Friday, February 24, 2006


In a recent post entitled "Inappropriate", I discussed a baby with holoprosencephaly and opined that it would be the best thing for him to die in his sleep, without a home monitor to help prevent that. Several people who left comments mentioned the issue of quality of life, implying that quality of life is a factor in deciding whether to withdraw or withhold life support for an impaired child.

It is my understanding of the laws in the U.S., though, that we are not allowed to use poor quality of life as a reason for withholding or withdrawing care. We cannot discriminate against people with impairments; withholding life support would constitute discrimination.

We are allowed to stop support, however, when continued treatment is futile or prolongs suffering. And there is the rub: how do we determine futility? And do we sometimes say we are stopping support because of futility when we are really stopping it because of quality of life issues?

For example, we are not allowed to withhold life saving treatment from a child with Down's syndrome, a chromosomal defect that causes mental retardation and other birth defects. However, we are allowed to - and usually do - withhold treatment from children with Trisomy 13 or 18, which are also chromosomal defects that cause mental retardation - a much more severe retardation than that of Down's syndrome kids - as well as other birth defects.

Eighty or ninety per cent of babies with Trisomy 13 or 18 die by age one year. They often die because they simply stop breathing; the respiratory center of their brain, responsible for regulating their breathing, doesn't work properly. They can also die because of other birth defects, such as serious heart problems. If a normal child were to stop breathing, we would begin support with a ventilator. This is not usually offered Trisomy 13 or 18 patients. We say we don't offer it because it would be futile; the baby would eventually die from another problem caused by the Trisomy. But the truth is, many of the Trisomy 13 and 18 children if put on a ventilator, if their heart defect were repaired, and a feeding tube placed, and anti-reflux surgery done, could possibly live for years. It would not, in my opinion, be a life of high quality (and I am not advocating it be done.) Most of these patients cannot even smile, let alone walk or talk or indicate their desires, but they would be alive. So when we withhold life support from them, are we really doing it because it would be futile, or because the quality of life would be so poor?

In reality, the distinction between futility and poor quality of life is not so clear.


Blogger mary said...

Trisomy 13 family stories.

Thought I'd throw that your way, for an alternate viewpoint. Just some food for thought.


"Emily's mom" (trisomy 21)

7:05 PM  
Blogger That Girl said...

What the law says and the actual reasons for our actions fall in that gray area - the area that few people want to discuss or even think about.
Some people make the case that parents and family are the least likely to be objective about care - they have other motives (their time/cost/sense of personal futility).
I find it amusing that a lot of parents of children with disabilities are the first to step up and protest against suggestions that we not prolong the lives (through ventilation, etc.) of severly disabled children because their children have defied the odds or some children have defied the odds.
What any honest parent of a child with severe disabilities will tell you is that it is exhausting, frequently gut-wrecnhing, stress-inducing, expensive, and prohibitively time-expensive to raise a severely disable child.
That said, parents who dont want to raise them, have reached the end of their rope/hope - why should they be denied the option of not continuing?
Some would answer that that is the worst reason to deny care.
But debating the answer gets us into the gray area of practicality vs. idealism - i.e., what is the life expectancy of a severely disabled child who is a ward of the state?
We have not (as a society) come up with an answer to many of these gray area questions which leaves us in this ethical no-mans land.
Personally, I think the balance we have right now where the decision rests between the parents and the doctors is okay.

7:59 PM  
Blogger neonataldoc said...

Mary, thank you. It was interesting to see children with full trisomy 13 smiling.

That girl, your comments are so true. I think most of society does not even think about these gray area questions.

10:24 AM  
Blogger clara said...

NeonatalDoc, I appreciate you really exploring these ideas.

I experienced this exact situation, my son lost oxygen during delivery and lived for 7 weeks with severe HIE. We had many discussions with neonat. about quality of life, futility & all of this. We had a DNR right from the start, but our faith (Catholic) made this easier for us. Not that losing our son wasn`t a huge test of that faith.

Anyway, he had a will to live that we respected and he breathed on his own. We supported him and made him comfortable and held him all day every day. As he moved to the PICU, we had another conversation about futility and met with the ethics board. We had always felt the Catholic belief that humans weren`t meant to live on machines, so as breathing got harder for him, we slowly let him go in a low tech way. These actual issues were not hard for us to navigate, because of our views on life and death, we worried more about "forcing" him to be here, and when it seemed he was ready, we supported him as he left, as a parent would do for their child.

