The new Epicure 2 data show encouraging trends in outcomes in the UK and Ireland for extremely preterm babies. Survival rates have improved significantly over the whole of the British Isles, Here for example is one of the figures from the long term outcome publication.
As you can see the proportion of survivors has increased, and the proportion of severely impaired survivors has not changed, when expressed as a proportion of the survivors. When expressed as a proportion of the number of babies who can be stabilized at birth and are admitted to NICU this means that the proportion of admissions who are severely impaired has increased. So in 1995 there were 266 survivors of 666 babies admitted, 39%; of them 19% (51 infants) were severely impaired, so the percentage of the original 666 babies who were admitted for intensive care who survive with a severe impairment is 51/666 = 7.7%. In 2006 there were proportionally many more survivors; 593 survivors of 1115 babies admitted, 53%. 19% of them (113) had severe impairments, the percentage of the original 1115 babies admitted for intensive care who survive with a severe impairment is 113/1115 = 10.1%.
Now you can interpret these data according to your own prejudices, and that is indeed what has been happening. The editorial accompanying the publications in the BMJ was very negatively slanted. That editorial, written by 2 individuals from the Netherlands, includes the following quotes ‘a greater absolute number of neonates had major morbidity’, ‘the prevalence of important adverse neurological and developmental outcomes had indeed not improved’ and ‘of 100 neonates born at 24 weeks, 60 will die despite intensive care, and of the 40 survivors 12 will have serious impairments’ (this is actually a serious error! Fifteen or nineteen percent of the survivors at 24 weeks gestation had serious impairments, which means that of the 40 survivors in their scenario there would be 6 or 7 with serious impairments).
One other quote: ‘Similar outcome data are seen in the Netherlands, and neonatal intensive care is therefore not offered routinely to neonates born before 24 completed weeks’ gestation.’ This is also untrue, there are no similar outcome data from the Netherlands. As there are no survivors at 23 weeks in the Netherlands, and until recently there were no survivors at 24 weeks either, they do not have data to make that statement.
I think that anyone looking at the graph at the top of this post without any pre-existing prejudices (like me for example) would say that there have been major advances. In the context of a huge increase in the absolute number of extremely preterm infants, there are major improvements in survival with a stable proportion of impairments among the survivors. With an overall prevalence of severe disability among the survivors of 19% why do we persist in focusing on those infants? I know it is vitally important that those infants receive early evaluation and effective services and interventions. But surely we can also emphasize that 81% of these infants, who would all be dead if it wasn’t for NICU, are free of serious impairment.
The authors of the editorial appear to be of the opinion that it is OK for 81% of the potential survivors to die to avoid the survival of the 19% who have severe impairments. I beg to differ.
Neonatal Research 
You make a great and important point, Keith. I would like to contribute some insight about the meaning and value of the life of some of the survivors with severe disability. I think the parents of many of these children would not agree with the medically accepted view that their child was a useless or futile life, who should not have been resuscitated at birth. Doctors make assumptions about those living with severe disability– where is the evidence?
Greggy’s legacy is a life savoured
By Jack Tourin
On Sept. 17, 1980, my son, Gregory Mark Tourin, died. He was 17.
The usual outpourings of sympathy after such an event were somewhat muted. A few close friends commiserated deeply and sincerely, but others were more restrained. Some stated openly what they thought, while others implied it: “How can any parent mourn the death of a severely handicapped child, a child with nothing to look forward to? They should be glad it’s all over.”
Well-meaning people would say to my wife and me, “It’s a blessing, really.” Or “Your 17 years of sacrifice are over. Now, you can live for yourselves.” They failed to see the other side of this coin of sacrifice.
What mattered to us wasn’t what we did for him, but what he did for us.
Greggy was born severely mentally disabled. He also had cerebral palsy. He spent most of his life in a wheelchair. During the last five years of his life, he was almost completely immobile. The only parts of his body he could move were his toes and wrists. All the natural functions of life that others accomplished automatically had to be done for him. Yet watching this child struggle to gain satisfaction, saluting each day with wide-eyed wonder at what was to come, drinking in and enjoying the tiny portion of life that was allotted to him, gave us a reward that can’t be measured in normal human terms.
Taking care of Greggy was a 24-hour-a-day job. He was a very demanding child because the only means he had of controlling his life was his voice, which he used constantly. He learned to baby talk when he was five and talked this way for the rest of his life. During all that time, we never heard from that baby talk one word of self pity or bewailing his fate.
What we did hear were demands to see, do, and go.
Greggy wanted to see everything, go everyplace, and learn everything he could. His world was restricted to a far smaller portion than that given to most people, but that tiny share of life was a constantly unfolding vista of wonderment for him.
