Uptravi 200/800mcg Therapy Pack
It is used to treat high blood pressure in the lungs.
Drug Class
Agents for pulmonary hypertension
Pharmacologic Class
Prostacyclin IP receptor agonist
Pregnancy Category
Not available
FDA Approved
Dec 2015
DEA Schedule
Not Controlled
Overview
What is this medicine?
Uptravi is a medication used to treat pulmonary arterial hypertension (PAH), a condition where blood pressure in the arteries leading to your lungs is too high. It works by relaxing the blood vessels in your lungs, making it easier for your heart to pump blood and improving your ability to exercise.
How to Use This Medicine
Taking Your Medication
To use this medication correctly, follow your doctor's instructions and read all the information provided. You can take this medication with or without food, but taking it with food may help minimize some side effects. It's essential to swallow the medication whole, without chewing, breaking, or crushing it.
Continue taking this medication as directed by your doctor or healthcare provider, even if you're feeling well. This will help ensure you receive the full benefits of the treatment.
Storing and Disposing of Your Medication
Store your medication at room temperature in a dry place, avoiding the bathroom. Keep all medications in a safe location, out of the reach of children and pets. When disposing of unused or expired medications, do not flush them down the toilet or pour them down the drain unless instructed to do so. Instead, check with your pharmacist for guidance on the best disposal method. You may also want to explore local drug take-back programs for a safe and environmentally friendly way to dispose of your medications.
Missing a Dose
If you miss a dose, take it as soon as you remember. However, if it's less than 6 hours until your next scheduled dose, skip the missed dose and resume your regular dosing schedule. Do not take two doses at the same time or take extra doses. If you miss three consecutive days of medication, contact your doctor for advice on how to proceed.
To use this medication correctly, follow your doctor's instructions and read all the information provided. You can take this medication with or without food, but taking it with food may help minimize some side effects. It's essential to swallow the medication whole, without chewing, breaking, or crushing it.
Continue taking this medication as directed by your doctor or healthcare provider, even if you're feeling well. This will help ensure you receive the full benefits of the treatment.
Storing and Disposing of Your Medication
Store your medication at room temperature in a dry place, avoiding the bathroom. Keep all medications in a safe location, out of the reach of children and pets. When disposing of unused or expired medications, do not flush them down the toilet or pour them down the drain unless instructed to do so. Instead, check with your pharmacist for guidance on the best disposal method. You may also want to explore local drug take-back programs for a safe and environmentally friendly way to dispose of your medications.
Missing a Dose
If you miss a dose, take it as soon as you remember. However, if it's less than 6 hours until your next scheduled dose, skip the missed dose and resume your regular dosing schedule. Do not take two doses at the same time or take extra doses. If you miss three consecutive days of medication, contact your doctor for advice on how to proceed.
Lifestyle & Tips
- Take the medication exactly as prescribed by your doctor, usually twice a day.
- Do not stop taking Uptravi suddenly, as this can worsen your condition.
- If you miss a dose, take it as soon as you remember, unless it's almost time for your next dose. Do not double your dose.
- Store at room temperature, away from moisture and heat.
- Report any new or worsening symptoms to your doctor immediately.
Available Forms & Alternatives
Dosing & Administration
Adult Dosing
Standard Dose:
Initial 200 mcg orally twice daily, titrated weekly in increments of 200 mcg twice daily to the highest tolerated dose up to 1600 mcg twice daily.
Dose Range:
200 - 1600 mg
Condition-Specific Dosing:
titration:
Titration should be done in increments of 200 mcg twice daily, usually at weekly intervals, to the highest tolerated dose. The 200/800mcg therapy pack facilitates this titration.
Pediatric Dosing
Neonatal:
Not established
Infant:
Not established
Child:
Not established
Adolescent:
Not established
Dose Adjustments
Renal Impairment:
Mild:
No dose adjustment required.
Moderate:
No dose adjustment required.
Severe:
Not studied in patients with severe renal impairment (CrCl < 30 mL/min) or end-stage renal disease requiring dialysis. Avoid use.
Dialysis:
Not studied in patients requiring dialysis. Avoid use.
Hepatic Impairment:
Mild:
No dose adjustment required (Child-Pugh A).
Moderate:
Initial dose of 200 mcg orally once daily. Titrate carefully. Maximum dose 800 mcg twice daily (Child-Pugh B).
Severe:
Initial dose of 200 mcg orally once daily. Titrate carefully. Maximum dose 400 mcg twice daily (Child-Pugh C).
Confidence:
High
Pharmacology
Mechanism of Action
Selexipag is an orally available, selective prostacyclin IP receptor agonist. It is hydrolyzed by carboxylesterase 1 (CES1) to its active metabolite, ACT-333679. Activation of the IP receptor leads to vasodilation, inhibition of platelet aggregation, and antiproliferative effects, which are beneficial in pulmonary arterial hypertension (PAH).
