type(s) of seizures: Started with tonic-clonic seizures later on all sorts of seizures.
frequency of seizures: There have been times where she didnot have a moment of rest she had permanent seizures.
There have also been times where she reacted good on her medication and had hardly any szs. at all.
additional health issues: She has been very ill. Had developmental delays extrapyramidal symptoms has suffered blindness but fully recovered. And splenomegaly. The last year of her life she had to be tube fed for several months.
Medications used (in chronological order)
Vigabatrin (Sabril)
still being used: Yes
effectiveness: Positive
max. dose: 1000 mg
positive effect: Controlled seizures
side effects: Non so far
comments: She was given sabril in stead of klonopin the depakine has later been changed into lamictal which she still uses so now (febr.'99) she is on lamictal and sabril she is now also slowely stopping with sabril seizures increase
comments: She was given Depakene to replace the phenobarbital.
Lamotrigine (Lamictal)
still being used: No
effectiveness: Positive
max. dose: 50 mg a day
positive effect: Seems to stop seisures
side effects: Rashes allergic reaction
comments: After the use of lamictal for over one year, with good results, Julia suddenly became severely allergic to the drug. So badly she had to be take to the intensive care for one week
Phenobarbital
still being used: No
effectiveness: Positive
max. dose: 50 mg.
positive effect: Controlled seizures for a while
side effects: None
comments: Needed increasingly higher doses
Clonazepam (Klonopin, Rivotril)
still being used: No
effectiveness: Negative
max. dose: Unknown
positive effect: None
comments: She had the combination of depakine and klonopin only for about one week. we are not sure at all but we think the combination was allmost fatal to her.
Topiramate (Topamax)
still being used: Yes
effectiveness: Positive
max. dose: 250 Mg
positive effect: Allert less seiz.
side effects: None
Personal Notes
Julia suffered her first grand-mal when she was three months old. With ups and downs it was controllable until august 1997 for three days she was given Phenobarbital which was poorly prepared seizures increased and did not stop after the error in the medication was corrected. She was hospitalized and set from Phenobarbital to depakine. Seizures increased she could not lay still. Then Rivotril was added to try and stop the continues seizures. She was transferred to an academic hospital on 16-09-1997. Nothing seemed to help and the doctors in the new hospital wanted to examine her. The next day she almost seemed to be in a coma and she started to slip away to a point where she was only breathing. Doctors told us on Thursday to call for our parents because they feared the might not make it through the weekend. They changed the rivotril to sabril and added B6 vitamin. After a few days she started to respond a little! We have the idea that the combination of depakine and Rivotril was almost fatal to her.
She has had some 20 EEG tests and 5 brain scans showing seizure activity throughout the entire brain. She has been tested negative on all known metabolic diseases and she also tested negative on chromosome deficits. She has never been able to walk nor talk and had poor fine motor skills (level of a child of about 7 months). However; she could understand us and found ways of letting us know what she wanted!
At the age of 3 and a half years old she was making progress slowly but steadily learning. She could now crawl and say 'Mom' and 'Dad' other words are still childhood murmur. We had good hopes she would learn to walk, she was very strong and persistent. Her medication had (again) been changed from sabril to topomax and seizure activity decreased form szs/per week to a few szs per month! After further reading and learning we become more convinced that the pertussis-shot rather than the poorly prepared medicine caused Julia to become the way she is now, she had her first sz three days after her first shot.
When Julia was 4 years old she was doing very well on Lamictal. Up until the day she developed this rash and it turned out that she had become allergic to the medication. (overnight without any change in her dose) She was immediately put of off the lamictal- went into a status epilepticus and was rushed to the IC. After two terrifying weeks she started to recover. From this point on Julia's life became harder and harder.
(During this time we were told she might have Rett so we learned all on Rett and became very scared. Later-on tests showed it was not the Rett-syndrome. What did affect our child and made her loss all of her abilities? We never knew.)
Szs returned, many and very heavy. Day's with over 20 szs. were very common again. Julia started to loose all of her abilities. She had to be tube-fed, could no longer crawl nor sit up straight. She lost interest in the Teletubbies, her all time favorites, and communicated on al much lower level.
Julia was hospitalized on many occasions, during 2003 over 17 times. It turned out that Diphantoine given IV in high doses could block the szs for almost 24hrs. Strange enough a permanent high dose was ineffective.
The last year of her life was very hard on our beautiful little girl and on Friday 3th October she had a cardiac arrest when she was on my lap ( I was feeding her) We were able to revive her together with the ambulance crew who were at our home in minutes.
When Julia was stable and at the IC-unit of our hospital we learned that there was a lot of damage done already and the doctors made it clear to us that this time she would not be able to recover.
Al through the weekend family and close friends had the change to come and say goodbye to our little girl.
On Monday the 6th of October 2003 the doctors stopped the respiration-machine and Julia breathed on her own again.
But the tests showed hardly any brain activity at all and the doctors were afraid that when the tube would be fully removed she might die shortly after.
The doctors agreed to bring Julia back to our home and to remove the tube there so that she could die with us in her own home.
It was heartwarming to see how all the medical-staff was working very hard to make Julia's last hours as easy as possible. She was brought to our home accompanied by two doctors and a nurse (who stayed for hours and returned again later that night!)
When the medics rolled her into the room she blinked her eyes for the last time and at 01:45 Tuesday morning she died in our arms.
Julia left us; her mother father and older brother, behind and we miss her dearly.
There are no words to express our pain but we will meet her again someday, somewhere.
If you would like to see why Julia was such a remarkable girl I invite you to some pictures of her at:
http://homepage.mac.com/bduijvelshoff/Julia/PhotoAlbum22.html
I have written a poem By which I mean to express
how good it is when people share our memories of her.
It is not scary to think of her. It is good.
Come travel together
through my darkest night
a sweet moon shall join us
like our gentle goddess
and the aroma of her beautiful life
shall find you in your eternal dream
Thanks for reading about Julia
Contact info
For additional information, please send e-mail to Bart (duijv003@wxs.nl)
