Monday, June 23, 2014

A bittersweet homecoming.

My last post had no resolution. I anticipated this one would, but I was wrong.

Zacchaio was discharged home on Tuesday night after a 6 night stay at UCSF. Being discharged from the hospital is normally reason to rejoice, but knowing we were coming home to a life full of epileptic uncertainties made our departure rather bittersweet.

Prior to leaving, we spoke with our epileptologist, Dr. Cilio. Based on Zacchaio's EEG, he has regular interictal spikes (electrical discharges between seizures) that last 1 second every 4 seconds all over the right hemisphere of his brain. They technically aren't considered seizures because they don't last at least 10 seconds. There had also been no signs of his blue seizures since his admission six days earlier. Although the episodic winking of his right eye appears to be seizures clinically, due to a lack of correlation between them and EEG spikes, she is calling them involuntary motor responses. Per Zacchaio's MRI, no structural abnormalities are causing his seizures, therefore we continue to live in the daily bewilderment of his being. Every epileptic child is a unique puzzle, and every passing day will provide us more clues as to how the pieces fit together.

Right temporal lobe epilepsy

Tuesday, June 17th
We enjoyed a decent night of sleep. The first in a long time.

Wednesday, June 18th
We started keeping a seizure diary.
138 winks.

Thursday, June 19th
5 blue seizures.
112 winks.

It was an unfortunately eventful day. The antiepileptic medication, Trileptal, was supposed to keep the blue seizures at bay. We were scared and discouraged and realized that Zacchaio needed 24-hr observation until his status was more stable. We alternated being awake with him through the night in 3-hour shifts, hopeful that his seizures wouldn't cluster or last longer than 3 minutes (thus requiring his rescue medication, a 9-1-1 call, and a prayer asking that he sustained no brain damage).

Aidan and I found ourselves struggling in our new role as parents of an epileptic child. Will it always be like this? Will we ever be able to leave the house again? Where do we put the rescue medication so we eliminate the possibility that it's ever forgotten? Can he ever sleep in his bed again? How do we know if he seizes at night? How do we know if he seizes in the car?

We always envisioned ourselves thriving as a family... Right now we were merely surviving.

Friday, June 20th
4 blue seizures (3 in one hour which was nearing the definition of a cluster).
170 winks.

In the moment it felt like his condition was worsening. His Trileptal blood levels should have been more therapeutic and therefore decreasing the frequency of his seizures. We called the on-call Neurologist multiple times on Thursday and just as many times on Friday.

Recommendations evolved with each call:
- "Just be patient and give the medication time to be therapeutic."
- "Is he recovering on his own after seizures? Is he tolerating his feeds?"
- "We're not concerned unless his blue seizures last at least 3 minutes or he clusters 5 seizures in one hour."
- "Let's increase his Trileptal to 2mL from the 1.5mL we've been giving him."
- "I'm thinking we add Phenobarbital back because he was so well-controlled with it in the hospital."

Zacchaio had been diagnosed with epilepsy just one week ago and he was already failing his first anti-seizure medication? I can't say I was surprised. A number of those diagnosed with Linear Nevus Sebaceous Syndrome and epilepsy had failed all mainstream anti-epileptic drugs and succumbed to taking non-FDA approved medications. A bit frightening.

I started taking advantage of my quiet night shifts with Zacchaio and began researching alternative treatments to epilepsy. The two we added immediately as adjunct therapies were:
- Fish oil supplementation (thought to help reduce inflammation in the brain and has decreased seizure frequency in some epileptic patients)
- Frankincense oil (has sesquiterpenes in its molecular makeup and is thought to cross the blood/brain barrier and stimulate the brain in positive ways)

We'll see...

Our Hail Mary treatment option will be called upon if traditional anti-epileptic drugs continue to be ineffective for Zacchaio... Medical marijuana. **Cue the ironical laughter!**

If you know Aidan or me well, you know we are straight arrows. Really straight. We have never had a drink, never tried a drug, and never believed in premarital sex. Neither of us are proponents of marijuana legalization... but desperate times call for desperate measures.

Charlotte's Web is a strain of medical marijuana that is high in cannabidiol (CBD) with no THC (the psychoactive component that leaves you "high").