He could read signs and large advertisements long before any teacher attempted to teach him. We discovered this when we were going through the yellow pages of the telephone book with him. There he was, calling out the names of the companies that had the larger ads. The finer subtleties of reading always eluded him, but he was able to determine that the larger sign with the big red logo meant K-Mart and the one with the circles meant something else.
When life wasn’t interesting to Greggy, it was funny.
When I became cross with him, he would say, “Daddy get mad,” his eyes sparkling with anticipation, so I would wave my arms, pretending to be wildly furious. This brought a deep, heartfelt, natural, all-encompassing burst of laughter from him. No one but a mother or father can understand how it feels to hear that delirious sound of pure innocent joy. My wife Beatrice and I would hear the same happy sound when we acceded to his request of “Mommy and Daddy dance!”
Once, when we were driving and he was in the front seat next to me, I said, “Greggy, if you say one more word or make one more sound, I’m going to throw you right out that window. Understand? One sound and out you go!”
He looked at me with an impish grin for a moment and then blurted out: “Baaaaaaah!”
My wife and I sang to Greggy constantly. He memorized many of the simpler songs. During his last two years, he sang himself to sleep every night. He woke each morning laughing and demanding to savour more of life.
His favourite place to go was our local shopping mall, where he liked to look in the store windows and ask us to buy him things. He knew we couldn’t buy him everything he asked for, but he seemed to enjoy just saying the words. In his own way, he was improving his vocabulary and his reading.
On the morning of his death, at one o’clock, on his way to the hospital emergency room, Greggy asked to be taken to the mall. We had no idea that he had only a few hours to live. We were on our way to get the cough and congestion in his chest relieved so he’d be able to sleep. He had had worse chest colds than this and had always recovered quickly.
Whether Greggy realized that he was dying we’ll never know because he was cheerful and funny right to the sudden end. However, I’ve always suspected that he knew a lot more than we ever dreamed was possible. If he did know that he was going to die, then he had decided to finish his life as he had pursued it, developing what he had to the fullest and learning what it was possible to learn right up to his last breath.
After the funeral and the departure of our friends, Beatrice and I returned to our strangely silent house.
Our friends were wrong. It wasn’t over.
It will never be over until my wife and I are gone and the last echoes of his singing, his laughter, and his constantly curious voice are buried with us.
For six days, I sat in the living room and cried. At the height of my agony, I made some wildly emotional decisions. I was going to quit my teaching job. I was going to retire from life. I would never smile or laugh again.
As I sat there struggling with my grief, many unusual questions came to me. One of them was: What would Greggy have done in a situation like this, if one or both of us had died before him? I knew he would have missed us deeply, but he wouldn’t have buried himself in a morass of self-pity. He would have continued to use whatever limited ability he possessed to develop, to learn, and to drink his tiny cup of life to the fullest.
To honour Greggy’s memory, I decided to do what he would have done: study, learn, teach, and use my life in the best way possible.
After my son was gone, I was never able to recapture his closeness by sitting in my chair and weeping. However, over the years, when I looked into the eyes of some of my students and saw a look of magical curiosity or a sparkling sense of humour, a strange thrill went through me. I knew that for that one heavenly suspended second I had looked again into the eyes of Greggy.
Taken from: http://bloom-parentingkidswithdisabilities.blogspot.ca/2012/12/greggys-legacy-is-life-savoured.html
Thanks for the comment. you are absolutely right, there is plenty of evidence that doctors (and other health care professionals) ascribe a lower value to the lives of disabled children and adults than the families do themselves, and less than other members of the population. We make assumptions about disabled children that have no basis in the experience of the families concerned. There are occasional families who find their experiences more negative, and some of them have been very vocal, sometimes blaming neonatologists for their suffering. I think a more balanced view is emerging, including the story you reproduce in your comment. Life is not easy when you have a severely disabled child, but there are rewards, joyful times, fulfillment, and hope. Positive experiences which for many outweigh the difficulties. If we recognize that then maybe our society can find more resources to assist the families and their children. Rather than finding reasons for not treating them.
Keith
We are living in a time when parents can choose, before birth or in the NICU. Parents can reflect on their values and capabilities and assess what raising a child with disabilities means to them. In the past, these choices did not always exist. Maybe those who are vocal about negative experiences did not make informed choices.
Are there any studies that report on the perspectives of parents of preemies who survive with significant disabilities who made informed choices to continue care for their child? Are these parents having a miserable time and do they regret their choice? Or is it the case that despite their many challenges they would not have it any other way?
