A post actually about Segev

My son is in decline. No one appears to be worried about this, the days just go by. There may be some help coming though, to slow it all down and this post is about that. But first a summary of the last few weeks.

We have seen this...

Segev began needing twenty four hour oxygen supplementation early in July, which lasted for one month. We went into hospital for him to have the tracheotomy surgery but not one specialist was willing to commit to an opinion about the relevance of the diaphragm paralysis (which I recently found, though it has apparently been present for six years) vis a vis the impending surgery. While the paralysis explains a great deal of Segev's breathing difficulties, the best treatment offered was no treatment at all, which I am suspicious of due to the physicians categorically disagreeing with me that it may be responsible for many symptoms, including bouts of pain, bowel difficulties, heart rate fluctuations and the need for constantly venting his stomach air, which comes out under high pressure.

Because of the lack of clarity I opted not to proceed with the surgery and seek further information and opinions.

This brought me to a pediatric pulmonologist in Jerusalem who I saw six years ago with Segev. An attentive and very knowledgeable man who, had we lived closer to where he treats, would certainly have been Segev's go-to doctor. The trip was arduous because of the distance, my having a splitting headache and the need to try and continue Segev's measured routine of treatment, suctioning, feeding and medication while in transit. Segev cannot sit in his wheelchair during such a trip because it is only recently that he was approved for an electric lift, which brings with it proper securing for his chair, but the finances provided are not enough to cover all the costs. So he is relegated to a child's car seat for the duration of the trip which, I'm certain you can imagine, is not the place for a fourteen year old with massive kyphosis and scoliosis.

change to this...

The meeting went well although Segev was not physically examined this time. Apparently his condition, based on the chest x-ray was sufficiently obvious and we left with some useful recommendations and the stern opinion, echoing all previous specialists, that the surgery to 'tie down' the paralyzed diaphragm was a great risk for Segev without offering any discernible reward. Also we received an unexpected round of applause over what wonderful care Segev has obviously been provided with.

Later I booked an appointment with a neurosurgeon to discuss the condition of Segev's phrenic nerve, which is apparently the culprit in the paralysis, in hopes of discovering another avenue of treatment. One neuro surgeon could see me in four months time, the other one and a half months from now, so guess which one I took.

Early September Segev's breathing, after a relative lull in difficulties, took another turn for the worse, this time with an affluent amount of phlegm clogging his lungs. Segev's bronchiectasis, despite that radiologic evidence shows minor damage, produces unbelievable amounts of phlegm. Together with bronchodilaters, viscosity reducing agents, repeated physiotherapy sessions and an effective herbal strategy Segev often received as much as six liters of pure oxygen with little effect, his saturation hovering as low as 84% until manual compressions finally succeeded (often at three O' clock in the morning) in opening the blocked passage. 

In my experience the blockage that Segev experiences, which I first saw several years ago in rare, isolated incidents where his entire body would jump upward like a person possessed, heaving violently in an effort to get air into his lungs, is one which completely closes off his left lung. Therefore supplemental oxygen, not entering that lung, simply didn't help.

The number of hours spent applying mechanical ventilation nearly brought me to tears from sheer physical exertion. I knew that something had to be done and if I didn't find something he would have to be admitted to hospital and put on a ventilator. I'm certain some would argue that the time for that, from the description of the events, was already long overdue and I can offer no resistance to such an argument, yet keeping Segev off of a ventilator somehow prevailed.

and this...

An echocardiogram was one of the suggestions due to the enlarged pulmonary artery visible on the chest x-ray. There the result showed not only that Segev's heart has been pushed upwards and considerably towards the right by his protruding, paralyzed diaphragm, but also somewhat twisted it. The cardiologist believes Segev may be suffering from Wolf-Parkinson-White syndrome, encouraged by the knowledge that on occasions when Segev is unconscious, non-reactive for up to ten days at a time, he has periods that his heart rises to 150, 160 and even 170 beats per minute. Further investigation is needed in this regard and the pulmonary hypertension was confirmed by the ultrasound.

I sent a letter to the pulmonary doctor in Jerusalem after I encountered several technologies which appeared to me could be helpful such as the regular Bipap and Intrapulmonary percussive ventilation. The doctor replied but evaded the question. I persisted and he suggested I speak with the head of ICU who specializes in issues surrounding tracheotomies and assisted ventilation, especially weaning patients from ventilators. 

After sending a very brief description of Segev and my expressing my need for some kind of informative consultation he suggested he come to my house to see Segev and discuss the possibilities.

For a man with his responsibilities to come to meet Segev is quite extraordinary and when he was here he related to Segev in a very natural and comfortable manner. There was certainly also a degree of matter-of-factness to his approach but he balanced this with a gentleness that was apparent when he held Segev's hand and spoke to him. I can tell you, despite the beauty of Segev's eyes and his stellar smile, few have been able to so casually sit with him, so I was duly impressed.

The good doctor discussed what he believed to be the best chance of helping my son breath more efficiently, slowing the pulmonary hypertension and possibly even put off the tracheotomy for an extended period, which is called biphasic cuirass ventilation. It is one of the technologies I saw when I was researching options but payed little attention to. There followed a number of amazing stories of patients taken off of ventilators with this (and another) device, including terminal children with cystic fibrosis, waiting for lung transplant but unable while they still needed their trach. The device allowed its removal and ten children had life-extending lung transplants as a result.

I was further invited to visit the doctor in the ICU to see the devices at work, which I did last week. The process of acquiring one will take time, in part due to its cost (here $16,000) which will need to go through the health insurance who, I have no doubt, will fight it tooth and nail. Before that though a serious recommendation will be needed by the pulmonologist, presently abroad, who in turn will most likely need some solid evidence of Segev's need for the machine, including a trial run to see that it indeed can help him.

go back to this...

After fourteen and a half years in an all out effort to entice, encourage, ensconce, berate, enlist and intimidate physicians into having a caring attitude for Segev it has been overwhelming to see these efforts being made. As the saying goes, 'it's who you know', right?  

Presently Segev has some better periods where he requires oxygen only during the night or conversely only during the day. The Great Phlegm flood has subsided which of course is a major factor in the relative improvement. Ongoing adjustments to Segev's anti-epileptic medications (three of them) is slow, erratic and mostly guess work, as any parent to a child with catastrophic epilepsy can attest, but at present there are improvements there as well.

And for now, we have this...