Specialist perinatal palliative care: a retrospective review of antenatal referrals to a children's palliative care service over 14 years.

BACKGROUND: Perinatal palliative care is an emerging branch of children's palliative care. This study sought to better understand the pattern of antenatal referrals and the role of a specialist paediatric palliative care (PPC) team in supporting families throughout the antenatal period. METHODS: A single-centre retrospective chart review of all antenatal referrals to a quaternary children's palliative care service over a 14-year period from 2007 to 2021. RESULTS: One hundred fifty-nine antenatal referrals were made to the PPC team over a 14-year period, with increasing referrals over time. Referrals were made for a broad spectrum of diagnoses with cardiac conditions (29% of referrals) and Trisomy 18 (28% of referrals) being the most prevalent. 129 referrals had contact with the PPC team prior to birth and 60 had a personalised symptom management plan prepared for the baby prior to birth. Approximately one third (48/159) died in utero or were stillborn. Only a small number of babies died at home (n = 10) or in a hospice (n = 6) and the largest number died in hospital (n = 72). 30 (19% of all referrals) were still alive at the time of the study aged between 8 months and 8 years. CONCLUSIONS: Specialist PPC teams can play an important role in supporting families during the antenatal period following a diagnosis of a life-limiting fetal condition and demand for this service is increasing. A large proportion of the cases referred will not survive to the point of delivery and a number of babies may survive much longer than predicted. PPC teams can be particularly helpful navigating the uncertainty that exists in the antenatal period and ensuring that plans are made for the full spectrum of possible outcomes.

The impact of prenatal counselling on mothers of surviving children with hypoplastic left heart syndrome: A qualitative interview study.

OBJECTIVE: To explore the role of antenatal counselling in how parents make treatment decisions following an antenatal diagnosis of Hypoplastic Left Heart Syndrome (HLHS). BACKGROUND: Antenatal counselling is a critical part of patient management following a diagnosis of fetal congenital heart disease; however, there is a very limited evidence base examining how parents actually experience antenatal counselling and make decisions in this context. METHODS: Semi-structured interviews were conducted with women who had received an antenatal diagnosis of HLHS. Interviews were digitally recorded, anonymised and transcribed verbatim. A thematic content analysis was performed using a constant comparative approach. RESULTS: Eight mothers of surviving children with HLHS were interviewed. Eight key themes emerged including new perspectives on how women receive antenatal counselling and how it affects their decision making. Three themes in particular are new to the literature: (a) Mothers of children with HLHS reported feelings of intense guilt that arose in the antenatal period around potentially causing the condition in their child. (b) For this group of women, recollections of perceived pessimism during antenatal counselling had a lasting impact. (c) Despite support from partners or extended family, women nevertheless experienced a strong sense that antenatal decision making was largely a 'maternal' responsibility. CONCLUSIONS: When recounting their experiences of antenatal counselling, mothers of surviving children with HLHS offer new perspectives that can guide fetal cardiologists in how best to support their individual patients. Further research is needed to comprehensively understand the experience of prospective parents counselled for severe forms of fetal congenital heart disease.

End of life care in the setting of extreme prematurity - practical challenges and ethical controversies.

While the underlying principles are the same, there are differences in practice in end of life decisions and care for extremely preterm infants compared with other newborns and older children. In this paper, we review end of life care for extremely preterm infants in the delivery room and in the neonatal intensive care unit. We identify potential justifications for differences in the end of life care in this population as well as practical and ethical challenges.

Paediatric palliative care in the NICU: A new era of integration.

We are entering a new era of integration between neonatal medicine and paediatric palliative care, with increasing recognition that the role and skills of palliative care extend beyond care of only the terminally ill infant. This paper addresses the principles of paediatric palliative care and how they apply in the NICU, considers who provides palliative care in this setting and outlines the key components of care. We consider how the international standards of palliative care pertain to neonatal medicine and how a fully integrated approach to care may be realised across these two disciplines. Palliative care is so much more than end-of-life care, offering a proactive and holistic approach which addresses the physical, emotional, spiritual and social needs of the infant and family. This is a truly interdisciplinary endeavour, relying on a harmonisation of the skills from both the neonatal and palliative care teams to deliver high-quality coordinated care.

