Siblings of children with cystic fibrosis: quality of life and the impact of illness.

OBJECTIVE: To asses self-reported quality of life (QoL) and perception of impact of illness on siblings of children with cystic fibrosis (CF). METHODS: The Child Health Questionnaire was used to assess QoL. The Sibling Perception Questionnaire was used to assess impact of illness. RESULTS: Siblings of children with CF (n= 39) rated their QoL higher than siblings of healthy children on most QoL domains (e.g. Physical Functioning, Behavior, Mental Health). Siblings older than the child with CF reported a higher impact of CF than younger siblings. Perceived impact of illness was higher when the child with CF had been hospitalized or was intermittent or chronically infected with Pseudomonas aeruginosa. CONCLUSIONS: Siblings of children with CF reported a good QoL. QoL and impact of illness were related to indices of CF severity. Insight into sibling-issues helps CF teams to provide family-oriented care.

Use of a mobile application for self-management of pancreatic enzyme replacement therapy is associated with improved gastro-intestinal related quality of life in children with Cystic Fibrosis.

BACKGROUND: Most patients with cystic fibrosis (CF) suffer from pancreatic insufficiency (PI), leading to fat malabsorption, malnutrition, abdominal discomfort and impaired growth. Pancreatic enzyme replacement therapy (PERT) is effective, but evidence based guidelines for dose adjustment are lacking. A mobile app for self-management of PERT was developed in the context of the HORIZON 2020 project MyCyFAPP. It contains an algorithm to calculate individual PERT-doses for optimal fat digestion, based on in vitro and in vivo studies carried out in the same project. In addition, the app includes a symptoms diary, educational material, and it is linked to a web tool allowing health care professionals to evaluate patient's data and provide feedback. METHODS: A 6-month open label prospective multicenter interventional clinical trial was performed to assess effects of using the app on gastro-intestinal related quality of life (GI QOL), measured by the CF-PedsQL-GI (shortened, CF specific version of the Pediatric Quality of Life Inventory, Gastrointestinal Symptoms Module). RESULTS: One hundred and seventy-one patients with CF and PI between 2 and 18 years were recruited at 6 European CF centers. Self-reported CF-PedsQL-GI improved significantly from month 0 (M0) (84.3, 76.4-90.3) to month 6 (M6) (89.4, 80.35-93.5) (p< 0.0001). Similar improvements were reported by parents. Lower baseline CF-PedsQL-GI was associated with a greater improvement at M6 (p < 0.001). CONCLUSIONS: The results suggest that the MyCyFAPP may improve GI QOL for children with CF. This tool may help patients to improve self-management of PERT, especially those with considerable GI symptoms.

Mental Health screening in cystic fibrosis centres across Europe.

OBJECTIVES: The Cystic Fibrosis (CF) International Mental Health Guidelines Committee published consensus statements for screening and treating depression and anxiety in individuals with CF and their caregivers. This work aimed to evaluate the dissemination and implementation of the guidelines in Europe two years following their publication. METHODS: A 28-item survey was developed by the multidisciplinary ECFS Mental Health Working Group and emailed to approximately 300 CF centres across Europe. The survey evaluated (a) who should be responsible for mental health (MH) care, (b) the current awareness and agreement of the guidelines, (c) the provision of recommended MH screening and follow-up care, and (d) successes, barriers and required resources/training needs. RESULTS: Responses were received from 187 centres (28 countries represented). There was consensus that a psychologist should be responsible for MH care, although members of the multidisciplinary team (MDT) believed they should also share this responsibility. Sixty-two percent of respondents were aware of the guidelines; 82% percent fully, and 12% partially, agreed with them. Fifty percent (94 centres) had implemented screening. In the past year approximately 6000 patients and 2000 caregivers had been screened, with 80% of respondents using the recommended screening tools. Respondents reported 551 referrals for moderate/severe psychopathology and 84 urgent suicide ideation referrals. CONCLUSIONS: The challenges of different healthcare systems and language barriers are being overcome with a greater awareness of the importance of mental health among the MDT. MH screening is feasible and gaining momentum in both Western and Eastern Europe.

Routine stresses in caring for a child with cystic fibrosis.

Difficulties experienced by mothers caring for a child with cystic fibrosis were assessed. Difficulties were categorized as: (1) developmental, which included tasks generic to all families (bedtimes, socialization, siblings and communication with others); and (2) illness, which were especially pertinent to the care of the child with cystic fibrosis (mealtimes, physiotherapy and home-based medical care). Age differences were found in stresses associated with developmental routines, with mothers of younger children reporting more difficulties. With regard to illness routines, more difficulties were reported for children in better health (higher Shwachman scores). The data suggest that parents experience increasing difficulties in implementing home-based medical care where the child is in better health. Clinic staff need to be more aware of these difficulties and target information to parents appropriately.

