A Birthday and a Flash Flood of Emotions

People often tell me I'm a very strong person. Internally I tend to not agree with them as I feel like I'm falling apart much of the time. I am disorganized, forgetful, impatient and a bit of a mess. But over time I have come to realize that I can be pretty strong. It takes a lot of mental effort for me to get out of bed every day knowing that I'll be greeted with a) a crying little boy who is in so much pain for which there is no help, b) an energetic pre-schooler who just wants as much of my time and attention as I can give him, c) a cat who thinks the whole world revolves around her need to drink water from the faucet, d)a busy workday full of twists and turns, e) a house full of clutter and dishes and footprints and shedding hair, f) all kinds of other things I'm too tired to think of. But I do it. I get out of bed every morning and throw myself into the task of getting Oliver as happy as I can as quickly as I can so that I can get ready for work and help Tobin with whatever he needs. I get the boys to school, usually spend another 20 minutes trying to calm Oliver down and then I'm heading into work.

I'm physically and emotionally exhausted by 9:30am every morning. But I do it, again and again and again. Often with a smile on my face, usually with a laugh and a high five for the adorable toddlers on my way out of the daycare. Those cute little faces remind me so much of the life Oliver won't get to have. They are walking and talking and learning their numbers. Some of them are potty training. One of them pointed at me today and asked me "where is Tobin?". Complete sentences! Amazing. Things a normal two year old should be doing, things MY two year old should be doing. I am good at allowing these thoughts to quickly enter my brain and then I push them aside just as quickly because wallowing in the misery that is my reality is just not acceptable for a functioning life.

So, yes, I'm strong. I can leap over typically developing toddlers in a single bound and I can speak frankly when other parents look at Oliver's ankle braces with a question in their eyes. I can come home from work every day and greet my boys with a hug and a smile and set right into getting dinner made, doing dishes, preparing Oliver's keto meals for the next day at school and hearing about Tobin's fun apple experiment that he did with his preschool class. But, sometimes, I break down and cry. This can happen at any moment, in the car on the way home from dropping off the boys or late at night while I try to fall asleep.

Last night, as I got into bed I looked at the clock and realized that if Oliver were going to have a seizure, it would probably happen sometime in the next hour. So I lie awake, listening to every little noise coming from the family room, tensing my body when I would hear him roll or move. I just couldn't let myself sleep thinking I'd be soon awakened to a seizure. As I was lying there, my thoughts were drifting and I remembered the day we learned he had CDG, and how I was sitting in the hospital room holding him. He was 5 months old and so sweet and innocent and I learned that his life would never be how I pictured it. I listened to the doctor for over an hour, trying to understand CDG, not shedding a single tear. When she walked out of the room, I put Oliver in his bed and I broke down and cried. That helplessness, that feeling of utter heart break, it comes back to me sometimes when I'm lying in bed at night trying to fall asleep. I can't stop it. It comes in waves and I have to cry and cry until I'm all cried out. And then I go and sit and I look at his gorgeous face and just try to put myself back together.

I don't know what prompted my sadness last night, I've been doing ok for a good while now. I think it's that his 2nd birthday is approaching so quickly. I haven't even bought him a present. I'm just thinking to myself that I'd rather forget about his birthday. Is that terrible? Other parents who have children with complicated medical disorders celebrate every day, every week, every month with their children. Some kids with CDG aren't expected to live past age 3 (which is really a crock, most doctors don't know much about CDG and just say that). So shouldn't I be celebrating his 2nd birthday with balloons and confetti and a 9 piece brass band? I just get stuck on the fact that Tobin was potty training on his 2nd birthday, and doing puzzles, and eating cake, and all the other things that normal 2 year olds do. Meanwhile Oliver still can't do so many things. He's never once tasted birthday cake because he started the keto diet before his first birthday. He can't open presents. Any toys we bought him he wouldn't be able to play with. He doesn't need clothes because he has so many wonderful things from his big brother. What do you buy for the boy who can't do anything?

Maybe it's good to have super sad days to get everything out so that the other days we can be strong and positive and full of hope and inspiration. And maybe, someday, Oliver will get to eat birthday cake. It will be chocolate, of course, and I bet he will smile big when he tastes it.

