Saturday, October 21, 2006
Welcome to Jannyworld
This is a blog about Janny and her changing world.
I'm not Janny - I live with her, care for her - love her - help her. She calls me Dev, and I guess you can, too.
Janny and I met donkeys' years ago when we were still students. It wasn't love at first sight. She wore scruffy jeans and baggy jumpers, she wore no make-up and her hair was all over the place. But then I saw her at a party in a Laura Ashley dress, her hair properly styled, her face radiant, happy, beautiful. And as far as I was concerned, that was that - I was in love.
It took longer to win her over to my point of view. I wasn't exactly the world's most handsome man. Shorter than the average for a start, I had no fashion sense whatsoever and virtually no social graces.
But I could cook. And bake. And so I did.
In those early months, we would talk long into the night about everything, anything and nothing, just for the sheer pleasure of being together. There was nothing more to it than that because Janny still had a boyfriend, a handsome bloke with a big bike. There was no way I could compete, and I settled for companionship even though I yearned for so much more.
It all changed on election night 1979 (like I said, it was donkey's years ago!) when we sat up and listened to the results come in. We held hands; later we kissed.
A few months later, we both graduated. A couple of years later, we married.
Let's fast forward to 1996. I dare say I'll say more about the past at some other time, but right now I need to explain why I've started this blog.
First, Janny fell off her bike. Then she felt a strange snaking motion in her arm when she reached out for something - a cup, say - which she knew wasn't quite right. She went for tests ... and tests ... and more tests. There was no relevant family history to give us any clues, so it was only after several months of blind alleys that we finally learned the truth. A simple blood sample, and two weeks later, the news that Janny had Huntington's Disease. This was a hell of a shock, because we knew of no other member of her family who had Huntington's. As it is a hereditary illness, we should have done. Our best guess is that the faulty gene came down from her mother, who died young - of breast cancer.
Most people get their diagnosis at a much later stage than Janny. It was because Janny was a doctor that she detected symptoms early. Other people would not notice, or would learn to live with the early symptoms. We therefore had several years of genuinely normal family life before Janny's problems started to become more serious. She could drive, walk, eat, look after the children (our youngest was only two in 1996), and recovered enough balance to go back to riding her bike.
Even so, we knew it wouldn't always be like that. Janny knew a lot about Huntington's from her work as a doctor, and I sneaked into Dillons and read the relevant passage in an expensive text book. Later, I learned more by joining the Huntington's Disease Association (www.hda.org.uk).
Frankly, the prospects didn't look all that appealing. Huntington's is an incurable degenerative illness that destroys brain cells. Scientists don't yet know exactly what causes the cells to die, but they do know that a faulty gene produces an excess of a protein (huntingtin) which forms clumps in the nucleus of the cell. From this knowledge, we hope they will one day work out how to control the problem, so that people with Huntington's do not develop the full range of symptoms.
Since you ask, the main symptoms are a loss of control over muscles and damage to the short-term memory. The muscles start to take on a life of their own, jerking and swinging as they see fit, not as a controlled response to a conscious decision to move. This makes it difficult to walk, to write, to pick things up without dropping them, to eat, to swallow, and to talk. The mind speaks, but the lips and tongue don't quite follow. The mouth swallows, but the throat fights back. Choking is a constant hazard. So is falling over; ditto spilling and dropping things.
As I say, the view from 1996 was pretty awful. All the more so when you add in the fact that around 50% of people with Huntington's experience episodes of clinical depression. Janny certainly did - and not just that, but chronic anxiety and panic attacks as well. Maybe I'll tell you about them one day.
Nowadays, Janny takes drugs which limit the involuntary muscular spasms and others which keep the lid on the depression and anxiety. They help a lot, but for now nothing can hold back the rising tide of Huntington's. Over time, the symptoms have become worse, and Janny needs more and more help. She uses a wheelchair outside the house and needs a special diet of mainly soft food, served with sauces of one kind or another and washed down with copious amounts of water from special spouted cups which are like adult versions of toddlers' beakers. Although she wears a bib, food and drink end up all over the place - not least because of a type of explosive cough which she now uses to forestall a choking episode. Anyone sitting directly opposite her at mealtimes is quite likely to be pebbledashed by the time they leave the table.
By now, you may have the impression that our lives have become grim, bordering on the intolerable. But here's the twist: they haven't. In fact, life's great - 75% of the time. The other 25% is scattered fairly randomly through our days and nights, and I will not pretend that they are easy. But the other times truly are happy.
I thought I should share some of our life and times, if only because there must be other people out there for whom 2006 is like our 1996: a year of horrible, nagging doubt about the future. I want to show that life with Huntington's Disease does become more challenging, but it can throw up some delicious surprises and pleasures along the way.
