When Women with Cystic Fibrosis Become Mothers: Psychosocial Impact and Adjustments

Advances in the treatment and life expectancy of cystic fibrosis (CF) patients mean that motherhood is now a realistic option for many women with CF. This qualitative study explored the psychosocial impact and adjustments made when women with CF become mothers. Women with CF () were recruited via an online forum and participated in semistructured telephone interviews about their experiences of becoming a mother. Transcriptions were analysed using Grounded Theory. Analysis revealed three core categories: (i) “Living with CF”: how becoming a mother impacted on health and treatment adherence, requiring a change in support from the CF team, (ii) “Becoming a Mother”: balancing issues common to new mothers with their CF, and (iii) “Pooling Personal Resources”: coping strategies in managing the dual demands of child and CF care. Participants experienced a variety of complex psychosocial processes. Most participants acknowledged an initial negative impact on CF care; however over time they reported successful adaptation to managing dual commitments and that adherence and motivation to stay well had improved. This study highlights the need for preconceptual psychosocial counselling and postpartum adjustment to CF care

The (ir)relevance of the abandoned criterion II for the diagnosis of serrated polyposis syndrome:a retrospective cohort study

The World Health Organization (WHO) recently updated the diagnostic criteria for serrated polyposis syndrome (SPS). One of the three previous diagnostic criteria (criterion II 2010) is now abandoned: ≥ 1 serrated polyp (SP) proximal to the sigmoid in a first-degree relative (FDR) of a patient with SPS. Individuals fulfilling this abandoned criterion now receive the same surveillance recommendations as all FDRs of patients with SPS. We aimed to compare the incidence of advanced neoplasia (AN) in FDRs with vs. without fulfillment of the abandoned criterion II 2010. We retrospectively recruited FDRs of patients with SPS who underwent a colonoscopy, and stratified them according to fulfilment of criterion II 2010 at baseline. Our primary and secondary outcomes were AN incidence during surveillance and at baseline, respectively. We included 224 FDRs of patients with SPS, of whom 36 (16%) fulfilled criterion II 2010 at baseline. One hundred and five underwent surveillance after baseline. Criterion II 2010-positive FDRs were at increased risk of AN, both during surveillance (hazard ratio 8.94, 95% CI 2.15–37.1, p =.003) as well as at baseline (adjusted odds-ratio 9.30, 95% CI 3.7–23.3, p <.001). FDRs of patients with SPS that underwent colonoscopy and fulfilled the abandoned criterion II 2010 for SPS diagnosis were at increased risk of AN at baseline and during surveillance in this small, retrospective cohort study. Our results should be interpreted with caution but suggest that adherence to surveillance recommendations for all FDRs of patients with SPS is important, especially for those that would have fulfilled the now abandoned criterion II 2010

Service user engagement in healthcare education as a mechanism for value based recruitment: an evaluation study

Aim Within the United Kingdom (UK) there is an increasing focus on Values Based Recruitment (VBR) of staff working in the National Health Service (NHS) in response to public inquiries criticising the lack of person-centred care. All NHS employees are recruited on the basis of a prescribed set of values. This is extended to the recruitment of student healthcare professionals, yet there is little research of how to implement this. Involving Service Users in healthcare educational practice is gaining momentum internationally, yet involvement of service users in VBR of ‘would be’ healthcare professionals remains at an embryonic phase. Adult nurses represent the largest healthcare workforce in the UK, yet involvement of service users in their recruitment has received scant attention. This paper is an evaluation of the inclusion of service users in a VBR of 640 adult student nurses. Background Design This study used a participatory mixed methods approach, with service users as co-researchers in the study. Methods The study consisted of mixed methods design. Quantitative data via an online questionnaire to ascertain candidates' perspectives (n = 269 response rate of 42%), and academic/clinical nurses (n = 35 response rate 34.65%). Qualitative data were gathered using focus groups and one to one interviews with service users (n = 9). Data analysis included descriptive statistics and thematic analysis. Findings 4 overarching themes were identified; increasing sense of humanness, substantiating care values; impact of involvement; working together and making it work, a work in progress. Conclusion The findings from the study highlight that involving service users in VBR of student healthcare professionals has benefits to candidates, service users and local health services. Appreciating the perceptions of healthcare professionals is fundamental in the UK and internationally to implementing service users' engagement in service enhancement and delivery. Findings from this study identify there may be a dissonance between the policy, the nurses' thoughts and their practice

Humanising the interview process: an evaluation of Service User/Carers contribution to value based recruitment in a Pre-registration Adult Nursing Programme.

Within the UK there has been move towards value based recruitment (VBR) as a response to some highly publicised poor standards of care with the National Health Service (Francis 2013). In 2014, Health Education England (HEE) published their VBR Framework which articulated how recruitment of healthcare practitioners needed to focus on how applicants’ individual values/behaviours aligned with core values of the NHS Constitution (DoH 2013). Higher Education Institutes were expected to comply as 50% of student nurse programmes are based in healthcare settings. During 2014-2015 the pre-registration adult nursing team redesigned the interview process to increase a focus on VBR: integral to this, we felt was the inclusion of Service Users/Carers within the interview process. Following training SU/Carers graded applicants in a group activity alongside academic and practice partners. There have been few research studies published evaluating SU/Carers engagement in Pre-registration Adult Nursing interviews probably due to the challenges of implementing SU/Carer engagement in the large cohort numbers that adult nursing attracts. This mixed-method evaluation analysed the perspectives of differing stakeholders (Candidates, SU/Carers, Academics and Practice Partners) regarding the role SU/Carer engagement in Adult Nursing Pre-registration interviews. Early findings from candidates have highlighted they value the involvement of SU/Carers in the interview process, SU/Carers add a “human dimension” ensuring a focus on the heart of nursing and its value base rather than the role of nursing and associated nursing tasks. This paper will present the full evaluation identifying areas of good practice, some of the challenges as well recommendations for future work. References Department of Health. (2013) NHS Constitution. Department of Health: London. Francis, R. (2013) Report of the Mid Staffordshire NHS Foundation Trust Public Inquiry; Executive Summary. London. Health Education England. (2014) Value Based Recruitment Framework. Available from: http://hee.nhs.uk/wp-content/blogs.dir/321/files/2014/10/VBR-Framework.pdf (accessed 07/02/15

