Specialist perinatal palliative care: a retrospective review of antenatal referrals to a children's palliative care service over 14 years
child; article; female; human; major clinical study; male; palliative therapy; hospice; uncertainty; school child; fetus; retrospective study; medical record review; infant; heart; hospital mortality; trisomy 18; prenatal period
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.
Bertaud S; Brightley G; Crowley N; Craig F; Wilkinson D
BMC Palliative Care
2023
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1186/s12904-023-01302-5" target="_blank" rel="noreferrer noopener">10.1186/s12904-023-01302-5</a>
Building trust and improving communication with parents of children with Trisomy 13 and 18: A mixed-methods study
death; article; child; female; human; major clinical study; male; palliative therapy; social support; decision making; ethics; thematic analysis; trisomy 13; trisomy 18; trust; adverse event; clinician; disability; questionnaire
BACKGROUND: Trisomy 13 and trisomy 18 are common life-limiting conditions associated with major disabilities. Many parents have described conflictual relationships with clinicians, but positive and adverse experiences of families with healthcare providers have not been well described. AIM: (1) To investigate parental experiences with clinicians and (2) to provide practical recommendations and behaviors clinicians could emulate to avoid conflict. DESIGN: Participants were asked to describe their best and worse experiences, as well as supportive clinicians they met. The results were analyzed using mixed methods. SETTING/PARTICIPANTS: Parents of children with trisomy 13 and 18 who were part of online social support networks. A total of 503 invitations were sent, and 332 parents completed the questionnaire about 272 children. RESULTS: The majority of parents (72%) had met a supportive clinician. When describing clinicians who changed their lives, the overarching theme, present in 88% of answers, was trust. Parents trusted clinicians when they felt he or she cared and valued their child, their family, and made them feel like good parents (69%), had appropriate knowledge (66%), and supported them and gave them realistic hope (42%). Many (42%) parents did not want to make-or be part of-life-and-death decisions. Parents gave specific examples of supportive behaviors that can be adopted by clinicians. Parents also described adverse experiences, generally leading to conflicts and lack of trust. CONCLUSION: Realistic and compassionate support of parents living with children with trisomy 13 and 18 is possible. Adversarial interactions that lead to distrust and conflicts can be avoided. Many supportive behaviors that inspire trust can be emulated.
Janvier A; Farlow B; Barrington KJ; Bourque CJ; Brazg T; Wilfond B
Palliative Medicine
2019
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1177/0269216319860662" target="_blank" rel="noreferrer noopener">10.1177/0269216319860662</a>
Implementation of Pediatric Palliative Care (PPC) in a Neonatal Intensive Care Unit (NICU) in St.Gallen
decision making; death; newborn; Switzerland; trisomy 18; major clinical study; newborn period; pregnancy; neonatal intensive care unit; conference abstract; human; child; female; male; palliative therapy; drug withdrawal; brain hemorrhage
Introduction: The Children's Hospital of Eastern Switzerland (OKS) is a hospital with 76 beds. A multiprofessional PPC and ethic team was established in 2007. After nine years of intensive development our PPC program reached full size (notification D) of sanaCERT Suisse certification. Until presently, the OKS is the only children's hospital in Switzerland which is certified in PPC. Furthermore, our PPC team is a member of the Pediatric Palliative Care Network Switzerland (PPCNCH). In order to focus on neonates, the biggest group needing PPC, we decided to develop a special program for Neonatal Palliative Care (NPC). Background(s): According to epidemiologic data, 400-500 children between the age of 0 and 18 years are dying in Switzerland each year. It's known from the PELICAN study (Bergstrasser; Zimmermann et al., 2016), that about 50% of these children are dying in the first year of life, of which 40% in the neonatal period. Four out of five children are dying in an ICU, the majority of them after a decision-making process with the decision to withhold or withdraw further treatment. Apparently, the neonates represent an important group and therefore it is reasonable to concentrate on NPC in the NICU. Case presentation: We present three neonatal patients suffering from severe, life threatening conditions: one newborn with trisomy 18, one premature of 32 week of gestation with a large intracerebral hemorrhage and a neonate with a life threatening conditions. The presentations demonstrate the difficulties and challenges and illustrate the importance of the involved teams networking in decision making and implementing care for these patients and their families under particular circumstances. The circumstances of death and the definitions of withholding or withdrawing therapy will be explained. Furthermore, the different requirements, discussions and the resources available in these cases will be presented. This indicates the possible improvements and developments in that area. Conclusion(s): Neonates are an important group to consider in a PPC program. Considering that the neonatal period is a very special phase of life for the child and his/her family deserve particular consideration and structures in order to treat them adequately. Caring for a neonate and his family needing PPC is a challenging task for the family and the multiprofessional team. Clear structures and allocated resources are very important to fulfill this need in a meaningful way.
