ALS: The Sentence

1.

What’s brutal is that anything, or almost anything, can be the beginning. For some, it starts with choking on a piece of bread; for others, with a stumble, a cramp in one hand, persistent fatigue, a little pain right here, a fall — there are so many other possibilities. At first, we all dismiss it as some minor nuisance: damn, I’d better watch where I’m walking or work a bit less or chew more slowly. But then it happens again, or other nuisances pile on. Then we see a doctor who says it’s probably nothing, that we should rest or get physiotherapy or change something in our diet. And we do it, but those nuisances keep coming back — more frequent, more bothersome — and then we go back and tell the doctor, and sometimes they will tell us not to worry, but other times they send us for tests. Then more tests. And more, more and more irksome each time, more terrifying, months and months of questions and setbacks: a variety of needles, electric shocks, lumbar punctures. And sometimes, when all is said and done, the doctor tells us to bring a family member to the next appointment, or maybe simply tells us to sit down, they have something important to tell us. Afterwards, each one of us will remember that date: it will be the day everything changed, when everything began to come to an end. It will be, some say, the worst day of their lives… and they are not exaggerating. 

Mario doesn’t speak. Yes, he makes a few low sounds that sometimes Blanca, his wife, manages to understand, but no other mortal does. So, Mario types on his phone what he wants to say — which isn’t easy for him — or selects one of the phrases it offers him, and then a slightly robotic voice speaks for him: “Yes, I try to keep living the life I had before, one way or another. But with so many differences…”

Mario’s grey beard has grown out (in general, we men with ALS grow our beards, since we can’t — or don’t want to — shave), his grey hair in disarray, his clothes half put together, his eyes small and bright, early fifties. Mario is an aeronautical engineer, and he used to work at Airbus, on the final assembly line for fighter jets. In April 2022, he began having trouble swallowing and speaking, then coughing; it was only in January 2023 that he was diagnosed with ALS. 

“Some say the worst part is the uncertainty. I’ve sometimes met patients who might have ALS and are wishing for a diagnosis once and for all, because not knowing creates this dreadful vertigo,” says Blanca, and I say, yes, but: 

“Yes, but once they tell you it’s ALS it isn’t vertigo anymore — you’re pushed off a cliff.” 

Mario smiles to himself and says that at first he didn’t even know what the disease was, and he looks at his wife and gestures as if twisting in his chair, so that she can translate and tell me that up to then, they had only heard of Stephen Hawking; but then you start realizing there are so many more than you thought. Mario nods; she interprets: “Well, you have to be… what’s that word everyone uses so much nowadays? Resilient, something like that…”

Mario has kept working remotely, he can drive and of course he can think. The most generous — and the cruellest — thing about ALS is that, in general, your mind stays alert as it watches your body fall apart. Mario keeps careful watch over his airplanes and his disease: he now shows me, for example, a message from the WhatsApp group for patients and relatives, where someone says they found an “isotropic supplement” that makes him feel better, and several say they want it. Some patients look over and over for ways to improve their situation; others abandon themselves to science and fate, which in this case feel very similar.

“Yes, I also think you have to try to keep on with your life as much as possible. But every time there are more things that you can’t do, that you would like to do and you can’t, and sometimes you become obsessed with the illness…,” he tells me, machine-voiced, and I answer yes, and that I don’t know what’s worse: chasing hopeful treatments in desperation or to resign oneself to the fact that there are none and try to make use of the time you have left without wasting it on hope. Mario smiles at me: you can tell he’s thought the same many times. 

I published a book not long ago that began by saying it: “I was told I will die. It’s silly; I shouldn’t need for someone to tell me. But it’s one thing to know that you will die someday — and insist on forgetting you will die someday — and something very different to be told there’s a deadline, and that it’s not even that long.” 

And that’s what they tell you when a very serious man in white says that you have amyotrophic lateral sclerosis, or ALS to its friends: 

“And, in the end, it all gets sorted out in a moment of overwhelming simplicity: a young man behind a desk with his little white coat, his face mask on, putting on a solemn voice. A moment that’s almost banal: a polite man in a polite conversation that didn’t even turn out to be dramatic. He said it to me, he said that no, there’s no cure for it, and that he was sorry, that it was best that I see a specialist in these sorts of things; he referred me to one, he said goodbye with a trace of affection. He had just told me the worst thing I had ever heard in my life, and I didn’t know what to do with it; he did (move on to the next patient); I’m the one who didn’t. I’m the one who still doesn’t,” said my book.

Mario wants to know and doesn’t want to know; it happens to all of us. You want to know, because you’ve been told you have one of the worst diseases known, and that your life from now on will be whatever that illness decides; you then wish to know what your life — and the end of your life — will be like. And you don’t want to know, because they’ve told you there’s no cure, so then what’s the point of anticipating all those horrors. 

Through the course of my brief life — any life is always brief — I have interviewed many people — hundreds, probably thousands. All types of people: rich and powerful, of course, but above all people in difficult, precarious, dangerous situations. In each of these interviews, I tried to approach the other person as much as possible, to understand them, empathize with them. And many times it seemed to me that I was managing it, but I was always left with a sharp twinge of guilt and distance: after our chat was over, I would go back to being a well-heeled white man who knows that he’ll eat every day; I would go back to being someone so different from them. This is the first time that I won’t: I’m speaking to my equals, to people suffering from what I am suffering, and they know it and I know it, and the difference is abysmal. We don’t speak of what is theirs; we speak of what is ours. I would never have imagined it, but our conversations are radically different, with an intimacy I’ve never had, with jokes I would never have dared to make, tears we don’t hide. Now we’re really talking. 

Mario has to leave for another appointment; we say goodbye with smiles, firm handshakes with hands that barely squeeze. For a long time, I felt very sorry for his difficulties with speech, but when all is said and done, when our conversation ends, he’ll get up and walk away like a little soldier, and I’ll wave at him sunk deep in my chair, and I will envy him terribly. ALS is like that — sly, capricious. 

 
2.

