Mentored by a Madman

by A. J. Lees

  Illich believed the medical profession were on a fruitless mission to eradicate pain, sickness and even death and was doing a great deal of harm by whipping up unrealistic expectations. Old age, handicap and death had been handed over by society to doctors to keep families from having to face them:

  Doctors deploy themselves as they like, more so than other professionals, and they tend to gather where the climate is healthy, where the water is clean, and where people are employed and can pay for their services.

  Illich warned of disease caused as a direct consequence of medical and surgical intervention:

  A culture can become prey of a pharmaceutical invasion. Each culture has its poisons, its remedies, its placebos, and its ritual settings for their administration. Most of these are destined for the healthy rather than the sick.

  Doctors had become the high priests of ‘a vast monolithic religion’ and the teaching hospital was geared for them rather than their patients. They had entered into a Faustian bargain that replaced healing with treatment, listening with investigations and caring with managing. The distressed human was divorced from the transaction.

  Illich’s doomsday prediction did not seem to have reached the National Health Service yet but it was a severe warning that I knew could easily come true. I wondered what Burroughs was thinking as he read Illich’s writings in his dark windowless room at 222 The Bowery (‘The Bunker’). He had written that schools should teach values, not facts, and shared many of Illich’s views about the shortcomings of modern medicine and the rottenness of Western Society. He also warned that technological advances could be highly dangerous in the wrong hands. He would have rejected Illich’s vicarious tolerance of pain even if he accepted his imputations of conspiracy by those who were paid to relieve it. Unlike Illich, Burroughs was not against painless dentistry, the use of modern anti-biotics and anaesthesia.

  In the end my fascination with neurology prevailed. I pushed Illich and his uncomfortable views to the back of my brain and returned to the pleasure of listening to patients’ stories in the clinics.

  5

  – Hanging Out with the Molecules –

  Many of the patients I was now seeing in the research clinic at University College Hospital had started to worry that they were becoming allergic or resistant to their drugs. They complained that some doses of L-DOPA, especially when taken with large meals, failed to work and that they could no longer rely on four or five hours benefit from each tablet. When their pill came on-stream it was as if someone had switched the light back on but they were having more and more Cinderella moments of disablement. Flinging helicopter movements of the limbs and distressing involuntary grimaces marred their sense of well-being even during their shrinking periods of mobility. One told me he now knew where the Monty Python Ministry of Silly Walks had come from. Another felt as if he was on a tightrope. The patients described tingling and stiffening followed by a terrifying sensation of their batteries running down. Some reported visions of motionless, silent Lilliputians and shadowy presences. Others had hallucinations of animals sitting in their homes or stalking the garden and grey mice scuttling across the floor.

  ‘DB’, a teacher who had developed Parkinson’s disease at the age of thirty-six, arrived in a wheelchair in great pain from contortions of his arms and legs to tell me tearfully that life with L-DOPA had come to resemble a never-ending big dipper ride. When he left the outpatient clinic twenty minutes later ‘cured’ and walking briskly, my reputation as a healer was greatly enhanced with the receptionists but all that had happened was that his medication had lit up his dopamine brain during the consultation. These were erratic ‘all or none’ occurrences. Everything was capricious. Nothing was predictable.

  I felt I was on a voyage of discovery and that if I could record and document accurately what I was now seeing I might be able to decipher a chaos that appeared to defy the laws of pharmacology. I began to think it was amazing that L-DOPA had ever worked in the first place. I also now accepted that making an individual whole again couldn’t be reduced to the prescription of a treatment however effective it was in improving quality of life. The art of medicine was not to be confused with artfulness. It had nothing to do with smoke and mirrors but was a skill born out of experience and practice and a knowledge of universals.

  Clinical pharmacology was a great strength at University College when I arrived in 1974. James Black, Nobel Laureate who had developed propranolol for the control of angina pectoris and cimetidine for the treatment of peptic ulceration, had moved from his post in industry to establish a new degree course in medicinal chemistry. Desmond Laurence, Professor of Clinical Pharmacology at University College Hospital, had written the definitive undergraduate textbook on his subject and was an inspiring presence at the Clinical Grand Rounds. As young doctors in training we were encouraged to test new drugs on ourselves and on patient volunteers in the wards.

  Not long after I had begun my specialist neurology training at University College Hospital, the head of the medical department at Sandoz approached my chief, Gerald Stern, with an offer to test a new molecule in the clinic. The new drug 2-brom-alpha-ergocriptine (bromocriptine), synthesised from the naturally occurring alkaloid ergocryptine, was a potent stimulator of brain dopamine receptors and a candidate anti-Parkinson drug. Although the Sandoz Company had been forced to discontinue the sale of LSD-25 (Delysid) in 1965 it had maintained a research interest in the medicinal potential of ergot compounds obtained from the rye fungus (Claviceps purpurea) and ergotamine was still in use for the treatment of acute migraine while ergometrine was used by obstetricians to expel the afterbirth.

