Pelvic vein incompetence and chronic pelvic pain: a case-control study.

OBJECTIVE: To investigate the association between chronic pelvic pain (CPP) and pelvic vein incompetence (PVI) or pelvic varices. DESIGN: Case-control study. SETTING: Gynaecology and vascular surgery services in two teaching hospitals in north-west England. SAMPLE: A total of 328 premenopausal women (aged 18-54 years), comprising 164 women with CPP and 164 matched controls with no history of CPP. METHODS: Symptom and quality-of-life questionnaires and transvaginal duplex ultrasound for PVI and pelvic varices. MAIN OUTCOME MEASURES: Venous reflux of >0.7 s in the ovarian or internal iliac veins (primary outcome) and presence of pelvic varices (secondary outcome). Statistical analysis compared the prevalence of PVI between women with and without CPP using the two-sided chi-square test. Logistic regression was used to compare the odds of having PVI and pelvic varices between women with and without CPP. RESULTS: Pelvic vein incompetence was found on transvaginal duplex ultrasound in 101/162 (62%) women with CPP, compared with 30/164 (19%) asymptomatic controls (OR 6.79, 95% CI 4.11-11.47, p < 0.001). Forty-three of 164 (27%) women with CPP had pelvic varices compared with three of 164 (2%) asymptomatic women (OR 18.9, 95% CI 5.73-62.7, p < 0.001). CONCLUSIONS: There was a significant association between PVI, as detected by transvaginal duplex imaging, and CPP. Pelvic varices were strongly associated with CPP and were infrequently seen in control patients. These results justify further evaluation of PVI and its treatment in well-designed research.

Transvenous occlusion of incompetent pelvic veins to treat chronic pelvic pain in women: A randomised controlled trial.

OBJECTIVE: To investigate the effectiveness of transvenous occlusion of incompetent pelvic veins in women presenting with chronic pelvic pain (CPP) in improving symptoms and quality of life. DESIGN: Patient-blinded randomised controlled trial with objective outcome measures. Results were analysed on an intention-to-treat basis. SETTING: Gynaecology and Vascular Surgery Services of two teaching hospitals in northwest England. POPULATION: Sixty women aged 18-54 years presenting with CPP after exclusion of other pathology, and who were found to have pelvic vein incompetence. METHODS: Participants were randomised and assigned to contrast venography alone or contrast venography plus transvenous occlusion of the incompetent pelvic veins. MAIN OUTCOME MEASURE: The primary outcome was change in pain score measured using the short-form McGill Pain Score (SF-MPQ) and the Visual Analogue Score (VAS) recorded at 12 months post-randomisation. Secondary outcomes included quality of life using the EQ-5D instrument, symptomatic improvement and procedure-related complications. RESULTS: Sixty participants were randomised to transvenous occlusion of incompetent pelvic veins or venography only. At 12 months, median pain scored 2 (3-10) in the intervention group versus 9 (5-22) in controls (p = 0.016). Pain on the VAS scored 15 (0-3) versus 53 (20-71), respectively (p = 0.002). Median EQ-5D improved after intervention from 0.79 (0.74-0.84) to 0.84 (0.79-1.00; p = 0.008) over 12 months. No major complications were reported. CONCLUSION: Transvenous occlusion of pelvic vein incompetence reduced pain scores, improved quality of life and diminished symptom burden with no major reported complications. TRIAL REGISTRATION: ISRCTN 15091500.

"Body as a Machine": How Adolescents With Sickle Cell Disease Construct Their Fatigue Experiences.

Research exploring illness experiences of young people with sickle cell disease (SCD) has, to date, ignored fatigue, despite the distinctive anemic nature of SCD. To examine adolescents with SCD fatigue experiences, we conducted narrative and picture-elicitation interviews with 24 adolescents in Ghana. A grounded theory, "body as a machine," was constructed from the narratives. Fatigue represented the most restrictive and disruptive aspect of growing up with SCD. Its meaning and significance laid in what it symbolized. Fatigue represented a socially undesirable feature that was stigmatizing, due to the expectations of high physicality in adolescence. Fatigue was therefore a major threat to "normalcy." The social significance of the physical body and its capacities shaped the adolescents' fatigue experiences. Managing fatigue to construct/maintain socially acceptable identities dominated the adolescents' lives. Consequently, there is a need for a recognition of the significance of fatigue to adequately support young people growing up with SCD.

