We have been navigating interesting roads the last week...
In an attempt to remain functional individuals, Aidan and I planned to take turns staying overnight with Zacchaio and sleeping on the heavenly mattress at the hotel just across the street. After volunteering to stay with the baby the first night, I pulled out what was supposed to be a mattress from the provided chair-bed and was completely appalled at what lay before me. In an effort to avoid being ridiculed as a Princess bothered by a mere pea, I decided to at least lie on the bed. To my dismay, I felt each of the 40 springs in the jack-in-the-box mattress putting painful pressure in 40 awkward places. As much as we appreciate Boston Children's, we are not very fond of their sleeping accommodations.
I proceeded to make a distressed phone call to Aidan which ended in, "...not suitable for a human!" My hero of a husband calmly replied, "If anyone should get a good night's rest tonight, it should be you, babe." Dramatic pause. "Plus maybe you won't be as cranky and mean to me." He spoke such truth.
For two nights Aidan battled the dreaded sleeping surface... until we had an idea.
Hello, Aero Bed! Thank you, donors, for giving us a sleeping space!
After 72 hours of EEG monitoring, we were given an update. In addition to Zacchaio's initial blue seizures, he was also having infantile spasms which, contrary to their understated name, are known as one of the more catastrophic childhood epilepsies. This is mostly due to the extreme difficulty of controlling them and their close association with mental retardation. They are so subtle that parents often do not realize they are happening until they go undetected for months, then worsen, and then eventually manifest more visibly. The earlier the diagnosis and treatment, the better the developmental outcome.
Normally this would be devastating news to a parent; however, three weeks ago Aidan and I took Zacchaio to our neurologist and said, "The winks have progressed into what appear to be absent gazes. Sometimes they're accompanied by left arm movement. I'm pretty sure he's having infantile spasms." To which we were asked, "Is he shrugging his shoulders when he does these movements with his eyes?" "No." "Then they are not infantile spasms."
Shrugging is not exclusion criteria.
I was demoralized and I raged with resentment, not at the fact that my son was diagnosed with infantile spasms, but rather because my best had failed. For the last three weeks we were told repeatedly merely to continue watching our son closely, as his "involuntary motor responses" (which we now know were infantile spasms) could evolve into more dangerous seizures. As much as I advocated for our son, our own physicians hampered our ability to obtain an accurate diagnosis and promptly begin treatment. Enter Aidan, my positive counterpart. He reminded me how intentional God has been in paving our path. Had we not become frustrated with our neurology team we wouldn't be traveling the country for second opinions, acquiring high-quality imaging, making new diagnoses, and gaining valuable insight into our son's condition. Your help continues to eliminate all financial barriers to accessing the best of the best. Thank you...
We are convinced more than ever not to take advice from individuals who seem to lack a personal investment in our son's well-being.
When I first reached out to Boston Children's, my initial contact was Dr. Joseph Madsen, a neurosurgeon and the director of Boston's Epilepsy Program. He forwarded my message to his neurophysiology colleague, Dr. Phillip Pearl, who we grew more and more fond of as our e-mail exchanges evolved. He was innately kindhearted and generous. He was considerate and took time out of his week on multiple occasions to validate my concerns and answer my 'what ifs.' He's the reason we came to Boston.
Upon getting admitted and settled at BCH, we were told Dr. Pearl was in clinic all week and would not be on call; in other words we were not going to have the opportunity to see him. It was Zacchaio's second night in the hospital when we heard a light knock at the door at 10pm. Emerging slowly from the shadows was a face I recognized; I was immediately uplifted at the sight of the Dr. Pearl!
He had a much different approach than any other. He didn't say much, but said just enough. He was an incredible listener; his face softened and then dampened as we told him of Zacchaio's journey in his short 7 months of life. He understood why we were exploring the road less traveled by. He recognized that we were trying to save our son's life, not just his existence.
As our conversation with him neared its end, he asked, "When was he born?" "January 9th, 2014." "No. Really? He and my granddaughter share the same birthday..."
And it is perfect.
