As promised, a medical run down of our week in
Minneapolis. You’ll know why I separated these posts about half way
through it.
Wes (yes, we are on a first name basis with our Dr.s) thought
he looked great! His chemistry was all in “normal” range. It will be a few
weeks yet before we get his engraftment and enzyme levels which I am always
anxious to receive. Last month we saw our local Phoenix geneticist and
they took GAG & Antibody levels. These 4 tests are the key indicators
of how Coleson is doing from a “Hurler”/BMT view.
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| Wes Miller - much better photo than 2 years ago. |
Enzyme levels – the amount of enzyme he is producing (0% =
Hurler), and he was within normal range last year at 49%. The higher the
better for this normal to eat away at some of the GAG (build up in his body
that occurred pre-transplant that wreaks havocs on joints, bones and muscles).
GAG – As mentioned above the amount of “Stored” build-up in
his body. He was at 85 last year and post-transplant has held steady at 5
for the past 2 years.
Antibody Level – Is he building antibodies against the
enzyme, rendering the transplant not effective? This came back negative
(which is good).
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| Paul Orchard, BMT, Paul and Coleson with his Recycle Truck |
We had X-Rays, EKG and Echo next. I say “we” as I
often have to hold Coleson during everyone one of these procedures to keep him
calm. He really is a trooper no doubt but can you imagine, we are already
at our 5th “procedure” on our first day and it’s not even noon!
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| We will miss Teresa! Good Luck at NP school. |
In the afternoon we had neurology and cardiology consults.
Neurology was painless but the wait was not! We were in the room
for 45 minutes before thankfully a music therapist showed up and Coleson got to
take out some much needed energy on the drums! He loves the drums.
Another 15 minutes later, the neurologist arrived and gave him a gold
start. He doesn’t show any signs of nervous system or brain impact.
He actually is quite smart!
The cardiologist consult was the last of the day and overall
his heart condition was great! He has minor thickening of the mitrial
valve but it is not impacting his heart function…but
Tuesday was an earlier morning and I pulled Coleson right
out of bed and into the car where he promptly said, “No hospital, No hospital!”
Broke my heart and we were only starting day 2! I promised him
no “owies” though I am not sure how honest I was being considering we started
the day with a 4 hr. neuropsych evaluation. It is one of the most dreaded
appts. for me, nothing like over analyzing everything your kid does/doesn’t
do. However, for him, hopefully it feels more like playing.
The Good
However, I guess the 4 hr. duration was worth it as he made progress in every developmental area, even his receptive/expressive communication which is the one area he had been a little lagging in. They stressed the continued need for speech therapy for his enunciation and OT for his fine motor skill development. He scored average in both areas while his cognitive scores were still above average (he can count to 100 already!) I often wonder what he would have been like had he not had Hurler but these are fleeting thoughts as there is no purpose on dwelling on the what ifs. We quickly squeezed in a pulmonary consult which happens to be on the same floor where which he sailed through with flying colors, despite the respiratory scare we had the week before. Then we were off to St. Paul to Gillet’s Children’s hospital for a hand ultrasound (to check for carpel tunnel) and a consult with the hand Ortho.
Customer Service (the good)
However, the receptionist provided great customer service which always makes these things a little easier - right HW colleagues? J and got us right into the Ortho for our consult and based on her range of motion exercises with Coleson determined that his wrists, elbows and shoulders had actually improved and we could skip the ultrasound! I guess it was worth the wait to skip another “picture taking” session!
Are you exhausted yet? We are only on Tuesday people!
The Ugly
Wednesday started early, with a 5:00am wake-up call and a 6:00am check-in time. After the usual 1:30mins of pre-op, meeting with the Dr.s, nurses and anesthesiologists (who I have the utmost respect for!), he was taken back to the OR. It never gets easier no matter how many times we have been through this routine. It is comforting to see familiar faces, although I would be happy if I never saw any of the surgical team ever again! J Its funny how even the smell of the surgical ward is familiar... and we have our typical routine. I struck a conversation with another mother whose son was getting his adenoids out, a first timer. I feel like a pro at the waiting room protocol and let her discuss all of her anxieties, nervousness, etc… Only then did I tell her that this was at least number 10 for Coleson (I have to finish documenting all of his surgeries to be sure and how do I count multiple procedures at one time?)
