AN EXPANDED SCORING SYSTEM INCLUDING AN INDEX OF NUTRITIONAL-STATUS FOR PATIENTS WITH CYSTIC-FIBROSIS

Evaluation of success or failure of therapy for patients with cystic f ibrosis (CF) commonly relies on the results of a single pulmonary func tion test (PFT). Most PFT measurements reflect different functional as pects of the lung. Although no single parameter can summarize all aspe cts of lung function, a combination of several may provide an advantag e by reflecting the overall abnormality of lung function in one number . Cropp et al. (1982, Am Rev Respir Dis 126:211-216) developed a multi parameter pulmonary function score (PFS) using the results of six sepa rate parameters obtained from a PFT. In CF, there is also the potentia l for declining nutritional status (NS), leading to malnutrition and s keletal muscle wasting. Our aim was to expand the PFS by including wei ghted information on NS, and to determine whether the expanded score ( nutritional-pulmonary function score, NPFS) was more sensitive in dete cting change in outcome variables than the PFS. Individual PFT paramet ers, percent ideal body weight (%IBW), and an index of anaerobic perfo rmance (AP) were measured in 21 patients on admission to the hospital and again at discharge. In the group as a whole, in-hospital therapy r esulted in improvement (P < 0.01) in individual PFT parameters, %IBW, PFS, and NPFS, and no change in AP. While the PFS more effectively ref lected improvement in lung function than did any single PFT parameter, the NPFS resulted in an even more sensitive index of change. Based on these results, we believe that the NPFS, which includes both lung and nutritional status, provides an effective and sensitive index of dise ase severity that can be used as a unifying measure to: 1) detect dise ase progression; 2) guide rehabilitation and training; 3) stratify pat ients for clinical trials; or 4) evaluate the effects of a therapeutic intervention. Pediatr Pulmonol. 1994; 18: 199-205. (C) 1994 Wiley-Lie s, Inc.