Pulmonary
- Critical Care Associates
of East Texas
Jeffrey M.
Shea, M.D., F.C.C.P.
Venkatesh Donty, M.D.
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Jeffrey M.
Shea, M.D., F.C.C.P. |
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What is Idiopathic Pulmonary Fibrosis? Idiopathic Pulmonary Fibrosis (IPF) is a specific disease of inflammation that results in scarring (fibrosis) of the lungs. In time, this scarring builds up to the point where the lungs are unable to provide oxygen to the tissues of the body.The word Idiopathic means "of unknown origin", Pulmonary refers to the lungs, and Fibrosis is scarring. In other words the cause of this scarring disease of the lung is not known at this time. There are however a couple of theories as to its origin. Some
researchers believe that IPF may result from either: It is estimated that IPF occurs in about 15 to 18 persons per 100,000 population. There are an equal number of men and women who contract the disease and it is generally diagnosed between the ages of 40 and 70. Often there is evidence of disease 2 to 5 years before the diagnosis is made. Prognosis of IPF is dependent on the stage at which the disease is diagnosed, the amount of inflammation on biopsy, the response to therapy, and disease-associated complications which varies with patients. What Are The Symptoms of IPF? Early symptoms of this disease are dyspnea (shortness of breath) and a dry cough (without sputum production). As the disease progresses, the shortness of breath will become more pronounced making otherwise simple tasks seem difficult. Daily activities such as climbing stairs, walking short distances, dressing, talking on the phone, and even eating will require a great deal of effort. A person may also be less able to fight off infection. Clubbing (enlargement) of the fingertips may also develop.The course of this disease varies greatly from person to person and usually develops slowly over a long period of time, sometimes years. Alveolitis (inflammation of the air sacs called alveoli in the lungs) is one of the early stages. This inflammation prevents the air sacs from doing their job in the lungs. The alveoli transfer oxygen from the lungs into the blood to be carried to the tissues of the body and pick up the carbon dioxide from the blood stream to be eliminated from the lungs and out of the body. As the disease progresses, the alveoli become damaged and scarred, thus stiffening the lungs. This stiffening in the lungs makes breathing more difficult and causes the simple activities of daily living harder to perform.
How is IPF Diagnosed? As we have already discussed, the first symptom of IPF is dyspnea (shortness of breath) frequently associated with a nonproductive cough. A personal medical history will be obtained from the patient. This is to evaluate the other causes of scarring in the lungs which can include occupational and environmental exposures, previous or concurrent illnesses, drug ingestions, family history of similar problems, etc. The diagnoses will be made on a series of tests ordered to rule out or confirm a diagnoses of IPF. There are approximately 130 other diseases that have been associated with scarring of the lungs and the history, physical exam and tests are done to help arrive at the correct diagnosis.
In IPF , the x-ray usually reveals shadows, mostly in the lower part of the lungs. In addition, lung size tends to appear smaller than normal.
This is a series of x-ray’s done through the top, sides, and back of the body that allows the physician to look at the lungs in more detail and from a different angle than the ordinary chest x-ray.
Arterial Blood Gases will be ordered to determine the amount of oxygen in the blood to see how well the alveoli of the lungs are working. Other blood tests may be drawn to determine if the fibrosis may be related to some other condition.
Pulmonary Function Tests (PFT’s) can be done in our office. This test requires the patient to do a series of breathing maneuvers through a mouthpiece connected to a carefully calibrated piece of equipment. These tests are used to measure the health of the lungs. They enable the physician to detect many changes and abnormalities in respiratory function, and is also useful in following the progression or response to treatment.
This is an examination of your airway using a small flexible scope that is inserted either through the nose or occasionally through the mouth. Lung washings (bronchoalveolar lavage) are done to remove fluid and other material from inside the lungs. The amounts of certain cells and proteins found in the materials are helpful in ruling out certain causes of pulmonary fibrosis and the possible response to therapy. Biopsies are also done to determine if other causes of fibrosis can be detected.
A chest surgeon will make several cuts between the ribs in the chest and remove several small pieces of lung tissue. The material is examined in the laboratory to determine how much inflammation and fibrosis are in the lungs. It is the only way to confirm whether the patient has IPF. How is IPF Treated? Medications are the primary form of treatment. They are usually prescribed for at least 3 months before an assessment of effectiveness is determined, usually by PFT’s and Chest X-rays. This gives your physician time to see if a particular treatment is effective. Prednisone is the drug of choice but other medications can also be used if prednisone is contraindicated. Oxygen is sometimes prescribed in addition to medication if a patients blood oxygenation becomes too low. Regular exercise may be useful for some patients with IPF. This can help in improving "muscle" strength and breathing ability and also increase overall strength. Lung transplantation is an alternative in some who have progressive disease despite treatment and in the severe, final stages of IPF. Living with Idiopathic Pulmonary Fibrosis!In addition to getting proper treatment, IPF patients just need to follow sensible healthy lifestyles which include: regular exercise, eat a healthy diet, maintain proper weight, get enough rest and above all Do Not Smoke.
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