Tough times and some good news

Oliver had a rough week last week. For the past month or so his seizures have been increasing to every 2-3 days. We had asked our keto team what the next best step would be…I wanted to decrease his diet ratio to 3:1 because I think maybe he does better at a lower ratio. He's been on the diet for almost a year now (WOW!) and they say their bodies get better at making ketones the longer they are on the ratio...also...small babies/little kids do better on a lower ratio most of the time. The keto experts wanted us to try increasing the fat content and going to the 4:1 ratio since we were already so close and since it would be best to force the extra fat content on him from the 3.5:1 ratio rather than going up from 3:1 ratio. So we started on Sunday August 21^st and it didn’t go very well. He didn’t eat much and when he did he just threw up the food. From Sunday to Wednesday he successfully held down one meal (220 calories!). He would not drink. He was lethargic and dehydrated. I had to pick him up early from daycare because he would not eat/drink and was crying all day. I brought him home, gave him some apple juice (not allowed on the diet) and some pedialyte and he instantly perked right up. The nurse said she didn't think he was too far into ketosis, or experiencing acidosis but I treated him as though he were. Whether it was that or just dehydration, the juice did the trick. After a few days I decided to put him back on the 3.5:1 ratio. We talked to the keto team and they agreed to keep him at 3.5:1 for now and repeat his blood work (which we did on Friday) and then talk to our neuro regarding next steps. We’re waiting on blood results now but I just think a lower ratio would give him some GI relief and maybe allow him to eat better and put back on some of the weight he’s lost.

On Friday we took him in for a GI scope to see what’s going on and if we could shed any light on his discomfort. They did biopsies so we will have the results on Wed /Thurs of this week. So far our GI doc has said his esophagus and bowels look fine but there was some inflammation in his stomach , which could be the source of the discomfort. For now we are increasing his zantac dose and hoping that being back on the 3.5:1 ratio will help him be more comfortable. My hope is that they let us try the 3:1 ratio. Even if it doesn’t help the recent seizure increase , my hope is that it will help him to be more comfortable, have fewer GI issues, and maybe gain some of the 2-3 lbs back that he’s lost. Also, while he was under the anesthesia, they removed his big toenail on his right foot since it’s been ingrown for a long time now and constant soaking/ointment/bandages have not fixed it. So at least he has some relief there!

Yesterday was the best day he's had in awhile and oh boy were we excited. He's an adorable baby even when he's crying and angry, but when he's happy there is nothing better in the world. The old saying "smile and the world smiles with you" certainly applies to our little Ollie. He brings us so much joy in his happy moments, his smile and laugh are contagious. He was eating pretty well and drank a bunch and I really think that made the difference. It's terrifying to think this diet may not be the miracle we were looking for, but in my gut I feel that we at least need to try lowering his ratio to get him more comfortable and eating and drinking better. Even with his increase in seizures I'd rather him be comfortable and seizing than miserable and seizing. Despite the increase in seizure activity he's making slow and steady progress.

Oliver is a rolling machine! He laughs! He smiles! He listens intently when I sing him silly songs! That alone is worth the struggle with the diet.

We started vision therapy a few weeks ago and we're working on creative ways to get his eyes engaged and focusing more. We also received some great news from EI, Oliver's equipment has been approved and in just a few weeks we will have a stander and a high/low seat for him to use at home (for feeding, therapy, sitting, etc). We are thrilled, and even though it took much longer to go through this process it also means we don't have to pay anything additional over our monthly EI contribution! I can't wait to get Oliver standing up and checking out the world.

Goodybe Reglan

Well we trialed Reglan for almost a full month and decided to take Oliver off the med. After a great first week he ended up eating less, being fussy more and having long bouts of inconsolable crying. He's still not eating great OFF the Reglan but he is MUCH more happy, comfortable and able to enjoy life. He has had 7 clusters of seizures so far in July, and we're not pleased with that number but we're hoping it's because of the Reglan and that things should settle down soon. If not, it could be the decreased calories because he hardly eats, the higher ketones we've been seeing, or something else entirely. Back to the guessing game!

A night in the life of Epilepsy

2:50 am: I hear a scream coming from the family room, where Oliver sleeps on the couch. Did I imagine it or is he having a seizure? I wake Jason and ask him if he heard anything. He didn't. A few seconds later I hear it again. We both jump out of bed and run to the family room where Oliver lays with his right arm stiff , convulsing.

2:51 am: I scoop Oliver into my arms and whisper "I"m sorry". I repeat this over and over again for the next five minutes. What am I sorry for? That he's having a seizure. That he was awakened from a peaceful sleep by a seizure. That I can't fine tune the diet well enough to stop the seizures. That doctors don't know which meds, if any, would help our son. Most of all, I'm sorry that I have a gene in my body that's been passed down through generations and generations resulting in Oliver having CDG. Isn't that silly? I had no idea, I had no control over this. But if Jason and I had somehow known we were carriers and that our children might be affected, well, Oliver might not be here to suffer like this.

2:56 am: I look at Jason and tell him "I'm really scared, he's been non stop shaking for over 7 minutes now. Get the Clonazepam".

2:58 am: Jason returns with Oliver's "rescue med" which is really just a benzo that his body is fairly used to and will likely help calm his brain but take 20 minutes to do so, it's our best bet at the moment though, to give him some relief.

3:00 am: Oliver is still having spasms , shaking in between. With each spasm he yelps like a wounded animal and lets out a little cry. Whoever says seizures don't hurt our kids has obviously not sat with Oliver in their arms while he seizes.