Familial Adenomatous Polyposis-Associated Desmoids Display Significantly More Genetic Changes than Sporadic Desmoids

Desmoid tumours (also called deep or aggressive fibromatoses) are potentially life-threatening fibromatous lesions. Hereditary desmoid tumours arise in individuals affected by either familial adenomatous polyposis (FAP) or hereditary desmoid disease (HDD) carrying germline mutations in APC. Most sporadic desmoids carry somatic mutations in CTNNB1. Previous studies identified losses on 5q and 6q, and gains on 8q and 20q as recurrent genetic changes in desmoids. However, virtually all genetic changes were derived from sporadic tumours. To investigate the somatic alterations in FAP-associated desmoids and to compare them with changes occurring in sporadic tumours, we analysed 17 FAP-associated and 38 sporadic desmoids by array comparative genomic hybridisation and multiple ligation-dependent probe amplification. Overall, the desmoids displayed only a limited number of genetic changes, occurring in 44% of cases. Recurrent gains at 8q (7%) and 20q (5%) were almost exclusively found in sporadic tumours. Recurrent losses were observed for a 700 kb region at 5q22.2, comprising the APC gene (11%), a 2 Mb region at 6p21.2-p21.1 (15%), and a relatively large region at 6q15-q23.3 (20%). The FAP-associated desmoids displayed a significantly higher frequency of copy number abnormalities (59%) than the sporadic tumours (37%). As predicted by the APC germline mutations among these patients, a high percentage (29%) of FAP-associated desmoids showed loss of the APC region at 5q22.2, which was infrequently (3%) seen among sporadic tumours. Our data suggest that loss of region 6q15-q16.2 is an important event in FAP-associated as well as sporadic desmoids, most likely of relevance for desmoid tumour progression

Familial Adenomatous Polyposis-Associated Desmoids Display Significantly More Genetic Changes than Sporadic Desmoids

Desmoid tumours (also called deep or aggressive fibromatoses) are potentially life-threatening fibromatous lesions. Hereditary desmoid tumours arise in individuals affected by either familial adenomatous polyposis (FAP) or hereditary desmoid disease (HDD) carrying germline mutations in APC. Most sporadic desmoids carry somatic mutations in CTNNB1. Previous studies identified losses on 5q and 6q, and gains on 8q and 20q as recurrent genetic changes in desmoids. However, virtually all genetic changes were derived from sporadic tumours. To investigate the somatic alterations in FAP-associated desmoids and to compare them with changes occurring in sporadic tumours, we analysed 17 FAP-associated and 38 sporadic desmoids by array comparative genomic hybridisation and multiple ligation-dependent probe amplification. Overall, the desmoids displayed only a limited number of genetic changes, occurring in 44% of cases. Recurrent gains at 8q (7%) and 20q (5%) were almost exclusively found in sporadic tumours. Recurrent losses were observed for a 700 kb region at 5q22.2, comprising the APC gene (11%), a 2 Mb region at 6p21.2-p21.1 (15%), and a relatively large region at 6q15-q23.3 (20%). The FAP-associated desmoids displayed a significantly higher frequency of copy number abnormalities (59%) than the sporadic tumours (37%). As predicted by the APC germline mutations among these patients, a high percentage (29%) of FAP-associated desmoids showed loss of the APC region at 5q22.2, which was infrequently (3%) seen among sporadic tumours. Our data suggest that loss of region 6q15-q16.2 is an important event in FAP-associated as well as sporadic desmoids, most likely of relevance for desmoid tumour progression

Evaluation of management of desmoid tumours associated with familial adenomatous polyposis in Dutch patients

Item does not contain fulltextBACKGROUND: The optimal treatment of desmoid tumours is controversial. We evaluated desmoid management in Dutch familial adenomatous polyposis (FAP) patients. METHODS: Seventy-eight FAP patients with desmoids were identified from the Dutch Polyposis Registry. Data on desmoid morphology, management, and outcome were analysed retrospectively. Progression-free survival (PFS) rates and final outcome were compared for surgical vs non-surgical treatment, for intra-abdominal and extra-abdominal desmoids separately. Also, pharmacological treatment was evaluated for all desmoids. RESULTS: Median follow-up was 8 years. For intra-abdominal desmoids (n=62), PFS rates at 10 years of follow-up were comparable after surgical and non-surgical treatment (33% and 49%, respectively, P=0.163). None of these desmoids could be removed entirely. Eventually, one fifth died from desmoid disease. Most extra-abdominal and abdominal wall desmoids were treated surgically with a PFS rate of 63% and no deaths from desmoid disease. Comparison between NSAID and anti-estrogen treatment showed comparable outcomes. Four of the 10 patients who received chemotherapy had stabilisation of tumour growth, all after doxorubicin combination therapy. CONCLUSION: For intra-abdominal desmoids, a conservative approach and surgery showed comparable outcomes. For extra-abdominal and abdominal wall desmoids, surgery seemed appropriate. Different pharmacological therapies showed comparable outcomes. If chemotherapy was given for progressively growing intra-abdominal desmoids, most favourable outcomes occurred after combinations including doxorubicin