Cognitive functioning and mental health in children with a primary mitochondrial disease.

BACKGROUND: Studies regarding cognitive and mental health functioning in children with mitochondrial disease (MD) are scarce, while both are important issues given their impact on QoL. Knowledge on these aspects of functioning and its relationship with disease parameters is essential to gather more insight in working mechanisms and provide recommendations for future research and patientcare. The aim of this study was to map the cognitive functioning and mental health in children with MD in relation to disease specific factors. METHODS: Pediatric patients (< 18 year) with a genetically confirmed MD were included. Demographic and disease specific factors (International Paediatric Mitochondrial Disease Scale) were assessed, as well as cognitive functioning (intelligence, attention, working memory (WM)), and mental health (psychological functioning and quality of life). Individual patient data was described. RESULTS: Thirty-three children with MD were included. Intellectual functioning ranged from a clinically low IQ (36% of the patients, N = 12/33) to an average or above average IQ (39%, N = 13/33). A higher verbal versus performance IQ was observed (36% N = 5/14), a lower processing speed (43%, N = 6/14), attentional problems (50%, N = 7/14), and verbal WM problems (11%, N = 2/18). Regarding mental health, general behavioral problems were reported (45%, N = 10/22), and on subscale level, attention problems (45%, N = 10), withdrawn/depressed (36%, N = 8/22) and anxious/depressed behavior (14%, N = 3/22). Furthermore, QoL impairments were reported (42%, N = 5/12). The specific intelligence profiles, cognitive impairments, behavioral problems and QoL impairments occurred in every intelligence subgroup. Children with an average or above general intellectual functioning had a generally lower and less variability in IPMDS scores, less frequently epilepsy, vision and hearing problems, and a relatively later age of onset, as compared to patients with a clinically low intellectual functioning. CONCLUSIONS: Despite considerable heterogeneity, overall results showed a high rate of impairments in both cognitive and mental health functioning. Also in children with an average or above level of intellectual functioning, specific cognitive impairments were observed. Children with a clinically low intellectual functioning more often had disease related impairments compared to children with a higher intellectual functioning. The importance of structural assessment of cognitive functioning and mental health is warranted, also in children with mild disease related symptoms.

Identifying trajectories of fatigue in patients with primary mitochondrial disease due to the m.3243A > G variant.

Severe fatigue is a common complaint in patients with primary mitochondrial disease. However, less is known about the course of fatigue over time. This longitudinal observational cohort study of patients with the mitochondrial DNA 3243 A>G variant explored trajectories of fatigue over 2 years, and characteristics of patients within these fatigue trajectories. Fifty-three adult patients treated at the Radboud University Medical Center Nijmegen were included. The majority of the patients reported consistent, severe fatigue (41%), followed by patients with a mixed pattern of severe and mild fatigue (36%). Then, 23% of patients reported stable mild fatigue levels. Patients with a stable high fatigue trajectory were characterized by higher disease manifestations scores, more clinically relevant mental health symptoms, and lower psychosocial functioning and quality of life compared to patients reporting stable low fatigue levels. Fatigue at baseline and disease manifestation scores predicted fatigue severity at the 2-year assessment (57% explained variance). This study demonstrates that severe fatigue is a common and stable complaint in the majority of patients. Clinicians should be aware of severe fatigue in patients with moderate to severe disease manifestation scores on the Newcastle Mitochondrial Disease Scale, the high prevalence of clinically relevant mental health symptoms and overall impact on quality of life in these patients. Screening of fatigue and psychosocial variables will guide suitable individualized treatment to improve the quality of life.

A conceptual disease model for quality of life in mitochondrial disease.

