July 21, 2013

Running on empty

This is more or less how things went.
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.
Department of Neurology, The University of Colorado at Denver Health Science Center, Denver, Colorado, USA.
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.


  1. 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.

  2. Big hugs. I'm so sorry that all this work to help Segev has hit a wall.

  3. Well, 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.
    Sadly, 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!

  4. 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.

  5. As 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.