We are planning to partially remove and completely disconnect the right half of our son's brain...
... and as disturbing as the above statement sounds, we consider ourselves lucky.
After meeting with Dr. Pearl (neurologist) and Dr. Madsen (neurosurgeon) at Children's Boston, we had the following options for managing Zacchaio's epilepsy:
Option 1: Try the anti-epileptic drug Vigabatrin, a medication specifically indicated for infantile spasms. If it were to work, Zacchaio would be seizure-free; but not without risk. The drug has potential side effects of permanent peripheral vision loss, reversible effects on deep brain structures, possible movement disorders, respiratory dysregulation (changes in breathing patterns), etc. Due to its high potential for toxicity, it can be used for a maximum duration of only 1 1/2 years.
The opportunity for Zacchaio to be seizure-free for a year and a half is enticing; we need to keep in mind that this year and a half would be during a critical developmental period where he would be under the influence of two anti-epileptic drugs (he is also currently on Phenobarbital for an additional seizure type) which by nature subdue the brain's electrical activity. Although he might make developmental progress, his gains would not be as great as if he were off anti-epileptic drugs completely. Following a year and a half of potential seizure freedom, we would find ourselves back at our only remaining option, surgery. The potential for permanent vision loss with Vigabatrin therapy coupled with the vision loss Zacchaio will definitely inherit with surgery could leave him almost completely blind. Bad idea.
Option 2: Try ACTH (injectible steroids) or similar drugs. These types of drugs are often most effective against infantilie spasms, but they also compromise heart function, decrease blood pressure, and suppress the immune system, among other things.
With Zacchaio having a heart arrythmia with a blood pressure that tends to run on the low side, this option is not safe.
Option 3: Surgery. A right functional hemispherectomy.
How is it performed?
The largest part of the brain is known as the cerebrum which is divided into two hemispheres. In a 5-8 hour procedure, Dr. Madsen would skillfully remove Zacchaio's right temporal lobe, then disconnect all remaining lobes in the right hemisphere. Part of what complicates the procedure, among many things, is that he will have to do all this while remaining superbly cognizant of Zacchaio's cerebral vasculature to avoid excess blood loss. Removing the temporal lobe would give him more direct access to the corpus callosum, a thick band of nerve fibers that helps the two hemispheres communicate, which he would ultimately sever in order to prevent the spread of seizures from the diseased right hemisphere to the healthy left hemisphere.
What would he be losing?
The right hemisphere controls all left-sided function, including its sensation and movement. In addition, it is responsible for the left half of vision in both eyes. By losing his right hemisphere Zacchaio would no longer have the ability to feel or move the left side of his body; he will also acquire a left-sided visual field loss. Zacchaio is likely to have decreased visual organization and visual memory skills, left-sided neglect, decreased non-verbal problem solving, impulsivity, and difficulty with math.
Although in choosing surgery we are electively disabling our baby boy, there is reason for us to believe in the potential for normalcy.
Why do the surgery?
Generally speaking, there is no cure for epilepsy, only life-long management. This is one of the reasons we consider ourselves so fortunate... Zacchaio's seizures have remained isolated to his right hemisphere, which is exactly what qualifies him for a surgical treatment option that has the potential to be curative. Time is now of the essence. The longer the seizures remain uncontrolled (as they are currently), the greater the potential that his healthy hemisphere becomes damaged or is recruited to seize similarly to the diseased hemisphere.
Seizure freedom and the promising concept of "brain plasticity" gives us hope that there is normalcy to be had. Brain plasticity is the concept that the brain has an innate ability to be shaped by experience, as well as a unique capacity to learn, reorganize, and recover following an injury. Regardless of the amount of traumatized, damaged, or entirely missing brain, it is thought that remaining healthy brain has the capability to create new neural pathways that can compensate for lost functions. Zacchaio will eventually regain sensation of his left side. He will learn to crawl by scooting on his bottom, will likely walk with a limp, and be able to move his left arm without fine motor movement in his left hand. At least that's what they say. In his 7 months of life, Zacchaio has managed to surpass everyone's limited expectations. Although outcomes following surgery remain variable, we focus on the remarkable recoveries made by determined half-brained children and their incredibly supportive families. A sweet 5-year old right hemi girl is in ballet fifth position with only one arm above her head and "lefty" dangling at her side looks just as graceful as her fellow dancers. A teenage right hemi girl posts a "how to" video on how one can independently tie their hair into a ponytail with only one hand. A tender moment is witnessed when a young left hemi boy helps guide his healthy baby brother down the stairs safely with his good hand.
