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.

A Better Place To Be

Recently, Adin and I were looking at a few older posts on Sadie's blog. Two years ago, Sadie was hospitalized emergently to start the Ketogenic Diet after having hundreds of seizures per day. One year ago, she was hospitalized again to start ACTH after having seizures every five minutes.  This year, she's recovered from two brain surgeries. Reading those posts was a stark reminder of everything she has been through in her short life. And how, as soon as you think you are in a better place, reality can knock you back down.



Sadie and Daddy enjoying pool time.

Now that she is finished with the Day Rehab program at Rehab Institute of Chicago (RIC), she is continuing with her regular therapy schedule, which includes weekly Physical, Occupational, Speech, Vision and Developmental Therapies. I'm pleased to say that despite recent setbacks, she continues to do well in therapy. Moving at a much slower pace than typically developing kids, obviously, but making progress nonetheless. Recently, her Physical Therapist has been working with Sadie in a Lightgait over a Treadmill, getting her to walk a few steps at a time so her body begins to understand what this feels like.

Additionally, I've been looking to enroll her in aquatic therapy. Aquatic therapy can provide valuable movement for kids who are low tone and struggle with holding up their own body weight. We tried Sadie out in a pool, and she seems to really enjoy it, so we are hopeful we can get her in aquatic therapy soon!

Sadie and Olivia contemplating
the Modern Art.

In spite of a busy therapy schedule, we have found time to do a bit of traveling this summer, as well as getting out and enjoying some Chicago sights. Sadie and her friend, Olivia, recently visited the Art Institute of Chicago to take in the new Modern Art wing. Sadie didn't quite grasp Modernism, she was much more drawn to the Impressionists - Monet, Seurat, and Van Gogh in particular.

We also recently traveled to St. Louis for the 2012 Aicardi Syndrome Family Conference. The Aicardi Syndrome Foundation holds this biennial conference for families with affected daughters. It was such a great opportunity to see old friends and meet new ones - quite a different experience than our first conference, which we attended four weeks after Sadie's diagnosis. This time, we found ourselves to be much more social with the other families, rather than just deer-in-headlights

Medically speaking, Sadie is still being fed the majority of her meals via NG tube. She is allowed a little bit of pureed food each day, which she eats with varying degrees of success. Sometimes she chews well and swallows, other days, the food sits in her mouth and she spits it out. We continue to work with her speech/feeding therapists to improve her eating skills. She was originally scheduled for a repeat swallow study in August, but we've decided to hold off until we find out the results from her recent brain surgery.
As I mentioned in my last post, the cyst fenestration surgery has a 50% change of being successful at alleviating Sadie's hydrocephalus. Sadie is scheduled for a CT scan in mid-September, which will show whether or not surgery was successful. If the fluid is building up in her brain again, she will have to get a shunt (an artificial drain) placed in her brain. We are saying lots of lots of prayers that the surgery WAS successful and she will NOT need this.

Prior to her recent surgery, had switched some of her seizure meds to see if we could gain more seizure control. She is finally completely off Dilantin (yay!), and we had begun a taper of Sabril (Vigabatrin). She also started a new medicine, Zonisimide, which we are still hopeful might provide some seizure relief. However, in light of the recent surgery, everything is on "pause."

Speaking of a better place to be, Adin and I booked our first "alone" vacation since before Sadie was born. We are looking forward to getting away for a few days and know Sadie will be in good hands with Grandma and her Aunt Megan.

We look forward with a mixture of hope and trepidation as we await the results of her brain surgery. She needs your prayers now more than ever.

Stronger

Sadie weight-bearing in the LiteGait.

Sadie is going into her third month of the Day Rehab program at RIC (Rehabilitation Institute of Chicago.) She has been attending RIC for three days a week/two hours a day. During this time, she gets physical therapy, occupational therapy and speech therapy. While it has been an intensely busy few months, we couldn't be more pleased with her progress.