  After we had obtained ethical approval I began to cautiously escalate the dose of bromocriptine in ten patients who had either been recently diagnosed with Parkinson’s disease or who had been unable to tolerate L-DOPA treatment. At doses above 40 mg a day their symptoms of slowness, stiffness and trembling started to lessen to a level that could be appreciated without the need to consult the rating scales I was using to give my study a pseudo-scientific robustness.

  These preliminary findings that were published in the 14 October 1975 issue of The Lancet encouraged me to use bromocriptine in more previously untreated patients with Parkinson’s disease in the hope that the drug might avoid some of the complications that were now occurring commonly in the clinic with L-DOPA. I also started to look for groups of patients with related parkinsonian disorders that had been unable to tolerate or had not responded to standard drug therapy.

  A few of the survivors of the pandemic of sleepy sickness were still living in the villas of the Highlands Hospital (previously the Northern Convalescent Fever Hospital), just north of Winchmore Hill in Enfield, where a special long stay unit for 100 children aged between three and sixteen had been established in 1925. Most of them had developed a unique neuropsychiatric syndrome that resembled Parkinson’s disease but differed in its presentation, course and response to medication.

  The surviving Highlands Hospital postencephalitics had been given a one-month trial of L-DOPA in 1969. After less than two weeks of treatment, pathological imbalances of the sort that I was only now starting to see in the patients with Parkinson’s disease at University College Hospital had emerged in several of the patients. Ten had experienced wild jerky twisting movements of their tongue, lips, face and limbs, six had become mentally agitated and manic, two had complained of shortness of breath and one woman had developed severe anxiousness with constant high-pitched moaning. Unfortunately, the severity of damage to the dopamine-containing nerve cells in their brains had rendered them exquisitely sensitive to the treatment and only two out of the forty had derived lasting, useful benefit from L-DOPA for more than a year. They were ideal candidates for the bromocriptine trial.

  Encephalitis lethargica (sleepy sickness) is a virus of the mind that causes a kaleidoscope of bizarre and bewildering neurological and psychiatric symptoms. About five million people fell prey to the disease between 1916 and 1927, many of
whom died. The brunt of the damage occurs in the ‘lizard brain,’ inherited from our pre-mammalian forebears and a sanctum for the interplay of motion and emotion. Impulsive, obsessional and enraged behaviours are common sequelae. The plague allowed tics and antics to mushroom in the dark cellars of the basal ganglia, nurtured by spurts of dopamine.

  During his time working at Beth Abraham Hospital in the Bronx, Doctor Oliver Sacks wrote several letters to The Lancet describing the effects of L-DOPA on the signs of parkinsonism in a similar postencephalitic colony. Under pressure from his medical director, who saw little point in adding to the correspondence section of a British medical journal, he was then browbeaten to write up his overall experience in sixty patients for the Journal of the American Medical Association. His article in the September 1970 issue drew particular attention to the hazardous reactions to the drug and the emergence of a phenomenon he referred to as incontinent nostalgia. The startling physical and mental rebirths of the patients had been followed by retribution and finally a painful re-adjustment and accommodation to their imprisonment.

  The entire correspondence in the letters section of the next edition was devoted to highly critical and bitter responses to his article, questioning his accuracy of observation and intimating that even if true he should have thought hard before publishing the findings because it would negatively impact on the atmosphere of optimism necessary for a positive L-DOPA response. The editor permitted no right of reply. Sacks’ article had cast doubt on predictability itself and needed to be censored. Rational discussion was scarcely possible. Much more distressing though was when the sister of Rose R., one of his patients, held up the New York Daily News that had reprinted word for word one of Sacks’ letters to The Lancet, complaining, ‘Is this your medical discretion?’

  Sacks now felt trapped. He knew he had something of importance to say but if he were to remain faithful to his experiences he would inevitably forfeit medical ‘publishability’ and the acceptance of his colleagues. He would be at risk of losing the serious stamp of science. If he were to write the detailed case histories of his patients, he would also need to obtain their unconditional consent and disguise their identity and the institution involved. His friend, W. H. Auden, gave him particularly good advice in the planning phase for the intended book. ‘You’re going to have to go beyond the clinical … Be metaphorical, be mystical, be whatever you need.’

  When Awakenings was published in 1973 it was reviewed positively in the newspapers and by the literary magazines and awarded the Hawthornden Prize for imaginative literature. Sacks had taken Auden’s advice and attacked the mechanical methodologies insisted upon by medical journals and epitomised by the early reports of L-DOPA in Parkinson’s disease that he described as ‘the ugliest exemplars of assemblyline medicine; everything human, everything living, pounded, ground, pulverized, atomized, quantized, and otherwise “processed” out of existence’.’the

  However, a strange silence lasting more than a year prevailed in most of the medical press before a few short and mixed reviews trickled out. Sacks found one particularly galling:

  This is an amazing book, the more so since Sacks is talking about non-existent patients in a non-existent hospital, patients with a non-existent disease, because there was no worldwide epidemic of sleepy sickness in the 1920s.

  – On the Move: A Life

  I did not meet Oliver Sacks for several more years but his experience following the publication of Awakenings provided me with a salutary reminder of the conservatism of the medical establishment. Doctor Sacks had crossed the line and was now considered an eccentric.

