Tuesday, February 14, 2006


Recently we sent home a baby who had holoprosencephaly. At first we thought he might just have a cleft lip and palate, but his eyes were a little too close and his head a little too small, and unfortunately a head ultrasound showed the holoprosencephaly. I say unfortunately because holoprosencephaly is a devastating defect of the brain, where the brain never divides into two hemispheres but remains a poorly functioning rudimentary brain. Children with it are severely impaired.

I discussed this finding with the mother and her father, the baby's grandfather, who are very nice people. Although I tried to be gentle, I was pretty explicit with them, using phrases like "he won't walk or talk." They took it about as well as you would expect a family to take it.

A few days ago I saw a pediatrician who had seen the baby as an outpatient. The child, not surprisingly, was having multiple problems, including seizures, dehydration, and an inability to keep his temperature up. But the part that bothered me was that some physician had put this baby on a cardiorespiratory monitor at home, for the purpose of preventing a death when the baby was unattended. This act, to me, was simply an abdication of responsibility. The best thing that could happen to this baby would be to pass away peacefully in his sleep. To provide a monitor not only prevents that (maybe), but also give the parents false hope. The pediatrician told me the parents were being very unrealistic and thought this baby was going to be fine. The response to that should be to keep talking truthfully but empathetically to them, not to buy into their unrealistic hopes.

Don't get me wrong. I'm not someone who thinks all impaired children should be killed or that we need to obtain an eugenic society. But prolonging a dismal life is no good either. Sometimes the best we can do for a patient is not so good, but merely the least bad thing. Whoever prescribed the monitor took the easy route in the short run, acquiescing to the parents' request instead of taking the time to tell them why a monitor was inappropriate.


Blogger Dream Mom said...

Your post today hit home with me. I totally understand your position and appreciate your honesty with the family.

I have a Special Needs child who now has a degenerative condition related to an rare gene. He is declining and is bedridden. He has never been able to talk or walk, is not toilet trained and has had a lifetime of seizures (had a VNS and is on 7 seizure meds). I hear him choke at night and I am up a lot turning him over to make him more comfortable. I have been told by our ped neuro of 14 years that he will not recover.

I do know that there will come a day when I may have to call 911. It will be the end. I often think about whether or not I should put a DNR in place. Not having a DNR in place will prolong his life when his body can no longer go on. I know this because I see this. It breaks my heart. He is deterioriating and I know it wouldn't be fair.

But putting a DNR in place is very uncomfortable to me. I don't think I can do it. So I stall. I pray that the Lord will take him swiftly when the time is right so I don't have to make that decision. It's a coward's way out. I also think about what will happen if I don't. When they hook him up to a ventilator and the agony for him goes on.

I remember a conversation that I overheard in my son's hospital room with our geneticist. He recieved a call on his cell phone; he was talking to this person who I am guessing was another physician and he said that this particular patient needed to have a DNR in place a long time ago. When he got off the phone, he explained to me that the patient was severely disabled and that her body was giving out. He talked to me about listening to that message.

So, today, when you talk about what's best for the child, I understand you. I hear you. I know you are right. But, it's very hard as a mother to make that choice.

Thank you so much for being honest with these parents.

8:35 PM  
Blogger Fat Doctor said...

I've thought this before but could never put it in words like you have. Thanks, Neonataldoc, for thinking of the child's pain and suffering, not society's burden, when discussing his future with his family.

12:41 PM  
Blogger neonataldoc said...

Thank you so much for your wonderful comments. My heart goes out to you. I can only begin to imagine the tremendous heartache this must be. Believe me, I don't blame you for having a difficult time putting a DNR order in place. It's a lot to expect of a parent, to say it's okay not to resuscitate their child.

I have read your blog and encourage you to keep it up. We need to see the perspective of parents of special needs children. In addition to enriching us, I hope the blog gives you some comfort or relief. I'm linking to it from my blog.

Best wishes in everything.

7:49 PM  
Blogger Dream Mom said...

Thank you.

6:02 PM  
Blogger mary said...

The best thing that could happen to this baby would be to pass away peacefully in his sleep. To provide a monitor not only prevents that (maybe), but also give the parents false hope. The pediatrician told me the parents were being very unrealistic and thought this baby was going to be fine. The response to that should be to keep talking truthfully but empathetically to them, not to buy into their unrealistic hopes.

Unrealistic hopes of what? That he not die in his sleep unattended? That mom gets a chance to stimulate him to breathe?

