A Rocky Start

Sadie with her EEG leeds (and bow) on.

Well, 2013 started off quite rocky for Miss Sadie. We were hoping that we would start to see a decrease in seizure activity after her shunt was placed. Unfortunately, that's not what happened.

Let me back up a bit. Sadie went in for shunt surgery on December 27th. The surgery itself went quite smooth, lasting only about 45 minutes. Post-surgery, she wasn't herself and seemed to be a bit uncomfortable. Despite pain meds, she had a restless night and was quite twitchy. A CT scan showed no issues with the shunt, so the docs ordered an EEG to check for seizure activity. About 20 minutes into the EEG, she had a big seizure - probably the scariest one I've seen to date. Sadie stopped breathing twice, turned an awful shade of blue, and oxygen levels dipped to 40% both times. A slew of nurses rushed in to administer oxygen. She seemed to come out of it fine, but it was quite scary nonetheless.

The EEG showed two focal seizures had occurred during her cluster of infantile spasms. Emergency seizure meds were administered, which calmed her brain down. The next day she was back to herself and was discharged from the hospital.

As I mentioned, our hope was that the shunt placement and subsequent fluid decompression in her brain would result in fewer seizures. Unfortunately, the seizures just continued to escalate in severity and frequency, until finally I wasn't sure if she ever fully recovered from one seizure before another started. On January 8th, we decided that we weren't comfortable managing this at home any longer and took her to the ER.

In the hospital bed with her angel doll.

Ultimately, she was in the hospital for a total of twelve days. The shunt was fully examined and was functioning properly. So...the question remained, what was causing the seizure increase? A full evaluation was run - CBC blood counts to check for infection, nasal swabs to check for signs of respiratory viruses, urine samples, chest x-ray, etc. No evidence of infection or viruses were found. In fact, other than the relentless seizures, she seemed to be perfectly healthy.

The doctor concluded that perhaps movement of some of the brain tissue was causing seizure activity, either that or she simply needed a tweak to her seizure medicines - or some combination of both.

The Epileptologist on hospital service happened to be Sadie's own doctor, who knows her quite well. She was able to quickly evaluate Sadie and recommend a course of action. She started her on a four-week course of high-dose steroids, Prednisone, to calm seizure activity. She also put her back on Dilantin - a medication that has seemed to work successfully for Sadie in the past. Additionally, we increased the dosage of a few of her daily seizure medicines, including a new medicine that she recently started, Banzel.

Over the twelve day hospital stay, she went from seizing almost non-stop, to having a few seizures an hour, to a seizure every few hours. While this may still seem like an awful place to be (and trust me, it is...) she's in a much better place now than when she entered the ER.

Smiling at Daddy.

Never short on challenges, Sadie decided to embark on a pee and poo strike while she was in the hospital. (Caution, if bodily functions gross you out, stop reading now...) Now, as many of you know, pooing daily has never been Sadie's specialty. Recently, it's taken a liquid suppository every three days to get the girl cleaned out (or have a poo-nami, as we call it). However, while hospitalized, she decided to escalate this to a whole new level. Two suppositories on day 3, two enemas on day 4...and still no poo. It finally took a third suppository on day 7 to get her to have a bowel movement (now escalated to a poo-splosion). How she kept all that inside her for seven days is beyond me.

Onto the pee situation. Sadie occasionally holds her urine. Sometimes, she'll go as long as 6 hours, then flood through everything she happens to be sitting on - chairs, beds, daddy's lap, etc. However, during this hospital stay, peeing was not something she was interested in. At one point, she held her pee for 16 hours before the nurses cathed her. The urology team then got involved in Sadie's care. They started her on a medicine called Cardura which helps with urinary retention. We were also instructed that we should cath her at home every four hours if she isn't going on her own (oh, joy). Sadie will go in for a urodynamics study in a few weeks to try to get to the bottom of the issue.

We've been so consumed with Sadie's surgeries and health issues, that everything else has taken a back-seat. We've been working with our local school system to try to determine an appropriate school for Sadie. At the age of 3 (which she will be on April 29th - yikes!), her in-home therapy services through Illinois' Early Intervention program end and she will receive therapy through the school system. We visited a local school in mid-December, which we really liked, but just didn't feel like it was the best fit for Sadie. We will visit a few more schools in February.

So, we hope that 2013 will begin to trend in a positive direction. Heaven knows we could use a break. And, more importantly, so could Sadie.

P.S. Like my Facebook page for more frequent updates on my adventures: http://www.facebook.com/friendsofsadiemccann

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.

An Eventful Few Weeks

A lot has happened in Sadie-Land in the past month. A few weeks back, Sadie had a series of sleepy days. As you know, sleepy days are few and far between for Miss Sadie. She is lucky if she gets a 30 minute nap each day. So, on a Sunday when she had slept 5-6 hours and barely woken up when roused, we were naturally concerned.

The following day, I mentioned this sleepy behavior to her Epileptologist during our routine visit. She shot me a concerned look and immediately sent Sadie to medical imaging to check if the fluid in her brain had started to build up again. At this point, we were only 2 1/2 weeks out from her cyst fenestration surgery.

Thankfully, the results came back fine. At this early stage, the fluid didn't look like it had started to accumulate again, but we wouldn't know for sure until Sadie's 6-week post-op CT scan. However, the doctor did express some concern about the tumor on the left side of her brain.

