Maternal Adaptation To A Hearing-impaired Child: A Comparison Of The Mediating And Moderating Effects Of Social Support And Personality

The current study assessed the relationships among parenting stress, social support, marital satisfaction and psychological adjustment in a sample of 96 mothers of hearing impaired preschoolers and 118 matched mothers of nondisabled children. Two competing models of the effects of social support and specific personality variables on adjustment were tested: the buffer and mediator models. Respondents completed a structured interview and a series of standardized questionnaires. Factor analyses of the independent variables yielded two dimensions of parenting stress and three dimensions of social support.;Significant group differences were obtained on the measures of parenting stress, marital satisfaction and psychological adjustment. Moderating effects for social support and the personality variables of endurance and nurturance were not obtained. However, path analytic techniques showed a significant mediating effect for perceived support and marital satisfaction. Higher levels of parenting stress produced decreased perceptions of emotional support, which in turn led to elevated symptoms of depression and anxiety. The results of the study were interpreted in light of the larger social support literature

Assessing disease disclosure in adults with cystic fibrosis: the Adult Data for Understanding Lifestyle and Transitions (ADULT) survey Disclosure of disease in adults with cystic fibrosis

<p>Abstract</p> <p>Background</p> <p>As more patients with cystic fibrosis (CF) reach adulthood and participate in age-appropriate activities (e.g. employment, dating), disclosure of medical status becomes more important. This study assessed rates of disclosure and its perceived impact on relationships using the Adult Data for Understanding Lifestyle and Transitions (ADULT) online survey.</p> <p>Methods</p> <p>Adults with CF participated in the survey via the United States national network of CF Centers. Descriptive and inferential statistics were utilized.</p> <p>Results</p> <p>Participants (n = 865) were more likely to disclose to relatives (94%) and close friends (81%) than to dating partners (73%), bosses/supervisors/teachers (51%) or co-workers (39%). Respondents generally reported a neutral/positive effect on relationships following disclosure. Negative effects of disclosure were infrequent, but more likely with dating partners or bosses/supervisors/teachers. Results also indicated that disclosure may be influenced by severity of lung disease and gender, with those having normal/mild lung disease less likely to disclose their diagnosis to both co-workers (p < 0.01) and bosses/supervisors/teachers (p < 0.01), and women being more likely to disclose to close friends (p < 0.0001) and dating partners (p < 0.05) than men.</p> <p>Conclusions</p> <p>Most adults with CF disclosed their disease to relatives and close friends. Individuals with severe CF lung disease were more likely to disclose their diagnosis to coworkers and supervisors/teachers. It may be helpful to provide support for disclosure of disease in situations such as employment and dating.</p