Welcome again, to Jannyworld.
I'm not Janny - I live with her, care for her - love her - help her. She calls me Dev, and I guess you can, too.
Janny and I met donkeys' years ago when we were still students. It wasn't love at first sight. She wore scruffy jeans and baggy jumpers, she wore no make-up and her hair was all over the place. But then I saw her at a party in a Laura Ashley dress, her hair properly styled, her face radiant, happy, beautiful. And as far as I was concerned, that was that - I was in love.
It took longer to win her over to my point of view. I wasn't exactly the world's most handsome man. Shorter than the average for a start, I had no fashion sense whatsoever and virtually no social graces.
But I could cook. And bake. And so I did.
In those early months, we would talk long into the night about everything, anything and nothing, just for the sheer pleasure of being together. There was nothing more to it than that because Janny still had a boyfriend, a handsome bloke with a big bike. There was no way I could compete, and I settled for companionship even though I yearned for so much more.
It all changed on election night 1979 (like I said, it was donkey's years ago!) when we sat up and listened to the results come in. We held hands; later we kissed.
A few months later, we both graduated. A couple of years later, we married.
Let's fast forward to 1996. I dare say I'll say more about the past at some other time, but right now I need to explain why I've started this blog.
First, Janny fell off her bike. Then she felt a strange snaking motion in her arm when she reached out for something - a cup, say - which she knew wasn't quite right. She went for tests ... and tests ... and more tests. There was no relevant family history to give us any clues, so it was only after several months of blind alleys that we finally learned the truth. A simple blood sample, and two weeks later, the news that Janny had Huntington's Disease. This was a hell of a shock, because we knew of no other member of her family who had Huntington's. As it is a hereditary illness, we should have done. Our best guess is that the faulty gene came down from her mother, who died young - of breast cancer.
Most people get their diagnosis at a much later stage than Janny. It was because Janny was a doctor that she detected symptoms early. Other people would not notice, or would learn to live with the early symptoms. We therefore had several years of genuinely normal family life before Janny's problems started to become more serious. She could drive, walk, eat, look after the children (our youngest was only two in 1996), and recovered enough balance to go back to riding her bike.
Even so, we knew it wouldn't always be like that. Janny knew a lot about Huntington's from her work as a doctor, and I sneaked into Dillons and read the relevant passage in an expensive text book. Later, I learned more by joining the Huntington's Disease Association (www.hda.org.uk).
Frankly, the prospects didn't look all that appealing. Huntington's is an incurable degenerative illness that destroys brain cells. Scientists don't yet know exactly what causes the cells to die, but they do know that a faulty gene produces an excess of a protein (huntingtin) which forms clumps in the nucleus of the cell. From this knowledge, we hope they will one day work out how to control the problem, so that people with Huntington's do not develop the full range of symptoms.
Since you ask, the main symptoms are a loss of control over muscles and damage to the short-term memory. The muscles start to take on a life of their own, jerking and swinging as they see fit, not as a controlled response to a conscious decision to move. This makes it difficult to walk, to write, to pick things up without dropping them, to eat, to swallow, and to talk. The mind speaks, but the lips and tongue don't quite follow. The mouth swallows, but the throat fights back. Choking is a constant hazard. So is falling over; ditto spilling and dropping things.
As I say, the view from 1996 was pretty awful. All the more so when you add in the fact that around 50% of people with Huntington's experience episodes of clinical depression. Janny certainly did - and not just that, but chronic anxiety and panic attacks as well. Maybe I'll tell you about them one day.
Nowadays, Janny takes drugs which limit the involuntary muscular spasms and others which keep the lid on the depression and anxiety. They help a lot, but for now nothing can hold back the rising tide of Huntington's. Over time, the symptoms have become worse, and Janny needs more and more help. She uses a wheelchair outside the house and needs a special diet of mainly soft food, served with sauces of one kind or another and washed down with copious amounts of water from special spouted cups which are like adult versions of toddlers' beakers. Although she wears a bib, food and drink end up all over the place - not least because of a type of explosive cough which she now uses to forestall a choking episode. Anyone sitting directly opposite her at mealtimes is quite likely to be pebbledashed by the time they leave the table.
By now, you may have the impression that our lives have become grim, bordering on the intolerable. But here's the twist: they haven't. In fact, life's great - 75% of the time. The other 25% is scattered fairly randomly through our days and nights, and I will not pretend that they are easy. But the other times truly are happy.
I thought I should share some of our life and times, if only because there must be other people out there for whom 2006 is like our 1996: a year of horrible, nagging doubt about the future. I want to show that life with Huntington's Disease does become more challenging, but it can throw up some delicious surprises and pleasures along the way.
Welcome again, to Jannyworld.
