This Is How I Liver

Ashleigh has become rather ill and she now needs a liver transplant as the doctors believe this will improve her quality of life and have a longer life expectancy . This will be held in Auckland Hospital (roughly a 2 hour flight from her home town)

Proceeds donated will go towards helping her family with some financial costs when Ashleigh is staying in Auckland over the three months . The ADHB (Auckland District Health Board) covers flights and accommodation costs for Ashleigh and one support person only , and although we are very grateful for them to do so we are seeking donations for Ashleigh's immediate family and partner to be up by her side . Unfortunatly we do not know when a liver will arise as Ashleigh is to have a whole liver therefore has to unfortunatly be a deceased donor which a match could arrive at any time weather its 3 months or 3 years , and booking flights last minnuite can be quite expensive along with accomodation cost etc all whilst still paying mortages and bills back home . So a little help financially will be hugely appreciated .

Ashleigh also wants to write and publish a book about her journey and to reach out to others whom are in a similar situation and to help raise awareness . Unfortunatly writing and publishing a book can be very expensive also and due to her health issues Ashleigh can not work nor study therefore can not fund this herself . Remaining proceeds will go towards Ashleigh's dream to get her book out there .

Every little bit helps and is much appreciated .

For those of you who know Ashleigh will understand how much she has gone through and know just how much she deserves this .

She would not wish this condition on anyone.

However as much as she despites the disease, she would not change a thing that has happened in her life as it has made her who she is today.

About Ashleigh's rare disease:

Ashleigh was diagnosed with a rare liver disorder called Glycogen Storage Disease - type 1b when she was 3-months-old.

Less than 20 people in New Zealand suffer from GSD and it only occurs once in every 100,000 births. This disease is genetic and caused by both the mother and father carrying this rare gene. They don't need to be suffering from this disease to be a carrier. If they were to have another child it is a 1 in 4 chance that child will have it too.

The main function of a person's liver is to make and store fuel from the carbohydrates they eat such as rice and potatoes. Some of the glucose produced travels into the blood and the rest into the body where it is used for energy.

The liver of a GSD type 1 sufferer does not do this function. They are missing the enzyme that allows this process to happen.

The defiency impairs the ability of the liver to produce free glucose from glycogen. Since these are the two main metabolic mechanisms by which the liver supplies glucose to the rest of the body during fasting, it causes severe hyperglycaemia and results an increase of glycogen in the liver or kidneys leading to enlargement of both.

Both organs typically function normal in childhood but are susceptible to a variety of other problems in adult years.

Frequent feedings of corn-starch or other carbohydrates is the main treatment. Ashleigh has to take 80 grams of cornflour every 5 hours, she has never had a full night's sleep. Corn-starch contains the highest amount of carbohydrate (1Tbsp contains 9 grams of carbohydrates) and when taken raw it is hard for the body to digest therefore giving her slow releases of energy.

Ashleigh goes through approximately eight boxes of cornflour a week and over her life time she has consumed about 1328kgs of it. To visualise this, it is estimated to be about 17 average size bath tubs packed full of cornflour! How can anyone take this disease seriously when her medicine is a gravy thickener brought at your local supermarket?

The main danger of not taking corn-starch is hypoglycaemia. It is the central problem and the one that is most damaging. Any seizure can cause severe brain damage or even worse, death. Having chronic low blood sugar scan lead to develop tumours in the liver called adenomas, typically becoming cancerous if not dealt with fast enough.

Having GSD comes with other problems such as growth failure, joint problems (gout), kidney effects, pancreatitis, bowel effects, high infection risk and blood clotting problems.

Without treatment, growth failure is common due to chronically low insulin levels. Intestinal involvement can cause mild malabsorption. Ashleigh was also diagnosed with Crohns Disease at the age of 16 due to this and suffers all of the above also.

Neutropenia is a manifestation of this disease. She has a very low white blood cell count , and even though she is on daily injections to boost her immune system she still picks up infections and viruses easy. Without adequate metabolic treatment, patients with GSD type 1 have not made it to adulthood and up until 1971 this disease was completely fatal.

There is no cure ! Doctors in medical school don't get taught about GSD and gets a mention in passing at best.