Linguistic validation of cystic fibrosis quality of life questionnaires

OBJECTIVE: The purpose of this study was to validate the Portuguese translations of four cystic fibrosis quality of life questionnaires (CFQ). The first three were developed for patients with cystic fibrosis aged from 6 to 11 years, from 12 to 13 years and 14 years or more, while the fourth was developed for the parents of patients aged 6 to 13 years. MATERIAL AND METHODS: The four CFQ translations contained from 35 to 50 questions covering nine domains and were validated as follows: translation from English to Portuguese, pilot application, back translation and then approval by the author of the English versions. The four translations were applied to 90 stable patients (30 from each age group) and the parents of patients aged 6-13 years (n = 60), on two occasions with a 13 to 17 day interval. Intraclass Correlation Coefficients (ICC) were used to measure reproducibility. This study was approved by the Commission for Ethics in Research at the institution. RESULTS:Reproducibility was good (ICC = 0.62 to 0.99) for the four translations in all domains, with the exceptions of the Digestion domain for the 6 to 11 and 12 to 13 years age groups with ICC = 0.59 and 0.47, respectively and the Social Role domain for the 14 and over age group (ICC = -0.19 ) CONCLUSION: The translation and cultural adaptation for Brazil resulted in four CFQ versions that are easy to understand and offer good reproducibility.OBJETIVO: O propósito deste estudo foi validar em português as quatro versões de questionários de qualidade de vida em fibrose cística, desenvolvidos para pacientes com fibrose cística de 6 a 11 anos, de 12 a 13 e mais de 14 anos, e para os pais de pacientes de 6 a 13 anos. MATERIAL E MÉTODOS: A validação das quatro versões de questionários de qualidade de vida em fibrose cística (de 35 e 50 questões, abrangendo nove domínios) constou de: versão inglês-português, aplicação-piloto, tradução retrógrada e aprovação da autora da versão inglesa. As quatro versões foram aplicadas a 90 pacientes estáveis (30 de cada grupo etário) e aos pais de doentes de 6-13 anos (n = 60), em duas entrevistas, com intervalo de 13-17 dias. Foi avaliada a reprodutibilidade pelo coeficiente de correlação intraclasse (CCI). O estudo foi aprovado pela comissão de ética em pesquisa da instituição. RESULTADOS: A reprodutibilidade foi boa (CCI = 0,62 a 0,99) para as quatro versões, em todos os domínios, exceto o digestivo (CCI = 0,59 e CCI = 0,47) para os grupos etários de 6 a 11 e 12 a 13 anos, respectivamente, e domínio papel social (CCI = -0,19 ) para o grupo acima de 14 anos. CONCLUSÃO: A tradução e a adaptação à língua e à cultura brasileiras das quatro versões de questionários de qualidade de vida em fibrose cística mostraram-se de fácil entendimento e boa reprodutibilidade.Universidade de São Paulo Faculdade de Medicina Departamento de PediatriaUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de MedicinaFaculdade de Medicina do ABCUNIFESP-EPM Centro de Reabilitação PulmonarUniversidade de São Paulo Faculdade de Medicina Hospital das ClínicasUniversity of Miami Department of PsychologyUNIFESP, EPM, Centro de Reabilitação PulmonarSciEL

Depression and anxiety in adolescents and adults with cystic fibrosis in the UK: A cross-sectional study

Background The International Depression/anxiety Epidemiological Study (TIDES) in the UK aimed: (i) to establish the prevalence of anxiety and depression amongst people with CF compared to a normative sample; (ii) to establish the association between mood, demographic and clinical variables; and (iii) to provide guidance for specialist-referral decision-making. Methods Patients (≥ 12 years) completed the Hospital Anxiety and Depression Scale (HADS). CF-HADS scores, expressed as percentiles, were compared with a normative sample. Multiple-regression analysis explored associations between demographic, clinical and mood variables. Results Thirty-nine CF centres recruited 2065 patients. Adults with CF were similar in terms of anxiety and depression to the general population. Adolescents with CF were less anxious and depressed. For adult patients, older age, unemployment for health reasons and poor lung function were associated with disordered mood. Gender-specific CF-percentile scores were calculated. Conclusion Surveillance, with attention to gender and risk factors is advocated. This work provides unique benchmark scores to aid referral decision-making

Validation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD)

