PF, which is also known as scarring of the lungs, is an uncommon and frequently fatal lung disease. This condition causes inflammation and scars to build up in the lungs, making them unable to transport oxygen into the bloodstream effectively.
Eventually, the lungs become stiff, forcing the breathing muscles to work extra hard. The brain senses this extra work and triggers a feeling of breathlessness in the patient.
“PF is a very serious, life-limiting illness that many are not aware of, and the process of diagnosis can be long and challenging,” says Dr Syazatul Syakirin Sirol Aflah, respiratory physician at Institut Perubatan Respiratori (IPR) in Kuala Lumpur.
“It encompasses a group of more than 200 disorders that cause scarring of the lungs. Most of the causes are not identifiable.”
According to her, patients have an average survival rate of two to five years after diagnosis, and there is no cure. “There is, however, treatment to reduce the progression of the disease,” she adds.
“Some known causes and risk factors are environmental exposure to harmful elements such as mould and animals with feathers; consuming long-term medication that affect the lungs; autoimmune diseases such as rheumatoid arthritis; as well as genetics and smoking.”
Observations during the SARS and MERS epidemics have shown a link to this condition. And while the implication of Covid-19 has not been not fully studied, growing clinical experience suggests PF is likely to be one of the pandemic’s most significant respiratory complications.
Mistaken as asthma
PF affects the patient’s quality of life, as daily activity becomes more limited and they would need to start relying on family members and other caregivers.
Sadly, PF is often mistaken for asthma or other respiratory diseases because of the similarities of symptoms. Shortness of breath and a persistent dry cough are the most common early indicators.
The patient might find it difficult to climb stairs. Later on, even walking or sitting can be tiring.
As the disease progresses, the patient usually develops widened and rounded tips of the fingers resulting from a shortage of oxygen, a condition known as “finger clubbing”.
Early detection is important to increase the effectiveness of managing PF, which primarily affects those over age 50 and men more than women. “But this does not mean the younger age group is not susceptible,” Syazatul cautions.
Treatments include oxygen therapy, pulmonary rehabilitation, medications, and lung transplantations, while clinical trials evaluating potential new medication are ongoing at five medical centres around Malaysia.
More information on pulmonary fibrosis can be found at the Malaysian Interstitial Lung Disease Awareness page on Facebook.