BACKGROUND: Previous studies in patients with a mitochondrial disease (MD) highlight the high prevalence of cognitive impairments, fatigue, depression, and a lower quality of life (QoL). The relationship with biological and physiological factors remains complex. The aim of this study is to investigate the status of and interrelationships between biological and physiological functioning, cognitive functioning as well as fatigue, depression, societal participation, health perceptions, and QoL, by using the Wilson and Cleary conceptual disease model, adapted to MD. METHODS: Patients with a genetically confirmed MD were included. The following health concepts in MD were investigated according to the conceptual model: (1) Biological and physiological: disease manifestation (Newcastle Mitochondrial Disease Adult Scale), (2) Symptom status: cognitive functioning, patient reported fatigue and depressive symptoms, (3) Functional health: societal participation, (4) Patient reported health perceptions, and (5) Overall QoL. Data were compared to healthy normative data and/or data from other patient groups. Correlations as well as a hierarchical regression analysis were performed to assess the relations between the different levels of health concepts in the conceptual model. RESULTS: Of the 95 included patients, 42% had a severe disease manifestation. Comparable or worse than normative data and other patient groups, 35% reported cognitive impairments, 80% severe fatigue, and 27% depressive symptoms. Patients experienced impairments in societal participation and QoL. Disease manifestation was significantly correlated with cognitive functioning, societal participation, physical functioning and overall QoL, but not with fatigue or depressive symptoms. Almost all outcome measures regarding functional health, health perceptions and QoL were correlated with symptom status variables. Overall QoL was significantly predicted by fatigue and physical functioning. CONCLUSIONS: Symptom status is related to the functional health, health perceptions and QoL in patients with MD. Moreover, fatigue and physical functioning are important contributors to the overall QoL of MD patients. In order to provide adequate patient care it is important to have a broad view on patients' functioning, not only by providing a proper clinical assessment, but also to screen for symptom status; cognitive functioning, fatigue and depression.

Determining the effectiveness of cognitive behavioural therapy in improving quality of life in patients undergoing endometriosis surgery: a study protocol for a randomised controlled trial.

INTRODUCTION: Endometriosis can cause chronic pain and subfertility thereby negatively affecting quality of life (QoL). Surgical removal of endometriosis lesions leads to improved health-related QoL, although not to the level of QoL of healthy controls. Pain intensity and cognitions regarding pain can play a crucial role in this health-related QoL following surgical treatment. Cognitive behavioural therapy (CBT) is a psychological treatment. In patients with chronic pain caused by a variety of medical conditions, CBT is effective in improving QoL. We designed a research protocol to investigate the effect of CBT on QoL in patients with endometriosis-associated chronic pain who are undergoing surgery. METHODS AND ANALYSIS: This is a study protocol for a randomised controlled trial in which 100 patients, undergoing endometriosis removal surgery due to endometriosis-associated chronic pain, will be randomised between post-surgery usual care with CBT and post-surgery usual care only. Participants in the CBT group will additionally receive seven sessions of CBT, focused on expectancy management, cognitions regarding pain and emotional and behavioural impact of pain. To determine the primary outcome Quality of life, both groups will complete questionnaires assessing QoL. The secondary outcomes pain intensity, pain cognitions, fatigue and perceived stress are also measured using questionnaires. Additionally, a marker for stress (cortisol extracted from a hair sample) will be assessed at T0 (baseline assessment), T1 (post-intervention; 2 weeks after completion of all CBT sessions) and T2 (follow-up; 14 weeks after T1). Statistical analysis will be performed using SPSS software. ETHICS AND DISSEMINATION: The study protocol has been approved by the Medical Ethical Committee of the region Arnhem-Nijmegen from the Radboud University Medical Centre on 2 September 2020. The findings of this study will be published in scientific journals and will be presented at scientific conferences. TRIAL REGISTRATION NUMBER: NCT04448366.

Cognitive functioning and mental health in mitochondrial disease: A systematic scoping review.

Mitochondrial diseases (MDs) are rare, heterogeneous, hereditary and progressive in nature. In addition to the serious somatic symptoms, patients with MD also experience problems regarding their cognitive functioning and mental health. We provide an overview of all published studies reporting on any aspect of cognitive functioning and/or mental health in patients with MD and their relatives. A total of 58 research articles and 45 case studies were included and critically reviewed. Cognitive impairments in multiple domains were reported. Mental disorders were frequently reported, especially depression and anxiety. Furthermore, most studies showed impairments in self-reported psychological functioning and high prevalence of mental health problems in (matrilineal) relatives. The included studies showed heterogeneity regarding patient samples, measurement instruments and reference groups, making comparisons cautious. Results highlight a high prevalence of cognitive impairments and mental disorders in patients with MD. Recommendations for further research as well as tailored patientcare with standardized follow-up are provided. Key gaps in the literature are identified, of which studies on natural history are of highest importance.

Psychological functioning in children suspected for mitochondrial disease: the need for care.

