Wednesday, July 28, 2010

Pay Day

The kids have been earning money around the house doing odds and ends. They started to do this so they could buy a Buzz and Jessie Doll.(Toy Story 3) It was really Kailyn's idea.  Her cousin had a Jessie doll and she wanted one.  I let her know that they were expensive and she could not just have one.  She asked if she could earn the money to get one and I said sure. I figured it would take quite some time. Maybe a month or more??
However, she really did "earn" that Jessie doll.  She cleaned and organized the house like I had never seen her do it before.  She did it like "I" would do it. Organized the closets, did the dishes, laundry, vacuumed, washed people's cars.  They both really got into it (Sam the window washer) and had the money well before I could even pay up.This led to Kailyn asking Seth when he got paid a few times because she knew that was when SHE would get paid.  Pay Day finally came and we went off to the store to spend their earnings.  This is the day that what Kailyn wanted changed.  She no longer wanted that coveted Jessie doll. She wanted to get something else, something more mature.  She ended up with this:
A sequin bag/purse, a butterfly ring, doubled layer scarf, necklace, lip gloss and hair products that the TV claims will make her hair full and shiny.  What happened to my little girl? She wanted nothing to do with all the toys in those toy aisles. It was her money she earned so she is allowed to do what she pleases with it.(within reason) She had a BLAST!!(We were in the store 3 hours!!)  Sam, on the other hand, ended up with just what he set out to earn and was quite happy too.

5 1/2 and 8(going on 18)

I don't want to offend anyone so this title is "Damné si vous faites damné si vous ne faites pas"

Hope you don't know french if you are offended by the phrase. lol   Anyway, Keira's Keppra med is not working. Her seizures have reduced, but not enough to make any of us feel comfortable (including Keira) Sometimes she cries after one and has refused to eat or drink because she knows that it is a trigger. The Ped Neuro is worried that she will a) suffer brain damage if we don't get the seizures under control or b) move on to the diagnosis of severe myclonic or some other Epilepsy if they don't stop. I knew that he said this was a possibility when he first diagnosed her, but we were so happy it was not infantile spasms and the MRI came out normal, any other form of Epilepsy was ignored.  We just felt relief.
At this point, she is not showing any signs of developmental delays, but she has lost skills in verbal areas. She was ahead in that area anyway. Regardless, she is still on track in all areas.  I was on top of that pre-diagnosis anyway.
The next line of medications to take on her type of seizures is Depakote.  This medication comes with a string of side effects with the most alarming one being liver failure. 1 in 1,000 infants end up with it.  He said the majority that develop liver failure have developmental delays already or an underlying metabolic disorder. It also can lead to pancreas problems (yes, he knows about K's T1D)  Knowing that our family is not favored when it comes to statistics, this makes me even more worried.  I just can't naively say, "What are the chances?" in my head, because my thought train is pretty good at predicting that ANYTHING can happen now.  Let's face it, lightening (so to speak) has struck us quite a bit lately.
After talking about it at length with Seth though, we have decided that we would rather get the seizures under control than risk permanent developmental delays. We will watch her like a hawk and get liver testing constantly if we have to. According to the Ped Neuro, he is pretty sure she has none of the underlying disorders.  So, tonight she starts Depakote. A hard core drug that is used as an anti-psychotic for bi-polar disorder etc.. too.  It's first use was for Epilepsy though. Here's to hoping it does not change our sweet babies personality, or makes her sick and gets the seizures under control.

