Epilepsy Families: Noah's Journey (pt. 2)

Welcome back!! Here is part TWO of Noah's journey, as told by his incredible mother, Mallory. If you missed it, you can read part one HERE.

Back to you, Mallory!

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September 2013 Noah had a neurologist follow-up and it was at this time his neurologist wanted to get down to the nitty gritty and figure out why Noah was having seizures.  Noah’s neurologist was going to schedule him for an up-to 7 day VEEG (Video EEG) as an inpatient.  While in the hospital they were ordering a high-resolution MRI, and a SPECT Test (with and without dye).

November 2013 Noah was admitted in the hospital for his VEEG, MRI, and SPECT Test.  We only had to stay for 2.5 days because Noah had enough seizures while at the hospital that they were able to get all the testing done as well as obtain enough information that they were comfortable in sending us home. 

This was the results of his testing:

Study recorded 13 electrical clinical seizures (5 night one, 1 at nap, 7 night two) that all began with a tonic seizure followed by a cluster epileptic spasms.

"The EEG onset appeared to have arised from over the right posterior hemisphere near an area that appears abnormal on the MRI scan representing a possible focal cortical dysplasia.  These results and potential for surgical intervention will be reviewed at an upcoming epilepsy conference after which time the parents will be called with a recommendation."

After receiving these test results our neurologist from Texas Children’s Hospital informed us he was leaving to go to a hospital closer to his home.  We, thankfully, got added to the head neurologist at Texas Children’s Hospital.  We hadn't actually met him, but we had an upcoming appointment that we were looking forward to.

By December 2013, Noah was having a terrible time sleeping because he was having so many seizures at night.  Noah’s neurologist then added a second medication Clonazepam for him to take with his other medication, Trilepital.  The Clonazepam helped Noah sleep better, but he was still restless.

In April 2014 we met with the head neurologist, Noah’s new neurologist, at Texas Children’s Hospital.  This is what we found out:  We finally found out WHAT is causing the seizures?  Noah's seizures are happening due to Focal Cortical Dysplasia in the right posterior (back) part of his brain.  Basically meaning he has one spot (focal) where the tissue (cortical) of the brain is abnormal/not developed correctly (dysplasia).  The doctor told me this was nothing I did during pregnancy or anything Noah did since being born; his brain just didn't completely developed properly in womb.  The exact type of seizures Noah is having is called "complex partial seizures."

Another answer to a question we have was, will he grow out of the seizures/epilepsy? Here's the answer we got.  Most kids who grow out of their seizure disorder will have normal MRI's and have their seizures easily controlled by medication.  Clearly this is not the case for Noah.  His neurologist told us that unfortunately, Noah will not grow out of the seizures.  He will live with them.

We were then informed of the four ways to control epilepsy.

There are four ways to control epilepsy; with specifics on my son’s particular case:
1) Medicine

2) Surgically remove the area where the seizures occur (as long as they are in one location; if the seizures are in multiple locations, surgery really isn't the best option)

3) Special diet called Ketogenic Diet (not recommend by our doctor because it's a hard diet to follow and he tends to only do this for those with NG-feeding tubes; plus once you hit teenage age/adulthood it’s not recommended)

4) VNS (Vagus Nerve Stimulation) which is a pace maker that sends electrical shocks to the area of the brain where the seizures are occurring.

Our doctor flat out told us that Noah could have surgery now, but he likes to have two failed attempts where medication does not control the seizures.  Although Noah has been on four different medications since 3 months old, our doctor is only counting the main one he is on now, Trilepital, because the first two weren't for exactly what Noah has, they were more for the spasms; and the Clonazepam medication is not used as a primary medication to control seizures, it’s typically a secondary medicine.  So only with this information, Noah has only had one failed attempt to control the seizures.  With that said, Noah is getting another medicine to start this week, Onfi.  If the Onfi fails, meaning Noah cannot be seizure free (not even one) for 3 consecutive months than that will count as another failed medication attempt.  At that time we will then take on the surgery journey to remove the portion of Noah's brain causing seizures.

I asked his neurologist that if Noah proves the medication route doesn't work, then how long it would take to get him in for surgery.  The neurologist said 3-6 months.

 

We left that appointment with Noah’s third medication, Onfi (added to his Trilepital and Clonazepam).

By mid-April Noah had already significantly failed the new medication and the process of a surgery date had been started.  We met with a neurosurgeon where we all agreed that a Subdural Grid placement followed by a resection was our best option for Noah’s surgery.  With this route they gird is like an EEG, but on the surface of the brain.  This gives them a more precise location of where the seizures are coming from; the other option was to just go in and remove the focal cortical dysplasia part.  We didn’t feel that was completely necessary, so we went with the grids.

