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1st BREATH OF FRESH AIR
Idiopathic pulmonary fibrosis presents with non-specific symptoms similar to those of other common conditions such as asthma, COPD and congestive heart failure. The patient’s general practitioner, once the disease is suspected, will refer him or her to a specialist. The tests used to confirm the diagnosis include spirometry (or lung function test), the 6-minute walk test, which measures the distance walked by the patient at his/her own pace in 6 minutes, or a high resolution CT scan (HRCT) to identify the pulmonary scar pattern.
HOPE: Diagnosing Idiopathic Pulmonary Fibrosis in its initial stages is fundamental for applying the most effective treatment.
1 American Cancer Society. Cancer Facts and Figures 2009. Atlanta, GA: American Cancer Society, 2009.
2nd BREATH OF FRESH AIR
More than 85000 people in Europe have Idiopathic Pulmonary Fibrosis, an orphan disease of unknown cause. It is estimated that 30,000 to 35,000 new patients will be diagnosed every year.1
IPF is more common in men than in women and it is normally diagnosed between the ages of 40 and 80.2,3 Although it’s cause is unknown, there are some risk factors, such as a family history of IPF, smoking and environmental factors.
HOPE: Research is crucial in order to find the cause of idiopathic pulmonary fibrosis and bring hope to patients, as is increasing the population’s awareness of the disease.
1 Eurostat News Release. European demography. 110/2010. 27 July 2010.
2 American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med 2002; 165:277-304
3 Raghu G, Weycker D, Edesberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006; 176:810-816
3rd BREATH OF FRESH AIR
IPF affects everyone differently. We could say that its natural evolution is unpredictable. One group of patients with IPF experience a gradual reduction in their respiratory function after diagnosis. Others appear to have episodes of acute clinical decline (acute exacerbations) before their pulmonary function deteriorates, possibly marking the beginning of the disease’s terminal phase.1, 2 Finally, the disease’s duration is shorter in another group of patients in whom it appears to progress faster.1 However, it is always a progressive condition, which means that the respiratory symptoms always get worse.
HOPE: Collaboration and experience-sharing among everyone involved in treating this disease can help to provide new insights related conditions that patients can experience.
1 Kim DS, Collar HR, King TE. Classification and natural history of the idiopathic interstitial pneumonias. Proc Am Thorac Soc 2006;3:285–292.
2 Collar HR, Moore BB, Flherty KR, et al. Acute exacerbations of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2007;176:636–643.
4th BREATH OF FRESH AIR
Over time, breathing difficulties affect everyday activities more and more, reducing the patient’s quality of life. Although IPF initially only affects the lungs, it is associated with right heart failure in it’s more advanced stages.
Patients can follow certain habits which will help them to feel better to a certain extent. In any case, the pulmonary specialist always assesses each individual for the best treatment and options, to prevent possible complications.
HOPE: A balanced diet, moderate exercise (under medical supervision), stopping smoking, oxygen therapy (in case of respiratory insufficiency) and, of course, compliance with the prescribed treatment, are some habits that can improve patient’s quality of life.
5th BREATH OF FRESH AIR
To date, the only cure available for Europe patients is lung transplantation – in selected cases. New pharmacological treatments are being investigated, one of them is already available in Europe.
HOPE: in the last few years there have been significant advances in research, focusing on anti-fibrotic drugs that bring a ray of hope to people with Idiopathic Pulmonary Fibrosis.