ACTH

I do a pretty good job, I think, at staying positive even when things get really hard.  Some of it may be keeping up appearances.  I don’t want to worry my family.  I want to protect my family any way I can so sometimes I don’t make a big deal out of something that maybe is a big deal.  I don’t want them to see me sad, or stressed, or worried.  I don’t want them to see me cry.  I let my tears fall under the disguise of the shower.  It’s usually the only time that I’m alone. Okay, maybe not completely alone.  I often poke my head out in response to the demands that I see the newest picture that Emily drew or the block robot that Justin built.  My sanctuary is not without its interruptions, but it’s a good place for me to release some stress and shed a few tears.  The shower drowns out some of the sounds of the children who are sword fighting in the hallway and I can hear my thoughts.  I can hear my prayers. I prayed the other day that Danielle would smile at me.  She hasn’t been her smiley social self for a while now, not since before her heart surgery.  It’s hard to know if it’s the Infantile Spasms that is taking my baby away from me or the medication that is masking her sweet character.  I prayed for God to heal her.  I prayed for a sign that everything would be okay.  I prayed that my baby would smile at me that day.  God answered my prayer.  She smiled at me later that day, and she’s been smiling at me every day since.  I really needed that reminder that God answers prayers.  Everything we’ve been through lately is enough to completely drain me physically and emotionally, but God never fails to restore me. 

Some of you who follow me on Facebook might have seen that I recently posted that Danielle’s latest EEG looked “perfect.”  Apparently when the neurologist said “perfect,” what she really meant was “very much improved.”  She had to explain to me later that day that while Danielle’s brain waves are much more organized now, she still has epileptic brain activity.  The prednisone that we added to the Topamax to make a “good cocktail” wasn’t effective in gaining complete control of her Infantile Spasms.  Danielle was admitted to the hospital to start ACTH (adrenocorticotropic hormone) treatment on Tuesday.  This is an aggressive treatment.  Although Infantile Spasms is tricky to treat because there is not one treatment option that works for everyone, ACTH is this is typically the first line of defense; the standard of care.  We didn’t have ACTH as an option until recently, after her cardiac team cleared her for the use of this heavy steroid, but at least we were able to rule out some things that didn’t work.  ACTH is a six week treatment; two weeks on a moderately high dose, followed by 4 weeks of weaning down.  Some of the side effects of the drug include: high blood pressure, irritability, high blood sugar, risk of infection due to suppressed immune system, water retention resulting in painfully bloated baby… the list goes on.  We’re treating her at home now, and there is a nurse that will come to our home twice a week to monitor her blood pressure.  I give her ACTH injections twice a day and monitor the ketones in her urine daily to make sure her blood sugar isn’t too high.  The promise that ACTH is effective has me hopeful, but very nervous.  The drug typically stops the spasms within a week.  3-5 days is most typical, but we need to give it a full two weeks before we know whether or not ACTH will be Danielle’s miracle drug.  Danielle is still having spasms today (treatment day 5).  Please keep praying for her.  Please pray that ACTH works.

EEG Monday night before starting ACTH.

Special handling.  One vial of ACTH.  I was annoyed that I had to jump through hoops to get this medicine, but looking back at the package set in the seat where my baby should be was very emotional for me.  This medicine is unbeleivably expensive and I am SO very thankful that it was made available for our baby girl.  There are mothers and fathers in other parts of the world that would sacrifice everything they have to get this drug for their child.