ramblinjaq

this is the second post about The Scary events of emmit having a seizure in january and us learning that he has a brain malformation. the first part is here.

we finally got a chance to sit down with dr. wilson, a developmental pediatrician at riley children’s hospital, to talk about emmit’s hydrocephalus and dandy walker malformation (DWM) and what the heck it means. all in all, we’re feeling pretty good and hopeful about emmit’s developmental potential. dr. wilson spent about an hour and an half with us and patiently answered our 2-month’s worth of questions. he also showed us the MRI images. emmit’s ears look supercute sticking out in the x-ray pictures of his noggin. imagine dumbo ears sticking out of a skeleton head. maybe that’s something only a mama would find cute?

i took notes during our appointment and tried to organize them to make sense, but what makes sense to me might not make sense to anyone else. or even be right, for that matter. there was a lot of medical-talk and emmit was climbing everything in sight and handling expensive medical equipment.

the most important thing that i learned and that i am compelled to communicate is this: emmit’s life expectancy is normal. there is no correlation of a shortened life span to hydrocephalus or DWM. as i mentioned yesterday, the one time i googled, i was punched in the gut by “sudden unexpected death” and it was the first question i had for the doctor.

alright, please bear with me. this is a lot of information. there are four separate issues that, of course, all tie together, but they each have their own luggage. the issues are: seizures, hydrocephalus, DWM and developmental delays.

seizures – which are a temporary alteration in brain function due to abnormal electrical activity (okay…maybe a little googling, but just for basic definitions) – are the most immediate issue to get a handle on. every seizure-prone person triggers for a different reason. fever (febrile) is the most common with children. in the ER, the docs asked if emmit had a fever recently. the looks on their faces when we said no scared me. how to control your “uh oh” face should be taught in med school. hard normal play, like jumping on a trampoline, is not a seizure trigger. the really icky thing? sometimes a brain just short circuits. isn’t that comforting? emmit is now on anti-seizure meds, which he takes twice a day. we’ll wait a certain amount of time (months? years?) that he is seizure free then try him off the meds. it’s all about the waiting. we also need to keep an eye on him for absence seizures, which basically look like spacing out, but you can’t get his attention. there is a chance that this seizure was the only one he was ever going to have.

now, ready for me to throw down my minimal brain knowledge? dandy walker malformation (DWM) concerns the formation of the cerebellum. the cerebellum is the communications center of the brain. emmit’s is under-formed, but he has one. some people with with severe DWM have none, I guess. the question will be what this means long term for his cognitive function, complex reasoning and motor skills, expecially coordination. there is also something about the connection to the 4th ventricle that, frankly, i don’t understand.

people with hydrocephalus have abnormal accumulation of cerebrospinal fluid in the ventricles, or cavities, of the brain. that can cause pressure inside the head. emmit is a big kid from a family of big-headed people. so there’s no way to tell, at this point, if his head size is communicating the hydrocephalus or if it’s just a family trait. josh’s head is 60cm, which officially counts as bigger than average (or abnormally large, as i’ve reinterpreted). emmit’s head, at 53 1/2cm is also above average (my head size is merely average, which some might find unbelievable). sometimes to relieve the extra pressure in the brain, a shunt is inserted. dr. turner, the pediatric neurosurgeon who saw emmit in the hospital two months ago feels that no shunt is needed at this time. we just need to watch for symptoms that the pressure has increased. symptoms like vomiting, headache, increasing developmental delay. but for now, no brain surgery – yay!

dr. wilson couldn’t make any promises or guesses about how severe emmit’s developmental delays will be. he suggested getting an IQ test done when emmit is older. he said there is hope becuase of the good prognostic indicators he observed in his office while emmit played and communicated with us, and from our anecdotal information. we need to work hard in the next few years on verbal communication. we want to make sure to give him every opportunity to learn.

what’s next?

1. we have an appointment in early april with dr. pappas, a pediatric neurologist. we’ll ask him the same things we asked dr. wilson, focusing on the hydrocephalus and DWM.
2. we are going to find a summer program for emmit since he responds so well to routine and school.
3. we are increasing his bi-weekly speech therapy to once a week.
4. emmit needs to go on a kinda-diet. at 42.5″, he is as tall as an average 5-year old and at 50lbs, he weighs as much as an average 7-year old. although he’s not fat, his bmi is high for his age – 99th precentile high. the extra weight could have a negative impact on his physical delays and low muscle tone. because he already eats quite healthfully – blueberries, yogurt, raisins, grapes, cottage cheese, crackers, cheese, tofu, brown rice - this mean fewer snacks and smaller portions. no more goat cheese and brie.  “i heavy” was one of the first full sentences he ever said. i heavy indeed, poor kid. we’re also going to try to just get outside and move as much as we can. that’s hard to do for one adult with emmit and jasper in tow, but there’s gotta be a way.