BACKGROUND: Mitochondrial diseases (MD) are generally serious and progressive, inherited metabolic diseases. There is a high comorbidity of anxiety and depression and limitations in daily functioning. The complexity and duration of the diagnostic process and lack of knowledge about prognosis leads to uncertainty. In this study, we investigated the psychological well-being of children who are suspected for MD and their parents. METHODS: In total 122 children suspected for MD and their parents, received questionnaires as part of standard clinical investigation. RESULTS: Parent proxy report revealed a lower quality of life (QoL) compared to norms and even more physical problems compared to chronically ill patients. They also reported more behavioral problems in general and more internalizing problems compared to the norms. Most frequent reported somatic complaints were tiredness and pain. Parents did not report enhanced levels of stress regarding parenting and experienced sufficient social support. At the end of the diagnostic process, 5.7% of the children received the genetically confirmed diagnosis of MD, 26% showed non-conclusive abnormalities in the muscle biopsy, 54% did not receive any diagnosis, and the remaining received other diagnoses. Strikingly, children without a diagnosis showed equally QoL and behavioral problems as children with a diagnosis, and even more internalizing problems. CONCLUSIONS: This study highlights the psychological concerns of children with a suspicion of MD. It is important to realize that as well as children with a confirmed diagnosis, children without a diagnosis are vulnerable since explanation for their complaints is still lacking.

Real-world implementation of electronic patient-reported outcomes in outpatient pediatric cancer care.

OBJECTIVE: The KLIK method is an online tool that monitors and discusses electronic patient-reported outcomes (ePROs), which has been shown to enhance outcomes. This study aimed (1) to determine the fidelity (ie, extent to which used as intended) of the KLIK method as implemented in outpatient pediatric cancer care and (2) to study health care professional (HCP)-reported barriers and facilitators for implementation. METHODS: Two hundred five children with newly diagnosed cancer (enrollment rate 85%) participated. At 1 (T1), 3 (T2), and 6 (T3) months after diagnosis, patients (8-18 years) or parents (of patients 0-7 years) completed health-related quality of life (HRQoL) questionnaires, which were transformed into an ePROfile and discussed by their HCP during consultations. Fidelity was determined by the following: percentage of website registrations, HRQoL questionnaires completed, and ePROfiles discussed. Implementation determinants were assessed with HCPs after the final T3 with the Measurement Instrument for Determinants of Innovations. RESULTS: Depending on the time point (T1-T3), fidelity was 86% to 89% for website registration, 66-85% for completed HRQoL questionnaires, and 56% to 62% for ePROfile discussion. Barriers were mainly related to organizational issues (eg, organizational change) and less frequently to users (eg, motivation to comply) or the intervention (compatibility). Facilitators were related to the user (eg, positive outcome expectations) and intervention (simplicity) but not to the organization. CONCLUSIONS: When implementing ePROs in outpatient pediatric oncology practice, HCPs report determinants that influence ePRO integration. To improve implementation and outcomes, tailored organizational (eg, formal ratification by management and time) and specific local (eg, individualized assessments) strategies should be developed to achieve optimal ePRO discussion.

Women's adjustment trajectories during IVF and impact on mental health 11-17 years later.