3:03 am: Oliver is still shaking but the spasms are smaller and getting further apart. I'm still in tears, sobbing and wondering how I'm supposed to do this for the next 10-20-30 years. I start apologizing to Oliver again. For things beyond my control, and things I think I should be able to control. I glance into my bedroom to make sure Tobin is still asleep and has not been disturbed by his brother's screams. Tobin is still sleeping on the air mattress on our floor most nights. He says he doesn't like to sleep upstairs in his room alone while we are downstairs because he gets scared, but I think it's really his way to be close to us since we spend so much of our time taking care of his brother.

3:10 am Oliver is still shaking and I contemplate calling an ambulance. With this new diagnosis of LGS I don't know what the protocol is. Wait it out or call for help? Then I think they probably wouldn't be able to stop the seizures any sooner. So I wait.

3:20 am: Oliver's spasms are few and far between and the general shaking seems to have subsided. He looks incredibly exhausted and sleepy. I hand him off to Jason to blow my nose and get a drink of water. My throat is raw from crying and my eyes are bloodshot. I look at myself in the mirror and think I've aged 3 years in the past 30 minutes.

3:25 am: Jason lays Oliver down to change his diaper. Oliver rolls to his left side which is his signal that he wants to go to sleep. Every 30 seconds another spasm rocks his body and he jerks awake, then settles back down.

3:30 am: Oliver's spasms seem to be gone and he is sleeping again. I notice the cat is almost out of cat food so I fill her bowl. I put away some clothes I had folded earlier in the evening. It's amazing how much our lives just "snap back to normal" once the seizure passes. Jason and I talk about the seizure Oliver just had and how it compared to the other ones we've had this month. We wonder if it's from the Reglan we started a few weeks back. Jason says "I hope it is, because the alternative is that his seizures are increasing/progressing because of the LGS".

3:35 am: We turn off the lights and crawl back into bed.

3:50 am: Tobin wakes up crying and asks to sleep in our bed with us. As he crawls in I grab him tightly and apologize to him as well. He asks "what for?". I tell him: "For everything".

LGS

After recently talking with our neurologist's office, we learned that Oliver's seizures and EEG pattern have progressed from Infantile Spasms with hypsarrthmia pattern to a slow spike and wave pattern consistent with Lennox Gastaut Syndrome (LGS). LGS is a devastating childhood epilepsy that can continue into the teen and adult years. Seizures come in many forms, can be hard to control and the constant background chaos in the brain leads to severe learning and developmental disabilities.

When Oliver first developed infantile spasms we were told he might never have another seizure disorder beyond Infantile Spasms, or they may progress into other seizure types/seizure disorders. At that moment, we had to hold on to the hope that our battle with IS would someday end and we would never see another seizure again. When Oliver was diagnosed with CDG, we were told he would likely always have seizures and that it was much more likely that his IS would be hard to control. IS that is uncontrolled generally turns into another seizure type down the road.

We started the ketogenic diet when Oliver was 11 months old, hoping that by the time he reached the age where IS turns into other seizure types (18-36 months usually), we would have things well under control with the diet and thus avoid other seizures types. As we went on with the diet, seeing improvements but not seizure freedom I began to feel the clock ticking faster and faster...against us. When we went in for our most recent EEG I had a feeling we'd get some bad news at the outcome. Somehow the "mommy intuition" never fails me even though outwardly I am hopeful and optimistic. I suspected all along, however, that some day we'd hear the words "LGS" in relation to Oliver's EEG.

So now we move forward, knowing that our son has just been given a life sentence for seizures. No matter how much we can help him to have fewer seizure episodes, he'll always have them. This chaotic brain pattern which can be worse than hypsarrthmia, will stick with him for a long time making every thing in his life that much more of a struggle. He's still our little guy and we'll continue to do every thing in our power to help keep him comfortable. And now that he's been given a pretty grim prognosis it means that each little inchstone he achieves will be that much more of a victory.

To read more about LGS: Click Here

June

Oliver did pretty well during the month of June. Back down to 3 big clusters for the month, which is much better than May so we're pretty excited. He did have some questionable seizures, small startles, staring spells and a possible complex partial seizure as well but we're still waiting to hear back about his latest EEG.

We started Reglan for his delayed gastric emptying last Saturday. So far we haven't seen a big difference in his eating or cranky episodes so we're not sure it will work. Luckily he hasn't suffered from any major side effects, so that's good.

He started July out with a bang and had a huge 45 minute seizure this morning that continued well after the administration of his rescue med. We'll see how the rest of the month goes, too early to tell if it's related to starting the Reglan or not.

Developmentally he's made some small strides, or "inchstones" as we like to call them. He has been doing a great job with our speech therapist, Emilie, trying to bring a rattle into his mouth when he's holding it in his hands. It's just the one rattle so far, so we have to go to Kohl's and see if we can find the exact one she brought over. It was so great to see him doing this though! He's also started to vocalize more and seems to be "taking turns" talking with us when he's in a vocal mood. He is just as smiley and cute as ever when he's happy and when he's cranky he gets very sad, but is still adorable.

