My Own Dose of Perspective

So, I was feeling a little guilty after my last blog post. You see, I didn't want anyone to think I was making reference to them when I was speaking about perspective. It was more of a general statement, not referencing any one person in particular. But that's the problem with generalities, folks tend to think you are talking about them. That was truly not my intention, so if I offended anyone, I apologize. Shortly after I posted that last blog entry, I was given my own dose of perspective.

We attended a holiday party put on by a great organization called Hope's Friends. (The photo above is from that party, with Sadie looking inquisitively at Santa.) The organization, under the Hospice umbrella, provides palliative care to children with life-limiting conditions. Attending the party were children with a variety of different diagnoses and conditions. It was a great reminder that even we need a little perspective from time to time.

Sadie has had a few busy weeks as we wrap up 2011. In addition to the holiday party mentioned above, we've spent time with our dear family and have gotten together with a few close friends for the holidays - all of whom showered Sadie with love and affection. She had her monthly visit to Lekotek (the special needs toy-lending program) where we picked out some great toys for her to play with over the holidays. We also heard back from our local park district, who agreed to install a special needs swing for Sadie at our neighborhood park. We're looking forward to warmer weather so she can enjoy her first time playing on a playground!

As we wrap up another year, I am reminded of all the wonderful people and organizations who have helped our family. From the friends and family who sat bedside with us during Sadie's five hospital stays this year to the volunteers who helped with caring for our lawn while Sadie was in the hospital. We are eternally grateful for everyone's support.

Here's to a happy and HEALTHY 2012.

My Problems > Yours

Recently, while speaking with a group of fellow special needs moms, the topic turned to our shared exasperation at some people's lack of perspective. These moms have kids with some of the most complex medical conditions - feeding tubes, daily seizures, non-verbal, non-mobile, oxygen requirements, etc.

One mom recanted a tale of a text message she received while she and her husband were writing up their daughter's DNR (Do Not Resuscitate) orders. The message of complaint from another mom detailed how her power had just gone out and she and the kids were forced out of the house to wait it out at the local Rainforest Cafe. My fellow special needs mommy wrote back, something to the effect of..."I'm sorry your day has been so hard. We're writing up our daughter's DNR Orders. Talk more later."

We all know these martyrs. The people who respond with a long sigh when you ask how their day is going. Always reminding you of their hardships, trials and tribulations. Even those who have legitimate things to complain about, they're just no fun to be around. I vowed never to be one of them.

Now, I realize that what may sound relatively trivial to me, is in fact, a big deal in these martyrs' lives. But, by about the sixth time I'm hearing about how they're just so worried that little Johnny is still using a pacifier, my head is about to explode. I want so badly to bring them back to reality and remind them that while they are googling the long-term dangers of pacifier reliance, we are debating whether or not we are going to have someone cut into our daughter's skull.

A good friend of mine suggested that this is exactly how I should respond. "Give them a little perspective," she said. No, instead I typically excuse myself from the conversation before I say something I'll regret.

Besides the lack of sleep, our little Sadie has been doing OK for the past few weeks. We are still dealing with seizures every day - but no diastat seizures for about 3 months now (quick prayer to seizure gods). She's still doing well OFF oxygen as well. Our recent challenge has been with feeding. As I mentioned in a previous blog post, we've been to see a second feeding therapist at Children's Memorial Hospital. She's provided us with a few additional tools to use with Sadie during her feeding times - such as a chewy tube (which promotes a natural chewing pattern), a maroon spoon, and a NUK brush.

These tools have provided for a bit more cooperation during feeding, but she'll still have quite a few mealtimes where she simply does not want to open her mouth and/or swallow her food. We've also been slowly introducing Sadie to a sippy cup during her feeding times, but some days she just has an aversion to any type of feeding. Luckily, we've still been able to keep her hydrated and get her daily calories in by using her bottle. We're just keeping at it.

In January, we will visit the docs at Cleveland Clinic again to talk about a possible surgery for Sadie. We were given the option to have surgery performed on this same trip, but decided that we'd need some time to process the conversations to make an informed decision. In the meantime, we're looking forward to spending a lovely Holiday Season with friends and family. And, when I encounter one of these martyrs, I'll just need to remind myself that it's not what happens to you, but how you react to it that matters.

P.S. The photo above is of Sadie meeting Santa for the first time - you can see she's overjoyed!

Giving Thanks

I recently read this post by a fellow parent, and felt inclined to share during this season of Thanksgiving (complete with a little tongue-in-cheek humor).

Ten Reasons to Give Thanks for Your Child With Special Needs (by Terri Mauro)

As if you need a reason! Parents of children with special needs know more than most that every child is a gift worthy of thanks. EVERY ONE, the ones who thrive and the ones who strive, whether they become our legacy or we become theirs. But on those days when there doesn't seem much to be thankful for, or others are suggesting that your child must be just a burden, or well-meaning charities suggest giving thanks for healthy kids, check this list for a smile and a little inspiration.

1. You never have to worry about worrying over nothing.
Let other parents obsess over the frivolous and the shallow. Your child will make sure you always have something worthy to worry about.
2. Developmental delays = more years of hugs, kisses, and little-kid sweetness.
My 13-year-old still wants to sit in my lap, give me hugs, and tell me he loves me. What mom of a sullen teen doesn't secretly wish for the same?
3. Maybe someday, Ty Pennington will come build you a house.
Hey, Extreme Makeover: Home Edition loves families of children with special needs. Your little one may be your ticket to a lavish living space.
4. Any little milestone is a cause to throw a party.
Your child works hard for every step, sit-up and syllable, giving you lots to be excited about.
5. Every day is a learning experience.
Some days it's a pop quiz, some days it's a crash course, but life with your child is always an education, for sure.
6. You have the privilege of putting several doctors' children through college.
After paying for all those appointments, you may feel like a one-family scholarship foundation. Put your child's name on some letterhead and take pride.
7. You meet a better class of parent in waiting rooms and support groups.
Your child frees you from having to hang out with those snotty parents on the playground, and gives you entry into an exclusive club of people who are sensitive, sarcastic, and sure of their priorities.
8. You have an iron-clad escape excuse for any occasion.
You'd love to stay at that boring party, crowded event, endless church service, but, you know, your child just can't tolerate it. (And if sometimes it's you who can't tolerate it -- who's to know?)
9. Coming up with new strategies every day keeps your brain sharp.
They say doing crossword puzzles helps ward off Alzheimer's. Figuring out your child's schedules and treatments and lessons and rights and restrictions must easily provide twice the protection.
10. Your blessings will always be fully counted.
Other parents may take the gifts that their children bring for granted. Not you. Not ever.