Gudrun J; Susanne A
Swiss Medical Weekly
2017
Experiences of children with trisomy 18 referred to pediatric palliative care services on two continents
palliative care; trisomy 18; pediatrics
Children with trisomy 18 that survive beyond the neonatal period have multiple congenital anomalies, neurodevelopmental disability, and high mortality rates. The experience of children with trisomy 18 who receive pediatric palliative care services is largely unknown. We conducted a retrospective review of children with trisomy 18 receiving pediatric palliative care services at both Boston Children's Hospital, USA and Great Ormond Street Hospital, UK from January 1, 2004 to January 1, 2015. Fifty-eight children with trisomy 18 were referred to pediatric palliative care, 38 in the United Kingdom, 20 in the United States. Median age at referral was 19 days (2-89) in the United Kingdom, and 25 days (1-463) in the United States. Median length of time being followed by pediatric palliative care was 32 days (1-1,637) in the United Kingdom and 67 days (3-2,442) in the United States. The only significant difference in the two cohorts (p = .001) was in likelihood of receiving cardiac surgical intervention-37% in the United States, 0% the United Kingdom. Children with trisomy 18 receive pediatric palliative care services, with variable age at referral and for a variable length of time. Further research is needed to understand the experience of children with trisomy 18 and their families receiving pediatric palliative care services.
Mullin J; Wolfe J; Bluebond-Langner M; Craig F
American Journal of Medical Genetics
2019
<a href="http://doi.org/10.1002/ajmg.a.61149" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.61149</a>
Trisomy 18: A survey of opinions, attitudes, and practices of neonatologists
Attitude Of Health Personnel; Health Care Surveys; Neonatologists/px [psychology]; Physician Attitude; Practice Patterns Physicians'; Trisomy 18; Trisomy/di [diagnosis]; Adult; Article; Attitude To Abortion; Caucasian; Christian; Chromosomes Human Pair 18; Clinical Practice; Correlation Analysis; Demography; Disease Management; Disease Severity; Family; Female; Fetus Malformation; Human; Humans; Intellectual Impairment; Male; Marriage; Medical Decision Making; Medical Society; Neonatologist; Newborn Care; Normal Human; Outcome Assessment (health Care); Palliative Care; Palliative Therapy; Parental Attitude; Prenatal Diagnosis; Priority Journal; Race Difference; Resuscitation; Survival Rate; Trisomy 18; United States
We conducted a survey-based study of the opinions, attitudes, and management practices of neonatologists across the United States regarding prenatally diagnosed Trisomy 18. The survey was designed based on previously validated surveys of severe fetal anomalies and collected demographic information on participants, as well as their attitudes, and management choices given a series of vignettes beginning in the prenatal period. The survey was sent to 3,143 American Academy of Pediatrics Section on Neonatal-Perinatal Medicine members of which 409 (13%) completed the survey. While the response rate was rather low, our respondent pool was representative of the national neonatologist population. Respondents were predominately white (81%), married (88%), Christian (54%), had children (86%), and were pro-choice in terms of abortion (68%). Eighty-three percent (83%) of respondents thought that trisomy 18 is a lethal condition and 60% thought that treatment is futile. Seventy-five percent (75%) expected that the best neurodevelopmental outcome in the case of infant survival would be profound intellectual disability. Regarding neonatal care, 95% stated that they would recommend palliative care only. Ninety-five percent (95%) would never recommend or recommend only if asked full code resuscitation for a neonate with full trisomy 18, yet, 44% would comply partially or in full with a full code request for resuscitation measures. The demographic features that correlated most significantly with these responses were clinician race and years in practice. The attitudes toward and management of infants affected with trisomy 18 seem to be largely driven by parental attitudes and wishes. � 2016 Wiley Periodicals, Inc.