Pharmacokinetics
Absorption:
Bioavailability:
Approximately 49% (selexipag), approximately 62% (active metabolite)
Tmax:
1-2 hours (selexipag), 3-4 hours (active metabolite)
FoodEffect:
High-fat meal decreases Cmax of selexipag but increases Cmax of active metabolite; AUC is similar. Can be taken with or without food.
Distribution:
Vd:
11.5 L (selexipag), 16.7 L (active metabolite)
ProteinBinding:
>99% for both selexipag and its active metabolite
CnssPenetration:
Limited
Elimination:
HalfLife:
1.3-2.2 hours (selexipag), 6.2-13.5 hours (active metabolite)
Clearance:
Not available
ExcretionRoute:
Primarily fecal (approximately 93%), minor renal (approximately 7%)
Unchanged:
<1% (in urine)
Pharmacodynamics
OnsetOfAction:
Not precisely defined, effects are observed with chronic dosing
PeakEffect:
Not precisely defined, effects are observed with chronic dosing
DurationOfAction:
Sustained with twice-daily dosing
Safety & Warnings
Side Effects
Urgent Side Effects: Seek Medical Help Right Away
If you experience any of the following symptoms, call your doctor or seek medical attention immediately, as they may be signs of a severe and potentially life-threatening reaction:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
New or worsening trouble breathing
Wheezing or coughing
Feeling extremely tired or weak
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only mild ones, it's essential to discuss any concerns with your doctor. If you experience any of the following side effects or any other unusual symptoms that bother you or do not go away, contact your doctor:
Headache
Diarrhea
Upset stomach or vomiting
Jaw pain
Muscle or joint pain
Pain in arms or legs
Flushing
Decreased appetite
Reporting Side Effects
This list is not exhaustive, and you may experience other side effects. If you have questions or concerns, call your doctor for medical advice. You can also report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
If you experience any of the following symptoms, call your doctor or seek medical attention immediately, as they may be signs of a severe and potentially life-threatening reaction:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
New or worsening trouble breathing
Wheezing or coughing
Feeling extremely tired or weak
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only mild ones, it's essential to discuss any concerns with your doctor. If you experience any of the following side effects or any other unusual symptoms that bother you or do not go away, contact your doctor:
Headache
Diarrhea
Upset stomach or vomiting
Jaw pain
Muscle or joint pain
Pain in arms or legs
Flushing
Decreased appetite
Reporting Side Effects
This list is not exhaustive, and you may experience other side effects. If you have questions or concerns, call your doctor for medical advice. You can also report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
Seek Immediate Medical Attention If You Experience:
- Severe headache
- Severe jaw pain
- Persistent or severe nausea, vomiting, or diarrhea
- Dizziness or fainting (signs of low blood pressure)
- Unusual bleeding or bruising
- Signs of liver problems (e.g., yellowing of skin/eyes, dark urine, severe stomach pain)
- Allergic reaction (e.g., rash, itching, swelling, severe dizziness, trouble breathing)
Before Using This Medicine
Before Taking This Medication: Important Information to Share with Your Doctor
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, its components, or other substances, such as foods or drugs. Be sure to describe the allergic reaction you experienced, including any symptoms that occurred.
If you have liver disease, as this may affect the safety and efficacy of the medication.
* If you are currently taking gemfibrozil, as this can interact with the medication.
This list is not exhaustive, and it is crucial to disclose all your medications, including prescription and over-the-counter drugs, natural products, and vitamins, as well as any health problems you have. Your doctor and pharmacist need this information to ensure safe treatment. Do not initiate, discontinue, or modify the dose of any medication without first consulting your doctor to confirm that it is safe to do so in conjunction with this medication.
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, its components, or other substances, such as foods or drugs. Be sure to describe the allergic reaction you experienced, including any symptoms that occurred.
If you have liver disease, as this may affect the safety and efficacy of the medication.
* If you are currently taking gemfibrozil, as this can interact with the medication.
This list is not exhaustive, and it is crucial to disclose all your medications, including prescription and over-the-counter drugs, natural products, and vitamins, as well as any health problems you have. Your doctor and pharmacist need this information to ensure safe treatment. Do not initiate, discontinue, or modify the dose of any medication without first consulting your doctor to confirm that it is safe to do so in conjunction with this medication.
Precautions & Cautions
It is essential to inform all your healthcare providers, including doctors, nurses, pharmacists, and dentists, that you are taking this medication. If you are pregnant, planning to become pregnant, or are breast-feeding, be sure to discuss this with your doctor. You and your doctor will need to carefully weigh the benefits and risks of this medication to both you and your baby.
Overdose Information
Overdose Symptoms:
- Severe headache
- Flushing
- Nausea
- Vomiting
- Diarrhea
- Hypotension (low blood pressure)
- Dizziness
What to Do:
In case of suspected overdose, seek immediate medical attention or call a Poison Control Center (1-800-222-1222). Management is supportive.
