Tracleer and PPH News Lung disease drug 'a revolution'
By Kim Arlington
A DRUG newly available in Australia would improve the life expectancy and quality of life of hundreds of people with a life-threatening lung disease, a medical expert said today. 01mar04 - Tracleer today became the first drug listed on the Pharmaceutical Benefits Scheme to help control the lung disease pulmonary arterial hypertension (PAH). The disease mostly affects women aged 20 to 45, but also occurs in young children and adult men. Left untreated, around half of those diagnosed will die within two years. In the past, heart lung transplants offered hope to Australian sufferers of the disease. Associate Professor Anne Keogh, a cardiologist at the heart lung transplant unit at Sydney's St Vincent's Hospital, said the drug would be "a revolution for the disease". Prof Keogh, who works to improve clinical practice for Australian PAH sufferers, said they would "just be ecstatic" that the drug was available. PAH is caused by excess levels of a hormone-like substance called endothelin which restricts and causes abnormally high blood pressure in the vessels transporting blood from the heart to the lungs. PAH eventually clogs up the vessels, leaving patients breathless. Prof Keogh said Tracleer rendered endothelin ineffective, immediately relaxing the arteries and unclogging them long term. The drug would allow patients to live normal - and longer - lives, she said. "The drug does improve life expectancy and instead of 50 per cent (of patients) dying in two years, it's more like 10 or 15 per cent dying in two years. "It has a dramatic effect on people's quality of life." "It doesn't have any horrible side effects, which is spectacular for a drug that's treating such a devastating disease." Prof Keogh said treating PAH with the drug also "takes a little bit of pressure off the transplant waiting list". "Each patient not requiring a heart lung transplant for PAH allows us to provide life-saving organs to another three patients with other conditions," she said. Tracleer, which was developed by Actelion Pharmaceuticals Australia, is the drug most commonly used to treat the disease in the USA, Canada and Europe. The federal Health Department has approved it to treat two of the most common forms of PAH - primary pulmonary hypertension and PAH secondary to scleroderma. Up to 400 Australians suffer from those forms of the disease in any one year.
New drug treats pulmonary hypertension Reported by Susan Aldridge, PhD, medical journalist A clinical trials shows that an oral drug, bosentan, can improve survival in patients who have pulmonary arterial hypertension.
It is only recently that there has been any treatment for pulmonary arterial hypertension (PAH), a condition where the blood pressure in the lungs goes up causing breathlessness, fatigue and increasing disability. Half of all patients die within two years if they go untreated. Now there is a new drug for pulmonary hypertension - the first in pill form - called bosentan. In a trial reported by researchers at the University of Michigan, bosentan has been found to improve survival among those with PAH. Survival in treated patients was 96 per cent at one year and 89 per cent at two years. On past clinical experience, these survival rates would have been put at 67 per cent and 58 per cent. After two years follow up, 70 per cent of patients were coping on bosentan alone. This adds to earlier, more short-term, work that shows that bosentan improves patient's walking ability and quality of life.
Source
European Respiratory Journal February 2005
First and Only Oral Medication Approved for Pulmonary Arterial Hypertension in HIV Patients
MONTREAL, CANADA -- July 24, 2006 -- Tracleer® (bosentan) is now indicated by Health Canada for patients with pulmonary arterial hypertension (PAH) secondary to HIV in those who did not respond adequately to conventional therapy.
PAH is a life-threatening lung disease associated with elevated blood pressure inside the pulmonary artery. PAH patients typically present with unexplained symptoms such as fatigue, breathlessness, chest pain and fainting. People living with HIV are six to 12 times more likely to contract PAH than the uninfected population. Although there are a number of theories as to why this happens, the precise reasons remain unknown.[i]
This new indication for Tracleer® adds another separate and difficult patient population to those PAH patients who can benefit from the drug. Tracleer® had previously been approved in Canada for the treatment of patients with functional class III and IV (meaning a moderate to severe form of the disease) primary PAH and PAH secondary to scleroderma. It can offer significant clinical benefits including improvements in exercise capacity and a decrease in the rate of clinical worsening.
Tracleer® is the only approved oral treatment in Canada that blocks the body's endothelin receptors. Endothelin is a substance produced by the endothelial cells that form a layer lining the blood and lymph vessels. It has the effect of narrowing the blood vessels and can also harden tissues, causing scarring. Endothelin is known to be a key component in the development of PAH.
A study evaluating the impact of starting treatment with Tracleer® on the survival of primary PAH patients has shown that 96% of patients were alive after one year and 89% were alive after two years. This is compared to a predicted one and two year survival of 69% and 57% respectively based on National Institutes of Health (NIH) registry formula.
"It is a reassuring development that bosentan is now approved for patients who have PAH secondary to HIV, as this is a population for whom specific therapy for PAH was not available. Similar to other patients with PAH, people with HIV can become quite ill and die from their disease" said Dr. John Granton, Respirologist, Toronto General Hospital.
About Pulmonary Arterial Hypertension (PAH)
PAH is a rare and life-threatening disease characterized by abnormally high blood pressure in the pulmonary artery, the blood vessel that carries oxygen-poor blood from the right side of the heart to the lungs. In PAH, there is increased blood flow resistance in the pulmonary arteries, raising pressure and forcing the right side of the heart to work harder. The heart muscle starts to weaken, resulting in right-heart failure. There are a number of treatment options for PAH, although not yet a cure.
Important Safety Information
In clinical trials leading to the marketing approval of bosentan, approximately 11% of PAH patients receiving the drug experienced abnormal but reversible liver enzyme elevations. It is therefore important that patients undergo monthly liver monitoring. Due to the risk of birth defects, women who are pregnant, or of childbearing age who do not use a reliable method of contraception, must not take bosentan.
Tracleer is a registered trademark of Actelion Pharmaceuticals Ltd, CH-4123
Allschwil, Switzerland.
REFERENCE:
[i] http://aids-clinical-care.jwatch.org/cgi/content/full/2001/601/6, accessed January 24, 2006.
SOURCE: Actelion
|