  With the help of Dr Joseph Sharkey, the Medical Director, I selected twelve of the most severely handicapped patients who had been inmates in the Highlands Hospital for more than half a century, and despite their negative experiences with L-DOPA they were still game to try another experimental drug. I felt it a great privilege to be allowed into this secret world that seemed to be suspended in time. For the next year, this out of the way hospital would become the home for my scientific experiments. One of the trial volunteers had been nicknamed Puskás by the nurses after the Hungarian football maestro of the 1950s. For most of the last forty-five years he had remained catatonic, barely able to move, and in need of help for all everyday tasks. However, when one of the nurses threw him a ball, he sprang to life, trapping the ball adroitly with his feet and dribbling skilfully down the ward. He could also juggle a matchbox on one foot, kick it in the air, catch it in his hand, drop it to the floor and kick it up again. On one occasion I watched him use this sensory trick to allow him to hop the length of the ward. If a fly landed on his nose he was able to whisk it away smartly with his hand.

  Another of the postencephalitics had for many years shared a room with a fellow sufferer of similar age in the 12A pavilion. Both showed little interest in their surroundings and were mute. One day a loud noise came from the usually silent room and on entering I found that the two living statues had come alive and were wrestling one another and bellowing insults. As soon as they were separated, they froze up again and remained inert on all my subsequent visits. I had witnessed my first examples of a phenomenon called Kinesia paradoxica that had allowed chairbound postencephalitics to run to escape house fires and earthquakes or swim to safety from a sinking ship. If I could understand better this natural unblocking mechanism and how to trigger it, a new drug treatment for Parkinson’s disease might become unnecessary.

  Some of the survivors at Highlands also had strange speech disturbances. Every time I questioned one woman she involuntarily repeated a meaningless phrase over and over again. When I then asked her to recite the Lord’s Prayer, she did so fluently with no trace of hesitation or repetition. Another patient echoed everything that was said to him. One of the elderly charge nurses told me that many years ago there was another lady who rarely spoke but would bellow foul obscenities for no apparent reason. These patients made me think about Burroughs’ view that language was an affliction, an alien organism that hi-jacked the perceptions of its unsuspecting host:

  From symbiosis to parasitism is a short step. The word is now a virus. The flu virus may have once been a healthy lung cell. It is now a parasitic organism that invades and damages the central nervous system. Modern man has lost the option of silence. Try halting sub-vocal speech. Try to achieve even ten seconds of inner silence. You will encounter a resisting organism that forces you to talk. That organism is the word.

  – The Ticket that Exploded

  Did these patients that had remained tight-lipped for so long continue to listen to stillness and communicate internally above the din? As Burroughs had said ‘the most addictive drug of all is silence’.

  I longed to release the brakes from these forgotten patients and bring them back to the land of the living. After I had got to know them and recorded their level of handicap, I started the trial drug in low doses. Each week, on my half day of study leave from University College Hospital, I travelled by bus to Southgate and walked down World’s End Lane. Although now an acute general hospital with five hundred beds, Highlands still had the look of an isolated concentration camp with a gothic central administration block, sinister chimney stacks and a winding lane of two storey L-shaped red and yellow brick buildings with coved eaves. Every time I entered pavilion 12A, my hopes of seeing improvement on the increased dose of bromocriptine were dashed. The drug was better tolerated than L-DOPA but its therapeutic effect on Puskás and the other volunteers was deeply disappointing.

  Although the clinical features of some of the postencephalitics closely resembled those seen in Parkinson’s disease, the extent and severity of damage that had occurred deep in their brains meant that although they still had the capacity to respond to dopamine replacement, their response to drugs was far more extreme and unpredictable. The landscapes in which the postencephalitics resided were far bleaker than people with Parkinson’s disease.

  Meanwhile, a few of the group of previously untreated pa
tients at University College Hospital, who had benefited for more than a year from large doses of bromocriptine, had started to run into difficulties. One day I was rung up by the duty psychiatric registrar to inform me that a woman who I knew well had been admitted to the North Wing at St Pancras Hospital with ideas of persecution. When I went to visit her, she spoke to me in a highly pressured uncharacteristic fashion and complained that the authorities wanted her back at University College Hospital ‘dead or alive’. She informed me that the doctors were ‘trying to change her mind’ and that the nurses had poisoned her food.

  As I moved to sit at the end of the bed, she became agitated in case I squashed a Siamese cat asleep on the cover. One thing that interested me as we talked was that despite her delirium, her signs of Parkinson’s disease seemed much less. I suggested to the psychiatrists that her bromocriptine be reduced down to 30 mg a day and that she continue with the mild tranquillisers they had prescribed for at least two more weeks. Within 48 hours her delusions settled, and the distressing hallucinations that had included a number of faceless men sitting in chairs had vanished. When I saw her in the clinic at University College Hospital, she told me that during her time in the North Wing she had seen flowing visions of enhanced colour and had felt as if time had stopped still for the days of her admission. She was now back to her usual lucid self but her greatly improved mobility that I had witnessed while she was confused had now disappeared and she was again scuffing her soles and not swinging her arms when she walked.