Adempas riociguat tablets for PAH for CTEPH

Bayer

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.

Marsha's Journey
Terri's Journey

Click through each chapter below to learn about Marsha’s Journey:

I could do anything.
And then things
changed.

Marsha’s life before CTEPH

I could do anything.
And then things
changed.

Marsha is a nurse who works in the emergency department of a hospital. Before she was diagnosed with CTEPH, she was a single mother of three children—the youngest still at home, while the others had made it out on their own. “I think most people would say I was just extremely energetic,” she says. “I could pretty much do whatever I needed to do, when I needed to do it.”

Raising three kids by herself was challenging, and her job was equally so. She relied on good stamina and a lack of physical constraints when it came to her work as a nurse. “I could transport people around on carts and beds and wheelchairs and I could do anything. And then things changed.”

The winding road to diagnosis

I was just very limited, and they [her coworkers] were very kind and were working around my physical limitations.

Before going to the ER, Marsha thought she had a collapsed lung that turned out to be a pulmonary embolism (PE). She was prescribed blood thinners for nine months and felt fine when her time on them ended.

But two years after the PE, she felt a change. Her stamina was failing, and she was having trouble walking. She called her doctor repeatedly, but wasn’t getting a response. Finally, she got an appointment. “He told me I had anxiety and prescribed me some anxiety medicine,” she recalls. “But I knew something else was wrong.”

The anxiety medication didn’t improve her condition, and her stamina continued to deteriorate. When she went to work one day and could barely walk, her coworker insisted on her seeing a Specialist. Upon examination, it was discovered that she had another PE—one that she, in her words, “did not bounce back from.”

She continued to work, with difficulty. “I was just very limited, and they [her coworkers] were very kind and were working around my physical limitations,” she said. One of her colleagues, an ER physician, suggested she see a pulmonologist, and she decided to give it a try. A battery of tests were conducted, including a right heart catheterization, and Marsha was diagnosed with pulmonary arterial hypertension (PAH), (WHO Group 1). “I feel like that pulmonologist really kind of saved my life,” she said.

I was just very limited, and they [her coworkers] were very kind and were working around my physical limitations.
I realized that I hadn’t had a V/Q scan yet, and once I did, my doctor discovered that I had CTEPH rather than PAH.
I realized that I hadn’t had a V/Q scan yet, and once I did, my doctor discovered that I had CTEPH rather than PAH.

She began routine medications for PAH and slowly began to gain back some stamina. “I still was having problems, like pushing carts, and anything on an incline.” Still, she felt better than she did before her diagnosis with PAH, and she continued to take her medications.

After being a single mother for fourteen years, Marsha remarried. She moved near a large city and transferred her care to a hospital that had a pulmonary hypertension program. She attended a pulmonary hypertension event with expert thought leaders, and that’s when she heard about CTEPH (WHO Group 4) for the first time.

“I did research and learned more about CTEPH and the different types of PH,” she said. “I realized that I hadn’t had a V/Q scan yet, and once I did, my doctor discovered that I had CTEPH rather than PAH.” A V/Q scan (along with other assessments), as Marsha learned, is the preferred and recommended screening test for CTEPH. It’s the most accurate scan available for CTEPH, with a screening sensitivity greater than 96%.

Once diagnosed with CTEPH, Marsha was assessed for surgery and determined inoperable because of how deep her clots were in her lungs. Her medications were updated, and she was prescribed the only approved medical therapy for CTEPH: Adempas (riociguat).

Continue Reading

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.

Living with CTEPH

I kind of know what my new normal is, and I avoid certain things.
I kind of know what my new normal is, and I avoid certain things.

After beginning therapy with Adempas, Marsha started to see improvements in some of her symptoms. “When my doctor evaluated me and told me my functional class had improved, I felt better because I was improving.”

She has continued to improve on other measures, such as her six-minute walk test, but still avoids stairs at work. “I kind of know what my new normal is, and I avoid certain things,” she says.

Marsha’s family is understanding of her illness, and her life at home with her husband is supportive. “It’s kind of odd because my husband — since we’ve been married for three and a half years — he’s never known me any different. He might not have liked the old me. I would have left him in the dust maybe! But he’s very, very understanding of it.” She is also close with her daughter and talks with her on the phone multiple times a day.

“We’re like best friends. I think we kind of were before, but maybe more so now—very close.”

Throughout her journey with CTEPH, Marsha has set goals that center around spending time with her family. “I have four grandchildren, and I try to see them every three months,” she says. “I have goals, though they’re probably not super long-term.” Over time, she has come to accept her condition and gained perspective on how to think about it. “You can’t reverse it,” she says. “You can’t just stop it where it is. It’s going to keep progressing.”

Even so, she has been encouraged by the progress she has shown. “I was kind of pleasantly surprised when I really started to notice changes in the way I feel and what I was able to do. My laundry is in the basement, and now I am able to go down there to bring a basket up.” For Marsha, living with CTEPH is a journey of highs and lows, but she is grateful for the time she gets to spend working towards her greatest goal: spending time with her family. “My son is getting ready to graduate college, and my dad is still living. I’m planning to take an Alaskan cruise next summer,” she says. “My goals right now are all about relationships with people.”

