Allergy & Pulmonary Medicine | Cystic Fibrosis Center

Overview

Dedicated to the treatment of patients with cystic fibrosis (CF) for more than 4 decades, the comprehensive, fully-accredited Cystic Fibrosis Center at Washington University Medical Center continues to flourish. The Cystic Fibrosis Clinical Center and affiliated programs has developed into a premier clinical and research program, and the numbers of patients seen here is steadily increasing. In 2004, the program treated more than 500 patients from several Midwestern states (including Missouri, Illinois, Kansas, Kentucky, Tennessee, and Arkansas), thus making it one of the largest centers in the country (Figure 1).

The Cystic Fibrosis Center at Washington University Medical Center is under the direction of Dr. Thomas Ferkol. Located at the St. Louis Children's Hospital, the Pediatric Cystic Fibrosis Clinical Center now cares for roughly 300 patients. The Center also sees CF children from across the United States have been referred for or have survived lung or liver transplantation and are followed by the Pediatric Transplantation programs. The long-established Adult Cystic Fibrosis Clinical Center at Barnes-Jewish Hospital, directed by Dr. Daniel Rosenbluth, follows almost 180 patients, including CF patients who have undergone lung transplantation. Both Centers are well-supported by nurses and ancillary health personnel, and has adult and pediatric subspecialty consultants available.

The research programs at Washington University School of Medicine in CF have expanded, and close collaborative relationships between the Cystic Fibrosis Center and investigators in clinical and basic science departments have allowed us to consider questions fundamental to our understanding of the disease. The clinical research program has rapidly grown and the Washington University Cystic Fibrosis Center is part of the Cystic Fibrosis Foundation Therapeutics Development Network (TDN). Moreover, Washington University and St Louis Children's Hospital are involved in a number of quality improvement programs, including multicenter projects sponsored by the National Initiative for Children's Healthcare Quality (NICHQ) and Cystic Fibrosis Foundation.

Patients with CF under 21 years of age routinely receive their care at St. Louis Children's Hospital. The Cystic Fibrosis Center Clinics are regularly scheduled in the Pediatric Ambulatory Clinics on Tuesday and Wednesday afternoons, as well as other times as clinically indicated. Individual physicians, who have primary responsibility for their patients, attend the clinic with CF nurses, dieticians, clinical social workers, respiratory therapists, and physical therapist. When necessary, children with CF are admitted to the St. Louis Children's Hospital pediatric wards (7E and 9E), under the care of pediatric residents and pediatric pulmonary fellows with attending supervision from the Division of Pediatric Allergy and Pulmonary Medicine.

Once the child reaches 18 years of age, discussions and counseling with the patient and families begins regarding transition of care to the Adult Cystic Fibrosis Center. With rare exception, all patients are transferred by age 21.

The Cystic Fibrosis Center Personnel

Karen Wilson, R.N., M.S.N., and Amy Rahm, R.N., are highly skilled clinical nurses who have extensive experience in the care of CF children and adolescents. Mary Boyle M.S.N. and Jane Quante R.N. are the research nurse coordinators for the Center, coordinating clinical trials and projects for both adult and pediatric CF patients. Their experience and dedication have been important ingredients for fostering the growth of our clinical and translational research program.

	Figure 1: Geographic distribution of children and adolescents seen in the Washington University School of Medicine Pediatric Cystic Fibrosis Center in 2003, excluding patients referred for transplantation.

Andrea Clark R.D. is the dietician for the Pediatric Cystic Fibrosis Center, and she has spearheaded many of the quality improvement efforts in the Center. She also works closely with the research coordinators for many of our studies examining nutrition in CF. Tracy Hartmann M.S.W. serves as the designated clinical social worker for the Center, and Martha Markovitz M.S.W., L.C.S.W. spends much of her time with CF patients, particularly those who have undergone lung transplantation. Rebecca Pendino, M.S.P.T provides physical therapy care and instruction in airway clearance techniques for our CF patients. Deborah White, R.R.T., R.P.F.T. continues to serve as director of the Pulmonary Function Laboratory at St. Louis Children's Hospital, essential for the clinical assessment of CF children.

The Pediatric Cystic Fibrosis Center team meets weekly to review inpatients and outpatients scheduled to be seen that week, discuss patient-related issues, research projects, and other center-related items. The adult and pediatric center directors meet frequently to review the programs and ongoing clinical research projects, and the entire adult and pediatric teams convene on a quarterly basis. Cystic fibrosis patients admitted to St. Louis Children's Hospital are also reviewed with the other members of the division (including physicians, nurses, respiratory therapists, dieticians, and physical therapists) during weekly patient care conferences. Finally, the Cystic Fibrosis Center and Pediatric Lung Transplantation teams hold joint meetings every three months to review common patients.

Laboratory Services

The Pediatric Pulmonary Function Laboratory at St. Louis Children's Hospital specializes in pediatric testing from infancy through adolescence. Newly remodeled suites are available for studies, which include 3 rooms for standard pulmonary function testing, an exercise testing area, and a dedicated room for infant pulmonary function (IPL) measurements. The laboratory serves more than 4000 patient visits per year.

Conventional and innovative thoracic imaging is available for clinical and investigational purposes though the Department of Radiology at St. Louis Children's Hospital. High quality, digital roentgenograms, computerized tomography, and magnetic resonance imaging to define chest and sinus anatomy can be performed on CF patients.

Microbiological analyses of specimens from the upper and lower respiratory tract are performed in the St. Louis Children's Hospital Microbiology Laboratory. The Laboratory is certified by the College of American Pathologists (CAP), the principal agency that oversees microbiology laboratories in the US, and are able to isolate and characterize common organisms from the CF lung, including Pseudomonas aeruginosa, Staphylococcus aureus, and Burkholderia cepacia, using specialized growth media. Suspicious isolates are routinely sent to the Cystic Fibrosis Foundation National Laboratory on Burkholderia cepacia (Ann Arbor, MI) for confirmation.

