Jordan A.'s Story

Jordan was born on February 5, 2004, at 7 pounds, 11 oz. He had neonatal jaundice and an umbilical granuloma, but otherwise was a perfectly healthy little boy. He was very pale skinned and bald as an eagle! During the first months of life the only thing we noticed that was different about Jordan was that he did not take good naps and suffered from multiple ear infections. Where my older son, and most babies I have ever met, take 3 to 4 hour naps throughout the day, Jordan preferred to nap for 15 minutes several times a day and appeared to "startle" awake. We noticed early on he had an elevated startle reflex, with him startling to noises like the dogs barking, phone ringing, or it being too loud in the house, etc.

Jordan moved along those first few months meeting his developmental milestones on time. He rolled over at 2 months, sat unassisted at 5 months and crawled at 7 months. He cooed and smiled and made noises. He loved to sit in his Exersaucer and play with toys, watch TV, and take baths! Some days he would have 3 baths just because he would crawl into the bathroom and want one. Jordan walked at 13 months.

There were two separate occasions between birth and 10 months (unfortunately I cannot remember the dates or his exact age) where Jordan did something I questioned, but with my paramedic training these episodes did not fit "seizure activity" completely and these episodes were blown off. On these two occasions I was holding Jordan and feeding him a bottle and at one point during the feeding he clamped down on the bottle tightly and kind of just "shuddered" for a few seconds. I remember asking my husband what that was and if it was a seizure and he assured me it was not. I remember looking back at these episodes later and thinking to myself how ironic it was that I thought Jordan was having a seizure when he wasn’t, and now he was having seizures. I did learn much later on from another mom on my Pyridoxine Dependent Epilepsy group that her son did the exact same thing, so maybe it was seizure activity after all.

Jordan's first seizure was on November 27, 2004 at 5:55 p.m. in the bathtub. I think that no matter how many seizures Jordan may have, that day, time and the circumstances will always stick in my mind clear as day – as that is the day our world changed forever. He got in the bathtub at 5:45 p.m. At 5:55 p.m. he sat forward and held his right foot with his right hand and made a face that appeared as though he was trying to have a bowel movement. I took him out of the tub as that was not a mess I wanted to clean up and realized that his right arm was "shaking". Feeling he was trembling from being cold I wrapped him in a towel and took him upstairs to his room where it was warmer. I lay him down on the floor and dried him off and put a diaper on, but noticed that he was still shaking in that arm. I picked him up and called my husband who was at work and told him I was afraid something was wrong. At that point Jordan began grunting. It was apparent he could no longer hold his head up and was beginning to drool. My husband, who is also a paramedic, came immediately to the house with an ambulance and off they went.

The seizure lasted 25 minutes. Jordan was diagnosed as having a febrile seizure, as he was suffering from an ear infection at that time. We were discharged 2 days later, being told it would probably never happen again. Unfortunately, it did.

Jordan had two more seizures that year, one on December 16th which was a partial of his right arm only while in the tub. He again had an ear infection, so we immediately pulled him from the tub and cooled him off with tepid water and this one stopped in less than 1 minute. The second was on December 24th when we were upstairs taking pictures of him in his first Christmas outfit and he went into another partial of his right arm. We stripped him down and this also stopped in less than a minute. He again had an upper respiratory infection. This put his seizure count for 2004 at 3.

On January 9, 2005 Jordan had a bilateral myringotomy and tube insertion to hopefully reduce his ear infections and upper respiratory problems and hopefully end the seizures. This didn’t go very well, as the left tube became blocked with blood and was never functional.

On January 13, 2005 Jordan was upstairs in his room playing and I was sitting in his rocking chair watching him. He turned to me, cried out and dropped to the floor in a full grand mal seizure. He was again taken by ambulance to the hospital and this time required intubation due to rapid breathing at 60 to 80 times a minute. This seizure lasted 45 minutes. They left him intubated for 18 hours while fighting over getting him an MRI. We finally won, he got his MRI and another EEG and both were normal. Jordan had croup.

Up until this point we had declined anti-convulsant medications, but due to the severity this time and the intubation we were scared enough to be convinced to start them. I still to this day wonder where we would be if we never had, but who can turn back the clock? Jordan was started on Trileptal. We were also given Diastat for home use of any seizures lasting more than 5 minutes.

For 20 days he went seizure free, but then the seizures actually got worse on this medication. He had a stint in March, 2005 where he had a seizure almost every other day. The Diastat did not work in stopping his seizures, so because we are paramedics we were given permission to use Versed (midazolam) injections to stop his seizures. This proved to be a wonder drug and always stopped his seizures and we no longer had to go to the hospital.

Jordan was diagnosed with Dravet syndrome in August 2005. This is a day that will also stick in my mind forever. I was sitting on the couch reading the papers that came with this horrible diagnosis while my perfectly "normal" child was sitting on the floor playing with his toys. He had not had a seizure in months and this diagnosis was extremely hard to accept. On one hand it is good that they are diagnosing this earlier as there are so many medications that make this condition worse, and an early diagnosis allows you the opportunity to avoid these; however, at the same time it steals away your baby’s childhood and all your dreams at a very early age.

Over the past five years since the diagnosis, Jordan has been tried on Topamax (which caused myoclonics), Depakote (which caused nighttime seizures), Keppra (which caused bad behavior), vitamin B6, phenobarbital and clonazepam. When the vitamin B6 was first put in Jordan had 7 months without seizures and we thought we had our answer. However, after having 10 seizures in one day in September, 2007 it was apparent that it had become toxic and was actually causing seizures. The best control Jordan has ever had is on phenobarbital, clonazepam and cyproheptadine. On this regimen from June 2008 to June 2009 Jordan barely had any seizures at all.

Jordan also has a diagnosis of autism. However, our neurologist now believes that his autistic traits are from long-term use of phenobarbital and may reverse if it is removed. We have been afraid to do this because of the good seizure control he has gotten on it. However, as always seems to be the case with Dravet, Jordan began doing worse again in July, 2009 and it has been found that his phenobarbital is now too high and toxic, which is actually causing seizures. So, we are weaning this and hoping to also wean clonazepam and see where we will go from there.

Jordan is currently almost 6 years old, five years after diagnosis, and is a happy, bright and wonderful child. He is currently nonverbal, but we hope that will change with the removal of the phenobarbital. He is a joy to be around and even with all of his challenges that we face every day, I wouldn’t change a thing about him. He goes to school and loves to learn. He loves to take car rides, go swimming and go to amusement parks. Although it took years to happen he did become developmentally delayed, but mainly in the areas of socialization with other children and speech. We are currently working on those. He also has challenging behavior at times, but we are working on that as well.

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