Primary sclerosing cholangitis (PSC) is a rare bile duct and liver disease which can considerably impact quality of life (QoL). As part of a project developing a measure of QoL for people with PSC, ...we conducted a systematic review with four review questions. The first of these questions overlaps with a recently published systematic review, so this paper reports on the last three of our initial four questions: (A) How does QoL in PSC compare with other groups?, (B) Which attributes/factors are associated with impaired QoL in PSC?, (C) Which interventions are effective in improving QoL in people with PSC?.
We systematically searched five databases from inception to 1 November 2020 and assessed the methodological quality of included studies using standard checklists.
We identified 28 studies: 17 for (A), ten for (B), and nine for (C). Limited evidence was found for all review questions, with few studies included in each comparison, and small sample sizes. The limited evidence available indicated poorer QoL for people with PSC compared with healthy controls, but findings were mixed for comparisons with the general population. QoL outcomes in PSC were comparable to other chronic conditions. Itch, pain, jaundice, severity of inflammatory bowel disease, liver cirrhosis, and large-duct PSC were all associated with impaired QoL. No associations were found between QoL and PSC severity measured with surrogate markers of disease progression or one of three prognostic scoring systems. No interventions were found to improve QoL outcomes.
The limited findings from included studies suggest that markers of disease progression used in clinical trials may not reflect the experiences of people with PSC. This highlights the importance for clinical research studies to assess QoL alongside clinical and laboratory-based outcomes. A valid and responsive PSC-specific measure of QoL, to adequately capture all issues of importance to people with PSC, would therefore be helpful for clinical research studies.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Background
Primary sclerosing cholangitis (PSC) is a rare incurable disease of the bile ducts and liver which can significantly impair quality of life (QoL). No existing QoL tools are entirely ...suitable for people living with PSC (PwPSC). We aimed to develop a measure of QoL for PwPSC in the UK, beginning by identifying relevant QoL issues. This paper describes our approach to this first stage, and discusses related benefits and limitations.
Methods
Scientific consensus on how to reliably stage PSC is lacking, due to its rarity and heterogeneity. We initially hypothesised four categories for PSC severity. After beginning the study, these were revised to six. For such a rare disease, the study could not recruit sufficient participants in each of these categories, particularly the more severe, in the time available. We therefore modified the design, adapting standard methodology for identifying potentially relevant issues. We started by conducting a thematic analysis of data from a previous survey of PwPSC, and extracting QoL issues from a literature review of QoL questionnaires of relevance to PwPSC. We then conducted group and individual interviews with PwPSC and clinicians, investigating the relevance, importance, phrasing, and breadth of coverage of issues identified. We also explored the validity of our hypothesised categories for disease severity.
Results
We identified 1,052 potentially relevant QoL issues from the survey and literature review and took 396 of these forwards for discussion with 28 PwPSC. We found 168/396 issues were considered relevant by ≥ 60% of these participants. We then discussed this subset of 168 issues with 11 clinicians. PSC and clinician participants identified some problematic phrasing with 19 issues, due to potential upset (n = 12) or problems with understanding (n = 7). We included one new issue from those suggested.
Conclusion
We identified a range of QoL issues relevant to PwPSC, with a good breadth of coverage, although lacking an in-depth understanding of the PSC experience. Our strategy effectively identified relevant QoL issues for people living with this rare condition, for which there is no consensus on stratifying for its severity. This strategy should however be considered specific to such circumstances, not a general recommendation for an alternative approach.
Parents of children who have a congenital anomaly can experience significant worry about their child's health. Access to clear, helpful, and trustworthy information can provide a valuable source of ...support. In this study the aim was to explore the information needs of parents/carers of children with congenital anomalies across Europe.
A cross-sectional online survey was developed in nine languages to measure parents' information needs, including: (1) the 'helpfulness'/'trustworthiness' of information received from eight relevant sources, and (2) overall satisfaction with information received. Parents/carers of children (0-10 years) with cleft lip, spina bifida, congenital heart defect CHD requiring surgery, and/or Down syndrome were recruited online via relevant organisations in 10 European countries from March-July 2021. Quantitative analyses using multivariable logistic regressions were performed.
One thousand seventy parents/carers of children with a cleft lip (n = 247), spina bifida (n = 118), CHD (n = 366), Down syndrome (n = 281), and Down syndrome with CHD (n = 58) were recruited in Poland (n = 476), the UK (n = 120), Germany (n = 97), the Netherlands/Belgium (n = 74), Croatia (n = 68), Italy (n = 59), other European countries (n = 92), and not specified/non-European countries (n = 84). Most participants were mothers (92%) and aged 31-40 years (71%). Participants were most likely to rate support groups (63%), patient organisations (60%), specialist doctors/nurses (58%), and social media (57%) as 'very helpful' information sources. 'Very trustworthy' ratings remained high for specialist doctors/nurses (61%), however, they declined for support groups (47%), patient organisations (48%), and social media (35%). Germany had the highest proportion of participants who were 'very satisfied' (44%, 95% CI = 34%-54%) with information, whereas this percentage was lowest in Croatia (11%, 95% CI = 3%-19%) and Poland (15%, 95% CI = 11%-18%). Parents of children with Down syndrome had significantly lower satisfaction ratings than parents of children with CHD; 13% (95% CI = 8%-18%) reported being 'very satisfied' compared to 28% (95% CI = 23%-33%) in the CHD group.
