Thursday, August 28, 2014

Do You Believe In Miracles?

On Monday we met with Zacchaio's neurologist, Dr. Wyllie, whose recommendation was to cease the discussion about a second surgery, start the anti-epileptic drug Vigabatrin to control his ongoing seizures, and discharge us home. Her intentions were to allow Zacchaio time to grow and develop, and to have him return for his second surgery once he was bigger (greater blood volume equates to a less risky surgery) and his seizures had worsened. At the end of our discussion I was left with a sour taste in my mouth. If we have to start Vigabatrin, why did we remove a quarter of our son's brain? We could have started Vigabatrin before touching him with a scalpel. Why are we toe-dipping in two different pools of epilepsy management? When we set out on our cross-country trek a month and a half ago, our goal was seizure freedom, not seizure betterment. Aidan and I were all in, and stopping now felt like half-assing it.

Zacchaio's neurosurgeon, Dr. Bingaman, was scheduled to meet with us shortly after Dr. Wyllie's departure. He did his usual: dramatic entrance, sarcastic remark, sit down, cross legs, frank conversation. It was as if he had a direct line from my brain to his lips (neurosurgeon's intuition?)...

"Alexis, I know we said we were going to go back in to finish the hemispherectomy if he continued having seizures...

"Well, what Dr. Wyllie was saying was-"

"You're unhappy with what she said. Unfortunately, you were born with the qualities you were born with." Dr. Bingaman's sarcasm at its finest. "You're a fixer by nature and you want to fix him. At the end of the day you want to say you did everything in your power to give him the best chance at developing and leading a seizure-free life."

"Right. And if it proves to be unsuccessful, I-"

"You're prepared to deal with it."

"I'm prepared to deal with it. Because regardless of how this turns out, we're going to be dealing with a whole lot of something for the rest of his life."

There was a longer than expected silence...

"Just to let you know, if I were you I would be doing the same thing."

"Thanks, Dr. Bingaman."

He proceeded to explain the surgery...

"We're going to completely remove whatever is left of his parietal lobe (his sensory and motor cortex).  He's obviously going to be paralyzed on his left side after we remove it. He'll eventually be able to move his shoulder and elbow, but he will have no fine motor movement in his left hand. He'll walk with a noticeable limp for the rest of his life. Back to surgery. I'll trace around the frontal lobe to complete its disconnection but I'll leave the brain tissue in place to reduce the chances of hydrocephalus developing."

"Gee, I was going to ask if you could leave it. Guess we're on the same page?"

"How does Thursday sound?"

"Wow, um. Thursday it is. Hey, might we be able to-"

"Sure. Let's go ahead and do some EEG before Thursday. We'll put it on now and monitor for 2 days? We'll make a final decision on Wednesday and I'll pencil him in the surgical schedule for Thursday. Sound good?"

"Sounds great. That way we can compare-"

"We'll compare it to the post-op EEG we did a week ago. More information. Good?"


And that was that.

I've never seen hospital time in such fast motion. Zacchaio's personal EEG tech, Betsy, had electrodes  decorating his scalp within an hour. It was very clear that when Dr. Bingaman issues orders, everyone's ears perk up.

By Tuesday, Zacchaio had been monitored for seizure activity for twenty-four hours. Overnight, Aidan diligently pushed the seizure button every time he witnessed a strange eye movement or behavior. Both of us assumed they would end the EEG monitoring a day early, as we had captured all of the behaviors we had been concerned about over the last few days. When neurology briefly popped their heads in, they asked if we could continue monitoring for one more night. We gladly obliged, though I'm not sure Zacchaio was as thrilled to give them the extra day.

And so after forty-eight hours of monitoring, our epilepsy team walked in. Everyone was in agreement. No surgery.

What? No surgery? I thought we discussed this!

A smile started to form on Dr. Gupta's face.

"Alexis, he hasn't had a single seizure for the last forty-eight hours."

There was a long silence while my eyes widened and awkwardly stared at him.

"Dr. Gupta, are you telling me that after surgery one week ago he was having as many as 400 seizures per day, and now he hasn't had one?!"

"That's what I'm telling you."

"I don't believe you."

"I thought you would say that."

I wish I could say he went on to explain how it was possible, but he couldn't. Aidan and I had been secretly in agreement that we weren't seeing as much abnormal activity in the last few days, but we were too afraid to admit it. We were both determined to be realistic about Zacchaio's condition and couldn't afford to have our hopeful subconscious mislead our neurology team. The intermittent eye blinks, eye glances, and startles we had been seeing in the last few days were definitely different than what we were seeing during Zacchaio's post-operative EEG one week ago, but we feared what that meant.

Dr. Gupta continued to explain that the behaviors we were witnessing are Zacchaio acclimating to his new brain (or lack thereof) and his new vision. Nothing he was doing was correlating to anything abnormal on the EEG. In fact, Dr. Wyllie kindly chimed in that while Zacchaio sleeps, his brain is doing all of the "smart things it should be doing," as in alpha, beta, and delta waves, spindles, etc.

