(BPT) - For Joanne Graham, life felt relatively normal. Having raised three wonderful children, she enjoyed spending time around her hometown, Seattle, Washington, and spending time watching her grandchildren play. But that quiet life she’d come to know was disrupted the day she began experiencing what would later be diagnosed as a lung disease called idiopathic pulmonary fibrosis (IPF).
IPF is a serious and rare condition affecting as many as 132,000 Americans that causes permanent scarring of the lungs. And the road to diagnosis can be a long and frustrating journey. On average, people with IPF visit two or three doctors over the course of nearly two years before receiving an accurate diagnosis. A reason for this is because IPF is often confused with other better known and more common respiratory diseases like chronic obstructive pulmonary disease (COPD), asthma, or pneumonia. Also, some primary care doctors may not be aware of IPF, which is why seeing a lung specialist called a “pulmonologist” is important.
Joanne’s journey began in August 2011, when she first suspected something wasn’t right. But it would take another 14 long months of seeing multiple healthcare specialists and wondering about her health before she would receive a definitive IPF diagnosis on Halloween in 2012.
“When my doctor said ‘IPF,’ I had no idea what it was. When I learned more about it, I thought, ‘this is ridiculous. This can’t be me,’” Joanne recalls. “How can some test be suggesting that my lungs may not be functioning normally? It just didn’t seem real.”
The gravity of the situation hit harder when Joanne learned that there were no medicines approved by the U.S. Food and Drug Administration (FDA) to treat IPF.
“At the time I was diagnosed, I was told by one doctor to just go home and live my life,” she said. “But this was simply not an option for me. Instead, I learned everything I could about IPF, worked closely with my pulmonologist and his team, and vowed to stay positive. I also knew I wanted to share my story and help others dealing with an IPF diagnosis.”
Once the U.S. FDA approved the first treatments for IPF in October 2014, nearly two years after her diagnosis, Joanne worked with her doctor to discuss options for treating her disease.
Every individual diagnosed with IPF has a unique experience with the disease, so treatment varies. Today, Joanne takes a medication called OFEV® (nintedanib), which is one of the first medicines approved for the treatment of IPF. OFEV has been shown to slow disease progression. In 3 out of 3 medical studies, IPF patients who took OFEV found that it slowed the decline in lung function by approximately 50% compared with taking a placebo. This was measured by the forced vital capacity (FVC), which is one of the measures of disease progression.
“An IPF diagnosis is shocking, and many patients can be easily frustrated by the lack of answers to some of their questions, especially if they are not under the care of a specialist,” said Rebecca Fox, LPN.
Rebecca works at a 24-hour nurse helpline at OPEN DOORSTM, a patient support program run by Boehringer Ingelheim Pharmaceuticals Inc., the makers of OFEV, and available to IPF patients taking OFEV. She explains that, particularly in rare diseases, these help lines – traditional phone hotlines, as well as online chats – offer support and resources to patients in need of information.
Rebecca explains, “At OPEN DOORS, we work with people one-on-one, and are available to provide advice and support. We work in partnership with a patient’s pulmonologist and healthcare team to provide additional support as needed.”
Joanne says that having a supportive health care team and increasing her knowledge of IPF has made a big impact in facing her illness. She also takes comfort in the support and guidance she receives from her pulmonologist and the nurses in the doctor’s practice.
“The most important thing is to understand your disease with your family and take all the necessary steps to be involved in your care. Above all, remember, you’re not alone. There is help available.”
IMPORTANT SAFETY INFORMATION
What is the most important information I should know about OFEV®
OFEV® can cause birth defects or death to an unborn baby. Women should not become pregnant while taking OFEV®. Women who are able to become pregnant should use birth control during treatment and for at least 3 months after treatment. If you become pregnant while taking OFEV®, tell your doctor right away.
What are the possible side effects of OFEV®?
OFEV® may cause serious side effects, including:
Liver problems. Call your doctor right away if you have unexplained symptoms such as 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 or feeling tired. Your doctor will do blood tests regularly to check how well your liver function is working during your treatment with OFEV®.Diarrhea, nausea, and vomiting. Tell your doctor if you have diarrhea, nausea, or vomiting or if these symptoms do not go away or become worse. Tell your doctor if you are taking over-the-counter laxatives, stool softeners, and other medicines or dietary supplements that can cause diarrhea. While you are taking OFEV®, your doctor may recommend that you drink fluids or take medicine to treat these side effects.Heart attack. Tell your doctor right away if you have symptoms of a heart problem. These symptoms may include chest pain or pressure, pain in your arms, back, neck or jaw, or shortness of breath.Tell your doctor right away if you have symptoms of a stroke. These symptoms may include numbness or weakness on 1 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, or wounds that do not heal. Tell your doctor 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.
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
What should I tell my doctor before using OFEV® (nintedanib)?
Before you take OFEV®, tell your doctor 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, are a smoker, or have any other medical conditions.
Tell your doctor if you are pregnant or plan to become pregnant. You should avoid becoming pregnant while taking OFEV®. OFEV® can cause harm, birth defects or death to your unborn baby. Use appropriate birth control methods while taking OFEV® and for at least 3 months after your last dose. Tell your doctor if you are breastfeeding or plan to breastfeed. It is not known if OFEV® passes into your breast milk. You and your doctor should decide if you will take OFEV® or breastfeed. You should not do both.
Tell your doctor if you currently smoke. 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. Keep a list of the medicines you take and show it to your doctor and pharmacist when you get a new medicine.
Please click here for full Prescribing Information, including Patient Information.
What is OFEV®?
OFEV® is a prescription medication used to treat people with a lung disease called idiopathic pulmonary fibrosis (IPF). It is not known if OFEV® is safe and effective in children.
For more information:
To learn more about OPEN DOORS patient support program, visit www.OFEV.com or call 1-866-OPENDOORS.
Visit the Pulmonary Fibrosis Foundation for information on IPF, additional resources, and to learn about local support groups.