Saturday, July 24, 2010

How things are

Things are going relatively well given all the excitement that has gone on.  Keira still has seizures, but they seem to have been reduced by the medication.  I think I counted around 20 today. Much better than the 80-100 a day I was counting.  The goal is to eradicate them altogether, however.  We had to increase the dosage and if they are not almost gone within 4 more days she is going to try another one. I would rather not do this because she does not seem to have any side effects from the Keppra. We notice the seizures the most when she has her bottle and this makes her not want to drink it. Not a good thing and all the more reason to make sure they stop.  I feel like the medication is allowing her to think more clearly and she has learned to clap and say yay, gotten back the ability to say bye bye, gives great kisses, high fives and says Dada like a pro.  I have tried repeatedly to get her to say "no no dada" like Kailyn, but my efforts seem to be fruitless so far. That was Kailyn's first official sentence.  She gets all over the place by rotating her body, rolling and occasionally pushing off on her feet to reach.
Kailyn is turning out to be quite the baker and little homemaker this summer.  She is trying to earn money for a Jessie doll (she actually has, just waiting for "payday" to buy it) and can now do the dishes, laundry, organize closets and drawers and do almost any chore for money.  I don't want her to do the toilets at this point.  Her blood sugar is good overall as long as we keep on top of her. She wants to do it on her own, but she forgets or calculates the carbs wrong.  The baking throws it off, but I am not going to deprive her of the experience just to keep blood sugar levels in check.  I just need to drive home that she needs to measure out what part of the bowl she licks or to not do it at all.  She made sugar cookies from scratch all by herself yesterday. I only put them in the oven.  I thought they were great, Seth thought he had an aftertaste of baking soda.  After she baked I had her check her blood sugar and she was 360 so I corrected and she ended up 38. Sooooo, we definitely have to watch her.
Sam had a ton of fun at basketball camp last week.  He did really well for never playing before and from what I could tell he was one of only a couple that had never done it before.  He came in last in the skills challenge (mainly because he can't reach the hoop), but it did not phase him and he wants to play this winter.  He did get the ball during one of the games and ran the ball all the way to the hoop (forgot to dribble), but when Kailyn did Kindergarten basketball this is how the majority of kids did it at first.  They don't call traveling at that age. It encouraged him to know that most of the other kids had played basketball for either 1 or 2 years.  It was fun to listen to other parents talk about how their child was a basketball prodigy (insert rolling eyes here).  We are talking 5-7 year olds. lol
We are working on getting healthier as a family.  No butter or treats in the house (unless Kailyn bakes) and the peanut butter will be hidden. We are also getting rid of extended cable/dvr.  Sam got in a habit this spring of eating out of boredom and watching more tv than he usually does.  Changing our cable package will only allow for mostly boring tv(with an added bonus of saving $35.00 a month).  We are making them go outside and I am walking again.  I got a 40 minute walk in today and my leg did NOT hurt. Woohoo!! My leg hurting afterwards was a huge deterrent for me.  That and sheer exhaustion.  Now that both have passed I look forward to my walks again. The kids start swimming again on Friday and then in the Fall Sam will do soccer and I am looking into Jukado for both of them.
I almost forgot to say that Keira has four bottom teeth now and is working on her top teeth.  She is pretty good natured about it.  I still say she is the best baby ever.  I love my other two just as much as her, but she is so easy I am in awe sometimes.

Friday, July 23, 2010

Keira has a new crib

and I think she loves it. (knock on wood)  She took a 1.5 hour nap in the am and almost a 3 hour nap in the pm. Having better sleep helps her seizures.  Being tired is a trigger.  Here are a few pics of her in her new digs. Thanks Nanie and Grampa!!

There are more pictures in the flickr photo stream below
She also has a new hair do. If I put her hair in a pony on the top it curls.  My Mom (Nanie) calls this a Tout. I have only ever heard that term from her, but whatever floats your boat. lol

Interesting Statistics

1 in 400-600 kids have Type 1 Diabetes (Kailyn), 1 in 100 kids have Autism (Sam) and 1 in 40,000 have Benign Myoclonic Epilepsy (Keira). I find myself wondering sometimes if there are any other people in the world like us. Not in a sad, this is not fair, woe is me kind of way. It is more like "Wow, what are the chances?"  There are groups on the Internet for Diabetes and Autism, but barely any Epilepsy ones let alone one for children with Keira's condition. If anyone knows how to calculate the statistics please feel free to do so and let me know the results.  I'm thinking it is in the millions. lol

Friday, July 16, 2010

Adventures of Benign Myoclonic Epilepsy in Infancy

Right before going in for her MRI. She was hungry and exhausted

In her hospital issued jail cell (I mean crib)

We have had a tiring 3 days trying to figure out exactly what Keira's "shuddering attacks" (as we described them) were.  When they first started happening during a viral illness around June 5th, they looked just like that. A shudder because of being startled. I decided to ignore the symptom in hopes that it would go away. People would comment here and there and it was in the back of my brain that there was something more going on, but I did not tell a soul I felt there could be something very wrong. I mentioned that I was worried to a few people, but I did not reveal the degree of my worrying. Seth even mentioned that he thought it was a seizure, but I tried to shrug it off, ignore it and put that worry in the outer most part of my head where it was hard to reach.  I did not want to talk about yet another illness this Robie family might potentially be handed to deal with. I shut down communications via Facebook so I would not talk about it to the masses. I felt that maybe I was being irrational in my thinking. I mean really, how many times can lightening strike the same family within a 3 year span? We had already filled one full hand. Could we really be starting to work on the second?

