Don't be nevus!
On January 9, 2014, our son Zacchaio was born via emergency c-section at 33 weeks old. Although our Zacchaio has overcome his acute medical issues in the NICU, we're now faced with his rare diagnosis of Epidermal Nevus Syndrome. We don’t know what developmental hurdles to expect with our son’s diagnosis in five years, in six months, or even tomorrow. We can only embrace his journey and all the discovery that comes with it, not looking too far ahead and enjoying the beauty that is today.
Friday, August 11, 2017
Saturday, June 13, 2015
God doesn't give us more than we can handle.
Zacchaio and I left home at 5am two Tuesday mornings ago for a 6am surgery call time. Though he awoke during the quick transfer to the car, he nodded sweetly back to sleep within minutes. We drove silently toward the dark skies of San Francisco and en route passed Children's Oakland, our original NICU home. On any given week we pass the medical center once or twice and it no longer elicits an emotional response. For some reason on this particular day, I was overwhelmed with an unsettling doubt. As the uncertainty seeped through the cracks of my confidence, I was left spiraling out of control with a disconcerting mistrust of my intuition. For the past three months Zacchaio has been an "average" boy without any need of a hospitalization. Now we were electing for one. I glanced into my rear-view mirror at our angelic boy once more before I looked forward again. It was during that short moment of adoration that the dark skies faded into a beautiful purple as God's light greeted the day. What a beautiful reminder that today our Zacchaio has the potential for a bright future. What a lovely reminder that there is always light after the darkness. And in that moment I was comforted that today would be no different from every other day: Zacchaio perseveres.
I gowned up in my bunny suit, donned my blue cap, and walked Zacchaio to the operating room. I laid him on the table and the anesthesiologist gently placed a mask over his nose and mouth. I playfully sang to him like I always do as his eyes roamed the bright, white, sterile room. Then he looked at me. He looked into me. And my smooth lullaby turned into a staccatoed teary mess because the past suddenly became the present. And he looked scared. Because I was scared. And then he fell asleep.
Just prior to surgery beginning, Zacchaio’s ENT doctor took advantage of his sedation and re-scoped his upper airway. The good news is that his airway is much less floppy than before. There remains, however, over a 50% narrowing (deemed Grade 2) of his subglottis. When we discovered the narrowing three months ago the doctor was hopeful it was due to inflammation related to acute illness, but his current state of health (nothing acute) confirms that it is yet another congenital anomaly caused by his syndrome. Another malformation? ::enter sarcastic gasp here:: You don't say! Time will tell whether or not he will need surgical intervention to help expand the narrowing, but for now it remains problematic only when he is ill. This explains our obsession with hand sanitizer.
Plastic surgery entailed more removal of Zacchaio's scalp nevus as well as a right eyebrow lift (look out, Hollywood!). Persistent scar tissue in Zacchaio's right eyebrow from his previous surgery had caused some sagging over his eye that Dr. Hoffman believed he could remedy.
A pleased plastic surgeon greeted me in the recovery room.
"He did great." He always has, thank God.
"So tell me... his right eye isn't always going to look surprised while his left looks sad in comparison, right? Because that would be confusing..."
"No... I'm pretty sure they're going to be mostly even." I guess we dodged that bullet... mostly?
"How much of the nevus were you able to get?" The surgeon's expectation was that he would require at least one more surgery to finish the job without skin expanders.
"Oh, well... I was able to get it all!" In my excitement I proceeded to shout out a series of ridiculous statements in the recovery room that I am mostly embarrassed to recount. “Ladies and gentlemen, the man with the magic hands!” “It doesn’t get more skilled than this guy over here!” With a lisp I hollered, “Unbelievable… truly inconceivable!” Then there was some kind of chant accompanied by melodic clapping like, “He did it, he did it, he really, really did it…” I was obviously enthusiastic about the end result.
It was one helluva day and two helluva nights riddled with breathing difficulties following surgery, but overall Zacchaio recovered with considerable ease and tremendous resilience. So much resilience, in fact, that he decided to pursue the art of walking the day following his surgery without his heavy head bandage slowing him down in the slightest.
Keep defying the odds, my love.
