Getting Closer

Ella continues to have seizures and they are getting stronger instead of weaker, more frequent, and are starting to cluster again. She is back on an IV. The MRI showed polymicrogyria in the right frontal lobe of her brain, which is a malformation or disorganization of the neurons that can be just coincidental or a cause for seizures. In Ella's case, the neurologist currently believes this is the likely cause of her seizures as no other factors have been discovered. But, we are still awaiting the results of all the blood and lab work from yesterday. The cause only partially determines the course of action to be taken. We still don't have a firm diagnosis, which means we don't have a firm treatment plan either. With seizures, the type is usually determined by which medicine is effective in controlling the seizures instead of the type being determined and then, prescribing a medication. Kids with polymicrogyria either have infantile spasms or other seizure disorders. So, it makes sense that she is showing signs of both infantile spasms and other disorders. The amount of developmental delay and disability associated with polymicrogyria is across a broad spectrum. In some, delay is so mild that polymicrogyria is not discovered until the child is much older and starts having seizures. In others, it's totally devastating from the start. There is no predictor of severity of delay. The neurologist believes her deficiencies aren't the most severe since she is responding to us, but there's no way to know how severe they are at this point. She's not the worst she could be, but not necessarily the best either. Since she initially responded to the Keppra, her dose is being increased to the maximum allowed. If that fails, we will start the ACTH. Even the doctor wants to avoid this if at all possible. Her prognosis is best if the seizures are stopped within the first thirty days of symptoms to avoid further damage. We now understand that it's not the number of seizures that do damage, but how long before the seizures are controlled that is the determining factor. The initial damage is done. We don't want it to be prolonged. We know she was having seizures as of May 25, but don't know if it started prior to that. Assuming May 25 was the start date, we still have enough time to take a final attempt with Keppra and if it fails, start ACTH before the optimal window of time closes. We'll know within the first 24 hrs. if Keppra was successful. If Ella requires ACTH, we will be here into next week. If not, we'll be home this weekend. We want to go home. In the future, her further treatment will likely require us to come back every so often. We are hoping that this isn't the case and that her treatment can continue at the Children's Hospital Clinic in Lowell, just up the road from our house. Please pray that the test results would give us a definitive diagnosis of something other than infantile spasms with the corresponding developmental delay and disability so that we can have a definitive treatment plan and avoid ACTH and the undesirable side effects that come with it. Pray that she would have no developmental delays from polymicrogyria and would continue to respond to us, laugh, coo, and smile, developing as a baby of her age should. Her laughing and smiling is a big answered prayer for us as it is the thing we missed the most. It lifts our hearts and spirits. Please pray that she would respond favorably to the Keppra and that her seizures would stop immediately and permanently so that damage is not prolonged, we can go home, and her diagnosis points to something other than infantile spasms with the accompanying developmental delay and disability. Please pray that we can continue her treatment in NWA instead of Little Rock. Please pray that we get approved for ARkids. Please pray that Ella avoids any extraneous illness while she is here as she is battling congestion and a cough that is moving toward her lungs and a diarrhea bug. Cooper is sick and is on antibiotics and breathing treatments. Pray that he gets well and doesn't get Jackson sick. Pray for our families as they care for our kids while we're gone. We're just waiting to see what happens next.

The Roller Coaster Continues

The morning was spent prepping for a day of tests. Ella had blood drawn at 10AM, then the EEG and IV removed, the MRI at noon, then the spinal tap. She has spent the rest of the day recovering. We are awaiting the results of all, but expect them tomorrow morning. Ella started smiling and laughing at us this afternoon, something we hadn't seen in more than 3 weeks. She had been seizure-free since starting the Keppra until 5:30pm today. Unfortunately, this has been her pattern, a medication change, 48 hrs. without seizures, then back to where we started. The roller coaster continues. She has had 2 more since 5:30pm. They've called the doctor and we are in a holding pattern. If the seizures cluster, increase in frequency or intensity, they'll consult with the neurologist again as to the appropriate course of action. There was talk of us going home tomorrow, but now that's up in the air. Yesterday, the neurologist had said if Keppra didn't work, ACTH was the only other option, which means more time in the hospital for monitoring, training, and more side effects. Thanks for all the phone calls, emails, messages,etc. A special shout out to my peeps at Arvest for the nice azalea. Thank you, Stefanie :) The receptionist was overrun by pollenating insects. I apologize for the run-on paragraph. The Tab and Enter button on this keyboard don't work.We are physically and emotionally drained, but we are hanging in.

