Dr. Sherr talks about ACC

Dr. Elliott Sherr is a Pediatric Neurologist at
University of California, San Francisco (UCSF).
Dr. Sherr is highly knowledgeable and an expert
on Agenesis of the Corpus Callosum. He sees many
patients, children, who have ACC and Dr. Sherr
is highly respected and well-liked by parents.
Dr. Sherr is on the Board of the National
Organization for Disorders of the Corpus Callosum
(NODCC) and he speaks at the annual Conferences.

Dr. Elliott Sherr is also involved in a very large
Brain Development Research Study on ACC.

I want to bring to your attention some excellent
resources that provide Dr. Sherr's knowledge
about agenesis of the corpus callosum. More
specifically:

At the 2004 NODCC Conference, Dr. Sherr gave
two presentations about Agenesis of the Corpus
Callosum. They are available as Conference
handouts [in outline form] to read below.

2004 Conference handout titled:
"ACC and Associated Features".

2004 Conference handout titled:
"How to Get the Most From Your Neurologist Visit".

In addition, both of the 2004 Conference Presentations
by Dr. Sherr, in their entirety, have been combined into
one DVD for viewing titled:

"Neurologic Issues in Disorders of the Corpus Callosum".
You can purchase the DVD from the NODCC store.

I own a copy of the DVD (recently purchased) and find
it to be very informative. My son, Matthew, who has
complete Agenesis of the Corpus Callosum was diagnosed
at four months and he is 17 years old.

The 2004 Conference DVD is divided into two separate
Presentations:

"How to Get the Most Out of the Visit/Relationship
with Your Neurologist"

and

"Agenesis of the Corpus Callosum and
Associated Features"

A few of the topics and information covered in
the "How to Get the Most Out of the Visit/
Relationship with Your Neurologist" 1st episode
of the DVD include:

Dr. Sherr says:

"I just want to say one thing: that there
are a lot of syndromes that are sometimes
associated with ACC where ACC is not
necessarily the principal issue. And that
doesn't mean that your child's not going to
have ACC related issues but it's important
to recognize that there's probably a lot
going on and ACC is a piece of that as
opposed to the primary driver of the issue."

Dr. Elliott Sherr was involved in an ACC Review
titled: "Agenesis of the corpus callosum: genetic,
developmental and functional aspects of
connectivity".

Genetic Syndromes occasionally and frequently
seen with ACC can be found on Page 8.

CHILD NEUROLOGISTS:

Dr. Sherr states that they should be board
certified in child neurology.

ADVICE REGARDING MRI FILMS:

He says:

"You should have the copy of the films with
you and in today's day and age you can have
a copy on CD. You can also, if you want,
have a film copy as well." (for yourself).

Dr. Sherr talks about asking for a film
copy of the MRI for you to have. Then you
are able to take the MRI film copy with
you to doctors and specialists that your
child sees.

Also discussed on the DVD:

What Type of ACC is this?

complete ACC
partial ACC-hypogenesis/dysgenesis
thin corpus callosum-hypoplasia

What are the associated CNS changes?
CNS = Central Nervous System
(in other words) - Dr. Sherr says:

"When you look at the MRI what other things
are there that can be seen?"

Is the MRI interpretation accurate?

Dr. Sherr says:

"I will tell you...so you know we're doing
research in my group on ACC and so we've had
families from around the country send us their
films and I can tell you that the accuracy...
the families always send us the films and
frequently the radiology report is included
in there."...

"for somebody who is not used to looking at
brains of children on MRI they're more likely to
make that mistake because they just don't have
the experience of looking at them. So the
people that I work with have probably looked
at in excess of 10,000 brain MRI's on children
and so they have a fairly strong wealth of
knowledge about what is what, what is normal,
what is abnormal and if it's abnormal, what is it,
and that is actually very important. And so when
we got the films--we've gotten over 100 set of
films--and I'll tell you that about 5 to 10% of
them were completely wrong"...

"Even the best radiologists are going to make
mistakes."

"So how do you address that?"

Neuroradiologist and
Pediatric Neuroradiologist

"Who's reading the film? Is it a Neuroradiologist?

So there are Radiologists, people who are board
certified in radiology, but then there are people
who do an extra two years of additional training
and all they look at are images of the brain and
the spine and the nerve roots throughout the body
so they're more likely to be experts in reading
brains."

"And then there's another level. It's not
a level that you can get a little certificate for
but there is another level of specialization where
they're Pediatric Neuroradiologists, where all they
do all day long is look at MRI's and other imaging
modalities of kids' brains and kids' spines and the
reason that's important is that the developing brain
looks a lot different on MRI than the mature brain.
There's a lot that's going on obviously inside of
your child's head when they're developing and that
is actually seen to some degree when you take
pictures. when you take this MRI and somebody who
is an expert in that knows about what a brain is
supposed to look like at 3 months and how it's
supposed to look at 3 years, cuz those are very,
very different. And how you interpret the results
is based on your knowledge of knowing how the brain
looks as it progresses during development. So this
would definitely be one of those cases where if
only a local radiologist has looked at it, you
should request that the MRI be reviewed by a Pediatric
Neuroradiologist. And there are ways to have your
local doctor send the films to another radiologist
[Pediatric Neuroradiologist] for a second opinion
and I would pursue those."

Dr. Sherr says:

"Have your Neurologist show you the films and go
over the films with your Neurologist."

He says to question the Neurologist about the
indecipherable terms on the MRI report and have
them explain each finding to you.

MRI's in Children

Dr. Sherr says:

"There are certain things that we can see at
3 years we can't see at 3 months and so if your
child's first scan was at 3 months of age I
would recommend getting a second scan when
they're 3 or 4 years of age."

He provides more detail about this topic on
the DVD.

