Showing posts with label liver disease gist. Show all posts
Showing posts with label liver disease gist. Show all posts

Saturday, October 13, 2012

Into the blue: Girl, 9, with rare liver disease has to sleep under bright lamps



A schoolgirl has to spend up to 12 hours a day under a bright blue ultra violet light to prevent her skin from turning yellow because of a rare liver condition. 

Brianna Minnich, nine, spends each night sleeping under UV lights that help battle a rare disease that can sometimes lead to brain damage.

Her skin and eyes turn yellow whenever she has a sudden change in mood caused by illness, injury, stress, being bullied at school or even arguing with her brother.

Brianna, from Whitehall, Pennsylvania, has Crigler-Najjar syndrome - a condition so rare that only around 200 patients in the world suffer from it.

Her illness means she has dangerously high levels of bilirubin - a naturally occurring waste product from the blood that is usually broken down by an enzyme in the liver - that the UV rays help to lower.

The UV rays from the blue light affect the bilirubin in her system when they come into contact with her red blood cells and skin tissue.

By helping to manage bilirubin levels in her body and keep them at an acceptable level, Brianna is saved from any long term damage.

Currently Brianna has to sleep under a 4ft bank of photo-therapy lights over her be but as she gets older it’s likely she’ll need a liver transplant.

Her father Bob, 29, said: 'Her bilirubin levels were considerably high, right up in the high twenties for the first month or so after being born.

'We were told soon after the diagnosis that she was only the 52nd person in the country to have the disorder and just the 212th in the world.

'In the past three or four years her body has maintained a much more steady level of around 10-15 - the average person has a level of around 0.5 so it’s still very high.
'She was started on Phenobarbital immediately, and used what was called a "bili blanket" which is a small photo-therapy blanket until she grew out of it.

'Whenever she is unwell and whenever she has a sudden change in her mood such as getting upset at her brother or if she gets bullied then her skin tone changes drastically to a much more visible yellow until she calms herself back down.

'She also sleeps under a 4ft long bank of photo-therapy lights every single night, for at least 9 hours a night which typically keeps her skin tone fairly ‘normal’.
'The only thing you can really see is her yellow eyes - people ask us all the time "why are your daughter’s eyes yellow?"'

After being born Brianna underwent tests and was constantly in and out of the hospital for two weeks before finally being transferred to the Children’s Hospital of Philadelphia.

She was eventually diagnosed with Crigler-Najjar syndrome at two-and-a-half weeks old and spent the best part of a month in hospital before being released.

Photo-therapy has helped Brianna but it becomes less successful as she grows up so her dosage of phenobarbital must increase.

The daily medication helps jump start her liver but whenever the dosage is increased it causes her to feel lethargic for weeks and constantly falls asleep at home and school as well as leaving her feeling depressed.

Tuesday, April 3, 2012

Never mind booze – you can get killer liver disease from biscuits


Tony Oakley liked to think he was pretty healthy — he drank only at weekends and went to the gym a couple of times a week.

The last time he’d had time off sick was six years ago, and that was only because he broke his leg.

So imagine his shock when last May he was warned he could be dead within ten years — because of liver disease.

‘I was really, really shocked,’ says Tony, 50, who is married with four grown-up children and works as a customer relations director for a City bank.

‘At the weekend I have a few beers but don’t drink during the week — at the most I have 16 units a week.’ (This is the equivalent of six pints of lager — comfortably under the recommended weekly maximum of 21 units for men.)

‘I had no symptoms either — my liver problem only got picked up because I had a blood test when I got diagnosed with gout in my big toe.

‘I knew I was overweight — I’d got into the habit of snacking at work on biscuits, and got up to 17st (I’m 5ft 10ins) but because I kept fit and was never ill I thought I was fine.’

Like Tony, few people realise it’s not just alcohol that can damage the liver — so can too much food.

He had what is called non-alcoholic fatty liver disease. Most sufferers are only moderate drinkers or may even be teetotal.

‘The risk factors essentially revolve around obesity,’ says Dr Ashley Brown, a liver specialist at St Mary’s Hospital in West London.

‘It’s a bit like pate de foie gras — which is made by force-feeding geese, making their livers swell. A similar thing happens in people.’

As we put on weight, our bodies become less sensitive to insulin. Insulin helps store sugar in the cells but if it is less effective, the sugar gets stored as fat in the liver.

‘This causes inflammation in the same way as too much alcohol does,’ says Dr Brown.

‘Over years this can lead to scarring, and that can lead to cirrhosis.’

