Month and a Half Post Fourth Surgery!

Life has been really nice these past couple of months without seizures. PJ is making huge strides cognitively, it's amazing! His memory was so bad prior to this surgery and now he knows what a cat, dog, lion, and pig say. All correctly...not only is that an awesome accomplishment in general for him buuut it's super awesome for a kiddo with global apraxia! His receptive language has sky rocketed as well, he is right in the middle of the normal range for his age....again amazing. His speech is still not there yet but we are working through that. He can make sounds and say a few words but those words have been programmed in to be used all the time. We are hopeful that now knowing he has apraxia and dysarthria we will be able to get him up to speed by approaching him correctly in ways that will help him develope speech. Right now prompt is a huge part of speech therapy.  He is off of his Topomax now, we are very relieved to be down to three seizure meds....THREE never in two years has he been down to only three. Without need for emergency drugs,Very happy parents over here!! We are having a hearing test scheduled here soon to make sure his hearing is good, just in case. Hopefully it's great and that'll be one less hill to climb for PJ. Although there is a concern that he may have some high frequency hearing loss. We will worry about that when and if there is a need too after his hearing test.

His Hemiplegia has caused him to be, what I like to describe, jellyfish like. He is very sloppy in his movements but we are working on getting that a bit better. His leg, arm, and hand are doing as good as they can. His leg is in worse shape then anything else. He can get around but trips a lot without his brace on. He actually likes wearing his brace...I think it is because he knows it helps him quite a bit. He still walks "sloppy" with it on because of the weakness in his knee and his hip. We are just thankful he is moving on his own :).   His arm gets postured sometimes, bends it at his elbow and cradles it some, but he still uses it when he has to....sometimes without being prompted. His hand is working marvelously. I don't see much of an issue with it although we haven't "compared" it to the other too much and haven't "pushed" it to do tedious tasks. His lack of support though is thought to be contributing to his apraxia so we have to keep trying to strengthen him up! There are plans on getting him into a physiatrist to see if medication may help reduce the tone in his shoulder and arm.

We aren't sure what PJ sees at this point. It's obvious he has left sided peripheral loss in his eyes but to what degree is unknown. He has to turn excessively to really see what's going on towards the left causing his posture to get crazy! Most the time, at home, I can't tell there is even vision loss.  Therapists though have found it to be causing him some subtle problems. We will be seeing a developmental pediatric ophthalmologist soon though...hopefully!

Emotionally PJ is becoming a bit of a wreck and very unpredictable. He can be set off so easily and sometimes there seems to be no reason for it at all. He will throw himself down, kick, scream, hit, and is completely unconsolable. He wants me to hold him but doesn't at the same time. It can be pretty chaotic!  We have found that he has SPD (sensory processing disorder) which may be a huge cause to the fits. What is hard is he goes from being an extreme sensory seeker to shutting down and needing very very little stimulation. It varies from day to day. On top of that I'm sure it is extremely frustrating having things to say and not knowing how to say it.

Trick or treating went soooo well for him this year. Finally the kiddo was able to enjoy it and really seemed to finally understand what was going on. I loved seeing how happy he was!! I'm even more happy to see him this thanksgiving and Christmas eating all that good food without limitations from that awful ketogenic diet!! :)

Hopefully there will be no more seizures and no more surgeries for him. He really seems to be making such great improvements!  Now to find time to add some pictures in here!! 

Surgery Four Complete..With Some Minor Tweaking

We have had so much stuff go in every different direction it's been chaos! PJ had his fourth surgery last Thursday. It was delayed due to them squeezing in an MRI to see if they could visibly see the malformation. In the areas that were left there was deeper tissue still left behind that his surgeon wanted to try and see if, hopefully, it would stop the seizures without having to resort to the full hemi. We were very very leery of this as a fifth surgery just made our stomachs turn BUT we do truly trust his group of doctors and agreed it would be worth a shot. The surgery went very well and for once no blood transfusion was needed. He has more deficits now, which we expected and honestly they are not too bad at all in the grand scheme of things. PJ has some issues with his left arm and hand. He has a little tone throughout it and has caused some motor planning issues along with some left side neglect...all of which we should see some improvement on. He has some left side peripheral vision loss and possibly some visual neglect.  He has already began compinsating for the visual changes and doesn't seem to mind too much. His left foot has more tone in it and we are looking at possibly getting a night time splint and a better fitted brace for extra support. Another change is PJ has started the thickened liquids diet...he was having a lot of problems tolerating regular drinks and fought us every time we offered it to him. Well come to find out he needed thinkened liquids, now he is a much happier boy. Oh the drool this boy can put out now with the weakness on his left side being more predominate...it is rediculous and he is hilarious with his sneaky ways of trying to wipe it (usually on my clothes when he conveniently buries his head with a hug). Good news, so far, no seizures....and trust me, I watch for them! More good news, PJ is officially a make a wish kid they accepted him about a month ago and we met his sponsors...who were so very sweet!! Overall I think he is a very blessed kiddo and we are even more blessed to get to say we are the parents of this strong willed little guy! Hopefully more good news posts to come. :-) 

Round Four??