The Beat Goes On

At this week's 18 month appointment with Sadie's pediatrician, I saw that her file had been upgraded. Most of the other kids' records are held in file folders. Sadie's records are now held in a large 5-inch three-ring binder. Luckily, we've made only a few additions to that binder recently, as the past few weeks have been relatively stable for Sadie. So we thought we'd take this opportunity to update you on what is going on with certain aspects of her life and general health.

Seizures - Seems like the logical place to start, since they (unfortunately) are such a big part of her life. Sadie is still having between 5-10 clusters of seizures a day. Mostly occurring when she is waking up. The clusters can last up to 10 minutes and vary in intensity. Relatively speaking, this is a better place than where she has been previously. We've now tried nine seizure meds (yes, nine), and have come to the conclusion that it is unlikely that we'll see significant improvement with medication changes alone. So we have been exploring surgical options. More on that to come.

Oxygen - Sadie has been off oxygen for about 2 months. (Yay!) She receives chest physical therapy (CPT) as well as an inhaled steroid (Flovent) twice a day. Chest PT is administered by tapping on her lungs with a little oxygen mask. It works to keep her lungs open and air moving freely. It's such a relief not to cart around oxygen tanks everywhere we go! We've even weaned ourselves off the pulse oximeter monitor (during the day, at least, she's still on it at night).

Eyes - Sadie's last MRI showed a growing cyst behind her right eye (the smaller eye). Her opthomologist will monitor the cyst by viewing her MRIs every 6 months. He is not terribly concerned and advised us that if it does create a problem, he can easily drain it. We did discover some good news recently with the help of Sadie's vision therapist. We now suspect that she does have some light perception in her right eye. Her therapist patched the eye with good vision and waved a light stick in front of Sadie. She promptly reached out and grabbed at it - much to our astonishment. Go Sadie!

Therapy - Sadie's therapy schedule is still going well. She is making strides with her therapies, and we've continued music therapy, and even added a massage therapist once a month! (Too bad massage therapy isn't also for mommy!) Sadie has also been using her stander during physical therapy. The stander is equipment that keeps her upright in a standing position. She stands for about two hours total every day. Because she isn't bearing weight on her own, the weight-bearing position helps for better development of her bones and joints. (The photos above show some of Sadie's activities during physical therapy (standing, sans stander) and swinging in the gym at the therapy center.)

Ketogenic Diet- Sadie has now been on the Ketogenic Diet for the past 15 months. We continue to measure out her food to the precise gram. She is taking her bottle pretty well, and we'll start to transition her onto a sippy cup soon. We've hit a stumbling block trying to get her from pureed foods to foods with a bit more texture. The high fat ratio of the Ketogenic diet has provided some challenges with transitioning to foods with more texture - there are only a few things that have such a high fat content. We're using a few recipes (such as a pancake made with her KetoCal powder, or a little bit of chicken mixed with mayo and avocado). She'll hold the chicken or pancake in her mouth, but won't chew or swallow. We're seeking out some supplemental feeding therapy from Children's Memorial to see if we can overcome this hurdle.

Sleep - Sleep is still a major challenge with Sadie. Her naps during the day rarely last longer than 20 minutes, then she is awakened with a seizure. After 18 months of not sleeping through the night, we put her on a medicine called Neurontin, which has helped slightly. She is still awakening at night with seizures, but will typically go back to sleep. Unfortunately, she rarely sleeps past 6 am. The past few nights, she's been up for the day at 4:45 am and 5:30 am respectively. We are considering hiring a night nurse to monitor her seizures at night so we can get some rest.

Surgery Update - We're still waiting on some test results requested by the doctors at Cleveland Clinic. We're planning another visit to Cleveland in the next month for a consultation with the doctors. If everything goes well, we'll likely schedule Sadie's brain surgery for the beginning of next year.

We're hoping that the upcoming Holiday season continues to proceed smoothly for Sadie. Please pray for no hospital visits or unexpected illnesses. We want to keep that medical record binder as thin as possible!

And the Verdict Is...

Rather anticlimactic. We heard back from Cleveland Clinic a few weeks ago. The docs want to conduct more testing on Sadie to explore the possibility of a metabolic or mitochondrial disorder on top of her Aicardi diagnosis. I mentioned to the doctor that we've gone down this rabbit hole before and come up empty-handed. See previous blog post. So, we're in another waiting game as they review the testing that was done on Sadie a few months back.

Ultimately, Cleveland Clinic believes that they could remove part of her right hemisphere of her brain, the area with most malformation, and yield positive results. However, they want to explore the metabolic/mitochondrial stuff before proceeding. This means we have a little more time to make what will likely be the most important decision of our lives. Unfortunately, it also means that Sadie may need to go through another round of testing. In the meantime, we are becoming more and more impatient as we await doctors to review tests. Have I mentioned that patience is not a virtue I possess?

In the meantime, Sadie is rolling like crazy, and has even started to put some weight into her knees and get her bum up in the air...which is a precursor to crawling! These recent acheivements are likely due to her weekly therapy schedule, and some new therapy gear.

Every morning, Sadie "suits up" in her therapy gear - she wears a SPIO vest under her clothes. The SPIO vest is a compression garment that looks like a little scuba suit. The vest provides her with compression on her trunk so she can start learning to utilize her muscles to sit up on her own. She also wears fancy shorts called "hip helpers." The hip helpers look like biker shorts that are connected in the middle. They help kids with low muscle tone to help their hips develop properly. Finally, she just got AFOs (Ankle Foot Orthotics). They are leg braces that will help Sadie as she begins to bear weight on her feet. Sadie's are pink (of course.) She wears all of this therapy gear every day under her clothes. While it is a pain to put all of these pieces on each morning, we know that they will help Sadie as she grows and develops.

Sadie has also undergone quite a bit of testing this month. A few weeks ago, she had a Urodynamics Study. Her urologist ordered the study to investigate why she has had recurring UTIs (four this year!). We are still awaiting the results, but based on the lab techs comments, she does not reflux urine into her kidneys (this is a good thing), and she is emptying her bladder completely (also a good thing). However, she does tend to hold A LOT of urine. She was able to hold close to 6 ounces during the test.

Sadie also finally had her FEES study. We had to reschedule a few times while Sadie was in a rough patch with her seizures. Similar to a swallow study, the FEES study evaluates swallowing of food and liquid. Sadie performed remarkably well during the study. A small camera was inserted through her nose and down her throat, the camera then watches as she swallowed liquid and baby foods. Unfortunately, the FEES study found that she still aspirates thin liquids, but luckily, she is still able to handle her thickened formula and pureed foods quite well.

