Structured nursing educational programs improve quality of life outcomes in patients with Cushing's syndrome

Impaired quality of life (QoL) has been reported in patients with Cushing’s syndrome even after biochemical cure, with worse QoL outcomes compared to patients with other pituitary adenomas [1]. A variety of physical, neuropsychosomatic, psychological, and sociodemographic factors have been found to influence QoL parameters in patients with Cushing’s syndrome [1, 2]. A systematic review by Andela et al. found that QoL outcomes such as physical functioning, bodily pain, and general health improved after surgical or pharmaceutical interventions in 6 of the 8 studies involving patients with Cushing’s syndrome

Glucocorticoid therapy for adrenal insufficiency: nonadherence, concerns and dissatisfaction with information

Objective: Appropriate self‐management of glucocorticoid therapy (GC) is crucial for patients with adrenal insufficiency (AI). We aimed to describe patients’ self‐reported nonadherence to GC, evaluate perceived doubts about need for GC, concerns about adverse effects, and dissatisfaction with information received about GC. Design: Cross‐sectional survey. Patients: Patients prescribed GC for AI (n = 81) from five European countries. Measurements: Online survey including the Medication Adherence Report Scale (MARS), Beliefs about Medicines Questionnaire© (BMQ Specific, adapted for AI) and Satisfaction with Information about Medicines Scale© (Prof Rob Horne; SIMS). Results: Most patients (85·2%) reported a degree of nonadherence to GC. The most frequent types of nonadherence concerned changing the timing of GC doses, for example taking a dose later in the day than advised (37·0%). Few patients doubted their personal need for daily GC, but most reported high concerns about GC including potential weight gain (50·6%), osteoporosis (53·6%) and the continuing risk of adrenal crisis (50·6%). Dissatisfaction with information about GC was frequent, with participants particularly dissatisfied with the amount of information they had received about potential problems with GC. People who expressed dissatisfaction with information about GC, and concerns about its adverse effects were also more likely to report nonadherence (P < 0·05). Conclusions: Nonadherence to treatment, concerns about potential adverse effects and dissatisfaction with the information provided about treatment were frequently reported by this European sample of AI patients. Many AI patients may need additional information about their GC and support to address concerns about GC and facilitate adherence

Quality of life for carers of people with type 2 diabetes: A literature review

This scoping literature review explores the impact of type 2 diabetes (T2D) in adults on carers' quality of life (QoL) and emphasises the importance of improving carer support in order to achieve integrated care. The review was conducted in February 2015 and included articles published in English in the past 10 years exploring carers' QoL. Five articles satisfied the inclusion criteria and were critically/thematically analysed. Evidence suggests that emotional wellbeing is the QoL domain that is most affected by T2D, with depression and anxiety being reported as the most common issues. Additional domains were social functioning and economic burden. Sociodemographic and cultural differences were identified as variables that can influence carers' QoL. This literature review suggests that T2D in adults has a significant impact on carers' QoL; however, there is limited evidence to demonstrate how healthcare professionals can support carers, so further research is needed in this area to improve the provision of integrated care

Factors Impacting Quality of Life in Patients With Klinefelter Syndrome: A Systematic Review with Narrative Synthesis and Meta-Analysis

