Patient journeys to diagnosis and living with PAH

Lindsay, Byron, and Kathy are real patients with PAH. Each of their photos were used to create a unique collage that shows the pieces of their life and who they are as individuals, and not just patients with PAH.

Lindsay
High school math teacher
OPSUMIT® patient since 2013

Lindsay is a 36-year-old high school math teacher who loves the outdoors and spending time with her nieces.

In 2007, Lindsay noticed her episodes of shortness of breath had grown in frequency to the point where she could not climb 2 stairs without stopping for breath, which she knew wasn’t normal for a healthy 23-year-old. After a cardiologist conducted a battery of tests, including a RHC, she was diagnosed with PAH in 2008. Her treatment was switched to OPSUMIT® while it was still in clinical trials.

Even though Lindsay has PAH, it has not stopped her from working, taking walks, or yoga classes. She hopes sharing her story with students inspires them to overcome challenges. “I feel like a lot of people have a really hard time with this disease and I want people to know it can get better.”

Learn more about Lindsay

WATCH VIDEO

Individual patient experiences may vary.

Byron
Blues musician
OPSUMIT® patient since 2017

Byron, a 65-year-old father of 3, has played electric bass and the electric guitar professionally for more than 50 years. He plays at festivals, dance halls, and restaurants. Aside from the occasional shortness of breath he began to experience in 2010, his medical check-ups indicated he was generally healthy. Byron had no reason to suspect a life-altering chronic condition until his symptoms worsened in January 2013. During those more detailed tests, a physician assistant noticed an abnormality in Byron’s echocardiogram and ordered a RHC. The results confirmed Byron had PAH.

When his PAH was not well controlled, it hampered his ability to perform, but now he is able to play again. His message to other patients with PAH is clear. “There’s hope.”

Individual patient experiences may vary.

Kathy
Aspiring author
OPSUMIT® patient since 2019

Kathy is 47 years old, an animal lover, and mother of 3. Before her PAH diagnosis, she enjoyed playing racquetball, studied laboratory animal science in college, and became a pet groomer.

In 2011, after several unusual medical experiences and misdiagnoses, it was discovered that she had an autoimmune condition, scleroderma. In late 2012, her rheumatologist noticed that her chest X-rays and MRI scan revealed an enlarged heart. Her rheumatologist referred her to a cardiologist who suspected the scleroderma had led to PAH, which was confirmed by a RHC.

In addition to other medication, Kathy began taking OPSUMIT® in 2019. “It gives me that inner peace knowing right now that I’m stable and can function...” These days, she enjoys encouraging other patients with PAH to be strong and find a support group. Kathy sits on the board of a pulmonary fibrosis support group and is also active in advocacy organizations. She is currently writing a memoir of her PAH experience.

Individual patient experiences may vary.

About the artist

Patrick Bremer is a creative from the UK chosen by Janssen PH to reimagine a campaign focused around how starting with the right foundational therapy impacts a patient’s life. As an artist who specializes in creating striking collages, he has been commissioned in the past by the likes of Google, The New Yorker, and AARP. Patrick’s unique portraiture style brings to life the pieces of a patient’s world and connects it to their experience with OPSUMIT®.

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IMPORTANT SAFETY INFORMATION

BOXED WARNING: EMBRYO-FETAL TOXICITY

  • Do not administer OPSUMIT® to a pregnant female because it may cause fetal harm.
  • Females of reproductive potential: Exclude pregnancy before the start of treatment, monthly during treatment, and 1 month after stopping treatment. Prevent pregnancy during treatment and for one month after stopping treatment by using acceptable methods of contraception.
  • For all female patients, OPSUMIT® is available only through a restricted program called the OPSUMIT® Risk Evaluation and Mitigation Strategy (REMS).

INDICATION

OPSUMIT® is an endothelin receptor antagonist (ERA) indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to reduce the risks of disease progression and hospitalization for PAH.

Effectiveness was established in a long-term study in PAH patients with predominantly WHO Functional Class II-III symptoms treated for an average of 2 years. Patients had idiopathic and heritable PAH (57%), PAH caused by connective tissue disorders (31%), and PAH caused by congenital heart disease with repaired shunts (8%).

CONTRAINDICATIONS

Pregnancy: OPSUMIT® may cause fetal harm when administered to a pregnant woman. OPSUMIT® is contraindicated in females who are pregnant. If OPSUMIT® is used during pregnancy, advise the patient of the potential risk to a fetus.

Hypersensitivity: OPSUMIT® is contraindicated in patients with a history of a hypersensitivity reaction to macitentan or any component of the product.

WARNINGS AND PRECAUTIONS

Embryo-fetal Toxicity and OPSUMIT® REMS Program

Due to the risk of embryo-fetal toxicity, OPSUMIT® is available for females only through a restricted program called the OPSUMIT® REMS Program. For females of reproductive potential, exclude pregnancy prior to initiation of therapy, ensure use of acceptable contraceptive methods, and obtain monthly pregnancy tests.

Notable requirements of the OPSUMIT® REMS Program include:

  • Prescribers must be certified with the program by enrolling and completing training.
  • All females, regardless of reproductive potential, must enroll in the OPSUMIT® REMS Program prior to initiating OPSUMIT®. Male patients are not enrolled in the REMS.
  • Females of reproductive potential must comply with the pregnancy testing and contraception requirements.
  • Pharmacies must be certified with the program and must only dispense to patients who are authorized to receive OPSUMIT®.

