PRIMARY
PULMONARY HYPERTENSION
INFO
CENTER
Primary
Pulmonary Hypertension is an extremely complex lung condition with
no known cure. A progressive disease affecting about 100,000
people worldwide, PPH may lead to congestive heart failure and respiratory
failure. PPH is most common in women between ages 21 and 40. Doctors
are not sure what causes the illness but it has been linked to the
recalled appetite suppressant known as Fen-Phen.
How
does PPH harm the body?
•
Pulmonary vessels may leak blood (patient may cough blood)
• Blood-oxygen content decreases
• The body produces more red blood cells
• The patient’s blood thickens
• The patient’s heart works harder to pump thickened
blood
• Arterial pressure increases to dangerous levels
Click
on the links below for more information about PPH
Causes
of PPH
Symptoms of PPH
Diagnosing PPH
PPH Treatment Options
—
CAUSES OF PPH —
There
is currently no known cause of PPH. Leading experts believe PPH
is likely caused by some type of infection that arrives in the pulmonary
vascular bed in genetically susceptible persons. A period of time
will pass, which may be years or decades, and the infection will
cause damage to the pulmonary vascular bed cells causing a spiral
hypertension. Although no cure currently exists, one encouraging
aspect for medical researchers is that PPH is likely not systemic
in origin, in that it has never recurred in a person who received
a lung transplant. Leading medical experts believe that causes of
PPH may be halted, damage may be reversed, and that the disease
will eventually be cured.
—
SYMPTOMS OF PPH —
Initial
symptoms of PPH are often very minor - almost unrecognizable. And
PPH is very difficult to detect in a routine medical examination.
Consequently, diagnosis may be delayed many years until symptoms
become worse. In the case of many Fen-Phen users, a troubling fact
is that PPH is a progressive disease. In other words, although a
person may have obtained a medical evaluation following the removal
of the Fen-Phen diet drugs from the market in 1997, the disease
could progress over a period of years, worsening until symptoms
are obvious and/or no longer confused with other conditions.
The
following symptoms have been associated with PPH:
•
Fatigue
• Light-Headedness
• Dizziness
• Fainting
• Chest Pain
• Physical Weakness
• Edema (Fluid Retention)
• Shortness of Breath
• Palpitations
• Swelling
• Increased Heart Rate
If
you or someone you know has taken Fen-Phen, Pondimin or Redux, make
sure you see a doctor right away. You may have PPH and not even
know it. The sooner this disease is discovered, the greater the
chances are for survival.
—
DIAGNOSING PPH —
It
is difficult to detect PPH in a routine medical examination. Even
when the disease has progressed, the signs and symptoms may be confused
with other conditions that affect the heart and the lungs. To determine
if a patient has pulmonary hypertension, a physician may recommend
a cardiac catheterization with an angiography. The way that PPH
is diagnosed is unique because it is basically a large process of
elimination. The doctor reaches a diagnosis of PPH by first ruling
out all other diseases and afflictions that can cause PPH. The following
tests are used to help determine if a patient has PPH:
•
Pulmonary function tests (to measure and evaluate lung
function)
• Connective tissue serology (to rule out
systemic connective tissue disease)
• Echocardiogram (to measure and evaluate
cardiac function)
• Cardiac catheterization (to measure cardiac
function)
• Perfusion lung scans (to rule out thromboembolic
disease)
• Pulmonary angiography (to rule out the
presence of clots and other lung blockages)
• Blood tests
• Sleep studies
• MRI
What
is a PPH Functional Classification?
Once
PPH is diagnosed, doctors classify the disease based on how well
a patient can perform daily life tasks and functions. Created
by the New York Heart Association, there are four classes:
Class
1 - Patients who have no symptoms
such as tiredness, palpitations, shortness of breath, and chest
pains.
Class 2 - Patients who are comfortable when resting
but experience tiredness, palpitations, shortness of breath, and
chest pains when performing basic life tasks.
Class 3 - Patients who are comfortable when resting
but experience tiredness, palpitations, shortness of breath, and
chest pains with less than ordinary life tasks.
Class 4 - Patients who experience tiredness,
palpitations, shortness of breath, and chest pains even at rest.
—
PPH TREATMENT OPTIONS —
PPH
is treated in a number of effective ways. While there is no cure
for PPH, the effects of the disease can be minimized. New treatments
discovered in recent years have made significant improvements in
the lives of PPH patients. Before the development of these drug
treatments, PPH was rapidly fatal with nearly two-thirds of patients
surviving only three years after the date of diagnosis. After
the development of these new therapies, more than 65% of patients
will survive longer than 5 years. After a positive diagnosis
of PPH, doctors will usually follow a PPH management program. The
treatment will proceed in stages depending on the patient's response.
The initial stages consist of evaluating the extent of the PPH by
measuring the vasoreactivity, or degree to which the pulmonary arteries
can be dilated or opened with drug therapy. The patient will be
given known vasodilators: inhalation of nitric oxide, intravenous
prostacyclin or adenosine.
If
these treatments create a notable fall in pulmonary vascular resistance,
the patient will be given oral calcium channel blockers
and anticoagulants which help control pulmonary
pressure. If the fall in pressure is moderate or minimal, the patient
will be given higher doses and more potent vasodilators and may
be a candidate for a heart and lung transplant.
