Multiple system atrophy

The author of the Harry Potter books wants better standards of care for people affected by MS. Her mother died of complications from rapidly progressive MS at the age of 45. The MS Society she refers to is the British MS Society, established through the efforts of National MS Society founder Sylvia Lawry. This article, originally published in Britain in 2001, encouraged protest that resulted in a bold reform, described in Getting Disease-Modifying Drugs in the U.K. on page 32.

My parents were both 18 when they met on a train going from King's Cross, London, to Scotland. My father had recently joined the Navy, my mother was a brand-new WREN; both were on their way to Arbroath, where they were stationed. The way my mother told it, a short, dark youth with a quiff pulled open the compartment door, took a good look around at all the assembled WRENs, strode over and sat down next to her. When she complained of feeling cold (an hour outside London on a summer's day in a nice thick serge uniform) he offered to share his duffel coat with her. From the complacent smirk on my mother's face as she told the story I gathered that things you don't want to associate with your parents might have gone on under that coat.

They were married at 19, though in their engagement photo they look about 12. My father left the Navy, with no regrets on either side, and joined Rolls-Royce in Bristol as an apprentice engineer. A year later my mother gave birth to a fat, blonde baby girl, and two years after that had a skinny, black-haired baby girl--my sister, Di.

Though she had all the Beatles LPs and retained an unhealthy love of cheesecloth all her life, my mother could not have been described as a true child of the Sixties. At 22 she settled down to raise her family and keep house, and I never saw the faintest sign, later in her life, that she regretted not having enjoyed the single life more. She had been brought up in a shambolic home by unhappily married parents, and I think she had a romantic idea of what family life ought to be, and never stopped striving to attain it. Her house was always immaculate, she made it an iron-clad rule that we ate together as a family, and she adored--never stopped adoring--my father.

She was the youngest mother I knew and one of the fittest. She played badminton, she gardened, she swam, she walked the dog for miles; she was the sort of woman who could not sit still until every last bit of ironing was done (a characteristic she completely failed to pass on to her elder daughter, who tended to sit down weakly at the very thought of ironing). When she did stop rushing around, however, it was usually with book in hand. She would read absolutely anything--classics, airport blockbusters, biographies, historical romances, thrillers, whodunits--and so fast that my father refused to believe she hadn't skipped alternate pages. She had one of the most contagious laughs I have ever heard, though you didn't really hear much--she literally doubled up, apparently unable to draw breath, while tears streamed down her face.

I was about 12 when she stopped being able to lift the teapot. She had a touch of rheumatism or a trapped nerve, she said vaguely; her right arm felt numb and weak, "like pins and needles." It was one of those little aches and pains you get used to hearing about, without the slightest tremor of alarm or foreboding. Some days she could lift the teapot, others she couldn't, but she was still zipping through the ironing like a demon, and working as a laboratory technician at my school, still playing the guitar, for God's sake, so obviously it wasn't anything very serious.

She was 34 when the odd pins-and-needles sensation spread up her arm and across the right side of her chest. She decided to see a doctor and was referred to a consultant, who wanted her to go into the hospital for unspecified "tests". When we went to see her in the evening we found her helping the nurses hand out trays of food to the other patients.

The test results came back; she was told that she had a "lack of protein" in her spinal fluid. Nobody interpreted this piece of information for her so she came home and brightly told polite inquirers that her arm was numb due to lack of protein in her spinal fluid.

She was 35, the age I am now, when the numbness and the pins and needles spread so far that she and my father went back to the consultant. He did not bother to send her for tests this time or even examine her. After a few polite nothings he told her calmly that she had multiple sclerosis--an as yet incurable complex disease of the nervous system, which impairs the brain's ability to transmit instructions to the muscles.

They came home and told my sister and me. Di and I went into the kitchen, cried, and swore that we were going to behave like angels from then on, a resolution I think we broke within 48 hours.