Background: Quality of life (QOL)-primary ciliary dyskinesia (PCD) is the first disease-specific, health-related QOL instrument for PCD. Psychometric validation of QOL-PCD assesses the performance of this measure in adults, including its reliability, validity and responsiveness to change. Methods: Seventy-two adults (mean (range) age: 33 years (18–79 years); mean (range) FEV1% predicted: 68 (26–115)) with PCD completed the 49-item QOL-PCD and generic QOL measures: Short-Form 36 Health Survey, Sino-Nasal Outcome Test 20 (SNOT-20) and St George Respiratory Questionnaire (SGRQ)-C. Thirty-five participants repeated QOL-PCD 10–14 days later to measure stability or reproducibility of the measure. Results: Multitrait analysis was used to evaluate how the items loaded on 10 hypothesised scales: physical, emotional, role and social functioning, treatment burden, vitality, health perceptions, upper respiratory symptoms, lower respiratory symptoms and ears and hearing symptoms. This analysis of item-to-total correlations led to 9 items being dropped; the validated measure now comprises 40 items. Each scale had excellent internal consistency (Cronbach's α: 0.74 to 0.94). Two-week test–retest demonstrated stability for all scales (intraclass coefficients 0.73 to 0.96). Significant correlations were obtained between QOL-PCD scores and age and FEV1. Strong relationships were also found between QOL-PCD scales and similar constructs on generic questionnaires, for example, lower respiratory symptoms and SGRQ-C (r=0.72, p<0.001), while weak correlations were found between measures of different constructs. Conclusions: QOL-PCD has demonstrated good internal consistency, test–retest reliability, convergent and divergent validity. QOL-PCD offers a promising tool for evaluating new therapies and for measuring symptoms, functioning and QOL during routine care

Children with disorders of sex development: A qualitative study of early parental experience

<p>Abstract</p> <p>Background</p> <p>Clinical research on psychological aspects of disorders of sex development (DSD) has focused on psychosexual differentiation with relatively little attention directed toward parents' experiences of early clinical management and their influence on patient and family psychosocial adaptation.</p> <p>Objectives</p> <p>To characterize parental experiences in the early clinical care of children born with DSD.</p> <p>Study Design</p> <p>Content analysis of interviews with parents (n = 41) of 28 children, newborn to 6 years, with DSD.</p> <p>Results</p> <p>Four major domains emerged as salient to parents: (1) the gender assignment process, (2) decisions regarding genital surgery, (3) disclosing information about their child's DSD, and (4) interacting with healthcare providers. Findings suggested discordance between scientific and parental understandings of the determinants of "sex" and "gender." Parents' expectations regarding the benefits of genital surgery appear largely met; however, parents still had concerns about their child's future physical, social and sexual development. Two areas experienced by many parents as particularly stressful were: (1) uncertainties regarding diagnosis and optimal management, and (2) conflicts between maintaining privacy versus disclosing the condition to access social support.</p> <p>Conclusions</p> <p>Parents' experiences and gaps in understanding can be used to inform the clinical care of patients with DSD and their families. Improving communication between parents and providers (and between parents and their support providers) throughout the early clinical management process may be important in decreasing stress and improving outcomes for families of children with DSD.</p

Coach to cope:Feasibility of a life coaching program for young adults with cystic fibrosis

Over the last two decades, lifespan has increased significantly for people living with cystic fibrosis (CF). However, several studies have demonstrated that many young adults with CF report mental health problems and poor adherence to their prescribed treatments, challenging their long-term physical health. Treatment guidelines recommend interventions to improve adherence and self-management. The aim of this study was to test the feasibility of a life coaching intervention for young adults with CF. A randomized, controlled feasibility study was conducted at the CF Center at Copenhagen University Hospital, Rigshospitalet. Participants were young adults with CF, aged 18-30 years without severe intellectual impairments. Participants were randomized to either life coaching or standard care. The intervention consisted of up to 10 individual, face-to-face or telephone coaching sessions over a period of 1 year. Primary outcomes were recruitment success, acceptability, adherence to the intervention, and retention rates. Secondary outcome measures included health-related quality of life, adherence to treatment, self-efficacy, pulmonary function, body mass index, and blood glucose values. Among the 85 eligible patients approached, 40 (47%) were enrolled and randomized to the intervention or control group; two patients subsequently withdrew consent. Retention rates after 5 and 10 coaching sessions were 67% and 50%, respectively. Reasons for stopping the intervention included lack of time, poor health, perceiving coaching as not helpful, lack of motivation, and no need for further coaching. Coaching was primarily face-to-face (68%). No significant differences were found between the groups on any of the secondary outcomes. Both telephone and face-to-face coaching were convenient for participants, with 50% receiving the maximum offered coaching sessions. However, the dropout rate early in the intervention was a concern. In future studies, eligible participants should be screened for their interest and perceived need for support and life coaching before enrollment