It's a miracle?

I was still skeptical. I asked Dr. Gupta about Zacchaio's interictal spikes (brief electrical discharges). Prior to surgery he was having them regularly, every 3 seconds. After surgery he was having small, intermittent clusters of them. And now? He said they were very infrequent and if he had to quantify them, maybe there was one per hour. He smiled again and reiterated that spikes are not seizures, just brief surges of electrical activity that last less than a second. He went on to say that there is potential for Zacchaio to have these spikes for the rest of his life and remain seizure-free with no concern from neurology.

He said slowly one last time... "He is not having anymore seizures."

I never thought receiving good news would be so challenging. One of the longest, most frightening chapters in our lives just ended, yet somehow in this moment I'm more afraid of the truth on subsequent pages.

Whenever I fell to my knees and prayed for our Zacchaio, I never asked for big miracles with little sacrifice. We were, and still are, willing to permanently disable our son for the chance of seizure freedom. I just never imagined God would be this good to us.

Zacchaio's seizure freedom is still not a guarantee. He may seize again in days, months, or years from now. But not today.

Today Zacchaio was supposed to have surgery. Instead Zacchaio brought our family home.

Sunday, August 24, 2014

Post-op Days 7-10

We've been riding the status quo wave since Thursday, hence this entry entails a handful of happenings while on this ride that has required an immense amount of patience.

Post-op Day 7

We begin to see intermittent interest in toys!

Post-op Day 8

Three hours of sleep per night. Delirium at its finest.

Post-op Day 9

Zacchaio has remained very resistant to taking food by mouth and whether that is because of nausea, vomiting, or pain related to his incision, we will never know. We have since started continuous feeds through his NG tube and we are happy to report that he is tolerating them with no vomiting!

We also discovered that he would sleep for longer stretches of time when he slept in our hospital bed as opposed to his crib. It's all yours, Your Highness.

After having a check-in with Alkaio back home in California, tears among endless "I miss yous" led us to buy him a one-way flight to Cleveland the next day. Although our family is still not living under the same roof, I forgot how amazing it felt to be living within the same city limits. This morning when Aidan, Alkaio, and I had a family hug we said, "Everyone think of Zacchaio right now so this is a REAL family hug!" And it was perfect.

Post-op Day 10

It seems as if Zacchaio is starting to turn a corner... 

And while I witnessed the sparkle in Zacchaio's eye begin to return, Aidan shared a day full of laughter and joy with our big boy. Balance is feeling really good right now.

Wednesday, August 20, 2014

Post-op Day 5 & 6

Zacchaio's last couple of days have been riddled with much struggle and few triumphs.

Finding the balance between patience and excessive intervention for Zacchaio's healing has been emotionally draining. As a nurse, I know the mantra: "Intervention begets intervention begets intervention..." Finding the perfect cocktail of supportive therapies has been a horribly disorienting rat race.

Zacchaio excels at getting his needs met, and he very clearly (and loudly!) made it known that we were not adequately managing his pain. In response, we began around the clock Morphine. Although Zacchaio achieved moderate pain relief with the narcotic, it also inadvertently slowed down his bowels (as narcotics do by nature) which led to subsequent constipation and food intolerance. We combatted his slow digestion with three different GI stimulants. Unfortunately his persistent low-grade fevers seem to have negated what progress they had made. He has continued to vomit his milk which puts him at risk for aspiration, and he has continued to vomit his heart medications which has perpetuated his arrythmia. His minimal caloric intake is now preventing his ability to maximize wound healing. In an attempt to break the cycle of negative side effects related to interventions, we recently stopped giving Morphine and switched to Motrin (ibuprofen). The anti-inflammatory and pain-relieving propertiies of Motrin have proven to be very effective for Zacchaio. In breaking one cycle we might have started another, as Motrin is blood-thinning by nature which increases Zacchaio's risk of bleeding which will likely push his surgery to a later date (though this may be what he ultimately needs).

In attempting to support him through his initial recovery, we have created so many more difficulties. I was beginning to feel like I was losing all control. Enter inordinate amounts of carbohydrates and refined sugars. Thank you, Fudge Brownie, for getting me through a difficult time.

For 80% of the day he is visibly uncomfortable. Even with Morphine on board he still whimpers with discomfort from his lacerated temporalis muscle and upset stomach. On rare occassion when he manages to find calm, he opens glazed eyes and stares toward a seemingly insignificant point in space. In a selfish attempt to reassure my soul that our joyful boy still exists within his pain-ridden body, I try to engage him during these peaceful moments. It turns out that the seemingly insignificant point in space he has discovered is his happy place, a place on which he focuses with such vigor so as to forget about the hurt. Who are we to be impatient? Take as long as you need, sweet guy.

Prior to Zacchaio's surgery I subconsciously created an expected post-operative course based on that of others who have had similar surgeries. In the last few days I became disappointed with his somewhat flattened trajectory of healing. 

