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.
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.
Chapter 2
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.
Chapter 3
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.
Come to us, speak to us, bring a list, make notes, ask whatever you'd like to do, and don't be shy.