Primary Pulmonary Hypertension Information

 

Primary Pulmonary Hypertension Information

Primary pulmonary hypertension (PPH) is the rare and deadly side effect, most recently linked to the use of the once popular diet drugs Fen Phen, Redux, and Pondimin. The incidence of PPH is about 2 in every 10,000 patients seen at the Mayo Clinic, however the frequency PPH is diagnosed has become more common, especially in women. A study that was published in the New England Journal of Medicine in 1996 found that primary pulmonary hypertension (PPH) occurs in 1 in 20,000 Fen Phen users, a number that was later found to be largely underestimated. With the number of Americans that used Fen Phen prior to its’ recall to be around 6-7 million people, PPH has affected, and continues to affect a large number of people.

Many instances of PPH are overlooked because the symptoms are very common amongst other non-threatening conditions. As a result, a delay in PPH diagnosis and treatment allows the PPH to progress into later, more threatening stages. Anyone whom has taken recalled diet drugs Fen Phen, Redux, or Pondimin should seek medical evaluation if they have not yet already done so. Studies show that the patients who had used the recalled diet drugs for a period of three months or longer had an increased risk of twenty-three fold for developing primary pulmonary hypertension.

Common primary pulmonary hypertension symptoms include:

  • New heart murmur
  • Chest pain
  • Shortness of breath
  • Fainting
  • Tiredness
  • Weakness

When PPH progresses to a more serious stage, symptoms can also include:

  • Death
  • Increased chest pain
  • Ankle and lower leg swelling
  • Bluish lips and skin

Primary pulmonary hypertension causes high blood pressure in the lungs leading to a feeling of constant breathlessness with minimal exertion, fatigue, dizzy spells, fainting, and chest pain. PPH somehow will cause some of the endothelial cell lining of the lung’s capillaries to peel off. The endothelial cells are what keep blood from leading out when blood cells pass through the small blood vessels, or capillaries. The leaking blood enters into the smooth muscle cells and causes it to continuously constrict as a reaction. Normally, the smooth muscle cells allow the capillary to get either narrower or wider dependent upon the amount of blood surging back an forth between the heart and lungs.

Receiving a PPH diagnosis is associated to a life expectancy of around three years, but recently the advancement of primary pulmonary hypertension treatments have prolonged the life expectancy. Flolan therapy has increased the expected length of PPH sufferers by 3-5 years. Back in the 1990s PPH patients could only expect to live three years, so while the prognosis has increased with the development of certain PPH treatments, there is still no cure for PPH as of yet.

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To find out what PPH treatment methods are currently being used, including Flolan and Tracleer click here.

 


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