one step forward, two steps back

Thanks for the theme song Paula Abdul!

So we had an amazing neuro appt on Friday, everything went better than we could have hoped. The results of our 24 hour VEEG were in and it appears that Oliver's odd behaviors were not seizures, and not only that but there weren't any minor subtle seizures picked up either. Which is great, even though he usually only has definite clusters several days to a week apart so we didn't catch one but it's good that there isn't something else going on that we've been missing. Not only that, but our neuro told us that his EEG is actually starting to look improved. There are 'state changes' which means that the patterns differ during asleep and awake times. Previously it was just 100% consistent chaos whether asleep or awake. So , he still has abnormal patterns but the suspicion is that they are trending towards improvement. Not a clean EEG but it's better than any previous ones so we hope that will continue to just get better and better.

Since Oliver had only had 2 small subtle seizures about a week apart prior to our appt, and his EEG looked better, and his development was completely stalled (perhaps due to meds), the neuro suggested weaning 1st one medication, and then maybe the other to try and figure out which med was helping. It seems that one (or maybe both) was doing something but we weren't sure which. The clonazepam is so similar to valium that it just has him very chill all the time, and perhaps that was limiting his development. So the thought was to wean that one first. We were going to drop a half tab each week until completely off the medication, calling neurology in the meantime if any seizure activity increased. If so, we'd adjust the taper of the meds and wean more slowly. If we successfully got him off of the clonazepam, we'd next tackle the Vigabatrin and try to very slowly wean him down so that he was off around his 1st birthday. Amazing. We left feeling so hopeful and optimistic. Our neuro congratulated us on being proactive by having all of our appts booked and going to mayo, etc etc. Smiles all around.

Several hours later, I got a call from daycare and could hear Oliver screaming in the background. He was having a seizure. Unbelievable. All weaning plans are now halted for a week to see what's going on. He sounded stuffy on Friday so we're hoping he is just sick and his defenses against seizures are lowered. He had another seizure Sunday afternoon as well. They haven't been this close together in a long long time. We're keeping him on motrin in case it's teething or an ear infection and I've been suctioning his stuffy little nose. Trying to keep him hydrated and happy. I am going to call his pediatrician today and see if we can pop over this afternoon to just peek in his ears and see if he has an infection brewing. Now we just have to hope that he gets over being sick, the seizures decrease again and the wean process can begin.

But this leaves me with so many questions. If he is going to still have seizures every time he's sick, shouldn't we keep him on medications to fight them? Why would a neuro recommend a wean of both meds if the EEG isn't normal and if the kid is STILL having seizures. granted they are few and far between but he's still having them. maybe he is just trying to figure out which of the 2 meds is helping the most and then he'll stay or go back on that one? Maybe he saw how horribly behind Oliver is in his development and wants to see where he would be without so much medication? I didn't question any of this at the time because he hadn't had these last two seizures yet and I agreed it would be nice to see if he had any chance of developing off the clonazepam.We'll never fully know if he is so behind based on the CDG, or the infantile spasms, or the meds. Or a combo of the 3. It's just so incredibly frustrating that every time we meet with our neuro, we start to let ourselves hope for a better outcome and then have all of that dashed when the seizures return. prior to Friday I had a very negative outlook on all of this, and then I allowed myself to be cheered up just a bit. But I think that's harder. It's hard to be positive all the time and just keep getting kicked down but if we don't stay positive than Oliver will think we're letting him down. It's just really hard day in and day out to pretend that things will be ok all the time. Even my super positive husband finally admitted last night that we might have to start thinking about things like wheelchairs and vans in the not so distant future.

