So, "How long will they live?"
There is no standard treatment for hydranencephaly. Treatment is symptomatic and supportive. Hydrocephalus may be treated with a shunt.
The prognosis for children with Hydranencephaly is generally quite poor. Death usually occurs in the first year of life.
In a survey completed by 88 families of children with Hydranencephaly from 2006-2007, 69% of the children who had died, did so after their first birthday. The oldest of the children who had died was in their 20s. 62% of the children represented in the survey were over the age of 1 at the time it was filled out. The first year is the hardest for children with Hydranencephaly but survival is possible.
The oldest known survivor was 32, and was observed to have noncortical responses to auditory stimuli.
While I maintain the accuracy of the newly optimistic version of the Wikipedia definition, I regularly receive emails that challenge my accuracy... because it is "medically impossible to thrive without a cerebral cortex as defined in hydranencephaly." (yes, those are the exact words used in most every email received) and sadly medical text supports this impossibility, though it is clear that it is possible.
The Rays of Sunshine website conducted a survey in 2002 showing the average age of a child living with hydranencephaly is nearly 8 years. That is only an average, so as you know there are many children who do not live to be that age, yet many that live well beyond those years. In some of the instances, believe it or not, life was shortened due to a doctor refusing to treat a child properly upon the onset of one of the many complications that can arise. But, in my opinion, that number does not mean anything.... in fact, it should not mean anything in treatment options for our children.
On a personal level, those definitions and negative prognoses really affected me in the beginning... as I am sure they do every parent when faced with a diagnosis of hydranencephly for their child. The not knowing "when" was the hardest for me, not wanting to become too attached because I knew my son would be ripped out of my arms at any second without even a moments notice. Feelings of great jealousy over those who were so fortunate enough to just have never made the greater connection with their baby before they were taken away, then the greater guilt I felt for feeling that way to the many parents of children lost to still births or miscarriages. There is no way to weigh the heaviest of the two losses, they're both unbearably devastating to a parent...
One of the best bits of advice I have been given and continue to share with other families, is to "take many pictures, since you will never feel that you have too many pictures... when they're gone, you'll still feel as if there are not enough!" I am scared to not have enough, or that one day I'll forget my precious boy. That my mission with this foundation will die when his earthly body has failed his soul. I now only hope that I am given some sort of warning, that he passes as nearly half of all do of an illness since I know that he is going to earn his angel wings sooner or later. I may be the only mother on the face of the planet that HOPES for an illness to take their child, but I want the warning. Or I want the weeks, or maybe even months, of time that it takes for all systems to just "shut down". I want to know that there is something wrong, not feel as if I missed some sign or neglected to notice a problem at the end. That it doesn't happen unexpectedly, or more importantly, during these times that I feel he is doing so wonderfully.
More oftentimes, it has been said, that children with hydranecephaly pass away from a multitude of problems that build-up, making it much more difficult for their bodies to overcome even the most minor of illnesses. Respiratory conditions prove to become fatal quite often, which is a reminder as to why a case of the sniffles is big business in our households. The fact that I know the pessimistic mentality of so many doctors, scares me as well. Parents are left wondering that if their child were in trouble, would they receive the same life-saving procedures that a "typical" child would receive?
I'll be the aggressor as long as I know that my son is benefiting from treatment I'm fighting for. If there ever becomes a point that his little body is too tired to stay with me, it will be hard but I will find the selfless strength to let him go. It brings tears to my eyes to even type about it, we do know that there will come a time that this will happen. We embrace those that have said goodbye while learning from their grief as well.