Drug Interactions
Contraindicated Interactions
- Strong CYP2C8 inhibitors (e.g., gemfibrozil)
Major Interactions
- Strong CYP2C8 inducers (e.g., rifampin) - may reduce efficacy
Moderate Interactions
- Moderate CYP2C8 inhibitors (e.g., clopidogrel, deferasirox) - monitor for adverse effects
- Moderate CYP3A4 inhibitors/inducers - less significant interaction
Confidence Interactions
Monitoring
Baseline Monitoring
Diagnosis of PAH
Rationale: Confirm appropriate indication for therapy.
Timing: Prior to initiation
Liver function tests (LFTs)
Rationale: Assess baseline hepatic function, especially important for dose adjustments in hepatic impairment.
Timing: Prior to initiation
Renal function (CrCl)
Rationale: Assess baseline renal function, important for dose considerations in severe impairment.
Timing: Prior to initiation
Routine Monitoring
Symptoms of PAH (e.g., dyspnea, exercise capacity)
Frequency: Regularly, as clinically indicated
Target: Improvement or stabilization
Action Threshold: Worsening symptoms may indicate need for dose adjustment or alternative therapy
Blood pressure and heart rate
Frequency: Regularly, especially during titration
Target: Within patient's normal range, avoid symptomatic hypotension
Action Threshold: Symptomatic hypotension or significant bradycardia/tachycardia
Liver function tests (LFTs)
Frequency: Periodically, especially in patients with hepatic impairment or if symptoms of liver injury develop
Target: Within normal limits or stable
Action Threshold: Significant elevation of liver enzymes
Symptom Monitoring
- Headache
- Jaw pain
- Nausea
- Vomiting
- Diarrhea
- Flushing
- Myalgia
- Arthralgia
- Hypotension
- Anemia
- Rash
Special Patient Groups
Pregnancy
There are no adequate and well-controlled studies of selexipag in pregnant women. Animal studies have shown adverse effects on embryo-fetal development. Use during pregnancy only if the potential benefit justifies the potential risk to the fetus.
Trimester-Specific Risks:
First Trimester:
Potential for embryo-fetal toxicity observed in animal studies.
Second Trimester:
Potential for embryo-fetal toxicity observed in animal studies.
Third Trimester:
Potential for embryo-fetal toxicity observed in animal studies.
Lactation
It is not known whether selexipag or its active metabolite are excreted in human milk. Due to the potential for serious adverse reactions in breastfed infants, advise women not to breastfeed during treatment with selexipag.
Infant Risk:
Unknown, but potential for serious adverse reactions.
Pediatric Use
Safety and effectiveness in pediatric patients have not been established. Use is not recommended.
Geriatric Use
No specific dose adjustment is required based on age alone. However, geriatric patients may have increased susceptibility to adverse reactions due to comorbidities or concomitant medications. Monitor carefully.
Clinical Information
Clinical Pearls
- Uptravi titration is crucial for patient tolerability. Patients often experience dose-dependent side effects (e.g., headache, jaw pain, nausea, diarrhea, flushing) during titration.
- The 200/800mcg therapy pack is designed to facilitate the initial titration phase, providing different strengths to reach the optimal tolerated dose.
- Do not interrupt Uptravi therapy abruptly, as this can lead to worsening of PAH symptoms.
- Patients should be educated on the importance of consistent dosing and reporting side effects to their healthcare provider for dose adjustment guidance.
- Concomitant use with strong CYP2C8 inhibitors (like gemfibrozil) is contraindicated due to significantly increased exposure of the active metabolite, leading to increased risk of adverse events.
Alternative Therapies
- Other prostacyclin pathway agents (e.g., epoprostenol, treprostinil, iloprost)
- Endothelin receptor antagonists (ERAs) (e.g., ambrisentan, bosentan, macitentan)
- Phosphodiesterase-5 (PDE5) inhibitors (e.g., sildenafil, tadalafil)
- Soluble guanylate cyclase (sGC) stimulators (e.g., riociguat)
Cost & Coverage
Average Cost:
Highly variable, typically >$10,000 per 60 tablets (e.g., 800mcg)
Insurance Coverage:
Specialty Tier (requires prior authorization, often subject to high co-pays or co-insurance)
General Drug Facts
If your symptoms or health issues persist or worsen, it's essential to contact your doctor for further guidance. To ensure your safety, never share your medication with others or take someone else's medication. Some medications may come with an additional patient information leaflet, so be sure to check with your pharmacist for more information. If you have any questions or concerns about this medication, don't hesitate to reach out to your doctor, nurse, pharmacist, or other healthcare provider. In the event of a suspected overdose, immediately call your local poison control center or seek medical attention. When seeking help, be prepared to provide details about the medication taken, the amount, and the time it happened.