My disease story
All started with a slight limp in September 1995. As it was not painful and I had plenty of teaching that semester, I only consulted a neurologist in December, taking advantage of a transport strike which was to last a whole month, and that caused a break in teaching at my university. The neurologist noticed an anomaly and recommended I should be hospitalized for more exams.
So I stayed a week at the hospital, but they did not find anything. The only thing I remember of that horrible week is a lumbar puncture which was traumatic. My daughter Marie, a neuropediatrician, warned the doctor that my spine was stiffened by an important osteoarthritis ; she replied that she would do it herself and that I had nothing to fear. When she arrived the next day, she was with a student obviously in charge of the lumbar puncture. I protested, but he did it, saying he was used to do them. He tried three times, and finally the doctor decided to do it herself.
After the puncture, I stayed in bed as long as recommended before going back home. My husband, in a traffic jam caused by those transport strikes, arrived late in the evening, and my back was already very painful. Coming home was hell. Back home, the only possible position was to tuck my legs under me, towards the ceiling. I stayed in that position all night. When I fell because I was asleep, pain would quickly wake me up. I suffered of rachidian liquid effusion, due to the puncture. My brother, also neuropediatrician, brought me back to hospital the next afternoon, where they did a "blood patch", which is taking blood out of my arm and then, introducing it at the bottom of my spine, in order to plug effusion. This "blood patch" immediately relieved me.
Later on, I learned that this problem never happens if you prick with a thin needle, but of course, thin needles are hard to enter, so you do not learn with thin ones. I also learned that in a disease like an ALS, brutal therapies are to be banned. Well done, doctor !
At the beginning of January, my brother came home to examine me, and he found I was doing "fasciculations", which are sorts of waves you do on your thighs when you start an ALS. Those fasciculations had not been detected by the physician in hospital, who wanted to undertake a very painful exam. Thanks to my brother, I avoided that exam. It is only on January 8th that I knew the name of my disease : an "Amyotrophic Lateral Sclerosis". Physician A... came in my bedroom, shared with an old lady, in company of the doctor who did the puncture and her student, to tell me what would be, in the future, a real tragedy. I found it unscrupulous, to come this way, and would have preferred being alone with him, in his office, for such terrible news. He explained that it was an accelerated aging. The word "Sclerosis" is hard to hear, but "accelerated aging" is absolutely unbearable. He promised there was now a miracle medicine, but I quickly understood he was laughing at me.
For diagnosis confirmation, I went for a few days in the hospital in Paris, called « La Salpétrière », specialised in all neurologic problems. I quickly understood my days were numbered, for the social worker to whom I asked the procedure to obtain a disability advertising sticker for my car, looked at me with such surprised eyes, saying that I had to wait at least three months before having it ! in other words, by that time I would be dead. Worst was the psychologist who recommended I should make peace with the people with whom I could have problems, for she said, when I die, it would be too hard for them. The message could not be clearer !
I was integrated in the "rilutek" protocol, but I quickly understood that this miracle medicine had no effect on ALS. So I rapidly gave up.
Weakness is the word which can characterise that 1996 year. I wanted to finish the teaching of that academic year. Luckily Bernard, my husband, mathematician in another university, substituted for me when I was too weak.
At Easter, to celebrate our 25 years of married life, we went to Egypt with our children, the last family travel. We took a wheelchair, lent for the occasion, so I could do all the visits. Marvellous recollection !
In June, I realised that teaching was over, I was too weak to continue. My writing on the blackboard was unreadable, I kept loosing my balance, and my fear was to fall in front of my students. Driving became dangerous, for I could not turn my head to check if someone was coming on my left. All the last term, my husband drove me to the university and back.
It happened that the year before, I had offered Jacqueline, in charge of the first academic cycle, to help her with the timetables, an enormous, complicated and very boring work, that she did alone, for she never found someone to do them. It was not my ALS that would stop me from doing that work, which could be done sitting near a telephone and a computer. So Jacqueline told me about what I had to do. Without knowing it, she saved my life. Another person who saved my life is the one in charge of the mathematical department, for understanding that I could not teach any more, she accepted to consider timetables work as an entire teaching service, and she lent me an old computer and a modem, so that I could do this work at home by e-mail. I am very grateful.
My walking became worse and worse, all along summer 1996, and only on crutches. My writing was unreadable. Walking and writing were too tiring. In October I was longing for the time I would not walk any more and would simply sit in a wheelchair ; it came quickly. I fell down very roughly in my flat, and a month later it became impossible for me to walk. At the same time, my hands became weaker and weaker, I could not knit, but I could still turn the pages of a book, but not for long.
