‘We are the ones who go back to their homes weeks after the researchers have been here and taken their samples back to the States. The patients ask us, ‘So what happened to those tests performed on us?’ But we have no answers for them, because they’re not our tests.’
The next morning John and Phil picked me up early. ‘You saw a little of the parkinsonism and dementia – the bodig – yesterday,’ he said. ‘Kurland felt this form of the disease was replacing ALS in the 1970s – but you must not imagine the ALS is extinct. I have lytico patients I have been following for years, and new cases as well – we’ll see some today.’ He paused, and added, ‘There is something unbearable about ALS; I’m sure you have felt it, Oliver – every neurologist does. To see the strength go and the muscles wasting, people unable to move their mouths to speak, people who choke to death because they can’t swallow…to see all this and feel you can do nothing, absolutely nothing, to help them. Sometimes it seems especially horrible because their minds remain absolutely clear until the end – they know what is happening to them.’
We were on our way to see Tomasa, whom John has known ever since he came to Guam. She had already had lytico for fifteen years when he met her; it has advanced steadily since, paralyzing not only her limbs but the muscles of breathing, speech, and swallowing. She is now near the end, but has continued to bear it with fortitude, to tolerate a nasogastric tube, frequent choking and aspiration, total dependence, with a calm, unfright-ened fatalism. Indeed a fatality hangs over her entire family – her father suffered from lytico, as did two of her sisters, while two of her brothers have parkinsonism and dementia. Out of eight children in her generation, five have been afflicted by the lytico-bodig.
When we entered her room, Tomasa looked wasted, paralyzed, but alert. With a cheery ‘Hello, Tomasa, how is everything today?’ John walked over to the couch where she was lying. He leaned over and touched her shoulder, and she followed his hand with her eyes, which were bright and attentive. She followed everything, with an occasional (perhaps sometimes reflexive, pseudobulbar) smile, and a slight groaning as she exhaled. She was dying in full consciousness, after twenty-five years of an implacable disease, in a bright sunlit room. John introduced me to Tomasa and to her daugher, Angie, who was with her. When I asked her date of birth, Tomasa produced a string of (to me) unintelligible sounds, but her daughter interpreted this as April 12, 1933. Tomasa could open her mouth on request, and put out her tongue. It was fearfully wasted, fissured, fasciculating, like a bag of worms. She made another unintelligible sound. ‘She wants me to bring you and Dr. Steele something to drink,’ Angie said. Tomasa’s manners have not deserted her, even at this point. ‘She has taught countless people about the disease on Guam,’ said John; Tomasa smiled. ‘Don’t worry, Tomasa – Angie will not get the lytico. No one in the younger generation gets this, thank God,’ he added softly.
Family, friends, neighbors, come in at all hours, read the papers to her, tell her the news, give her all the local gossip. At Christmas, the Christmas tree is put by her couch; if there are local fiestas or picnics, people gather in her room. She may scarcely be able to move or speak, but she is still, in their eyes, a total person, still part of the family and community. She will remain at home, in the bosom of her family and community, in total consciousness and dignity and personhood, up to the day of her death, a death which cannot, now, be too far off.
Seeing Tomasa surrounded by her large family made me think of a 1602 description of the Chamorros by an early missionary, Fray Juan Pobre, which I had seen while browsing in John’s office:
They are naturally very compassionate people… The day when the master of the house, or his wife, or a child falls ill, all the relatives in the village will take dinner and supper to them, which will be prepared from the best food they have in the house. This is continued until the patient dies or recovers.
This acceptance of the sick person as a person, a living part of the community, extends to those with chronic and incurable illness, who may, like Tomasa, have years of invalidism. I thought of my own patients with advanced ALS in New York, all in hospitals or nursing homes, with nasogastric tubes, suction apparatus, sometimes respirators, every sort of technical support – but very much alone, deliberately or unconsciously avoided by their relatives, who cannot bear to see them in this state, and almost prefer to think of them (as the hospital does) not as human beings, but as terminal medical cases on full ‘life support,’ getting the best of modern medical care. Such patients are often avoided by doctors too, written, even by them, out of the book of life. But John has stayed close to Tomasa, and will be with her, with the family, the day she finally dies.
