Children living with a diagnosis of hydranencephaly are possessing very little to no cerebral cortex. Seizures occur when neurons within the cerebral hemispheres of cortex misfire and create somewhat of a electrical brainstorm. With those two facts presented, many doctors disbelieve in the fact that hydran children can have seizures... no cortex, no seizure... contrary to the finding of most with a confirmed diagnosis, are also being diagnosed and treated for obvious seizure activity with special diets and/or pharmaceutical interventions. This information is not only for those parents/loved ones... but for everyone, with another reminder that epilepsy does not discriminate, in fact affecting about 3% of the world's population.
I personally lived in somewhat of a denial for a couple of years, that we just may be the "lucky ones". The parents who did not have to watch their child, already battling so much living with hydranencephaly, also have seizures. However, even when my own 'lil man's "spells" progressed in to more defined episodes, as opposed to the typical absence 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. After all, the EEG showed no abnormal activities, which the neurologist quickly pointed out as evidence against possible seizures. After further thought, of COURSE the results were inconclusive without 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 many 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.
Brayden does not have any visible cortex while some children do have varying degrees of remaining cortex. Where on earth, then, would seizures come in a child missing cortex which just so happens to be the site of ignition in seizure activity? 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.
BRAIN STEM SEIZURES
Unfortunately, brain stem seizures are not believed to exist by many neurologists, in fact I have yet to meet one who does believe them to be possible. However, while little research on the topic exists, it is a medically possible phenomena which is supported by many articles and medical journals.
For Brayden, he started having typical absence 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, especially if you're not his parent and used to his "normal" eye movements.
These seizures never really went away, but they're definitely not as common. Part of the issue was believed to be weak eye muscles, which were strengthened with the addition of his infamous glasses. These seizures have progressed a bit in to more atypical absence seizures, meaning that they last longer, are much more obvious of being a complete mission to another world, 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 one living with a condition deemed incompatible with a conscious life.. it's huge!! We can't afford to risk these moments of consciousness with unconsciousness at any rate. This gave Brayden the official diagnosis of complex partial seizures, simply due to this lack of consciousness.
The scariest to experience: tonic-clonic seizures. 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, then awful shades of blue, blotchy, and his eyes dilate considerably making these episodes look even more scary (as his sisters would say, "zombie-like"). They happen quite often during sleep, which is why I'm thrilled to now be the "un" proud owner of a pulse oximeter to alarm us to these episodes. They do not have any rhyme or reason since they happen during the day too; no known triggers either. Because of the rapid jerks, or myoclonic jerks, during and sometimes separate, he is also experiencing another epileptic symptoms associated with various other seizures.
Seizures pose a severe risk to these little lives and definitely diminish their well-being. Heart rates skyrocket, for Brayden up in to the 180's or more, and because of the intense stiffening of the body, breathing and swallowing become compromised as well. Although Brayden sleeps propped up at an angle, saliva will cause him to choke and vomit if he is not helped upright, with lungs open wide, 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 are the worst episodes, which began by lasting simply seconds... but have progressed to about a minute or 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.
Please learn the signs of possible seizure activity, then do not let a medical professional convince you that treatment is not an option for your child with hydranencephaly because seizures just are not possible. The best proof to present to the neurologist: a video. Capture an episode on video for evidence, next to impossible when it lasts only seconds. But when seizures are suspected and you're battling medical impossibilities, video evidence is the best.