and some other interesting stuff

1. emmit has always been a crappy sleeper so we asked if any of these new-to-us things could be contributors. dr. wilson said that he might have a heightened anxiety level due to his delays. there might be emotional/psychological reasons behind his sleep disruptions rather than physical reasons. could be bad dreams that he isn’t able to communicate just yet. we’re going to start keeping a sleep log (fun!) that includes what happens during the day before he has disruptions. perhaps change in routine sets it off. we’ll look for patterns and maybe follow up with a sleep study in the future.
2. emmit’s esotropia (the crossy eyes) were not caused by or a symptom of the hydrocephalus or DWM.
3. emmit’s cognitive age seems to be around 2-2 1/2 which means that time out is okay for discipline and that we should not be too worried about potty training, he’ll do it when he’s ready. until then, we hope pampers comes out with a bigger diaper size than the sevens he has nearly outgrown.

thanks to everyone for all the love and kindness that has come our way. it is amazing to know that we’re not alone as we go through this new adventure and that there is a small army of people who love our boy.

a lot has happened since i last wrote. a lot a lot. life altering scary-ass a lot. and i’ve wanted to write about it. but i couldn’t do it. i was afraid that writing about The Scary would give it a voice so i’ve let a couple months go by without a word.

i thought maybe i could ignore The Scary and write about other very important topics. things like josh & my conversation about who the best male rock singers in history are (josh had to make two separate lists, one for regular boys and the other for metal boys). things like the struggle i’m having with loving nursing, but wanting to free my boobs from the peskiness of pumping. things like my new love for pink wines (to be clear, rose not zinfandel). things like 24, battlestar gallactica and how lame the new cycle of america’s next top model is. and things like jasper starting to walk and say the word “down.” okay, those last two shouldn’t be uttered in the same breath as a reference to david lee roth and freddie mercury, but my point is i’ve been blocked because of The Scary. and being blocked by The Scary makes it all the more scarier. so i shall jump in and write about it. and get it out of the way.

emmit had a seizure in january. it sucked. at the hospital, the doctors asked, “has his head always been this big?” i wanted to point to josh and my heads, like, “you think his head is big? i’ll show you big heads!” boy comes by the big head honestly is all i’m saying. after many tests, including an EEG and a CT scan, the docs told us that emmit has hydrocephalus and something called a dandy walker malformation (DWM) of his brain, which is a rare congenital malformation that involves the cerebellum and the fourth ventricle. my beautiful, silly, funny, loving, evil genius boy has a brain malformation. what on earth does a mama do after being told that?

the answer to that question, actually, is bake. then eat baked goods until they are gone. then bake again. in some cases, baking can happen before the previous baked item is gone. cakes, cookies, cupcakes, olive bread, boules. lather. rinse. repeat.

i am generally a researcher, but i’ve had to stay away from the internets on this one. the only time i poked around looking at DWM, i came across the phrases “poor intellectual development” and “sudden unexpected death.” i decided to only let the doctors tell me what i need to know. trouble is, we couldn’t get appointments until two whole months after the seizure. that’s a lot of time to sit with “sudden unexpected death” tooling around in my head. but it has also been enough time for my mama-confidence to grow in terms of making sure emmit is a person to the docs and not just a patient. it has been the right amount of time to help me remember that emmit is still emmit and he’s going to be okay and to stop sleeping on the floor next to his bed every night for fear that he would have another seizure, which shouldn’t happen because he’s on anti-seizure meds. strangely, the seizure was kind of a good thing. as it turns out, emmit’s DWM and hydrocephalus are the cause of his developmental delays. without the seizure, we wouldn’t have known to look at his brain. um…thanks seizure?

the most important thing to mention at this point is that emmit is doing great. wonderful, in fact. lately, his speech has taken huge strides. he’s making more full-on sentences and picking up vocabulary words like crazy. he’s a madman at the park, jumping and climbing and sliding and swinging. he’s just emmit. there is a huge spectrum of people with DWM (dandies?). some are very seriously sick. and some don’t know they have it until they are adults and something else brings it to the light. we already know, based on how healthy he is otherwise, that he’s on the better-case side of the spectrum. knowing what we face gives us the power to help him reach his potential.

tomorrow, i’m going to write about the MRI and doctor appointment we finally had last week. wow. it’s kind of great to write about it. i’d like to kick myself for being silly about The Scary, but i also trust that it feels good to write about it now because now is the right time.