Sarah's home away from home. Hospital bed where she has spent much of her 20s. Photo / Supplied
Sarah's home away from home. Hospital bed where she has spent much of her 20s. Photo / Supplied
Sarah Grant is facing the biggest battle of her life - the fight to stay alive.
In 2008, the then 21-year-old discovered she was carrying an ultra-rare genetic disease after she was diagnosed with the life threatening condition Atypical hemolytic-uremic syndrome (aHUS).
The disease acutely damages major organs like the kidneys, liver, heart and brain due to blood clots in small blood vessels, and can cause death through strokes, kidney failure, heart attacks - or drowning.
aHUS patients go through peaks and troughs, and is often described as a "sleeping dragon" to those with the condition.
Some days, Sarah is OK. But other times, when the condition "flares up", that's when the problems start.
Sarah, now 29, has spent the past eight years in and out of intensive care, and tied to a dialysis machine to keep her alive.
While she still has both the kidneys she was born with, they don't function - so she is forced to go through a blood washing dialysis 15 hours each week.
The painful process, which involves "washing and filtering of the blood" eliminates excess fluids the kidney would usually dispose of on a daily basis in a healthy person.
"If I have anything to drink, because I don't have kidneys, I will hold it all in my body," she said.
"They put two needles in my arms, one takes the blood out and goes through lots of different chemicals to clean it, then pumps it back into me.
"This will happen over and over again for five hours. I come out incredibly tired, and have a migraine the next day."
If Sarah didn't have dialysis, she would die from fluid overload.
"Liquid would go around my heart and cause pressure so it wouldn't beat properly," she said.
"If I didn't have dialysis, it would take maybe two weeks for me to go. And that's if I hardly ate or drank."
Unable to work, spend much time with friends or live a regular 20-something lifestyle, Sarah "lives in fear" of dying every day.
"If my heart beats funny, I panic because I think something bad is about to happen," she said.
"I'll sit and stay up at night, and during the day I make sure I don't drink too much because it may damage me even more. It's terrifying."
The obvious treatment for Sarah would be to replace her kidney - a transplant operation which she has tried once before.
In 2010, her mother donated a kidney to help her daughter. But just three years after the operation, aHUS reared its ugly head and attacked the new kidney, meaning it had to be removed from her body.
Sarah was back on dialysis immediately, and hasn't been able to try a transplant again because of one simple restraint - she doesn't qualify to access the only drug that will stop aHUS from attacking her organs.
Known as eculizumab, and sold under the name Soliris, the drug can be used to treat aHUS with weekly, fortnightly or monthly instalments.
Listed as the most expensive medication on Australia's Pharmaceutical Benefits Scheme, costing upwards of $500,000 per year, those who have access to the lifesaving treatment are few and far between.
But one of the lucky recipients was Sarah's older sister Rebecca, who was diagnosed with aHUS just two years ago.
In 2014, six years after Sarah was confirmed as having the condition - her sister fell ill and showed the same symptoms. The results came back positive, which lead to further testing of the pair to confirm the condition was in fact genetic.
The drug Soliris wasn't around when Sarah first got sick, but it was available for Rebecca. Being in the early stages of diagnosis, and because she hadn't reached the point of requiring a renal transplant, she was given access to the drug her sister so desperately needs.
"The drug allowed her to go back to work just four weeks after being diagnosed with aHUS, whereas I spent the first 18 months after my diagnosis in hospital, and half a year in ICU when I got sick in 2008," Sarah said.
"It's great for her, I was so worried what would happen and that she would go through what I have. My whole family was terrified."
But with Rebecca's kidney function back to normal, and only requiring a 20-minute Soliris infusion once a month - Sarah only wishes she could get the same dosage as her sister.
While the drug is readily available and saving lives in the US, Spain, France, England, Germany, Russia, Argentina and Italy, by stopping the body inflammatory response to those in need, that's not the case for people in Australia.
In 2014, the Australian government made the decision to fund Soliris for a number of aHUS patients who met a select criteria - Rebecca qualified, Sarah didn't.
"It has been available for people with acute episodes of aHUS who have not progressed to end stage renal disease (ESRD)," a Health Department spokesman told news.com.au.
This means, it is not listed for patients on long term dialysis, or waiting on a transplant - such as Sarah.
While some studies suggest the drug will not work over long periods of time, Sarah says her older sister is a shining example of how Soliris allows an aHUS patient to live a better life. Currently, Sarah's doctor's will not conduct another kidney transplant until she gets the drug, because a transplanted kidney would suffer the same aHUS attack as her native kidneys.
The Australian Government said the cost of the drug was another factor in regulating who received the treatment.
While Sarah understands the parameters around funding such an expensive drug, she only hopes Health Minister Sussan Ley will hear her plea and reconsider funding Soliris for her situation.
"I don't want to be tied to a machine and live the rest of my years like this," she pleaded.
"I'm young and don't want to die young. My last eight years have been taken away from me, and I want to get the most out of what I have left.
"This drug should be for everyone, I just want to stay alive."