Childhood Development after Cochlear Implantation (CDaCI) study: Design and baseline characteristics

Children with severe to profound sensorineural hearing loss face communication challenges that influence language, psychosocial and scholastic performance. Clinical studies over the past 20 years have supported wider application of cochlear implants in children. The Childhood Development after Cochlear Implantation (CDaCI) study is the first longitudinal multicentre, national cohort study to evaluate systematically early cochlear implant (CI) outcomes in children. The objective of the study was to compare children who have undergone cochlear implantation, with similarly aged hearing peers across multiple domains, including oral language development, auditory performance, psychosocial and behavioural functioning, and quality of life. The CDaCI study is a multicentre national cohort study of CI children and normal hearing (NH) peers. Eligibility criteria include informed consent, age less than 5 years, pre- or post-lingually deaf, developmental criteria met, commitment to educate the child in English and bilateral cochlear implants. All children had a standardised baseline assessment that included demographics, hearing and medical history, communication history, language measures, cognitive tests, speech recognition, an audiological exam, psychosocial assessment including parent-child videotapes and parent reported quality of life. Follow-up visits are scheduled at six-month intervals and include a standardised assessment of the full battery of measures. Quality assurance activities were incorporated into the design of the study. A total of 188 CI children and 97 NH peers were enrolled between November 2002 and December 2004. The mean age, gender and race of the CI and NH children are comparable. With regard to parental demographics, the CI and NH children's families are statistically different. The parents of CI children are younger, and not as well educated, with 49% of CI parents reporting college graduation vs. 84% of the NH parents. The income of the CI parents is also lower than the NH parents. Assessments of cognition suggest that there may be baseline differences between the CI and NH children; however the scores were high enough to suggest language learning potential. The observed group differences identified these baseline characteristics as potential confounders which may require adjustment in analyses of outcomes. This longitudinal cohort study addresses questions related to high variability in language outcomes. Identifying sources of that variance requires research designs that: characterise potential predictors with accuracy, use samples that adequately power a study, and employ controls and approaches to analysis that limit bias and error. The CDaCI study was designed to generate a more complete picture of the interactive processes of language learning after implantation. Copyright © 2007 John Wiley & Sons, Ltd.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/56091/1/333_ftp.pd

A quality-of-life measure for adults with primary ciliary dyskinesia: QOL-PCD

Primary ciliary dyskinesia (PCD) is characterised by chronic suppurative lung disease, rhino-sinusitis, hearing impairment and sub-fertility. We have developed the first multidimensional measure to assess health-related quality of life (HRQoL) in adults with PCD (QOL-PCD). Following a literature review and expert panel meeting, open-ended interviews with patients investigated the impact of PCD on HRQoL in the UK and North America (n=21). Transcripts were content analysed to derive saturation matrices. Items were rated for relevance by patients (n=49). Saturation matrices, relevance scores, literature review, evaluation of existing measures, and expert opinion contributed to development of a preliminary questionnaire. The questionnaire was refined following cognitive interviews (n=18). Open-ended interviews identified a spectrum of issues unique to adults with PCD. Saturation matrices confirmed comprehensive coverage of content. QOL-PCD includes 48 items covering the following seven domains: Physical Functioning, Emotional Functioning, Treatment Burden, Respiratory and Sinus Symptoms, Ears and Hearing, Social Functioning, and Vitality and Health Perceptions. Cognitive testing confirmed that content was comprehensive and the items were well-understood by respondents. Content validity and cognitive testing supported the items and structure. QOL-PCD has been translated into other languages and is awaiting psychometric testing

Primary Ciliary Dyskinesia: First Health-related Quality of Life Measures for Pediatric Patients

Rationale: Primary ciliary dyskinesia (PCD) is a rare disease. There are no available data on disease-specific pediatric patient–reported outcomes