Little Jonpaul update-June 12th

Hi all,
We've given this new medication a try (Clobezam) for about two weeks now, and still not working entirely. While he's having less clusters, he's still not seizure free. We're incrementally upping his dose in hopes of getting seizure freedom.

We'll give this medicine a few more weeks or so, and then it's off to a few more possibilities. Likely either a special high-fat diet called ketogenic diet, or another round of steriods. In the meantime, little JP seems to like doing his exercises, to a point :). Most days he's pretty socialable and happy to be around all the family (especially his sisters!), but other days he's a bit more dazed. Considering how medicated he is he's doing pretty well overall in terms of his interaction. We have those weekly physical therapy appointments that benefit him greatly, and every day Cortney and I work with him doing exercises.

The girls are great. Skylar and Stella are both done with school and enjoying summer so far, although Cortney and I with they would take to sleeping in a bit now that there's no school! Soleil is turning into quite a little ham and brings quite a comedic element to every day life around here....They all have quite different personalities, it makes the house quite uptempo and fun...and busy too :)

I hope you all are enjoying summertime!

Little Dude Update May 30th-he rolled over!!

Hi everybody. A quick update on little dude JP....

We started a new medicine this last Friday in hopes it would help corral or stop the clusters of spasms little Jp is having. We are at about 4 or 5 days into it so far and while it's helped, it's too soon to declare it help or failure yet. He's still having one or two clusters of spasms per day.

He was officially evaluated by developmental specialists last week. He was scored at 3 or 4 months for his development (both motor and cognitive), and he's almost 7 months old. So, as we expected, he's behind a bit. The good news is that we have a fantastic therapist that will be coming to our house once a week who will work with him, and show Cortney and I exercises we can do with him to help out on a daily basis.

I think little JP may be a fighter...I don't mean to sound cheesy here, or to use an old cliche, but...so far, he's doing something that is very unusual for kids who have his condition. When he had his first therapy session last week, he was simply laying on his back, not moving around much to his sides. After one session, and then Cortney and I doing exercises with him every day, he actually ROLLED OVER yesterday onto his back. This is a BIG change from only week ago, and when we do exercises, he really gets after it, and pushes himself. It's been wonderful to see just that little bit of progress since we started therapy...

The reason this is unusual is that nearly all kids who have hyppsarythmia like he does will have total arrest (or complete halting) of any development. They usually stop cold turkey and stay that way until the hypps is gone, or often times go backwards quickly. For whatever reason, little JP is fighting forward, albeit slowly, despite having this severe disorder.

He may continue this way, he may stop and regress tomorrow, we just don't know how this will end. BUT, Cortney and I are trying to take small victories when we can, and yesterday with the rolling over is one of them. We root him on, as do his three older sisters, during every exercise session. The therapist had him on an exercise ball yesterday, and I'll try to post the great picture we got of that on here soon.

We are so thankful to have found this great therapist, who is just fantastic with JP and the entire family as well, as she has the girls help out as to make it a family thing. Hopefully little JP can continue progressing forward :), one rollover at a time!!

Will post again in a week or so....

Love,
The Seivane Clan

Little JP update - May 21st

Little JP is still having his spasms unfortunately, although we're still fighting them tooth and nail with medicine changes. We saw a specialist at Stanford Hospital last week who is a premiere pediatric epileptologist, specializing in pediatric seizures. While he obviously didn't have a "magic cure" for this, he did clearly lay out our options and some sort of plan of attack. We are continuing to raise his current medication level of vigabatrin incrementally, which is now at FOUR times the dose (and is now maxed out, we can't go any higher) it was at when the seizures stopped in March.

Next up is a new medication that while not specific to Infantile Spasms, it helps all seizure types to some degree. If that new medicine doesn't work, then we'll likely try a new round of steroids again. For now, if the seizures don't stop by tomorrow we'll be adding that new general medication, which is called Clobezam.