I feel like so much has happened since our previous blog entry that I don't feel like it is possible to even put it all into words. We had an overnight EEG that turned into a two night EEG. The results were not easy to read for his doctor with healing and things going on but PJ is having subclinical seizures as well as his other episodes. It is all right sided and isn't spreading to the left which is really good to know. Unfortunately the area that is showing as dysplastic is an area that will impair both his vision and his motor if removed. We are still weaning off of Topomax currently and maybe keppra but it is too dangerous, at this point, to take his clonazepam away.  He has been on it for nearly two years twice a day and taking it away could trigger seizures not related to his condition just purely from the withdraw. His trileptil is slowly on the rise though in hopes, that eventually, it will be his only medication. We talked to his neurosurgeon last week and he feels that the next step is a hemispherectomy for PJ. Our latest date for surgery is October 3 but he is amazing and said that if he can he will do it sooner. I am going through a lot of conflicting emotions regarding this decision, although I know it needs to be done.  I always have said "I'm not worried, I just want him seizure free." Deep down though I feel horribly sickened that we will have a new set of difficulties for PJ to overcome and suddenly all of this has become very very real to me. I am kind of feeling it is a lose lose situation but when thinking of what I would want I would pick better cognition and communication with no or drastically reduced seizures and impaired mobility over good physical mobility with multiple seizures and very impaired cognition and communication. Making the decision for my child though is nothing short of heartbreaking. Of course I am a worrier so I also can't help but worry about the left hemisphere which has always been perfect on tests but that "what if.." Always creeps up on me and gets me wondering if that other side is as good as the tests have shown. Ugh. My husband is so relaxed and together and keeps reminding me that just because PJ has a good day doesn't mean it's gone, and he is right. We were told numerous times that once this type of dysplasia becomes active it doesn't stop...we have always found this to be true even on good days he was having subclinicals and even more unnerving seizures not even readable through the EEGs. It's just tough and has proved to not get any easier on any of us. All we can hope and pray for is a successful surgery with no complications, that he does become seizure free, and that he has a speedy recovery.

Pushing Forward

After speaking with Paul's doctor we are beginning to wean him off of all but, hopefully, one medication. We have upped his trileptil and significantly lowered his Topomax all the others are staying the same for now. We did find out he is having up to 8 seizures a day. All the extreme fits I have seen of him crying in pain that I thought could be pressure is in fact seizures from the motor/sensory area. They are wanting to do an overnight EEG but want to try to get him on one medication first but if things get really out of control we will go in sooner. Along with his fits from his seizures triggering pain he is having tonic clonic appearing seizures every other day (except for this weekend he had one every day). Not sure what the next step is but at this point the medication has made absolutely zero difference. What is most frustrating is knowing there is something causing this yet we still try to medicate the issue...I know we have to try but we know it isn't the answer as it has never previously worked so why in the world would it now?

We also put in to see if PJ could get a wish through the Make a Wish Foundation. All his life at this point has been seizures, tests, medication, surgeries, therapies, and doctors....I would really love to have him have something done for him that is full of fun and far from all the things he has become accustomed to. He needs some fun times with his siblings!

Spoke Too Soon...

PJ is back to 0 with his awesome record. Had a seizure during nap time. :(

Five Weeks Seizure Free!

We are coming up on a new record!! Post op with his first surgery he went six weeks seizure free so we are almost there. It has been a very nice break for all of us in this household! We did go in a week ago for some concerns regarding hydrocephalus (extra fluid on his brain). They did a haste MRI which is done just as the name says...hastily.  He was swaddled and the MRI took a quick two minutes tops. The results came back with him having extra fluid but not enough to cause concern right now. We go in again here soon for another haste MRI to check on the fluid.  Most importantly what we discovered from that trip from Dr.Oxford (an AMAZING neurosurgery resident) that we were totally informed incorrectly about PJ's EEG. It was, in fact, abnormal but only from the all the empty areas which should be abnormal. He said in the thirty minute read there wasn't anything that seemed seizure related!! Big, HUGE, relief!! So for now, he is good to go! :-D

He also gets his orthotic this coming week to help his left foot. He is a little tight still on that side and is turning his foot in again until it has been worked out...then it straightens out but then the muscles are exhausted and he can't clear his toes from the ground with every step so he is accident prone right now. He is very very smart though and has already learned to compensate for his left "apraxic" side...it really looks to be causing him minimal difficulties, from what we see anyway.

