Diagnosis and treatment of rare disease in the world is still being fought for to be made easier and known by many people.

Disease is said to be rare if there are less than 2,000 people in the world. There are around 6,000-8,000 rare diseases in the world, and it is only about 120 of them succeeded in diagnosing with certainty. One of them is Idiopathic Pulmonary Fibrosis (IPF). The term “idiopathic” has an unknown meaning, which makes it a suitable name for a disease that is unknown to many people. It is also not clear why idiopathic pulmonary fibrosis (IPF) exist.

The popular case of this pathology is about Crown Princess of Norway, Mette-Marit, when she was diagnosed with a chronic lung disease called pulmonary fibrosis, or lung disease that occurs when lung tissue becomes damaged and injured. The princess was treated at the University Hospital in Oslo and was also handled by experts from abroad. The royal doctor, Professor Kristian Bjøro, said that Princess Mette-Marit had experienced gradual changes in her lungs over several years and that her illness was not possible based on lifestyle.

Pulmonary fibrosis is a lung disease that occurs when lung-tissue becomes damaged and injured. It is a respiratory disorder caused by the formation of scar tissue in the lung organs. When the lung-tissue tightens and thickens, it makes it harder for your lungs to work properly. When the condition worsens, you become increasingly short of breath. This condition makes you experience difficulty in breathing naturally. Moreover, in severe conditions, breathing becomes more difficult. This condition will cause the lungs to not function normally. This abnormal lung function will cause a person to experience shortness of breath, even when only doing light activities, such as walking or wearing clothes.

In Indonesia, IPF is not yet popular and that’s why this disease experiences nearly 50 percent misdiagnosis because many of its symptoms are similar to other chronic lung diseases, such as TB (tuberculosis), pneumonia, and asthma. IPF occurs because of scarring that appears in the lung tissue which causes oxygen to be difficult to find a way into the body. The nature of the disease is irreversible, that is, it will not return to normal, and this scar will continue to appear. Pulmonary fibrosis is a lung disease that worsens slowly and is not contagious. This condition can be caused by various factors and can be experienced by anyone, but it is more common in adults and the elderly.

A pulmonary specialist in Indonesia Dr. Agus Dwi Susanto, Sp. P, stated that IPF cannot be cured. Because this disease progressively decreases lung function and causes the sufferer to have difficulty breathing, leading to death. A common problem faced by rare diseases in Indonesia is the lack of available medicines and treatments. However, in January 2018, the Food and Drug Supervisory Agency (BPOM) agreed that one of the drugs for IPF could enter Indonesia through the fast lane and was legally registered.

Symptoms of pulmonary fibrosis

Besides shortness of breath, other symptoms that may be experienced are dry cough, weight loss without reason, you get tired quickly, muscle and joint pain, and widening and rounding of the fingers or toes. The severity of symptoms can vary from person to person. There are people who experience symptoms quickly for several days. There are also symptoms that occur slowly but last for several months or years. If the condition persists, it can cause serious health complications, including high blood pressure in your lungs (pulmonary hypertension), difficulty breathing, lung cancer, and other lung complications.

Causes and risks

When searching for the cause of this condition, doctors often cannot determine the cause of the scar tissue that forms in the lungs. However, it can be various factors, including long-term exposure to certain poisons, certain medical conditions, radiation therapy, and several types of drugs. For exposure to a number of poisons and pollutants, including dust, silica dust, asbestos fibres, metal dust, coal dust, or animal dung. Meanwhile, factors from radiation therapy, could be derived from radiation therapy for lung or breast cancer.

The severity of the damage may depend on how much the lungs are exposed to radiation, the amount of radiation given, whether chemotherapy is added, and the administration of drugs. Drugs that are at risk of damaging the lungs are chemotherapy drugs, such as methotrexate and cyclophosphamide, heart medications such as amiodarone, antibiotics such as nitrofurantoin and ethambutol, and anti-inflammatory drugs such as rituximab and sulfasalazine.

Other factors that can cause pulmonary fibrosis are autoimmune diseases, your immune system attacks itself, for example lupus, scleroderma, polymyositis, dermatomyositis, or vasculitis. In addition, it could also be due to bacterial or viral infection of hepatitis C, adenoviruses, and herpes viruses. In many cases, the cause of pulmonary fibrosis is unknown. When this happens, this condition is called idiopathic pulmonary fibrosis (IPF).