As I could not bring the phone to my ear, Bernard brought me a telephone with a speaker, so that I could do the timetables of the second academic semester, with Bernard typing my e-mails, which was now impossible for me. People were surprised to hear me on the phone, for I had a funny nasal voice, but still quite understandable ; some thought I was mentally retarded.
In January 1997, a year after I had been diagnosed, I could not walk, I could not sit without falling on one side or the other, and I could not control my hands. I was fed and Bernard had to wash and dress me. I could not turn the pages of a book, so the ALS association in Paris, lent me an electric page-turner, but I never managed to make it work, my hands were too weak. So the only occupation I had, was to listen music, lying on my bed ; and that is what I did, every day, from January to June.
Two events changed my life in June 1997. The first was the purchase of a very comfortable wheelchair, seen at a disability exposition and presented to us in a hospital, which could tip-up and had an antimacassar. Those wheelchairs are very expensive, but happily the mathematical department of my university had made a collection at my intention, that was nearly the price of the wheelchair. Thanks to them, I could now sit out of my bed.
The second event was a visit at one of the AFM association departments, in Evry (very near Paris), called the DRAC. They have plenty of money, because of a charity event done on television in December, called "Telethon". Most of this money goes to medical research, but a small part is for children suffering from myopathy. They give them computers, essentially for games, but also to be able to write, and for the elders to go on internet. We were welcome. It was a young lady suffering from myopathy, who explained to me how the access to computer software used by the DRAC works. This software was Discover, at that time. It puts itself before every other software, and it is totally opened, so you can do your own switch keyboards, according to your needs. The DRAC lent me the software and a blowing switch. Unfortunately the computer lent by my university was too old and not powerful enough. So the head of the DRAC lent me one for the summer ; I can say, she also saved my life.
We brought this computer with us on holiday, and I had two months to get to know how Discover worked. I was nearly giving up the timetables, for as I could not write, I needed someone who would take down my dictation, which was becoming very difficult because my speech was now incomprehensible. When I realised that with Discover, I could do the timetables on my own, I was delighted, and more, I felt saved, because those timetables had to be done. I had to change switch, because I salivated too much for the blowing one. Bernard found a tongue for the cheek that I kept until September 2001. I spent all summer on that marvellous toy !
Back to Paris in September, Bernard bought me the same computer and we gave back theirs to the DRAC. My life was taking another shape. I was a dead weight, an every day and permanent charge, without possibly to give anything in exchange, and now I became a mother again who could write to her children, who could manage her bank account, who commanded groceries and did her shopping by Internet, etc... I even had a real professionnal activity.
Morally, I was much better, but physically much worse. When I was eating, I kept on choking ; sometimes meals would last two hours, which was unbearable for whom made me eat. In January 1998, they put me a feeding tube, and since then, I do not eat. Not eating any more caused me a very bad taste in the mouth which is worse nowadays. I tried all sorts of treatments to get rid of it, but none ever worked. A few months after that bad taste appeared, my jaw blocked up, leaving me no hope to eat again some day. I learned later on, that it is the brains that send the jaw the order, to stay in that closed position. As if, by fear that food with bad taste was introduced into my mouth, it preferred not receiving any food at all. Yet in the opposite, the bad taste comes from the stomach, I believe gastric juices come up, into my mouth. As I am conscious of it, Freud is once again disavowed !
During all this first term of 1998, I did Discover panels, with one main idea : speeding up writing. Someone told me about a small software for secretaries, TypeIt4me, which does abbreviations. You enter in the software a word or a group of words and its abbreviation, and then when you type the letters abbreviation and a space, it restores the word or group of words. So I introduced all sorts of words following logical rules, so as to find them back. Unfortunately this software is too small to build a real accelerating writing method, for you cannot introduce more than 2700 abbreviations.
As my speaking became incomprehensible, I made, with the help of a speech therapist, a language where each phoneme had a sign made with my eyes and eyelids. That language turned out to be the best way to communicate with anybody who would learn it, when I was not at my computer. With the people who do not know that language, I have a range of phonemes made out of cardboard.
In July 1998, I had a serious phlebitis and food regurgitated into my mouth more and more. We were on holidays, in our house in the Jura. The emergency physician of the nearest hospital to whom Bernard phoned, said that if I came, he would give me a blood test, and if he found it necessary, he would not let me go without doing me a tracheotomy. I got scared, and never went to his consultation. It was a mistake, for I had that tracheotomy in January (1999) after enormous sufferings, which could have been avoided if I had accepted it earlier. But nobody ever told me reflux was a sign of respiratory failure, apart from this emergency physician whom I did not want to believe.