We have both been very busy at work and Tobin is enjoying summer being here so that he can go to the pool and the beach and get Italian ice down the street!

The Waiting Game

Today we met with our neuro to talk about Oliver's recent weaning of meds and next steps with the diet and seizure treatment. We shared with him that February, March and April were kick ass months for us because of reduced seizure activity and that May and June have not been so great, mainly because of the increase in clusters since fully weaning Vigabatrin. Oliver had a 25 minute very intense cluster yesterday that stopped about 5 minutes after his rescue med was given at school. Scary and it really knocked him out for the rest of the evening :(

However, because the seizures haven't increased too much, the neuro believes there isn't any reason to medicate right now. If we had gone from 3 clusters per month to 20 clusters per month, we might consider adding a medication or going back on his previous Vigab dose. But since we went from 3 to 6...he still considers Oliver to be doing "very well" and doesn't think it's worth adding a med for a little bit of benefit when it would come with very big side effects. I tend to agree with him but still feel like a quitter for saying it's ok for Oliver to have seizures, as long as he doesn't have too many. Though we can all agree he's shown great alertness and awareness since coming off the medications and I wouldn't trade those smiles for anything!

Oliver was actually awake in his appt and in a good mood for the first half (the 2nd half he spent crying in his stroller while Jason pushed him around the waiting room). The doctor got to see all his new moves (he rolled both ways! he held his head up while lying on his tummy! he smiled! he laughed! he had more spontaneous movement in his arms and legs!).

Unfortunately our most recent EEG report was not ready and in the system waiting for him (we expected it would be as it usually takes a week and the EEG was a week and a day ago) , so now we wait to find out how much worse it looks compared to before when he was having fewer seizures....hopefully we'll find out very soon as it may change our decision to not medicate him at the moment.

Will post back when we know more!

June

We've had a lot going on in the past several weeks, hoping to figure out some answers for Oliver with his GI issues. After lots of phone calls and appts we finally got Oliver in for a gastric emptying study and determined he does have delayed gastric emptying. Basically the food sits in his stomach much longer than it should, and that is why he's never really hungry and also explains a lot of the reflux and GI pain he suffers. The full report is being sent to our GI doctor and then we'll find out next steps when we meet with him again.

Oliver also had an overnight 24 hour VEEG (Video EEG) this week. He did a great job but didn't have many of his staring spells while hooked up so I don't know that we'll actually know if those are seizures or not. He did have several of his small little random jerks and startles so it will be nice to see what types of seizures they are. He didn't have a big cluster while hooked up, which was expected because he never does...and because he was having a really good streak. Prior to today, his last big cluster was May 26th. Unfortunately, today the daycare called and said he was having a bad/long cluster and they were giving him Clonazepam to stop it. But this was his longest streak between big clusters since before he started the diet so that's good.

Oliver has been consistently rolling over just with his upper torso and always to his left side but it's a bit of progress. He also has been smiling a ton lately, which we're very grateful for. his bath chair arrived last night, woohoo! So we're excited to see if that helps him enjoy baths more. it will certainly make bath time easier on all of us.

next steps...

Oliver's seizures are increasing. We are holding our breath waiting to see if they will settle back down or if we will have to make a change to his diet or med plan. We finished the Vigabatrin on 4/28 and since then he has had 5 clusters of spasms, numerous random spasms and jerks at night while sleeping, staring spells before going to bed and some convulsing/shaking for 30 seconds or so randomly throughout the day. Aside from the big clusters of spasms, we're not sure if any of the other things are seizures or not but we're assuming they are.

Oliver did so great during his med wean but that last dosage drop seems to have really thrown him for a loop. I don't know if it is his brain protesting the last bit of the drug that we took away or something else. We have our next neuro appt June 15th and will be doing an overnight EEG just before that on June 7th. We will then plan our next step in the fight against seizures.

I got to a comfortable place. I was ok with his 3 clusters per month, happy to be taking steps toward more therapy and feeling confident in everything we could do to make him as happy and comfortable as possible.

Today I feel angry, frustrated and defeated. I feel bitter that I spent the weekend photographing and listing all of the baby gear and toddler gear for sale. Items that Oliver should have used like his exersaucer, bumbo seat and baby carrier that couldn't be used because he lacks head control. Tobin's old push car with a handle that we used to take him out for walks with. Oliver was supposed to be riding around in that this summer. He was also supposed to get Tobin's tricycle now that Tobin has outgrown it. All of those items were sitting in storage, taunting me every time I passed by them. I know it's healthier for me to get them out of the house (and maybe make a few bucks towards Oliver's savings account) but I can't help but think of the life that we imagined we'd live. Instead, we received a parking placard for our rearview mirror, to allow us to park in the handicapped spots when Oliver is in the car with us. It's official, the State of Illinois has granted our son "disabled" status. While the placard will certainly make it easier for us to get him out of the car and into his stroller or wheelchair, it is a concrete reminder that Oliver really isn't developing at all on the physical side. He's over 19 months old now and still can't hold his head up, sit, or roll over. Increased seizures on top of that are just not what we need right now.

