Special Needs Spotlight: Emma-Kate

Miggy:  Hi Sara!  So I loved what you said about your daughter in your
email to me; “She just turned two and has been
through the medical wringer, having just added her 9th surgery to her rap
sheet.  On paper, she’s a nightmare.  In person, she’s the smiliest, sassiest little peanut of a
firecracker ever.”  I think this “on paper/but in real
life” sorta summarizes up a lot of our experiences as special needs
parents.  While life with our kiddos can sound horrible from the outside,
the reality is often the complete opposite.  So please, tell us about your
little Emma-Kate, when did you first find out about her special needs and what
was reaction?  Have those feelings changed in the last 2 years?    

Sara:  Our sonographer saw something funky with our baby girl’s
heart at the routine 20 week ultrasound. 
Long before then, I knew something was not right.  I could not, for the life of me,
connect with this child growing in my womb.   Through the subsequent months of peri-natal
assessments and fetal echocardiograms, we saw and found out more than was
possible for us to process. 
Somewhere along the line, amused that I had not miscarried with evidence
of so many anomalies, I distinctly remember a very defining conversation my
husband.  We decided we felt like
we were on a foreign shore, isolated and holding hands, waiting for this
tsunami to consume us. 

But somehow, amidst our devastation, we decided that the
most important thing was to give this baby girl a chance of birth and
life.  And that we would just love
her, no matter what the outcome.  That
last part has never changed.  

She was born with “multiple congenital anomalies” which
includes a few things like: atrial/ventricular septal defects (holes in the
heart), coarctation of the aorta (narrow aorta), hypothalamic hamartoma (a
benign mass in her brain), severe tracheomalasia (narrow airway), micrognathia
(short chin which adds to airway complications), and polysyndactyly (extra and
fused toes).  These are just a few
of her more severe anomalies which were tagged under the oral/facial/digital
branch of syndromes, more specifically Pallister-Hall Syndrome.  Despite all of these oddities, she
shows no signs of any genetic modifications.  This, of course, caught the attention of the folks at the
NIH in D.C.  They enrolled all of
us in the Human Genome Research Project two years ago and have still found no
significant genetic markers to identify what happened to cause all of this for
her.  She is only the 21st female
to ever have her genome sequenced. 
They pronounced to us last May when we drove out for a visit that she is
a true miracle, a true mystery, a true gift to science and human understanding.  She is one in six billion. 

Take that, severe congenital anomalies!

Plus, despite all the crazy medical stuff, she’s the
happiest kid on the planet.  I
hyped myself in to thinking that she was experiencing gelastic (laughing)
seizures because she was so stinking happy all the time.  I mean, it just didn’t make sense.  SO, I made them do a repeat MRI.  She was just fine.

Miggy:  Explain how Emma-Kate’s needs affect your day-to-day life?  

Sara:  Our day is often composed of the happenings between feedings (every four
hours), meds and various appointments. 
Now that Emma-Kate is older, she has this adorable little backpack that
houses her feeding pump and supplies, so she can roam about rather than be
tethered to a pole for the duration of her feed (which is now about an hour,
she used to be fed continuously). 
As far as meds go, she used to be much more dependent on the timeliness
of her diuretics.  She came home on
a total of seventeen medications, five of which were related to cardiac
function.  We just recently
celebrated her complete diuretic/electrolyte wean and are now down to just
three (fairly benign) maintenance meds (plus inhalers). 

Now, because I have seen how the fragility of life can lead to long
bouts of bed riddance,  the
feeds/meds/breathing treatments/supplies simply come with us.  And we are on the GO!  We mainly go to various therapies, but
I am determined to get this kid out there to do all the little kid things I did
with my oldest…so we schlep our baggage merrily along.  And hilariously enough, I have
connected with so many strangers over odd needs for sterile gloves, saline,
bacitracin and alcohol swabs. 
These are some of the (un)common medical supplies that we keep with us
on a daily basis. 

I have also begun to notice funny things about people- scars, small
chins, signs of various conditions- that I would have never otherwise paid
attention to.  These complicated
kids teach you so much about the body and that “everyone has their disease.”

There are perks to all this trach business, you know, in case this all
sounds a little deranged.  While it
took us some time to get used to, we have nurses that care for Emma-Kate at
night.  They take care of all her
feedings through the night and clean/restock her supplies while we get a
glorious, worry-free sleep.  And
she plugs in a movie for our six year old when he wakes up at five a.m.!!

However, there is one particular way that Emma-Kate affects our daily
life.  And that is that her scars
constantly emphasize the beauty of the moment at hand, of the distance we’ve
all come together, of life.

Miggy:  What are the biggest worries you face for her?