In needing to find a more productive approach toward his recovery, I am adopting the theory of tabula rasa (blank slate) in hopes of new perspective and opportunities to celebrate even the smallest of his successes.

The first of these successes occured on the morning of Day 5 when I rejoiced with tears. Zacchaio opened his eyes, gazed purposefully into mine, and responded to my greeting with a beaming smile. He said what I needed to hear most; "Mama, I'm still here." For the next few hours he responded to our signature sound effects with smiles and coos. The last time he was this receptive to us was months ago before this whole business of seizures. He remembered. He was really still in there.

On Post-op Day 6 Zacchaio participated in physicial therapy for the first time. Seeing as he was unable to take part in days past due to lethargy and pain, this was another success! Though a bit resistant to start, he embraced his physical therapy as his therapists embraced him. He visually tracked for the first time and reached for his toys with a tentative smile. Atta baby!

Though Zacchaio's seizures continue, we have noticed a surprising decrease in frequency. We don't want to set our expectations too high just yet, but we're obviously happy to see an improvement from his pre-surgical state.

Lastly, we received his post-operative MRI results that revealed nothing unexpected! There was no sign of stroke and no excessive swelling or bleeding present. Phew!

With such little progress made these last couple of days, it would have been easy to fall into despair. Though tears have been shed, the most recent that have fallen from our faces are not because of Zacchaio's struggles... They are because of you.

A sweet 12-year old is celebrating his entrance into his teens and has asked his guests to offer donations toward Zacchaio's journey in lieu of the joy of presents. Dearest friends continue to research and contact foundations on our behalf, inquiring as to how they can further assist with medical costs. Entire high school classrooms have paused in prayer, and prayer circles full of strangers continue to speak our baby boy's name. As you all continue to light candles worldwide, know that you are feeding our souls and Zacchaio's drive to heal. 

We thank God every night for you, our family.

Better days are on the horizon...

Monday, August 18, 2014

Post-op Day 3 & 4

Days 3 and 4 have been overwhelmingly volatile.

On Day 3 Zacchaio was either inconsolably screaming in pain, or asleep and constantly awoken by spasms. After increasing his Morphine frequency to every two hours, he found moderate pain control. 
Today on Day 4, he requires no morphine. His whimpers are quieted as I gently hum with my lips softly pressed against his cheek. 

On Day 3 Zacchaio's right eye remained large, bruised, and swollen shut.
Today on Day 4, definition in his eyelid returned.

On Day 3, Zacchaio had a continuous fever that necessitated around the clock IV Tylenol.
Today on Day 4, he has remained without a fever and has been without Tylenol for 14 hours. 

On Day 3 we attempted to give Zacchaio Pedialyte through his NG tube on account of his frequent vomiting of hospital formula. Although his vomiting episodes ceased while on Pedialyte, we found he was still retaining full volumes of fluid in his stomach six hours later. 
Today on Day 4 we began giving him medications to help stimulate motility in his gut. We will try to give him incredibly small (2mL) amounts of his own formula hourly to see if he is more tolerant of a familiar formula. There is no IV equivalent for his anti-arrhythmic medication, therefore maintaining therapeutic blood levels of it has been problematic with his upset stomach. His heart rhythm has become increasingly irregular.

On Day 3 he was still on an IV beta blocker to control his heart rate.
Today on Day 4 he is maintaining a safe rate without any beta-blocking assistance.

On Day 3 he remained lethargic and absent with his eyes persistently closed to us and the world.
Today on Day 4 he briefly opened his eyes, reached for and softly touched my face and in silence managed to say, "Hi mama... I'm still here." He fell back asleep immediately. With every passing day we don't see him, we fear he may not ever come back.

On Day 3 he continued having strange eye movements that the epileptologist said were highly suspicious of seizures. We placed EEG leads and waited.
On Day 4 they confirmed he's still having seizures. 

He's still having seizures. 

I could feel the large hope-filled balloon we came to Cleveland with losing air, and it was losing air so quickly that while we frantically searched for the leak we only kept losing more. Aidan and I found ourselves with wavering endurance for this new life of ours. 

The two of us had the opportunity to go on a 45-minute mini date while Zacchaio was in MRI. Our options were one of two restaurants in the clinic... How's that for romance? The quiet and intimate moment we managed to find felt disappointedly unfamiliar. Though we were enjoying a sit down meal together, somehow we both still missed one another. While we're together during the day we tend to Zacchaio. At night we take turns between staying at his bedside and sleeping at the Ronald McDonald House. The day we get to share a bed together is going to feel like our honeymoon! I'm looking forward to it. 

The current plan is to await MRI results and allow Zacchaio one week of recovery time, as most of his acute swelling will have subsided by then. It is at this point that we will gain a more accurate account of his seizure type and frequency. Following final evaluations we will make the decision as to when Zacchaio will go back into surgery to remove the remaining brain tissue of his right hemisphere, effectively completing the hemispherectomy we came here for.