I agree with ThatGirl, the way this is currently done seems fine to me, between doctors and parents. I had doctors and nurses that encouraged us to be with him and love him and because we treated him like any other loved child, I have no regrets. Of course the loss is permanent and life changing though. I would encourage all doctors to make sure parents spend time with their very sick babies.

9:46 PM  
Blogger neonataldoc said...

Thanks, Clara, for your comments. I'm sure it was a tough time, but it sounds like you and your family were comfortable with your decisions.

2:14 PM  
Anonymous Anonymous said...

"That Girl" I must say, as the parent of a child with what society deems a "severe disability", I dont appreciate you putting words into my mouth or the mouths of the hundreds of other families I work with who have the same disability. I am an honest person, and in my honesty I will tell you the words *I* choose to use regarding my, spirit-enhancing, love-building, trust requiring, spirit soaring, gentleness, equality, courage, strength, depth, humanity, kindness, unconditional, unwaivering, fullfilling...I could go on and on.
The only things so far that I have found "exhausting, frequently gut-wrecnhing, stress-inducing" are societies impressions of my life and their placement of little value on the life of my child.
The rhetoric that says that we are militant, angry moms and dads (you know, the kind who amuse you when we "step up and protest") may be your opinion based on watching us have to fight for the right to have our CHILDREN seen as worthy in the eyes of the doctors and nurses who so often try to take away any chance at hope we have. Fighting for medical care should not be what we waste our energies on. But somehow, through all the bitterness of knowing the world sees our kids as "less than", we are able as a group to find more good in the world than bad, more decency than cruelty, and more love in the living of every minute than many people with high IQs and higher salaries will EVER see. We treasure every minute, every single accomplishment, every breath of life. It is called love, and it is the best gift any of us have to give the world. Unconditionally, no holds barred, perfection or imperfection.
PS Doc, keep thinking about these things, they truly are important. But dont forget that behind every parents decision making is a WHOLE lot of love and a whole lot of suffering to make the right decision when there are no good outcomes, only better outcomes. We who have disabled kids appreciate every ounce of yourself that you pour into understanding them (and us) and recognize that you too want what is best for them.

8:18 AM  
Blogger Dream Mom said...

I have to agree with anonymous in the sense that I too, was outraged at that girl's post. While I do agree it is exhausting work at times, I disagree with her suggestion that those who have reached the end of their rope/hope should be the allowed the option of not continuing.

I have written in previous comments about the challenges as my child reaches the end of his life. While his quality of life is not what it should be, my comments center more around listening to "his" body at the end due to his suffering than choosing not to go on because he can't do as much. I had hoped I had made that clear.

After that girl's post, I wrote a post but never published it in response to that girl. I think the larger issue is that we need to do a better job at helping parents of disabled children and I outlined things we could do to help them. I will publish it today on my blog.

11:36 AM  
Blogger neonataldoc said...

Thanks Dream Mom and anonymous. These are tough issues. Thanks for the encouragement to keep discussing these issues - but some days I'm going to have to lighten up and talk about the good things!

8:17 PM  
Anonymous Anonymous said...

I can share a few good things with you, hows that?

1) tonight my daughter giggled herself to sleep because a friend teasingly asked her if "her daddy went to the vet for a checkup"
2) I got a note home from her school actually thanking me for "letting" them be a part of her life
3) Her echocardiogram revealed a VSD that is ALMOST healed...wooohooo.
4) her big brother read her a bedtime story and laid in bed with her letting her sing to him in her warbly, offkey voice, and told her it was beautiful...and meant it.
5) the work you do, wether it be today or tomorrow or next week, will save a child's life and allow their family to experience the same depth of love that we enjoy here in my home.

8:59 PM  
Anonymous armchair ethicist said...

Neonatal Doc,

Thanks for your blogging - I just recently found you.

Medical futility is a topic that really scares me -- to the core. I'm not sure how I feel about your discussion - and revelation - that it's not so clearcut of a concept. On the once hand, it reassures me. It means there is still room for discussion, that even with physicians making decisions, perhaps there is some room for advocating for a child who may not have the standard of quality of life that particular physician needs to see. On the other hand, it scares me.

As a neonatal doc, you don't see the children much beyond their time with you, I'm assuming. A baby with trisomy 13 is just that, a baby. She may no more smile at 1 week (or 2, or 3 weeks) than a genetically perfect child at 1, 2, or 3 weeks. Is mental retardation *in* an infant "poor quality of life" at that time? When does it become an issue of "poor quality of life" for the child? How do we know?

