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There is no cure available for idiopathic pulmonary fibrosis (IPF), so the current treatment involves symptom relief and the slowing the progression of the disease. The optimal treatment depends greatly on the individual circumstances and the severity of the condition.
It is important that patients with IPF are encouraged to take care of their body and help to protect their lungs from further damage, in addition to treatments. This may involve stopping smoking, regular exercise, a nutritious diet and avoidance of triggers.
The most common complaint from people with IPF is breathlessness, which may affect them constantly throughout the day or become worse during light movements such as walking.
Oxygen support can be beneficial when the oxygen levels in the blood are lower than normal, as a result of insufficiency of the lung function. Oxygen can be administered via a mask or nasal tubes from a portable oxygen concentrator.
Pulmonary rehabilitation can also be used to manage breathlessness by aiding patients in their day-to-day function and coping with respiratory issues. This usually helps patients to understand the condition and encourages them to eat well, stay active and learn breathing techniques to improve symptoms. It is also a good place to find a network of other individuals facing the same issues and getting psychological support.
The suitability of medications in the treatment of IPF is highly variable. For example, patients with severe symptoms are likely to benefit than those with mild symptoms. Additionally, the risks of treatment may out weight the benefits for elderly patients that are more susceptible to side effects.
Pirfenidone can be used to slow the progression of disease and scarring of the lung tissue, due to its immunosuppressive action. This is usually recommended for adult patients with a lung capacity 50-80% of the expected normal value, and therapy should be ceased if lung function reduces during treatment. Side effects may include gastrointestinal effect, fatigue and photosensitivity.
N-acetylcysteine is an antioxidant that may be able to reduce the scarring and damage to the lung tissue. There is some evidence to support its use in the treatment of IPF, but the extent of its action has not been established.
Nintedanib is an anti-fibrotic kinase inhibitor that is currently in the development process and has not yet been approved for public use but is a promising solution for the future.
In the past, steroid medications were often recommended due to the belief that the pathology of the condition involved inflammation of the lung tissue. However, this therapy had a relatively low success rate, and it is now controversial if inflammation is involved in the mechanism at all. Steroid medications may be recommended as a trial of several to test the effect on each individual and continued if a positive effect without side effects is noted.
In some cases, the symptoms of IPF continue to worsen with treatment and there may be the need for a lung transplant. This is highly variable and depends on the severity and progression of the condition, in addition to individual factors about the patient that may affect the outcomes, such as age and other health conditions.
A lung transplant can be very useful to improve both quantity and quality of life for individuals with severe disease. Rejection and infection are the two major concerns involved in this surgery and patients that undergo the procedure require ongoing treatment to reduce the risk of infection for their lifetime.