Facilitators and barriers to the delivery of palliative care to children with life-limiting and life-threatening conditions: a qualitative study of the experiences and perceptions of healthcare professionals.

OBJECTIVE: To understand healthcare system facilitators and barriers to the delivery of palliative care for children with life-limiting and life-threatening conditions and their family members. DESIGN: Focus groups with children's palliative care professionals. Data were analysed using thematic analysis. SETTING: Four regions of England (West Midlands, South West, Yorkshire and Humber, and London) from December 2017 to June 2018. PARTICIPANTS: Healthcare professionals (doctors, nurses and allied healthcare professionals) working in children's palliative care services. FINDINGS: A total of 71 healthcare professionals participated in the focus groups. Three overarching themes were identified which influenced whether and when children were referred to and started to receive palliative care: (1) the unspoken background of clinical uncertainty which often delayed palliative care; (2) the cultural 'collusion of immortality', where conversations about the possibility of dying can be avoided or deferred; and (3) the role of paediatric palliative care teams in 'illuminating the blind spot' of palliative care as well as providing hands-on care. CONCLUSIONS: Palliative care is a holistic approach to care that focuses on quality of life for people living with life-limiting and life-threatening conditions that can be delivered alongside active treatment. There is a need to prioritise and integrate this into healthcare services for children more effectively if improvements in care are to be realised. While more specialist paediatric palliative care services are needed, the unspoken background of clinical uncertainty needs to be addressed together with the collusion of immortality within healthcare culture and organisations.

Use of home parenteral nutrition in severely neurologically impaired children.

OBJECTIVE: To review the outcome of children with severe neurological impairment (NI) and intestinal failure (IF) referred to our specialist multidisciplinary IF rehabilitation service and to discuss implications. DESIGN: Case report series, descriptive analysis. SETTING: IF rehabilitation programme at a tertiary children's hospital in the UK. PATIENTS: Children with severe NI referred to our IF rehabilitation programme from 2009 to 2019. MAIN OUTCOME MEASURES: Demographic and social data, diagnosis, clinical condition, use of home parenteral nutrition (HPN), complications, ethics review outcome and advance care plans. RESULTS: Six patients with severe NI were referred to our IF rehabilitation service. Consent for publication was obtained from five families. After thorough medical review and clinical ethics committee assessment, three children started HPN, one had intravenous fluids in addition to enteral feed as tolerated and one intravenous fluids only. The HPN children survived 3-7.08 years (median 4.42 years) on treatment. Objective gastrointestinal signs, for example, bleeding improved without excessive HPN-related complications. Symptomatic improvement was less clear. Analgesia was reduced in three of the five children. All cases had detailed symptom management and advance care plans regularly updated. CONCLUSIONS: HPN can play a role in relieving gastrointestinal signs/symptoms in children with severe NI and IF. HPN can be conceptualised as part of good palliative care if judged to be in the child's best interests. However, given its risks and that HPN has the potential to become inappropriately life-sustaining, a thorough ethics review and evaluation should be performed before it is initiated, withheld or withdrawn in children with severe NI.

Right ventricular morphology and function following stage I palliation with a modified Blalock-Taussig shunt versus a right ventricle-to-pulmonary artery conduit.

OBJECTIVES: The Norwood procedure for hypoplastic left heart syndrome (HLHS) is performed either via a right ventricle-to-pulmonary artery (RVPA) conduit or a modified Blalock-Taussig (MBT) shunt. Cardiac magnetic resonance (CMR) data was used to assess the effects of the RVPA conduit on ventricular shape and function through a computational analysis of anatomy and assessment of indices of strain. METHODS: A retrospective analysis of 93 CMR scans of subjects with HLHS was performed (59 with MBT shunt, 34 with RVPA conduit), incorporating data at varying stages of surgery from two congenital centres. Longitudinal and short-axis cine images were used to create a computational cardiac atlas and assess global strain. RESULTS: Those receiving an RVPA conduit had significant differences (P< 0.0001) in the shape of the RV corresponding to increased ventricular dilatation (P = 0.001) and increased sphericity (P = 0.006). Differences were evident only following completion of stage II surgery. Despite preserved ejection fraction in both groups, functional strain in the RVPA conduit group compared with that in the MBT shunt group was reduced across multiple ventricular axes, including a reduced systolic longitudinal strain rate (P< 0.0001), reduced diastolic longitudinal strain rate (P = 0.0001) and reduced midventricular systolic circumferential strain (P < 0.0001). CONCLUSIONS: Computational modelling analysis reveals differences in ventricular remodelling in patients with HLHS undergoing an RVPA conduit insertion with focal scarring and volume loading leading to decreased functional markers of strain. The need for continued surveillance is warranted, as deleterious effects may not become apparent until later years.