A Busy Day Tomorrow

Ella was hooked up to the EEG and had a few seizures that have been documented as we are under constant audio and video surveillance. She will be monitored until the MRI tomorrow. We saw the neurologist this morning. Ella had a seizure while he was here. He thinks the EEG further points toward a diagnosis of infantile spasms, but the other neurologist thinks it could be something else as she is not a classic case of infantile spasms, but has half the symptoms of infantile spasms and half of something else, which they haven't named. Ella is to have an MRI tomorrow and will be on the EEG until then. We're told that we'll be here at least this week if not the beginning of next. Ella's prognosis is better if the MRI shows nothing. That leaves the possibility, however slight, that Ella could eventually not have seizures. She is also scheduled for more bloodwork and a spinal tap tomorrow. She has been put on Keppra to see if that will stop the seizures since this is one of the main goals. A reduction is seizures doesn't really do much good. It's all or nothing. We'll know after 24 hrs. if Ella is responding to the Keppra. If so, this points to something other than infantile spasms. If Ella does have infantile spasms, she'll be put on a course of ACTH, which has a host of undesireable side effects, for up to 8 weeks depending on how well she responds. The quicker she responds, the better the prognosis. Please pray that the MRI comes back clear, that she responds well to Keppra and has no more seizures, and that the spinal tap goes well. We'll also meet with the financial aid dept. to determine whether Ella qualifies for ARKids or TEFRA insurance. ARKids is the better option. So, we hope she qualifies.
It's a slow, methodical process of elimination. We're just waiting to see what tomorrow brings.

We've arrived

We are here at Arkansas Children's Hospital. Ella is hooked up to the EEG and has already had a few seizures that have been documented as we are under constant audio and video surveillance. She will be monitored through tomorrow until we see the doctor. We'll then know if Ella is to have an MRI, but we suspect so. It's one step at a time. We hope that we are only here a few days before we are sent home with a diagnosis and an action plan. Please keep us and our families in your prayers, especially Jackson and Cooper.

Headed to Children's

The dr. called regarding Ella's EEG. It was "abnormal" and "worrisome." She thinks it may be infantile spasm. Not good. We are out the door to AR Children's hospital to get Ella admitted for an overnight EEG, MRI, bloodwork, and other tests. This is all we know and will update once we know more. Please pray.

Back to the Lab and Then the EEG

Ella has been having seizures more frequently and they are becoming more clustered together. So, we went back to the lab to have a phenobarbital level drawn again yesterday. We'll see if her medication is increased. We are still waiting to hear the results from the doctor.

Today, Megan had to keep Ella up all day in preparation for the EEG. It was a tough task, but she was successful in her endeavor. We had to keep her awake for the first portion of the EEG. She slept for the middle portion. Then, we had to wake her up again for the final portion.

To start, they measured her brain activity while they flashed a strobe light with different patterns in her eyes. We think she might've had a seizure just after the lights, but couldn't tell because it was so dark in the room and she was swaddled very tightly so as to restrict her movement. We were told it wasn't necessary for her to have a seizure while being monitored as the doctor would be able to tell regardless. Then, they measured activity during sleep, then wakefulness. She was so tired that she wouldn't wake up with me clapping as hard as I could in her ear. We used noisemakers and were jostling her to no avail. She finally woke up when we were about to put a wet rag on her face. Megan took pictures of Ella hooked up to all the electrodes, which I'm sure will be posted soon.

Megan writes:


"The EEG went well. We don't have results yet, but we have a little bit better understanding about what they might learn from it based on what may or may not have been in the tracing.

Praise: We happened to run into a social worker as we were checking out who hooked us up with an application for supplemental medicaid for chronic illnesses (called TEFRA). She was incredibly helpful and friendly. She advised us to wait until we are billed to pay anything on the test right now, whereas we were originally told to expect to pay for it in full today (over $600!). The application for TEFRA is a long process and the sooner you get started the better, so it was great to have this information essentially at the beginning of Ella's illness. And the coverage backdates three months from when you put in the paperwork, so if Ella is approved (please pray that she will be :), it would even cover her recent hospitalization, as we understand it. That's huge!

Thank you for your prayers."


