What support do nurses need to provide palliative care for people with dementia?

The aim of this project was to identify the support required by registered nurses and unregistered healthcare support workers to provide palliative care for people with dementia in an acute hospital in England. A quantitative approach was taken and participants were asked to fill in a questionnaire. Data were collated and analysed to identify support needs and any emerging themes. Respondents were confident in identifying the different stages of dementia. There was less confidence in identifying a patient with dementia for palliative care referral compared with a patient without dementia. Further needs were identified by respondents in supporting the family/carer of the person with dementia and being aware of available support to facilitate palliative care for people with dementia and support for end of life care (EoLC) planning. The findings suggest that further work is required in relation to dementia and EoLC. Practical and educational collaboration with EoLC/palliative care practitioners and dementia leads would be beneficial.

Person-centred dementia care in acute settings.

A dementia activities coordinator role was created in an acute hospital environment with the aim of improving the experience of patients admitted to an elderly care ward who have dementia or cognitive impairment. Patient and carer feedback has confirmed the benefits of the role for patients and the increased confidence of carers in the support offered during hospital admission.

Time for a gut check: Pancreatic sufficiency resulting from CFTR modulator use.

Pancreatic exocrine insufficiency in cystic fibrosis is genetically determined and generally felt to be irreversible. However, recent studies in young children started on cystic fibrosis transmembrane conductance regulator (CFTR) modulators have suggested improvement of pancreatic functioning over time. Here, we present the case of a 10-year-old child with pancreatic exocrine insufficiency since birth who regained pancreatic functioning after 4 years on the CFTR corrector drug, ivacaftor.

Alternative donor hematopoietic stem cell transplantation for sickle cell disease.

Most patients who could be cured of sickle cell disease (SCD) with stem cell transplantation do not have a matched sibling donor. Successful use of alternative donors, including mismatched family members, could provide a donor for almost all patients with SCD. The use of a reduced-intensity conditioning regimen may decrease late adverse effects. Ten patients with symptomatic SCD underwent CD34+ cell-selected, T-cell-depleted peripheral blood stem cell transplantation from a mismatched family member or unrelated donor. A reduced-intensity conditioning regimen including melphalan, thiotepa, fludarabine, and rabbit anti-thymocyte globulin was used. Patients were screened for a companion study for immune reconstitution that included a donor lymphocyte infusion given 30-42 days after transplant with intravenous methotrexate as graft-versus-host disease (GVHD) prophylaxis. Seven eligible patients were treated on the companion study. Nine of 10 patients are alive with a median follow-up of 49 months (range, 14-60 months). Surviving patients have stable donor hematopoietic engraftment (mean donor chimerism, 99.1% ± 0.7%). There were no sickle cell complications after transplant. Two patients had grade II-IV acute GVHD. One patient had chronic GVHD. Epstein-Barr virus-related posttransplant lymphoproliferative disorder (PTLD) occurred in 3 patients, and 1 patient died as a consequence of treatment of PTLD. Two-year overall survival was 90%, and event-free survival was 80%. A reduced-intensity conditioning regimen followed by CD34+ cell-selected, T-cell-depleted alternative donor peripheral blood stem cell transplantation achieved primary engraftment in all patients with a low incidence of GVHD, although PTLD was problematic. This trial was registered at clinicaltrials.gov as #NCT00968864.

A near lethal case of plastic bronchitis following truncal valve replacement.

Plastic bronchitis is a condition in which large, rubber-like, bronchial casts develop in the tracheobronchial tree causing airway obstruction. It is an unusual disorder that occurs in various disease states. Most case reports of plastic bronchitis associated with congenital heart disease are in patients palliated with Fontan physiology. We describe a 13-year-old girl with DiGeorge syndrome and truncus arteriosus who underwent an uneventful truncal valve replacement. The child developed plastic bronchitis on postoperative day eight requiring extra-corporeal membrane oxygenation support and daily bronchoscopy to remove bronchial casts. Our patient did not have elevated systemic venous pressures or Fontan physiology. We speculate the etiology of plastic bronchitis may have been surgical trauma to the lymphatic channels surrounding the bronchi and disruption of pleural adhesions. A comprehensive evaluation revealed no specific cause and the diagnosis of idiopathic plastic bronchitis was made.