Name: Teigan

Sex: female

Date of birth: 23 April 1998

Now lives in: Alberta Canada


Epilepsy info

Diagnose:
Dravet's Syndrome, September 2000

type(s) of seizures:
Tonic-clonic, complex partials, myoclonic jerks, simple partial. Prone to tonic clonic status events

frequency of seizures:
She is having about 2 per month now, could be any type mentioned

Medications used

Current:
Lorazepam (Ativan), Used only to stop status seizures. We can give up to 4mg at home, then hospital gives more if seizure persists.



Previous:
Phenobarbital, This is the first med she was put on. Teigan is on a relatively low dose now, just as a back up in case we need to pull out of a new med quickly. The docs want her to have something still in her system if we have to pull off another med.
Clobazam (Frisium), Teigan started having 2-3 seizures per week while on frisium. Most wouldn't become status, but it was still unacceptable
Topiramate (Topamax), Topomax seemed to be working at 50mg per day, then as amount was increased, so was seizure frequency
Carbamazepine (Tegretol, Carbatrol), Tegretol aggravated the seizures majorly. Teigan began to have myoclonic jerks, which she had never had prior to tegretol. Once Tegretol was stopped, jerks reduced (from 200/day to 50/day) but they never went away
Gabapentin (Neurontin), Nothing on this med. No side effects, no control
Valproic Acid (Depakene, Epival)
Lamotrigine (Lamictal), Another terrible med for us. Teigan began having 1-5 complex partials/day, and 500-600 myoclonic jerks/day. Although we never went status in the time we were on Lamictal, it was a terrible time.It has since been brought to my attention that tegretol and Lamictal are known irritants for SMEI, but Teigan was not diagnosed back then



Alternative Treatments

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Personal Notes

At the age of 4 months my daughter had her first seizure. It was a focal seizure, affecting her right arm only. The arm was totally limp after for about 1 hour. The doctors said it was a fluke thing and would probably never happen again. At 6 months she had a really big seizure lasting 1 hour, 45 minutes. They called it tonic-clonic and said it was status epilepticus. I now have been told that with tonic-clonic, you always lose consciousness. My daughter is awake during these seizures, and can follow me with her eyes. She does not seem to be aware that her body is seizing. This seizure was stopped by the doctors after administering 3 doses of valium rectally, 1 dose of ativan, then 1 loading dose of phenobarbital. At 7 months (Nov. 19) she had another big seizure same as above, but lasting only 50 minutes. The right side of her body stopped after 35 minutes, the left after 50 minutes. This one was stopped with 2 doses valium, and 1 of ativan. Her CT scan and EEG have come back normal, and we are still waiting for the results of the MRI which was done on the 25th. This is horrible ! Our little girl is the picture of health otherwise, and we don't understand why this is happening. The doctors love to use the word 'idiopathic meaning 'no known cause' which is driving us nuts! We want answers. The doctors and neurologists don't seem to know what they are talking about. The most helpful person to help us has been the neurological RN. She has been dealing with epilepsy patients for 21 years and seems to have better knowledge than any doctor! We are lost. Any one have similar story? We seem to be having a hard time finding anyone who has experience with these types of seizures, would appreciate any info on what to expect now. Thanks.
Update 1/28/99: Teigan has had a few more seizures. Dec.10 1998 she had a seizure which we stopped at home with rectal ativan. It lasted 24 minutes before we stopped it.We were so excited that we didn't have to go to the hospital to have them stopped anymore! Then on Jan.13 1999 she had another seizure which we couldn't stop. The hospital stopped it again. The neuro said that he didn't want us stopping the seizures here anymore as he wanted to start isolating the meds that worked best for stopping the seizure. On Jan 19 1999, she had another. The hospital tried Dilantin to stop it, but it was ineffective without the ativan. Numerous tests have been done now, 2EEG's, 2 SEP tests(long and short versions), SPECT scan, and they attempted a lumbar puncture, but after 7 pokes couldn't get it. We are still awaiting most results. The EEG which was done after the seizure on the 13th showed very slight suppresion on the left side. The one after the 19th was normal. I really wish I understood why these things are showing my daughter as 100% fine when we know there is something wrong. I will update again when we get some results.

UPDATE: Teigan is 6 now! Thanks to the internet, I was able to locate a family whose child sounded so much like Teigan. They had a diagnoses of Severe Myoclonic Epilepsy in Infancy (SMEI) I approached our neuro, after a few months, he agreed. SMEI has now been renamed Dravets Syndrome. There is a gene mutation commonly seen (although this is a new finding) and Teigan has tested positive to a missense mutation in the SCN1A gene. Teigan has not been status in almost 3 years! She is on a non-FDA approved medication from France called Stiripentol, along with Clonazapam. She has a 2-5 minute t/c every 2-4 weks, and a few myoclonic jerks in the AM, but is doing fabulous. She is developementally delayed, and we know that she will be dependant for life, but she is definately in the higher end of the spectrum for this disorder. We are thrilled with how great things are. If anyone thinks their child may have this syndrome, please mail me. It is so lonely to be suffering with status events, and no one understands exactly.Best wishes to all!


Contact

Mum: Paula (janfield@shaw.ca)


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