Frequently Asked Questions
Key topics for people with pulmonary fibrosis and those
caring for people with the condition.
Frequent questions from patients
Diagnosing pulmonary fibrosis can be difficult and take some time. Doctors will look at your medical history and a team of specialists may perform a range of tests. These may include lung function tests, X-rays and blood tests that help rule out other conditions to make a confident diagnosis.
This is important because once lung function is lost, it cannot be regained. So, starting the appropriate treatment early on will allow you to preserve valuable lung function.
While the symptoms can affect how you live your life, they don't have to rule it. Together with your doctor or nurse, you can put together a plan to help you manage your symptoms and make the changes you need without compromising on the things you enjoy. Your healthcare team has the knowledge and experience to address any questions you have (or at least to point you in the right direction), so don't be afraid to ask.
It's up to you. It depends on how you feel and how your symptoms affect your ability to perform your duties at work. Speak to your employer about any concerns you have and talk about how flexible working could benefit you both. Do some research into your workers' rights as well, just so that you're well prepared. Make sure you know what you're entitled to, as well as any benefits that may help you along the way.
Exercise can help to maintain your physical and mental health. Of course, your ability to exercise depends on a variety of factors, including how advanced the disease is, as well as your age and general health. Just trying simple, light exercises such as a short walk can be a good start. Talk to your doctor before embarking on any exercise regime, as they can help to evaluate your condition and determine an appropriate level of activity.
There are ways you can help yourself when living with pulmonary fibrosis. Vaccinations are important and effective ways to help protect yourself from infections. Practicing relaxation techniques and trying complementary therapies can also help reduce stress and improve your overall well-being. Don’t be embarrassed to ask for support from friends and family. You can also join a support group for further help with living with pulmonary fibrosis.
Yes, but it is always wise to check with your doctor first. You will need to plan in advance, and make arrangements to consider your condition. High altitudes can worsen breathlessness, as can the climate and terrain. You may also need to inform your insurance and travel companies in advance. Your healthcare team can advise you on how to prepare and plan for your trip.
There are various non-pharmacological treatment options such as pulmonary rehabilitation, oxygen therapy and lung transplantation. Your healthcare team knows your individual situation and condition and are best able to talk to you about possible therapeutic options.
There are also currently two antifibrotic medicines available — nintedanib and pirfenidone. Visit the medication page to learn more about these medicines.
A clinical trial is a medical study designed to investigate how effective and safe a new treatment is. All new treatments have to go through many years of development to ensure they are safe for use in humans and that they work as expected.
You may see a lot of different doctors and other healthcare professionals over the course of your diagnosis and care. Members of your healthcare team can include your doctor, respirologist, nurses, psychiatrists, occupational therapists and others.
Idiopathic pulmonary fibrosis (IPF)
Certain factors, such as cigarette smoking, some viral infections, genetics, gastro-esophageal reflux disease (GERD) and prolonged exposure to occupational or environmental contaminants or dusts are risk factors for IPF; however, its origin and onset are not fully understood.
Idiopathic means of no known cause. In most cases of lung fibrosis (scarring), an exact cause is never established.
The signs and symptoms of IPF include:
- Shortness of breath – only during exercise at first
- A short, dry (hacking) cough that doesn't get better
- Rapid, shallow breathing
- Gradual, unintended weight loss
- Tiredness and generally feeling unwell
- Finger clubbing (widening and rounding) of the tips of the fingers or toes
- "Velcro-like" crackles heard when the doctor listens to the lungs with a stethoscope
- Muscle weakness
IPF is a rare condition that affects about 1 in 3,800 Canadians.
Diagnosing IPF can be hard and take a long time. Doctors will look at your medical history, and a team of specialists may perform a range of tests. These may include lung function tests, X-rays and blood tests that help rule out other conditions to make a confident diagnosis.
IPF is a progressive disease, meaning that it becomes worse over time. Currently it cannot be cured. However, IPF is different for every patient. Some patients may find their disease progresses quickly, while others may find it only changes slowly.
