Szyndler Janina E, Towns Susan J, van Asperen Peter P, McKay Karen O
Department of Adolescent Medicine, The Children's Hospital at Westmead, New South Wales, Australia.
J Cyst Fibros. 2005 May;4(2):135-44. doi: 10.1016/j.jcf.2005.02.004.
The life expectancy of individuals with CF has increased to 33 years. Thus, issues such as quality of life and psychological well-being, previously thought to be of lesser importance than physical well-being, are now recognised as significant factors. This study examined the interrelationships between quality of life, family functioning, individual psychopathology and optimism of adolescents with CF.
Adolescents attending the CF clinic completed a number of questionnaires. Quality of Life was measured using the Cystic Fibrosis Questionnaire, family functioning by the Family Environment Scale (3rd edition), general psychopathology with the Symptom Checklist-90-Revised and optimism for the future by the Hunter Opinions and Personal Expectations Scale. Disease severity was assessed using the Shwachman score and spirometry at the time of questionnaire completion.
The level of psychopathology (12.5% of those 13 years and over) in the group was lower than that reported for young people in Australia (15-20%). The results indicated that young people with a delayed diagnosis and those who are alienated from their families may be in need of additional psychosocial support. The group was hopeful and positive about their future and these attributes were independent of clinical measures of disease severity. In general, these young people scored relatively highly on the quality of life scale. For example the mean standardised score for physical functioning was 70 points, for respiratory symptoms was 63 points and for emotional state was 78 points. Increased levels of psychopathology and lack of hope for the future were however associated with lower ratings on a number of quality of life measures. Family cohesiveness, expressiveness and organization were associated with better psychological functioning in the young people.
Adolescents with CF appear to be a psychologically well functioning and well-adjusted group. These findings support the importance of a more sophisticated model of well-being for adolescents with CF, which explores the young person's views on their quality of life and wider support frameworks rather than relying solely on measures of physical health to gauge well-being.
患有囊性纤维化（CF）的个体预期寿命已增至33岁。因此，诸如生活质量和心理健康等问题，以前被认为不如身体健康重要，现在则被视为重要因素。本研究探讨了CF青少年的生活质量、家庭功能、个体精神病理学与乐观主义之间的相互关系。
在CF诊所就诊的青少年完成了多项问卷调查。使用囊性纤维化问卷测量生活质量，用家庭环境量表（第3版）评估家庭功能，用症状自评量表修订版评估总体精神病理学，用亨特观点和个人期望量表评估对未来的乐观主义。在完成问卷调查时，使用施瓦赫曼评分和肺功能测定评估疾病严重程度。
该组的精神病理学水平（13岁及以上人群中的12.5%）低于澳大利亚年轻人报告的水平（15 - 20%）。结果表明，诊断延迟的年轻人以及与家人疏远的年轻人可能需要额外的心理社会支持。该组对自己的未来充满希望且积极乐观，这些特质与疾病严重程度的临床指标无关。总体而言，这些年轻人在生活质量量表上得分相对较高。例如，身体功能的平均标准化得分为70分，呼吸症状为63分，情绪状态为78分。然而，精神病理学水平的升高和对未来缺乏希望与多项生活质量指标的较低评分相关。家庭凝聚力、表达性和组织性与年轻人更好的心理功能相关。
患有CF的青少年似乎是心理功能良好且适应良好的群体。这些发现支持了为CF青少年建立更复杂的幸福模型的重要性，该模型探索年轻人对其生活质量的看法和更广泛的支持框架，而不是仅仅依靠身体健康指标来衡量幸福。