Preliminary Findings of ACC:
White Matter

Dr. Sherr states:

"Is there a correlation between what we see on
their MRI and how they're doing clinically?"
We did find something preliminarily. We found
that the clinical score correlates with the
amount of white matter and white matter is
insulation in the brain."

"So the more insulation [white matter] that
you're missing the more severe your clinical
condition was. And if the brainstem was
involved that was also correlating with
severity."

Dr. Sherr further states:

"Knowing more about the MRI is going to
provide you additional information about how
your child is likely to do. And I say likely
because this is just a rough correlation. It's
not a guideline. It's not an absolute-ism."

MRIs and ACC

Dr. Sherr says:

"Everybody should have one MRI at diagnosis
and, unless the diagnosis was made later in
life, should have a second MRI."

The DVD includes many other topics and
information about ACC.

At the end of the DVD Dr. Sherr took
questions from the audience. One
question was:

"Why do some people who have ACC function
fairly normally and live a relatively
normal adult life?"

Dr. Sherr replies:

"Some of that might have to do with other
places that fibers cross...so there's
something called the anterior commissure
and it's a place that fibers cross and it's
in front of and a little down from where the
corpus callosum is."



"And actually when we reviewed some people with ACC
we noticed that they had big anterior commissures,
suggesting that maybe fibers that should have gone
through the corpus callosum crossed through there.
So that's one possible explanation."

note: Anterior commissure. Small band of approximately
50,000 axons that connects the cerebral hemispheres. The
anterior commissure connects the temporal lobes and is
located at the base of the fornix.


A few of the topics and information covered in the
"Agenesis of the Corpus Callosum and Associated
Features" 2nd episode of the DVD include:

MRI features that can be associated with ACC:

*COLPOCEPHALY:

"This is a posterior enlargement
of the lateral ventricles that results from the
absence of the corpus callosum."

"Similar phenomenom is the 'steer-horn' shaped
lateral ventricles seen more anteriorly."

*UPWARD DISPLACEMENT OF LATERAL VENTRICLES

Dr. Sherr says:

"The first two [colpocephaly and upward
displacement of lateral ventricles] are
almost always seen with ACC...not always
but almost always...and it's just what
happens to the brain in the absence of
the corpus callosum."

"So it's not a separate diagnosis
all by itself. It [colpocephaly]
is intrinsic to the diagnosis of
ACC."

He also states:

"So it's like if you're building a building
and you take out scaffolding in one of the
main areas of the building then the
building's going to look different. It's
going to have support structures in
different places and so that's what's happening
to the brain."

"But it's not a separate diagnosis. It's
just what happens when the scaffolding,
which is the corpus callosum in a way, is
missing. The ventricle's going to bulge out."

"So colpocephaly and what radiologists
call steer-horn shape ventricles are a
consequence of the absence of the corpus
callosum."

*PROBST BUNDLES

The corpus callosum is made up of 200 million
axons (also referred to as fibers).

Dr. Sherr says:

"Axons are like the telephone wires that are
connecting all the phones together and they are
these wires and they go from one side of the brain
and they cross over to the other side of the brain.
And if they cross over to the other side of the brain
then the corpus callosum is formed and if they don't,
then where are they going to go...

So in certain individuals those fibers go to the middle,
they can't cross, presumably because the structure that
needs to be made in development, (what's called a callosal
sling) is not there and so instead they turn to the left
or they turn to the right and they go alongside the edge
of the callosum (or where the callosum would have been)
and form the Probst bundles. And it's unclear what this
means.

Some people think that if you have Probst bundles that
it correlates with a better diagnosis [outcome]. We
don't know that yet but that's hopefully one of the
things we'll be able to answer more definitively."

Dr. Sherr (in the DVD) goes on to point out visually
on an MRI image where the Probst bundles are.

He then says:

"Not everybody with ACC has Probst bundles but a lot
of people do have Probst bundles but we don't know the
significance of that yet."

*INTERHEMISPHERIC CYSTS:
*a. type 1 - communicate with ventricles
*b. type 2 - not communicate with ventricles
*c. continuum with lipoma

Dr. Sherr says:

"These cysts are fluid filled structures outside
of the brain matter proper."

"So actually when the brain is formed there
actually is a space between the two hemispheres
so it's not within the brain proper...it's sort
of like in between the two sides."

"And the current thinking is that cysts develop
in lieu of the corpus callosum."

*LIPOMA:

Dr. Sherr says:

"One thing that happens instead of a cyst
forming is that a little structure, that's
basically a fat collection, which is called
a lipoma, will form instead.

ACC Brain Images

In the DVD, Dr. Sherr also speaks in detail
about other brain anomalies seen with ACC.

Dr. Sherr said:

"If you just look at the number of people who
have just ACC and nothing else that's a smaller
percentage than the total, so most kids with
ACC are going to have ACC and something else."

Dr. Sherr explains the different views of
the brain on MRI films and shows examples.

Dr. Elliott Sherr advises:

"When you get an MRI scan you need people to do
Axial, Coronal and Sagittal views to get a true
picture of the structure of the brain."

Also discussed:

Can My Child Have ACC and:

Autism
Attention Deficit Hyperactivity Disorder
Obsessive Compulsive Disorder
Cerebral Palsy
Mental Retardation

Dr. Sherr says:

"The short answer is yes."

However, in the DVD he goes on to answer
in more detail.

ACC Syndromes with Molecular Genetic
Testing:

Dr. Sherr says:

"So these are examples where the gene is known
and it causes ACC."

Andermann Syndrome
Mowat-Wilson
ARX related syndromes
L1CAM

He also says that in his experience they are
very uncommon causes of ACC.

The DVD covers a lot more topics and
information about ACC.

