In many ways, Tara McNaught is a typical 15-year-old—she's a student, junior lifeguard and member of her school's soccer team.
But she's also a teenager who faces extraordinary challenges on a daily basis, including cystic fibrosis (CF) and CF-related diabetes.
Much like a prize fighter, Tara has knocked down every challenge she's encountered along her courageous journey: major breathing treatments, gastrointestinal issues, CF-related diabetes and an overwhelming daily medication regiment. Her partners on this journey are a loving and supportive family, and an extended family of a different sort—the staff at the Outpatient Specialty Centers at Miller Children's Hospital Long Beach.
DIAGNOSIS/GETTING TO KNOW
THE EXTENDED CARE TEAM
The first indication that something might be wrong came in the delivery room. Doctors at Miller Children's Hospital Long Beach told Tina and Terry McNaught that their newborn daughter had a bowel obstruction, a condition that required immediate surgery. It was also a warning sign of a disease the McNaughts had barely heard of: cystic fibrosis. Two weeks later, further testing confirmed the diagnosis.
"We were shocked," recalls Terry, who is a superintendent for Del Amo Construction in Torrance. "We didn't know anything about cystic fibrosis, and had no idea what to expect." Baby Tara spent her first month in Miller Children's neonatal intensive care unit. During that time, the cystic fibrosis team at Miller Children's began preparing Tina and Terry for the journey ahead.
Treating CF is a complex and lifelong process. This chronic disease affects multiple organ systems, causing serious complications—each of which requires highly specialized care. Coordinating this treatment, especially in a growing, developing child, takes a unique blend of expertise and teamwork that only a children's hospital can offer.
Fortunately, Tara is able to get the advanced care she needs at the Outpatient Specialty Centers at Miller Children's Hospital Long Beach. This major referral center is made up of more than a dozen individual specialty centers, housing the majority of Miller Children's subspecialists. With doctors representing over 30 different pediatric specialties and subspecialties, the centers provide care for children, adolescents and adults with chronic and complex medical conditions. Each center is designated to an exclusive specialty with physicians and care teams trained specifically in certain childhood illnesses.
These Outpatient Specialty Centers serve as Tara's "medical home." Tara attends as many as three different specialty centers throughout the year, including a comprehensive multispecialty visit four times a year. Without Miller Children's, Tara would probably be referred to different community clinics throughout the region and would not have access to her own personalized, expert medical care team.
"Many kids with chronic illnesses have multisystem diseases that require pediatric expertise in more than one area," says Barry Steinmetz, M.D., careline director of the Outpatient Specialty Centers at Miller Children's. "In Tara's case, she needs three different specialty doctors for her condition. Our approach is to provide patients with a 'medical home'—a place where there is integrated and coordinated expert pediatric care available in one location. It's very much a family-centered, team-oriented dynamic, with the family considered an important member of the care team."
Tara and the group review her current condition from a multidisciplinary perspective, and discuss whether any adjustments are needed in her care plan. Her mother, Tina, always accompanies Tara to these appointments, offering her daughter support and encouragement. The availability of so many pediatric specialists and services in one location simplifies the lives of both mother and daughter.
Cystic fibrosis is a genetic disease that affects about 30,000 children and adults in the United States, with more than 1,000 new cases diagnosed each year. While both parents must be carriers of the gene that causes CF in order to pass it on to their child, that's not always the case. In fact, neither of Tara's older siblings—Adam, 23, and Brittany, 20—have the disease.
CF causes the body to produce thick, sticky mucus that builds up primarily in the lungs, as well as in the pancreas and other organs. "The mucus clogs the lungs, making it difficult to breathe, and also blocks the digestive system, which interferes with the body's ability to digest food and absorb nutrients," explains Eliezer Nussbaum, M.D., chief of pediatric pulmonary medicine at Miller Children's and medical director of the Pulmonary, Cystic Fibrosis, Allergy & Asthma Center. He is also a professor of clinical pediatrics, step VI. "The mucus is a perfect breeding ground for bacteria, which can cause life-threatening infections, especially in the lungs."
Since Tara's primary diagnosis is CF, much of her care takes place in the Pulmonary, Cystic Fibrosis, Allergy & Asthma Center, which provides comprehensive respiratory care by pediatric board-certified specialists. The Miller Children's cystic fibrosis program is one of only 115 in the nation accredited as a CF Care Center by the Cystic Fibrosis Foundation. "In order to earn this accreditation, a center must meet very rigorous standards of care, demonstrate a multidisciplinary approach, and use the most advanced national guidelines and protocols for CF care," says Dr. Nussbaum.
Treating cystic fibrosis is a complex and lifelong process that requires highly specialized care.
The multidisciplinary aspect of CF programs is critical. There are more than 25 members from different specialties on Tara's team, including experts in gastroenterology, pulmonology, endocrinology, infectious disease, respiratory and physical therapy. Most of the team's members, including Dr. Nussbaum, have been involved in Tara's care since she was a newborn and have guided the McNaught family on their journey.