At first, life went on much as usual--perhaps too much as usual. My mother made few, if any, concessions to her illness. The casual observer would not have known there was anything wrong; she carried on working fulltime, the house was still spotless; she was determined to meet her self-imposed standards. She was relentlessly cheerful whenever anyone asked about her health.

Her one admission that she had the illness was very much in character; she read everything she could about it. But none of her pamphlets or booklets could tell her why she had the illness or how severely her life would be affected. All they really underlined, as I remember them, was how little was known about the disease. It could affect different parts of the body to widely different degrees. One person might have slightly blurred vision, another might not be able to walk. In most cases the symptoms would increase in number and become more severe over time, requiring ever-increasing provision of care. One odd fact from those booklets stuck fast in my mind (no doubt because Mum was half-Scottish and she and Dad had met and married there): the country with the highest incidence of MS in the world was Scotland.

Her deterioration was slow at first. There were bad spells when her walking became jerky and her general coordination suffered, then she would seem to recover for a while. After a few years, what had once been a bad spell became the norm, and the bad spells grew steadily worse. She was just past 40 when she started using a wheelchair outside the house and 42 when she needed a walking frame inside it.

I have said that little was known about the illness itself, but the medical profession did know that some treatments helped patients manage the symptoms. Adequate and regular provision of physiotherapy (PT in the U.S.) greatly helped people to keep mobile, for instance, when muscles would otherwise seize up or atrophy. It is here that a note of bitterness is going to creep into this article. I would estimate that my mother saw a physiotherapist fewer than 10 times in the l0 years following her diagnosis. She lived in the country and couldn't drive; for a very brief period a physiotherapist came to visit her, but that somehow fell through, and the physiotherapist stopped coming. The same thing happened with the home help who was sent once Mum had given up work and was reduced to crawling upstairs instead of walking. There never seemed to be quite enough money to provide services for people with MS unless they were hospitalized.

She was stubborn. She really needed a chair lift installed in the house but she wouldn't admit it. She tried, in a rather half-hearted way, to follow the dairy-free diet * that had been advised; but as the scope of her weekday life narrowed to within the four walls of the house, and then to the ground floor alone, I would imagine that a bit of milk in her coffee did not seem a dreadful indulgence.

I saw her for the last time just before Christmas 1990. She was extremely thin and looked exhausted. I don't know how I didn't realize how ill she was, except that I had watched her deteriorate for so long that the change, at the time, didn't seem so dramatic. I said goodbye to her and left to spend Christmas with my then boyfriend's parents, the first time I had spent it away from her.

She died on New Year's Eve. When the telephone rang at half past seven in the morning and my boyfriend's mother called up the stairs, "It's your Dad," I knew. Fathers don't call their daughters that early except for the worst of reasons. She was 45 and I still can't write about her without crying.

I need to pause here, not just to blow my nose, but also to state that the rapid spread and severity of my mother's condition was not typical. She had a "galloping" form of the illness. The effects of MS differ widely from person to person and I would hate to think that somebody newly diagnosed with MS is reading this and assuming their illness will follow the same course.

I went to live in Portugal nine months after she died. MS is virtually unknown in the south of Europe; my Portuguese (now ex-) husband didn't understand what had killed her. It was only when I came to live in Edinburgh at the end of 1993 that I started to hear MS mentioned, or read about it again.

Let's fast-forward now--broke, single mother wrote bestseller in cafe--to last year, when I became Patron of the MS Society, Scotland. You might think that I have already explained why I wanted to help, but the fact that the illness took one of the people I loved most is not the full story.

MS, as I have already said, is more likely to strike a Scot than a person of any other nationality. I live in the MS capital of the world. Surely, then, our governing bodies would want to set world standards in research and care? Well--you be the judge.

While research into MS is proceeding in a number of academic and medical centers, most of this is funded by the MS Society. None of the research is funded by the Scottish Parliament and only a tiny amount is paid for by the British government.

While there is still no cure for MS, beta interferon (actually three different drugs) has been licensed for use in Britain for six years, and has recently been joined by glatiramer acetate (Copaxone). These revolutionary drugs slow down the course of the disease for many patients.

