Tuesday, August 23, 2011

The What, The When, The How...

Sometimes reality hits you with a sucker punch, when you least expect it. My reality always strikes when I read of another little bumblebee buzzing away to join the angels... something that, sadly, has been happening far too often lately. It always seems to come in clusters... 

The questions regarding death... these are the questions everyone wants to ask, but never actually does. Instead, they make assumptions or feel extreme pity and share their sympathies. Those that know the lives of the children living with hydranencephaly well, feel pity for the parents and/or caregivers, that they are either in denial of the actual prognosis or that they will be saddened to days without their child one day and not able to cope. Those that know of the condition only, and maybe just a little more about the wonderful days they live, feel sheer sadness for their inabilities rather than happiness in their exuberant joy of life. As if parents are merely prolonging the inevitable, in a selfish quest to sustain  life beyond a level of comfort. 

To fuel these thoughts are those in the medical field who encourage parents to terminate pregnancies upon prenatal diagnosis, or recommend starvation or over-medication to end the suffering of their child once they have made their unexpected debut. I cannot ignore that this happens, I witness it on a regular basis, and I only wish that more would refuse to ignore these cases. I lived it myself, battling doctors against termination and refusing to medicate with morphine as Brayden was an infant while also choosing to conquer feeding issues despite being told that my son would not thrive... where are you now, doctors, now that he is three??

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 this condition that my own son, and many of his friends across the globe, are living with...

So, "How long will they live?" 

I think that every time someone reads a post I've made stating that Brayden has hydranencephaly (in mention of a small milestone he has achieved), they immediately Google the condition and read the definitions found there. After personal research, if I've told them that he is missing a large majority of his brain, it is unfathomable to most that he can achieve anything at all. Fortunately, I took it upon myself to update the following pessimistic Wikipedia definition that ended with the below outlined prognosis, with a much more optimistic version that you should take a few minutes to read:

Prognosis
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.

Not only was this the first thing that people used to read, but the last thing they read is about the poor prognosis. I was given this information from Wikipedia as a resource from the doctors that presented the diagnosis after Brayden's birth, as I am sure other parents are give as well. The fact that death usually occurs in the first year of life, immediately assuming that I must be wrong in thinking that so many of these little ones are doing wonderfully... incomprehensible. I guess you have to be a parent of a child with a "terminal" condition to understand that there will always be the textbook definition and then there are the real-life versions. The truth you learn from others living similar life experiences as yourself, that is what ends up being more factual in the end, not the doctors and certainly not those evil textbooks. This is why a large portion of our mission is networking families, they are the true experts in the field.

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. 

It has been said that the first year of life with 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 my experience, and in networking with other families, the quality of life is what you strive to make it... the right support, resources, and positive influences are essential in helping these little lives thrive.

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...

It took a great amount of time, but I came to a realization and accepted that I was the fortunate one. The remembrance that no life is guaranteed, 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 had 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 lesson, 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 gift received.

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? 

This only proves another reason, why I want to know everything I can, why knowledge is powerful... and to continue to be the aggressor, helping other parents along the way, when it comes to treatment for our children.

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. 

Until then, parents can only provide their child with the greatest quality of life possible. I always encourage parents to allow their child to participate in everything they do, even if it is sometimes with a little less enthusiasm than the rest of the family. They can go everywhere the family goes, even if it's not a stroller/wheelchair friendly adventure, there are ways to accommodate and I'll be happy to share how we have. Always accommodating to be sure that they are able to live their little lives fully and, more importantly, at the highest quality possible. 

We will wake up each day remembering that the day is a blessing, and go to sleep every night thankful for that blessing... hoping and praying for another wonderful day. But only He knows the plan...regardless of what is written. I have yet, in all my research and family networking, found one child who had an expiration date stamped on their little bodies... 

"Believe in the Impossible"

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