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| The flowers in Providence are a gift all unto themselves. |
Posted with Naomi’s permission and her lovely blessings.
This is the third summer we have been to Providence, Rhode
Island. We haven’t come because Rhode Island is the Hope state, or because we
are drawn to a town named Providence, God’s protective care. (However neither of those facts are lost on
us, and we have found more hope here than we can measure.) Each time we have come it has been to work
with specialists who care for the various complications surrounding Ehlers
Danlos Syndrome. In early 2018 we
learned that Naomi has Hypermobile Type Ehlers Danlos. Simply put she has a bendy body. Or in a world of Subaru’s, she’s a
Jaguar. Both are cars. Both have pretty
awesome features. But while a Subaru
fares perfectly well on the old Montana wash board, pot-holed back roads, a
Jaguar wouldn’t do very well. Just about
any competent mechanic can fix up a Subaru, but it takes a specialty mechanic
to keep a Jaguar running well and if needed repaired. She was born with a variation in the stretch
of her body. Long slender arms and legs, slender hands, and skin that is as
soft as velvet, adorned with a beautiful long neck are all a part of her
beauty, and are a direct result of the way God made her with collagen that moves
more than most.
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| A favorite house on the corner of Angell and Hope Streets |
On May 31
st, 2018 she and I sat across from Dr.
Petra Klinge at Hasboro Children’s Hospital.
At the time she was here working on neck rehabilitation following our
wreck. The ligaments in her neck that already stretch too far at baseline, had
basically been stretched beyond even their limits that night.
And where my neck would have broken, hers
stretched and left her with extreme pain, headaches, numbness in her arms and
hands, and visual blurring among other symptoms.
She was working with Dr. Mike, our EDS
physical therapy guru, to strengthen her craniocervical (head to neck)
instability.
While were here we decided
to have the leading pediatric neurosurgeon in the country for EDS related
conditions see her and give her opinion on what would be best for Naomi.
Dr. Klinge definitely discouraged neck fusion
surgery for Naomi’s neck, preferring rigorous PT.
And then after listening to our story, and
examining her said, “I really wonder if she may have a tethered spinal cord?,”
which up until that time we had never heard
of.
Thankfully, Naomi hadn’t developed
leg pain or numbness at that point, and she didn’t have any urinary symptoms
then either.
Looking back now, and
knowing all we do, I suspect that many of her symptoms were directly related to
a spinal cord that was tightly tethered down, and stretched on that night in
2017 when we nearly died.
But at the
time we didn’t see a reason to investigate further.
Dr. Klinge graciously told us that if Naomi
ever did progress, we could certainly return.
And I have to admit that when we left I never fathomed that we would be
back.
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| This door has a story... |
In the last 2 years so much has happened. God has directed our steps so
beautifully. Naomi has gradually and
consistently improved. Every day has
consisted of a physical therapy regimen, and she just finished her first full year
of completely uninterrupted schooling. I
am going to take a mom moment and brag just a bit on her. She has blown this year out of the ball
park. She is not only caught up on
school, she will finish high school next spring just 6 credits short of an
associate’s degree. She was able to work
last summer, and since November she has exercised every day, walking at least
2.5 miles. She sees one of her favorite
doctors, Dr. Kris French, a neurologist, for injections into painful neck
muscles every 3 months. And after an
albeit shortened trip to Maryland in March, to work with Dr. Alissa Zingman and
the Prism Spine and Joint Clinic, Naomi can manage almost every ache or joint
issue she has on her own. She is
learning to handle her Jaguar body with the care it needs, and even though she
has truly rough days, she is different person than the girl who sat across from
Dr. Klinge on that late May day in 2018.
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| Old brick buildings are stunning |
In honesty, I am a different mom and doctor than I was on
that day as well.
The last 2 years I
have done what I do best; learning.
Learning absolutely everything I can about EDS.
My medical practice has changed because of
it.
In med school and residency I would
literally cringe when a patient had every symptom positive in the book.
Now my EDS patient intakes have a review of
systems that in years past would have quite literally caused me to run; but
knowing all the details helps me see them fully and understand the totality of
their collagen related troubles.