There are those who say ALS doesn’t exist. That, really, saying ALS is a bit like saying cancer — a single word for so many different diseases. Certainly, science has no idea of its origins or mechanisms; certainly, each patient evolves in very different ways. What we know — or what is known, let’s say — is that amyotrophic lateral sclerosis is a disorder of the central nervous system that begins by degenerating or destroying motor neurons in various parts of the body, thereby deactivating muscle after muscle. And that there are two large groups: bulbar, which does its job faster, and which above all begins in the head; soon, the patient will be unable to swallow or cough or breathe. Meanwhile, spinal ALS starts by paralyzing arms and legs, which are necessary but not quite as necessary, and then continues on its path. It’s sometimes slower. 

Those of us afflicted with ALS remember with dreadful precision how and when we were given the diagnosis, when and how our world was turned upside down. Elena also remembers the exact date, over a year ago. And that she had been waiting for it since March of 2023, when she had gone with Paco, her husband, to Rome and had fallen three times in the street. Elena is not yet sixty, her very blue eyes very moist, decades of working for a driving school, her family, her kids: “I knew there was something and that it wasn’t normal… My foot would drop, then my hand, then I couldn’t lift a pen…”

The diagnosis took time, as always, but Elena suspected before that, and she looked up ALS online, and it was a dreadful moment: “I couldn’t believe it, I didn’t want to believe, I thought: It can’t be that this is happening to me.”

And she kept hoping that it wasn’t, and suspecting that it was; after some time, she knew. Elena’s voice grows thick; her throat gets very dry; Paco then brings a glass with a straw in it close to her so she can moisten her throat. And even then, Elena stays quiet, and her eyes speak more than she does.

The most widespread image of an ALS patient, the one most commonly shared in the media, is that of a prostrate body hooked up to medical devices. Fortunately, these are the rarest cases, the extreme ones, those who have been living the longest with the disease and resort to every resource to survive. Most of us still get around, with or without a wheelchair, with or without a voice, with or without despair, trying to stretch the present for as long as possible in the absence of a future. 

Once they knew, Elena and Paco tried to face it. Many of us do; we try those “bucket lists,” only that, suddenly, dying isn’t somewhere far off in the haze anymore. Elena and Paco decided to go with their kids to New York, which they had always wanted to visit, and had a good time; they were dazzled. But the next trip to Punta Cana was difficult: Elena felt worse, the beach isn’t kind to wheelchairs, and they decided to stop trying. Elena and Paco now spend a lot of time in their home in a suburb of Madrid. She rests, watches television, tries not to think about it; he, a manual labourer, is on leave because of anxiety, and says: “What I do is to stay with her.”

“Take care of me,” Elena says, and smiles at him.

“Yes, take care of you,” Paco assents. 

ALS affects its patients brutally, but also, and very much so, those around them. A husband or a wife, a daughter or a father suddenly become the indispensable extension without which the sick person can do very little. They have to learn to bring us and take us, feed us and clean us, hold us together and accompany us, and frequently, to conceal their own anguish in order not to anguish the other even more. Those with more money can hire people to take over some of the care and manage to work or rest for a few hours. Even so, the burden is heavy. The new law is supposed to ease that burden. For now, though, it’s a compendium of good intentions and hasn’t been regulated or funded yet; it’s paper and promises. And as long as it’s not put into effect, patients keep dying, loved ones keep suffering. 

“Yes, it’s very hard for us, too. You do what you can and many times what you can’t, but, well…,” says Paco, and I ask him if he’s been learning about the disease, about what he could and couldn’t do.

“Well, I’ve gone through different stages. I started when Elena was diagnosed, because I couldn’t believe it either; until the neurologist told me, I thought it was impossible. From then on, I’ve been on several online forums, I’ve been joining some groups for patients, I’ve been reading up more on what the disease is like. But of course, in these groups there are patients in different states, people who are very ill, and then you see where this is all heading and you feel…,” Paco doesn’t say what he feels, but he doesn’t have to: we know. “Anyway, I have to learn to move her better. I’m afraid of making a movement that I shouldn’t and hurt her… For example, when I sit her up and that sort of thing, well, I place an arm here and the other here, and I lift her whole back, but for now she can still hold onto my neck. But when her arms begin to drop…,” says Paco, and Elena looks at him through tearful, loving eyes. In this illness, the future is always a threat. 

 
3.

The son of a carter, Dr Jean-Martin Charcot graduated as a physician in Paris in 1853, and shortly thereafter was appointed to direct an old asylum for the worn and the dying known as La Salpêtrière, near the Seine. When he left forty years later, La Salpêtrière was one of the most reputed hospitals in the world. 

There, with his disciples and patients, Charcot discovered and described dozens of diseases — among them, the one that, because of its numerous variants, no one had ever thought of as a single disease, and which he named amyotrophic lateral sclerosis. “Sclerosis” refers to the lateral hardening of the spinal cord, which stops sending electrical impulses to the muscles (a-myo), depriving them of nourishment and function, and ultimately atrophying them. But the name didn’t stick: for decades, everyone called it the maladie de Charcot. (Doctor Charcot also oversaw a pavilion housing one hundred and fifty “hysterical and epileptic” women. True to his method, he defined four stages in their condition: epileptoid attacks, acrobatic contortions and positions, emotional verbalization disorders, and finally, delirium. Around 1890, his theories on hysteria were rebutted by one of his disciples, a young Viennese physician named Sigmund Freud, who, from that disagreement, began to develop his own theory of psychoanalysis.)

