Manic Monday :)

Yesterday we headed out to have P.J's FMRI (functional MRI) done. How I understood it, Basically, it allows them to see what areas are working properly by showing the blood flow and oxygen use areas of the brain are using to perform some basic tasks. P.J was sedated during the test and the actual FMRI lasted about an hour. This did give my husband and I the chance to have a coffee date in the hospital, which was nice...sadly when we have time like that we usually end up reflecting on everything going on---sometimes that leads no where good. It's hard to think of anything but what is going on when your sitting in a children's hospital surrounded by children who are dealing with more then I ever had in my lifetime and in most cases more then I ever will experience...it's heartbreaking.

Pauly came out of sedation very well, thankfully. The only hard part this time around was that now he recognizes the MRI bed....poor kid looked at me screamed and repeated "uh-uh". He use to take things so well but the older he gets the less compliant he is becoming---totally understandable though, I know I would be the same way. We are just happy that this should be the last of the tests before they go "all in".

Our next appointment is next week with his neurosurgeon and also,next week, for his bayley test through the hospital to get a better idea of where he is developmentally. Until then though, we are going to enjoy this beautiful weather up here in the northwest. A little sun shine and fresh air goes a long way!! :)

"Hanging in There."

We received appointment dates for his FMRI and for our meeting with his neurosurgeon all of which will happen next week and the week after that.  In the meantime we are just counting down the days till his appointment dates, and trying to get by with as little extra hospital trips as possible.

Sweet little P.J is making great strides with his communication (most of the time things he babbles aren't clear at all but we know exactly what he means most the time...makes caring for him much easier) he is also trying so very hard to jump...so cute!  The Little Gym has helped him so much physically (and in many other ways) every week I see this little boy learning something new from them! Funny, his new word is "nany" for candy (of course, haha!)...if we happen to say "No" he does a wonderful thing...throws a fit like a typical two year old! :)

Recent, not so great, developments......
He is now having up to nine hard seizures a day and if what they say about his subclinicals are true, it means he has had at least eighteen all together (on his worse day). They are beginning to last longer as well, around four minutes being the longest so far...you can see him struggle to come out but just can't kick it.  His clusters, strangely, seem to have taken a break though...but I can't say that I mind, I really hate days where he has them. His break days have also disappeared...yet another sign that things are heading in a bad direction. He has begun staring off again as well, which makes me a bit sad. I had been trying to ignore it but I brought it up to my husband and he had noticed it too. He doesn't blink, his eyes dilate, he just stares blankly, unresponsive for a minute or so during these spells....we have not seen him do this since his first surgery but he commonly did this before it. Another new development, he has now begun getting very flushed randomly throughout the day....I can't help but wonder what in the world is going on now.  The redness looks almost exactly the same as it did while he was on the ketogenic diet when he got too ketotic. Maybe somebody will have some insight with this? It isn't that he is over dressed and he isn't sweating so I'm thinking maybe it has something to do with his seizures?

I worry so much for him....it just breaks my heart. We are hanging onto the hope, and trying to keep our faith strong, that things will get better soon.

So Here is the Game Plan....

Seizures here lately have been relentless...Grandma even got to experience one of his "Good Morning" seizures before we left for his appointment today.  At this point we were unsure of how much the people at the hospital believed us so we have been recording every seizure with our phones just in case.  Thankfully, we didn't need them as his doctor totally believed everything that was going on.  He told us that he did get some information from the PET scan and his seizure that he had before the scan itself was at the absolute perfect time.  He had just had the radioactive sugar injection about 10 minutes prior to his seizure and any activity in that 45-60 minutes after the injection can be tracked.  They found that the seizure that he had was coming further back on the right side of his brain...  The only bummer is that it is most likely going to impact his motor skills.  To us, right now it is no big deal, he CAN NOT live with these seizures....they are detrimental to his development.  They are causing the speech and cognitive delays and at this point, we feel, he can deal with the motor delay way easier then the speech and cognitive delays.  He wants so badly to communicate with others and play with others but at this point he just can't and you can see the frustration in his sweet little face.  It was so heartbreaking today, he walked up to a woman sitting in on our appointment and stood there staring at her...you could see that he wanted so bad to talk to her. He literally stood there for ten minutes just looking at her right in the face just like somebody would be doing if they were holding a conversation...made my heart ache for him!