Introducing a model for emotional distress in respiratory disease: A systematic review and synthesis of symptom management models.

AIM: To undertake a theoretical systematic review to develop a conceptual model of illness-related emotional distress in the context of symptom management in chronic respiratory disease. DESIGN: We performed a systematic search to identify conceptual models. DATA SOURCES: Electronic databases MEDLINE, CINAHL, EMBASE and PsycINFO were searched and papers included from inception of the search term until June 2017. REVIEW METHODS: The review was conducted following Pound and Campbell's and Turner's theory synthesis. Conceptual models were appraised using Kaplan's criteria. Models were excluded if they referred to a specific condition and/or lacked clarity. RESULTS: This synthesis, which includes five models and additional evidence, yielded a new conceptual model describing the processes of regulation and symptom self-management in chronic respiratory disease. Identified sources of illness-related emotional distress are new or increased symptoms, additional treatment, new restrictions in performance of daily life roles and increased unpredictability. People goals and self-efficacy were identified as further drivers of symptom self-management. The regulation process is embedded in contextual factors. CONCLUSION: Theory synthesis provided transparent guidance in developing a model to understand of the factors driving self-management decisions. Therefore, the model has the potential to guide development of interventions that support symptom self-management in chronic respiratory disease. IMPACT: This newly presented conceptual model of illness-related emotional distress provides an understanding of the factors that drive self-management decisions when peoples experience new or increased symptoms. Such understanding is critical for nursing practice to developing appropriate interventions, especially in support of people decision-making.

"Thrust out of normality"-How adults living with cystic fibrosis experience pulmonary exacerbations: A qualitative study.

AIM AND OBJECTIVES: To explore the experience of pulmonary exacerbation from the perspective of adults with cystic fibrosis. BACKGROUND: While management of pulmonary exacerbations is a pillar of cystic fibrosis care, little is known of patients' perspectives. Understanding the patient's experience is essential for developing and evaluating interventions in support of patient self-management. DESIGN: Qualitative study with longitudinal study in a subsample. METHODS: The study took place from 2015-2016 in a university hospital. Eighteen patients with cystic fibrosis were included who were ≥18 years of age and had no solid organ transplant. Patients' experiences were explored through semistructured interviews and analysed using framework analysis. They each participated in one interview, with a subsample (N = 7) being interviewed twice during and once after antibiotic therapy. RESULTS: Patients (11 men and 7 women; median age 29.5 years, range 19-55 years; median FEV1 45%, range FEV1 23%-105%) experienced pulmonary exacerbations as disruptions of their normality, which led to a substantial increase in their emotional distress. Exacerbations represented a period of threat and domination by CF; that is, symptoms and treatment consumed energy, restricted physical activity and daily life roles. "Noting change," "waiting until antibiotics help," "returning to normality" and "establishing a new normality" characterised their descriptions of the pulmonary exacerbation trajectory. Emotional distress was the major driver for patients' self-management, and personal goals and illness beliefs influenced also patients' self-management decisions. CONCLUSION: The experienced degree and source of emotional distress are drivers for self-management decisions in patients with cystic fibrosis who experience a pulmonary exacerbation. RELEVANCE TO CLINICAL PRACTICE: Our data provide new understanding that will be essential to informing clinical practice, future patient-reported outcomes measures and intervention development.

Principles for discharging patients from acute care: a scoping review of policy.

BACKGROUND: Acute medicine units (AMUs) are part of acute hospital care, where length of patient stay is short and turnover is high. Expediting patient discharge safely is a major function of AMUs, which need up-to-date bespoke guidance. AIM: To carry out a scoping review of UK discharge policy to critically consider, compare and contrast the relevant discharge policies and guidance that underpin the assessment of acute patient discharge. OBJECTIVES: To inform the production of bespoke patient discharge guidance for AMUs. Design and stages: Identify the review questions; identify relevant studies; select the studies; chart the data; collate, summarise and report the results. FINDINGS: 28 patient discharge policy guidelines were identified that had no specific guidance for patient discharge from AMUs. New bespoke principles for AMUs were created through a pragmatic interpretation of current relevant policies. CONCLUSION: There is a gap in specific patient discharge guidance for AMUs. New guidance should contribute in practice to improve patient discharge.

