The fear and the hope

(I've been working on this since yesterday (6/16) afternoon, but am only posting it Wednesday morning. You know you're in a pediatric hospital when the computer in your room locks at midnight and tells you to go to bed. So, you know what? I followed that advice, and here I am at 6am trying to finish this)

Many of you get Chris' email/facebook updates, so some of this may not be new to you, but this is my attempt to process the vast amount of information we have received today.

The neurologist came to see Ella around 10 a.m. He told us that the EEG was abnormal and combined with our description of her spells that "cluster", he felt that the likely diagnosis was infantile spasms. As inocuous as that sounds, it's a type of epilepsy that is actually pretty terrible (as you might see if you google it). To quote the doctor, "This is not a good thing to have."

(This is the same condition that Dr. B from NWA who read the original EEG suspected. She told me as much on the phone yesterday morning, and my heart just sank. You see, when Ella first started having seizures three weeks ago, I began to do some internet research about them, trying to find a description of symptoms that matched what we were seeing. I kept coming back to infantile spasms. And, truth be told, I freaked out. Especially about the odds--better than 70%--of an association with mental retardation and long-term, profound developmental delay. Yuck. Yuck. Yuck! Pretty much my worst fear of the probable diagnoses. So I quit searching and tried to not to think too much about the what-ifs.)

Anyway, while we were talking to the neurologist (known to me until further notice as Dr. I because that's my best understanding of what his name is) this morning, he asked about Ella's history of these spells. While he did key in on their clustering nature, he was surprised to learn that she had only had isolated seizures since the phenobarbital had been started. He even saw one of these seizures while he was talking to us.

He continued by saying that we would definitely do an MRI, probably tomorrow or even the day after that. He also said that the treatment for infantile spasms is ACTH, which is a course of injections for up to eight weeks. I asked if we had to be hospitalized for the duration. He said that it would be outpatient after the initial 3-5 days of treatment here in the hospital--monitor Ella for a positive response (reduction/elimination of seizures) and any side effects, teach us how to give the shots, and so on. At that time we were looking at likely being here the rest of the week.

I had tried to be prepared for the diagnosis of infantile spasms and meeting with Dr. I made it that much more of a reality. Especially when he talked about the treatment as though it were definitely going to happen. He finished by saying that he was going to have an epileptologist also look at Ellie's ongoing EEG to confirm the diagnosis. We were very sad for our baby, to say the least.

BUT (and this is a big but)

. . .Many things do not add up for Ella to be diagnosed with infantile spasms. For one, she has responded to the phenobarbital to a certain extent, in the sense that it gave her a couple seizure-free days and reduced her spasms from clusters to isolated events. Usually I.S. (my abbreviation for infantile spasms since I'm tired of typing it) does not respond to phenobarb or many of the other anticonvulsants at all. Also, Ella does not show ANY problem with feeding--namely losing her ability to suck and becoming failure to thrive--which is apparently very common in I.S. babies, so common in fact that Dr. R. the neuro who saw her in the Springdale ER thinks that her continued ability to eat so well means that she does not have I.S.

So, a little later in the day (after the IV team started an IV for Ella on the first try--can I get a woot, woot!), the nurse came in with a new anti-seizure medicine to start. I didn't know why we were starting something else but found out it was going to be in addition to the phenobarb (not replacing it). Soon after the IV infusion of keppra was done, Dr. I came back in to say that the keppra was a trial to see if Ellie responds to it; if she does this is a very good thing and likely means that I.S. is not her problem. He also told us that the other neurologist (the epileptologist perhaps?) agreed with him that she didn't have all the classic signs of a baby with I.S. Up until that point, we didn't know he wasn't sure of the diagnosis.

And you want to know where my hope is this morning (6/17), several hours since the first keppra infusion? The seizure that Ella had when the medicine had just started infusing was the last one she's had in several hours!

I asked the resident yesterday evening when we might know if the keppra was working (and therefore likely ruling out I.S.), and she did not have a definitive answer but she thinks 24 hours is a good start.

So, that's our hope, our prayer. That Ella would (continue to) be seizure-free on the keppra for at least 24 hours. We are at least 15 hours and counting so far.

At noon today (6/17), Ellie goes for an MRI under sedation. While still "under," she will have a lumbar puncture which along with some blood work will test for some specific metabolic disorders.

Oh, and right before she goes for the MRI, she will finally get to come off of the EEG that has been running from the moment we got here Monday night. When I found out yesterday (6/16) that E would continue to be monitored for another night, with video and audio and everything, I gave up my modesty and opted to breastfeed her like normal (or as normal as possible considering all the things currently attached to her).

After the MRI, I am unsure of what will happen next. I assume it depends on what if anything is found on the MRI and whether the keppra continues to control her seizures.

I will try to update when we know more.

♦