Saturday, August 16, 2014

Post-op Day 2

Post-op day two has proven to be a difficult one.

Overnight, Zacchaio spiked a temperature as high as 103 degrees that has unfortunately persisted through today (minus intermittent relief from Tylenol). He also vomited an entire feed in the middle of the night and two more during the day. He continues to be very lethargic and has preferred to rest more than anything else. The good news is that all of his vital signs have remained stable and labs continue to look good; blood cultures are pending. Last night the neurosurgical team wasn't too worried about the fever, but as it continued into today they began having increasing concerns. The swelling in his right eye is much worse today and was very bothersome for Zacchaio. Aidan said, "Babe, everyone keeps calling him a fighter, and now he actually looks like one! What a shiner..." Normally the remark would make me at least grin or sarcastically roll my eyes, but today I'm finding it difficult to keep the mood light.                                      

Though his overnight concerns have me a bit down, I have to remember to celebrate the successes even when they're muddied with setbacks. Zacchaio became much less tubular today! His wound drain was removed (3 inches of tube under his scalp), his arterial line was removed, a peripheral IV was removed, and his foley catheter was removed. On account of the high fevers, they also removed his central line (long IV that ends in the heart), as such an invasive line poses additional infection risk. His head dressing was also removed which ultimately exposed his scar to us for the first time. It is intense. The average incision made for this type of surgery looks like a question mark extending up and around the ear. We requested that Dr. Bingaman take a different approach with Zacchaio since he will be having plastic surgery to remove his scalp nevus in a few months. In trying to minimize the number of scars on his scalp, we asked that the incision be somewhat of a tracing of Zacchaio's scalp nevus. Dr. Bingaman did exactly that. We now hope that Dr. Hoffman (plastic surgeon) will work his magic and revise portions of Zacchaio's current scar as opposed to creating new ones. Scar photos will be posted at the end of the blog post so those of you who have queasy stomachs can avoid viewing it.

After Zacchaio opened his eyes for the first time after surgery, we noticed that he had a strong right gaze preference. He would make attempts to track something from his right to straight ahead, though every time he tried his eyes would strongly pull back to the right. As of today he has started to find more neutrality with his eyes which is great progress. I'm looking forward to his right eye swelling subsiding so he can begin to explore more of his visual field. 

There have been a handful of times that we have noticed Zacchaio having eye movements that closely resemble pre-surgery seizures. While he's fast asleep, he suddenly opens his eyes widely as if startled, he gazes straight ahead for one second, and then closes his eyes while remaining asleep throughout the process. We were told prior to surgery to be prepared to witness breakthrough seizures post-operatively for a number of different reasons. It's difficult to know whether these slightly altered seizures are due to the swelling in his brain, a seemingly normal frontal lobe that is microscopically abnormal, or his healthy hemisphere having abnormal electrical activity. The neurologist mentioned that if it persists we could do a 24-48 hour EEG, though it is not standard procedure. We'll definitely be taking him up on that offer.

That's a wrap on Day 2. Praying for better days ahead for our baby warrior.

*** Post-op Day 2 Update ***

The day evolved from "not great" to "bad" to "worse." 

This evening Zacchaio's heart rate spiraled out of control and reached as high as 300bpm. Our best guess as to why is the persistent fever. Fortunately our subsequent intervention was successful, as starting him on a continuous IV infusion of a beta blocker aided him to regain rate control. We also decided to start him on around the clock IV Tylenol to help minimize the fevers and regular doses of Morphine for the extreme discomfort he appears to be in today. 

Zacchaio threw up again, and again, and again. It had been 10 hours since he had last taken formula yet he still managed to vomit a strange pasty brown substance. He is now NPO (nothing by mouth) and will remain hydrated exclusively with IV fluids. Just as we established his NPO status, one of his two remaining IVs began to leak/bleed. This was not good news, as finding IV access on Zacchaio is considered a superhero's feat. Prior to starting surgery on Thursday, it took our anesthesiologist and her team three hours to gain adequate IV access. We counted the stick marks left on the undersides of both wrists; 13 attempts in one arm and 15 in another.

Prayers for our little man to find peaceful rest and comfort. Prayers for his continued strength and patience as we await the healing of his little body.

Friday, August 15, 2014

Post-op Day 1

Zacchaio is a rockstar.

