Commentaries - Ethics
These publications include commentaries, narratives and ethics-related papers about medical care for children with trisomy 13 and 18. As a result of support groups, blogs and websites on the internet that reveal happy, surviving children with these conditions, parents have begun to request consideration of treatment for their children. This request often conflicts with the traditional management of care, causing disagreement and distress to both parents and medical staff. Click on the link to access the paper.
Lorenz & Hardart, Curr Opin Pediatr 2014. Evolving medical and surgical management of infants with trisomy 18.
Lorenz and Hardart have reviewed the literature on management and prognosis of infants with trisomy 18. They discuss an interesting range of articles, including older and newer studies on intensive care and cardiac surgery. The older case series, they state, suggest that the two dominant beliefs – that cardiac surgery would not prolong life and that children with trisomy 18 do not die from their cardiac anomalies - were mistaken. Babies with cardiac anomalies and trisomy 18 just did not survive long enough to die from their cardiac anomalies. The new reports on outcome after intensive care and cardiac surgery show increased survival. The authors recommend that parents are included in the decision making process, and also that burdensome interventions such as cardiac surgery are deferred until it becomes likely that the child may survive for longer than six months to a year. They conclude their article with these words: “Healthcare management approaches or policies that reject out of hand the goal of prolonging the life of any infant/child with trisomy 18 are not defensible.”
Thiele et al. 2013. More than a diagnosis. Acta Paediatrica
Three mothers who had children with trisomy 13 or 18 describe the difficulties in the medical care they received before birth, when baby was newborn and as an older infant. They make recommendations to physicians regarding how care of mothers expecting babies with trisomy 13 or 18 and babies with trisomy 13 or 18 could be improved.
Two of the authors of this paper, Dr. Siri Fuglem Berg and Barbara Farlow, are co-founders of ITA.
Janvier et al. 2013. Medical Interventions for children with trisomy 13 and 18: what is the value of a short disabled life? Acta Paediatrica
A Canadian neonatologist and Australian pediatrician comment on the changing view of trisomy 13 and 18, the benefits of interventions for some children, the ethics of providing care and the quality of life of the children. The doctors describe the purpose of the children as 'moral enhancers' and the enrichment of parents' lives as a result of having a baby born with trisomy 13 or 18. 'They learned to love and expect nothing in return, they learned that imperfection does not mean inferior, they learned to not take life for granted, they learned to celebrate every day as if it was the last.'
Berg et al. 2013. Why were they in such a hurry to see her die? Am J Hosp Palliat Care
Parents of a baby with trisomy 18 share the experience of their baby’s birth and short life including the lack of understanding of the staff. Both parents of the baby are physicians.Commentary about this article posted in the Neonatal Research Blog (Dr. Keith Barrington). http://neonatalresearch.org/2013/06/07/why-were-they-in-such-a-hurry-to-see-her-die/.
One of the authors of this paper, Dr. Siri Fuglem Berg, is a co-founder of ITA
Boss et al. 2013. Pediatrics Trisomy 18 and complex congenital heart disease: seeking the threshold benefit
This is a case study about parents who receive a prenatal diagnosis for trisomy 18 and desire to create an optimal birth plan for their child. The unborn baby has a severe heart condition, hypo plastic left heart syndrome (HLHS). The article describes their journey to obtain approval before birth including multiple meetings with staff.
Bruns, D. A. 2013. Erring on the side of life: Children with rare trisomy conditions, medical interventions and quality of life. Journal of Genetic Disorders and Genetic Reports.
In this opinion article, the author argues that children with trisomy 13 and 18 should be examined as individuals and considered for invasive interventions to prolong life pursuant to Human Rights Conventions.
The author of this article is a co-founder of ITA.
Carey, Curr Opin Pediatr 2013 Perspectives on the care and management of infants with trisomy 18 and trisomy 13: Striving for balance
In this article, Dr. Carey (geneticist, Board member and a Founder of the Support Organization for Trisomy (SOFT USA) provides recommendations for counseling parents with a child who has trisomy 13 or 18 in consideration of the most recent information about the diagnoses, the spectrum of the condition and the values of the family.