In the last two years there has been great buzz surrounding the topic with a couple of documentaries (Weed/Weed 2) giving light to the highly controversial and experimental treatment. The treatment was first made available in Colorado upon the legalization of marijuana last year. Hundreds of families with epileptic children uprooted their lives and fled across state lines pursuing a miracle... and a miracle many of them witnessed.

Our epileptologist is currently spear-heading a CBD drug trial for children with intractable epilepsy out of UCSF. Zacchaio cannot qualify to be in the study until he has failed at least four anti-epileptic medications; it's nice to know, however, that incredible resources are at our fingertips.

We're not there yet, but at least mentioning it now will lessen the shock value when you read a future post saying Zacchaio was the first in our family to try weed.

Saturday, June 21st
1 blue seizure.
NO winks!

I was finally feeling hopeful...

Sunday, June 22nd
1 blue seizure.
139 winks.

I think I need to get used to this being a really, really, really slow process. 

So where do we stand? We stay home all day taking turns holding our sweet baby boy. We keep tweaking medication doses to find a balance between seizure control and tolerable side effects. We log every blink and prepare for the worst with rescue med in hand every time he starts to seize. We elicit the help of our loving family to take a 3-hour night shift so we're of relatively sound mind to take on another day.  

Alkaio remains the incredible big brother he always promised to be. Lots of hugs, lots of kisses, lots of empathy for his brother's hardship, and lots of concern for his upcoming surgery. In an effort to not overwhelm him with Zacchaio's heavily medical environment, we bounce him from activity to activity with friends and family. We're grateful for his understanding and patience as he takes a back seat to his brother.

We continue to fight the good fight as soldiers for Zacchaio. I thank God I have Aidan, who often carries me, the walking wounded, on his shoulders. Praying for peace, strength, and patience. Sending love from our family to yours...

Tuesday, June 17, 2014

Waiting... and waiting... and waiting.

I recall talking to a few moms of children with Linear Nevus Sebaceous Syndrome who also had epilepsy... They referred to this process (meaning the first time their child ever seized) as some of their worst times ever. I now see why. Oh how I wish it was as easy as taking a single medication and never seizing again.. Unfortunately, finding seizure management is trial and error, with breakthrough seizures and the horrible side effects of psychotropic drugs along the way. We've watched Zacchaio be euphoric over nothing, thrash his head dangerously against his bed, stay asleep and limp for 12 hours straight, become terrified amid deep sleep, make eye contact with us and not recognize we are his parents... All things I never thought I would experience as a mother. And so with bated breath following every new intervention, we wait to see which Zacchaio awakens and maintain hope that in the end it's the one with that signature Zacchaio sparkle in his eye. 

Zacchaio kept the EEG monitor overnight and after seeing constant firing for hours on end within the right hemisphere of his brain, they gave him Ativan (a sedative) in an attempt to calm the activity and provide him restful sleep.

Pediatric neurology signed off today, though not in the traditional sense. They passed the neuro baton to a pediatric epileptologist, one specializing in his new diagnosis. And we wait...

Having seen much success in Zacchaio's hemi-hypertrophy after beginning his homeopathic remedy, we also elicited the help of our homeopath to begin taking an additional remedy for his seizures. And we wait...

I haven't seen Alkaio for 5 days now and my heart is aching for a large dose of his 4-year old joy. After talking to him on the phone yesterday he told me he had another dream. "Dad, you, me, and Zacchaio were all at Disneyland!" We talked about him riding Buzz Lightyear and Space Mountain with Zacchaio one day. He and his brother riding together, and Aidan and me riding behind them. "And after we ride Space Mountain, me and my brother will say, 'Let's do it again!' And we do!" I was flooded with emotion and the feelings seemed all too familiar. It was because him telling me about his Disneyland dream reminded me of his elevator button-pushing dream while Zacchaio was in the NICU and how I dreamed about such a silly dream coming true one day. The unknown is impossible to contemplate, yet I find myself doing it constantly. I cried knowing that Alkaio was missing his little brother. I cried at the vision of Zacchaio not having the brain capacity to enjoy the childrens wonderland. I know that his future isn't defined yet, but right now the only way I can cope is to assume the worst in hopes that I'll be pleasantly surprised with an outcome that's any better.