Findings suggest that informal sources of information (e.g. support groups) are of value to parents, however, they are not deemed as trustworthy as specialist medical sources. Satisfaction ratings differed across countries and by anomaly, and were particularly low in Croatia and Poland, as well as for parents of children with Down syndrome, which warrants further investigation.
ObjectiveTo survey parents and carers of children with a congenital anomaly across Europe about their experiences of healthcare services and support during the COVID-19 pandemic.DesignCross-sectional ...study.SettingOnline survey in 10 European countries, open from 8 March 2021 to 14 July 2021.Population1070 parents and carers of children aged 0–10 years with a cleft lip, spina bifida, congenital heart defect (CHD) requiring surgery and/or Down syndrome.Main outcome measuresParental views about: the provision of care for their child (cancellation/postponement of appointments, virtual appointments, access to medication), the impact of disruptions to healthcare on their child’s health and well-being, and satisfaction with support from medical sources, organisations and close relationships.ResultsDisruptions to healthcare appointments were significantly higher (p<0.001) in the UK and Poland, with approximately two-thirds of participants reporting ‘cancelled or postponed’ tests (67/101; 256/389) and procedures compared with approximately 20% in Germany (13/74) and Belgium/Netherlands (11/55). A third of participants in the UK and Poland reported ‘cancelled or postponed’ surgeries (22/72; 98/266) compared with only 8% in Germany (5/64). In Poland, 43% (136/314) of parents reported that changes to their child’s ongoing treatment had moderately to severely affected their child’s health, significantly higher than all other countries (p<0.001). Satisfaction ratings for support from general practitioners were lowest in the UK and Poland, and lowest in Poland and Italy for specialist doctors and nurses.ConclusionA large proportion of participants reported disruptions to healthcare during the pandemic, which for some had a significant impact on their child’s health. Regional differences in disruptions raise questions about the competence of certain healthcare systems to meet the needs of this vulnerable group of patients and indicate improvements should be strived for in some regions.
Background:
Multiple sclerosis (MS) is a complex disease with new drugs becoming available in the past years. There is a need for a reference tool compiling current data to aid professionals in ...treatment decisions.
Objectives:
To develop an evidence-based clinical practice guideline for the pharmacological treatment of people with MS.
Methods:
This guideline has been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology and following the updated EAN recommendations. Clinical questions were formulated in Patients–Intervention–Comparator–Outcome (PICO) format and outcomes were prioritized. The quality of evidence was rated into four categories according to the risk of bias. The recommendations with assigned strength (strong and weak) were formulated based on the quality of evidence and the risk-benefit balance. Consensus between the panelists was reached by use of the modified nominal group technique.
Results:
A total of 10 questions were agreed, encompassing treatment efficacy, response criteria, strategies to address suboptimal response and safety concerns and treatment strategies in MS and pregnancy. The guideline takes into account all disease-modifying drugs approved by the European Medicine Agency (EMA) at the time of publication. A total of 21 recommendations were agreed by the guideline working group after three rounds of consensus.
Conclusion:
The present guideline will enable homogeneity of treatment decisions across Europe.
Most research on interventions to counter stigma and discrimination has focused on short-term outcomes and has been conducted in high-income settings.
To synthesise what is known globally about ...effective interventions to reduce mental illness-based stigma and discrimination, in relation first to effectiveness in the medium and long term (minimum 4 weeks), and second to interventions in low- and middle-income countries (LMICs).
We searched six databases from 1980 to 2013 and conducted a multi-language Google search for quantitative studies addressing the research questions. Effect sizes were calculated from eligible studies where possible, and narrative syntheses conducted. Subgroup analysis compared interventions with and without social contact.
Eighty studies (n = 422 653) were included in the review. For studies with medium or long-term follow-up (72, of which 21 had calculable effect sizes) median standardised mean differences were 0.54 for knowledge and -0.26 for stigmatising attitudes. Those containing social contact (direct or indirect) were not more effective than those without. The 11 LMIC studies were all from middle-income countries. Effect sizes were rarely calculable for behavioural outcomes or in LMIC studies.
There is modest evidence for the effectiveness of anti-stigma interventions beyond 4 weeks follow-up in terms of increasing knowledge and reducing stigmatising attitudes. Evidence does not support the view that social contact is the more effective type of intervention for improving attitudes in the medium to long term. Methodologically strong research is needed on which to base decisions on investment in stigma-reducing interventions.