 

 

Well onto the doctors plan of action. P.J is being scheduled for an FMRI and after that he will have an appointment with the Neurosurgeon and then will have the Grid placement done which will lead directly to the surgery.  Grid placement is similar to an EEG but they will place them directly on the brain (brain surgery part 1) he had this done the first time around but it was done on the operating table so he didn't have to sit around with them on...this time he will be monitored in the hospital for a while.  This will allow them to see exactly where the problems are and what is "good" brain and what is "bad" brain. It will also tell them what exactly they will be impacting if the area of brain is removed. The doctor said that with as often as he is seizing it should only take a couple of days as opposed to the week or sometimes more it can take (so in this case more seizures are better, yay).  After they get the information he will go straight back and have the dysplasia removed for a second time in hopes of FINALLY being able to know the real Pauly....not the highly medicated, seizing, little boy.  The guessed time frame for all this is two and half or three months till we will be on our way to recovery....I can't help but hope it happens a bit sooner...totally ready to move forward with our lives as a family....without the dysplasia!  So ready or not here we go with our fingers crossed and praying hard....onto a second brain resection!

Headed Down a Familiar Road?

This week has been nothing short of a nightmare. PJ had his PET scan on Tuesday which was a step that was needed to try and pin point where all his dysplasia is at. The scan itself took 30 minutes but for a child it is a full day of appointments.  We got in the hospital at 7:30 in the morning and waited around until he was called back to get hooked up the EEG.  PJ did so well hanging around the hospital...he was eating up all the one on one time he was getting from Mom and Dad!

 

 

Once he had the leads on we had to wait around for his IV hook up and his Injection of Sugar (slightly radioactive) fluid for a 45 minute "quiet time" to let the fluid get to his brain then wait for the team to come in for sedation.  Really most of the day was just a whole mess of waiting on different people. Very exhausting for all of us...expecially when their play room is for children three and up.  Big bummer for Pauly! 

On a happy note PJ did, however, perform greatly with the leads on and recording.  He had one of his hardest physical seizures to date and struggled to come out of it.  I was so glad it was recorded and I am hoping it leads the doctors to more answers! Once PJ got out from his PET scan at around 1:45 we watched him sleep off the meds for 40 minutes and then FINALLY let the boy eat some food...Poor thing was starved! He didn't stop shoving food in his little mouth till we got home around five.  By far the worst part of the scan is having to keep food and drink from your child...even more so when they absolutely have no idea why it is necessary.

 

 

 

So onto a quick update of Pauly overall.  Things are starting to really worry me with him here recently.  He is now starting to have these clusters of seizures very similar to how they started out last year before he started going into status and eventually was put into a coma to save him.  Actually I don't even think he stops seizing during these clusters but is seizing inside and not physically.  You can see in his eyes that he is not with us...not the look he gets postictal but the look of being waaayy off.  We ended up giving him a dose of versed on Wednesday during on of his clusters and went to the ER and were given the okay to leave after 45 min of being there only for him to have another seizure in the car. He began jerking hard in his sleep once we got home and at that point we drove to his hospital in Seattle where his specialists are which after checking him over and observing him allowed us to go home and wait for more.  I am petrified that we are headed down the same road we walked over a year ago.  Things are slowly getting worse and the intensity of his seizures are getting to be more and more.  We also found out that the PET itself didn't show anything but they said with these PET scans the EEG readings are the most important (?) and those results would be there next week on our appointment day with the epileptologist.  He is going on the board on Monday as well to see what the other doctors think should be done.



 

One Year Post Op and his Worst Day Since Surgery

Today marks the anniversary of PJ's life saving surgery!!! We thank God daily for blessing us with this amazing little boy!!! We also are thankful to the Mayo hospital for doing all they could to find answers and for taking such remarkable care of him through his long stay there. He is our miracle child. :).

Yesterday, however, was our worst day since surgery. PJ went through a five and a half hour period of cluster seizures. He got some breaks but just couldn't completely pull himself together. Finally the hospital said if we see one more would need to administer his emergency meds and call 911. We were pretty upset at the thought of being hospitalized and giving him medicine that would take two days to ware off (we despise the emergency drugs it makes it impossible to see if he is no longer seizing...but know they are also necessary to help his muscles from being damaged).  Thankfully though he snapped out of it and like magic was back to normal. It drives us crazy...there is no trigger, no method to the mayhem, no reasons what so ever for his episodes. That's what makes things tough. Even tougher and more scary to accept is that no medicine can stop his seizures it just truly does what it wants for however long it wants. Soon I hope he will be free of these seizures...five more days till we find out if it can be done!!
                                                                                                          

Neuropsychology Appointment

Well today was PJ's appointment with a neuropsychologist.  They like to get a child's developmental skill level before a surgery and compare after.  It went really well, it was just sad to see his weak spots. Hopefully though once we stop these seizures he will be able to thrive like any other child.  Next week is his PET scan and I am worried.  I want them to be able to see it and I don't want it to be a large area....preferably teeny tiny and most importantly operable.  I know we probably won't get all that we want though but we can still hope! Paul has had such a slow month with seizures though...which is odd... until yesterday. He had a couple (possibly more between them) within three hours so he was done for that whole morning.  We love this little boy to pieces, it hurts to see him go through all this! Hoping to get things fixed soon....we would be on cloud nine if we never saw our son have a seizure again..

