Saturday, December 13, 2014

New Developments

Aww Genes!

Genetic testing results came back on Zacchaio's scalp nevus tissue (biopsied during his plastic surgery in October). The meeting with the geneticist was long and didn't offer much positive news. Zacchaio has a mutation of the KRAS gene. Since he was first diagnosed with his syndrome, we knew it was caused by one of two mutations (KRAS or HRAS).  KRAS is less frequently seen in LNSS cases (1%) and Zacchaio has the rarest form of the KRAS mutation.

KRAS is one of the most frequently mutated oncogenes in human cancers. A mutated KRAS directs cells to grow and divide in an uncontrolled way. This mutation has been found in high percentages of pancreatic cancers (90% of cases), lung cancers (25% of cases), colorectal cancers (50% of cases), as well as some stomach, esophageal, and thyroid cancers and leukemia. ::insert deep breath here:: Due to the role KRAS plays in cellular pathways, those who have cancer and the mutation are rarely treated with chemotherapy because it is ineffective. Despite continued efforts, there is no known treatment to stop KRAS' function.

What does that mean for Zacchaio? A plethora of proactive screening. He is at a much higher risk of developing the above cancers as well as secondary melanomas of his nevus skin. We are now also plugged into a GI doctor (to monitor his digestive system), an oncologist (to dictate screening frequency), an endocrinologist (to monitor his thyroid and hormone levels), and a GU doctor (to monitor his urinary system). Because treatment options are limited if Zacchaio were to be diagnosed with cancer, there isn't a single body system we can afford to overlook. We will likely alternate between ultrasounds and MRIs of his entire body to keep a watchful eye on any developing tumors/malignancies.

Dem Bones!

We received official biopsy results for Zacchaio's right arm bone tumor. UCSF had difficulty making a differential diagnosis of the tissue, so they forwarded the sample to the Mayo Clinic for further evaluation. The tumor continues to baffle physicians and pathologists. The next few sentences will contain loads of medical jargon, but I like including it in my blog in the event that there are others searching for similar diagnoses. His tumor has features of a nonossifying fibroma (a common benign bone tumor seen in children), but the spindle-shaped cells are too small for what is typically seen in a nonossifying fibroma. Doctors do NOT see evidence to suggest a malignancy. Zacchaio's lesion has unusual features associated with scalloping of the cortex. Some features suggest it's an intracortical lesion, but based on the x-ray it seems to have a benign appearance.

Since receiving these results, I have e-mailed a few of the top orthopedic departments in the country in hopes that they have seen something that resembles above description. We'll see.

I wasn't thrilled with our last orthopedic visit with UCSF, so we visited a Stanford doctor for a second opinion. He recommended another xray, which I agreed to, as I was anxious about the progression of the tumor. You can see from the picture above that it has grown quite a bit since August. Although the growth is quite alarming and one would logically assume his condition was worsening, the physician said it actually looked better! The cortex of his entire bone looks healthier and stronger. His humerus bone is also behaving in a more "normal" way in that it seems to have further quarantined the tumor with a more distinct border. Zacchaio's arm doesn't appear to be bothering him at all; he is still weight-bearing without any visible discomfort. He will have a skeletal survey (full-body xrays) at the end of January to check for tumors in any other bones.


OMG...another EEG!

First EEG since August 2014 brain surgery

Zacchaio had a 1-hour EEG last week and was incredibly cooperative with lead placement and fell asleep at the technician's request. We just heard back from the neurologist, and although there were rare spikes (bursts of electrical activity), he reiterated there are absolutely no seizures present. This is great news! We still have quite a few questions for the neurologist regarding spike origination location, whether there will be changes to the anti-epileptic drug weaning plan scheduled for February, etc. We will let you know more as we learn more!


Those of you who follow on Facebook are already aware; those of you exclusively on Zacchaio's blog are not yet. The Anat Baniel Method (ABM) therapy Zacchaio attends each week (3-6 sessions) has been absolutely tremendous for his development!

Fun with ABM practitioners Sylvia!

A month ago, Zacchaio was quite behind developmentally. When I last posted we were celebrating his rolling from front-to-back and back-to-front. On Thanksgiving morning, Zacchaio surprised us with...

...and within a week he had advanced to...


...and right after I posted this blog, he did this...