On a sad note, we learned this week that another Aicardi family lost their little angel at the age of 12. It always hits us hard to hear this. Additionally, an Aicardi family who we are close to are again in the PICU with their little one. Dad writes a lovely blog, which I have shared before. Sadie has coded once in her life - in February when we were in the trauma room of the ER - and I hope it never happens again. This father's recent post speaks of the crazy place in which we live where we hold our breath each time we step out of our child's hospital room.

We are again reminded to appreciate the little things in life - like Sadie's beautiful smile, the joy we get seeing her roll onto her tummy, and her feisty nature. It makes the waiting game with the doctors a little more bearable. And we are grateful for each day we have to share with her.

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.

That's How I Roll.

Well, we figured out the mystery from the last blog post. Like I mentioned, for the past few weeks Sadie had been acting restless and was just not her smiley, interactive self. Feeling exhausted and defeated, we took her to the ER at Children's upon the advice of her pediatrician. Boy am I glad we did. Her bloodwork showed that her Dilantin level was 3x higher than it should be. Dilantin toxicity is the official term. Symptoms include darting eye movements, dizziness, nausea, etc...this explains why Sadie had been out of sorts.

She was admitted to the cardiac floor where they did a STAT EKG to monitor her heart. She was in the hospital for a total of 4 days until her Dilantin level dropped back into the normal range. Dilantin is a tricky medicine to get a good level in kiddos due to the fluctuation in metabolism. But, we are hesitant to give it up since it's one of the few medicines that has had a positive effect on her seizures.

We are excited to have our Sadie back. She has been giving Mommy and Daddy wonderful toothy grins lately. While visiting her Grandma last weekend, she rolled over for the very first time! Of course she did it while Mommy was out of the room...but we were able to capture it in the video above. Now that two of her grandchildren have rolled over at her house for the very first time, Grandma is convinced that Grandpa Chris in heaven is giving the babies a little nudge.

We've also just returned from our visit to Cleveland Clinic with Sadie. We'll post more about the experience later this week. For now, we're cherishing the moment we are in with Sadie.

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.

A Dose of Honesty

Recently, I received a Facebook message from a family who was considering adopting a newborn baby with Aicardi Syndrome. I don't know why the biological parents were giving up their daughter. Perhaps it was a decision they made before they realized this baby would have medical complications. Hopefully, it was not a decision made after the fact.

It struck me as amazing that there are people out there who would actually VOLUNTEER for this life. I came to the conclusion that this family must be truly special to accept a child with such severe special needs. As hard as it is to admit, if given the choice, I honestly can't say I would do the same.

It doesn't mean I don't love my precious Sadie for the special little baby she is. However, as she gets older and her disabilities are more visible, I struggle with how to respond to questions about her. After seeing Sadie in her baby wheelchair with an oxygen tank and pulse oximeter underneath, a man in the store asked "What's wrong with her?" I was taken aback. He likely was not aware of the insensitivity of his question. I stopped in mid-aisle and stood there for a few seconds, unsure of what to say. I finally uttered quietly, "Oh, she just needs a little oxygen," and scurried away before he could ask anything else. After thinking it through, a simple explanation "She has a rare neurological condition called Aicardi Syndrome," would have sufficed.

We did not ask for this life. Sometimes I feel like the deer-in-the-headlights cartoon character who is the only one in line who didn't take a step back. When people say "I don't know how you do it." I simply respond, "She is my daughter. You would do the same."

For the past few days, Sadie has been off the oxygen. She needed it, again, for a few weeks post-hospital. We have also seen some improvement with her seizure activity after a recent Dilantin increase. However, she is restless, not sleeping, and not eating very well. (Which means Mommy and Daddy are averaging about 4 hours of sleep a night.) If it's not one thing, it's another.

As for this special family and their unselfish desire to adopt this precious baby, the biological parents ultimately chose another adoptee family for the baby. I can only hope this special child went to a family who will cherish her for the beautiful angel she is.

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.

Cause and Effect

Why do certain things happen? Growing up, I would always ask my parents, "Why?" Their canned response, "Because I said so."

Why was Sadie given these challenges in life? As a parent, it's hard not to blame yourself. Maybe it is god's way of punishing me for being mean to that girl in high school? Maybe it was that one night before I knew I was pregnant where I had too many martinis? Maybe it's because I didn't spend enough time volunteering, going to church, caring for those less fortunate...you see how it can drive you crazy??

For two analytical individuals like Adin and me, these mysteries that happen with Sadie are even more frustrating. After a few relatively calm weeks (only 3-5 seizures a day), the random twitches have again returned. Our initial guess (unsteady Dilantin levels) has proved to be untrue, so we're now exploring a secondary hypothesis - that her new medicine (Ospolot) is the culprit, as the twitches have seemed to occur a few days following an Ospolot increase. So...we're now taking her off the Ospolot and trying what will now be our eighth seizure medicine, Keppra.

Keppra has been used successfully on many girls with Aicardi Syndrome. Many docs like the drug due to it's limited side effects. When the twitching was occurring every 5 minutes this weekend, we ended up in the ER where Sadie received a loading dose of Keppra. Loading doses of meds are often given in a hospital setting to immediately reach a therapeutic level. While we thought we saw some initial improvement in the ER, the next morning the twitching was back...again every five minutes. It's just maddening to have her on five seizure medicines and the ketogenic diet and to still be experiencing such high seizure activity.

The emotional side of me wants to just take her off of everything and start from scratch. The logical side of me realizes the danger in this and will just keep plugging away hoping that these rough patches even out once we figure out the right medicine cocktail.

In other news, Sadie had her first visit with an otolaryngologist last week, or more commonly known as an ENT (ear, nose and throat) doctor. He is recommending a FEES (Fiberoptic Endoscopic Evaluation of Swallowing) study on Sadie. It is similar to a swallow study in that the study evaluates the effectiveness of the swallow, but will also tell us more about how Sadie is handing secretions and if there are any other issues with her swallowing.

While we can't help ourselves to try to figure out what the causes are...we keep plugging along, hoping that this rough patch will come to an end soon.

P.S. I just HAD to include the photo above in her blog. Sadie and Baxter, sibling love.

It Gets Better?

When Sadie was a newborn, she and I would take daily walks around our neighborhood. There was a local park we'd circle every day with a playground, pool, and softball fields. After Sadie's diagnosis, there were some days when making that loop was just too painful. Every child jumping off monkey bars, running by the pool, pitching a ball - was a vivid reminder of my own child's limitations.

