Life on ACTH therapy and Vigabatrin for Infantile Spasms

Savanna had been on the ACTH for about 8 days when she experienced her last Infantile spasm.  When she did not have a cluster on December 28th, we were beside ourselves and honestly didn’t know what to think.  She was healed, right?  Wrong.  A routine EEG would confirm lack of hypsarrythmia, lack of discontinuity, presence of normal sleep patterns, but also some abnormalities.  Savanna was still experiencing some clinical events typically effecting her eyes and head.  Even though we were just reaching the highest dose of the ACTH, it was decided that its affect on Savanna (the good part anyway) was at or near maximum.  She was started on Sabril (Vigabatrin) January 1, 2012.  That was the second front-line medication intended to treat the Infantile Spasms.

Within a couple of weeks of the start of ACTH, Savanna had lost most milestones she had reached as a result of the seizure activity.  She stopped smiling and laughing.  She was considered low tone clinically.  She slept the majority of the day, only awake about a half hour out of every four hours.  At least half of that time, she was really fussy.  She appeared to be blind, as she did not track anything with her eyes, and never seemed to be ‘looking’ at anything – just blankly starring. We were told her pupils were normal, and the problem with her vision was her brain.  It was a very dark period for us as a family.  Trying to understand why God allows such suffering was a recurring thought in my mind. 

By the middle of January, Savanna was about half way through the ACTH therapy when her health turned for the worse.  We noticed she was having an increasingly difficult time feeding.  She had now lost command of the breathe/swallow muscle control and was aspirating feeds into her lungs.  She had grown enormously in her face and legs.  Her cheeks were the widest part of her face.  Her hair had turned wiry and was growing fast.  She began to grow facial hair.  One day I came home from work and sat down to give her a bottle, which was our normal routine, and she didn’t  take the feeding as she seemed really congested.   She was coughing a lot and seemed almost like she was choking.  I administered a dose of inhaled albuterol, and it seemed to make the problem worse.  I opened her outfit to look at her respiration rate and was shocked to see her belly contracting inward so hard.  (This in the end, was the result of chronic and increasing aspiration of feeds combined with her inability to adequately clear the fluid due to her low tone.)  She was in respiratory distress and we had act quickly.  I hastily packed a bag, and took off for the children’s hospital.   

Once there, it did take some time to penetrate the membrane that surrounds the ER and in-patient facility.  Triage took all of 15 seconds as the nurse listened to her and said she was in real trouble.  I was told I would be taken back very soon, despite the overflowing waiting room.  An excruciating 45 minutes later, the calm before the storm was over.  I walked quietly through the doors to meet doctors, nurses, technicians into what seemed like utter chaos.  More or less dismissed as an important person in her care, I finally met the doctor orchestrating this effort involving at least 20 people, and I could see the genuine concern for her well being in his eyes.  Within a few hours, she was admitted to the children’s hospital where the gravity of the situation became more clear.  Her respiration rate was steady in the high 80’s, and she could not stay saturated without a strong flow of oxygen.  What became the next 18 days, was a very difficult experience as a parent.  Getting a successful IV started was in and of itself a significant event, as what normally takes a couple of people a couple minutes, took teams of PICU and NICI nurses hours.  It was painful to watch.  

In and out of the PICU, the helpless feeling was ever present. The doctors seemed to have few options left as the treatment eventually became a wait and see event, (of course with a plethora of drugs being administered around the clock).  Depending on which doctor you spoke with, the consolidation in her lungs was probably bacterial pneumonia according to some, and viral pneumonia according to others.  She was given 3 different strong antibiotics, a lung drying agent, and stronger blood pressure medication.  She contracted a fungal infection so bad on her rear, that the Infectious Disease team became involved.  We lived at the hospital, and it was a real strain as we each tried to maintain a presence at home and in our workplaces.  Being ‘fun’ for the other kids was very difficult after a night at the hospital and then a day at work.  Rebecca and I barely saw each other for 3 weeks and the strain on the marriage was significant.  More than once I found myself in that dark hospital room late at night with tears in my eyes trying to grasp the magnitude of the overall situation.  I found myself weak in the face of some of the adversity in front of me.  The effects of those 3 weeks permanently changed my perspective on certain aspects of human existence. 

As if it wasn’t difficult enough… I remember getting a call one day near the end of this particular ordeal at about 8 am from my boss.  He was wondering where I was and when I  was coming in to work, as though I had slept through my alarm.  I had just dropped the big kids off at daycare after Rebecca and I made the early morning switch at the hospital and I knew at that moment something had to change in our lives. While I was working a lot overnight remotely from the hospital, it went mostly unseen.  It did not replace my presence in the facility.  I remember that phone call feeling very cold and inconsiderate.  Over time though, I gained an understanding of  the other side of the relationship.  Once this happens to your family, you experience a paradigm shift while those around you do not as normal life does go on for everyone else.  Recognition and acceptance of this fact is critical in order to move forward.  Your perspective changes (along with your circle of friends) over time.  You see things around you that you may not have ever seen before.  You see other families with special needs kids living life at times, and this becomes an area of interest rather than something you ‘look through’ when observing from afar.  This is an evolving process and we are still learning as Savanna is changing.

Savanna finally went home in early February with oxygen tanks, a pulse-ox monitor, a nasal-gastro-intestinal tube installed, a feeding pump, and a significant panel of medications. The day before she went home, I was with her, and witnessed a smile and almost laugh that we had never seen before.  I captured it on my phone and will never forget it.  The feeling was like your first breath after being underwater too long.  It was a glimmer of hope for us. The next few days saw the final ACTH injection and numerous medications discontinued.  While the side effects from the ACTH were present for months afterward, her neurological progress was remarkable. 

Looking back, we initially had to manage a situation that was very task oriented while coming to grips with reality.  We had not yet started any occupational therapy or physical therapy, so the magnitude of her global developmental delay was still somewhat hidden.  We were being prepared emotionally (and didn’t know it), as the trivial tasks such as medicine compounding and delivery would transform into learning how to be Savanna’s best therapist.  At first I found this more difficult, but now find it very rewarding.

Dad
(Ken Lininger)

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2 thoughts on “Life on ACTH therapy and Vigabatrin for Infantile Spasms

  1. Hi Ken,
    Just read your story, it was very touching and heart breaking at the same time. My daughter is going through the exact same thing, and I can remember perfectly the first time she smiled at me (once her acth injections level dropped) . I wish nothing but the best for you and your family.
    Ps- I found this story to feel closer to home, because from the pictures our daughters look VERY Similar.

    Like

    • Hi Amanda,

      Thank you for reading, and thanks for taking the time to comment. The blog started more for me to communicate to family what was happening, but has transformed into a therapy for me and a sounding block for awareness of epilepsy in infants as a result of focal cortical dysplasia.

      I can’t tell you how thankful we are for the God-given intervention in Savanna’s life we commonly refer to as ‘modern medicine’. Just today we have come home from a long day of follow up appointments with the neurosurgeon and neurologist/epileptologist and learned, she is infact ‘seizure free’ with an official impression from a 23hr VEEG on October 7th.

      Has your daughter had surgery too? Is your story somewhere to be read? Feel free to pm me if you want, anytime.

      -ken
      kenlininger@gmail.com

      Like

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