Today he keeps trying to sneak through the kitchen and out the garage door for some outdoor play with his brother! He has almost caught up with his 9-month benchmarks... There is no containing his excitement for the world and the people in it! Given where we started 11 months ago, I never imagined we would find ourselves here today, almost to Christmas with a beautifully decorated tree, a hilarious 4-year old ham, and a remarkably joyful 11-month old who makes every attempt to undecorate the aforementioned evergreen. Everywhere we go, the festive atmosphere reminds us of the miracle we will be celebrating on Christmas Day. We are so grateful for His love and healing, the continued miracles we witness, and the joy you all bring to our lives.

Wishing you all the Merriest of Christmases! May God bless you with his love and peace, and may you find joy in every minute of every day!

Also, please refer to our previous post, "Save the date!" We would love for you to join us for Zacchaio's 1st birthday celebration!


Sunday, December 7, 2014

Save the date!

Save the date!!

What: Zacchaio's 1st Birthday! A Celebration of Survival!

When: Saturday, January 10th, 2014 at 12:30pm

Who: All who have prayed, loved, and supported us and Zacchaio over the last year!

Where: Hayward, CA
(Please refer to our Facebook invitation for exact location. If you do not have access to this invitation, please contact us via Zacchaio's blog Contact Form)

RSVP: Please RSVP via e-mail, phone, or blog Contact Form the number of people that will be attending. We want to be sure we have accurate numbers for catering purposes!

Saturday, November 15, 2014

Cruise Control

Since my last post three weeks ago, our family has found itself in cruise control. Not the kind of cruise control where we go unconscious and miss four miles of the road; it's the kind where no one around us is in a hurry, all the windows are rolled down, and every breath of fresh air reawakens our soul as we drive toward the sunrise.

Aidan and I accidentally stumbled on one of Zacchaio's old seizure videos and found ourselves lost for words while tears streamed down our faces. I've mentioned the idea of brain plasticity and how it has benefited Zacchaio's developmental progress, but it's just as incredible how the minds of his parents can so skillfully filter out the bad memories and glorify the beautiful ones. The moments of heartbreak we experienced in the last 10 months seem like a bad dream. We have left desperation behind. We have officially exited survival mode, and I've started envisioning what could be a realistic future for our boy.

November 2014

I heard this song on the radio the other day and found myself surprisingly emotional.

The music video spotlights a cystic fibrosis patient and resonates deeply (around-the-clock nebulizer treatments included). It sings of the many dreams I have for Zacchaio. I dream that he one day experiences heartbreak, because he was capable of falling in love. I dream that he one day breaks a bone after falling from his bike, because he was capable of riding one. I dream that he one day inspires nonbelievers to trust in God's miracles, because modern medicine can't explain it any other way. I never want his disabilities to hold him back; I want them to inspire him to celebrate every moment he's given. I'm so excited to see what God had in store for our baby boy!

Zacchaio's plastic surgery incision is healing well. For the time being he is unable to lift his right eyebrow due to the severing of the nerves in his forehead, but the surgeon is hopeful that he will regain mobility in the next few months. As for his brain surgery scar, after just three months it has almost faded completely. My memories from his epileptic period have disappeared along with his scar; I can't imagine that's coincidental. Wounds do heal, no matter how deep.

Progression of healing...

After seeing the dermatologist, we learned that an interesting feature of Zacchaio's nevus skin is that it lacks sweat glands and subsequently the ability to sweat. This was consistent with some of the overheating and mottled skin that he experienced during the last few months; we're now, however, more proactive with strategies to help him compensate.

As for his brain, we are no longer seeing any of the strange happenings we were seeing before. Not a single one in weeks! This allows me to say with much more confidence that he remains seizure-free! Zacchaio will have a follow-up EEG to check for sub-clinical seizure activity in December and we hope it further validates our feelings! We will also start to wean his epilepsy drug, Phenobarbital, in February. While there is potential for new seizures to breakthrough during the weaning period, I am excited for what may be on the horizon. I see how fast Zacchaio is learning while on a drug that inhibits his brain activity and his capacity to learn. I can only imagine what he will be capable of as we give him back all of its function!

Zacchaio's heart has responded tremendously to drug therapy. His cardiologist said we could start weaning him off is heart medications; instead we will approach it conservatively and start the process in January.

We got a call from the orthopedic surgeon's resident today with final biopsy results. Although we received preliminary results that Zacchaio's bone tumor was benign, we were still awaiting further pathology to give us insight about the tumor. UCSF was unable to provide a differential diagnosis for the specimen, so it was forwarded to the Mayo Clinic which explains the three-week wait. Final results stated that he has a benign spindle cell bone lesion. Unfortunately, there isn't much more I can say about it, as I don't have precise enough terminology to do my own research. We should be meeting with the surgeon next week to get a better idea about what it means, whether it needs to be surgically removed, whether it can show up elsewhere, etc.