Careda, Carey. 2012. Orphanet J Rare Dis The trisomy 18 syndrome
This article is a thorough, up-to-date overview of what is known about trisomy 18. One of the authors is Dr. Carey, Board member and Founder of SOFT USA.
Heuser et al. 2012. J Med Ethics Survey of physicians' approach to severe fetal anomalies.
This article describes survey results of 732 members of the American Society of Maternal Fetal Medicine. Data are provided on the manner in which women who receive a prenatal diagnosis for trisomy 13 and 18 are counselled including options for pregnancy management and delivery of conditions including trisomy 13 and 18.
Thiele et al, 2011. BMJ Destined to die
An Australian mother of a baby who receives a prenatal diagnosis for trisomy 18 chooses to continue her pregnancy and make plans for his birth. She experiences hurtful comments by many providers and even a refusal for delivery by one hospital. Finally, she meets a doctor who understands and who respects her decision and her son’s life.
Farlow 2011. Curr Probl Pediatr Adolesc Health Care Choosing the road less traveled –
A Canadian mother describes her family’s choice to consider interventions for their child with trisomy 13, the child’s short life, and their troubling experience with medical professionals.
The author of this article is a co-founder of ITA.
Everett BJ et al. 2011. J Clin Ethics Ethical care for infants with conditions not curable with intensive care.
The authors, from British Columbia, Canada, suggest a framework for palliative care.
Fenton 2011. Am J Med Genet A Trisomy 13 and 18 and quality of life: treading ‘softly’
The article recounts the experience of a former American neonatologist who attends his first family conference for children with trisomy 13 and 18. He is quite impressed by the children and families he meets. He concludes that the medical system needs to fully support parents in the decisions they make for their children with trisomy, regardless of the level of care they choose.
Janvier et al. 2011. Pediatrics An infant with trisomy 18 and a ventricular septal defect
This is an ethics case involving an infant with trisomy 18 in the United States whose parents request cardiac surgery. A perinatologist, neonatologist and the mother of a child with trisomy 13 offer their perspectives on various aspects of the baby’s medical care..
ITA believes that this article is helpful for the insight it offers parents about the ethics and perspectives providers have about caring for an infant with trisomy 18. It also allows understanding of the reason that conflicts in the level of care sometimes arise. One of the authors of this article is a co-founder of ITA.
Mercurio 2011. J Perinatol The role of pediatrics ethics committee in the newborn intensive care unit
The author reviews two cases that have come before the hospital ethics committee in an American hospital. One of the cases involves a baby with trisomy 13 whose parents are requesting cardiac surgery. The article analyzes the request according to all bioethical principles. The author challenges the traditional standard of care for children with trisomy 13 (no treatment) and the basis upon which the standards were developed. The committee approves the surgery and the baby survives surgery and lives for several years afterward.
ITA believes that this article provides good insight into bioethics and the complexity of making decisions. Dr. Mercurio, the author of this paper, makes an excellent common about traditional standards of care and how they must be challenged. He writes, "The standard of care at this institution was to provide children with Trisomy 13 with comfort measures only and allow them to die without surgery or any intensive measures such as mechanical ventilation. For many questions in medicine, the standard of care is often used to determine the best course of action, but a good physician will always want to know upon what that standard is based. Good medical practice requires knowing the strength of the evidence and arguments that support any standard, and basing one's degree of flexibility with regard to the standard on the strength of its foundation."
Morrison et al. 2010. Circulation 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science Part 3: Ethics
This article is based on guidelines for resuscitation developed by the International Liaison Committee on Resuscitation (ILCOR). ILCOR’s guidelines have been adopted by most countries, including the European Resuscitation Council. ILCOR recommends that infants with trisomy 13 and similar conditions not be candidates for newborn resuscitation based on risk of early death and what has been deemed by those involved on the committee to be “unacceptable morbidity” among survivors.
ITA notes that this guideline was developed in the absence of the parental perspective of surviving children. Articles related to the quality of life of surviving children have subsequently been published (Janvier, 2012 and Kosho,2013).
Wilkinson 2010. J Med Ethics Antenatal diagnosis of trisomy 18, harm and parental choice.
