The majority of patients with cystic fibrosis (CF) will grow into adulthood. Despite this improvement in survival, the disease is progressive, especially with respect to the decline in pulmonary function, which in turn may have an impact on a patient's quality of life. In this study we evaluated the quality of life in CF adults and examined the relationship between quality of life and pulmonary function, exercise capacity, and dyspnea. We assessed in 15 patients in stable clinical condition their forced expired volume in the first second (FEV1) inspiratory vital capacity (IVC), cycle exercise capacity (Wmax), and subjective degrees of dyspnea during daily living (MRC dyspnea scale). Quality of life was assessed with the Sickness Impact Profile (SIP). A sample of 100 healthy individuals, ranging in age from 18 to 30 years, served as the control group. Mean (SD) age of the patients was 25.9 (7.3) years, FEV1 was 38 (16) % predicted, IVC was 65 (17) % predicted, FEV1 IVC ratio was 46 (10) %, and Wmax was 90 (54) W. The overall SIP and physical SIP scores in CF patients were significantly higher than in the controls, indicating more impairment in overall and physical functioning In the patients than in the control group (P < 0.001). The psychosocial SIP score did not differ significantly between the two groups. Overall SIP score correlated poorly with FEV1 % predicted (r = −0.33; n.s) and IVC % predicted (r = −0.36; n.s.) but showed a better and significant relationship to the maximal exercise capacity (r = −0.57; P < 0.05). MRC dyspnea scores showed a strong correlation with overall SIP scores (r = 0.75; P < 0.001). These results show that CF affects quality of life in adults primarily due to a limitation in physical functioning. Psychosocial functioning did not differ from that of healthy controls. Exercise capacity and dyspnea scores were related to the Impairment in the quality of life. Therefore, the effects of programs aimed to improve exercise capacity and reduce dyspnea on CF patients' quality of life need to be evaluated. Pediatr Pulmonol. 1997; 23:95–100. © 1997 Wiley‐Liss, Inc.

The median age of survival in patients with cystic fibrosis (CF) has improved considerably. Despite this improvement, deterioration of pulmonary function and decrease in exercise capacity are still the main problems for many patients. Although dyspnoea is a common complaint in CF patients, relatively little regard has been paid to this symptom. This study examined the relationship between dyspnoea, bicycle exercise capacity and pulmonary function in patients with CF. In 14 patients in a stable clinical condition, pulmonary function [forced expiratory volume in 1 s (FEV1), inspiratory vital capacity (IVC)], bicycle exercise capacity [maximum exercise capacity (Wmax)], subjective degree of dyspnoea during daily living [Medical Research Council (MRC) dyspnoea scale], and during exercise (Borg scale) were assessed. The mean (SD) age of the patients was 25 (6.8) years, FEV1 was 41 (19)% predicted, IVC was 63 (17)% predicted and FEV1/IVC ratio was 47 (10)%; median (range) Wmax was 55 (0-79)% predicted. Bicycle exercise test performance appeared to be mainly determined by pulmonary function and MRC dyspnoea grade; multiple regression equation containing FEV1 and dyspnoea accounted for 76% of the variance in Wmax (% predicted) (Wmax = -7.9 dysp + 1.1 FEV1, +24). Exercise dyspnoea, assessed by the Borg scale, showed a significant linear correlation with minute ventilation. (VE), maximal voluntary ventilation (MVV) (%) (r = 0.76; P < 0.001). Medical Research Council dyspnoea score correlated relatively poorly with FEV1 (% predicted) (r = -0.17; n.s.) and IVC (% predicted) (r = -0.48; n.s.). Borg score at maximal exercise did not correlate with MRC dyspnoea score (r = -0.07). Borg50% score correlated significantly with MRC dyspnoea score (r = 0.61; P < 0.05). These results show that dyspnoea has an influence on exercise capacity. Dyspnoea score showed a large inter-individual variation, not strongly related to pulmonary function. It is concluded that dyspnoea deserves more attention in CF patients and needs to be assessed in rehabilitation programmes and other intervention studies in these patients.

Data on the pharmacokinetics of antibiotics after inhalation are limited. The aim of this pilot study was to assess the pharmacokinetics of tobramycin under optimalized and standardized aerosol circumstances and, furthermore, to be able to consider possible treatment of exacerbations with inhalation therapy. Six patients were studied after inhalation of 600 mg tobramycin. A jet nebulizer loaded with a 10% solution of tobramycin in water was used. The percentage of the dose that was systemically absorbed ranged from 1.0% to 16.6%. The maximum serum levels of tobramycin ranged from 0.77 mg/L to 3.63 mg/L (mean 1.70 +/- 1.01). The pharmacokinetic data were best described by a two-compartment model. Compared to intravenous administration, the long terminal half-life (mean 9.47 h +/- 3.28 h) could be explained by the slow absorption of tobramycin from the site of administration (flip-flop model). Despite standardized aerosol conditions, considerable interpatient variability was observed. However, the relatively low serum levels allow a further increase of the dose.

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Renal function impairment is common after solid organ transplantation, due to the nephrotoxicity of cyclosporine. Moreover, in patients with severe respiratory failure, renal function is often impaired. This renal function impairment may predispose patients to further renal function impairment after lung transplantation. Therefore, renal hemodynamics were measured in 44 patients before lung transplantation and 1, 6, 12, 18, 24, and 30 months after transplantation. After transplantation, a decline in renal function occurred, with a progressive fall in glomerular filtration rate (GFR) of 33 +/- 4% at 12 months and 42 +/- 9% at 30 months. Effective renal blood flow fell by 22 +/- 5% at 12 months and remained stable thereafter. Changes in effective renal plasma flow (ERPF) were less pronounced than those of effective renal blood flow, due to a fall in hematocrit after transplantation. Blood pressure and renal vascular resistance increased significantly, consistent with the effects of cyclosporine. Prior to transplantation, renal function impairment with intense renal vasoconstriction had been found in a subset of the patients. Remarkably, the decrease in renal function after transplantation was less pronounced in patients with renal function impairment prior to transplantation, as indicated by significant negative correlations between pretransplantation GFR and the percentage change in GFR after transplantation, and pretransplantation ERPF and the percentage change in ERPF after transplantation. This suggests that the net course of renal hemodynamics after lung transplantation is the result of the opposed effects of cyclosporine nephrotoxicity and the favorable effects of the normalization of respiratory status. In conclusion, after lung transplantation a decline in renal function occurs that is less pronounced in patients with renal function impairment and intense renal vasoconstriction prior to transplantation. Such a renal function impairment, therefore, should not be considered a contraindication to lung transplantation.