Zacchaio continues to receive ABM therapy and is making great progress! It's a shame he doesn't enjoy it at all...

His posture has improved (he sits up much higher in his high chair), he is rolling back-to-front and front-to-back consistently, he is making consonant sounds, he is creeping forward, and he is moving too well in reverse (every once in awhile we can see the hope fade from his face as he gets further and further away from his intended target!

He is eager to be a part of the action and even attempts to propel himself out of arms to get to where he wants to go.  He is eating enormous amounts of pureed fruits and vegetables and is rapidly achieving new percentiles on the growth chart. He is playful and has a fondness for music (to the point where we sometimes find him entranced!). The love and admiration Zacchaio has for his older brother is endearing; he is captivated by his every move and every touch. Our big boy, Alkaio, falls a little more in love with his baby brother every day. He validates his developmental gains, comments on his beautiful smile, and especially loves Zacchaio's shared interest in light sabers...

People continue to show their love and support for our Zacchaio in surprising ways! Alkaio has been a toy tester for Leap Frog a number of different times (side note: if you happen to have an interested 3-7 year old who would like to earn Target gift cards for testing toys, let me know!) and had an opportunity to test yet another toy just last week. As we were leaving the offices, a sweet member of the Leap Frog team showed up with four brand-new toys. She handed them to me and said, "We have all been so taken by this little guy's story. He looks so incredible and we are so proud of how far he's come! We can't wait to see what else is he's going to do!"

We pray that his miraculous healing continues, that we never find ourselves complacent, and that we appreciate the beauty in every minute that is to come.

January will mark one year in our baby boy's life and we are thrilled at the opportunity to celebrate all he has overcome! We are planning to have Zacchaio's birthday party in January and look forward to celebrating with all of you!

Yoda & Luke Skywalker

Happy Halloween! 2014

Friday, October 24, 2014


We arrived promptly at UCSF's intake desk at 8:30am this morning. After an hour in the waiting room we were called back to meet with the anesthesiologist, the physician who had the final say as to whether or not surgery was a-go. Multiple specialty doctors came through Zacchaio's pre-op room in an effort to coordinate care; all briefly hesitated at the sound of Zacchaio's barky cough and gurgly breaths. The anesthesiologist listened to his lungs at length, then asked for my sentiments. We jointly agreed... Our little warrior would prevail!

A couple of hours into surgery we received a call from Zacchaio's orthopedic surgeon. An incision was made along his bicep, his muscles were parted, a burr hole was drilled into his humerus bone, and just enough tissue was scraped out to be sent to pathology. Within minutes the preliminary results were in... the right arm lesion was benign!! I can't put into words how it felt. I took a deep breath for the first time in three weeks (when we first learned of the incidental finding). Aidan and Alkaio were in Arizona prepping for Aidan's brother's wedding at the time. The text I sent to Aidan was clear and concise, "NOT CANCER! V! SUDHSPABZM" To which he replied, "Dhsufuwgdid!!!!!!" I knew exactly what he meant.  

Zacchaio did very well in surgery and Dr. Hoffman (plastic surgeon) did not encounter anything unexpected. Most of the nevus beginning at Zacchaio's right eyebrow and ending on the top of his head was removed, but Dr. Hoffman was unable to remove it all. With Zacchaio's recent loss of skull, the surgeon couldn't safely stretch his scalp any further due to the detrimental risks associated with tearing the dural layer (membrane that covers the brain) in the problematic area. In the end, 75% of his scalp nevus was removed, as well as his largest cutis aplasia. The remaining area of nevus and cutis aplasia blisters will be removed at a later date at least six months from now. 

It may not look like much now, but we hear Dr. Hoffman is world class in his quality of work. The visible purple coating is waterproof DermaBond glue that will fall off in the next ten days. The healing process will be long and require much patience, but I'm eager to see what it looks like by Christmas!

Thank you all for your love, prayers, supportive attitudes, and well-wishes! I read many of your Facebook comments aloud to Zacchaio as he drifted off to the psychedelic lands of Morphine and Fentanyl. 

Your faith in our son inspires us... 

************* Update: Saturday, October 25, 2014 *************

We're home and healing!