STUDY QUESTION: Do patients present different adjustment trajectories during and after IVF treatment? SUMMARY ANSWER: Most women show resilient trajectories during and after IVF treatment but 37% show temporary or chronic maladjustment during IVF and 10% are maladjusted 11-17 years after treatment. WHAT IS KNOWN ALREADY: Research on patient psychosocial adjustment during treatment has contributed to identifying the most distressful stages of IVF treatment and profiling patients at risk for emotional maladjustment at these specific stages. This knowledge is currently driving the deliverance of psychosocial care at fertility clinics by tailoring it to patients' risk profiles and specific treatment stages. However, current care does not take into consideration how individuals adjust across the entire treatment pathway. This can be assessed by profiling individual adjustment trajectories. STUDY DESIGN, SIZE, DURATION: A longitudinal cohort study with five assessment moments that combines data from two different studies, the STRESSIVF and OMEGA projects. Participants enrolled in the STRESSIVF study (started IVF in 1998-2000) were assessed before and after the first IVF treatment cycle and 6 months and 2.5 years after the last IVF cycle. A subset participated in the OMEGA project (started IVF in 1995-2000) and reported on their mental health 11-17 years after treatment. PARTICIPANTS/MATERIALS, SETTING, METHODS: Three hundred and forty-eight women participated in the STRESSIVF project and 108 of these in the OMEGA. Anxiety was measured with the State and Trait Anxiety Inventory, depression with the Beck Depression Inventory and mental health with the Mental Health Inventory. Latent class growth mixed modelling was carried out to identify distinct anxiety and depression trajectories over the four STRESSIVF study assessment moments. Multinominal logistic regressions were conducted to investigate predictors of trajectory membership, and stepwise linear regressions were performed to investigate if adjustment trajectories predicted mental health 11-17 years after IVF treatment. MAIN RESULTS AND THE ROLE OF CHANCE: A total of 67 and 86% of women showed normal levels of anxiety and depression, respectively, throughout treatment (resilient trajectories), 24 and 33% experienced anxiety and depression only during treatment (recovery trajectories), 4.6 and 4.9% experienced anxiety and depression only after treatment (delayed trajectories), and 4.3% showed chronic anxiety (chronic trajectory, not identified for depression). Non-resilient trajectories were associated with unsuccessful treatment, marital dissatisfaction, lack of social support and negative infertility cognitions. One in 10 women had a delayed or chronic trajectory and these trajectories predicted serious mental health impairment 11-17 years after treatment. LIMITATIONS, REASONS FOR CAUTION: The study only focuses on women. In the OMEGA project adjustment was assessed using a mental health measure. Although we could investigate how trajectories predicted mental health, it would have been preferable to map anxiety and depression trajectories up to 11-17 years after treatment. Missing analysis showed selective dropout from the study but this was accounted for by using mixed models and imputation procedures. Finally, data on other life stressors were not collected; therefore any contribution from these events cannot be assessed. WIDER IMPLICATIONS OF THE FINDINGS: Fertility health-care providers have been called upon considering their responsibility in supporting patients in the aftermath of treatment. Results show it is possible to profile different groups of at-risk women at the start of the treatment and tailor psychosocial support to risk profile to promote health adjustment during treatment and thereafter. STUDY FUNDING/COMPETING INTERESTS: This study was supported by a grant from the Dutch Cancer Society (2006-3631) and the Praeventiefonds (28-3012). No competing interests exist.

e-Therapy to reduce emotional distress in women undergoing assisted reproductive technology (ART): a feasibility randomized controlled trial.

STUDY QUESTION: Is it feasible to evaluate a personalized e-therapy program (Internet based) for women during fertility treatment aimed to reduce the chance of having clinically relevant symptoms of anxiety and/or depression after unsuccessful assisted reproductive technology (ART) treatment within a randomized controlled trial (RCT)? STUDY ANSWER: The evaluation of a personalized e-therapy program is feasible, reflected by good acceptability and integration within current guidelines, but adjustments to the e-therapy program and study design of the RCT have to be made to enhance demand, practicality and efficacy. WHAT IS KNOWN ALREADY: Internet-based interventions are promising in reducing psychological distress, especially when treatment is personalized to specific risk profiles of patients. However in fertility care, the beneficial effects of personalized e-therapy on psychological distress and its implementation in daily clinical care still have to be evaluated. STUDY DESIGN, SIZE, DURATION: To evaluate the feasibility of a personalized e-therapy program, we conducted a two-arm, parallel group, single-blind feasibility randomized controlled trial with a 1:1 allocation. Feasibility was assessed in terms of demand, acceptability, practicality, implementation, integration and limited efficacy. Women were included between 1 February 2011 and 1 June 2013. Women in the control group received care as usual, whereas women in the intervention group received in addition to their usual care access to a personalized e-therapy program. Women were monitored until 3 months after the start of their first ART cycle. PARTICIPANTS/MATERIALS, SETTING, METHODS: In a university hospital in the Netherlands women who were screened as at risk for emotional adjustment problems and intended to start their first ART cycle were invited, and of them 120 were randomized. Of these women, 48% in the intervention group were compliant to the intervention. Outcome measures associated with the feasibility to analyse this e-therapy program within an RCT were assessed. MAIN RESULTS AND THE ROLE OF CHANCE: It is feasible to evaluate a personalized e-therapy program within an RCT. The acceptability was good, as was the integration within current clinical guidelines and care. However, the demand reflected by a participation rate of 44% was low, since most women declined participation because they felt no need for support at that moment. The practicality of the intervention was moderate illustrated by a relatively high dropout rate (30%) due to practical concerns. The intervention was effective, shown by a reduction in the percentage women having clinically relevant symptoms of anxiety and/or depression in the compliant intervention group compared with the control group 3 months after the first ART cycle; risk difference of 24% (95% CI: 2-46%; ITALIC! P = 0.03). LIMITATIONS, REASONS FOR CAUTION: The large non-participation rate (56%) needs further evaluation. This also could have influenced results on limited efficacy. Barriers for participation could be assessed more in-depth. Moreover, ∼30% dropped out. This percentage is comparable with other e-health studies. Finally, this is a single-centre study. Generalizability could be enlarged by a multi-centre approach. WIDER IMPLICATIONS OF THE FINDINGS: In clinical fertility care, personalizing an e-therapy program to the patients' risk profile is promising and feasible. However, in future studies, we recommend modification of the study protocol by for example offering the intervention to the preferred moment in the treatment process. Moreover, adjustment of the study protocol tailored to the found barriers and facilitators is needed. When performing a multi-centre consecutive RCT to assess the effectiveness of personalized e-therapy in fertility care, the findings of this study, for example concerning the preferred timing or reasons for non-participation, could be helpful. STUDY FUNDING/COMPETING INTERESTS: NutsOhra (Study Number 0702-94) funded this study with an unrestricted grant. There were no competing interests. TRIAL REGISTRATION NUMBER: ClinicalTrials.gov NCT 01283607. TRIAL REGISTRATION DATE: 21 January 2011. DATE OF FIRST PATIENT'S ENROLMENT: February, 2011.

