Taking control of IPF
A tale of two perspectives
Rainer Karpavicius had suspected that something was wrong for quite some time.
People were asking me: 'Have you got any idea what is wrong with you?'
But it was only after two very stressful years of uncertainty that he was diagnosed with idiopathic pulmonary fibrosis (IPF).
Michael Kreuter, who is Rainer Karpavicius's doctor, is an eminent pulmonology and respiratory specialist at the Thoraxklinik at Heidelberg University Hospital. Scroll down to hear his expert advice for living with IPF alongside Rainer Karpavicius’s personal experience of the condition.
Chapter 1
Journey to diagnosis
Rainer Karpavicius reflects on his two-year journey from first becoming aware of his symptoms to being diagnosed with IPF. “When I found out that I have lung fibrosis, I was first confused. What is a ‘fibrosis’? I had never heard that word before.“
Michael Kreuter explains what IPF is and why early diagnosis is so important. “IPF is a progressive and chronic lung disease characterised by progressive scarring of the lungs. We don’t want to lose lung function, so we think early diagnosis is essential.“
Rainer Karpavicius, who is from Schwetzingen, Germany, first noticed some troubling symptoms on one of his regular hikes with friends – he was very out of breath, had a persistent cough and struggled to keep up with the group.
I didn’t look forward to going out anymore. When the group said they were going hiking, I no longer looked forward to it because I would be the one at the back and start the coughing again. I simply couldn’t go with them.
After going from doctor to doctor trying to seek clarity, Rainer Karpavicius was eventually referred to a specialised lung disease clinic, where he finally found out after two years of uncertainty that he had IPF. He recalls how incredibly stressful his long journey to diagnosis was; he felt physically exhausted and depressed by his symptoms, and uncertain about what his future would hold.
It was a terrible time for me. People were asking me: ‘Have you any got any idea what is wrong with you?’ And I had to say: ‘No, I don’t know.’
Later, Rainer Karpavicius would learn that the earlier IPF is diagnosed, the better for patients managing the condition. He therefore looks back on his long journey to diagnosis as wasted time – time that he wishes could have been spent more meaningfully.
Michael Kreuter stresses that early diagnosis is essential in order to preserve lung function, as eventually all patients with IPF will experience disease progression over time.
[00:00]
IPF, Idiopathic Pulmonary Fibrosis, is a progressive and chronic lung disease characterized by progressive scarring of the lungs. And why do we think that early diagnosis is so essential? Well, we know that whether a patient is in the early stages, moderately impaired, or severely impaired, the progression over time is the same. So we don't want to lose time, we don't want to lose lung function — that's why we think early diagnosis is essential. How to do that? If you're suffering from cough or dyspnea, it's not just because you're aging — it's because you may have some kind of disease. So, if you have these symptoms, go to your GP, and maybe you'll need to be referred to a pulmonologist.
[00:35]
And what is also essential? The physician who is seeing you should use their stethoscope to auscultate your lungs. If they hear something we call Velcro crackles — and you really can hear that your lungs have crackles — then the probability that you're suffering from pulmonary fibrosis is very high, and further diagnostic procedures should be pursued.
[01:08]
And what kind of early treatment is essential? Well, we know that it is a progressive disease, and we want to halt that progression. We have a couple of treatment options: pharmacological and non-pharmacological. The non-pharmacological ones are especially important to prevent, for instance, infections — like vaccination, which is very helpful — or engaging in physical activity, like pulmonary rehabilitation.
[01:38]
And there are also drugs which can influence the further course of the disease.
Chapter 2
The burden of cough
Rainer Karpavicius shares his personal experience with cough – a debilitating and often embarrassing symptom of IPF. “You don’t really feel comfortable going out in public anymore, because you’re always scared that you will start coughing.“
Michael Kreuter talks about cough, one of the most distressing and disabling symptoms in people with IPF that is associated with a deterioration in their quality of life. “Sometimes the cough is so severe that it can really impact your life and, especially, it can really impact your social life.“
Can you imagine having the urge to cough that cannot be relieved by actually coughing? That is the reality for people living with IPF, who sometimes cough up to 3,000 times a day.
For Rainer Karpavicius, daily life is disrupted by coughing. Simple activities such as walking upstairs and participating in business meetings become physically and mentally exhausting when a cough attack strikes. On many occasions he has chosen to cancel dinner plans or concert outings as his cough makes him afraid to sit in a big room full of people. He fears distracting or attracting the attention of others, who will inevitably wonder what is wrong with him.
One evening we were having a nice dinner at a restaurant, and we were talking and making jokes. And in that moment, as I started laughing, the laugh turned into a continuous cough. I had to cough loudly and cough and cough. I couldn ’t get any breath and it got so loud. When I looked to the right and left, I noticed that all the tables were looking at us. It was a very uncomfortable situation for me.
Michael Kreuter witnesses first-hand the impact that cough has on his patients’ quality of life – every single day.
[00:00]
So what are the main symptoms in patients suffering from IPF? There are two. One is dyspnea, and the other — a very debilitating one — is cough. What does cough mean? Sometimes cough is so severe that it can really impact your life, and especially your social life. Because we all know: if you go to a concert, the cinema, or the theatre, and people are coughing, then not only are you disturbed — the people around you are disturbed too. So what could cough mean?
