In 2006 I lobbied a neurosurgeon
to implant a vagal nerve stimulator in my son in order to attempt to better
control his myriad seizures. The ketogenic diet then was in its infancy here in
Israel. As a matter of fact when I had approached a pediatric neurologist on
the matter his answer had been, “well if you want to occupy yourself with
voodoo, you are on your own.”
It took me six months to convince
the surgeon to go ahead with the VNS device. Coming to Segev in the recovery
room I saw him on the table arching his back, screaming without a voice,
convulsing to get air into his lungs. The anesthesiologist stood next to the
bed, looked at me endearingly and asked me, “is he always like this?”
Within a week he developed an
enormous hematoma at the site of the surgical intervention. After 12 hours in
emergency of the same hospital, and a consultation by phone with the chief ENT
surgeon who had done most of the actual work (but who never bothered to show
despite being present in hospital at the time), we were admitted to a ward and
a conservative approach was adopted. This is another way of saying, nothing was
done. And the hematoma did subside spontaneously. Immediately after the surgery
I had asked the chief of pediatric ICU about the loss of his voice and its
possible origins, “coincidence” was his reply.
Later the chief pediatric ENT
surgeon was consulted and with the help of fibre optic investigation it was
determined that, yes he had bilateral paralysis of his vocal chords. Prognosis?
“Most likely permanent”, was his response.
After some four months Segev slowly regained his voice.
After one year, in consultation
with the pediatric neurologist specializing (and overseeing Segev’s treatment
with the VNS) concurred that it had proven ineffective and agreed to turn it
off. In his hands was all the relevant information concerning the chronology of
events since the surgery, of that I made quite certain. But he mentioned nothing.
J Neurosurg. 2010
Apr;112(4):829-31. doi: 10.3171/2009.6.JNS09422.
Insulation discontinuity in a
vagus nerve stimulator lead: a treatable cause of intolerable
stimulation-related symptoms. Spitz
MC, Winston KR, Maa EH, Ojemann SG.
Source
Department of Neurology, The
University of Colorado at Denver Health Science Center, Denver, Colorado, USA.
Abstract
Discontinuity in the silicone
insulation over an electrode of a left vagus nerve stimulator (VNS) allowed the
aberrant leak of current to the phrenic nerve and other structures. This
resulted in ipsilateral diaphragmatic dysfunction, inability to vocalize, and
severe radiating pain into the jaw and upper incisor for the duration of each
stimulation. The device was explanted and a new device was implanted. All
stimulation-related symptoms ceased immediately. A similar discontinuity in the
silicone insulation is the likely explanation for several prior reports of
poorly understood pains and phrenic nerve stimulation in patients with VNSs.
The findings and analysis of this case establish a rationale for consideration
of replacement of the VNS lead in all similarly symptomatic patients.
PMID: 19558306 [PubMed - indexed for MEDLINE]
The above scenario should
somewhere, somehow have triggered a red flag with anyone of these noted and senior
physicians. But apparently the responsibility all lay with me.*sad face*
One year ago I took Segev to
RAmbam hospital, here in the north, for him to finally undergo the surgery that
so many physicians had insisted was critical to his survival, a tracheostomy. For
years I postponed this by strategically placing cervical collars to support his
regressed jaw, thus maintaining a proper airway.
I went to a very senior pediatric
ENT surgeon who also specialized in reconstructive surgery to ask him what
could be done. Surgery? Bipap? No, he quipped, both would be not only useless
but actually dangerous for Segev. Tracheostomy was the only hope.
When time for the surgery finally
arrived due to Segev’s worsening breathing, requiring constant oxygen for the
first time, not only to support him during his many exacerbations of
bronchiectasis and multiple pneumonias or to allow more swift resolution of a
particularly large seizure, a chest xray was done prior.
The radiologist looked at the
film and pronounced every thing to be fine. At the first instance to view the
film myself, at home, I immediately saw the diaphragmatic paralysis and
together with Segev at the admission protocol for the surgery, drew everyone’s
attention to the paralysis. While interesting no one offered any thoughts on
the matter and the surgery was scheduled to go ahead. Because his intestines
were protruding upwards (as far up the chest as the level of his nipple) my
concerns were regarded as requiring the consult of the chief pediatric gastroenterologist.
So I hurriedly, and quite obtrusively, consulted him. He said that since it was
causing the collapse of his left lung the decision whether to go ahead really lay
with the chief pediatric pulmonologist. So I hurriedly made my way to her and
asked for her opinion. Her response was that this was the realm of the chief anesthesiologist
since the relevance during the operation could be known only to him. So I
hurriedly made my way to the chief anesthesiologist who proclaimed that such
matters were in the hands of the pulmonologist.
I cancelled the surgery.