I love,
LOVE, my bendy patients.
And living day
to day with the complexity of a poorly understood and often brushed aside
medical condition in my child, I have become a better human…result?
I am a better doctor.
And even with those changes when Naomi began
to have some new neurologic symptoms in mid December I initially dismissed them
as just “odd EDS” stuff that would hopefully pass.
(Side note:
be a mom to your kids, not their doctor if at all possible.)
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| 5th Floor Hasboro Children's Hospital |
I remember that mid December walk with her on our road.
It was a sunny day, warm by Montana
standards, and I had nearly cried from the joy of walking with her as if the
last 3 years hadn’t really ever happened.
The walk had been lovely.
And
then about an hour after we returned she shared with me that she had
experienced some leaking urine while we walked, and hadn’t felt it happen; not
even noticing it until she returned home.
She had also begun to have the soles of her feet go numb while we were
walking.
My first reaction was something
like, “Uhhhh.
That’s awful.
I’m sorry.
Maybe you just didn’t realize that you needed to pee?
Tell me if it happens again.”
And in my heart I skipped forward, towards
optimism.
Surely nothing else was going
on, it couldn’t be.
She’d been doing so
well.
But a quiet voice, which has often
guided me in medicine, far in the back of my mind nudged me to request an MRI
of her low back.
You see, along with the
above mentioned issues she had also had burning pain into her bottom and legs,
heaviness and weakness after walking or standing for a while, base of her skull
headaches had increased, and a whole bunch of other issues that go along with
tethered cord syndrome if you so feel the desire to read about them.
She had also been telling me that she felt
like she was being shocked up and down her spine if she would curl up or bend
over to tie her shoes.
And even though
my mom heart wanted to move forward and dismiss it all, the doctor/student part
of me was cautioning me.
So we ordered
an MRI, and according to the radiologist it looked great.
And with that report I gladly moved on, and
quit asking about any other symptoms.
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| Even the sidewalks have a personality here |
Meanwhile, Naomi began to have progressively worsening
symptoms. That short lived numbness that
would start with walking began to last for over an hour after every walk. The urinary symptoms became daily and nightly
recurring issues. And being the warrior
that she is, rarely complaining about anything, she carried on. Continued to
exercise and actually had continued to do extremely well, all things being
considered. Then about 3 weeks before
she was to leave for Maryland, I asked how she had been doing. 2.5 months later. 2.5 months of my blissful optimism and her actual
reality, I finally thought to ask if she had continued to experience
incontinence or numbness. When she told
me the extent of the issues, and how they had progressed, I reached out to her
doctor shared about the urinary and nerve symptoms, which were now daily and
progressively worsening. Dr. Alissa responded
about a minute later with the name of a uro-neurologist to see in Baltimore,
and told me to get her in ASAP. (There are like 4-5 uro-neurology groups in the
US, and one of them just happened to be 20 miles down the road from the Prism
Clinic in Maryland.) She confirmed what
I feared, that Naomi could be having symptoms of tethered cord syndrome. So, I prayed (and cried just a bit), and
called Dr. Gordon’s office in Baltimore.
“Oh honey, this is great, we have an opening in 4 weeks, on March 17th! She’ll be here then right?,” said the sweet
nurse on the other end of the line. Within
15 minutes Naomi was scheduled for a highly specialized form of urodynamics
combined with nerve conduction followed by an office visit on March 17th,
right in the middle of her planned time in Maryland. Perfectly timed by a perfect Father who loves
her and goes before her always.
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| Opened gates of iron |
So in the middle of the early days of COVID, she underwent
urodynamics studies.
Folks if you have
had these done, you know. If you haven’t, then I pray you never have the
need.
And despite the invasive nature of
having her bladder and pelvic floor nerves investigated by a team of male
urologists, at 16 years old, with only her dad present, she felt relief that it
was done.
The next day she abruptly
returned home to Montana, just as COVID closed the country down.
Her results were conclusive.
Her bladder was not communicating with her
spinal cord well, a result of a tethered spinal cord and her symptoms were
unlikely to go away without surgery.
In
fact the doctor told me that they would become permanent and likely progressive
without surgery.