Following the conclusion of Zacchaio's surgery, we had the opportunity to speak with his surgeon, Dr. Bingaman. Surgery went very well with no unanticipated difficulties or surprises. For once Zacchaio was run-of-the-mill. We were overjoyed with Zacchaio's extraordinary ability to be average! ;)

Prior to beginning the posterior resection, Dr. Bingaman did an intracranial EEG (where electrodes were placed directly on the surface of Zacchaio's brain to record electrical activity) and saw precisely the same electrical patterns we've seen in previous EEGs (interictal spikes every 3 seconds, infantile spasms, as well as additional types of seizures). Following the partial removal of his right parietal lobe, and complete removal of his right occipital and temporal lobes, his intracranial EEG of his frontal lobe (the only lobe left) had changed. Ideally we would have seen NO abnormal electrical activity of the frontal lobe, as our hope was that the seizure-generating tissue lied within the posterior (back) portion of his right hemisphere. Dr. Bingaman saw some interictal spikes in the frontal lobe, however they were infrequent and not regular. He did not see any seizure activity. He did tell us that upon removing Zacchaio's occipital lobe he noticed it felt abnormally firm. This reiterated the fact that there was likely cortical dysplasia (abnormal neuronal migration), and reaffirmed that we made the right choice in having surgery.

I found myself a bit disappointed with the news... 

Dr. Bingaman was perceptive and picked up on my lack of enthusiasm surrounding surgery's outcome and did tell us this. It is still possible that all of the seizure-generating brain tissue was localized in Zacchaio's posterior right hemisphere, which has now been removed. We should keep in mind however, that because his posterior brain has been seizing so repeatedly for the last three months, his frontal lobe has grown rather accustomed to receiving and perpetuating chaotic impulses. It IS possible then, that as his right frontal lobe stops receiving impulses from the now non-existent brain behind it, it too will stop propagating abnormal impulses. We have faith.

To give you an idea as to what Zacchaio looks like currently, he has 3 peripheral IVs (one in each foot and one in his hand), an arterial line, a central line, a foley catheter to drain his urine, an NG tube, and a JP drain which is draining excess blood and cerebrospinal fluid from the surgical area. During rounds with the physicians today we said, "He's a tubular little dude!" I think they appreciated the lightness we like to maintain in the room.

Overnight he had a few developments that aren't too concerning but are being watched closely. Zacchaio's right pupil is more dilated than his left, though Dr. Bingaman said it was due to all the trauma and subsequent swelling around his third cranial nerve and should resolve within a month. He was also bleeding more than anticipated out of his drain, though frequent hematocrit/hemoglobin lab draws are convincing the team that he is not in need of another blood transfusion (he received one in the OR). Keep it up, buddy...

I was with Zacchaio when he opened his eyes for the first time today, though he looked a bit like Quasimodo due to the swelling in both eyes with more extensive swelling in his right. One just never knows with a surgery like this; we were so grateful his desire to see the world was still intact. I captured the first few moments following his first eye opening.....

I was finally able to hold our little warrior today... and a true warrior he is! He has been getting by on only Tylenol and love for his pain. I suppose our love is pretty darned powerful.

Team Zacchaio, your prayers, messages, and well-wishes are powerful healers! Prior to surgery Zacchaio was unable to get peaceful rest due to frequent epileptic spasms. I am thrilled to report that he has been resting comfortably without any signs of seizures!


Today will be another day of rest, and we hope with every passing day we see more of our baby boy come back to us! 

Love from Ohio.

Thursday, August 14, 2014

Surgery Text #7!!!

So proud of our baby boy... Keep it up, love!

Surgery Text #6

Surgery Text #5

We like this one....

Surgery Text #4

Surgery Text #3

Surgery Text #2

Surgery Text #1

The Morning of Surgery

Just as anticipated, we both slept very little last night. Zacchaio never left my arms which allowed me the perfect amount of closeness with him before surgery this morning. He was so angelic while he slept, though he was only able to find short stretches of peaceful rest. Seeing him seize as much as he did through the night is more than enough validation for our choice.

With every new face coming through Zacchaio's room this morning, we are feeling more and more at home. Another local priest, Fr. Dean, showed up unexpectedly as we were accompanied back to pre-op. He blessed Zacchaio with holy oil and left behind icons. 

So far so good...

Zacchaio's anesthesiologist came in for a pre-surgery briefing and greeted us with a joyful, "Kalimera!" (Greek: Good morning!) Everything about her was beautiful, and she fell in love with Zacchaio immediately. She was perfect.

Next the entire team came by for a huddle. Surprisingly, we found ourselves joking and laughing more than anything else. In sharing such genuine laughter with the team, I feel we all made deep and personal connections that spurred a more personal investment in our baby's well-being. We are convinced that he is in calm, confident, and capable hands. 

Before they took him back to the operating room, our anesthesiologist asked, "A prayer before we go?" 

This is so right.

While encircling Zacchaio, the team and Aidan and I spoke the Lord's Prayer. 

May the surgical prep begin. Surgery should begin in about an hour.

God bless you baby boy.

Wednesday, August 13, 2014

Tomorrow is THE day.

 As I write this entry I'm sitting next to Zacchaio in bed and watching him fall asleep on my pillow; except he hasn't been able to fall asleep for the past 2 hours on account of his constant seizures and spasms. Since beginning our quest for brain surgery one month ago we have witnessed a disheartening evolution of his seizures and regression in his development. His seizures have increased in frequency (now up to 400 per day), and they have increased in intensity (contracting muscles, uncontrolled dropping of his head, intense pulling of his eyes to one side).