From Tomasa’s house, we drove north across the island, up through the cycad-dappled hills, and past placid Lake Fena, Guam’s only reservoir of fresh water.63 Everything looked very dry on the plateau; at one point, John pointed out the charred trees and large areas of blackened ground which were the legacy of a great forest fire the previous summer. And yet here, even in these blackened areas, were new shoots of green – shoots which came from the stumps of cycads.
Dededo is a more modern village, now the largest on Guam after Agana. It has a somewhat suburban look, with each house set at a little distance from the others, so there is more sense of ‘privacy’ (though this seems to be more a Western concept than a Chamorro one). It is in one of these houses that Roque lives. He is a strong, muscular man in his early fifties – robust, covered with tattoos from his tour of duty in the army – in perfect health, apparently, until fourteen months ago, when he started to complain of something blocking his throat. He soon noticed symptoms in his voice, his face, his hands, and it became clear that he had a rapidly progressive, almost fulminating, form of lytico. While he is not too disabled at this point, he knows he will be dead in a few months. ‘You can talk to me about it,’ he said, seeing my reluctance. ‘I have no secrets from myself.’ Part of the problem, he said, was the mealymouthed doctors in Agana, who were evasive, who wished to convey hope and reassurance – an optimistic, false view of the lytico, which might prevent him from coming to terms with it, with his rapidly narrowing life and the certainty of death. But his body told him the truth – and John did too.
‘I was a very athletic man, and now the disease has pulled me down,’ he told us. ‘I accept it, but sometimes I feel so depressed that I feel like doing something drastic…To commit suicide is no good. It’s not right. But I wish the Lord would take me rather than wait for no result or no cure. If there’s no cure, I would have the Lord take me.’
Roque was deeply sad, he said, that he would not see his children grow up, and that his youngest son (just two now) might not retain any memories of him; he was sad that he would be leaving his wife a widow, and his old parents, still in good health, bereaved.
What will happen with him, I asked John – will he die at home, like Tomasa, or will he go to a hospital? ‘That depends,’ said John, ‘on what he wants, what the family wants, the course of the illness. If you have complete bulbar paralysis, and respiration is affected, you have to have assisted breathing, a respirator, or you die. Some people want this, some do not. I have a couple of patients on respirators at St. Dominic’s – we’ll see them tomorrow.’
Later in the afternoon, Phil and I had planned to go down to the beach at Sumay, said to be the finest for snorkelling in Guam. This was on the military base, so Phil had arranged permission for us to go. We arrived around four, and presented our papers. But our reception at the gate was surly and suspicious, especially when the guards saw that Phil was a Chamorro.
When I tried to put a good-natured, genial spin on things, I was met by a blank, faceless stare – I was reminded, unavoidably, of the hateful episode on Kwajalein, the helplessness of civilians, civility, in the face of military bureaucracy. Phil had warned me that I had best say nothing, that we both had to behave in the most deferential, abject manner, or they would find reason to deny us entrance at all. I had thought, at the t
ime, that his advice was a little overstated – but now I saw that it was not. In the event, we were kept waiting at the gate for an hour, while the guards phoned for various permissions and confirmations. At five o’clock, we were told that our admittance had been approved – but also that it was too late, because the base was now closed. At this point, fortunately (for I was about to explode with rage), a senior officer came along; we could override the regulations this time, he said – we could enter and have our swim, but we would have to be accompanied by military police as long as we were on the base.
Phil choked at this, and the feeling of supervision made me furious, but having come this far, we decided to go ahead and have our swim. Changing into our swimming gear in full sight of a jeep with four police was slightly unnerving, and an antino-mian part of me wanted to do something outrageous – but I controlled myself, with some regret, tried to put the police out of mind, and surrendered to the water.