Dr. Gordon explained so
kindly what was causing her issues and told me that he would call Dr. Klinge
and discuss.
Apparently the few
specialists surrounding tethered cord have a weekly call in meeting and discuss
patients.
(In my mind it’s like a Zoom
meeting with super geeks, discussing super “interesting” cases.
And they are all heroes as far as I am
concerned.)
And God did what He does
best, He opened the doors for Naomi to “see” Dr. Klinge virtually in the middle
of the country being shut down due to COVID.
But before I go on, I must take this short break to explain tethered
cord, and be a bit of a science geek.
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| Baby tails. |
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| Naomi is now filum free. |
Did you know that at one point in our development we all had
a tail?
Well, we did.
As a human embryo develops there is tail,
which eventually shortens, and forms into the tissue which is known in fully
developed humans as the filum terminale.
(Oh my, I cannot tell you the joy it brings me to have searched for
images of these very things…yes.
I am
fully a nerd.)
Well the filum terminale
is a wisp of tissue that comes off the end of the spinal cord, and in healthy function
it serves as a bit of a shock absorber for the spinal cord.
The spinal cord is the long thick bundle of
neural tissue that transmits information from the brain to the body and vice
versa.
The filum doesn’t help with that
transfer of nerve information once we are fully developed.
And to be honest, I hadn’t really thought
much about it until the last 7 months.
In
some people tumors or spinal column defects can bind the filum up, and literally
pull the spinal cord down very early in life.
In EDS, for whatever reason, the filum can become thickened and very
taught as the child grows, or has trauma (both of which occurred in 2017 for
Naomi, 2 inches of growth followed after several thousand pounds of metal
collided with her in the middle), and rather than shock absorb, it become a
shock transmitter to the spinal cord.
This
shock transmission can then lead to problems with the autonomic nervous system.
Please allow one more digression…this one way, way back in
time.
Our nervous system is so amazing.
It has a parts that sense the world around
us, and those that control our muscles for movement, fine tuning components
that help us balance, and among many other complicated systems, there is the
autonomic nervous system.
The autonomic
nervous system is like the fine tuner of our bodies operations and functions
(think digestion, heart rate, blood pressure, etc).
So now imagine a cave man being chased by a
saber tooth lion.
When he sees the lion,
before he decides to run away his body needs to prepare for that to occur.
His heart rate will elevate, and his blood flow
will increase to the areas that are going to have need (like the muscles).
It’s the “fight or flight” response.
Well that response is driven and regulated by
the autonomic nervous system which has two parts:
sympathetic and parasympathetic.
The sympathetic part gets him ready to run, and
the parasympathetic part helps to calm him down.
So when he is running his awareness of
whether or not he needs to pee is turned down (of course it is…he cannot be
focusing on finding the most convenient boulder rest area as he is fleeing the razor
sharp teeth of his foe).
Also, the
muscles in his pelvis better tighten up (wouldn’t want the poor cave man to pee
down his leg when he is running).
The sympathetic system also diminishes the
awareness of hunger because he cannot be daydreaming about digging into a
mammoth steak while simultaneously trying to run his fastest.
Once he has survived his parasympathetic
system would have to kick in to calm him down.
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| Balance is key in life. Sympathetic and Parasympathetic need an equal voice. |
When the spinal cord is tethered down by a thickened filum
terminale, or a tumor, or whatever, the sympathetic nervous system continues to
fire off, because the spinal cord is under more tension that it should be. The pelvic floor muscles tighten up into
spasm, and have a very hard time relaxing.
The bladder begins to ignore that it is full, but it continues to fill
because the kidneys don’t quit working. Hence
the urinary symptoms Naomi has had; which
do not respond to medications, they respond to removal of the tension. But unlike the cave man who survived the
saber tooth attack, the parasympathetic nervous system doesn’t get to kick in
fully as the tension never really lets up fully. The pain down into the legs and numbness come
because the spinal cord is being pulled down on as well. And when Dr. Klinge reviewed Naomi’s MRI of
her low back, she felt that it wasn’t normal as originally read. She explained that the filum shouldn’t be
traceable. But Naomi’s was thickened to
about 10 times the thickness it should be, and rather than being whip-like and
slack it was more like a thickened anchor.