The author of this paper, who is a physician and ethicist, concedes that a small proportion of children with trisomy 18 survive and benefit from surgeries. However, the author believes that for most children, there is little chance of benefit from the child enduring painful surgeries and treatments. He encourages that high quality palliative care be an option offered to all parents to ensure that babies don’t suffer when there is little chance of benefit.
ITA believes that often, how well a child will do and the optimal plan of care cannot be known with certainty before birth. Some parents choose to create a birth plan that allows for different options, depending on how the baby is doing after birth.
McGraw et al. 2008. Pediatrics Attitudes of neonatologists toward delivery room management of confirmed trisomy 18: potential factors influencing a changing dynamic.
This article provides information about the attitudes of neonatologists and their willingness to resuscitate a newborn with trisomy 18 with a cardiac anomaly. Only 44% of neonatologists were willing to resuscitate and most would do so only because parents requested resuscitation.
There are two published responses (Dr. S Showalter and Dr. John Carey) to this article:
1. http://pediatrics.aappublications.org/content/123/3/e548.long
2. http://pediatrics.aappublications.org/content/123/3/e547.long
ITA encourages parents to read the excellent responses to the McGraw et al. article. It is important to remember the uniqueness of every baby born with trisomy 13 and 18. ITA believes that it is wrong to withhold or limit care on the basis of the genetic label alone.
Siegel, 2006 Mt Sinai J Med When staff and parents disagree: decision making for a baby with trisomy 13
This article describes the struggle between staff and parents related to the appropriate level of care for a baby with trisomy 13. The article offers helpful insight into the providers’ perspective in such a situation.
Guinan 2005 Virtual Mentor. Patau syndrome and perinatal decision making.
This article describes a case related to a prenatal diagnosis of trisomy 13 and the perspective of the family along with commentaries from two representatives of different religious faiths.
Koogler et al 2003 Hastings Center Report Lethal language, lethal decisions
This article challenged the futility of certain conditions that had commonly become known to be “lethal”. The authors argue that some of the conditions that cause death might be resolved through interventions but because physicians describe the conditions as lethal, parents opt not to seek treatments. The authors furthermore argue that physicians might be using lethal language to impose a quality of life judgement that is not theirs to make.
Lorenz & Hardart, Curr Opin Pediatr 2014. Evolving medical and surgical management of infants with trisomy 18.
Lorenz and Hardart have reviewed the literature on management and prognosis of infants with trisomy 18. They discuss an interesting range of articles, including older and newer studies on intensive care and cardiac surgery. The older case series, they state, suggest that the two dominant beliefs – that cardiac surgery would not prolong life and that children with trisomy 18 do not die from their cardiac anomalies - were mistaken. Babies with cardiac anomalies and trisomy 18 just did not survive long enough to die from their cardiac anomalies. The new reports on outcome after intensive care and cardiac surgery show increased survival. The authors recommend that parents are included in the decision making process, and also that burdensome interventions such as cardiac surgery are deferred until it becomes likely that the child may survive for longer than six months to a year. They conclude their article with these words: “Healthcare management approaches or policies that reject out of hand the goal of prolonging the life of any infant/child with trisomy 18 are not defensible.”
Thiele et al. 2013. More than a diagnosis. Acta Paediatrica
Three mothers who had children with trisomy 13 or 18 describe the difficulties in the medical care they received before birth, when baby was newborn and as an older infant. They make recommendations to physicians regarding how care of mothers expecting babies with trisomy 13 or 18 and babies with trisomy 13 or 18 could be improved.
Two of the authors of this paper, Dr. Siri Fuglem Berg and Barbara Farlow, are co-founders of ITA.
Janvier et al. 2013. Medical Interventions for children with trisomy 13 and 18: what is the value of a short disabled life? Acta Paediatrica
A Canadian neonatologist and Australian pediatrician comment on the changing view of trisomy 13 and 18, the benefits of interventions for some children, the ethics of providing care and the quality of life of the children. The doctors describe the purpose of the children as 'moral enhancers' and the enrichment of parents' lives as a result of having a baby born with trisomy 13 or 18. 'They learned to love and expect nothing in return, they learned that imperfection does not mean inferior, they learned to not take life for granted, they learned to celebrate every day as if it was the last.'
