The Next Fire

Sadie smiling in her chair

Life with Sadie is a series of putting out fires. We're always waiting for the next one to pop up.
Luckily, the biggest fire of this Fall was extinguished. We heard back from the docs that the CT scan showed her brain looked stable. No fluid was building up, and the cyst fenestration surgery appeared to be doing it's job - allowing the fluid to naturally circulate again. We all exhaled.

However, soon after hearing this good news, another set of fires popped up. This Fall began with the first "fire," Sadie's mild pneumonia. She spent a few days on oxygen and recovered quickly. Shortly after finishing her antibiotics for pneumonia, she was diagnosed with a Urinary Tract Infection (UTI). Sadie used to get UTIs frequently when she was younger, but since starting a maintenance course of Bactrim, she has been UTI-free. Well, this one must have been a bit too much for the antibiotic to handle. So...she started another 10-day course.

In the middle of treatment for her UTI, the third "fire" erupted. Sadie awoke last Saturday morning very lethargic and with a "barky" cough. The pediatrician diagnosed croup. Her oxygen continued to drop throughout the day, until our home oxygen tanks just couldn't keep up anymore. Down to Lurie Children's Hospital we went...

She was diagnosed with a respiratory virus (rhinovirus) which led to the croup. When adults develop rhinovirus, it manifests itself as the common cold. When Sadie develops rhinovirus, it turns into an ER visit and a two-day hospital stay. By Monday morning she was completely off oxygen and back to herself.

You see, the problem with these fires is that the embers from one fire can ignite another. Along with an xray of her lungs, the docs xrayed Sadie's belly to get a better look at the GI issues she's been experiencing (as mentioned in the last blog). While the lung xray looked fine, the docs were concerned enough about her belly scan that they called in the surgery team to look for an obstruction.

Luckily, no obstruction was found, but the belly was full of poo and gas - even though she had a series of bowel movements in previous days. She is scheduled to see a motility specialist to see why her bowels aren't moving like they should be. The docs mentioned that the full belly could have contributed to the breathing troubles as well.

Staring at the Jellyfish at the Jellies exhibit!

And there are some fires just refuse to go out no matter how much water you throw at them (case in point, the seizures). Sadie's seizures have been pretty awful lately. She's still having her typical infantile spasm clusters a few times daily, but has also started to have lip-smacking seizures. Her face gets red and she'll smack her lips and move her mouth around strangely. These seizures have been occurring about 20 times a day. On Thursday, she was back at Lurie for an overnight EEG. In the short time she was hooked up, I pressed the seizure button twenty-two times. Yes, twenty-two times.

We had been trying to wean her off her heavy medicine load for some time, but it looks like the medicine we were trying to wean (Vigabatrin), may have been doing some good.  So, now we are titrating the dose back up. Ugh...it's a horrible game of trial and error.

On a lighter note, in between these illnesses, we were able to get Sadie out to the Shedd Aquarium to look at the fishies! She just loved the jellyfish! I think it was a combination of the dark room and the lit-up aquarium. You can tell just by looking at her face how much she enjoys it. (And, no...sorry folks...we will not be getting jellyfish at home.)

So...in the meantime, we'll keep working on these fires until we find a way to extinguish them once and for all.

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.

Meanwhile in Cleveland...

We recently returned from a visit to Cleveland Clinic. On our way to Cleveland, we stopped in South Bend for a family wedding. Sadie loved interacting with everyone at the wedding - the photos above are from the cocktail hour.

The purpose of our Cleveland visit was to get a second opinion on Sadie's treatment and to see if the docs there would recommend her for surgery. The facility at Cleveland Clinic was breathtaking...a lobby filled with beautiful artwork - even a few Plensas, and a lovely rooftop terrace with views of the Cleveland skyline.

On Day One, we met with Dr. Elaine Wyllie who heads up the Epilepsy Center at Cleveland Clinic. Dr. Wyllie said that their team will only recommend surgery if they truly believe it will be helpful to Sadie. If surgery is not offered, they will provide their input on her treatment and other options to consider. Our goal all along has been to allow Sadie to have the best developmental outcome possible, so that worked for us. After our meeting with the doctor, Sadie checked in to the Epilepsy Monitoring Unit to be hooked up to the Video EEG for the next four days.

The Video EEG process at Cleveland Clinic is quite thorough. From the placement of the sensors on her head, which was precisely mapped out with a measuring tape, to the re-application of sensor conducting gel...the process is quite intensive. As with Sadie's previous video EEGs, we press a button each time we see a seizure. Each time the button is pressed, at least 4 people rush into the room to note the seizure presentation, as well as to ensure the video is capturing the movement accurately. The techs review the entire EEG, both push-button and non-push-button events and note any abnormal activity for the doctors.

We had been informed by our docs at Childrens that Sadie has no sub-clinical seizures (i.e. seizures that we do not see...with no physical presentation.) While her brain waves are a little messy on her EEG, the lack of subclinical seizures was re-confirmed at Cleveland Clinic, which is comforting to know. They did mention that her brain waves during sleep are pretty active, which could explain her horrible sleep patterns.