Hepatotoxicity

  • ERAs have caused elevations of aminotransferases, hepatotoxicity, and liver failure. The incidence of elevated aminotransferases in the SERAPHIN study >3 x ULN was 3.4% for OPSUMIT® vs 4.5% for placebo, and >8 x ULN was 2.1% vs 0.4%, respectively. Discontinuations for hepatic adverse events were 3.3% for OPSUMIT® vs 1.6% for placebo.
  • Obtain liver enzyme tests prior to initiation of OPSUMIT® and repeat during treatment as clinically indicated.
  • Advise patients to report symptoms suggesting hepatic injury (nausea, vomiting, right upper quadrant pain, fatigue, anorexia, jaundice, dark urine, fever, or itching).
  • If clinically relevant aminotransferase elevations occur, or if elevations are accompanied by an increase in bilirubin >2 x ULN, or by clinical symptoms of hepatotoxicity, discontinue OPSUMIT®. Consider re-initiation of OPSUMIT® when hepatic enzyme levels normalize in patients who have not experienced clinical symptoms of hepatotoxicity.

Fluid Retention

  • Peripheral edema and fluid retention are known consequences of PAH and ERAs. In the pivotal PAH study SERAPHIN, edema was reported in 21.9% of the OPSUMIT® group vs 20.5% for placebo.
  • Patients with underlying left ventricular dysfunction may be at particular risk for developing significant fluid retention after initiation of ERA treatment. In a small study of pulmonary hypertension due to left ventricular dysfunction, more patients in the OPSUMIT® group developed significant fluid retention and had more hospitalizations due to worsening heart failure compared to placebo. Postmarketing cases of edema and fluid retention occurring within weeks of starting OPSUMIT®, some requiring intervention with a diuretic or hospitalization for decompensated heart failure, have been reported.
  • Monitor for signs of fluid retention after OPSUMIT® initiation. If clinically significant fluid retention develops, evaluate the patient to determine the cause and the possible need to discontinue OPSUMIT®.

Hemoglobin Decrease

  • Decreases in hemoglobin concentration and hematocrit have occurred following administration of other ERAs and in clinical studies with OPSUMIT®. These decreases occurred early and stabilized thereafter.
  • In the SERAPHIN study, OPSUMIT® caused a mean decrease in hemoglobin (from baseline to 18 months) of about 1.0 g/dL vs no change in the placebo group. A decrease in hemoglobin to below 10.0 g/dL was reported in 8.7% of the OPSUMIT® group vs 3.4% for placebo. Decreases in hemoglobin seldom require transfusion.
  • Initiation of OPSUMIT® is not recommended in patients with severe anemia. Measure hemoglobin prior to initiation of treatment and repeat during treatment as clinically indicated.

Pulmonary Edema with Pulmonary Veno-occlusive Disease (PVOD)

Should signs of pulmonary edema occur, consider the possibility of associated PVOD. If confirmed, discontinue OPSUMIT®.

Decreased Sperm Counts

OPSUMIT®, like other ERAs, may have an adverse effect on spermatogenesis. Counsel men about potential effects on fertility.

ADVERSE REACTIONS

Most common adverse reactions (more frequent than placebo by ≥3%) were anemia (13% vs 3%), nasopharyngitis/pharyngitis (20% vs 13%), bronchitis (12% vs 6%), headache (14% vs 9%), influenza (6% vs 2%), and urinary tract infection (9% vs 6%).

DRUG INTERACTIONS

  • Strong inducers of CYP3A4 such as rifampin significantly reduce macitentan exposure. Concomitant use of OPSUMIT® with strong CYP3A4 inducers should be avoided.
  • Strong inhibitors of CYP3A4 like ketoconazole approximately double macitentan exposure. Many HIV drugs like ritonavir are strong inhibitors of CYP3A4. Avoid concomitant use of OPSUMIT® with strong CYP3A4 inhibitors. Use other PAH treatment options when strong CYP3A4 inhibitors are needed as part of HIV treatment.
  • Moderate dual inhibitors of CYP3A4 and CYP2C9 such as fluconazole and amiodarone are predicted to increase macitentan exposure. Avoid concomitant use of OPSUMIT® with moderate dual inhibitors of CYP3A4 and CYP2C9.
  • Concomitant treatment of both a moderate CYP3A4 inhibitor and moderate CYP2C9 inhibitor with OPSUMIT® should also be avoided.

INDICATION

OPSUMIT® is an endothelin receptor antagonist (ERA) indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to reduce the risks of disease progression and hospitalization for PAH.

Effectiveness was established in a long-term study in PAH patients with predominantly WHO Functional Class II-III symptoms treated for an average of 2 years. Patients had idiopathic and heritable PAH (57%), PAH caused by connective tissue disorders (31%), and PAH caused by congenital heart disease with repaired shunts (8%).

Please see full Prescribing Information, including BOXED WARNING.

cp-113979v5
MRI=magnetic resonance imaging; PAH=pulmonary arterial hypertension; RHC=right heart catheterization.

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IMPORTANT SAFETY INFORMATION

BOXED WARNING: EMBRYO-FETAL TOXICITY

  • Do not administer OPSUMIT® to a pregnant female because it may cause fetal harm.
  • Females of reproductive potential: Exclude pregnancy before the start of treatment, monthly during treatment, and 1 month after stopping treatment. Prevent pregnancy during treatment and for one month after stopping treatment by using acceptable methods of contraception.
  • For all female patients, OPSUMIT® is available only through a restricted program called the OPSUMIT® Risk Evaluation and Mitigation Strategy (REMS).

INDICATION

OPSUMIT® is an endothelin receptor antagonist (ERA) indicated for the treatment of pulmonary arterial hypertension (PAH, WHO Group I) to reduce the risks of disease progression and hospitalization for PAH.

Effectiveness was established in a long-term study in PAH patients with predominantly WHO Functional Class II-III symptoms treated for an average of 2 years. Patients had idiopathic and heritable PAH (57%), PAH caused by connective tissue disorders (31%), and PAH caused by congenital heart disease with repaired shunts (8%).