In the speech world PJ has made improvements. He is making tons more sounds. He has "words" but not all are exactly correct words for things.  He can make most single sounds (a,b,d,e,h,I,m,n,o,p,s,t,w, and y) he struggles with a couple of them but can do them. Putting two sounds together is very very tough for him right now. He has a handful of words that are more complex but getting him to say new things is like pulling teeth. We have started using Talk Tablet and it seems to help...he repeats whatever button he pushes (as best as he can) and with every repetition he does sound a bit better.  It has also helped us test his cognition by asking him to find a certain picture...best App we could have gotten (Thanks Mom and Dad!!!).

Overall, we couldn't have asked for a better outcome for PJ with everything he has been through. Next hurdle is keeping an eye on his fluid and seeing how his body responds to it...hoping it will start regulating it.

EEG Spot Check Results

We got the results from PJ's spot check EEG and in the 30 ish minutes of recording he had no seizures that they could see. It unfortunately still showed abnormal on the right side.  It also showed signs of some swelling which doesn't come as such a shock to us with the surgery being done about three weeks ago.  We were told that it had improved...I had to chuckle with that being said as nearly all that side was removed so it better had improved! We have an MRI and an appointment with his neurosurgeon in a month and in the meantime we will watch and wait for seizures since with it reading abnormal we are thinking that it is most likely due to more dysplastic brain---at least until we are told otherwise anyway. I was really hoping to have them begin lowering meds but with his EEG not being clean I am not thinking that they will...who knows what will be said next month though..got to try and be a little optimistic!! :)

Two Week Post Surgery Neurosurgery Check Up

PJ had his two week check up today and his wound looks great, no concerns there. There are some concerns however with his twitches happening throughout the night more frequently, predominately on the left side from facial twitches all the way down to his toes. His right leg is now also showing random bouts of extra tone causing him to trip which is odd.  He is getting an AFO soon, for now as he heals, for his left leg and we were told that it looks as though,now, he may need one on both legs as his usage of both of his ankles seem pretty unpredictable (left more then right). He is being scheduled for an EEG to see if his motor strip is acting up any and perhaps causing some of these issues (twitching and right ankle tone).  He is also complaining of his head hurting towards the back and is unconsolable at times and these episodes stop abruptly.  They are not too concerned about this right now though as it could be a result of the recent surgery and could happen up to a month after surgery.  Even still though if his occipital (visual) lobe was/is acting up an EEG would (hopefully) pick up the abnormal waves. We are hoping his reading comes back clean but also know that if it doesn't its okay because we know there are other options to get PJ to lead a more normal life.  Blah, tests. They are so great for getting answers but such a pain for a two year old.

PJ and his "sissy" holding hands while he sleeps :)

Home 40 Hours After Surgery

The surgeon made his rounds yesterday morning and was so impressed by PJ's accomplishments that he released us home. It is so shocking the small amount of deficit he has physically compared to the large amount of improvements we already notice in other areas....now, like last time, we know to keep a close eye on things since he showed tons of signs last time that he was still seizing.  For having nearly all his right side removed he is just blowing us away!

Physically what we have noticed: He walks with a bit of a lean to the left and has still been consistently dragging his toes on his left foot. We are working hard at getting him to use his left hand but at this point he is doing a great job of forgetting about it being there. When he uses it he is able to pinch at things but it seems considerably weaker then his right hand. But we are only two day out of surgery so we know things could get a little better quickly as he heals.

Positive things we have seen: PJ is not flushing so far and his twitching during his sleep is very minimal. He has already started saying the few words he spoke before surgery and his response time has been very quick when answering "uh huh" (or nodding) or "uh uh" (or shaking his head). He is also saying thank you (more humming it then pronouncing it, but he does it consistently when somebody does something for him or we tell him to say thank you).  He has started more "baby babbles" which is a good sign of him trying to communicate more. He is also back to using two words together. We are hoping since he is doing this so soon after surgery that soon or at least eventually he might start spitting out some more words!

