Adempas riociguat tablets for PAH for CTEPH

Important Safety Information
Adempas can cause serious birth defects if taken during pregnancy. Females must not be pregnant when they start taking Adempas (riociguat) tablets or become pregnant during treatment with Adempas. Females who are able to get pregnant must have a negative pregnancy test before beginning treatment with Adempas, each month during treatment, and 1 month after you stop treatment with Adempas. Females can only receive Adempas through a restricted program called the Adempas REMS Program...Continue reading below.

What is Adempas?

Adempas is the first and only medicine approved by the Food and Drug Administration (FDA) for treating two types of pulmonary hypertension in adults:

  • Chronic thromboembolic pulmonary hypertension (CTEPH) (WHO Group 4) treated with surgery but who continue to have high pulmonary blood pressure (persistent) or it comes back after surgery (recurrent), or that cannot be treated with surgery.
    • CTEPH is a type of high blood pressure in the arteries of your lungs caused by blood clots that narrow or block blood flow. Adempas can improve your ability to exercise (measured by 6-minute walk distance) and can help to improve some of your symptoms.
  • Pulmonary arterial hypertension (PAH) (WHO Group 1)
    • PAH is a type of high blood pressure in the arteries of your lungs. Adempas can improve your ability to exercise (measured by 6-minute walk distance), improve some of your symptoms, and help slow down the worsening of your physical condition [this includes death, heart/lung transplant, a surgery to reduce pressure in your lung, hospitalization for worsening of pulmonary hypertension, start of a new PAH-specific treatment, continued decline in your ability to exercise (measured by 6-minute walk distance) and some of your symptoms].

The Adempas clinical studies included mostly patients with WHO functional class II-III symptoms. It is unknown if Adempas is safe and effective in children.

What is the most important information I should know about Adempas?

  • Serious birth defects.

Adempas can cause serious birth defects if taken during pregnancy.

  • Females must not be pregnant when they start taking Adempas or become pregnant during treatment with Adempas.
  • Females who are able to get pregnant must have a negative pregnancy test before beginning treatment with Adempas, each month during treatment, and 1 month after you stop treatment with Adempas. Talk to your doctor about your menstrual cycle. Your doctor will decide when to do the tests, and order the tests for you depending on your menstrual cycle.
    • Females who are able to get pregnant are females who:
      • Have entered puberty, even if they have not started their period, and
      • Have a uterus, and
      • Have not gone through menopause (have not had a period for at least 12 months for natural reasons, or have had their ovaries removed).
    • Females who are not able to get pregnant are females who:
      • Have not yet entered puberty, or
      • Do not have a uterus, or
      • Have gone through menopause (have not had a period for at least 12 months for natural reasons, or who have had their ovaries removed)

Females who are able to get pregnant must use two acceptable forms of birth control, during treatment with Adempas and for one month after stopping Adempas because the medicine may still be in the body.

  • If you have had a tubal sterilization, have a progesterone implant, or have an IUD (intrauterine device), these methods can be used alone and no other form of birth control is needed.
  • Talk with your doctor or gynecologist (a doctor who specializes in female reproduction) to find out about options for acceptable birth control that you may use to prevent pregnancy during treatment with Adempas.
  • If you decide that you want to change the form of birth control that you use, talk with your doctor or gynecologist to be sure that you choose another acceptable form of birth control.
  • Do not have unprotected sex. Talk to your doctor or pharmacist right away if you have unprotected sex or if you think your birth control has failed. Your doctor may talk with you about using emergency birth control.
  • Tell your doctor right away if you miss a menstrual period or think you may be pregnant for any reason.

If you are the parent or caregiver of a female child who started taking Adempas before reaching puberty, you should check your child regularly to see if she is developing signs of puberty. Tell your doctor right away if you notice that she is developing breast buds or any pubic hair. Your doctor should decide if your child has reached puberty. Your child may reach puberty before having her first menstrual period.

Females can only receive Adempas through a restricted program called the Adempas Risk Evaluation and Mitigation Strategies (REMS) Program. If you are a female who can become pregnant, you must talk to your doctor, understand the benefits and risks of Adempas, and agree to all of the instructions in the Adempas REMS Program. Males can receive Adempas without taking part in the Adempas REMS Program.

How far might Adempas take me?

Adempas can help increase your ability to walk farther as measured by a 6-minute walking test.

