Pulmonary Arterial Hypertension is treated
in a number of effective ways. While there is no cure for PAH,
the effects of the disease can be minimized. New treatments,
notably vasodialators such as prostacyclin, discovered in recent
years have made significant improvements in the lives of PAH
patients. Before the development of these drug treatments, PAH
was rapidly fatal with nearly two thirds of patients having 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.
The most significant advances were made in the late 1990s by
Sir John Vane, a Nobel Prize winner, whose work with prostaglandins
has revolutionized PAH treatments. These treatments have focused
on very specific chemicals the body uses to control the behavior
of the lung's blood vessels.
Course of Treatment
After a positive diagnosis of PAH, doctors will usually follow
a PAH management program (developed by researchers Dr. Rubin
and Rich). The treatment will proceed in stages depending on
the patient's response. The initial stages consist of evaluating
the extent of the PAH 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 or given intravenous prostacyclin or adenosine.
If the 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.
Drug Therapy
Anti Coagulation Agents
Most PAH 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 considered to be a more conventional oral vasodilator
therapy. CCBs are the most widely used type of drugs used to
treat PAH. 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 thereby
increasing the hearts output of needed blood flow.
New Drugs
The three main pulmonary vasodilator therapies currently used
are:
Epoprostenol (Flolan)
Treprostinil (Remodulin)
Bosentan (Tracleer)
These substances are widely hailed as major advances in PAH treatment.
These molecules work by triggering a reaction in the tiny vascular
endothelium cells (see What is PAH)
and cause pulmonary vasodilation (opening of the lung blood vessels).
Flolan or Epoprostenol was approved by the FDA in 1995
for patients with PAH. The drug is extremely effective in opening
up the lung arteries and takes immediate effect, 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.
Treprostinil or Remodulin The drug is very similar to
Flolan but does not need to be stored at as low a temperature
as does Flolan and it lasts much longer so that it does not need
to be constantly infused and 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.
Read more on Remodulin >>
Bosentan (Tracleer) This drug 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. This drug
is called an "endothelin-A and endothelin-B receptor antagonist."
In tests and clinical trials the drug proved its effectiveness
by improving arterial capacity and reducing pressure in the blood
vessels of the lungs.
Lung Transplants
If the PAH 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
2) Single Lung
3) Double lung
A Heart and Lung Transplant will replace all the damaged
organs, but is generally disfavored because the heart's right
ventricle can recover if the pulmonary resistance is lessened
with a new set of lungs.
A single lung may be an option, for older patients who do no
qualify for a double lung transplant. Additionally the surgery
is easier and many patients tolerate the procedure better. On
the down side there may be a mismatch with the blood all flowing
in the new lung, causing complications.
The most favored of these options is the double lung transplant.
Doctors believe it avoids the complications of a single transplant,
while offering more protection if something goes wrong. Unfortunately
the surgery is more difficult to perform and the wait for two
lungs is much longer.
If you or a loved one has been diagnosed
with PAH and have used Fen Phen or related diet drugs, you may
be entitled to substantial compensation to help pay for treatment.
Contact us to see if we can help
you.
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