It was, indeed, exquisite. There are more than three hundred species of coral native to Guam, and the colors of these at Sumay seemed far richer than those at Alma’s, or even those of the glorious corals off Pohnpei. A little farther from shore, we could see the outlines of the wreck of a Japanese warship, richly and strangely metamorphosed by a crust of barnacles and corals – but it would take more time, and scuba gear, to examine it properly. As we swam back in, I could see the shape of the waiting jeep shivering through the transparent waters, and the stiff figures of the MPs, distorted by their shifting refraction. As we dried ourselves in the gloaming, I seethed to think that this perfect reef was denied to the people of Guam, hoarded and locked up by institutional order.
But Phil’s anger had a deeper layer. This was the site of the old village of Sumay, he said as we drove back to the entrance of the base. ‘It was the most beautiful village in the whole of Guam. It was bombed by the Japanese, the first day they attacked Guam; then all the inhabitants were evicted or killed. When the Allies came, the Japanese retreated to those caves in the cliffs you can see, and trying to get them out, the Americans bombed the whole place into dust. That fragment of the church and the graveyard – that’s the only thing left. My grandparents were born here,’ he added, ‘and they are buried here too. Many of us have ancestors in the graveyard here, and we want to visit the graves, pay our respects – but then we have to go through the bureaucratic process you’ve seen. It is a great indignity.’
The next day, John and I set out for St. Dominic’s, a beautiful new hospital, or, as the nuns prefer to call it, Home, with gardens, patios, a tranquil chapel, perched on Mount Barrigada, overlooking Agana. Here were two more patients of John’s – both, like Roque, still in their fifties, and stricken by lytico in its most virulent form. Both had been in perfect health, seemingly, eighteen months before; both had now reached a point where the muscles of respiration were paralyzed, and mechanical ventilation was needed to help them breathe. As we approached their rooms, I heard the heavy, animal-like breathing of their respirators, and the unpleasant sucking sounds made as their throats were suctioned dry (for they could no longer swallow their own secretions and had to have these sucked out mechanically, lest they be aspirated into the trachea and lungs). I could not help wondering whether life was worth it under these conditions, but both patients had children with them – an adult son in one case, an adult daughter in the other – with whom some contact and simple communication was still possible; they could still be read to, watch television, listen to the radio. Their minds were still alive and active, even if their muscles were not, and both had indicated that they wanted to go on, to stay alive as long as they could, even if this meant being maintained on a machine. Both were surrounded by religious pictures and icons, which they gazed at with unblinking eyes. Their faces, I wanted to think, seemed to be at peace, despite the heaving, gurgling bodies below.
Many patients with very advanced bodig come to St. Dominic’s too, in some cases suffering not only from parkinsonism, but from a severe dementia and spasticity as well. In such patients, in the final stages, the mouth hangs open, drooling with saliva; the palate hangs motionless, so that speech and swallowing are impossible; and the arms and legs, severely spastic, become bent in immovable flexion contractures. Patients in this state can hardly be looked after at home by even the most devoted families, and are usually brought to St. Dominic’s, where the nuns are devoted to their care. I was deeply moved by the dedication of the nuns who undertook this care; they reminded me of the Little Sisters of the Poor, an order of nuns I work with in New York. Unlike what one sees in most hospitals, the Sisters’ first care, and continuing concern, is with the dignity and state of mind of each patient. There is always a sense of the patient as a total individual, not just a medical problem, a body, a ‘case.’ And here, where family and communal ties are so close, the patients’ rooms, the corridors, the patios, the gardens of St. Dominic’s, are always thronged with family and neighbors – the family, the village, the community, of each patient is reconstituted here in miniature. Going to St. Dominic’s does not mean a removal from all that is dear and familiar, but rather a translocation of all this, as much as is possible, into the medical milieu of the hospital.
