Until my father was finally diagnosed with Idiopathic Pulmonary Fibrosis (IPF), I had never heard of IPF before. But for my father, finally having a diagnosis meant an end to his sense of helplessness and frustration.
In his own words, “Finally, my disease has a name.”
For a long time my father had had this hacking, hacking cough that everyone assumed would just go away. He gradually got worse and was making such a fuss during the conversation that we were very concerned and insisted that he see a doctor immediately.
Little did we know that he was battling such a debilitating disease! Years later, and numerous tests, she didn’t realize it and no doctor could explain why her condition continued to deteriorate.
Finally, within a fortnight of being admitted to Mount Sinai hospital in New York and undergoing further tests, he finally received a dual diagnosis of IPF and squamous cell cancer.
Although cancer is still the most feared disease, IPF was the worst for my father. The good news was getting a diagnosis. The bad news was that 87% of patients diagnosed with IPF die within two years.
Despite his prognosis, my father bravely decided to fight the cancer with chemotherapy and radiation until his lungs gave out six weeks later.
Losing my father to IPF opened up a whole new world for me; and a relatively unknown and often misdiagnosed disease that claims up to 5 million lives worldwide.
What is Pulmonary Fibrosis?
Pulmonary fibrosis (PF) literally means lung scarring. Scarring develops when the tiny air sacs that transfer oxygen to the blood become damaged and inflamed. The body attempts to repair the damage by scarring the lung tissue. But this scarring thickens and stiffens the lungs, causing an irreversible loss of the ability to transfer oxygen into the bloodstream.
Symptoms of pulmonary fibrosis include shortness of breath, chronic cough, fatigue and weakness, chest discomfort, loss of appetite, and rapid weight loss.
What causes IPF?
Traditional theories have postulated that it could be an autoimmune disorder, in which the body’s immune system attacks its own tissues. A growing body of evidence points to a genetic predisposition. Other known causes of PF are inhaled environmental and occupational pollutants.
In most cases, like my father’s, when the cause is unknown, it is called Idiopathic Pulmonary Fibrosis (IPF), which means “unknown cause.”
How widespread is IPF?
- At least five million people suffer from Pulmonary Fibrosis worldwide.
- It claims as many lives as breast cancer each year and affects both men and women, with a slightly higher incidence in men.
- The age of onset is between 40-70 years, but it can occur at any age. Although unusual, it has been diagnosed in children as young as three years of age.
- It does not have a specific geographic location, and can be found in equal proportions in urban and rural settings.
- Marlon Brando died of pulmonary fibrosis last July at the age of 80
There are currently no effective treatments or cures for IPF, nor is there a known prevention.
The biggest problem for PF patients is their invisibility; they are the unseen victims of a disease most people have never heard of.
My last memories of my father were having his oxygen equipment and the tubes that led to the concentrator or cylinder wherever he went. He could barely get away from his bed for fear that my two year old nephew would grab him and pull the tubes.
During our last phone conversation, the day before he died, he could hardly breathe but he insisted on talking.
It was too late for my father, yet through public education, advocacy and increased funding for research, 5 million patients around the world wait in hopes of finding a cure for Idiopathic Pulmonary Fibrosis. .