He is often disengaged from the environment and people that surround him. I miss his blue eyes looking deeply into mine.

His smiles and joyful expressions are no longer generated by our silly faces or the tickling of his feet; they are seizure-induced. There were multiple occasions where I found myself resenting his seizures for eliciting the euphoric emotions they did, but after hearing the bloodcurdling screams that preceded the seizures of other children in the epilepsy unit, I am grateful.

He no longer has the strength or stamina to hold his head up during tummy time. The gains he made with persistent therapy and hard work have been lost.

His sweet baby coos have been silenced. He often moves his mouth in anticipation of generating sound without ever producing one.

In refusing to lose anymore of the charming and resilient baby boy God has blessed us with, we're taking a tremendous leap of faith tomorrow, an average Thursday, with hopes of him being seizure-free and leading a normal life..

Tomorrow, on August 14th, Zacchaio will have a right posterior quadrant resection. 

In other words, Dr. William Bingaman (from the Children's Cleveland Clinic) will remove a quarter of Zacchaio's brain; most of his right parietal lobe, all of his right occipital lobe, and all of his right temporal lobe. This contrasts the right hemispherectomy that was recommended by Boston Children's.

Fiducials (brain markers) are in place, now off to MRI!

In removing the back half of Zacchaio's right hemisphere, he will permanently lose the left half of his vision in both eyes. To have a better idea as to what this would look like, Zacchaio would perceive a landscape as depicted in the image below. Our understanding from Dr. Bingaman as well as parents of children with identical deficits, is that children learn to compensate for the loss very well. Though Zacchaio might only be able to see half of our faces, if he can truly see half of our faces, I will consider ourselves incredibly fortunate.

The distiction between a quadrantectomy and a hemispherectomy for Zacchaio is the preservation of his sensory and motor cortex, allowing him complete sensation and fine motor skills of the left side. I feel inclined to mention that this surgery is by no means a guarantee of seizure freedom. There is a chance that Zacchaio continues having his current seizures post-operatively. If this is the case, Dr. Bingaman will take Zacchaio back into surgery to remove the remaining right-sided brain tissue, which ultimately completes a hemispherectomy.

There is so much more I can write, however my goal in getting this blog post published tonight is to unite Team Zacchaio in faith, prayer, well-wishes, and love for a successful surgery.

We will arrive at surgery check-in at 5:15AM EST tomorrow morning. Once the 5-hour surgery is underway, we will receive text updates via hospital pager as surgery progresses. I will be posting photos of pager messages in almost real-time for you all (if any of you are up that early)!

May God grace our sweet baby boy with the strength to survive surgery and overcome his epileptic hardships. May He steady and guide Dr. Bingaman's hands with His mighty hands while instilling in him the necessary wisdom. May He work miracles through Zacchaio so he can one day live with a renewed spirit in His light.

A partial baptism the night before surgery by local Greek Orthodox priest Father Steve.
We rejoice knowing he is truly in God's hands!

Friday, August 8, 2014

Celebrating the little things, because in this life they're BIG things!

Our lives for the last 2 1/2 weeks have been that of refined nomads. At one point in Boston we had to pack up and check out of our hotel daily due to sold out and overbooked hotels city-wide. We were scrambling to find accommodations so often that when we were finally grounded in the same room for 3 consecutive days, we felt as if we had found our eternal abode. In Cleveland we managed 3 nights in a row within 1 block of the Cleveland Clinic before we had to head to another hotel in the heart of the city. 

We put ourselves on the waiting list for the Cleveland Ronald McDonald House a few days ago. Although the waiting list was lengthy, we hoped that by the time they called we would still be in need of accommodations. We got the call yesterday that they had a room available for us! 

If you are not familiar with the Ronald McDonald House, it is a charity that provides a "home-away-from-home" for families while their children are receiving medical care for serious conditions. They allow families to access the best care possible, regardless of their distance from home, by providing free, comfortable, and supportive accommodations. 

The Cleveland Ronald McDonald House is located two blocks from the clinic and houses 52 families from around the globe. It goes beyond offering a comfortable night's rest by offering pool and fitness center access, free tickets to local events, in-house organized activities, and 1 or 2 meals a day free of charge through the generosity of local volunteers. In our short time getting settled last night, we met one of the volunteers who cooked us dinner. His son battled leukemia in his teens and he recalled how integral the house and its residents were in compassionately supporting their son's journey. We also met a retired pediatric orthopedic surgeon who volunteered regularly. His genuine interest in and friendliness toward others was a breath of fresh air. The accommodations they have provided us are beautiful and we feel so fortunate to have a "home" for as long as we are in need.

