CommentaryImproving Subspecialty Healthcare: Lessons from Cystic Fibrosis
Section snippets
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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Speaking of pandemics…
2021, Journal of Cystic FibrosisTobacco smoke exposure and socioeconomic factors are independent predictors of pulmonary decline in pediatric cystic fibrosis
2020, Journal of Cystic FibrosisCholestasis affects enteral tolerance and prospective weight gain in the NICU
2019, Clinical Nutrition ESPENCitation Excerpt :For example, only 37% of patients were seen by a gastroenterology consultant while in the NICU. Multidisciplinary team approaches are known to positively impact outcomes in healthcare settings [48–51], and patients who develop PN associated cholestasis could benefit from this care model as well. After this study ended, our center started a weekly multidisciplinary round-table discussion that included neonatologists, gastroenterologists, surgeons, radiologists, nurses, nutritionists, and pharmacists.
Impact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis
2018, Journal of Cystic FibrosisCitation Excerpt :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].
European cystic fibrosis society standards of care: Quality management in cystic fibrosis
2014, Journal of Cystic Fibrosis
Supported by Cystic Fibrosis Foundation grant CFF SCHECH01C0QI.