As Sadie is our first baby, Adin and I have no previous experience in typical child development. We only have the children of friends and family with which to compare. Now that summer is into full swing, we find ourselves at friends' barbecues, pool parties, and family gatherings. As I mentioned in a previous blog post, it's quite difficult NOT to compare your own child to others.

Friends of ours, parents of another Aicardi girl, compare it to "the bends." A feeling you get when resurfacing from a deep sea dive. You are thrown into an alternate reality, then just as quickly, you're catapulted back into your own reality. Psychologists call it "chronic sorrow," a pervasive grief experienced by parents of children with special needs. There is no "It Gets Better" campaign for parents of children with special needs. You're simply handed a copy of a poem about Holland and sent on your way.

Don't get me wrong, I LOVE seeing our nieces, nephews and friends' children, and am fascinated with how quickly they grow and develop. But will there always be a twinge of pain when seeing a child do something that Sadie might never do? Does this mean our friends and family should feel guilty for having healthy children? No - absolutely not. We simply hope they cherish every healthy, typically-developing moment.

And while I hate hanging out in the land of "I feel sorry for myself", perhaps I've been thinking more about this lately because Sadie has been experiencing one of the most rocky seizure periods she's ever had. Her Dilantin levels have been all out of sorts - high one week, low the next. We also started a new medicine, Ospolot, a few weeks ago. Since then, she's been having periods of time where all she does is twitch. Sometimes it's mild - just the foot and hand twitching in a synchronized pattern, other times, her whole body twitches. This can go on for over an hour. We tend to err on the conservative side when it comes to administering Diastat (her emergency med), otherwise, we'd be giving it every day. We have given it three times in the past two weeks.

Just when we thought things were starting to calm down, the seizures spiked again for no apparent reason. We also noticed that Sadie's heart rate was extremely high and she was running a temperature. Come to find out, Sadie had a urinary tract infection (UTI). We started her on antibiotics, but the fevers continued to spike. Back to the pediatrician a few days later and they gave her an antibiotic shot - to which she had an allergic reaction. Hives all over her arms and legs.

And, while we thought we had gotten rid of the nasal cannula, she's needed to be on oxygen for the past week. According to her pulminologist, this can happen when kiddos get sick. Grrrrr...the girl simply can't catch a break.

So, I'm patiently waiting for things to get better. Patiently.

One Year Ago Today.

We walked in for our appointment with the neurologist to get the results of Sadie's MRI. That morning, I had a sinking feeling in the pit of my stomach...I just knew that it was not going to be good news.

The neurologist who diagnosed Sadie was an extremely kindhearted woman who began by having us explain to her what had been going on with Sadie. She then showed us photos of Sadie's MRI and pointed out the various cysts and abnormalities in her brain. It was during this appointment that she told us Sadie had Aicardi Syndrome. As Adin wrote notes in our notebook, he asked her how it was spelled.

She gave us literature about Aicardi Syndrome and began to explain what it would mean for Sadie. As Adin and I read the markers of the Syndrome: missing corpus callosum, seizures, infantile spasms, retinal scars and micropthalmia, we thought, "Yes, that's Sadie."

I remember the neurologist saying that Sadie would only develop mentally to 6 months to one year of age and would only have a 40% chance of making it into her early teens. I had to ask her to repeat herself...as my brain wasn't able to process this so quickly. She stepped out of the room to allow us time to digest this information. When she came back, we talked about seizure medications and follow up appointments with neurosurgeons and epileptologists.

Driving home was a blur. Adin and I didn't say one word to eachother. All that we had imagined for our daughter - playing softball, soccer, singing in the school choir, going to her first prom - had been taken from us in that short one-hour meeting.

Once arriving home, we began the hard task of making phone calls to our immediate family. How do you begin to deliver such bad news? I would always preface the conversation with "You're not going to know what to say when I tell you this....and that's OK."

The next few days were filled with profound sadness. I awoke the next morning hoping the day before had been a dream. Unfortunately it was not. Our families were (and continue to be) our saviors. It was comforting to be surrounded by people who shared in the sorrow.

Little did we know what the upcoming year would have in store for us - therapy sessions, numerous doctor's appointments, seizure medicines, hospital visits, oxygen tanks. But the past year has also given us a beautiful little girl who laughs when mommy coughs, smiles up at us from her crib, gets cranky when she's tired, puckers her lips when her Aunt goes in for a kiss, and looks with surprise when Baxter cries over a squirrel in the backyard.

Sadie has helped us become more tolerant, patient, kind and forgiving. The past twelve months have given us a broader perspective on life and reminded us to cherish every moment, because you never know when it could be taken away.

The only thing constant

Recently, I've felt like the only thing constant is change. We're aching for some regularity in our lives. Seizures are changing, medications are changing, feeding is changing.

Some changes are for the better....

Like Sadie is now taking all her nutrition by mouth. NO MORE NG TUBE! She is excited about the bottle, excited about solid foods...NOT so excited by her medicine syringes. So we've been experimenting with different methods of getting the medicines in her belly. What complicates the issue is the thickening of the liquid. As mentioned in the previous blog post, Sadie's swallow study showed that she aspirates thin liquids and all of her medicines are thin liquids (compounded liquid or crushed tablets mixed with water.)

Another positive change is Sadie's EEG. She had another video EEG study done last week to look at her brain waves. Sadie's neurologist reported that her recent EEG showed "more organized brain activity."

We are also happy that Sadie is slowly shedding her cords. She has been doing great off oxygen during the day. She even gets through her daily naps off O2. She is, however, still needing a trickle of oxygen at night. But we hope to get rid of the nasal cannula soon! I can't say when we'll be comfortable taking her off the pulse oximeter. There is a certain level of comfort in knowing her heart rate and oxygen levels at all times...but I know that in order for us to live a more "normal" life, we'll have to get rid of it soon.

Some changes are for the worse...

Sadie's seizures are still a little out of control. We've had to use diastat five times in the past six weeks. The most recent episode happened while her aunt and I were out shopping. Sadie began to have a seizure that lasted upwards of 20 minutes. We quickly headed to the car and administered the Diastat in the back of the minivan. As we headed home, her O2 levels dropped quickly and she became unresponsive. We gave her some emergency oxygen, but the O2 levels only rose into the mid-90s. So, we again found ourselves in the ER at Lutheran General. By the time we arrived, Sadie's O2 levels were back up, but she was a little woozy from the Diastat.

Luckily, this time we were released within a few hours. The docs found that Sadie's Dilantin level was high (33.6). Therapeutic range is from 10-20. If the level gets too high, it can cause increased seizures. So, we're working with the doc to reduce her levels. We also started a new medicine this week, Ospolot. Ospolot is not yet FDA approved in the US, but is used to treat seizures in Europe. Clinical trials look promising for Sadie's seizure types. One of Sadie's Aicardi friends, Josie, has used the medicine for years and it has worked well. We are hopeful that this new medicine will also work for Sadie.

