Pulmonary fibrosis is the typical type of lung disease caused by the damaging and scarring of the lung tissues. The normal functioning of the lung is drastically degraded due to the stiffness of the tissues as a result of the scarring of the lung. The patient is incapacitated from breathing normally. Many factors may be behind this disease though in most cases the cause is unknown. At such times, it is termed as idiopathic pulmonary fibrosis. Pulmonary fibrosis damages the lungs irreversibly, however, the treatments and therapies can still help to mellow down its symptoms. A lung transplant could be the final solution but there are no guarantees on it.
The chronic symptoms of pulmonary fibrosis are as follows:
- Dyspnea or shortness of breath
- Dry cough
- Inadvertent weight loss
- Paining muscles and joints
- Clubbing around the tips of the fingers or toes (widening and rounding of the tips of the fingers or toes)
The symptoms and the severity of pulmonary fibrosis may vary from patient to patient. Few patients may also experience a rapid degradation in their physical condition with symptoms like acute exacerbation (severe shortness of breath) that may prolong for days or weeks. Such type of patients might be placed on ventilators to stimulate breathing. Doctors may advise antibiotics, corticosteroid medications or other medications in these cases.
In order to diagnose the severity of the condition, the doctor might review a patient’s medical and his/her family’s history. Further discussions on the undermining symptoms, reviewing any exposure to dust, gases and chemicals can be held followed by a physical examination. The doctor will listen carefully to your breathing mechanism with a stethoscope. Few or more of the following tests can also be advised:
- Chest X-ray
- CT scan (Computerized tomography)
Lung Function Tests
- Pulmonary testing: Pulmonary tests may be conducted to measure how much air your lungs can inhale and exhale at a given time.
- Pulse oximetry: In this test, a small device is placed on one of the fingers to measure the saturation level of the oxygen in the blood. Oximetry helps in determining the course of the disease. Exercise stress test. An exercise test on a treadmill or stationary bike may be used to monitor your lung function when you're active.
- Arterial blood test: The oxygen and carbon dioxide levels in the arterial blood sample are measured.
Tissue Sample (Biopsy)
A biopsy may also be required to determine Pulmonary Fibrosis as other tests may not be able to diagnose the condition accurately. The sample may be obtained through one of these steps:
- Bronchoscopy: A small and flexible tube passed through the mouth or nose into the lungs may be used to remove a small sample for biopsy.
- During bronchoscopy, the doctor may conduct another procedure called bronchoalveolar lavage. In this procedure, salt water is injected through a bronchoscope into the lung, and then it is immediately sucked out. The ejected matter contains cells from the lung.
- Surgical biopsy: This procedure, though difficult with possibility of complications, but helps to get accurate diagnosis. It may be done with the help of video-assisted thoracoscopic surgery (VATS) or a thoracotomy, an open surgery may be performed. In VATS, the surgeon inserts the surgical instruments and a small camera through two or three small cuts made between the ribs. With these, the surgeon can easily see inside the lungs while taking the sample from the patient’s lungs. This procedure is performed after a general anaesthesia is given to the patient. In thoracotomy, a lung sample is removed through an incision made in the chest between the ribs. This procedure is also conducted after a general anaesthesia is given to the patient.
In order to gauge the liver and kidney functioning, the doctor may suggest a blood test. This might help to determine the presence of pulmonary fibrosis.
Though there is no cure for Pulmonary fibrosis, however, there are few treatments that may improve the condition of the patient temporarily or slow the onset of the disease. These treatments depend on the severity of the condition of the patient.
Medicine such as pirfenidone (Esbriet) and nintedanib (Ofev) can drastically slow the elevation of the idiopathic pulmonary fibrosis.
Side effects such as diarrhea and nausea are well known to be associated with Nintedanib. The side effects of pirfenidone are skin rash, nausea and diarrhea. Doctors may also advise certain anti-acid medications to counter gastroesophageal reflux disease (GERD). It is an abnormal digestive condition that is mostly found in people with idiopathic pulmonary fibrosis.
Oxygen remotely can't restrict lung damage in itself, but it can certainly help in the following ways:
- Breathing is better and panting for breath is also reduced
- Complications that arise as a result of low oxygen levels in the blood can be reduced
- Blood pressure in the heart is regulated
- Sleep disorder is rectified giving a feeling of well being
Receiving oxygen when sleeping or exercising is common. There are chances that some people might have to use it all the time. Some people may also carry a mobile canister of oxygen wherever they travel.
Restorative activity can somehow help to manage the symptoms and enable the patient to live life with ease. This includes the following:
- To enhance the level of endurance through physical activities
- Improvising better breathing techniques to make the lung stronger
- Dietary and nutrition counselling
- Psychological consultation
- Getting vivid knowledge about the disease and patient’s current condition
A medical option for treating pulmonary fibrosis is a lung transplant. However, it also comes with certain malice such as the rejection of the transplanted lung by the body and the risk of having an infection. It will be up to the doctor to decide if a lung transplant would be appropriate for the patient’s condition or not.
Cost of Treatment for Pulmonary Fibrosis
The following are the primary factors that determine the cost involved in treating pulmonary fibrosis:
- Hospital that patient chooses for his/her treatment
- Fees for the doctor/physician
- Cost of medicines
- Cost of tests and diagnostic procedures
- Cost of surgery (if required)
- Cost of follow-up care
- Cost of other related therapies