Prenatal maternal psychological stress and childhood asthma and wheezing: a meta-analysis.

The aim of this study was to systematically review and meta-analyse observational studies on prenatal maternal psychological stress and the subsequent development of asthma and wheezing in early childhood.All available published literature from 1960 until November 2013 was systematically searched through electronic databases (PubMed, Embase, PsycInfo and Web of Science). All observational studies assessing associations between any form of prenatal maternal psychological stress and respiratory morbidity in the child were included. Data extraction, quality assessment and meta-analyses were performed.The overall meta-analysis included 10 studies and showed that the prevalence of wheezing, asthma and other respiratory symptoms is higher in children of mothers who were exposed to or experienced some form of psychological stress during pregnancy than in mothers who did not (pooled OR 1.56 (95% CI 1.36-1.80)). Comparable results were observed in subgroup analyses of stress exposure, perceived stress, asthma and wheezing.This study demonstrates that prenatal maternal psychological stress is associated with respiratory morbidity, including asthma and wheezing in the child. Future studies examining the early origins of asthma and wheezing need to account for the impact of prenatal maternal stress.

Illness cognitions and family adjustment: psychometric properties of the Illness Cognition Questionnaire for parents of a child with cancer.

PURPOSE: Illness cognitions are an important mediator between disease and psychological adjustment. This study assessed the psychometric properties of the Illness Cognition Questionnaire (ICQ), adjusted for the parents of an ill child. METHODS: Participants were recruited from two multicenter studies: sample 1 included 128 parents of a child diagnosed with acute lymphoblastic leukemia (ALL) (response rate 82 %) and sample 2 included 114 parents of a child diagnosed with cancer (response rate 74 %). Parents completed an adapted version of the ICQ (Illness Cognition Questionnaire-Parent version (ICQ-P)), together with the Profile of Mood States (POMS; sample 1) or the Hospital Anxiety and Depression Scale (HADS; sample 2). The factor structure of the ICQ-P was examined by means of principal component analysis. Cronbach's alpha for each subscale and correlations between the ICQ-P scales and the HADS and POMS were calculated. The illness cognitions of parents with and without psychological distress were compared. RESULTS: Factor analysis confirmed the hypothesized structure of the ICQ-P in our sample (n = 242). The three scales Helplessness, Acceptance, and Perceived Benefits explained 9.8, 31.4, and 17.9 % of the variance, respectively. Cronbach's alpha showed adequate internal consistency (.80-.88). Concurrent and criterion-related validity were appropriate. CONCLUSIONS: The results confirm that the ICQ-P reliably assesses the illness cognitions of the parents of a child with cancer. Psychologically distressed parents showed less acceptance and more helplessness. The availability of a short and valid illness cognition questionnaire will help clinicians gain insight into parental cognitions regarding the illness of their child, information that might be helpful for targeting interventions.