As a result, the normally smooth liver becomes full of nodules and hard gristle, meaning it cannot function as normal.

This can have a profound effect, for not only is the liver vital for processing digested food, it clears the body of infections and helps neutralise toxins and drugs. So cirrhosis can be life-threatening.

And, as the British Liver Trust warns, obesity — not alcohol — will become the main cause of liver cirrhosis if current trends continue.

Obesity - not alcohol - will become the main cause of liver cirrhosis

It’s not just cirrhosis: non-alcoholic disease can lead to liver cancer, stroke and other cancers, too.

It’s estimated more than half-a-million people in the UK could soon have non-alcoholic liver disease, although Dr Brown says it may be much more, as it is ‘significantly under-diagnosed’.

In the past ten years the number of hospital admissions has more than doubled to nearly 2,000, and already it causes almost 700 deaths a year.

Last month a report from the National End of Life Care Intelligence Network warned that while deaths from other serious conditions such as cancer and heart disease were falling, those from liver disease have increased over the past decade by a quarter.

‘Non-alcoholic fatty liver disease is already a big problem and one that’s going to get even worse,’ says Dr Phillip Harrison, a liver specialist at the London Liver Centre, London Bridge Hospital.

‘It’s like a juggernaut about to hit us.’ Worryingly, even children are developing it because they’re overweight.

And you don’t need to be obese to have a fatty liver. For some people being just a few stone overweight is enough.

‘There is a genetic element to it: not every overweight person will develop it, and some people don’t need to be obese for this to happen,’ says Dr Brown.

‘Some people who are quite thin but what we call TOFI — thin on the outside, fat on the inside — can get it.

'They’ve been overweight and lost weight but they may still have fat stored round their organs.’
This is because losing weight by cutting calories isn’t enough to shift liver fat — you need to exercise, too.

There are also a tiny minority who get fatty liver disease yet have never been overweight — why this happens is not clear.

As well as obesity, fatty liver disease is linked to high cholesterol, high blood pressure and type-2 diabetes.

‘If you have any of these conditions, your risk of developing it increases,’ says Dr Brown.

Yet while drinkers know they’re putting their livers at risk, those who are overweight don’t, and because the disease is silent with no obvious symptoms, it can go undetected until it is too late. It is usually spotted by chance when people are in their 40s and 50s.

‘Normally it gets picked up when someone is having a general health screening or blood test for another condition,’ says Dr Brown.

It is diagnosed with blood tests to check the levels of enzymes that can indicate inflammation. Scans or even a liver biopsy may also be done.

Often it causes no symptoms other than mild tiredness, says Dr Brown.

‘Whereas heart problems might lead to breathlessness, for example, the liver is like a plodding donkey that carries on without any complaint — until it is too late.

‘And GPs and the public are not particularly switched on about it, as it was only first described about 15 years ago.

‘I work in A&E, too, and I see people whose liver disease has not been picked up who are now in liver failure.

'Or they are referred to me with late-stage liver cancer, and this is the first they have known of their liver disease.’

Tony, from Dartford in Kent, knows he is lucky to have been diagnosed when he was.

‘I’d had a pain in my toe for a while and decided to use the private health cover I get at work,’ he says.

‘I didn’t even know my liver function was being tested, and when the doctor said “one of your liver function tests is not right” I didn’t really take it seriously.’

Soon after, in May 2011, he had more blood tests and an ultrasound.

‘I went back for the results a few days later, and the doctor read me the riot act,’ says Tony.

‘He said my liver was suffering as a direct result of my weight. I had a BMI of about 30, putting me in the obese category.

‘What the doctor said made me realise it was time to do something.’

The good news is that a fatty liver is treatable, says Dr Brown.

‘If you start to lose weight and exercise then the liver can start to heal and the fibrosis or scarring will be broken down faster than it is produced.’

Sometimes weight-control drugs such as orlistat (which reduces the amount of fat the body absorbs) may help. Scientists are also developing ‘anti-fibrotic’ drugs to stop the liver scarring, but these are in the early stages.

After his diagnosis Tony cut out cake, biscuits and foods that quickly break down into sugars, such as white bread, and had brown or wholemeal alternatives.

He went to the gym more regularly and stopped drinking completely for three months. When he next saw his specialist six months later, he had lost more than 1½ stone.

‘This time the tests showed that my liver was fine,’ he says.

‘The doctor was amazed, and I was really chuffed that I’d managed to undo the damage I had done.


 
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