How many patients with cystic fibrosis receive recommended care? How have outcomes for cystic fibrosis patients changed over time?

Among patients with cystic fibrosis, a life-shortening genetic disease, 64 percent to 94 percent of children and adolescents and 51 percent to 87 percent of adults received recommended care during 2004. From 1990 to 2004, lung function improved by 13 percentage points among cystic fibrosis patients ages 6 to 30.

Why is this important?

• Cystic fibrosis is a life-shortening genetic disorder that affects approximately 30,000 Americans. The disease is characterized by chronic respiratory infections and gradual loss of lung function, typically accompanied by digestive disorder and malnutrition.
• In a comparison of process and outcomes of care across cystic fibrosis specialty centers, patients who received care in accordance with guidelines issued by the Cystic Fibrosis Foundation had better lung function—an important outcome associated with survival (Johnson et al. 2003).

Findings

Among cystic fibrosis patients whose treatment was represented in a national disease registry during 2004, up to one-third of children and adolescents (6 percent to 36 percent) and up to one-half of adults (13 percent to 49 percent) did not receive recommended care to help prevent disease complications.

• From 2002 to 2004, the performance of lung function measurement (spirometry to anticipate and treat complications and establish patterns of response to treatment) increased 2 to 3 percentage points.
• Performance decreased slightly (by 2 to 7 percentage points) for three other measures: outpatient monitoring visits to anticipate and treat physical and psychosocial problems, cultures to detect and treat respiratory pathogens, and testing for glucose intolerance or cystic fibrosis-related diabetes.

Among cystic fibrosis patients ages 6 to 30, lung function (measured as a percentage of the predicted value for healthy, nonsmoking people) improved by 13 percentage points on average from 1990 to 2004 (CFF 2003; CFF 2005b).

Implications

• Improvement in lung function probably reflects better nutrition and use of antibiotics and other medicines, along with patient airway clearance and exercise (CFF 2005b). Given average decline in lung function of about 1.5 to 2 percentage points per year in cystic fibrosis patients, the 13 percentage-point improvement from 1990 to 1994 represents many years of better quality life.
• Through improvements in treatment, the life expectancy for people with cystic fibrosis has increased from less than five years in the 1950s to 37 years in 2005 (CFF 2005a, 2006).

Improvement Ideas and Resources

The Cystic Fibrosis Foundation recently converted its patient registry, created in 1964, to a Web-based system that can be used by frontline care providers for real-time tracking and monitoring of patient care. The foundation is working with its network of care centers to train staff in quality improvement methodologies and to identify and share best practices for improving processes and outcomes of care (CFF 2004; Gawande 2004; Marshall 2003).

Measure:

For the first chart, the denominator is patients with cystic fibrosis in the Cystic Fibrosis Foundation patient registry. The numerator is the subset of the denominator population who received four monitoring and evaluation measures recommended by the Cystic Fibrosis Foundation for this condition. The second chart depicts the median forced expiratory volume measured over one second on a spirometry test, expressed as a percentage of the predicted value for healthy, nonsmokers (CFF 2005b).

Limitations:

These data do not include all individuals with cystic fibrosis. The measures do not include all guideline-recommended care processes, such as receipt of X rays and nutritional assessments.

Source:

The Cystic Fibrosis Foundation Patient Registry collected data in 2004 on 22,714 patients with cystic fibrosis who received care at more than 115 Cystic Fibrosis Foundation–accredited care centers across the U.S. Three of five (58.2%) of these patients (13,220) were younger than age 18 in 2004 (CFF 2003, 2005b).

References:

* Indicates source of data used in the chart(s).* CFF (Cystic Fibrosis Foundation). 2003. Patient Registry Annual Data Report 2002. Bethesda, Md.: Cystic Fibrosis Foundation.CFF (Cystic Fibrosis Foundation). 2004. The CF Foundation's Quality Improvement Initiative. Bethesda, Md.: Cystic Fibrosis Foundation. CFF (Cystic Fibrosis Foundation). 2005a. Facts About CF. Bethesda, Md.: Cystic Fibrosis Foundation. * CFF (Cystic Fibrosis Foundation). 2005b. Patient Registry Annual Data Report 2004. Bethesda, Md.: Cystic Fibrosis Foundation. CFF (Cystic Fibrosis Foundation). 2006. New Statistics Show CF Patients Living Longer. Bethesda, Md.: Cystic Fibrosis Foundation. Gawande, A. 2004. The Bell Curve. The New Yorker (Dec. 6). Johnson, C., S. M. Butler, M. W. Konstan et al. 2003. Factors Influencing Outcomes in Cystic Fibrosis: A Center-Based Analysis. Chest 123 (1): 20–7. Marshall, B. 2003. Quality Improvement is Priority One. Bethesda, MD: Cycstic Fibrosis Services, Inc.