David’s Story with F.I.R.E.S.

David’s story began a couple of weeks before his seizures started.

Around January 17th, David came down with what seemed to be a cold.  I took him to the doctor, where he was prescribed cough medicine.  He had no fever, just a cough and runny nose.  A week and a half after his visit he started throwing up, and then threw up a few times more.  I kept him home from school the day after, giving him Pedialyte – I wanted him to go 24  hours without throwing up before sending him back to school.  He met that criteria, and was his normal happy-go-lucky self, so I sent him back to school on Wednesday, January 25th. That evening, we were on our way to church, when David started throwing up again in the backseat of the car.  I turned the car around, and headed home.  He didn’t throw up any more that night.  When I tucked him in that night he was calm and happy.  The next morning is when everything changed.

When I went to bed that night, I decided I wouldn’t send David to school the next day.  He had thrown up the night before, and obviously needed to fight off whatever bug was in his system.  It was for that reason that I did not wake up my children early that morning. I was able to sleep in until 9am that morning, which was nice- I needed that.  When I walked into David’s room to wake him up, he was in the fetal position on his bed.  When I tried to rouse him, he would groan.  This was not his normal behavior.  I tried to pick him up to hold him in my lap, but he did not want to move out of the position he was in. Finally, I got in his face, scared, and said loudly, “David, please open your eyes, you are scaring me!”  He opened his eyes, but looked scared himself.  He started acting like he didn’t recognize me.  He wouldn’t speak, he wouldn’t walk, and he didn’t even know his own mother.  I decided to take him to the E.R.  It sounds crazy since I was taking him based on his behavior, but I knew something was wrong.

Now would be a good time to mention that my husband was overseas while this was going on.

I couldn’t get David to walk of course, so I had to carry him to the car.  I have another son, Gabe, who was 4 years old at this time, so of course, I had to bring him with us.  I called my pastor and his wife to let them know what was going on.  They knew my husband was out of town, and that I would need help.  When I parked my car, I had to figure out how I was to carry my six year old, and still hold my four year old’s hand across the parking lot.  Thankfully, halfway through walking, a gentleman saw me and asked if I needed help.  He carried David into the waiting room and laid him down on a chair.  I left him in the chair, with his little brother sitting next to him, and walked up to the line to register him.  While I was in line, he started shaking.  He was having a seizure. I no longer had to worry about waiting in line, as a gentleman working there immediately came to help, grabbed David, and took him into a room.  It was there that I learned David must have had a seizure that morning before I woke up.

Once Gabe and I were in David’s room, all the questions came.  Has he ever had a seizure before?  No.  Was he on any medications?  Just Bromphed cough syrup.  I went over the past two weeks with the staff, explaining everything I could think of explaining. They asked if anyone had been out of the country recently.  The day I took David to the doctor for his cough, his dad came home for just one day, his birthday, and then had to go back overseas for a couple more weeks.  I explained that my husband was in the military, so yes, his dad had recently been out of the country, and in fact, was at this moment. After that it dawned on me that I needed to get in touch with him so he could come home.  His command was great, sent out a red cross message to him, and had him home at 2 am the next morning.  My pastor’s wife arrived, and was just a huge help with Gabe.  The staff explained that they would have to transfer David to another hospital.  My pastor’s son then arrived, and took Gabe with him, so that I could ride with David in the ambulance to the new hospital, and our pastor’s wife could follow us.

That night my husband finally arrived.  What a relief.

David was attached to all kinds of monitors, an EEG, IVs…everything you can think of. The initial thought was that David had viral encephalitis.  We had contagious disease doctors come in, spinal taps, everything and everyone you can think of, trying to figure out what was wrong with David.  After a week at this hospital though, we saw no progress.  His seizures became worse and got closer together.  On his last day at this hospital, he had 3 seizures within ten minutes.  The staff then decided it was time to transfer him to a different hospital, and they knew the neurologist there specialized in cases like David’s.

David was life-flighted to Nicklaus Children’s Hospital in Miami.  He was put in a medical coma to stop his seizures while they treated him, and the unofficial diagnosis at this hospital was F.I.R.E.S.  As with most F.I.R.E.S. cases, they put him in a medical coma several times, and hoped that once they brought him out of it, that his brain would sort of reset, and he would stop having seizures.  That never happened.  About two weeks in, we were told that if he kept having seizures like he was, that either his heart would stop, or if he kept living, that he would be a vegetable, completely dependent on life support.

