Learning to live with cystic fibrosis

OKLAHOMA CITY — I’m pushing my two-year-old son in a shopping cart at a discount grocer when we hear the chest-rattling hacks nearby.We both turn around.

“Sick! Sick baby!” Eli says, pointing.

“Shh shh shh, it’s OK,” I say, pushing his finger down.

We walk past the toddler as a fit rages through his tiny body. His parents pat his back.

A look of disgust crosses one shopper’s face. “Why would they bring him out?” utters another. Others just stare.

I only have this thought: “Maybe that child has cystic fibrosis, like my son.” I get angry as I watch them gawk. I want to yell: “Stop staring. Stop judging. It could be a genetic disease. Genetic diseases aren’t contagious. What do you know about that child?”

Instead, I slink away, wondering when Eli will have his first experience like that, one that makes others stare in some combination of judgment and disgust. What would I do if I were there? What would I say? Would I tell them to stop staring at my child or would I move along without a word?

My son’s illness sometimes drives me to the badlands of my mind. Cystic fibrosis, which Eli was diagnosed with soon after birth, is a fatal and progressive genetic illness that obliterates lung functioning over time and often claims people in their 20s.

At the moment, he has a mop of floppy golden curls, a gap-tooth grin and a fierce love of trains. The science to cure cystic fibrosis is moving fast, but I don’t yet know if it will be fast enough for him.

This time, I manage a quick escape from negative thinking. Wondering if or when a bad thing is going to happen, I’ve learned, wastes energy. It wastes time. And I don’t know how much time Eli and I will have together.

Eli Keeping
Eli Keeping
Courtesy Juliana Keeping

Meanwhile, in Chile, a schoolgirl with cystic fibrosis begs to die in a “euthanasia plea” she posts on You Tube.

Valentina makes her request from a hospital bed. She’s thin and pale with big, serious eyes. She takes oxygen through tubes in her nose.

I’m Valentina Maureira. I’m 14 years old. I suffer from cystic fibrosis, she says.

I need urgently to speak to the president. I’m tired of living with this sickness and she’s the only one who can authorize euthanasia for me. I want her approval so I can get a shot that can make me sleep forever.

Some are compassionate, others outraged. Pundits weigh in. Her friends campaign for her right to die. Yet most people miss the headlines — few seem to notice or care.

As is the case with Eli, the mucus in Valentina’s body is thick and sticky. Their lungs accumulate bacteria and viruses that would otherwise cycle out of a healthy person. Even a small cold can lead to a bad infection that threatens to scar lung tissue. The way things are now, so long as they fight illness after illness, the day will come when Eli and Valentina will no longer be able to breathe.

Even though I knew this, I’d never considered a scenario in which my son would decide to stop fighting his illness. Eli will possess the ironclad desire to keep breathing, won’t he? I don’t want to bury my little boy.

Valentina’s story

I’m on the phone with Valentina’s father, Fredy Maureira, a 53-year-old who works in the laboratory of a chicken farm. Valentina’s mother, Jaqueline, stays at home. In addition to Valentina, the couple has a 23-year-old daughter, Karmine, who has a 5-year-old son, Vincente. They live in a town southeast of Santiago called Melipilla. The Pacific Ocean is a 40-minute drive west.

Melipilla is full of chicken farmers and factory workers, Fredy tells me. There has never been proper medical care for Valentina in town; the family has always traveled to Santiago. When Valentina is 5, Fredy breaks the news. He tells her that she had an older brother, Mike, who died of cystic fibrosis, the same disease that she has. Fredy describes her reaction as intelligent, aware.

Valentina, a happy child, takes the bad news in stride.

She’s got things to do.

She rides a black horse without a name through fields near her home. She grows her fawn-colored hair long, dances with friends in the living room to Daddy Yankee and cheers for the Chilean soccer team Colo-Colo. Meanwhile, her parents beat on her back, chest and sides daily to purge her of mucus.

Eli receives a similar therapy, but it’s automated. For an hour a day I strap him into a vest with two dark blue ridged tubes hooked to a bread-box-sized compressor. When I press a button it inflates and pounds him. At two, he often screams, hides or throws himself down when he sees me coming with his vest, which has an Army fatigue motif.

Doesn’t she hate that, the pounding, I ask Fredy?

“She got used to it,” he says.