Monday, June 16, 2014

Is there an end? Nothing on the horizon yet...

I'm desperately hoping that by writing this blog I can distract myself just enough to avoid a complete meltdown.

As I mentioned in my last post, our first couple of days on Phenobarbital were rough, but they did stop the seizures. The plan was to stop the Phenobarbital and start Trileptal, a drug with more tolerable side effects. The first 24 hours on Trileptal went well. Zacchaio remained seizureless and although very lethargic at first, he eventually came back to his baseline activity level.

I've never believed in the superstition that is Friday the 13th, but after experiencing our own a few days ago, I now have eternal friggatriskaidekaphobia.

Sometime mid-day on Friday before I gave Zacchaio Propranolol (one of his heart meds), I noticed the label stated 1.3mg instead of 1.3mL (of a standard concentration). It turns out 1 of his 3 meds was ordered incorrectly and he had been getting just under a quarter of his usual daily dose since Wednesday. Although the error threw me for a loop, I was more enthusiastic about the fact that his heart rhythm/rate was looking better now than it has ever looked before! The physicians said and did all the right things to remedy the error, but following its discovery, our cardiologist decided to trial him off the medication altogether. We changed a variable, and now it was a matter of waiting and seeing...

Later that night I was holding Zacchaio and noticed that he suddenly felt warm. His temperature came back 101.4 degrees. Great, that's what we need. Within the next few hours he started getting congested and began having lots of secretions that made it slightly more difficult for him to breathe. Great, that's what we need.

A few hours after that we noticed a strange winking of his right eye. We initially wrote it off as "weird baby behavior," but he began doing it rather repetitively. So we took advantage of modern day technology and took a video on our smart phones. We let the nurse know, who let the resident doctor know, who then came to the bedside and was able to witness a blinking event. I was convinced it was a seizure. She said, "maybe he just has an eye irritation?" I gave her a dirty look, because I don't like being dismissed, yet managed to kindly request that she let the neurologist know. After a game of telephone was played in the reverse order, we were told to keep an eye on the behavior and to let them know how many times it happened overnight. Fine.

On Sunday morning the doctors were feeling good about sending us home. I played along, asked many questions, but as much as I tried to be on board with the discharged; something irked me. One of the pediatric doctors picked up on my hesitance and after explaining he had only been fever free for 8 hours, he had only been off his Propranolol for 12 hours, and he was still doing the eye wink... She understood. There were too many variables haunting my dreams, too many loose ends. So we stayed overnight.

Aidan and I were not aware that Neurology had "signed off" on Zacchaio's case on Saturday, but that explains why we never had an opportunity to see them on Sunday... even though he kept winking.

Fast-forward to this bright and lovely Monday morning where doctors came through in waves reminding us of our impending discharge. I felt comforted knowing Zacchaio hadn't had any fevers since Friday night AND was still managing an amazing heart rate and rhythm without any Propranolol. All great news! Except he was still blinking. Neurology didn't have any intention of coming to see us this morning (since they had signed off on Saturday), but I was annoyingly persistent in my request to see them.

Eventually they came, and after listening to me describe the winking event, they didn't think it was a seizure. So I showed them the video to convince them otherwise. And they agreed that the repetitive winking mimicked seizures. Now we're taking a closer look.

Zacchaio had the EEG monitor placed again earlier this afternoon and they are in fact seizures. The neurologists are having a hard time identifying where they originate because the muscle spasms in his eye are muddying up the reading.

The short-term plan is to increase the Trileptal (anti-seizure) dose and see if his winking spells go away.

We're feeling discouraged. Zacchaio's life story has been, "But wait, there's more!" My plate is full, too heavy, and I don't think I can take much more. The last 5 days have been over-saturated with new findings, frustration, disappointment, and grief. I've managed to keep it together all 5 days until today. My emotional reserves are empty and we're praying for a break, as we always do, so we can refill the tank and continue on this path of hope, love, and patience.

Sunday, June 15, 2014

Seizing the day? No, he seized today.