This is an example of one patient’s experience. Always talk with your doctor to see if Adempas is right for you.

Real Patients with Real Journeys

As you continue on your treatment journey, it’s important to remember you are not alone. There are others who are on similar paths as you. myJourney stories share the real-life experiences of patients to provide you with insights into the lives of patients with PAH (WHO Group 1) or inoperable or persistent/recurrent CTEPH (WHO Group 4).

Terri’s Journey

myJourney

Click through each chapter below to learn about Terri’s Journey:

I went from being perfectly fine one day to passing out another. I didn't even feel dizzy before I fainted.

Unusual symptoms

I went from being perfectly fine one day to passing out another. I didn't even feel dizzy before I fainted.

Terri had a full-time job and managed a T-shirt business with her husband right out of their home. She was energetic, active, and felt like she could do anything when her health suddenly took an unexpected turn.

It began when she started experiencing fainting spells. The first time it happened, she was drying her hair, and then she lost consciousness and woke up on the floor. “I went from being perfectly fine one day to passing out another,” she remembers. “I didn’t even feel dizzy before I fainted.”

More problems arose when she’d walk across her yard, from the T-shirt shop on her property to the front door of her home. “I would have to sit, I’d be completely out of breath.” She found the episodes strange because they’d go as quickly as they came. “I really never sought help with them because they were just random,” she said. “When I passed out, I would wake up and feel fine.”

The fainting spells and breathing difficulties continued, and she passed out 3 times over a 6-month period. Concerned, she went to the doctor and was told that she was out of shape. She began an exercise regimen, but that seemed to make matters worse. “At that point, I started having what I would liken to asthma attacks, where I just completely couldn’t catch my breath.” Terri’s symptoms escalated from there.

Fighting for a diagnosis

Once we got all the bad news, we started trying to fix it.

One day, Terri was out with her husband in their yard when she felt like she was going to pass out again. She reached for him, and he caught her as she fell, noticing on the way down that she wasn’t breathing. “He said I immediately turned blue,” Terri recalled. As a firefighter, he knew that meant Terri had been without oxygen for too long, and he performed CPR as a result.

When she woke up, Terri’s husband had brought her to the emergency room of their local community hospital, where a battery of tests were performed. Her blood pressure and electrocardiogram results came back normal, so she was misdiagnosed with syncope and sent home.

An hour after getting home, she fainted again. “We went back to the emergency room and we said, ‘Look, this is our second trip here in less than 2 hours. Something is not right.’ ”

Terri was in the hospital for about 4 days until she was seen by the hospital cardiologist, who performed an echocardiogram and right-heart catheterization (RHC). After seeing her results, the doctors finally had an answer. Terri was informed that she had pulmonary arterial hypertension (PAH, WHO Group 1), and was in WHO functional class (WHO FC) III.

After diagnosis, Terri’s cardiologist said he was surprised by the significant enlargement of her right heart. A month later, she finally saw a specialist. “I’d say it went uphill from there. Once we got all the bad news, we started trying to fix it."

Terri was prescribed medications to manage her PAH, including Adempas, and her doctor informed her about the Adempas REMS Program and the risk of embryo-fetal toxicity.

Continue Reading

Once we got all the bad news, we started trying to fix it.

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.

Living with PAH

My goal was to get to where I was feeling better. … Finally getting the correct diagnosis helped me get to this point.
My goal was to get to where I was feeling better. … Finally getting the correct diagnosis helped me get to this point.

Since being diagnosed with PAH, Terri and her husband have had to make adjustments in their lives. “We gave up our T-shirt business because I couldn’t be on my feet and bent over as many hours as that took,” she said. “We quit doing a lot of things. We just did not get out as much.”

Still, Terri decided to set goals for herself. “I had gotten to where I couldn’t do anything, and I didn’t work. We are avid campers and we didn’t go camping,” Terri said. “My goal was to get to where I was feeling better so that I could at least enjoy my family and enjoy everyday activities that we used to take for granted. Finally getting the correct diagnosis helped me get to this point.”

Since being prescribed PAH medications, including Adempas, Terri has seen some improvements. Her doctor informed her about the Adempas REMS Program and the risk of embryo-fetal toxicity. Terri’s stamina is better and her 6-minute walk test has improved, which has led to improvements in her ability to exercise. “I can walk and do a little bit of a treadmill, but I can’t do any major cardio or anything of that nature.” As far as her part-time job goes, Terri said, “I limit myself as far as I don’t try to pick up a hundred folders and walk them down the corridor at work.”

Terri is aware and realistic about her limitations, but she still tries to challenge herself every day. “I feel like it’s easy to give up with this disease. It’s easy to give up and just say, ‘I’m not going to be able to do that anymore,’ or ‘I don’t think I can do that.’ But if I feel it’s safe to do it, then I’m going to try it.”

This is an example of one patient’s experience. Always talk with your doctor to see if Adempas is right for you.

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.