Sending love!

Thursday, October 23, 2014

Twas the night before surgery...

Within an hour of the publication of my last post, Zacchaio's upper respiratory issues became a bit more distressing. His barky cough was more frequent and higher-pitched, his nose began to run again, and he was having increased difficulty clearing the junk that sat cemented in the back of his throat.

The following morning we had a phone appointment scheduled with the pediatric anesthesiologist. I was sure she was going to cancel surgery, as protocol typically states that any cold-like symptoms experienced within a week prior to surgery deems one unsafe for anesthesia. Surprisingly, she said she would check in with us today, two days later, to determine whether or not Zacchaio was in an upward or downward trend. 

I obsessively reevaluated Zacchaio's health every hour today in hopes of having a clearer idea whether or not he would be having surgery tomorrow. Anesthesia called again this afternoon and I reported the latest. They said we could either cancel surgery or arrive at UCSF tomorrow as scheduled so the anesthesiologists could personally evaluate him and weigh the risks.

We saw a UCSF neurosurgeon today to get his insight on a couple of anatomical concerns. There is an area of Zacchaio's bone flap (the bone originally removed and replaced for brain surgery) that has, well, disappeared. Bone resorption is the process by which bone is broken down and its minerals are released.  This is supposed to be coupled with bone generation, but Zacchaio is missing a silver dollar-sized area of his skull that isn't coming back. We will continue to monitor its size, as well as take precautions to ensure it isn't forcefully contacted. The hope is that his skull will regrow and eventually fill in the gap, however considering that the gap has only grown in size over the last month, I am not very confident in the growth process. If it continues to widen, the plastic surgeon and neurosurgeon will have to intervene. We'll have more on that in the coming months.

As for tomorrow's surgery...

Our arrival time is scheduled for 8:30am with a 10:30am surgery time. Total time in the operating room will be 1.5 hours. 

Praying our boy wakes up healthier in the morning...

Praying for good news regarding the biopsy.

Knock it out of the park, kid.

Sunday, October 19, 2014


We met with the pediatric orthopedic surgeon on Wednesday to review Zacchaio's test results. Unfortunately I can't characterize the news as either "good" or "bad."

- What we verified through the MRI of his right arm is that there is a very large lesion/mass/tumor that has eroded away what now looks like 80% of his humerus bone. 
- His bone scan was negative which means one of two things. Either the lesion is benign, or contrastingly, is one of the most aggressive and nasty cancers known to man. In this moment I'm feeling as if the only thing a bone scan is good for is instilling an unprecedented amount of fear in parents. It's really good at that.

The plan was to update the blog from the beaches of Venice in Los Angeles, though where I find myself currently is on our own living room couch in oversized sweatpants. A few days ago, Zacchaio woke up with a runny nose, the earliest indication of yet another upper respiratory infection which has historically sent him to the hospital for 1-week long admissions. With plastic surgery scheduled on Friday, we couldn't take any chances and decided to cancel our trip, quarantine him in his own environment, and focus on getting him healthy. Our plastic surgeon is booked solid for the next six months so we can't afford to lose our surgery date! We took it upon ourselves to try something new and have been proactively giving Zacchaio saline nebulizer treatments (normal saline solution converted into mist for inhalation) in hopes of lessening and thinning his secretions. It's been four days of nebulizer treatments every four hours and it seems to be halting the progression of whatever virus he might have contracted. In fact, he is no longer showing signs of a runny nose and we're praying that continues until Friday!

As for Friday...

The timing worked out quite well between the diagnosis of Zacchaio's arm lesion and his plastic surgery date. As soon as the anesthesiologist puts him under on Friday, the orthopedic surgeon will come in (on his day off I might add), make a small incision in his right arm, and biopsy the lesion. I asked how long until we find out if it's malignant? He said we'll know immediately. Friday morning calls for a lot of prayers. 

I haven't been nearly as cheerful since Wednesday; I find myself often spiraling out of control with "what ifs." Life has been unimaginably beautiful and it is hard to fathom that cancer may be trying to dampen its brightness. Although the last nine months of Zacchaio's life have been riddled with obstacles, I somehow felt optimistic he was going to overcome each one. When I entertain the realistic possibility of cancer, I'm stricken only with fear. I shouldn't get too far ahead of myself.

Wednesday, October 8, 2014

The latest!

