 |  | | | | Laney Siems, left, with mom, Caryl, and pulmonologist Pam Zeitlin. |
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CF: One Family’s Experience
At 5 months of age, Laney Siems continued to suffer a nagging cough and infections that wouldn't go away despite an array of antibiotics. Also troubling, she wasn't growing.
"Our pediatrician would see some improvement, but then a decline," says Laney's mom, Caryl Siems. "There was something really, really wrong here."
Her instincts were confirmed when Laney began to struggle to breathe one night. On her pediatrician's advice, Caryl took her to the Johns Hopkins Children's Center emergency room, where asthma was ruled out. Then, recognizing that Laney had the two hallmark symptoms of cystic fibrosis—persistent cough and failure to thrive—physicians ordered a sweat chloride test. Laney had CF. Caryl was devastated. Then she became an advocate.
"My response was to cry a lot and get all the information I could," she says. "We had to figure out how we were going to handle her care from that point on."
Pediatric pulmonologists Peter Mogayzel and Pam Zeitlin helped get Laney's lung infections under control, but improving her nutrition was a bigger challenge. In CF, the same mucus secretions that block lungs also block pancreatic ducts, preventing digestive enzymes from getting to the gastrointestinal tract. Laney couldn't absorb food. At 5 months, she weighed just over 9 pounds. Soon after, she was sent home with an NG (nasogastric) feeding tube, and her nutritional status began to improve.
At the same time, Caryl began surfing the Internet for advocacy groups like the CF Foundation, and for a pediatrician who knew CF. She found one, but she also quickly discovered how challenging and time-consuming managing a CF child can be. In addition to daily compression and nebulizer treatments, Laney had to be given antibiotics and pancreatic enzymes three or more times a day.
Caryl also connected with the CF Foundation in ways she hadn't expected. She started out volunteering for its Maryland chapter; now she works there full time, doing everything from fund raising to organizing special events.
"One of the harder things about this disease is the feeling of helplessness," Caryl says. "Raising funds for research for a cure is one way to give you more control."
A decade since diagnosis, Laney, now a sixth grader active in gymnastics and lacrosse, is doing well. But Caryl still struggles with managing her daughter's disease. Families need to network, bounce experiences and management ideas off each other, Caryl concludes. But that's not easy, because CF kids can't be near each other because of infection risks.
Well aware of that isolation, Mogayzel notes that the CF Center at Johns Hopkins has developed its Web site for patients and their families to connect via the Internet.
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