LAKEVIEW — If she had been born 20 years earlier, Alexis Justice’s life, at age 7 would be nearly half over.
As it is, the charming Lakeview Elementary School second-grader, who wants to grow up to be the next Taylor Swift, may live long enough to celebrate her 35th birthday, maybe her 40th.
Anything beyond that will require major advancements in medical science.
Diagnosed with cystic fibrosis about five years ago, Alexis’ life — and the lives of her parents, Lorena and Kirk Justice — changed virtually overnight. Suddenly, the pert, active toddler was undergoing three hours of physio-therapy every day in an effort to keep her breathing passages clear. There were nebulizers, pills, enzymes to help her properly digest food. Doctors. Regular blood draws.
It would have been easy to let the diagnoses and resulting fallout rule her life. But like her folks, this kid is a fighter.
peaking with Alexis, you’d never know her average day was any different from the days of her classmates. She’ll talk about the hours spent each afternoon wrapped in a vibrating vest that shakes free the mucus that accumulates in her lungs. She’ll tell you about the daily visits to the school office to pick up the pills that allow her to digest her lunch. She will even talk about the doctor visits, blood tests, and what she thinks of Cystic Fibrosis Month, which is observed in May.
But for her it’s all matter-of-fact; boring stuff. What she really wants to tell you about are her plans for her future career as a singer-songwriter.
“I’m a pretty good singer,” Alexis says. “I got a microphone for Christmas and it makes my voice sound really good.”
At this point, the only possible speed bump she sees in her rise to stardom is competition in the form of her 10-year-old sister, Arianna, who, Alexis admits, is also a “pretty good” singer.
According to Lorena, she and Kirk first suspected something was wrong with their younger daughter when she was still toilet training. Problems arose that resulted in a visit to the doctor, and eventually, the CF diagnoses.
An inherited disease, CF affects the secretory glands, including mucus and sweat glands. It damages the liver, pancreas, intestines, sinuses and sex organs. Mucus becomes sticky and accumulates in the lungs and airways, causing infections that in turn cause additional damage to the lungs.
Lung function begins to decline in early childhood and over time, results in serious breathing difficulties.
Though symptoms — and their severity — vary from person to person, a CF diagnoses is never going to be mistaken for a good thing.
“She gets really tired,” Lorena said. “Every day she takes a nap right after school. Even so, she gets a lot of infections. She’s on antibiotics about eight months out of every year. She does miss a lot of school. She’s sick often and has to have the blood draws every few months. She’s had to have a bunch of surgeries on her sinuses for polyps. It’s really pretty awful for her.”
Yet Lorena has faith that medical technology will one day find a cure, or at least an effective treatment, for CF. As it turns out, there is good reason for Lorena’s faith.
Recently, Harvard stem cell researchers at Massachusetts General Hospital have made significant inroads toward finding a drug that will control CF, or at least its most debilitating symptoms.
As reported in the May edition of the Harvard Gazette, these researchers have grown — using skin cells of CF patients — tissues that line CF patents’ airways and prevent damage there. The tissue — which can be grown in unlimited quantities in the laboratory — contains the gene responsible for around 90 percent of CF cases in the United States.
This research has already lead to a drug, Ivacaftor, which alleviates CF symptoms in 2 percent of patients. Sadly, Alexis is not among that 2 percent. However, Ivacaftor serves as “proof of concept” for similar drugs researchers expect to introduce to the market in the foreseeable future.
Twenty years ago, the average life expectancy of a CF sufferer was 16. Today it is 37.
And so Lorena remains hopeful that her daughter will grow up, will grow old. Research continues.
And Alexis? She still plans to beat out her sister and be the next Taylor Swift.