Parenting stress in mothers with cystic fibrosis.

AIM: To assess the parenting experience of mothers with cystic fibrosis (CF) and to compare with normative data. METHODS: Cross-sectional study with a validated generic parental stress questionnaire (PSQ). This PSQ differentiates four components of parental stress: main factor "parental stress", compounding factor "role restrictions", protective factors "support from spouse", and "social support". Cut-off scores categorise results as "normal", "borderline" or "concerning". SAMPLE: Seventy-three women were informed by their local CF centre. Of these, 36 enrolled and had a first-born child aged 1-12 years (consistent with reference values of the PSQ). Of these, 31 (86%) returned the PSQ. Mean age of mothers was 32.6 years ± 6.9 years, mean age of first-born child was 5.2 years ± 3.4 years. Most of the mothers had one biological child, five women had two children and one had three children. RESULTS: Parental stress scores were normally distributed, the same applies for contributing factors and for the two protective factors. Favourable scores were twice as frequent as concerning scores. Mothers of younger children scored slightly better than mothers of school-aged children. CONCLUSION: In line with the only comparable study, mothers with CF seem to be a remarkably resilient group who mostly cope well with parental stress even in the face of a progressive, chronic disease requiring time-consuming treatment. IMPLICATIONS FOR REHABILITATION: Today, motherhood is increasingly becoming an option in fertile women with cystic fibrosis. The additional burden of parenting seems to be rewarded by fulfilling essential personal goals. CF clinics should routinely address a possible wish for a child and to discuss it, openly.

Patient experience in cystic fibrosis care: Development of a disease-specific questionnaire.

OBJECTIVES: The aim of this study was to develop valid and reliable disease-specific questionnaires for adult patients with cystic fibrosis and for parents of minors with cystic fibrosis for assessing patient experience with cystic fibrosis care. METHODS: The pilot versions of the questionnaires were developed based on a literature review, interviews with health professionals and focus groups. A postal survey with two reminders was conducted in 56 German cystic fibrosis centres recruiting 2874 participants. Psychometric evaluation was done via exploratory factor analysis and reliability and regression analysis. The questionnaires' ability to differentiate between subgroups and between cystic fibrosis centres was evaluated. RESULTS: Response rates were 74% for both adult patients and parents. Ten factors were extracted for both the adult and the parents' models (Cronbach's alpha between 0.6 and 0.9), explaining 50% and 48% of the variance, respectively. The factors organisation & access and the doctor-patient/parent-interaction had the highest relevance for a good overall care experience. The questionnaires were able to distinguish between different cystic fibrosis centres. DISCUSSION: The questionnaires are well suited for use in internal and external quality management of cystic fibrosis care due to their good psychometric properties, the ability to differentiate between centres and its practicability.

European Cystic Fibrosis Society Standards of Care: Best Practice guidelines.

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in Table 1. We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.

Guiding principles on how to manage relevant psychological aspects within a CF team: interdisciplinary approaches.

Managing CF can be emotionally and physically challenging for patients and their relatives. The disease and its treatment influence the ability to tackle normal tasks of daily living and unexpected life events. The context within which psychologists work varies according to different cultural backgrounds and their professional and theoretical memberships. The benchmarks presented here focus on four crucial issues: (i) identifying a common base of tools and theoretical reflections through suggested readings, (ii) interdisciplinary work within a CF team and its importance for both persons with CF and other healthcare professionals, (iii) the benefits of an eclectic approach utilising cognitive-behavioural theories for specific psychological problems and, (iv) effective and evaluated transition programmes from paediatric to adult healthcare services.

Lung transplant recipients' views on the integration of their new organs.

AIM: Although donorship issues and the integration of the new organs are the most distinguishing aspects of lung transplantation (LTx) compared to other kinds of 'high-tech' medicine, there is a paucity of papers on that matter. Therefore, we aimed to evaluate these aspects in young adult LTx recipients with at least 1-year survival. METHODS: Semi-structured interviews; content analysis of specific parts of the interviews; frequency distributions of resulting categories. SAMPLE: Forty-five adults aged 18-42 years (mean: 32 +/- 5.5 years). Post-transplant survival ranged from 1 to 11 years. RESULTS: The majority of recipients (60%) fulfilled criteria of denial towards the foreign organ and/or the donor. However, they were rather sensitive towards the ethical and psychological impact of the upcoming option of living donorship in LTx. Also, the majority of recipients (79%) understood that there might as well be good reason not to opt for a LTx. CONCLUSIONS: LTx recipients appeared at first sight 'pragmatic' towards the gift of life, but they remained sensitive to its ethical and psychological challenges.