Now, we are waiting to see Dr. Balmakund on Thursday to go over the results of the EEG.

Holding Pattern

Below is the latest from Megan:

We don't have any major news on Ella, still waiting for what we hope will be a very productive and information-gleaning neurologist appointment in eight days. We go for the EEG on Friday, but I don't expect that they will be able to tell us anything right then; we will instead get the results from the neuro. After the pediatrician upped Ella's medicine on Thursday and a short adjustment period, E went almost 48 hours without seizures. They've started to breakthrough again in the last few days, about four to six times a day right now.

I wrote the following on my facebook page yesterday:

"I wanted to update, but I don't want to sound despondent or hopeless. I'm actually in a pretty good place right now.The seizures that she's had since starting the medicine are not as bad as before we went to the hospital, in my opinion. They used to come in clusters and now they are rare and isolated events. This is incredibly encouraging to me.I think we can manage with just a few breakthrough seizures if that's the best we can get with the medicine, but if we can find a dose, a different med, or a combo of meds that gives her seizure-free days for good, that of course is the goal.(She's at the maximum recommended dose (mg/kg/day), but her blood level was low enough last week, that maybe there's room to increase it yet?)I told Chris that I think (and hope) that this waiting period before our first appointment with the neurologist will be the most uncertainty we will experience."

Chris asked if there were any specific prayer requests that we could share regarding the EEG. First off, they want her to be sleep-deprived. (How exactly do you do that? Isn't it that they only sleep when you don't want them to? :) Please pray that I would know how to manage her day so she will stay awake and meet this requirement. For the test itself, pray that they would get a good reading and get it as quickly as possible. I think she may have to be strapped down during what I understand is--best-case scenario--a twenty-minute evaluation, so it is important to me that this be swift. Pray that she will hold still and not be too upset. And finally, please join us in praying that it is a normal EEG. True, if there is nothing to be found on the tracing, we may not get a diagnosis right now, but a normal result may be able to help the doctors rule out some of the more serious types of seizure disorders, which is our hope.

Thank you for checking in on us.

Grieving the loss of the Perfect Child

I'm reposting Megan's latest blog entry below. It's a good assessment of what's happening with us. Today is the first day since this all began that we haven't noticed any seizures. But, I can say that I haven't been watching very closely as constant vigilance is exhausting. We've hit the maximum dose of medicine allowed for Ella. So, we hope that it does the trick....

In the spring of 2004, I spent six weeks in maternal and newborn nursing. I did not have children of my own at the time and would not be pregnant with Jackson until that summer. In clinicals (hands-on experience in the hospital), I got to witness the miracle of birth and hold itty bitty babies in their early hours of life. It was an exciting, baby-fever-inducing season.

In addition to our hospital hours, we also spent time in the classroom. One lecture stands out to me above all the others, even five years later. During one tearful morning, our instructor guided us through a discussion about providing care and support to families in perinatal loss and the birth of special needs children. We talked about the grieving process in miscarriage and stillbirth, but then she said something that floored me; it was something along the lines of: "Additionally, when a child is born alive but with birth defects or chronic health problems, the family often needs to grieve the loss of the Perfect Child."

Whoa.

Being healthy myself, I assumed, as many women do, that I would have uneventful pregnancies and healthy babies. It never occurred to me to think otherwise. Intellectually, I knew that these things happen and that no one is immune, but no sense in being all doom and gloom, right? And besides, pregnant women have a tendency to be a bit on the paranoid side anyway, so why try to prepare oneself for such eventualities that statistically do not happen all too often (thankfully).

Even when--during my very first shift as an RN, while pregnant with Jackson--three out of my four pediatric patients had significant birth defects, I did not linger too long on the what-would-I-do-if's.

And fortunately, all three of my babies were born healthy. I counted fingers and toes, watched them take breath after breath, and felt peace in knowing that they were okay. Therefore I thought (assumed is a better word) that we were out of the woods on this one.

Fast forward to now. Where I'm trying to wrap my brain around this new reality we find ourselves in. The one where our baby girl has seizures and needs medicines and all manner of tests and such. In digging into why I have been tearful the last few days about all this, I am realizing that it is because I am indeed in this process of grieving (the loss of) the perfect child.

