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Book: Caring for Your Child With Hydranencephaly

Hydranencephaly data base

 

 

 

A Note of Encouragement

Chances are that if you have just found out that your child has Hydranencephaly, you were given very little information or encouragement from the Dr. Yes, Hydranencephaly is a very serious condition and children with it have many many struggles.  However, they have lots of triumphs as well.

I asked the families on the mailing list what they would most have wanted to know when their child was diagnosed.  The most frequent answer was, what will my child be like.  The thing they least wanted to be told was that their child would be a “vegetable” and not live beyond a few months or a year at the most.  So, I’ll try to answer a bit based on my own experience and knowledge gained through talking to families of children with this condition.

Your child with Hydranencephaly is going to look just like any other child.  As a baby, children with this condition look and act, for the most part, “normal”.  Over the last couple of years we’ve had several families join our list, who are expecting a baby that has been diagnosed as having Hydranencephaly. They often ask what to expect when their child is born. So, I’m including a few pictures of children within a few days of birth and some of the birth experiences of families. These are letters in response to a question from an expectant mom as to what to expect.

Kimbra, mom to Dillion, age 1: don’t' feel bad about not buying clothes or anything I was told not to expect to bring a baby home so I had nothing no bed no clothes only a car seat I had gotten before I knew. We did take the car seat to the hospital but didn't bring it in till the day we were ready to come home. We even went the week before Dillion was born to funeral homes and cemetery to make funeral arrangements. Yes I was very convinced that I would never bring Dillion home. But when he was born he looked so normal that the doctors who originally diagnosed him with Holoprosencephaly took him for an ultrasound about 20 minutes after he was born to check and make sure Dillion had anything wrong with him.  It wasn't until he was about four months old right before his first shunt surgery we found out he actually had Hydranencephaly.  Dillion never had a problem with his breathing but he did have a problem with keeping his temp up and also he made ALOT of mucus se we were constantly suctioning out his mouth the first day but after that he was ok. I hope everything goes really good for you and If your doc are anything like, mine were they will even let you have the baby in the recovery room and may let your family be with you too. I was put in a private recovery room so that I could be with my baby and my family. Just talk to your hospital when you go in for surgery they will let you know. Make sure everyone knows you are delivering a hydran baby and they most likely will definitely make arrangements for something special for you.

Holly, mom to Kristen age 12, when my Kirsten was born she did better then my "normal" child when he was born.  She had apgars of 9 & 10.  She had beautiful coloring and screamed right away.   Now I know every birth is different and also I know that with a Csection it can take them a few minutes to catch their breathe sometimes because they haven’t been through the normal birthing process which helps to bring mucous up.  But I see no reason why you shouldn’t have an uneventful birth (except for the 200 Drs that will be there)

Donald, dad to Kyana age 6 months: Kyana was diagnosed with Holoprosencephaly (diagnosis changed after birth to hydranencephaly). We were told that she probably would not be able to breathe on her own..... The anxiety for those 10 weeks liked to drove us crazy.... when it came time for her delivery also by c-section there must have been @ least 8 people in the room waiting just in case...anticipating the worst she finally came out and after about 10 seconds she let out a huge first cry although that 10 sec. seemed like eternity, it was the best sound I've ever heard. As for your other concerns. Her color was normal...the only thing that stood out was that her left eye lid would not open all the way (corrected by a minor procedure @ 4mos) and the fact her head was larger.. Although she spent 2 weeks in the hospital they waited until she was 6 weeks old before putting in a shunt. That was Sept 13th

Lisa, mom to Noah age 20 months: When Noah was born we didn’t even know he had hydran - he was fine - just a little trouble breathing but it wasn't a problem. We literally thought we had a 'normal' baby for 9 weeks - he doesn’t have hydrocephalus - so his head size was normal until then - now it is a lot smaller than normal - he has microcephaly. He did cry - a LOT! That is why we started to question if there were problems and discovered that he had hydran

Our children respond to their families, as does any other child.  There may be a few differences such as increasing intracranial pressure and a need for a shunt, problems with controlling the child’s temperature, sleep problems, and feeding difficulties.  For the most part though your child will be a treasure to be enjoyed.

  As your child grows older, various problems are common.  Among these are feeding problems, susceptibility to illnesses and infections, seizures and respiratory problems.  However your child will still most likely respond to you, know who you are and know when he/she is at home and also the difference between family members and strangers.  This sounds like any other child doesn’t it?  Yes, our kids are more like other children than different from them.

Throughout this book you will find references to a research study.  This was simply a questionnaire that was filled out by many of our families in order to give a clearer idea of similarities and differences among our children

According to the results so far (February 2003), 57% of the children are happy*, 74% are aware of their surroundings, 41% are aware of objects and many of the children have security items-mom is the most common security “item”.  This doesn’t sound like the common descriptions of hydranencephaly that you will find referred to later in this book.  My hope is that this information will counteract the pessimism that you will often feel that you are inundated with.  (Full results of the study can be seen in Chapter 6, )

*Please note that 58% of the children are fussy and irritable during their first year. The low percentage of the children described as being happy is due to the fact that 13.7% of the children are less than one year of age. Most families have found that after one year the child becomes much happier. So, if your child is under one and is very irritable, take heart; that should change after the first birthday.

