Boehringer Ingelheim announces nintedanib expanded access program (EAP) for children and adolescents with fibrosing interstitial lung disease

Ridgefield, Conn., October 17, 2022 – Today, Boehringer Ingelheim Pharmaceuticals, Inc. announced an expanded access program (EAP) for nintedanib for children and adolescents with fibrosing interstitial lung disease (ILD) who meet certain eligibility criteria. Nintedanib is approved by the U.S. Food and Drug Administration (FDA) for the treatment of adults with idiopathic pulmonary fibrosis (IPF), chronic fibrosing ILDs with a progressive phenotype, and to slow the rate of decline in pulmonary function in adult patients with systemic sclerosis-associated ILD (SSc-ILD). Nintedanib has not been approved for use in children and adolescents. Expanded access to nintedanib would provide an investigational treatment for children and adolescents who otherwise have no approved therapies for this illness.

“With no established diagnostic criteria, and no approved therapies, childhood interstitial lung diseases can place a significant burden on patients and their loved ones,” said Craig Conoscenti, M.D., FCCP, ATSF, Executive Director/Therapeutic Area Head, Respiratory IPF/ILD, Clinical Development and Medical Affairs, Boehringer Ingelheim. “Given the debilitating and progressive nature of this rare disease, Boehringer Ingelheim remains committed to making nintedanib accessible to children and adolescents who are unable to take part in our ongoing clinical trial program.”

EAPs are intended to allow patient access to an investigational medicine for a serious or immediately life-threatening disease or condition where no comparable or satisfactory alternative therapy exists and when the potential benefit of the medicine justifies the potential risks and those risks are not unreasonable in the context of the disease or condition being treated. Healthcare professionals and patients can obtain details about participation in the nintedanib EAP by calling +1-800-542-6257.

Additional information about Boehringer Ingelheim’s EAPs can be found here.

About the EAP
The nintedanib EAP is a treatment protocol to provide access to patients not able to join the ongoing clinical trial program (InPedILD^™-ON [NCT05285982]), and to evaluate the safety and tolerability of nintedanib in children and adolescents with fibrosing ILD. Enrollment criteria are available on clinicaltrials.gov and include patients with the following characteristics at first visit:

• Children and adolescents aged 6-17 years
• Confirmed diagnosis with clinically significant fibrosing ILD who are unable to participate in the ongoing InPedILD^™-ON trial
• Evidence of fibrosing ILD on high-resolution computed tomography (HRCT) within 12 months of first visit
• Forced vital capacity (FVC) predicted ≥25%  

About Childhood Interstitial Lung Disease (chILD)
Childhood interstitial lung disease (chILD) includes more than 200 rare disorders with debilitating symptoms that can include cough, difficulty breathing and rapid breathing. Its exact prevalence is unknown, but it can be considered very rare with a reported incidence ranging from 1.5 to 3.8 per million. Pulmonary fibrosis within chILD is even less frequent, with no known global prevalence estimates, and no international studies prior to InPedILD. chILD is associated with significant mortality and morbidity. When their condition deteriorates, many pediatric patients will need oxygen to go about their daily lives and require lung transplants. There are no established diagnostic criteria and few management guidelines. The current standard of care involves off-label use of treatments including steroids and steroid-sparing immunosuppressants.

About OFEV (nintedanib) Capsules
OFEV is approved in the U.S. and more than 80 countries for the treatment of adult patients living with IPF. In September 2019, OFEV was approved in the U.S. to slow the rate of decline in pulmonary function in adult patients with SSc-ILD, and then in March 2020 to treat adult patients with chronic fibrosing ILDs with a progressive phenotype.

What is OFEV?

• OFEV is a prescription medicine used: 
  • to treat adults with a lung disease called idiopathic pulmonary fibrosis (IPF). 
  • to treat adults with a long lasting (chronic) interstitial lung disease in which lung fibrosis continues to worsen (progress). 
  • to slow the rate of decline in lung function in adults with systemic sclerosis-associated interstitial lung disease (SSc-ILD) (also known as scleroderma-associated ILD). 
• It is not known if OFEV is safe and effective in children. 