I apologize for the delay in getting another post published! Finding a single quiet moment to sit and type seems to be near impossible nowadays. Between being a parent of two (how did you all do it?!) with one of them being special needs requiring numerous appointments every week, sometimes I feel like I can't get my head to stop spinning!

Zacchaio was discharged last Saturday evening from UCSF's PICU with his admission amounting to a 6-day stay. His previous admission in July for identical reasons lasted well over a week, so our decision to take him in early most definitely served him well.

Here is where we stand today...

- We still don't think we're seeing any seizures. Hallelujah!!

An inside look. Goodbye rotten brain!
Left: Zacchaio's brain prior to surgery.
Right: Zacchaio's brain after surgery. The white is empty spaced filled with cerebrospinal fluid.
Note: MRI images are reversed. He had most of his right hemisphere removed.

- A couple of posts ago I mentioned that we were seeing Zacchaio's ophthalmologist for his lazy eye. On the morning of his appointment, we were playing in usual fashion while I incorporated eye tracking exercises. I couldn't believe what I saw... his lag had completely disappeared and it hasn't shown itself since! He's amazing at problem-solving.

- Zacchaio is developing tremendously! ABM therapy is doing wonders for him. He is so alert, engaged, and attentive. I can't get him to stop moving during diaper changes; his excitement for rolling over and discovering what awaits him on his play mat cannot be restrained! When we're in the car he leans forward and out of his chair, laughing as he looks out the window to the left. To his right he consistently searches for Alkaio in hopes of some kooky 4-year old entertainment. He continues to explore what his voice is capable of and is making new sounds every day. At the moment he is showing a preference for the bear cub growl. Ultimately the timing is perfect, as Cal volleyball season has started and we're hoping to attend a game soon. Go bears!

His visual field deficit is now barely noticeable. He does startle from time to time if you come toward him from his left periphery, but he is otherwise tracking 180 degrees, he turns his head both directions to sound, and he most definitely recognizes his name. He loves to gnaw on his teething biscuits, he thinks squeaker toys are hysterical, he intermittently bears complete weight on his legs, and we're seeing just the beginnings of creeping when he's on his belly. He is not yet caught up to his adjusted age of 7-months, as he is not sitting up unassisted or rolling from front to back, but he is catching up quickly and I have faith that one day he'll find himself ahead of the curve! His brain is unbelievable and his new neurologist out of Stanford attested to the fact. She says he should be the poster child for brain resection surgery! She couldn't believe how incredible he looked!
We continue to have four sessions of ABM therapy per week in San Rafael, and my gratitude is unending for your generous donations. Thank you again for eliminating the financial barriers that would otherwise limit our access to such tremendous healing! His therapists marvel at what he has overcome and the way in which he continues to overcome.
- In my last post I wrote of our newest incidental finding related to Zacchaio's right humerus bone. Following further review of his ultrasound they realized that whatever they were looking at was extraosseous, or outside of the bone. This "something" (likely a tumor per the physicians) has been putting pressure on his bone long enough to have caused it to erode half of its width. On Friday Zacchaio will have an MRI of his arm as well as a full-body bone scan while under anesthesia. The MRI should give us a good idea of what exactly this large mass is, and the body scan will show "hot" and "cold" spots which signify overactive and underactive bone activity/metabolism. We'll have a follow-up with an orthopedic surgeon five days after the scan so it's likely I won't post an update until after that meeting when a short and/or long-term plan has been established. I'm taking a deep breath heading into Friday and will continue to pray that whatever they find will be manageable.
Erosion of Zacchaio's right humerus
- A plastic surgery surprise. After rescheduling plastic surgery multiple times due to acute illness and uncontrolled seizures, we finally had a surgery date of December 5th. The problem with having a date so far in the future is 1) Zacchaio's health is incredibly unpredictable and there is no telling whether or not he will be healthy enough to keep it, and 2) we are nearing his one year birthday (January 9th) which means his scalp will inevitably be much less mobile and malleable, decreasing the chance of complete removal of his scalp nevus. The wonderful news is that an October 24th surgery date became available and we were given first claim to it! The bad news is that this happens to be the same day as Aidan's brother's wedding. Unless anything changes, Aidan and Alkaio will attend Evan and Beth's wedding in Arizona while Zacchaio and I send our love from afar.
The plan remains the same with plastic surgery. Remove as much of his scalp nevus as possible (without leaving the incision too taut), remove the large cutis aplasia (area where skin didn't form completely) and large lipoma (fatty tumor) toward the back of his head, and remove all the smaller cutis aplasias on the right side of his head.
- Although Zacchaio had a clinical baptism in Cleveland prior to brain surgery, we had the opportunity to complete his baptism in our own church last week with two phenomenal Godparents. Fr. Nebo and Presbytera Stephanie have not only taken our son under their spiritual wing, but have blessed our lives in more ways than they know by joining our family. As much as I would have loved to open the baptism for all to attend, there was no telling whether or not Zacchaio would be healthy at any particular date or time so this was incredibly last-minute. God willing, we hope to celebrate his 1-year birthday in your spirited presence, surrounded by your unbridled love and support!
As a family we are taking off next weekend for UCLA Children's for a second opinion from a neurologist who specializes in infantile spasms. During downtime we hope to find an opportunity to introduce Zacchaio to the ocean and beautiful sand of Venice Beach for the first time!
September 2014
Trying to keep up with big brother!