  • 443 adults taking Adempas for pulmonary arterial hypertension (PAH) (WHO Group 1) were able to walk 118 feet farther in a 6-minute walking test than patients taking a placebo (no Adempas) at the end of a 12-week study (-20 feet without Adempas therapy, +98 feet with Adempas therapy).
  • 261 adults taking Adempas for chronic thromboembolic pulmonary hypertension (CTEPH) (WHO Group 4) that was inoperable or recurrent/persistent after surgery were able to walk 147 feet farther in a 6-minute walking test than patients taking a placebo (no Adempas) at the end of a 16-week study (-20 feet without Adempas therapy, +127 feet with Adempas therapy).

The Adempas clinical studies included mostly patients with WHO Functional Class II–III symptoms. It is not known if Adempas is safe and effective in children.

Who can fill my Adempas prescription?

Adempas will be provided to you by a certified pharmacy. Your doctor will give you complete details.

What special information should I share with my doctor before taking Adempas?

Before you take Adempas, tell your doctor if you:

  • smoke; have recently had serious bleeding from your lungs, or if you have had a medical procedure called bronchial arterial embolization to stop you from coughing up blood; have problems with your heart or blood circulation; have low blood pressure; have liver problems; have kidney problems or are on dialysis; have narrowing of the pulmonary veins, a condition called pulmonary veno-occlusive disease or PVOD; have any other medical conditions.

Tell your doctor about all the medicines you take, including prescription and non-prescription medicines, vitamins, and herbal supplements. Adempas and other medicines may affect each other causing side effects. Do not start any new medicine until you check with your doctor.

How often do I take Adempas?

Take Adempas 3 times each day, about 6 to 8 hours apart, with or without food. Do not take more than a total of 7.5 mg of Adempas in one day unless your doctor tells you to. Do not take Adempas within 24 hours of sildenafil. Do not take Adempas 24 hours before or within 48 hours after tadalafil. Always take Adempas exactly as your doctor tells you. Don’t stop taking it or change your dose without talking to your doctor.

What if I miss a dose?

If you miss a dose, take your next dose at the regular time.

What if I miss several days?

Call your doctor if you miss 3 or more days of Adempas. Don't stop taking it or change your dose without talking to your doctor.

Can I take Adempas with heartburn medicine?

If you take a heartburn medicine (antacid) that contains aluminum hydroxide or magnesium hydroxide, do not take it within 1 hour of taking Adempas.

Could my doctor prescribe other medications for PAH with Adempas?

Adempas can be taken alone or together with endothelin receptor antagonists or prostanoids, which are medications your doctor may also prescribe for PAH. Do not take Adempas if you take certain other medicines used to treat PAH, called Revatio (sildenafil) and Adcirca (tadalafil).

Who should not take Adempas?

Do not take Adempas if:

  • you are pregnant, plan to become pregnant, or become pregnant during treatment with Adempas. Adempas can cause serious birth defects.
  • you take:
    • a nitrate medicine to treat high blood pressure or heart disease, such as nitroglycerin, or a medicine called a nitric oxide donor, such as amyl nitrite
    • certain other medicines that contain sildenafil (Revatio or Viagra), tadalafil (Adcirca or Cialis), vardenafil (Levitra or Staxyn), dipyridamole, or theophylline. Revatio and Adcirca are also used to treat PAH
  • you have pulmonary hypertension associated with idiopathic interstitial pneumonias (PH-IIP).

Ask your doctor or pharmacist if you are not sure if you take any of the medicines listed above.

What dose of Adempas will I take?

The Adempas starting dose is 0.5 mg or 1 mg. During the first weeks, your doctor may increase your dose. When you begin treatment, your blood pressure should be monitored about every 2 weeks to help your doctor decide the correct dose of medicine for you. Do not take Adempas within 24 hours of sildenafil. Do not take Adempas 24 hours before or within 48 hours after tadalafil. Always take Adempas exactly as your doctor tells you. Don’t stop taking it or change your dose without talking to your doctor.

When do I need to call my doctor?

Call your doctor if:

  • You have symptoms of low blood pressure such as dizziness, lightheadedness, or fainting
  • You ever experience any side effects
  • You take too much Adempas
  • You miss 3 or more days of Adempas
  • You stop smoking or start smoking
  • You are a female patient and miss a menstrual period, think you may be pregnant, or have questions about feeding your baby (it is not known if Adempas passes into your breast milk)

Do not take Adempas within 24 hours of sildenafil. Do not take Adempas 24 hours before or within 48 hours after tadalafil.