Sweat chloride measurements for all patients in the Washington University Medical Center are performed in the Clinical Chemistry Laboratory at St. Louis Children's Hospital.

Newborn Screening for Cystic Fibrosis in Missouri

Newborn screening for CF is routinely performed in several states. Although Missouri does not currently have such testing, it is tentatively planned for 2006. The program will be an important part of a comprehensive approach for combating CF here in the state. More details will be provided in the coming months.

Lung Transplantation for Cystic Fibrosis at Washington University

Based on our most recent statistics, St. Louis Children's Hospital has performed more organ transplants than any other pediatric center in the world. Of the solid organ transplants performed, the leading organ has been the lung. More than 300 pediatric lung transplants have been done here, and the majority of those were performed on CF children with advanced lung disease. The Cystic Fibrosis Clinical Center remains committed to lung transplantation as a potential option to those eligible with severe pulmonary disease, and we will continue to care for all CF patients in need of lung transplantation in the United States and world-wide.

Education

Fellows: Washington University provides a fully-accredited, board-certified, 3-year training program in Pediatric Pulmonary Diseases. We accept 2 fellows per year to our program, who rotate through our clinical services and directly care for CF patients both in clinic and when they are hospitalized. The program currently has 6 pediatric pulmonology fellows. Several clinical conferences are held regularly for the fellows, including a weekly lecture series that comprehensively reviews pulmonary physiology and pathophysiology, where CF is extensively covered in the curriculum, and clinical care conference that has a heavy emphasis on CF. Additional research conferences are held weekly throughout the Washington University Medical Center, many involving pulmonary infection, inflammation, host defense, lung injury, and tissue destruction both directly and indirectly relate to CF. The pulmonary fellows attend national conferences, including the North American Cystic Fibrosis Conference. We have areas of strong clinical and basic research, and several National Institutes of Health Clinical Training (T32) Grants, administered through the Departments of Pediatrics and Internal Medicine, support our fellows during their research training.

Residents: The Department of Pediatrics at the Washington University School of Medicine continues to attract highly talented and dedicated house officers. Residents are intimately involved in both the inpatient and outpatient care of our CF patients. As residents rotate through the Division of Pediatric Allergy and Pulmonary Medicine, which the majority of them do during their second- or third-year of training, they have the opportunity to participate in the care of CF patients in all stages of disease. They receive more formal education in the scheduled clinical conferences held by the division and department, and CF has been a regular topic for discussion in Department of Pediatrics Grand Rounds, Case Management Conference, and Early Bird rounds.

Medical students: Washington University School of Medicine is distinguished as one of the most competitive in the nation, and has the largest Medical Science Training Program (MSTP) in the country. Medical students are exposed to CF in their pulmonary pathophysiology courses, and pre-clinical students have been increasingly attracted to both clinical and basic CF research. Cystic fibrosis is also a topic of the Markey Program, which exposes Ph.D. students and post-doctoral fellows to important clinical topics.

During their third year clinical training, medical students are involved in the care of hospitalized CF patients, and the division faculty review cases with students who are rotating through pediatrics. These students also participate in the departmental lectures that deal with CF. As fourth-year students, many medical students from Washington University and other schools rotate through the division, participating in Cystic Fibrosis Clinics and the divisional educational programs.

Community outreach: The Cystic Fibrosis Center produces a biannual newsletter for families, and is involved in several community activities, including the Washington University Mini-Medical School, family educational programs, like Cystic Fibrosis Parents Night Out, and Center Parent Advisory Committee. The Washington University Cystic Fibrosis Center also supports monthly Cystic Fibrosis Family Network for families of CF children.

Cystic Fibrosis Research: Clinical Trials

The Cystic Fibrosis Clinical Center at Washington University is devoted to improving the health and well-being of patients with CF, whether it involves conventional treatments, applying novel therapies and pharmaceuticals, or, in patients with advanced disease, organ transplantation. The Center has considerable experience with single and multi-center trials, and the clinical research program in CF has rapidly grown, focusing on: (i) infection and inflammation in the CF lung; (ii) genetic and environmental determinants of lung disease; and (iii) growth and nutrition of CF patients.

During the past 5 years, patients from the Washington University Cystic Fibrosis Center have participated in numerous Human Studies Committee (IRB)-approved protocols, including multi-center research studies and clinical trials, and the number has escalated with our Center's inclusion in the Therapeutics Development Network (TDN). Please contact Mary Boyle M.S.N. or Jane Quante R.N. at (314) 454-2353 if you would like additional information regarding active clinical trials at our center.

Cystic Fibrosis Research: Basic and Translational Research

The Center has maintained close, active collaborative relationships with clinical and basic science departments, most notably with physicians and scientists from the Departments of Internal Medicine, Molecular Microbiology, Cell Biology and Physiology, the Mallinckrodt Institute of Radiology, and Washington University Genome Sequencing Center.

Affiliate Cystic Fibrosis Center Programs

There are two Affiliate Cystic Fibrosis Clinical Centers at the Memorial Medical Center at the Southern Illinois University School of Medicine in Springfield, Illinois (Director: Lanie Eagleton) and the Carle Clinic in Urbana-Champaign, Illinois (Director: Dr. Donald Davison). Both programs care for approximately 20 to 40 CF patients, and have the facilities and necessary complement of physician, nursing, and ancillary staff to accommodate their patients. Our Center continues to see patients with Dr. John Carlile, a board-certified pediatric pulmonologist, and his staff at the Cystic Fibrosis Outreach Clinic, located at Cox Medical Center in Springfield, Missouri three times per year.