Conflicting realities experienced by children with life-limiting and life-threatening conditions when transitioning to adult health services.

AIMS: The aim of this study was to report a secondary qualitative analysis exploring the cultural and practical differences that young people and parents experience when transitioning from children's to adult services. BACKGROUND: Despite two decades of research and quality improvement initiatives, young people with life-limiting and life-threatening conditions still find transition unsatisfactory. DESIGN: Secondary analysis: 77 qualitative interviews with children and young people (20), parents (35), siblings (1), professionals (21). METHODS: Qualitative framework analysis completed 2017. FINDINGS: Six conflicting realities were identified: Planning to live and planning to die with different illness trajectories that misaligned with adult service models; being treated as an adult and the oldest "patient" in children's services compared with being treated as a child and the youngest "patient" in adult services; being a "child" in a child's body in children's services compared with being a "child" in an adult's body in adult services for those with learning impairments; being treated by experienced children's professionals within specialist children's services compared with being treated by relatively inexperienced professionals within generalist adult services; being relatively one of many with the condition in children's services to being one of very few with the condition in adult services; meeting the same eligibility criteria in children's services but not adult services. CONCLUSION: Inequity and skills deficits can be addressed through targeted interventions. Expanding age-specific transition services, use of peer-to-peer social media, and greater joint facilitation of social support groups between health services and not-for-profit organizations may help mitigate age dilution and social isolation in adult services.

Mothers' and health visitors' perceptions of the support provided to mothers who have experienced domestic violence: A systematic review.

BACKGROUND: Domestic violence has been described as a public health epidemic, with victims of domestic violence encountered in all health care settings. Within the United Kingdom the role of the health visitor (specialist community public health nurse) is to promote health in the whole community; every family with a child under five years has a named health visitor. Preparation for the health visitor role is unique to the United Kingdom. Health visitors are particularly well placed to identify and support mothers who are experiencing domestic violence. OBJECTIVES: The objective of this review was to synthesise the best available evidence relating to support provided by UK health visitors for mothers who have experienced domestic violence, from both the mothers and the health visitors' perspectives. INCLUSION CRITERIA: The participants of interest were mothers who have experienced domestic violence and health visitors who offer support to those mothers.The self reported experiences of health visitor support provided to mothers who have experienced domestic violence, from the perspective of both the mothers and the health visitors providing the support.This review considered studies that focus on qualitative data including, but not limited to, designs such as ethnography, phenomenology, grounded theory, action research and feminist research. SEARCH STRATEGY: Studies published up to April 2011 were included in the review. The search was restricted to English language studies. The databases searched were: Medline, CINAHL, PsycINFO, PsycARTICLES, EMBASE, British Nursing Index and Archive, ASSIA and TRIP. METHODOLOGICAL QUALITY: Studies were assessed for methodological quality using the standardised critical appraisal instruments from the Joanna Briggs Institute. DATA EXTRACTION: Data were extracted using standardised data extraction tools from the Joanna Briggs Institute. DATA SYNTHESIS: Data synthesis used the Joanna Briggs Institute approach for meta-synthesis by meta-aggregation. Findings were synthesised into categories, which were aggregated into synthesised findings. RESULTS: Four qualitative papers were included in this review. Forty-five findings were synthesised into eighteen categories, from which six synthesised findings were derived which answered the review objectives. CONCLUSIONS: Mothers who have experienced domestic violence were reluctant to disclose this to health visitors; those who did disclose felt unsupported. Health visitors perceive domestic violence differently and their knowledge of strategies to deal with mothers experiencing domestic violence is limited. IMPLICATIONS FOR PRACTICE: Health visitors require appropriate training to enable them to recognise and discuss domestic violence, respond appropriately where they identify that mothers are experiencing domestic violence and provide support and guidance including information about, and appropriate referral to, support agencies. IMPLICATIONS FOR RESEARCH: Further research should determine what type of continuing training and support is effective for health visitors in identifying and supporting mothers who are experiencing domestic violence.

Supporting trail-blazing: A systematic review of the factors that facilitate or inhibit the implementation of new nursing roles: the experiences of UK consultant nurses.