Jacobs AP; Subramaniam A; Tang Y; Philips JB 3rd; Biggio JR; Edwards RK; Robin NH
American Journal Of Medical Genetics Part A
2016
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ajmg.a.37807" target="_blank" rel="noreferrer">10.1002/ajmg.a.37807</a>
Moving Toward A Shared Process: The Impact Of Parent Experiences On Perinatal Palliative Care
Decision Making; Neonatal; Palliative Care; Parent Experiences; Perinatal; Trisomy 13; Trisomy 18
Hasegawa SL; Fry JT
Seminars In Perinatology
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
10.1053/j.semperi.2016.11.002
Surgical Intervention In Trisomy 18-supporting Pallative Care
Trisomy 18; Birth Weight; Clinical Article; Diagnosis; Female; Gastrointestinal Symptom; Heart; Hospital; Human; Infant; Intestine Obstruction; Male; Meningomyelocele; Palliative Therapy; Pathology; Pregnancy; Prognosis; Surgery; Tracheoesophageal Fistula
Aims Patients with Trisomy 18 have a poor prognosis-only 5%-10% of children survive beyond the first year; however various clinical features of Trisomy 18 such as gastrointestinal, cardiac and central nervous system anomalies may benefit from surgery. Providing intensive care including surgery must be carefully considered. We aimed to review surgical intervention in babies with Trisomy 18 and its outcome. Methods A retrospective case note review was undertaken of babies with a confirmed diagnosis of Trisomy 18 admitted to NICU over a 5 year period (01/09/2011 to 31/08/2016). Results 15 babies with Trisomy 18 were identified, 12 male and 3 female. Mean birth weight was 1.91 kg. Babies were born at a mean gestation of 39 weeks (range 31-41 weeks) (Tables 1, 2 and 3). Out of the 8 babies who had surgical pathology, 6 were stable enough to receive surgical intervention. 1 infant with a TOF/OA and 1 with complex cardiac problems were too unstable for surgery and died in hospital. Of the patients operated on, 1 baby died in theatre but the remainder were discharged home. Gastrointestinal problems were the main indications for surgery. 3 operations were undertaken for tracheo-oesophageal fistula, 2 for intestinal obstruction and 1 myelomeningocoele repair. Three of the 6 operations took place before a diagnosis of Trisomy 18 was confirmed. Conclusion Patients with Trisomy 18 who had surgical lesions that were amenable to intervention received surgery and had good short term outcomes with the majority of these patients discharged home. Surgery was performed both before and after the diagnosis of Trisomy 18 was confirmed, although it was suspected in all but one case. We therefore consider that a diagnosis of Trisomy 18 should not be a contraindication to surgical intervention. Our data shows that despite the poor long term prognosis of Trisomy 18, surgery should be considered to enable good quality palliative care, enable discharge home and improve the quality of a short life. (Table presented).
Masood Y; Spiers H; Cragie R; Edi-Osagie N
Archives Of Disease In Childhood
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
10.1136/archdischild-2017-313087.503
The Impact Of A Sibling's Life-limiting Genetic Condition On Adult Brothers And Sisters
Family Attitude; Sibling; Sibling Attitude; Trisomy 13; Trisomy 18; Adult; Article; Brother; Coping Behavior; Female; Genetic Disorder; Grief; Human; Male; Parental Attitude; Peer Group; Priority Journal; Psychological Resilience; Qualitative Analysis; Sister; Structured Interview; Wellbeing
It is estimated that rare diseases affect the lives of over three million people in the United Kingdom. Of these, a significant proportion are children and young people with genetic life-limiting or life-shortening conditions. This study used a qualitative approach with in-depth semi-structured interviews to explore the experiences of 10 adult siblings of a baby diagnosed with Trisomy 13 (Patau syndrome) or Trisomy 18 (Edward syndrome). Findings illustrate that parental grief from the time of their child's diagnosis onward is also experienced by siblings. Although young adults may have conflicting feelings as a bereaved sibling, there is evidence that the experience impacts on their world views and their attitudes about prospective and expectant parenthood. The study highlights the importance of providing siblings with short-term and long-term support from the time of their brother's or their sister's diagnosis onward and provides new understanding about benefit of professional and peer support in helping young adults develop resilience and coping strategies.
