Please keep in mind that our plan for managing DJ's seizures was conceived after several discussions with the pediatric neurologist and the pediatrician. We also update our seizure management plan at regular intervals with our doctors. This plan was also tailored specifically for DJ. Other children with Tay-Sachs have been known to present with seizures and receive different medications . . . so what works for one child may not work for another child.

For the first two years, DJ's seizures were always short in duration, usually lasting under a minute. Therefore, we initially decided not to put DJ on daily medications for seizures. We were opposed to seizure medications because the doses need to be carefully controlled. Most of the medications that were suggested (Phenobarbital, Dilantin, and Tegretol) are toxic to the liver and bone marrow, so you need to take frequent blood draws to check the levels of the drug in the blood with regard to liver function. In addition, one common side effect of anticonvulsants is excessive sedation. We did not want to lose DJ's ability to respond to us. Therefore, we decided to use a medication called Klonopin; we used it as needed, i.e. just on bad days when the seizures interfered with his ability to eat. Klonopin is in the class of benzodiazepenes, a tranquilizer that also serves as a muscle relaxant. The one drawback of Klonopin was increased secretions. After he turned 4 years old, we noticed that DJ's seizures were especially bad after 2 AM and it was severely interfering with his sleep. We decided to start him on a daily dose of Ativan (lorazepam), another benzodiazepene, at bedtime.

Over the course of his life, DJ has exhibited a whole range of seizures. Some seizures come and go, while others seem to disappear as he ages. The first sign was nystagmus - a rapid, exaggerated, involuntary eye movement when he tilted back too quickly. He would also stare off into space - another sign of seizures. Then he started having seizures where his arms and legs would tremble and shake. He graduated to "flying" - his arms would fly straight up to the sky, he would arch his back slightly, and he would cry out. Although these seizures looked painful, they were not. Afterwards, he seemed exhausted and would sleep for a long time. After DJ became 2 years old, he had bigger, jerky movements of his limbs as well as involuntary facial grimaces. Our friend, Joel Du Vall, called this seizure the "Popeye" face, because of the lopsided grimace. The most physically demanding seizures were the tonic-clonic seizures where his back would arch and his limbs would literally drum against the floor. Thankfully, tonic-clonic episodes are very rare. DJ's seizures are generally short episodes, but can come in clusters and last several hours, so it always good to have Diastat (valium) on hand to help break them.

DJ also has some seizures where he does not move at all. He sometimes has his eyes would roll back into his head, he would be comeletely limp like a piece of seaweed. He would lose consciousness, for anywhere between a couple of minutes to a few hours. The most disturbing seizures where the ones where he would hold his breath for a minute or two, then suddenly take a deep breath and start up again. Luckily, these were very rare.

After age 3 years, we noticed a few changes. DJ outgrew his startle reflex. He stopped making noise (crying out) during seizures. Today, he tends to turn/pull and twist his body very strongly to one side. We occasionally give Tylenol (acetaminophen) after the seizures are over, to help with lingering muscle ache or pain.