Siamese
I saw in the news a couple weeks ago that Siamese twins were surgically separated somewhere in the U.S.A., and that reminded me of my own experience with Siamese twins. My first brush with them was actually my roommate's experience in medical school. He was at a supposedly fairly routine cesarean section for twins when the attending obstetrician said matter-of-factly, "Hey, they're Siamese," which seemed like an anticlimactic way to describe an extremely unusual occurence.
I have personally seen Siamese twins only once. The babies were born at 26 to 27 weeks gestation - about 13 weeks early - and we knew from prenatal ultrasounds that many of their internal organs, such as their liver, were shared, although their hearts were separate. I was the attending neonatologist at their delivery and subsequent admission to the NICU. When they were born it turned out they were fused from their chins down through their chest. Their heads were thrown back by the fusion, their necks permanently extended. They were born alive, with beating hearts, but without much breathing effort or other signs of life. What to do? Their heads and neck were such that it would have been difficult to intubate them and put them on ventilators if we wanted to. We didn't know if their lungs were fused or anomalous in some other way, besides being premature. It was a number of years ago, and the survival rate for babies at that gestation, which is now about 80% to 90%, was less then, and much less for very premature babies with major birth defects. The parents were aware that they might be so severely affected they might not survive.
Should I give them a try? Should I attempt to put both on ventilators and call our pediatric surgeons to try to sort out the awkward mess of fused organs? Should I start treatment that, if they survived, would lead to months of hospitalizations, several surgeries with painful post-op periods, and a next to nothing chance of anything near a normal existence?
No. I let them die a quiet, peaceful death.
I have personally seen Siamese twins only once. The babies were born at 26 to 27 weeks gestation - about 13 weeks early - and we knew from prenatal ultrasounds that many of their internal organs, such as their liver, were shared, although their hearts were separate. I was the attending neonatologist at their delivery and subsequent admission to the NICU. When they were born it turned out they were fused from their chins down through their chest. Their heads were thrown back by the fusion, their necks permanently extended. They were born alive, with beating hearts, but without much breathing effort or other signs of life. What to do? Their heads and neck were such that it would have been difficult to intubate them and put them on ventilators if we wanted to. We didn't know if their lungs were fused or anomalous in some other way, besides being premature. It was a number of years ago, and the survival rate for babies at that gestation, which is now about 80% to 90%, was less then, and much less for very premature babies with major birth defects. The parents were aware that they might be so severely affected they might not survive.
Should I give them a try? Should I attempt to put both on ventilators and call our pediatric surgeons to try to sort out the awkward mess of fused organs? Should I start treatment that, if they survived, would lead to months of hospitalizations, several surgeries with painful post-op periods, and a next to nothing chance of anything near a normal existence?
No. I let them die a quiet, peaceful death.
posted by neonataldoc at 7:56 PM
15 Comments:
A quiet peaceful death can be a good thing, but did the parents contribute to this decision or did you arrive at it on your own?
If I were the parent of these "conjoined" children, I would want to make that call.
When you say "not much effort" are you saying they were not making respiratory effort or they were making no respiratory effort?
I don't doubt you (or you and the parents) made the right decision. I might have made the same one.
But if the babies were trying to breathe, it's a little bit of a stretch to characterize their deaths as "peaceful". Did you sedate them?
best,
Flea
Sorry - not enough coffee! I should have asked "Were they making some respiratory effort or no respiratory effort? The difference matters, I believe.
Flea
NeonatalDoc, I understand what you're saying and I don't think I disagree with the decision you made... but...
Would you have made the same choice as quickly for a baby (or a pair of babies) born premature with, say, severe arthogryposis, HLHS, Tetralogy of Fallot, severe esxtrophies etc? How about now, with today's advances in medical support and assistive technology for long-term survivors?
It wasn't all that long ago that Baby Doe was starved to death because he was born with Down syndrome and esophageal atresia. The question is, who draws the line?
Reflecting overnight, I admit, I'm a bit troubled by what you have written. I have been with a child through post-op, several times. Maybe some surgeons and teams are better than others, but at our facility, children are kept comfortable. Bottom line: pain is not good for recovery.
I have never liked the excuse of "painful" post-operative periods to deny life to a child (or possibly in this case, deny life-saving treatment). Besides, it doesn't last long. Painful post-op (assuming there are places that allow children to suffer miserably)is temporary. Death is permanent.
Without more information doc, it's difficult for me to push thoughts of Groningen out of my mind. Help me out here. What is this story missing? Did the parents want to let their children die peacefully, or not?
