Pulmonary Fibrosis

What is pulmonary (lung) fibrosis?
Lung fibrosis, or connective tissue formation (densification) in the lung tissue, is a chronic condition in which the lung tissue does not function as well. In pulmonary fibrosis, the lung tissue has been transferred into scar tissue (fibrosis). This scar tissue will not go away. Often more and more healthy lung tissue disappears. The result is that the lungs are less able to absorb oxygen; breathing becomes more difficult. Normally the lung is able to take in enough oxygen for daily needs. In the case of pulmonary fibrosis, the ability to absorb oxygen is reduced. As a result, the person concerned becomes short of breath, is quickly tired and has little energy.

The most important complaints with pulmonary fibrosis are: shortness of breath: first when exercising later also at rest, coughing, tiredness and lacking energy. In addition, patients may suffer from concentration problems, headache and depressive feelings.
Some patients have an abnormal shape of the fingernails (watch glass nails) and thickening of the fingertips (drumstick fingers). Very rarely these deformations also occur with the toenails. The complaints continue to increase over time; the ability to make an effort decreases.

Cause and prevention
Lung fibrosis can be caused by inhalation of all kinds of harmful substances, the use of certain medicines and also radiation (radiotherapy). Familial forms of pulmonary fibrosis have also been described. In many cases, however, the cause remains unclear. Lung fibrosis occurs in both women and men. Every year an estimated 1000-1500 new lung fibrosis patients are added in the Netherlands.

Establish the disease
Determining the diagnosis of lung fibrosis is difficult: it requires specialized knowledge. Reference to or consultation with a specialized center is then often desirable. In the Netherlands there are a number of centers that specifically deal with this group of rare lung diseases, including the ILD Expertise Center of the St. Antonius Hospital in Nieuwegein.

The course and the prognosis can vary enormously. Full recovery almost never occurs. Adequate treatment is not yet available. The course of lung fibrosis is difficult to predict. This differs per person and per form of the disease. However, the course is always characterized by deterioration. Sometimes fast; sometimes gradual and barely perceptible. Lung fibrosis is a chronic disease that cannot be cured. The damage to the lungs by the scars is permanent.

If the cause is known, it is first of all important to remove this cause. For example, you must ensure that you no longer inhale certain harmful substances. Or you must stop taking a certain medicine. In this way you prevent the disease from getting worse.
Inhibiting the process
There is no medicine for lung fibrosis. There are, however, means that prevent the process of scarring in the lungs. These drugs therefore inhibit deterioration, sometimes delay the course of the disease and can in any case help to reduce the symptoms. Regular monitoring by the attending physician is required for all forms of pulmonary fibrosis to monitor the course of the disease. The doctor can then intervene on time and possibly start or intensify treatment. Anti-oxidants and stomach acid inhibitors may have a positive effect, as well as the prevention of infections. New drugs are being searched diligently. In addition, oxygen administration and physiotherapy can have a positive effect on functioning. Checks can be checked in time to see whether your lungs still absorb enough oxygen. If this is not the case, oxygen can be prescribed. The patient often only uses this at night first, and later also during the day.
IPF treatment
In the case of pulmonary fibrosis with unknown cause (IPF), the treatment consists of combating the symptoms in order to try to maintain the highest possible quality of life. For the first time, there are now two drugs, pirfenidone and nintedanib, which inhibit deterioration in lung function and improve prognosis for mild to moderate IPF. They are not drugs that can cure IPF, but it can help to reduce the symptoms and slow the process of deterioration. Both funds are reimbursed by people who qualify for it (strict criteria have been set for this). Both drugs may only be prescribed in specialized centers. If you want more information then it is advisable to discuss with your pulmonologist what the options are and / or a referral to one of these centers makes sense in your case.
Lung transplantation
If the lungs are damaged in such a way that medication and oxygen do not help sufficiently, lung transplantation can be a last option. If this is the case, your treating doctor will discuss this with you in detail.

Lung fibrosis is a disease that can have major social and emotional consequences for the person concerned and his or her environment. This condition requires expert guidance, with attention for medical aspects, but especially for the influence on the quality of life. The Dutch Long-fibrosis Patients Association has set itself the goal of providing information and encouraging contacts with fellow patients.

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