“First there’s the uncertainty when the person begins to notice that something isn’t working, there’s something odd, and starts getting worried, because they can give it a thousand names, they can think it’s a thousand things, and they’ll keep worrying, wondering all the time, ‘What’s wrong with me?’ What’s the first thing you imagine could be happening? Cancer. That moment of uncertainty is the one of greatest suffering,” says Teresa Salas, the psychologist in the unit. Teresa was born in Peru, where she studied psychology, but in the eighties she came to Madrid to do a specialty, and she settled here. In the mid-nineties, at a point when she was unemployed, someone suggested she go to the Carlos III to speak to Adela. She assumed Adela was some woman that could give her a job; it wasn’t until she got there that she discovered it was the name of the Spanish Association for Amyotrophic Lateral Sclerosis (Asociación Española de Esclerosis Lateral Amiotrófica), founded years before by Dr Jesús Mora. Teresa found another job, but she went back as a volunteer to work with those people. Now, along with her colleague Saúl Marín, she is one of the unit’s veterans — over twenty-five years. Those pioneers trained her, gave her her place there; in her office, which is as austere as the rest, she continues to talk of that horrible moment when they tell you that’s what you have:

“The diagnosis is given and the patient relaxes a bit, because their fear finally has a name. Many say, ‘Oh, at least it wasn’t cancer,’ because they don’t know about this disease, they’ve never heard of it. Then they say ‘Yes, I’ve heard something about the muscles, something weird like that, a strange name, but it isn’t cancer. How lucky!,’ and then they ask about the treatment, if they’ll get better, what it will be like; at that point, they hardly ever wonder ‘How long do I have left to live?’ or anything of the sort. But if the patient has heard about ALS, they fall apart because what they see before them are three years of life, wheelchairs, I’ll die from suffocation; and then a very critical phase begins, the worst of all. And then the moment of readjustment arrives, the ‘What will I do with the time I have? How much is left? What is left?’ and so on; and from then on, the chronic phase begins, and then it all depends on the patient’s personality and characteristics. But I insist, the diagnosis is where they have it the worst: first the uncertainty, then the certainty.”

The ALS unit at the Hospital Carlos III was started in 1990 by one of the few Spanish physicians who, at the time, specialized in the disease. Jesús Mora was forty years old and had worked in Spanish and North American hospitals. Among them was Massachusetts General Hospital, the leading centre for ALS, from which he returned convinced the disease could not be treated without an interdisciplinary team including neurologists, pulmonologists, physiotherapists, psychologists, nurses, speech therapists, endocrinologists and a few other specialists. He set it up with the usual difficulties and delays, and kept it going until 2017, when he had to step down. But the unit continued operating and is now directed by Dr Mascías, a neurologist who had trained under him for many years: “When we began, we were very much alone. We had no palliative care. Rehab therapists would ask, ‘What for?’ There weren’t any nutritionists or pulmonologists willing to adjust a patient’s ventilatory support, or no one prepared to perform gastrostomies…” 

Many call Dr Mascías “doctor,” a few “Javi,” and some “Javier.” He is a large man, solid, with youthful white hair, in his fifties and with a smile that isn’t always forthcoming. When he graduated in 1992, he wanted to become a neurologist, and he became one, but his specialization in ALS “was a fluke,” he says now. Towards the end of last century, Mora convinced him to join him on this path, and has been there ever since. “No doctor likes ALS. It’s thankless; these patients have no satisfactory treatments. It’s a progressive disease where a patient will always come in saying they’re the same or worse; breathing becomes difficult, swallowing… You can’t do much, and then the general neurologist prefers an escape valve — which is us. Saying, ‘Hey, look, I’ll send you there because I know they will take better care of you;’ and that way they’re free of a chronic, complex patient that they really have few satisfactory things to offer to.”

(ALS is a disease that primarily affects people between the ages of forty and seventy, more men than women but not that many more. There aren’t many of us: around four thousand in all of Spain. At some point I came to think that the care provided to us was disproportionate, until someone explained to me that the number doesn’t grow, because for each new patient, there’s one that dies: three or four a day, I was told.)

In the century and a half that passed since Charcot saddled it with that ugly name, the science of ALS has advanced very little. Doctors still don't know how or why it begins and, above all, how to deal with it. They diagnose it, they regret it deeply, and they prepare — at least, the best ones do — to accompany you throughout the degradation. The last medicine approved for its treatment is called riluzole, was launched in 1995, and (according to the most optimistic calculations) manages to add four or five months to the patient's life. It's a matter of faith: we take it to last longer, but nobody knows how long they would have lasted without it. We take it anyway: it's the only thing there is, a minor ritual of sorts every morning, every evening, the way of imagining that at least we do something to stop the monster. 

The comparison to cancer, the king of contemporary fears, is inevitable. More than once I found myself envying those with cancer, who could at least find hope through treatments and throw themselves into that pathetic commonplace that is “their fight against the disease.” And yet I’m thinking now of another radical difference: cancer is something that doctors discover, monitor, confront; in contrast, ALS is a disease you yourself begin to feel. And once diagnosed, it’s the patient, and not an X-ray or a test, who discovers its progression when they realize they can’t move their left arm or pronounce the word “tremendous.” Unlike almost all the rest, ALS is not a disease someone tells you you have; you live it instead, feeling it advance step by step every morning when you wake up.

“How are you feeling today, darling?

“Oh, so-so. Maybe a bit limper.”

“I wouldn’t worry… It’s probably just in your head.”

I ask Javier Mascías how he can bear caring for patients with no cure, who are already condemned: “Well, I think about how I can help them with all those small problems or symptoms they have over the course of their illness, and that those are things we can do something about. That the person has no pain, that they sleep well, that they don’t suffer unnecessary pain, that they’re in a good mood, that they feel less fatigued, that they can breathe better... this whole series of things, and above all that feeling of being accompanied, that you have somebody taking care of you. That’s what offers some consolation; it lets you tell yourself that you can still do things. It seems like a small thing, but it can be a lot.

“Patients are all different. And they’re also different in their way of adjusting, of confronting the disease,” says Teresa, and that perhaps those who suffer most are the ones that from the very beginning have to abandon their usual activity. Once again, class: most of those who can’t continue are the ones who did manual labour, in the fields, the factory, in construction, in the service sector, at home. We, the privileged in the disaster, can keep up our activities for a much longer period, and that, they say, helps survival.

 
4.