The greatest worry about the shuddering attacks, which we now know are seizures, was that it could be infantile spasms.  Infantile spasms can cause major brain damage leading to significant developmental delay and permanent intellectual impairment.  The prognosis is not good.  I did not think that this was what was wrong with Keira because she is on track and even ahead in some developmental milestones.  Most infantile spasm patients are delayed at onset. However, it was very hard to find other forms of seizures written about on the Internet because infantile spasms are the most frequent cause of them in children her age. I did finally find Benign Myoclonic Epilepsy, but you don't know for sure until you have an EEG and an MRI.

As of Sunday night,early Monday morning, I decided it was time to get it checked out.  The seizures were happening up to 80-100 times a day, came in three's and her eyes were rolling up in her head. They were clearly getting worse. I did not count them before then in my attempts to convince myself (which I really never did) that they were simple shuddering attacks. I got one of them on video and called our Pediatrician on Monday. We got an appointment on Tuesday. The Pediatrician saw them multiple times during the appointment and talked to the Neurologist right away while we were in the office. They scheduled the EEG for the next day (Wednesday), which clearly showed the myoclonic pattern.(A huge relief for us) We were given the diagnosis and sent to the hospital for observation, to start medication (Keppra) and get the MRI. Once we had the MRI done and they did not find any brain abnormalities the diagnosis would be set in stone.  Thankfully the MRI was unremarkable.

We are happy with the diagnosis. Of course, we would rather her not have this or take medication, but in the grand scheme of things it is minor.  We are on to the second hand and fully expect to add more fingers to it. We do hope that the pace of filling our hands will slow down. I still worry about her development and about the seizures, but I would be worrying about her development anyway. I was right on top of it before and will continue to do so. Keira is happy, otherwise healthy and the easiest baby I have ever known. A huge blessing to our family. :)

Here are pictures from our adventures.  She was quite a ham in the hospital and all the nurses/doctors were drooling over her. They could not get over how good she was.

This form of Epiliepsy is written about in detail on the website:

"Benign Myoclonic Epilepsy of Infancy
Of all the myoclonic epilepsies, benign myoclonic epilepsy of infancy (also known as benign infantile myoclonic epilepsy) is distinguished by its appearance early in life and its favorable prognosis. This belongs to the group of primary generalized epilepsies and is probably the equivalent of juvenile myoclonic epilepsy. Since its first description by Dravet and Bureau in 1981, many other cases have been described.


In 2005, Dravet and Bureau made an updated review of benign myoclonic epilepsy of infancy, in which they mention 120 cases.5 The syndrome occurs in 7% of children with myoclonus and 2% of epileptic children aged 1-36 months; 0.39 of epilepsies that begin in the first 6 years of life are benign myoclonic epilepsy of infancy. The male-to-female ratio is about 2:1. Age of onset is usually between 4 months and 3 years.5 However, as reported by Rossi et al in 1997, later onset up to 4 years and 8 months is possible.6


The genetics is unknown. In 78 cases, family history of febrile seizures was present in 17% and history of epilepsy was noted in 27%.

Clinical symptoms

Generalized myoclonic seizures occur in otherwise healthy infants, some of whom (about 25%) have a history of isolated febrile convulsions. Myoclonia involves the axis of the body and limbs, causing head drops, upward and/or outward movements of the upper limbs with flexing in the lower limbs, and possible rolling of the eyes. Seizures vary in intensity from severe forms (eg, causing the child to fall suddenly) to mild forms (eg, eye closure). Episodes are brief, lasting 1-3 seconds. Less commonly, episodes may be repeated up to 10 seconds, especially in older children. They do not occur in clusters and are not favored by awakening, but rather by drowsiness. Alertness may be slightly reduced with repeated seizures. The seizures are not associated with other seizure types such as absence or tonic seizures.

