Developmentally appropriate transitional care during the Covid-19 pandemic for young people with juvenile-onset rheumatic and musculoskeletal diseases: the rationale for a position statement

From Springer Nature via Jisc Publications RouterHistory: received 2021-03-26, accepted 2021-06-06, registration 2021-07-31, pub-electronic 2021-08-25, online 2021-08-25, collection 2021-12Publication status: PublishedAbstract: Background: The importance of developmentally appropriate transitional care in young people with juvenile-onset rheumatic and musculoskeletal disease is well recognised. The Paediatric Rheumatology European Society (PReS) / European League Against Rheumatism (EULAR) Taskforce has developed international recommendations and standards for transitional care and a growing evidence base supports the positive benefits of such care. However, there is also evidence that universal implementation has yet to be realised. In 2020, against this background the COVID-19 pandemic arrived with significant impact on all our lives, young and old, patient, public and professional alike. The unfortunate reality of the pandemic with potential for unfavourable outcomes on healthcare provision during transition was acknowledged by the PReS working groups in a position statement to support healthcare professionals, young people and their caregivers. Aim: The aim of this review is to present the literature which provides the rationale for the recommendations in the PReS Position Statement. Summary: The following areas are specifically addressed: the prime importance of care coordination; the impact of the pandemic on the various aspects of the transition process; the importance of ensuring continuity of medication supply; the pros and cons of telemedicine with young people; ensuring meaningful involvement of young people in service development and the importance of core adolescent health practices such as routine developmental assessment psychosocial screening and appropriate parental involvement during transitional care

Experiences of employment amongst young people with juvenile idiopathic arthritis: a qualitative study

Purpose: This study explored expectations and experiences of employment amongst young people with JIAjuvenile idiopathic arthritis, and the role of health professionals in promoting positive employment outcomes. Methods: Semi-structured interviews (n=13) and three focus groups (n=9,n=4,n=3) were conducted with young people (16-25y) and adults (26-31y) with juvenile idiopathic arthritis JIA and semi-structured interviews (n=9) were conducted with health professionals. Transcripts were analysed thematically. Results: Young people with juvenile idiopathic arthritisJIA have concerns about employers’ attitudes towards employees with long-term health conditions and lack knowledge of antidiscrimination legislation. Young people not in education, employment or training identify arthritisJIA as a key barrier. Challenges associated with JIA arthritis (e.g. pain, psychological distress) may not be visible to employers. Decisions about disclosing arthritisJIA are challenging and cause anxiety. Young people associate good disease management and access to flexible and convenient care with their capacity to succeed in employment. Psycho-social and vocational interventions have benefited some young people, but are not routinely available. Conclusions: Low expectations of employers may affect young people’s decisions about disclosure and seeking appropriate support in the work place. Health professionals can equip young people with knowledge and skills to negotiate appropriate support, through signposting to Young people, employment and arthritis. 2 anti-discrimination information and offering practice of transferable skills such as disclosure in consultations

Radical surgery versus organ preservation via short-course radiotherapy followed by transanal endoscopic microsurgery for early-stage rectal cancer (TREC): a randomised, open-label feasibility study