The hospital lights are merciless, because it’s assumed that here it’s best to see everything, even though it’s never better to see it all. So, hospitals have very few shadows: their white walls, their yellow chairs, those colours that are more or less an absence of colour, those gummy floors made of some material that might be plastic or stone or rubber, or some peculiar particle the Lord created for hospital floors. In any case, hospitals present themselves as radically clean spaces; they simulate a kind of cleanliness that is meant to calm us: viruses can’t be seen, but if everything is shiny we believe there aren't any here, I suppose, or something of the sort.

“For me, it’s comforting to come here: they take good care of you, they make you feel supported, they smile at you. What’s strange is seeing all these people and thinking that they’re going through more or less the same thing I am,” Marta, who comes from Cuenca, tells me with a tearful voice. She used to be a health care worker and liked her job, and leaving it was very tough. But worse, perhaps, was accepting that she had to ask her husband, a retired banker, for almost everything:

“Sometimes I might tell him, ‘Go have some coffee, bring me bread, we don’t have bananas, come on, go and have some coffee.’ That way, I can put on the cough machine by myself and relax quietly, and I spare him the chore. Poor man, who’d never held a frying pan or an iron in his life, and now has to take care of everything…”

Many things are done in hospitals, but they’re done, above all, to reassure people. That’s why in a hospital almost nothing very visible ever happens. The worst cases, the worst situations take place out of sight, in the ICU, in an operating room, in places that are apart. But here, in the hallways of the ALS unit, we see each other and we recognize each other, we smile at each other with our eyes: I know that you do too, and you know that I do. It’s an intimate closeness, a solidarity of the doomed: sometimes silent, sometimes talked about, above all with looks and smiles. Morituri te salutant and all that bullshit.

And we have to learn words, submit to words we never should have heard. 

‘And what’s that, child?’ 

‘E-lec-tro-myo-o-gram, Mother, that’s what they told me.’ 

‘And does that hurt, child?’ 

‘I don’t know, Mother, but they tell me it’s something they have to do to you so they can see how your electricity is working.’ 

‘My what?’

‘Yes, Mother: your electricity.’ 

There are three kinds of people in hospitals, three classes that are both more and less than social classes. There are those of us who are sick, afflicted, carried back and forth, lost in some way in our error; there are the relatives, so filled with questions about what is happening to their loved one and what it means for them and how and when and even why; and finally, there are the health care workers, the cream, those who know what they’re doing there; the ones that at some point chose to be there; those who are where they should be and give orders and get paid. Patients tend to be older than the average person; family members tend to look more frightened than the average person; health care workers tend to look busier than the average person. But the health care workers are also divided into well-marked classes: they all dress with their white or light blue pants and loose coats, and maybe their crocs, but the doctors wear their stethoscopes around their necks as epaulettes. More and more doctors are women, but Spanish refuses to admit it: the language still denies them the feminine form that is by now undeniable. We Sudacas — South Americans, more brutish — tend to call female doctors médicas, and Hippocrates still hasn’t risen from his grave: he must be busy with something even more terrible.

“And there’s also fear, the feeling of saying… well, if I’m alone, I fall and I can’t get up, because I don’t have enough strength in my arms any longer. I still have a bit of strength in the right one, but none in the left. I can’t open a bottle of water anymore, I can’t pull down my underwear or pull up my trousers, I can’t shower alone…,” says Marta, from Cuenca, two years after her diagnosis.

Alberto, her husband, sums it up: 

“It’s a bastard of a disease.” 

And she nods: 

“Yes, one day if I’m alone and I have no one, I’ll starve, because I can’t even break off a piece of bread.” 

That’s what they call dependency: learning that you’re useless and wishing to remain so for a long time.

“And all of that creates increasingly more problems. The patient may be in one place and the relative in a completely different one, they sometimes are very out of step, very unbalanced, but it’s what I was telling you: let ALS in your life, in the life of any relative, of any person, and it’s a catastrophe. After four days even the best couples are… well, of course, you have to deal with yourself, your feelings, your emotions, and your own grief, and your relative is dealing with a different grief…” says Tere, the psychologist, “and then I ask for things I never thought I would ask anyone for, and the other must do things they never imagined they’d do, and the other person doesn’t know how to help you either.” 

“Sometimes they want to help you more or help you less than you’d like, or help you in ways you’d rather they didn’t, and sometimes unspoken agreements are made. No one talks about the disease. Or, I don’t tell you I’m not well; the caregiver wonders, How can I say that I’m unwell, that I’m tired, that I haven’t slept, that I can’t take it anymore, that I’m scared, if he’s more frightened than I am, right...?”

“It’s hard to ask someone who little by little is suffering the loss of someone they love — that is, yourself — to cheer up,” I say, though I’m not sure why. 

There are people who travel down the hallways of the hospital — perhaps not yet patients, or patients who hope to remain so for a long time — with a paper in hand searching for a door, a number on a door, a place where they’ll be told what will become of their lives. Some walk slowly, as if they never want to get there. Others hurry, as if they cannot bear so much uncertainty; surely there’s no place in the world where words determine your life so decisively as in hospitals. 

And some stop you, tell you things, try to help you: ‘When the time comes when you can’t talk anymore, there are some excellent communication devices… the government already approved them three years ago, but since health care is decentralized, here, the Community of Madrid hasn’t released the cash yet; so you’re left speechless,” Don Santiago tells me. He is an elderly gentleman whose wife died of ALS six years ago; he has devoted himself ever since to helping patients. Don Santiago is enthusiastic and encouraging, but I can’t stop hearing the prophecy, the certainty of his phrase: “When the moment comes when you can’t speak anymore…” We know but we don’t know, we don’t want to know. 

Even if here, in these hallways, are those of us who can still move somehow — those of us who aren’t connected day and night to a respirator, those of us who still maintain the illusion that we have a body. Or something like one. 

Those of us who don’t know how long we can hold on to it. 