There are also people who are at risk of developing this disease, among others, elderly, men, who are treating cancer, smoking, genetic factors, and working in mining, agriculture or construction. So, can this condition be cured? Lung damage caused by pulmonary fibrosis cannot be repaired, but medications and therapies can sometimes help relieve symptoms and improve quality of life. Lung transplantation may be done for some patients with very severe cases.

Here are several factors that can trigger the formation of scar tissue, including:

Work environment

Hazardous chemical particles, such as asbestos fibres, coal powder, and metal dust, risk damaging lung organs if the exposure lasts for a long time. Chemical particles can be found in mining, agriculture and building construction areas.

Certain diseases

Pulmonary fibrosis can develop from several diseases, such as pneumonia, rheumatoid arthritis, scleroderma, and sarcoidosis.

Certain medications

Several types of drugs can damage lung tissue, such as chemotherapy drugs (methotrexate and cyclophosphamide), heart disease medications (amiodarone), antibiotics (nitrofurantoin and ethambutol), and anti-inflammatory drugs (rituximab and sulfasalazine).

Radiotherapy

Radiation therapy or radiotherapy that is generally given to treat cancer risks damaging the lungs, especially if done for a long time. Symptoms of lung damage can be seen in several months to several years since the patient is exposed to radiation.

In addition to some of the causes above, there are also factors that can increase a person’s risk of pulmonary fibrosis;

Age and gender

Most sufferers of pulmonary fibrosis are people aged between 40-70 years. However, this condition can also be experienced by infants and children. Pulmonary fibrosis is also more experienced by men than women.

Smoking habit

The risk of active smokers or people who have smoked to develop pulmonary fibrosis is higher than people who have never smoked at all.

Heredity

Pulmonary fibrosis can be inherited in families. In some cases, people with pulmonary fibrosis are known to have family members who also suffer from this disease.

When you should see a doctor?

Every worker who is at risk of exposure to hazardous particles, such as silica dust or asbestos fibres, needs to carry out regular checks to the doctor regularly, usually once a year, depending on company policy. In addition, these workers must also wear personal protective equipment to prevent damage to the lungs. Immediately consult a doctor if you experience coughing for more than 3 weeks, especially until shortness of breath. This condition requires special treatment from a doctor.

After asking the patient’s symptoms and history, the doctor will use a stethoscope to check the sounds in the lung organs. The doctor can also do additional tests to confirm the presence of pulmonary fibrosis. Here are few tests to do:

Imaging test

Imaging is done by chest X-ray, CT scan, or MRI, to check the condition and structure of the lungs.

Lung function test

This test can be done with spirometry, oximetry, and blood gas analysis, to check the performance of the lungs as well as the levels of oxygen and carbon dioxide in the blood.

Biopsy

This procedure is carried out to confirm the diagnosis and detect the severity of pulmonary fibrosis, through examination of lung tissue samples.

Blood tests are also carried out to check the function of other organs, such as the kidneys and liver, and detect infections. Because the symptoms of pulmonary fibrosis are similar to the symptoms of heart disease, your doctor may perform a cardiac echo examination and a treadmill ECG to check heart function.

Treatment of Lung Fibrosis

The doctor will determine the type of pulmonary fibrosis treatment based on the severity. Here is some treatment which can be done for pulmonary fibrosis besides medications like pill etc.:

Additional oxygen

Oxygen is given to prevent the body from lacking oxygen, as well as to improve sleep quality.

Lung rehabilitation

Pulmonary rehabilitation is done by practicing physical endurance and breathing techniques to improve the workings of the lung organs, so that it will relieve symptoms.

Lung transplantation

Lung transplantation is done if the condition of the lungs is severe and other treatments are not effective for treating pulmonary fibrosis. This method is done by replacing damaged lung organs with healthy lungs from the donor. However, this procedure risks causing the body to reject new organs.

In addition to medical treatment, the doctor will also encourage patients to change lifestyles, so that the treatment and recovery process takes place faster and there are no complications. The steps that need to be done are stop smoking and avoid cigarette smoke, eat foods which high in fibre, such as fruits and vegetables, and avoid foods high in salt and fatty foods. The most important is try to have more time to rest and doing exercise regularly.

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