However, always take Adempas exactly as your doctor tells you. Don’t stop taking it or change your dose without talking to your doctor.

What should I avoid while taking Adempas?

  • Do not get pregnant while taking Adempas. If you miss a menstrual period, or think you might be pregnant, call your doctor right away.
  • It is not known if Adempas passes into your breast milk. You should not breastfeed if you take Adempas. Talk to your doctor about the best way to feed your baby if you take Adempas.
  • Adempas may make you feel dizzy. Do not drive, operate machinery, or do other activities that require mental alertness or coordination until you know how Adempas affects you. Talk with your doctor if you are concerned about when it is safe for you to do these activities.
  • Smoking. Adempas may not work as well if you smoke during treatment. Tell your doctor if you stop smoking or start smoking during treatment with Adempas, because your dose of Adempas may need to be changed.

What is pulmonary hypertension (PH)?

Pulmonary arterial hypertension (PAH) (WHO Group 1) and chronic thromboembolic pulmonary hypertension (CTEPH) (WHO Group 4) which is either inoperable or recurrent/persistent after surgery are two different types of pulmonary hypertension (PH). The Adempas clinical studies included mostly patients with WHO Functional Class II–III symptoms.

Pulmonary hypertension (PH) is the name of the condition that causes people to have high blood pressure in the blood vessels of their lungs. Smaller openings or blockages in blood vessels make it more difficult for blood to get to the lungs. Because of this increased pressure, the heart has to pump harder and harder.

Over time, increased pressure in blood vessels can cause the right side of the heart to get bigger and weaker. When the right side of the heart is weakened, it has a harder time pumping enough blood and oxygen to the left side of the heart and the rest of the body.

What is PAH?

PAH is a type of high blood pressure in the arteries (blood vessels) of your lungs. An artery is a blood vessel that carries blood away from the heart. The pulmonary arteries carry blood from the heart to the lungs.

This high blood pressure is caused when the openings in the blood vessels of the lungs get smaller and smaller. These smaller openings can be caused by the following:

  • The walls of the arteries tightening
  • The walls of the arteries becoming stiff and narrow from an overgrowth of cells

How is PAH diagnosed?

Some of the tests used to diagnose PAH are echocardiography (images produced by sound waves bounced off the heart and lungs) and right heart catheterization (a tube inserted in the pulmonary artery that measures blood pressure).

Can PAH be treated?

The increased pressure in the pulmonary arteries strains the right side of the heart and it begins to fail. There is no cure for PAH, but several medications are available to help slow the progression of changes in the pulmonary arteries and help reduce symptoms. However, even with medication, about half of patients are still in WHO Functional Class III.

To treat your PAH (WHO Group 1), your doctor may give you Adempas. Adempas is a prescription medicine used to treat adults with PAH (WHO Group 1). The Adempas clinical studies included mostly patients with WHO Functional Class II–III symptoms.

What is CTEPH?

CTEPH is a type of high blood pressure in the arteries (blood vessels) of your lungs caused by blood clots that narrow or block blood flow. In most cases of CTEPH, blood clots in the blood vessels of your legs break off and travel to the lungs. Sometimes, however, blood clots or tissue growth may form in the blood vessels of the lungs. Over time, extra tissue forms over these clots and creates a blockage that can’t be removed with blood-thinner drugs alone.

How is CTEPH diagnosed?

Some of the tests used to diagnose CTEPH are echocardiography, right heart catheterization, ventilation/perfusion (V/Q) scan (measures the amount of air and blood flowing through the lungs), and computed tomography (CT) angiography (computerized imaging of the blood vessels in the heart and lungs).

Can CTEPH be treated?

CTEPH may be treated with an operation called pulmonary endarterectomy (shortened as PEA). During this very specialized operation, clots and other blockages of the pulmonary arteries are removed. CTEPH is potentially cured after the surgical procedure.

However, if you are not a candidate for PEA surgery, or your high pulmonary blood pressure continued (persistent) or came back (recurrent) after PEA surgery, your doctor can treat your CTEPH (WHO Group 4) with Adempas. The Adempas clinical studies included mostly patients with WHO Functional Class II–III symptoms.

Can PAH and CTEPH become more serious?

As PAH and CTEPH progress, symptoms get worse. The World Health Organization (WHO) developed a scale for doctors to rate the severity of PAH and CTEPH symptoms. Your doctor will determine which WHO Functional Class (FC) you belong to. A lower class number (I or II) means that you have less severe symptoms. Identifying your WHO FC is one of the ways your doctor determines your best course of treatment.