Juan Pedro was diagnosed three years ago, when he was already seventy-five. In his previous life he was a prosperous man, a professional, an athlete, lyric singer during the hours he lost and family man in the hours he gained, and he still rode a bike every day. But when I ask him what he used to do, he responds in the present tense. The good thing about some professions is that you don’t practice them: “I’m an industrial engineer. I’ve worked in many places and with many people working under me…” he tells me with a sad smile, as if it were hard to believe. Because one day he began to feel cramps on the index finger of his right hand, and after months of trial and error, he was told he had ALS: Juan Pedro didn’t know what that was, “so I didn’t think much of it. But I’ve never been afraid of anything, and that’s been my great defence. Fear is no more than an emotion, and I’ve been able to master it.”

I ask him how, and he tells me of a long-ago day when he’d been certain that everything was ending. It isn’t easy for him: the words get tangled in his mouth, they fall apart before they are formed. There comes a moment in the disease where you still speak but you don’t really speak — the desperation of being on the verge of forming words, and then you drop them, you break them. I make an effort to reconstruct them: “I was windsurfing in Tarifa, and suddenly the currents and the wind turned bad. Then I knew it would be my last day, and I was surprised that I wasn’t scared. And I thought it was because fear is tied to uncertainty: since I was certain I’d die, I had no uncertainty, and I wasn’t afraid. And so, I’ve spent over thirty years without fear of anything.”

But moving doesn’t come easy to him. His caretaker, a big Latino in his fifties, pushes his chair, and his wife and daughter accompany him, two blonde, long-lined women. Juan Pedro’s face is very composed, almost severe, almost arrogant. One could say he’s a man who once enjoyed the pleasure of power… and now he has to be carried back and forth.

“And with the disease?”

“No, it’s only affected me physically. Fear is something intangible, a toxic emotion that’s only in our minds and that we must avoid.” I ask him what he regrets the most among the things ALS has taken from him, and he tells me, almost haughtily, that he regrets nothing, that he accepts what he has and that he must survive without letting his physical condition bother him; but a short time later, he tells me he does miss music, singing the way he used to. And he decides to show me that technique still allows him a few flourishes, and he lets out a loud, high-pitched scream. He then smiles at me and tells me to try. I try, and he says no, sharper, this way, and he belts out another one. Then I try to match him with another one, and with another one he shows me: No, and there we are, both of us, screaming at the top of our lungs, when a nurse bursts in to see what’s happening to us. Juan Pedro and I laugh like children. 

 
5.

We’re visible, very visible. We’re the ones who move weirdly, slowly, twistedly, and sometimes someone is pushing us or accompanying us. We’re the ones who are unable to keep doing on our own what we always did. There are many of us, even though we are few, and we might be so different from each other. Some walk with dexterity, others can’t lift a fork; some can’t pronounce a word, others talk your ears off; some need a machine to breathe, others to inject the food that their glottis can’t swallow; some drool, some can’t hold up their heads. There are many of us, even if we are few, and so different; and yet, we all know that we will end up the same. 

(As will all of you, but probably sooner, and without much mercy.)

Even so, coming to the ALS Unit at the Carlos III is an almost pleasant moment. I tell Yoli that it’s a bad joke: in general, when you arrive, they welcome you by name, with smiles and hugs, and they almost convince you that having ALS is a privilege that allows you to join the party, greet old friends, let yourself be fussed over, get to a place where each person wants to look after you in their own way. 

Yoli laughs and I tell her I’m serious.

They manage it. It’s funny, but they do.

“My first boss, Dr Mora, would say that we have to create the best environment possible, because this illness is very hard; patients need to feel at home, to feel that we’re close, that we care for them. We approach the patient and greet them, we hug them… and that’s something that ordinary doctors never do.

Yoli’s name is Yolanda Morán and she can’t recall her first contact with ALS, but she knows, of course, that she has had daily encounters with it for 18 years. 

Yoli is a nurse and very red-haired. She is generally smiling, busy, concerned, caring. 

Two decades ago, Yoli was starting to work in the neurology ward, and Jesús Mora brought her into the unit he was then organizing. Since then, and increasingly more so, Yoli is the red-headed centre of the operation, the one who welcomes and cares for both the newcomers and the regulars, the one that organizes the rounds with the different doctors, the one who always attempts a word of encouragement or hope.

“I love my job. I always say, I’m a nurse first of all, then a woman, then I’m a mother and then I’m a daughter, or I’m… My way of seeing life is the way I think a nurse should see it,” she says, and perhaps what impresses and excites her the most is that intimacy that forms between her and the patient that might not be able to speak, that has to say it all with their eyes, who suffers and doesn’t want to make others suffer, who can look at her “with their soul, you know, and that isn’t always easy to bear.” 

Yoli is also very proud to work in a public service, a place where we are all equal: “I always say that ALS doesn’t care if you have money, if you don’t have money, if you have a high level of education, if you don’t. There aren’t these differences here that can appear in other diseases. When it comes to ALS, we’re all equal; everyone comes here, yes, and that’s very good and very enriching for everyone. For example, Francisco Luzón, the banker, when he was diagnosed, he went to all the most prestigious places and they’d all send him to ours. It doesn’t matter if you’re somebody’s son, somebody’s father, the marquise of who-knows-what, it’s all the same. Everyone ends up here. There’s no one in private care that will do it better because they charge six hundred euros. In that sense, there’s a kind of equity here.” 

It’s true: this unit is a heartening breach of that general rule that says the more you pay, the more you receive. Care is as free, of course, as in any public hospital, but it’s led by a group of people that feel like a group and that feel strongly about their work and that want to do everything that is possible whether there’s money or not. Here, excellence isn’t a question of money. If anything, what makes a difference is finding out it exists, arriving all the way here; for once, information truly matters.

(A brief personal aside: For two years, my treatment consisted of going every three months to a large hospital in Madrid where, after getting lost down infinite hallways, first on foot, then in a wheelchair, a pulmonologist would measure my lung capacity and then, after getting lost again and a long wait, a very kind neurologist would take note of my deterioration. It was like going to a municipal office to say that, in principle, I was still who I was, although a bit less each time. I got tired when, encouraged by the French news of a new medicine, I wrote to that doctor a couple of times to enquire about it and he never answered. I was his patient and I imagined I deserved an answer; there have been few times when my irritation with medical authority have been so well founded. When I was enthusiastically told about the ALS Unit in the Carlos III, I didn’t get my hopes up, until one day I asked for an appointment.)