Disease impact and disease-unrelated strain in parents of children with rheumatic disease: is there an influence on disease-related information needs?

OBJECTIVE: To explore the information needs of parents of children with juvenile idiopathic arthritis and other rheumatic diseases with emphasis on possible intervening factors, such as disease-related or disease-unrelated strain. METHODS: A cross-sectional, anonymous survey using a purpose-designed questionnaire, incorporating, among others, the Impact on Family Scale. The questionnaire was sent to 146 families continuously attending a pediatric rheumatology outpatient clinic. The response rate was 80%. The mean age of the children was 6.9 (± 4.3) years. Mean CHAQ score was 0.259 (± 0.45; range 0.0 to 2.13). RESULTS: Parents considered themselves well-informed. They frequently mentioned having received information on core domains of medical aspects. They described deficits related to psychosocial impact, to vocational education and to complementary therapy. However, their interest in further information was high, almost irrespective of the amount of prior information. Unexpectedly, neither disease impact on the family nor the presence and number of disease-unrelated life events seemed to have an influence (positive or negative) on the parents' information needs. CONCLUSION: With respect to individually tailored information strategies, it should be taken into account that even for topics largely covered by usual medical advice, residual interest and information needs remain high. We were furthermore able to ascertain topics of particular interest but failed to substantiate our clinical impression that families who feel burdened with disease-related and or disease-unrelated stressors are less likely to be interested in further disease-related information.

Parental knowledge and behaviour to prevent environmental P. aeruginosa acquisition in their children with CF.

BACKGROUND: Chronic infection with Pseudomonas aeruginosa (PA) is associated with an accelerated decline of lung function in patients with cystic fibrosis (CF). Precautions to avoid contact with PA from the environment have been recommended by caregivers and are being carried out by many families with CF children. The present study was conducted to portray the spectrum of hygienic measures and to evaluate the restrictions and impact caused by these measures. SUBJECTS AND METHODS: In a multi-centre survey, parents of children below 13 years of age responded to mailed questionnaires. The items covered parental knowledge of PA, information provided by caregivers, the parents' feelings and thoughts about PA infection, and measures taken in daily life to prevent a possible contact with PA in the environment. RESULTS: 130 parents from 10 CF centres responded to the questionnaire (63% response rate). 76% of the respective children had always been PA negative. Most parents displayed erroneous beliefs regarding PA infection (mean: 3.5 correct replies to 9 questions). Families performed a mean of 11 different hygienic measures, e.g. they prevented their child from being the first person to use the bathroom in the morning (72%) or from bathing in gravel pits and standing water (52%). The majority of parents felt markedly (44%) or somewhat (44%) stressed that their child might acquire PA, and many parents felt markedly (16%) or somewhat (43%) restricted and stressed by the hygienic measures. Less stressed parents tended to have more knowledge and undertook fewer measures. CONCLUSION: When informing and teaching parents on the nature of PA infection, caregivers should provide clear recommendations on reasonable actions to be taken. Also, physicians should anticipate and adequately respond to parental fears and misconceptions.

Consulting the "experts": a pilot study on perceptions of professional support among lung transplant recipients and accompanying relatives.

BACKGROUND: Lung transplantation is extremely stressful for patients and accompanying support persons. PURPOSE: To improve delivery of care, we designed a cross-sectional study about unmet needs and perceived helpfulness of staff. METHODS: The sample consisted of 30 adult lung transplant recipients with cystic fibrosis (and 22 relatives) and 20 age-matched recipients with other reasons for transplantation (and 17 relatives). Mean survival since transplantation was 5 years. Data were collected via questionnaires (numerical rating scales and fill-in-the-blank items). RESULTS: Most patients in both groups were satisfied with staff support (nurse, doctor) especially during the acute stage of illness. Relatives were less satisfied at all stages. Patients' satisfaction with doctors' support was higher after than before transplantation, but the opposite was true for relatives. Insufficient continuity of care was the most frequent critical comment from patients and support persons. Recommendations to improve delivery of care included providing access to psychosocial professionals and broadening the information provided before transplantation. CONCLUSIONS: Regular screening of customer satisfaction should become routine. Particular attention should be paid to support persons.