"We get to know our patients and their families extremely well, and believe that translates to better care," says Dr. Nussbaum. "We've known Tara since she was an infant. We've seen her go through kindergarten, graduate from elementary school—and now we're hearing about how she's hoping to get her driver's license."
MANAGING CF/ DAILY REGIMEN
For Tara, a typical day begins at about 5:45 a.m.—early enough for her to complete her breathing treatments before school. The primary focus of CF treatment is clearing the lungs and airways of mucus to help patients breathe better and reduce their risk of infection. Tara uses a special therapy vest attached to a machine that vibrates her chest at high frequencies. The vibrations help loosen and thin the mucus in her lungs, making it easier to expel. She also inhales several medications through a device called a nebulizer. Together, the vest and nebulizer treatments take about 40 minutes. The whole process has to be repeated every evening—and if she's sick or very congested, as many as four times a day. It can be an exhausting ritual. "Sometimes Tara falls asleep with the nebulizer in her mouth," says Terry. "We all do our part though, and go over there and hold it for her while she sleeps."
CF creates other problems as well. Because the disease causes mucus to build up in the pancreas and digestive tract, it blocks a key digestive process. "The pancreas is responsible for producing various enzymes that help the body process food," says Richard Mathis, M.D., medical director of the Gastroenterology, Hepatology & Nutrition Center at Miller Children's, and a member of Tara's care team. "When CF blocks or disrupts this process, it causes major problems for patients, including malabsorption of fats and nutrients, and a failure to grow over an extended period of time."
To prevent this, Tara must take six enzymes each time she eats or snacks. It's part of a regimen called pancreatic enzyme replacement therapy, which aids in the digestive process. "CF patients typically have difficulty gaining weight, so a special diet that's high in fats and calories is prescribed," says Dr. Mathis. To ensure that patients like Tara can absorb the nutrients that will help them grow, Dr. Mathis and other specialists from the Gastroenterology, Hepatology & Nutrition Center work very closely with the CF program. "We have a unique collaboration that not many CF Care Centers can claim," says Dr. Mathis. "Having gastroenterology care integrated into the CF program makes us stand apart."
"I love that we can go to one place and take care of everything at one time," says Tina, who works as a part-time health assistant at Tara's school. "It's so much more convenient than having to make multiple appointments on different days all around the city. And the care is great. The Miller Children's team loves Tara like she's their own."
ANOTHER SETBACK/COURAGE TO FIGHT ON
With breathing treatments, a special diet and medications, Tara's daily regimen seems daunting enough. In 2007, however, she was faced with yet another challenge when she was diagnosed with CF-related diabetes. But true to form, it wasn't long before Tara simply took the news in stride. "After three or four days, it was as if she'd had it all her life, and it just became part of the routine," says Tara's father, Terry. "That's typical Tara."
The additional diagnosis added yet another dimension to Tara's care. "Diabetes is fairly common in people with CF, but it's not the same type of diabetes that you see in the general population," says Mario Brakin, M.D., medical director of the Endocrine & Diabetes Center at Miller Children's. Two types of diabetes occur in the non-CF population: type 1, also called insulin-dependent diabetes, and type 2, also called non-insulin dependent diabetes. "CF-related diabetes (CFRD) includes features of both types," says Dr. Brakin. "CF damages the pancreas, causing it not to produce enough insulin, which is a hallmark of type 1 diabetes. CF also causes insulin resistance, which prevents the body from using insulin normally—the hallmark of type 2 diabetes." CFRD becomes increasingly common as individuals with CF grow older: It's found in 35 percent of adults aged 20 to 29 who have the disease, and 43 percent of those over 30. "Diet is a major part of managing diabetes," says Dr. Brakin, who has become one of the most recent members of Tara's "extended family" at Miller Children's. "One of the challenges in caring for patients with CF-related diabetes is combining the high-fat, high-protein diet needed by cystic fibrosis patients with the low-carbohydrate, low-sugar diet required for people with diabetes."
Managing her diabetes means that Tara pays strict attention to what she eats, keeps a close watch on her blood sugar and carries an insulin "pen" with her so she can self-inject when she needs to. Although her daily treatment regimen is very demanding, Tara is matter-of-fact about the challenges. "Sometimes, it's frustrating but I just deal with it," she says. "I just try to focus on staying as healthy as I can."
Living with CF and all its complications hasn't slowed Tara down in the least—or kept her from doing what she loves. As a straight-A student, she's won numerous academic awards and plays multiple sports, including varsity soccer. "She was offensive player of the year in 2009 for JV soccer," notes Terry, proudly. She also competes in roller hockey, runs track and spends summers taking junior lifeguard classes. "Soccer and roller hockey are my favorites," Tara says, with a characteristic laugh. "They're fast-paced and thrilling."
Her parents and medical care teams admire her strength and tenacity. Together, they do their best to always support her. As Tara's mother points out, "We've never lowered our expectations because of her condition, and she's certainly never lowered hers."