Coping and confidence among parents of children with diabetes.

Sixty-two mothers and 45 fathers of children with diabetes completed questionnaires to assess their confidence to manage the disease and treatment. Implications of parental confidence for attitudes to (1) child-rearing, (2) coping and (3) perceived restrictions of the disease were considered. Mothers and fathers differed in reported child-rearing behaviours and strategies for coping. More confident fathers reported that information was helpful in coping with the disease, while more confident mothers reported that support from the family was more helpful. Predictors of children's locus of control scores differed between mothers and fathers. However, children expressed more external locus of control beliefs where parents believed their children to be more restricted.

Healthy children's understanding of their blood: implications for explaining leukaemia to children.

Explanations of leukaemia to children usually include details of blood function and structure. In study 1, 3-year-olds (N = 25), 4-year-olds (N = 25), and 8-year-olds (N = 27) were interviewed about their understanding of the properties of blood and any occasions when they remembered seeing blood. They were also asked whether other animals and objects had blood or not, and to give reasons for this. Children justified their decisions in three ways: only people have blood, they had seen blood on a particular occasion, or only creatures which shared a human property (walking or talking) could have blood. In study 2, an intervention to increase children's understanding of the function of different blood cells is reported. It was partially successful. Children who reported an incident in which they saw blood were more likely to recall information about the function of red cells, white cells and platelets compared with children who did not report a previous experience (p < .001). In terms of explaining leukaemia to a child, we conclude that explanations should build on previous experience, and not be based simply on chronological age.

Measuring health-related quality of life in clinical trials in cystic fibrosis.

The inclusion of health-related quality of life (HRQoL) as an outcome measure in cystic fibrosis (CF) clinical trials can supply important patient-reported information not captured by other endpoints. Both an appropriate HRQoL measure and sound methodology are required in order to draw valid inferences about treatments and HRQoL. This paper provides the current consensus of the HRQoL Outcomes Group. Particular consideration has been given to the appropriateness of measurement scales, the rationale for including specific domains as endpoints, the importance of considering baseline ceiling effects and the difficulties of data interpretation. Guidance is provided on HRQoL measurement in National and European CF clinical trials.

Siblings of a child with cancer.

A total of 21 healthy siblings were interviewed about their experiences when a brother or sister is diagnosed with cancer. Information about the illness, opinions about who should inform siblings, social support, specific worries and worst memories were studied, as well as perceptions of any differences in the way they were treated by their parents. A short General Impact Scale was developed to assess the extent to which the siblings felt their lives had been disrupted by the illness. Results on these measures were compared with scores on four dimensions of the Sibling Perception Questionnaire (Carpenter & Sahler 1991). Siblings who reported some positive effects as a consequence of the illness (they had become more empathic toward others, or valued life more) perceived their interpersonal relations to be more negatively affected, i.e. there were greater difficulties in their relationships with others especially their parents. Higher scores on communication were related to less of an impact of the illness on life generally. However, these siblings also reported heightened concerns that their brother or sister might die. The data suggested that siblings should have opportunities to talk about implications of the disease, especially worries about death, and more efforts should be made to prepare siblings for visits to hospital and seeing the sick child.

Mothers' perceptions of parenting a child with spina bifida.

Mothers of 19 children (mean age = 8.8 years) with spina bifida were interviewed about medical and psychological services available, school placement and restrictions experienced by their children in school and social activities. Mothers also rated their own confidence to care for the child, perceived social support and difficulties experienced by the family. In addition, a measure of the child's disability status was derived. Mothers whose child scored higher in terms of disability reported greater everyday difficulties. In addition, mothers' confidence was related to perceived social support. Mothers reported regular use of medical services but little reliance on specialized nursing or psychological services.

Development of a measure to assess the perceived illness experience after treatment for cancer.

The development of a scale to measure perceived illness experience in young people with cancer is described. Areas of concern were first identified through semistructured interviews conducted with children and adolescents. As a result, 78 items were generated to cover the main areas identified (physical appearance, interference with activity, peer rejection, integration in school, manipulation, parental behaviour, disclosure, preoccupation with illness, and impact of treatment). These items were rated (on five point scales) by 41 patients (mean age 14.6 years) and 35 of their parents. Measures of physical functioning (symptoms, functional disability, and restrictions) and psychological functioning (symptoms) were included for validation purposes. Test-retest reliability was calculated on the basis of ratings made by a subsample of parents on two separate occasions. A 34 item scale was constructed with four items in each of the areas identified above, except for physical appearance (n = 2). The scale has adequate internal reliability and validity. There were significant correlations between parents and their children on all subscales except for illness disclosure and impact of treatment, suggesting that parents may be less reliable informants for their children in these contexts. The scale has potential use in clinical contexts, in evaluating the psychosocial impact of different treatment regimens, and as an outcome measure in intervention work.