Brown E; Coad J; Franklin A
American Journal Of Medical Genetics Part A
2017
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
10.1002/ajmg.a.38213
Trisomy 18: A Survey Of Opinions, Attitudes, And Practices Of Neonatologists
Genetics & Heredity; Infant; Intervention
Attitudes; Management; Neonatology; Trisomy 18
We conducted a survey-based study of the opinions, attitudes, and management practices of neonatologists across the United States regarding prenatally diagnosed Trisomy 18. The survey was designed based on previously validated surveys of severe fetal anomalies and collected demographic information on participants, as well as their attitudes, and management choices given a series of vignettes beginning in the prenatal period. The survey was sent to 3,143 American Academy of Pediatrics Section on Neonatal-Perinatal Medicine members of which 409 (13%) completed the survey. While the response rate was rather low, our respondent pool was representative of the national neonatologist population. Respondents were predominately white (81%), married (88%), Christian (54%), had children (86%), and were pro-choice in terms of abortion (68%). Eighty-three percent (83%) of respondents thought that trisomy 18 is a lethal condition and 60% thought that treatment is futile. Seventy-five percent (75%) expected that the best neurodevelopmental outcome in the case of infant survival would be profound intellectual disability. Regarding neonatal care, 95% stated that they would recommend palliative care only. Ninety-five percent (95%) would never recommend or recommend only if asked full code resuscitation for a neonate with full trisomy 18, yet, 44% would comply partially or in full with a full code request for resuscitation measures. The demographic features that correlated most significantly with these responses were clinician race and years in practice. The attitudes toward and management of infants affected with trisomy 18 seem to be largely driven by parental attitudes and wishes.
Jacobs AP; Subramaniam A; Tang Y; Philips JB; Biggio JR; Edwards RK; Robin NH
American Journal Of Medical Genetics Part A
2016
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
DOI: 10.1002/ajmg.a.37807
Parental Hopes, Interventions, And Survival Of Neonates With Trisomy 13 And Trisomy 18
End-of-life Decision Making; Palliative Care; Perinatal Hospice; Perinatal Palliative Care; Prenatal Diagnosis; Trisomy 13; Trisomy 18
Trisomy 13 and 18 are life-limiting conditions for which a palliative approach is frequently recommended. The objective of this study was to examine parental goals/decisions, the length of life of their child and factors associated with survival. Parents of children who lived with trisomy 13 or 18 that were part of English-speaking social networks were invited to participate in a questionnaire study. Participants answered questions about their hopes/goals, decisions regarding neonatal interventions, and the duration of their children's lives. The participants were 332 parents who answered questions about their 272 children (87% response rate based on site visits; 67% on invitations sent). When parents were asked about their hope after the diagnosis, the main themes invoked by parents were the following: meet their child alive (80% of parents with a prenatal diagnosis), spend some time as a family (72%), bring their child home (52%), and give their child a good life (66%). Parents wanted to give them a chance, but also reported their fears were medical complexity, pain and/or life in the hospital (61%). Healthcare providers recommended comfort care at birth to all parents. Life-sustaining interventions "as for any other child" was chosen as a plan of care by 25% of parents. Of the 216 children with full trisomy, 69% were discharged home after birth and 40% lived >1 y. The presence of a prenatal diagnosis was the strongest independent factor negatively associated with longevity: 36% of children with a prenatal diagnosis lived <24 hr and 47% were discharged home compared to 1% and 87%, respectively for children with a postnatal diagnosis (P < 0.01). Male gender, low-birth weight, and cardiac and/or cerebral anomaly were also associated with decreased survival (P < 0.05). After a prenatal diagnosis, palliative care at birth consisted of limited interventions, whereas after a postnatal diagnosis (median age of 6 days) it consisted of various interventions, including oxygen, ventilation, tube feeding and intravenous fluids, complicating the analysis. In conclusion, the goals of parents of children with trisomy 13 or 18 were to meet their child, be discharged home and be a family. Having a postnatal diagnosis was the independent factor most associated with these goals. Children with a postnatal diagnosis were treated "as any other children" until the diagnosis, which may give them a survival advantage, independent of palliative care. Rigorous transparency regarding specific interventions and outcomes may help personalize care for these children.
Janvier A
American Journal Of Medical Genetics Part C: Seminars In Medical Genetics
2016
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
DOI: 10.1002/ajmg.c.31526