It is so amazing the amount of trauma a child can go through and still pull through without skipping a beat.

 This kid is our miracle.

The Day After

PJ is doing remarkably well. His surgeon came by to check on him and told us that by looking at the post op scans it doesn't look like PJ should be using his arm and leg yet he was moving around all four limbs.  Today Paul took things even further and WALKED! He is dragging his toes pretty bad but is still walking unassisted. His grip has improved a bit but is still weak with his left hand. His surgeon is not worried about the seizure PJ had since the motor strip left had quite a bit of trauma during the surgery, he seemed to kind of expect it. This does not mean we stop being on our toes....we know now that only time will tell us if this surgery did the trick or not. On another great note, he hasn't thrown up at all, which makes us happy and he is eating and drinking well. Now we wait for him to poo. We are so amazed at how well he is doing :)

Surgery Three Completed

PJ came out of surgery today like a trooper! He heard my voice and said "momma up" reaching towards my voice (his eyes were already swollen shut). They wrapped his head this time around which is kinda nice also. Sadly PJ had a seizure about two-three hours after surgery. It showed all the signs of PJ's old seizures minus the staring spell before it. The areas he has remaining on that side is his motor area and his occipital lobe.  He did get as close as he could though to his motor as he could without totally wiping him out. Unfortunately we knew that there was a decently high possibility of  more surgeries but will wait in high hopes of seeing nothing more---but also totally accepting the possibility of needing another.  we were told during this surgery once they got through to the insula(r) lobe that it appeared to be the most obviously malformed. It was all scar tissue from "burning itself out"-- it was also constantly firing off deep in there.  They also let us know finding dysplasia there like they did was very rare. Good news is PJ didn't stroke from what they could see on his scan after surgery but the vessels supplying blood to the motor area were, from how I understood it, pulled away from abnormal tissue so we are hoping they don't start "spazzing out" on him.  The deficit on his left side currently is more pronounced but we anticipate it getting a little better before we see his new baseline since healing will take a bit of time. Will be updating as PJ comes around more, thank you to all our friends and family for the support and prayers!

Round Three...

Seizures have increased to up to 20 visible episodes a day. Most of them occurring at night and during nap time....Leaving PJ, daddy, and I to be horribly sleep deprived. We have been giving midazolam almost daily and sometime twice a day with little relief. They upped his Topamax drastically to try and slow things down which has worked a little allowing us to give him a break from his midazolam.  Paul's neurosurgeon wanted to see him ASAP last week to discuss another surgery so we met up the day after I called letting them know the severity of things (so thankful they moved so quickly). He let us know upfront that this may take four or five surgeries (depending on whether or not seizures return again) to get the dysplasia as it "acts up" because  he is really wanting to try and spare as much of that right side as he can. The only lobe that will be left alone this time is his occipital lobe (vision) and maybe a part of the periatal lobe depending on whether or not it reads clean once they are in there.  The temporal, part of the periatal, and an area really deep in the brain called the insula lobe has to be remove. The insula lobe puts him in a very high risk for stroke which will essentially cause the same damage to Paul's physical abilities as a full hemispherectomy would cause but there is still that chance that he won't stroke and he will have fewer issues physically and that is what his surgeon is aiming for. We aren't afraid of any of this though because we know he is in very good hands and these seizures must be stopped so he can cognitively and verbally progress. Every day is one day closer to helping PJ. Our other two blessings are taking everything so well. They have sacrificed so very much during all this...makes me happy that they are all so young I think they don't know any different right now and don't have a clue that this isn't normal.

PJ's third surgery is scheduled for this Friday the 5th. Praying hard that my baby comes out of surgery as well as he has in the past. My only fear, with either living with seizures or surgery, is losing my baby. I hope and pray this gives my little PJ some relief from all these seizures.

And We Start Again..