At the end of the Presentation Dr. Sherr
took questions from the audience.

(a few of the questions are):

Question:

"Is it acceptable to request a typed up
copy, in layman's terms, of what the MRI
interpretation is?"

Dr. Sherr replies:

"The answer is absolutely yes."

Question:

"Symptoms that might trigger you to get
another MRI?"

Dr. Sherr replies:

"I think that the symptoms that would trigger
somebody to get another MRI down the road
would be a significant worsening. So if a child
is developing at his or her own rate and then
all of a sudden they stop developing or they
decline in development, that would be a reason
to get an MRI. If there's new symptoms that
are significant and not sort of part of things
that they're already doing, you need to
potentially get another MRI, and the last one
would be something that might need to be
discussed with your doctor, depending on what
they are."

Also available from the NODCC is Dr. Sherr's
presentation at the 2008 Conference on podcast, at
no cost, which includes some of the same information
on the DVD from the 2004 Conference plus even
more information. In my opinion, the DVD and the
2008 Conference podcast are BOTH excellent
resources about ACC.

note: The DVD from the 2004 Presentation
gives the advantage of being able to see the exact
area of the brain Dr. Sherr is talking about (because
he uses a red light pointer to visually point to the specific
brain structure in the picture that he is discussing).
The 2008 Conference podcast displays still frame pictures
of the brain and you only hear Dr. Sherr talking about
specific areas of the brain, but do not get to visually
see them being pointed out.

note: you will need to download the TechSmith
Screen Capture Codec (TSCC) to view the podcast:
download here.

2008 Conference podcast with Dr. Sherr

(a few interesting highlights are):

3 family generation of complete ACC and
partial ACC in one family. Dr. Sherr discusses
one family where ACC and partial ACC is seen
in a grandfather, children and grandchildren.

seen at time frame 12:44-15:26

Dr. Sherr states:

"All of these individuals with a corpus
callosum disorder did well or average in
school and the adults have jobs. So these
are folks who are functioning at a very
high level."

ACC and Heart Defects:
seen at time frame 32:53-33:49

Dr. Sherr says:

"Almost 1/4 of the kids had heart problems.
Now these are not innocent murmurs that then
go away. These are actually kids who have real
heart disease. So my recommendation on any child
with ACC is that they get a comprehensive heart evaluation."

Colpocephaly is discussed:
seen at time frame 38:52-40:29

Dr. Sherr says:

"And so if doctors say your child has hydrocephalus
you want to be very careful to ask them, well does
the child have hydrocephalus or does he or she just
have colpocephaly?"

Interhemispheric Cysts discussion
and other ACC features
seen at time frame 43:20

Question regarding Interhemispheric Cysts:

"Do they enlarge or do they stay the same?"

seen at time frame 49:09-50:44

Dr. Sherr's answer:

"I don't think I have good enough numbers yet
but I would say that the short answer is most
don't change in size and a few will change
in size.

"If a cyst enlarges it will result in the
symptoms of hydrocephalus and so usually what
that will mean is that the child will present
with some sort of symptoms...usually it could
mean things like vomiting, particularly vomiting
when you first wake up in the morning, if a child's
eyes seem to be pointing down too much instead of
being right in the middle of the eyelids, if the
child seems to be too sleepy (lethargic) that
could also be a sign of hydrocephalus."

"So for my patients in my clinic who have an
interhemispheric cyst, when they're little I'll
get an MRI every year for the first three years
of life and if there's no growth after three years,
then I'll do one more when they're 5 years of age and
then after that I won't do anything unless they have
symptoms. But that way...I think most of the kids that
have cyst enlargements, it happens early on."

It's definitely worth exploring these valuable
ACC resources mentioned in more depth for yourself.

You can also find additional resources about ACC
(including Conference handouts, podcasts, and other
items) available at the NODCC website.

Do you want to enroll in the ACC Research Study at
UCSF where Dr. Sherr and his colleagues are involved?
Please contact: Brieana Fregeau at 415-502-8039.

Got an Idea?

Do you have an idea?

I have ideas about topics to include here on
the Agenesis Corpus Callosum blog and I'm usually
working on one or have some ideas stirring around
in my mind.

But I am very interested in knowing YOUR Ideas!

What are they?

What do YOU want to see here?

When it comes to Agenesis of the Corpus Callosum,
what type of information are YOU seeking?

It's especially important to me to include a wide
variety of information here that pertains to ACC.

Is there a specific topic here that you like and
want to see more of?

What's not here that you wish was?

School issues?

Speech and Language?

Adults & ACC?

Some other topic?

If you have an idea or several ideas, then here's
a place for you to express them. Share an idea
once or twice or as often as you would like.

Please leave a comment with any ideas that YOU
have.

Go ahead, be the first to share YOUR idea.

I can't wait to read them all.

A little girl, learning to read & Bob Books Giveaway

March 1, 2011 announcement

The WINNER of the Giveaway is:

Family H and their little girl, Violet.

Sabra let me know that her daughter put 8 pieces
of paper, numbered 1 through 8 (for the 8 entries)
into a bowl. She drew out the piece of paper with
number 6 and that represents Family H.

Sabra told me that after her daughter chose the
number....6 she said:

"This is cool to pick another child who has ACC like
me to get Bob books the same as me. I hope they help
reading with the other child."

Her mom said:

She then asked me who is number 6. I told her
I don’t know but we can check tomorrow to
see who the winner is. She was very excited about
seeing who won.

THE BOB BOOKS GIVEAWAY IS CLOSED.

Thank you to everyone who entered.


You are going to LOVE this story!
AND the prize revealed at the end of the rainbow…♥

I was contacted by the mom of a little girl who has
partial agenesis of the corpus callosum. I have known
the mom, Sabra, for a few years. Her daughter struggled
with learning to read and I remember when she told the
jump for joy story in the ACC support group about how
her child learned to read her first book.

