Tammy Ledkins sensed something was wrong.
Her infant daughter, Erin, went through 10 to 12 diapers a day.
She consumed enormous amounts of food, but never gained weight.
“We thought she had a food allergy,” the Marne woman said.
But then came the testing. And the answer she didn’t want to hear.
Erin, then 15 months old, had cystic fibrosis, an inherited disease that causes the body to produce thick and sticky mucus. The condition mainly affects the lungs and pancreas.
“We didn’t even know what the word cystic fibrosis meant, but she had all the signs of a CF kiddo,” Tammy said.
Erin, now 8 years old, was recently admitted to Spectrum Health Helen DeVos Children’s Hospital for a “tune-up,” which consists of a heavy dose of IV antibiotics.
Erin is one of about 30,000 people in the United States who suffer from cystic fibrosis.
“Erin is a lovely girl,” said Charles McCaslin, MD, a pediatric pulmonologist with the Helen DeVos Children’s Hospital Cystic Fibrosis Clinic. “She’s always smiling and laughing. Her mother is incredibly dedicated and takes great care of her.”
Dr. McCaslin said the outlook is much better for cystic fibrosis patients because of advancements in medication, education and testing, and the efforts of the national Cystic Fibrosis Foundation.
Cystic Fibrosis Foundation statistics show that, in 1986, only 30 percent of people with CF reached age 18 or older. In 2013, that number jumped to 50 percent. The typical lifespan is now 40.
Tammy is thrilled with the progress.
“You for sure want your kids to outlive you,” she said. “When you think of all the treatments coming down the pipeline to extend life expectancy, it makes me so happy that she is going to have a wonderful future.”
Tammy is also part of the solution.
For the past year, she has served as a patient representative for a Spectrum Health-Cystic Fibrosis Foundation-Dartmouth University collaboration to make the transition from pediatric to adult care easier and smoother.
“I was very excited to be a part of it,” Tammy said. “At some point you have to let your kid take responsibility for their own care. That’s really hard to do as a parent when you’re so ingrained to help them stay healthy. You think if you don’t help them, that you risk their health declining. But you don’t want them to be thrown into the big world of adult care if they have no clue.”
The transitions program teaches children how to manage their own disease. And, beginning in infancy, the program teaches parents how to raise their children to be self-reliant and involved in the process. It also teaches parents how to slowly let go and let the kids take charge. Children start to see doctors on their own at age 13 to learn how to report their health status and prescription status, then the parents go in to review the visit and ask follow-up questions.
Some children with cystic fibrosis may take as many as 15 medications and spend more than two hours a day taking breathing and airway clearing treatments.
“Sending a young adult with CF into the adult world without teaching self-management of a chronic disease would be disastrous,” Dr. McCaslin said.
The self-management education includes time management, prescription management, equipment maintenance, and an in-depth understanding of cystic fibrosis.
Tammy said Erin is already taking an interest in her medical needs.
“Starting at an earlier age is a wise thing to do because kids soak that information up,” Tammy said. “If I forget to put a pill out, Erin says, ‘Hey, Mom, you forgot this pill.’ She knows what meds to take. She knows it’s non-negotiable, that they’re going to make her feel better when she’s done.”
Tammy still attends appointments with Erin, but she knows it will be important in a few years to take a back seat.
“That still gives responsibility to the child,” she said. “It’s not by any means taking the parent out of the picture, but it’s starting to teach them what’s going to happen when they go off to college.”
The transitions program is big on education, even for young children.
“They found targeting nutrition early in life makes a big difference in lung health,” Dr. McCaslin said.
Spectrum Health is one of eight health organizations in the country to participate in the Cystic Fibrosis Foundation-Dartmouth collaborative. Spectrum Health’s transition program recently completed its first year.
“It wasn’t just the Cystic Fibrosis Foundation telling us what to do and how to do it, but it was improving processes,” Dr. McCaslin said. “You find out where the problems are, where things can be fixed and what gaps can be filled to make it a seamless process.”
Spectrum Health’s children and adult cystic fibrosis clinics started operating in separate spaces three years ago to better personalize care programs based on age group. The children’s clinic is at 35 Michigan Street. The adult clinic is on Lake Drive.
“We prepare for the transition for years and years before the transfer to the adult clinic,” he said.
Success comes with developing strong team dynamics between the patient, family and health care professionals.
Eventually, when it’s time for a young patient to leave the nest at about age 18, he or she has a goodbye visit with the pediatric team. The adult team comes to the pediatric clinic at that time to meet the patient and help smooth the transition.
“We clearly do our best to prepare children to be adults with CF,” Dr. McCaslin said. “As people are living longer we have to do a better job of preparing them for adulthood with a chronic illness. It’s a fantastic problem to have.”