What day are we on again?!
The Bad
Ah yes, another pull him out of bed right into the car for the audiology testing. I was hoping for good results since the ABR he had in December showed normal hearing activity and his vocabulary and communication skills have improved greatly. However, the pressure in both ears was not equalizing and he does have fluid in his left ear (his eustachian tube is not as vertical as it should be). So they only got one recordable event which happened to be normal range but recommended he get tubes in his ears again (3rd time is a charm)!
Next stop endocrinology with a new Dr. Dr. Polgreen
who had seen the Hurler kids for years left to go to California to do research
and clinical trials for Hurler. I was skeptical to see Dr. Miller (Brad–
not Wes) but was pleasantly surprised and really like him. He was so good
with Coleson and explained everything very thoroughly, though it did confirm
that the thyroid medicine that Coleson has been on for about 3 months is
keeping his TSH levels in normal range (which is a good thing) but just confirms
that he has hypothyroidism. I was hoping his TSH would be too low and
would indicate Thyroid medication is not needed. It’s not the end the
world but it is a daily medication… for which I have found a new transport
vehicle… Reese’s Peanut Butter Cups! The crushed pill sticks to the PB
and he is none the wiser!
Other than the above news, he is growing according to the
chart and actually gained a %. He is now on the 2% growth curve! We
talked in great detail about Growth Hormone, a controversial topic and one that
we do not need to get to deep into yet. We traveled to the Spine Center
to see Dr. Schwender, neck and spine ortho. This was probably one of the
most positive consults. After more “pictures”, Coleson’s kyphosis had
improved from 42% arc to 15% arc. That’s from all that swimming and
climbing! I tell you, he tries to do everything his sister does and she’s
a daredevil herself! Not sure if that is a good thing or not! His
spine and neck looks great and foresees no spine surgery in his future much to
my relief… however, he also was formerly the hip and knew ortho and he prepared
us for what Dr. Walker, our next appt. would be telling us, and what I knew but
was hoping for a miracle would not be required…
Dr. Walker (appropriately named considering he is a hip and
knee specialist) was the appointment I was dreading the most. Yes, even
more so than surgery and neuropsych eval. After another round of
“pictures” (we’re getting good at bribing) we had a “extended consult” with the
Dr. It’s never good when your appointment is labeled, “Extended”.
We spent nearly 2 hrs. Discussing the surgery Coleson will need next summer,
Pelvic Osteotomy and Proximal Femoral Osteotomy. In layman’s terms, Hip
Reconstruction, on both hips. Basically, they will cut his hips and
create a wedge and cut off another piece of his hip to insert into these wedges
so that it completes the hip sockets. At the same time, they cut his
femurs and reposition the thighbone into the hip socket. They screw a
metal plate into his femurs (which requires another surgery to remove).
Sounds fun, huh? Oh, then he will need to wear a cast from his thighs to
below his knees for at least 4 weeks. Ideally they will do both sides and
both procedures at the same time. These surgeries are fraught with
risk. They are cutting the very place where bone marrow is made with high
risk of blood loss and transfusions required, potential of the bone dying (and
therefore not ever growing), spinal injuries (from being sedated &
intubated for so long), infections, etc. They have 2 surgeons working on
him at the same time to reduce the surgical time and hook him up to electrodes
to monitor his central nervous system. Did I leave anything out?
Oh, yeah, that outside of the BMT, this is probably going to be the most
stressful, worrisome procedure he will need to have. And we will do this
in Minnesota so we will be living away from home for the summer again.
Though I love the RMH, I do not want to live there again necessarily.
It has its pros and cons and it was there when we needed it most, but I think
for this procedure, it is better for Coleson and our whole family to be
somewhere I can protect him better.
Anyone have a rental house in the Minneapolis area? J
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