Wednesday, September 24, 2014

A speed bump gone wild.

Life has been incredibly busy! Up until Sunday morning I am proud to say that it was because we were living the life of a normal family! Walks, farmers' markets, errands, the park, dinners out, baseball out front... Unfortunately, busy has taken on new meaning since Sunday night.

For the last week and a half, Zacchaio had what we thought was an insignificant cough. It was so mild and infrequent that I considered it a teething cough resulting from increased amounts of saliva in his mouth at any given time. Surprisingly, on Saturday afternoon he woke up from a nap with a horribly runny nose and worsening cough, and within hours he was struggling to take a bottle due to excessive mucus and congestion in his nose. I couldn't believe how quickly it evolved. As resistant as Aidan and I were to putting the NG tube back in, we soon realized that a few days with an NG tube much outweighed a week of treatment at the hospital.

Putting the NG tube back in was no easy task. We had both forgotten that the last time he had one in was over one month ago when he had uncontrolled seizures, minimal purposeful movement, and little energy. Zacchaio made it abundantly clear that he did NOT want the NG tube and had us sweating bullets in the wee hours of the morning as we struggled to get it down. After a successful placement and subsequent feed, we put him back to bed only to discover four hours later that we had underestimated our baby boy. Still wearing the two pairs of socks we put on his hands to limit his dexterity, Zacchaio looked up at me wide-eyed from his bed as his NG tube dangled from his cheek. Really, kid? As frustrated as I was knowing we would have to battle the dreaded nasal passages yet again, a part of me was encouraged to see how inspired and capable he was to achieve a desired outcome. Your capabilities are endless, baby!

On Sunday we found him having longer coughing fits where his lips would start to turn blue. His work of breathing and respiratory rate suddenly increased and after seeing that his oxygen concentration was only 91% on our home pulse oximeter, I decided to pack our overnight bag and head to UCSF's ER.

We were a well-oiled machine. We knew exactly what to pack in his bag. Aidan and I started making our long-term plan regarding who would be staying at Zacchaio's bedside and when we would make our swaps. We prepared Alkaio for what the week ahead would look like and he was beyond understanding. Once in the ER, I described Zacchaio's clinical picture for the last two days. In order to remove the possibility for the staff to make inappropriate care plans, I informed them of which supportive interventions he would be in need of for his admission. And then he was admitted.

It was on his first day in the PICU that Zacchaio had a chest xray to rule out pneumonia. Thankfully it was ruled out, however the radiologist accidentally stumbled upon a concerning finding. Zacchaio's right arm was unintentionally caught in the xray and there was an area of lower density noticed in his right humerus (the bone that connects your shoulder to your elbow). They decided to do another xray of just the humerus for a closer look and after reviewing the images determined that the area was either a bone infection, tumor, or an entanglement of malformed vessels. To review it further, they did an ultrasound which has allowed the staff to say with certainty that there is an area of bone in Zacchaio's humerus that has been eaten way. What has caused the erosion? They aren't sure. A previous infection that is no longer present? An infection that is persisting? A tumor that is pushing on the bone? More questions than answers overwhelm the day.

We're awaiting further input from our genetics and orthopedics teams following their review of the images. An MRI of his humerus will give us the most information, however he is unable to complete the test now due to his acute illness and congestion (doesn't complement anesthesia well).

I'm hopeful that at some point in Zacchaio's life we will stop getting new and unanticipated diagnoses  every month. Until then I'll just continue marveling at what our little boy is capable of...

Eyeing his IV... Plotting how to rid himself of it...
So he pulled it out the next day.

Tuesday, September 9, 2014

Home Sweet Home!