We have a few options at this point. We can give his brain more time off the drug to see if it will settle down in the coming weeks, we can go back up to the small nightly dose of Sabril we were on before we lost control or we can increase his diet ratio. There are drawbacks to all of these options and I'm not sure which choice will be the right choice.

Once again I find myself angry that no one has answers for us, not even the experts. Epilepsy is totally unlike any other disorder because the brain is such a mystery. There isn't one solution for every situation. there are dozens, maybe hundreds of options/med combos/diet tweaks etc. It is mentally draining spending your whole day going through every little thing your son has eaten, drank, the time he ate or drank, what time of day he had his supplements, etc etc etc. I am tired. I am over it. There is still a long road ahead of us and some days I just don't have the strength to keep going. It's a lot of pressure as a parent to try and be your child's doctor, nurse, therapist, cheerleader, chef, teacher...when all you want most is to just be his mom.

Keto Appt

We had our 3 month keto appt yesterday. No changes to the diet for right now. Oliver had blood taken (he was not a fan of the process) and we'll know in about a week if he has any levels that are too high or low or of concern. He also received another vaccination and we've been told after his chicken pox vaccine in a month he'll be caught up on all the big stuff (pheww!). He slept through that shot, how nice!

Not much else going on, knock on wood but we're fairly stable with the same level of seizure activity, development, etc. Just trying to live life and enjoy some of the increased sunshine of late. It's wonderful!

May!

We made it through the month of April with only 3 clusters of seizures to record...but we were very nervous we wouldn't be able to do so. Oliver's last cluster was the 19th of April and we thought his clusters were getting closer together again. On 4/25 we saw single spasms throughout the morning so I gave him his first bit of Clonazepam in over 3 months. Just a 1/4 of a pill to see if I could head off any impending cluster. It seemed to work because Oliver did not have any clusters that day or during the rest of April!

He has , however, started May out with a bang. I woke up last night at 1am and I could hear Oliver yelling during intervals up in his bedroom. I finally came out of my sleepy fog enough to realize he was having spasms and woke Jason up to see if he though it sounded like a seizure. I don't know how long he'd been seizing and I feel awful that I may have left him up there seizing for more than a few minutes. The spasms were mild and not as close together when we brought him downstairs so I can only assume he had already been seizing for a good 5-10 minutes. We were nervous it had been going on for awhile so we gave him half a Clonazepam to settle him down and he eventually did , and was able to go right back to sleep immediately. He slept in our bed all night, because we were both so nervous about not hearing him. Luckily, Tobin didn't make his usual trip down to our bed!

I keep hoping this current bit of seizure activity is related to the end of the Vigabatrin wean and that Oliver will settle down in time and continue to see great seizure control in the coming months.

He's had a very rough weekend, lots of fussiness and crying (hopefully wean related) so we are all a bit tired today. The diet continues to be going well and we're even getting a lot of his therapies in now that he's more awake during the day.

Keto Tips

Someone on our keto diet group mailing list is assembling a list of tips for new parents starting the diet. She goes to Johns Hopkins and is acting as a parent advocate for other parents who are beginning their keto journey. I emailed her a list of my tips this morning to add to her handout for new parents but I thought I'd post them here as well for anyone about to embark on the wild roller coaster that is the keto diet:

My tips:

1. Supplies:
- masking tape and sharpies (for labeling pre-made meals quickly and easily).
- Gerber bowls: http://www.amazon.com/NUK-Gerber-Bunch---Bowls-Colors/dp/B000RFC3BM/ref=sr_1_1?ie=UTF8&qid=1298911571&sr=8-1
- good spoon shaped spatulas (I got mine at home goods)
- 2oz and 4 oz portion cups from costco or restaurant supply store

2. Foods:
- Don't make it a huge challenge for yourself the first weeks by trying tons of different recipes and experimenting. choose 2 simple dishes for each meal and alternate between them to first get your child into a good consistent level of ketosis and know that there are only a few ingredients that can be affecting the diet at first (keep it simple with whole foods, real fruits and veggies and only 4-5 ingredients per meal at most).
- Don't be afraid to give yourself a keto cooking break once in awhile and just use the ketocal formula or simple meals like eggnog. If you're rushing or going out to eat and you want to make it easier on yourself, these are great quick solutions.
- cook in bulk on the weekend. We make all our meals and elements of meals on the weekend so during the week we only spend about 10 minutes per day doing keto assembly (or weighing out fresh cream for the bottles,e tc).

