PAH Overview
The following is a simple explanation of pulmonary arterial hypertension (PAH) and how it may be affecting your health. Knowing this may help you to better understand prostacyclin therapy and how it works.
- PAH is high blood pressure in the blood vessels in the lungs
- In people who have PAH, the blood vessels in the lungs become too narrow
- This makes it harder for blood that is being pumped to the lungs from the heart to pick up the important oxygen it needs and carry it to the rest of the body
- It also puts extra stress on the heart
Effect of PAH on the Lungs
In a healthy person's lungs, the vessels are wide enough for the blood to flow freely through the lungs to collect oxygen for the rest of the body.
In a person with PAH, the vessels in the lungs become narrowed, making it much more difficult for the blood to flow through them.
Effect of PAH on the Heart
The heart in a healthy person works efficiently to pump blood to the lungs and throughout the rest of the body.
PAH causes the heart to work harder. Over time, the right side of the heart can become larger and the walls of the heart thicker. These effects can lead to symptoms of PAH.
Symptoms of PAH
Classifications of PAH
A well-known classification system was created by the New York Heart Association (NYHA) to measure disease severity in patients with PAH.* Patients with these symptoms could benefit from REMODULIN therapy.
*The World Health Organization (WHO) developed a similar classification system that is also commonly used for patients with PAH.
NYHA Functional Classes
Class I: No symptoms with ordinary physical activity
Class II: Some symptoms with ordinary activity and slight limitation of physical activity
Class III: Symptoms with less than ordinary activity and increased limitation of physical activity
Class IV: Symptoms with any activity, possibly even while at rest
Blood Vessels of the Lungs: A Closer Look at the Effect of PAH
Healthy Vessel: In healthy vessels, the blood flows normally, allowing oxygen to get to the body as needed.
Affected Vessel: In vessels affected by PAH, the blood flow is restricted, resulting in less oxygen getting to the body.
Severely Affected Vessel: In vessels severely affected by PAH, there is very little blood flow and much less oxygen gets to the body.
Common Pathways
The levels of prostacyclin, endothelin, and nitric oxide in the body can become unbalanced. When this happens, it can cause the blood vessels to narrow. This can lead to PAH.
REMODULIN® (treprostinil sodium) Injection
Indication
REMODULIN is indicated for the treatment of pulmonary arterial hypertension in patients with NYHA Class II-IV symptoms to diminish symptoms associated with exercise. It may be administered as a continuous subcutaneous infusion or continuous intravenous infusion; however, because of the risks associated with chronic indwelling central venous catheters, including serious blood stream infections, continuous intravenous infusion should be reserved for patients who are intolerant of the subcutaneous route, or in whom these risks are considered warranted.
In patients with pulmonary arterial hypertension requiring transition from Flolan (epoprostenol sodium), REMODULIN is indicated to diminish the rate of clinical deterioration. The risks and benefits of each drug should be carefully considered prior to transition.
Important Safety Information:
Chronic intravenous infusions of REMODULIN are delivered using an indwelling central venous catheter. This route is associated with the risk of blood stream infections (BSI) and sepsis, which may be fatal. REMODULIN should be used only by clinicians experienced in the diagnosis and treatment of PAH. REMODULIN is a potent pulmonary and systemic vasodilator. It lowers blood pressure, which may be further lowered by other drugs that also reduce blood pressure. REMODULIN inhibits platelet aggregation and therefore, may increase the risk of bleeding, particularly in patients on anticoagulants. Initiation of REMODULIN must be performed in a setting with adequate personnel and equipment for physiological monitoring and emergency care. Therapy with REMODULIN may be used for prolonged periods, and the patient's ability to administer REMODULIN and care for an infusion system should be carefully considered. Dose should be increased for lack of improvement in, or worsening of, symptoms and it should be decreased for excessive pharmacologic effects or for unacceptable infusion site symptoms. Abrupt withdrawal or sudden large reductions in dosage of REMODULIN may result in worsening of PAH symptoms and should be avoided. Caution should be used in patients with hepatic or renal insufficiency.
The most common side effects of REMODULIN included those related to the method of infusion. For subcutaneous infusion, infusion site pain and infusion site reaction (redness and swelling) occurred in the majority of patients. These symptoms were often severe and could lead to treatment with narcotics or discontinuation of REMODULIN. For intravenous infusion, line infections, sepsis, arm swelling, tingling sensations, bruising, and pain were most common. General side effects (>5% more than placebo) were diarrhea, jaw pain, vasodilation, and edema.
Please see the REMODULIN full prescribing information for further details.
