Development and validation of a short form psychometric tool assessing the caregiving Challenge of Living with Cystic Fibrosis (CLCF-SF) in a child.

OBJECTIVE: Caring for a child with cystic fibrosis (CF) is a rigorous daily commitment for caregivers and treatment burden is a major concern. We aimed to develop and validate a short form version of a 46-item tool assessing the Challenge of Living with Cystic Fibrosis (CLCF) for clinical or research use. DESIGN: A novel genetic algorithm based on 'evolving' a subset of items from a pre-specified set of criteria, was applied to optimise the tool, using data from 135 families. MAIN OUTCOME MEASURES: Internal reliability and validity were assessed; the latter compared scores to validated tests of parental well-being, markers of treatment burden, and disease severity. RESULTS: The 15-item CLCF-SF demonstrated very good internal consistency [Cronbach's alpha 0.82 (95%CI 0.78-0.87)]. Scores for convergent validity correlated with the Beck Depression Inventory (Rho = 0.48), State Trait Anxiety Inventory (STAI-State, Rho = 0.41; STAI-Trait, Rho = 0.43), Cystic Fibrosis Questionnaire-Revised, lung function (Rho = -0.37), caregiver treatment management (r = 0.48) and child treatment management (r = 0.45), and discriminated between unwell and well children with CF (Mean Difference 5.5, 95%CI 2.5-8.5, p < 0.001), and recent or no hospital admission (MD 3.6, 95%CI 0.25-6.95, p = 0.039). CONCLUSION: The CLCF-SF provides a robust 15-item tool for assessing the challenge of living with a child with CF.

Development and preliminary validation of the challenges of living with cystic fibrosis (CLCF) questionnaire: a 46-item measure of treatment burden for parent/carers of children with CF.

OBJECTIVE: Treatments for cystic fibrosis (CF) are complex, labour-intensive, and perceived as highly burdensome by caregivers of children with CF. An instrument assessing burden of care is needed. DESIGN: A stepwise, qualitative design was used to create the CLCF with caregiver focus groups, participant researchers, a multidisciplinary professional panel, and cognitive interviews. MAIN OUTCOME MEASURES: Preliminary psychometric analyses evaluated the reliability and convergent validity of the CLCF scores. Cronbach's alpha assessed internal consistency and t-tests examined test-retest reliability. Correlations measured convergence between the Treatment Burden scale of the Cystic Fibrosis Questionnaire-Revised (CFQ-R) and the CLCF. Discriminant validity was assessed by comparing CLCF scores in one vs two-parent families, across ages, and in children with vs without Pseudomonas aeruginosa (PA). RESULTS: Six Challenge subscales emerged from the qualitative data and the professional panel constructed a scoresheet estimating the Time and Effort required for treatments. Internal consistency and test-retest reliability were adequate. Good convergence was found between the Total Challenge score and Treatment Burden on the CFQ-R (r=-0.49, p = 0.02, n = 31). A recent PA infection signalled higher Total Challenge for caregivers (F(23)11.72, p = 0.002). CONCLUSIONS: The CLCF, developed in partnership with parents/caregivers and CF professionals, is a timely, disease-specific burden measure for clinical research.

Unravelling complexities involved in parenting a child with cystic fibrosis: an interpretative phenomenological analysis.

We conducted a qualitative study with caregivers for a contemporary understanding of the challenge of caring for a child with cystic fibrosis (CF). A single case is presented that details one woman's experience from her unique perspective of both, 'mother to a child with CF' and 'CF healthcare provider' using an interpretative phenomenological analysis. Emergent themes include: 'trying to keep things normal,' which includes different types of normality and routine management of treatment; 'when things become difficult,' which includes dealing with symptoms and battling with CF related decline; and, 'the complexity of decision making.' The discussion section expands on how, for this mother contrasting modes of managing CF (everyday life/full-on alert) co-existed and were further complicated by (i) role discrepancies (mother/nurse), and (ii) a dialectic between affect and reason. The CF parenting challenge increasingly involves responsibility for complex healthcare interventions and this study suggests a need for further enquiry into how caregivers are involved in the treatment plan and decision-making about treatment. Practice implications are proposed.

Through a mother's lens: a qualitative analysis reveals how temporal experience shifts when a boy born preterm has cystic fibrosis.