Psychosocial and demographic correlates of the discontinuation of in vitro fertilization.

In this study, we investigated the relationship between psychosocial factors assessed pre-treatment and the discontinuation of in vitro fertilisation (IVF) treatment after one year. A cohort study was performed in a Dutch fertility clinic and included 667 couples. Demographic characteristics, scores for psychosocial factors, discontinuation rates, reasons for discontinuation and outcome data of the treatment were measured. Discontinuation due to personal reasons was differentiated from discontinuation because of changing a clinic or physicians' advice. The results showed that 37.5% of the couples became pregnant, while 40 (6%) discontinued IVF treatment because of personal reasons. A sample size of 288 women remained for analysis. A longer duration of infertility, less perceived social support in women and higher scores on acceptance of infertility in both men and women were significantly correlated with discontinuation. Multivariate analysis, using these variables including the age of the women, showed that these factors could explain 29% of the discontinuation. These results point to a differentiation between couples who choose positively choose to discontinue treatment and those discontinuing from a more negative perspective. Opportunities to tailor interventions to this second group of couples need to be investigated.

Do children make you happier? Sustained child-wish and mental health in women 11-17 years after fertility treatment.

STUDY QUESTION: Are fertility treatment-related factors, parenthood status and sustained child-wish associated with women's long-term mental health? SUMMARY ANSWER: Sustaining a child-wish is more strongly associated with women's long-term mental health than fertility treatment-related factors and parenthood status. WHAT IS KNOWN ALREADY: About one-third of the couples starting fertility treatment do not achieve parenthood and have to adjust to an unfulfilled child-wish. In women, remaining childless after treatment is associated with less favourable mental health. It is unclear if this is only related to their childlessness or if adjustment after unsuccessful treatment is affected by other variables. These include diagnostic and treatment-related factors (cause of fertility problems, age at first consultation, type and number of treatments) and the psychological ability to come to terms with the unfulfilled child-wish. Differentiating the relative contribution of these factors to women's long-term mental health will provide useful knowledge to support patients adjusting to negative treatment outcomes. STUDY DESIGN, SIZE, DURATION: A cross-sectional study with a nationally representative sample of 7148 women who started fertility treatment at any of the 12 IVF hospitals in the Netherlands from 1995 through 2000. Of 16 482 women who were invited to participate, 7148 (43.4%) provided psychological data. PARTICIPANTS/MATERIALS, SETTING, METHODS: The average age of women was 47 years and the average age at first fertility consultation was 30 years. Fifty-one per cent of women did IUI and 85% did IVF/ICSI. Ninety per cent of women were married/cohabiting, 20.9% remained childless and 5.9% had a child-wish. Women completed a questionnaire assessing diagnostic and treatment factors (retrospective data), parenthood status, sustained child-wish and mental health. MAIN RESULTS AND THE ROLE OF CHANCE: A multiple regression analysis controlling for background variables showed that, first, male factor (P < 0.05) and/or idiopathic infertility (P < 0.001) were associated with better mental health. Secondly, starting fertility treatment at an older age was associated with better mental health (P < 0.01). Thirdly, the interaction between parenthood status and sustained child-wish was significant (P < 0.01). Having a child-wish was associated with worse mental health for women with (ß = -0.058, P < 0.01) and without children (ß =-0.136, P < 0.001), but associations were stronger for the latter. Predictive factors accounted for <5% of the variation in mental health status in the study population. LIMITATIONS, REASONS FOR CAUTION: The sample was large and nationally representative. Response rate was in line with other studies but women without psychological data were less likely to have biological children and 15.9% of non-responders considered the questionnaire to be too confronting or to elicit too emotional memories. This could reflect an underestimation of the proportion of women with a sustained child-wish. WIDER IMPLICATIONS OF THE FINDINGS: Sustaining a child-wish is a more important risk for long-term adjustment problems than parenthood status. Women adjust better when they start treatment at older ages and know they were not responsible for the cause of the fertility problem. Fertility staff can play an important role in preparing patients for the possibility of treatment failure and the associated grief process. They can also inform patients about the positive effect of refocusing their life goals. STUDY FUNDING/COMPETING INTERESTS: This study was supported by a grant from the Dutch Cancer Society (2006-3631). No competing interests exist. TRIAL REGISTRATION NUMBER: N/A.