Different opinions of physicians on the importance of measures to prevent acquisition of Pseudomonas aeruginosa from the environment.

BACKGROUND: Since chronic infection with mucoid Pseudomonas aeruginosa (PA) is associated with deteriorating lung function, many parents of young children with cystic fibrosis (CF) fear the first PA positive throat swab as a milestone in the progression of the disease. To reduce the risk of PA acquisition from the environment, they perform preventive measures at home or outdoors. METHODS: In an attempt to evaluate the attitude of CF physicians towards these measures and the respective consulting practice, we mailed a questionnaire to all 65 certified paediatric CF centres in Germany. RESULTS: Physicians from 54 (83%) CF clinics replied. They expressed widely different ideas about the impact of the environment for the acquisition of P. aeruginosa, and recommended a large spectrum of preventive measures. Some physicians proposed only few precautions, which focussed on the prevention of cross-infection between patients, whereas others suggested prevention of any contact with moist or wet places, e.g. use different toothbrushes for mornings and evenings, or do without air-conditioning in the car. CONCLUSIONS: CF physicians have different opinions on the risk of PA acquisition from the environment. Doctors who recommend strict precautions could engender a parental fear of a ubiquous threat from invisible bacteria. The resulting extended safety measures might impair the family's quality of life.

How do adolescents with juvenile chronic arthritis consider their disease related knowledge, their unmet service needs, and the attractiveness of various services?

AIMS: To explore the information needs of adolescents with juvenile chronic arthritis (JCA) with respect to patient education and other measures to promote self-management. METHODS: Standardized cross-sectional inquiry concerning disease-related knowledge, perceived importance of information giving, unmet needs as well as perceived attractiveness of a range of services (lecture, structured patient education, support group, self-help group) to promote self-management. SAMPLE: N = 48 adolescents (68% of all adolescents with JCA of our outpatient clinic); mean age x = 14.9 (+/- 2.1) years; 56% female; 17% had the oligoarthritis form of JCA, 40% juvenile spondylarthritis, 25% polyarthritis and systemic form, 19% other rheumatic diseases. RESULTS: The majority of adolescents considered themselves as sufficiently well-informed and voted in favour of detailed information giving. However, 30% were unsatisfied with their current information and knowledge. Information needs predominantly related to the prognosis, course, and treatment of JCA, whereas the psychosocial impact (except sports and job matters) were judged as less important. Adolescents with a lower level of education were generally more interested than those with a high level of education. As for the attractiveness of services nearly half of the adolescents judged all of them as not very attractive. CONCLUSIONS: The majority of adolescents is interested in detailed information giving and some of them point to unmet needs, but nearly half of them is hesitant towards services which are delivered in a group format (such as structured patient education or support groups).

Parental fears of Pseudomonas infection and measures to prevent its acquisition.

BACKGROUND AND AIM OF THE STUDY: Chronic infection with Pseudomonas aeruginosa (PA) is associated with accelerated worsening of lung disease in patients with cystic fibrosis (CF). Fears of PA are widespread among parents of CF children, and many parents take precautions at home to prevent acquisition of the bacterium from the environment. The present study was undertaken to describe the type and intensity of these activities. METHODS: Parents of 21 CF children (7 without prior PA infection, 10 with intermittent and 4 with chronic PA infection) were investigated using semistructured interviews. These were analyzed descriptively and with respect to predominant themes. Additionally, a German personality test was used to evaluate the influence of psychological factors. RESULTS: The clinical impression of widespread parental anxieties of PA infection was confirmed. Misunderstandings concerning PA infections were related to a simplistic concept of the underlying biological mechanisms. Some parents which we classified as 'bacterium-focussed' thought that each contact with PA would lead to bacterial infection. These parents used a large variety of measures, which concerned both domiciliary and outdoor surroundings and activities. At the other end of the spectrum were parents which we classified as 'child-focussed' who mostly supported (and relied on) the child's defense mechanism instead of hygienic measures. CONCLUSIONS: Recommendations by physicians on how to prevent PA acquisition from the environment should take into account possible non-intended side effects, since some parents will exaggerate daily precautions to the detriment of the child's (and the parent's) quality of life.