Anthony Herbert

Components and Principles of a Pediatric Palliative Care Consultation: Results of a Delphi Study

BACKGROUND Pediatric palliative care is a distinct specialty that requires input from pediatric and palliative medicine specialists to provide comprehensive high-quality care. Consultations undertaken early in a childs illness trajectory, when end-of-life care is not anticipated to be required, enables relationships to be established and may enhance the quality of care provided. OBJECTIVE To define optimal components of an early pediatric palliative care consultation. DESIGN Consensus of an expert group was sought in a five-round Delphi study. SETTING/PARTICIPANTS Based on the literature and existing standards for specialist palliative care, components of an early pediatric palliative care consultation were derived. In rounds 2 and 3, experts from around Australia participated in online surveys to review and prioritize the components and principles. Consensus of survey items was determined by defined criteria. A flowchart was developed in the fourth round and the final round involved review and refinement of the flowchart by the expert group. RESULTS Nineteen experts participated and prioritized 34 components and principles in the first survey round, and 36 statements in the second survey round. There was consensus from all participants that the first priority of a consultation was to establish rapport with the family, and examples of how to achieve this were defined. Other components of a consultation included: establishing the familys understanding of palliative care; symptom management; an emergency plan; discussion of choices for location of care, and a management plan. Components considered suitable to defer to later consultations, or appropriate to address if initiated by family members, included: spiritual or religious issues; discussion around resuscitation and life-sustaining therapies; end-of-life care; and the dying process. CONCLUSION We have provided the first published framework from expert consensus that defines the components and principles of an early pediatric palliative care consultation. This framework will provide guidance for clinical practice as well as being useful for education and research in this area.

Home telehealth and paediatric palliative care: clinician perceptions of what is stopping us?

BackgroundAdvances in technology have made the use of telehealth in the home setting a feasible option for palliative care clinicians to provide clinical care and support. However, despite being widely available and accessible, telehealth has still not been widely adopted either in Australia or internationally. The study aim was to investigate the barriers, enablers and perceived usefulness for an established home telehealth program in paediatric palliative care from the perspective of clinicians.MethodsSemi-structured interviews (n = 10) were undertaken with palliative care clinicians in a tertiary paediatric hospital to identify attitudes to, satisfaction with, and perceived benefits and limitations of, home telehealth in palliative care. Iterative analysis was used to thematically analyse data and identify themes and core concepts from interviews.ResultsFour themes are reported: managing relationships; expectations of clinicians; co-ordination, and the telehealth compromise. Core concepts that emerged from the data were the perceived ability to control clinical encounters in a virtual environment and the need to trust technology. These concepts help explain the telehealth compromise and low utilisation of the home telehealth program.ConclusionsEffective communication between caregivers and clinicians is recognised as a core value of palliative care. Home telehealth has the potential to provide a solution to inequity of access to care, facilitate peer support and maintain continuity of care with families. However, significant limitations and challenges may impede its use. The virtual space creates additional challenges for communication, which clinicians and families may not intuitively understand. For home telehealth to be integrated into routine care, greater understanding of the nature of communication in the virtual space is required.

Home telemedicine for paediatric palliative care

Children with life limiting conditions require intensive, complex management of the physical, psychosocial and spiritual issues that evolve for the child and their family as the focus of care moves from curative treatment to palliation. Optimal palliative care for children can be limited by the lack of health professionals with the necessary skills and experience to confidently and competently manage the care of both the child and the family. The University of Queenslands Centre for Online Health has been working closely with the Paediatric Palliative Care Service (PPCS) at the Royal Childrens Hospital (RCH) in Brisbane, Australia, to investigate the potential of telemedicine for the delivery of specialist support in the home or local community. Research conducted in Queensland has shown how telemedicine can be effectively used to link the specialist paediatric palliative care team into the home of families caring for a child receiving palliative care, regardless of their physical distance from the RCH. This qualitative report describes two case studies, each illustrating the value of home telemedicine for families at this vulnerable and distressing time. This work should provide a useful insight for other specialty disciplines contemplating home telemedicine applications.