Important Safety Information 

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

OFEV can cause harm, birth defects, or death to an unborn baby. Women should not become pregnant while taking OFEV. Women who are able to become pregnant should have a pregnancy test before starting treatment and should use highly effective birth control at the start of treatment, during treatment, and for at least 3 months after your last dose. Talk with your doctor about what birth control method is right for you during this time. Birth control pills may not work as well in women having vomiting, diarrhea, or other problems reducing the drug absorption. If you have any of these problems, talk with your doctor about what highly effective birth control method is right for you. If you become pregnant or think you are pregnant while taking OFEV, tell your doctor right away. 

What should I tell my doctor before using OFEV?

Before you take OFEV, tell your doctor about all of your medical conditions, including if you have: 

• liver problems. 
• heart problems. 
• a history of blood clots. 
• a bleeding problem or a family history of a bleeding problem. 
• had recent surgery in your stomach (abdominal) area. 

Tell your doctor if you:

• are pregnant or plan to become pregnant. 
• are breastfeeding or plan to breastfeed. It is not known if OFEV passes into your breast milk.
        You should not breastfeed while taking OFEV. 
• are a smoker. You should stop smoking prior to taking OFEV and avoid smoking during treatment. 

Tell your doctor about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements such as St. John’s wort. 

What are the possible side effects of OFEV?

OFEV may cause serious side effects.

TELL YOUR DOCTOR RIGHT AWAY if you are experiencing any side effects, including: 

• Liver problems. Unexplained symptoms may include yellowing of your skin or the white part of your eyes (jaundice), dark or brown (tea-colored) urine, pain on the upper right side of your stomach area (abdomen), bleeding or bruising more easily than normal, feeling tired, or loss of appetite. Your doctor will do blood tests to check how well your liver is working before starting and during your treatment with OFEV. 
• Diarrhea, nausea, and vomiting. Your doctor may recommend that you drink fluids or take medicine to treat these side effects. Tell your doctor if you have these symptoms, if they do not go away, or get worse, and if you are taking over-the-counter laxatives, stool softeners, and other medicines or dietary supplements. 
• Heart attack. Symptoms of a heart problem may include chest pain or pressure, pain in your arms, back, neck, or jaw, or shortness of breath. 
• Stroke. Symptoms of a stroke may include numbness or weakness on one side of your body, trouble talking, headache, or dizziness. 
• Bleeding problems. OFEV may increase your chances of having bleeding problems. Tell your doctor if you have unusual bleeding, bruising, wounds that do not heal, and/or if you are taking a blood thinner, including prescription blood thinners and over-the-counter aspirin. 
• Tear in your stomach or intestinal wall (perforation). OFEV may increase your chances of having a tear in your stomach or intestinal wall. Tell your doctor if you have pain or swelling in your stomach area. 
• Increased protein in your urine (proteinuria). OFEV may increase your chances of having protein in your urine. Tell your doctor if you have any signs and symptoms of protein in the urine such as foamy urine, swelling, including in your hands, arms, legs, or feet, or sudden weight gain.

The most common side effects of OFEV are diarrhea, nausea, stomach pain, vomiting, liver problems, decreased appetite, headache, weight loss, and high blood pressure. 

These are not all the possible side effects of OFEV. For more information, ask your doctor or pharmacist. You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088. 

Please see full Prescribing Information, including Patient Information.

CL-OF-100056 01.18.2022

About Boehringer Ingelheim
Boehringer Ingelheim is working on breakthrough therapies that improve the lives of humans and animals. As a leading research-driven biopharmaceutical company, the company creates value through innovation in areas of high unmet medical need. Founded in 1885 and family-owned ever since, Boehringer Ingelheim takes a long-term perspective. Around 52,000 employees serve more than 130 markets in the three business areas, Human Pharma, Animal Health, and Biopharmaceutical Contract Manufacturing.  

Learn more at www.boehringer-ingelheim.com/us

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