Referral and management options for patients with chronic kidney disease: perspectives of patients, generalists and specialists.

BACKGROUND: Chronic Kidney Disease (CKD) is increasing in prevalence and significance as a global public health issue. Appropriate management of CKD stages 3-4 in either generalist or specialist care is essential in order to slow disease progression. As various consulting options between services may be used, it is important to understand how patients and practitioners view these options. OBJECTIVE: To elicit patient and practitioner views and preferences on the acceptability and appropriateness of referral practices and consulting options for CKD stage 3-4. DESIGN: A mixed methods approach involving a semi-structured interview and structured rating exercise administered by telephone. SETTING & PARTICIPANTS: Adult (18+) patients with CKD stage 3-4 were recruited via their General Practitioner (GP). Practitioners were recruited from both general and specialist services. RESULTS: Sixteen patients and twenty-two practitioners participated in the study between July and September, 2011. Both patients and practitioners preferred 'GP with access to a specialist' and least preferred 'Specialist Review'. Computer review and telephone review were acceptable to participants under certain conditions. Practitioners favoured generalist management of patients with CKD 3. Specialists recommended active discharge of patients with stabilised stage 4 back to generalist care. Both generalists and specialists strongly supported sharing patients' medical records via electronic consultation systems. CONCLUSION: Participants tended to prefer the current model of CKD management. Suggested improvements included; increasing the involvement of patients in referral and discharge decisions; improving the adequacy of information given to specialists on referral and encouraging further use of clinical guidelines in practice.

Perceptions, experiences and needs of patients with idiopathic pulmonary fibrosis.

AIMS: To understand the perceptions, needs and experiences of patients with Idiopathic Pulmonary Fibrosis. BACKGROUND: Idiopathic pulmonary fibrosis is a progressive interstitial lung disease, with a mean life expectancy similar to some forms of cancer of 2-4 years from diagnosis. Unlike the cancer literature, which is rich with studies exploring the needs of their disease group, few publications exist on patient needs with this severe fibrotic lung disease. DESIGN: A Qualitative study which took place between 2007-2012. METHODS: Seventeen patients with a multidisciplinary team confirmed diagnosis of Idiopathic Pulmonary Fibrosis, with moderate to advanced disease severity and six of their informal carers were interviewed. An interview topic guide was developed by the researchers and service user group. The interviews were audio-recorded, semi-structured and took place at a regional respiratory and lung transplant centre in North West England. Interviews were transcribed verbatim and data analysed using Framework Analysis. FINDINGS: Three main themes were identified: 'Struggling to get a diagnosis'; 'Loss of the life I previously had'; and 'Living with Idiopathic Pulmonary Fibrosis'. Patients reported struggling to get a diagnosis and coping with a life-limiting, rapidly progressive illness with no good treatment and few support structures. CONCLUSIONS: There is an urgent need for a better understanding of the difficulties faced by people with Idiopathic Pulmonary Fibrosis and their carers. This can be used to develop better supportive care in the United Kingdom and ultimately improve the quality of life of these patients.

Exploring the Impact of Bereavement During the COVID-19 Pandemic on Children and Young People: A Scoping Review.