Here
is a description of the various treatment options for PPH:
ANTI-COAGULATION
AGENTS
CALCIUM CHANNEL BLOCKERS
DIURETICS
DRUG THERAPY
LUNG TRANSPLANTS
ANTI-COAGULATION
AGENTS
Most PPH studies have shown longer survival rates when patients
are treated with anticoagulant therapy. Agents like Warfarin are
commonly used to prevent pulmonary blood clotting and scarring.
The blood is thinner and can travel through the constricted pulmonary
blood vessels with less resistance.
CALCIUM
CHANNEL BLOCKERS (CCBS)
CCBs are the most widely used type of drugs available to treat PPH.
Doctors believe that these drugs work by triggering the vascular
smooth muscles in the lungs to open, which lowers resistance and
in turn lowers the pulmonary artery pressure. CCBs nearly always
bring about a reduction in pulmonary vascular resistance by lowering
pulmonary artery pressure and increasing the heart’s output
of needed blood flow.
DIURETICS
These medications help to remove excess fluid from body tissues
which may accumulate due to high pressures in blood vessels.
DRUG
THERAPY
These substances are widely hailed as major advances in PPH treatment.
These molecules work by triggering a reaction in the tiny vascular
endothelium cells and cause pulmonary vasodilation (opening of the
lung blood vessels). These medicines enable the vessels in the lungs
to expand and allow the blood to move through them with less resistance.
The
main therapies currently used are:
Flolan
(Epoprostenol)
Tracleer (Bosentan)
Remodulin (Treprostinil
or UT-15)
Ilioprost
Beraprost
Flolan,
the brand name for Epoprostenol, was approved by the FDA in 1995
for patients with PPH. Flolan is extremely effective in opening
up the lung arteries and producing immediate results. Unfortunately,
its effect only lasts about 5 minutes. The treatment is given
intravenously through an indwelling catheter connected to a pump
that continuously infuses the lungs with the substance. Patients
who elect Flolan treatment must wear a battery operated backpack
or bag that keeps the drug at a proper temperature. The treatment
is very expensive, in the $150,000 per year range. Flolan therapy
has been credited with raising the life expectancy of patients
with PPH by 3 to 5 years or more.
Side
effects can include jaw pain, headache, flushing, nausea, diarrhea,
and vomiting. Interruption of Flolan can be life-threatening,
even a brief interruption can result in a sudden reoccurrence
of symptoms.
Visit the Flolan Information Network
Tracleer,
or Bosentan, is an alternative therapy to the more invasive prostacyclin
agents Flolan and Remodulin. This drug is taken orally and is
the first oral PPH therapy to get full FDA approval in the US.
In tests and clinical trials the drug proved its effectiveness
by improving arterial capacity and reducing pressure in the blood
vessels of the lungs.
Visit the Tracleer Information Network
Remodulin,
also called Treprostinil or UT-15, is still pending full FDA approval,
but the drug is in the final clinical trials. The drug is very
similar to Flolan but it does not need to be stored at as low
a temperature as does Flolan, it lasts much longer, it does not
need to be constantly infused, and it uses a smaller pump. In
some patients, Remodulin may be introduced under the skin in a
process called subcutaneous infusion. Testing indicates Remodulin
carries with it a lower risk of infections caused by the catheter.
The side effects of UT-15 include jaw pain, headaches, nausea,
diarrhea, flushing and localized pain at the delivery site under
the skin.
The
reported results of Remodulin have been encouraging. Patients
using the drug seem to experience improvement in their condition
including: decreased fatigue, decreased shortness of breath, decreased
pulmonary artery pressures as well as overall improvement
in quality of life.
Iloprost
is an inhalable form of prostacyclin, an analog (imitator) of
our body's own vasodialator, prostaglandin. Prostaglandin prevents
blood clots, and researchers believe that people with PPH do not
have enough of it. Prostacyclins have been shown to improve survival
rate, exercise capacity, and hemodynamics (blood circulation)
of patients with severe PPH.
Iloprost
is effective and reduces shortness of breath, and may lower pulmonary
pressures according to an August 2002 study that appeared in the
New England Journal of Medicine. So far Iloprost has only been
approved for treatment of pulmonary arterial hypertension (PAH)
in a few countries, and is not available for PPH in the United
States except through clinical trial.
Beraprost
is an oral form of prostacyclin currently being developed by United
Therapeutics Corporation. Currently in Phase III clinical trials
in the United States and Canada, Beraprost is intended to be used
in early stages of pulmonary hypertension and peripheral vascular
disease only as oral doses of Beraprost do not provide the continuous,
consistent levels of prostacyclin required to treat PPH. United
Therapeutics Corporation has commenced a 100+ patient study of
Beraprost for pulmonary hypertension. In Japan, small, uncontrolled
studies have led to Beraprost being approved for the treatment
of primary pulmonary hypertension.
LUNG
TRANSPLANTS
If the PPH symptoms cannot be controlled using drug therapy, a patient
may opt for a lung transplant. This operation is very risky, but
can extend a patients life for an average of five to seven years.
There are three types of transplants:
1)
Heart and Lung – replaces all damaged organs, but
is disfavored because the heart’s right ventricle can recover
if the pulmonary resistance is lessened with a new set of lungs
2) Single Lung – may be an option for older
patients who do no qualify for a double lung transplant. This surgery
has less risk associated with it and patients generally recover
quicker.
3) Double lung – the most favored type of
lung transplant, but surgery is more difficult and the wait for
two lungs is much longer.
If
you or a loved one has been diagnosed with PPH,
contact your doctor to decide what treatment options may be right
for you.
For
more information on PPH and your legal rights,
Contact a PPH Attorney
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