Wednesday morning was no different from any morning. Zacchaio was his usual joyful self when his eyelids started turning a bit pink and his sleepy nose and eye-rubbing ensued. I laid him on his stomach on the couch next to me and patted his back to help him drift off to sleep. Just as he was getting himself comfortable, he took on a belly flop position (with an arched back) and started to have full-body shakes. I continued patting his back for a full 5 seconds with the initial thought that he was more anxious and restless than usual, but something began to not feel quite right. I gently turned him over whispering, "you're okay love bug..." but he wasn't. He had turned a dusky red color and gone as rigid as steel. I yelled his name and he didn't respond. I shook him somewhat violently and he didn't respond. I slapped his cheek and he didn't flinch. I pulled him into my chest thinking my warmth would magically wake him up... but that failed, too. When I pulled him away from my body one last time, he went from rigid to completely limp. He started to turn purple, blue-lipped, then a dark blue/gray from head to toe. Even though I was holding him tightly in my arms, I felt as if his soul was slipping between my fingers. I screamed in hysteria. I was frantic, then debilitated, mad at Zacchaio and then mad at God, and then I got it together.

I checked for a pulse, and there wasn't one. I started doing chest compressions while trying to dial 911 with trembling hands. 9-9-1-1-1-1-1. Come on! 9-8-8-1-1. Damnit! And finally after a deep breath and a pause from compressions, 9-1-1.

He came back. God let him come back! He might have only been gone for a single minute, but his short absence felt like a lifetime of loss.

The paramedics were there within 10 minutes and Aidan within 15. I caught Aidan just before the school buses departed for the 8th grade Great America trip. Thanks, God. Sorry for being mad at you earlier.

The paramedics had Children's Oakland in mind as our final destination, but after a convincing monologue about the rarity of our son's condition and all his specialists being at UCSF, they somehow pulled strings and were able to get him there. I was grateful for their persistence, as seeing the familiar faces of our medical family was incredibly comforting, especially during a time of such anxiety and fright. Thanks, God. Sorry for being mad at you earlier.

I had to tell the story of my son's departure and return to every doctor that came through the ER. It never got any easier to tell. We were soon transfered to the ICU where he was closely monitored while physicians bought themselves time to work up whether he had a cardiac or neurological event. Within an hour he had another episode, this time without the violent shaking. He suddenly appeared to be stuck in a gaze, started turning grey-blue (not as dark as before), then came to within 30 seconds and got his pink color back. Physicians ordered an EEG (which looks at the electrical activity in his brain), however before the technician could arrive, it happened again. With a couple of dirty looks from Aidan and me, they put some urgency on the EEG technician and it was on JUST in time to catch his third seizure, which I managed to capture on video about 10 seconds in...

We immediataely started a loading dose of Phenobarbital (a powerful sedative/anti-convulsant) to get the seizures to stop, which it was successful in doing... at a price. Believe it or not, it was almost as painful watching him wake up a phenobarbital zombie as it was watching him seize in the hospital. It was unknown to us whether or not our sweet and cheerful son would ever return to us post-seizures and medication. An entire 24 hours went by before the light in his eye returned. In fact, he was back with more energy than ever before! Although he exhibited the severe lethargy associated with phenobarbital, he also had the rare side effect of hyperactivity! He was almost dangerously spastic, but I was grateful to have a spastic Zacchaio than the alternative.

Test results...

The EEG captured one focal/partial (isolated to one part of the brain) seizure with other small random misfirings in Zacchaio's right temporal lobe. The right temporal lobe is responsible for long-term memory, and visual/auditory recognition and processing. The neurologist told us they would not expect to see any irreversible or long-term brain damage based on the small number and short durations of his seizures. They validated their theory with MRI imaging that showed no brain injury. Thanks, God.

Zacchaio's brain MRI showed no structural abnormalities (often the cause of seizures), which leads the neurologists to believe that his seizures are being caused by mutated brain cells that fire at incorrect times in incorrect directions. 

The MRI did find thickening in the back right portion of Zacchaio's skull. Their finding is consistent with his hemihypertrophy and one-sided overgrowth. Their concern is that if it continues to thicken, it could potentially infiltrate the space into which his brain needs to grow. 