In the meantime, we are having him evaluated tomorrow officially by the pediatric development medical folks to assess if and how far "behind" all this has put him. We are quite certain that physically he is behind, although his improvement over the last month has been quite good.

Many folks ask me what this means for him, as far long term. Well, there is no way to know that at this point. The fact is that the spectrum for these kids is so vast that we won't know how he's going to end up until he's older and things continue to move along...obviously during these episodes when he's having his spasms, it means that his brain has the hyppsarhythmia going on which is very, very bad for his cognitive development. We will continue to fight for this little guy and do all we can to get that hypps removed from his little brain...in the meantime we are loving him up at every turn and he's quite the happy little guy (when he's awake, this increased medicine dose is making him sleepy).

The family is doing well. Skylar is almost done with Kindergarten!! Stella and Soleil are doing great, all the girls are growing so fast and are little sweethearts. They are all so kind to little JP. Cortney and I are doing okay considering the circumstances, we realize this may be a long, drawn out battle with these seizures and are taking the motto of just continuing to move along with life as much as possible and not dwell. It's difficult sometimes however when we think of little JP and how he will be when he's older and life may be like....but we are doing all we can for him and that's what we have to hang our hat on every day. It's certainly not easy though...Cortney and I probably don't go more than 5 minutes in a day without thinking about it at some point...

Anyway, I'm blabbing on and on I know..but writing this is a sort of therapy for me as well....I'll update here in a week or so...I hope you all our family and friends are doing well and ready for the summertime!!

Love,
JP, Cortney, Skylar, Stella, Soleil and Little Jonpaul

Little JP's seizures are back - May 5th

Hi all, after finally gaining some momentum and little JP showing really positive signs of developing along, those little horrible spasms came back around Saturday afternoon (5/5), and have worsened quite a bit since then. We have doubled his dose of medicine in hopes of slowing them down or even stopping them if possible. We did get an EEG a few days ago and the hyppsarhthmia is back officially.

We did enjoy a good six weeks or so with little JP with him not seizing and it was great, we feel like we finally got to know him a little....we are obviously very disappointed that he is seizing again and his socialabililty is decreasing slightly now which is a major bummer.

I'll try to post an update here in the next few weeks, hopefully with better news. We are doing our best to not lose our minds, however this situation has just been so difficult on us all. The girls are doing well, progressing along great, the older two seem to have an idea that little JP isn't doing well and seem concerned about him. It's quite sweet really. Anyway, that's all for now.

The Seivane Clan

A few more days in the hospital...Saturday April 7th

Hi everybody, we had an eventful last few days and just returned from the hospital late last night. On Thursday morning, JP came down with a rash and low grade fever and his fontanelle (the baby soft spot on the top of the head) was bulging. Both Cortney and I knew this was not normal, and we took him to the ER. After spending 12 hours in the ER, we were finally admitted for the night and stayed another 24 hours or so.

What was found was that little JP's CSF (cerebrospinalfluid) was too highly pressurized, causing his fontanelle to bulge. He doesn't have an infection, and an MRI on his head was clear of any problems, so at this point they are chalking the pressure up to a likely virus which may be causing the rash also. At this point the pressure in his CSF is stable and not affecting his eyes or causing other problems. If at any time the pressure worsens more and starts to affect his vision or cause problems, he will likely get a shunt placed in his head, which is a small tube put in his head that drains extra fluid down into his stomach. Again, this is only if his CSF pressure worsens...for now, if it remains stable or gets better, he won't get the shunt. We just have to wait and see and watch him closely over the next several days.

It is totally unknown if this new problem is related to his seizures (he's still not having any seizures at all). After talking to multiple specialists over the last few days, one thing that is clear is that nobody knows the relation between this new issue and his seizures, or his medications, etc. We are certainly in a bit of grey area in that regard. We are meeting with one of the only pediatric neurosurgeons in the Sacramento area this Thursday to discuss the shunt possibilities should that become necessary.

