DCSIMG

Staying strong in the face of tragic illness

LITTLE Katie Hogg is just like any other six-year-old girl. She loves to shower her new baby sister with kisses, she adores her Disney theme bedroom and her new Sleeping Beauty dress makes her eyes light up with joy.

She also throws a major strop when she doesn't get her own way, oozes girly attitude and despises having to brush her teeth.

And in two weeks' time her typical six-year-old excitement levels will hit fever pitch on a trip to Disneyland Paris with her mum, where they'll wave to the characters in the big parade and Katie will wear her special dress and be a princess for a day.

It's a fun-filled jaunt that many families make with their children. But for mum Charlene, the trip will be a poignant and emotional journey that at one stage almost seemed impossible.

For a rare and incurable condition means Katie might not survive long enough to enjoy anything like it ever again.

She is one of only four Scottish children known to suffer Batten disease – a genetic blip that gradually destroys movement, sight and speech. Eventually sufferers become increasingly immobile and mentally impaired, typically falling into a vegetative state before they die.

No-one knows how long Katie may have left, however her form of the condition often means sufferers do not survive into their teens.

"I don't really want to know exactly what stage she is at," says mum Charlene, softly. "The condition causes the brain to shrink, so the neurologist could probably tell us how far things have gone.

"But what's the point in being told? Right now she's on a plateau and that's all that matters."

Today the family's priority is simply getting through the next two weeks without any setbacks – and stepping on board that long awaited flight to Paris. For Charlene knows that even at this stage, Katie's condition could suddenly worsen, throwing their dream of spending precious days together before it's too late, into chaos.

Friends, family and wellwishers had raised more than 3000 to send Katie and her family on the special trip two years ago, only for Katie to suffer a string of setbacks that left her desperately ill in hospital. It's only now, having managed to get through the past year without a single night in hospital, that medics have given her family the go-ahead to make the trip.

"I can't wait," laughs Charlene. "I think I'm more excited than she is. I know it's going to be very emotional for me, but what's important is that Katie enjoys it."

Katie, Charlene's daughter from her previous relationship, was just a year old when the first signs emerged that something wasn't right. "She wasn't progressing as she was supposed to," recalls Charlene. "The health visitor was saying then they were a little concerned.

"I was told there seemed to be some global developmental delay, which basically meant she was a bit slow and that she would need a speech therapist and a physiotherapist,"

"I was devastated. But now I wish that's all that was wrong."

Instead, Katie's degenerative condition was already taking hold. The first of hundreds of terrifying seizures occurred when she was three – a signal of the worrying news to come.

Medics diagnosed Batten disease in July 2006 – a rare genetic condition that affects only a handful of unlucky people every year. Doctors warned Charlene and new fiance Derek Lusk, 22, that Katie's lifespan would be dramatically shortened.

Charlene, who was just 16 when Katie was born, found herself plunged into a medical nightmare. Today she talks knowingly of feeding tubes – Katie can't eat normally and is fed through a tube directly into her stomach four times a day – and the side effects of seizure medication, but it was unknown territory two years ago.

"I had to grow up really quickly," she reflects. "Too quickly really. But you've just got to get on with it.

"There have been long, sleepless, teary nights, but I've got lots of support around me. And right now she's doing well, so I'm grateful for that.

"I try not to dwell on what might happen."

Katie's condition spiralled downwards rapidly. Today she is confined to a wheelchair, cannot speak and is at constant risk of suffering further seizures.

Even the medication to help avoid fits carries the risk of making her ill – liver and blood clotting problems caused by drugs in the wake of another seizure scuppered the last Disney trip and left her in hospital for over two months. Yet despite it all, Katie has thrived at The Hub special needs wing of Sanderson's Wynd Primary School, Tranent, and Elphinstone Primary School, where she is among the most popular pupils in the school.

"Sending Katie to the village school is one of the best things that's happened," says Charlene. "Children used to stare at her because they didn't know what else to do. But now Katie is part of the community and children come over to speak to her and ask to play with her.

"Kids are very accepting, once they know a bit about Katie, they don't mind that she has a tube in her stomach or she's in a wheelchair.

"It's made a huge difference."

Another major difference in the family's life is the arrival of baby Faith – whose name carries a poignant reference to the hope Charlene and fiance Derek have for Katie's future. The couple underwent nail-biting genetic tests to ease concerns that any children they might have together would not be affected by the same condition.

"I'm a carrier, but it turned out that Derek isn't," explains Charlene, "so the chances of any child we had of having this were almost nil. But it's still a worry and the decision to try for a baby was huge.

"To make it harder, Katie wasn't very well when I was in the early stages of pregnancy – so I was having my early scan while she was extremely ill. Then I tried to hide the pregnancy because I didn't want people to know. And there were terrible feelings of guilt – I started to wonder if it was all a big mistake."

To make life even trickier, Faith is lactose intolerant. "She screamed and screamed," recalls Charlene. "So it wasn't easy at first. But now Katie adores her, she loves to give her little kisses on her nose and cuddles her."

Charlene knows, however, that the nature of the condition means Katie is destined to progressively deteriorate. That, says Charlene, means she faces a difficult decision. "I wouldn't want Katie to suffer," she whispers. "I wonder if slowing down the progress of it just slows down the suffering too.

"Every day I see Katie choking and having seizures. There are days she is really well, bright and full of laughs and smiles.

"We take things day by day. You set little targets. One was Easter, then next is Disney, then it's birthdays, Christmas.

"You can't look too far forward," she says with a shrug. "But then, none of us knows what's ahead. . ."

For more information about Batten disease go to www.bdfa-uk.org.uk/

Defective gene lies at the root of the problem

Batten disease was first identified in 1903. It is a rare inherited disorder of the nervous system which can affect children and adults without warning.

The condition occurs when a couple who are both carriers of the defective gene go on to have children. Each of their children faces a one in four chance of developing the condition and a one in two chance of inheriting the defective gene – making them carriers.

Early symptoms include loss of vision or seizures, often accompanied by learning difficulties and clumsiness.

As the condition develops, patients often suffer more severe seizures and loss of motor skills. They often have problems eating, walking and talking.

Victims become blind, bedridden and increasingly mentally impaired until they fall into a vegetative state and die.

There are two main types: infantile, which usually causes victims to die before they reach six years old, and late infantile where they live until they are about eight to 12.

Few of the estimated one in every 150,000 people in the UK with the disease survive into their teens.

 
 
 

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