Breaking Records!

Right now I'm going through this naive stage that maybe the dysplasia just disappeared..or perhaps his brain connections have weaved their way around it completely and he will be fine.  He is nearing his record of no visible seizures. Well I say nearing....but it is as close as he has gotten to it. Seven days! So proud! There has been so much focus and developmental improvements this week that I just want to think he is "normal" now! I do know...deep down....that this IS the calm before the storm for him.  What is scary is the longer he goes without a physical one the worse they will be when his bad week gets here.  I thoroughly enjoy seizure free times but when it gets to be this long I have to snap out of my dream world and be on my toes.  We never go anywhere without his midazolam and try not to let his disorder pull the rug out from under us. It must be so frustrating for him...he takes three steps forward and two steps back during good times like this. He learns so much just to have to start over again after his seizures get him.

**Update**

Well...maybe I jinxed things! P.J had a seizure while his daddy was rocking him to sleep.  He went a good while without a hard "physical" one. Now to conquer this bad week or so and hope for a good break again!

Most Recent MRI

Picture of Paul before the MRI :)

 

So, this would be P.J's eighth MRI in the Past year.  Unfortunately, even with the best MRI it is near impossible to tell what is damaged brain and what isn't...but we will see what this MRI says, we should know in a few days.  This is how we have understood it; Since his brain is still developing at his age it is really hard to see the dysplasia--as he ages it will become more and more visible---Problem is the longer we wait to stop the problem areas the more damage could be done and the ability to recover from a brain surgery is more difficult.  Plus at this point his brain is a ticking time bomb....we don't know how bad this could truly get or when it could happen.  Anyway, we are waiting on his PET scan here in a couple of weeks to get a better idea of what is going on inside the cute little head of his :) 

 

After the MRI...not the happiest coming out of sedation!

First "Real" Word!!

So P.J has made me come so close to tears today!! His speech therapist came by the house today for his weekly visit (she is so awesome, and P.J absolutely loves her) and P.J did something unexpected...and AMAZING!

Speech has been a constant struggle for him and he really just babbles a lot.  He does say Mom, Momma, Up, Uh-oh, Uh-Uh (for no), and Yeah.   Recently, this past week, he has started saying Hi, Bye-Bye, and most recently Bubbles and Mine.  The problem with P.J is his words are not super clear and most times he doesn't quite get the words out right.  Like he will say Ba for Bubbles or just resort back to babbling. 

At this point he refuses sign language so that, at the moment, is out of the question for him although we still try to incorporate it into what we are telling him sometimes.

Anyway, onto the great news.....P.J said "Barbie"!!!! Not just babbled it or said it broken up---he straight out said "Barbie".  :)   It wasn't totally perfect but he did it and I about passed out from shock! He covered up his sisters barbie picture and I said "Bye bye" and he uncovered her and I said "Boo Barbie" and he recovered her and uncovered her again and said "Barbie"!! Hopefully he will hold onto this word for a while, we will see when he starts having more of the terrible "S's" whether or not he was able to keep it!  We are so proud of our little man! Fingers crossed the word sticks! :)

More and More Tests!

P.J's two night stay at the Children's Hospital.  Of course big Brother and Sister were there for support for a bit Arietta said "Oh, Pauly are you okay???! Were you brave!!??"   :)   I will say though--this little boy was thrilled to have a bag of snacks.  Obviously he couldn't keep his hands out of the bag! 

We thought for sure there would be a seizure since we usually see at least one a day.  This boy just wouldn't do it.  He made a liar out of us for quite a while!

Daddy visited the hospital on the second day and I literally RAN to Starbucks and loaded on caffeine...something about hospitals I just HAVE to have my caffeine!! On the last morning there P.J decided to have a seizure and I was thrilled (only because I knew we were getting the information that we needed to help him).  I wasn't thrilled about the timing...we were sleeping and I heard him grunting and had to hurdle off the hospital bed...I'm sure the EEG tech had a good laugh at the video!We were warned that we may need to stay to at least record three seizures but the doctor came in and told us he got everything he needed from the one he had.  His right side (not quite sure where yet or how big the area is) is seizing sub clinically and when we see the physical seizures it is the seizure spreading to the left side of his brain...which is not a good thing.  We have an MRI scheduled and will be going in for a PET scan in a couple of weeks also.  We have to save the functional spots of our sons brain and since the medication isn't working our other option is another brain resection if operable.  We are hoping that it is a tiny little spot and totally easy to get to and also that if it is operable it will be the last time he has to go under the knife.  Good news though---P.J's medication has been lowered.  He is still on the Topamax, Trileptil, Keppra, and Clonezapam BUT the Dilantin is gone and instead of three times a day he takes his medicine two times a day now! Soooo much better!