Psychological concomitants of cystic fibrosis in children and adolescents

Thirty cystic fibrosis (CF) and 30 matched control children and their parents were administered several psychiatric inventories including the child (DICA) and parent (DICA-P) versions of the Diagnostic Interview for Children and Adolescents, the Child Behavior Check List, the Hopelessness Scale, and the Piers-Harris Children's Self-Concept Scale. Data analysis revealed few differences in either psychopathological symptoms or psychiatric diagnoses between the CF and control children. The differences which did emerge were either physical in nature (reflecting somatic complaints) or did not depart enough from normal scores to merit the label of high psychopathology. The results are discussed in terms of the growing evidence that CF children do not suffer from greater psychopathology than do normal children.     

Newborn screening for cystic fibrosis: Educational implications

We examined the educational implications of newborn screening for cystic fibrosis (CF) as performed by combining the measurement of immunoreactive trypsin with analysis for the most common CF mutation, F508. Four out of 77 (5%) of maternity staff from 11 hospitals in rural New South Wales, Australia had learned about the salient features of the screening protocol from a pamphlet distributed from a central laboratory. In comparison, a didactic lesson resulted in a significantly greater (p<0.00006) number of maternity staff learning about the salient features of the screening protocol. Most maternity staff expanded their explanation to parents of newborn babies because of the didactic lesson.     

Family and childhood adjustment in cystic fibrosis

Family adaptation has been commonly associated with the psychological adjustment of chronically ill children. However, few studies have attempted to systematically evaluate this association and its relationship to illness severity. We studied 44 children ages 7 to 15 and their families at a large cystic fibrosis center and obtained measures of 1) impact of illness on the family; 2) family functioning; 3) behavioral adjustment; 4) social competence; 5) ratings of anxiety, depressive, and eating disorder symptoms; and 6) ratings of illness severity and duration. Impact of illness on the family and overall family dysfunction were significantly correlated with illness severity, but not duration. However, impact of illness on the family was significantly correlated with internalizing behavioral symptoms, while family dysfunction was correlated with depressive symptomatology. These findings suggest that illness-related stress is primarily reflected in general emotional and behavioral symptoms, with familial adaptation either ameliorating or exacerbating their development into depressive symptomatology.     

A survey of reimbursement for cystic fibrosis carrier testing

To assess the current status of reimbursement for cystic fibrosis (CF) carrier testing, we surveyed individuals tested in the Mid-Atlantic region. Results show that CF testing was covered by insurance in part or in full for greater than 50% of respondents. The test was nearly always covered when performed during pregnancy because of a positive family history, but it was also covered for more than 50% of pregnant respondents with a negative family history. There were no significant differences in coverage by type of insurance. Many respondents needed to supply additional information about the testing to their insurance company before a coverage decision could be made. Before population-based CF screening programs are initiated, more data are needed on insurance reimbursement for testing, especially when performed pre-conceptually.     

Attitudes toward genetic testing for cystic fibrosis among college students

The knowledge and attitudes of 25 college students toward clinical and genetic aspects of cystic fibrosis (CF) were assessed before and after an 80-minute presentation about the disease. The students were asked about their plans regarding genetic testing for cystic fibrosis prior to, and during future conceptions. While their knowledge about the disease increased significantly following the lecture, there were no significant changes in their attitudes.     

Education and testing strategy for large-scale cystic fibrosis carrier screening

Population-based screening for cystic fibrosis carrier mutations presents a number of challenges for genetic counselors, owing primarily to the inability of current DNA testing technology to identify all possible mutations and the difficulty involved in conveying the concept of residual risk to those patients who test negative. To address these issues, we are conducting a pilot study, as part of a consortium established by the National Center for Human Genome Research, to explore the efficacy, acceptance, and psychosocial impact of various approaches to carrier screening in an ethnically diverse Southern California population. This article reports the patient instructional and screening strategies we developed in the initial phase of the project in order to optimize our chances of answering these questions and delivering this service on a large scale.     