What serious side effects should I be aware of?

Adempas can cause serious side effects including:

  • Serious birth defects.
  • Reduced blood pressure. This may cause symptoms of low blood pressure, such as lightheadedness, chest pain, and dizziness especially in people who are dehydrated, have a severe blockage of blood flow out of the heart, or have certain other medical problems. Your doctor will check you for any of these problems.
  • Increased risk of bleeding, including bleeding from the respiratory tract. Tell your doctor right away if you cough up blood during treatment with Adempas.
  • Worsening of symptoms in people with Pulmonary Veno-Occlusive Disease (PVOD). If you have PVOD, treatment with Adempas may cause a build-up of fluid in your lungs (pulmonary edema) which may result in shortness of breath. Your doctor may tell you to stop taking Adempas and switch you to a different medicine.

What are the most common side effects?

The most common side effects of Adempas are:

  • Headache; dizziness; indigestion; swelling of your hands, legs, feet, and ankles (peripheral edema); nausea; diarrhea; and vomiting

Tell your doctor if you have any side effect that bothers you or that does not go away. These are not all the possible side effects of Adempas.

What nursing services are available through the Aim Program?

As directed by your physician, you can access the assistance of nurses affiliated with your specialty pharmacy in two ways:

Toll-free Assistance, 24/7

  • Representatives on-call to address your questions
  • Provided by your specialty pharmacy

At-home Nurse Visits

  • Administer a clinical assessment
  • Follow-up on your specific needs
  • Provide Adempas information

What financial assistance services does the Aim Program offer?

The Aim Support Center gives you access to a variety of financial assistance programs.

Co-pay Assistance Program*

  • Up to 100% coverage of co-pays or coinsurance directly to the insurer

*Patients must have private commercial insurance with a percentage coinsurance or co-pay requirement. Medicare or Medicaid patients are ineligible. Assistance is for one year, after which, patients must re-apply. Patients must notify the program of any change in their insurance status. Patients in certain states may be ineligible. The co-pay assistance program has an annual cap per patient year.

Temporary Patient Assistance Program (TPAP)

  • For temporary situations and uninsured or underinsured patients

Patient Assistance Program (PAP)

  • Uninsured or underinsured patients can apply to receive medicine free of cost

Additional Resources

  • Lab coordination services (for females of reproductive potential only) to assist with scheduling and reminders


Important Safety Information

Adempas can cause serious birth defects if taken during pregnancy.

  • Females must not be pregnant when they start taking Adempas (riociguat) tablets or become pregnant during treatment with Adempas.
  • Females who are able to get pregnant must have a negative pregnancy test before beginning treatment with Adempas, each month during treatment, and 1 month after you stop treatment with Adempas. Talk to your doctor about your menstrual cycle. Your doctor will decide when to do the test.
    • Females who are able to get pregnant are females who:
      • Have entered puberty, even if they have not started their period, have a uterus, and have not gone through menopause (have not had a period for at least 12 months for natural reasons, or have had their ovaries removed).
    • Females who are not able to get pregnant are females who:
      • Have not yet entered puberty, do not have a uterus, or have gone through menopause.

Females who are able to get pregnant must use two acceptable forms of birth control, during Adempas treatment and for one month after stopping Adempas.

  • If you have had a tubal sterilization, have a progesterone implant, or have an IUD (intrauterine device), these methods can be used alone and no other form of birth control is needed.
  • Talk with your doctor or gynecologist (a doctor who specializes in female reproduction) to find out how to prevent pregnancy during treatment with Adempas.
    • Do not have unprotected sex. Talk to your doctor or pharmacist right away if you have unprotected sex or if you think your birth control has failed. Your doctor may tell you to use emergency birth control.
    • Tell your doctor right away if you miss a menstrual period or think you may be pregnant for any reason.

If you are the parent or caregiver of a female child who started taking Adempas before reaching puberty, you should check your child regularly to see if she is developing signs of puberty. Tell your doctor right away if you notice that she is developing breast buds or any pubic hair. Your doctor should decide if your child has reached puberty. Your child may reach puberty before having her first menstrual period.

Females can only receive Adempas through a restricted program called the Adempas Risk Evaluation and Mitigation Strategies (REMS) program. If you are a female who can become pregnant, you must talk to your doctor, understand the benefits and risks of Adempas, and agree to all of the instructions in the Adempas REMS program. Males can receive Adempas without taking part in the Adempas REMS program.

