The questions regarding death...these are the questions everyone wants to ask, but never ever actually do. Instead, they make assumptions or feel much pity and share their sympathies. Those that knowBraydenwell, feel pity for me as his momma, that I'm either in denial of his actual prognosis or that I will be without him one day and not able to cope. Those that know ofBrayden'scondition only, and maybe just a little more about his days, feel sheer sadness forBrayden. As if I'm prolonging theinevitable, sustaining his life beyond a level of comfort. To break the boundaries, and prove that I'm not in any sort of denial at any moments time, I'll share what I know in regards to life expectancy and longevity, answer some "whats" or "hows" or "whens" of his condition...
So, "How long will he live?" I think thatevery timesomeone reads a post I've made stating thatBraydenhashydranencephaly(and mention a small milestone he'sachieved),Google'shisconditionand reads the definition. After if I've told them that he is missing parts of his brain, it's unfathomable to most that he can achieve anything at all.After all, the first thing that comes up is theWikipediaversion:
Hydranencephaly is a type of cephalic disorder. This is a rare condition in which the cerebral hemispheres are absent and replaced by sacs filled withcerebrospinalfluid.
This should not be confused with hydrocephalus, which is an accumulation ofcerebrospinalfluid in the ventricles.
In "hemihydranencephaly", only half of the brain is filled with fluid.
Usually the cerebellum andbrainstemare formed normally, although in some cases the cerebellum may also be absent. An infant withhydranencephalymay appear normal at birth or may have some distortion of the skull and upper facial features due to fluid pressure inside the skull. The infant's head size and spontaneous reflexes such as sucking, swallowing, crying, and moving the arms and legs may all seem normal, depending on the severity of the condition. However, after a few weeks the infant usually becomes irritable and has increased muscle tone (hypertonia). After several months of life, seizures and hydrocephalus may develop. Other symptoms may include visual impairment, lack of growth, deafness, blindness, spasticquadriparesis(paralysis), and intellectual deficits.
Some infants may have additional abnormalities at birth including seizures, myoclonus (involuntary sudden, rapid jerks), and respiratoryproblems.
Hydranencephalyis an extreme form of porencephaly, which is characterized by a cyst or cavity in the cerebral hemispheres, and may be caused by vascular insult or injuries, infections, or traumatic disorders after the 12thweek of pregnancy.
Diagnosis may be delayed for several months because the infant's early behavior appears to be relatively normal. Transillumination, an examination in which light is passed through body tissues, usually confirms the diagnosis.
Preliminary diagnosis may be made inuterovia standard ultrasound, and can be confirmed with a level II or higher ultrasound.
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.
Not only is this the first thing that people read, but the last thing they read is about the poor prognosis. The fact that death usually occurs in the first year of life, and immediately I must be wrong in thinking Brayden's doing wonderfully. I guess you have to be a parent of a child with a "terminal" condition to understand that there's a textbook definition and then there's the real-life lessons. The truth you learn from other's living similar life experiences as yourself, that's what ends up being more factual in the end, not the doctors and certainly not the textbooks.
TheRays of Sunshinewebsite conducted a survey in 2002 showing the average age is nearly 8 years. That is only an average, so as you know there are many children who don't live to be that age, yet many that live well beyond those years. In some of the instances, 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 doesn't mean anything. It's been said that the first year of a child with hydranencephaly is the hardest, and most pass away during those first 12 months...it is supposed to be easier after that point. Some live for 20 years, or even more. In our case we have almost 12 months down...it's a mystery to how many more to come.
Those definititions and prognosis descriptions really affected me in the beginning. The not knowing "when" was the hardest for me, not wanting to become too attached because I knew he 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. It took a great amount of time, but I came to a great realization and accepted that I was the fortunate one. The rememberance that no life is guaranteed, and that I have two other children that can just as easily be taken from me without warning, not to mention any other single person in my world that could leave it at any second. Life is not a guarantee, we are not insured to see another day. I finally came to a point when I could really enjoy the fact that I'd been blessed with the most wonderful gift I could ever receive, and am still in the processes of learning everything that this gift will teach me. The greatest gift, my ability to see every little moment as a blessing and to treasure every moment as if it could be the last, has been the ultimate first lesson learned.
One line I read, probably at the Rays of Sunshine website, was "take many pictures, you will never feel that you have too many pictures...and when they're gone, you'll still feel as if there are not enough!". I'm scared to not have enough, or that one day I'll forget my precious boy. I now only hope that I'm given some sort of warning, that he passes as nearly half of all do of an illness. 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's 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's doing so wonderfully.
More oftentimes, it's 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. I've mentioned before that I had to call 911 because Brayden couldn't recover from a cough (which later turned in to RSV). Respiratory conditions prove to become fatal quite often, probably the reason I freaked out so. The fact that I know the pessimism of so many doctors, scares me as well. I wonder if he were in trouble, if he'd receive the same life-saving procedures that a "typical" child would receive. Just proves another reason, why I want to know everything I can...and be the aggressor when it comes to treatment for him.
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, I'm selfless enough to let him go. Hard as it may be, and it brings tears to my eyes to even type about it, I do know that there will come a time that this will happen. Until then, he will live the greatest quality of life possible. He will always participate in everything we do, sometimes with a little less enthusiasm than the rest of us. He will go everywhere we go, even if it's not a baby stroller friendly adventure, and there have already been many of those. I will always be sure that he lives his life fully and, more importantly, lives his life at the highest quality possible. We will wake up each day remembering that it's a blessing, and go to sleep every night thanking the Lord for that blessing...and praying for another wonderful day. But only He knows the plan...regardless of what's written.