We had Sadie out and about in the past few weeks. The photo above shows us at the finish line of the Epilepsy 5K Walk. Sadie also enjoyed her very first tea party for her friend Carmen's fourth birthday! The photo above shows Sadie in her fancy party dress with her Mimi. (Can you tell she likes her hands?)

The changes remind us not to get too comfortable. So, Sadie's journey continues as we change course...and change course again.

The Little Things. A Dash of Realism.

Much has happened in Sadie Land since our last blog post...swallow and sleep studies, a glorious period of seizure freedom, and a lot of new activity.

Sadie had her sleep study last week. The premise of a sleep study is to evaluate sleep patterns and determine if any sleep disorders (such as apnea) exist. As you can see by the photo above, multiple wires and sensors are placed on the body to evaluate breathing, leg movement, jaw movement, EEG patterns, etc. It's no wonder that Sadie slept horribly during this study. She was also recovering from a bad cold, so she was already a little congested. She slept about 4 hours that night.

When the nurse called to give the report, she informed me that no apnea was detected, but Sadie needed a little bit of oxygen (big surprise) and never got into REM sleep.

I asked, "Does apnea sometimes show up only in REM sleep?"
Nurse: Sometimes
Me: So do we have to repeat the sleep study once she's better and off oxygen?
Nurse: That probably isn't a bad idea.
Sigh...

I also asked for a copy of the report. It amazes me how much medical jargon is included in these reports. You'd think it was a contest between doctors. Example, the title of the sleep study report - "Nocturnal Polysomnograph Report." Interpreter, please.

Sadie's seizure activity has been all over the place in the past few weeks. She'll have a few good days, then a few really bad ones. Strangely enough, two weeks ago, the seizures stopped entirely. No medications were changed, nothing...we had 6 beautiful days of seizure freedom. Then the seizures came back 7 days later. We had to administer diastat (emergency med) after she had a long seizure that didn't go away after 40 minutes. It was a hugely disheartening.

Because the seizures now are looking a little different than even a few weeks ago, the doctor ordered another video EEG to determine if she is having different seizure types. This information helps the docs determine which medications will work best.

In positive news, Sadie is slowly coming off oxygen. She can go most days without. We do still have to put her nasal cannula in for naps and at night. Sadie has also delighted us the past few weeks by doing some pretty amazing things.

Reaching for and grabbing a toy - The past few weeks, Sadie has gotten pretty good at reaching and grabbing. I know she is interested in a toy when she lifts her right arm immediately when I bring the toy in front of her. She has started to grasp a few toys as well, even pinching some between her thumb and finger. This is a huge accomplishment. Previously, she wouldn't grasp at all and toys would simply fall out of her hand. She's still working on her grasp with the left hand, but I think she'll get there.

Because of this recent interest in toys, we've enrolled Sadie in Lekotek. Lekotek is an organization that helps families with special needs children learn how to play with toys. They have a variety of different toys that are more accessible to kids with special needs. Sadie has a hard time playing with toys due to her low muscle tone and lack of coordination. She can't press buttons on toys like most kids - many of the Lekotek toys are attached to switches which make them easier for Sadie to use.

Rolling over (almost) - Sadie can now get herself up on her left side. It's actually her preferred sleeping position. She'll wind up and flip her right arm and leg over her body - she has yet to fully roll onto her belly, but she has gotten her entire body over except for her head.

Eating - Sadie actually cooperated and we got through swallow study last week. The good news is, she was awake and swallowed her food. The bad news is she is aspirating some of it. The x-ray showed that some of her formula was getting into her lungs. The solution for the time being is to thicken her liquids. We are using a thickener called Simply Thick, which makes the formula the consistency of a thick nectar. The thicker liquid is more difficult to aspirate. We have an appointment to visit a feeding clinic at Easter Seals this week, and we'll work closely with her feeding therapist to tweak her feeds in the coming weeks.

We started to feed her orally the evening after the swallow study. She was a champ with the solid foods and took them great. Tears of joy filled my eyes as she began to suck on her bottle again. For parents of "normal" children, these proud tears are reserved for graduations, little league games, piano recitals, first proms. As parents of a special needs child, it's these small things that keep us going and give us hope.

There many people who say "you'll never know what she'll be able to do." And, while we appreciate the optimism, we have to temper it with a dash of realism, otherwise, we'd be constantly disappointed.

For Sadie, it's the little things that make us so happy - (almost) rolling over, giggling, sucking her bottle, reaching for a toy, grasping an object. We hope for more of these small achievements in the coming weeks.

Sadie Time

We always joke that Sadie works on her own schedule - "Sadie Time." I have to laugh when people ask me questions like "When does she typically nap?" "What's her schedule?" The girl does what she wants, when she wants. We do our best to have her awake and alert during the day, and keep her sleeping at night - but most of the time, even this proves to be a challenge.

Case in point, we've now had to reschedule her swallow study twice. The first time around, she came down with a cold, so we cancelled and rescheduled for a week later. The second attempt proved beyond a doubt that Sadie is in charge. Despite sleeping relatively well the night before and getting in a good morning nap, she simply refused to wake up for the 1 pm swallow study. We tried taking off her shirt, putting a wet washcloth on her face, turning the lights up high, playing music, etc. The more we tried, the more she slept.

So frustrating when there are three technicians, a speech therapist and doctor in the room all asking why she's not waking up? Isn't she hungry? Did she not get a good nap? I wanted to scream at them and tell them that she does things on her schedule - and four seizure medications doesn't help either. Arrrghhh...

We attempt the swallow study again on Tuesday. I'm fearful because she hasn't taken anything by mouth for the past month, that she's lost some of her swallowing ability. I guess we'll know for sure on Tuesday (if Sadie cooperates.)

Sadie has been having a hard time keeping her oxygen levels up on her own. This week will mark one month on oxygen and the NG feeding tube. We had been slowly weaning her off the O2 when she came down with a slight cold and we had to boost her up again. After speaking with her pulminologist last week, she suspects that Sadie's low O2 levels may be the result of her having a difficult time coughing stuff up. Apparently this is a problem for many kids with low muscle tone. I have an appointment with a respiratory therapist next week to learn how to do chest PT, which will help with this. My nursing repertoire expands.

The pulminologist also suspects that Sadie may have sleep apnea. So, they will conduct a sleep study in the next few weeks where they will observe her overnight. Perhaps this will finally answer our question about her horrible sleep patterns.