Experiencing bereavement as a child or young person (CYP) can have long-lasting effects. The societal and environmental burdens of the SARS-CoV-2 pandemic exacerbated the experience of loss and grief for many CYP, who were unable to access their usual the support networks. However, it is still unclear what is currently known and not known about the experiences of CYP bereaved during the SARS-CoV-2 pandemic. This review used the framework of Arksey and O'Malley and included five stages: (1) identifying the research question; (2) identifying relevant studies; (3) study selection; (4) charting the data; (5) collating, summarizing, and reporting the results. The methodological quality of the included studies was also assessed using the Critical Appraisal Skills Programme tool. The PRISMA framework was used for reporting the results. The electronic databases Medline, PsychINFO, CINAHL, and PubMed were searched for relevant articles. A total of three papers meeting the inclusion criteria were included in this review and two main themes identified: (1) support (which included sub-themes; social isolation and the impact on support; support from family and friends; wider support networks); (2) Emotional impact of bereavement during a pandemic. Access to support networks is crucial for CYP to understand and process their emotions relating to their bereavement experience. The pandemic meant that many usual support networks such as family and friends were inaccessible to CYP, who struggled to deal with their experience of grief during this time. Schools are a valuable support mechanism and can help CYP understand their emotions through open discussions about their bereavement. The limited empirical evidence currently available in this area of research demonstrates an important need to further understanding of the long-term impacts of dealing with pandemic-related loss in childhood.

Understanding the experience of patients with chronic obstructive pulmonary disease who access specialist palliative care: a qualitative study.

BACKGROUND: Palliative care for people with life-limiting non-malignant disease is increasingly prioritised. People with end-stage chronic obstructive pulmonary disease are among a key group of non-cancer patients likely to benefit from specialist palliative care, but it remains uncertain whether the needs of this group are met by existing services. AIM: To evaluate the experiences of patients with chronic obstructive pulmonary disease who accessed specialist palliative care. DESIGN: Data from semi-structured interviews were analysed using a hermeneutic phenomenological approach. SETTING/PARTICIPANTS: Eight patients accessing specialist palliative care within one city in North West England. RESULTS: Perceived benefits of specialist palliative care included reduced frequency of hospital admission, improved physical and psychological symptoms, reduced social isolation and a broadened physical environment. Participants were mainly aware of their poor prognosis, but discussion of referral to palliative care sometimes caused distress owing to the historical associations between dying and hospice care. Following engagement with services, participants' perceptions changed: palliative care was associated with social inclusion and opportunities to engage in reciprocal and altruistic social action. Negative associations were replaced by uncertainty and anxiety about the prospect of discharge. CONCLUSIONS: Much within existing services works well for people with chronic obstructive pulmonary disease, but opportunities to enhance palliative care for this underserved group remain. Future research might focus on prospectively evaluating the impact of key components of palliative care on core patient-centred outcomes. Additionally, work must be done to raise awareness of the benefits of specialist palliative care for non-cancer patients, as negative associations can form a barrier to access.

Parents' emotional and social experiences of caring for a child through cleft treatment.

Little is known about the experiences of parents caring for a child through long-term treatment for cleft lip and/or cleft palate. We conducted in-depth interviews with 35 parents with children between the ages of 20 weeks and 21 years to explore experiences across the treatment program. We analyzed the data using a constructivist grounded theory approach and present in detail in this article one subcategory from the analysis: managing emotions. Throughout childhood and adolescence, parents experienced conflicting emotions about their child's impairment, uncertainty about cleft treatment, and stigmatizing attitudes. Although parents attempted to manage emotional tensions by pursuing cleft treatments, the interventions could themselves be a source of conflict for them. We suggest that routine assessment of parents' emotional and social well-being should be included in cleft treatment programs, and access to psychosocial support made available.

National survey of the current provision of specialist palliative care services for patients with end-stage renal disease.

BACKGROUND: Patients with end-stage renal disease (ESRD) experience a significant symptom burden and have complex needs. However, involvement of specialist palliative care (SPC) services with these patients has previously been shown to be limited. This study assesses the current provision of and access to SPC services for ESRD patients in the UK and considers how the provision has evolved over recent years. METHODS: A questionnaire was sent to the lead clinician for all UK adult hospital, hospice and community SPC services, identified from the Hospice and Palliative Care Directory 2008. Non-responders were mailed again after 5 weeks. Descriptive statistics and qualitative thematic analysis were performed. RESULTS: Three hundred and eighteen of 611 (52%) questionnaires were returned. Ninety-six per cent stated that SPC services have a role in caring for patients with ESRD. Two hundred and eighty-one of 318 (88%) accepted referrals, and 185 of 281 (66%) reported that 'none or few were referred'. Only 7% and 17% of respondents used specific ESRD referral and treatment guidelines, respectively; whereas 79% used the Liverpool Care Pathway for the Dying Patient. Seven per cent undertook joint renal and SPC multi-disciplinary team (MDT) meetings, and 3% held joint out-patient clinics. Forty percent of respondents proposed initiatives to improve palliative care for ESRD patients, with mutual education and collaborative working being key themes for improvement. CONCLUSIONS: The majority of SPC services accept ESRD patients, but limited numbers are referred. Respondents indicated that this barrier could be addressed by closer collaboration and better communication and education between renal and SPC services. Other initiatives to enable delivery of SPC to increased numbers of ESRD patients include the use of specific referral and clinical care guidelines and expansion of joint MDT meetings and out-patient clinics.