“It’s a hell of a thing, but I don’t complain that it happened to me. It’s a lottery, so I’ll get through it as best I can. I’ve been upbeat ‘til now, I laugh a lot, I keep busy and the days pass quietly. I can’t complain. From here onward, I don’t know.”

Manuel was an executive at a bicycle factory when he began to feel cramps in one hand, weakness in his arms. It was October 2019; after tests and more tests, in June 2020 in the middle of the pandemic, he was told that what he had was ALS. Manuel was 54 years old and for many months he was able to keep riding a bike and even ski. Now he can’t, and last year he began to have trouble speaking; his son Manolo accompanies him and sometimes translates. In a few days he will have a PEG placed, a tube inserted directly into the stomach, feeding him without passing through the glottis and other passages that don’t work well anymore and could choke and suffocate him. It may seem banal, but choking like that is a typical death for ALS patients.

“It’s very simple, and it’s quality of life. On the other hand, what I don’t want is the tracheostomy, I don’t want to live with respiratory support, I don’t want my family to see me like this and have to bear all of this… I don’t want my family to suffer. Since I had very few symptoms when I was diagnosed, I spent a year without telling them anything; I thought that the later they found out, the happier they would be.”

Patients usually come to the clinic every two or three months. And each time, almost everyone sees us: Tina and Begoña intercept us when we arrive, and Yoli or Pilar send us along to see neurologists Mascías or María Hernández or Francisco Rodriguez or Rafael Martinez, pulmonologist Gómez Mendieta, pulmonary rehab specialist Rosario Urbez or motor rehab specialist Susana Chajma, psychologists Teresa or Saúl, or even María Varela, who is in charge of palliative care. Sometimes they draw our blood for tests, or they give us a vaccine, or they tell us we will have to wear a mask to sleep or use a small tube to feed ourselves. But they all make an effort each time in showing that they want to take care of you. A few months ago, I went for my check-up and, through a passing comment, one of the doctors heard I had an ingrown toenail. She then called the surgeon on call so he could take care of it for me, and in ten minutes they had it sorted. It’s that strange pleasure of seeing people who aren’t in it for the money: they’d be paid the same either way. 

And they really work as a team. They all meet twice a week before they begin seeing patients, and each one discusses details about the patients they’re treating to see how they can approach them. These meetings also serve to support each other, to confront the pain a life so close to death. 

Some patients don’t want to say anything to their loved ones; there are loved ones who don’t want to say anything to their patients. When Mrs. Pilar began to complain too much about pain in her left arm, her children decided to take her to their local hospital. There, they ran tests and more tests on her — months and months of them — until at last they were convinced that she had ALS. The neurologist didn’t tell her but told her family instead — her husband, her children. Then Susana, the eldest, a nurse, thought it best for her mother not to know what she had; they discussed it, they agreed. So, every time they bring their mother to the unit, Susana goes for a few days without sleeping out of fear that a doctor or a nurse or a patient or a poster on the wall will say too much, reveal the secret.

“My mother has had several episodes of depression throughout her life. What we’re afraid of is that if she finds out she has this disease, she will get so depressed that she would lose all the time she has left.”

“And don’t you think she has a right to know, to decide?”

“Sometimes I think yes, I wonder if we aren’t making a mistake. But then I imagine the word ALS comes up, and she searches for it somewhere and finds all those horrible things that appear, and I really am afraid she will fall apart.”

“I think it’s important for the patient to know it, that they have to know it…” says Tina Lázaro, another tireless nurse, another smiling organizer, short-haired, solid, twenty years on the ALS unit. I ask her why. 

“Well, because it’s their life. It’s their life, and each person has to be able to decide for themselves.” 

“Yes, but you could also say that since you won’t be able to decide practically anything anyway….” 

“Or you can. You can decide the life you have left, how to live it, what you will do with it. There are so many things you can decide…”

It’s true: one of the worst traps we patients can fall into is that feeling that there’s nothing else left. There is, for now there is, and it’s worth doing whatever is worth doing with it.

But there are many that don’t want to know. Details tend to be difficult, and we prefer to avoid them. Although, to comfort ourselves, we always insist that each case is different: “Every patient carries their own backpack of ALS, and their ALS is personal; don’t look at what’s out there, each one has their own, no two are the same,” says Elena. But some do want to, they look online for the latest updates, more precise details; others don’t. Elena has already told her husband and children she doesn’t want them to tell her anything, anything at all.

“I don’t know, you see the way some patients live and you say I don’t want that, but how can you know what will happen when you get to that moment. At first when we’d come here, I’d have an awful time, I’d see very sick patients and I would get depressed, anguished. That’s why it’s better not to think of it; whatever comes will come. Maybe tomorrow I will drop dead from something, and look…

“Yeah, I’m sometimes envious of a good heart attack at the right time,” I tell her, and I remember several others that have told me the same: Why find out? Why know, if it will happen to you and there won’t be a way to avoid it? Why then suffer beforehand? 

 
6.

To learn and forget, learn and forget, learn and forget: the disease makes you learn and forget all the time. One of the most odious cruelties of ALS is the obligation to find solutions that are so ephemeral that the illness itself will sweep away with disdain in a few weeks or, with luck, in a few months. One day you realize you can’t lift a glass the way you used to, without thinking about it, and then, with effort, you manage to invent another way — helping yourself with the other hand, bringing your head closer to the table, finding a lighter glass, using a straw… and you manage to solve the problem, only to discover not much later that you can’t do that anymore, either, and then you have to find another solution and sometimes you don’t find it, sometimes you do, and before long it stops working. The disease laughs at you: it forces you to invent methods that you very soon will be unable to use. It forces you into a state where the only constant is the provisional, the road toward what is final. 

(In the large WhatsApp group for the “ALS Family” there are two or three hundred people: patients, relatives, friends — the group is very active: information is offered on doctors or medications or services or devices, a variety of activities and meetings are advertised, words of encouragement and comfort are exchanged. Today, Sunday, a member of the group, Martín, left a very sparse message: Good afternoon family I want you to know my fight against ALS ends this Tuesday a big hug. 