How about your peers? Cystic fibrosis questionnaire data from healthy children and adolescents.

BACKGROUND: The Cystic Fibrosis Questionnaire (CFQ) is widely used in research as an instrument to measure quality of life in patients with cystic fibrosis (CF). In routine patient care however, measuring quality of life is still not implemented in guidelines. One of the reasons might be the lack of consensus on how to interpret CFQ scores of an individual patient, because appropriate reference data are lacking. The question which scores reflect normal functioning and which scores reflect clinically relevant problems is still unanswered. Moreover, there is no knowledge about how healthy children and adolescents report on their quality of life (on the CFQ). With regard to quality of life the effect of normal development should be taken into account, especially in childhood and adolescence. Therefore, it is important to gain more knowledge about how healthy children and adolescents report on their quality of life and if there are any difference in a healthy populations based on age or gender. Without these data we cannot adequately interpret the CFQ as a tool in clinical care to provide patient-tailored care. Therefore this study collected data of the CFQ in healthy children and adolescents with the aim to refer health status of CF youngsters to that of healthy peers. METHODS: The CFQ was completed by 478 healthy Dutch children and adolescents (aged 6-20) in a cross-sectional study. RESULTS: The majority of healthy children (over 65%) did not reach maximum scores on most domains of the CFQ. Median CFQ-scores of healthy children and adolescents ranged from 67 to 100 (on a scale of 0-100) on the different CFQ-domains. Significant differences in quality of life exist among healthy children and adolescents, and these depend on age and gender. CONCLUSIONS: Reference data of quality of life scores from a healthy population are essential for adequate interpretation of quality of life in young patients with CF. Clinicians should be aware that the perception of health-related quality of life is not as disease-specific as one might think and also relies on factors such as age, normal maturation and gender.

Psychological characteristics associated with the onset and course of asthma in children and adolescents: a systematic review of longitudinal effects.

OBJECTIVE: to systematically review all available studies that investigated the longitudinal relationships between the psychological characteristics of children and adolescents suffering from asthma and those of their caregivers, and the onset and course of the asthma. METHODS: relevant studies were identified using Medline, PubMed, and PsychINFO between 1970 and September 2009. RESULTS: twenty studies matching inclusion criteria were reviewed. Six studies focused on child-specific psychological characteristics in relation to the onset and course of asthma. No compelling evidence was found for an association with asthma onset, but there was some evidence that the child's psychological characteristics can contribute to the subsequent course of asthma. Fourteen studies considered the effects of the psychological characteristics of the caregivers. Eleven studies found significant relationships between the psychological problems of caregivers and the subsequent onset and unfavorable course of the asthma in the child. CONCLUSION: in pediatric asthma both the psychological characteristics of the affected children and their caregivers appear to contribute to the course and possibly also to the onset of the condition.

Weaknesses, strengths and needs in fertility care according to patients.

BACKGROUND: The patients' role in assessing health care quality is increasingly recognized. Measuring patients' specific experiences and needs generates concrete information for care improvement, whereas satisfaction surveys only give an overoptimistic, undifferentiating picture. Therefore, this study aimed to investigate possible weaknesses, strengths and needs in fertility care by measuring patients' specific experiences. METHODS: Mixed (qualitative and quantitative) methods were used to identify weaknesses, strengths and needs in fertility care. Four focus groups with 21 infertile patients were used for documenting care aspects relevant to patients. The fully transcribed qualitative results were analysed and converted into a 124-item questionnaire, to investigate whether these aspects were regarded as weaknesses, strengths or needs in fertility care. The questionnaire was distributed to 369 eligible couples attending 13 Dutch fertility clinics. Descriptive statistics were used to determine the quantity of the weaknesses, strengths and needs. RESULTS: Overall, 286 women (78%) and 280 men (76%) completed the questionnaire. Patients experienced many weaknesses in fertility care, mostly regarding emotional support and continuity of care. Respect and autonomy and partner involvement were considered strengths in current care. Furthermore, women expressed their need for more doctors' continuity during their treatment, and couples strongly desired to have free access to their own medical record. The questionnaire's internal consistency and construct validity were sufficient. CONCLUSIONS: Infertile couples experience strengths, but also many weaknesses and needs in current fertility care. Lack of patient centredness seems to be a major cause herein. Using mixed methods is a sensitive means for identifying these weaknesses and needs.

