Another of the articles that have been keeping me busy over the last couple of months has just been published. Boutillier B, Biran V, Janvier A, Barrington KJ. Survival and Long-term Outcomes of Children Who Survived After End-of-Life Decisions in a Neonatal Intensive Care Unit. Journal of Pediatrics. 2023:113422.

Discussions with parents about the progress of their baby, when we have concerns about their survival, or poor prognosis, are not infrequent in the NICU. We may decide to continue as before, or to limit interventions, or to withdraw some, or all, life sustaining interventions. One of our fellows, Beatrice Boutillier had collated the results of a large number of such discussions in a French tertiary NICU, and investigated the outcomes, who died, who survived after treatment limitation or withdrawal decisions, and the long term outcomes of those who survived. Annie Janvier and I collaborated with Beatrice, and with Valérie Biran, the chief of the NICU at Robert Debré hospital, to publish the data from their NICU.

There is very little other such information in the literature. Although there are several publications about the proportions of deaths that follow end of life discussions, many of those have collected data only on babies who died, there is little about those who survive after a decision to limit or withdraw care.

Although each case is unique, we squeezed them into general categories, using a revision of the categories of Eduard Verhagen et al. In some, all ICU care was withdrawn, including assisted ventilation, this could either be because it was thought there was little chance of survival in an unstable infant (category C), or because the long term prognosis for an acceptable quality of life was thought unlikely (category D). There were some infants where there was a decision to not escalate care, such as deciding to not go to the OR, or to not start an inotrope, or sometimes just to withhold CPR, these were category B. Those with withholding some interventions or limiting care were the babies most likely to survive, 18/29. But even among those with withdrawal of life-sustaining interventions, 4/41 unstable, and 12/94 stable infants survived.

All of the babies who survived to discharge required some medical follow up, and for many there were multiple specialties involved. Of the 34 who survived to 2 years, we had functional outcome information on 32, 8 of whom had outcomes between functionally normal, up to functional limitations with likely supervised living in the future, the remaining 24 were predicted to need help with activities of daily living or to be partially or totally dependent. We used the Glasgow Outcomes Score-Extended, as adapted for children (which I think gives a much better picture of the functional abilities of the children than a label of “NDI”).

The results point out the uncertainties inherent in our practice; even unstable babies that you think will likely die, who are so sick that you decide to withdraw their life-sustaining interventions, may still survive. After doing neonatology for over 40 years now (I know, I don’t look that old) I find myself often humbled by seeing things I would have thought impossible. Recently I looked after a baby with a pH <7.00 for 12 hours, (the baby was on extreme support, the parents weren’t ready to withdraw LST, so we continued while assuring good analgesia), who survived and is doing OK after discharge.

I am sometimes tempted to give up trying to predict anything! But I think it is better just to be transparent and honest and be open about the uncertainties of life, and of neonatology. We must always recognize that we are lousy at predicting survival, and even worse at predicting long term functional disability. Even with all the tools at our disposal, it is rare that we can say anything definitive.

One thing that doesn’t seem to help parents is to try and develop predictive models which give a percentage likelihood of survival or of poor long term outcomes. Although it is a different time of life, a recent study is relevant McDonnell SM, Basir MA, Yan K, Liegl MN, Windschitl PD. Effect of Presenting Survival Information as Text or Pictograph During Periviable Birth Counseling: A Randomized, Controlled Trial. Journal of Pediatrics. 2023. This study, using a vignette of a threatened delivery at 22 weeks gestation, showed that it didn’t make any difference how outcome data were presented (text or pictogram) to treatment decisions, it also showed that it didn’t make any difference what data were presented! Whether a 30% or 60% survival probability were revealed to a randomly selected group of 1000 women of child-bearing age didn’t make any difference to decisions, it didn’t even make any difference to what the participants thought the chance of survival was; when told the baby had a 30% chance of survival the participants thought the baby had a 68% chance of survival. Participants, however, thought that even with palliative care the baby was likely to survive (median 58% chance of survival), which does show that the presentation of the information, no matter how it was done, did not lead to the respondents having accurate knowledge of the results of palliative care.

The article is reviewed by a really nice editorial from Naomi Laventhal (Laventhal N. Negative Studies and the Future of Prenatal Counseling at the Margin of Gestational Viability. Journal of Pediatrics. 2023:113440.) who points out that the data are consistent with other studies, and that although there are several tools for calculating percentage survival and disability, there is no good data to show that such percentages are what parents want, or that it makes a difference to decisions made, even if they accept the figures they are given.

“These seemingly nihilistic study results are illuminating. Should we be spending our time trying to get better at sharing outcome probabilities with expectant parents if the probabilities aren’t what matter to them?”

I never give percentage survival figures, unless parents ask for them (which they almost never do), and, to be honest, I am not at all sure that the difference between 30% survival and 60% should make a difference in decision-making. You might as well say 50:50, and leave it at that for both cases. There is a reasonable chance of survival, and ICU care is probably worth a try, I would say, and most of the respondents in the article by McDonnell seem to say the same thing. It might be different if the chances were 1% vs 99%, but it is hard to think of a scenario which could be included in such a study where the chances would be 1% survival.

To return to the case series that we just published, some of the considerations in counselling in the NICU are similar. We can try and calculate survival rates, but each case is different, each individual baby can only be 100% survival or 0% survival, and parental (and physician) attitudes and values are probably more important to the outcomes of such decisions than percentages. We have to always remember that neonatologists are remarkably bad at predicting the future.

We should also remember that parents of surviving preterm infants almost never regret their life and death decisions. In another study that we published recently, Thivierge E, et al. Guilt and Regret Experienced by Parents of Children Born Extremely Preterm. Journal of Pediatrics. 2022, 113 of 248 parents of former preterm infants, seen in the follow up clinic, expressed some regrets about the NICU stay. None of them regretted decisions made to start or continue NICU care. We know from other data that decisional regret is more common after decisions to opt for palliative or comfort care alone. The regrets that parents did express were more associated with lack of self-care during the NICU stay, regrets about having a preterm baby (for which mothers often blamed themselves) and regrets related to their role as parents in the NICU. Addressing those issues could help to improve parental mental health after discharge.