Talking is increasingly difficult for Jackie Thompson, walking impossible. But few people have heard of the degenerative disease which has affected this formerly athletic woman. Now her sister wants to do something about that, she tells Health Editor Barry Nelson.

THE closest Jackie Thompson gets to playing tennis these days is watching it on TV. A keen schoolgirl tennis player, she was forced to give the game up when she reached her early teens because of a cruel, little-known degenerative disease which attacks the nervous system.

Jackie, 29, from Shotley Bridge, County Durham, finds it difficult to even talk now, though her mind is unaffected by the creeping paralysis which means she is now confined to wheelchair.

"I always watch Wimbledon on the telly every year," says Jackie, who struggles to articulate each and every vowel and consonant. She takes a big breath and tries again: "It did make me sad to watch it at first."

After a pause, Jackie manages a smile as she says: "Now we will never know whether I could have been a champion."

Jackie, who now lives in a specially adapted flat in Newcastle, has had to endure the slow degeneration of her condition, making life increasingly difficult. And while she remains positive and has a keen sense of humour, a constant source of irritation for her is the lack of understanding of her rare illness.

Friedreich's Ataxia is very unusual. So unusual that only one or two fans out of an average Newcastle United home gate of 50,000 would have it.

"It is so unusual that we have come across some doctors haven't got a clue about it," says Jackie.

A source of strength and inspiration is her younger sister, Tracy, who has followed in her father, Brian's, footsteps and is a teacher at Delves Lane Infants School near Consett. The two sisters are close and Tracy calls in to see how her big sister is getting on as often as she can.

But Tracy, 25, is also frustrated at the blank looks she gets when she talks about ataxia to anyone other than her immediate family or friends.

"You have to explain all the time. It makes it very difficult because no-one has heard of ataxia," says Tracy.

That's why, when her school broke up for the long summer break, Tracy decided to try to do something to make more people aware. Although she has never done anything like it before, she decided to organise a fund-raising dinner dance. As well as raising money for research into a possible cure for ataxia she hopes the event at Beamish Hall Country House Hotel next month will increase awareness across the region. "It will be great. We are putting on a three course dinner, a raffle, a disco and an all-girl band called Dream On," says Tracy, who has written to many businesses in the Consett area asking for a donation.

Poignantly, one of the Dream On members, Nicola Brown from Great Lumley, County Durham, is the sister of another Friedreich's Ataxia sufferer, part time entertainments organiser Gary Brown.

But sadly - and this may well reflect the low profile ataxia has in the North-East - so far, not a single firm has responded positively to her letters.

Determined to make the event a success, Tracy asked The Northern Echo if it was interested in their story and now hopes that the event will help to put ataxia on the map in the region.

The first signs that all was not well with Jackie happened when she was just 11 years old. Her parents, Brian and Maureen, suspected there was something seriously amiss when Jackie took part in a school egg-and-spoon race.

Although most of the children kept dropping their eggs, 11-year-old Jackie was noticeably much worse than any of her classmates. Shaking, swaying and constantly spilling the egg from her spoon, she came last in the race.

Jackie's parents became increasingly worried about their elder daughter and decided to have her properly checked out by hospital specialists in Newcastle. After a battery of tests lasting more than a year, Jackie's parents were given a positive diagnosis of Friedreich's Ataxia, an incurable, often life-shortening condition.

The shattered couple decided it would be kinder to allow Jackie to have a few more years of carefree childhood without burdening her with the grim news.

Caused by a rogue gene carried by an estimated 500,000 Britons, the condition leads to the gradual paralysis of the victim's arms and legs and seriously affects speech.

While patients usually need a wheelchair, they are normally unaffected intellectually and many sufferers manage to lead full and active lives and live to a ripe old age.

Named after the doctor who was the first to describe the symptoms of this form of ataxia, Friedreich's Ataxia is caused when nerve cells in the body become damaged and eventually die.

According to the website of the main ataxia charity, Ataxia UK, the nerves that are mainly affected are those of the spinal cord and those connecting the spinal cord to other parts of the body, such as arms and legs.

This leads to a range of serious symptoms, usually starting around the age of 15, but sometimes showing in very young children or even in adults.

The early symptoms usually involve poor co-ordination and clumsiness, followed by balance problems when walking. Eventually, speech becomes slurred and legs become so weak that sufferers will need a wheelchair to get around.

Ataxia UK estimates that between one and two in every 50,000 of the population have Friedreich's Ataxia, usually shortened to 'FA'.

The term ataxia literally means 'absence of order' and is associated with co-ordination problems caused by damage to the nervous system.

Ataxia UK will help anyone affected by ataxia but the charity focuses on the so-called 'cerebellar ataxias', which include FA. This group of diseases affects the cerebellum, the area of the brain which coordinates information relating to balance, posture and fine voluntary movement.

Carriers of the faulty FA gene only have one copy of the gene instead of two. If both parents are carriers, the chance of having a child with the condition is one in four and the chance of having a child who carries the gene is one in two. If one parent has FA and the other is a carrier there is a one in two chance of having a child with the condition. A simple blood test will establish whether an individual is a carrier of the FA gene or has the condition.

Apart from wanting to raise the profile of ataxia, Tracy also wants to help the work of scientists who are trying to find a cure for FA. Ataxia UK is also celebrating its 40th anniversary this year, another reason for having the charity dinner.

There is great excitement in ataxia circles at the prospect of developing some form of gene therapy to combat symptoms of the disease. Earlier this year the Ataxian, the magazine of Ataxia UK, reported that experiments involving injecting pieces of genetic material into the brain of mice suffering from ataxia led to much improved coordination.

However, the Ataxian states: "It still remains to be seen whether this technique can be applied successfully to humans. More research is needed to assess its safety and efficacy."

Jackie hopes that many people who had not previously heard of the disease will buy tickets to next month's event. But when asked about the prospect of an imminent cure, Jackie's reply is healthily sceptical: "Don't hold your breath," she says.

l Anyone who wants a £25 ticket for the summer ball at Beamish Hall Country House Hotel, near Stanley, Co Durham, on Friday, September 30, should contact Tracy directly on (07810) 448862.