If that wasn't enough, this past month her seizure activity has been up and down. She'll go for a few days having very low seizure activity (2-3 is a good day for Sadie.) Then, out of nowhere, she'll have a day where she'll be seizing every 3 minutes. We've had to give diastat (her emergency medication) twice in the past month. The seizures have been looking a little different lately as well. Instead of her typical infantile spasm "crunches," she's been having seizures that look more like your standard seizure (some shaking/trembling, eyes rolling). These look a little more frightening than her old seizures.

I was a little (OK, a lot) disappointed that Sadie had all of her cords attached for her first birthday party. But as you can see in the photo above, she looked adorable and had a wonderful time with friends and family.

We forge ahead, hopeful that we can get her off of oxygen and the feeding tube very very soon. Which, Sadie will do when SHE is good and ready.

P.S. Sadie's Journey now has an option to "Follow by E-mail." To receive e-mail updates with new blog posts, enter your e-mail address in the field to the right.

One Year Down. Sadie by the Numbers.

One Year Old
365 Days
3 hospital stays
100+ doctor appointments
251 therapy sessions
15 different medications
2 intubations
5 days on a ventilator
5 prosthetic shells

Countless smiles, coos, smooches, and loving glances

Happy 1st Birthday darling Sadie.

The Wrong Side of the Crib. Team Sadie.

Since Sadie's been home from the hospital she's been a little crabby. Adin likes to say that she woke up "on the wrong side of the crib."

She is still attached to three cords - one for her oxygen, another in her nose (NG tube) for feeding, and a third attached to her foot (her pulse oximeter which monitors her O2 levels) Most likely, she is mad about all the darn cords attached to her. Although she may also be pi*&?ed that she can't wear her cute outfits. Her current attire is old sleepers with one foot cut off. You see, she currently can't wear clothes that go over her head due to her oxygen tube, and she needs one foot free for her O2 sensor. Thus, our one-footed sleeper invention. Adin says we should market it and make millions ;)

Our days have become exponentially busier as Sadie is always cycling between feeding time, oxygen monitoring, breathing treatments, and medication administration. Nursing was never a career I considered, and I'll admit, it's a little stressful. Good thing I love my patient. We are hopeful that we can wean her off of the oxygen soon, and the NG tube will go away once she shows no aspiration in her swallow study.

Our hospital experiences with various medical professionals have made us extremely grateful for the team we have in place to oversee Sadie's care.

Dr. Lori Walsh, Pediatrician - I knew Dr. Walsh was a keeper from the moment I met her in our prenatal visit. She was truly interested in Adin's PKU (Phenylketonuria). She even called at 8 pm one evening to discuss what she learned about the genetics of PKU. Dr. Walsh has been the key player in coordinating all of Sadie's care. She truly understands Sadie and her condition and has been the ultimate partner throughout Sadie's journey.

Dr. Srishti Nangia, Epileptologist (a long word for "seizure doctor") - Both times Sadie was inpatient at Children's Memorial, Dr. Nangia visited us every single day - including weekends. We've been on the roller coaster with Dr. Nangia since the very beginning. She helps us through ups and downs, listens carefully, and knows Sadie quite well. She is a wonderfully compassionate, thoughtful, and caring doctor.

Dr. Hawke Yoon, Opthomologist - We met Dr. Yoon when Sadie was just three weeks old. Our journey to her diagnosis started with Sadie's eye exam under anesthesia. He quickly won our hearts when we asked about the procedure. Not finding a piece of paper handy, he pulled out his pen and started drawing a diagram right there on the bedsheet. The first few months of Sadie's life, we saw Dr. Yoon almost weekly. And he just has a cool name.)

Dr. Tadanori Tomita, Neurosurgeon - Dr. Tomita monitors Sadie's choroid plexus papilloma. Sadie receives an MRI every few months to check the growth of the tumor. If it gets too large, it can cause a condition called hydrocephalus, which would require a shunt and brain surgery. We know that we are in good hands with Dr. Tomita.

Roland Scott, Ocularist - Roland Scott fabricates Sadie's eye prosthetic (conformer). He fits Sadie's eye very carefully, and given how sensitive her eye is, we see him quite frequently. She gets larger conformers every few months as she grows, and he is always very careful and patient as he molds and fits each of them. Ocularists are hard to find - there are only about six of them in the Chicago area. Roland is actually the second that we've seen as we weren't entirely happy with the first. We joke that his lab resembles Dr. Frankenstein's - pots boiling over, him in a white lab coat, but overall, we've been very happy with his work.

Robyn Blackford, Ketogenic Dietician - Robyn oversees Sadie's ketogenic diet. Even though she was booked with patients, she helped us get Sadie into the hospital to start the diet on an emergent basis when her seizures were almost non-stop. She is a wonderfully knowledgeable and kind individual.

Michelle Johnson, Home Nurse - It was Michelle who ordered Sadie emergency oxygen for us to have in the home. A week later, Sadie ended up needing that oxygen to get her through until the ambulance arrived. Had we not had the oxygen, Sadie would likely be in much worse condition. Michelle was introduced to us through a great organization called Hope's Friends.

Team Sadie will be growing this week, as she has appointments with a few new specialists - a pulminologist (a doctor specializing in respiratory issues), and a urologist. We hope the pulminologist will be able to offer some insight into why these darn respiratory viruses have hit Sadie so hard. And, although she is now pee-ing more frequently (pause for knocking on wood), we initially made the urologist appointment after she was holding her urine for up to 12 hours between diapers.

I'll brag about her therapists (who are also wonderful) in a later post. Although we would change it in a heartbeat, we are grateful that we have gotten to know such wonderful medical professionals and feel confident in their care for our darling daughter.

Sadie's first ambulance ride. Prayers, prayers, prayers.

Looking at the title of the last blog post, I hope I didn't jinx poor Sadie. After battling cold symptoms for a few days, Sadie awoke early Tuesday morning with labored breathing and a "barky" cough. We hooked her up to the pulse ox to check her O2 levels. The machine read 83%...we started panicking. Luckily we had the emergency oxygen her home nurse had sent over the week prior. We started her on O2 and she quickly rose to 100%. Adin and I knew that we had to call an ambulance to transport her to the hospital, as we couldn't take her without supplemental oxygen.

The ambulance arrived at 2 am, and delivered Sadie and I to the ER at Lutheran General Hospital. Ambulance protocol is to take the patient to the nearest ER. I was a little nervous being at a different hospital than Childrens, which we know so well. Although I prefer Sadie's doctors at Childrens, we've been quite pleased with the staff and facility at Lutheran General.