[00:28]
It could mean that you start to avoid going out to social interactions. So what should you do about cough? First of all, think about where it may come from. It could be related to reflux, or to other medications that have nothing to do with your disease. But in many cases, it is related to your disease. So, we have to treat the disease — in order to treat the symptoms — and thereby improve your health-related quality of life. Treatment could include inhaled therapies, drug treatments,
[00:58]
or non-pharmacological options.
Chapter 3
Living an independent life with IPF
Rainer Karpavicius's advice to others with IPF is to continue going out and doing activities rather than sitting at home waiting. “It is extremely important, despite the illness, to stay active.“
Michael Kreuter shares his advice for maintaining independence in life, even when IPF is holding you back. “What does independence from a disease mean? Well, I think the best thing to say is, you should control your disease and not let the disease control you.“
Independence to me is meeting with friends, relatives, keeping in touch. That I regularly communicate with them… I am also active in a sports club, I play in a golf club, regularly with friends and co-workers. In other clubs I am also active, so this freedom, this independence is something that I want to keep as much as possible. And all these activities, I can feel myself, it is very, very helpful. If I don’t do it, something is missing, and I can feel this is not good for my lungs and my head.
It is extremely important, despite the illness, to stay active. To stay fit, go to a sports group and support groups where you can exchange with one another. The social environment should be kept, you should stay active. This is one thing that I can recommend to everybody: not to keep quiet, to sit at home and wait – but to go out and to do activities.
You have to be independent for yourself and your quality of life, with your family, with your friends, and to not to have the disease control you.
[00:00]
So what does independence from a disease mean? Well, I think the best way to put it is: you should control your disease — don’t let the disease control you. You need to be independent for yourself, for your quality of life, for your family, and for your friends. Don’t let the disease take over. Don’t avoid going to the cinema, or concerts, just because you have the disease. Yes, you have it — but you can control it. If you have options to treat the symptoms, then you have options to maintain your quality of life,
[00:36]
meaning you can still have social interactions. And that, to me, is independence. Is independence something we can read in a book or something clearly defined? No — independence is in your book. You know what it means to you, and you know what you want to do about it. Talk to your friends about it, your family, your caregivers — and especially speak with your doctor about what independence means for you. If you start to feel like your disease is controlling you and impacting your hobbies — whether that’s sports or something else — and you think you can’t maintain them,
[01:05]
then think about other ways to approach them. For example, is there another way to enjoy your hobbies? Is there an alternative? If you were interested in literature before, maybe you can pick that up again. If you love going to the cinema, perhaps you can still do that — or explore new ways to enjoy it. Are there alternative sports or physical activities that you can try? Think of these alternatives — and I’m sure that will help you.
Chapter 4
Encouraging open conversation
Reiner Karpavicius's relationship with his doctor helps him to better take control of his IPF.
During the patient-doctor meeting, I tell them about what activities I am still doing and what is important to me, so that the doctor can refer to these things and give me advice.
Michael Kreuter encourages his patients to be completely open with him about symptoms and their life with IPF.
Write everything down you want to know, that you want to ask your physician, or that you want to ask your caregivers. Come back to the people you spoke to, they will help you at any time.
For Rainer Karpavicius, it’s important to feel well-prepared ahead of his appointments with his doctor to be able to have an open and positive conversation about his life with IPF.
[00:01]
When I got the diagnosis — lung fibrosis — I was confused. Because I didn’t know what it meant for me, and I didn’t know what it was. Later, I learned that it is very important to be prepared when you go to the doctor — that you have noted your questions, what you want to ask, on a piece of paper. So I wrote down all the questions I wanted to ask the doctor. And then, regularly, I also noted the answers I received. That gives you a very good picture at the end of the conversation with the doctor.
[00:37]
When I go to the doctor, it’s very important to me that we maintain open communication. I tell him about my achievements, about my goals — and I expect that he is going to help and support me in how I can achieve those goals.
Come to us, speak to us, bring a list, make notes, ask whatever you'd like to do, and don't be shy.
[00:00]
What is the best way to educate yourself — or be educated — on the disease you're suffering from? Well, I think we are all human beings, and we are all very different. That means you are different, and we — as the ones providing education — are different too. However, there’s a very useful framework we use to help guide education. Together with a couple of colleagues, we call it the A to E of care for patients with the disease you are suffering from — in this case, idiopathic pulmonary fibrosis.
[00:30]
"A" means we want to assess — to see where you are. Then we want to back you up, and that’s "E" for educate. In our interaction with you, we can learn what kind of information you want to know. What’s on your list? And by the way, having a list is a good thing. When you first hear about your disease, just take some time at home to write everything down — what you want to know, what your physician should know, what your caregivers need to ask — and then bring that list to your appointment. At the end of the day, your list should become our checklist — everything should be answered.
[01:02]
So what is education? Education means we want to tell you everything you want to know about the disease. That might include reliable educational materials you can access: trustworthy websites, helpful books, or other sources. We also aim to connect you with patient support groups, because they often know things we may not — from real-life experience with the disease. That’s part of backing you up too. Then we come to C, D, and E. "C" is for comfort care — helping you manage your symptoms, because you asked for that. "D" is for disease-modifying agents — treatments that can slow disease progression.
[01:33]
And "E" — well, it’s something we also have to talk about. You’re living with a severe disease, one with a high burden, and sometimes we need to talk openly about end-of-life care. That, too, is essential. So, come to us. Speak to us. Bring a list. Make notes.
[02:04]
Ask whatever you’d like to ask. And don’t be shy — come back to the people you spoke to. They’ll help you, any time.