Neurology
The neurologist we saw last month was out of Children’s
Oakland and we weren’t very impressed with the quality of the appointment. What’s
new, right? We have high expectations! With our vow to never
settle, we sought out a new neurologist out of UCSF and ultimately connected
with Dr. Litwin. She went above and beyond and showed me EVERY image from the
MRI of his brain (there were hundreds!). Fortunately, she has had quite a bit
of experience with children diagnosed with neurocutaneous (brain-skin)
disorders and was feeling rather optimistic about his brain and current neurological
development. His neurological exam was mostly normal, but she did notice a
moderate head lag when trying to pull Zacchaio up to a seated position from a
lying positions by his arms. She didn’t seem very concerned, as not only was
Zacchaio preemie, but he had a very rough start to life that put him a bit
behind in his development. With that, she urged physical therapy and looked
forward to a 3-month follow-up to monitor his progress.
BVAC! (Birthmark and Vascular Anomaly Clinic)
We had the opportunity to attend UCSF’s invitation-only BVAC
a couple of weeks ago. An interesting experience indeed! The 7 invitees were
placed in exam rooms and physicians rotated into each room (stations,
everybody!) to see, diagnose, and recommend treatments for each patient. The
first few rounds were clusters of med school students who were in LOVE with
Zacchaio but maybe a bit more in LOVE with his nevi. It was hilarious to watch…
They would run their fingers over his skin lesions while a breathy, “woah,” “oh
my,” and my favorite, “so beautiful” were uttered. Now don’t get me wrong, I
love my son and think he is beautiful WITH his nevi, but do I think the nevi
themselves are beautiful? No! In the students’ defense, they were getting to
put eyes on something that dermatologists rarely witness in their
lifetimes. I digress…
The largest and most notable group finally came through and
it was at that point that we had short and long-term plans to manage his skin.
The world-renowned dermatologist, Dr. Frieden, told us it was only in the last
few years that the genetic mutation that causes Zacchaio’s nevi was identified.
Having this information makes her hopeful they will come up with treatments for
his body-wide nevi sometime during his early years, so she discouraged us from
intervening anytime in the near future. The laser surgeon said he could work on Zacchaio's right cheek if he would like in the future, but not until he is at least 5 years old. Dr. Frieden also highly recommended removing
the nevus skin on Zacchaio’s scalp. The nevus skin on his scalp is already at a
higher risk for cancer formation due to its nature of over-producing cells, and
being that this skin is on the top of his head and so frequently exposed to the
sun, it’s even higher risk. It made sense.
At this point the craniofacial surgeon, Dr. Hoffman, chimed
in and stated that were we to do surgery in the next few months, it would be a
1-day procedure and he could get MOST of the nevus removed. It turns out that
the scalp of a baby is extremely mobile and pliable which makes surgery easy if
done early enough. If we wait any later than age 1, the scalp is mostly fixed
and doing the same surgery would mean inserting saline bags surgically under
his scalp, filling them weekly with injections, and continuing this process for
4-5 months until the skin has stretched/expanded enough to make of for the skin
that would be excised. Not only that,
you have to be very aware of your child’s activity to avoid potential popping
of the balloons and be vigilant about preventing infection. The choice seems simple enough… Do the early
1-day procedure, duh! If only there weren’t confounding factors.
I put on my nursing hat (metaphorically, because I would
never allow that little white bonnet to sit atop my head) and began researching
anesthesia risks in infants. A number of retrospective studies have been done
that show a mighty correlation between 2 or more exposures to anesthesia (under
age 4) and learning and memory deficits/disabilities. Remember that Zacchaio is
already high risk for intellectual disability due to his syndrome. On one hand,
we would like to preserve as much of his brain function as possible, and doing
so would mean to hold off on the traumatic 5-month long surgical journey until
he is over 4 years old. On the other hand, we can avoid the torturous weekly
saline injection visits, multiple opportunities for infection, and being
home-bound for 5 months by doing surgery early.
Surgery is scheduled for July 11th. He will be a whopping 6 months old (or 4
months adjusted).
It took us a long while to come to our conclusion, but Aidan
and I agreed that 1) The retrospective studies are not CONCLUSIVE and are
unable to take into account many other factors that play a role in the
development of learning disabilities, and 2) We’re making an instinctive
decision, and truth be told, so far our instinct has proven to be the gold
standard in guiding Zacchaio’s care and getting him exactly what he needs.
We have to trust that if we’re making the wrong choice, God
will make us aware of it and help us change it accordingly.
Pulmonary
In trying to minimize his exposures to anesthesia, Zacchaio
will also have a bronchoscopy (a scope will be put down his mouth to visualize
his airway) piggybacked on his surgery. I
mentioned before that Zacchaio had a long bout of croup and viral illness, but
the croup seems a bit chronic now and he has a signature bark anytime he coughs
or sneezes. He has also been having some difficulty swallowing food. Just as he
gains momentum sucking and swallowing during his feed, his nose will all of a
sudden seem stuffy, he can’t take a breath in and he unlatches to avoid
choking. The pulmonologist isn’t sure what’s causing the problem (he thinks
maybe floppy cartilage of his trachea?) but the bronchoscopy should shed more
light on the problem.
Physical Therapy
We had our first physical therapy appointment yesterday and
are seeing Graciella out of Baby Builders in Oakland. We tried using our insurance through Alta
Bates and Children’s Oakland but the first appointments they had weren’t until
the end of June. I was frustrated to
have to go out of network, but Zacchaio is in such a critical developmental
period that every day we don’t get therapy is that much further behind he may
become.
After having our initial evaluation, our concern that
Zacchaio had right-sided torticollis (asymmetric head/neck position) was
validated by the therapist. We noticed in the last couple of weeks that his
head has been progressively tilting more to the right and that his head would
fall or flop when in a position requiring his left-sided neck muscles. We were
given a packet of exercises to help rectify the weakness and we definitely have
our work cut out for us! We’ll continue physical therapy once a week for now
and hope that in the next few months the torticollis will resolve.
So much happened in only 2 weeks! I’m copying and pasting my
facebook post from this last week during our Disneyland vacation to end on a
positive note. I’m so thankful our entire family was healthy enough for the
trip!
“After being hospital and home bound for over 4 months, we
feel so fortunate to be outside and enjoying what life, and Disney, have to
offer! Having an opportunity to feel "normal" (with baby care center
members knowing us by first name due to frequent visits) has been a wonderful
way to celebrate our completed family of four! Eternal thank yous to Aidan's
co-workers at CVUSD for their priceless gift of time through sick day
donations. The outpouring of love and support from everyone has been
overwhelming and has helped us navigate the unthinkable. This trip has served
as a true respite from the constant focus on Zacchaio's ill health (though
we're unfortunately still not getting any sleep with tube feeds). We will
adjust yet again as Aidan and I head back to work in the next couple of weeks;
until then much love from the Happiest Place on Earth, because who knows what
tomorrow brings...
And so we move forward, ‘to infinity, and beyond!’"