I lived in denial for a couple of years, that we just may be the "lucky ones". The parents who did not have to watch their child, living with hydranencephaly, have seizures. Around 75% of hydran-miracles have seizures, mostly controlled with pharmaceutical intervention. However, even when my 'lil man's "spells" progressed in to more defined episodes, as opposed to the typical absence seizures (or petit mal seizures) that Brayden experienced since birth, I still allowed myself to play in to the neurologist's belief that these were not seizures... rather an exaggerated startle reflex. Afterall, the EEG showed no abnormal activities, which the neurologist quickly pointed out as evidence against possible seizures. After further thought, however, of COURSE it did since there is no cortex for the EEG to read activity from! And to add to this inability to read any sort of cortex activity is the fact that my 'lil man also has hydrocephalus, a severe build-up of cerebrospinal fluid (CSF) which also clouds the EEG's ability to see what's going on in there.
A quick recap: hydranencephaly is a condition where both sides of the cerebral hemispheres are damaged in utero, or sometimes afterwards, and replaced by sacs of CSF. There is a wide array of levels of destruction, but images do not really define who the child is or what the child will do. For example, Brayden does not have any visible cortex while some children do . Where on earth, then, would seizures come from since a majority of the brain is made up of the cerebral cortex... which just so happens to be the site of ignition in seizure activity?
Rest assured, seizures definitely exist in our little ones. Some instances may be stemming from preserved cortical mantle and/or cortex that is obviously abnormal. Other instances may be derived right from the stem... the brain stem, that is. Unfortunately, brain stem seizures are not believed to exist by many neurologists, mine included. However, while little research on the topic exists, it is believed to be a possible phenomena.
Sadly, this is another small portion of a life's journey... a huge reality to the hundreds of families who have a little loved one living with hydranencephaly. Now to convince the doctor, and not let him convince me otherwise.
First step in defining seizures, you can visit either the Epilepsy Foundation Web site or my previous blog post on the topic of Seizures for symptoms to look for and to help you classify what is going on with your little one during these scary moments.
For us, Brayden started having typical absence seizures (also called petit mall seizures) as a baby. These are pretty harmless, causing him to just stare off in to space for a few seconds at a time. There are no convulsions, no hypertonicity or muscle rigidity, no crying, no slowing of his breathing... literally easy to miss if you're not paying attention.
These seizures never really went away, but they're definitely not as common. They have progressed a bit in to more atypical absence seizures, meaning that they last longer and present quite a bit of tonic movement with them.Otherwise, they've been relatively replaced by a host of other seizures activities, which have gotten progressively worse as of late.
Most importantly, whatever is going on in there, I know that his consciousness is hugely impaired. Not a huge biggy for some, but for a 'lil man living with a condition deemed incompatible with a conscious life.. it's huge!! We can't afford to risk his moments of consciousness with unconsciousness at any rate. This titles him with complex partial seizures, simply due to this lack of consciousness.
The scariest part is the tonic stage. His muscles stiffen up so tight that he even stops breathing at times, if not completely than it is deeply shallowed. He turns completely red, blotchy and his eyes dilate considerably making these episodes look even more scary. They happen quite often during his sleep, which is why I'm thrilled to now be the "un" proud owner of a pulse ox machine. But, they happen during the day too... after tracking them for a while, there is no rhyme or reason to these episodes. Because of the rapid jerks, or myoclonic jerks, during and sometimes separate, he is also experiencing another epileptic symptoms associated with various other seizures.
I was against pharmaceutical intervention when these episodes did not seem to cause him much harm. Unfortunately, I'm now desperate for some because they pose a severe risk to his life and definitely his well-being. His heart rate skyrockets well above 160, generally around 180+, and because of the intense stiffening of his body, his breathing and swallowing becomes compromised too. Although he sleeps propped up, saliva will cause him to choke and vomit if he is not helped upright and helped to get things back on track. Other times, since it happens so suddenly, he bites his tongue or cheek causing it to bleed :(
These began by lasting simply seconds... but have progressed to about a minute, and sometimes more. If he doesn't have a serious choking episode, he will generally come out of it smiling as if nothing ever happened. If he comes out of it choking, he is so exhausted afterwards that he will either take a nap or remain pretty dazed for an hour or so.
So, today's the day I will present all of this info to his neurologist. I had hoped to capture an episode on video for evidence, but that's next to impossible when it lasts only seconds. But when seizures are suspected, this is definitely the best way to describe them to your neuro!