Objective: To identify how Klinefelter’s Syndrome affects patients’ quality of life (QoL) and to determine which subsections of QoL are affected guided by the WHOQOL100-tool as an overarching framework for QoL. To determine the effects and impact KS has on patients’ QoL when compared to the QoL of healthy controls and general population. DESIGN - Systematic review of studies reporting QoL factors among patients with KS which included narrative synthesis and thematic analysis of 17 studies and a meta-analysis of intelligence quotient (IQ) completed in 7 studies. QoL factors were reviewed based on the parameters of the WHOQOL-100: physical health, psychological, level of independence, social relations, environment, and religion/spirituality/personal beliefs. DATA SOURCES - Medline, Cochrane, Embase, Psychinfo, CINAHL, BASE and grey search from the reference lists of key publications. Eligibility Criteria: RCT’s, Cohort studies, cross sectional studies and Epidemiology studies involving patients with KS and reporting on QoL parameters. Only human studies published in English were considered with no limits for publication date. Results: Out of all studies included (n=1266), (87.5%) had suggested KS negatively affected the outcomes measures tested, where recorded (91.1%) of studies had small/medium/large effect sizes (Cohen’s d). Narrative synthesis suggests all subgroups of QoL excluding ‘environment’ and ‘spirituality/faith/personal beliefs’ were negatively impacted for patients with KS, whilst meta-analysis showed statistical significance (P &amp;lt;0.00001) which identified patients with KS having lower full-scale IQ compared to healthy controls. Psychological parameters were the most affected in this patient group, showing that patients with KS experienced greater social anxiety, distress during social interactions, self-esteem, self-injuries behaviours and symptoms or traits related to Autism spectrum. Conclusions: This review identifies the significant evidence supporting that QoL is reduced in patients with KS. There is a large spectrum of symptoms and no standard phenotype for KS suggesting that multiple facets of QoL are negatively impacted in these patients due to the complex nature of KS and the severity of symptoms and phenotype associated with KS. PROSPERO REGISTRATION NUMBER - CRD4202017343</jats:p

Association between domains of quality of life and patients with klinefelter syndrome: a systematic review.

Klinefelter syndrome (KS) is the second-most prevalent chromosomal disorder in men, though late diagnosis is very common and 50-75% of men remain undiagnosed. Evidence suggests that men with KS have impaired Quality of Life (QoL) but research on how the diagnosis of KS is associated with different QoL domains and what factors influence patients' QoL is limited. This study aimed to provide a systematic review of the published evidence on factors that influence QoL in men with KS. DESIGN: Systematic review and meta-analysis with narrative synthesis. METHODS: Medline, Cochrane, Embase, Psychinfo, CINAHL, BASE and relevant publication reference lists were searched in January 2021. Eligible studies included RCTs, cohort studies, cross-sectional studies and epidemiology studies on KS and its effect on QoL and all domains of WHOQOL-100. Clinical studies with no date restriction published in English were included. RESULTS: Thematic analysis was completed on thirteen studies, with a meta-analysis of intelligence quotient (IQ) completed on seven studies. Twelve out of 13 studies suggested that KS negatively affected QoL outcomes and KS was associated with impairments in physical, psychological, level independence and social relationship domains of WHOQOL-100. Meta-analysis suggested men with KS have significantly lower full-scale Intelligence Quotient versus controls (P <0.00001). CONCLUSIONS: This is the first evidence synthesis of QoL in men with KS. Current evidence suggests that combined physical and psychological impairments affect men with KS who also experience impairments in relationships and independence in society. Further research is needed to identify factors that influence QoL in men with KS

Using the behaviour change wheel and person-based approach to develop a digital self-management intervention for patients with adrenal insufficiency: the Support AI study protocol

Introduction: Most patients with Adrenal insufficiency (AI) require lifelong glucocorticoid replacement. They need to increase glucocorticoids during physical illness or major stressful situations and require parenteral hydrocortisone in the event of an adrenal crisis. Patients with AI have impaired quality of life and high mortality; approximately 1 in 6-12 patients are hospitalised at least once/year from a potentially preventable adrenal crisis. Adoption of self-management behaviours are crucial; these include adherence to medication, following “sick day rules” and associated behaviours that aid prevention and treatment of adrenal crisis such as symptom monitoring, having extra tablets, carrying a medical-alert ID and injection kit, and self-injecting when necessary. Current patient education is ineffective at supporting self-management behaviour change or reducing adrenal crisis-related hospitalisations. This research study aims to gain an in-depth understanding of the barriers and enablers to self-management for patients with AI and to develop an evidence-based digital self-management behaviour change intervention. Methods: The study is conducted in accordance with the MRC Framework for developing complex interventions. Underpinned by the Behaviour Change Wheel (BCW), the Theoretical Domains Framework (TDF), and the Person-Based Approach, this research will be conducted in two phases: Phase 1 will involve a sequential qualitative/quantitative mixed-methods study involving focus group interviews followed by a cross-sectional survey with patients with AI recruited from patient advocacy groups and endocrine clinics in the UK. Phase 2 will develop the Support AI, a website-based digital behaviour change intervention (DBCI) informed by Phase 1 findings to support self-management for patients with AI. The most appropriate behaviour change techniques (BCTs) will be selected utilising a nominal group technique with an Expert Panel of 10-15 key stakeholders. The design of the Support AI website will be guided by the Person-Based Approach using an Agile iterative “think-aloud” technique with 12-15 participants over 3 usability testing iterations. Conclusion: A theory- and evidence-based digital behaviour change intervention will be developed which will be tested in a feasibility randomised trial following completion of this study. The projected benefit includes cost-effective health care service (reduced hospitalisations and demand for specialist services) and improved health outcomes and quality of life for patients with AI