Dream mom's thoughtful response tells us about a child with a degenerative disorder and the agonizing waiting state she is in. The child Neonatal Doc is describing, though, does not have a degenerative condition. He does have a very signficantly disabling condition which will include serious cognitive impairment.

The baby described may have lobar holoprosencephaly, the most severe kind. But morbidity and mortality rates change over time as people treat the associated disorders. I see you describe a child with two eyes and a nose, although a cleft palate. He may surprise you and live past his first birthday. Heck, I know a child with the most severe kind of associated facial anomolies as well as lobar holoprosencephaly who is still alive at 3 years old. And mom, she doesn't seem to mind having her baby there.

Just a thought. Maybe the monitor just makes mom more comfortable. Maybe this is mom's call?

Current understanding of the life course of HPE is: we don't. Understand much, that is. As these children's associated problems are treated, they are surprising us (see: http://www.emedicine.com/RADIO/topic347.htm as well as http://www.geneclinics.org/profiles/hpe-overview/)

You have been truthful to mom. It's your job to present truthful information and your best prognosis, which you did. It's not your job to force her to give up her dreams. Her dreams and hopes belong to her, after all, don't they?

7:19 AM  
Blogger neonataldoc said...

Thank you, Mary, for your comments. I respect your opinion, although I don't agree with all of it. There is much we could write about this topic; I'll keep my comments brief for now.

If the mother's hopes and dreams were only to be with the child when he dies, or to stimulate his breathing, I would not mind the monitor so much. But her hopes and dreams were that he would be cured of the holoprosencephaly and turn out normal or near normal. No matter what kind of progress we are making with holoprosencephaly now, this child will never turn out any better than severely impaired. As I said before, I'm not into eugenics, but we do have to be realistic.

Also, It's not just about what the mother wants. We also have to think about the baby and whether we are prolonging suffering or not.

12:16 PM  
Blogger PaedsRN said...

Nice post, good discussion. My pick of the week from this edition of Grand Rounds.

Linked from this GeekNurse post.

3:27 AM  
Blogger Becca said...

But, NeonatalDoc, is this baby honestly doomed to suffer?

Yes, seizures are not fun, but is the distress his or his mother's? Is there any reason to think that they will not respond to the right anticonvulsant, eventually? Will he always be dehydrated or is this a transient feeding problem that might be resolved by changing formula or having a g-tube placed? Will the temperature control issues resolve somewhat as he grows bigger and older, and are they uncomfortable for him or just known not to be terribly healthy?

Mum and dad will, at some point, accept that their son won't walk or talk, but they'll do so in their own time, and it is after all very difficult to be sure with a very young baby, even if he does have a serious brain abnormality.

Baby has some problems, yes, but is he actually suffering? Do any of them hurt?

I think sometimes physicians can get a little confused between quality of life (how happy and comfortable he is: the sky's the limit) and functional ability (which can obviously in this case be predicted to remain rather low).

I think it's fantastic that you were able to send him home; he's clearly got loving parents and for all we onlookers know may enjoy a (relatively) long and comfortable life. Thankyou for giving him the chance to do so.

12:46 PM  
Blogger That Girl said...

Thanks so much for your post! As the mother of a disabled child I am totally behind you.
Parents can always choose to have hope despite dire medical warnings but to temper medical warnings for the sake of "feelings" is harmful.
The reason that parents get to make the ultimate decision is for exactly the reason illustrated in the comments - different people have different ideas of what life is.
My greatest fear was never that my son would die but that his quality of life would be so comprimised that it didnt meet my definition of life at all.
Thanks for advocating for your paient in the most caring way you can.

9:52 AM  
Blogger neonataldoc said...

Thanks to all for your thoughtful comments. Becca, your points are well taken. I don't really know if the holoprosencephalic baby is suffering, although if we become too aggressive with treatment (surgeries, needle pokes)we can cause suffering. Whether that suffering makes life not worth living is hard to say.

That Girl is very true when she says: different people have different ideas of what life is.

Regarding quality of life versus functional ability, etc., this is a huge subject. Rather than write about it here, I'll save some of those thoughts for future blog postings, so I don't run out of topics!

12:29 PM  
Blogger mary said...

No matter what kind of progress we are making with holoprosencephaly now, this child will never turn out any better than severely impaired

Very true, but not my point.

I hope you will blog more on this subject; particularly if you can think about the statement above and how you have made a value judgment. Please don't mistake my own statement as condemnation-you can't hear tone of voice in a blog comment. :-) But "quality of life" arguments disproportionately affect people with severe cognitive impairments--and those who love them. I am interested in seeing how things look from a neonatalogy POV.