Berg et al. 2013. Why were they in such a hurry to see her die? Am J Hosp Palliat Care
Parents of a baby with trisomy 18 share the experience of their baby’s birth and short life including the lack of understanding of the staff. Both parents of the baby are physicians.Commentary about this article posted in the Neonatal Research Blog (Dr. Keith Barrington). http://neonatalresearch.org/2013/06/07/why-were-they-in-such-a-hurry-to-see-her-die/.
One of the authors of this paper, Dr. Siri Fuglem Berg, is a co-founder of ITA
Boss et al. 2013. Pediatrics Trisomy 18 and complex congenital heart disease: seeking the threshold benefit
This is a case study about parents who receive a prenatal diagnosis for trisomy 18 and desire to create an optimal birth plan for their child. The unborn baby has a severe heart condition, hypo plastic left heart syndrome (HLHS). The article describes their journey to obtain approval before birth including multiple meetings with staff.
Bruns, D. A. 2013. Erring on the side of life: Children with rare trisomy conditions, medical interventions and quality of life. Journal of Genetic Disorders and Genetic Reports.
In this opinion article, the author argues that children with trisomy 13 and 18 should be examined as individuals and considered for invasive interventions to prolong life pursuant to Human Rights Conventions.
The author of this article is a co-founder of ITA.
Carey, Curr Opin Pediatr 2013 Perspectives on the care and management of infants with trisomy 18 and trisomy 13: Striving for balance
In this article, Dr. Carey (geneticist, Board member and a Founder of the Support Organization for Trisomy (SOFT USA) provides recommendations for counseling parents with a child who has trisomy 13 or 18 in consideration of the most recent information about the diagnoses, the spectrum of the condition and the values of the family.
Careda, Carey. 2012. Orphanet J Rare Dis The trisomy 18 syndrome
This article is a thorough, up-to-date overview of what is known about trisomy 18. One of the authors is Dr. Carey, Board member and Founder of SOFT USA.
Heuser et al. 2012. J Med Ethics Survey of physicians' approach to severe fetal anomalies.
This article describes survey results of 732 members of the American Society of Maternal Fetal Medicine. Data are provided on the manner in which women who receive a prenatal diagnosis for trisomy 13 and 18 are counselled including options for pregnancy management and delivery of conditions including trisomy 13 and 18.
Thiele et al, 2011. BMJ Destined to die
An Australian mother of a baby who receives a prenatal diagnosis for trisomy 18 chooses to continue her pregnancy and make plans for his birth. She experiences hurtful comments by many providers and even a refusal for delivery by one hospital. Finally, she meets a doctor who understands and who respects her decision and her son’s life.
Farlow 2011. Curr Probl Pediatr Adolesc Health Care Choosing the road less traveled –
A Canadian mother describes her family’s choice to consider interventions for their child with trisomy 13, the child’s short life, and their troubling experience with medical professionals.
The author of this article is a co-founder of ITA.
Everett BJ et al. 2011. J Clin Ethics Ethical care for infants with conditions not curable with intensive care.
The authors, from British Columbia, Canada, suggest a framework for palliative care.
Fenton 2011. Am J Med Genet A Trisomy 13 and 18 and quality of life: treading ‘softly’
The article recounts the experience of a former American neonatologist who attends his first family conference for children with trisomy 13 and 18. He is quite impressed by the children and families he meets. He concludes that the medical system needs to fully support parents in the decisions they make for their children with trisomy, regardless of the level of care they choose.
Janvier et al. 2011. Pediatrics An infant with trisomy 18 and a ventricular septal defect
This is an ethics case involving an infant with trisomy 18 in the United States whose parents request cardiac surgery. A perinatologist, neonatologist and the mother of a child with trisomy 13 offer their perspectives on various aspects of the baby’s medical care..
ITA believes that this article is helpful for the insight it offers parents about the ethics and perspectives providers have about caring for an infant with trisomy 18. It also allows understanding of the reason that conflicts in the level of care sometimes arise. One of the authors of this article is a co-founder of ITA.