BACKGROUND: If emerging 'trail-blazers', such as the consultant nurse, are to be successful in developing and sustaining new ways of working then factors that support or inhibit new role developments need identification. There is a growing body of evaluative and anecdotal evidence about the experience of consultant nurses since the introduction of the role in the UK thirteen years ago. OBJECTIVE: To synthesise the evidence on the experiences of UK consultant nurses in implementing a new role in order to identify inhibitors and facilitators of role development. INCLUSION CRITERIA: This review sought qualitative and mixed methodology studies that yielded qualitative data about the phenomenon of interest, together with narrative opinion papers i.e. consultant nurses' experiences of role development. SEARCH STRATEGY: The search in 14 databases considered studies and opinion papers published between January 1999 and April 2010 in English. METHODOLOGICAL QUALITY: All retrieved studies and opinion papers were assessed by two independent reviewers using the standardised Joanna Briggs Institute critical appraisal tools. DATA COLLECTION: Data were extracted from included papers using the standardised Joanna Briggs Institute data extraction tool. DATA SYNTHESIS: Data synthesis used the Joanna Briggs Institute approach for meta-synthesis by meta-aggregation. Findings were synthesised into categories and categories were aggregated into synthesised findings. RESULTS: On the basis of critical appraisal, no opinion papers were included in the review. A total of 11 qualitative studies were included, yielding a total of 313 findings. These were synthesised into 64 categories which were further synthesised into 11 synthesised findings about factors that facilitate and inhibit the role's implementation and development. CONCLUSION: Drawing directly on consultant nurses' experiences, these findings add evidence to what is already known about what does and does not work in developing a consultant role. They indicate that an understanding about the role's core functions is needed, as is a supportive environment in which the consultant can fully operate. IMPLICATIONS FOR PRACTICE: The implications for practice are derived from the results of the synthesised findings. An organisation, through its policies, practices, procedures and individuals, must support the consultant's leadership and collaborative goal directed approach to care delivery and service development. This can be achieved by allowing the consultant both autonomy and authority. The synthesised findings reinforce the need to optimise the facilitators and minimise the inhibitors of role development. They can also make a contribution to the conceptual understanding of the consultant role. IMPLICATIONS FOR RESEARCH: Further research into the experiences of consultant nurses is necessary - in particular, how nurse consultants interpret their relationships with others, negotiate resources and demonstrate the impact of their role in each of the core functions, notably the leadership function.

Promoting and supporting self-management for adults living in the community with physical chronic illness: A systematic review of the effectiveness and meaningfulness of the patient-practitioner encounter.

