- DIFFICULTY EXERCISING
- LOSS OF INDEPENDENCE
- FEELING LOW
MANAGING MY IPF:
- HOW CAN I SLOW DOWN MY DISEASE PROGRESSION?
- WHAT THERAPIES AM I CURRENTLY RECEIVING?
- HOW CAN I CONTROL MY COUGH BETTER?
- WHAT CAN I DO DAY-TO-DAY TO HELP?
- HOW CAN I MANAGE OXYGEN THERAPY?
- WHAT OTHER THERAPIES MIGHT BE SUITABLE FOR ME?
- WHAT ARE THE BENEFITS AND RISKS OF THERAPIES?
- WHEN CAN I START ANTIFIBROTIC TREATMENT TO SLOW MY ILLNESS DOWN?
- WHAT EMOTIONAL SUPPORT IS AVAILABLE TO ME?
- WHAT SUPPORT IS AVAILABLE TO MY LOVED ONES?
What it means to have IPF
Idiopathic Pulmonary Fibrosis, or IPF, is a disease that affects the lungs. It's a chronic condition that can take a long time to diagnose, and it's relatively rare. While many people have never heard of it, over one million people around the world live with IPF toaday and 30,000 people may be affected by IPF in Canada alone.1-3
IPF affects your breating, making it harder to catch your breath when yoe're active. Depending on how far IPF has progressed, that can mean anything from shortness of breath when exercising, to difficulty doing normal day-to-day activities, like walking outside or going upstairs.
This shortness of breath occurs in IPF because fragile tissue on the lungs is damaged and becomes scar tissue, in a process called fibrosis. This scarring gradually progresses over time, spreading to more of the lungs, making it harder to breathe.
IPF behaves differently in each person who has it, so it's hard to predict how fast the scarring will spread. Sometimes it happens very quickly, over a period of months, other times it takes several years.1 Unfortunately, whether IPF spreads slowly or quickly, eventually it will be fatal in most cases.
What treatment options are there for IPF?
A lung transplant is the only cure for IPF at the moment, but only a few people are eligible for a transplant. There are risks involved, and importantly, you will need to be matched with a suitable donor, which can take a long time. Your doctor will be able to help explain how the donor process works.
There are medicines which have been developed over the past few years to treat IPF. While it is not completely understood how these medications work, studies show they can help reduce inflammation and fibrosis, which in turn may slow down the progress of the disease but are not a cure. Slowing the disease progression offers people with IPF the opportunity to continue doing the things that matter the most to them. With these medicines, side effects can include nausea, diarrhea, vomiting, decreases in weight and/or appetite and abnormal blood tests, but your doctor can usually help you to manage these.
Health Canada has authorized for sale two medications for the treatment of IPF:
What the medication is used for:6
ESBRIET is used for the treatment of idiopathic pulmonary fibrosis (IPF) in adults.
What it does:6
How ESBRIET works is not yet fully understood. It may reduce inflammation and fibrosis in your lungs. It may slow down worsening of your IPF.
ESBRIET should only be prescribed and monitored by physicians with the appropriate training and experience in the diagnosis and treatment of IPF.
Side effects may include:6
- Increased Sensitivity to Sunlight: skin reactions after going out in the sun or using sunlamps, sunburn
- Skin problems such as rash, itchy skin, skin redness, dry skin
- Tiredness, feeling weak or feeling low in energy
- Indigestion, heartburn, acid reflux, loss of appetite, anorexia, changes in taste, bloating, abdominal pain and discomfort
- Infections of the throat or the airways going into the lungs and/or sinusitis, influenza and/or common cold
- Difficulty sleeping, feeling sleepy
- Muscle pain, aching joints/joint pains
- Weight loss
- ESBRIET may cause liver problems and other abnormal blood test results. Your doctor will decide when to perform blood tests and will interpret the results
What the medication is used for:7
Use OFEV to treat Idiopathic Pulmonary Fibrosis (IPF) in adults.
What it does:7 OFEV works to reduce the fibrosis in your lungs.
OFEV should only be prescribed and monitored by physicians with the appropriate training and experience in the diagnosis and treatment of IPF.
Side effects may include:7
- Diarrhea may lead to a loss of fluid and important electroylytes in your body
- OFEV can cause abnormal blood test results. Your doctor will decide when to perform blood tests and will interpret the results
Pulmonary rehabilitation is a programme of exercise, training and education designed to maximize the use of your lungs. Over time, the exercises and techniques will help you manage the day to-day symptoms of IPF and stay as active as possible.
Therapies for IPF symptoms4
Your doctor may offer you other therapies to help with the symptoms of IPF, such as breathlessness, but they do not manage how the condition progresses. Oxygen therapy can help with the feeling of breathlessness as you get more oxygen to your body. However, this is often from a tank and so can limit how easy it is to move around. Tell your doctor about other symptoms you are experiencing.
- Nalysnyk L, etal Eur Resp Rev. 2012; 21(126): 355-61
- Canadian Pulmonary Fibrosis Foundation. IPF Patient Information Guide. 2016 Accessed: http://cpFf.ca/understanding-pf/cpff-patient-guide/. Last accessed: September 2016.
- Meltzer E. Noble P. Orpharet J Rare Dis 2008; 3: 8.
- Raghu G. Collard HR, Egan J, et al Am J Respir Crit Care Med. 2011.183: 788-824.
- Raghu G, Rochwerg B, Zhang Y. etal. Am J Crit Care Med 2015; 192(2)-238-248.
- Esbriet® Product Monograph, April 2016.
- OFEV™ Product Monograph, October 2015.