Brian had his first seizure at 4 months old and it lasted 35 minutes. It took the ER doctors a long time to stop it. He had to be transported to a larger hospital by ambulance where he was kept for several nights, released, put on phenobarbital and told it probably wouldn't happen again. Unfortunately, it did, over and over again for the next few years. When he was one year old, we went to see French epileptologist, Dr. Olivier Dulac, in Paris, France, upon the recommendation of our pediatric neurologist, Dr. Karsten Gammeltoft, in Knoxville, TN. Dr. Dulac said that Brian had SMEI (Severe Myoclonic Epilepsy of Infancy soon to be known as Dravet syndrome after Dr. Charlotte Dravet who first discovered/diagnosed it). Dr. Dulac told me that the life expectancy was 10-12 years and that Brian would develop normally for a few years and then regress. I sat in his office and cried. When we returned home, I took Brian to see a developmental and genetic specialist to get a baseline for his development. He was already behind. I began seeking OT and speech therapy and doing all in my power to push him to be “normal”.

Unfortunately, the seizures continued. We tried more than 20 different medications in different combinations in an attempt to find the right “cocktail” to control the seizures. We tried to figure out what caused the seizures, knowing that illness and fevers did, but oftentimes there seemed to be no reason. Because he had so many ear infections, we decided to have his tonsils removed and tubes put in his ears just before Christmas in 1994, when he was 18 months old. He stayed overnight in the hospital for this, but unfortunately, when I gave him his medicine the next day at home through a syringe, he seized, aspirated, and developed ARDS (adult respiratory distress syndrome). He ended up in the PICU (pediatric intensive care unit) for 14 days on a respirator fighting for his life with a 50/50 chance of survival. We prayed incessantly; talked, sang, and read to him; massaged him; and did all we could to help him while he was in an induced coma. God answered our prayers and he was able to come home in mid-January. He had to have intensive PT, OT, and speech therapy for several months as well as being on oxygen.

For the next 18 months he was seizure free. However, this didn't last. The seizures returned as did the feeling of being a guinea pig experimenting with various seizure medications trying to get control. We tried everything, even medications that were later found to be contra-indicative for Dravet syndrome. We even tried the ketogenic diet before it was “in vogue”. We stayed on it for 18 months, and it did stop the myoclonic jerks, but not the seizures and we were never able to wean off all his medications.

When Brian was nine, his seizures became worse again and nothing was working. We were considering the VNS, but I was hesitant. We went to France in May and saw Dr. Dulac again. I told him that I was at my wits end and asked about the VNS. He discouraged me from doing it. He said that if Brian were younger, he would try the Clobazam in combination with the Stiripentol (which was still in the experimental stage in Europe at that time). I begged for him to let us try it as we were out of options, and he agreed. Because the Stiripentol was in trial, we were able to get it for free for several years. The Clobazam had been in use for many years. These two drugs in addition to the Depakote and Topamax that he was already taking proved to be our magic medication cocktail and we were relatively seizure free for many years, having only three or four seizures a year.

As Brian grew older, his seizure types changed. He began to have more nocturnal seizures, and drop seizures lasting for only 2-3 minutes.

Brian is now 31 years old, and functions on the level of a 6-10 year old. He loves pretty girls, knows no strangers, wants to pet every dog we meet, and wants to thank every veteran for their service. He is loving and very extroverted. He loves to go and do, and we enjoy sight seeing together.  He continues to have small seizures on a regular basis but comes out of them without the need for rescue medications.  

I am so thankful to the DSF for all that they have done to move the FDA to approve new medications for the treatment of Dravet syndrome, and for all the encouragement and support that they provide families.