Uptravi

Pulmonary Arterial Hypertension

Uptravi is indicated for the long-term treatment of pulmonary arterial hypertension (PAH), which is high blood pressure in the vessels that carry blood from the heart to the lungs. It is specifically used for patients with WHO Group 1 PAH, which includes PAH that is inherited, caused by certain drugs or toxins, or associated with conditions like connective tissue disease or HIV infection.

Treatment Goals

The primary reasons healthcare providers prescribe this medication include:

• Delaying disease progression: It helps slow down the worsening of PAH symptoms over time.
• Reducing hospitalization: It is used to lower the likelihood that a patient will need to be hospitalized for complications related to their pulmonary hypertension.

By helping to manage the pressure in the lung's arteries, the medication makes it easier for the heart to pump blood through the lungs, which can help improve the patient's long-term outlook.

Mechanism of Action

Uptravi belongs to a class of medications known as selective prostacyclin receptor agonists. It is designed to act like prostacyclin, a natural compound in the body that helps regulate blood flow in the lungs.

How It Affects Blood Vessels

In people with pulmonary arterial hypertension (PAH), the blood vessels in the lungs become narrow and stiff. Uptravi works by targeting and activating specific receptors on the smooth muscle cells of these blood vessels. This activation causes the following effects:

• Vasodilation: The blood vessels relax and widen, allowing blood to flow through more easily.
• Reduced Pressure: By widening the vessels, the medication lowers the high blood pressure within the pulmonary arteries.
• Heart Support: Because the vessels are wider, the right side of the heart does not have to work as hard to pump blood through the lungs.

By improving blood flow and reducing the workload on the heart, Uptravi helps to delay the progression of the disease and reduce the risk of being hospitalized for PAH symptoms.

Common Side Effects

Common side effects of Uptravi often occur when the dose is being increased. These may include:

• Headache
• Diarrhea
• Jaw pain
• Nausea and vomiting
• Muscle or joint pain
• Flushing (redness, warmth, or tingling)
• Pain in the arms or legs
• Decreased appetite
• Rash

Serious Side Effects

Seek medical attention immediately if you experience any of the following serious symptoms:

• Signs of pulmonary edema, such as severe shortness of breath or a cough with foamy mucus
• Signs of anemia, including pale skin, unusual weakness, or cold hands and feet
• Symptoms of a severe allergic reaction, such as hives, swelling of the face or throat, or trouble breathing

Drug Interactions

Uptravi must not be used with strong CYP2C8 inhibitors, such as gemfibrozil. Using these medications together can cause the levels of the drug to rise significantly in the bloodstream, which may lead to an increased risk of side effects.

Pulmonary Veno-Occlusive Disease

There is a risk of pulmonary edema in patients with pulmonary veno-occlusive disease (PVOD). If signs of fluid in the lungs develop, such as increased shortness of breath, healthcare providers will consider the possibility of PVOD. If this condition is confirmed, the medication should be stopped.

Liver Health

The liver is essential for processing this medication. For individuals with moderate liver impairment, the starting dose is typically lower, and dose increases occur less frequently. Use of this medication is generally avoided in those with severe liver impairment.

Pregnancy and Nursing

The safety of this medication during pregnancy or while breastfeeding has not been fully established. It should only be used if the potential benefit justifies the potential risk to the fetus or nursing infant.

Pregnancy

It is currently unknown if Uptravi will harm an unborn baby. There are no adequate and well-controlled studies of this medication in pregnant women to determine its safety. While animal studies using high doses did not show evidence of harm to the fetus, animal results do not always predict how a drug will affect humans. This medication should be used during pregnancy only if the potential benefit justifies the potential risk to the fetus.

Breastfeeding

It is not known whether Uptravi or its components pass into human breast milk. Because many drugs are excreted in human milk and there is a potential for adverse reactions in nursing infants, healthcare providers typically recommend weighing the benefits of breastfeeding against the potential risks. Some clinical guidance suggests that breastfeeding should be avoided while using this medication. Consult with your doctor to determine the safest course of action for you and your baby.

Dosage Forms

Uptravi is available in the following forms:

• Oral Tablets: These are film-coated tablets taken by mouth.
• Intravenous Injection: This is a powder for solution that is reconstituted and administered as an infusion into a vein.

The injection is typically used as a temporary replacement for patients who are unable to take the medication orally.

FDA Approval History

The U.S. Food and Drug Administration (FDA) first approved Uptravi (selexipag) tablets on December 21, 2015. It was introduced as a selective prostacyclin IP receptor agonist for patients with pulmonary arterial hypertension (PAH).

In July 2021, the FDA expanded the approval to include an intravenous (IV) formulation. This injection is designed for use in adult patients who are already prescribed Uptravi but are temporarily unable to take the medication by mouth.

Approved Indications

Uptravi is indicated for the long-term treatment of pulmonary arterial hypertension (WHO Group 1) in adults. The regulatory approval is based on its ability to:

• Delay the progression of the disease.
• Reduce the risk of hospitalization related to PAH.

It may be used as a standalone treatment or in combination with other types of PAH medications, such as endothelin receptor antagonists or phosphodiesterase-5 inhibitors.

Regulatory Status

Uptravi is available only by prescription. Unlike some other medications used for PAH, Uptravi does not currently require a Risk Evaluation and Mitigation Strategy (REMS) program or carry a Boxed Warning for severe safety risks. It is available in multiple tablet strengths to allow for gradual dose titration.

Factors Influencing Cost

Uptravi is a specialty medication used to treat pulmonary arterial hypertension. Because it is a specialty drug, it is often more expensive than standard medications and may require fulfillment through a specialty pharmacy. The total price you pay can be affected by several factors:

• Insurance Coverage: Your specific health insurance plan, including your deductible, co-pay, or co-insurance requirements, will significantly impact your out-of-pocket costs.
• Dosage and Form: Uptravi is available as oral tablets and as an intravenous injection. The dosage strength and frequency prescribed by your doctor will influence the overall cost.
• Pharmacy Choice: Prices may vary between different specialty pharmacies.

Financial Assistance Programs

The manufacturer of Uptravi offers support programs to help eligible patients manage the cost of the medication. These may include:

• Savings Cards: Commercially insured patients may be eligible for co-pay cards that reduce out-of-pocket expenses.
• Patient Assistance: For patients without insurance or those who are underinsured, the manufacturer may provide the drug at a reduced cost or for free through specific foundation programs.

It is recommended to contact your insurance provider or the manufacturer's support program to get the most accurate estimate of your costs.