Elsevier

The Journal of Pediatrics

Volume 147, Issue 3, September 2005, Pages 295-301
The Journal of Pediatrics

Commentary
Improving Subspecialty Healthcare: Lessons from Cystic Fibrosis

https://doi.org/10.1016/j.jpeds.2005.03.044Get rights and content

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Cystic Fibrosis

Treatment advances have dramatically improved patient survival since CF was first described in 1938 (Figure 1), changing the face of the disease in a relatively short period of time.5, 6, 7 The median predicted survival age was 33.5 years in 2003, and about 40% of patients with CF are currently over the age of 18. Nonetheless, there is considerable variability in age at death among the CF population (Figure 2). Much of this variation can be explained by individual patient differences in genetic

Variation in Care is Associated with Variation in Outcomes

Reports from both the CF Registry and the Epidemiologic Study of CF (ESCF), an industry-sponsored patient registry that operates independently of the CFF, demonstrate that patients with CF do not consistently receive optimal care. For example, although the CF Foundation has formulated relatively conservative guidelines for the regular monitoring of clinical status (timing of clinic visits, pulmonary function testing, airway cultures, and so forth), these routines are followed in only 58% to 79%

Conclusions

In its report, Crossing the Quality Chasm,1 the Institute of Medicine identified problems in the system of health care delivery rather than deficiencies in individual physicians' practice as the major impediment to attaining quality health care for all Americans. Effective interventions are available to slow or reverse the progress of many chronic diseases of childhood, and our patients are best served by ensuring that they consistently receive indicated treatment. Variations in disease outcome

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      The documentation of significant inconsistencies in care was a starting point of the CFF Quality Improvement program, with a reduction in treatment variations its goal [28, 29]. Steps needed to achieve this goal include an appreciation of the need for system-based changes that bring together the entire care team, including patients and families; microsystem-based changes in delivery system design; development and utilization of decision support tools such as care algorithms; and the use of data to provide feedback on the effectiveness of the effort [30–33]. The CFF benchmarking project from a decade ago found a number of key characteristics of programs with top-quintile clinical outcomes, including strong leadership, close tracking of clinical details and outcomes, high expectations and low thresholds for treatment, team consensus on standard approach to care, and education of patients/families on high outcome expectations and need for early aggressive intervention for declines [4, 12].

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    Supported by Cystic Fibrosis Foundation grant CFF SCHECH01C0QI.

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