Background: Radical surgery via total mesorectal excision might not be the optimal first-line treatment for early-stage rectal cancer. An organ-preserving strategy with selective total mesorectal excision could reduce the adverse effects of treatment without substantially compromising oncological outcomes. We investigated the feasibility of recruiting patients to a randomised trial comparing an organ-preserving strategy with total mesorectal excision. Methods: TREC was a randomised, open-label feasibility study done at 21 tertiary referral centres in the UK. Eligible participants were aged 18 years or older with rectal adenocarcinoma, staged T2 or lower, with a maximum diameter of 30 mm or less; patients with lymph node involvement or metastases were excluded. Patients were randomly allocated (1:1) by use of a computer-based randomisation service to undergo organ preservation with short-course radiotherapy followed by transanal endoscopic microsurgery after 8–10 weeks, or total mesorectal excision. Where the transanal endoscopic microsurgery specimen showed histopathological features associated with an increased risk of local recurrence, patients were considered for planned early conversion to total mesorectal excision. A non-randomised prospective registry captured patients for whom randomisation was considered inappropriate, because of a strong clinical indication for one treatment group. The primary endpoint was cumulative randomisation at 12, 18, and 24 months. Secondary outcomes evaluated safety, efficacy, and health-related quality of life assessed with the European Organisation for Research and Treatment of Cancer (EORTC) QLQ C30 and CR29 in the intention-to-treat population. This trial is registered with the ISRCTN Registry, ISRCTN14422743. Findings: Between Feb 22, 2012, and Dec 19, 2014, 55 patients were randomly assigned at 15 sites; 27 to organ preservation and 28 to radical surgery. Cumulatively, 18 patients had been randomly assigned at 12 months, 31 at 18 months, and 39 at 24 months. No patients died within 30 days of initial treatment, but one patient randomly assigned to organ preservation died within 6 months following conversion to total mesorectal excision with anastomotic leakage. Eight (30%) of 27 patients randomly assigned to organ preservation were converted to total mesorectal excision. Serious adverse events were reported in four (15%) of 27 patients randomly assigned to organ preservation versus 11 (39%) of 28 randomly assigned to total mesorectal excision (p=0·04, χ2 test). Serious adverse events associated with organ preservation were most commonly due to rectal bleeding or pain following transanal endoscopic microsurgery (reported in three cases). Radical total mesorectal excision was associated with medical and surgical complications including anastomotic leakage (two patients), kidney injury (two patients), cardiac arrest (one patient), and pneumonia (two patients). Histopathological features that would be considered to be associated with increased risk of tumour recurrence if observed after transanal endoscopic microsurgery alone were present in 16 (59%) of 27 patients randomly assigned to organ preservation, versus 24 (86%) of 28 randomly assigned to total mesorectal excision (p=0·03, χ2 test). Eight (30%) of 27 patients assigned to organ preservation achieved a complete response to radiotherapy. Patients who were randomly assigned to organ preservation showed improvements in patient-reported bowel toxicities and quality of life and function scores in multiple items compared to those who were randomly assigned to total mesorectal excision, which were sustained over 36 months’ follow-up. The non-randomised registry comprised 61 patients who underwent organ preservation and seven who underwent radical surgery. Non-randomised patients who underwent organ preservation were older than randomised patients and more likely to have life-limiting comorbidities. Serious adverse events occurred in ten (16%) of 61 non-randomised patients who underwent organ preservation versus one (14%) of seven who underwent total mesorectal excision. 24 (39%) of 61 non-randomised patients who underwent organ preservation had high-risk histopathological features, while 25 (41%) of 61 achieved a complete response. Overall, organ preservation was achieved in 19 (70%) of 27 randomised patients and 56 (92%) of 61 non-randomised patients. Interpretation: Short-course radiotherapy followed by transanal endoscopic microsurgery achieves high levels of organ preservation, with relatively low morbidity and indications of improved quality of life. These data support the use of organ preservation for patients considered unsuitable for primary total mesorectal excision due to the short-term risks associated with this surgery, and support further evaluation of short-course radiotherapy to achieve organ preservation in patients considered fit for total mesorectal excision. Larger randomised studies, such as the ongoing STAR-TREC study, are needed to more precisely determine oncological outcomes following different organ preservation treatment schedules. Funding: Cancer Research UK

Universal newborn hearing screening and early identification of deafness: Parents' responses to knowing early and their expectations of child communication development

This article presents results from an interview study of 45 parents/caregivers (representing 27 families) whose infants were correctly identified as deaf during the first phase of the implementation of the national universal Newborn Hearing Screening Programme in England. Average age of children when parents were interviewed was 25 weeks. Two issues are explored: (a) how parents talk about significance of knowing early that their child is deaf and (b) parents ’ expectations of their child’s development in light of early identification. Although results demonstrate clear support from parents’ perspective of knowing early, they also identify the psycho-logical complexities of recognizing both the grief and reas-surance that early knowledge brings; the risks of early knowledge-inducing timetables of expectations that create distress when not met speedily; the extent to which parental models of the developmental advantages of early identifica-tion are underpinned by notions of normal speech and the possibility of being like hearing children; and the pervasive-ness of deficit and illness models associated with having identified deafness early. Implications for parental support and professional responses are also discussed. With the rapid expansion of Universal Newborn Hear-ing Screening (UNHS) programs across both the de-veloped and the developing world (Newborn Hearing Screening Conference, 2006