Thursday, January 15, 2009

January 15th, 2009 AKA ACTH Therapy

We didn't see an end to her seizures on the Nitrazepam, we saw a what seems like a reduction with the combination Nitraz/Vigabatrin but I can't say she's been seizure free.
It looks like ACTH is the next thing on the agenda.  Janna will be admitted to BCCH next week.  We're waiting on a phone call from Neurology to find out when we're to head in.
Because of the risk of some serious side effects I told them I'd rather be admitted with her so she can be monitored closely.  This will be a "low dose" ACTH course and if she tolerates it after the first few doses we'll be released and continue with it to see if we see a break in seizure activity.   If that doesn't work we'll go to regular dose ACTH and if that doesn't work, on to the next thing although I'm not sure what that will be.
On a positive note, she has gone back to lifting her head and she's much less floppy now than she was with the pheno/nitraz/vig combo so we're seeing more movement out of her.
I have this love/hate relationship with her NG tube.  She coughs and gags on it quite a bit and I know it makes her uncomfortable but it's the only way to get nutrition into her at the moment.  She has gained weight, she's now at 12lbs 8oz and on track for weight gain.  Our G-tube surgery consult is on Feb 3rd and I still wince when I think of her with a G-tube and worry about how well she'll be able to tolerate it, after surgery pain etc.  I know she needs it right now so I'll get over my own issues with it and move on.
I do find I'm giving her more belly tickles and kisses now that I know there is a countdown to the surgery.  I know I'm going to miss that beautiful little tubeless belly.
And onward we trod.  Waiting for that call back from Neuro.

Monday, January 12, 2009

January 12th, 2009. AKA Vigabatrin

There's alot to tell.

At our 3 month EEG Janna was diagnosed with Infantile Spasms and we started on Vigabatrin.  I was not in the mood to update the blog, I wasn't in the mood to do much else but bury my head in a pillow but hey - no time for that really when you're caring for an infant with a serious chronic condition.

The medication seemed to be doing it's job, we were getting Janna back.  Still no real smiles and she was very floppy but we were seeing her more focused which was a bit of a relief.  But a few weeks later I noticed some strange, very subtle rhythmic movements she was making with her arms and legs.  I called Neuro, we had an emergency EEG and It turned out Vigabatrin wasn't our wonder drug.  Janna was still having a large number of seizures.

We were admitted to BCCH and a new drug was added to the regimine; Nitrazepam.  The idea was to completely wean Phenobarb (which she's been on in low dose since NICU) and begin a wean of Vigabatrin and introduce nitrazepam. 

At first she had a cocktail of all three.  This basically turned her into a drooling rag doll.  Another side effect of the Nitraz is incoordination of suck and swallow so it became impossible to bottle feed her.  She was incredibly floppy and sleepy but we were seeing more focus from her when she was awake and we were not seeing as many seizure clusters from her.  Once the levels for Pheno and Vig came down, she was fitted with an NG feeding tube I was shown how to manage it and we procured the necessary equipment for interal feedings; we were released with a plan for weaning two of her anti-convulsants and continuing on with the Nitrazepam.

The wean started well enough.  We even began some vision therapy. (We'd discovered during our Ophamology referral in-hospital that she has a Cortical Vision Impairment) but when we stepped down to only 125mg of Vigabatrin per day, on the third day - we began seeing an increase in seizure clusters and the duration of each was longer than we'd seen since we'd started the Vigabatrin.  I placed an emergency call to the resident Neuro on call at BCCH, chatted with him and it was decided that we'd step back in our Vig wean to 250mg per day and keep the Nitraz steady until our follow up EEG scheduled that week.
So fast forward to that EEG - we found she was still having seizures and many of them were very subtle with almost no outward signs.  In total neuro counted 30 in a 30 minute EEG time span.  That was last week.
Our next plan of attack is to up the nitrazepam and keep the Vig at the same dose.  We were to try this for a week and follow-up report to neuro.  If we are still seeing outward signs of seizure, we'll arrange to be admitted to BCCH again and start low-dose ACTH.

One positive thing that came out of last week is that we were introduced to Canuck Place.  Janna is now officially a "Canuck kid" and we'll have access to that resource when we need it.
As of today, I'm starting to feel a bit cautiously optimistic that the Nitraz and Vigabatrin might just be helping.  She's regaining some head control and I counted less seizures today. 

Hopefully this trend continues.