Little JP, since he stopped the steroids, has been coming back to a normal little baby (aside from his fat little cheeks)!! He's smiling, even cooing at us, loves to smile and touch his mom and dad's face, and generally seems much more comfortable. He's a wonderful little guy and we just love getting to know him better again...

Love to all, and thank you for the continued notes and calls of encouragement and love! We certainly appreciate it very much!

Love,

The Seivane Clan

Little JP update, Thursday March 29th

Hi everybody, a quick update on little JP.

Since the good news of last week, things have kind of settled a bit, although we did get word that while his EEG didn't have any hypssarhythmia, it did have a bit of other irregular seizure like activity (waveforms in his brain, not ACTUAL seizures that we can see). While we knew very well that he will always be prone to other seizure types now, this news confirmed that while the infantile spasms are now controlled, there is potential for him to have other seizures essentially at any moment. It's possible he may not have any ever, or it could be tomorrow that he has several. We simply don't know. All we do know is that day by day we try to appreciate the fact that he is not seizing, and that is a good thing.

We are currently trying to figure out whether we can treat this irregular brain activity or not. Pediatric Neurologists are split on whether to treat these irregular brain waves even if there isn't actual seizures going along with it. Our Neurologist is on the side of not treating the abnormal EEG findings unless there is actual seizure activity along with it. For now, we'll be monitoring him closely as normal.

This EEG finding really reminded us clearly that we are on thin ice at the moment. We hope to get to more solid footing at some point with a true clean EEG that doesn't show abnormalities. It may take awhile but we hope we are there at some point.

As we are currently weaning him off of the ACTH (the steroids),  his personality is coming back just slightly, as we are catching a few smiles here and there and seeing more of his personality. It's very refreshing as we really haven't seen much of that for nearly a month. He only needs 5 more injections of this medication until we are finished. He will continue to stay on his Vigabatrin indefinitely as this point as that is the medication that controlled the spasms.

That's it for now. Take care everybody!
The Seivane Clan

Little JP update, Wednesday March 21st - Finally some good news!!!!!

Hi everybody, just got back from the hospital and finally received some good news. The hypsarrthymia, or abnormal brain wave, was GONE from his brain wave study. He had a normal brain wave reading...So, for now, he is officially considered "seizure controlled". This is great news as having no seizures is only part of the path to recovery, the other was the absence of that hypsarrthymia, and he now has both. This was GREAT NEWS!!! He will stay on his new medicine that helped him at least one year if not longer. He can't stay on the medicine forever as it can permanently damage his vision. We will be seeing a pediatric ophthalmologist every 3 months to closely assess his vision while he is on this medicine.

So, for now, here is his situation. The reality is that they (the seizures) could come back at any time...they could come back as IS, or, he could develop other seizure disorders at some point. Or, he could go on to live a totally seizure free life and never look back. We just won't know for sure until he's about 20 years old...That's going to be a long wait :). But, for today, we know his brain isn't seizing all the time anymore and that the Vigabatrin was our answer. So, we're celebrating that!!

In terms of development, we will be taking him to a professional group that assesses children and determines if any special needs are present. There is no doubt that physically this situation, along with the medications, has taken a toll on him, but again we may not know the extent of any disability, if there is any at all, that may be present for many months until he gets older. For now we'll just watch him close and see how it goes.

The immediate future will likely be a lot of the same as now...he continues to take lots of medication, but will be weaning off of the ACTH for a few weeks..he still has a depressed immune system and will for at least another 1-2 months from the steroids, so he'll still be on lockdown in our room for the most part.
Otherwise we'll be trying to regain some sort of "normal" lifestyle again, whatever that may be :)

Thank you so much everybody for the endless string of prayer, positive thoughts, food deliveries, cards, flowers, other assorted gifts, Cortney and I have been truly humbled by it all, thanks for supporting our little guy so much through all this....The amount of prayers for JP have made the difference :)

Love,
The Seivane Family