[Psychosocial adjustment in children with a cleft lip and/or palate]. / Sociaal-emotionele gezondheid bij kinderen met schisis.

OBJECTIVE: To gain insight into the psychosocial health of children aged 9 to 12 years with a cleft lip and/or palate; to determine the relation between their health and the nature and severity of the cleft as well as other individual characteristics. DESIGN: Descriptive, cross-sectional study. METHOD: Questionnaires completed by parents, teachers and children were used to obtain information about the psychosocial health, nature and severity of the cleft lip and/or palate, and individual characteristics of 80 children. The interrelationship between these parameters was assessed using chi-square tests, single-factor analysis of variance and correlational analysis. RESULTS: In general, the psychosocial health of children with a cleft lip and/or palate did not differ from that of the norm groups. Parents of children with a cleft lip/and or palate reported more withdrawn or depressive behaviour in their child than parents from the norm groups. Children with a cleft lip and/or palate exhibited less rule-breaking behaviour. Teachers reported relatively more social problems. One-third of the children had learning problems. A better psychosocial health was associated with fewer speech problems but not with a more or less abnormal physical appearance. Self-image showed a negative correlation with psychosocial health problems, while learning problems showed a positive correlation. CONCLUSION: In general, the psychosocial health of children with a cleft lip and/or palate does not differ from children without this condition. However, children with a cleft lip and/or palate do exhibit more learning problems.

Effectiveness of a multidisciplinary itch-coping training programme in adults with atopic dermatitis.

The short- and longer-term effectiveness of a brief, multidisciplinary itch-coping group training scheme in adults with atopic dermatitis was evaluated. Clinical severity scores (Eczema Area and Severity Index) and validated self-report measures were obtained in a waiting-list control condition (n=30) and a treatment condition (n=61) at pre- and post-treatment and in the treatment condition at 3- and 12-month follow-ups. Relative to the control condition, all post-treatment measures showed improvements in terms of enhanced skin status, reduced itching and scratching and improved itch-coping patterns. In the treatment condition, the changes were sustained or further improved at both follow-ups. Also, the dermatological healthcare use was significantly reduced during the follow-up periods, in terms of fewer visits to the dermatologist and decreased use of topical corticosteroids and itch-relieving medication (histamine antagonists). The brief multidisciplinary itch-coping programme in adults with atopic dermatitis considerably reduced itch-scratching patterns, improved their skin status and reduced the use of dermatological care, both in the short and longer term.

Predicting emotional response to unsuccessful fertility treatment: a prospective study.

The predictive value of a comprehensive model with personality characteristics, stressor related cognitions, coping and social support was tested in a sample of 187 nonpregnant women. The emotional response to the unsuccessful treatment was predicted out of vulnerability factors assessed before the start of the treatment. The results indicated the importance of neuroticism as a vulnerability factor in emotional response to a severe stressor. They also underlined the importance of helplessness and marital dissatisfaction as additional risk factors, and acceptance and perceived social support as additional protective factors, in the development of anxiety and depression after a failed fertility treatment. From clinical point of view, these results suggest fertility-related cognitions and social support should receive attention when counselling women undergoing IVF or ICSI treatment.

Patient participation in discussing palliative radiotherapy.

Cancer patients' participation in doctor-patient interactions has been shown to be an important factor in the emotional processing of their condition, particularly when only palliative treatments can be offered. In this study, we assessed incurable cancer patients' participation in initial consultations with their radiation oncologists (ROs). RO stimulation of patient participation and discussions about treatment decisions were also measured. The entire consultation was videotaped and analyzed using the Roter Interaction Analysis System (RIAS). Patients' participation proved to be low on medical information, but high on discussing their experiences and life circumstances. The ROs stimulated patient participation mainly by providing medical information and giving patients opportunities to tell their stories. Decisions about radiation treatment had previously taken place and were rarely discussed in the consultations studied. The results suggest that patient participation in palliative treatment consultations might be improved for facilitating patients' emotional processing of the incurable nature of their cancer.