And began a year long journey
trying to find a way to help my son breath more easily, as his breathing became
more labored and the requirement for oxygen simply increased, often becoming
useless as the incidents of obstructions and complete closure of the lung
climbed to multiple daily episodes.
Plication, where they simply sew
the diaphragm in place, preventing it from rising (or being used) was not
something that anyone was willing to do. To much risk involved with fragile
(yet so strong!) Segev. If he were required to be on a ventilator permanently,
then they would consider the surgery.
I investigated the possibility of
a diaphragmatic pacemaker. The chief pediatric neurosurgeon of one hospital
stated that they do make that kind of surgery in Israel but that first you
would have to understand if the phrenic nerve was still functional and there was
no way of doing that. Besides, using such a pacemaker only on one side was
fraught with poor results and required lengthy intubation. This was again
something all the physicians agreed upon wholeheartedly; put my son on a
ventilator and he will not only never come off of it but it would surely signal
the beginning of the end. His body simply wouldn’t have it.
But I continued. I contacted the
chief of ICU in a major hospital, an amazing man, highly regarded as the expert
on ventilators and ventilator weaning, who also specializes in emergency transport of
critical care patients, himself acting as pilot, who came to my home to assess
Segev. He recommended not to do the treacheostomy since this would require a
ventilator post-op which would be the beginning of the end and the hayek RTX cuirass which uses negative
pressure as an external ventilator to reduce CO2 buildup and allow Segev to
breathe easier, even possibly expanding the collapsed lung.
Subsequently I admitted Segev
(after a long waiting period) to the children’s rehabilitation hospital in
Jerusalem to test the RTX and, at the suggestion of the head of pulmonary
rehabilitation, the bipap machine which had been regarded as an absolute
contraindication. We were not able to try the RTX as the doctor was reticent
but we did try Bipap and IPV (interpulmonary percussive ventilation) with good
results. Segev’s CO2 was measured as being as high as 47.
After much bureaucracy and
wrangling I finally received authorization for the Bipap but the IPV was deemed
“excessive” by health insurance. For four months I tried to make it work but
the results were not merely disappointing but the machine became
counterproductive, so I stopped its use.
Once again, after cancellations
and much gnashing of teeth, we were back at the rehabilitation hospital to
check the IPV and convince the insurance of its validity in providing my son
with some help. Not only did we test it a second time successfully but I was
also able (with sleight of hand) to arrange a test run of the RTX. Fantastic
results all ‘round. I was both happy and
dejected. Happy is obvious, but dejected since there was not one known case in
the country where the health insurance had authorized the RTX for in home use.
The authorization for the IPV
actually came as a surprise to me but it is with us and has had much
use, though the extent to which it works under the trying and complicated
circumstances of Segev’s lungs is not the panacea I had hoped it would
be. Still, it contributes and this warrants its continued inclusion in Segev’s
varied daily schedule of treatments.
Eventually I was able to visit a
pediatric ENT surgeon in Jerusalem, after three aborted attempts, to try
and establish whether it was possible to see the extent of damage to my son’s
phrenic nerve, which caused the paralysis. Transcutaneous stimulation was a
possibility but, together with a further researched method of specialized MRI
called magnetic resonance neurography, this will necessarily lead to a dead end
unless there is the possibility of finding a surgeon who would be willing to
undertake reconstruction of the nerve. The difficulty of such specialized
surgery and the inherent danger of surgery for Segev in general has found me,
as of this morning, with empty hands and the feeling that, when it could
possibly matter most, all that I have for my son, are empty promises.
Thank you for putting in words your struggles and naming some of the most difficult emotions that permeate, even motivate a parent to seek a better life for their child.
ReplyDeleteBig hugs. I'm so sorry that all this work to help Segev has hit a wall.
ReplyDeleteWell, my friend, as we have learned in many cases the medical establishment treats or children as anatomical mannequins and in many cases with less skill than an auto mechanic. They, although I will acknowledge, not all, see the pieces: brain, lung, diaphragm, etc There often forget that they are treating a whole child, a whole being and it should be with the care for a whole child. I do not think that medical schools teach this novel idea.
ReplyDeleteSadly, people also view our kids as pieces that somehow don't quite fit together.
You experience is raw, unending, and beyond weary...but I do not believe that the tank is on empty. Warmest regards to you both!
There is great value in your careful documentation; I hope the information can be used to help someone, somewhere, sometime. And while the two of you are thrown against yet another wall, and you are beyond depleted (how well I know both those miserable states) I've witnessed how you regroup, again and again, to move forward in No Man's Land.
ReplyDeleteAs you well know, there is nothing more frustrating and disheartening than traveling this journey without the support of the medical professionals we need to help us help our children. After all you've been through with Segev, how can you not be running on empty? I hope knowing that so many of us out here in cyber-land care deeply about Segev and about you brings you strength so that you can keep on keeping on.
ReplyDelete