Her head and trunk control have improved significantly - and she can even sit with very little assistance. During speech therapy, she is working on communication by using the switch - shown in the photo to the right. Knowing her preference for pop music a la Beyonce and Katy Perry, her sweet speech therapist put a photo of Katy Perry on her switch. Each time Sadie presses the switch, we play music and have a dance party.


Sadie's switch with Katy's photo

Unfortunately, there are a few skills which Sadie used to practice, that she has since lost - like reaching for and grabbing a toy, bringing her hands to midline, and rolling over. Even before her surgery, she stopped demonstrating these skills. We are hopeful that with the help of her therapists that she will be able to regain these abilities.

In addition to her therapies at RIC, we are still working with her home therapists through the Early Intervention (EI) program. We recently visited our neighborhood park with Sadie's home PT, where Sadie experienced sitting on a swing and sliding down a slide - with the help of Mommy and Karen, her PT.

Sadie exploring the neighborhood park

While she is making great strides with her therapy, Sadie continues to struggle with daily seizures. As I've mentioned, we have seen some improvement post-surgery- particularly with the duration and severity of her seizures. Unfortunately, she's still having about the same number of seizures per day.

So, we are again in discussions with her doctor about switching up seizure meds. Our first order of business is to try to wean her off a few of these meds (she is currently on five - Vigabatrin, Keppra, Dilantin, Clonazapam, and Neurontin) - not to mention her Ketogenic Diet. Weaning the meds that aren't working will allow us greater flexibility to try new things. The challenge is figuring out what IS and IS NOT working.



Snoozing at the Zoo

Her blood draw a few weeks ago showed that her Dilantin level was a bit low. So, upon her doctor's recommendation, we are going to try weaning that first. If we can get her off Dilantin without upsetting the apple cart, then we are going to try a new medicine called Zonisimide (Zonegran). Zonisimide has been recommended for Sadie by a few doctors. So, we're hoping that it will work well for her. But first...a five-week wean off Dilantin. I never said things happened quickly.

In the meantime, Sadie experienced her first visit to the Zoo while visiting friends in Milwaukee. Of course, she spent most of the trip napping, but she did see a few of the animals. Mr. Cheetah made an appearance right in front of the window where Sadie was looking - and I think he caught her eye!

So, as Sadie's therapy sessions at RIC begin to wind down this month, we'll have a bit more free time to enjoy the warm summer weather, and to get her outside practicing some of her new skills!

An Unexpected Turn

All tuckered out from opening birthday gifts.

Raising a special needs child is often compared to a roller coaster ride. Along with the many ups and downs are unexpected twists and turns. We experienced an unexpected turn last week during Sadie's swallow study. This was Sadie's third exam to look at her swallow. Her first swallow study showed she aspirated thin liquids, and we've since been thickening her bottles using a gel called "Simply Thick."

During the swallow study, a substance called barium is added to her food. The barium lights up on the x-ray showing where the liquid is going during the swallow. (You can see a pretty cool video here.) Her therapist and doctor at the Rehabilitation Institute of Chicago ordered the swallow study to check her post-surgery swallow. We went in assuming a routine appointment - after all, she had a post-surgery bedside swallow examination in Cleveland which she passed with flying colors. She had not been getting sick and her lungs have always sounded clear (two red flags for aspirating).

However, the results showed otherwise. While drinking her bottle, even thickened, a small amount of the liquid was making it's way into her lungs during each swallow. Even at it's thickest consistency (honey thick), it was still going into her lungs.

Sadie eating her breakfast via NG tube.

Sadie was sent directly to Children's Memorial Hospital for an immediate admission and NG tube placement. (Standard protocol when a child fails a swallow study.) We were told by the pulminology team at Children's that if we had not found the aspiration, Sadie could have come down with a horrible life-threatening pneumonia. And, if she were to continue to drink, the aspiration could cause lung disease. The pulminology docs said that there are over 70 different mechanisms that must occur in the throat to ensure a safe swallow. Seems amazing that all of us aren't aspirating all the time, right?