Sabra enthusiastically wrote a heartfelt letter to the
Bob Books company:

"I would like to say thank you to whomever wrote these wonderful
books. I have a 6 ½ year old daughter born with congenital
hydrocephalus with a VP shunt and partial agenesis corpus
callosum. I tried hooked on phonics since this is what I had
used with my other children but she still struggled with trying
to read. She started first grade the day after Labor Day in
regular classroom with resource for language arts. She brought
home books from the library, AR books that were 0.6 and 1.0
reading level and cried because they were too hard and said
she couldn’t read. This about broke my heart when she said
this and how discouraged she was in regards to reading. When
I was in Barnes and Noble a week and a half ago to buy books
for my other children and myself, I asked the sales clerk on
what they recommended to help my daughter with reading. They
recommended your books and said they have teachers coming in
all the time purchasing them. I had never heard of these before
but I decided to give them a try and bought the first set,
beginning readers.

You have to understand this has been hard on me since we are
so big on reading. I have been working with her and trying to
find what worked for her to help her learn to read. It was
amazing and beautiful to see her little face light up and the
biggest grin after she read the first book. She currently
finished book 9 tonight and is doing amazing, we read a book
a night. She may not remember she just read that word on the
previous page but sounds it out and reads it without getting
discouraged. What helps for her is the small size of the book
and only a few words on each page. She may also struggle with
comprehension and understanding that reading a book is a story
but I know we will get there eventually. Your books have helped
us give her the confidence to read and the foundation on which
reading begins.

I will be going back to Barnes and Noble this weekend to purchase
set 2 for my daughter. Again, thank you so much for having this
program available and the insight to whomever wrote and developed
these books to give my precious daughter a chance to learn to read.
I don’t normally write comments and feedback but I am so impressed
with the books and the positive rewards, I felt compelled to send
one in regards to the Bob Books.

I now tell everyone I know including parents on hydrocephalus and
ACC online groups I belong to about how wonderful Bob books are.
I will make sure I have the money available when she is ready for
the next set."

A very happy parent in Virginia,
Sabra




Bob Books Reply Letter to Sabra:

"What an amazing and wonderful letter. Thank you so much for writing.
I am forwarding your letter to the author, Bobby Maslen. She will be
thrilled to hear about your daughter's success. After all this time,
she still loves to hear about each and every child that learns to
read with Bob Books. (She wrote Bob Books over 35 years ago.) We
are so proud when we can make a difference, especially for
children that struggle.

You might like to see a video (just posted today) about how Bob
Books came to be. I hope you can tell from her face, how much
Bobby cares.

http://www.bobbooks.com/bob_books_story.php

Thank you again for your very touching and heart-warming letter.
I am so glad we were able to make a difference for your daughter.”

Best, Lynn
-------------------
Lynn Maslen Kertell
Author and Managing Partner, Bob Books Publications, LLC
www.BobBooks.com



This was a story with a very happy ending. But
that’s NOT the end...

Sabra, the little girl’s mom, also let me know that
Bob Books would like to give away a Bob Books
Set 1 Beginning Readers and a Bob Books Set 2
Advancing Beginners to a family who has a child
with Agenesis of the Corpus Callosum.

Sabra asked if I would host the Bob Books Giveaway
on the ACC blog on behalf of her and her daughter.
I said…absolutely YES, with pleasure.



THE GIVEAWAY INCLUDES:

1 Bob Books Set 1 * Beginning Readers



and

1 Bob Books Set 2 * Advancing Beginners



CONTEST GIVEAWAY RULES:

1. Please enter only if you have a child who has agenesis of the corpus callosum or a corpus callosum disorder.

2. Only US residents age 13+ are eligible to enter (due to
licensing issues we do not ship to Canada or overseas).

3. One entry per username & e-mail.

4. Answer the following questions:

Why do you want to win Bob Books for your child who has ACC and How do you think it would help your child with reading?

5. Please include your e-mail and username in your entry so
you can be reached if you are the winner.

6. Contest winner will be randomly chosen on February 28,
2011 by Sabra’s daughter, who inspired the Bob Books
Giveaway. : )

7. Contest Begins: February 14, 2011

8. Deadline Entry: February 27, 2011 11:59 pm, pacific standard time

WINNER WILL BE ANNOUNCED ON: March 1, 2011

If you are the Winner you will be asked to give out your mailing address which will be forwarded to Bob Books who will ship your free set of Bob Books 1 & 2 directly to you.

Be sure to explore the "Entire Family of Bob Books"...

Thank you to Bob Books for sponsoring the Giveaway
from your heart in an effort to help another child,
who has ACC, learn to read. ♥

Thank you also to Sabra for allowing me to host the
Bob Books Giveaway for you.

A great BIG congratulations to Sabra’s little girl
for learning to read. You did it!!


THE BOB BOOKS GIVEAWAY IS CLOSED.

Thank you to everyone who entered.

*note: I did not receive any merchandise or monetary gift
for hosting this Giveaway. This Giveaway comes from the
heart of Bob Books and is the result of a mother’s joy in
hearing her child who has partial ACC learn to read for the
first time and seeing her child’s confidence level soar.

ACC and Moms-To-Be #9 Story

I am so thankful to all of the Moms who want
to tell their story about being pregnant
and having a baby with Agenesis of the
Corpus Callosum. Each story is as unique as
each child who has ACC.

Several weeks ago I received an e-mail from
Amanda who expressed a desire to share
her ACC Pregnancy Story here on the blog
for others to read.

Thank you very much, Amanda. It is a
privilege to post your story and I thank
you from my heart for your willingness to
help other people through your own
personal experience.