ESE Clinical Practice Guideline on functioning and nonfunctioning pituitary adenomas in pregnancy

Pregnancies are rare in women with pituitary adenomas, which may relate to hormone excess from secretory subtypes such as prolactinomas or corticotroph adenomas. Decreased fertility may also result from pituitary hormone deficiencies due to compression of the gland by large tumours and/or surgical or radiation treatment of the lesion. Counselling premenopausal women with pituitary adenomas about their chance of conceiving spontaneously or with assisted reproductive technology, and the optimal pre-conception treatment, should start at the time of initial diagnosis. The normal physiological changes during pregnancy need to be considered when interpreting endocrine tests in women with pituitary adenomas. Dose adjustments in hormone substitution therapies may be needed across the trimesters. When medical therapy is used for pituitary hormone excess, consideration should be given to the known efficacy and safety data specific to pregnant women for each therapeutic option. In healthy women, pituitary gland size increases during pregnancy. Since some pituitary adenomas also enlarge during pregnancy, there is a risk of visual impairment, especially in women with macroadenomas or tumours near the optic chiasm. Pituitary apoplexy represents a rare acute complication of adenomas requiring surveillance, with surgical intervention needed in some cases. This guideline describes the choice and timing of diagnostic tests and treatments from the pre-conception stage until after delivery, taking into account adenoma size, location and endocrine activity. In most cases, pregnant women with pituitary adenomas should be managed by a multidisciplinary team in a centre specialised in the treatment of such tumours

Service evaluation suggests variation in clinical care provision in adults with congenital adrenal hyperplasia in the UK and Ireland

Background: Congenital adrenal hyperplasia (CAH) encompasses a rare group of autosomal recessive disorders, characterised by enzymatic defects in steroidogenesis. Heterogeneity in management practices has been observed internationally. The International Congenital Adrenal Hyperplasia registry (I‐CAH, https://sdmregistries.org/) was established to enable insights into CAH management and outcomes, yet its global adoption by endocrine centres remains unclear. Design: We sought (1) to assess current practices amongst clinicians managing patients with CAH in the United Kingdom and Ireland, with a focus on choice of glucocorticoid, monitoring practices and screening for associated co‐morbidities, and (2) to assess use of the I‐CAH registry. Measurements: We designed and distributed an anonymised online survey disseminated to members of the Society for Endocrinology and Irish Endocrine Society to capture management practices in the care of patients with CAH. Results: Marked variability was found in CAH management, with differences between general endocrinology and subspecialist settings, particularly in glucocorticoid use, biochemical monitoring and comorbidity screening, with significant disparities in reproductive health monitoring, notably in testicular adrenal rest tumours (TARTs) screening (p = .002), sperm banking (p = .0004) and partner testing for CAH (p < .0001). Adoption of the I‐CAH registry was universally low. Conclusions: Differences in current management of CAH continue to exist. It appears crucial to objectify if different approaches result in different long‐term outcomes. New studies such as CaHASE2, incorporating standardised minimum datasets including replacement therapies and monitoring strategies as well as longitudinal data collection, are now needed to define best‐practice and standardise care