On the other hand, some of the children aren’t as aware as others, and some do die within their first year as predicted by the Drs. Just as every “typical” child is different from all the rest, the same is true among children with hydranencephaly.  Something that is often a major factor in what the children do or are aware of, is their health.  Our children have very few reserves.  Therefore if all of their energy is being used up just in breathing or eating they’re not going to have any left over to use in playing or interacting.  So, if at a young age your child doesn’t seem to be doing much, take heart, chances are they will, as they grow older and healthier.  Try to solve as many of the health problems your child has so as to maximize their potential.  

 

For instance, my daughter did almost nothing until after her 5th birthday.  We don’t know all the reasons for that. What we do know is that around the time she started to show an interest in her surroundings and relate to objects, her health was better than it had been in many years. A few months before that when she joined our family she had started to receive more stimulation than she had previously too. Once she started showing an interest in things; it started with chewing and touching a toy, we just were on the alert to see what would interest her next. We let her be our guide.

  Many of the new families on the mailing list have been a little overwhelmed with the difficulties and health problems many of the older children have.  It’s usually not so much that the children’s health deteriorates as they get older but that they’ve just been around longer and have had more time to develop problems.  It’s sort of like senior citizens.

The fact remains, though, that a brain stem is not meant to control a whole body for many years. Therefore the bodies of many of the older children just seem to wear out and they start to develop more problems.  This certainly doesn’t happen in all cases. Our 20-24 year old members can attest to that. Once again, all the children are different. 

 

 

Something Special

I asked the families that are on the Hydranencephaly and Hydranangels Mailing lists to tell about something special that their child had done or accomplished, as a way to help new parents realize that their child would have a quality life and that there will be triumphs and joys in their lives.

Lisa, mom to Noah age 20 months in Tasmania: We decided to come home after the football game to get Noah as they were having a function after the game where you could meet the team players, so we rushed home and got Noah and went back as we have lots of photos of Jalen with players from the team but not Noah.  When we got there there were sooooo many people crowding the players, getting autographs, photos etc.  We waited back in the crowd with Noah in his wheelchair and the captain of the team who also has the most fans had two security guys next to him to settle the crowd down.  The captain then looked up (for Emma and Jasia - it was Shane Crawford from Hawthorn) and saw Noah in his wheelchair and straight away he pushed his way through the crowd of people to come over to Noah and was soooooo nice to us and was more than happy to get his photo taken with him.  The crowd of people there actually started to cheer and clap him for coming over to Noah, which was nice!!

Diane mom to Josh, age 4, UK: Josh started school full time last Sept, he also won a art competition & got his certificate presented by the Lord Mayor.

 

Alsie, mom to Stasia: Stasia was only with us just shy of three years.  She went home to be with the Lord two months before her third birthday.  That was what she did.  She hung on and stayed with us for almost three years.  With less than 10% of a brain, she was never supposed to survive the NICU.  And in those incredible three years, she taught us more about honesty, purity, strength, courage, unconditional love, grace, patience, tenacity, giving and happiness than anyone or anything in the 51 years I've been on this earth.  And it was more than we ever hoped or dreamed she was capable of.  And not a day goes by that my heart doesn't fill up with gratitude for all that God gave us in so short a time through so small a child.

April, mom to Chris who died recently at the age of 14: Chris got an award one year for student of the month his name was also in the paper.

 Agnes, mom to Rachel, age 9: Rachel got first prize in bonnie baby contest aged 3 wks. She has also been featured in chat magazine and also appeared on tv. Rachel goes on a lot of school outings, swimming and loves sweet food. She is very good at saying yum yum.

Karen, mom to Heather, age 2: With Assistive Technology, Heather is able to read books.  We put story programs onto the Macintosh Computer and load them up.  As the story plays, a person reads the story and Heather sits on my lap in front of the computer listening.  At the end of each page, the person stops reading and a little bit on music comes on.  Heather pushes the mouse, which turns the story to the next page.  At first, I helped Heather by hand-over-hand pushing on the mouse and telling her, "Turn the page."  The next time, I just told her to turn the page.  The third time, she turned the pages herself, without any prompting from me.  She read the whole book Green Eggs and Ham by Dr. Seuss to her vision teacher!

Erica, mom to Matthew who died recently at the age of 2 ˝:He could not usually hold up his head or even sit very well but put him in the jonney jump up and he could make it move. He would be so happy in it till he got too big. But it was his favorite time in his jump up he loved to make it spin mostly.

Dee Dee, mom to Elizabeth, age 14: Elizabeth will be going to her school Prom next year.  

Barb, mom to Kayda who died at the age of 11˝ : One of the special occurrences in Kayda’s life was welcomingg families to the school for the Christmas concert. Her big Mack said: Welcome and she kept pushing it. The other really special thing for Kayda was how her hands were always busy and touching things. She also knew what time Star Trek came on and fussed if I forgot to turn it on.

