, a progressive genetic disease that affects breathing, digestion and other functions while slowly destroying the lungs. She had been dropping weight for months, her once plump arms reduced to sticks, her belly distended. A sweat test detected CF, which a blood test confirmed.
“The good news is the life expectancy for someone with CF is almost 31 years,” her pediatrician said.
My husband and I sat in stunned silence. I was 31 at the time.
CF is caused by a mutation in the gene that controls how salt passes through cells. This causes a buildup of thick, sticky mucus in the airways that impairs breathing and leads to lung infections. It has been described as trying to breathe through a narrow straw all day long.
After her diagnosis, my daughter’s daily routine was daunting. She took more than a dozen prescription drugs, including pancreatic enzymes at every meal to digest her food. She learned to swallow capsules as a toddler after watching me take a birth control pill. We celebrated with high-fives when she made her enzyme “all gone.”
She did twice-daily respiratory therapy, breathing a nebulized cocktail of drugs to thin the mucus in her airways, while an oscillating vest on her little body shook the mucus loose. When she was small, we pounded on her chest and back with cupped hands, distracting her with videos of “Barney & Friends.”
“Treatment should be a treat, right Mommy?” she’d say, parroting what I told her.
My husband and I worked as a team. We acclimated the way parents do when living with chronic illness, a current of anxiety always humming in the background. As her mom, I was more than a caretaker. I was the eyewitness, record-keeper, connector of dots, making sure every doctor, nurse, insurance provider, pharmacist and teacher was aligned.
Raising a child with CF was like driving through a blizzard. Gripping the wheel, it took all my strength and determination to keep from careening off the road. I focused only on what I could see in my headlights, relying on blind trust to get through the most difficult patches. I was terrified to look too far ahead.
For a while, my daughter’s lung function held steady, and it felt like we were in control. Her bimonthly sputum cultures sometimes tested positive for lung infections, which we treated with more drugs and rounds of airway clearance. Only once in the first decade after her diagnosis and initial hospitalization did she need to be admitted for a grueling two-week course of IV antibiotics — what we in the CF community called a tuneup. Many kids with CF were hospitalized yearly or even more often.
I knew people died young from CF, but I refused to believe she’d be one of them. Then my worst nightmare struck my inner circle — a small group of local CF parents who leaned on each other for emotional support — when I was jarred awake by my phone ringing at 3:45 a.m. My friend’s name glowed on the caller ID.
“Kath? What’s wrong?” I said.
“The hospital just called.” Her voice was a monotone. “Sam’s gone.”
Kathy’s daughter Samantha was 22, a college senior. She’d flown home for Thanksgiving break and checked into the hospital for a short course of IV antibiotics to help clear out a lung infection. It was a routine admission, no red flags. She was supposed to come home.
Sam’s death was a devastating gut punch. I was probably going to outlive my child.
Our family life was structured around my daughter’s disease, a constant checking of boxes — meds refilled, therapies completed, blood tests and chest X-rays scheduled, pulmonary checkups every two months.
Still, I never treated my daughter as if she had a fatal illness. I saved my rare emotional breakdowns for the privacy of my shower or a phone call with a CF mom-friend. My husband and I saved for college, talked about the future.
To reduce my daughter to a diagnosis was to dismiss everything about her. Her intelligence and sharp wit. Her ability to make me laugh so hard I cried. Her creativity and kindness, the strength of her will.
In middle school, she developed a chronic cough. Laughing hard would reduce her to a coughing fit. Her doctors adjusted her medications but there wasn’t more we could do; coughing is a defining characteristic of this disease.
In high school, my daughter and I argued regularly as she pushed back against my hypervigilance. The more she struggled for independence, the more tightly I held on. My ability to compartmentalize my fears began to crumble. If she missed a respiratory treatment, I went ballistic, accusing her of being cavalier with her life.
The truth was, I was terrified.
The summer before her senior year of high school, my daughter was hit with a triple lung infection. She was hospitalized, given intravenous antibiotics and airway clearance therapy every three to four hours, around-the-clock. Despite weeks of intensive treatment, first at the hospital and then at home, she didn’t bounce back as we had hoped, her lung function unable to rise above its new, lower level.
At 18, her routine included hardcore prescription pills with terrifying warning labels and insulin shots for CF-related diabetes, a common development. It felt like my daughter had veered down a path with narrowing possibilities.
Another friend’s daughter succumbed in her 20s. At her funeral, I sat between two other CF moms. We were the lucky ones whose daughters were still alive. Silent, we stared ahead, clutching one another’s hands. I envisioned the three of us on a rowboat, in the middle of a storm-tossed ocean, sharks circling — waiting to tear us apart.
At 22, my daughter joined a clinical trial for a triple-combination drug to target the underlying cause of CF, its mutated protein. For years, we’d heard about this new generation of drugs in the pipeline that focused on restoring that protein’s function. Less powerful versions had come to market for smaller CF populations with rare mutations and those were game-changers. This new drug would address the most prevalent mutation, and the Phase 2 trial results looked promising.
For the first few months of the Phase 3 trial, nothing changed for my daughter. We assumed she was among those taking the placebo. Then the study entered its open-label stage when all participants received the drug. Within hours of taking her first pills, my daughter began coughing violently as her airways were purged of mucus. Within a week, her lung function skyrocketed, and her chronic cough disappeared. Within months, her blood sugars normalized, and she could stop injecting herself with insulin, although she continues on other medications.
The drug, Trikafta, received approval by the Food and Drug Administration in October 2019. It arrived like the hero in an action film, snipping the wire on a ticking time bomb with only seconds remaining.
I was an emotional mess the week Trikafta was approved. It felt like I’d been holding my breath for 20 years and could finally exhale. I inhaled stories about young adults with CF suddenly moved off transplant lists or able to have kids. My daughter got tired of hearing me talk about it.
“I know you’re excited about the drug, and I get it, but you have to understand. I was never afraid of dying from CF,” she said.
I sat with that a moment.
Parenting a child with a chronic illness is, in many ways, like parenting any child. It requires trust and a willingness to live with uncertainty. Eventually, you have to let go and hope your child has absorbed the lessons you tried to impart.
“Good,” I said. “That means I did my job well.”
My daughter has grown into a remarkable young woman. Strong, confident and capable. She no longer needs me to be her caretaker. I can just be her mom.
Abby Alten Schwartz is writing a memoir about parenting, illness and hope.