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Conscious Children: A Brief Look at Hydranencephaly

merry2e's picture

Conscious Children: A Brief Look at Hydranencephaly

Web Paper #2

“It is on the whole probably that we continually dream, but that consciousness makes such a noise that we do not hear it” Carl Jung once wrote.  But, what happens if a child is born with a rare neurological condition where they are missing 80% of their brain?  Are they conscious of what is going on?  Do these children live in a ‘vegetative state’ or are they aware of sensory inputs and outputs?  Do they smile when happy and become agitated when in pain if consciousness originates in a region of the brain they lack?  As we have explored consciousness throughout the semester in class discussions, we have tried to tease out the definition of consciousness and where consciousness originates.  These questions have been researched by many scientists for decades with countless theories evolving on many sides of the debate.

What Is Hydranencephaly?

Hydranencephaly, is a disorder where the cerebral hemispheres are completely or near absent at birth, including the cerebral cortex and basal ganglia and are replaced by cerebrospinal fluid (1).  Small amounts of tissue from the frontal, temporal and occipital lobes may be left intact and the thalami, pons, and cerebellum are generally present and functioning (2).  In most cases, evidence has shown the disorder is a non-inherited condition and usually develops after the second trimester of pregnancy.  It has been linked to injuries to the fetus, placental anomalies, and cytomegalovirus, herpes simplex, and Toxoplasma gondii infections affecting the mother (2).

After the infant is born, there may be no initial detection of hydranencephaly and it may go unnoticed for several months until the child misses developmental targets such as motor control (holding up head and neck), lack of eye contact or enlarged head (3).  The first year of life for the child with hydranencephaly and their families presents the most challenges and difficulties, both physically and emotionally.  It is at this time complications arise and medical emergencies can be a part of daily life.  Seizures, pneumonia due to aspiration, and problems with temperature regulation are a few of the complications that commonly occur (3).  Due to many hurdles, some children do not make it through their first year of life, but some have gone on to live into their late twenties with medical intervention and deeply caring family members.

Does the Cortex Reign Supreme?

Neuroscientists have paved the way for the development of further research into the controversial subject of consciousness and the view of the “supremacy of the cerebral cortex” (3) continue to be debated.  Until 1949, it was assumed that the regulation of the stages of sleep and wakefulness were ruled by cortical functioning (3).  Scientists Moruzzi and Magoun researched this regulating function through animal experimentation with astonishing results.  They found, “local stimulation of circumscribed cell groups in the pons and midbrain of experimental animals exerts a global activating influence on the cerebral cortex as well as on behavioral state, and that experimental lesions in these brainstem sites are capable of rendering animals somnolent and even comatose” (3).  This, in turn led neurosurgeons Penfield and Jasper to look further into the higher functioning debate of consciousness by studying patients with epilepsy (3). In surgical operations on patients who suffered with severe epileptic seizures, Penfield and Jasper removed cortical tissue while the patient was conscious.  They found that even with substantial removal of cortex, including hemispherectomy, the patients continued responding and maintaining consciousness (3 & 5). Through many years of study, Jasper and Penfield concluded that consciousness could not be an independent function of the cortex but a combination of brain connections, with the upper brainstem system serving as the conductor (3).

Exploring Levels of Consciousness

In understanding children with hydranencephaly, I feel it necessary to think about the possibility of the evolution of consciousness in the brain.  In the article, “Consciousness without a cerebral cortex: A challenge for neuroscience and medicine” by Bjorn Merker, he details his theory and research into consciousness and demonstrates evidence of his research with his experiences of children with hydranencephaly.  There are differences in the range of possibilities in consciousness as Bjorn Merker explains:

                “to see, to hear, to feel, or otherwise to experience something is to be conscious, irrespective of whether in addition one is aware that one is seeing, hearing, and so forth, as cogently argued by Dretske.  Such additional awareness, in reflective consciousness or self-consciousness, is one of many content of consciousness available to creatures with sophisticated cognitive capacities. However, as noted by Morin (2006), even in their case, it is present only intermittently, in a kind of time-sharing with more immediate, unreflective experience. To dwell in the latter is not to fall unconscious, but to be unselfconsciously conscious. Reflective awareness is thus more akin to a luxury of consciousness on the part of certain big-brained species, and not its defining property” (3). 