We are on day 4 in the pediatric ward. What started as cold symptoms turned out to be metapneumovirus, which is similar to RSV (the virus that hospitalized Sadie in January). Sadie is also showing some cloudiness in her lungs, which could be a mild case of pneumonia. The doctors are trying to figure out why these respiratory viruses are so hard on Sadie. They suspect aspiration, which is where some of the liquid one consumes goes into the lungs instead of the stomach. She will be having a swallow study to explore this further. In the meantime, all liquids are going through an ng tube (through nose, down throat, in tummy). She REALLY did not like that ng tube going in, and actually punched the nurse with her little bandaged IV hand.

Throughout her hospital stay, her seizure activity has been quite low. And, we finally were blessed with her first seizure free day*. I always feel like I need an asterisk after that statement, as it is possible she had a twitch here or there that I didn't see, or a mild episode in her sleep. The picture above is the page from Sadie's journal showing her first day with no seizure activity. We have been keeping these journals since she was 4 weeks old (we're now on journal #3.) Each page is a different day. We track the time and duration of the seizure. I used to count every single spasm per cluster, but once they got up into the hundreds it was just too depressing.

We also track her fluid intake and diapers in the journal (since she has decided to hold her pee for hours and hours, and poo for days. More about that in a future blog post.) We have also been advised to see a pulminologist to oversee Sadie's respiratory issues. Her team of doctors grows.

The analyst in me wants to overanalyze exactly why her seizure activity has been so low. (I see a spreadsheet in our future:) But for the time being, I'll be grateful for this blessing. Maybe all of your prayers are working. Keep them up!

Social Sadie. Warning, sickness ahead.

The past few weeks, Sadie has been quite verbal and is often taken to having long conversations in her crib in the middle of the night. She's not cranky or upset, she just wants to chat. She'll do this for hours on end, but simply prefers to do it between the hours of 12 and 4 am. We hope this bodes well for her future verbal ability.

She has also been quite active, and has gotten close to rolling over. She keeps herself entertained talking and moving, moving and talking for hours at night in her crib. Adin and I will alternate getting up to check on her. The conversation usually goes something like this.

Cathy: Honey, can you get up and check on her?
Adin: Arrgh...
Cathy: I think she just said "daddy." She obviously wants you.
Adin: Arrgh... (rolls onto his side and climbs out of bed)

Seeing as how she likes to communicate with us, we've started to teach her sign language. We're currently teaching her two signs - "milk" and "more." We've done better with the "milk" sign, and swear that she is trying to do it on her own!

She also does a funny thing when she's dosing. Her eyes are little slits and we ask her if she's "fake sleeping." She typically responds with a big smile although her eyes are still mostly closed.

Speaking of sleep, we broke down and tried the melatonin one night. She slept for a record four hours (four hours, people!). Unfortunately, the next day was a crazy high seizure day for her. Its hard to discern whether it was the melatonin or another factor. So for the time being, we're holding off on the melatonin until we're at a more stable place with her medications.

Speaking of medications, Sadie is still on her four seizure meds (Topomax, Vigabatrin, Clonazapam, and Dilantin). The latter two are the ones that seem to be showing some positive improvement. We're still weaning Topomax, albeit very slowly. Vigabatrin will be weaned next. It will likely take us until June or July to get her off both, as long as we see no adverse effects.

Speaking of adverse effects, we've heard from many people that seizure activity often spikes right before a child gets sick. We think that is what happened with Sadie earlier this week. We spoke with the on-call neurologist after she had a really rough night and morning - seizures happening every 5 minutes. The neuro advised us to give her Diastat (emergency medication). This is a huge thing for us, as it was the first time we've administered Diastat at home. It was a little nerve-wracking, but it stopped the seizures. She, of course, was way out of it for the rest of the day. Not suprising, as Diastat is a form of Valium.

Because Diastat can suppress breathing, we hooked her up to her new pulse oximeter to monitor her O2 level. Pre-diastat, her O2 was about 95%, post-diastat she hovered around 85%. I was again on the phone with doctors, and was trying to avoid the dreaded "take her to the ER" response. (Sidebar - love love love the folks in the ER at Children's, but we feel like the past few times we've been there, Sadie's been hit by a wrecking ball as they try to get her O2 levels back up.)

So, we took her to the pediatrician. Luckily by that point, her O2 had gone back up into the 90s....whew - crisis averted. In the meantime, our lovely home nurse (who sees Sadie once every two weeks) had an oxygen compressor and tanks delivered to our home. So - we've added to our beautiful collection of medical equipment. Lo and behold, two days later, Sadie comes down with a horrible cold. The seizure increase served as our warning sign.

We forge ahead as Sadie approaches her first birthday at the end of this month. So hard to believe that our little girl will be one soon. Even through all the hospital visits, doctor appointments, therapy sessions, etc., she has been our joy and the light of our lives.

Gratitude.

Last week, friends of ours hosted a benefit fundraiser for Sadie. You can view photos of the event here. We cannot properly express in words our gratitude to those individuals who put this event together - Chip & Lisa, Jeff & Erika, and Josh & Heidi - we are forever indebted to you for offering up so much of your time and energy.

Proceeds from the fundraiser will go to Sadie's Special Needs Trust. The funds will be used to pay for anything that is not covered by insurance - prescription co-pays, specialized equipment, extra therapy sessions. While we are thankful that insurance is picking up a huge tab for Sadie's care, we are learning very quickly that there are a number of things that simply aren't covered.

A portion of the proceeds will also be donated to the Aicardi Syndrome Foundation. The Foundation connects families affected by this disease through a biennial Family Conference. They also assist with funding two major research teams who are doing research on Aicardi Syndrome.

Many, many other people also donated time, items for the auction, money - we will do our best to thank each and every one of you personally. We have been completely overwhelmed by the support we have received, and the people who have supported us...many of whom we have not seen in years (yes, you). It's beyond heartwarming to know that Sadie is loved and so many people want to help out. Please know that your donations will go to help our little girl live the most fulfilling life that she can.

Sadie's godmother always says, "that little girl is going to change the world." We think she already has.

If there's anything we can do (continued)...

As we learn more about Sadie's condition, we also learn more about our needs as parents as we navigate this new reality. So, as a continuation of our "If there's anything..." post, we decided to add a few more things to the list.