BACKGROUND: There has been a reported rise in the number of people with chronic illness (also referred to as long-term disease) in the Western world. One hundred million people in the United States have at least one chronic condition and in the United Kingdom (UK) as many as 17.5 million adults may be living with chronic disease. New models of care have been developed which recognise the complexities of managing care where there is overlap between the wider community, the health care system and provider organisations, for example, the Chronic Care Model and the Expert Patient Programme. These new models herald a shift away from the idea of chronically ill patients as passive recipients of care towards active engagement, in partnership with health professionals, in managing their own care.Partnership, ideally, involves collaborative care and self-management education. This may support self-care alongside medical, preventative and health maintenance interventions. In this context the nature of the patient-practitioner consultation in promoting self-care takes on a new importance. OBJECTIVE: The overall objective of the review was to determine the best available evidence regarding the promotion and support of self-care management for adults living in the community with chronic illness during the patient-practitioner encounter. Specifically the review sought to determine: What is the effectiveness of the patient-practitioner encounter in promoting and supporting self-care management of people with chronic illness? What are the individual and organisational factors which help or hinder recognition, promotion and support of chronic disease self-care management strategies? What are the similarities and differences between how 'effectiveness' is defined in this context by patients and different practitioners? INCLUSION CRITERIA: The review focussed on self-caring adults aged nineteen years and older living in the community, with a physical chronic illness, and not currently being treated as an in-patient. For example, people with diabetes, asthma, arthritis, coronary disease, lung disease, heart failure, epilepsy, kidney disease and inflammatory bowel disease. Since patients meet various professionals in a variety of community settings regarding their care, a practitioner in this review included doctors (physicians and General Practitioners), nurses, nurse specialists, dieticians, podiatrists and community health workers.A variety of outcomes measures was used to evaluate effective self-care management. These included physiological measurements such as: HbA1c, blood pressure, body weight, lipids; lifestyle measurements, for example physical activity; and self-care determinants such as knowledge, attitude; and self-care behaviours regarding, for example, diet and physical exercise, and medication. The outcome measures used to explore the meaningfulness of the patient-practitioner encounter, concerned patients', physicians' and nurses' views and perceptions of self-care management and support.The review considered all types of quantitative and qualitative evidence regarding the patient-practitioner encounter where self-care in chronic illness was the focus. The quantitative studies reviewed included systematic reviews, randomised controlled trials (RCTs), quasi-experimental studies, and survey studies.Qualitative studies reviewed included interview designs, vignette technique, qualitative evaluation, grounded theory, and exploratory descriptive design. SEARCH STRATEGY: The search sought to find both published and unpublished studies between 1990 and 2005. The year 1990 was deemed appropriate since it precedes the development of the Chronic Care Model in which self-management support for people living with chronic illness is heralded as an important part of care-management. An initial search of CINAHL and MEDLINE databases was undertaken to identify appropriate search terms regarding self-care and chronic illness. A search strategy was then developed using all identified MeSH headings and key words and the following databases were searched: - Ovid CINAHL; Ovid MEDLINE (R); Ovid EMBASE; Ovid EBM Reviews (CDSR, ACP Journal Club, DARE, CCTR); ASSIA; SIGLE; Digital Dissertations; and British Library's Zetoc Services. DATA COLLECTION: Thirty-two papers were considered applicable to the review topic from the title and abstract. Two reviewers used the appropriate critical appraisal instruments designed by the Joanna Briggs Institute (JBI) to assess methodological quality of papers retrieved for review, and agreed on the papers for inclusion. A total of 18 papers reporting 16 studies were included in the review (3 papers reported from the same study): 12 quantitative studies, 5 qualitative studies and 1 study using mixed methods. These papers were heterogeneous in nature, diverse in subject matter and considered a wide range of physiological, psychological, sociological and behavioural self-care outcome measures. Data were extracted by the two independent reviewers using a variety of data extraction instruments developed by JBI. DATA ANALYSIS: The heterogeneous nature of the quantitative studies prevented meta-analysis and so these studies are presented in narrative summary. Meta-synthesis of the qualitative data was performed for the six qualitative pieces following the process of meta-synthesis set out in the JBI-QARI software package. The process of meta-synthesis embodied in this programme involves the aggregation or synthesis of findings. Seven syntheses were produced from fifty findings. RESULTS: For effective patient-centeredness to be established patients should be able to discuss their own ideas about self-care actions, including lifestyle management in an unhurried fashion and with a practitioner who has the time and who is willing to listen. Patient-centred interventions aimed at providers such as patient-centred training and patient-centred materials were shown to have a positive effect on the patient-centeredness of an encounter, but their effect on self-care outcomes was not clear. Interventions directed at enhancing patient participation in the encounter were shown to effect diabetes self-care and self-behaviour.Nurses were shown to have an effective role in educating patients and facilitating adherence to treatment. Patients found nurses approachable and some studies showed that when given the choice, patients were more likely to contact a nurse (than a doctor) regarding their care.Professional interventions such as education, and organisational interventions such as management of regular review and follow up, were shown to improve process outcomes in the management of a patient-practitioner encounter. When patient-orientated interventions were added to professional and organisational interventions, in which patient education and / or the role of the nurse was enhanced, patient health outcomes were improved.The different patient-orientated interventions reviewed highlighted some of the elements that can effectively support self-care management during a patient-practitioner encounter. These are information giving, including the use of a guidebook, the use of care plans, the structure of treatment using checklists, and education and support for staff in 'collaboratives'.Comprehensive, well-paced, user-friendly information is effective in supporting and promoting self-care management in a variety of ways. It informs and reassures patients and their families. It can be used during a doctor/patient consultation to assist communication between doctors and patients, and may help patients feel more involved in their care.For information to effect self-care management, it is important that it is given at diagnosis and from then onwards so that the implications of good self-care management in relation to long term health outcomes are established.Care plans and self-management plans can be useful in facilitating patients' discussion of self-care actions and lifestyle management.Organisational factors affect opportunities for professionals to support patient self-care management. These include time, resources, the existing configuration and expectations of a consultation, the opportunity for open access to appointments, the ability to see the same doctor and early referral to other professional groups.Correlational design studies indicated that individual psychological factors, such as attachment style and autonomy support given to a patient during a patient-practitioner encounter, have a relationship to self-care behaviours and outcomes.Correlational design studies indicated that both general communication and diabetic specific communication used during a patient-practitioner encounter have a positive effect on patient self-care management and outcomes for patients with diabetes.Consultations about self-care for patients with chronic illness tend to be medically focussed and do not always include discussion of patients' views of the routines and self-care actions. This can lead to tension and unresolved issues between the patient and professional.Studies in the context of diabetes self-management reveal that professionals can effectively support patients in a number of ways. These include assisting the orientation of patients towards skills and competencies needed for self-care; sharing knowledge and information; endorsing the patient's view that he or she is the most reliable and accurate source of information about his or her physiological function; trusting the patients' interpretations of their physiological function, and modifying advice in response to patients in accordance with their bodily cues and experiences. CONCLUSION: The nature of the patient-practitioner encounter is multifaceted involving patient, professional and organisational factors. Patient-orientated interventions are the most effective in effecting positive self-care behavioural and health outcomes. Patient participation in thepatient-practitioner encounter is a key factor in influencing self-care outcomes. Patients' self-care management involves social as well as medical management. Professionals need to recognise and value patients' views and experiences in order to support their self-care management. IMPLICATIONS FOR PRACTICE: Patients need information at diagnosis and from then onwards to enable good self-care management. It is important to enable patient participation during the patient-practitioner encounter.For patients' self-care needs to be addressed opportunities for patients to talk about their diet, routines and lifestyle management need to be incorporated into the encounter. Extra time in consultations may be required. Care plans can help to facilitate this discussion.To support patients with their self-care management, both sharing of medical and nursing knowledge, and recognition of the value of patient's knowledge and experiences are vital.Nurses relate well to patients who want to discuss self-care management.Professional interventions and organisational interventions can improve the management of a patient-practitioner encounter. Patient-orientated interventions in addition to good management of the encounter can improve health care outcomes. IMPLICATIONS FOR RESEARCH: Patient focussed interventions have a positive effect on patient self-care outcomes. Further research regarding patients' self-care and health outcomes and behaviours is needed to establish which patient focussed interventions in particular are effective.Qualitative research has proved to be important in understanding the different ways that professionals and patients approach self-care management during an encounter. More qualitative research would assist an understanding of the processes that inspire effective partnership between patients and professionals to support the establishment of self-care management of chronic illness.