3. General tips when starting the diet:
- Set a goal for yourself that is realistic. Don't expect "seizure freedom" from Day1 and don't be surprised if things get worse before they get better...treat the diet like a med in that sometimes you have to titrate up to the right "level" before you see good results.
- Don't make your goals related to just seizures...include things like increased awareness, development, attitude, etc and the ability to wean off of 1 or all of your seizure meds as a goal as well. This way you can still achieve something even if you don't get seizure freedom right away.

And my biggest tip of all: DON'T CARRY THE KETO WEIGHT ON YOUR SHOULDERS ALONE! Enlist a partner, husband, wife, mother, brother, sister, neighbor or cousin. Get 1-2 other people educated on the diet and have them ready to pitch in once in awhile with the cooking, meal prep, recipe invention etc. It's so helpful if you come down with the flu or have an off week or if you're just so sick and tired of weighing out cream and oil and butter to be able to take a break.

Weekend Update

Here's some photos of the past weeks....

Pictures soon, I promise!

We have the ankle braces and the vest! Insurance won't cover the hip helpers so the rehab clinic gave us the measurements so we can order them ourselves out of pocket. They are essentially stitched up shiny bike shorts and only cost about $15 so it's no big deal for us to buy a couple pairs on our own...

I will get some pictures of Oliver in his new kicks as soon as we buy him some big boy shoes this weekend and then I will post...

Today we had our stander fitting at RIC. We're getting this stander: Prime Engineering Super Stand in bright shiny candy apple red! The diva in me wanted the violet purple...the mom in me thought blue was more appropriate for a boy...and the mother to a special needs child with vision issues decided that bright red is best since it's the first color babies can see beyond black and white. It will probably be a long time before we receive it but I'm very excited!

Oliver was very fussy and cranky due to some GI discomfort when they placed him in the stander, so I spent most of his first time standing trying to soothe and comfort him...it was probably a good distraction because it brought me to tears to see him standing. I was so happy to see him standing and so sad at the same time that he even needs a stander. Very bittersweet moment. Luckily Oliver's crying and distress served as a great distraction and didn't allow me to wallow in my own misery. Smart boy!

We also test drove some chairs and will be placing an order through EI for a feeding/therapy/general hanging out chair very soon.

Oliver's ear infection is clearing up and he has been having a pretty good week aside from a seizure on Tuesday.

Spring is Here!

We've finally been able to get the boys outside at the park and enjoy some warm spring weather. It's been such a relief to get fresh air and sunshine! Oliver has been doing really well in the stroller as opposed to last spring/summer when he cried nearly all the time. It is nice for us to be more confident in bringing him out with his brother.

Oliver has his first ear infection since getting his tubes in. The nice thing about the tubes is that the ear just starts discharging all the gunk so we knew right away that it was infected and started the drops. Also, since he has the tubes to drain everything out, there was a lot less pressure in his ear during the infection so he never really got fussy or spiked a fever. It's so nice to be able to diagnose something in our son quickly and easily and start a treatment right away!

Oliver is now the proud owner of some ankle braces and a Benik support vest for his trunk support. We are building up his tolerance to the ankle braces by having him wear them for an hour more each day until we get to the full 8 hours. This weekend we are going to go shopping and buy him his first pair of shoes to fit over the braces, then we can start some weight bearing exercises! Thursday he'll be fitted for a stander, very exciting.

Eyes

I have been thinking about Oliver's eyes lately. I hate that he can't see. It might sound like one of the more minor issues considering everything Oliver has to deal with, but developmentally it really limits us.

When we first chose Vigabatrin for him as a medication we were told the risks would involve a decrease in retinal vision with long term/high dose use. I remember thinking "so what?". If it cures the spasms and gets rid of the IS, I can give up some vision. So what if he has to live in a city and ride public transportation instead of driving...so what if he needs a seeing eye dog? Those dogs are cute! I didn't quite grasp at the time that it wouldn't be his total vision but just his peripheral vision that was affected...I also didn't consider blindness to be that severe of a disability in general if he still had cognitive abilities and was physically able to walk, dress himself, etc.

Now as we're faced with accepting the fact that Oliver is going to be severely impaired both cognitively and physically, it seems rather unfair that he doesn't at least get the gift of vision. I think so many of his setbacks come from the fact that he can't see. Why should he roll over? what's tempting him on the other side, if he can't see a toy, a ball, mom, dad or brother? Why should he reach for a toy if he doesn't know it's there. How can he grab a cup and take a sip if he has no idea it's sitting in front of him? We asked for a vision evaluation from EI quite awhile ago. After months of hearing that they had no record of the paperwork we finally confirmed that our coordinator did submit the paperwork and that the vision therapists were going to contact us soon. It's been over a week since that promise. I , once again, have to pick up the phone and start bugging people. It's very frustrating. Hopefully we can get his vision eval sometime this month!

We received a referral from our developmental pediatrician to an organization called Lekotek. They have adaptable toys for impaired children and I'd love to know what they have that might help Oliver.

