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She was six years old when she was diagnosed with the disease and the illness has been part of her life ever since.
Something as simple as the common cold can turn serious and leave her struggling for every breath.
A small pharmacy of pills and syrups becomes necessary to fight every infection – but after each bout of illness her body is left a little more resistant to antibiotics, a little more vulnerable to sickness.
Although Cheri Nel (38) fights hard for her quality of life, living with cystic fibrosis isn’t easy. Which is why she’s embarked on a David vs Goliath battle for the one drug that will make a significant difference to her life.
It’s called Trikafta and right now it’s cystic fibrosis patients’ best hope for a longer and better life.
“It’s not a cure but it’s the closest thing we have to it,” Cheri says.
Trikafta contains three precision medications – ivacaftor, tezacaftor and elexcaftor – which don’t only treat symptoms but target the basic defect from specific genetic mutations that cause cystic fibrosis.
“It’s a miracle drug,” says Dr Marco Zampoli, paediatric pulmonologist and head of the cystic fibrosis clinic at the Red Cross Children’s Hospital in Cape Town.
“If started early in life, it will prevent irreversible damage to the organs, especially the lungs.
“Instead of often dying at a young age, people with cystic fibrosis can look forward to a much healthier and normal life.”
Alan Dunn, chairman of the SA Cystic Fibrosis Association, says the difference the drug makes to people’s lives is remarkable.
“People who previously couldn’t work can start working again. Those who didn’t have energy before can exercise regularly.”
But there’s a catch – and it’s a big one. Trikafta isn’t available in developing countries, and that includes South Africa.
It’s only available in North America, Europe, Australia and New Zealand.
The cost to bring it into SA would be exorbitant: a year’s supply of the drug would cost around R6 million per patient, excluding import charges.
US medication giant Vertex Pharmaceuticals manufactures the drug and it has registered patents for it in South Africa.
But the company isn’t producing or distributing it here.
As Vertex hasn’t applied to register the drug with the SA Health Products Regulatory Authority (Sahpra) – but has registered patents here – it means other drug companies aren’t allowed to produce generic versions.
Cheri, a chartered accountant and investment banker who lives in Sandton, knows she’s one of the lucky ones: she’s made it into her thirties, which is rare for cystic fibrosis patients.
But she wants to improve the quality of life for all sufferers – which is why she’s taking Vertex to court in a bid to permit the generic in SA, so local patients have access to affordable medication.
Cystic fibrosis is a genetic condition that causes the cells that produce mucous, sweat and digestive juices to thicken and become sticky, causing blockages in the airways and lungs.
“You have to fight for every breath,” Cheri says.
Symptoms include coughs, lung infections, the inability to gain weight and it can cause secondary conditions, such as diabetes.
Over time, infections damage the lungs and patients often require lung transplants or die of lung-related complications.
“When you’re sick, your lung capacity decreases,” Cheri says.
“The biggest fear of people living with cystic fibrosis is that your lungs won’t bounce back to the level they were before the exacerbation.”
At the hint of a cold she has to go to the doctor to find out what she’s picked up as any bacteria or virus could be deadly. Some of them have a mild impact, she says. “But then there are the bad guys. If you pick up one of the severe ones you have to come out guns blazing and take all kinds of antibiotics and meds.”
Infections quickly become resistant to antibiotics, so you have to keep trying different ones, she adds.
Cheri has check-ups every six weeks and is hypervigilant about hygiene.
“I’m very wary of shaking people’s hands and I’m constantly aware that my cellphone can carry germs. And my hands feel ugly because I have to wash them all the time.
“I also have to manage my stress levels and make sure I get enough sleep – stress and lack of sleep is a recipe for a cystic fibrosis exacerbation.”
Cheri doesn’t know how much time she has left with her husband, Rob, but she’s trying to live life to the fullest.
Things are going well at work and she enjoys hanging out with her husband and friends and playing with their three dogs.
She admits she and Rob sometimes become negative about her illness. “But you can sit and cry, or you can play the cards you were dealt. That’s what I do.”
Cheri has also chosen to fight. Since Vertex came onto the market in 2019, at least 30 South Africans have died of cystic fibrosis, she says in court papers submitted to the court of the commissioner of patents, a specialist court of the Gauteng division of the high court in Pretoria.
If the drug had been available in South Africa, she says, those patients might have survived.
The only alternative for Cheri and the 550 other South Africans on the cystic fibrosis registry is medication that suppresses their symptoms – and even that costs a whopping R50 000 a month, says Cheri.
She’s asking the court for a declaration that Vertex is abusing its patent rights. She’s also asking for a compulsory licence that will end the company’s monopoly protection and allow for the supply of a more affordable generic version.
In court papers she says Vertex’s conduct violates the constitutional rights of people with cystic fibrosis by effectively denying them lifesaving treatment.
According to Cheri, Vertex indicated last week that they would be opposing her action. Similar legal action is under way in other countries, including India and Ukraine.
Responding to YOU’s written enquiry from the US, Daria Munsel, Vertex’s director of foreign relations, says the company cannot discuss the case as it’s in court.
“As seen in other rare disease areas, it’s challenging to bring medicine to patients in South Africa as the reimbursement system and willingness to invest do not support a viable path to sustainable access,” she says.
She adds Vertex is in talks “with relevant stakeholders in the private insurance system” to see how they can provide better access to patients.
“We can also confirm we recently signed a contract with a local distribution partner for our cystic fibrosis therapies in South Africa. While access to medicines for rare diseases remains challenging in SA, we’re committed to supporting access to our cystic fibrosis treatments as soon as possible.”
For Cheri, “as soon as possible” isn’t good enough, which is why she’s launched this court action.
She says people shouldn’t have to live in fear of catching a cold.