Bipolar disorder is associated with an increased risk of suicide and physical illness, such as ischaemic heart disease, diabetes, chronic obstructive airways disease, pneumonia, and unintentional ...injury. 2 Around two thirds of people with bipolar disorder also experience another mental disorder, usually anxiety disorders, substance misuse disorders, or impulse control disorders. 1 The risk of recurrence in the year after a mood episode is especially high (50% in one year and >70% at four years) compared with other psychiatric disorders, 3 and this has important implications for the long term management of the disorder. (New recommendation.) Based on the experience and opinion of the GDG Key symptoms of mania and severe depression Mania Expansive, grandiose affect Inflated self esteem Increased talkativeness Decreased need for sleep Increase in impulsive risk taking behaviour Depression Depressed mood Profound loss of interest in activities Feelings of worthlessness Weight loss or gain Suicidal thoughts or actions Monitor the physical health of people with bipolar disorder when responsibility for monitoring is transferred from secondary care, and then at least annually.
Background: Primary sclerosing cholangitis (PSC) is a rare disease of the liver and bile ducts which can impact on quality of life (QoL). This research aimed to develop and pre-test a measure of QoL ...for people with PSC (PwPSC) in the UK. Methods: The study followed a two-stage mixed-methods design, drawing on existing guidance for tool development. Stage 1 identified QoL issues of relevance to PwPSC from an online survey and literature review of relevant QoL tools. Consensus on how to reliably stage PSC is lacking, so six categories of disease severity/co-morbidities were hypothesised, including: co-morbid inflammatory bowel disease, awaiting liver transplant, PSC-related cancers. Issue relevance, importance and phrasing were explored with PwPSC and clinicians in an empirical study. Following the development of a conceptual framework, the issues list was refined and the provisional UK-PSC-QoL constructed. Stage 2 pre-testing involved exploring item acceptability, comprehension, relevance, redundancy, and response distributions with UK PwPSC grouped in an expanded eight categories; the two least severe Stage 1 categories were each subdivided into mild and moderate-severe. Results: Stage 1 identified 396 QoL issues, explored with 28 PwPSC and 11 clinicians. Following issue reduction, 83 items were constructed in the six domains of the UK-PSC-QoL, including a separate six-item module for PwPSC with a stoma. Stage 2 pre-testing was conducted with 60 PwPSC who had comments about the wording of 54 items, mostly due to item ambiguity/clarity. One positive item had an unexpectedly positive skew. Fifty-five items are recommended for retention, 22 items modified to address problematic phrasing, six items deleted, and one new item added. Conclusion: The revised measure comprises 66 items in five domains, complemented by a six-item stoma module and an eight-item experience of care and treatment module. The measure remains provisional and recommended modifications require further testing in larger samples of PwPSC.
Background:
The use of observational measures to assess palliative care patients’ level of consciousness may improve patient care and comfort. However, there is limited knowledge regarding the ...validity and reliability of these measures in palliative care settings.
Aim:
To identify and evaluate the psychometric performance of observational level of consciousness measures used in palliative care.
Design:
Systematic review; PROSPERO registration: CRD42017073080.
Data sources:
We searched six databases until November 2018, using search terms combining subject headings and free-text terms. Psychometric performance for each identified tool was appraised independently by two reviewers following established criteria for developing and evaluating health outcome measures.
Results:
We found 35 different levels of consciousness tools used in 65 studies. Only seven studies reported information about psychometric performance of just eight tools. All other studies used either ad hoc measures for which no formal validation had been undertaken (n = 21) or established tools mainly developed and validated in non-palliative care settings (n = 37). The Consciousness Scale for Palliative Care and a modified version of the Richmond Agitation–Sedation Scale received the highest ratings in our appraisal, but, since psychometric evidence was limited, no tool could be assessed for all psychometric properties.
Conclusion:
An increasing number of studies in palliative care are using observational measures of level of consciousness. However, only a few of these tools have been tested for their psychometric performance in that context. Future research in this area should validate and/or refine the existing measures, rather than developing new tools.
General principles of care Working with people with a learning disability and behaviour that challenges, and with their families and carers When providing support and interventions: -Take into ...account the severity of people's learning disability and their developmental stage, any communication difficulties, and physical or mental health problems -Aim to provide support and interventions in the least restrictive setting (such as the person's home or in other places where the person regularly spends time) -Aim to prevent, reduce, or stop the development of future episodes of behaviour that challenges -Offer support and interventions respectfully and aim to improve quality of life -Ensure that the focus is on improving people's support and increasing their skills rather than changing people -Ensure that people know whom to contact if they are worried about care or interventions, including their right to a second opinion -Offer independent advocacy to the person and to family members or carers.
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