So, in reflective awareness (the “I”) would be what Merker defines as a “luxury”, essentially unnecessary for

 consciousness to occur in human beings, thus allowing for those without a cortex to experience conscious

states, including children with hydranencephaly.

                Children with hydranencephaly demonstrate evidence for Merker’s argument that the brain stem holds many answers for understanding consciousness. On the website, Rays of Sunshine, families of children with hydranencephaly support one another and exchange information. In three separate questionnaires, they gathered data about their experiences, asking numerous questions ranging from the age, sex, and time of diagnosis to medical conditions, allergies and medications their child was currently prescribed. The data proved to show an enormous amount of surprising and sometimes, contradictory information compared to what had been previously believed of the children (5). The most common health concerns with the children were respiratory, neurological issues involving problems with the shunt used for drainage of fluid from the brain (approx. 65% of children have shunts), seizures (approx. 77%), feeding problems and spasticity (5).

                As far personal contact with the child’s family and caregiver including motor functioning processes and sensory input, the following questions were asked:

1.       Can your child move his/her hands? Approx. 50% Yes

2.       Can your child give hugs or kisses? Approx. 20% Yes, Sometimes 11%

3.       Can your child cry? Approx. 91% Yes

4.       Can your child hear? Approx. 93% Yes

5.       Can your child make sounds? Approx. 96% Yes

6.       Is your child aware of his/her surroundings? Approx. 74% Yes

7.       Does your child understand what is said to him/her? Approx. 22% Yes

8.       Does your child use a communication device? Approx 14% Yes

The data collected shows evidence of consciousness and awareness of the primary caregiver and other family members and the ability in some children to even communicate needs and wants. This gives rise to understanding implications to many other important issues surrounding ethics including pain management in neonates, understanding fetal pain and pain for those who live in so-called “vegetative” states.

                Since hydranencephaly is a rare condition affecting just 1 in less than 10,000 births, and the mortality rate is extremely high for newborns, much extensive research still needs to be done regarding hydranencephaly. On July 25, 2006 in the Omaha World-Herald, there was an article posted, “Q & A: Meet the mother of a 27-year-old with hydranencephaly.”  This was the first article I read on hydranencephaly and knew I wanted to investigate the condition further when I read her response to one simple question, “How much brain tissue does she have?”  The mom’s response,

“I don’t really know. Back when Katie was diagnosed with this, I tried to do some research on it and it was so frustrating. Even going to a library, you might find a paragraph or two lines, and that was it. Even now you go to a doctor and say ‘hydranencephaly’ and half of them don’t understand. They’ll write ‘hydroecephalic.’ You have to say, ‘No, it is hydranencephaly (4).’” 


May the noise of consciousness get out of our way so we can some day find a cure for the Katie’s of the world.


WWW Works Cited

(1)  “Definition of Hydranencephaly.”

(2)    Wagner MD, Andrew L., and Dennis Rohrer, MD. “Hydranencephaly.”  eMedicine. Eds. Charles M. Glasier, MD., Bernard D. Coombs, MB, ChB, PhD, Marta Hernanz-Schulman, MD, FAAP, Robert M Krasny, MD, Lawrence M. Davis, MD.  18 Feb. 2008. WebMD.

(3)    Merker, Bjorn. “Consciousness without a Cerebral cortex: A Challenge for neuroscience and medicine.”   Behavioral and Brain Science. (2007) 30. 63-134.  Tripod. BMC Library, Bryn Mawr, PA.

(4)    Aksamit, Nichole. “Q & A: Meet the mother of a 27-year old with hydranencephaly.” Omaha World Herald. 25 July, 2006.