 

 

 

Another Point of View, by Vikki Stefans: Vikki is a physiatrist in Arkansas and is on one of the mailing lists I’m on. I am including the entire article as it needs to be used in it’s entirety to meet the conditions of it’s use. I found this article very interesting as, when I first joined this list I wrote telling everyone how alert Kayda was. Vikki responded that likely Kayda was misdiagnosed and just had severe hydrocephalus. It appears that she has changed her mind. Yeah for Kayda!!!!!

 

A Rehab Doc's Take on Brain Injury
As posted to Our-Kids on May 20, 2000

OK , you asked for it, you got it...a rehab doc's take on brain injury. :-)

My answer to this is basically that kids all have an underlying and incredibly powerful drive to grow, develop, and learn. They will use whatever brain tissue they have and do as much as possible with it. All of us know kids with severe progressive neurodegenerative diseases who for years went undiagnosed because they were making as much progress as they did despite it for so long. And we all know that even kids with Hydranencephaly, having just a little basal and gangliar tissue, will use it to connect with and recognize caregivers, enjoy some music, etc...I guess they save what they've got for the truly important things in life!!

A bad looking CT or MRI is just a picture of an injured brain, and does not in itself tell you exactly how much that brain can still do, or predict lifespan.

The other, related part of this answer is "plasticity". The younger the brain the less committed certain parts are to doing certain specialized functions. Plasticity is not infinite and it is not a free lunch. For example, kids with right hemiparetic cerebral palsy don't have aphasia like an adult with a left sided cortical stroke and right hemiparesis most often does. But the child with CP is more likely to have some spatial-perceptual and learning or milder language problems. For the amount of brain tissue lost, the child's hemiparesis will also be a lot milder than the adult's, but there will generally be at least a little. This is why people in general are amazed at what a child who has a hemispherectomy can do and how "normal" they look...but some parents, who were expecting totally normal, have been disappointed with their real results.

So there's not a lot of white matter. So what there is works a little overtime to get through what messages it can. When not enough messages that control excess tone are getting through (when it comes to motor functions especially, the cortex is largely inhibitory in function) we see a lot of spasticity and basically we try various ways to send the message to relax with the various medications and such we use. Then whatever messages to move a certain part a certain way can get through will better be able to accomplish something. White matter also has some function in learning, connecting the different areas of gray matter together and connecting the hemispheres with each other. The "diffuse axonal injury" seen after head trauma is largely a white matter injury, disrupting lots of connections, and generally results in overall inefficiency of operation of the brain. Exactly how new connections get established and how brain function continues to improve over months to years afterwards is still more than a little mysterious.

Remember that the resolution of even the best neuroimaging is still a little limited. There are many things we could not see on CT scans that we can see now on MRIs but you can't see down to the level of an individual nerve cell by any means. The famous cases where the person was thought to have hydranencephaly but actually had severe hydrocephalus with enough cortex to learn algebra would be less likely to be mistaken today; These were recently brought up in an ethics discussion for a baby with hydranencephaly (Barb- you'll be pleased to know that the neurosurgeon finally DID do the shunt after all!) and the question was brought up whether the shunt should be done just to see if in fact this was the case instead. This is no longer at all likely; and in a way, that was the wrong reason to do the shunt. The point is that the child obviously had capability to have a quality of life despite the obvious limitations and needed the shunt to live and to avoid having a huge head that would have
limited mobility and care. (That's what persuaded the neurosurgeon to do it.)

So remember that every brain is designed to grow and develop, and even an
injured brain will grow and develop to whatever extent is possible. Your child's abilities and potential to maximize them matter more that the picture of how the brain looks. Also, this has come up before on this list- but if someone is diagnosed with mental retardation (about 50% of people with CP have some degree of MR) it does not mean they cannot learn. It means that they may learn more slowly, require more repetitions, may have to be more explicitly taught things that others just learn "naturally". We all face the challenge of pointing out that the cup is 3/4 full instead of empty- there is a world out there that tends to see it as either 100% full or nothing.

Vikki Stefans, pediatric physiatrist (rehab doc for kids), Arkansas Children's Hospital/ U of A for Medical Sciences, Little Rock, aka vstefans@care.ach.uams.edu

References:

A Rehab Doc's Take on Brain Injury, by Vikki Stefans, Pediatric Physiatrist, Arkansas Children’s Hospital, Our-kids Mailing list archives:
http://www.our-kids.org/Archives/A_rehab_docs.html


Picture Index:
Image1: Kayda, 3 days old, 2: Kirsten, 3 days old.
Image 3: Noah newborn, 4: Brennan 1 year , 5: Drew: happy boy
Image 6: Kayda age 5 playing with her hands, 7: Travis, age 22 Grad picture, 8:Joshua age 3 with art that won an award, &9:  with Lord Mayor and Mom.
Image 10: Chris, 11: Kayda greeting people at school concert  

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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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