1996 - The Island of the Colorblind (17 page)

‘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 time, 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 disease, 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.

She had not had any medication for several hours when we stopped by, and she was in an ‘off’ state, sitting completely motionless in a chair, her head bent, almost jammed, on her chest, only her eyes still capable of any movement. There was extreme rigidity in all her limbs. Her voice was very soft, flat, almost inaudible, and devoid of any animation or expression. She drooled constantly.

John introduced us, and I took her hand and squeezed it gently She could not speak, but she smiled back, her eyes crinkling, and I could feel a faint squeeze in response.

With a conspiratorial wink to Euphrasia, I said to John, ‘I’ll show you something – or Euphrasia will.’ I managed, with some difficulty, to get her to her feet. Walking backward in front of her, holding her gnarled hands, cueing her all the time, I was able to guide her, with tiny, tottering steps, to the garden just outside. There was a rock garden in the form of a little hill, with irregular ledges and slopes. ‘Okay,’ I said to Euphrasia, pointing to a rock, ‘climb over this, you’re on your own – go!’ To John’s horror, and the nuns’, I took my hands off her, and let her go. But Euphrasia, who had been almost incapable of movement on the flat, featureless floor of the dayroom, lifted her leg high, and stepped boldly over the rock, and then over another one, and another, up to the top of the rock garden, without difficulty. She smiled, and climbed down again, as surefootedly as she had gone up. As soon as she reached the level ground, she was as helpless as before. John looked rather stunned at this, but Euphrasia still had a ghost of a smile on her lips – 
she
was not in the least surprised at all. And had she been capable of speaking, she might have said, like so many of my post-encephalitic patients, ‘If only the world consisted of stairs!’

It was two o’clock, the nun said, time for her medicine. She brought Euphrasia, now sitting in the dayroom once again, a tiny white pill with some water. Fourteen minutes after receiving her L-DOPA – we timed this, as if waiting for a chemical reaction, or explosion – she suddenly jumped to her feet with such energy her chair fell over backward, hurtled along the corridor, and burst into lively, even rambunctious, conversation, bursting with all the things she had wanted to say, but could not while she was frozen. This was not just a disappearance of her parkinsonism, her motor problems, but a transformation of her senses, her feelings, her whole demeanor. I had not seen anything like this in more than twenty years, and was both stunned (though I had half expected her to show such a reaction) and a bit nostalgic – Euphrasia especially reminded me of my postencephalitic patient Hester, in whom there was a similar, instantaneous transformation, with no intermediary state, no warming-up period whatever.