Havi Carel: My 10-year death sentence

"So, how long have you got?" The first time I was asked this question, I was dumbstruck. The horror of it, and the casualness with which it was asked, was too incongruous for words. Was it simply curiosity? Ignorance? A clumsy attempt to "connect" with me? What else could motivate someone to ask such a horrific question? Yet, it's a question I have been asked again and again - by friends, acquaintances, even strangers who have seen me sitting in a café with an oxygen cylinder beside my feet.

Once you are ill, I realise, you become fair game. You slide down an implicit social ladder. Others begin to perceive you as weak and unimportant, an object of pity and fascination. In asking: "How long have you got," they compress all their horror, anxiety, pity, and relief that this is someone else's story. How else to explain how people find the obtuseness and cruelty to ask you - in so many words - "When are you going to die?"

I am not impressed. I feel like screaming like that old lady on The Catherine Tate Show: "What a fucking liberty!" To the people who really piss me off, I quote the figure from Wikipedia: five years. I watch them deflate, shoulders sagging, thinking: "How awful. Gosh, I'm glad it's not me."

To others, I provide the official figure: 10 years. I can see them doing the sum in their heads: "Wow, that's really young. I wonder what she feels like, poor thing." To the people I feel deserve more respect, I give a slightly more complex answer, explaining the possibility of medical advance and lung transplantation. To my loved ones, I say that everything is open, we don't know what the future holds, and anyway, no one knows when they are going to die.

Imagine the opposite situation: me approaching one of my colleagues smoking a fag at lunchtime and asking, amicably, in that faux-concerned tone, "So, how are you coping with your mortality? Do you often think about the fact that an average smoker your age has only another 10 years to go?" That wouldn't really cut it, would it.

But somehow, when you are my age (36) and ill with one of the rarest diseases known to mankind (lymphangioleiomyomatosis, or LAM), asking you the most intimate of questions becomes legitimate. The rules change when you are ill. You are, as Erving Goffman put it bluntly, stigmatised. You become an outsider to the world of the healthy, an offensive reminder of the ugly underbelly of life.

Having written as a philosopher on death, years before my diagnosis, I often think about the real meaning of the question. What those people seem to be asking me is: "What is it like to be acutely aware of your mortality? How do you prepare for death? What does helplessness feel like?" They ask because they know that I have, unusually, been given access to a terrible and unwanted vision of my future.

Something has happened to my temporal existence. My future has folded in on itself. It has exposed itself to me, contrary to both the laws of nature and of human nature. We are not meant to be able to see into our future. We are propelled into our future, thrown into our projects with no premonition, no peeking. Our life stories are meant to unravel as we go along, at a rate of one second per second. No slower, and certainly no faster. But I was given a crystal ball through which, with painful accuracy and nauseating detail, I can see my future: further decline, hopeless prospects, premature death.

The Greek philosopher Epicurus argued that the fear of death was irrational. "Where death is, I am no longer," he said, "and where I am, death is not." So long as you are alive, death is nothing to you. And once you are dead, you are no longer there to feel anything, fear included. Life and death are mutually exclusive. If you think about it carefully, Epicurus said, you realise that what you are really afraid of is not death but dying, the pain of illness and decay. There is nothing to fear in death itself, because death is a state of non-existence. It is incoherent to say that you fear not existing. Epicurus thought that those who fear death were confused and should use rational arguments, like the ones he provides, to overcome their fear.

Another philosopher, Martin Heidegger, thought differently. He argued that death is a constant presence in life, and renamed human existence "being towards death". What is human existence, he said, if not a limited stretch from birth to death? Human existence is marked by finitude and limitation, and those of us who ignore this fact are engaged in a futile pursuit, trying to escape the inescapable. So Heidegger rejects the Epicurean idea that death is irrelevant to our lives. To understand life fully, Heidegger argued, one must understand oneself as finite.

Which of these two views is more compelling? In some ways, Epicurus is right. If I do not believe in the persistence of my mind after the death of my body, what have I to fear? I am not afraid of non-existence. But on the other hand, there are other things to regret in death. Like the grief of others, missed opportunities and having to take a different course in life than the one I wanted.

I have always thought about death. In fact, only a month before my diagnosis, my first book was published: Life and Death in Freud and Heidegger. After my diagnosis, I was petrified by the coincidence. The futility of philosophical arguments was suddenly glaringly obvious to me in the face of the practical problems I was facing. Its intellectual approach seemed evasive; its explicit analysis of death, crass.