For at least two hours after Sadie was admitted, a steady stream of hospital staff was in and out of the room - doctors, nurse practitioners, dietitians, technicians. The anesthesiologist reviewed instructions for Sadie's PET scan the following day - no food after 4 am. This presents a challenge as all her meds are given in a bottle of formula at 6:30...so for each day we were there, we were waking up at an ungodly hour to feed her and give meds. We had to laugh when the nurse, seeing Sadie asleep at 8 pm on the first day, asked "So, is she out for the night?" Don't we wish!

Sadie's first test was a PET Scan. A solution was injected through her IV. The tech mentioned that they would monitor her awake brain waves for 30 minutes prior to going under anesthesia. I swear, as soon as she heard that she needed to stay awake, her eyes promptly shut. That's our Sadie for you...always battling authority. This started a 30 minute circus act of mommy loudly singing songs, playing with toys, wet washcloths, tummy time...whatever I could do to keep her awake. Unfortunately, I was only moderately successful.

Sadie was put under anesthesia for all three of her tests. On Wednesday, Sadie had her first ICTAL SPECT test. For this test, a radioisotope was injected via IV at the onset of a seizure, and her brain was scanned to determine where the seizure originated. On Thursday, the same test and scan was conducted during non-seizure brain activity to get a baseline. We were informed that the radioactive isotope would leave Sadie's body through her urine, so she had blue radioactive diapers for 48 hours.

This morning of Sadie's first Ictal SPECT test, the nurse sat by Sadie's bedside and waited for seizure activity. Luckily for her, Sadie cooperated and had a seizure in the first 30 minutes. Unfortunately, when the nurse injected the radioactive isotope, a little of it spilled out of Sadie's IV...so the docs needed to calculate how much got into her system. So, this took a little more time, but it also got Sadie into anesthesia for the scan a little quicker.

Before we left Cleveland, the doctor revealed that she thinks surgery might be an option for Sadie. She was initially cautious about surgery because her brain abnormalities are in both hemispheres, and she shows abnormal EEG activity from both sides. But she mentioned that based on the test results she's seen thus far, she thinks she can identify an area of the brain that could be removed to improve her seizures and developmental outcome, while preserving most of the vision in her left eye.

Vision has always been one of our major concerns with surgery. Removing the right hemisphere of her brain would also impair the vision in her left eye - the eye that has good vision. Sadie gets such pleasure from visual interaction, we are hesitant to take this away...even partially. Of course this is a preliminary assessment, and we will hear their final recommendation after the entire team has had a chance to review her info during their Patient Management Conference.

So now we wait...

Overall, we were very pleased with the staff, nurses, and doctors at Cleveland Clinic. We are anxious to hear the final set of recommendations this week after the doctors' conference on Tuesday. Sadie definitely earned an "S" on her chest - which will make her Supergirl Halloween costume even more appropriate this year.

An Enigma Wrapped in a Riddle

"It is a riddle, wrapped in a mystery, inside an enigma; but perhaps there is a key." - Winston Churchill

We always say "if it's not one thing, it's another." Miss Sadie continues to be a mystery. The seizures are relatively low (well, for Sadie's standards), but she has not been herself lately. She is restless, irritable, not eating and sleeping well. She is also not as interactive as she once was. It's been about 2 weeks since we've seen a smile (very un-Sadie-like). This lack of recognition and interactivity just tugs at the heartstrings, especially because this was once one of her strengths. We're trying to figure it all out, but it's as if we have 12 puzzle pieces from 12 different puzzles.

We thought we solved one piece of the puzzle when the docs found another UTI last week. Yes, her fourth UTI...which we think is happening because she tends to hold her urine for hours on end, then bacteria builds up and before you know it, another infection.

However, we are now on day 4 of antibiotics and Sadie has not gone back to her happy-go-lucky self. My current theories are a Dilantin level that is way too high (when in doubt, blame the Dilantin) or a side effect of weaning the vigabatrin (maybe it was doing more than we thought).

Sadie's oxygen requirements have also been up and down. She'll do great for a few days off oxygen, then for some unknown reason, she'll need it again. We had a great appointment last week with Mary Massery, a PT who specializes in kiddos with respiratory issues. Since Sadie has developed her propensity for oxygen, Mary's name has been brought up by numerous people. She is in such high demand, that it took 6 months to even get in to see her! She was able to provide some great recommendations for Sadie. Maybe we can get her off this oxygen once and for all!

The biggest news of the past few weeks is that we've heard back from the doctors at Cleveland Clinic regarding a second opinion on Sadie's treatment and possible surgery. We will be traveling there in early October for some testing and a surgical evaluation. While she is there, Sadie will meet with Dr. Elaine Wyllie and will undergo a PET Scan and an ICTAL SPECT . The ICTAL SPECT is a test where dye is injected during the onset of a seizure, the dye pinpoints the location of the seizure activity in the brain. It can be very helpful for doctors in surgical planning.

Sadie will be hooked up to a Video EEG in the Epilepsy Monitoring Unit during her entire stay at Cleveland Clinic. This will enable the docs to capture as much brain activity as possible.

We hope and pray that this team of doctors can help us determine the right path for Sadie's treatment. Perhaps they can find the key to unwrap this mystery.