Cystic fibrosis and the transition to adulthood

Cystic fibrosis (CF) has long been thought of as a childhood disease but the life expectancy for those with CF has been steadily on the rise and now reaches well into adulthood. There has been little or no research on the psychosocial impact of CF on becoming an adult. This exploratory, qualitative study examined the perceptions of young adults with CF regarding the impact the disease has had on their lives, especially during the transition from adolescence to adulthood. The findings indicate concerns about health insurance and finances, as well as the achievement of independence and optimism for the future.     

Feasibility study of community control programmes for cystic fibrosis: memorandum from a WHO/ICF(M)A meeting.

As a result of improved medical services and diagnosis of cystic fibrosis (CF), it has in recent years become clear that this is a common genetic disorder with a worldwide distribution. The average life expectancy of CF patients is very low in the developing world, but in developed countries this fatal childhood disease is becoming a chronic disorder persisting into adult life. In western Europe and North America the average life expectancy is now about 25 years, with a fatal outcome, so that CF represents one of the most serious of inherited life-threatening conditions. It remains to be shown whether very early diagnosis and treatment can further improve the prognosis.     

Adults with cystic fibrosis: spiritual coping with lifelong disease

Abstract

Cystic fibrosis (CF) is a chronic life-shortening disease requiring significant coping. Spiritual belief relates to treatment behaviors. Little is known about spirituality’s role in adults diagnosed as children, nor how it compares with adults diagnosed as adults. Adults over 18?years, diagnosed as children completed a questionnaire; some were randomized to also participate in an interview or daily phone diary to measure adherence. Qualitative analyses of 25 adults are presented. Participants reframed their disease as part of a Divine Plan, in which Divine assistance was conditional upon adherence. Linear regression models of spiritual constructs on airway clearance, nebulized medication, and exercise are presented. Adults diagnosed as children related spirituality to CF in ways both consistent and different from adults diagnosed as adults. Spiritual beliefs were related to adherence determinants and intentions. Increased understanding of the relationship between spirituality and health behaviors is important to providing person-centered care.     

Child health on a dollar a day: some tentative cross-country comparisons

Children living on a dollar a day-the international extreme poverty line-appear to have radically different chances of dying in childhood and being malnourished, depending on the country in which they live. In Kazakhstan, a child living on a dollar a day, has only a 10% risk of being underweight, while the risk facing a child living on a dollar a day in India is nearly 60%. The Kazakh child has a risk of less than 40 per 1000 of dying before his first birthday, while a child living on a dollar a day in Niger faces a risk of nearly 160 per 1000. Countries where mortality and malnutrition risks at a dollar a day are high are not typically those where there are large gaps in child survival and in malnutrition between the poor and better-off. The two concepts of inequality and health risks at the poverty line are not only conceptually distinct-they are empirically distinct too. The large differences between countries in the risks of mortality and malnutrition in childhood beg the obvious question-what accounts for these differences? Some regression results presented in the paper suggest that these differences may be due to differences across countries in levels of per capita expenditure on the health sector. Regressions find that higher levels of per capita public spending on the health sector are associated with significantly lower levels of mortality and malnutrition amongst children living on a dollar a day.     

Child depression: anthropological approach

OBJECTIVE: To understand the sociocultural meanings of childhood depression, from the medical-scientific concept of the disease. METHODS: This was a qualitative study carried out in the metropolitan region of S?o Paulo, State of S?o Paulo, Brazil, in 2003. It consisted of ethnographic observation and in-depth interviews with eight psychiatrists from a public health service and nine relatives (parents or guardians) of children who had been diagnosed with and were being treated for childhood depression. The analysis sought to identify categories that would make it possible to isolate different notions of the disease, as expressed in the discourse of these groups. RESULTS: Different notions of the disease were identified, in accordance with the cultural patterns of the discourse. For the psychiatrists, the concept of childhood depression was related to inappropriate child behavior, or "bad functioning", which then had to be adjusted by medical intervention. For the relatives, childhood depression meant "dissatisfaction" and "discomfort" with life and "intolerance" of adults to child behavior. It was seen that the discourse of the psychiatrists and relatives interviewed showed great diversity of subjects, concepts and categories, according to the logic of their particular understandings and explanations for childhood depression. CONCLUSIONS: In the light of the results obtained, the phenomenon of childhood depression can be analyzed not as an event determined by medical-scientific knowledge, but as a dynamic process of "creative reinvention" of categories and concepts that are fundamental to this discourse. It is therefore concluded that childhood depression presents as a differentiated disease, in the form of a broad category that is capable of integrating different connotations and contexts under the same term.     