It was then that we were told about a trial drug that had promising results.  It was a double-blind study, so we wouldn’t know if he received the trial drug or not, but that if after a week it looked like he still needed the actual drug, he would receive the real thing. My husband and I went home, and did our own research.  After looking through all we could find, we agreed to the trial.  (http://www.statustrial.com/fflop/) *(Update- this drug is no longer available as it was not approved by the FDA for status seizures.)*

I will never know for sure if we received the actual drug, but I believe we did, because soon after the trial, David’s seizures became responsive to medication- they were actually controllable!  It wasn’t all smooth sailing though.  He had withdrawals from several of the drugs he was being weaned off of (not the trial drug).  A 6-year-old having drug withdrawals, poor thing. He was given different medications to ease the withdrawals from others.  David endured a lot during this time.  Two collapsed lungs, one of them a couple of times, severe withdrawals from drugs, at one point we couldn’t open any windows in his room because his skin was too sensitive and would react with a rash to light.  At another point doctors thought they may have to perform surgery on David because they saw what seemed to be a tear in his stomach lining.  They had to take him off two of the drugs that were controlling his seizures because of the tear, and he had more seizures.  The stomach lining issue eventually fixed itself and doctors were able to put him back on the anti-seizure meds he needed.    It was a long road.  Finally, he started to look like himself again.

And on March 5th, he finally woke up from the medically induced coma (which was part of the trial).

He was very groggy when he woke up, but he pointed to the tv that was playing the Minions movie.  I asked him if he wanted to watch it where it was, or if he wanted me to start it from the beginning, and he weakly said, “from the beginning.”  He was able to communicate clearly with me!  I was thrilled!  The above photo was taken the next day, March 6th- I asked him if he could smile to let everyone know he was doing okay.

David’s personality was intact!  He began making jokes, laughing, and saying some of his normal David-isms.  Soon after, he began physical and speech therapy sessions.  During this time, he was still very loopy- he was on a lot of medication and had his days and nights mixed up for a while.  And when I say he was extremely loopy, I mean he was high as a kite.

David was doing so well that he was moved from the PICU to the neurology floor, which meant that after 7 long weeks apart, Gabe was finally able to see and play with David.

Soon after, the staff worked on getting his ng tube out.  Once it was out, the first thing he asked for was a hot-dog.  The cafeteria didn’t have hot-dogs, so I went to a fast food place down the street and brought him a hot-dog.

Gabe began to visit his brother almost every day with us.  We truly believe that this helped shorten David’s recovery time.  They were able to snuggle and watch movies together.

It took a while for doctors to get David’s medications right before he could leave the hospital.  Seizures are different for everyone, and finding the right cocktail can be tough.  Every time doctors would lower his Phenobarbital dose, he would have a seizure, so we were on a pretty high dose for a while.  Another medication he was dependent on was Ativan.

Initially, David was released from the hospital on March 30th.

However, after having him home for a day, he began having seizures again.  We took him to the E.R., he was readmitted, and then it became clear that the problem was taking him completely off Ativan.  He was discharged again on April 4th, with an additional prescription of a tiny dose of Ativan.  That was all he needed.  A few days later, we had a birthday/welcome home party for David. (He spent his 7th birthday in a medical coma.)

David has now been home for 14 weeks.  There is one medication he has been on that has been completely terrible for him- Keppra.  I know there are many people it works out great for, but on Keppra, David experienced Kepprage.  He became very hostile, angry, and aggressive- it was horrible.  He would have bouts of behavior where it felt like we were interacting with a feral animal.  That is honestly the best way I know how to describe it, even though it sounds terrible.  We would question ourselves:  Is he really okay?  He was acting fine in the hospital, so what is going on now?  After bringing this behavior up to our neurologist, she agreed it was best to wean off Keppra.  As of tomorrow, we will have successfully ended a 2-month wean of Keppra.  We didn’t see a huge difference until about 3 weeks ago.  So for a while there, it was rough.  Really rough.  But now, he is behaving much more like himself- the more medication we wean, the more of David we see.  He is also on Phenobarbital, the small dose of Ativan mentioned, Vimpat, Onfi, and Topamax.  We have have lowered his doses of Phenobarbital and Topamax from the original, and he has handled it well so far. He had a very mild seizure this past Friday, but we think it was a delayed reaction to lowering his Phenobarbital.  He hasn’t had any since, which means he is breaking his old seizure patterns (thank God)!  He will be starting 2nd grade in August.

The bottom line:  When looking for success stories of those diagnosed with F.I.R.E.S., we found very little.  We’ve seen a couple cases of kids whose seizures were controlled on the Ketogenic Diet, and a couple that were doing well taking Epidiolex, but what worked for us was the Sage Trial.  Again, I can’t guarantee that we got the actual drug during this trial, but I can say that now David’s seizures are responsive to medication.  David is a walking success story of a child who has not only survived F.I.R.E.S., but is thriving, and continues to improve every day.  Please see the link I attached if you or your doctor think your child may have F.I.R.E.S., or Status Epilepticus.

http://www.statustrial.com/fflop/

PS- An extra special thanks to all of the doctors, nurses, and staff that helped David during his hospital stays, and to our family, framily (friends we claim as family), church family, and friends who continually prayed, supported, loved, and kept us sane throughout.  We love you, all!