"So, you're done, right?"

It's a question I've been hearing for a while now, even before our girl joined our family in fact. I never know what to do with such a query. I mean, I know what the asker WANTS me to say. You can't be the daughter of a Perry Mason fan and not want to shout, "Objection, your honor, leading question!" But being a people pleaser all my life and generally wanting to avoid the Weirdo label makes me play nice, and instead I say, "oh yeah, we're SOO done! Three kids in less than four years is quite enough for me." Chuckle, chuckle, chuckle. And the person nods in agreement as if the proper number of children were a constant like Avogadro's and goes on about his or her day (it's almost always a "her," by the way).

It's not really that I'm being dishonest, per se. In many ways, I really do FEEL done, if such a thing can be felt. Like when I chase my middle child in the parking lot yet again, heart pounding, baby strapped to my chest, oldest left to his own devices, hoping none of them will be harmed, and wondering why children his age are so good and intent on weaseling out of momma's handhold, I certainly feel like I have enough children to manage. Or when I'm up a second or third or even a fourth time in the night holding babies or quieting bad dreamers, I can't imagine adding to the number of sleep deprivers I already have. And certainly, the finances can argue their own case of being stretched to the limit by our current numbers.

The truth of the matter, though, is that it is not up to me. That is not code for "I want more but I know better than to say it outright because people will judge me" either. I promise. It's just that I know better than to think that I am in control of this part of our life (or any other part for that matter).

I know Who is in charge here, and I know how blessed we are. We wanted kids, and we had kids. It was as simple as that for us. But I've seen more than enough who are trying to conceive or grieving the loss of a child, to the point that I realize this has very little to do with mere desire or fervor of prayers or potential parenting ability or any other human thing. God works in mysterious ways, no more apparent than in how he builds families. It does not always makes sense in the valleys of deferred hope and grief, but He is not done writing our stories yet, and, knowing the Author, I have a feeling they will be masterpieces.

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My FDA speech

"Good morning, and thank you for having us. I, along with my husband, stand before you today to share the still unfolding story of our daughter Ella's journey with infantile spasms.

In May of 2009, while not yet 5 months old, Ella began to have odd staring spells. Up to this point, she was on track developmentally, but, being a nurse, I feared right away that something very serious was wrong with our baby. Within the week, her spasms became more pronounced and began to cluster in short succession several times a day. By mid June, after an EEG that was determined to be "very abnormal and worrisome" by our neurologist, Ella was admitted to Children's Hospital for treatment of what was presumed to be infantile spasms.

We had already researched the possible causes of what we were seeing and were familiar with the term. And its devastating prognosis. I will never forget one of the first things I read about this catastrophic seizure disorder: that the incidence of mental retardation associated with it is estimated at 75 to 90 percent. There were also grim statistics about shortened life expectancy that I quickly banished from my mind.

After a few days of EEG monitoring and observation, Ella was officially diagnosed with infantile spasms. Quickly, the neurology team decided that ACTH would be the best course of action for our baby; we weighed the risks and agreed, and she started treatment the very next morning.

The case manager immediately began communicating with our insurance company to cover the prohibitive cost of the medication, knowing it would be a hardship on our family.

Because Acthar is not FDA-approved for use in infantile spasms, our insurance company denied coverage, calling it "experimental." An appeal was also denied for the same reason.

But the National Organization of Rare Disorders, along with the Acthar Support and Access Program, had already come on board to help us get this medication our daughter so desperately needed. Through their combined efforts, even though our insurance company never approved our claim, we were given Ella's entire six-week-course of treatment free of charge and without delay. We will be forever grateful for their assistance.

On her eighth day of treatment with ACTH, and exactly a month after her "staring spells" began, Ella had her last spasm, well over 10 months ago. 

Now 15 months old, our daughter is a happy, beautiful, active, loving little girl who charms everyone she meets. She attends a full-time early intervention program that provides her with daily physical and speech therapies, and, though she is delayed, we have great hope that she will continue to progress and will some day reach her optimal development.

We take nothing for granted, knowing that hers could have easily been a very different story. We believe that ACTH gave our daughter back to us. We hope that you would strongly consider approving Acthar for use in the treatment of infantile spasms, so that many more children like Ella would have access to this life-giving medication."

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A few images from our trip to tide you over until I have time to actually write about it

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Procrastinating

Wow. 10 days. Pretty sure I haven't gone that long without blogging in a long time. And I didn't really notice it'd been a while until yesterday. When I stopped to try to remind myself what day it is.

See, I've been working a lot. Partly to make up for the lost income from Chris, and partly to cover the time we will be out of town this week and/or entertaining (read: trying not to overwhelm) family coming to town. Nights mostly. And not sleeping enough in between. So lot's of things have been pushed to the side for now, including being on the computer.

But then I realized that I have to actually write a speech if I want to come across at least slightly coherently when I testify before the FDA on Thursday, so onto the computer I logged. But not really knowing where to start, I started reading a few blogs, and then remembered how neglected my own blog had become, and, voila, here I am.

It occurs to me that I may not have mentioned this little detail about the FDA on the blog before now, since it was only finalized a couple of weeks ago, so let me backtrack.

Ella, as many of you know, was treated with a medication called ACTH last summer that was effective in eradicating her infantile spasms (so far anyway, 10 months and counting!). It was costly and not covered by our insurance (through the generous gifts of an amazing organization called NORD and the drug manufacturer itself, we were able to get her six-week course of treatment free of charge). The main reason our insurance company cited in denying the claim was that the Acthar was not FDA approved for use in infantile spasms and was therefore considered "experimental" (though it has been used to treat IS "off label" for more than forty years). So when we found out (through our ongoing involvement in the IS awareness campaign) that the drug company was petitioning the FDA for approval for Acthar in the treatment of IS and was looking for parents to testify about their children's experience with ACTH, we were immediately interested; it is our hope that with FDA approval, the medication will be more rapidly and readily available to the children who need it in the future. We formally petitioned the FDA to speak at the hearing, and our request was granted a little while later. After much planning and not enough speech-writing, the day is almost upon us.

In addition to not knowing what I will say before the FDA yet, I am feeling a bit unprepared in other ways. I need to pack for myself, pack for my children who will spend the night at the grandparents--including all that they will need at school, make sure I have properly identified my parents as authorized to pick up my darlings at their respective schools, not to mention trying to rendezvous with a few other parents of IS children while in DC.

I haven't a thing to wear to the hearing either. Sigh.

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