 

It has been a while since I have updated...We had an overnight EEG last week that turned into a two night EEG. Sadly PJ is still seizing from the right side. He had multiple seizures the first night we were recording with one physical episode. They asked us to stay to try and get more information and information they received...PJ had his worst night, with seizures mimicking the ones he use to have before his first surgery.  It was long and stimulation set him into a hard physical seizure. Needless to say, I was a petrified mess. We were told that his seizures are remaining on the right side and, at this point, not spreading to the left. His flushing episodes are seizures and his extreme fits could be seizures or repercussions of a seizures. Right now the big bulk of his seizures are happening at bed time and nap time but abnormal waves remain steady from everywhere remaining except the occipital lobe (or at least from what the EEG reads anyway). Right now there is a possibility that he is seizing more deeply in the remaining right side making it also undetectable on regular EEG like before.  Right now we are at a place where a hemispherectomy is Paul's best shot at living a normal seizure free life....We are very aware that chipping away at that side doesn't seem to be doing the trick and we don't want to put him through more surgeries then necessary and our gut is saying this is PJ's cure. God has a big plan for this sweet little boy. He has taught us so much already and I pray that PJ and all of us can look back at these couple years and smile knowing how far we have come. We should be hearing back from the hospital this week for further appointments and are really hoping that things roll as quickly as they did last time. We are also, possibly, going to be taking away some more medications sometime soon...but we will see how that all turns out.  It just breaks our hearts seeing PJ suffer.

Neurosurgery Two Week Check Up

Yesterday was a flood gate of emotions....pretty common for this house hold!  We met with neurosurgery and they were so very impressed with PJ and how well he is doing developmentally...he is really thriving right now which I can't get enough of seeing.  I actually am feeling a little bad because my other two young ones are lacking a little of my attention during the day.  Still really trying to work out some sort of balance.

They also informed us that pathology came back as cortical dysplasia  ll b, which was a relief because we were told that it was likely more then just one type in there.

  I did bring up my concern with his face starting to flush again and him twitching again (the twitching has slowed down a lot but is still happening) during nap and bed time like he did before this surgery.  I brought up that I knew the brain was healing so I was hoping it may have something to do with it.  I was quickly told that those symptoms weren't healing related but were unfortunately signs of seizures.  Deep down I knew that the flushing wasn't good. I even watched him do it a couple of times right after surgery....use to though, most of the time, (before the surgery) this flushing and twitching would lead to a full on tonic clonic episode....so I thought in the hospital he was going fall into a big episode but once he didn't and the redness faded I had hope it was nothing...I was in a bit of denial with it all..I think, even though I didn't admit it openly, I had loads of hope that this would be it even though I repeatedly said "I'm not very hopeful".

Now we are waiting for him to heal some to see about a third surgery. They told us they typically wait a bit before they go in again--not things we wanted to have brought up after we all successfully survived this second round. This dysplasia will only get worse again though if it isn't all removed, so we got to do what we have to, to give this little guy the best life we can. At this point it is being seizure free and try and reduce the ample amounts of zombifying seizure medications he is on. As of right now we begin waiting again---waiting for harsher seizures, waiting for doctors phone calls, waiting for appointments, and waiting to have our little boy be healthy, happy, and rid of cortical dysplasia. He will get through all this!

A Turn for the Best! :)

On the 22nd Paul woke up and surprised everyone. He decided, on his own, that he was ready to walk, nearly run, play, and begin communicating. He even said two words together consecutively! Both totally clear! Amazing! We did move to the rehab unit to ensure that things were going as well as they seemed and he blew them away.

He does have problems with his left arm and remembering it is still there and will need an orthotic for his left ankle just for outings where he will be doing lots of walking to keep him from tripping.  Really though things were not expected to be this well.  Unfortunately, PJ did have a "possible" seizure at night in the hospital.  His eyes rolled back a few times and he began lip smacking then his jaw went tight, he took a deep breath, then went back to sleep.  We were told not to worry to much as it could be the brain healing and not anything related to his disorder....can't lie though, I am worried...petrified actually, but we will see.  I have a feeling though that if he has an abnormal EEG again that they will end up graphing him to make sure he is not constantly seizing again deeper in his brain....maybe not, actually I'll word it better HOPEFULLY not...I would prefer his EEG be nice and normal and us not even need to wait to find out what they would do!   They released us from the hospital Friday afternoon and we were able to bring all our babies home.  I snapped a picture before my oldest had his swim class...which he wasn't very happy about haha!

 

Since being home I have noticed him still flushing a deep red color during his sleep.  I am not too sure what is causing this and will be asking the docs to see what they think....I can't help but fear that the seizures are still at it in there.  He has, however, had a HUGE reduction in his twitching in his sleep and has had a big boost in his balance overall.  Even with his bad leg he seems to be way more coordinated now then before.  His cognition still waivers a bit.  In fact, he doesn't understand if we say more then three or four words to him.  It has to be straight to the point or he just goes "uhhhhhhhh" and then walks off...which could be a result of many things...he isn't at a very cooperative age as it is! Needless to say we will be giving PJ lots of TLC to try and see how we can try and get him to a comfortable level developmentally.