Shared decision making or paternalism in nursing consultations? A qualitative study of primary care asthma nurses' views on sharing decisions with patients regarding inhaler device selection.

BACKGROUND: Although patients with asthma would like more involvement in the decision-making process, and UK government policy concerning chronic conditions supports shared decision making, it is not widely used in practice. OBJECTIVE: To investigate how nurses approach decision making in relation to inhaler choice and long-term inhaler use within a routine asthma consultation and to better understand the barriers and facilitators to shared decision making in practice. SETTING AND PARTICIPANTS: Semi-structured interviews were conducted with post-registration, qualified nurses who routinely undertook asthma consultations and were registered on a respiratory course. Interviews were recorded, transcribed and analysed using the Framework approach. RESULTS: Twenty participants were interviewed. Despite holding positive views about shared decision making, limited shared decision making was reported. Opportunities for patients to share decisions were only offered in relation to inhaler device, which were based on the nurse's pre-selected recommendations. Giving patients this 'choice' was seen as key to improving adherence. DISCUSSION: There is a discrepancy between nurses' understanding of shared decision making and the depictions of shared decision making presented in the academic literature and NHS policy. In this study, shared decision making was used as a tool to support the nurses' agenda, rather than as a natural expression of equality between the nurse and patient. CONCLUSION: There is a misalignment between the goals of practice nurses and the rhetoric regarding patient empowerment. Shared decision making may therefore only be embraced if it improves patient outcomes. This study indicates attitudinal shifts and improvements in knowledge of 'shared decision-making' are needed if policy dictates are to be realised.

Exploring differences in referrals to a hospice at home service in two socio-economically distinct areas of Manchester, UK.

In order to provide equitable access to hospice at home palliative care services, it is important to identify the socio-economic factors associated with poorer access. In this population-based study we aimed to test the inverse care law by exploring how socio-economic status and other key demographic indicators were associated with referral rates in two distinct areas (Salford and Trafford) served by the same service. Secondary data from the UK National Census 2001, North West Cancer Intelligence Service (2004) and hospice at home service referral data (2004-06) was collated for both areas. Descriptive analysis profiled electoral ward characteristics whilst simple correlations and regression modelling estimated associations with referral rates. Referral rates were lower and cancer mortality higher in the most deprived areas (Salford). Referral rates were significantly associated with deprivation, particularly multiple deprivation, but not significantly associated with cancer mortality (service model and resources available were held constant). At the population level, the socio-economic characteristics of those referred to hospice at home rather than service provision strongly predicted referral rates. This has implications for the allocation and targeting of resources and contributes important findings to future work exploring equitable access at organizational and professional levels.

Recommendations for Developing Support Tools With People Suffering From Chronic Obstructive Pulmonary Disease: Co-Design and Pilot Testing of a Mobile Health Prototype.

