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!