In a subgroup of patients, myoclonic jerks may be triggered by tactile or sudden acoustic stimuli, referred to as reflex benign myoclonic epilepsy of infancy. These may or may not be associated with spontaneous jerking. Although some authors have tried to distinguish between reflex benign myoclonic epilepsy of infancy and the classic form, Dravet and Bureau did not think such a distinction is necessary in their 1996 article.


Findings on interictal waking EEG usually are normal for age. Spontaneous spike-wave (SW) discharges are rare. Almost all SW and polyspike-wave (PSW) discharges have some form of clinical expression. Drowsiness and early stages of sleep activate generalized SW. During light sleep, they almost always are accompanied by myoclonus. The discharges gradually disappear during slow-wave sleep, as does the myoclonus. Clinical and EEG photosensitivity is present in one third of patients. Ictal EEG recordings show generalized fast SW or PSW accompanying the myoclonus. Darra et al reported on 22 patients with benign myoclonic epilepsy of infancy. They reported that the ictal EEG discharges were often focal, limited to the rolandic and vertex regions and concluded that seizures are rarely truly generalized.7


Seizures usually are controlled easily by sodium valproate monotherapy. If seizures are not controlled, other proposed medications include benzodiazepines (ie, clobazam, nitrazepam, clonazepam) or ethosuximide. Phenobarbital has also been tried.

Evolution and prognosis

The seizure outcome is favorable. The myoclonias have disappeared in all reported patients who were monitored. In most patients, they lasted less than a year, with the longest duration up to 6 years and 4 months. On long-term follow-up, 18% of patients had other seizure types, including some with rare generalized tonic-clonic seizures.

Clinical features as well as the associated EEG findings help differentiate benign myoclonic epilepsy of infancy from both nonepileptic conditions and other epileptic syndromes.
The following are nonepileptic conditions that may be mistaken for benign myoclonic epilepsy of infancy:
•Physiologic myoclonus

•Benign nonepileptic myoclonus

•Hyperekplexia (startle disease)

•Shuddering attacks

Epileptic syndromes that may resemble benign myoclonic epilepsy of infancy include the following:

•West syndrome

•Severe myoclonic epilepsy in infancy

•Early onset Lennox-Gastaut syndrome

•Myoclonic-astatic epilepsy (Doose syndrome)

Benign Partial Epilepsy With Complex Partial Seizures

Described by Watanabe et al in 1987 , complex partial seizures often occur in clusters and are manifested in the following ways:

•Arrested motion

•Staring spells

•Decreased responsiveness

•Automatisms with mild convulsive movements

Features of benign partial epilepsy with complex partial seizures include the following:

•Family history of benign seizures (often)

•Normal development prior to onset

•Onset at 3-10 months usually (ranges to 20 mo)

•No underlying disorders

•Normal findings on interictal EEGs

•Good response to treatment

•Normal developmental outcome"

Sunday, July 11, 2010

We have been busy Beavers

Sort of.  At least as busy as you can get with an 8 month old in tow.  Seth had the last week off and we went to China Lake, Nanies Beach and Toy Story 3.  In between all that, Kailyn hurt her eye, the kids swam in the pool, played make believe, made cakes and brownies and went to swimming lessons.  It's hard to do a lot with the baby since she loves her two naps a day, which are typically around two hours.   We can get away with one day messing up the schedule, but two days and she is toast.  Sam can't handle too much either.  He was in bed at 6pm this evening.  Kailyn is at that magic age where she can pull it together now whether tired or not.  Sam still has melt downs. 
The summer is flying like all the others and the older I get the faster they go by.  The kids start back to school on August 30th, which means we will have to start looking for school supplies soon.  They have not talked about school at all.  We did order new backpacks already.  Both of the rolling backpack zippers broke that they got from LL Bean last year so back they went. They are getting new Deluxe ones that should hold snow pants and shoes in the winter on top of all the other stuff they tote back and forth.
Here are a few pics from our adventures.  Others can be found in the flickr photo stream in the bottom right hand corner of the blog