Usual Adult Dose for Pulmonary Hypertension

Initial dose: 2.5 mcg inhaled orally once; if tolerated, increase to 5 mcg and maintain this dose
Maintenance dose: 2.5 or 5 mcg inhaled orally 6 to 9 times per day, no more than once every 2 hours while awake, based on individual need and tolerability
Maximum dose: 45 mcg/day (5 mcg/dose 9 times per day)

Comment: Avoid initiation of therapy in patients with systolic blood pressure below 85 mmHg.

Use: For the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to improve a composite endpoint consisting of exercise tolerance, symptoms (New York Heart Association [NYHA] Class), and lack of deterioration. Studies establishing effectiveness included predominately patients with NYHA Functional Class III-IV symptoms and etiologies of idiopathic or heritable PAH (65%) or PAH associated with connective tissue diseases (23%)

Renal Dose Adjustments

Renal dysfunction (not on dialysis): No adjustment recommended.

Liver Dose Adjustments

Child-Pugh Class A liver dysfunction: Data not available
Child-Pugh Class B or C liver dysfunction: Consider increasing dosing interval (e.g., 3 to 4 hours between doses) based on the patient's response at the end of the dose interval

Dose Adjustments

Elderly: Cautious dose selection recommended due to increased likelihood of concomitant hepatic, renal, or cardiac dysfunctions or concomitant disease or other drug therapy.

Precautions

Safety and efficacy have not been established in patients younger than 18 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• For oral inhalation using the I-neb(R) AAD(R) System.
• To avoid potential interruptions in drug delivery, the patient should have immediate access to a backup I-neb(R) AAD(R) System.
• Nebulizer solution should not come into contact with the skin or eyes; oral ingestion should be avoided.

• Do not mix with other medication in the I-neb(R) AAD(R) System.
• For each inhalation session, the entire contents of the ampule should be transferred into the medication chamber immediately before use.
• After each inhalation session, any solution remaining in the medication chamber should be discarded.

• The 20 mcg/mL concentration should be reserved for use in patients who are maintained at the 5 mcg dose and who have repeatedly experienced extended treatment times which could result in incomplete dosing.
• Transitioning patients to the 20 mcg/mL concentration will decrease treatment times to help maintain patient compliance.

• General: Monitor vital signs during initiation.

• Follow the manufacturer instructions for proper administration techniques, including dosing frequency, ampule dispensing, and I-neb(R) AAD(R) System operation and equipment cleaning.
• Stand up slowly as a fall I blood pressure may occur, causing dizziness and possible fainting.
• Consult your physician if fainting gets worse.