We present a qualitative case study of one woman's experience of bringing up a child with cystic fibrosis (CF), born prematurely, using interpretative phenomenological analysis (IPA). This mother's existential account portrays time sequences for developmental milestones and synchronization of lifecycle events as atypical. Her perception of her child fluctuated radically; illness seemed to displace time, which collapsed with adversity. The temporal relations of CF and preterm birth are blurred at certain points. Although the challenge of compromised health may in some ways have promoted her son's development, his immature self-expression moved this mother towards an insider perspective of his experience. Cystic fibrosis is an ominous presence that demands respect even when the child is well. Anticipating the psychological impact of biomedical interventions can help to minimize trauma and maximize adaptation. Talking and play at transitional time points may assist the way parents and children assimilate temporal disruptions.

Psychological interventions for people with cystic fibrosis and their families.

BACKGROUND: With increasing survival estimates for cystic fibrosis (CF) long-term management has become an important focus. Psychological interventions are largely concerned with adherence to treatment, emotional and social adjustments and quality of life. We are unaware of any relevant systematic reviews. OBJECTIVES: Assess whether psychological interventions for CF provide significant psychosocial and physical benefits in addition to standard care. SEARCH STRATEGY: Trials were identified from two Cochrane trial registers (CF 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: September 2007. SELECTION CRITERIA: Randomised controlled trials of a broad range of psychological interventions in children and adults with CF and their immediate family. DATA COLLECTION AND ANALYSIS: Two authors independently selected relevant trials and assessed their methodological quality. MAIN RESULTS: The review includes 13 studies (five new at this update) representing data from 529 participants. Studies mainly assessed behavioural and educational interventions:1. gene pre-test education counselling for relatives of those with CF;2. biofeedback, massage and music therapy to assist physiotherapy;3. behavioural and educational interventions to improve dietary intake and airway clearance;4. self-administration of medication and education to promote independence, knowledge and quality of life; and5. systemic interventions promoting psychosocial functioning.A substantial proportion of outcomes were educational or behavioural relating to issues of adherence, change in physical status or other specific treatment concerns during the chronic phase of the disease. Some evidence was found for relative's acceptance of a genetic test for carrier status when using home-based rather than clinic-based information leaflets and testing. There is some evidence that behavioural interventions improve emotional outcomes in people with CF and their carers, and that psychoeducational interventions improve knowledge in the short term. There was no consistent effect on lung function, although one small study showed that biofeedback-assisted breathing re-training helped improve some lung function measurements. Some studies point to educational and behavioural interventions aiding nutrition and growth in people with CF. Currently there is insufficient evidence for interventions aimed at other aspects of the disease process. AUTHORS' CONCLUSIONS: Currently no clear evidence exists on the best psychological interventions to help people with CF and their carers manage the disease. Trials of interventions to improve adherence to treatment are needed. Multicentre approaches, with consequent funding implications, will increase the sample size of trials and enhance the power and precision of their findings.

Parental depression following the early diagnosis of cystic fibrosis: a matched, prospective study.

OBJECTIVE: To assess risks for parental depression following the diagnosis of cystic fibrosis (CF) in a child. STUDY DESIGN: Matched cohort study in NW England; 45 parental couples with a child diagnosed with CF were compared with 45 control couples matching for age, sex, and position in the family of the index child. The Beck Depression Inventory (BDI-II) with a clinical cut-off > or = 13 for dysphoria (mild depression) was the main outcome. A stratified analysis was conducted using the Mantel-Haenszel risk-ratio estimator (RR(MH)) with eight strata for each of the matching variable combinations. RESULTS: Heterogeneity was found within the dataset. Parents with a child with CF < or = 9 months of age at baseline had an elevated prospective risk of depression (mothers RR(MH) [95% confidence interval (CI)] = 2.6[1.05,6.42], fathers RR(MH) [95%CI] = 2.26 [0.97,5.28]). The absence of a group effect for depression at follow-up after adjusting for the matching (mothers RR(MH) [95%CI] = 1.1 [0.59,2.05], fathers RR(MH) [95%CI] = 1.42 [0.66,3.08]) masked this heterogeneity. CONCLUSION: This hypothesis-generating finding suggests that parents may be more vulnerable to depression when their child is diagnosed with a life-shortening condition during the first few months of life. Mood in parents of infants diagnosed early needs to be monitored longitudinally and preventative strategies devised.