in Bostin who also had thought of it as ‘one’ distant possibility (a ‘Dr. Fish’ or something) was informed.
Then came the time to prepare, write and publish the case of the young girl with C.J.D. -as the youngest case of C.J.D. in the world- in a prestigious medical journal. Both Dr. Martin and Dr. Collier wrote the paper. But being Dr. Martin Mediterranean-and English being his second language, his professional diction ‘not as good,’ Dr. Collier’s version prevailed. Dr. Martin’s paper had emphasized the interesting clinical history-so unusual in a young girl, and THAT was what made the paper important- while Dr. Collier’s version, as a pathologist, was mostly about the tissue findings (which he had missed anyway!)… Dr. Martin was not happy with the basic ‘laboratory tone’ of Dr. Collier’s version, this guy having had little to do with arriving at a clinical -or pathological!- diagnosis; but Dr. Martin gave up his version in favor of that of his senior ‘with better English’ and pretty color tissue photographs which he did not have.
But then another conflict aroused: On top of the wrong person’s version having prevailed to be sent to the New Britain Journal of Medicine, Dr. Collier had put himself as THE FIRST AUTHOR! (having placed Dr. Martin’s name and that of the guys at the NIH ‘following’). Usually the first author is the only one who counts in important scientific papers, and Dr. Martin felt that all the weight of the case and impetus to a final diagnosis had been only his (even if the words in the paper were Dr. Collier’s).
He protested. The doctors at the NIH were very familiar with such disputes of authors claiming but denied first authorship of many papers. They acted as mediators in the dispute and their opinion was in favor of Dr. Martin as first author – as opposed to Dr. Collier. They themselves would be named last. And that is how it was done; the paper was published in its first presentation by The New Britain Journal of Medicine. It gave Dr. Martin (real name changed again) no substantial glory since the nuances of his clinical story had been omitted, and it would later leave in the Faculty of the University against hima negative atmosphere that would haunt perversely to his demise…
Dr. Martin later inquired -as in the previous case where Dr. Bear had learned about the prior transmission by a corneal transplant- more details of the young girl’s preceding medical history. And as in the case of our young girl here with C.J.D., he found that approximately six months prior to the onset of her symptoms, that she had visited a local neurologist because of a blackout that he’d diagnosed as ‘just fainting.’ It turns out -interesting along the same lines of a possible human-to-human transmission, that he had done in the girl an EEG (=Electroencephalogram) using ‘needle,’ intradermal electrodes in her scalp! Even if ‘boiling’ of the needles had been carried out, it is known now that slow viruses -such as the one of C.J.D.- easily survive such – done then with alcohol or by ‘immersion in boiling water.’ Thus one can conjecture that our girl here also had ‘transmitted' C.J.D. by such needle electrodes! (that probably had been previously used in someone ‘with a fast neurological deterioration’ (perhaps diagnosed then as ‘another case of Alzheimer’s’).
These C.J.D. cases tell the reader first, interesting tales of rare medical conditions, and how the best specialists struggle to diagnose them; second, they tell us of a Dr. Frank Martin, foreign-born somewhere in Southwestern Europe who was at least as smart and sleuth diagnostician as the best of his US contemporary colleagues; thirdly, of a guy a bit outspoken who took risks as he ‘told’ his mind -to his own perdition eventually- some times clashing with his academic colleagues; these would eventually bear considerable weight -retaliatory- against him to bury him, as this author has mentioned in the previous case (chapter #6).
CHAPTER 8
More Clashes of Dr. Martin with Academia
A very handicapped young girl with Down’s syndrome who resided in a group home had had seizures since her first year of life. Her parents were unable to care for her and Social Services took over her residence and all her needs. At some point at the age of 6, after she had been followed for years in the University Hospital Pediatric Clinic by Dr. Carlo Porky -due to ‘not getting along issues’ between him and the group home's staff- they asked Dr. Martin if he would take over the care of her seizures. Though she was covered by Medicaid -of course- and the reimbursement was for Dr. Martin at a loss both (for the periodic visits AND for her EEGs) he agreed to see her. Bertie Bones was her name (changed, of course; she is print #18 in the non-fiction book FRANKLY, to which this is its Sequel).
She was no problem and her seizures (Grand Mal) were not very frequent. She was then on Phenobarbital and Tegretol. He figured he would need to see her no more often than once every 6 months… But not too long after that, she began to have the spells every month, almost by the clock; and they started to be harder and longer… When they called him from the group home he suggested that they bring her to the E.R. He saw her as such a couple of times. No significant changes were made except a minor adjustment of her meds. In a third similar visit to the E.R. her physical exam revealed no significant changes from her basic Down’s condition; but her blood work was… fine… except -unusual!- that of her electrolytes, her serum Sodium (= her Na+) was a bit low; it is normally in all of us around 140 mEq/L, but hers was only 130 mEq/L. It is known that low Sodium (called by doctors hyponatremia) can cause seizures, especially it it dips down to the 120s. She was admitted to the pediatric ward, upstairs. Dr. Martin would be her attending for her seizures and the pediatric residents would monitor any general care needs.
With her law Na+, and with no apparent reason for it, the peds resident called the Pediatric Endocrinology attending who came to see her with his team (a resident and two students). Extra tests of her adrenal and kidney functions were done. He thought that the Tegretol she was on, was most likely the cause of her low Sodium. But with the girl being unusually small and slender, even for a child with Down’s syndrome, Dr. Martin ventured that she might have mild adrenal insufficiency causing the drop in her Sodium and that a pinch of steroids taken on regular basis might be what she needed. The endocrinologist shook his head. ‘I don’t think so, Frank. I think it is just from her Tegretol.’
No big deal: ‘Why don’t you switch her to a different drug for her seizures?’ So our compliant Dr. Martin switched her from Tegretol to Dilantin and he discharged her… It was all well… but like previously, in another month exactly she had another, big and long Grand Mal seizure. Back to the ER, her serum sodium was again low, this time even lower, just 126 mEq/L. She was again admitted to the floor. Her anticonvulsant blood levels were fine. This time Dr. Martin mentioned in somewhat stronger terms that she probably had adrenal insufficiency and instead of changing her anticonvulsants again, suggested to start her on Prednisone. Without listening to Dr. Martin for that, the peds resident called again the endocrinology team. And again the big guru balked, shaking his head: ‘This is NOT adrenal insufficiency Frank! Try her on something else.’
So again Dr. Matin cowed and went along against his gut feeling, took her off the Dilantin -though this drug is not supposed to change sodium serum levels- and placed her on Depakote instead. ‘Perhaps the next time this happens I’ll see her just myself in the E.R. without bringing her to the floor and I’ll be the one in charge!’ he thought.
And as he anticipated, exactly again a month later, our little Birtie Bones had another huge seizure. This time, when he brought her to the E.R. he kept her there without calling the pediatric resident for an admission. Her serum Sodium was again low, at about 127 mEq/L. This time, once her seizure was stopped (with I.V. Diazepan, that is Valium), he started her as he had suggested earlier, on steroids. ‘Steroids insufficiency’ -that is, adrenal- is commonly accompanied by a low Sodium level. It was NOT a crazy thought of his. She was fairly small, nearly squalid, and his hint of an insufficient adrenal gland was not off the wall. So he placed on just 10 mg of Prednisone by mouth to be given initially daily, every morning, but just for 10 days. Then that would be cut back to just every other morning, equivalent to 5 mg daily – steroids cause less side effects -if given on long term basis- when they are taken just on alternate days.
So he ordered, no one there to tell him no, and so it was done.
And it worked! She went for three months without any seizures. When then he saw her in the office