We didn't waste much time. Within twenty four hours of discharge we were on a plane heading home to good ol' California!

That's not to say that leaving the Ronald McDonald House wasn't a difficult task. We said goodbye to a Greek family whose daughter contracted a disease from a mosquito bite which necessitated intestinal transplants she was disappointingly rejecting. We lovingly waved farewell to the dad who lost his 5-year old son to a leukemia days prior to our departure. The day before he passed the house threw him a birthday party even though his birthday wasn't until November. Alkaio yelled an enthusiastic, "See ya later, alligator!" to the young girl he played with every day; she struggles with seizures that remain resistant to medication. Behind every door was a family with a story of struggle that no one wants to hear. Each tell it so gracefully. We couldn't have asked for a better home away from home where a simple, "Good morning!" from one resident to another really meant, "I hope today is a better day. May our children find peace and healing, and may we as parents find patience as we watch our children overcome." If you're ever looking for a charity to which to donate your time or money, we highly recommend any Ronald McDonald House. Know that you would be making an irreplaceable difference in the lives of ill children and their families.

Though stepping foot in our own home was utterly magical, home was familiar yet strange. Our nomadic life from one medical facility to the next had become our new normal. After tucking in each boy in their own beds on our first night home, Aidan and I dramatically collapsed onto our pillows. We looked at each other with eyes full of wonderment that we survived it all, that we had wiped the slate clean, and that our small family of four was still a cohesive unit after all we had been through. We couldn't help but sigh with relief and amazement that life felt manageable in that moment.

Zacchaio in his own crib!
Home sweet home!

Family mornings are back! All four of us under one roof.

All of you are asking about how Zacchaio's seizures are doing... Unfortunately, the only answer I can give with 100% certainty is, "We're not quite sure." Only days after our arrival home we sat amongst some of our favorite company at our neighbors' dinner table. Zacchaio was fast asleep in my arms when his eyes opened abruptly, his legs stiffened, and he leaned forward while appearing to be in a trance. The behavior exactly mirrored one of the many types of seizures Zacchaio had prior to surgery (this one in particular we labeled the "Rise of Frankenstein"). Aidan and I looked at each other and felt our hearts break. We lasted as a normal family for four days. Though surgery helped us push the reset button, we were now following the exact path we traveled just two months prior. I lost my composure at the dinner table as tears welled and began to stream.

After some minutes of mourning the loss of seizure freedom, I immediately found myself in "fix it" mode and began brainstorming our next steps (I find that I sustain myself by planning and taking action on behalf of Zacchaio). I deliberately left my suitcase packed for this exact reason. I knew it was too good to be true. While frantically drafting e-mails to different neurologists with my proposed plan, I had a moment of clarity. If this was in fact a seizure (which I still believe it was even though it technically hasn't been correlated clinically yet), it was just ONE seizure. ONE seizure is not reason to remove the remainder of our son's right hemisphere. ONE seizure is not reason to start a new anti-epileptic drug. ONE seizure is manageable. The new plan was to continue watching and documenting the frequencies of the "likely seizures." If they become daily occurrences, we'll go in for an EEG, capture the behaviors, and clinically diagnose them as seizures if that is indeed what they are. Until then we continue living in the gray area in between. I don't particularly care for the color gray... We're learning to embrace the color gray...

If you recall my last post, the neurologists at the Cleveland Clinic reiterated prior to our departure that we should expect Zacchaio to have a number of strange happenings and odd behaviors as he adjusts to his new brain and altered vision. We have seen a number of these, though it seems to be happening less frequently with every passing day. They range from a half wink in one eye to startles that awaken him from a deep sleep to strange eye rolls to somewhat absent gazes into the distance. We have not seen a "likely seizure" since last week's "likely seizure." I pray the trend continues...

Besides strange behaviors, what has been happening in the last week and a half since we have been home? Well, life has been extraordinarily normal and I never knew there could be such exceptional delight in normal!

