Cochrane corner: psychological interventions for individuals with cystic fibrosis and their families.

Psychological issues associated with cystic fibrosis may arise from the patients' lifelong disease- and treatment-related burden. This Cochrane Review aimed to determine psychosocial and physical outcomes of psychological interventions. Trial registries, databases and professional networks were used to identify relevant studies. Altogether, 16 studies involving 556 participants were included. They were heterogeneous in their methods, design, target groups, and outcomes. Overall, the current evidence for psychological interventions is insufficient. Preliminary evidence was available for interventions targeting specific aspects of the treatment regimen, such as behavioural nutrition interventions.

Fear of disease progression questionnaire for parents: Psychometric properties based on a sample of caregivers of children and adolescents with cystic fibrosis.

BACKGROUND: Parents caring for a child with a chronic somatic condition are at risk of increased distress and impaired quality of life. Fear of disease progression (FoP) is known to be an important source of distress in patients and their partners, and may be of relevance for parents as well. Existing measures are not applicable to parents. This study describes the adaptation of the FoP questionnaire for parental caregivers and investigated its psychometric properties. METHODS: Sixteen items appropriate for parents were derived from existing measures and from interviews with clinical experts in family-oriented psychosocial care. Factor structure, internal consistency, validity and sensitivity to change were analyzed in a clinical sample of 162 caregivers (M(age)=42.07 years, SD=6.0 years, 87.8% female) of a child with cystic fibrosis. RESULTS: The exploratory factor analysis reveal ed a two-factor structure, which was not supported by confirmatory analysis. Cronbach's α was examined for total score (.91) and significant positive correlations of the total score with anxiety (HADS: r=.70) and depression (CES-D: r=.60, BDI-II: r=.59), and a significant negative correlations with quality of life (r=-.66) could be demonstrated. A significant decrease in FoP was found (d=1.11) in a group of highly distressed caregivers undergoing web-based cognitive behavioral intervention. CONCLUSIONS: The FoP questionnaire is a reliable and valid instrument for parents of children with CF. Further studies in larger samples are needed to clarify dimensionality and validity among parents of children with other chronic conditions.

A web-based psychological support program for caregivers of children with cystic fibrosis: a pilot study.

BACKGROUND: Parents caring for a child with Cystic Fibrosis (CF) are at high risk for psychological distress and have limited access to psychological care. Therefore, a web-based psychological support program for severely distressed parents of children with CF (WEP-CARE) was developed and evaluated for its feasibility and efficacy. METHODS: A clinical expert panel developed WEP-CARE based on principles of cognitive-behavioral therapy. This web-based writing therapy comprises nine sessions, tailored for the specific needs of caregivers. The pilot study was conducted as a single-group intervention with pre-post-follow-up design. Out of 31 participants, 23 parents completed the intervention (21 female; mean age 37 years; SD = 6.2 years, range 25 - 48 years). Psychological symptoms and quality of life were assessed online by self-report measures at pre- and post-treatment and were followed up three months later. RESULTS: On average, the caregivers' symptoms of anxiety decreased statistically significant and clinical relevant about five points from an elevated (M = 11.4; SD =2.6) to a normal level (M = 6.7; SD = 2.6; p < .001) between pre and post treatment. Fear of disease progression (p < .001) and symptoms of depression (p = .02) significantly decreased as well. Quality of life significantly improved (p = .01). The effects were maintained at the 3-months follow-up assessment. CONCLUSIONS: WEP-CARE is feasible and promising regarding its efficacy to improve parental mental health and quality of life.

Symptoms of depression impact the course of lung function in adolescents and adults with cystic fibrosis.

BACKGROUND: Epidemiological studies report high rates of depression among patients with cystic fibrosis (CF). Assuming a causal relationship between depression and the progression of CF, our hypothesis is that elevated symptoms of depression would be a predictor of worse lung function after two years. METHODS: In the context of the TIDES study, 473 German patients with CF (age 12-53 years, FEV1% predicted M = 66.2, range 13-137) completed the Hospital Anxiety and Depression Scale (HADS). Lung function (FEV1% predicted) was assessed at baseline and followed up two years later. Repeated measures analysis was performed involving the level of FEV1% and the level of depressive symptoms at baseline as independent factors and FEV1% at the 2-year follow-up as the dependent variable. RESULTS: Interaction between lung function and depression at baseline significantly affected the change in lung function at the 2-years observation interval. The largest decline in FEV1% occurred in depressed patients with good lung function at baseline. In contrast, patients without any clinically relevant depressive symptoms and with poor lung function at baseline showed a slight increase two years later. CONCLUSION: The findings emphasise the need to screen patients with CF for symptoms of depression and to treat co-morbid depression.