So it seems that one has to look into a crystal ball and make prognoses about the future to attribute current "quality of life" standards. But how can a neonatal doc do that? Do you rely on other specialists to tell you what a child with say, hlhs, or hydrocephalus will be like when she is 10? Do you ever consult with families to learn about what a diagnoses could mean down the road?

Please don't interpret these questions as "grilling" -- this is an area I am genuinely concerned about and would *value* your input.

9:56 AM  
Blogger neonataldoc said...

Thanks, anonymous, and good questions, ethicist. I usually don't see my patinets in long term follow up, unless the parents bring them to the NICU for a visit, but I remember some older kids with disabilities from my days as a pediatric resident. There are, of course, a wide range of disabilities. I remember two older kids with either trisomy 13 or 18, and they didn't look so good, but that's just my opinion. They weren't smiling or laughing.

As far as consulting with parents of children with disabilities - this is one reason I like these blog comments, because people like Dream mom and anonymous really have a lot to offer us health professionals.

2:13 PM  
Anonymous Anonymous said...

I am not the other "anonymous." Just call me "anonymous 2" if you'd like. I believe that anonymous 1 is talking about a child who has Down syndrome and is otherwise healthy. It is true that some consider this a "severe disability" but I do not believe that in this context, neonatal doc was including children with Down syndrome in his discussion of children with severe disabilities. I do not want to speak for the doctor but I wanted to point this out.

That said, it makes all of us who have children not classified as "normal" understandibly queasy when we hear of life-preserving actions routinely taken on "normal" children but not those classified abnormal or with prognoses that are statistically poor. Neonatal doc, we who have children with Down syndrome sure hope that in contexts such as your fifth paragraph, children with Down syndrome are thrown in with the "normals" (and I believe, from what you've written, that they are, but we're not sure how far that extends when/if complicated issues arise.) It makes us equally squeamish to contemplate where a line should be drawn, with our children just on one side of it and less fortunate children just on the other. At the very least, though, when unable to mentally tackle the entire issue, we feel compelled to speak up in behalf of our own children. It is unclear to us, upon meeting smiling medical professionals who seem to have our children's best interests at heart, whether the underlying, unspoken (heaven forbid that anyone would be so candid to our faces) assumption is that the child (in an idealistic sense) shouldn't be here at all or should not have been as aggressively brought over a particular hump or on the contrary, whether the smiling professional believes, as we do, in the worth of our child's life, imperfect as it is in the eyes of some. Pardon my sensitivity, and I wanted to clarify what might have been a little unclear with Anonymous 1's post.

3:17 PM  
Anonymous Glenda Parkman said...

Because of my inexperience as a blogger, and, possibly, because of the very long days I've put in lately, I managed to place my reply to this portion of your blog with the comments for another story (on home births, I believe). My apologies. Here I go again.

My name is Glenda, and I have a 20-year-old daughter named Rebecca who has a translocation trisomy. This translocation consists of part of the long (q) arm of chromosome 13 and part of the long (q) arm of chromosome 15. These portions are combined to make one extra chromosome in all of Rebecca's cells. When Rebecca was born, she had a "funny-sounding" heart (due, according to the pediatric cardiologist, to the fact that one wall of her heart was thicker than the other). This was determined by echocardiogram after birth. Rebecca also had Trigonocephaly, an extra toe (complete with bone and joints), severe jaundice, clenched fists, lowset ears, and several "strawberry" hemangiomas. (The term "strawberry" was used by the doctors present.) In spite of these anomalies, no genetic tests were ordered, and we were at one of the largest hospitals in the country. By the time of Rebecca's repeat "echo," at about 8-weeks-of-age, there was no sign of a heart problem. We give God the glory! The Trigonocephaly was surgically corrected at 8-and-1/2-months-of-age (with no worries about heart issues!), and her extra toe was removed by a hand/foot surgeon while she was under anesthesia for the Trigonocephaly surgery. The large umbilical hernia she developed shortly after birth healed over time without surgery. Rebecca also developed strabismus in early childhood, and this was surgically corrected at 5-years-of-age. She began to develop scoliosis in later childhood, but it never became enough of a problem to warrant surgery. Other issues with which she's dealt are sleep pattern problems (the lack of a sleep pattern, actually), autistic tendencies, and developmental delay.