Martín has decided to put an end to it, and dozens and dozens of messages send him strength, love, admiration, and promise him memory. He replies to almost all of them.)

“Unfortunately, I don’t like people very much. Or I do, but just for a while: it’s hard for there to be someone that offers something interesting for more than ten minutes.” 

Roberto and I have the same electric wheelchair, and we chat for a while about its virtues and flaws. 

He moved to Asturias a few months ago, near Oviedo, in the countryside, in a prefabricated house he built with wide spaces where he circulates without problems.

“You know, I’ve had seventy-one well-lived years and I don’t have a single friend: not one. Acquaintances I have many, but friends, none.”

When he was young, Roberto thought he would be a pianist. He graduated with honours from the Madrid Conservatory at a very young age, taught lessons and gave concerts, but gave in to the pressure from his parents to do something “more serious,” and they found him a job in banking. High positions, deals worth millions, a comfortable life, a wife, two children: a very well-organized life that shattered at fifty, when his wife was diagnosed with ALS. He spent a year and a half caring for her with their daughter, until she died.

“To a large extent it was because of love, but also from a sense of guilt. It’s not that her illness was my fault, but I did think that I should have spent less time making money and more time with her.”

But within his self-critique, Roberto says that with ALS people become more selfish and demanding than they really are, and that his wife was so much so that, months after her death, their daughter attempted suicide.

“With my wife it was like that, and then with my sister, when she was diagnosed…”

“With ALS?”

“Yes, my sister died from ALS three years ago,” says Roberto, his grey hair dishevelled, his beard scruffy.

“Your wife, your sister…”

“Yes, and now me. My sister’s and mine are connected, it’s genetic; my wife’s had nothing to do with it. But I’m telling you, both of them, who were strong, generous women, became terribly selfish; they could only think of themselves, they didn’t care even about their children.” 

“Yes, being unable to cure people gives you an emotional overload, it’s tremendously frustrating. But it gives you other things, such as caring for someone at a moment when very few people will care for them, accompany them at a decisive moment.” Maria Varela had planned to become a family doctor — médica, in the feminine — but her path took her elsewhere: she’s been trying for years to help people die better. Maria is around fifty; she is tall and thin and manages to smile even when speaking of these things: “Because we’re going to die, we’re all going to die, and only one in four people dies suddenly. So, we need someone to be there. So, then I say, well, that’s what I want to do, no?”

It’s called “palliative care,” and it was the invention of a Canadian urologist in the sixties who, shocked by “how badly people died in hospitals,” tried to improve it. Since then, with very varied contributions, this branch of medicine grew, aiming not to cure but to care: that, when there is no alternative, we can die better.

“The hospital is prepared to treat illnesses, but three out of every four of us will die in a hospital, and they have to be treated well, they shouldn’t die badly. That’s our goal,” says Maria. The problem is that in the most immediate interpretation, if they send you to “palliative care”: they are telling you there is no cure for you. But even so, the road doesn’t have to be so bad.

 (As always, it’s all relative: for a healthy person, to think of palliative care sounds like hell; for a sick person who is aware of their illness, it’s a comfort and a relief.)

One could think that few people know as much about ALS as Roberto: his wife, his sister, and now himself, and he insists on the selfishness it produces. However, Dr Mascías told me a few days before of something that is like the opposite:

“In my experience, I’ve always been surprised that in general patients with this disease are very agreeable; it’s curious. A few years ago, at an important congress, a presentation titled ‘Why are ALS patients so nice?’ caught my eye. And the study reviewed the behaviour of a number of patients who were being subjected to electromyography tests because ALS was suspected. Electromyography tests are unpleasant, a series of electrical discharges in different parts of the body. And when they finished diagnosing them, they found that the ones that did have ALS had complained much less than those who had other illnesses….

When I tell him, Roberto manages a bitter smile: “Yeah, nice for appearances, but in private… yes, we’re nice. My sister was tremendously nice, and she’d move around in her wheelchair, apparently everything wonderful, and her tendency was ‘I’m very well, I’m very well.’” His sister, he tells me, was a Professor of Law at the University of Salamanca, “very well-known, a formidable person, and she couldn’t stand seeing herself with this deterioration.

“How the hell could you be ok? You’re screwed, hell, it’s a bitch, it’s a bitch of a disease, it’s a bitch. You’re completely lucid observing how you’re falling apart day after day, day after day, without being able to do anything. Not for yourself when you are suffering from it, like in my case, nor for the person you love, as in the case of my wife or my sister. And I’ve got to tell you that she couldn’t stand it: in the end, breathing perfectly well and swallowing perfectly well, she asked for euthanasia, and it was granted.”

But Yoli, who’s almost always optimistic, disagrees:

“This disease brings out things that you never imagined you have. It blows me away. Often, someone new comes in and you think, Wow, this one’s going to have a terrible time of it. And then they suddenly turn a corner; two or three months later you see them unbelievably well adapted, pushing on, rowing forward. And the family tells you, but they were so negative, this was a guy who, when he couldn’t find underwear in his size, would go, ‘This always happens to me,’ and now he comforts and cares for all of us…

Death, in any case, is still there.

7.

We all think of death, we speak of death. It’s logical: it’s been promised to us, and soon. But sometimes I wonder if we really take it seriously: if we really understand that in a few months, in a few years, we will die (The same could be asked, I suppose, about everyone else, all of you, except that here it seems clearer, more urgent.)

“The most brutal condition of ALS is the sentence — the sudden presence of death as something inevitable… In general, we feel immortal, because since you’ve never thought of it… but here you think of it daily, we are conscious of Damocles’ sword; we all have it, but the ALS patient sees it continuously, so they don’t stop thinking of it,” Teresa the psychologist tells me. “And I bring it up with them, sometimes in the first appointment I suggest they write a living will. In that document they ask you if you want a tracheostomy, which isn’t reversible; if you want to be resuscitated, that sort of thing. Let’s say: your life plan. Coitus interruptus. And I tell them that when you start, you think you won’t be able to stand particular things, that when this or that point arrives, you won’t want to keep going; when you can’t move your hands or your legs, when you can’t swallow, when you can’t speak; and then it turns out you adapt: your capacity to adjust, your survival instinct, your will to stay in this world and your fear of what comes after make you endure things you thought you wouldn’t.