I wonder, if one was to reflect on the complete lives of these children (such as the essay about Greggy) how often the conclusion would be that it would have been better had the child not lived?
Keith…the willingness of many colleagues to diminish and dismiss the value of the lives of those judged to be severely developmentally impaired is astonishing. While I fully support complete informed consent when entering into conversations related to the resuscitation of infants at the edge of viability, we are living in an age when peers equate severe disability with death. This may be their view of the world but it is not the view of the world for many of our families. Having had the incredible fortune to care for large numbers of preterm infants in my general pediatric clinic in a prior life, I can unequivocally state that I never met a parent who wished their medically complex child had not survived. Life with a “retarded child with cerebral palsy” is not an easy one but these families all seem to accept at least two simple facts….there are no guarantees and we were never promised a life free from sacrifice. My families are not unique. And Barb, the work of Sigal and other investigators makes it clear that young adults and adults born extremely prematurely do rate their quality of life on a par with peers.
Neonatology has been the tip of the spear in many areas of research and quality in medicine. Unfortunately it appears we may also be the tip of the spear for utilitarians marching under the “quality of life” banner. The NICHD “estimator” was promoted immediately after Tyson published his data in the NEJM on morbisdity and mortality of ELBW infants in 2008. The impact of this publication has been variable,and the potential impact of the “estimator” (calculator) was never been assessed…though NICHD put it up on its website at the time of the publication. Tyson’s article added much to our knowledge but it did not support the publication of a calculator which many neonatal units have used to determine their resuscitation policies.
I can state that in many units percentages of survival and severe neurodevelopmental outcome based on the Tyson calculator determine whether infants are resuscitated or not. To restate, some units use this calculation to determine whether families will be offered resuscitation for their infant. This in and of itself is not objectionable. We know there are limits to our ability to resuscitate infants. What is objectionable is that more than a few units equate severe neurodevelopmental delay with death! In these units at a percentage for severe neurodevelopmental outcome or death, neonatologists will not offer families resuscitation. In effect these physicians have decided that death and severe neurodevelopmental outcome are equivalent!
We are living in a utilitarian medical world in which we are openly or subconsciously encouraged to appreciate the “greater good.” These are challenging times for physicians who see the incredible light of life, and perhaps soul, of all the patients they care for. I know the challenge and the gift families with a special needs child have been given. My daughter is moderately retarded. I never imagined I would have a 12 year old child who would not be toilet trained…but we do. That said, our family has been given a great gift. Everyday our daughter teaches us how handicapped we are.
I understand every family may not be able to accept such a responsibility Keith….and such a challenging gift. I do not expect all 23-24 week infants to be resuscitated. I do, however, expect our colleagues to love life and possess the humility to understand that the child they may not be capable of handling does not make it a life unworthy of being lived. I expect colleagues in those circumstances to speak honestly with families about survival and severe handicap, not equate the two, and allow families to make decisions which are in their best interests.
I said neonatology has been the tip of the spear in many areas…and perhaps in carrying the utilitarian banner. Now that we have a prematurity calculator what will be next? The Alzheimer’s calculator, the CHF calculator, the recurrent breast cancer calculator? They are doing cartwheels in the halls of cubby holes of government health care organizations struggling with how best to reduce costs for infants who cost so much and whose lives are…equated with death…by physicians.
Thanks very much for the comment Marty. I think that, as you say, we have been very remiss in neonatology to promote the use of predictions of death or disability as if they had the same implications.
I think there may be some good reasons for this, that is that everyone wants to improve ‘intact survival’, so we work to reduce the combined burden of death or disability.
But there are also bad reasons, able-bodied intelligent health care workers, especially those who spend their lives in intensive care, only see the crises in the families’ lives, and not the day to day happiness they may experience. They suppose that families have the same negative view of impaired lives that they themselves have developed, so we present the numbers of deaths and of impairments to potential parents combined together, which is not appropriate. When so much disability among former preterm infants is minor or moderate, and even severely impaired children have a good quality of life.
I also don’t think that every baby should be resuscitated, gestational age is only one part of the puzzle, there are babies at every gestational age that we need to discuss treatment options with the parents, and I think that sometimes it can be in the child’s best interest to have limits put to the intensity of their care, but the calculators and things like the awful new Canadian Pediatric Society position statement (more about that in another post) reduce these appallingly difficult situations to a single number, or a simplistic threshold.
I do think we need to find strategies to help our colleagues to recognize the value of the lives of others, whether they have disabilities or not. I think Annie’s research with Barb Farlow has been quite influential already, we need to do something along the same lines for the ex-premie! I don’t quite know how at the moment, all suggestions gratefully accepted.