For many families the first information they find on the internet about Hydranencephaly is this "Fact Sheet" from the National Institute of Neurological Disorders and Strokes. http://www.ninds.nih.gov/disorders/hydranencephaly/hydranencephaly.htm
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.
Hydranencephaly Fact Sheet
Please 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 internet.
Description: 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.
Hydranencephaly 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 pregnancy. (NINDS)
Cause of Hydranencephaly
Children 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)
Treatment: 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).
Other Conditions Children with
Hydranencephaly may have:
Some children also develop obstructed or difficult breathing and need to have a tracheostomy. At this time 12% of the children have a trach. Several of the children have also needed help from a Bipap or a Ventilator as they get older.
Prognosis: The outlook for children with Hydranencephaly is poor. Death generally occurs before age 1 (NINDS)
Of the 350 children we have information on, 162 are living and 188 have died. 16% died before their first birthday. Out of those who have died 31%** died before their first birthday. However of the children who are alive, only 5 are under the age of 1. Our oldest member is 32. 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.
Raising 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 community activities.
Please 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.
* Survey was conducted via a questionnaire online. The 1st had 107 participants and was conducted from 2005-2007 & the 2nd had 106 participants and was conducted from 2010-2011.
** 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
National Institute Of Neurological Diseases and Stroke website: http://www.ninds.nih.gov/health_and_medical/disorders/hydranen_doc.htm
Neurology Channel Cephalic Disorders
This article is also available as a pamphlet/brochure to be downloaded: Hydranfactbrochure11