Although our original admission date for yet another 72-hour EEG was today, we received a call yesterday with the great news that they had space for Zacchaio a day early! As excited as we were to get the ball rolling, Zacchaio didn't seem thrilled to repeat the same 3-day process that he very patiently endured a couple of weeks ago in Boston. We at least got a comical picture out of it...

Some of you may have noticed the last bit of good news, as it's very (in)visible in the picture above. No, Zacchaio's NG tube was not inadvertently pulled out; we very intentionally said good bye!

Ever since Zacchaio was diagnosed with epilepsy 2 months ago, we stopped attempting to make gains with oral feeds. Not only was he an increased aspiration risk (due to laryngomalacia and the potential for him to seize while eating), but we also didn't want to introduce new variables while frustratingly navigating his epilepsy management. After 2 months of taking nothing orally, Zacchaio decided to surprise us with his tenacity by taking an entire bottle by mouth! And then he shouted for more! Within two days he was eating full volumes by mouth for every feed, and yesterday we decided to further support his newly acquired skills by removing the tube entirely. 

Talk about perspective... Feeding him by bottle takes 15 minutes compared to the one hour it would take on the pump. Feeding him by bottle requires only a bottle compared to a pump, bag, IV pole, syringe, and water flush. Our lives just got a whole lot easier! His life just became a whole lot more normal! We thank God that our baby boy has refused to become complacent; he continues to rise to each successive challenge. 

Team Zacchaio, thank you for your unwavering encouragement! We love broadcasting good news and pray that many more successes await...

Thursday, August 7, 2014

Nothing ventured, nothing gained.

We are planning to partially remove and completely disconnect the right half of our son's brain...

... and as disturbing as the above statement sounds, we consider ourselves lucky.

After meeting with Dr. Pearl (neurologist) and Dr. Madsen (neurosurgeon) at Children's Boston, we had the following options for managing Zacchaio's epilepsy:

Option 1: Try the anti-epileptic drug Vigabatrin, a medication specifically indicated for infantile spasms. If it were to work, Zacchaio would be seizure-free; but not without risk. The drug has potential side effects of permanent peripheral vision loss, reversible effects on deep brain structures, possible movement disorders, respiratory dysregulation (changes in breathing patterns), etc. Due to its high potential for toxicity, it can be used for a maximum duration of only 1 1/2 years.

The opportunity for Zacchaio to be seizure-free for a year and a half is enticing; we need to keep in mind that this year and a half would be during a critical developmental period where he would be under the influence of two anti-epileptic drugs (he is also currently on Phenobarbital for an additional seizure type) which by nature subdue the brain's electrical activity. Although he might make developmental progress, his gains would not be as great as if he were off anti-epileptic drugs completely. Following a year and a half of potential seizure freedom, we would find ourselves back at our only remaining option, surgery. The potential for permanent vision loss with Vigabatrin therapy coupled with the vision loss Zacchaio will definitely inherit with surgery could leave him almost completely blind. Bad idea.

Option 2: Try ACTH (injectible steroids) or similar drugs. These types of drugs are often most effective against infantilie spasms, but they also compromise heart function, decrease blood pressure, and suppress the immune system, among other things. 

With Zacchaio having a heart arrythmia with a blood pressure that tends to run on the low side, this option is not safe.

Option 3: Surgery. A right functional hemispherectomy. 

How is it performed?
The largest part of the brain is known as the cerebrum which is divided into two hemispheres. In a 5-8 hour procedure, Dr. Madsen would skillfully remove Zacchaio's right temporal lobe, then disconnect all remaining lobes in the right hemisphere. Part of what complicates the procedure, among many things, is that he will have to do all this while remaining superbly cognizant of Zacchaio's cerebral vasculature to avoid excess blood loss. Removing the temporal lobe would give him more direct access to the corpus callosum, a thick band of nerve fibers that helps the two hemispheres communicate, which he would ultimately sever in order to prevent the spread of seizures from the diseased right hemisphere to the healthy left hemisphere.

What would he be losing?
The right hemisphere controls all left-sided function, including its sensation and movement. In addition, it is responsible for the left half of vision in both eyes. By losing his right hemisphere Zacchaio would no longer have the ability to feel or move the left side of his body; he will also acquire a left-sided visual field loss. Zacchaio is likely to have decreased visual organization and visual memory skills, left-sided neglect, decreased non-verbal problem solving, impulsivity, and difficulty with math. 

Although in choosing surgery we are electively disabling our baby boy, there is reason for us to believe in the potential for normalcy.

Why do the surgery?
Generally speaking, there is no cure for epilepsy, only life-long management. This is one of the reasons we consider ourselves so fortunate... Zacchaio's seizures have remained isolated to his right hemisphere, which is exactly what qualifies him for a surgical treatment option that has the potential to be curative. Time is now of the essence. The longer the seizures remain uncontrolled (as they are currently), the greater the potential that his healthy hemisphere becomes damaged or is recruited to seize similarly to the diseased hemisphere.