6:46 PM  
Blogger wunderkind said...

neonate student nurse here:

Your post reasonated within me how we are on such a quest to save lives that we are forgetting dignity. Even a child deserves dignity.

i dearly love my babies - i cherish dignity.

1:37 PM  
Blogger neonataldoc said...

Thanks, Mary and Belinda. Interestingly, Mary, I wrote today's (2/24/06) posting before reading your comments.

3:25 PM  
Anonymous Anonymous said...

Neonatal Doc,

I just noticed this today. It's nearing 4 yrs when I was at my level 2 u/s nervously & excitedly waiting to find out what gender my baby was going to be. Well, I only had concerns of a cleft lip & cleft palate since I am with one. I couldn't imagine anything much more worse than that.

It was my nightmare that really came true because at my level 2 u/s everything was fine except the brain with severe facial anomlies. They considered it holorosenchephaly the severe alobar kind. She also had the 18 p minus syndrome(which I have the same syndrome).

So they had a very poor outlook didn't even think she was going to 'survive' and if she did it wouldn't be much of a life & she would more likely not live a long life either.

So in my sense I decided I could not go along with the pregnancy because how could you carry a pregnancy with so much sorrow & risking a chance of your special needs child living with 'severe issues.

I also thought what if the choice came down what if she was stuck on life support like a respiratory to help her breathe then after meeting her to do a decision to take her off it . I thought it would be more easier on me & her so she would 'never feel the pain of the severity and I would never feel the pain of seeing her suffer.

Somedays I do question how bad could she have been could she had been better than what doctors had said but it's just wondering and knowing I did the choice with my heart.

12:11 AM  
Anonymous Marcela said...

I have a young baby who was diagnosed with HPE in utero. The perinatologist and neonatalogists that we saw at UCLA gave met he same info - your baby will not have a meaningful life, he will not survive and if he does, his life will be miserable.

Well Nathan is 2 months old now and his life has already been very powerful and meaningful. He has taught many people about faith and hope and is loved by many.

I have spoken with many parents that have childrend with HPE and they continue to surprise everyone. Many have learned to walk and talk, contrary to expectations by doctors, and all of them have feelings and personalities and enjoy life in their own way.

So I strongly encourage you to rethink your position on this.

All of the parents that I have spoken with are so grateful to have these children in their lives, and deeply joyful that their children are alive. Many have had many scares where their children could've died, but they have fought for their child's life and are very grateful to have done so.

And for all of these families, these children have taught the parents and siblings so many lessons, so much love, so much kindness.

Me, personally - I don't care whether my son can walk or talk. I will do my best to help him. But if he feels loved and cherished he will have a happy life, and that is very powerful for all of us.

So before you sentence a child to death due to anticipating what his life could or couldn't be like, please get to know a couple of families that have gone through this and your ideas might change.

You can read more about my baby here: www.prayfornathan.org

1:02 AM  
Anonymous Anonymous said...

I recently gave birth to a baby diagnosed at 20 something weeks with holoprosencephaly. I understood the prognosis and what the doctor's concerns were, but my husband and I had hopes that our baby would be healed in the name of Jesus. When he was born with the condition and died, it was a very hard thing to cope with, but I did not regret choosing to continue the pregnancy because we wanted the Lord to have the last say. What I don't understand is how these doctors can get upset for a parent making such a choice, as this is America, and we reserve the right to make such a choice. I prepared myself for the worse and the worse did happen, but it is no one's business if the parents decide to carry through the pregnancy after being fully informed. This means that you did your job and can be nothing but supportive to that family until and after the baby's birth. My husband and I are college educated, entrepreneurs and capable of making choices for ourselves. Remember, the Lord does not give us more than we can bear, and if as Christians, we have FAITH, we trust that these life lessons become a part of our lives for a reason.

9:18 PM  
Anonymous Anonymous said...

Hi Neo do, I am an FP resident and the mom to a 5mon old baby who had HIE. She is very delayed and you have made a lot of good points. I've thought about them a million times. I think we do too much, i really do. She was rescuctiated for 8 minutes and is soo neurologically compromised; when do we say stop? When is it our selfish desires and not our kids? And not to mention the shattered dreams hurt like hell (understatement, need a stronger adjecitve) but these babies physically suffer too. For us we have decided that we brought her back once and we will let nature take it's course based on the circumstances at that time. God will decide,not humans b/c God's will is perfect. Vanessa

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