Pulmonary fibrosis mainly affects the elderly and smoking is a risk factor. Some variants are part of rheumatic diseases; others are hereditary. But most commonly, the cause is unknown.
The most severe form (IPF, idiopathic pulmonary fibrosis) historically had a median survival of 3–5 years - but there are now drugs that slow the progression. The only way to recover completely is a lung transplant.
What is pulmonary fibrosis?
This means that the lung tissue is converted into connective tissue, a type of scar tissue. This causes the lungs to shrink in size and become stiff, and the patient experiences shortness of breath and reduced oxygen uptake, says Magnus Sköld, professor of pulmonary medicine at Karolinska Institutet.
How serious is pulmonary fibrosis?
Pulmonary fibrosis is always serious. The most severe type of pulmonary fibrosis has a prognosis worse than most types of cancer, says Jesper Magnusson, senior physician and associate professor at Sahlgrenska University Hospital.
How quickly or slowly does it get worse?
"It depends on which type you have. It also tends to worsen in episodes. There are periods of deterioration that come quite suddenly," says Jesper Magnusson.
Are you healthy after a lung transplant?
It is less serious than pulmonary fibrosis, but not the same as being completely healthy. You have a shortened survival. About one in ten people who have a lung transplant die in the first year, says Jesper Magnusson.
How is the rehabilitation?
Rehabilitation is active, combined with immunosuppressive drugs. But patients can go from severely impaired lung function to essentially regaining normal lung function. It is a fantastic effect, says Magnus Sköld.
What is required to get a lung transplant?
"Generally, it only requires that you are ill only in your lungs. If you are ill in other parts of your body as well, you won't be able to do it," says Jesper Magnusson.
How much reduced lung capacity do you have to have to get oxygen?
In the event of a serious reduction in oxygen uptake, oxygen can be provided. The first step is to provide it during exertion. The second step is to provide oxygen basically around the clock. Then you should have reduced oxygen uptake even at rest, says Magnus Sköld.
Each year, approximately 60 lung transplants are performed in Sweden, divided between Skåne University Hospital in Lund and Sahlgrenska University Hospital in Gothenburg.
Rehabilitation takes an average of about 3–4 weeks, but there are those who remain in hospital for two months.
About half of lung transplant recipients experience chronic rejection after five years. It is a condition for which there is no cure, but which can be lived with for a longer period.
Source: Jesper Magnusson, Sahlgrenska University Hospital.