Sara:  Among the millions of worries for this child and her infinite trips to
the OR bring us, one of my biggest worries for her was (this sounds vain, I
know, but seriously…) that she would be smelly.  When Aiden (her big brother by three years and 50 weeks) was
barely four, he always commented that she smelled icky.  She used to be a mega puker.  These cardiac babies just are.  In our experience, cardiac surgeons
like their little babies chunky so that if/when they need to go back in for
additional repairs, the babies go into the OR nutritionally strong and have
some weight to shed in recovery. 
Otherwise, they tend to be super fragile.  This isn’t always the case, but it was for Emma-Kate.  It took forever for us to realize that
cardiac nutrition was calculating her max caloric intake for us to give
her in a day, and she truly was like a super chunk for a while.  She had these round bulbous cheeks that
tube fed babies can get.  And
despite my obsessive smell-masking efforts, she still often had this nasty sour
stink cloud following her around. 
She already had all these medical accessories that people were likely to
notice and gawk at first, but I always had this irrational fear that people
would be even more disenchanted by her awful stench.  I recognized that it would be difficult to romanticize a
tracheostomy or feeding tube, or to hide them, but I really felt like it should
be easy enough to normalize her a little with that amazing baby scent that
everyone loves. 

It was harder than I thought.

In the last six months or so, the puke fest has more or less
subsided.  We learned after talking
with docs and other parents that we could monitor her weight gain on our own so
as not to over-feed her and perpetuate the puking.  We were in crisis mode for so long that it took awhile to
concern ourselves with the more mundane things of being home, like regular weight
gain.

Anyways, this desire for me to normalize her and her medical accessories
(like bedazzling her feeding backpack 
or constantly pestering the DME companies to make cute trach ties…) is
constant.  Somewhere along the line
I learned that these trite things can be conversation starters with onlookers
who aren’t sure how to converse about or cope publicly with special needs.  For me, there is aways this worry that
I am not giving her the proper tools to cope with her accessories and deal
those who gape. 

Miggy:  Now for a lighter question, have you ever had any funny
conversations/moments you never imagined due to your special needs situations? 

Sara:  Funny you should ask. 
Emma-Kate is a little confused about her anatomy because she has two “buttons”
(her belly button and her Mic-Key feeding tube Button), two noses (one of which
is a type of filter that goes at the end of her trach.  It’s commonly known as the “artificial
nose” or just plain “nose” as we refer to it), and a “zipper” (the giant
franken-stitch of a scar from cardiac surgery).  We also like to refer to her Mic-Key Button extension as her
“tail.”  We have gotten plenty of
strange looks when we ask Aiden to go get his sister a new nose or to tuck her
tail in.  We think it’s funnier not
to explain to people what we mean and watch their reactions…

We always talk about BMs too- color, consistency, frequency, you name
it.  Between the poop, the tail,
and the lung secretions, most of our conversations must seem less than savory!

Miggy:  How can people best approach or respond to your child? Is there
something you wish other people knew so as to avoid awkward or hurtful
situations?  

Sara:  I love when people approach us or ask questions about her.  I especially love when other little
kids point and stare and ask hilariously loud questions about “that thing
sticking out of her neck.” It’s kind of humorous to watch people fuss and
squirm until I break the ice.  I
try to always launch a little anatomy lesson by asking people what they think
it might be for.  It’s amazing to
hear what kids come up with!  In
the end, I would much rather people ask about it than not.  I don’t want people (including some
close family members…) to be scared of her.  In fact, her airway is way more stable than most
people’s. 

Miggy:  What is the biggest lesson you’ve learned since becoming Emma-Kate’s
mom?

Sara:  After having spent so much time in the hospital and observing how people
react to all the medical drama, I have learned that Emma-Kate and I were meant
for each other.  I love going to
the hospital and so does she.  In
fact, she’s the only kid I know that claps when she sees a person in a white
coat.  I love to learn about all of
her cool anomalies and the lingos of all the different specialties that follow
her (there are 11).  She has also
taught me that I thrive in chaos. 
I never ever ever thought that I would be as comfortable with the
medical setting as I am today. 
When I grow up, I want to become a survival flight nurse.

My husband and I have learned that we are team.  We are on the same side as each other.  We are on the same side as the medical
professionals.  We must all work
together, not against each other. 
There are days when I want to slap some people (or just go on a manic
slapping spree!) but then I step back, realize that something most likely needs
more time, more thought, or rephrasing and a better solution always comes to
mind.

Along those same lines, we have learned to be friends with the
trach.  We do not let it control
us.  As cliché as it is, Emma-Kate
has taught us to enjoy the little things and to be grateful for the little
things each day.  We still go on
dates, we still pack the van full of all of her mounds of crap and schlep it
three hours to see family, we still play in the snow and (very carefully) in
the water. 

Miggy:  If you could say something to the mom who just starting on this journey
of special needs–especially a journey that includes a lot of medical
issues–what would you say?   What would you say to yourself if you
could go back in time?  

Sara:  Life
with these kids is an adventure.  I
was talking to my mom recently and she told me very somberly that she prays
every night that we will have the strength to go on doing what we do for
Emma-Kate.  I laughed and told her
that I have never been happier in my life than since she came along.  She takes me to places I would never
venture to on my own.  She has
taught me more about life and love in her two years than I have learned
throughout the rest of my whole life. 
Now, when something unexpected comes up with Emma-Kate- like the cardiac
surgery she sprung on us last August (and in relation to the tsunami comment in
pregnancy…)- Brad and I just look at each other and say, “Welp, grab your
surfboard!  Here we go!”  We know, no matter what the outcome, we
will be delivered in the end.