What I Did This Summer

Sadie working on an art project
during school.

It's been a while since my last blog post - and a lot has happened in that short time. At the end of the last post, Sadie was being treated for pneumonia in the PICU at Lurie Children's Hospital. She was able to come home in mid-May after about a week in the hospital. She went back to school a few days later. Her regular school year ended in late May, and Sadie started her ESY program (or Extended School Year) in early June. ESY is essentially the same thing as school - same hours, days, etc., but it runs only through the end of June.


In early July, we took a short family vacation to a lovely beach house on the east side of Lake Michigan. The weather was great - "Sadie weather," as we call it - mid-70's with a nice breeze. Because Sadie has a hard time regulating her temperature, we try not to take her outside if it is too warm.  Sadie got to enjoy the beach for the first time and put her little toes in the sand. She also enjoyed laying on her towel in the shade - a regular bathing beauty!

The following week, we were preparing Sadie for surgery. For some time now, we had been speaking to her neurosurgeon and epilepsy doctor about placing a VNS (Vagus Nerve Stimulator). I mentioned the device and how it works in a previous blog post. Essentially, it helps to control erratic brain waves through a series of pulses to the vagus nerve. The device also comes with a magnet that can be "swiped" at the onset of a seizure - and has the potential to stop or decrease the severity of a seizure.



Staying cool at the beach.

However, in typical Sadie fashion, a curveball was thrown at the last minute - and the VNS surgery was changed to a shunt revision procedure. You see, in addition to speaking to the doctors about the VNS, they had also been monitoring her shunt function. Despite multiple shunt taps and MRIs, Sadie's team of doctors wasn't convinced that her shunt was working properly. The MRI continued to show enlarged ventricles - which is an indication of excess fluid in the brain. So, two days prior to her scheduled VNS surgery, the procedure was changed and Sadie underwent a shunt revision.

During a shunt revision, the doctor checks all the components of the shunt - the catheters into the brain and down into the belly, as well as the valve function. The doctor explained that he knew there was an issue as soon as they opened up the incision, as fluid was pooling around the shunt. This is an indication that the valve was not working the way it should. The shunt valve - which was a fixed valve - was replaced with a programmable valve. With this new valve, the rate of flow can be adjusted if necessary.

The procedure itself took about 2 hours, and Sadie did great in recovery and was out of the hospital the next day. An MRI is scheduled for this week to see if this new valve is set to the correct setting. But, we are hopeful that this procedure corrected the issue with her shunt once and for all!

We are anxious to proceed with the VNS surgery. Sadie's seizures are still quite difficult to control. She has now tried 15 different treatments - a variety of medications, ketogenic diet, surgery - and she is still having seizures daily - sometimes more than 20 per day. We are hopeful that the VNS will help control at least some of these seizures. Another Aicardi mom conducted an informal poll of parents, and of the 20 parents who responded to her survey - 50% saw a reduction in seizures by half or greater. Pretty good odds, in my opinion.

How a VNS works

While we wait to schedule her VNS surgery, Sadie will again be enrolled in RIC's (Rehabilitation Institute of Chicago) Day Rehab program where she will receive intensive therapy until school starts back up. Three days a week, two hours a day. I heard her give an exasperated sigh as the doctor and I discussed this therapy schedule. However, she progressed so well when she did this therapy post-brain surgery last Spring, I can only hope that it will help her just as much this time around.

Hope. It's something I've been thinking a lot about lately. As I mentioned, Sadie's seizures are still terrible. I'd be lying if I said there aren't many days when I feel hopeless - as if there is nothing else we can do for her. I do understand that there is the possibility that VNS therapy will not work for her. But, all we can do is HOPE.

It's this hope that keeps me from staring at the bottom of a bowl of brownie sundae daily. On the contrary, we've been doing our best to stay healthy - (trying to) eat right, getting to the gym, etc. - attempting to stay healthy for HER. But that's a topic for another blog post. For now, we are hanging by our fingernails to those strands of hope.

One Year Ago. Hindsight.

One year ago, we were preparing for what would be the biggest procedure of Sadie's life. On February 23rd, 2012, she had part of the right side of her brain removed. Similar to a hemispherectomy, the surgery removed her right frontal and parietal lobes of her brain. The hope was that by removing these areas of seizure focus, she would experience fewer seizures and have a better quality of life. The full surgery story can be found here.

It feels a little surreal to be approaching the one-year anniversary of her surgery. In some respects, it feels like it happened so long ago. It's been a rough year for Miss Sadie - a long recovery in Cleveland followed by two subsequent brain surgeries - a cyst fenestration in August to alleviate hydrocephalus, and a shunt placement in December when the hydrocephalus returned.

We knew going into the surgery last year that there could be a range of outcomes - anywhere from significant reduction in her seizures to no reduction. While we did see some initial reduction in the months following the surgery, the seizures seem to have found a new focus area on the other side of her brain.

Is this disheartening, yes. Does it make us question our decision? Of course. But we know that these are the unfortunate decisions that we as parents of a medically fragile child have to make in order to improve her quality of life. They are decisions that we wish we never had to make. We simply have to move forward and continue to do everything we can to make sure she has the best life possible.

On that note, a bit of good news is that her pee strike seems to be coming to an end - yay! After having to cath every four hours for a few weeks, she finally decided to start peeing again on her own. I was never so happy to see a wet diaper. Champagne was popped and celebration ensued. She is now peeing on her own about 90% of the time. There have been a few times every other day or so, when we have to go in to get the pee, but it seems that her bladder has regained it's function.

Ultimately, who knows why she started retaining her urine. Although the pee strike seemed to coincide with her treatment on the seizure medication, Banzel. While urinary retention is not a listed side-effect, Banzel proved to be an awful medicine for Sadie. It intensified her acid reflux - causing her to vomit a few times and actually seemed to make her seizures worse. Once we started weaning her off the medicine, she had fewer seizures. Case made that every girl with Aicardi Syndrome is so different, as Banzel has been a great medicine for many of Sadie's Aicardi sisters.



Activating her light box during therapy

While the pee issues seem to be getting better, the constipation issues continue. We are following the treatment plan of Sadie's GI Motility Specialist who has ordered an anorecal manometry study. She will undergo this test next week to determine whether her muscles "down there" are operating properly to pass stool. Fun stuff.

Another bit of good news, is that Sadie completed her steroid treatment a few weeks ago - whew! We were hoping that getting her off steroids would help with her sleep issues. Unfortunately that does not seem to be the case. As a matter of fact, her sleep patterns have been getting progressively worse lately. She has been a bit hyperactive lately and is unable to nap during the day. At night, she is able to get to sleep, but staying asleep has been an issue. On a typical night, she is up between 3-6 times. The entire house is sleep-deprived.



How the Vagal Nerve Stimulator (VNS) works

We had been holding off on investigating the sleep issue in great depth, thinking that the sleep problems were related to her seizure activity - hoping that better seizure control would equal better sleep. After almost three years, and only a handful of nights where she's slept the entire night - we're ready to pursue an in-depth sleep analysis. More to come on that.

We have also scheduled an appointment to discuss a Vagal Nerve Stimulator (VNS) for Sadie. A VNS is like a pacemaker for the brain - helping keep the brain waves from getting too crazy. VNS has been a highly effective tool for girls with Aicardi Syndrome, and we can only hope that it is just as helpful for Sadie.

So, lots going on in Sadie-Land. She is back in the swing of things with all of her therapies. And overall is more alert and attentive - giving out lots of smiles, which makes this crazy journey a lot more enjoyable!