In Finland, 15% of the MS population get one of these drugs, in Germany and Italy, 13%, France and Greece, 12%, in both the Irish Republic and Northern Ireland, 8%. And in the U.K.? Between 2 and 3%. You've got a better chance of being prescribed an MS-modifying drug in Turkey.

It would cost around 46 million [pounds sterling] a year to give these drugs to every person with MS in the U.K. who would clinically benefit from them. That's a tiny 0.09% of what the National Health Service will spend this year.

Those with MS who will not benefit from drugs can be taught to minimize the impact on their daily life if given access to professionals such as dieticians, occupational therapists, and physiotherapists. Such services are available only erratically and inadequately across most of the U.K.

In 1999 the MS Society conducted a large survey of people with MS all over Britain, to find out what they thought of the medical and care services available. Some 16,500 people completed the survey. Here are a few of the results: 81% said clinic staff did not discuss the implications of having MS at the time they were diagnosed; 68% were unable to meet a neurologist to discuss their concerns in the month following diagnosis; 74% felt they did not receive adequate support from the health service around the time they were diagnosed; 66% received no advice about how to manage day-to-day activities to reduce impact on their symptoms; 85% had not been advised by their local health service about the range of services it offered to people with MS.

Because of the nature of the disease and the complete inadequacy of the services available, the 85,000 people in Britain who have MS are not the only ones affected. Many care needs are met by members of the family and friends, whose sometimes heroic services are unpaid and unsung.

The MS Society is campaigning to improve these standards of care. One of the quickest and most cost-effective ways of improving care is the designation of specialist nurses. There are only 100 MS nurses across the U.K. Standards of care could be significantly improved overnight, at very small cost, by doubling that number.

Physical aids and adaptations are usually needed. Wheelchairs are the most obvious, but there are hundreds of other aids and adaptations that can make a huge difference to the quality of life. The big ones involve alterations to homes: lifts, doors, showers, hoists, kitchens. With such adaptations, a degree of independence and dignity is possible for the person with MS. Without them--heavy dependence on others and an ever-narrowing life.

MS is progressive--it generally gets worse, so the needs of people increase over time. But are their needs regularly re-assessed? No.

The key specialists involved in getting adaptations are the occupational therapists (OTs). There is an acute shortage of OTs everywhere in Britain. Currently, in Glasgow, there is a waiting time of 29 weeks to get an assessment of needs from an OT. Then there is a long waiting time to find out if the Social Work Department approves the alterations. Then (assuming they do), there is a further long wait until the work is done. It can take up to two years to get a simple modification. By this time for many people with MS, the disease has progressed and their needs have outgrown the adaptation.

The MS Society is the largest organization fighting for the rights of people who have been overlooked for too long. Its help line, pamphlets, and booklets are the main sources of information for most people affected by MS--and for many professionals. Its 370 branches across the U.K. provide activities, support, information, and advice for people with MS, wherever they are. It is by far the largest single source of funds for research into MS. Its welfare grants help give thousands a quality of life, including funding for adaptations and vacations, which would otherwise be impossible. Its specialist respite vacation centers are vital to the continued functioning of thousands of families. Its lobbying and PR have been vital in getting MS onto the political agenda. The Society's work is entirely funded by voluntary contributions.

I miss my mother almost daily, and I feel desperately sad for all she missed. She died before either of her daughters got married, died before Di gave up nursing and became a lawyer; she never met her granddaughter, and I never told her about Harry Potter.

And while she would have preferred me to have cleared away the remains of the take-away in the kitchen before I sat down to write this, I might have got away with it once I pointed out that support for people living with MS is as woefully inadequate today as it was 10 years ago. She would have found that difficult to understand, and so do I.

* While a low-fat diet is advised for general good health, there is no clear medical evidence that any special diet can influence the course of MS.

COPYRIGHT 2002 National Multiple Sclerosis Society
COPYRIGHT 2002 Gale Group