Unrelated cord blood transplantation in a girl with Hoyeraal–Hreidarsson syndrome

Hoyeraal–Hreidarsson syndrome (HHS, OMIM 500545) is a multisystem disease believed to represent a severe variant of dyskeratosis congenita (DC, OMIM 305000). We report the case of a 40-month-old girl with classical features of HHS, including cerebellar hypoplasia, oral ulceration and BM failure. She is the 19th case of HHS and only the 4th female reported in the medical literature. She received an unrelated cord stem cell transplant with a fludarabinebased reduced-intensity conditioning (RIC) regime. To the best of our knowledge, she is only the fourth HHS patient to receive a haematopoietic stem cell transplantation (HSCT). Besides reactivation of CMV infection, there was minimal regimen-related toxicity associated with the stem cell transplant. This case highlights the classic features of HHS, the most likely locus of heterogeneity associated with HHS and the importance of considering the diagnosis before transplant. Hoyeraal–Hreidarsson syndrome is a severely progressive multisystem disorder characterized by intrauterine growth retardation, microcephaly, cerebellar hypoplasia, mental retardation, oral aphthous ulcers, immune deficiency and aplastic anaemia. HHS creates significant mortality and morbidity, with BM failure and immunodeficiency being the most serious manifestation of the condition, occurring in over 80% of patients. The natural history of HHS is that of death secondary to complications of BM failure or infection that usually occurs in the first decade of life. The patient reported here was the only child to nonconsanguineous Caucasian Australian parents. Gross motor and fine motor delay and ataxia became evident during the first 2 years of life. Persistent oral ulceration began at 22 months of age. Cerebellar hypoplasia was detected on a magnetic resonance imaging scan at 24 months of age. At 30 months of age, thrombocytopaenia was diagnosed during the treatment for an intercurrent respiratory illness. A BM aspirate at this time revealed a hypocellular marrow consistent with trilineage BM failure. The coding regions for DKC1 and TERC, in DNA extracted from peripheral blood, were screened for mutations by denaturing HPLC and they failed to identify any mutations. Immunological work up showed normal Ig and complement levels. Tand B-cell subsets were within normal reference ranges. Stimulation with phytohaemagglutinin and DHR-123-oxidative burst was normal. There was no increase in chromosomal breakage with diepoxybutane. In both peripheral and BM chromosome analyses, an abnormal cytogenetic clone was not detected. Owing to progressive marrow failure and no matched related or unrelated donor being available, an unrelated cord blood transplant was performed. Following local Human Research Ethics Committee approval and with informed consent from the family, the fludarabine-based RIC regimen reported by Barker et al. was used as follows: fludarabine, 40mg/m once daily i.v. on days –6 to –2 (total five doses); CY, 50mg/kg daily i.v. on day 6 (total one dose); and TBI on day –1 (200 cGy 1 fraction). Antithymocyte globulin (equine) (ATGAM), 15mg/kg twice daily i.v., was administered for 3 days (total six doses). The unrelated cord unit was an HLA 5/6 match with total nucleated cell and CD34 doses of 11.5 10/kg and 2.6 10/kg recipient body weight, respectively. The patient received G-CSF (5mcg/kg daily) after transplantation until neutrophil engraftment. GVHD prophylaxis consisted of CYA and mycophenolate. Myeloid engraftment defined as an absolute neutrophil count of 40.5 10/l for 43 days occurred on day þ 13, and platelet engraftment defined as a count of 450 10/l for 43 days without support occurred on day þ 45. The last packed RBC transfusion was given on day þ 28. Donor alleles were identified on engraftment studies performed by PCR for variable nucleotide tandem repeats (VNTRs) of the apolipoprotein B gene at day þ 34 with complete donor chimerism by day þ 100. Grade 2 GVHD of the skin and the gastrointestinal tract was confirmed on biopsies at day þ 25. This responded well to treatment with prednisolone. The patient developed CMV reactivation on day 47 and on day 145 post transplant, as detected by rising CMV DNA titres (PCR). Both of these episodes were successfully treated with ganciclovir. The patient is currently 2 years post transplant with ongoing steroiddependent chronic GVHD of the skin and the gastrointestinal tract. She is continuing to progress in speech and motor developmental abilities and is attending a special needs preschool. This patient displayed features consistent with a diagnosis of HHS, including intrauterine growth retardation, cerebellar hypoplasia, leukoplakia, developmental delay and aplastic anaemia. HHS and DC exhibit clinical and genetic heterogeneity with more than one locus implicated. Vulliamy et al. report that almost 50% of patients with DC and HHS do not harbour a mutation of the DKC1 gene. Clear genotype–phenotype correlations are yet to emerge. However, null mutations have not been described, Bone Marrow Transplantation (2008) 42, 293–294 & 2008 Macmillan Publishers Limited All rights reserved 0268-3369/08