Tomorrow we will start trying to wean Zacchaio off Phenobarbital and onto Trileptan, an anti-siezure medication with fewer serious side effects.

God is forgiving and blessed us in ways that I couldn't see until now. I'm grateful our baby boy is still with us and in the hands of our loving God and UCSF family.

Thursday, June 5, 2014

Our new normal still feels really abnormal.

Aidan officially went back to work last week and I didn't realize how much of a crutch he was until he was gone. The two of us had a system in place that worked so well. One could even call us a well-oiled machine, just one in need of a "sleep mode!" For the next two weeks I have to put my big girl pants on and try my best to come out the other end with a smile, or at a least a crooked one.

I/We feed Zacchaio every 3 hours by first attempting an oral feed (for 30 minutes), then finishing however much milk is left via his NG tube (takes about 40 minutes). As soon as I take a deep breath following the stressful process, we have to start it all over again an hour and a half later. Sprinkle in diaper changes, physical therapy exercises, heart medications, and the endless washing of tubes/bottles/syringes; I'm looking at no more than 10 minutes to remember I'm a human being that has innate needs to remain hygienic, hydrated, and fed. Lately I've been forgetting to do those things.

In the last few weeks I've found it very easy to lose my sense of self. The only way I rediscover myself is when I'm out and about, running errands, spending time with friends and family, throwing Alkaio a mini birthday bash, feeling the warm sun on my ghostly (what is the antithesis of Greek) skin... Pretending we're normal. As difficult as it is to leave home with a special needs baby (and all the supplies that come with him), I've found it's 100% worth the forethought and stress. My alternative is hiding away in our house-cave where all I have to think about is providing non-stop special care to my baby boy. That in and of itself sometimes gets me spinning out of control with grief that "normal" will never be on the horizon. In looking for a little more balance and a little less insanity, I've decided to stop nursing and pumping. I barely have enough time to pump between feeds, and in reality, the quickest way to get Zacchaio's NG tube out is to get him bottle-fed since I'm going back to work at the end of the month.

Through coughs and gags, Zacchaio takes anywhere between 10mL-50mL per feed by mouth with his goal being 110mL. Although our progress is slow, I have to remember to acknowledge that it's progress nonetheless. We continue to pray that the soft cartilage that makes up his larynx stiffens sooner than later. I have found quite a bit of support from other moms of children with laryngomalacia and it seems as if its end ranges anywhere from 6 months to 2 years. It's hard not to wish the time away. Though I cherish my time with Zacchaio, part of me wants to push fast-forward just until the airway problem disappears so we can finally go about our life tubeless. Until then I'll keep experimenting with sleep wedges and milk thickeners in hopes of helping Zacchaio be more successful.

Aidan and I were getting about 4 hours of sleep per night while we were on Zacchaio's every-three-hour feeds. The good news is our pediatrician okay-ed us to trial skipping his 3AM feed, and without it he is still gaining weight! Sleeping 6 hours straight is MAGICAL! Though I'm still somewhat dysfunctional with the 6-month sleep deficit, I'm not nearly as clumsy or reactionary as I was before. I think Aidan appreciates it. :o)

Zacchaio continues to have weekly physical therapy and his neck is most definitely getting stronger. His head still lags when pulling him up from his arms, but if we pull him up from his shoulders he's now able to maintain a neutral head position until he's sitting upright. His head is also still cocked a bit to the right, but its much better than a couple of weeks ago!

We see cardiology in a couple of weeks and then have surgery scheduled on July 11th (to remove his scalp sebaceous nevus  and aplasia cutis blister). It seems like just yesterday surgery was a distant to-do; it's now frighteningly near.

His scalp sebaceous nevus and largest aplasia cutis to be surgically removed in July.
Thank you to Fr. Tom and Fr. Ninos for Zacchaio's "churching" on Sunday. I was surprisingly overwhelmed by emotion as he was blessed and taken to the altar. We are so grateful for our church family and the prayers that have propelled Zacchaio's healing. He is a child of God and continues to prove that in faith, miracles happen!

Although Aidan and I feel frayed at the edges, there's no untying the knot of unconditional love that holds us and our family together. 

Just when I feel like surrendering, I remember that the struggle will make living this life that much more worth it.