Unknown and irreversible and deadly: pulmonary fibrosis is a respiratory killer

EVERY 15 minutes, somebody in Europe dies of Pulmonary fibrosis (PF). Pulmonary fibrosis is the fifth biggest respiratory killer in Europe after lung cancer, COPD, pneumonia and Covid-19. It accounts for over 1% of all deaths and kills more people than either leukemia or ovarian cancer. 

Pulmonary fibrosis, which is also known as scarring of the lungs, is an uncommon and frequently fatal lung disease that makes walking across a room seems like scaling Mount Everest. This condition causes inflammation and scars to build up in the lungs, which makes the lungs unable to transport oxygen into the bloodstream effectively.

Eventually, the lungs will become stiff and this makes your breathing muscles work extra hard just to pull air in with each breath. Your brain senses this extra work, and it lets you know there is a problem by triggering a feeling of breathlessness (or 'shortness' of breath) while exerting yourself.

In an effort to educate the public on the disease, Dr Syazatul Syakirin Sirol Aflah, respiratory physician of the Institut Perubatan Respiratori (IPR) shared, “PF is a very serious life-limiting illness which many are not aware of and the process of diagnosis can be long and challenging. It encompasses a group of more than 200 disorders that cause scarring of the lungs.

"Most of the causes are not identifiable and this is called Idiopathic Pulmonary Fibrosis (IPF) which usually has a median survival of 2-5 years after a diagnose and to date there is no cure. However, there is a treatment to reduce the progression of the disease.”

Early detection is of prime importance when it comes to pulmonary fibrosis as the faster you detect it the better chances you have in managing the disease. Dr Syakirin added, “The disease primarily affects patients over the age of 50 and affects more men than women. However, this does not mean the younger age group is not susceptible.

"Some known causes and risk factors of PF are environmental exposure to harmful elements such as mold and animals with feathers such as birds; consuming long term medication that are known to affect lungs; autoimmune disease such as rheumatoid arthritis; genetics and smoking.”

Viral infection has shown an association in PF. The PF learnings observed in Sars and Mers (Middle East Respiratory Syndrome) epidemics highlighted a subsequent outcome into PF which puts into question the implications of Covid-19. Although, the implication of Covid-19 is not fully studied, growing clinical experience suggests that PF is likely to be the most important respiratory complication.

Although a direct relationship between respiratory viral infection and development of progressive fibrosis has not been fully established, evidence from the previous global Sars outbreaks with Sars‐CoV and Mers shows a clear link between coronavirus infection, persistent impairment of lung function and abnormal radiological findings consistent with PF.

Hamshah Ali, a 65-years-old, an IPF patient said, “Every day I would play football, rugby, jogging then all of a sudden everything is down. The simple activity that I used to do such as gardening becomes harder as I easily get tired after 1 hour.”

PF affects the patient’s quality of life, your activity will become limited and you will start relying more on your family members and caregivers. 

PF is often mistaken as asthma or other respiratory diseases due to the similarities of the symptoms. Although hard to detect, the most common symptoms and signs of PF is shortness of breath and a persistent dry cough. At first, you may find it difficult to climb up the stairs and later even walking on flat ground or sitting is tiring.

As the disease progresses the patient will develop widened and rounded tips of the fingers (finger clubbing) resulting from a shortage of oxygen. If you faced any similar symptoms or signs of PF, do seek medical advice as early and accurate diagnosis is important. 

With the advancement of research, clinical trials, and technology there are treatments designed to specifically manage the disease such as oxygen therapy, pulmonary rehabilitation, the use of medications and lung transplantations.

Clinical trial evaluating a potential medication for idiopathic pulmonary fibrosis is being conducted in 5 centres Institut Perubatan Respiratori (IPR), Hospital Raja Perempuan Zainab (HRPZII), University of Malaya Medical Centre (UMMC), Universiti Kebangsaan Malaysia Medical Centre (UKMMC) and Universiti Teknologi MARA Medical Centre (UiTMMC). 

Get more information on PF from the first official Malaysian Interstitial Lung Disease (MY ILD) Awareness Facebook page. – The Vibes, November 29, 2021