‘I just want to make it to my 40th birthday’

When Canberra woman Vanessa Counsel began to have numbness in her back in her late 20s, it was the start of

“I underwent testing and they discovered I had a series of tumours in my spine,” says Vanessa.

She has Von Hippel-Lindau Syndrome (VHL). Her father died from the disease at just 42 and her brother also has it.

The disease sees tumours and cysts grow in various parts of the body, which can include the brain, spinal cord, retinas, ears, pancreas, adrenal glands, kidneys and more.

Sign this petition to make treatment for VHL available on the Pharmaceutical Benefits Scheme (PBS).

Up until this point, the only treatment option has been removing tumours surgically – treatment that the 34-year-old Vanessa is all too familiar with.

“Since then I’ve had lower spine surgery, neck surgery and I’ve had half of my kidney removed on the left side. They had to take 50 per cent to remove the cancers.”

About 12 months after this, surgeons were forced to remove Vanessa’s entire right kidney as well, leaving her with just half a kidney on her left side.

“The surgeon has told me that the next time I have to have surgery, it will be to remove the entire kidney, leaving me on dialysis for the rest of my life,” says Vanessa.

For this reason, Vanessa is in a race against the clock to gain access to a new drug called WELIREG (marketed as Belzutifan), which has been proven to shrink certain VHL tumours.

Dr Kathy Tucker, a geneticist who has been treating VHL patients since the 1990s, says the drug is life changing for people with the condition.

“Depending on the different cancer, or tumour types, there is between a 50 and 90 per cent chance of [Belzutifan] decreasing the tumours,” she says. “So, that’s not just stopping the tumours getting bigger, but actually making them shrink. This is the most exciting thing that’s happened for me in my career in terms of being able to turn around the lives of people rather than just screen for things that are going to get worse. Belzutifan has the potential to change outcomes.”

The catch? Currently, Belzutifan costs patients $12,000 a month. Should it be added to the PBS, that cost to the patient could be brought as low as $7.70.

“Obviously, both my brother and I are getting closer and closer to the age my father was when he died from VHL,” she says.

“I can’t afford this drug at its current cost, and I don’t know how long it will take – if at all – until it’s added to the PBS. In the meantime, it’s reasonable to think I’ll need another kidney surgery – one that could leave me on permanent dialysis – within the next year. It’s literally a race against time.

“If this drug was added to the PBS and I could start taking it, there is a real chance the tumours would shrink and I could start to have hope for the rest of my life – because I would actually have a life to look forward to,” Vanessa continues.

“It feels so incredibly cruel that for the first time in my life – for many families, the first time in generations of suffering – that there is hope of being able to treat this with medicine, it’s so far out of reach still.”

“I have been told I can’t have children as the risk with the tumours is too high. It’s taken so much from me, but if I could access this drug, it could give me a future,” Vanessa continues.

“I want to be able to think about big events, like having a 40th birthday or maybe travelling or just having the reassurance that I might not have to have a really scary invasive surgery every year. It would change everything.”

Vanessa, who is also at risk of losing her pancreas and contemplating the very real possibility of a future reliant on insulin and dialysis, says that without access to Belzutifan, her future is “very scary, harsh and unpredictable.”

“If Belzutifan can be approved by PBAC and placed on the PBS that would be

wonderful,” says Meredith Cummins, CEO of NeuroEndocrine Cancer Australia (NECA).

“It would be so much more beneficial if this approval could be fast tracked

to allow patients who are rapidly deteriorating to have some hope, and enjoy the time

they have.”

“Every person deserves to have their best life and to have fair rightful options for the

management of their condition. Every person deserves to have quality of life. How

can you put a price on a life when considering approving a medication or not?”

Add your voice to the campaign to get Belzutifan added to the Pharmaceutical Benefits Scheme (PBS) to reduce its price to as little as $7.70 per month for patients in desperate need. Please take 30 seconds to sign this petition.

Dr Tucker would like to disclose that she’s authored educational material about Belzutifan for MSD Australia.

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