I know it has been quite some time since we have updated everyone on Zacchaio’s progress. I have found that with every passing day I have become much better at living in the moment. In living in the moment, however, I can often become preoccupied with the happenings and logistics of each day. Allow me, then, to celebrate some of the incredible milestones Zacchaio has reached in the last few months.
It has been 9 months since Zacchaio had a quarter of his brain resected…
…it has been 9 months since his last seizure.
It has been 12 weeks since Zacchaio’s last ER visit/ICU admission. That’s a record.
It has been 12 weeks since we started Zacchaio on overnight oxygen for his breathing difficulties... and it has been 9 weeks since his airway problems spontaneously improved to the point where he no longer needs said oxygen.
It has been 7 weeks since reuniting with our incredible neurology/neurosurgery team at the Cleveland Clinic. Tears of joy were shed, terrifying recollections were revisited, and joyous new memories were made. The Ronald McDonald House was able to accommodate our short stay, and A and Z were ecstatic about the kid-friendly lodging. It was heart-warming to witness Zacchaio enjoying all the house had to offer when just 9 months ago he was bound to a hospital bed following brain surgery. After getting ourselves settled, we ran into a Greek mother whom we had met during last year's stay. It turns out she had never left. And in that moment we were abundantly grateful for the perspective and the forward momentum our lives had gained.
Zacchaio was the life of the neurology waiting room with beaming smiles, bright eyes, and a desire to personally meet and greet every seated patient. Each physician on our team rejoiced in surprise when they saw our boy, no longer a baby, full of life and thriving in every possible way. We thanked them for their relentless efforts, dedication, and success, and they thanked us for the lessons on love, trust, and patience.
Since returning home, Zacchaio has been busy being a flourishing learner. In being a witness to his learning, I believe his brain's comprehension has been the most astonishing. Across the living room we yell, "Zacchaio, go get the block and put it in the box." In pure amazement we watch him crawl to one end of the room to grab a block, then scurry back to the opposite end where the box awaits its deposit. My favorite moment of it all is how proud of himself he is in the end. If only he knew... He's gotta know...
Zacchaio spends majority of his days shadowing his older brother and swiping his toys with sly looks of artificial innocence. When not entertained by Alkaio, he entertains himself by opening, emptying, and closing a variety of cabinets and drawers throughout the house. We never knew whether Zacchaio would ever reach this point, and I must say that I’ve taken a liking to finding his toys and miscellaneous items in unexpected places. They are daily reminders of his remarkable journey; a treasure trove of his stupendous accomplishments.
Zacchaio plays a serious game of peek-a-boo with our electronic garbage can and relishes the responsibility of throwing away his own dirty diapers. He takes much delight in lifting up our shirts to tickle our belly buttons and elicit giggles. When he receives a guffaw, he reciprocates with his own chuckle, an adorable smile, and a priceless accompanying nose-scrunch. I can't wait to catch them on video.
It has been 3 weeks since he took his first 3 steps from the table to my arms and it has been 2 weeks since he walked across the entire living room on his own. With every passing day we see an insatiable desire to master his new skill.
His right leg remains significantly longer than his left (due to his hemihypertrohpy), so while being a new walker comes with its own challenges, his asymmetry adds that little extra obstacle that Zacchaio has grown so accustomed to. I find the slight hitch in his giddy-up rather endearing. He's come a long way in just a a few weeks...
A couple of weekends ago Zacchaio was highlighted as a case study at UCSF's Regional Dermatology Clinic in an effort to further the research and understanding of Linear Nevus Sebaceous Syndrome. We were excited to be a part of the rare learning opportunity for a large number of clinicians. Our hope is that the clinicians in attendance came away with an improved ability to recognize sebaceous nevi and its syndromic characteristics, as these identification skills will help lead them to earlier diagnoses, treatment, and improved outcomes.
The last 3 months have left us feeling very encouraged, yet it seems as if not much time ever passes before we are handed a reminder of our vulnerability. It has been gratifying and humbling and filled with love.
I remember during our challenging first year with Zacchaio how our family was generously showered with affirmations of faith from all of you in hopes of inspiring strength and optimism. One of the most common declarations was that God doesn't give us more than we can handle. Z's first year not only put us to the test, but also helped us gain an incredible amount of insight into our capability to love, to learn, to accept, and to parent. It felt like we might just be moving into a time in our lives with a little more stability....
