According to a story from BBC, John Mason, age 80, is one of over 2,000 people in Wales who currently have idiopathic pulmonary fibrosis (IPF), a rare and ultimately lethal lung disease. Unfortunately, awareness about this disease is limited there, and many patients and rare disease organizations think that access to effective treatment needs to be greatly expanded.
About Idiopathic Pulmonary Fibrosis
Idiopathic pulmonary fibrosis is a rare, progressive, chronic lung disease in which the functionality of the lungs progressively worsens. As an idiopathic disease, the exact cause of the illness is not really understood in the medical field currently. However, some definitive risk factors have been identified, and include smoking, certain viral infections, and occupations involving farming or agriculture. Exposure to a variety of particles such as metal, wood, coal, and stone dust, mold spores, hay, and silica have also been implicated. A small number of people may be genetically predisposed. Symptoms include distinctive, crackles that can be heard with a stethoscope, a dry cough, clubbed digits, shortness of breath, loss of exercise tolerance, and oxygen deficiency in the blood. Treatments include a number of medications, lung rehabilitation, oxygen therapy, and lung transplant. Five year survival rate is around 40 percent. To learn more about idiopathic pulmonary fibrosis, click here.
Living With IPF
For John, his idiopathic pulmonary fibrosis has progressed severely. Climbing a flight of steps can be a fifteen minute procedure. He takes medication daily as well as oxygen supplementation, and has visits with a specialist in six month intervals. John has also lived seven years with the disease, which is longer than what is doctors originally told him and longer than the average patient in general.
Slow Treatment is No Treatment
A lung transplant is an option, but a wait time of about nine months is not uncommon. The British Lung Foundation is an important advocate for idiopathic pulmonary fibrosis, and according to their statistics, 32 patients have died waiting for a transplant in the last eight years.
The organization has also released a report calling for improvements in treatment access for idiopathic pulmonary fibrosis patients, who need consultation with specialists, anti-fibrotic medications, and more personalized treatment approaches overall. In addition, the report highlights major disparities in treatment between different health boards.