Do not take Adempas if:

  • you are pregnant, plan to become pregnant, or become pregnant during treatment with Adempas. Adempas can cause serious birth defects.
  • you take:
    • a nitrate medicine to treat high blood pressure or heart disease, such as nitroglycerin, or a medicine called a nitric oxide donor, such as amyl nitrite
    • certain other medicines that contain sildenafil (Revatio or Viagra), tadalafil (Adcirca or Cialis), vardenafil (Levitra or Staxyn), dipyridamole, or theophylline. Revatio and Adcirca are also used to treat PAH
  • you have pulmonary hypertension associated with idiopathic interstitial pneumonias (PH-IIP).

Ask your doctor or pharmacist if you are not sure if you take any of the medicines listed above.

Before you take Adempas, tell your doctor if you:

  • smoke; have recently had serious bleeding from your lungs, or if you have had a medical procedure called bronchial arterial embolization to stop you from coughing up blood; have problems with your heart or blood circulation; have low blood pressure; have liver problems; have kidney problems or are on dialysis; have narrowing of the pulmonary veins, a condition called pulmonary veno-occlusive disease or PVOD; have any other medical conditions.

Tell your doctor about all the medicines you take, including prescription and non-prescription medicines, vitamins, and herbal supplements. Adempas and other medicines may affect each other causing side effects. Do not start any new medicine until you check with your doctor.

What should I avoid while taking Adempas?

  • Do not get pregnant while taking Adempas. If you miss a menstrual period, or think you might be pregnant, call your doctor right away.
  • It is not known if Adempas passes into your breast milk. You should not breastfeed if you take Adempas. Talk to your doctor about the best way to feed your baby if you take Adempas.
  • Adempas may make you feel dizzy. Do not drive, operate machinery, or do other activities that require mental alertness or coordination until you know how Adempas affects you. Talk with your doctor if you are concerned about when it is safe for you to do these activities.
  • Smoking. Adempas may not work as well if you smoke during treatment. Tell your doctor if you stop smoking or start smoking during treatment with Adempas, because your dose of Adempas may need to be changed.

Adempas can cause serious side effects including:

  • Serious birth defects.
  • Reduced blood pressure. This may cause symptoms of low blood pressure, such as lightheadedness, chest pain, and dizziness especially in people who are dehydrated, have a severe blockage of blood flow out of the heart, or have certain other medical problems. Your doctor will check you for any of these problems.
  • Increased risk of bleeding, including bleeding from the respiratory tract. Tell your doctor right away if you cough up blood during treatment with Adempas.
  • Worsening of symptoms in people with Pulmonary Veno-Occlusive Disease (PVOD). If you have PVOD, treatment with Adempas may cause a build-up of fluid in your lungs (pulmonary edema) which may result in shortness of breath. Your doctor may tell you to stop taking Adempas and switch you to a different medicine.

The most common side effects of Adempas are:

  • Headache; dizziness; indigestion; swelling of your hands, legs, feet, and ankles (peripheral edema); nausea; diarrhea; and vomiting

Tell your doctor if you have any side effect that bothers you or that does not go away. These are not all the possible side effects of Adempas.

Indication

Adempas is a prescription medicine used to treat adults with:

  • chronic thromboembolic pulmonary hypertension (CTEPH) (WHO Group 4) treated with surgery but who continue to have high pulmonary blood pressure (persistent) or it comes back after surgery (recurrent), or that cannot be treated with surgery.
    • CTEPH is a type of high blood pressure in the arteries of your lungs caused by blood clots that narrow or block blood flow. Adempas can improve your ability to exercise (measured by 6-minute walk distance) and can help to improve some of your symptoms.
  • pulmonary arterial hypertension (PAH) (WHO Group 1)
    • PAH is a type of high blood pressure in the arteries of your lungs. Adempas can improve your ability to exercise (measured by 6-minute walk distance), improve some of your symptoms, and help slow down the worsening of your physical condition [this includes death, heart/lung transplant, a surgery to reduce pressure in your lung, hospitalization for worsening of pulmonary hypertension, start of a new PAH-specific treatment, continued decline in your ability to exercise (measured by 6-minute walk distance) and some of your symptoms].

The Adempas clinical studies included mostly patients with WHO functional class II-III symptoms. It is unknown if Adempas is safe and effective in children.

For important risk and use information please see the full Prescribing Information, including Boxed Warning and Medication Guide.