Except we weren't.
It has been 14 years since my mom was diagnosed with breast cancer and it has been more than 10 years since she was deemed in remission. Just 7 weeks ago she was diagnosed with Stage IV (terminal) metastatic breast cancer.
Though we have had much practice receiving heartbreaking news over the last 16 months, being experienced didn't make receiving the news any easier. Getting the diagnosis was a bomb thrown into the middle of our already tumultuous lives. I cycled through anger, frustration, and disbelief, just as I had when we were in the thick of it with Zacchaio. I questioned God's decisions of who He burdens with challenges such as cancer, as well as his choice of someone who had already proven victorious against such a lethal opponent... Then I stopped being unproductive and took a self-guided 3-day crash course in mom's condition.
Metastatic breast cancer (MBC) is breast cancer that has spread beyond the breast to other organs in the body. Strangely enough my mom does not have any cancer in her breasts, nor has she since her original diagnosis 14 years ago. I suppose that was part of my frustration with Kaiser's breast cancer surveillance plan. Once a person reaches 5-10 years in remission following their first breast cancer diagnosis, the likelihood of cancer returning to the primary site (breast) is less than 5%. So why was there such a push for her to be doing yearly mammogram screenings and nothing else? Kaiser says there is no research to support a different approach. Hmph.
My mom's cancer has spread to most of her bones (except the long bones) and is thankfully not in any other organ or tissue. When looking at the chemical makeup of her cancer, it is estrogen-receptor positive and HER2-negative which is ideal in that the cancer should be receptive to hormone therapy (and hopefully not require chemotherapy) and is slower growing and less aggressive than HER2-positive cancers.
The reality of metastatic breast cancer is that it is terminal; there is no cure, only treatment to prolong life. I suppose it can be alluded to as a chronic illness which leaves the individual always in treatment. Our goal then, is to give my mom the highest quality of life for the longest number of years. My research has led me to conclude that there is a wide spectrum in terms of life expectancy which ranges from 6 months to 15+ years. We're doing everything in our power to be right-winged extremists.
The probable diagnosis of MBC was given to my mom in mid-April with the official diagnosis pending her biopsy pathology which would be received weeks later. My mom was pale, frail, and ridden with pain from various fractures that had left her unable to walk and wheelchair-bound. The light in her eyes had dimmed and the energy in her voice had waned. If you don’t know my mother personally, it was only a few months prior that she, as a 62 year-old, was shaming 40 year-olds in her fitness classes. Talk about life-changing.
Kaiser seemed to move in slow motion once they gave my mom her official diagnosis, and rather than being sitting ducks until the start of traditional therapies, we took matters into our own hands. On 4/20 (the national unofficial day to celebrate marijuana; anyone else enjoying the irony as much as we did?), my mom, dad, and I found ourselves sitting around a table at Constance Pure Botanical Extracts receiving my mom's newest treatment to be started immediately... cannabis (marijuana) oil.
If you’re familiar with Zacchaio’s story, you might remember a time when we opted to use marijuana oil for his seizures when his traditional anti-epilepsy medications failed him. Though my mom's oil is similar to what we gave Zacchaio, her oil has higher concentrations of THC (the psychoactive component of marijuana) compared to Zacchaio's CBD-rich (non-psychoactive and highly anti-inflammatory) oil. My research led me to discover a number of in vivo (live mice) studies that have showed my mom’s combination of THC and CBD cannabis oil to inhibit gene expression in aggressive breast cancer cells, induce death of breast cancer cells, and reduce the potential for metastasis. All of this without the chronic and toxic side effects of traditional cancer treatments. Though politics continue to limit the opportunities for cannabis oil use in human trials, I came across much anecdotal evidence of its miraculous healing properties. The testimonials are inspiring, to say the least, with many given no more than 6 months to live yet still living years later with baffled oncologists. Some even went into remission, a word never used for an individual deemed terminal.
Within a couple of days of first ingesting the oil, my mom's overwhelming bone pain began to dissipate. After 1 week she ditched the wheelchair and was able to stand and walk independently with only a moderate amount of discomfort. Only 2 weeks after her first dose she was completely pain-free. We were all flabbergasted. A month of Motrin and being bound to the couch had afforded her no relief from her symptoms, yet 2 weeks of oil and her pain was nonexistent.