Parents' attributions about childhood cancer: implications for relationships with medical staff.

Improvements in therapy have resulted in increasing numbers of children being successfully treated for cancer. However, the aggressiveness of therapy and uncertainty about prognosis are associated with many adverse effects, psychological as well as physical, for both the child and family. This study investigates family coping with treatment in relation to attributions about cause and responsibility for onset of the disease. We interviewed parents of 30 children with cancer (28 mothers and 23 fathers) about their attributions of causality, and perceptions of responsibility for the diagnosis and care of the child. Coping strategies which were found to be helpful were assessed using the Coping Health Inventory for Parents (CHIP). Fathers were more likely than mothers to accept that there was no known cause for the child's illness. Fathers who blamed doctors for failing to diagnose the condition found medical staff less helpful up to 2 years later. The theoretical importance of blaming others more than oneself is discussed, along with practical implications for services in paediatric oncology.

Adjustment to chronic disease in relation to age and gender: mothers' and fathers' reports of their childrens' behavior.

Investigated age and gender differences in adjustment to chronic disease in children suffering from one of five conditions: diabetes, asthma, cardiac disease, epilepsy, and leukemia. Ratings of adjustment and disease-related restrictions were obtained separately from mothers and fathers. Factor analysis of the adjustment scale yielded 6 subscales which differentiated between children in terms of age and disease type, and to a lesser extent, gender. Mothers' and fathers' ratings of adjustment and restrictions were comparable, though fathers made less differentiation on the basis of disease or age. For both parents, perceived restrictions of the disease were associated with poorer adjustment in the child, and this was particularly reflected on indices of peer relations and work.

Validity of the Rotterdam Symptom Checklist in paediatric oncology.

In order to determine the validity of the Rotterdam Symptom Checklist (RSC) for use with paediatric patients, a sample of 47 mothers with a child with acute lymphoblastic leukaemia (ALL) was asked to complete the RSC, the Play Performance Scale for Children PPSC and a measure of daily activity (HDI: Questionnaires were completed during routine outpatient visits. There were no effects of child age on number of symptoms reported. The physical symptom subscale of the RSC distinguished between children in terms of treatment status and number of hospitalisations. However, the psychological symptom subscale did not distinguish between these groups. Limitations of the scale for work with children are considered. These include difficulties experienced by patents in reporting psychological symptoms for their children, and the inappropriateness of a scale developed for adults to assess children. In the absence of other measures, the RSC can be used for children, but a more developmentally appropriate measures is needed.

Clinic audit for long-term survivors of childhood cancer.

Follow up for survivors of childhood cancer is considered essential in order to document any continuing impact on growth, fertility and other systems, as well as provide appropriate care and information to individuals themselves. Appropriate follow up needs to take into account the survivors' own views about reasons for attendance and perceived satisfaction with the services provided. Information was sought from 93 survivors (more than five years from diagnosis) and 68 of their parents regarding current attendance, understanding of the purpose of the clinic and satisfaction, and future preferences. Patients' main reasons for attending were to gain reassurance that they were well and information about the disease. There were some discrepancies between the types of information patients would like from clinic attendance and what they remembered being given. Parents were more positive than patients. Our data suggest that (i) knowledge in survivors is poor and (ii) it may be possible to define a subgroup for whom less frequent follow up is appropriate. A key component of care must involve education of patients, both about their past and the implications for future health.

Coming of age with diabetes: patients' views of a clinic for under-25 year olds.

This study is both an evaluation of patients' perceptions of a diabetes clinic for under-25 year olds, and an assessment of experiences in transferring from a paediatric clinic. Sixty-nine patients with Type 1 diabetes (74% of the total clinic), including 41 females, completed evaluations of the clinic and recall of experiences in transfer. Mean age on transferring clinic was 15.9 (range 12-20) years. Paediatricians were perceived to emphasise family and social life, school or work progress; adult physicians tended to stress the risk of long-term complications, importance of exercise and need to maintain strict levels of glycaemic control. Our data suggest that transition to adult care can generally be achieved non-traumatically, but patients may perceive some difficulty because of different emphases and treatment advice favoured by paediatricians and adult physicians.

Quality of life in young adults with type 1 diabetes in relation to demographic and disease variables.

Sixty-nine young adults (mean age 21 (range 15-25) years) with Type 1 diabetes completed measures of diabetes knowledge and quality of life. Factor analysis of the quality of life scale resulted in the identification of three subscales (social relationships, diabetes concerns, and impact). There was no relation between any of the quality of life subscales with knowledge, or with multiple versus twice-daily insulin injection regimens. Higher self-rated diabetes satisfaction was related to lower fructosamine levels and better clinic attendance (p less than 0.05). Females reported a more negative impact of diabetes on their lives compared with males (p less than 0.05).