Sick babies in the heat are not much fun for anyone. So Oliver spent most of the weekend inside with Jason while I embarked on fun adventures with Tobin. I know if a regular 8 month old had a cold and slight fever we probably would have done the same, but it feels more difficult because we have SO many of these weekends where we divide and conquer. Awhile back, before Oliver was ever diagnosed, I was watching an Oprah episode about a family who has a schizophrenic 7 year old. They live in such fear of her hurting her little brother that they live in separate houses. The parents take turns staying with the girl and her little brother. So the parents are never together. One is always with one child while the other is with the other child. I thought to myself, that must be so difficult on a marriage, but what choice do they have? I never would have thought that would be us one day. of course, we're not in separate houses but as I was out and about with Tobin all weekend I kept thinking of Jason and Oliver at home and wishing they were out with us. Tobin didn't seem to mind since he's a mama's boy. He had a blast at his first movie (Toy Story 3) and we went to the pool and swam for hours. He loves "swimming". He can't really swim yet but he likes hanging out in the water. Yesterday he learned to hold his breath and go under and then pull himself back up on the wall. I think that's a big step toward getting to be more comfortable in the water. He slept great last night as a result of no nap and lots of swimming!

Jason and I seem to have both caught Oliver's cold, which means it's likely viral and not bacterial so that's a good thing. But we both have sore throats and I am super stuffy with lots of sinus pain. No wonder Oliver is so whiny. Hopefully this will pass soon and the seizures will decrease again and we can move forward with our med wean plan. fingers and toes crossed!

now we wait

Spent the night at CMH for our overnight 24 hour VEEG. Relatively uneventful but I hit the button for any behaviors that seemed questionable. They will write up a report which we're review with our neuro next Friday. Until then, we wait and hope the seizures don't continue and keep praying that the behaviors we see are not actually seizures but just funky baby behaviors. We'll see.

I have a feeling we'll only get bad news as that seems to be all we hear lately. Reading up on CDG is depressing. So many children without the ability to walk or communicate with their family members. I know it's not the case for all, and I know it's too early to tell what will become of Oliver, but it's still really hard to think about all we're going to go through with him. It becomes so overwhelming that I try to just shut it out, but these thoughts always creep back into my head. How much pain and heartache can one family take?

A very silly nurse asked me this morning if Oliver was my first baby. I told her I had another son at home who is a great big brother. Then she asked when I was going to try for a girl. I very quickly replied, "we're not". She said, wow you answered so fast, how are you so sure. Um, lady, you saw Oliver's chart. He has a disorder that he likely inherited from us. Why on Earth would we bring another baby into the world and risk them facing the same struggles and pain that Oliver is? Why would we do that to ourselves? People are crazy. It's those comments that get to me. I can understand them coming from someone who doesn't understand that this is not something Oliver will outgrow. But it's unacceptable coming from a medical professional who should know better. It's things like this, and my stupid babycenter emails that tell me "your baby is 8 months and 3 weeks and is likely pulling to a stand". I don't know why I haven't unsubscribed to those. He doesn't pull to a stand, he can't even hold his own head up, and at this point is in worse shape than when he was 3 months old. What chance does he have to ever be "normal"? Absolutely none. So we push on, day by day, trying to be grateful for what we DO have. But it's really really hard.

busy weeks ahead

We've got a lot going on the next couple of weeks after a relatively quiet spell. We had 18 seizure free days. It was a good run. About a week into our dosage drop with clonazepam Oliver had an afternoon seizure this past Saturday. It was short and the spasms stopped after a few minutes. Afterwards he went back to sleep and woke up 20 minutes later in a good mood. Then, last night he had a really rough time but it didn't match his typical seizure pattern so we're unclear if it was actually a seizure or some other pain response or a new type of seizure or what. We tried an extra pill of the clonazepam, waited a bit and then tried motrin. He eventually settled to sleep. The hardest part is that 8 month old babies sometimes cry, have nightmares, have teething or gas pain, etc. We were so used to a baby that never cried unless seizing that we assume any time he cries that he's having a seizure. The crying wasn't like his typical cry that he has when he has a spasm but it was coming in waves like a spasm. Lots of lower lip pouting. Poor little guy. I just hope he gets through the next 2 days at daycare without a seizure and that we get some good feedback at his 24 hour Video EEG this week.