Written by Amanda:

My name is Amanda and I am the lucky mother of three
boys.

On August 28th of 2009 I found out I was pregnant
which also was a week after my brother-in-law was
killed in a car accident.

My pregnancy was normal for the most part but it
took a month or so before I was able to see an
Obstetrician because Medicaid was dragging their
feet and no doctor would set me an appointment
without insurance. I was very angry.

When I was finally able to see a doctor because of
insurance (and where I live) I only had two choices
for doctors. So I picked the closest doctor.

When I finally got a doctor, I let my doctor know
I was hurting and having pain on and off with this
pregnancy. He checked me out and said I was fine.
With my second pregnancy I hurt and couldn't walk
far and had to take meds to control contractions.
At the end of my second pregnancy my son was born
five weeks early but was healthy. So I made sure
my doctor knew about my problems with my second
pregnancy when I saw him at my first appointment
for this pregnancy. But he didn't seem concerned
or seem to care about the pain I was having.

I remember hating to go to doctors because it would
take three hours to get seen and then ten minutes
to be seen. Because of my insurance you are only
allowed 2 ultrasounds.

About a month or two before Landon was born is when I
had an ultrasound done (my first and only ultrasound).
After the ultrasound, they said everything looked
good. They said due to his head size I was further
along than they thought and they changed my due date
from May 1st to April 26. My doctor said take it
easy but don't worry everything was good.

But in reality I now know he just had complete
agenesis of the corpus callosum (c-acc) and
plagiocephaly and many other things that were
not yet discovered.

Then soon after my ultrasound I went to a routine
appointment on March 15 (six weeks before my original
due date). I told my doctor everything again about
the pain and problems with my second pregnancy and
the pain I was having with this pregnancy and I also
told him that while doing kick counts the baby hadn't
moved much. So he said go to the hospital, let them
hook you up and check everything.

As soon as they put me on the monitors to watch me
I started having bad contractions.

I was in labor for 24 hours. On March 16, 2010 our
beautiful son, Landon, was born weighing 5 lbs.
4.9 oz and 18 1/2 inches long.



The cord was wrapped around his neck twice and he was
not breathing right so they took him to the Special
Care Nursery which is the same thing as a Neonatal
Intensive Care Unit (NICU). Landon was considered
to be preemie when he was born.

I didn't get to hold my son for a week and a half.
He was on machines helping him breathe and we couldn't
even touch him. They were afraid too much stimulation
could make his lungs collapse.

He was on the breathing machines for almost two weeks
and on feeding IV's for about a week.

He didn't seem to see or notice anything when he was
messed with or touched so they weren't sure about his
eyes. They always said oh all preemies have to learn to
eat, breathe right and keep his own body temp up in his
own time.

Once he could breathe right and keep his body
temperature up on his own he was moved out of the
warming bed (closed incubator) into an open bed for
almost another week. Then we were finally able to
bring him home.

He was in the Special Care Nursery a month before we
could take him home. No one could tell us why he
wasn't gaining weight and not eating right or
focusing.

Once we brought him home he still couldn't eat without
it all coming back up right after. He started losing
too much weight so his Pediatrician and stomach doctor
put him in the hospital. He was put on Zantac when he
was born then changed to Prilozec then to Reglan but
changed back to Zantac because he had a reaction to
Reglan. Also he was on Neocate and Polycose--both are
supposed to make him gain weight but still didn't.

When he was four months old he was admitted to the
hospital because of inability to eat without vomiting.

While in the hospital they did ultrasounds of stomach
and head, a lot of blood work an MRI and CT scan. Finally
they had a diagnosis. They informed us that Landon had
complete Agenesis of the Corpus Callosum (c-acc),
Deformational Plagiocephaly, Septo Optic Dysplasia,
Optic Nerve Hypoplasia, Gerd and Gastroparesis and now
they say he has a growth hormone deficiency.

The doctors said they had never really dealt with
anything like this and all they really knew was seizures
were a big possibility as was delays, he may need a
feeding tube, may never walk or talk so I was horrified.
I could do nothing but cry and blame myself and
everything else.

He doesn't see very well, he doesn't move much, he
is over 10 months and he just now learned to hold his
head up. He still has problems with eating baby food.
At 10 months he is only 15 lbs.



Nobody around here seems to be able to tell me
anything but wait and see. They say he may never walk
or talk and are not sure where he will be mentally.



But no matter what happens Landon is a God send. He
changed everything. He has made me a better stronger
person and I wouldn't trade it.



I just wish I could take his place so he wouldn't
have to go through it.


Landon


There are many different stories and outcomes of
ACC and Moms-To-Be.

I welcome every story.

Do you:

Have questions?
Need support?
Want information?
Have encouragement to give?

Do you want to share your own ACC story here?

If you do, please leave a comment below or you can E-mail me:
hope@aracnet.com

Want to talk to other moms who have been there and
understand? Join the ACC Listserv e-mail support group.

I hope to continue sharing more of these ACC and Moms-to-Be
stories out in the open in an effort to inform, encourage,
support and help other moms (and dads) out there who are being
told that their baby in utero has agenesis of the corpus
callosum.

This ACC and Moms-To-Be section will always remain open
and available to anyone who would like to tell their story.

there is always...

hope is the sparkle
from the depths of the hole

hope is the candle
you light in your soul

hope is the glimmer
you sense in your being

hope is the jewel
so brilliant and freeing


© Sandie L. Davis

ACC and Moms-To-Be #8 Story

I was recently contacted by the mom of a little
boy who has Agenesis of the Corpus Callosum who
expressed her desire to tell her own Mom-to-Be
Story about receiving the diagnosis of ACC when
she was pregnant.

This Mom shared something with me....in her own
words:

"I am very passionate about helping others and encouraging
others through those hard times."