Chillin' in her stroller during a walk.

You see, Sadie has a tumor in her left hemisphere called a choroid plexus papilloma. We've known for some time that it was close to the third ventricle in her brain and had the potential to cause hydrocephalus (fluid buildup) should it shift or grow. Prior to her recent brain surgeries, she had been undergoing MRIs every 6 months to check for tumor growth and to ensure it doesn't block the ventricle. The doctor mentioned that THIS imaging showed the tumor to be partially obstructing the third ventricle.

So, now it seems we have two things to be concerned about - first, that the scar tissues doesn't re-form and cause blockage, and second, that the tumor doesn't get in the way and cause blockage. If either of these occur, then it appears that the only way to fix the problem is by placement of a shunt. We will know for sure if either of these things is occurring after her CT scan scheduled for this Thursday.

In other news, Mommy and Daddy were able to get away for vacation for a few days. But, despite a long talk with Sadie about NOT getting sick, she decided to do the opposite - just like a toddler! 

On Saturday night, her oxygen dropped and she developed a fever. When her temperature increased a few hours later, Grandma and Aunt Megan decided to take Sadie to the ER for assessment. Turns out, she had a mild pneumonia which was likely caused by aspiration from vomiting a few days earlier.


Sadie looking lovingly at Aunt Megan (Goo).

Another issue Sadie has been dealing with lately is slow motility. Or, in layman's terms - she can't poop. In the four weeks since her brain surgery, she has not been able to have a bowel movement without assistance of a suppository. We visited the GI doc this week who gave us some tips on how to get her to go...yes, it's a glamorous life we lead.

We did receive a bit of good news last week. After a visit with a doctor at the Chicago Lighthouse for the Blind and Visually Impaired we received confirmation that Sadie has normal vision in her left eye. Yay! We had heard this from her regular opthomologist, but I've become a bit of a sceptic when it comes to doctors, so it is always a relief to get confirmation from another source!

Please keep Sadie in your thoughts and prayers as we hope for good news out of Thursday's CT scan.



Where to Begin...

Sadie post surgery

I've been procrastinating writing this post because so much has occurred in the past few weeks. Where to begin...

I suppose I'll start with the biggest piece of news. In case you hadn't heard, Sadie had a second brain surgery a few weeks ago. During her 6 month post-surgery follow up appointments at Cleveland Clinic, her MRI showed water in her brain cavity that was unable to drain. This fluid-filled pocket (or cyst) was causing pressure to build up and press against her brain stem and other structures in her brain. We saw this clearly on the MRI.

The doctor was not 100% sure why this occured, he said that it is possible that scar tissue from her first surgery formed a membrane over the brain's natural drain causing a buildup of fluid. Apparently this can happen in 5-10% of patients who receive brain surgery.

The doctor spoke about a few symptoms that we might have seen. Headaches, sleepiness, loss of head control, etc. Obviously, Sadie can't tell us if she has a headache, but we hadn't noticed a change in her behavior. We did, however, notice that her head control hasn't been quite as good and she has been excessively sleepy lately - both things that we had been attributing to a recent change in seizure medicines. 

Our pager that provided updates
during Sadie's surgery.

The doctor layed out a few options for treatment. First, we could choose a cyst fenestration - which essentially is a procedure where they go into the brain to lance the cyst and create a natural hole for the brain to begin to naturally regulate the pressure. The second option he offered was a cyst fenestration and shunt. A shunt is an artificial drain which is implanted in the head and drains the spinal fluid into the belly where it is reabsorbed. The drawbacks of a shunt is the probability that it could malfunction, get infected, etc. This occurs quite often. Lastly, he mentioned that some parents may choose NOT to treat the condition and hope for the best. The obvious drawback to this choice is that this condition can be fatal.

Adin and I decided that we were not willing to give up fighting for Sadie, and we would obviously choose to treat this condition. Which left us with surgery or surgery as options - not a great choice. So, we chose to do the cyst fenestration and hope that it works to drain the fluid and create the natural circulation of fluid in her brain. Here are some articles we read which describes the procedure in more detail:

http://cornellneurosurgery.org/pedneuro/endo_cyst.html

http://www.ncbi.nlm.nih.gov/pubmed/3226511

The drawback of the cyst fenestration without a shunt is there is a possibility that it might not work. The doctor told us that it has a 50% chance of working. Sadie will have a CT scan of her brain in six weeks which will show whether the surgery was a success. If the fenestration alone isn't effective, we'll have to consider a shunt.

Doors to the PICU.
I've seen these much too often.

Surgery was performed at Cleveland Clinic by Dr. Bingamin, the surgeon who performed her first surgery. Although this is not what we expected when traveling to Cleveland for follow up appointments, we knew she would be in good hands.

Unlike Sadie's first surgery, this inpatient recovery stay was relatively short. She was discharged in three days. She is now recovering at home and is doing quite well.

Please keep her in your thoughts and prayers throughout the next six weeks as we pray this surgery is successful and she will not need a shunt.

P.S. In our next blog post, I'll fill you in on all the FUN and exciting things Sadie has been doing this summer (trips to the Museum, the bienniel Aicardi Family Conference, etc.)