She assured us that Naomi had a very good prognosis and chance of
recovery if surgery was done. A surgery that entailed her spending just over 2 hours on her stomach, exposing her low back, needles up and down her legs to assure nerve health and safety, and cutting down to the natural space between 2 of her back bones right down into the sac around the spinal cord. Then just about an inch and half below her spinal cord, well below nerve tissue they clamped the filum, and cut it away from the spinal cord. That tissue was removed fully, and with it the remaining parts of Naomi's embryo tail were cut away. Good-bye anchor that has bound her. (The tissue they removed will be studied and Naomi is being followed in a research project to help better understand the details of both EDS as well as tethered cord.)
And here is just another fun fact as a bonus.
Dr. Klinge, the pediatric neurosurgeon who
specializes in EDS neurosurgical care, and just happened to be across the
street from the Ronald McDonald House where we have stayed for neck care while
in Rhode Island, happens to do the most tethered cord release surgeries in the
world.
How is that for PROVIDENCE?
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| Katy Perry, the Chaplain, not the singer |
So here we are, post operative day 5; in Providence, Rhode
Island.
And God has been faithful.
I won’t go into all the details, but Naomi
can feel her feet differently than she has been able to in over 8 months.
Her surgery was “completely straight forward
and smooth.
No bleeding.
No unexpected or excessive bone removal
needed.”
And on post op day one, after
24 hours of being completely flat all she needed to do was to stand up.
But she stood, went to the bathroom on her
own, and walked all over that pediatric ward.
She has carried on and moved forward, as she has always done with gentle
strength and quiet resolve.
Her urinary
symptoms have already started to lessen, and even medicated, her joy remains
infectious.
Her IV nurse called her “Petunia”
and every person on the ward enjoyed her presence.
She was given a gift with a nurse, 'Maverick', who
for 3 straight days made her laugh and smile, a lot.
Having him as a nurse, was like having our
sweet Carla Barker, in male form, as a constant cheer leader in the middle of
the fight.
I am convinced fully, that
God within Naomi, shines like the sun through her.
People are drawn to her and she literally
gives away joy wherever she goes.
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| She doesn't always smile.... |
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| But when she does the world is better. |
To be clear, her days since March 31, 2017 have been
anything but carefree and easy.
She has
suffered and she has grieved the loss of the life she thought she would have as
a teenager.
But she has also pressed
into God and received the fullness of His promise to bring her hope and a
future.
He has been with her, and He has
opened the paths for her healing while also growing and deepening her as a
human in the middle of suffering.
Her
future is bright, and she is learning even more how to care for the body she
has been given.
(Which, by the way, we
all need to do.)
She is not an EDS
patient.
She is child of God, who lives
and thrives
with EDS and some of the
problems that go along with it.
In closing this very long post, I will leave you with the
verses that we have been praying over her.
God hears our prayers, and He is FAITHFUL.
Hebrews 12:1-2 “Therefore, since we are surrounded by so
great a cloud of witnesses, let us also lay aside every weight (every tie that
binds), and let us run with endurance (perseverance) the race that is set
before us, looking to Jesus, the founder and perfecter of our faith.”
Isaiah 45:2-3, 7 “I will go before you and level the exalted
places (the mountains), I will break in pieces the doors of bronze and cut
through the bars of iron, I will give you the treasures of the darkness and the
hoards in the secret places, so that
you may know that it is I, the LORD, the God of Israel, who calls you by your
name. I form light and create darkness;
I make well-being and create calamity; I am the LORD, who does all these
things.”
Isaiah 42:16 “I will lead the blind by ways they have not
known, along unfamiliar paths I will guide them; I will turn the darkness into
light before them and make the rough places smooth. I will
not forsake them.”
Isaiah 40: 29-31 “He gives power to the faint, and to him
who has no might He increases strength.
Even youths shall faint and be weary, and young woman shall fall
exhausted;
but they who wait for the
LORD shall renew their strength; they shall mount up with
with wings like eagles; they shall run and not be weary, they shall
walk and not faint” (and this is totally my addition), "they shall walk and not
pee their pants."
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