That’s not to say that the oil road hasn’t been difficult. There have been good days and hard days. There were days when she slept for 20 hours in a 24-hour period and there were days when she didn’t know up from down. There was an extended period of time when my mom appeared to be a mere shell of her original self. The whole family was frightened. Now, however, 7 weeks after the start of her oil regimen, her fire appears rekindled and her energy restored. We're all eager to see what next month's scans look like compared to April's. As thankful as we are that the oil has improved her quality of life, we're obviously praying that it is having anti-tumor effects as well.
Sunday, March 8, 2015
“Just keep breathing, just keep breathing…”
After 13 months of almost never leaving Zacchaio's side, I went back to work.
The transition was easier than I expected, though much of that is owed to a mother and daughter caregiver dream team. They are both abundantly joyous and faithfully patient; they mirror Zacchaio’s energy perfectly! Our boy has been more than receptive to their abrupt arrival and we feel lucky to have found them. My favorite part of my workday is receiving candid photos of him in their care. We couldn’t ask for anything more!
|Photos courtesy of Zacchaio's caregivers!|
Zacchaio had a hearing test to evaluate for hearing loss since brain surgery; the removal of his right temporal lobe has the potential for negative effects on his hearing, language, and memory. He was placed in a small room with sounds of differing frequencies and volumes projected to his left and right. If he turned the right direction, a pleasant image would appear in hopes of reinforcing the correct behavior. The wonderful news is he scored as an average child and passed his exam! All signs point to normal hearing in both ears. I remain awed by the ability of his brain to compensate for loss. God has constructed these bodies of ours so skillfully… It has been an honor to slowly uncover the hidden potential He has infused into every cell in Zacchaio’s body.
Over the past couple of months, Aidan and I have noticed a worsening of Zacchaio’s baseline snoring. He began falling into cycles of snoring where each subsequent inhale would increase in pitch until he would meet complete resistance, quickly readjust his sleep position, and start the cycle all over again. Though there is a certain amount of snoring and stridor (harsh, noisy breathing) expected with his laryngomalacia/tracheomalacia (floppy airway), it was causing restless enough sleep to warrant a sleep study. It was on a Tuesday night that Aidan and Zacchaio checked themselves into a hotel in Japan Town (UCSF tries its best to mimic your home sleep environment), and it was soon after that the sleep technician went to work attaching an inordinate number of wires, sensors, and gadgets to Zacchaio’s head, face, chest, arms, legs, and stomach. So much for mimicking his home sleep environment…
|Sleep study hotel|
|Going into battle!! For a sleep study, that is.|
Based on the results, Zacchaio has been diagnosed with severe obstructive sleep apnea. During his 8-hour sleep study, he had over 110 hypopneic events (decreased amounts of air movement into the lungs that cause oxygen levels to drop), and just under 10 apneic events (complete cessation of breathing). What was somewhat reassuring is that his oxygen levels stayed above 90% for majority of the study with lower levels in the 80s occurring only 0.5% of the time. Add it to the list…
Now for the latest.
About a week ago Zacchaio’s nose began to run. The infamous runny nose served as an alarm for Aidan and me to don our battle gear. We put ourselves into a hyper-aware state and initiated our regimen of proactivity in hopes of avoiding potentially debilitating outcomes. During the next two days, Zacchaio gnawed on every surface of the house; his two front teeth finally broke through and his runny nose ceased. A victory, indeed! With heavy sighs of relief, we resumed our normal levels of awareness until Zacchaio woke up Friday night with higher-pitched breathing and a very high-pitched cough. We immediately recognized it as croup, and that coupled with Zacchaio’s history of airway disease got us a prescription for a steroid to help lessen the inflammation in his airway. The drug worked beautifully and Zacchaio was asymptomatic within hours of his first dose. That is, until Saturday night.
