Rare illness makes life a struggle

07.06.2013

Source: IOL News

July 6, 2013

Jenna Lowe was a dancer and a swimmer, but now she uses a mobility scooter to get to her matric classes at Herschel, and can’t walk any distance without becoming dizzy and weak.

4006711295.jpgJenna Lowe has pulmonary hypertension. At 18, she struggles to maintain a normal lifestyle, having to be on oxygen constantly, suffering from shortness of breath. Picture: Thomas Holder


 

 

 

Every day, the 18-year-old Claremont girl breathes oxygen through a tube, swallows warfarin to prevent her blood from clotting and takes imitation Viagra designed to dilate the vessels in her lungs.

But it’s bosentan that will help prolong her life.

Bosentan is FDA-approved but not available in South Africa, so has to be imported from Switzerland. And it comes at a crippling cost: R32 000 a month, and a swamp of paperwork.

Jenna is one of 50 to 80 South Africans living with pulmonary arterial hypertension (PH).

And she’s the only one who has access to bosentan, which she gets thanks to a special permit and a special favour from a pharmaceutical import company.

Getting permission from the Medicines Control Council (MCC) to import it is a harrowing process, says Jenna’s mother, Gabi Lowe: “The first time we needed to get permission, it took five months. When you’re looking at a life-threatening illness, five months is a long time.

“All the drugs my daughter needs are FDA-approved, so the question is why are they not registered in this country? People are dying because the figures don’t work out.”

For a drug to be legally available here, it has to be registered with the MCC, a government body.

According to MCC registrar Mandisa Hela, anyone can apply to register a medication, as long as there is a pharmacist to take care of the technical aspects of the application.

Approving new medicines takes the MCC 30 to 36 months, while generics take 18 to 24 months, says Hela.

Only a licensed company is allowed to handle and distribute them.

As Kelly du Plessis, founder of the Rare Disease Society of South Africa, says: “By the time you get the right diagnosis, you don’t have time to wait around for the Medicines Control Council’s approval.”

Denneys Niemandt, who suffers from pulmonary hypertension and is chairman of the Pulmonary Hypertension Association of SA, explained: “Before my medicine was registered in South Africa, I got it free of charge on a compassionate basis. The moment it was registered I had to pay R6 000 a month.”

Even so, registration is the best hope for Niemandt and his community of pulmonary hypertension patients. He is busy putting together an advisory body of 11 specialists to try to speed up the process.

“The first step would be to legalise the medication and get it at a cost that is viable,” he says, “and then also get the medical aids to buy into it so that they can cover it.”

Niemandt, like the Lowe family, has been continually frustrated by the long line of legal hoops they have to jump through for the life-saving medicine.

“People in Europe told us we can get a container full of medication free of charge,” says Niemandt, “but because of medical red tape, if that container lands in Cape Town it will be confiscated and destroyed.”

Before treatment even begins, though, there is the monumental difficulty of diagnosis.

“A GP can’t just diagnose you,” says Niemandt, who spent nearly R40 000 on the testing procedures that detected his pulmonary hypertension.

“It’s very expensive and also very complicated.”

The cost of testing for rare diseases continues to prevent the vast majority of sufferers from ever being diagnosed.

Children are more likely to be diagnosed early because their symptoms present severely, but for adults, a persistent tight chest could easily be passed off as a cold or asthma, and never be treated properly.

Meanwhile, Jenna and hundreds of other rare disease patients are running out of time.

“I’m concerned about whether I’ll always have a secure supply chain,” she says. “Knowing you’re the only one on a drug and you can’t just pop down to the pharmacy and get more is daunting.”

- To raise the funds she needs to import medication from Switzerland, Jenna wrote a song called I need more time, which her younger sister Kristi recorded with the help of jazzy pop duo Goodluck.

It is available for download on iTunes. - Pretoria News

Recent News

Subscribe

Get the latest on PH treatments, community events and more when you subscribe to our newsletter

ImagineCan_Accreditation_Trustmark_ENGFR_WHT-FNL_white.png