Like my son, Valentina also takes enzymes to grow and keep on weight. Without the enzymes, neither would be able to process fats in their pancreas, and both would either starve or succumb to illness. The disease can mess with other organs too, though I refuse to think about this.

Valentina Maureira in the hospital in Santiago
Valentina Maureira in the hospital in Santiago
Courtesy Maureira family

I learn that Valentina’s trips to the hospital are nothing like Eli’s.

“Here, there are so many kids that they are crowded in the hospital rooms,” Fredy tells me. “The waiting rooms are crowded. It’s very disorganized.”

Cystic fibrosis clinics throughout the United States adhere to a different standard of care. When I take Eli to the lung doctor, the receptionist hands me a brand new pen I keep with me throughout the visit to avoid the transfer of germs. Nurses and his doctors slip on bright yellow protective gear before they swab his throat. His antibiotics are tailored to his most recent lab results. Certain bacteria are extremely harmful to cystic fibrosis patients, and if detected must be treated with an aggressive regimen of drugs.

When Valentina is six, her father makes a trip to the United States. He applies for a visa so she can travel with him and receive better medical care. It’s denied.

When she is 10 she gets very sick, Fredy tells me. Trips to Santiago become more frequent. Her parents always sleep on the hospital floor. When healthy, she attends school but has little interest in friends her own age. She is always with her older sister.

At 13, Valentina becomes determined to live, though by this point she needs a double lung transplant to keep breathing. No organs are available, and the family can’t afford a transplant. She campaigns by handing out fliers outside of a Santiago hospital. Though the family is featured on local news reports, fundraising efforts bring in “very little,” Fredy says.

It’s getting harder and harder to breathe. Valentina can’t keep weight on. The headaches come all the time. She spends much of 2013 in the hospital.

In February 2014, after nine months in the hospital, she is ready to die.

She posts her plea to YouTube one night. Within two hours it has been viewed 25,000 times. At 7 a.m. the next morning the family starts getting calls from Israel, Japan, Britain, all over the world.

Her heartbroken parents fall apart but never question her decision. We support her “100 percent,” Fredy says. She is “smart and aware and we want to respect that.”

Valentina Maureira with Chilean president Michelle Bachelet
Valentina Maureira with Chilean president Michelle Bachelet
Courtesy Maureira family

The media visit her in the hospital, and one reporter tells her she will be cured.

“Si,” she says. She manages a quick smile and casts her eyes downward. You sense she’s just being polite.

Valentina is excited but also scared and overwhelmed by all of the attention. The family hire bodyguards to stand outside her hospital room. She receives a visit from the Chilean president, Michelle Bachelet.

Valentina tells the president she wants to enjoy life.

There is no money. There are no organs. Valentina is too small to endure any transplant.

She has little breath to waste and she has a message. Her requests are humble; she’s not asking for medical breakthroughs or a cure, like I am.

She wants housing near hospitals throughout Chile so the parents of sick children don’t have to sleep on hospital floors. She dreams of a marathon — a big one — tied to cystic fibrosis, and she wishes for a book and a movie to be made about her life. But more than anything Valentina wants to see the formation of public clinics in Chile to help children like her, but with all kinds of rare diseases, not just cystic fibrosis.

She has always been like that, a humanitarian, her father tells me.

Valentina does not end up getting the shot to help her sleep forever.

Her breath grows weak; she strains to stay alive through quick, shallow pants. “Help,” she says. “Help. Help.” Those were her last words. On May 14 she succumbs to the same genetic illness my son carries.

What the future might hold

I wondered after Eli and I left the supermarket why people are so quick to judge, instead of looking at a sad or confusing person with understanding — or at least a desire to understand.

I am putting my son to bed now. He wears dinosaur pajamas and a goofy grin. I put on “Rainbow Connection” by Kermit the Frog, who strums along on guitar.

Each night, as I put my little boy to sleep, soulful Kermit, the everyfrog, contemplates the meaning of life with us.

I think about Valentina sometimes, her life, her dreams. Even in failing health, she was kind. Valentina never lost hope. She was only being realistic. Her lungs failed but not before she gave the world her to-do list. She never once stopped believing, stopped insisting in her quiet, determined way, that things could be better for kids like her. She gave hope, even as death drew nearer with her each passing breath.

“Someday we’ll find it, the rainbow connection, the lovers, the dreamers and me,” Kermit sings.

Valentina found it. What else is there?

I kiss my son goodnight.

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