What is Idiopathic Pulmonary Fibrosis?

Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis

What is Idiopathic Pulmonary Fibrosis?

Idiopathic Pulmonary Fibrosis is the appearance of scar tissue in the lungs that cause damage and disruption of lung function. This damage causes the tissue around the air sacs in the lungs (alveoli) thickened and rigid, making it difficult for oxygen to enter the blood.

A person with Idiopathic pulmonary fibrosis will have difficulty breathing due to the conditions that occur in the lungs. This condition can be triggered by various factors, but the real cause is still unknown until now so that the disease is commonly referred to as Idiopathic Pulmonary Fibrosis .

The elderly are a group of people susceptible to the disease, which is 50 years of age and above. The incidence rate in men was higher than in women. Treatment of this disease aims to reduce the symptoms felt by the sufferer because the lungs damaged by this can not be repaired.

Causes of Idiopathic Pulmonary Fibrosis

Damage to the Idiopathic Pulmonary Fibrosis can be caused by several different factors, including radiation therapy or from certain drugs. Although the exact cause is still unknown, here are some key factors and risk factors that can trigger Idiopathic Pulmonary Fibrosis .

  • Medical condition. Pulmonary fibrosis can develop from several diseases possessed by a person, such as pneumonia, rheumatoid arthritis, sarcoidosis, dermatomyositis, and various connective tissue diseases.
  • Certain medications. Some drugs that can damage tissue in the lungs, including chemotherapy drugs, heart disease, anti-inflammatory, and antibiotics.
  • Treatment using radiation therapy. This treatment will damage the lungs if done in large quantities, a long time, there is an underlying lung disease, and combined with chemotherapy. Signs of pulmonary damage can begin to appear months to years since the first patient is exposed to radiation.
  • Environmental factors related to work or occupation. An environment or work that exposes a person to toxic substances or sources of pollution in the long run can also harm his lungs. Some risky substances and jobs include coal powder, asbestos, silica, hard metals, dust granules, animal or bird droppings, miners, construction, and agricultural labor.
  • Age and gender. Idiopathic pumonary fibrosis is more common among elderly and adult men than women or children.
  • Hereditary factors. Some Idiopathic pulmonary fibrosis is inherited in the family so that gene factors can be a risk factor of this condition.
  • Smoking habit. Tobacco smoke, smokers, and people who have smoked, and people with emphysema will have a greater risk of Idiopathic Pulmonary Fibrosis than people who do not smoke at all.

Symptoms of Idiopathic Pulmonary Fibrosis

Symptoms and severity of Idiopathic Pulmonary Fibrosis may vary with each patient. Patients with one can experience severe symptoms with rapidly deteriorating conditions, while others experience only moderate symptoms with slower progression.

Symptoms of Idiopathic Pulmonary Fibrosis develop regularly, usually lasting for more than 6 months, with symptoms most commonly experienced are shortness of breath and cough. Here are other symptoms of Idiopathic Pulmonary Fibrosis that need attention:

  • Shortness of breath until the patient has difficulty breathing well (dyspnea), even when doing activities that are mild, such as dressing. Not a few people who consider these symptoms as a result of age or lack of exercise.
  • Fatigue
  • Dry cough
  • Muscle and joint pain
  • Reduced weight for no apparent reason
  • The tips of fingers and toes are wide and rounded

Patients with worsening symptoms in a matter of days or weeks will require special treatment from a doctor. Immediately see a doctor if you have trouble breathing for some time and cough that lasts for more than 3 weeks.

Diagnosis of Idiopathic Pulmonary Fibrosis

Before recommending what treatment is appropriate for patients with Idiopathic Pulmonary Fibrosis, the doctor will perform a diagnosis process consisting of a history of patient and family history, physical examination, and various support tests.

In addition, the doctor will also check the symptoms and ask if the patient had been exposed to certain substances that could be the trigger of this disease. On physical examination, your doctor will check your lung ability by listening to the patient breathing.

There are several types of supplementary tests that the patient may have to go through to confirm the symptoms and diagnosis of Idiopathic Pulmonary Fibrosis :

Blood test. This test is performed to evaluate liver and kidney function and at the same time to exclude other health conditions.

Idiopathic Pulmonary Fibrosis function test. Some types of tests that may be done are:

  1. Spirometry – Measures how much air can be inhaled, retained, and excreted from the lungs.
  2. Pulse oximetry – Measure air saturation in the blood.
  3. Pressure test – Conducted when the patient is on a treadmill or a static bike for pulmonary function while on the move.
  4. Blood gas analysis – Measures the levels of oxygen and carbon dioxide in the blood by using patient blood samples.