As Aidan and I were heading to bed, Zacchaio woke up for what we thought was a midnight bottle. When I picked him up, he attempted to cough and clear his throat; he could barely expel any air. He tried to take a breath in but his efforts were met with complete resistance, a brick wall of sorts, and nothing got through. His head drooped in front of him and he began to drool. He tried to cough again, but nothing. He started to gag. He tried to take another breath in, but his airway was clamped shut. He started to panic. We started to panic. All I could think about in that moment was the incredible amount of durable medical equipment we had all over our house, yet the only equipment we didn’t have and desperately needed in that moment was oxygen. In an effort to calm him down, I ironically began to frantically slap his chest. My actions could easily have been interpreted as agitating, but I was making my best attempt at a thymus thump while contemplating our next move. Aidan was ready to call 9-1-1, but I told him I could get Zacchaio to Eden’s ER faster than an ambulance could get to us.
Aidan stayed home with Alkaio and began to pack the overnight bag. At 12:30am I got into the front seat of our car with Zacchaio on my lap (I couldn’t stand the idea of him turning blue or going into cardiac arrest while strapped safely and legally in his carseat). I thumped his chest and disobeyed all speed limits (and maybe a few signals) down Castro Valley Boulevard. By the time we stepped foot into the ER, Zacchaio had an extremely high-pitched whistle upon inhale. At least he was inhaling. I made the decision at that point that he would be better served at UCSF where they specialize in little people and were exceedingly familiar with his medical history.Zacchaio was admitted to the ICU that night for a two-night observation. Nobody could explain the ALTE (Apparent Life-Threatening Event). They believe it was a combination of his airway defects, the acute croup, and the sleep apnea, though we still lack definitive answers. On the morning of his discharge, Zacchaio had a flexible laryngoscopy (a small flexible camera was put down his nose to view his airway) which not surprisingly showed some swelling and floppiness. Ultimately his admission comprised more of doctors and nurses aww-ing at his cuteness and giggling at his silliness than actual intervention. He didn’t seem acutely ill in the least and rather enjoyed his time in his “bubble-top crib.” It was obvious that Zacchaio was the talk of the unit, as he got multiple social visits from doctors who no longer see him and many “hellos” and peek-a-boos from passing nurses and ancillary staff.
Thankfully our ICU visit lasted only a couple of days, and when Zacchaio heard he was going home he responded with… (a first!)
|Bubble top hospital bed|
|Playing with mama|
Our time home was short-lived; only two days later we were back in the ER due to Zacchaio’s nighttime apnea worsening. The admission prompted doctors to shorten their timeline in terms of diagnostic testing and intervention, and Zacchaio was put under anesthesia for a bronchoscopy (a scope placed down his airway to visualize his throat, larynx, and trachea). If doctors were to find an isolated narrowing, the plan was to inflate a balloon to expand the narrowing in hopes that the expansion would remain once the balloon was removed. Doctors were disappointed to find that Zacchaio’s entire upper airway was hypotonic (low tone) and floppy. We were told airways like these are typically seen in children with severe cerebral palsy, not in children as vibrant and active as Zacchaio. Our little man has proven time and time again that he excels at puzzling experts in every medical specialty. We will support him enthusiastically when he is ready to find a new hobby…
The easiest surgical solution to the problem would be to bypass his defective airway with a tracheotomy (an incision made into the neck that creates a new airway directly to the trachea). This is something typically done on CP children who don’t speak and are hypoactive. Another option would be a uvulopalatopharyngoplasty, a trimming of Zacchaio’s soft palate, to make the airway opening larger. This surgery is rarely done in children and has the risk of creating an airway leak which would leave Zacchaio speaking in somewhat of a whisper with a speech impediment similar to a lisp. Even if we did decide to move forward with the palate trimming, there is no guarantee that it would remedy Z’s airway problems, as the rest of his airway would still be floppy and could continue to close in on itself.
|In recovery after his bronchoscopy.|
The plan moving forward is to bring him home on overnight oxygen. Though his oxygen levels remain in a satisfactory range during his episodes of apnea, our hope is that the positive pressure from the oxygen flow helps keep his airway slightly more open while he sleeps. We’ll have a repeat sleep study to quantify how much the oxygen is helping (or not) and then move on to an undetermined (as of yet) Plan B.
Until then his joy continues to radiate and his heart remains pure.
There’s never a dull moment in “The Story of Z.”
|Zacchaio and Alkaio both at one year...|
|Downtime between neurology and orthopedic second opinions|
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