The stories are inspirational, and I know Zacchaio's will be one of them.
Research has shown that the earlier in life brain trauma occurs, the greater the opportunity to recover function. Traditional epilepsy management asks for the exhaustion of drug therapy prior to the consideration of epilepsy surgery. With that said, most children who are having epilepsy surgery are much older than Zacchaio, have already failed at least 8 anti-epileptic drugs, and are showing severe developmental delay or regression. There is a reason that every physician we mentioned surgery to 6 weeks ago thought we were crazy. Zacchaio has failed only 2 anti-epileptic drugs. He has very minimal developmental delay and continues to make huge gains in spite of having as many as 300 seizures per day.
We're in the fast lane, and we're headed straight toward a better life.
Just prior to leaving Boston to return home to California, Cleveland kept nudging its way into my consciousness. I mentioned in a previous post the fantastic opportunity we had to receive an online second opinion from the Cleveland Clinic so as to avoid another trip, but a nagging feeling was telling me that it just wasn't good enough. Trusting our instincts, we boarded a plane and arrived in Cleveland 3 days ago. Much gratitude to Team Zacchaio for allowing us to follow our intuition!
We met with Dr. Elaine Wyllie, an epileptologist who has publications surrounding epidermal nevus syndromes as well as Zacchaio's specific diagnosis. She was... incredible. Her process was a reflection of her extraordinary expertise. She exhibited great compassion, understanding, and dedication to our son's well-being. She complimented us repeatedly for our proactive methods and being remarkable advocates for our son. Everything about her and Cleveland felt right.
In reviewing all of the data we acquired from Boston, she first offered Boston's options #1 and #2. After respectfully declining the two, she followed with a slight difference in Boston's #3. Her preliminary surgical proposal was a right posterior quadrant resection, or in other words, a removal of only the back half of Zacchaio's right hemisphere. She believes that most of Zacchaio's abnormal electrical activity (based on EEG, MRI, and PET) is originating from his temporal, parietal, and occipital lobes, not his frontal lobe. In having the posterior resection instead of the hemispherectomy, we would be preserving Zacchaio's motor and sensory cortex which would eliminate the left-sided weakness/paralysis he would acquire with the other proposed surgery. With her approach Zacchaio would still lose half of his visual field and seizures could eventually manifest within the preserved brain. If seizures were to return, another surgery to complete the entire removal of his right hemisphere would quickly follow.
Where do we stand today?
A right functional hemispherectomy is scheduled for August 22nd at Boston Children's.
Dr. Wyllie at Cleveland was able to work impressive last-minute magic and schedule Zacchaio for a 72-hour EEG this Friday. On the following Tuesday morning Zacchaio's case will be presented at Cleveland Clinic's surgical conference, where 70 sets of eyes will have an opportunity to weigh in on proposed surgical intervention. Immediately following the conference, we will meet Dr. Bingaman, the neurosurgeon who will be doing Zacchaio's surgery if we ultimately select him over Dr. Madsen at Boston Children's. We will make our final decision regarding which procedure and which medical facility on Tuesday. If we select Dr. Bingaman at Cleveland, surgery will follow shortly after.
We have received only rave reviews for both surgeons and believe that Zacchaio will be in skilled hands regardless of our choice. As his parents, we have to listen to our instincts carefully, as our decision will greatly impact the framing of the foundation for his new life. I imagine the moment Zacchaio is lying on the operating room table and his surgeon looks at him one last time before turning toward the monumental task ahead of him... my hope is that he sees him not only as a brain, but as the angelic soul that he is, the understated champion, and the powerful being that unites communities through faith and love. I want them to be a part of the miracle that defines Zacchaio's next chapter. May God continue his miraculous work through our baby boy...
As I'm reading I'm thinking people should never have to be confronted by decisions like this. But you are. And you're sharing with us in the most tender way, bringing us along with you. I'm an old friend of Erin's. My heart is with you.ReplyDelete
Team Zacchaio here! The prayer warriors are lifting up the Thiele family everyday through this journey. My life verse is Jeremiah 29:11 and every time I read this it reminds me of Zacchaio. His story has been orchestrated since before anyone knew about him. God has major plans for Zacchaio and his story has been told. This tiny baby has had (and still having) such a great impact on so many already. Alexis and Aiden I admire you both! Seeing you all walking together strong as one through Christ is amazing. We love you all (Alexis, Aiden, Alkaio, and Zacchaio) so much and are here for your family if you need anything. XOXO
For I know the plans I have for you,” declares the Lord, “plans to prosper you and not to harm you, plans to give you hope and a future.
Thank you for keeping us update through this voyage.