Living with IPF

IPF (idiopathic pulmonary fibrosis) is a terminal pulmonary disease with a life expectancy of 2 to 5 years after diagnosis. The lungs become fibrotic, "rubber-like", unable to function and maintain adequate oxygen levels in the blood. IPF affects more men than women and the incidence of the disease is increasing with age. The most likely patient is a 60 to 80 years old male. The cause of the disease in unknown, therefore, the name "idiopathic". There are no drugs approved for the therapy of IPF. Professional societies suggest steroids (e.g. prednisolone) and immunosuppressants (e.g. azathioprine). This therapy has not been shown to prolong life and is associated with severe side effects. Therefore, some pulmonologists recommend only oxygen as the sole therapy. The first symptom of the disease is dry cough, particularly in the morning. Gradually the patients develop shortness of breath, particularly in climbing hills or steps. The patients "desaturate" , this means that their blood oxygen saturation (as measured with finger oximeter) is easily diminishing, particularly with bending or any exercise. Normal oxygen saturation is 95 to 100%. while in patients with IPF it can easily decrease to 85 or even 75% after climbing a few steps. Oxygen saturation below 90% is associated with dizziness and further decline of oxygen levels can lead to loss of consiousness. The eventual death is usually multi-organ failure due to chronic oxygen deficiency. Pneumonia or any respiratory infection is likely to be deadly for patients with IPF. Only lung transplant offers a chance for IPF patients to avoid premature death. Many elderly patients are not eligible for lung transplant and, those who are, have to wait years for the availble lungs. It is not easy to live with IPF. Cough attacks and inability to perform daily tasks are particularly disturbing. Exercise under oxygen protection and psychological counseling can make the life of an IPF patient a liitle easier. Cough attacks do not respond to usual over-the counter antitussive drugs, but can occasionally respond to hydrocodone/homatropine mixture. Antioxidants have been shown to prevent experimental fibrosis in animals and are often used as a supportive therapy in patients with IPF. All IPF patients wait impatiently for development and release of new drugs, but curative therapy is not yet on the horizon. Alexander Scriabine, MD and also a patient with IPF