BACKGROUND: Gaps exist between developers, commissioners, and end users in terms of the perceived desirability of different features and functionalities of mobile apps. OBJECTIVE: The objective of this study was to co-design a prototype mobile app for people with chronic obstructive pulmonary disease (COPD). We present lessons learned and recommendations from working on a large project with various stakeholders to develop a mobile app for patients with COPD. METHODS: We adopted a user-centered, participatory approach to app development. Following a series of focus groups and interviews to capture requirements, we developed a prototype app designed to enable daily symptom recording (experience sampling). The prototype was tested in a usability study applying the think aloud protocol with people with COPD. It was then released via the Android app store, and experience sampling data and event data were captured to gather further usability data. RESULTS: A total of 5 people with COPD participated in the pilot study. Identified themes include familiarity with technology, appropriate levels for feeding back information, and usability issues such as manual dexterity. Moreover, 37 participants used the app over a 4-month period (median age 47 years). The symptoms most correlated to perceived well-being were tiredness (r=0.61; P<.001) and breathlessness (r=0.59; P<.001). CONCLUSIONS: Design implications for COPD apps include the need for clearly labeled features (rather than relying on colors or symbols that require experience using smartphones), providing weather information, and using the same terminology as health care professionals (rather than simply lay terms). Target users, researchers, and developers should be involved at every stage of app development, using an iterative approach to build a prototype app, which should then be tested in controlled settings as well as in the wild (ie, when deployed and used in real-world settings) over longer periods.

A review of the information and support needs of family carers of patients with chronic obstructive pulmonary disease.

AIMS AND OBJECTIVES: The objectives of this narrative review were to identify: (1) The information and support needs of carers of family members with chronic obstructive pulmonary disease; (2) appropriate interventions to support carers in their caregiving role; (3) information on carers' needs as reported in studies of patients living with COPD in the community. BACKGROUND: Chronic obstructive pulmonary disease is a major health problem in the UK resulting in significant burden for patients, families and the health service. Current National Health Service policies emphasise, where medically appropriate, early discharge for acute exacerbations, hospital-at-home care and other models of community care to prevent or reduce re-hospitalisations of people with chronic conditions. Understanding carers' needs is important if health care professionals are to support carers in their caregiving role. DESIGN: A narrative literature review. METHODS: Thirty five papers were reviewed after searching electronic databases. RESULTS: Few studies were identified which addressed, even peripherally, carers' needs for information and support, and no studies were found which described and evaluated interventions designed to enhance caregiving capacity. Several studies of hospital-at-home/early discharge, self care and home management programmes were identified which included some information on patients' living arrangements or marital status. However, there was little or no detail reported on the needs of, and in many cases, even the presence of a family carer. CONCLUSIONS: This review highlights the dearth of information on the needs of carers of chronic obstructive pulmonary disease patients and the need for future research. RELEVANCE TO CLINICAL PRACTICE: There is little research based knowledge of the needs of carers of chronic obstructive pulmonary disease patients and interventions to assist them in providing care. This knowledge is critical to ensure that carers receive the information they need to carry out this role while maintaining their own physical and emotional health.

Judgements about fellow professionals and the management of patients receiving palliative care in primary care: a qualitative study.

BACKGROUND: Policies emphasise the importance of collaborative working in community palliative care. Collaborations are generally formed through formal and informal referral processes, but little is known about what influences professionals' decisions to refer to such services. AIM: To explore the influences on referrals within general and specialist community palliative care services. DESIGN OF STUDY: Qualitative, multiple-case study. SETTING: Three primary care trusts in the north-west of England. METHOD: Multiple data collection methods were employed, including documentary analysis, observation of referral team meetings and interviews. This paper primarily reports data from interviews with 47 health professionals, including GPs, district nurses, and specialist palliative care professionals. RESULTS: Judgements -- positive and negative -- about aspects of fellow professionals' performances appeared to influence referral decisions and ongoing collaboration and care. Attributes upon which these judgements were based included professional responsiveness and communication, respect, working and workload management practices, perceived expertise, and notions of elite practice. The effects of such judgements on referral and healthcare practices were altered by professional "game playing" to achieve professionals' desired outcomes. CONCLUSION: Palliative care policies and protocols need to take account of these complex and subtle influences on referrals and collaboration. In particular, teamwork and partnership are encouraged within palliative care work, but critical judgements indicate that such partnerships may be difficult or fragile. It is likely that such judgemental attitudes and practices affect many aspects of primary care, not just palliative care.

Adolescents' experiences of living with sickle cell disease: An integrative narrative review of the literature.