Reproductive issues in adults with cystic fibrosis: Implications for genetic counseling

This article reviews reproductive issues faced by the growing number of individuals with cystic fibrosis (CF) who reach adulthood. Approximately 97–98% of males with CF are infertile and they may have an increased risk for genitourinary anomalies. Females with CF may experience delayed puberty, irregular menstrual cycles, and decreased fertility. Women with CF who have good clinical scores, good nutritional status, and normal lung volumes with mild to moderate airway obstruction, have a better chance for successful pregnancies. The role of the genetic counselor in counseling adults with CF is discussed and resources for CF adults are provided.     

Homeostatic interactions: a longitudinal study of biological, psychosocial and family variables in children with cystic fibrosis

A two-Year longitudinal study of thirty-five children used data gathered from pulmonary function tests, clinical measurements and standardized tests of depression, self-esteem and family environment to illustrate homeostatic interactions among biological and psychosocial adaptation to cystic fibrosis. Despite physical deterioration over the course of the study, children with cystic fibrosis reported lower depression and higher self-esteem than population norms, and improvements in psychological functioning. Decline in biological indicators of health was associated with increases on an index based on Family Environmental Scales (FES), subscales of cohesion, expressiveness and organization, and maintenance of high self-esteem and low depression. Presence of additional major life stressors was associated with deterioration in pulmonary function, higher levels of depression and poorer scores on the FES. These findings suggest that homeostatic interactions between children's health status and family functioning may moderate the physical and psychological sequelae of cystic fibrosis.     

Parent group education: What does it do for the children

The Downing Program for Parent Training in Family Relationship and management Skills was introduced to the parents of students attending an alternative education school at the secondary level. With 58 parents completing all phases of the program, the results indicated that students with parents completing the Dowing program were significantly or markedly different from students whose parents did not participate in the Dowing program, using two measures of classroom behavior.     

Predicting medical compliance among adolescents with cystic fibrosis

This investigation evaluated the utility of a new assessment tool based on a competency/coping skills model in predicting medical compliance of adolescents with cystic fibrosis. The Medical Compliance Incomplete Stories Test (M-CIST) was administered to 40 adolescent and young adult inpatients aged 13 to 23. These data were compared to objective measures of the patient's medical compliance. The M-CIST was positively correlated with compliance (multiple R = .72, p less than .001) and discriminated compliant from noncompliant patients. Data derived from patients' responses on the assessment tool could also be used projectively to provide information useful to clinicians treating adolescents with cystic fibrosis.     

Visual routines for extracting magnitude relations

Linking relations described in text with relations in visualizations is often difficult. We used eye tracking to measure the optimal way to extract such relations in graphs, college students, and young children (6- and 8-year-olds). Participants compared relational statements (“Are there more blueberries than oranges?”) with simple graphs, and two systematic patterns emerged: eye movements that followed the verbal order of the question (inspecting the “blueberry” value first) versus those that followed a left-first bias (regardless of the left value’s identity). Question-order patterns led substantially to faster responses and increased in prevalence with age, whereas the left-first pattern led to far slower responses and was the dominant strategy for younger children. We argue that the optimal way to verify a verbally expressed relation’s consistency with visualization is for the eyes to mimic the verbal ordering but that this strategy requires executive control and coordination with language.     

Body image and cystic fibrosis: a critical review

A slight frame and poor appetite are common among patients with cystic fibrosis (CF) yet healthy body weight has been related to a better prognosis. A review of studies exploring body image (BI) among adults and adolescents with CF was conducted. Seven electronic databases were searched for potential papers. They located 128 references, of which 24 were read in full and 12 included in the review. Accepted papers suggested females with CF had a better BI compared to males, but this could compromise survival, given their preference for a low body weight. Males may be more motivated to adhere to nutritional advice because they favor a larger form. Practitioners should broach the topic of BI at clinic appointments to ensure this does not have a detrimental impact on self-management, although more research is required to guide professionals in this task.     

A comparison of two approaches to education about carrier testing for cystic fibrosis

We tested the efficacy of two types of educational materials for genetic counseling: a traditional information brochure and one adding a role model story. Brochures were alternated weekly at a prenatal genetics center. Subjects were asked to read the brochure and fill out a questionnaire covering demographics and variables from the health belief model (impact, barriers, motivation, susceptibility, knowledge, severity). A group of 409 pregnant women and 251 male partners participated. Study design was quasiexperimental, using a post-test only comparison group. The brochure with modeling enhanced the perception of both risk and the severity of the disease and was inversely associated with the assessment of barriers, but did not directly impact on the decision to pursue testing; only 12% chose to be tested, with no significant differences between groups. While suggestive, the study is not confirmatory and should be repeated with a more heterogenous group of women.     

