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.

A Long February's Journey Into March. A Surgery Story.

In the lobby of the Cleveland Clinic is a projection of a tree that transitions through the four seasons. Spring, Summer, Fall and Winter. We arrived in Cleveland to cold weather and snow on February 20th and left to 70-degree weather on March 17th.

Sadie arrived at the Surgery Center at 5:30 am on Thursday, February 23rd. I carried her into the surgery room at approximately 7:30 am. I was able to stay with her until she fell asleep from the anesthesia. Leaving her was one of the most difficult moments of my life. Surgery lasted approximately 5 1/2 hours. We waited in the surgical lobby and received periodic updates via pager.

Except for needing a bit of a blood transfusion, the surgery itself went fine. Following the procedure, Sadie's surgeon, Dr. Bingamin, provided us with an update on the procedure. He mentioned that the structure of her brain is a little smaller than is expected of a toddler her age (microcephaly). We weren't terribly surprised, knowing that this is common for girls with Aicardi Syndrome. He explained some of the work they did during surgery, mentioning the use of electrodes on the brain tissue during surgery to see which areas were emitting seizure activity. He mentioned that her most malformed area in the frontal lobe was constantly spiking with electrical discharges.

The photo above shows what Sadie looked like post-surgery - puffy and pale. While the surgery itself went as expected, Sadie's recovery was quite difficult. As she awoke from surgery, she began having a few seizures - full body convulsions that we had not seen before, which began on the right side of her body. Apparently this is not uncommon for kiddos who have undergone brain surgery due to the trauma to the brain tissue. Still a little disheartening.

She was given a number of different emergency meds to stop the seizures. Dr. Bingamin also ordered a CT scan to check for bleeding, which came back normal. Due to the additional seizure meds, there were a few days where we were extremely concerned that Sadie would not be able to continue breathing on her own. Which means intubation and be placement on a ventilator. In fact, we had a ventilator hanging out in her room in the PICU for quite a while.

Sadie also had some trouble post-surgery with nausea. She was vomiting for a few days after surgery, so much so that docs didn't even try giving her any nutrition until 5 days post-surgery. And, even then, they just placed her on a TPN IV bag (a type of nutrition that goes directly into her bloodstream via IV). Sadie's nausea and strange right-sided movements continued for a few days post-surgery.

On Saturday, they removed her head bandaging revealing her scar, which was shaped like the upper part of a question mark looping from her right ear around the side/back of her head and around down the middle. Immediately after removing her head bandaging, Sadie was hooked up to a Video EEG to monitor her brain waves. It was Saturday that we learned Sadie was in "status epilepticus" or constant seizure. She was then placed on additional medication and the risk of breathing impairment increased.

Sadie stayed in status epilepticus for a few days until her brain slowly calmed down. She stayed on the Video EEG for a total of 12 days until the docs felt comfortable enough to take her off.

Meanwhile, she did have a bit of trouble breathing and required a few treatments by respiratory therapy, but luckily no intubation was required. She also suffered from some issues with her belly being full and distended, requiring docs to place an NG tube to pull some of the excess "juice" out of her belly. Sadie also had a feeding tube (ND or "CorePack" tube) placed which went directly into her intestine, this was used to administer medicines and to feed her formula.

After her central line was taken out of her neck, she received a PICC line in her arm. A PICC line is basically an IV that is meant for long-term administration of IV drugs, it also can be used to pull blood, so the child doesn't have to be continuously poked. Sadie was put under anesthesia and intubated for the placement of the PICC line.

Originally, GI docs wanted to conduct a gastric emptying study and a swallow study. After a little prodding by mommy and daddy, the tests were cancelled and replaced by a simple bedside swallow evaluation, which Sadie passed with flying colors. We began to feed her orally about 2 1/2 weeks after surgery. While her suck was a little weak, she quickly regained this skill and is again eating like a champ.

In addition to Sadie's brain surgery, she also had two additional diagnostic procedures performed - a spinal tap and a skin biopsy - to test for an underlying mitochondrial disease. We have some preliminary feedback from the spinal tap that no mitochondrial abnormalities were found, but we are awaiting final results, as well as the skin biopsy results.

We were hoping to transfer Sadie into an inpatient rehab facility, but after a few days of fighting with the insurance company, they denied her transfer. We will be starting a day rehab program with Sadie this week.

Overall, Sadie performed in her typical super-hero fashion. Lots of pokes, prods, tests, wires, cords, etc. but she still managed to give smiles to Mommy and Daddy. We're not quite sure where things will end up post-surgery. It will take some time to know for sure. So far, she's been very vocal, very interactive, very happy, and while the seizures still remain - they are less frequent and less severe.

After twenty-seven long days in Cleveland, we are enjoying the beautiful Chicago Spring and are happy to be home.

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.