(5)     Rays of Sunshine.>

Other helpful resources:

Paul, Annie Murphy. “The First Ache.” The New York Times. 10 Feb. 2008. 28 Mar. 2008.

Schwartz, Jeffrey M. and Sharon Begley. The Mind and The Brain: Neuroplasticity and the Power of Mental Force. New York:  HarperCollins 2002.

Bower, Bruce. “Consciousness in the Raw.” Science News Online. 15 Sept. 2007. Science News.  5 Apr. 2008.



erica's picture

new mommy of baby boy with hydranencephaly

my name is Erica I have a 12 day old baby boy who has just been diagnosed with hydranencephaly at five days old my world turned up side down I have been looking and looking for answers and the hospital he was at gave up on him they told me to let nature take its course they told me he wouldn't cry but I have video of him crying they told me he could not feel but when I place my warm hand on him he didn't move but if I place a cold one he would flinch they said he would never open his eyes but he has four times now they said he would not learn how to breath but he has already started to improve I put my foot down and demanded to be se3nt to a new hospital he has on going seizures all the time and has been put on sedation the hole time I have found support groups and done lots of research on this topic but I am still at a lost a I have a almost 2 year old that I had to leave at home that I am worried about how will I show him that he cant play with his brother like other kids how will I tell him he cant be to loud how do I make him not feel left out knowing that I will be bringing home a baby that will need my undivided attention I have so many questions and seeing no new studies being done to improve this condition please help......

support group I found was on facebook) global hydranencephalyfoundation

Serendip Visitor Breezi Elyea's picture

Hi my name is Brian and I

Hi my name is Brian and I know it's been years since you posted but I was wanting to know how your son is doing?

Please message me if you find the need or want

Cognosium's picture

Cortex v Cerebellum

It is notable that no specific regard is given to the cerebellum here.

It is not a trivial matter because, as suggested in my first book "Unusual Perspectives: An Escape From Tunnel Vision" (free digital download), this section of the CNS has greater processing power than the entire cortex.

A proposition that has been substantiated in recent years by the finding that the cerebellum has far more neurons than the cortex. By a factor of four!

A fact that, along with its implications, does not seem to have yet filtered through to workers such as those mentioned here.

A limitation commonly imposed by today's high degree of specialisation which often excludes the "big picture"

While traditionally relegated to the "merely motor control" bin, the cerebellum very likely pays a much more important role in consciousness and cognition than hitherto imagined.

A paradigm shift in this area may be long overdue.

Serendip Visitor's picture


There have been people born without a cerebellum who have been completely conscious. There was this chinese woman who doesn't have a cerebellum and she was completely conscious. There are people without a cerebral cortex who are completely conscious and functioning. I don't think consciousness has anything to do with one specific region of the brain. I don't even know if it has to do with many specific regions of the brain.

debbie's picture

Hydranencephaly, consciousness, and what it is to be human

As long as a child reacts to their environment they are having an internal experience and are aware of their surroundngs to a degree.... on a moment to moment basis.....As far as the comment about someone being "deserving" of being called a human being.....rediculous!!! DNA of a child without "consciousness" will still be human DNA....We are not humans because we desrve to be....we are just a product of our DNA.....My grandson has Hydranencephaly and one thing I know....Zeke is...he just doesnt know why he is.....he exists on a basic level physically, thats no reason for anyone to question his "humanity" To me, they are just to ignorant to debate!

Anonymous's picture


Thank you for citing verifiable sources! It is a pain to have to fact-check blogs with no citations.

Angela's picture


Oh My, Tokyo, what a small brain you must have! I wonder about YOUR human(ness).
As a mother of an 8 year old child with hydranencephaly, I can assure you she is indeed "human". She is aware, she sees, she plays with switch toys, she is happy and she is for the most part extremely healthy as well. Her first year was not even very difficult - only the start of seizures made it somewhat so. She is currently on seizure meds to control that issue. She does respond, not just to family, but her therapists and her teachers. She loves hand over hand activities, she loves music, and she likes to watch TV along with her siblings. She is a happy, sweet little girl and very loving. She is A joy to our lives and we would not trade one minute of time with her - we love her just the way she is! By the way, we adopted this precious little one at seven days of age, knowing her diagnosis, and were told she would only live perhaps days or weeks. Her will to live is as strong as you or I! She is our hero!
Meredith, thank you for your article and attention to hydranencephaly. I have met and spent time with Bjorn Merker (my child is one of those he observed for his research). He is a wonderful, caring individual as well as a most inspiring and intelligent scientist.