Toolbox-based routines for Macintosh timing and display

Pascal routines are described for several real-time operations on Macintosh computers. Methods are presented for millisecond timing and for high-speed transfer of arbitrary bit-image displays to the screen. All routines are based on Toolbox procedures available on virtually all Macintosh computers.     

Depression in children: Parent,teacher, and child perspectives

Depressed and nondepressed children were found to differ in the types of behavior problems manifested at home and at school. Children rated as depressed by their parents on the Personality Inventory for Children evidenced significantly more conduct problems, anxiety, impulsive hyperactivity, learning problems, psychosomatic problems, perfectionism, and muscular tension at home than children rated as nondepressed. Depressed children were rated by their teachers as displaying more inattentionpassivity than nondepressed children. A significant but modest relationship was found between parent report and child selfreport of the child's depression. Depressed children attributed positive events to external causes and negative events to internal causes significantly more than did nondepressed children. The specificity of these results to depression was also examined;the particular features of childhood depression are compared to the features of adult depression. The investigators would like to thank Jim McFerren and Ralph Zalazar for their work as research assistants and David Watson for his help with the statistical analyses. The helpful comments of Eric Klinger and Auke Tellegen are gratefully acknowledged. The cooperation of Dr. Loren Benson, director of personnel services of the Hopkins School District #274; Mr. Edward Ryshavy, principal of Glen Lake Elementary School; and the teachers of that school is greatly appreciated.     

Parent support is less effective in buffering cortisol stress reactivity for adolescents compared to children

The goal of the present study was to investigate developmental differences in the effectiveness of parent support to alleviate hypothalamic‐pituitary‐adrenal (HPA) axis stress responses of children (ages 9–10, N = 40) and adolescents (ages 15–16, N = 41). We experimentally manipulated the provision of parent support during the speech preparation period before a modified Trier Social Stress Test (TSST) and examined its effect on levels of salivary cortisol secreted in response to this laboratory stressor. Analyses revealed a significant interaction of condition and age group such that social support from the parent (versus a stranger) significantly eliminated the cortisol stress response in children, but had no effect on the response among adolescents.     

Coping styles in adults with cystic fibrosis: implications for emotional and social quality of life

As life expectancy increases, interest has grown surrounding the factors that may influence quality of life (QOL) for people with cystic fibrosis (CF). The aim of the current study was to examine which specific coping styles were positively or negatively associated with social and emotional QOL in a CF sample. One hundred and twenty-two respondents aged 18 and over were recruited through an online support group. Respondents completed the ‘CF Questionnaire-Revised (CFQ-R)’ and the ‘Brief COPE’. The CFQ-R is a disease-specific instrument designed to measure the impact of CF on nine QOL domains and the Brief COPE is a 28 item questionnaire which assesses 14 coping scales. A multivariate regression model revealed that higher substance abuse and disengagement was associated with lower emotional QOL whereas greater use of religion, instrumental coping and acceptance was positively associated with emotional QOL. Active coping was linked to better social QOL and a negative association was reported between distraction coping with both emotional and social domains. Given the burden of CF, ascertaining which factors enhance or diminish emotion and social well-being is now an integral component of QOL research. The current findings may therefore have value in informing clinical interventions which aim to cater for the psychological needs of individuals with CF.     

Sweet sorrow: the color-shading response in an adult cystic fibrosis population

Research on the color-shading response on the Rorschach has been limited previously to its use as a predictor of suicide. The theoretical understanding of this response was addressed by some of the major Rorschach authors. Adult cystic fibrosis patients were administered a battery of psychological tests, including the Rorschach. While these patients face a premature death, none of these patients were suicidal. A higher than usual incidence of color-shading responses occurred. Rapaport's hypothesis that the color-shading response is an expression of a simultaneously conflicting emotion, or similar to "sweet sorrow," was suggested as a possible hypothesis.