Haley Is My Hero

Haley started a new medication last week.  The new addition brings her pharmaceutical “cocktail” up to a four drug mixture.  Haley has been on over a dozen different medications and combinations of medications since she was two years old.  None of which have controlled her seizures for any length of time.  Some of the prescribed medications made her seizures worse, even though that was not supposed to be a side effect of that particular drug.  For Dravet patients, Lamictal and Dilantin are two drugs that can really make things worse.  Haley has been fortunate in that side effects have been minimal with the drugs that she has taken.  Here is a brief overview of what she has been on and what my small brain can remember about each one.  This is not the exact order of how they were prescribed.  I wish I had kept better records, but during all of this the twins were born prematurely…I consider that time and the months that followed, my “black hole”…

Tegretol- We started on this drug and did not see any results right away (within a week) so we stopped and went onto the next drug.          

Phenobarbital- We had poor seizure control with this one.  Haley was lethargic. We went off of it and then back on it and back off it a second time.  When Haley had the multitude of ER visits in 2005 due to clusters of seizures (status epilepticus), the ER would give her a loading dose of Phenobarbital.  I would bring home a “rag-doll” of a child.  The first 24-hours she was a zombie; the next 24-hours she was stumbling all over the house.  She might recover about and be “normal” about 4 days out.  By the seventh day we were back in the ER with another loading dose.  Her treating neurologist at that time said that “one of the side effects of withdrawal from Phenobarbital is seizures…”  I do not like this drug.

Depakote- Some control.  She stayed on this the longest, but eventually went off it, then on it, then off it, and now she has been on this the longest of all the drugs.

Trileptal- She was in a study for this drug.  She was fortunate to get her first video EEG as part of the study.  Unfortunately she did not have any seizures for the 72-hours she was in the hospital.  However, once we got home she had a severe tonic clonic within the first hour, figures!

Topamax- When Haley was on this drug, she lost some of her language or ability to speak.  It was heart breaking to hear her say, “ma..ma….ma….ma…..(then finally) mom.”  Her regular speech came back within 24-hours of going off the drug.

Zonegran- This was another drug that took away Haley’s speech.

Tranxene- This is a valium-like drug that worked well at first.  Then is started blocking the receptor sites for her “rescue drugs” which landed us in the ER and hospital repeatedly in 2005.  We finally went off of it because of the resistance she had against the “rescue meds”.

Lamictal- In hindsight this was the worst drug!  We were put on it by our first neurologist and as he raised the dosage, her seizures seemed to increase to me.  When I told this doctor my thoughts, he immediately ( and safely) stopped the drug.  Our next neurologist wanted to try it again.  I told him what happened the last time we were on it, but he was not concerned.  As he increased the dosage, we saw the same increase in seizures.  This doctor, however, did not take her off the drug…he said, “that is not a side effect of Lamictal”.  Once we were diagnosed with Dravet syndrome we did learn this is the ONE drug that these kids should stay away from!  If the second prescribing doc would have “thought outside the box” we might have had a diagnosis earlier.

Dilantin- Another drug that is not good with Dravet kids.  We used this as well as a “loading dose” when the Phenobarbital was in question.  Haley was on Dilantin while on the Ketogenic diet.  When she was on the diet she had to take the IV solution orally, YUCK!  I truly think her tastebuds were ruined by that.  It was nasty stuff!

Keppra- She first went on Keppra in conjunction with Lamictal.  Since Lamictal causes seizures with Dravet kids we thought we would revisit it.  The second time she was on it she was mean!  It made her nasty, which was kind of amusing for us.  Haley is so sweet normally, but on keppra we saw a different child.  She liked to tell her physical therapist, “you can leave now”.   She was also very disruptive in the classroom.  Once we went off the drug she was back to her sweet self. 

Klonopin- Haley is on this drug now and it seems to have some positive effects, maybe.  The down side is that the longer you are on it, the more of it you need.  We are waiting for another drug to get FDA approval that is similar without some of the lethargy.

Felbatol- This was the first drug that the neurologist said, “I would like to put Haley on Felbatol/Felbamate.  I would like you to go home and read about it and take about two weeks to think about this drug before you answer”.  Felbatol was pulled off the market in the 1990’s because there was a link to aplastic anemia.  So it was very scary putting her on this one.  She spent a year on it with regular blood tests and no negative side effects.  Haley went off it because we did not think it was doing anything for her.  Then seizures picked back up and we went back on it.  She is still on this drug today.

Lorazepam-  This is another valium-like drug.  Haley spent a short time with this one as her daily medication. The problem with this is that the more you take, the more you need.  We only use it now as a “rescue drug”.  We will administer this orally if she is alert after having a series of seizures.

Diastat- This is our life saver…and a very, very expensive one at that!  This is a rectal syringe of valium (diazepam).  When we first purchased this it cost us an $86 co-pay for two syringes.  That was in 2001.  Now in 2009 it would cost us almost double that with a co-pay.  The manufacturer says “we are saving you a trip to the ER”.  In 2007 and 2008 our use of this drug caused us to hit Haley’s drug cap of $5000 by July and June respectively.  These syringes without insurance are about $175 a piece!  In 2007 we used 10 in one month!  That is no fun!

Banzel- This is our most recent addition to Haley’s drug regimen.  So far we don’t know.  She started it on a Thursday night and her seizures picked up.  We upped her dosage the following Tuesday and so far (this is Friday) we have been seizure free since then (3 days…).  Only time will tell.

One thing Haley has learned from all these medications is a talent for swallowing some big pills, even two or three at a time.  She unfortunately also knows where a tourniquet goes on her arms for all the blood draws.  She doesn’t even flinch now.  She just watches the whole process.  When it is all over, she pronounces, “I am very, very brave….God made me strong”.  Yes, sweetheart, God did make you very strong and very brave!