Angela (mother to Chrissy, 8 years old) Texas

Brandi Frazier's picture

my 5 month old baby was just diagnosed with hydranenephaly....

My 5 month old baby girl Isabella has been diagnosed with hydranenephaly and I'm sick to my stomach a parent's worst nightmare come to life. I felt as if a had been punched in the head I was confused. "What do you mean without a brain?" I said. Will my daughter ever recognize me? Will she be able to show love? Please helping answer these painful questions. Is there a support group? I don't where 2 start I'm a single parent of 2 kids. My son William is a active 2 year old..And my daughter Isabella has been diagnosed with hydranenephaly at 5 months old & I don't know what to do... Are you able to still have a job with a daughter that needs my full undivided attention. How do I make sure my son doesn't feel neglected do to his sister's circumstances? Please helping I know am probably overwhelming you with questions. I'm sorry. Sincerely A mother in need.... Thank you.

Eiah's picture

Hi mommy, im also a mother of

Hi mommy, im also a mother of a 9 days old baby girl with the same condition as your daughter. If ever you have a chance to find a support group please... E mail me. I wanted to join.

Michelle47's picture


I know how u feel and still ask these questions
I live in nebraska and if u can call or text I would love to
Talk to u

Seth is my only son and he is 18 months

merry2e's picture

Why do higher mental processes define "human (ness)" ?

I am just wondering about the question, "Does something that "looks like" a human being but lacks any higher mental processes still deserve the name "human being"?" Because someone has a physical illness that affects brain functioning does not take away their humaness. Can you imagine a world without people in it who had IQ's less than a certain set number? Or a world where people with blue eyes were not human, or people who were deaf or blind were not human? What is the difference? Why would a child born with hydranencephaly be any less human than you or me?

Thank you for taking the time to read the paper,


gina's picture


I have an relative whom is 3days old and born with hydranencephaly,i am concern of her health and questions is it okay to bring her home at this crucial time?i do not know what to say,my relative is only 19yrs old that had this baby recently.I am trying to get an resource and an start somewhere for help.What can i do?

Tokyo's picture

What is a human being?

While I have every sympathy for the parents of hydranencephalics, I can't help finding the subject fascinating in relation to our fundamental definition of "person" or "human being." Does something that "looks like" a human being but lacks any higher mental processes still deserve the name "human being"? I also strongly question self-reports by care-givers claiming "hearing" and "awareness of surroundings." Yes, there are degrees of hydranencephaly, and a relative minor case might display such awareness, but a severe case, lacking any cerebral lobes, could hardly be said to "hear" or be "aware" as I understand it. Such beings may respond to stimuli of various sorts, but surely that alone doesn't amount to awareness.

Serendip Visitor's picture

The survey recruited 108

The survey recruited 108 primary caregivers through a parent support group and was conducted online via a commercial survey hosting facility. As part of a more extensive questionnaire, participants answered 106 questions bearing on the environmental responsiveness, emotional reactivity, mood and agency of the child in their care.

The survey elicited a many-facetted and detailed set of caregiver answers and written observations regarding the child's behaviour. A conservative measure of agreement among respondents' answers yielded a generic portrait of the responsiveness and expressive behaviour of a hydranencephaly child.

First of all a self-report is a report about oneself, not about someone else. If I were talking about myself that would be a self-report. So this study is not based on self-report. Plus the methodology is clear. Detailed questions were asked 106 of them about the behavior of the children.

