Child Dementia

Reporter: Andrea Burns

Jessica Sheehan should have the world at her feet. But in the cruelest of genetic twists, Jessica's beautiful mind is being stolen. Jessica's only nine. She has a rare condition called Niemann Pick Type C. Childhood dementia. It's terminal.

Parents Trina and Rex were told the diagnosis two years ago. Trina says "people think of kids or adults with diseases , that you've gotta look abnormal but she's not, she's perfectly healthy, beautiful nine year old and she still does as much as what every other kid does" Big sister Charmaine doesn't have the condition - that Jessica does, is diabolical bad luck .

Genes are carried in pairs in all of our bodies - when a child is conceived, each parent passes one gene from every pair, on to the baby. To inherit Niemann Pick Type C, both parents must be carriers, each with one normal gene, and one abnormal gene.

Any baby can inherits one of four combinations.

Two normal genes, in which case the child would be okay.

One normal, one abnormal - here, the child would be a carrier, but would not have the condition.

Or, in Jessica's case, two abnormal genes.

Childhood dementia. Trina says "she can start a book, she can read not even the first chapter and she'll forget what she's already read from the beginning" Sickly as a baby, Jessica saw doctor after doctor.

"When she was 2 they thought she might have had leukemia because all her blood results were always abnormal" Not leukemia, but her spleen was swollen - a key medical clue. The first indicator of Nieman Pick type C IS an enlarged spleen or liver. The cells become clogged by fatty material called lipids and can't process them. Later, they also start to accumulate in the brain, preventing normal function, and eventually affecting speech, memory and coordination.

Dr Lakshmi Nagarajan, Pediatric neurologist at Perth's Princess Margaret Hospital says the condition is terminal because it causes the body's systems to eventually break down, but its progression varies from patient to patient. "There are studies suggesting children that present later in childhood, about half of them will not see their 30's but there are children who present in childhood who are into their 5th decade"

Trina says "pretty hard to take for a kid who was 7 at the time"What did you tell her? "The truth, we told her that she had a rare disease and we told her what it was called and we've just worked with her with the Drs, day by day with her"

Jessica's currently on an experimental drug trial - medication costing one hundred thousand dollars for a year's supply. Not a cure by any stretch, but it has stabilized her, preventing symptoms from getting worse. For her family, giving Jessica an everyday childhood is a difficult task that requires planning and patience. Trina says "she knows her memory is going and she always makes a joke - oh, but I forgot"

Jessica's a bright nine year old, fighting the disease as hard as she can. "How does it make you feel when you forget things? Um sad really" Trina and Rex help Jessica with memory jogs around the house. "And what sorts of things do you do to help you try and remember? I write down a list to help me and does that work? yes"

In year 4 at her local primary school, where mum's also teacher, classmates understand Jessica's not always well, but she's doing her very best. Tossing the coin for her beloved Dockers was one dream... A trip to Sydney is next on her list, thanks to the kindness of strangers. "That's one little dream that we look like we can answer for her, through the Apex and so she's got a lot of other little things that she wants to do and go and see and so"

Medical science is making some advances in treating child dementia, having tried cholesterol drugs, and bone marrow transplants. The next hope is genetic research in animals. Meanwhile, Rex and Trina say they'll make as many memories as they can, and cherish every single moment when their little girl can't. Trina says ""we'll just work day by day and try to keep answering as many of her dreams as we can."Jessica does the disease worry you? no. why not? cos I just forget that it's there"

For Further Details

Apex Web site www.apex.org.au

other Apex Sites www.aatfa.org.au WA Apex started this one over 17yrs ago

www.apexfoundation.org.au

You can visit any West Pac Bank and donate to the "Apex Bunbury Lets Make Dreams Happen" account.

Or post donations to: (a receipt will be sent back).

Bunbury/Koombana Apex Club inc

Po Box 42

Bunbury

WA 6231

David Briggs from the Bunbury Apex club, can be contacted on 0427 971773