But after a while, I began to see what I have gained from thinking long and hard about death. It helped me to formulate the questions and, later, develop a view that addresses the issues that are so close to my heart, both professionally and personally. How can I make sense of a finite life? What is the role of finitude within human life? Can a limited life still be a good one? These were questions I had grappled with as a philosopher, long before I had any idea how my own life would turn out. But now, these abstract questions have a real urgency for me.

I need to face these questions in order to forge a way forward. I feel obliged to find a way to remain sane, to enjoy what little time and health I have, and to refuse bitterness and envy, the most tempting vice of all. Moreover, I want not just to be able to accept my illness - for I have no choice, really - but to make my life a good one, with all its limitations and truncated future. With Heidegger, I think that in order to understand my life I must understand it as finite. I see my actions and possibilities as limited and ultimately nested within a finite existence. Perhaps some people - the healthy - can live in accordance with Epicurus's ideas. But where I am, death is. It is a constant presence, a perpetual shadow.

I had been feeling breathless for a while, probably since 2004. But it was only in February 2006 that I went to the doctor. Why did I wait so long? The sad and surprising fact is that we can lose up to 50 per cent of our lung capacity without noticing much change. I felt a little breathless, especially going uphill and climbing stairs, but I thought that was normal.

I was living in Australia at the time, leading an active life, which probably masked my breathlessness, as my muscles were efficient and I wasn't overweight. But sometimes on walks I would see other people climbing hills effortlessly, while I was struggling to breathe. In my kick-boxing class I could do everything as well as the next person, but for the life of me I couldn't skip rope. I bought a skipping rope and practised at home, but nothing happened. I never improved.

By the time we were back in the UK, I could no longer deny that there was something wrong. I went to a specialist who said that it was "unusual asthma". She gave me inhalers and told me to plot my breathing. Over two months, it just got worse. I began to lose weight and got a nasty cold that stayed with me for two months. I'd never had a cold like that. When boxes arrived from Australia, one of them contained my scales. I weighed myself and my heart sank. I was a meagre 48kg, just under 8 stone. There I was, coughing up gack, unable to wear half my clothes, going to work in a taxi because I couldn't cycle, giving lectures sitting down, wrapped in my coat. Yes, I was ill. And yes, I was scared, but I didn't know what of.

In April, I went to visit my parents in Israel. When I arrived, my father, who works in a medical institute, said that he'd arranged a chest CT scan for me, just to make sure everything was OK. I had the scan and went on to see friends in Tel Aviv. I bought sunglasses that afternoon and sat in a café with an old schoolfriend. We talked about Michael Haneke's film Hidden. I wore a lavender jumper that my sister had just given me. Everything was normal.

My parents picked me up from the café. My father suggested that we swing by the medical centre to collect the test results. He parked and walked into the building, while my mum and I waited in the car. Ten, 15, then 20 minutes passed. He was not back. By then I understood something was wrong. I walked to the radiologist's office. The secretary tried to stop me, but I told her my father was in there and I needed to see him.

I entered a dimly lit room. The radiologist was showing my father something on a computer screen. My father was holding his head in his hands. The radiologist looked up, surprised and displeased to see me. "Oh, it's you," he said. "Do you know what's going on?"

"No," I answered. "What is it?" He smiled awkwardly and handed me a big book, a diagnostic manual. He pointed to a word I didn't know: lymphangioleiomyomatosis. "What's that?" I asked. He simply pointed to the text below and said: "Read about what you've got."

Words flashed before my eyes: bullae, pneumothorax, chylous effusion... I didn't understand any of them. I skimmed further down. "Prognosis: 10 years from onset of severe respiratory symptoms." That would make me 45, I thought.

I was suspended in mid-air with one thought only: my life will end in 10 years. I continued sitting there. All the while, the radiologist was speaking to my father and pointing to what I now understood were my lungs. The black balls on the screen were not meant to be there, they were cysts created by LAM cells. I stared at the screen. I stared at the radiologist. I stared at my father. No one looked at me. After what seemed like a very long time, I said the only thing I could think of: "Can someone get my mother?"

