That quotation is the title of a touching and thought provoking article just published (Berg SF, Paulsen OG, Carter BS. Why Were They in Such a Hurry to See Her Die? American Journal of Hospice and Palliative Medicine. 2013;30(4):406-8.) It is the personal story of a couple who had a baby with trisomy 18. Their individual history mirrors much of what Annie Janvier, Barb Farlow and Ben Wilfond described in their survey of many hundreds of families. It relates the lack of comprehension of many that they did not want an abortion, and the sometimes outright refusal to consider any options in the care of their baby.
At one point the couple go to see a neonatologist to discuss options of care: this was the response ‘The physician gave us the impression that this monster should have been removed long ago and that she was as good as dead.’
The most disturbing part of the story occurs when Evy is born. Despite an agreement to provide modest resuscitation if she doesn’t breathe immediately, no-one intervenes and the father, an intensive care physician/anesthesiologist, has to take her and do the resuscitation himself.
The parents describe how much the 3 days of Evy’s life affected them for the good, which helps us to remember that the value of a life cannot be measured in hours.
The story is accompanied by a discussion by Brian Carter, in which he addresses the issues, and the need to individualize our counseling and decision -making to the desires and values of the family. I think I can be proud that he then uses a list that I created for a previous blog post. It is a list of tips and suggestions to use when talking to parents who have a fetal, or neonatal, diagnosis of a potentially life-limiting condition. It was based on comments from families in Annie Janvier’s paper referred to above, of things that they appreciated, and things that they disliked, in their interaction with physicians, and other care-givers. Unfortunately he improved it a little (and also toned down the language a bit for a respectable journal rather than a blog). So here is the Carter revision of the Barrington tips for being a humane doctor (or other caregiver) in that situation.
(You can click to enlarge)
Neonatal Research 
I was actually wondering where those articles of trisomy 18 are from Dr Janvier’s presentation at le congrès de soins palls; I was having a conversation with some pediatricians here in Rouyn and they would like to see the sources / her presentation.
Dr Janvier’s first article about the trisomy 18 work is here. there are at least 2 other articles ocming out, one which is close to being published about the obstetric management issues and attitudes, then another about what predicts how long a baby will live.
We published an abstract at the last PAS meeting which I reproduce below: (there is a table that I can’t reproduce in the comments here, but you can find if you go on the website of the abstracts from the meeting.)
[2924.467] Not Lethal Anymore: Interventions, Hospitalizations and Outcomes of Children with Trisomy 13 and 18
Annie Janvier, Barbara Farlow, Keith Barrington. Patients for Patients’ Safety, Canada, Mississauga, ON, Canada; Clinical ethics and Pediatrics, Université de Montréal, Hôpital Sainte-Justine, Montreal, QC, Canada.
BACKGROUND: Trisomy 13 and 18 (T13-18) are often classified as lethal conditions because of their poor outcomes.
OBJECTIVE: To examine outcomes of T13-18 children.
DESIGN/METHODS: Anonymous online questionnaire. Parents who lived with T13-18 children answered questions about outcome, medical needs and quality of life (QOL).
RESULTS: 332 parents answered questions about their 272 children, (87% resp rate). 40% of diagnosis were prenatal (PND). The 216 children who had “full” T13-18 were analyzed. After the diagnosis, 25% chose active interventions for their child. After birth, children who had PND had significantly less medical interventions than children with postnatal diagnosis, including non-invasive interventions (IV, Oxygen, tube feeds). 150 children (69%) were discharged home after birth and 40% lived more than one yr. The following were associated with an increased likelihood to live up to one yr: postnatal diagnosis, absence of holoprosencephaly, greater birth weight, female sex, absence of comfort care at birth when PND. At home, 11% needed oxygen continuously and 45% had a gastrostomy. Children were hospitalized on average 4 wks their first yr of life. 60% of parents report that as their child gets older, they need less medical care, but many children still have significant hospital needs. 25 children had cardiac surgery (mostly VSD), 2 died within 1y of the interventions and the others survived more than one year (many still living). 37 children had abdominal surgery, 33 survived > 1yr after the procedure. Children have severe disabilities but progress over time
The subjective QOL reported by parents was similar between all children/families and not related to medical needs and disabilities. 50% of parents report their children had more pain then other children, but all described happy children who enriched their family life, irrespective of longevity.
CONCLUSIONS: T13-18 are not universally lethal conditions. Some children can have prolonged survival and demonstrate developmental progress. Parents who live with these children report a positive experience despite severe disabilities.
Thank you for engaging in this subject! I am happy to see that there are physicians out there who seem able to appreciate the value of short lives and of lives with disabilities. And I appreciate your list of do´s and don´t´s.
And thank you for sharing you story, I just visited your website, I was glad to find the page in English, especially to read the wonderful poems written by Evy’s older brother. Your story will make a difference, and help other families to find the care that they want.
Keith
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Evy’s story makes my heart break. We had the opposite experience, even with a prenatal diagnosis of T18. I didn’t know at the time how very rare that was, to be given full autonomy to make decisions for our son and then have our wishes followed, not questioned. Today is my son’s third birthday, yes, three years old, and he is one of our greatest blessings. He keeps on growing and thriving and loving life. I have not met a happier child. Thank you so much for keeping this dialog going.
Thank you for helping us, neonatologists from all over the world, to see things from a new perspective (in my opinion, the right one). Annie’s paper on parental feelings on babies with T18 and T13 should be of compulsory reading for all of us.
I do believe you and Annie are helping us to become better physicians.
Let me share with you a spanish Facebook Group that Barbara Farlow made me aware of. There are over 2000 members in that group. This might be helpful to Spanish speaking families. Here is the link: http://www.facebook.com/pages/Sindrome-de-Edwards-Trisomia-18/163899444398