We did recently have another ERG to check retinal function. The results are similar to the ERG we had back in July 2010 and October 2010. They don't think his eyes are being affected negatively by the meds as of this point. That's good news and we hope that continues. In the doctor's note she mentioned nystagmus (which we knew about), IS (yup), CDG (yup) and exotropia (HUH?!?!?!). Apparently exotropia is a strabismus, or a turning of the eye. I have heard of CDG kids having this, but never Oliver. We'll have to ask her about it at our next appointment. In the meantime I've been researching the various diagnoses Oliver has been labeled with and found this great blog written by a young woman who has had nystagmus her whole life. It's interesting to hear about it from the perspective of someone who has grown up with it since birth. Click here to read more about it: The Shifty Eyes Blog

Seizure wise we were able to close out both February and March with 3 clusters each month. That's 6 clusters in the past 2 months. That's really low for Oliver, meaning we cut his seizures in half (at least) and the past 4 seizures haven't required any rescue meds. This is such great progress and is so encouraging to us to continue weaning his medications and plugging along with the keto diet.

So far for April our seizure cluster count is: ONE. we're only 4 days into the month so I'm hoping that he won't have many more but we are in the final stages of our Vigab wean and I know this could be the roughest part of it. This morning he was just off, and I could tell he'd be having a seizure at some point during the day. I was not surprised to hear my phone ringing 15 minutes after I left the daycare this morning with the report that Oliver was seizing. Today's seizure was a bit different. He had a ton of general whole body shaking throughout the 10 minute cluster of spasms. My poor boy. He goes through so much.

Tomorrow he has his 18 month checkup at the pediatrician. I assume our Dr will want to continue our efforts to catch Oliver up on the shots. I know he needs at least one more of the pentacel and the prevnar...I also know 18 months is generally the time our practice likes to give the MMR. So we'll likely do one shot per month for the next three months and then see what else we have left to give. I hope the shot does not derail our recent level of seizure control.

After the dr's appt we have a visit from a developmental therapist from EI. I'm not sure what developmental therapy entails but I'm excited to find out.

On Friday we go into RIC to pick up Oliver's AFOs, hip helpers and Benik vest. Then we'll be ready to do some shoe shopping to find REAL shoes that will fit over his ankle braces. I am so excited to get him standing a bit more now that he'll have the proper support. Fingers crossed there won't be any insurance hiccups that prevent us from getting the equipment!

I will try to get some updated pictures of Oliver with his new stuff and post them after the weekend.

EI Meeting

Our EI meeting went fairly well as we are going to start Oliver on more therapies and see how he tolerates them. He'll have PT and OT once per week each and then Speech twice a week with one of the sessions focusing on feeding. We are also going to have him evaluated for vision (finally, should have a phone call by the end of this week) and by a developmental therapist. We'll be meeting with a social worker once per month to help navigate resources available in the city/state.

A year ago , when Oliver was diagnosed, I never would have imagined a meeting in which I blatantly discussed the disabilities of my child and what we can do to make it easier on all of us. Even as I was sitting in the meeting looking around at all of the therapists, I was thinking to myself "I can't believe this is my life". It took a lot for me to be positive and ask the right questions and write down the tasks I need to complete without breaking down into tears.

I am learning more about navigating the special needs world and I'm finding that a lot of it is unfair. Kids that need so much receive so little. It really amazes me. I hope that we're lucky enough on our journey to get what we need for Oliver with minimal fighting but I think many things will be a battle.

The good news is that Oliver's bath chair has been ordered. The bad news is they never did order more supportive seating for him for meals and therapy. So we're going to go through the physiatrist at RIC and see if she can help us at least get him fitted for all of the equipment he needs and then we can try and order it through EI even if it takes a long time to arrive.

He has been fairly stable health-wise and we are so grateful for that. He's been teething up a storm and now has 12 teeth! The seizure count is lower the past 2 months and we're hoping that trend continues. We have about a month left on Vigabatrin and then he'll be seizure med free. We're crossing our fingers we can get to that point and see how he does without meds. During the wean we were hoping to see more alertness and strength. I'm a bit disappointed that we haven't as it means that a lot of his delays are likely caused by the CDG and not so much the medication. We'll see how his next EEG looks, hopefully he'll have one in the next two months.

On the GI front things are about the same though he seems to be doing better having bowel movements every day or every other day without help from suppositories. This is since starting the carnitor supplement. We hope to try the motility meds in May and see if that gives him the relief from the awful reflux that keeps him unable to eat most foods.

Tobin is happy, healthy, thriving and looking forward to warmer weather so he can go to the park and the beach. We consistently have temps in the 30s and we're all ready for spring to finally arrive!

ENT, ERG, AFOs , OH MY!