I felt drained by seeing these patients with lytico and bodig in their final, terrible stages, and I wanted desperately to get away, to lie down and collapse on my bed, or swim again in a pristine reef. I am not sure why I was so overwhelmed; much of my practice in New York involves working amid the incurable and disabled, but ALS is rare – I may see only one case every two or three years.
I wondered how John, who has forty or more patients with advanced lytico-bodig, dealt with his feelings. When he was with patients, I noted, he often adopted his booming, professional voice, and an optimistic, bracing, cheery manner – but this was only a surface, behind which he remained intensely sensitive and vulnerable. Phil later told me that when John is alone, or thinks he is alone, he may weep at the plight of his patients, and at his impotence, our impotence, to do anything about it.
After lunch we visited a different part of St. Dominic’s – a pleasant, open room looking onto a garden, where some of the day patients had collected for their afternoon session. St. Dominic’s is not just a chronic-care hospital, but also has an active day program for ambulatory patients who come from all over the island. It is a place where they can meet, enjoy meals together, walk in the gardens, or work in a workshop, and receive therapy of all sorts – physiotherapy, speech therapy, arts and music therapy. It was here that John brought me to see Euphrasia, another patient of his. She is seventy, but looks much younger, and has had a parkinsonian form of bodig for twenty-four years, though not the least memory impairment or dementia. She had moved to California as a young bride soon after the war, and did not revisit Guam for many years. Nevertheless, she came down with bodig in 1969, despite having lived out of Guam for twenty-two years.
Seeing Euphrasia brought home to me the immense lag which might exist between exposure to whatever it is (or was) on Guam, and the subsequent development of lytico-bodig. John told me, indeed, that he had heard of one patient in whom the gap between leaving Guam and developing the disease was more than forty years – and that there might be similar lags in those who came to Guam. No Caucasian, as far as he knew, had ever contracted the disease, but he knew of a few Japanese and Filipino patients who had come to Guam, married Chamorros, entered the culture completely, and then come down with apparent lytico or bodig many years later.64
This, for him, was the most convincing clinical evidence of the extraordinary ‘silent’ period in which the lytico-bodig must, in some sense, be present – but subclinical or latent. Was it burning away slowly beneath the surface, all through these years? Or did there have to be a new event, which might ignite a previously harmless, perhaps arrested, process and turn it into an active one? Sometimes he favored the first thought, John said; sometimes the second – though seeing a patient such as Roque, in whom there had been so explosive an onset of dis
ease, erupting in the midst of seemingly perfect health, one had less sense of a steady, ongoing process finally surfacing than of a sudden, lethal transformation.
I thought of how von Economo, the physician who had first identified the encephalitis lethargica, had spoken of postencephalitic patients as ‘extinct volcanoes.’ This seemed an apt comparison until L-DOPA came along, when I began to think of them as sleeping volcanoes, which might suddenly (sometimes dangerously) erupt with this new drug. But these patients were already manifestly ill – frozen, catatonic; whereas the lytico-bodig patients, seemingly, were perfectly well and active before their symptoms began. ‘But you can’t be sure of that on purely clinical grounds,’ said John. ‘You have no way of judging what may be going on at the cellular level.’ What had been going on, we wondered, in Euphrasia during those twenty-two years after she had left Guam?
Euphrasia was started on L-DOPA by her doctor in California in 1969 (this intrigued me, as it was the same year I had started my own post-encephalitic patients on L-DOPA). In ordinary Parkinson’s disease, the initial effects of the drug are smooth and steady and last for many hours, though sooner or later, its effects may become unstable, giving patients a brief period of fluidity, sometimes accompanied by chorea and other involuntary movements, followed in an hour or so by an intense immobility – a so-called on-off effect. Such on-off effects, I had found, tended to set in much earlier with my post-encephalitic patients – sometimes, indeed, from the very start, and Euphrasia too, John said, had shown reactions which were extreme and hyperbolic from the beginning. And yet despite its ups and downs, she continued to get a crucial benefit from L-DOPA, for it allowed her a few hours of relatively good function each day.