Now, please don't misunderstand me. I am not saying this perfect-child-ness is lost forever. I do not know what the future holds for Ella. I have no idea if this is a disorder that she will grow out of with no lasting effects or on the opposite side of the spectrum if she will be profoundly handicapped by it. I DO know that the latter is certainly possible. And that makes me sad.

Regardless of the outcome, however, I know that God has not forsaken my daughter. I know that I love her as much as I ever did, and that He loves her more than I ever could. By His grace, we will walk this road for as long as it is necessary, and we will continue to praise Him in the journey.

Appointment with Dr. Simmons

We met with Dr. Simmons, our pediatrician. He increased Ella's medication and prescribed another to give in an emergency if she has any more big seizures. If she doesn't improve, we'll go back to Dr. Simmons to increase the dose. We will keep a seizure journal. Ella will get another blood level drawn the day before her Friday, June 12, EEG. Now, we wait.

Off to the lab

Megan just heard from the doctor's office. We will go to Washington Regional to have a phenobarbital level drawn in the lab. No other changes to the plan unless Ella has worsening symptoms between now and her (previously scheduled) follow-up appointment tomorrow afternoon.


Thanks for weathering this storm with us. You guys are awesome!

The latest and thoughts from Megan

Ella had more seizures this morning that were like her first ones, but stronger than those from last night. We are waiting to hear back from the doctor. Below are Megan's thoughts on the situation:

If I might share my nurse-y thoughts for a moment:


My theory is that the IV medicine that Ella got in the hospital is wearing off (it takes a long time with this one). The reason I think this is because the two periods of time where she's had seizures so far were just before her next dose of twice daily oral medicine was due. So, if that's what is going on, I'm guessing the next issue becomes: "Can her dose of phenobarbital be increased without risk of toxicity (undesirable side effects)? Or does she need a second medicine to try to keep the seizures at bay?"

If she is on medicine for any length of time, which does seem to be necessary *sigh*, they will routinely draw blood levels on her (I'm not sure how often) to make sure that the medicine is not reaching toxic levels or conversely that it is not too low to be considered therapeutic (effective). Since she is a baby and is growing exponentially right now, I expect that her dose will continue to be tweaked up to meet that growth.


I thought I would be overcome with fear if Ella continued to have seizures, but that has not been the case. Thank you for praying for me in this; I have no doubt that it is a supernatural peace that I feel. Thank you for loving on our daughter and loving on us.

Here we go again

Ella has had more seizures. Megan is home and confirmed my observation. The plan remains unchanged.

Update

To catch up, you can read the updates by friending me or Megan on Facebook or by checking here and Megan's blog, http://sermonsinstones.blogspot.com. You can also find Megan on Twitter: @ms_megan

Ella had 2 little seizures at 9pm. They weren't as intense as those at the end of this week, but more like they were at the beginning of the week as shown in the video I posted. They were short and less pronounced. But, I knew what I was witnessing this time around. We will contact the doctor in the morning. It may be that her medication needs to be increased a little.

Megan's Prayer Requests

Could you join us in praying that God will be glorified in this, that we will lean not on our own understanding or strength but fully on God, that Ella will remain free from seizures, that we will continue to have unity in our marriage, that the accuser will have no foothold to try to point blame on us or stir up guilt, that we will have answers to our (many) questions, and that our boys will be insulated from any fears/anxiety they may sense in us?

Also, I am trying to work extra hours this week to make up for the hours I missed while we were in the hospital this weekend and perhaps will continue to work extra in the weeks to follow as we anticipate the medical costs we've incurred/will soon incur; please pray for grace for the boys as I am out of the house a little more, especially Cooper as he is really struggling with separation anxiety right now anyway; and pray for favor for Chris as he is the caregiver when I am gone.

Finally, I am struggling through some of the strong emotions I've felt since this journey began last week, emotions that I shoved down inside while in "survival mode" in the hospital. I don't think I've ever been more scared or felt more helpless than I did as I observed Ellie's major seizure on Friday morning. It continues to affect me, and I pray that God would help me release it soon (though I'm admittedly afraid that if I try to forget that I'm somehow not being a good mother to Ella--does that make sense? I'm also afraid to try to close that off only to have it ripped back open if she has another seizure.) I would like to deal with these emotions in a genuine way while leaving room/pacing myself for the possibility that we are not "through the woods" just yet. I believe that through Christ alone, and with His perfect provision of just enough manna for one day at a time, we will be sustained, no matter the outcome.