Mom defies cystic fibrosis odds

DELTONA, Fla. (AP) - Danielle Tirella's daughter Alyssa is 7. That is the same age the mother wasn't supposed to see. She wasn't supposed to live beyond 7. Then medicine advanced and it became 12. Then 15. Here she is, now 28, a mother still facing a death sentence. Maybe she can make it to 34.

For as long as she can remember, she has been sick. She was diagnosed at 4 with a fatal genetic lung disease called cystic fibrosis.

Her lungs produce thick, sticky mucus that restricts her airways and can cause digestive problems. To counteract the mucus, her life has become a regimen of taking 30 daily pills, sitting for treatments and following doctor's orders. No running. No alcohol. Eat certain high-calorie foods.

The daily treatments are remarkable. Ever since she was a small child, she had to have her dad or stepmom beat on her chest, sides and shoulders with a gizmo they called "the hammer" for an hour twice a day to loosen the mucus, all so she could cough it up. Today, she wears a special vibrating jacket that produces the same results in less time. But all that pounding has taken its toll.

"My body hurts so bad it feels like I have been beaten with a metal bat," she said in a handwritten journal. "I have to grit my teeth and squeeze my nails into my hands, into tight fists, and get up ... That's about the point I say ... 'OK, sweet relief. It's pain-meds o'clock!"

Now she has learned her lung function has dropped to 30 percent, triggering a whole new set of dire projections.

She could die in a year.

The last resort is a double-lung transplant. Sunday night she headed to a Jacksonville hospital for two weeks of daily tests, in hopes she will be deemed a good candidate for a transplant. The tests are daunting. She will have to be up before 5 a.m. to get ready for them each day, and her whole body will be prodded and poked in ways it never before has.

If, in the end, she is deemed a good candidate for a transplant, that could buy her another five or six years.


FACING DEATH ALL HER LIFE

Danielle Tirella was born on July 9, 1982, in Queens, N.Y. Her family -- headed by father Steve, an electrician, and stepmother Shirley -- moved to different parts of Florida and settled in Deltona when Danielle was 11.

The whole time she was sick.

Steve, her dad, didn't know what to think. "You're told the average life span is 9 years old. You prepare," he said. "Now she'll be 29 years old. Things have changed."

So in some sense, talk of Danielle's impending doom is no different than it's been much of her life -- things looked bad for awhile, but got better. She survived.

Reality didn't let her get too carried away, though.

When she was a teenager, she made friends with a fellow cystic fibrosis patient named Ryan.

"That was kind of like, he was just here and in the hospital ... and he's dead," she said. "That was a grim reminder we don't last that long."

When she was a bit older, Danielle was sick again, coughing up blood for three weeks. While in the hospital, she befriended a girl named Heather.

A couple of months later, Heather died. "I was like, 'OK, that's it. They die. They all die,' " Danielle said.

Her stepmother, Shirley, recalled the struggle to get Danielle through school. She attended Pine Ridge High School her first two years, but school officials wouldn't allow her to ride on a golf cart between classes, so she was home-schooled, completing her work in time to graduate in 2001. But even that was a challenge.

The school initially told her she couldn't join her Pine Ridge classmates at the graduation ceremony. Her stepmom, Shirley, got involved.

"I told them no, my daughter is going to walk with her class," Shirley said. "I had to fight it, but I did it."

Danielle wore her cap and gown and strode across that stage, shook hands and took her diploma home.


BECOMING A MOTHER

Danielle knew she didn't want to go to college.

She enjoyed baking, and thought about attending a culinary school, but she figured the heavy uniform and the hot temperatures in the kitchen might be too much for her to handle.

Really, all she wanted was one thing from adulthood: To become a mom.

She met a guy, John Selph, through his stepsister, Danielle's best friend Mandy.

"Opposites attract," Danielle said. "He likes mud and trucks and fishing. I like beaches and books."

Unmarried, they're still a committed couple after 11 years, although money is a constant struggle. She lives in a rental home on Medicaid and federal Supplemental Security Income.

At age 20, Danielle felt good, and she knew time was not on her side. She became pregnant with Alyssa. Doctors were not thrilled, she said.

Her Orlando cystic fibrosis specialist, Dr. Daniel Layish, said he is seeing more and more women like Danielle with both the disease and a child.

"I think she's fairly typical, unfortunately," Layish said in a phone interview last week. "Other women with CF who are becoming moms are finding it's a huge burden of time just for their basic CF care."

Layish's clinic, Central Florida Pulmonary Group, has a social worker to help people like Danielle navigate the challenges posed by the disease and motherhood, as well as the financial burdens.

Danielle said she understands some people might feel she's selfish for having Alyssa, knowing she might not live to see her through adolescence. Because cystic fibrosis is genetic, Danielle knew she was a carrier. John isn't. So Alyssa has the gene, but not the disease.

"There's stuff I don't know if I'm going to be there to teach her. Back then (when she was 20), it didn't weigh on my mind that much. I was doing good."

And Danielle had a motherly instinct.

"Ever since I was little, I was motherly -- with my baby dolls. I always wanted to be a housewife and have a child. ... I knew this was something I deserve to have."

The need for a transplant has given Danielle a new sense of urgency. She has started writing more in her journal, things she hopes Alyssa might someday read.

Her writings are honest. She struggles with the idea of even going through with the transplant, knowing the pain she already suffers.

"Even if I do (have the transplant), my body will eventually start rejecting the new lungs anyway, maybe as soon as five years," she wrote in one entry. "So what's the point ... 1 year or 5 years? I guess that's the point. But the thing no one is paying attention to is this: What if I don't want (it) to be done?"

She writes that is not an option because of her 7-year-old daughter, but she remains conflicted.

"I could say ... 'It was a good 30 years, 29 years, whatever,' ... but I feel like I don't get a say in this ..."

In another entry, she struggles with her calling in life.

"It just seems like I am here to ... get poked by needles and do a large amount of meds every day ...

"But I think I have a big heart and I'm good at some stuff. I'm good at being a mom."

She's nervous about the testing, uncomfortable with being away from home for two weeks and uncertain about what lies ahead.

One thing she writes about with certainty, though, is perhaps the thing that carries her through each day. Love. Love for her family, her boyfriend, her daughter. Her life may end up shorter than most, but it won't have been lived without love.

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