“It’s just that no one wants to die. When the euthanasia thing happened, people would tell me, ‘You’ll see, we’ll have so many requests,’ and there were hardly any.”

And yet, before the law, a few years ago, Teresa and the people in the clinic had, among others, a much talked about case. José Antonio Arrabal was an electrician in his fifties who began feeling that his hands weren’t responding. After many fruitless consultations, he arrived at Carlos III, where tests determined that he had ALS. Teresa remembers that after the diagnosis, José Antonio was desperate, he said: “And now with what hands will I work, I’ll have to shut down my company, my wife will have to take care of me, what will we live from?” ALS can be, on top of everything, an economic catastrophe. 

“And a few months later, during one of his visits, in front of his children, he told me, ‘I’m planning to get out of the way,’” Teresa tells me, and she says he explained it very seriously: “‘If I disappear, my wife can find a job and care for my kids and on top of it she would have a widow’s pension, which is more than what they give us now,’ that man told me. 

“His children and his wife were crying but I think that in some way they understood him.”

A few weeks later, José Antonio was sitting on his sofa in his apartment in Alcobendas with a video camera in front of him and a glass that seemed to hold water. He waited until his wife and kids were out so they couldn’t be accused of collaborating in his suicide and end up in prison. His apartment wasn’t adapted for his needs: José Antonio had preferred not to invest in that. At the time, he told a journalist from El País that “After all, it would be for a few months, and I would have had to spend money that instead remains for my family.”

That morning, next to a sheet of paper that said, “Do Not Resuscitate” and another in which he donated his brain to science, José Antonio gave a brief speech: “If you’re watching this video, it means that I’ve succeeded in becoming free…,” he said, to begin with. And he then made an effort to lift the glass with his right hand and drink it down in one gulp. Leaning back on the sofa, he managed to say “Goodbye, everyone,” and he closed his eyes.

His death, which was widely reported at the time, helped the euthanasia law campaign to move forward. And it helped show the differences between the rich sick and the poor sick, crudely, in black and white: how, for many, ALS could be, on top of everything else, financial ruin for the family.

Now another law aims to remedy this. But, as we said: for now that law remains a compendium of good intentions. 

María Varela, the palliative care specialist, has seen many people die: it must be a heavy burden. She says that in any case it doesn’t become normal, that no one ever gets used to watching someone die. I ask her what the most important thing she’s learned has been in all this time of living alongside death.

“Well, look, death itself isn’t the problem. Physically, a person can die well. I believe we can die well. But those of us who provide support, both family and professionals, must make that path as easy as possible. That’s what I’ve learned. That death itself is the simplest part. A person can die well, I think.”

“What does dying well mean?”

“Well, that despite what one could say, that dying must be horrible or I will have a horrible time or all of that, the moment of death can be planned; preparation can allow patients not to suffer as much… That’s it, that path has to be prepared.” 

“But in the end, it’s true that one also holds on to a little bit of that hope that something will happen, that some medicine will appear…,” says Elena, with that voice that’s already trembling, her oh-so-blue eyes moist. “Yes, they have to come up with some medicine, because there are always advances. The problem is when. At some point there will be a cure, of course. But when?”

(It’s part of the cruelty of ALS — telling you that you’ve arrived too early at a death sentence that in a few years will no longer be one.)

So, in short, there are those who for one reason or another resign themselves to death and those who can’t bear the idea of it. There are those kept alive by machines, with a tube feeding them directly into the stomach, another tube in the neck breathing for them, a screen where letter by letter they try to shape the words that a voice will speak for them; and, sometimes, like that, they last a few years. 

“Those people tend to hold on to the hope that a cure will be invented. Many of them want to stay alive because they have faith in science and that the situation can be reversed. If you opt for euthanasia, there’s no remedy,” Elena’s husband Paco tells me. He still doesn’t quite know what he’ll want when the time to decide comes, which has a lot to do with the tracheostomy: to do it or not do it. Manuel doesn’t want to, and he says it with a half-broken voice: “No, I haven’t thought much of death. What scares me isn’t dying; I’m afraid of how I’ll die. One of my sisters-in-law died recently in the hospital, and she was sedated for several days while she lay dying, and you could tell she was suffering, she was finding it hard to breathe; I couldn’t look at her because I was thinking that the same thing could happen to me. That moment scares me, but dying in and of itself doesn’t anymore. That it takes a long time, that it takes time in arriving…. But when I become dependent on the respirator, I think then nothing will make sense anymore. Then I hope they can admit me and sedate me so I don’t feel the anguish of not being able to breathe. And whatever that lasts….

María Varela had told me that the best way of confronting death was planning with the patients the steps they’d be taking, and to think about how they want to die.

“The third stretch of the path is how I want to die. What would I like? And where? In my home, in the hospital… and how? Some people prefer to die asleep, without being conscious — to die in their sleep. There are people who don’t; they don’t want to sleep. There are people who don’t want to die with respiratory support; then we have to sedate them so they don’t suffocate. And almost all of us want to die without pain and without knowing too much about it. In general, that’s what I perceive when I speak to people.” 

It’s striking, in the end, that a few minutes of your life take up so much of it.

“And if it’s fast, all the better. Control that moment, right? The feeling of control also gives us security. Sometimes. Sometimes it doesn’t. There are people who can’t speak of this situation. That’s fine: we’ll accompany them and decide. There are other people who feel secure talking about how to die and where, no?,” Maria says. 

Let it take a long time to arrive, let it take as long as possible; but when it comes, let it be swift: living with ALS is living against the clock. 

Living, more than ever, at the centre of that whirlwind we call time.