In October 2000, the Adult Cystic Fibrosis Program began at the University of Alabama at Birmingham Hospital due to a Cystic Fibrosis Foundation mandate requiring all pediatric programs with more than 40 patients over the age of 21 to establish an adult program. The cystic fibrosis program at Children’s Hospital had more than 40 patients over the age of 21, so the program at UAB was launched with the instrumental help of Dr. Randall Young, the current assistant program director, and Joy St. John, MSN, CRNP, Adult Cystic Fibrosis Program coordinator, who worked with patients at Children’s Hospital before moving to UAB.
Fortunately, adult programs have become necessary due to the extended lifespan of cystic fibrosis patients. The current median lifespan is 36.4 years. Adult cystic fibrosis patients have unique medical, behavioral and social needs, and care in children’s hospitals could not meet them, according to Mary Wade, MSN, CRNP, a nurse practitioner with the program. She cares for program patients who are admitted to the UAB pulmonary ward. On average, 4 to 12 patients are on the floor at a time, and Wade is responsible for their daily care.
“Adult patients need to be treated with an adult paradigm versus a children’s paradigm,” Wade said. “They need more encouragement for self management.”
Wade stated that adult patients must learn how to function on their own and take on a different level of responsibility, such as keeping up with their medications and going to treatments without their parents. As they become adults, they must learn to become independent; deal with adult issues and problems; and, if possible, live on their own and manage their own care.
“Patients are encouraged to get jobs or go to school. Many have children or, because many men are infertile, adopt,” Wade said. Being in an adult-care facility also gives the patients an opportunity to see other people sick with other illnesses. “They have a broader outlook on illness in a broader adult population.”
St. John, a nurse practitioner and the program coordinator, is responsible for the outpatient care of program patients. Besides a patient’s medical care, she keeps track of many measurements for each patient, including sputum cultures, pulmonary functions, chest X-rays, number of times a patient is admitted to the hospital and whether a patient needed oxygen. “This data is collected and maintained in the Cystic Fibrosis Foundation’s national registry,” St. John said.
The data is used for national trend tracking and may help determine the appropriateness of a lung transplant for a patient. Since a lung transplant can mean a lifetime of management, the trend information is used to offer advice and counseling in regards to lung transplants to help patients decide if a transplant is the best decision. The mean life expectancy is five years with a 50 percent survival rate.
“Re-infection — including lung and sinus infections — while patients are on immunosuppressant drugs is a concern,” Wade said.
In general, cystic fibrosis patients have a wide range of unique medical issues and treatment needs, and ensuring that all these needs and requirements were addressed was one of the challenges in bringing this program to UAB eight years ago.
“Cystic fibrosis is a multifaceted disease affecting nutrition, lung function and bone health,” Wade said.
First, treatment of the disease can be expensive for patients. A program social worker was necessary to help them handle, among other things, the financial burden of purchasing equipment (such as chest vests and nebulizers) and medicine (such as bronchodilators and enzymes).
The program also needed respiratory therapists specially trained to care for cystic fibrosis patients. They teach patients proper coughing techniques, good respiratory care and chest physiotherapy. The respiratory specialists also perform chest physiotherapy for patients who are admitted to the hospital. The number of therapists needed for the program was monumental: each patient may need a minimum of 1½ hours of chest physiotherapy three times per day.
Cystic fibrosis patients also face dietary issues and need guidance with nutrition planning. “Simply obtaining a program-dedicated dietician was a hurdle,” Wade said.
Many cystic fibrosis patients need to take enzymes each time they eat. “Dr. Young put a (flexible) system in place to allow patients to have their enzymes in their rooms,” Wade said. “(He) is to be credited greatly. … We are indebted to him for that.”
Wade also appreciates the program staff, both licensed and unlicensed personnel. “We are able to offer the best quality (care because) our nurses are very dedicated to our patients. They are always here to hold the patients’ hands and offer support.”
Wade stated that the staff gets to know the patients well and celebrates when they have successes. “However, that makes it difficult when we have to face (their) not doing well or when they die.”
PHOTO CUTLINE: Seated front left to right: Gibian Goolsby (social worker),
Dr. Kevin Leon (director of Adult CF Program)
Standing left to right: Joy St. John, CRNP (program coordinator),
Mary Wade, CRNP (inpatient coordinator),
Dr. Randall Young (assistant program director), Dr. Steven Rowe, Dr. Amit Gaggar
February 2008