Weak linkage at 4p16 to predisposition for human neuroblastoma.

The most frequent genetic alterations described in neuroblastoma (NB) are amplification of MYCN oncogene and deletion of chromosome 1p, although somatic deletions have been demonstrated at other chromosomal intervals. Since loss of heterozygosity (LOH) at distal 4p has been observed in about 20-29% of neuroblastomas, we have evaluated deletions in 41 Italian NB samples by LOH analysis at loci mapping to 4p as follows: pter-D4S2936-D4S412-D4S2957-D4S432-D4S3023-D4S431-cen. Our analysis showed allele losses in eight out of 41 samples (19.5%) and allowed the identification of a smallest region of overlapping deletion (SRO) of 3.0 cM, delimited by D4S412 and D4S3023. Two of these tumors with 4p LOH are from patients belonging to a family with recurrent NB. Interestingly the genotyping of this family revealed an identical haplotype that includes the nonrecombinant loci D4S412, D4S2957 and D4S432 shared by all affected children and demonstrated that this haplotype is retained in the two tumors carrying somatic deletions from patients of this family. Furthermore linkage analysis was performed in two NB families and yielded an overall lod-score of 3.0 in the interval including the haplotype. This provides a confirmatory indication that the region delimited by D4S2936 and D4S3023, which also includes the new defined SRO, may harbor NB predisposing gene/s.

Linkage analysis in families with recurrent neuroblastoma.

Neuroblastoma is a neural crest-derived tumor of childhood with a serious prognosis; only 20% of patients with stage 4 disease survive 5 years from diagnosis. Mechanisms involved in neuroblastoma development are unclear, but the engagement of many neuroblastoma-related gene(s) is suggested by specific chromosomal alterations. Most prominent among these is the amplification of the MYCN oncogene and the deletion of the 1p36 region. Other genetic aberrations have been discovered over the years such as deletions of 11q and 14q and gain of 17q. Although tumor aggressiveness greatly depends on the most frequent genetic abnormalities, to date no neuroblastoma-related gene has been discovered. Neuroblastoma usually occurs sporadically, but 1.5% of all diagnosed cases show familial recurrence with an autosomal dominant inheritance and incomplete penetrance. A comparison between hereditary and sporadic neuroblastomas led Knudson and Strong to gather that the two-hit hypothesis, proposed for retinoblastoma, could be applied to neuroblastoma. To determine if the 1p36 region harbors a predisposition gene for familial neuroblastoma, we carried out linkage analysis at 1p36 loci in two families with recurrent neuroblastoma. Similarly, we analyzed loci of chromosome 16, where a predisposition locus was recently mapped. We also analyzed markers located close to several candidate genes (RET, NF1, GDNF, GFRA1, EDNRB, and EDN3) involved to a different extent in other neurocristopathies. Our findings indicate that the candidate chromosomal regions and genes analyzed are not in linkage with neuroblastoma.