Cystic Fibrosis: A Tale of Two Women
by Mary Ann Littell
Lourdes Laraya-Cuasay, MD (right), with her long-time patient Barbara Duggan
he year is 1973. A 13-year-old girl comes to St. Christopher’s Hospital in Philadelphia with an assortment of
symptoms: a severe, nagging cough,
shortness of breath, nasal drip, and an inability to gain weight. After many tests, including the infamous sweat test for
cystic fibrosis (CF), she is finally
diagnosed with this serious genetic
disorder. Among those treating the child is a young attending physician named Lourdes Laraya-Cuasay. A native of the Philippines, she came to the U.S. in 1966 for further training and stayed to become a pioneer in the growing field of
The patient, Barbara Duggan, sees the physician off and on until 1979, when they go their separate ways. Duggan attends college, gets married, and begins a career as a dietitian. Cuasay comes to UMDNJ-Robert Wood Johnson Medical School in 1980 as an associate professor of pediatrics and continues to
pursue her lifelong interest in CF.
In 1983, the two meet up by chance at a pediatric nutrition symposium, and their lives became intertwined once again. “We talked and got caught up,” says Duggan. “Shortly after that, I became very sick and started coughing up blood. So I became her patient again.”
Cystic fibrosis, a disease where abnormally thick, dense mucus clogs the lungs and leads to serious infections, once was a childhood killer. Complications of CF include diabetes,asthma, digestive problems and male infertility. In the 1950s, those born with it rarely survived past the age of 5.
Today, however, better diagnostic techniques and new therapies have improved the life expectancy of CF to around 34 years. “It’s no longer a childhood illness, because people are living much longer with it, but it still falls under the auspices of pediatrics,” observes Cuasay, who is director of the UMDNJ-Robert Wood Johnson Medical School/Bristol-Myers Squibb Children’s Hospital Cystic Fibrosis Center, located in downtown New Brunswick.
Cuasay’s interest in CF is somewhat ironic, considering that Filipinos, and in fact most Asians, are not affected by the disorder. She started the Center in 1980 at what was then the Middlesex General Hospital to have a more central location in New Jersey. She actually started a CF program in Camden in 1977, the first in south Jersey, but most of the patients moved to the New Brunswick CF center later.
“Around this time, New Jersey was starting to move away from just being a satellite to New York or Philadelphia,” she says. “The state health department decided it was time to fund programs that would address several diseases of
children, one of which was CF.
“We began with one patient, but soon 33 others — all New Jersey residents — transferred here from St. Christopher’s,” she continues. “Until we opened, they had been unable to get care close to home.” Later, some CF patients transferred from centers in New York and Philadelphia, as well as other CF programs in the state.
Many of the adult patients at the Center have been cared for by Cuasay since their infancy. In addition to medical staff, the multidisciplinary center also includes a clinical geneticist, social worker and nutritionists, respiratory therapist, and other specialists. The patient population is 40 percent adult, one of whom is Barbara Duggan. The CF center is accredited by the National Cystic Fibrosis Foundation.
The physician explains that Duggan’s particular type of CF is not typical. The disease has some 1,300 mutations, and Duggan has one gene of one of the rarer types. For instance, in many CF patients, thick secretions obstruct the pancreas and prevent the digestion of food. Duggan’s pancreatic function was normal at diagnosis but more recently started to be abnormal. She does not suffer from the complications of diabetes, but does have asthma and reactive airway syndrome, which is triggered by temperature changes and inhaling irritating substances.
Cuasay describes her patient as “something of a medical miracle.” When you look at Barbara Duggan, it’s hard to believe she has a debilitating and serious illness. Years ago, she was told by a physician that she wouldn’t live to see her 16th birthday. Yet here she is today: lively and exuding well-being. At 45, she’s already exceeded the current life expectancy by several years. She’s also the mother of four children — highly unusual for someone with CF. (Cuasay is also the mother of four children born within five years — another bond between the two women.)
“There have been only 300 documented cases of babies being born to CF mothers,” says Cuasay. “Barbara has four of them. That makes her very unique.”
Duggan jokingly describes herself as a “soccer mom.” Her daily “to-do list” includes activities that most women take for granted: She’s up early to drive her children, who range in age from 5 to 18, to school and a host of other activities. She does extensive volunteer work at her children’s schools, and until a few years ago, also worked full-time. And she does it all while dealing with a disease whose treatment takes hours every day.
The Changing face of CF
As someone who has spent years in the trenches fighting CF, Cuasay says, “It’s very gratifying to see more and more patients reach adulthood. Adult CF centers are opening in several states to respond to the needs of these patients. Improved survival is credited to advances in nutrition, earlier diagnosis, newer antimicrobial and anti-infective therapy, newer and better pancreatic enzymes, and advances in critical care.”
CF is heritable, passed on by both parents carrying the recessive genes, but it skips a generation. This means that a family history of CF will not always be present and is not a requirement to diagnose CF. Duggan says her parents were healthy, but a grandmother died in her 20s. She had been diagnosed with pneumonia, which actually could have been CF. “Back then the disease was very difficult to diagnose,” says Cuasay.
By pursuing a course of aggressive therapy, Duggan is able to control the symptoms of CF. “I decided long ago that I wasn’t going to let this disease stop me,” she says. “With four children, I don’t have time to be sick.”
Her daily regimen is rigorous. She takes 26 different medications every morning, including a cocktail of antibiotics designed to keep serious lung infections at bay, and another 10 pills before she goes to bed. She also undergoes chest physiotherapy twice a day and treatments with a nebulizer seven times a day. Typically chest physiotherapy involves manual chest percussion, or pounding, aimed at loosening the secretions from the walls of the lungs. “My 5-year-old daughter is very good at doing this,” Duggan jokes. She also has a special vibrating vest she wears, which gently loosens the secretions from the chest walls.
Despite all these efforts, infections flare up. When they do, patients either go into the hospital for intravenous antibiotic therapy or have it done at home. Duggan prefers the latter. A PICC line, or peripherally inserted central catheter, is inserted in her arm and she administers her IV medications herself. A respiratory therapist comes to her house daily to provide chest physiotherapy.
Duggan volunteers her time speaking to groups about CF. Among them are parents of newly diagnosed children. “What they hear about CF is not promising,” she says. “They hear the disease is progressive and the life expectancy is short. I try to show them the positives. CF has changed from a potentially fatal illness to a chronic life-limiting one. I’m living proof that you can live a happy and productive life with it.” Every November she also speaks to medical students about life as a CF patient.
Duggan prides herself on not having been hospitalized in two years. “I stay healthy by doing everything Dr. Cuasay tells me to do,” she says.
She credits her well-being to the dedication of her physician: “I really don’t think I would be sitting here, 45 years old, without her.”