PPH has only been the subject of intense medical investigation in the past 10-15 years, although the disease has been known since the 1950s. Most doctors originally believed that the disease stemmed from preexisting lung diseases such as emphysema. Although it is often the result of other conditions, PPH today is recognized as existing without any other known cause. Unfortunately, PPH is still a disease that doctors know little about. But in recent years many specialists have emerged to break new ground on research involving how the lungs function and have used that knowledge to develop treatments.
What they have learned is that the disease arises inside the tiny blood vessels of the lungs. These blood vessels produce complicated chemical compounds that control their behavior and size. In some people, a chemical called endothelin which causes these blood vessels to constrict is overproduced. The reasons for this are still unknown. In some cases the cause is genetically programmed or the person is predisposed genetically and gets PPH after being exposed to a certain drug.
The information on this web site is geared for people who have PPH as a result of taking diet drugs.
Although no one knows what the true number of people with PPH is, the numbers have increased dramatically in the past 6 years. Doctors and researchers have concluded the cause of this increase was the widely prescribed diet drug Fen Phen. Before these drugs were used by more than 6 million people in 1996 and 1997, the best estimates were that only one or two in a million people could get PPH. In the years since Phen Fen was used, the incidence has jumped to ten times that number by some estimates. According to a New England Journal of Medicine study, 1 in 20,000 Fen Phen users is at risk of getting PPH. Thus, by taking diet drugs, a person with a one in a million chance of getting PPH, increases that likelihood by several orders of magnitude.
According to doctors who treat patients with PPH, those who contract it through Fen Phen and diet drugs, tend to have more severe cases. Dr. Stuart Rich, one of the world's foremost authorities on PPH, writes of patients who have PPH related to diet drugs: "Although the syndrome is indistinguishable from primary pulmonary hypertension, our experience suggests these patients tend to have a more aggressive disease with a poorer prognosis than similar patients with PPH. This may be a result of the fenfluramines triggering a unique molecular pathway that produces pulmonary vasculopathy."
Fen Phen Lawsuits
Fen Phen is an abbreviation of a very popular diet drug combination: fenfluramine and phentermine. Similar drugs, Redux (dexfenfluramine) and Pondimin (fenfluramine) were also widely prescribed in the mid 1990s.
After wide-spread reports of heart valve disease linked to Phen fen, American Home Products was forced to recall the drug in 1997. The heart valve disease had little to do with PPH, but that was the issue that initially attracted regulators and lawyers attention.
PPH was a side issue, because of its rarity as compared to heart valve disease. However, several lawyers began to explore what the company knew about this disease. They discovered in the course of litigation that PPH was a known risk, that the company chose to ignore and hoped that doctors would follow suit.
In an 1995 internal memo discovered by lawyers, the marketing department of the diet drug maker advised:
"A weight-maintenance position strategy is still possible with a pulmonary hypertension warning, because physicians may overlook the warning as they currently do with other drugs."
The drug maker had known that its product could cause PPH because of a similar product, called Aminorex marketed in Europe in the late 1960s and early 1970s. That diet drug resulted in a ten fold increase in PPH in the years following its use. The American manufacturer of Fen Phen apparently hoped that the incidence of PPH would be so small that it wouldn't be noticed. If it had not been for the heart valve disease controversy, it might not have been noticed until millions more people had taken these dangerous drugs.
The lawsuits mounted, and the company eventually opted to settle for 4 Billion dollars, however this amount does not include claims relating to PPH. The vast majority of PPH claims are treated as individual cases against the company. The average person with a confirmed PPH diagnosis is likely to receive a several million dollar settlement.
A recent book, Dispensing with the Truth: The Battle over Fen-Phen (pictured at left) tells the story of a 29 year old Massachusetts woman who wanted to lose weight before her wedding in 1996. After taking Fen Phen for only three weeks, she began to experience the symptoms of PPH including dizziness and shortness of breath. She stopped taking the drugs, felt somewhat better, but then the symptoms returned with a vengeance. Unknown to her and her doctors she had primary pulmonary hypertension, her lung capillaries were closing, slowly suffocating her and destroying her heart. Within three months she was dead.
Her family hired a lawyer, which resulted in the first significant trial on the PPH issue. The company settled her case for many, many millions of dollars.
The book goes on to outline the legal battle on both the PPH and heart valve damage issues. The author follows several different cases from Boston to Texas. In the process, the book reveals through court filings and documents uncovered during the litigation, the extent to which American Home Products was aware of the health risks and chose to ignore them. The reader will likely conclude that the company made a business decision to endanger the health of millions of Americans in order to sell a diet drug.
If you or a loved one has been diagnosed with PPH and have used Fen Phen or related diet drugs, you may be entitled to substantial compensation.
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