6) Advocate. We were disappointed to learn that Illinois ranks dead last in many services to individuals with disabilities. Read more about that here. (For example, Sadie is currently on a wait list of 20,000 people to get any sort of assistance for her disability.) Send a letter or e-mail to your Illinois representative about providing more programs and services for individuals with disabilities.
7) Give to Sadie's Special Needs Trust. We've set up a special trust for Sadie to pay for items that are not covered by insurance. (i.e. therapies, medications, special equipment, etc.) The trust ensures that any funds set aside to care for Sadie will not be counted against her once she turns 18, and will enable her to access public funds such as Medicaid and Social Security Income. It also will provide for Sadie's care if, heaven forbid, anything were to happen to Adin or myself. If you'd like to make a donation to Sadie's Special Needs Trust, please let us know. You can also donate with a credit card through PayPal (simply click the link on the right).
8) Attend a benefit fundraiser for Sadie and the Aicardi Syndrome Foundation. We are blessed to have great friends who are planning a fundraiser next week in South Bend, IN. It will be a fun party with great music :) Click here for more info. We hope to see many of you there.

Home Sweet Home. Sleep Sweet Sleep.

Sadie has been home from the hospital for about a week. She is still struggling with kicking this recent respiratory virus. At her pediatrician's request, she is now the proud owner of a pulse oximeter and a nebulizer machine. We will use the pulse-ox at home to monitor her O2 levels should she get sick again. We've been using the nebulizer machine to give her albuterol breathing treatments three times a day. We'll continue with the treatments until the virus works its way out and her breathing returns to normal.

While in the hospital, we finally weaned Sadie off of one seizure medicine. While Clobazam looked promising at first, it seemed to be having an adverse affect, as Sadie's seizures went up with each increase in dosage. Once we backed off the dosage, her seizure activity went down. We also started Sadie on Dilantin when she was having a rough time with seizures at the hospital. I hesitate to say that her current seizure medication cocktail (dilantin, vigabatrin, topomax and clonazapam) seems to be showing some improvement. The seizures are at their lowest level. She still has episodes daily, but we've gone from 20+ seizures a day down to 3-5. Our next step is to slowly wean a few of these meds that we're not entirely sure are working, something we've been wanting to do for quite some time.

We also received some troubling news a few months ago that I didn't feel comfortable blogging about at the time. The doctor noticed some new brain abnormalities in Sadie's recent MRI. According to the doctor, there were a few areas that showed some "disbursed white matter." She said the changes looked consistent with a mitochondrial or metabolic disorder. "Instead of her Aicardi diagnosis?" I asked. "In addition to her Aicardi diagnosis," she responded. Wonderful.

This time around, I did no googling, no researching...I simply just ignored the fact that our daughter was being tested for an additional disease and kept on living. As a friend so wisely stated, "no point in going down paths that aren't yours."

I've come to the conclusion that we are the big losers when it comes to the battle of the odds. If a medication has a extremely rare side effect, Sadie is bound to get it. As it turns out, that's what the doctors think is happening with these MRI abnormalities. So far, all the mitochondrial and metabolic tests have come back within normal ranges. So that leaves the medicine. Apparently, one of Sadie's seizure medications, Vigabatrin, can cause MRI abnormalities that are similar to those shown in Sadie's MRI. The problem occurs with high doses and when children are very young (check, check). The good news is these abnormalities often revert back to normal once the medication is weaned. So, the plan is to slowly get her off vigabatrin and hope it is a transient finding and the MRI goes back to its original state.

We are overjoyed to be home after our 7-day stay at Children's Memorial. Sadie has been delightful this week. Smiling, chatting, happier than we've ever seen her. She dove headfirst into her therapies this week and did a great job. The picture above was taken during one of her monthly music therapy sessions. She liked the rain stick toy, but she liked her therapist's diamond ring even more. Smart girl.

The only trouble we've had with her is sleep. The girl simply doesn't sleep. And when I say that, I mean she is up about every hour at night. I found that one side effect of dilantin is insomnia. However, I'm not willing to give up seizure control for sleep. Insomnia combined with the coughing make for long nights for the McCann clan. We've been encouraged by her doctors to explore melatonin, which is a mild, naturally-occurring sleep aid for children. With all the medicine that she's on, we've been hesitant to add one more thing...but give us a few more nights and we may be there. For now, we thank God for espresso.

Hospital, the sequel.

We again found ourselves in the ER at Children's Memorial after Sadie was coughing and struggling to breathe. Our ER visit began the same way as our visit last month. She was assessed by a triage nurse who raced her back to the trauma room when she saw her O2 levels at 80%. It was as if we had roles in a poorly-scriped sequel where the plot line is the same, but all the characters are different.

The doctors tried their hardest not to intubate her, but just couldn't get her blood pressure or oxygen levels high enough. So, again she was intubated and put on a ventilator. A scary procedure for us to witness, as they had trouble getting the tube in her throat through a swollen airway. We saw her oxygen levels dip into the 10s, twice.

We are now at day 6 of her hospital stay in the ICU. Sadie seemed to be recovering and in good spirits until yesterday when out of nowhere her seizure activity spiked. She was given three sedatives to get the seizure to stop...then another dose after another cluster started an hour later. One step forward, two steps back.

Our strong little girl continues her feisty behavior - pulling out her nasal oxygen tube, squirming out of her arm immobilizer. She also decided that the x-ray table was the perfect place for a poonami. We hope this bodes well for her release soon.

Perspective Redefined - Part 2

It's been a while since Adin and I attended the Aicardi Family Conference last July. I think we needed some reflection time before being able to properly communicate our experience. The Aicardi Syndrome Foundation hosts this bi-annual conference for families affected by Aicardi Syndrome. As fate would have it, it just so happened to be three weeks after Sadie was diagnosed, and in the western Chicago suburbs.

We received a call from another mother encouraging us to go. We weren't sure if we were ready to face this reality, but decided that it would definitely be helpful for us to learn more about this new world we were about to enter.

I still remember our first day at the conference. Pulling into the hotel parking lot, I had ideas of what I might see - lots of handicapped vehicles, girls in wheelchairs - my heart was beating out of my chest as we entered the registration room. Everywhere we looked, there were girls affected by the Syndrome who seemed very limited in their abilities. Many were unable to walk, speak, hold their heads up independently...it was very emotional for both of us. As another new parent so appropriately put it,"It's like looking into a crystal ball...but you don't like what you're seeing."

We were greeted with open arms by the families. We were so grateful to attend this conference and learn more about Aicardi Syndrome, as well as the many issues that we will be dealing with as Sadie gets older - advocacy, special needs trusts, therapies, specialized equipment, etc. But most of all, it gave us a support group of familes going through the same thing. We met some great people who we will keep in touch with for many years.

When you're raising a disabled child, your perspective of others with disabilites is quickly adjusted. By the end of the three-day conference, I realized that I was no longer seeing these children in terms of what they could NOT do, and instead was seeing what they COULD do.