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

Little JP update, Sunday March 18th

Hi all, it's been an eventful week for little JP.

With his clusters of seizures not stopping entirely as of last Monday night, we began our new medication called Vigabatrin. This medication is being given concurrently with the ACTH that he's getting from us via injection once a day. They will overlap for a few weeks while we wean him off the ACTH but will stay on the Vigabatrin for an extended period should it work.

After starting the Vigabatrin on Monday night, he had last cluster of seizures on Tuesday afternoon. We haven't seen a "classic" cluster or singular seizure since then, so nearly 5 or 6 days now. On Tuesday, Wednesday, and Thursday JP seemed very groggy but relaxed and easygoing and easily the most calm e can remember him during this entire process. We were so excited that it appeared we had found our drug that would work.

However, Friday evening, he began to start having a very distinct pattern of grunting and flexing at his midsection that seemed very odd and not normal. It initially began as one or two, but then now is being done a lot of the time he's awake. These motions that he is doing are different than the previous seizures he had been having. Because they are so different, we are not convinced that they are seizures...we know due to the meds he's on he's very irritable and could have stomach pains.

So, those activities are still going and we have no idea what they are. They are either a) Still IS but the spasms are different in nature, b) a new onset of some other kind of seizure disorder, or c) an upset stomach or unknown pain causing them and not seizure related. We are obviously praying for C.

On Wednesday coming up, he will have an EEG done of the brain again. There is a very specific brain wave associated with IS, called hypsarrhythmia. We will know from the EEG if the hypsarrhythmia is gone, and whether any other kind of seizure disorder is present. For Jp to be considered "free" of IS, that hypsarrhythmia must be gone. Thus, Wednesday, is an important day in terms of determining where we are.

So, the good news is that his clusters are gone with the new medication...the uncertain news is we don't know what this new behavior is that he's doing. We'll know a lot more by Wednesday, and I will try to post here that night.

Thank you again everybody for the prayers, the words of encouragement, the meals, etc. We sincerely appreciate it each and every day.

Love,
The Seivane Clan

Jp Update #2-Sunday, March 11th

Hi family and friends..I wish had I better news for you all by now, but I simply don't. After upping his medication last Monday to max dose, he remains having at least 2 or 3 clusters of 15-20 seizures each a day. We had a small stretch over Friday/Saturday where he went nearly 27 hours without a cluster and we began having thoughts that perhaps we had turned a corner, but that was followed by several clusters within the next 12 hours.

Now we'll be turning our hopes tomorrow, Monday the 12th, to a new medication called Vigabatrin. When coupled with the ACTH (he'll continue that medication for several more weeks) this combination has proven relatively effective with other children with IS. This medication is also our last true hope of controlling the seizures effectively.

Cortney's mom has been here helping since we came home from the hospital, and continues to be so helpful to us all, in particular to the three girls when Cortney and I are tending to little dude. My fire department has continued to bring us dinners each and every night and that has been truly wonderful. If it weren't such fantastic support from family/friends/fire dept., Cortney and I may have already lost our minds....The emotional roller coaster of waiting for his clusters and then the heartbreak that accompanies each one of them has been very draining...the support from everybody has certainly helped Cortney and I keep our chins up a bit and keep trudging ahead...

Take care everybody..will post again later in the week after JP has taken his new medication for a few days...

JP and Cortney

JP Update #1 - Thursday, March 8th

A quick update on little JP. His dose of his hormone drug ACTH was raised on Monday as one final attempt to quell the seizures before we have to consider additional medications. So far it has helped a little bit as we've had a few longer spells with no seizure activity, but they are still there. He's still averaging around 2 clusters a day with a few little intermittent single spasms. If they are not entirely gone by Monday the 12th, we'll begin mixing in Vigabatrin, which is the only other proven medication for Infantile Spasms besides ACTH,  however it can cause permanent blindness if taken too long.

We are obviously hoping to avoid that medication and keeping our fingers crossed that the ACTH takes hold 100% over the next several days. We're still hoping it'll work and so are the doctors!!

Things in the house are as you can expect. Busy, waves of different emotions but overall doing okay, lots of doctor visits this week and a home health care nurse comes twice a week to check him to ensure the possible side effects (hypertension, bleeding in stomach, or spilling of sugar into the urine) of ACTH aren't hurting him. So far he's been clear of all that so we're hoping his upped dose doesn't change that as well.

On a plus side, little JP has been his little self quite a bit recently, several times a day he's cooing and babbling at us grabbing our face and such, and loves to be talked to and tickled just like before all this started. It's wonderful to see regular little JP sometimes as it gives us all hope things may stay that way at some point.

That's it for now...thanks again for all the wonderful support...
The Seivane Clan

Little Jonpaul's Infantile Spasms

Hi family and friends! So we are digging out this old blog that hasn't been updated in several years to use as a place for family and friends who are curious about little JP and his situation.

First, Cortney and I wish to thank everybody so much for the continued prayers and encouragement, we know they are helping and hope they continue on as he's still fighting his seizures on a daily basis...Thank you for the phone calls, the deliveries of food and general words of encouragement and love. We have told little JP how many people out there are praying for his little body to get better...