Illness changes everything. It changes not only my internal organs, but my relationship to my body, my relationship to others, their relation to me, to my body... In short, illness changes how one is in the world. Moreover, the world of the ill person changes; it transforms into a different landscape, filled with obstacles. Distances increase. It becomes uncanny. The world of the sick belongs to a different universe from that of the healthy, and the interaction between them is clunky, difficult, abrasive.

Stairs used to lead somewhere interesting or important. Now, stairs go nowhere. Offices and rooms on the fourth floor no longer exist for me. Slight inclines have become hills, hills have become mountains. I cycle a circuitous route to work, avoiding a hill. I think twice before going downstairs to collect the post. Groceries now come from the internet, books are no longer carried to and from work. The geography of my world is no longer a shared one. It belongs to me alone, and separates me from the people I walk with. The healthy ones who don't even notice they are healthy.

The future used to be something to look forward to. Now it is something to dread. I know the route my future will take. Nearly all LAM patients decline. I know of women dying in their sleep, going in and out of hospital with collapsed lungs, sitting at home for years waiting for a call from the transplant centre, a call that may never come. I also know of women dying of chronic rejection, weeks, months or years after their transplant. A great wave of helplessness envelops me when I think about the future. I face a dead end, a cul-de-sac, literally: the bottom of the bag.

A few months ago, I went to a conference in Durham. The porter took me to my room: the third floor, of course. He looked at me with a mix of curiosity and pity, a mix I now know well: a young woman with a big rucksack and an oxygen cylinder, looking so independent and yet so disabled. I had to ask him to move me to the ground floor.

It suddenly hit me that I have to tell people, in situations like this, that I am disabled. I still have to take a deep breath before saying this. Being able to engage in projects, to carry out actions, to act freely - we take all that for granted. Our actions define who we are, give us a sense of potency and agency. But being disabled means being unable to do the things you love. Unable to go on long walks, to run, to play with your dog. What kind of being is being disabled, being unable to be?

Illness breaks down the neutrality and transparency of our bodily existence. But it has also given me perspicuity. I observe my life and the lives of others and see them for what they are: brief, full of emotion and agony, activity and joy. I see people arguing over nothing, worrying about wrinkles and careers. Illness makes you immune to that. From the loneliness into which my illness forced me, I became able to see the world anew. My horizons and expectations shifted. I was made aware of the many healthy years I enjoyed. I cherish the things I can still do, like cycling along a canal in summer, the visceral joy of fresh smells and colours.

I learnt to pace myself and rejoice in small pleasures. I became generous towards myself and others; less worried about a looming deadline. I no longer cringe when children ask: "Mummy, what's wrong with that lady?" I am grateful for every moment that is not consumed by sorrow, pain or despair. I try not to think about the future.

I discovered that there can be health within illness, joy within difficult circumstances, and emotional freedom within the adversity of disability. I learnt to be less controlling and more accepting of my inability to do things, my inability to be. Within this inability, I found an ability to be unable, to face myself as unable and to embrace this unable self.

This is not to say that there is nothing that can be done. I have become a patient-activist, working for the LAM Treatment Alliance, trying to push forward research and drug trials. LAM is a disease that has few advocates because it is so rare. That is why I have become my own advocate, like many other women with LAM. We find it hard to accept that the world carries on as before, while we suffer from a terrible disease about which so little is known.

I continue to live as before. Despite the awkwardness and the stares, I still cycle, go to (non-smoking) pubs, do yoga. A pannier with an oxygen cylinder was followed by an electric bike. When people offer help, I no longer refuse it. This is not the life I wanted, but I refuse to indulge in "what if". The first rule I made for myself is never to ask "Why?" Never say, "if only". Never think that things could have been different.

LAM: the facts and figures

Lymphangioleiomyomatosis (sporadic pulmonary LAM) is a rare fatal lung disease that affects women from all racial backgrounds in their childbearing years. There currently is no effective treatment for the disease. In LAM, abnormal cells proliferate in the lungs, lymphatics and blood vessels, ultimately leading to respiratory failure. There are an estimated 250,000 women worldwide suffering from LAM. A lung transplant is sometimes an option if a matched donor can be found, but there is a critical shortage of lungs for transplant.

To support a runner for LAM at the London Marathon go to www.justgiving.com/ kategamez. For more information on LAM in the UK, see www.lamaction.org. For research news, see www.lamtreatmentalliance. org. For the NHS Organ Donor Register, see www.uktransplant.org.uk.

Source: www.lamtreatment alliance.org