Back home, they gave me a treatment for the phlebitis, but I still had that enormous reflux against which they never found anything to do.
During all 1998 last term, my capacity to work gradually decreased. In December, I could not work more than ten minutes, just the time to write a few words for Bernard, as speaking was impossible. I did not know what was happening with me, and as my neck was becoming too weak to support my head, I thought it was the reason why I could not activate scanning. At the end of December, I felt always too hot, and needed the window open though it was freezing outdoors. I slept very badly. It is only in January that I understood that I was missing air. In the middle of January (1999), I spent a week in the pneumology department of my hospital, to try a mask joined to a ventilator, with the idea that it would be enough to have it just at night. As I knew that if it did not work, it would end up with a tracheotomy or in the cemetery, I put all my energy so that it would work. But how can you imagine putting a mask on someone who has no hands to take it off, and no possibility to ask for help when he is suffocating. The test failed completely.
So I had to choose between tracheotomy and cemetery. Being a dead weight for my family and specially for Bernard, my husband, was unbearable. The physician in charge of me, clearly said that if I chose tracheotomy, I could go on working, that life would be possible again, but that nobody would ever accept to stop the ventilator, even if life became insufferable. The decision was impossible to take, but in fact Bernard had taken it for me. He always passionately tried to make me live. I do not know if it is will to live, but I let him decide. The operation was done under local anaesthetic and lasted ten minutes. I had it at the end of January 1999. Bernard stayed with me all the time, day and night, except when he had to go to the university for his teaching. Staying with me, was very kind, as I could not speak, thus could not communicate with the nurses.
This tracheotomy, which frightened me so much, was a real relief. Again I could work all day, which is very important for me, otherwise I am very unhappy. Apart from the problem of my head that was becoming too heavy for my neck, the disease stopped its evolution. But nights were still hard, because of sucking up problems and often ankylosis problems.
At the beginning, I feared not to be able to call when I needed something, for with tracheotomy I was now unable to do those sorts of growlings that helped me to call. But quickly, one of my brothers made an alarm that Bernard puts on my wheelchair. I activate it with light movements that I can still do with my legs.
In January 2001, they operated me a second time to make the tracheotomy orifice bigger. This time I was totally anaesthetized, and got to wake up in an observation ward, which was real hell. Bernard had asked to be with me when I woke up, for he knew that if I needed something, as I cannot speak, it would be impossible for me to ask. As they refused, he warned them that my head should always be upwards, because of my enormous salivation that could choke me. Of course they replied their usual : "we are used to it". And what happened ? They left me with the head tilted backwards all the time, and for two hours I had to swallow my saliva, in order not to choke. They put my feet under a horribly heavy warning blanket, which was very painful. Bernard, waiting outdoors, and very impatient, asked how I was, to someone coming out of the observation ward, and she replied that I was very calm ! Of course Madam, if you were so used to paralysed persons who cannot even send out a whisper, you should know that they seem obviously calm ! Nevertheless this operation was useless, for changing cannula was still as painful.
Unlike what I thought, tracheotomy did not stop me going out nor travelling. I went three times to the opera, and we kept on going on holidays normally. At the beginning, it was a real move : two ventilators, two aspirators, all my food for two months, the anti-bedsore mattress, my toilet chair, my wheelchair, the computer, etc... All that needed several trunk-fulls, and all the family used to help us going to our country house, 500 kilometres from Paris ! In June 2000, we bought a sort of small van in which Bernard can rise my wheelchair, with the help of two rails forming an inclined plane. The wheelchair is fastened to the van, so I can travel in it. The wheelchair takes the place of three people, so only the driver and another person can travel with me. With this van, we can go and visit brothers and sisters in all parts of France, go to weddings, and as Bernard is now retired, we stay in our country house, in the Jura, a great part of the year. We had this house arranged for me, with a bedroom downstairs, at the same level than outdoors, and with a bathroom next to it. In the bathroom, there is a special shower, so that Bernard can wash me entirely. In that house, I am much more comfortable than in Paris.
For tracheotomy care, we took a nurse when I came out of hospital. But nurses are very difficult to find for those sorts of cares, which are long, meticulous and badly paid. Back from holidays, in September, our nurse said she could not go on with me. We tried to find someone else, but never succeeded, so it is Bernard who does those cares, plus toilet and dressing. He says it is nothing compared to the strain of nights and pressures, he cannot cope with.
