Growing Older with Cystic Fibrosis: Psychologic Adjustment of Patients More Than 16 Years Old

Abstract

The 79 female and 147 male patients constituting the population with cystic fibrosis (CF) aged 16 years and older attending The Hospital for Sick Children were asked to complete the Cornell Medical Index (CMI) and Tennessee Self-Concept Scale (TSCS); 64 female (81%) and 112 male (76%) subjects participated. Analysis of CMI results showed 43% of female subjects to have moderate to severe emotional disturbance compared to 19% of male subjects. This female : male ratio for severity of emotional disturbance is found in ostensibly healthy groups, but the percentages of disturbance approach values for medical patient populations. The frequency of emotional disability is greater in those more than 20 than in those 16-19 years old. The TSCS results portray a generally normal self-concept except for scores of positive physical self and psychosis for patients aged 20 years and older; these scores approach psychiatric values, suggesting that some reality distortion facilitates emotional adjustment to adult life with CF. The TSCS and CMI results correlate significantly, indicating a connection between self-concept and emotional status. However, TSCS and CMI scores do not correlate with measures of disease severity except for correlations between lung function and physical self-concept in older male patients. These results suggest that psychologic functioning is independent of the degree of physical impairment in older patients with CF, with the long-surviving male patients more realistically appraising the limitations their disease imposes and utilizing denial and minimization to a lesser degree. Demographic data on the clinic population reveal that most patients aged 16 years and older cope with their intellectual, developmental, and socioeconomic tasks commensurate with normal age expectations.

Work disability in adults with cystic fibrosis and its relationship to quality of life

“…Vocational attainment is an important consideration and many individuals with CF are highly motivated to work with 94% indicating a current or past employment history. Studies surveying workforce participation rates range from 61% [16], 77% [17] to over 90% for combined work and advanced education participation [4,18]. The collective findings highlight that most CF patients have aspired to, or participated in, paid employment.…”

Quality of life in patients awaiting lung transplant: Cystic fibrosis versus other end-stage lung diseases

“…Similarly, Cowen et al 9 found that 59% of women with CF over age 20 were emotionally distressed (as measured by the Cornell Medical Index), and 22% of adult men with CF were distressed.…”

“…Nineteen participants reported having, on average, 1.2 children living at home. The average number of years of education for subjects was 13.9 (SD, 2.7; range, [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. The median age of the patients with CF was 26 years (range, 18-39); the median age of non-CF patients was 46 years (range, 18-62).…”

The role of biomedical and psychosocial processes in the intellectual and academic functioning of children and adolescents with cystic fibrosis

“…di Sant' Agnese and Davis (1979) report that 85% of their adult cystic fibrosis patients were involved with school, career, or household chores. Similarly, Cowen et al (1984) report that 93% of the male and 85% of the female cystic fibrosis patients aged 16 or older either were attending school or were employed full-time.…”