Plus there is no relative minor case of hydrenancephaly. A relative minor case of the entire cerebral cortex being missing along with much of the basal ganglia?! So minor. Hydrenancephaly is defined with the cerebral hemispheres being almost completely or completely absent.

Serendip Visitor's picture

These aren't self-reports.

These aren't self-reports. Self-report is when you talk about yourself.

Serendip Visitor's picture

There are degrees of

There are degrees of hydrenancephaly. Yes. But the best possible case is when there is just a small amount of material in the three lobes of the cerebral hemispheres. The worst is no brain. Responding to stimuli outside of motor reflexes means awareness. This is a scientific study. The "self-reports" have been validated by the lead researchers.

Serendip Visitor's picture

These are not self-reports.

These are not self-reports. Not at all. They are reports about the children.

Serendip Visitor's picture

In hydrenancephaly the

In hydrenancephaly the cerebral lobes are absent or nearly absent. That is required. So technically no minor case is possible cause that would not be hydrenancephaly.

Serendip Visitor's picture

If you can respond to stimuli

If you can respond to stimuli in situationally contingent ways then yes you are conscious. Plus its unclear whether these children completely lack cerebral lobes.

Serendip Visitor's picture

Well no one here is saying

Well no one here is saying that these children are geniuses. They are just conscious. They don't have the mental capacities of other children. Plus there was the case of this woman with half a brain and 5 percent cortex who was almost completely functional. Plus the French man in 2007 infamous case. Hydrocephalus.

Ettina's picture

I think it's foolish to

I think it's foolish to ignore the reports of people who see hydranencephalic individuals 24/7, just because they violate medical orthodoxy. Who has more chance to observe a child than their parents? You don't need to be a trained professional to realize that a child's reactions are contingent on stimuli.

Paul Grobstein's picture

anencephaly: consciousness?

Very interesting issue, in part because it is so hard (at the moment?) to settle. How does one decide whether another human (or animal?) is having internal experiences? Is "reflective awareness" a "luxury" on top of consciousness? Or might it be that without which there is no internal experience at all?  I wonder what the parents would say if the question was posed to them this way?
Cognosium's picture


With regard to consciousness:

Rather strangely, we can trace the evolutionary roots of this phenomenon right back to the divergence of the human ape which involved the trading of the snout for the hands to serve the primary food-processing function.

The near-obligate use of tools which arose thereby having triggered the co-evolution of language and its correlate, an enormously enhanced level of consciousness.

As I have had occasion to point out many times before, the mystical notions that have arisen in the past are purely illusory, an inevitable result of approaching the question by introspection. This, of course, was the only option available to earlier philosophers and many still have trouble escaping from that trap with its inevitable recursive loops.

Today, although the details of nervous system function of ourselves or other animals is very far from complete, we have sufficient information to have a rough idea of the gross workings of these systems.

From evolutionary considerations we can also now see how the essentially navigational function we like to call "consciousness", "self-awareness" "sense of agency" and so forth is bound to arise.

Most, if not all organisms must, in principle, have some degree of consciousness (self-awareness). Even if only as the locus of its sensory and effector interactions with the external world.

This, of course, includes such creatures as bacteria and plants. Here's why:

From our understanding of biological evolution by natural selection it becomes quite clear that provision of a navigational feature that involves some degree of self awareness is required for an organism to interact optimally with its environment. It is a measure of its fitness for the prevailing environment and subject to selection pressure accordingly.

In the case of our own species, the co-evolution of language with an exceptionally high level if innervation of our hands and vocal apparatus has led to a situation such that we are not only self-aware but aware of being self-aware.

Furthermore, from a quite different discipline, we now have an excellent understanding of functionally analogous computational systems. And the composite that we call the Internet has, even now, comparable processing power to the human mind and is rapidly becoming endowed with the semantic linkages required of our particular kind of consciousness.

With these new tools at our disposal we can now view the phenomenon in a truly objective way.

This issue is discussed at length in chapters 23 and 27 of my latest book "The Intricacy Generator: Pushing Chemistry and Geometry Uphill", now available from Amazon, etc.