Thank you very much, Brandy. I am so grateful to
you for your passion to help others, for your
willingness to reach out to people in need and for
allowing me to post your ACC Pregnancy Story.



Written by Brandy:

My pregnancy with this child (child #2 for me) was
never really normal. It started off rough as I had
started to bleed early on during my pregnancy. I
felt in my heart that I was losing my baby. However,
my baby was a fighter and wasn't going anywhere!
After about the 10 week mark, I was taken off bedrest
and was able to resume normal activities. At 20 weeks,
I went in for my ultrasound. Like many others, I had
no idea that they actually checked for so much during
those ultrasounds! He was given a good checkup other
than he was a little on the small side and they wanted
to do a repeat ultrasound at 28 weeks just to check
his growth. Thinking everything would be normal
(this was my second child and my first child had no
issues at all), my husband went on his trip out of town
for work, leaving me to go alone for the ultrasound.
This was the first and only ultrasound that he had
ever missed with either of my kids. Everything
seemed ok during my appointment. The ultrasound
tech never said anything was unusual or made any
faces. She printed off my pictures and told me
that he was growing on schedule. She then told me
that she was going to put me in a room so that the
doctor could talk to me. My stomach sank. I knew
that this was not normal! The ultrasound showed
that his brain ventricles were enlarged. I had never
heard of ventricles in the brain! I asked the doctor
what all that meant. He was very upbeat and honest
with me. He wanted me to go to a maternal-fetal
doctor to be followed. I got an appointment the next
day. On my way home after hearing the news, I was
pretty numb. I didn't know what any of this meant.
I didn't cry, which was VERY unusual for me. I even
went to the grocery store on the way home. It
wasn't until I got home and called my best friend
that I broke down. My husband flew home the next
morning so that he could go to the specialist with
me.

The specialist basically told me that same thing.
Hydrocephalus and he would need a shunt. I did
lots of research on hydrocephalus. Some days I
was worried and other days I wasn't. At 36 weeks,
I was sent to the children's hospital for a fetal
MRI. A big pregnant woman in a little bitty
MRI tube = MISERY! Anyway, the results came
back that he had Agenesis of the Corpus Callosum
(ACC) and not hydrocephalus. His ventricles were
enlarged but stable and not changing. I was told
that his ventricles weren't necessarily enlarged,
they had just taken on a different shape since
there was no corpus callosum.

I opted to have a c-section to deliver him.
My first delivery was vaginal and did not go
well at all. I also wanted to schedule his
delivery so that we could have all necessary
doctors on stand-by just in case we needed them.
The delivery turned out to go smooth. He did have
to go to the Neonatal Intensive Care Unit (NICU)
for a night to monitor his breathing (not related
to the ACC) but was allowed to spend the rest of
our stay with me in my room. While in the NICU,
they did an ultrasound of his head and it confirmed
the ACC diagnosis. He also had an EEG which came
back normal. He was having a little difficulty
eating and that prolonged our hospital stay
by a day, but he caught on like a champ
(and he is now the best eating child I have
ever seen!). The neurologist visited him in
the NICU and then came to see me. He gave me
the best piece of advice regarding the ACC.
He told me to learn all I can about ACC and then
to forget it - to treat him like I would treat
any other child. My son is now 3.5 years old
and he has taught me so much in his short life.

He first started showing physical delays when
he was a few months old. He did not sit until
he was nearly 8 months, crawled at 11 months,
and walked at 13.5 months. He had been in
physical therapy through our Early Intervention
program since he was 6 months old. He was
discharged from physical therapy around 14 months
since he was really thriving in that area once
he started to walk. From 14 months until
2.5 years he was in a little occupational
therapy through the Early Intervention program,
however he really needed speech more than
anything. We fought and struggled with our
Early Intervention program because they just
could not provide what he really needed.

Finally at 2.5 years old, we got into a
private therapy clinic and had a wonderful
speech therapist who made leaps and bounds
with him. At 3 he graduated out of the
Early Intervention and we enrolled him in the
special needs preschool through our school
system. I was very hesitant to make that move.
In fact, I had pretty much decided that I was
not going to send him to school at the age
of 3. However, I have a wonderful friend who
had been down this road with her son who had
similiar issues. She pushed me to enroll him
in the special needs school. Boy am I glad
I did!

Until Logan was about 2.5, he was nonverbal.
He would say "mama" and "dada" on occassion,
but basically nonverbal. He made huge
improvements with his speech therapist, but
the special needs school has REALLY made him
bloom. Just thinking of his three angel
teachers makes me tear up. He now has a
vocabulary of about 150-200 words and is
doing two to three words sentences, though
many of his words aren't really clear
(but we can understand him!)

He has a sweet personality. He is pretty
quiet in a group and is very well behaved,
but once he is at home, he really opens up!
His behavior is pretty much perfect,
especially for a 3 year old. But we are
having one big issue and that is he screams
when his dad is at home. He loves his dad
and wants to always be with him and play with
him, but for some reason he just screams.
When he is just with me (or anyone else) he
does not do the screaming. As for Logan's
"screaming"... it is the most high pitch noise
you can imagine to come out of a three year old.
If it happens in a store (ie. Walmart) you can
definitely hear him on the completely opposite
end of the store. It literally makes me feel
like my head is rattling. I can't really say
what prompts the screaming. It seems to me that
he wants his dad to do everything with him
(or for him is more like it!) and wants his dad
to do it right that second. I was talking with
my mom today, who babysits Logan sometimes. She
said it is just like a Jekyll/Hyde transformation.
Logan is about as perfectly behaved as a 3 year
old can be with her (and everyone except his dad).
Then when his dad walks in the room, he just starts
with this high pitch, stop-in-your-tracks scream.
I'm beginning to think it is a mixture of language
barrier frustration, being spoiled by his dad,
and just being a stubborn and impatient three
year old. It has by far been the must frustrating
bump we have faced on this road.