Psychological interventions for individuals with cystic fibrosis and their families.

BACKGROUND: With increasing survival estimates for individuals with cystic fibrosis, long-term management has become an important focus. Psychological interventions are largely concerned with adherence to treatment, emotional and social adaptation and health-related quality of life. We are unaware of any relevant systematic reviews. OBJECTIVES: To determine whether psychological interventions for people with cystic fibrosis provide significant psychosocial and physical benefits in addition to standard medical care. SEARCH METHODS: Studies were identified from two Cochrane trials registers (Cystic Fibrosis and Genetic Disorders Group; Depression, Anxiety and Neurosis Group), Ovid MEDLINE and PsychINFO; unpublished trials were located through professional networks and Listserves. Most recent search of the Cystic Fibrosis and Genetic Disorders Group's register: 19 December 2013.Most recent search of the Depression, Anxiety and Neurosis Group's register: 12 November 2013. SELECTION CRITERIA: Randomised controlled studies of a broad range of psychological interventions evaluating subjective and objective health outcomes, such as quality of life or pulmonary function, in individuals of all ages with cystic fibrosis and their immediate family. We were interested in psychological interventions, including psychological methods within the scope of psychotherapeutic or psychosomatic mechanism of action (e.g. cognitive behavioural, cognitive, family systems or systemic, psycho-dynamic, or other, e.g. supportive, relaxation, or biofeedback), which were aimed at improving psychological and psychosocial outcomes (e.g. quality of life, levels of stress or distress, psychopathology, etc.), adaptation to disease management and physiological outcomes. DATA COLLECTION AND ANALYSIS: Three authors were involved in selecting the eligible studies and two of these authors assessed their risk of bias. MAIN RESULTS: The review includes 16 studies (eight new studies included in this update) representing data from 556 participants. Studies are diverse in their design and their methods. They cover interventions with generic approaches, as well as interventions developed specifically to target disease-specific symptoms and problems in people with cystic fibrosis. These include cognitive behavioural interventions to improve adherence to nutrition or psychosocial adjustment, cognitive interventions to improve adherence or those associated with decision making in lung transplantation, a community-based support intervention and other interventions, such as self-hypnosis, respiratory muscle biofeedback, music therapy, dance and movement therapy, and a tele-medicine intervention to support patients awaiting transplantation.A substantial proportion of outcomes relate to adherence, changes in physical status or other specific treatment concerns during the chronic phase of the disease.There is some evidence that behavioural interventions targeting nutrition and growth in children (4 to 12 years) with cystic fibrosis are effective in the short term. Evidence was found that providing a structured decision-making tool for patients considering lung transplantation improves patients' knowledge of and expectations about the transplant, and reduces decisional conflict in the short term. One study about training in biofeedback-assisted breathing demonstrated some evidence that it improved some lung function measurements. Currently there is insufficient evidence for interventions aimed at other aspects of the disease process. AUTHORS' CONCLUSIONS: Currently, insufficient evidence exists on psychological interventions or approaches to support people with cystic fibrosis and their caregivers, although some of the studies were promising. Due to the heterogeneity between studies, more of each type of intervention are needed to support preliminary evidence. Multicentre studies, with consequent funding implications, are needed to increase the sample size of these studies and enhance the statistical power and precision to detect important findings. In addition, multicentre studies could improve the generalisation of results by minimizing centre or therapist effects. Psychological interventions should be targeted to illness-specific symptoms or behaviours to demonstrate efficacy.

Quality of life among parents of children with phenylketonuria (PKU).

BACKGROUND: Parents of children with chronic conditions are known to be at risk of impairment in their quality of life (QoL). Studies considering other chronic conditions proposed diverse factors to have an impact on the parent's QoL. So far, there has been little research on parents who have a child with phenylketonuria (PKU). This study was designed to evaluate the parental quality of life (PQoL) of parents of children and adolescents who have PKU and identify possible predictors of PQoL. METHODS: In this cross-sectional study 89 parents completed self-report measures of PQoL, family stress, social support, and parental coping. To determine the impact of these potential predictors on PQoL, regression and mediation analyses were performed. RESULTS: Most parents coped well with their children's metabolic disorder. Family stress (ß = -0.42; p < 0.001) and perceived social support (ß = 0.33; p = 0.001) were proven to be the most powerful predictors, accounting together for 45% of the variance of PQoL. Social support mediated the association between family stress and PQoL. CONCLUSIONS: The current study indicates that parents of younger children are an especially vulnerable group. Members of health-care teams should be able to identify and empower vulnerable parents to seek and maintain social support.