Rare Disease Story: Jeremiah

Lennox Gastaut Syndrome

Jeremiah was born healthy, except for a bit of jaundice that cleared up with bili lights after a week. He had the normal childhood illnesses and was on track with his milestones. At age 2 1/2, he was finally big enough to move from a crib to a toddler bed. On Sunday, August 24 2008, my then 15 year old son went to put Jeremiah to bed. He was laying down with him, trying to get him to sleep when he said that Jeremiah dropped his bottle so he picked it up. It was then that Jeremiah started shaking so he thought that Jeremiah was cold and he pulled the blanket up. But Jeremiah wouldnt stop shaking and he started making noises. He turned the light on and noticed that Jeremiah was having a seizure. Alex came running into my room and said: "Jeremiah is having a seizure!" I said,  "How do you know its a seizure?" and told my husband I would go check on him to find out what was going on. I saw Jeremiah in a full blown tonic/clonic seizure. I yelled for my husband and, after he came running in, I called 911. That was Jeremiah's first of MANY seizures to come. 

3 weeks later, on a Sunday night, he had another seizure at bedtime. At that point, we took him to a neurologist at Primary Children's Medical Center. They got him in for an EEG and when we got home, he had his first day time seizure. He was standing up in the dining room about to get lunch, dropped to the floor and had a seizure. 

After 3 months of sporadic seizures, they admitted him to the hospital for an over night eeg. It was early December. They had enough information after an hour but decided to keep him hooked up and finish the 24 hour eeg. We found out that he was even seizing while carrying on a conversation with the doctor! After that EEG, Jeremiah was diagnosed with Lennox Gastaut Syndrome. 

Jeremiah then took a turn for the worst: he was in the hospital in Status Epilepticus every month for a week or more, sometimes twice a month, for an entire year. 

In February of 2009 he was started on IVIg. In March 2009 he had the VNS placed. We were to the point that he was on 6 oral meds plus IVIg. He was like a zombie. He stopped talking, went back in diapers and 100% bottle fed. While in Status, he was having over 300 seizures per day, and over 100 when not in Status. My parents started the plot search and the doctors didn't think he was going to live. The prognosis was not good. No one ever thought he would get potty trained, talk or even learn. We were told to expect him to be a toddler his whole life. He stopped growing and putting on weight. 

Today, Jeremiah is a healthy 7 year old. He is in first grade and doing well! He goes in every 2 weeks for his IVIg infusion and is on 2 oral meds. He is also taking an experimental medication. He was potty trained 2 weeks before kindergarten and off the bottle in January 2012. He is talking and learning, but in a functional skills class due to his being behind. He is a 2 year old mentally. He talks like a 2 year old, and it is hard to understand him at times. But, he defied the odds and he is thriving! He is down to about 20-30 seizures per month. We see the light at the end of the tunnel now, where a few years ago, we didn't. 

LGS no longer defines who Jeremiah is.