I love my son to pieces. He has taught me so
many lessons about life. If I had the chance,
I would not change him for the world!


3-year-old Logan


There are many different stories and outcomes of
ACC and Moms-To-Be.

I welcome every story.

Do you:

Have questions?
Need support?
Want information?
Have encouragement to give?

Do you want to share your own ACC story here?

If you do, please leave a comment below or you can E-mail me:
hope@aracnet.com

Want to talk to other moms who have been there and
understand? Join the ACC Listserv e-mail support group.

I hope to continue sharing more of these ACC and Moms-to-Be
stories out in the open in an effort to inform, encourage,
support and help other moms (and dads) out there who are being
told that their baby in utero has agenesis of the corpus
callosum.

This ACC and Moms-To-Be section will always remain open
and available to anyone who would like to tell their story.

ACC and Moms-To-Be #7 Story

Soon after posting the previous ACC Pregnancy Story,
I was contacted by another mom, Andrea, who has a
son who was born with Agenesis of the Corpus Callosum
and she expressed an interest in sharing her own story.

Several months ago I came across Andrea's blog about
her baby boy and I have been reading her blog posts
so it was a very nice surprise to get an e-mail from
Andrea, for the first time, and read about her desire
and willingness to tell a very private part of her
life in the hopes that it may help others and to let
them know "that they are not alone." (in her own words)

It is truly a privilege to be able to post Andrea's
Pregnancy Story here for you to read.

Thank you very much, Andrea, for reaching out to
others from your heart.



Written by Andrea:

This pregnancy started off a little differently
from the very beginning. When I had discussed
with my OB-GYN that my husband and I were
thinking about trying to have baby #3, he went
ahead and sent me for some testing with a
high-risk maternal-fetal specialist due to the
fact that I had miscarried two times previously.
He didn’t want me to have to go through that a
third time before we did some testing to find
the cause. The initial testing showed that I
have a blood clotting disorder known as MTHFR,
which makes me prone to blood clots. So I was
put on a baby aspirin every day as well as 5
folic acid tablets per day. I also had to be
followed closely by the maternal-fetal specialist
throughout the pregnancy. This was actually a
blessing in disguise, because it’s this high
tech equipment and the highly-qualified
sonographer, who discovered Anderson’s ACC
before he was born.

We were so excited to find out whether or not
this 3rd child would be a boy. We already had
2 girls and while we would have loved another
girl just as much, my husband and I were hoping
for a boy. To be honest, I really thought that
was the main purpose of the 20 week ultrasound.
In fact, even though this was my 3rd child
(5th pregnancy), I didn’t realize how detailed
these ultrasounds are. I had no idea how many
things they check and measure. I mean, they
check for 3 bones in the baby’s pinkie finger!
Did you know that?

Throughout the ultrasound, we kept hearing the
sonographer ooh and ahh over our precious baby.
She kept saying how beautiful everything looked
and how wonderful the images were that she was
getting. She did reveal that the baby was a boy,
and we were overjoyed! It was about 10 minutes
later that her demeanor and voice both completely
changed all at once. She became quiet, concerned,
and intensely focused on something that she did
(or didn’t) see. She left the room, came back in
with another sonographer who then took her turn
looking at the screen, pointing to things on the
screen, and giving “looks” to the other lady.
Then they both left the room. At this point, my
husband and I both realized that something was
wrong, but we didn’t know what. All we knew,
was that they kept looking in the baby’s head at
his brain. The third time the lady came back
into the room, she told us that she was going
to put us in a room so the doctor could talk to
us and discuss the ultrasound results. I
stopped her mid-sentence and asked, “What is
going on? What is it that you are looking for
and can’t find? Or is there something you do
see that shouldn’t be there? We are completely
in the dark. We are scared. We need to know
what is happening.”

At this point, I hear the words “corpus callosum”
for the first time. I had no idea what it was,
or what it meant for my baby to not have it.
This is where my story really begins to differ
from that of others’. We were never told, nor
was it ever implied that we terminate the
pregnancy. In fact, at this first visit, quite
the opposite happened. When the doctor came
in to speak to us, she explained what the
corpus callosum is and what its job is in the
brain. She told us that the only effects of
not having one could be coordination problems
and possible seizures. I specifically asked
her what effects it would have cognitively on
the baby and she replied, “None at all.”

We actually left the visit feeling okay- not great,
but we still felt all would be fine. The doctor
told us that everything would be fine and more
than likely he would have no problems. It wasn’t
until I decided to do my own research that I
realized that this doctor had given me false
information. I began searching and searching
for anything I could find relating to ACC, and I
kept reading stories of people with ACC and it
didn’t take me long to realize 2 things: 1.
While all of the stories might share some
commonalities, each person with ACC is very
different. It is not a cookie-cutter diagnosis.
and 2. The doctor had not been completely honest
with my husband and me about the prognosis of
our baby.

I was outraged. I knew I could not continue
seeing a doctor who would not be up-front and
honest with me. I called my OB-GYN and told
them I wanted a 2nd opinion. They immediately
scheduled me an appointment with a different
group of doctors. In the meantime, we were
left to wait, to wonder, to worry, and to have
more questions than we had answers.

We received the letter in the mail to confirm
our appointment, along with the paperwork to fill
out for our first visit, and I noticed that I had
been made 2 appointments- 1 with the maternal fetal
specialist and the other with a geneticist for
“Genetic Counseling”. Keep in mind that at this
point my husband and I have not even accepted that
something is wrong with our baby. We are still
dreaming that when we go for the 2nd opinion the
ultrasound will reveal that this had all been a
mistake and the baby is doing well. Oh how we
prayed for the corpus callosum to be seen!