In neonatology, it is not so unusual to have a newborn with severe and highly probable fatal anomalies be allowed to die during the transition period because someone makes a hard call. Who makes that call should be a discussion that occurs before birth otherwise, the call should be "do everything". But to get back to why I wrote in the first place, micropremies appear comfortable because they have no secondary apnea reponse; i.e. they don't know they're asphyxiating. To Armchair, while there would be nothing wrong with sedating such patients, there's no access initially and I have to ask where you're going to find rapid access in the conjoined siamese twins that NeoDoc describes? I have also been the presiding doc for my share of these cases (severe anomalies, not necessarily siamese twins) and I don't know about "peaceful" but relatively quick and certainly "natural" in the sense that the patients were not meant to survive. Quick because there's usually a reason why they never take a first breath.
Cherubsinthelandoflucifer.com
Just in case I ever visit your city, wherever that is, and I happen to deliver a very sick baby that you think would suffer in life no matter what, then please know way ahead of time that you have my permission to do nothing. And if you don't ask my permission but do the right thing anyway, I'm cool with that, too.
Thanks, everyone, for the great comments. My post of April 1 will address many of the comments. It does not, though, address the issue Flea raises, about their breathing and comfort. I don't remember everything about their breathing, but they were not struggling. They died quietly and fairly quickly. I admit I don't know if that means peacefully. Should I have given them morphine to ease their suffering? Maybe. But that could also hasten their death by depressing their breathing effort. Also - and this is a lousy excuse - this occured before the emphasis of the past ten to fifteen years about addressing neonatal pain.
Becca, I think most of the situations you bring up should be dealt with on an individual basis. I'm not trying to dodge the question; it's just impossible to decide many of these things without specific information about the case.
Dr Cherubs(etc),
I don't know that these conjoined babies could have been saved and if not, the question of should have is not really at issue. I did think doc's post was a little provocative - it had a "punch" to it, and that is what I'm taking issue with.
That is, it wasn't a foregone conclusion that these children would pass quickly and quietly, and that was the point. Doc made a decision that they would. He is telling us that he made the decision, not that the outcome forced itself on him.
Again, was it the right one? I don't know. If I was mom, however, every single detail of this decision would be important to me. To us, on the outside, it's just an interesting case. But to some mom and dad out there, it's their very hearts.
Maybe it is merciful to make such a call without them? But maybe it is merciful to choose to delay death (if possible) so that parents can be absolutely sure their babies will not live and that they have done all they can do. After all, they have to live with the decision too.
I'm sure doc will clear it all up in his next post.
I have been thinking about this post all day. I think you did the right thing. These babies weren't meant to survive. As a parent, I just don't think I could have made that call fairly. I think it would be easy to say, "save the baby at all costs" without really knowing what that means. One the other hand, the difficulty of their future life shouldn't really be an issue as to who lives and who dies.
I have said it before, I think the key is listening to the babies (or the kids) and understanding when their bodies are ready to give up that fight. You did that. Could a new parent know to do that? I sure don't think I would have.
You made a good decision.
P.S. This is what I like about your blog-you always give us important issues to think about.
I think you made the right decision and your post did not cause me to lose any sleep.
In surgery I find myself explaining time and time again that I am not the executioner. Yes, we can take an elderly dying patient with ischaemic gut for a laparotomy knowing that they have almost no chance of surviving a week and certainly not enough physiological reserve to ever make it out of the hospital. But for what?
We can amputate the ischaemic and non-viable lower limb of a nursing home resident and perhaps extend their life by a few weeks or months.
We can do a laparotomy on a patient with advanced metastatic cancer that has caused a bowel obstruction, knowing that the patient will die very soon anyway.
The surgeon needs to know when not to operate. The neonatologist needs to know when not to resuscitate. Yes, we need to let patients and parents participate in the discussion and make an informed decision. From time to time we perform operations and resuscitations that are not, in our professional judgement, in the best interests of the patient. Because the stated wishes of patients and parents can be extremely persuasive, even though we suspect or know that they are making decisions based on emotion, not reason.
Ethical quandaries indeed... you can lose sleep over them or you can learn to make your decision and accept it for what it was. I prefer not to make my life a series of "what if's".
Unlike the majority of those who read your post, I have seen conjoined twins in very similar configuration to yours. Ours were near term, their chins weren't fused, and intubating them was a major endeavor requiring 3 physicians, 3 nurses and a couple of respiratory therapists. They very nearly didn't survive the intubation and they didn't survive the workup to determine whether or not they could be separated. Bag/mask ventilation simply wasn't going to happen with ours. Obviously it wasn't possible with yours either.
I think it's important for people to understand that chest compressions as a part of resuscitation would have been impossible. You had to either intubate immediately or not at all. Not an easy call to make ever, but I think you made the right one.
Dream Mom: Thank you. I hope you don't mind, though, if sometimes I write about less weighty issues.
Barb: Thanks. Just because we can do something doesn't mean we should. And if you don't want to make some hard decisions, don't go into medicine.
Judy: Excellent point. Thanks.
Who makes that call should be a discussion that New Trends
occurs before birth otherwise, the call should be "do everything".
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