As a group we have learned that things aren't always as dismal as stated in the NINDS Fact Sheet and have prepared our own "Fact" Sheet based on our experiences and our informal surveys done over the years.
note: Barb Alemán on behalf of the International Hydranencephaly Support Group
writes the following information. We are families of children with
Hydranencephaly, not physicians. The information is based on our experiences and
on the results of several informal surveys*, which we have done. It is meant to
be a balance between what information is currently available and what Doctors
tell parents. Text in italics is quotes from a variety of sources on the
Hydranencephaly is a rare neurological condition in which the brain's
cerebral hemispheres are absent and replaced by sacs filled with cerebrospinal
fluid. (National Institute of Neurological Disorders and Strokes NINDS)
The amount of brain tissue that each child with Hydranencephaly has varies from
child to child. Many of the children are missing most of their brain tissue
above the brain stem. Others have large sections of such tissue left, for
example parts of the cortex along the midline or in the occipital lobe (the
cortex at the back of the head). Sometimes the cerebellum and basal ganglia
are present and normal. ( http://chorus.rad.mcw.edu/doc/00194.html
). Sometimes they are also missing. Or there are small pockets of brain
tissue throughout. Some children also have damage to their brain stem in
addition to loss of tissue above the brainstem.
is considered to be an extreme form of porencephaly (a rare disorder
characterized by a cyst or cavity in the cerebral hemispheres) and may be caused
by vascular infections or traumatic disorders after the 12th week of
with Hydranencephaly usually appear normal at birth. Sometimes the head is
enlarged. "The infants' head size and spontaneous reflexes such as
sucking, swallowing, crying, and moving the arms and legs may all seem normal.
However after a few weeks the infant usually becomes irritable and has increased
muscle tone. After a few months of life seizures and hydrocephalus (excessive
accumulation of cerebrospinal fluid in the brain may develop). (NINDS)
There is no definitive treatment for Hydranencephaly. Treatment is
symptomatic and supportive. Hydrocephalus may be treated by a shunt (a
surgically implanted tube that diverts fluid from one pathway to another).
Conditions Children with Hydranencephaly may have:
children also develop obstructed or difficult breathing and need to have a
tracheostomy. At this time 7% of the children have a trach. Several of the
children have also needed help from a Bipap or a Ventilator as they get older.
The outlook for children with Hydranencephaly is poor. Death generally occurs
before age 1 (NINDS)
the 146 children we have information on, 93 are living and 53 have died. Of the
children who have died 40%** died before their first birthday. However of the
children who are alive, only 10% are under the age of 1. Our oldest member is
24. It appears that the first year is the most difficult for our children. If
they survive that year then many will live a good number of years further.
a child with Hydranencephaly has many challenges but also many joys. You
will learn that caring for your child cannot be done alone. You will need the
services of a variety of health professionals. But, you will also become the
expert on what is best for your child. With love and care a child with
Hydranencephaly can have a full active and happy life. They can even attend
school with typical children, go on trips, attend parties, and participate in
note: All the “statistics” here only represent what we feel are a small
percentage of the actual number of children with this condition around the
world. All the families have some sort of computer access to have found us.
Therefore these numbers may not be a true representation. The majority of the
families live in the US. We’re in contact with a few that live in the UK,
Australia, New Zealand, Canada and then one or two in other countries. We have
no members who live in Europe, Scandinavia, China, etc. There are also a number
of families who chose not to participate in the survey.
was conducted via a questionnaire online and via snail mail. The majority of
responses were from those who participated online.
This number includes those children who were still born, those where Drs
insisted on a too early delivery, those for whom treatment was denied, and those
who only lived a few days due to severity of their condition.
We have also put this fact sheet into a brochure in pdf format (you will need Adobe Acrobat to read it) that can be printed out and shared