By Megan Flaherty
October 9, 1997

Twenty years ago, most cystic fibrosis patients didn’t live past their teens.

Healthcare providers are developing a different outlook on their care too. As patients with what were traditionally considered "childhood diseases"—such as CF, hemophilia, and muscular dystrophy—live well into adulthood, healthcare professionals are making decisions about where to treat these patients, and how to best educate them for their futures.

One of the biggest decisions involving chronically ill young adults is whether to treat them at children’s or adult hospitals. Either way, the situation is challenging for this growing group of patients and their providers, said Susan Dull, MBA, MSN, RN, an associate director in the child health and financing division at the National Association of Children’s Hospitals and Related Institutions. The pediatricians and nurses in a children’s hospital may be experts on chronic childhood illnesses, but are unfamiliar with adult health issues. And the physicians and nurses in an adult facility may not know anything about childhood diseases, Dull said.

Some hospitals make an organized effort to transfer their chronically ill young adult patients to adult facilities. In the past five years, Childrens Hospital of Los Angeles (CHLA) has transferred most of its older CF patients to nearby USC University Hospital or Cedars-Sinai Medical Center, said D.J. Kaley, RN.

Kaley, the hospital’s cystic fibrosis clinical coordinator, said that since many of her former patients are now middle-aged, "they need adult practitioners who know about arthritis pain, mammograms, and menopause—things that pediatric caregivers don’t traditionally deal with."

Some patients initially resisted leaving, Kaley said, because they were emotionally attached to physicians and nurses who cared for them when they were young. It was equally difficult for the providers to say goodbye, Kaley said. The hospital kept older patients with severe cases, and still sees about 30 CF patients regularly who are over 18.

There are ways to make the transition less traumatic for patients, Dull said. Transfers shouldn’t be rushed, and physicians, nurses, and respiratory therapists from the children’s hospital should share the patient’s history with their adult hospital counterparts. The care providers from the children’s facility should be available to answer questions before and after the transfer, Dull said.

This continuous communication is easy at a children’s hospital that is part of a larger one, such as University of California, Los Angeles (UCLA), Children’s Health Center.

"It’s very easy for us to be integrated," said Margaret Ecker, MS, RN, a clinical nurse specialist there. "It’s a matter of sending the patient to another floor." The care providers make judgment calls about when to move a patient. For example, if a patient has had a lot of trouble with a liver transplant throughout his or her life and is comfortable with certain physicians, the patient may still receive treatment on the children’s floor at 19, Ecker said.

"By and large those patients with cystic fibrosis and muscular dystrophy know us. They’ve had a hard life and we welcome them," Ecker said. "However, if our census is putting us in a tough place, our first choice is to send up an older child rather than a younger one." An older child is still followed by pediatric medical services, she said.

Although most children’s hospitals transfer at least some of their young adult patients, many remain at children’s hospitals. In those cases, it is important for pediatric nurses and other care providers to recognize the changing psychosocial needs of young adults. "Someone who is 25 years old doesn’t need to be told to go to bed at night," Kaley said.

Older teens on the fringes of adulthood may be particularly sensitive to the way they are treated. One factor nurses should keep in mind with CF patients is that they often look much younger than they are, said Marty Smith, executive director of the Center for Children with Chronic Illness and Disability at the University of Minnesota, Twin Cities Campus. They may be smaller in stature than others their age and enter puberty later than average. CF patients stress that care providers should "treat them by their chronological age, not how they look," Smith said. Pediatric nurses who are treating adults should also keep in mind that their patients may have questions about birth control or may need special arrangements made to accommodate sexual activity.

Price was about 12 when her parents and practitioners, including Kaley, began teaching her about her treatment and medications. "I felt a little bit more control then. And I knew if my parents couldn’t be at the hospital with me, I could give someone my history."

Price’s situation is typical, Smith said. Often when a family raises a child with a severe life-threatening illness, the parents invest a lot of time in the child’s care. As the child gets older, there is a shift in responsibility, she said. "It is important to recognize when parents stop calling all the shots and the kids become equal partners in decision-making," Smith said. Any provider who cares for adolescents should talk to them confidentially without their parents, at least part of the time.

Kaley has watched many of her CF patients thrive: Among her patients are an engineer, a comedy-show writer, a nurse, and a physician.

Older CF patients are an inspiration for the newly diagnosed, she said. "The physicians give the bad news about a fatal disease. I go in and say our oldest patient is 57. It provides a ray of hope for them."