160219. Cape Town. Razelle Shulman was diagnosed with an extremely rare lung disease called Pulmonary Arterial Hypertension and now state hospitals cant afford her medication. Picture Henk Kruger/Cape Argus/Reporter Helen Bamford

Cape Town - A Cape Town woman suffering from pulmonary hypertension, a rare lung disease, has been given six months to live after she says doctors at Groote Schuur Hospital reduced dosages and cut back on her medication which could potentially have prolonged her life.

Razelle Shulman, 56, of Ysterplaat, was diagnosed with the condition in 2013 and had been treated privately until she was forced to go to a state hospital last August when she no longer had medical aid.

“That is when everything went downhill,” she said. “They changed my medication and reduced the dosage. When I told them I was getting worse they said there was no proof.”

But she said a private doctor had told her that if she did not receive the additional medication, she had about six months to live. Although there is no cure for pulmonary hypertension, medication can increase life expectancy.

Shulman had been using oxygen to help her breathe because the disease causes fatigue and extreme shortness of breath, but she was told at Groote Schuur she no longer qualified.

They also declined to give her a drug called Bosentan, which is not available in South Africa – it has to be imported – telling her it was too expensive.

Pulmonary hypertension is the same disease that killed 20-year-old Jenna Lowe, three years after she was diagnosed with the disease and six months after a bilateral lung transplant.

Like Lowe, Shulman was also misdiagnosed several times before doctors realised what she was suffering from.

Shulman said the Pulmonary Hypertension Association of SA eventually referred her to cardiologist Dr Jan Smedema, and she had been responding well under his treatment until forced to go to the state facility.

“I could feel the difference within weeks. I stopped being able to climb stairs and I couldn’t even walk two steps without having to stop to catch my breath. Everything is an effort.”

Smedema confirmed that Shulman’s condition was deteriorating and said it was disappointing her dosages had been reduced at Groote Schuur.

He said he often had to fight to get the right drugs for his patients.

“I sit on the phone and harass the medical schemes so it’s disappointing to see the treatment she is getting.”

He said pulmonary hypertension was a rare but deadly condition, much like terminal cancer, and it often affected young, productive women.

Smedema questioned why expensive drugs were given to transplant patients yet not to patients such as Shulman.

“It seems there are some doctors that hide in big state institutions without caring for their patients yet still get paid their high salaries,” he said.

Denneys Niemandt, the former chairman of the Pulmonary Hypertension Association of SA, who was diagnosed with the disease in 2006, said people who did not have medical aid were often left “stranded”.

Even with medical aid, he has to re-apply every year for his chronic medication, and his doctor has to provide written motivation for it. He said the experience had been different when he had been diagnosed with cancer last year.

“Within two days all my medication had been approved.”

Niemandt said he understood Shulman’s frustration at battling to access the medication. “In Europe and the US, there are 15 or 16 types of medication for pulmonary hypertension, some of it free. In South Africa, there are only two officially registered ones that have to be imported at huge cost.”

What was also frustrating, he said, was that his European connections had indicated they would be willing to send medication to South African free of charge but, because of red tape in South Africa, it would be seized and destroyed.

Alaric Jacobs, spokesman for Groote Schuur Hospital, said Shulman was receiving the available medication for her condition.

He said that Bosentan was not a registered medicine in South Africa and there was no generic for it in the country.

“In order to use it, approval must be given from the Medicines Control Council after motivation from the treating doctor, and even then acquiring the drug depends on its affordability within the current budget. If medicine is not affordable, then appropriate alternatives will be considered to treat the patient.”

* Pulmonary hypertension is a disease affecting the arteries of the lungs. It can strike anyone regardless of age, gender, social or ethnic background.

People affected with this disease suffer from continuous high blood pressure in the lungs, which results in an enlargement of the heart, and can lead to heart failure.

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Cape Argus