As you know from me typing our story, it wasn’t
there. However this doctor was much more open
with us. She wasn’t doom and gloom, but she would
present us with what she knew, and didn’t mind to
tell us that some things we would just have to
wait and see. We continued with this doctor the
remainder of the pregnancy.

As the pregnancy progressed, there was mention of
a small cyst, and also the fluid in the baby’s
ventricles was being monitored closely. However,
at each visit, the fluid stayed within the
“high-normal” range. Until the 34 week visit.
At this visit, the amount of fluid had increased
significantly- to the point that they were
concerned that the baby’s brain may be under
pressure. So it was decided to deliver the baby
via C-section at 37 weeks.

I was scared out of my mind the day of the
delivery. We still really didn’t know what to
expect. Would the baby have facial deformities?
Would he breathe okay? What problems would he
have when he was born? What about the fluid?
Will he need surgery for a shunt? There were
so many questions that couldn’t be answered
until he was out of my body and welcomed into
this world.

Anderson Knox Spears was delivered at 8:47 on
Tuesday, June 1, 2010, at a hefty 8 pounds and
2 ounces. Shortly after he was born, it was
determined that he was struggling to breathe
on his own and he was whisked away to the NICU
after I got one quick peek at him. I remember
that he was absolutely beautiful. I remember
the delivery being very peaceful. I remember
not being worried that they were taking him to
the NICU. I felt at peace with everything
now that he was here.



Later that night, I was wheeled to the NICU to
get to see my baby. I was not prepared for what
I saw. He seemed so fragile laying there with
all of the tubes and lines and machines. He
actually only ended up on a CPAP machine to help
his breathing, and he had a feeding tube in his
nose. He had an IV in his arm, and monitors on
his chest. He was in good condition, but to see
your newborn lying there helpless after delivering
2 healthy babies and getting to hold and love on
them immediately, there are really no words.



Anderson’s breathing condition had nothing to do
with his ACC. It had more to do with the fact
that he was born at 37 weeks and his lungs needed
a little more time.

While he was in the NICU, the testing began.
Chromosome testing, blood testing, MRI’s, xrays,
hearing tests, vision test, etc. Lots and lots of
tests.

It was determined that Anderson had hydrocephalus,
complete agenesis of the corpus callosum, and an
interhemispheric cyst. However, his chromosome
testing showed that he had no chromosomal
abnormalities.

At exactly 1 week old, Anderson underwent surgery
to have his VP shunt placed. His shunt is actually
placed in the cyst- rather than in the ventricles
in his brain. Come to find out, the fluid that
they had been watching was not in a ventricle- it
was actually in the cyst. It appeared at first
that the cyst had actually grown in place of the
right hemisphere of his brain. However, as the
shunt continues to work properly and the cyst
gets smaller, more and more brain tissue is
appearing. Apparently it is there, it is merely
hidden by the large cyst. He tolerated the shunt
surgery so well, that we actually got to come
home the day after the surgery.

Still not knowing what to expect, our family
prayed, we loved, we rocked, and we treated him
like a normal baby. His 2 sisters absolutely
adore him, and they have been the best form of
interaction and stimulation that he could get.
Anderson began to amaze all of us as he grew.



He is now 7 months old and continues to amaze
everyone who knows him. In fact, he is even
amazing the doctors. When they see his MRI
results and then they look at him- the 2 don’t
fit together. He is doing things that right
after he was born we were told he would never do.
In fact, when he was 6 days old, a doctor told
us he would be severely delayed in all areas
and we should go ahead and file to put him on
disability. We told him we were not giving up
on him. We were going to give Anderson every
opportunity to be successful…and what a wonderful
decision that has been!

At his 6 month developmental check up, Anderson
was on an 8 month cognitive level, and on a 4 month
verbal and motor skills level. He is sitting up
unassisted, but he isn’t rolling very well. He
can do it, but not without lots of encouragement.
He is getting a head-shaping helmet this month-
because he would not lie on the right side of his
head due to his shunt so his head is misshapen.
He is talking (babbling) more and more each day.
We couldn’t be more pleased with how he is doing.
He is a true blessing to our family. He is
defeating the odds that are against him. We know
he may hit some obstacles, but we take each day at
a time and focus on what he CAN do, rather than
worry about what he WON’T be able to do.

If you are expecting and have received this
diagnosis, it is not the end. It is the beginning
of a very special journey. Anderson is a wonderful,
happy child who brings joy to everyone around him.
We can’t imagine our lives without him. The truth
is that the doctors really don’t know. Every
child/person is different. The doctors do not
have all the answers. We have found that our God
is much bigger and more powerful than the doctors.
He is allowing Anderson to do things that are not
medically possible. We look forward to watching
him grow, develop, and continue to amaze all of us!



If you have any questions or would like to get in
touch with me, feel free to email me at: spears.andrea22@gmail.com

Also, if you would like to read more about our
journey with Anderson, visit our blog at http://spearsfamilyblog.blogspot.com


There are many different stories and outcomes of
ACC and Moms-To-Be.

I welcome every story.

Do you:

Have questions?
Need support?
Want information?
Have encouragement to give?

Do you want to share your own ACC story here?

If you do, please leave a comment below or you can E-mail me:
hope@aracnet.com

Want to talk to other moms who have been there and
understand? Join the ACC Listserv e-mail support group.

I hope to continue sharing more of these ACC and Moms-to-Be
stories out in the open in an effort to inform, encourage,
support and help other moms (and dads) out there who are being
told that their baby in utero has agenesis of the corpus
callosum.

This ACC and Moms-To-Be section will always remain open
and available to anyone who would like to tell their story.