This is what a G-Tube looks like.

So...you may ask, where does that leave Sadie? Well, there are few options for a child who aspirates. The most common being a G-Tube. Many girls with Aicardi Syndrome also have G-Tubes. In fact, one of Sadie's good Chicago Aicardi friends, Olivia, just got her G-Tube a few months ago.

In order to mentally prepare for this possible next step, we met with the surgeon this week who would perform the G-Tube operation. He provided us with pros and cons of G-Tubes. The pros being ease of medication administration, hydration, and most importantly - no aspiration. We have spoken to Sadie's team at Children's Memorial who have advised us to take our time with making this decision. They have offered to repeat a swallow study in 3-6 months to see if her swallow has improved. By that point, we can do more research and become more comfortable should the G-tube be the path she must go down.

Luckily, she is still able to eat pureed food (baby foods), so we've been feeding her her favorite keto-friendly food - mashed banana with butter. While we are disheartened and saddened by another "normal" being taken away from Sadie for the time being, we are grateful that it didn't develop into something even worse.

A Spring of New Beginnings (and Hats!)

It has been three weeks since Sadie returned home from her stay at Cleveland Clinic. We have been enjoying getting back into our regular routine. Sadie's scar from surgery is healing up well and her hair is growing back slowly. She received an assortment of spring sun hats from her Grandma. We joke that it is the "Spring of Hats," for Sadie. Being the trendsetter that she is, I'm sure that soon everyone will be wearing them.

The seizures have been holding steady. They are still less intense and frequent than before surgery, but she still has multiple episodes per day. She continues to be quite vocal and active...even a bit hyperactive at times. She particularly likes to kick around and talk around 3:30 am. Once she awakens, it's fruitless to try to get her back to sleep, so we typically pull her into bed with us and just endure the yelling and swatting until one of us gives in and gets up for the day. It's really quite comical...and tortuous. I now understand how sleep deprivation is used as a method of torture.

We have one more week until she is completely weaned off Phenobarbitol, so we are hopeful that the hyperactivity and sleep issues will settle out once her body has adjusted. Anecdotatally speaking, Phenobarbitol is notoriously difficult to wean and can cause all kinds of withdrawl symptoms in kids. We've spoken to many parents who have experienced similar difficulties during their child's wean.

Her doctor mentioned that we have the option of increasing her sleepy medicine (Neurontin) to try to get her to sleep a little longer. But, my mind was having a hard time making that leap...given that she just went through surgery in the hopes of weaning her OFF some of these meds. So, we decided to endure the sleep deprivation for a few more weeks until she is completely weaned off Phenobarbitol. Let's hope we can both keep our sanity until then...

Sadie's therapy schedule has been keeping us quite busy lately. Three days a week she is in the Day Rehab program at the Rehabilitation Institute of Chicago (RIC). She receives Physical Therapy, Occupational Therapy and Speech Therapy in Day Rehab. She also sees her regular therapists through Early Intervention (EI), but we have scaled back a bit with EI until she completes Day the program at RIC. Presently, we think she'll be in Day Rehab for at least two more months. The duration will be based on how well she is doing and how close she is to her pre-surgery baseline.

We've already seen HUGE improvements with her head and trunk control. When she left Cleveland Clinic, her muscles were quite floppy - a wet noodle. Now, she is managing her head quite well. She still needs to be supported while sitting, but that has also improved immensely. The therapists at RIC have ordered a Benik Vest for Sadie to help with her trunk control. Of course, Sadie's will be hot pink!

A bit of good news - we've heard back regarding Sadie's diagnostic testing for a mitochondrial disease, and so far, everything has come back negative. There are still a few tests outstanding, but this is great news.

In the meantime, we are beginning to plan for her two year birthday! It's hard to believe that Sadie will be turning two at the end of the month. Of course, we'll be on the hunt for the perfect hat for the occasion.