BACKGROUND: Sickle Cell Disease is the commonest monogenic haemoglobinopathy worldwide. Living with a long-term condition such as sickle cell disease during adolescence constitutes a significant challenge for the key stakeholders due to the combined effects of chronic illness and adolescent development. For adolescents with sickle cell disease to be cared for and supported appropriately and effectively, it is crucial that health professionals have a comprehensive knowledge and understanding of how adolescents experience living with the condition. While there is developing literature about how adolescent's experience sickle cell disease, this body of research has not been critically reviewed and synthesised. OBJECTIVE: To identify, critically appraise and synthesise primary research exploring adolescents' experiences of living with sickle cell disease to make recommendations for practice and research. DESIGN: Integrative narrative review. DATA SOURCES: A systematic search of 10 electronic databases and key journals was conducted to identify studies from the inception of databases to September 2016. REVIEW METHOD: Inclusion criteria: adolescents with sickle cell disease aged 12-19 years, primary data on adolescents' own perspectives, and published in English. Data were extracted on study contexts, methodology/design, theoretical constructs, participants, and key findings. Findings from included studies were synthesised using the integrative narrative approach. Additionally, the methodological quality of studies was assessed using the Hawker et al. (2002) appraisal checklist. RESULTS: 683 studies were identified, of which 40 fulfilled the inclusion criteria. Nine broad themes emerged: knowledge and understanding of the condition, symptom experiences, self-management, attitude to treatment, healthcare experiences, social relationships, difference and striving for normality, school experiences, and emotional well-being and coping. Majority of the studies were of moderate quality methodologically. Quality assessment demonstrated a high risk of bias in three studies. CONCLUSIONS: Sickle cell disease impacts on multiple facets of an adolescent's life. While there are similarities in the experience of living with sickle cell disease and living with other chronic illnesses, there are essential differences in relationship dynamics and healthcare experience. The adolescents expressed less confidence in generic healthcare providers. The review highlights areas relating to symptom management and health service provision that has been under-researched and need further exploration to understand adolescents' experiences and their support needs fully. Nursing care and research should focus more on adolescents' developmental wellbeing, promote peer support network among adolescents with the condition and with adolescents with other chronic illnesses and collaborate with adolescents to ensure service development are developmentally and culturally appropriate.

Patient-Reported Outcome Measures for Symptom Perception During a Cystic Fibrosis Exacerbation.

BACKGROUND: Symptom burden increases during pulmonary exacerbations of cystic fibrosis (CF), and patient-reported outcome measures (PROMs) are often used to evaluate symptoms as either primary or secondary outcomes. However, there is currently no guidance on the use of PROMs to assess symptom burden during pulmonary exacerbations. METHODS: A systematic literature search was conducted to identify PROMs measuring symptom experience, management, or influencing factors, which were developed for CF patients and had been used at least once during pulmonary exacerbations. The PROMs included were assessed for relevance and psychometrics, according to the criteria of the United States FDA guideline and the Consensus-Based Standards for the Selection of Health Measurement Instruments (COSMIN) checklist. RESULTS: Five PROMs were identified, all measuring symptom perception. The CF Respiratory Symptom Diary (CFRSD) and the Symptom Scoring System were developed to assess symptom severity during pulmonary exacerbations. Of the other 3, which also included symptom scores of 2 quality of life measures, one assessed symptom severity exclusively, and 2 measured symptom severity in addition to other dimensions (such as symptom distress). All 5 instruments measured respiratory symptoms. Other relevant symptoms, such as energy and emotions, were covered by 4 instruments; pain and gastrointestinal symptoms were covered by 2 measures. All of the instruments demonstrated good internal consistency and sensitivity to change over a period up to 4 weeks. The symptom scores of the 2 quality of life measures with longer recall periods are not suitable for measuring assessed changes in a period of < 2 weeks. Criterion validity for gastrointestinal subscores has not been established. Discriminant validity was established in all of the instruments reviewed except for the Symptom Score System. CONCLUSIONS: Of the current PROMs used during CF pulmonary exacerbations, only 2 have been developed for this purpose, and only the CFRSD fulfilled all FDA guideline criteria. To date, there is no instrument that assesses exacerbation-specific symptom distress.