Genetics and genes may play only a small part in the risk of getting scleroderma. Those who have a close family member with scleroderma, for example a child, parent or sibling, have a slightly higher risk of developing scleroderma than everyone else. One US study observed that among the patient groups studied, scleroderma occurred more frequently in families with scleroderma (1.6%) than in the general population (0.026%). It is not a disease that you can pass directly to your children or inherit from your parents.
Scleroderma is not an infection and cannot be passed directly from one person to another like chicken pox or flu. Scleroderma is not contagious.
People are affected by scleroderma in different ways. It can be difficult to predict how it will progress. If scleroderma progresses to your lungs, it can impact your breathing and your ability to do everyday activities. Check your breathing often to note any changes that may be occurring, and don't be afraid to ask your doctor or nurse about your lung health. It's an important thing to ask.
People with scleroderma might develop pulmonary fibrosis that reduces their lung capacity (measured by forced vital capacity) and affects how they go about their life. In very serious cases, these symptoms can affect how long they live.
Pulmonary arterial hypertension (PAH) can have an impact on how long somebody with scleroderma will live, but it depends on the type and nature of PAH.
Your doctor will be able to give you more information that's specific to your situation.
People are affected by scleroderma in different ways. It can be difficult to predict how it will progress. Your type of scleroderma, your symptoms and how they progress can give an idea of what might happen in the future. Don't be afraid to ask your doctor/nurse this question. It's an important thing to ask. Also, check your breath often, and if you notice any changes in your breathing during everyday activities, or other symptoms that could be related to your heart or lungs, tell your doctor as soon as you can. It’s important to start treatment for some symptoms without delay.
It can be difficult to fit your medical appointments in around your other responsibilities. If it’s not physically possible to go to your clinic, request a virtual appointment — most clinics are now able to provide virtual doctor-patient meetings.
The tests and check-ups give the doctor an idea of what's happening and how you're doing. It can help to talk with family, friends and colleagues about your scleroderma. They will be able to find ways to help you make it to your appointments. Don't be afraid to ask. Most people will be glad to have found a way to help you.
Frequent questions from caregivers
Providing support and help that your loved one needs can be a big help. You may feel frustrated or guilty at times, but the care you give makes a big difference.
Among other things, you can help your loved one with their physical and mental well-being, plan for the future, get a power of attorney and organize end-of-life care.
To provide the most effective care for your loved one, it is vital that you are supported too. Patient associations and community support groups can support you by providing a space for you to discuss your feelings with other people in similar situations. Complementary therapies such as yoga and relaxation techniques can also be beneficial for you as a caregiver.
Just a few hours off every now and again can help you relax and maintain social relationships. Your doctor may also be able to help with sorting out some assistance for you. Making sure you take time to care for your own health is an important part of caring for someone.
We know that caregiving does impact relationships, careers, finances and a person’s health and well-being both positively and negatively.
However, the answer is that every caregiver’s experience will be unique. You may be a spouse, a child or a friend caring for someone with scleroderma. Given the variety of symptoms scleroderma can cause, everyone will need different types and levels of help. Whatever you do and whatever your role, how you define it is up to you.
People with scleroderma can develop symptoms that have an impact on their relationships. Skin tightening and discomfort, joint pain and other symptoms can affect the sex life between partners where one or both have scleroderma.
If these symptoms become a problem, talk it over with your partner and consider talking to your doctor. In some women, another autoimmune condition called Sjögren’s syndrome seems to accompany scleroderma but does not happen directly as a result of having scleroderma.
In Sjögren’s syndrome, the immune system is attacking the glands that make tears and saliva. Women with Sjögren’s syndrome can experience vaginal dryness, but this can be overcome by using vaginal lubricants.
Up to 8 out of 10 men with systemic scleroderma (SSc) experience erectile dysfunction. One reason may be that scleroderma decreases blood flow to the erectile tissues. Erectile dysfunction is a common problem that can be addressed by the doctor or a urology specialist.
With sexual problems, it is a good idea to try and provide support for each other, while seeking help from a healthcare professional. It might be a bit embarrassing at first, but it is something you can work at together.
Your loved one’s healthcare professional should be able to provide general direction for when it is best to take the medicines. Your local pharmacist can also help with this too. They may help organize your loved one’s medications and help you remember when to give them. It can also help to keep a list of the current medicines, as they may change every now and then.