Presently, at age 20, Rebecca functions about like a mid-to-upper elementary schooler academically and somewhat lower socially. She reads fluently on a a 5th-grade level at least, can perform arithmetic up to the level of basic fractions, can tell time and count money, and has some of the neatest (if somewhat large) cursive handwriting you'll ever see. Rebecca also loves singing in church and knows the words to almost every chorus, and she loves to play baseball on her special needs team. She can hit a machine-pitched ball out of the air. Rebecca has, more and more, overcome the autistic tendencies and learned to make better eye contact and broader conversation. (Just don't get her started on baseball!)

Rebecca was not diagnosed until the age of 12, at the suggestion of a sleep clinic pulmonologist who thought her medical history too complicated not to indicate a syndrome.

I homeschooled Rebecca until a couple of years ago (along with her brother, until he was in 9th-grade), and she now attends a private, special needs day program focused on academics and life skills.

I am so glad that we did not know Rebecca's diagnosis until she was older. I know so many families with T-13 kids (Full, Partial, Translocation, and Mosaic), and the majority of those who found out prenatally were encouraged (even pressured) by doctors to abort. Many of these kids can sit up, crawl, cruise, and/or walk (some with help, some unaided). Others can babble, sign, use PECS (a picture-based communication system), type, and/or talk. All but the few most profoundly affected can smile, coo, laugh, respond to (or play with) simple toys, and can definitely recognize family members and close friends.

There are almost 60 of these amazing survivors on a website at There you'll see stories and pictures of these babies/children/adults posted on the Children Living With Trisomy 13 Album. One Partial T-13 woman is 47-years-old!

There is also a Treasured Memories Album there, devoted to honoring the T-13 children who have already gone on to be with the Lord. The postings on this album tell over and over of parents glad to have had even a small amount of time to hold and to love their child and to make memories.

Your comment about yourself basically indicates that the longer you practice medicine, the greater your realization of your need to know more. Please don't go another day basing your beliefs about a T-13 person's ability to experience "quality of life" on the few dismal pages found on T-13 in medical books and in journals and on the few patients with this diagnosis you'll encounter in your years of practicing medicine. Look at our lovely, amazing kids and be educated by the people who know first-hand about T-13.

Glenda Parkman

12:55 AM  
Anonymous Anonymous said...

Anonymous ny
As a first time blogger, i have read all the blogs and thank everyone for their imput. But now i would like to add a little of my own, as a mom of a beautiful trisomy 13 baby myself i would like to ask the medical profession to take a little time to study these children again and i dont mean from their text book the 3 or 4 pages written, maybe talk with the families who have lived and continue to live with these children as far i can tell we are asked to sign D.N RS before we even get to hear what problems the have ,and everything that comes about is associated with the disorder even if it's really not i would also like to add that 'incompatable with life' be erased from medical journals , if we were all to listen to society nobody would add up as some are to fat. to skinny ,to much hair, to little and so on. my son is very compatable hes truly happy hes truly loved. So far he's had to have some surgeries to help him but has'nt a lot of regular kids also? why are our children considered a waste of time and money by some dotors { not all}im hoping my doctors were not the norm , I hope nobody else was told to go home they had two other children your just sitting here waiting for him to die just let him go , eight months later at home im still holding my son but now hes a lot more active so i guess what im trying to say is that parents are not looking for doctors to cure our children but to treat them with the knowledge and respect the treat any other child if parents are willing to care for their children with love and hope ,why would any doctor have a problem with this?
thank you.

8:24 PM  
Blogger Nichole said...

As a mother of a child with severe disabilities and a very rare chromosomal condition (12q deletion), I had to comment here. We were told our son would likely not survive the birth, from a geneticist who was basing this from only a couple of case studies. Yet here we are, 16 months later holding our amazing little one. We have been asked numerous times by the medical community if we would sign a DNR for our son. And before his birth this was all quite confusing for us. But after one look into his eyes, we knew we had to give him every chance we would give our other healthy children. Interestingly enough, even though his case is so rare that the medical community really doesn't know what to expect, we are still offered the choice to withhold treatment from him.
Yes, his care can be exhausting, and he spends more time in hospital than at home, but he brings more joy than I ever knew even existed. Yes, he can't crawl, walk, talk, eat or grow, but he is content and always smiling. And so who is to decide whether our son has "quality of life"? If the laws don't allow us to use poor quality of life to withhold treatment, then how can we be asked to withhold treatment? How can anyone know in an extremely rare case such as ours, whether the care is futile? Our son's days are unknown, just as mine or yours are unknown. And so we will continue to fight for our son to receive the best medical care possible, futile or not.

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