Imaging test (scan) body. Some types of possible body scans are:

  1. Chest X-ray – To find out the scar tissue in the lungs and monitor the progress of the disease and the treatment that is being undertaken.
  2. CT scan – To find out how much damage the lungs experience more clearly.
  3. Echocardiogram – To know the structure and function of the heart especially the level of pressure on the right ventricle of the heart that can trigger complications of heart failure.

Test tissue or biopsy. This test is done by taking a small portion of the lung tissue to be examined in the laboratory. Some common types of biopsy are:

  1. Bronchoscopy – For smaller tissue samples using a small flexible tube inserted through the mouth or nose to the lungs.
  2. Biopsy through surgical procedure – To obtain a larger tissue sample using a video thoracoscopic surgical procedure (VATS) or a thoracotomy procedure ie open surgery. Both of these procedures are performed by making slices in the chest area, exactly between the ribs, and with general anesthesia in patients.

Treatment of Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosisdamage in these conditions can only be slowed down and reduced the impact of the symptoms by administering drugs and performing a series of therapies. If conventional therapy with drugs fails, lung transplantation may be recommended by your doctor. Here are some common methods of Idiopathic Pulmonary Fibrosis treatment.

  • Drugs – To slow the progression of Idiopathic Pulmonary Fibrosis and gastrointestinal disturbances commonly experienced by the owner of this condition. Some examples of drugs, namely pirfenidone, nintedanib. Some of the side effects of these drugs, among others rash, nausea, and diarrhea.
  • Pulmonary rehabilitation – To reduce symptoms and support body functions, exercise physical endurance, and improve breathing techniques to train lung efficiency.
  • Oxygen therapy – To make exercise and breathing itself easier, reduce the risk of complications due to low oxygen levels, and reduce blood pressure on the right side of the heart. This therapy will also help improve the quality of sleep and welfare of the patient. Therapy can be given when the patient is sleeping or exercising, and some are using it all the time.
  • Pulmonary transplant – In addition to the good benefits of lung transplantation, the doctor will also discuss the risk of complications in the form of a body rejection of a replacement organ to a patient.

In addition to medical treatment, Idiopathic Pulmonary Fibrosis sufferers may also start taking active action in their ongoing care to maintain and improve their own health. Some steps that can be done, are:

  • Follow the ongoing treatment plan, doctor’s appointment, doctor’s instruction, medication schedule, and follow the recommended diet and exercise schedule.
  • Maintain a balanced and more balanced nutrition with smaller portions of food. This step is important because people with Idiopathic Pulmonary fibrosis generally lose weight due to the condition that makes it uncomfortable when breathing also when eating. Discuss food menu choices that suit your condition.
  • Quit smoking. If you have trouble stopping this habit, you can discuss what programs are suitable to be with your doctor. Avoid also being near people who smoke because inhaling cigarette smoke can also affect the lungs.
  • Regular body work can help maintain lung function and control stress. Activities such as walking and cycling can be done but will take time and process. Discuss with the doctor what type of exercise suits your condition.
  • Make sure you have enough rest time so you will have more energy and can reduce the stress felt due to this condition.
  • Make sure you and your family get the annual pneumonia and flu vaccine to avoid respiratory infections that can worsen the condition of Idiopathic Pulmonary Fibrosis. Avoid also meeting with many people in the flu season is spreading.

In some people, treatment of Idiopathic Pulmonary Fibrosis can have a good effect so they can not be disturbed by symptoms from this condition for years. But there are also conditions that deteriorate rapidly.

Idiopathic Pulmonary Fibrosis is one of the most common diseases that can worsen over time. Being active and learning about this disease can help you as well as the people around in through the treatment process.

Being open with your doctor is also recommended especially when stress and depression strike. Your doctor may refer you to a mental health professional or suggest other alternative measures to relieve your condition.

Routine health monitoring can help patients understand the development of the disease and its health condition. As the condition develops, the doctor may begin discussing the end-of-life care and other steps to be prepared with the patient and family.

Complications of Idiopathic Pulmonary Fibrosis

Here are some complications that may develop if Idiopathic Pulmonary Fibrosis is not treated immediately.

  • Pulmonary complications, such as blood clots in the lungs, lung infections, or lungs that fail to function.
  • Respiratory failure as a result of severe lung disease, when oxygen levels in the lungs are at very low levels
  • High blood pressure in the lungs or pulmonary hypertension when scarring affects the pulmonary blood vessels and causes impaired blood flow.
  • Right-sided heart failure or cor pulmonale, which is caused by a lower right ventricle of the heart that works too hard to pump blood through a clogged lung vein.
  • Lung cancer that develops from long-term pulmonary fibrosis.
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