Caution: Surveying Crew at Work
Zacchaio's brain MRI was on Wednesday and his PET scan on Thursday. Our little guy had his EEG electrodes removed prior to his MRI (it's a giant magnet that is dangerously attracted to metal) then had to have them reapplied prior to his PET scan. We were unpleasantly surprised to see that some of the electrodes had caused ulcerations on his forehead from being cemented in the same place for an extended period of time. In spite of the sores, our Zacchaio has reacted with nothing but smiles and gratitude for their removal.
The results? Get ready for a whole lot of medical jargon without much context.
MRI: There appear to be characteristics of hemimegalencephaly (one side of the brain larger than the other), specifically in the right temporal lobe compared to the left. It is likely that there is dysmyelination (a defect in the myelin sheaths that coat our nerve cells which help to transmit impulses) in the right cerebral white matter. Due to Zacchaio being only 7-months old, he is in a natural intermediate state of myelination, which can obscure our view of a more subtle malformations. Therefore, it is recommended that a follow-up MRI occur after further myelination takes place to see if more specific focal malformations can be identified. Lastly, the right temporal lobe cortex is thicker than the left which is consistent with polymicrogyria (a malformation consisting of too many small gyri, or infolds, on the surface of the brain).
PET: There is clear asymmetry in Zacchaio's two hemispheres with decreased metabolic activity in the right temporoparietal lobes relative to the left. These regions of abnormality are consistent with the findings on MRI.
None of this has a whole lot of context yet. Obviously, it is not good news. All we can do is continue to manage our expectations and pray that we haven't run out of options.
We were pleasantly surprised to hear that Zacchaio's case was scheduled to be examined at Boston's neurosurgical conference this week. Following today's conference we will have an opportunity to discuss the team's recommendations with Dr. Pearl. In the meantime, we eagerly await evaluations of the imaging and recommendations for treatment from UCLA and the Cleveland Clinic. Every set of eyes brings unique perspective and experience that allow for a more holistic approach to managing Zacchaio's seizures.
Following Zacchaio's initial diagnosis of seizures, we stopped feeding him orally due to his increased risk of aspiration. After six weeks of strict NG feedings, we attempted an oral feed five days ago. My assumption that he would pick up right where he left off was grossly miscalculated, as all he did was bite and lick the bottle's nipple.
I am thrilled to report that after a few additional attempts, Zacchaio regained his sucking abilities yesterday! Though he may be off to a very slow start, I believe he'll gain momentum quickly after the enthusiastic acknowledgement he received from us following his first bottle. Way to go, baby!!!
|Learning to eat all over again at 7-months old (5-months adjusted)|
Zacchaio was discharged from Boston Children's on Friday evening; however, we were advised to remain local until surgical conference today. Not knowing what news we would receive following today's conference, we decided to fly Alkaio into Boston from California.
|Sleeping with my boys with hopes of a life full of the same opportunities.|
I was flooded with joy when I felt Alkaio's arms wrap around me for the first time in a week. All I wanted in that moment was for him never to let go, but his unadulterated excitement for his Boston trip (and the hotel pool) made it clear that he didn't understand the fragility of our family's circumstances. We have come to realize that each new experience we have as a family may be our last. I suppose this is one of the many blessings God has given us in this new profoundly difficult reality.
While taking walks through Little Italy and downtown Boston, we stop every 2 hours to set up Zacchaio's feeding pump. People walked quickly through picturesque plazas to reach final destinations while doing double-takes at the silver IV pole starkly juxtaposed with the beautiful brick backdrop.
There is nothing easy about our new life. It's not convenient, it's not predictable, and it's not balanced.
But you know what our new life is? It's a constant reminder to live and to love. Because of Zacchaio, we stopped and enjoyed our sandwiches on a plaza bench, instead of on the run. He helped us savor sweets from a historic chocolate shop in the shadow of Paul Revere. And then there is all the excitement! We found ourselves power-walking through crowds when Zacchaio was on the verge of vomiting in the carpeted Old North Church. And just last night during a feed in the park, a thundershower sent us scurrying for a patio umbrella to keep Zacchaio and his pump dry. It would be easier to stay home. It would be easier to go with the most convenient option. It would be easier to stick to what we already know. But that's not what we want for Zacchaio, that's not what we want for Alkaio, and that would not be us.
|Not your average hotel kitchenette counter.|