Seizure freedom and the promising concept of "brain plasticity" gives us hope that there is normalcy to be had. Brain plasticity is the concept that the brain has an innate ability to be shaped by experience, as well as a unique capacity to learn, reorganize, and recover following an injury. Regardless of the amount of traumatized, damaged, or entirely missing brain, it is thought that remaining healthy brain has the capability to create new neural pathways that can compensate for lost functions. Zacchaio will eventually regain sensation of his left side. He will learn to crawl by scooting on his bottom, will likely walk with a limp, and be able to move his left arm without fine motor movement in his left hand. At least that's what they say. In his 7 months of life, Zacchaio has managed to surpass everyone's limited expectations. Although outcomes following surgery remain variable, we focus on the remarkable recoveries made by determined half-brained children and their incredibly supportive families. A sweet 5-year old right hemi girl is in ballet fifth position with only one arm above her head and "lefty" dangling at her side looks just as graceful as her fellow dancers. A teenage right hemi girl posts a "how to" video on how one can independently tie their hair into a ponytail with only one hand. A tender moment is witnessed when a young left hemi boy helps guide his healthy baby brother down the stairs safely with his good hand. 

The stories are inspirational, and I know Zacchaio's will be one of them.

Research has shown that the earlier in life brain trauma occurs, the greater the opportunity to recover function. Traditional epilepsy management asks for the exhaustion of drug therapy prior to the consideration of epilepsy surgery. With that said, most children who are having epilepsy surgery are much older than Zacchaio, have already failed at least 8 anti-epileptic drugs, and are showing severe developmental delay or regression. There is a reason that every physician we mentioned surgery to 6 weeks ago thought we were crazy. Zacchaio has failed only 2 anti-epileptic drugs. He has very minimal developmental delay and continues to make huge gains in spite of having as many as 300 seizures per day. 

We're in the fast lane, and we're headed straight toward a better life. 

Just prior to leaving Boston to return home to California, Cleveland kept nudging its way into my consciousness. I mentioned in a previous post the fantastic opportunity we had to receive an online second opinion from the Cleveland Clinic so as to avoid another trip, but a nagging feeling was telling me that it just wasn't good enough. Trusting our instincts, we boarded a plane and arrived in Cleveland 3 days ago. Much gratitude to Team Zacchaio for allowing us to follow our intuition! 

We met with Dr. Elaine Wyllie, an epileptologist who has publications surrounding epidermal nevus syndromes as well as Zacchaio's specific diagnosis. She was... incredible. Her process was a reflection of her extraordinary expertise. She exhibited great compassion, understanding, and dedication to our son's well-being. She complimented us repeatedly for our proactive methods and being remarkable advocates for our son. Everything about her and Cleveland felt right.

In reviewing all of the data we acquired from Boston, she first offered Boston's options #1 and #2. After respectfully declining the two, she followed with a slight difference in Boston's #3. Her preliminary surgical proposal was a right posterior quadrant resection, or in other words, a removal of only the back half of Zacchaio's right hemisphere. She believes that most of Zacchaio's abnormal electrical activity (based on EEG, MRI, and PET) is originating from his temporal, parietal, and occipital lobes, not his frontal lobe. In having the posterior resection instead of the hemispherectomy, we would be preserving Zacchaio's motor and sensory cortex which would eliminate the left-sided weakness/paralysis he would acquire with the other proposed surgery. With her approach Zacchaio would still lose half of his visual field and seizures could eventually manifest within the preserved brain. If seizures were to return, another surgery to complete the entire removal of his right hemisphere would quickly follow. 

Where do we stand today?
A right functional hemispherectomy is scheduled for August 22nd at Boston Children's. 

Dr. Wyllie at Cleveland was able to work impressive last-minute magic and schedule Zacchaio for a 72-hour EEG this Friday. On the following Tuesday morning Zacchaio's case will be presented at Cleveland Clinic's surgical conference, where 70 sets of eyes will have an opportunity to weigh in on proposed surgical intervention. Immediately following the conference, we will meet Dr. Bingaman, the neurosurgeon who will be doing Zacchaio's surgery if we ultimately select him over Dr. Madsen at Boston Children's.  We will make our final decision regarding which procedure and which medical facility on Tuesday. If we select Dr. Bingaman at Cleveland, surgery will follow shortly after.

We have received only rave reviews for both surgeons and believe that Zacchaio will be in skilled hands regardless of our choice. As his parents, we have to listen to our instincts carefully, as our decision will greatly impact the framing of the foundation for his new life. I imagine the moment Zacchaio is lying on the operating room table and his surgeon looks at him one last time before turning toward the monumental task ahead of him... my hope is that he sees him not only as a brain, but as the angelic soul that he is, the understated champion, and the powerful being that unites communities through faith and love. I want them to be a part of the miracle that defines Zacchaio's next chapter. May God continue his miraculous work through our baby boy...

Brotherly love - July 2014

Who loves Ohio!?!

Pure joy on the swings!

People watching at the coffee shop

Hello, big boy!