So ... Paige put this together this evening. I will give her full credit as she is the resident medical expert. It is a bit lengthy but we decided to err on the side of full disclosure to satisfy the more inquisitive minds in the crowd.
Feel free to skim if you don't fit in that box.
---
Dear friends, colleagues, and family,
We pray and hope that
as of Wednesday the source of Hollis's ongoing health issues has been
identified and the proper solution has been found (and applied). For most of
you, a quick background is likely to be needed in order for the solution to
make sense. So here goes ...
When Hollis was three
he was diagnosed with a Chiari I malformation of the brain. The entire brain is
supposed to be positioned so that it sits neatly in the skull. People with
Chiari I malformations have the bottom portion of the back of the brain (i.e., the cerebellar
tonsils) sagging down into their necks where it causes crowding. Because the
back of the head is big and the neck is small, having that extra tissue
herniated into the neck area can cause a lot of problems (e.g., blocking the
flow of spinal fluid and putting pressure on the surrounding structures and
tissues). A herniation of 5 or
more millimeters is a Chiari I malformation. When he was first diagnosed back
in 2007, Hollis had a 23 millimeter herniation and almost complete blockage of his
spinal fluid. Because Hollis had symptoms
showing that damage was being done by the tonsil position, he had surgery in
2007 to take out a window in the back of the skull, put a synthetic pouch in that area, and file down vertebrae C1 and C2 of the spine to clear some space (like a leaf in a table) so
that those tonsils can move up and back to be closer to their proper position. Letting
the tonsils have more room allows for better spinal fluid flow and eliminates
the bottleneck situation and its resulting symptoms. The surgery was deemed successful and follow-up MRIs
continued to confirm the success as recently as 2009. Even though the tonsils only moved up to the 21 millimeter
position, spinal fluid flow appeared to be adequate.
During the first week
of September 2011, Hollis had really bad headaches that we now know were due to extremely high spinal fluid pressure in the brain. By late
November there had been repeated pain bouts as well as evidence of bleeding
(with no identifiable source) into the spinal fluid. As most of you know, while there were many tests and additional
hospitalizations, the source of the bleeding remained a mystery. The neurologists and neurosurgeons that
we worked with in Houston generally thought that there was bleeding, that the bleeding
was clogging up the spinal fluid flow in the ventricles of his brain, and that the
clogging and increased pressure were causing extreme pain. For each incident, Hollis
had a lumbar puncture to remove spinal fluid and relieve pressure. He would then return to normal, return home, be fine for a few weeks, and
then the cycle would repeat.
This past week, a neurosurgeon
(and her partner in neurosurgery) here in San Antonio went through all of the
information, scans, etc., from all of Hollis’s previous Houston tests. Because
he was in a bit of a crisis situation (as he always is when he has these
episodes), they decided to put an external drain in his brain that let
them monitor his spinal fluid pressure for several days. They determined that
what appears to have happened is kind of backward from the scenario described above
of bleed, clog, pressure, pain. Rather, they think that something had been
causing pressure surges (Chiari patients often have pressure issues) that swept
the tonsils (that are already greatly herniated even after surgery) even
further into the neck. Over the
years, the area in the neck where the 2007 surgery had been performed had
developed scar tissue so that the existing space in Hollis’s neck is even smaller
than it was before his surgery. They believe that the cerebellar tonsils pulled
down and caused more blockage, and the resulting increased pressure on those
tonsils caused a capillary or capillaries to break. The break in the
capillaries leaked blood into the spinal fluid which then blocked the
ventricles, making the pressure even higher. So ... pressure surges, tonsils get pulled, more pressure
builds, capillaries break and bleed, pressure builds, and on and on. The lumbar punctures were letting off a
lot of fluid and allowing relief, but at some point the whole cycle apparently
repeated.
With the external
drain in place Hollis was keeping his pressure controlled while lying
still. After a few days, a new MRI
revealed that his brain tonsils were only 13 mm herniated (his previous low
after pressure-reducing lumbar puncture was 17). The neurosurgeons reasoned
that if no event ever caused another surge in pressure for which his brain
could not compensate, he would be good to go because the tonsils were higher and
in a relatively good position (away from the scar tissue). The problem, of
course, is that absent intervention the probability of those tonsils staying “high
enough” would be close to zero. After all, he’s had half a dozen episodes since
August alone. So ... the lead neurosurgeon placed a permanent shunt in his brain
that drains excess spinal fluid (once the pressure reaches a certain level)
from the ventricles of the brain into his abdominal cavity. As long as the
doctors are correct about the source of the bleeding and as long as there are
no problems with the shunt, Hollis should be totally normal. Other than the fact that about 1/3 of
his head has been shaved, he looks and acts totally normal.
I apologize for the
long-winded note. The situation is/was very complicated, and it took some
excellent minds in both Houston and San Antonio, a lot of time, many episodes of suffering and fear, and a truly miraculous series of events to put the numerous pieces of information together (over 18
MRI, CT, and LP items on the radiology records up to the most recent
hospitalization) to come to a solution for Hollis. The last piece of the puzzle was our neurosurgeon in Houston being out of town during Hollis's most recent episode and recommending a friend of his in San Antonio. This friend (the woman who performed Hollis's surgery) had a patient many years ago in Hollis's same situation (Chiari malformation, bleed, high pressure, no detectable source). She put a shunt in that patient, and the patient has not had any further problems.
We thank God for the extremely non-coincidental series of events that has brought us to this point, and we thank all of you for your prayers, positive thoughts, love, and/or support. We appreciate your continued support as we keep our eyes on Hollis’s situation moving forward.
We thank God for the extremely non-coincidental series of events that has brought us to this point, and we thank all of you for your prayers, positive thoughts, love, and/or support. We appreciate your continued support as we keep our eyes on Hollis’s situation moving forward.
Sincerely,
The Wilkins Family