Since March 2000, my neck kept on loosing its physical power, and it became impossible to get the cheek switch moving. I corrected that weakness by little movements of all my body, which were possible because certain of my thigh muscles still work. These same muscles let me lift up my legs a little, so that I can reach an alarm that Bernard puts on my wheelchair ; they also let me stiffen them up, when I am taken off my wheelchair ; without that possibility, life would be much more difficult.
As I could not get the cheek switch moving any more, Bernard contacted a team in Marseille that advocates a fluttering eyelashes switch, which consists in putting an optical fibre on glasses, sensible to all eyelid movements. Unfortunately, I have no control on my eyelid movements, sometimes I cannot open my eyes, and other times I do not succeed to shut them. So the experience failed, and I took back my cheek switch.
That cheek switch was becoming so difficult to handle, that writing was too tiring and I was not able to do the timetables any more, so I gave them up in summer 2001. I spent my days on Internet and reading books downloaded on internet, for books on computer have a great advantage, no need to have someone to turn pages.
One day, Bernard showed me a photo of a man with a switch on his eyebrow. We bought the same muscular switch and I tried it on my eyebrow. Disastrous result. To the movements I tried to do with my eyebrows, were added uncontrolled ones, so it was impossible to write without making an enormous amount of smudges. Once again, I took back my cheek switch.
One day, in September 2001, the switch strip was at such a place on my cheek, that without moving all my body, I could nearly have it moving with little jaw movements. I hastened Bernard to mark with a felt pen, the spot on my cheek, and then to put the muscular switch on that exact spot. It was a real discovery, once again, I could do everything with the computer, and once again I could write all day, without feeling tired immediately. Once again, I could go full speed, I was delighted. I even offered to do the timetables again, but under certain conditions, as those conditions were not accepted, and as I was already retired, I stopped them.
Apple has changed it's operating system for a few years now. Mac OS X is based on UNIX language. Gradually all softwares only offered Mac OS X versions, and it became impossible to find softwares working with the classical Mac OS 9 operating system. I wrote to Discover several times, asking when they would adapt Discover to OS X ; at the beginning, they replied it was a question of a few months, but later on, they did not even reply. So I asked the association that had let me know Discover, and I was told that for Discover, I had to wait, but that recently a new scrolling software, named SwitchXS, had appeared. I bought it in July 2003, and proposed myself as tester, which turned out to be very interesting, for David Niemeijer, the author, is a very open-minded person, seizing any new idea he receives. SwitchXS turned out to be much better than Discover, and I was now using OS X system, which makes life easier. I go on nowadays, doing my job of tester, through chats, when David is online.
In April 2005, once again, like in January 1999, I was suffocating in the afternoon. I had to have the window open, though it was freezing outside. My blood test was perfect, no need for extra oxygen. My lung specialist, who is well informed on ALS, nevertheless gave me extra oxygen, and it turned out to be very effective. I ask it when I need it, generally in the evening. It is a machine that extracts the oxygen out of air, and puts it in the ventilator. The only problem, is that it needs electricity, so it cannot work when travelling. Coming back from Nantes, in June, after our daughter's wedding, I suffocated so much, that we had to stop at each highway rest area, to branch the oxygen extractor. As soon as we left the rest area, I started suffocating again. Horrible travel ! now we always have a cylinder of compressed oxygen in the van. And nowadays, in 2007, I need oxygen, nearly all afternoons.
In France, one is very well taken care of, but only on the strictly medical level. Home ventilation is covered, and all appliances, such as ventilators, aspirators, oxygen extractors, and even feeding bags are entirely free. It is an enormous advantage, to which I owe to still be alive.
Yet a crucial problem remains, which does not seem
to have been sufficiently taken into account. It is the problem of home guards
for severely handicapped and totally dependent persons, like I am. Nurse,
physical therapy and speech therapy fees are entirely repaid, but home guards
are left to family care, and in my case, it means to my husband and my children.
As my children have too much to do, it is Bernard who is in charge of me,
day and night. When he had his teaching, he asked one of our children to
keep me. This led him to be so exhausted, that you would think he was a living
corpse. Now that he is retired, he feels better, but it is very hard to be
stuck indoors and never go out. Hundreds of times, I told him, he should
put me in a home, but he does not want to hear about it. For me, it is real
suffering, having to depend on my family this way ; I cannot stay ten minutes
alone without a risk of suffocation. Authorized day-care workers would cost
us more than we earn. We tried several times to have one at our own expense,
but they all failed, because of my difficulty to communicate. What
can we do? We battled against that horrible disease, but finally our success
condemns us. Bernard has stopped all his activities to stay with me, the
children who helped us a lot, are fed up, which is understandable ; life
which we restored, is each day more difficult. Each day is a battle.