So, Cortney and I noticed a few weeks ago that JP was having sudden jerking motions that initially looked like normal baby startling, but within a few days began to worsen. The morning after we noticed they were worsening we went to the UCD ER, where the pediatric neurologist immediately recognized the movements as infantile spasms, which was confirmed by an EEG (brain wave reading). Initially upon hearing that we thought, "okay, some infant seizures, no problem, he'll probably grow out of it or it's something easy to fix up". We thought that until we googled it in our phones, and then the realization that our lives may have just changed significantly hit us.

Infantile Spasms is a severe form of Epilepsy that doesn't respond well to most typical seizure medications and is very difficult to control. It involves several "clusters" of small seizures (6 to 8 clusters a day), usually with each cluster having approximately 10-20 seizures. If not treated quickly and effectively, it will worsen causing major physical and mental developmental problems (and will serve as a gateway to many other potential neurological disorders. You can google infantile spasms to see how potentially bad this can become if we can't get control the seizures...) This is an extremely rare condition found in 1 of 4,000 kids. Many of those cases are kids who have other underlying brain damage or syndromes. However, JP doesn't fit that category.

So far JP's cause for this is idiopathic, or without a known cause. Meaning, every single test known to man has been run on this little guy, including a brain MRI, which ALL have come back normal. 30% of Infantile Spasms cases are idiopathic like JP.

So what are we doing now? There are two major medications that have been known to effectively stop the spasms permanently. The front-line medication here in the USA is ACTH, which is a growth hormone with a slew of potential side effects, and it was overnighted to us from Florida for immediate treatment. Cortney and I give him one shot in his thigh every afternoon of this hormone. Upon receiving this medication, his clusters slowed down and we were hopeful they would stop...but he hasn't been that lucky...while they've slowed down, after 10 shots he's still having several clusters of seizures a day and unfortunately each cluster he's having more and more spasms now, upwards of 20 seizures or more. For this medication to "work", it must eradicate the seizures entirely. He only receives the medication for 1 month total so they must be gone before then and STAY gone after he weans off the medicine. Because this medication often works quickly (and hasn't for little JP), our neurologist today upped his dose again in hopes of helping the cause .

If this medication dosage bump doesn't show some improvement by the middle of this week, we will be adding another medication to the fight. It's called Vigabatrin, which is used front-line in most other countries in the world however it has one major side effect...it potentially causes permanent peripheral blindness. So, Cortney and I are now having to decide whether to let him seize uncontrolled causing severe developmental problems, or, try to stop them with this Vigabatrin possibly blinding our son in an attempt to stop the seizures. Like I said, our world has suddenly changed quite a bit.

We will try to post here at least every few days so that all of you can follow his progress if you so desire. I know many of you text me and ask and I can't always reply as things are quite hectic around here as you can imagine. The emotional toll is heavy on Cortney and I and we are trying to just go day by day and keeping trudging along. The girls are doing well with it so far, Cortney and I are trying our best to keep things as normal as possible with them as well.

Little JP will have a severely depressed immune system for awhile from his ACTH, and we are basically quarantining him for now in the master bedroom to keep him safe from as many germs as possible so obviously the Seivane house is on lockdown for the most part. Lots of hand sanitizer and hand washing :)

We have Cortney's mom Pam living with us on/off for now helping out, and she has been absolutely wonderful in helping us out while we navigate this. The fire department has been bringing us meals every single day for over a week, and that has been so unbelievably helpful as well.

One thing that all of the research has shown clearly with infantile spasms is that quick diagnosis and quick treatment greatly enhances the chances for a "normal" prognosis so-to-speak. Thus, getting his seizures under control ASAP is paramount to his development. This is a very big week coming up for little JP. We'll keep you all updated as often as we can.

Again, thanks for all the love and encouragement!!
Jonpaul, Cortney & Little Jonpaul

Surprise Disneyland Trip

April 2009

I unfortunately had checked the mail and saw a envelope from Disneyland- I asked Jonpaul about it... turns out it was supposed to be a surprise trip for us to Disneyland at the end of April. Disneyland was NOT supposed to send the information to our house- somehow it was overlooked and well, I innocently found out. Jonpaul was SO bummed out- and I was too- I felt bad for finding it and that now I was not surprised and ruined it for Jonpaul. Well the girls and I were about to head out the door one morning in the beginning of April to visit with daddy at work. I called his cell and I told him I was probably out the door in about 40 minutes or so. Then all of a sudden I heard the alarm for the house sound when you open the door- I was pretty freaked out!!! The next thing I knew it was Jonpual home from work! What??? I had just talked to him about when we would be there to see him at work! Well sure enough it was a big surprise! He had been talking to me from down the street and not from work- very sneaky!!! He appeared in the hallway upstairs with Mickey Mouse ears on and big Disney balloons for the girls. Not only were we so excited about his revamped surprise- I was also very relieved it was he that opened the door and sounded the alarm! He changed the dates of our trip from the end of April to us leaving the very next day! So we had some packing to do! We had a blast! Skylar and Stella loved it.

Stella kept trying to put bacon on Minnie's nose!

Jonpaul with his girls.

This might be as fun as Disneyland!

We had just left the hotel for home...

Two seconds later!