  • For the first time in Zacchaio's eight months of life we have been able to attend church two Sundays in a row to give thanks for the life with which we've been blessed. Many members of our church family were brought to tears seeing him in person for the first time. Zacchaio's spirit somehow transcends his blog and lives in every individual who knows of him.
Zacchaio on church Sunday at 8-months old (6-months adjusted)

Dressed in our Sunday best!
  • We are hearing Zacchaio's voice for the first time in months! Since he was 3-months old his mouth and throat would try to utter sounds with quiet intensity and no success. Now his constant chatter is perfectly orchestrated music that is the new soundtrack to our lives.
  • Zacchaio laughed. He laughed! There have been many times in Zacchaio's life when I questioned whether or not our advocacy was in his best interest. I found myself often asking God to help me clarify why I was saving his life; for him or for me? Was I prolonging a life of poor quality? Would Zacchaio find more peace in Your heavenly arms than here on earth with us? It was in this moment when our boy was overwhelmed with pure glee that my questions were emphatically answered. Every minute was worth it and every future minute will continue to be.

  • Zacchaio now has an insatiable interest in the world around him! He stares out the car window wide-eyed at the shapes and colors passing by. He investigates each of his toys with intense focus, then playfully shoves them in his mouth to work his gums as his teeth advance. Prior to surgery he either slept all day due to exhaustion from seizures or attempted to fall asleep throughout the day because seizures woke him as he transitioned between sleep states. After so much of his life passed him by, he now doesn't want to miss a thing!
  • Our little preemie boy is now packing on quite a bit of weight! He has had a ferocious appetite with no difficulty taking all of his recommended formula volume by mouth (sometimes he even takes extra). He even started solid foods and is loving his rice cereal, apples, and pears. Vegetables are on their way!
  • Zacchaio had lost many of the developmental gains he had made in the last few months because of uncontrolled seizures. Even after surgery he could no longer roll over, brace himself on his elbows during tummy time, or steadily hold his head up (it would bob and then fall with each spasm). Though we have referrals for traditional therapies such as physical therapy, occupational therapy, speech therapy, and an infant developmental specialist, a referral from Aidan's dear cousin Tracy led me to newer and more innovative approaches to improve Zacchaio's development. She mentioned the Anat Baniel Method (ABM), located in San Rafael, which has had incredible success with special needs children, especially those with autism and cerebral palsy. The goals lie in creating new connections in the brain through integrative movement. Seeing as it is rather non-traditional and also very pricey, it is a leap of faith (though the leap was easier to take after reading and watching success stories). After only five 30-minute lessons in the last week, Zacchaio's head control has improved tremendously to the point that he can now face forward in a Baby Bjorn carrier. Prior to ABM, he also lacked the flexibility required in his spine to bring his feet to his mouth as a baby his age should. In one week of lessons ABM has brought awareness and rediscovery of his feet. He can now hold onto them for an extended period of time and bring them very close to his mouth! It is only because of your generous donations that we have the means to access these amazing therapy sessions. We are so grateful that you continue to play such a direct and active role in Zacchaio's recovery!

Head control gained after 1 week of ABM therapy.
Zacchaio graduated to forward-facing!
  • He's healing... and rocking an incredible battle scar.

What's next on the docket?

We have left UCSF neurology and will be seeking guidance from a new neurology team at Stanford's Lucile Packard Hospital.  Stanford will fill the need for a local neurologist (as long as they live up to our high expectations), but we will also seek a second opinion from a neurologist out of UCLA's Children's Hospital who specializes in infantile spasms. Having additional and objective professional opinions surrounding Zacchaio's epilepsy was integral in shaping the course we took with his epilepsy management. Seeing as how well it served us, our plan is to follow up with the Cleveland Clinic in December (standard 3-month post-op appointment), to see a Stanford neurologist regularly, and to have the UCLA physician add his expertise specifically for infantile spasms.

We see Zacchaio's ophthalmologist on Wednesday due to increasing concerns of a lazy eye. We were told that lazy eye is expected initially after surgery and that with time it should improve. We have noticed it has worsened in the last couple of weeks and when Zacchaio looks to the right, his right eye is much lower than and mismatched with his left. There may be an eye patch (Arrgghh!) or eyeglasses in his future... We'll see!

Aidan is heading back to school next week. I'm afraid to be without him for the sake of losing his positive and grounding voice. He somehow transforms his physical presence as a single man into that of ten men. On a daily basis he bends over backward to lessen the overwhelmingly heavy load I often find myself taking on. Although we have been at this "together" for the last eight months, we miss one another tremendously and look forward to one day getting some extended quality time. Assuming we maintain Zacchaio's health, I plan to return to work in November. We'll be on the lookout for someone with LVN/RN experience to care for him a twice per week while Aidan and I are at work. If you happen to have any recommendations please send them our way!

And that's that.

We can't wait to take on another day in this typical, ordinary, average, humdrum marvelous life that is now ours.

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...