Child health on a dollar a day: some tentative cross-country comparisons

Children living on a dollar a day – the international extreme poverty line – appear to have radically different chances of dying in childhood and being malnourished, depending on the country in which they live. In Kazakhstan, a child living on a dollar a day has only a 10% risk of being underweight, while the risk facing a child living on a dollar a day in India is nearly 60%. The Kazakh child has a risk of less than 40 per 1000 of dying before his first birthday, while a child living on a dollar a day in Niger faces a risk of nearly 160 per 1000. Countries where mortality and malnutrition risks at a dollar a day are high are not typically those where there are large gaps in child survival and in malnutrition between the poor and better‐off. The two concepts of inequality and health risks at the poverty line are not only conceptually distinct – they are empirically distinct too. The large differences between countries in the risks of mortality and malnutrition in childhood beg the obvious question – what accounts for these differences? Some regression results presented in the paper suggest that these differences may be due to differences across countries in levels of per capita expenditure on the health sector. Regressions find that higher levels of per capita public spending on the health sector are associated with significantly lower levels of mortality and malnutrition among children living on a dollar a day.     

Factors influencing health insurers' decisions to cover new genetic technologies

OBJECTIVE: To examine the relative importance of factors influencing health insurers' coverage of new genetic technologies. METHODS: A national survey in which the decision makers for private health insurers were asked whether they would cover cystic fibrosis (CF) carrier screening, testing for genetic susceptibility to breast cancer (BRCA test), and medical costs of a clinical trial of gene therapy for CF under a variety of conditions. RESULTS: Respondents' coverage of the two tests and of medical costs of clinical trials was low at the time of the study (4%-15.5% of insurers). Their coverage of CF carrier screening and BRCA testing would be increased significantly if the group tested was restricted to those at high risk, if detection rates were higher and costs lower, and if testing was endorsed by a national professional group or consensus conference. Coverage of the medical costs of a trial of CF gene therapy would be significantly more likely if the trial was restricted to children or adults with severe CF, safety and effectiveness was proven, and therapy could be administered in a regional hospital or an outpatient setting rather than in a research hospital. CONCLUSIONS: Health insurers play a critical role in the diffusion of new genetic technologies. The validity of genetic tests and the safety and effectiveness of new therapies are primary factors influencing health insurers' coverage. Lower costs and approval of professional groups are other factors associated with increased coverage.     

Using Spirituality After an Adult CF Diagnosis: Cognitive Reframing and Adherence Motivation

Chronic illness is a significant stressor; the majority of Americans cope utilizing spirituality. Numerous studies demonstrate links between spiritual coping and health outcomes. The purpose of this study was to determine whether persons diagnosed with cystic fibrosis (CF) as adults use spirituality to cope and influence disease management. Semi-structured interviews were completed and analyzed using grounded theory.

Data saturation was reached following twelve interviews (83% female); representing 100% participation of those approached and 48% of eligible adults. Persons with late-life CF diagnoses used spirituality to make meaning, understanding themselves in a collaborative partnership with their pulmonologist and God. Supporting themes were: (a) God's intervention depended on treatment adherence and (b) spiritual meaning was constructed through positively reframing their experience.

The constructed meaning differed from that of adult parents of children with CF. Late-life diagnosed adults focused on personal responsibility for health. Clinical and research implications for chaplains are presented.