Respite needs of families receiving palliative care

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Principles of a paediatric palliative care consultation can be achieved with home telemedicine.

The care of a child with a life‐limiting condition proves an emotional, physical and financial strain on the family that provides care for their child. Respite care is one way which allows carers to receive some relief and support in the context of this burden of care. The provision of and the requirements for respite in this context is poorly understood. This survey aims to describe the types of respite care families receive, the respite that they would ideally receive and the barriers that prevent this.

Exploratory study of sleeping patterns in children admitted to hospital

We compared the records of paediatric palliative consultations undertaken face-to-face, with telemedicine consultations undertaken in patients’ homes. A convenience sample of consecutive paediatric palliative care patients was identified from the hospitals palliative care database. A total of 100 consultations was reviewed (50 telemedicine consultations during home visits and 50 face-to-face consultations) according to 14 established principles and components of a paediatric palliative care consultation. In the telemedicine group there was a higher proportion of patients in a stable condition (58% vs 7%), and a lower proportion of patients in terminal phase (2% vs 17%). Discussion about pain and anorexia were significantly more common in the telemedicine group. Discussion about follow up was significantly more common in the telemedicine group (86% vs 56%), whilst resuscitation planning was more common in deteriorating patients receiving inpatient care. All other components and principles of a palliative care consultation were documented equally regardless of method of consultation. The findings confirm that palliative consultations via telemedicine are just as effective as face-to-face consultations in terms of the documented components of the consultation.

Adequacy of palliative care in a single tertiary neonatal unit.

Sleep is considered an important time of healing and restoration during illness. The primary aim of this study was to determine the prevalence of self‐reported sleep disturbance in children admitted to a tertiary childrens hospital with a variety of medical diagnoses.

Development of a state-wide pediatric palliative care service in Australia: referral and outcomes over two years.

End‐of‐life care remains part of the scope of practice in all neonatal units. This study aimed to characterise the end‐of‐life care provided in an Australian tertiary neonatal centre, where paediatric palliative care was accessible via a consultative service.

Affording opportunities to discuss deterioration in paediatric palliative care consultations: a conversation analytic study

BACKGROUND Pediatric palliative care is an evolving specialty that aims to improve the lives of children with a life-limiting condition. As an emerging specialty, there is much to be learned about service provision and the expected outcomes that can be achieved. Additionally, quantification of the needs for pediatric palliative care is complicated by the uncertainty of defining the population that requires care. OBJECTIVES Our aim was to define the characteristics of the population cared for by a newly formed state-wide service in Queensland, Australia, and describe the development of the service over a 24-month period. DESIGN Data on all referrals and outcomes were collected. Descriptive statistics were used to describe patterns including the variation in outcomes between children with oncology and non-oncology diagnoses. Other factors influencing the development of the service including involved health professionals and the model of the Australian health care system are also described. RESULTS Over a 24-month period, 150 patients were referred of whom 117 subsequently died. There was a wide range of diseases and ages, and significantly, 58% of children were from regional or rural locations where there are can be limited access to specialist pediatric services. The average length of service was 83 days. A variety of factors were identified as being important for providing optimal care including ensuring equity in access, timing of referral, and continuity of care. CONCLUSION The importance of a population-based approach to pediatric palliative care in a state that is geographically large and diverse like Queensland is highlighted. This article may provide valuable information to other health care providers who care for children with life-limiting illnesses.