July 8, 2014 Noah went in for part 1, subdural grid placement, for his brain surgery.  Surgery for placing the subdural grids took 8 hours from the time he left our side until we got to see Noah in recovery.  He had an 8x6 grid placed, 48 little EEG monitors, and 4 internal probes that help determine the depth of the seizure.  I will not lie, seeing Noah in recovery was the worst feeling ever.  I have never heard such a painful, hoarse cry.  

I remember just looking at him and my husband and thinking, “what have we done?”  He had a drain from his head, a catheter, two IV’s (in the end he had 7 IV’s placed), an aterial line, blood pressure cuff, and the no-no’s (which are Velcro arm braces to keep him from bending his arms at the elbow).  The first 24-hours he didn’t sleep at all and was quite uncomfortable, yet in good spirits.  He was placed in ICU for 24-hours, and then we were moved into the Epilepsy Monitoring Unit.  Noah had multiple seizures the second night at the hospital including one that lasted 4 minutes long, which is the longest that he’s ever had to my knowledge. 

By July 10, 2010 (this happens to be my husband and my wedding anniversary, 2010) they were taking

Noah back in for his resection at 1:30pm.  This surgery, the right occipital lobe resection, took 12 hours.  When we finally got to see him in recovery he was in much better spirits then the first time.  The neurosurgeon told us that everything went well and that when they tested for seizure activity before closing him up that the EEG went from active before surgery to completely inactive after surgery.  We were thrilled.  Noah was placed, again, in ICU for 24-hours and then moved back to the Epilepsy Monitoring Unit.  

We were able to leave the hospital by July 13, 2014.  

Noah has been seizure free since July 10, 2014!!

This is the longest he has been seizure free in at least a year. 

Noah is a completely different kid.  He is learning so much in the past few weeks.  He can sing almost all the words to “Twinkle Twinkle”, before he only knew “twinkle”; he’s copying saying his ABC’s; he’s counting; and overall he’s more interactive with everyone.  Noah is also not nearly as, shall I say, naughty, as he was prior to surgery.  He listens more and understands what’s being asked of him, so that results in less time outs and more fun times.  My heart is filled with overjoy.  I will not lie; I fear that one day this will all end and the seizures will come back.  My husband and I have talked about this; we both agree that if he starts having seizures today that these past four weeks have been absolutely worth the surgery.

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Thank you, Mallory!! What a week it has been, sharing your story!

Now, for any new Epilepsy families out there, Mallory has also added a list of meds that Noah has been on, as well as their side effects. This information can be very helpful, as you are learning more and more about the condition that is a part of your lives, now. 

Remember, 1 in 26 people have Epilepsy.

Medicines & the Effects on Noah PRIOR to Surgery:

·         Predisone

o   Made him go from small/petite to very chunky

o   Made him very sleepy (slept easily 18-20 hours a day)

·         Phenobarbital

o   Made him tired

o   Made him irritable

o   Caused excessive learning delays

o   Eventually hated the taste and refused to take it

·         Trileptial

o   Made him tired

o   If increased too much too fast it would make him extremely nauseous and make him walk like a drunken sailor

o   Made him irritable, but not as bad as the Phenobarbital

·         Clonazepam

o   No bad side effects

o   Helped him get some sleep at night and control his bedtime seizures

·         Onfi

o   Made him tired

o   Made him irritable

Epilepsy Families: Noah's Journey (pt. 1)

The family we are talking about this week is near and dear to our hearts. They just endured watching their son have a lobectomy as well. The difference? Noah is THREE.

Mallory, Noah's mom, had a difficult time condensing his super long story down to one post. I assured her that that makes TOTAL sense, and that it's 100% okay. In his short lifetime, Noah has been through so much. But I can promise you, this journey has a happy ending... or is it happy new beginning?

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My son, Noah, was born May 19, 2011.  Noah started having infantile spams at 10 weeks old.  However, we didn't know that’s what they were.  I just remember holding him or watching him play on the floor and then all of a sudden start twitching uncontrollably for what seemed like forever, but in reality it was more like a minute or two. 

 Just to give a little background, I had the absolute perfect pregnancy with no complications.  He was born 38w6d via emergency c-section only because I was in the beginning stages of labor, he had been complete breech the entire pregnancy, and the OB/GYN thought my amniotic fluid was infected because I wasn't feeling well that day at all.  My fluid was infected, but Noah was perfectly healthy and had no complications.