Mercurio 2011. J Perinatol The role of pediatrics ethics committee in the newborn intensive care unit
The author reviews two cases that have come before the hospital ethics committee in an American hospital. One of the cases involves a baby with trisomy 13 whose parents are requesting cardiac surgery. The article analyzes the request according to all bioethical principles. The author challenges the traditional standard of care for children with trisomy 13 (no treatment) and the basis upon which the standards were developed. The committee approves the surgery and the baby survives surgery and lives for several years afterward.
ITA believes that this article provides good insight into bioethics and the complexity of making decisions. Dr. Mercurio, the author of this paper, makes an excellent common about traditional standards of care and how they must be challenged. He writes, "The standard of care at this institution was to provide children with Trisomy 13 with comfort measures only and allow them to die without surgery or any intensive measures such as mechanical ventilation. For many questions in medicine, the standard of care is often used to determine the best course of action, but a good physician will always want to know upon what that standard is based. Good medical practice requires knowing the strength of the evidence and arguments that support any standard, and basing one's degree of flexibility with regard to the standard on the strength of its foundation."
Morrison et al. 2010. Circulation 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science Part 3: Ethics
This article is based on guidelines for resuscitation developed by the International Liaison Committee on Resuscitation (ILCOR). ILCOR’s guidelines have been adopted by most countries, including the European Resuscitation Council. ILCOR recommends that infants with trisomy 13 and similar conditions not be candidates for newborn resuscitation based on risk of early death and what has been deemed by those involved on the committee to be “unacceptable morbidity” among survivors.
ITA notes that this guideline was developed in the absence of the parental perspective of surviving children. Articles related to the quality of life of surviving children have subsequently been published (Janvier, 2012 and Kosho,2013).
Wilkinson 2010. J Med Ethics Antenatal diagnosis of trisomy 18, harm and parental choice.
The author of this paper, who is a physician and ethicist, concedes that a small proportion of children with trisomy 18 survive and benefit from surgeries. However, the author believes that for most children, there is little chance of benefit from the child enduring painful surgeries and treatments. He encourages that high quality palliative care be an option offered to all parents to ensure that babies don’t suffer when there is little chance of benefit.
ITA believes that often, how well a child will do and the optimal plan of care cannot be known with certainty before birth. Some parents choose to create a birth plan that allows for different options, depending on how the baby is doing after birth.
McGraw et al. 2008. Pediatrics Attitudes of neonatologists toward delivery room management of confirmed trisomy 18: potential factors influencing a changing dynamic.
This article provides information about the attitudes of neonatologists and their willingness to resuscitate a newborn with trisomy 18 with a cardiac anomaly. Only 44% of neonatologists were willing to resuscitate and most would do so only because parents requested resuscitation.
There are two published responses (Dr. S Showalter and Dr. John Carey) to this article:
1. http://pediatrics.aappublications.org/content/123/3/e548.long
2. http://pediatrics.aappublications.org/content/123/3/e547.long
ITA encourages parents to read the excellent responses to the McGraw et al. article. It is important to remember the uniqueness of every baby born with trisomy 13 and 18. ITA believes that it is wrong to withhold or limit care on the basis of the genetic label alone.
Siegel, 2006 Mt Sinai J Med When staff and parents disagree: decision making for a baby with trisomy 13
This article describes the struggle between staff and parents related to the appropriate level of care for a baby with trisomy 13. The article offers helpful insight into the providers’ perspective in such a situation.
Guinan 2005 Virtual Mentor. Patau syndrome and perinatal decision making.
This article describes a case related to a prenatal diagnosis of trisomy 13 and the perspective of the family along with commentaries from two representatives of different religious faiths.
Koogler et al 2003 Hastings Center Report Lethal language, lethal decisions
This article challenged the futility of certain conditions that had commonly become known to be “lethal”. The authors argue that some of the conditions that cause death might be resolved through interventions but because physicians describe the conditions as lethal, parents opt not to seek treatments. The authors furthermore argue that physicians might be using lethal language to impose a quality of life judgement that is not theirs to make.
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