de Jong W, Kaptein A A, van der Schans C P, Mannes G P, van Aalderen W M, Grevink R G, Koëter G H
Department of Rehabilitation, University Hospital, The Netherlands.
Pediatr Pulmonol. 1997 Feb;23(2):95-100. doi: 10.1002/(sici)1099-0496(199702)23:2<95::aid-ppul4>3.0.co;2-n.
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.
大多数囊性纤维化（CF）患者会长大成人。尽管生存率有所提高，但该疾病仍在进展，尤其是在肺功能下降方面，而这又可能对患者的生活质量产生影响。在本研究中，我们评估了成年CF患者的生活质量，并研究了生活质量与肺功能、运动能力和呼吸困难之间的关系。我们对15例临床状况稳定的患者进行了评估，测量了他们的第一秒用力呼气量（FEV1）、吸气肺活量（IVC）、循环运动能力（Wmax）以及日常生活中的主观呼吸困难程度（MRC呼吸困难量表）。使用疾病影响量表（SIP）评估生活质量。选取100名年龄在18至30岁之间的健康个体作为对照组。患者的平均（标准差）年龄为25.9（7.3）岁，FEV1为预测值的38（16）%，IVC为预测值的65（17）%，FEV1/IVC比值为46（10）%，Wmax为90（54）瓦。CF患者的总体SIP和身体SIP评分显著高于对照组，表明患者在总体和身体功能方面的受损程度高于对照组（P < 0.001）。两组之间的心理社会SIP评分无显著差异。总体SIP评分与预测的FEV1%（r = -0.33；无统计学意义）和预测的IVC%（r = -0.36；无统计学意义）相关性较差，但与最大运动能力呈现出更好的显著关系（r = -0.57；P < 0.05）。MRC呼吸困难评分与总体SIP评分显示出强烈的相关性（r = 0.75；P < 0.001）。这些结果表明，CF主要通过身体功能受限影响成年患者的生活质量。心理社会功能与健康对照组无差异。运动能力和呼吸困难评分与生活质量受损相关。因此，需要评估旨在提高运动能力和减轻呼吸困难的方案对CF患者生活质量的影响。