Oliver had his one month checkup at the ENT. He had an audiology screening to do the hearing test. They were unable to get the thing working properly in his right ear but his left ear passed the newborn hearing test again. They couldn't do the other tests because he's not 7-8 months developmentally :(

They recommended we do an ABR (sedated hearing test that measures what the brain processes through the ears) if he is to be sedated for any reason, perhaps when he's closer to 2 if we do a repeat MRI. His ear tubes are in and look fabulous. His ears look very clean and healthy.

He had his 4th ERG today to check his retinal function as required by SHARE while on Vigabatrin. NO changes since the last ERG and everything looks NORMAL! :)

We are just about 5 weeks from being completely off the Vigab medication so we're hoping that was our last ERG (maybe we'll do one more in about 6 months just to make sure everything still looks good).

GI Issues still there though he has some really good days with minimal crying (maybe 10-15 min fits instead of 3 hour long ones). We started Prevacid for the reflux in the am, while continuing Zantac at night just today so we'll give that about a month and then move on to the motility meds as recommended by the GI doc (we go back to GI on May 3rd).

Oliver should be getting a Benik vest, AFOs for his ankles/feet/legs and a hip helper on April 8th. On April 14th we go in to get fitted for a stander.

We have our annual EI meeting tomorrow afternoon and I will be asking again when our vision eval will happen (was requested a few months ago...apparently the place that does the evals is giving our EI coordinator the run around ), We also need to inquire about the bath chair and feeding chair we requested 4 months ago and ask for more services (would like speech and OT once per week and PT twice per week, along with feeding once per week). Wish us luck!

March Madness

March is full of many appointments for us. This morning Oliver went to the Rehabilitation Institute of Chicago (RIC) where we see a great physiatrist. He was fitted for AFOs (ankle braces), a hip helper (basically bike shorts that keep his hips in line) and a Benik Vest (it's a neoprene vest to help give more trunk support). We were waiting for all of this through early intervention but it was taking way too long to get going. The physiatrist said they could do it all through their office and had us scheduled for fittings within two weeks! Now we hold our breath praying insurance will cover all of this cool gear. It is very vital for Oliver to have the support that his body cannot provide to avoid risky surgeries in the future. No baby should have to go through hip surgery!

Next week we'll be having our first hearing test since Oliver was born and then we will follow up that same afternoon with the ENT to check out the tubes in his ears and make sure they look good. Later on in the week he has his next (and hopefully last) ERG to check his retinal function as a part of the Vigabatrin prescription. It's required by the company that makes the drug because there is a known issue of kids having permanent vision loss when they take the drug in high doses for long periods of time. On Friday we'll finish up our week with a meeting with our EI team. The coordinator, physical therapists, occupational therapists and feeding / speech therapists will all attend to make plans going forward for Oliver's therapy. We're hoping to get everyone to agree that he needs speech/feeding and OT more than he currently gets.

March also has us continuing our Vigabatrin wean. Oliver is now on 250mg in the morning and 500mg at night. This is step 3 of our 6 step wean plan so we're about halfway done with our wean. Knocking on wood as I say this but so far we've seen about 1 seizure on average during each 2 week stage of the wean! Pretty good!

Oliver's development is still at a bit of a standstill though he does seem to have more moments where he seems very strong as we continue on the wean. He is able to get some good head lifts in while on his tummy though he cannot hold his head up for very long. His vision is still pretty bad and that bothers me a bit as I had hoped to see improvement as he got older. I have to keep reminding myself that he has a lot of chaos in his brain and that maybe some day it will get better.

His GI issues are still there but he has some good days. I think the Carnitor supplement has helped a bit and we have not had to use suppositories in quite awhile. We have a prescription for Prevacid we picked up but we haven't started using it yet because we are nervous it will bring the seizures on (it contains aspartame, a known seizure trigger). Once we get settled into this current phase of the vigabatrin wean we will try the prevacid in the mornings with the zantac at night and see if that improves Oliver's reflux issues. They aren't too bad but he squirms a ton while eating and we can only think it's reflux or perhaps the infant high chair that he has outgrown (still waiting on a feeding chair from EI, hopefully that will come along soon!)

Everyone else in the family is doing pretty well. Tobin moved up to his Pre-K classroom at school and is really thriving there. He loves his friends and his teachers. We all took a one night trip up to Wisconsin to a hotel and water park while Oliver stayed behind with Grandma Patti. Tobin loved the attention and the fast waterslides. Jason and I really had a great time being able to just focus on Tobin and playing without worrying about meds, meals or bouncing the cranky baby around the house.

We are looking forward to spring and walks outside and the chance to get Tobin to the park to burn off some of that four year old energy.

Sabril Wean Step 2

Oliver had a great ear surgery. Did very well, no pain meds needed.

We met with a physiatrist and she is going to help us with some equipment and stuff for Oliver. Stander, Core/Trunk Support Vest and a hip helper to keep his hips from turning out all the time while he's laying down.

We dropped our evening Sabril dose last night and have now embarked on Step 2 of the Sabril wean....