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What Doctors May Say and What They May Mean

We hear many things from our children's doctors. Sometimes they're right and sometimes they're not. 

The thing families hear the most is: "he/she is shutting down", "his/her brainstem is shutting down", "brainstem deterioration", etc

From Dr Bjorn Merker in Sweden: 
These are not technical terms with well-defined meaning. They sound like shorthand expressions used by clinicians for a progressive deterioration of global functioning heading towards a comatose state. It is informal language, and parents could simply ask a physician who uses such expressions to clarify and explain. Even then, there will be times when no precise answer is forthcoming, because of the difficulty of knowing the functional conditions of the parts of the brain present in a child with Hydranencephaly and just how those parts interact. Despite the incredible progress made in imaging and scanning methods, their resolution still does not give a good view of the details of the brain stem. Multiple possible interacting sources of malfunction can easily turn into a diagnostic nightmare. That may occasion vague language such as the above, and when it does, and no precise explanation is forthcoming, parents should also know that no precise prognosis is possible. That is, when a physician cannot pinpoint the cause of a child's condition, he also cannot know what that child's condition might be the next day, the next week, or the next month. Numerous experiences of parent gathered in the Hydranencephaly group confirm this. 

Here's a letter from a mom and Bjorn's response to the statements in it:
From Valerie mom to Brianna (Jan. 22, 2003-Dec. 8, 2003)

"After the shunts and everything else, we got home last Wednesday and first thing Friday morning, when I picked Brianna up from her bassinet, lo and behold..... Seizures!!!!

The doctor said the seizures are from her sodium level and she is on Phenobarbital. My doctor and the neurologist advised me that everything is basically missing. Growth hormones, steroids, her potassium level is going up, her acid in her blood is low, etc. The doctor thought it was great that I had the group to write to, but he advised me that Brianna is more severe most likely than all of your children. 

He said Brianna has only the brain stem (which is distorted) and a very small amount of the very back part of her brain. she only has a few months. H said all different things we could do but basically, we would be keeping her alive, but for what purpose....hers or ours.  

I don't know what to do.... Basically, I guess we just forget the treatments, keep her on the seizure medicine and love her for however long she decides to bless our family. She started laughing already and smiling...It's just so hard!!!!"

Reply from Dr Bjorn Merker: When the doctor says "Brianna has only the brain stem (which is distorted) and a very small amount of the very back part of her brain" that sounds like a typical situation for the diagnosis Hydranencephaly. Without having compared Brianna's brain images with those of the kids living on for year after year in the loving care of their families it is not possible to say anything like "Brianna is more severe most likely than all of your children." My guess is that such a comparison would show a broadly similar picture with, naturally, all kinds of detailed variation from one child to the next. I further guess that the doctor is guessing or assuming (and the "most likely" indicates as much) that BECAUSE these children are doing better than he expects Brianna to do, they must be "less severe" than Brianna, that is, must have less brain tissue missing than what he sees on Brianna's brain images. But that, of course, is putting the cart before the horse: It is what the Hydranencephalic children receiving proper care do and respond to that shows us what they are capable of, and what to expect after a diagnosis of Hydranencephaly has been made. There is a lot here that we do not yet know, and much to be learned, and this is a process that has only begun, as Dr. Shewmon's paper indicates.

 One has to be careful even with statements such as the one about Brianna’s brain stem being distorted. It is very difficult to know what this means without having the details, and the reason is this: What we know is that most of Brianna's cerebral hemispheres are missing. That means that her motor cortex is missing, and the motor cortex is the origin of the so-called "pyramidal tract" which is a big bundle of nerve fibers going right through the brain stem down to the spinal cord. That bundle of nerve fibers, and some other ones as well, will be missing in Brianna's brain stem, and that in turn may give her brain stem a distorted appearance. But such a distortion is really not saying any more by itself than that large parts of the cerebral hemispheres are missing, and so the mere fact of distortion is not informative by itself. Similarly, if parts of the cerebellum are missing, which is not uncommon in Hydranencephaly, the massive bundle of fibers connecting the two halves of the cerebellum with each other (in the "pons") will also be reduced, and this may add to the distorted appearance of the brain stem. So, some kinds of distortions of brain stem appearance are expected in Hydranencephaly simply as a consequence of the cerebral hemisphere and other damage.

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August 16, 2001- January 12, 2005

This website is funded in loving memory of Jason S. by his mother Kammy

The information on this site is provided by families, caregivers, and professionals who are or have been caring for a child with Hydranencephaly.

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