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.

In The Weeds

As a former waitress, when things were starting to spiral out of control, you'd say that you were "in the weeds." If you were lucky, coworkers would help out until you were in a more comfortable place.

We're on week three of Sadie's ACTH treatment. While the steroid seems to have gotten Sadie out of her every-five-minute-seizure-pattern, she is still "in the weeds." We've gone from seizures every five minutes to seizures every fifteen minutes - still not a great place to be. You know things are bad when medical professionals (epileptologists, even) look at you in amazement when you tell them the number and duration of seizures she has, as well as the numerous medicines and treatments she is on.

We've started making some slow changes with her medicines. We're finally starting to wean her off of Vigabatrin - a medicine she's been on now for a year. The jury has always been out on Vigabatrin. We initially thought it was making things worse, then after a few weeks, she got better...but she had also started the ketogenic diet around the same time. Of the four seizure medicines she is currently on, Vigabatrin has the most severe side effect - potential vision loss - and with Sadie's existing vision impairment in her right eye, it's the logical choice to start weaning first.

On a parallel track, we've also started testing to determine whether Sadie is a candidate for brain surgery. After talking with few other parents of children who have undergone hemispherectomies, we've started an initial conversation with Cleveland Clinic, who has a great epilepsy center and neurosurgeon who specializes in the surgical procedure.

In Sadie's situation, we have epileptologists, pediatricians, dietitians - all helping to get Sadie out of "the weeds." But no matter how hard we tug, we just can't seem to get her to where she needs to be. We'll simply keep trying.

Desperate Measures

Since my last blog post, Sadie's seizures have gotten significantly worse - the twitching episodes are now happening every 2-5 minutes. After giving Diastat 3 times in 48 hours, we felt uncomfortable managing the seizures at home, and decided that she needed to be in the hospital. Upon our arrival to the ER, Sadie was given a dose of phenobarbitol to calm her brain activity. The medicine completely knocked her out. We were hopeful that it would give her some much-needed rest and seizure relief while allowing for the prednisolone to take effect.

Let me backtrack...after 10 days of crazy seizure activity (every 5 minutes,) we decided to start a steroid treatment, prednisolone. We had previously rejected the steroid route due to their intense side effects - suppressed immune system, irritability, increased blood pressure, weight gain, etc. Also, steroids are typically a short term treatment and sometimes the seizures come right back once treatment ends. Well, after dealing with seizures all day long, we were desperate for anything that might help.

So...back at the hospital...once the phenobarb wore off, Sadie's seizures returned. It seemed that the more mild steroid treatment (prednisolone) was not working. Time to call in the big guns. Desperate times call for desperate measures. Sadie starts her ACTH treatment tomorrow. ACTH is another steroid treatment that we had previously dismissed due to all the scary side effects listed above as well as a high mortality rate due to illnesses contracted because of a suppressed imune system. It was a difficult decision, but we feel like we need to give this a try for Sadie's sake. Our list of options narrow and the choices after ACTH are pretty shitty (excuse my french)...and would include possible hemispherectomy (removing part of Sadie's brain.)

I hope the day is close in the future when I will be able to share some positive news. Thanks to everyone for your contiued support.

P.S. The photo above is my view from my hospital chair...where I get very little sleep.

For Better or Worse. Here Goes...

We're bracing for impact. What that impact is, we're not sure. After a week of various opinions, we've made a decision.

Let me start at the beginning...

Sadie saw a new neurologist last week to get a second opinion. In the meantime, her current neurologist presented Sadie's case to the team of doctors at Children's Memorial. Apparentely, the doctors do this periodically when they have difficult cases. The result was two differing opinions.

Doctor A recommended weaning a few medicines to try to determine what is and isn't working. "A" also suggested she try a few new medications. Doctor B recommended we start aggressively treating her with prenisolone, an oral steriod. Steriod treatment has always made us a little nervous, given the many side effects, including a weakened immune system. We've opted out of the most commonly used steriod for infantile spasms, ACTH, due to the high mortality rate (1 out of every 12 children), and the fact that many Aicardi parents don't think it works very well for girls with the Syndrome.

After giving ourseleves a few days and seeking out numerous opinions, we decided to forgo the steriod treatment for the time being. For better or worse, we're trying another seizure medication, Depakote.

Sadie was also at the hospital most of the day last Thursday for her Electroretinagram (ERG). We received good news and bad news. The good news is that the ERG showed Sadie to have decent vision in her left eye. The bad news was as we suspected, she most likely has no vision in her right eye....sigh. Although we had mentally prepared for this, it doesn't make the news any easier.

We've started working with two great non-profit organizations. The first is an organization called Hope's Friends. They send a home nurse to see Sadie as needed, a service that will be even more important as cold weather approaches. The nurse came out this week to check out Sadie's bowel movement issues. The ketogenic diet really stops her up :( They'll also be starting Music Therapy with Sadie this week. We'll see if we can get any Katy Perry or Beyonce in the mix, you know how Sadie loves them :) Additionally, an aromatherapist is developing aromas for Sadie to see if it will help with her bowel movements and seizure control. It's worth a try, right?

Sadie also visited the local Easter Seals chapter for a feeding evaluation. She will go back in a few weeks for a seating/positioning clinic to help determine what, if any, special equipment is needed as she gets older. Specifically, we're looking at special feeding chairs, strollers, and car seats. Both organizations have been wonderfully helpful for Sadie.

So, we brace for impact as Sadie starts what is now her third seizure medicine this week. We hope and pray that the third time's a charm.

From bad to not so bad. Yay for ketones!

The last week of July things went from bad to worse. Sadie had been on vigabatrin, her new seizure medicine, for about two weeks. The medicine seemed to have an adverse effect, as her seizures started INCREASING. Each time the doctor would increase her meds, her seizures would go up. The increased medication was also making her drowsy. By the last weekend in July, her only awake time was when she was having spasms.

We were on the phone with the neurologists every day talking about scary remedies, from administering diastat (an emergency medication given rectally to stop seizures), to admitting her to the hospital to begin ACTH, an injectable steriod treatment. We had already decided that ACTH had too many scary side effects for us to try, one of which is possible death. So we were stuck with a baby who is constantly having seizures.

On top of the seizures, Sadie had a conformer put in her eye last week, and the conformer ended up scratching her cornea. Back to the ocularist to get it taken out, and then to the opthomologist to get medicated drops. Ugh. If only I could get paid for coordinating doctors appointments.

Needless to say all of this was super stressful. We were at the end of our rope when the hospital called to tell us they were able to get her in to start the ketogenic diet right away. Sadie was admitted on Monday. She received her first bottle of KetoCal (a special ketogenic formula) on Monday night. It smells like a vanilla milkshake. Sadie loved it...but of course she's never turned away food. We anxiously awaited the arrival of ketones in her urine, which indicates that the diet is working. I don't think I've ever looked forward to diaper changes as much.

Monday and Tuesday went by without ketones. Sadie's seizure activity held steady. She had a few scary episodes at the hospital, one of which lasted 50 minutes. FINALLY late Tuesday night she had a trace of ketones in her urine. Yay! The doctors came in saying they wanted to pop the champagne.

Speaking of the doctors, I can't say enough good things about the staff at Childrens Memorial Hospital, many of whom are aware of sadie's condition and pulled strings to get her in earlier to start the diet. Sadie was released from the hospital on Thursday night and seems to be getting better, slowly...

We are cautiously optimistic.