The importance of early involvement of paediatric palliative care for patients with severe congenital heart disease.

Growing numbers of patients with severe congenital heart disease (CHD) are surviving into late childhood and beyond. This increasingly complex patient group may experience multiple formidable and precarious interventions, lifelong morbidity and the very real risk of premature death on many occasions throughout their childhood. In this paper, we discuss the advantages of a fully integrated palliative care ethos in patients with CHD, offering the potential for improved symptom control, more informed decision-making and enhanced support for patients and their families throughout their disease trajectory. These core principles may be delivered alongside expert cardiac care via non-specialists within pre-existing networks or via specialists in paediatric palliative care when appropriate. By broaching these complex issues early-even from the point of diagnosis-an individualised set of values can be established around not just end-of-life but also quality-of-life decisions, with clear benefits for patients and their families regardless of outcome.

Technical and anatomical factors affecting the size of the branch pulmonary arteries following first-stage Norwood palliation for hypoplastic left heart syndrome.

OBJECTIVES: Branch pulmonary artery (BPA) size is one of the factors that influence the efficacy of the Fontan circulation. Central pulmonary artery stenosis and small left pulmonary artery (LPA) are well-known problems following Norwood palliation for hypoplastic left heart syndrome (HLHS). We investigated anatomical and technical factors that may stand behind these problems. METHODS: A total of 47 consecutive patients were included in the study. All had complete magnetic resonance imaging (MRI) study pre-second-stage palliation. Measurements were taken using a first-pass 3D angiography technique after intravenous injection of an extravascular contrast agent. Factors investigated included the following: size and site of the pulmonary artery bifurcation stump in relation to the Damus-Kaye-Stansel (DKS) anastomosis, interaortic distance/ratio (neoaorta to descending aorta distance/antero-posterior dimension of the chest) (IAD/IAR), distance from the under surface of the arch and the size of native aorta and pulmonary artery. IAD/IAR were compared between two different arch reconstruction techniques. RESULTS: Stenosis occurred either centrally, at the origin of the BPA, or more distally, in the mid-LPA (posterior to DKS). There was a significant lower incidence of central BPA stenosis when the pulmonary artery stump was placed in the mid-position compared with right/left position (26 vs 67%; P = 0.011). A more bulky pulmonary artery stump was also found in those patients with central BPA stenosis (186 vs 137 mm(2)/m(2); P = 0.047). The mid-LPA consistently showed antero-posterior compression (mean cranio-caudal diameter 3.82 mm vs mean antero-posterior diameter 3.07 mm, P < 0.001). Indexed mid-LPA area was only correlated with IAD/IAR (r = 0.49 and 0.51, P < 0.001). No correlation was shown with the distance to the under surface of the arch (r = 0.14, P = 0.37), again confirming antero-posterior compression of the LPA rather than cranio-caudal. In multivariable analysis, the only predictor of indexed mid-LPA area was the IAR (P < 0.001). There was no significant difference in the IAD or IAR between the two arch reconstruction techniques [mean IAD 15.5 vs 13.5 mm (P = 0.14)]; [mean IAR 0.17 vs 0.19 (P = 0.21)]. CONCLUSIONS: Of all studied factors, IAR and the size and position of the pulmonary artery bifurcation plays the main role in LPA growth and central BPA stenosis.

Diffuse primary large B cell lymphoma of leg type presenting on the breast: a rare case of surgical excision.

Primary breast lymphoma is a rare subtype of breast malignancy whose diagnosis relies on histopathological characteristics and a high index of suspicion in clinically unusual presentations of breast pathology. The authors report a rare case of diffuse primary large B cell lymphoma of leg type presenting as a single lesion confined to the breast. Unusually for this subtype of lymphoma, the lesion was surgically excised and the patient, to date, has made an uneventful recovery.