We go into the hospital on Wednesday morning and should go home Thursday morning. He'll be hooked up to the EEG machine the whole time we're there so hopefully it will give us some insight as to what goes on in his brain at various points in the day. If he is going to have more seizures this week, it would be best if they happened while he is hooked up to the machine. But hopefully soon after that we can increase his dose or change his medication again to get another seizure break. In the week on the lowered clonazepam dose he has been waking up a lot more at night, usually to laugh but we don't know what he's laughing at. I can't just enjoy it, because I have a feeling it's seizure related....but wouldn't it be great if those were a few hours of clarity and he was just making up for lost time by talking and laughing and practicing those skills? He has been more alert and aware during the day time too, which is nice. Feeling a little muscle tone come back and he's been reacting to the sound of Tobin's voice, which Tobin really likes.

He's still eating great and generally happy so we can't complain too much, but these seizures are just really painful all around. I hate that they are so stubborn and keep finding their way around / through his medication.

He has physical therapy tomorrow, then our 24 hour hospital stay and then next week we have eye dr appts for both boys , PT for Oliver, and a follow up with our neurologist. we also have been contacted by the speech therapist assigned by EI. She will be coming once a month and we should have our first appt in the next two weeks. Still waiting for a PT and OT to be assigned. We're doing as much as we can with him during his awake hours at home.

Aside from the seizures we had a fairly decent weekend, Tobin got a cute new haircut and Jason go to see the Cubs/White Sox battle it out at Wrigley Field. I took Tobin to a local street fest with our neighbors yesterday and the boys got to bounce in a bouncy castle, have corn dogs, lemonade, and funnel cake and then Tobin took a nice long nap and also feel asleep fairly quickly at bedtime last night. Might need to get a bouncy house for the backyard....

Minnesota or Bust

So we haven't seen any seizures since starting the clonazepam on the 24th. We were back in the ER on Sunday because Oliver had a fever of 102 and we had to follow our standard protocol of blood culture, cbc to check his absolute neutrophil count, and possible iv or shot of antibiotic. Jason spent the 7 hours there with Oliver while I put Tobin to bed and caught up on laundry. Sad that it's become a little routine. The good news is there isn't a bacterial infection and it's likely just a virus. He is now fever free and still has a bit of diarrhea but seems ok otherwise. I , unfortunately, caught the same bug and was super sick yesterday and still feel "off" today. Hopefully it passes quickly for all of us.

Interestingly enough, Oliver's ANC (Absolute neutrophil count) was 1192. anything above 1500 is normal. 1000-1500 is mildly neutropenic, 500-1000 is moderately neutropenic, and anything under 500 is severely neutropenic. Oliver has been in the severe category with counts under 300 for the past 2 months. Not sure if the increased count was due to the virus and his body fighting the infection or if his neutropenia is cyclical or if it's actually on its way back up. We have a hematology follow up tomorrow so they will likely do another CBC to see. if it's still up tomorrow and then up again in another week or so , I am going to ask the neuro to reconsider doing a short course of ACTH as long as his levels stay up while in the hospital. Would love it if the counts would go up long enough for us to take the opportunity to try a treatment that might clear his hypsarrthmia.

I got a voicemail today from the mayo clinic and they have us scheduled for Aug 16-18th! Finally, something to look forward to. I am very excited to have the chance to sit and talk with this dr who knows so much more about CDG and IS than anyone else we've met! Waiting on a call back from the neuro's office about the Clonazepam as they gave us a 30 days supply but never specified if he was to take the full course or if we need to gradually wean him down before stopping (or if they intend to stop)?

That's about all the news. We had a nice weekend before the ER visit. Spent Sat at a bbq at the neighbors and Tobin had a blast playing with his buddies. Sunday before the fever hit we went to the Morton Arboretum and Tobin got to catch tadpoles, play in the stream, and walk in a netted cage in the tree tops!