WHAT IS IPF? IF YOU HAVE IPF, IT’S VERY IMPORTANT THAT YOU EMPOWER YOURSELF BY UNDERSTANDING AS MUCH AS YOU CAN ABOUT THE DISEASE.
Idiopathic Pulmonary Fibrosis, or IPF, is a rare, scarring disorder of the lungs without a known cause, which tends to develop in people in their late 6Os to early 7Os. In IPF, scar tissue – similar to that you get after an injury – develops around the edges of the lung, causing them to become stiffer and less effective at getting oxygen into the bloodstream.
It’s a progressive condition where the lungs become increasingly scarred over time. As that happens, the lungs become less and less efficient, and that means people become breathless, even when doing very little.
In the end stages of the condition, people with IPF become breathless at rest, so even just going to the toilet or washing themselves becomes a major effort.
What symptoms do people with IPF develop?
The earliest symptom that people with IPF notice tends to be breathlessness, but only when they’re exerting themselves, perhaps walking upstairs, carrying heavy bags back from the shops.
Another symptom that people sometimes notice is a persistent cough. For a small proportion of people a cough is the very first symptom that they get, while others get a cough at the same time as the breathlessness.
Diagnosing IPF can take a long time – can you tell us why? GETTING FROM THE POINT WHERE SYMPTOMS ARE FIRST REPORTED TO A DOCTOR, TO AN ACTUAL DIAGNOSIS OF IPF CAN SOMETIMES TAKE AS LONG AS 9, 12 OR EVEN 18 MONTHS.
One of the challenges in diagnosing IPF is that there are lots of causes for breathlessness.
People can become breathless due to asthma or emphysema or heart disease. In some cases, people can end up seeing cardiologists first, or receiving treatment for emphysema and that adds time to the patient’s journey before the final diagnosis is achieved.
For a patient, getting from the point where symptoms are first reported to a doctor, to an actual diagnosis of IPF can sometimes take as long as 9, 12 or even 18 months.
What is the impact of such a lengthy journey to diagnosis?
Shortening the time taken for patients to be diagnosed with IPF is a key challenge. This is particularly the case because during this period of time, the condition may be progressing and getting worse – this deterioration is irreversible.
One of the really important things that we need to do is to shorten that journey so that we make a diagnosis as quickly as possible. The later treatment is left, the worse the patient’s lung function is before that treatment is started.
Are there therapies for managing the symptoms of IPF?
There are treatments available to manage symptoms like cough and breathlessness. These can either be medications to treat cough or breathlessness or they can be things like pulmonary rehabilitation. Pulmonary rehabilitation is a course of exercise, guided by a physiotherapist, which aims to teach exercises to people with IPF that they can do within the confines of their symptoms, and provide education so they can manage their own symptoms effectively.
Oxygen therapy can also be prescribed when people find that their oxygen levels drop rapidly during exercise; it’s that rapid drop in oxygen that causes people to feel breathless and stops them doing physical activities. In the later stages of IPF, oxygen therapy can be very useful and doctors will prescribe oxygen for long periods of time to be used as a way of alleviating the stress of lower oxygen levels on the body.
Are there therapies to help manage the progression of the disease?
Yes, there are treatments available, called antifibrotics, which can actively slow down the scarring process in the lungs. Antifibrotic therapy is an important consideration as it specifically targets the scarring process itself.
Are there ANY OTHER TREATMENTS I SHOULD BE AWARE OF?
Another treatment option to consider is lung transplantation. Because of the limitations of lung transplantation, it is only an option for a minority of patients with IPF. However, it is very important that transplantation is considered early enough so that both the patient and their doctor can decide whether it’s an appropriate treatment for that individual.
How can someone with IPF make sure they understand their options? IT’S IMPORTANT YOU HAVE A HEALTHCARE PROFESSIONAL WHO UNDERSTANDS IPF AND CAN PROVIDE YOU WITH BALANCED ADVICE ON THE BEST MANAGEMENT OPTIONS.
If you have IPF, I think it’s very important that you, first of all, empower yourself by understanding as much as you can about the disease and there are a number of good websites that will help (see Key Links below for more information). But it’s also very important that you have a healthcare professional, or a team of healthcare professionals, who understand IPF and can provide you with balanced advice on the best treatment options and the management of other things related to your disease.
What can patients do to make the most of discussions with their specialist?
IPF symptoms are not easy to identify; people can’t see your symptoms. So I think it’s very important in any conversation with healthcare professionals for patients to communicate the impact that IPF is having on them, in preventing them from doing things.
I think for a person with IPF, trying to slow disease, and thus trying to maintain quality of life over the longer term is a very important goal and I think it’s critical to get that message across to healthcare professionals who perhaps only have a very short period of time to get to know you in clinic.
What can someone living with IPF do on a daily basis to help themselves?
If you are a smoker and have been diagnosed with IPF, you should stop right away. Equally, if any of your family smoke, you should ask them not to do it in the same house. You should certainly avoid direct exposure to cigarette smoke.
It’s also important to remember that infections can really cause problems for people with IPF.
Infections often exacerbate the scarring process in the lungs, and you should take all possible steps to avoid them. You should remain alert for symptoms of an infection and should ideally have antibiotics at the earliest signs, as well as an annual flu vaccine.
How can the family and friends of someone with IPF help?
When people are diagnosed with IPF, one of the first challenges they face is trying to communicate or talk about their diagnosis with their loved ones, their family and their friends. This is partly because very few people know about IPF.
So in all likelihood when people go home and tell their family that they’ve been diagnosed with IPF, nobody is going to know or understand what this means. It’s going to be very important for friends and family to find out about the disease for themselves, so they can understand the impact it is having on the patient.
It’s also really important that someone with IPF stays active, is positive about the future and engages with healthcare professionals. Family, friends and loved ones are really important in giving the help and support people with IPF need on their journey.