Fast-Forward: When I explained these “twitches” over the phone to my pediatrician at the time, at Ochsner Medical Center in New Orleans, she thought it sounded like when someone is just about to fall asleep and they bob their head and jerk it back.  At the time that seemed feasible to me, so I went with it.  The longer this continued the more I knew that this wasn’t right.  He was having these “jerks” anywhere from 10-20+ more a day, and each “jerking” session had at least 30-100+ “jerks.”  

At around 4 months old my husband and I took our son in to the pediatrician because he was having bad gas problems.  Coincidentally, Noah had one of his “twitches” in front of her and she immediately looked at us and said, “Is this what he’s been doing?”  We replied, “Yes.”  She then left the room, came back, and said, “I believe your son may be having seizures.  I’d like for him to go upstairs to get an EEG done right now, as well as blood work, and a urine collection.”  My husband and I were in complete shock.  Never in our minds did we think our son was having seizures; that seemed impossible.  Nonetheless, we went upstairs and Noah had his first EEG.  The EEG came back with abnormal results, blood & urine work came back normal.  We were then referred to a neurologist & genetic doctor.  Noah was sent home on a prescription for a steroid, Prednisone. Noah took Prednisone from September 2011 until December 2011.  Once Noah was weaned off Prednisone he started Phenobarbital; they actually started weaning him off the Prednisone while slowly adding the Phenobarbital in December 2011.  He had also undergone an MRI to check for any abnormalities in the brain; results came back “normal.”  

By January 2012, Noah was only on Phenobarbital and we thought it was the miracle drug.  Noah stopped

having his infantile spasms and was seizure free from January 2012-May 2012.  Just after Noah’s first birthday, the seizures started to come back; and these seizures were stronger than ever.  By this time, Noah had been in the Early Childhood Intervention (ECI) program for his learning delays.  He started ECI at 5 months old.  Noah did absolutely nothing, but sleep.  He had stopped rolling over, he didn't smile, he didn't laugh, and he didn't engage with anyone.  Noah was seeing a speech pathologist (who worked on feeding as well), a physical therapist, and occupational therapist, and a specialty instructor who helped with pretend play.  

We saw the genetic doctor to rule out any other abnormality.  The genetic doctor did all of his tests; they all came back normal.  However, the genetic doctor felt he had “secondary mitochondrial disease.”  Oddly enough, we found out that this was his “go-to” diagnosis when he couldn’t give the parents an exact diagnosis.  This genetic doctor couldn’t even tell us what it was secondary too.    Of course we wanted a second opinion and we were just in luck.  My husband had accepted a job in Houston where we were then able to take our son to the #4 ranked hospital nationally, Texas Children’s Hospital.

Finally, we moved to Houston.  Noah went to his first neurologist appointment in Houston in the Epilepsy Clinic at Texas Children’s Hospital in November 2012.  First of all, this hospital is absolutely amazing and everyone is super friendly; I highly recommend them if you’re able to go there.  This is when we were actually told for the first time that Noah had a “seizure disorder” called Epilepsy.  Before now, the doctors in New Orleans just referred to it as infantile spasms; wow was that a bit of a shock to the system, especially when the Houston neurologist said that it was already in Noah’s charts as Epilepsy. We then started discussing everything that had been going on.  As soon as our Houston neurologist heard Noah was taking Phenobarbital the first thing he said is, “We are going to wean him off that ASAP.”  Of course my husband and I were thrown off by this and we asked them why.  Noah’s neurologist explained that Phenobarbital is one of the first medications on record to treat Epilepsy and it’s proven that Phenobarbital causes learning delays on top of other complications. So our neurologist added Trilepital with Noah’s Phenobarbital.  Noah also had two additional EEG’s at this time, an MRI, and blood work.  Both of Noah’s EEG’s came back abnormal, and then his MRI and blood work came back normal.  Noah started his wean off of Phenobarbital in December 2012 and was completely off of it by January 2013. 

Between January 2013 and May 2013 we basically monitored Noah and his seizures.  I have kept a chart since he was about 5 months old where I tallied each seizure, AM or PM, what it looked like, what he was doing, how long it lasted, etc.  I take these charts, which I still use today, to each appointment to be analyzed with Noah’s neurologist.  Almost immediately after Noah was completely weaned off the Phenobarbital he started learning and doing things he had never done before; it was like night and day.  We were happy that he was picking up on things and we loved the new change in Noah.

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Thank you, Mallory for part one of this story!! 

Please join us, as we look forward to part two, on Wednesday!