Interstitial lung disease

Lifestyle and home remedies

Being involved in your own treatment and staying as healthy as possible are essential to living with interstitial lung disease. For that reason, it's important to:

• Stop smoking. A strong association exists between smoking and idiopathic interstitial lung disease, the most severe and potentially lethal of the disorders. Talk to your doctor about options for quitting, including smoking cessation programs, which use a variety of proven techniques to help people quit.

• Exercise regularly. Exercise is a double-edged sword for people with lung disease; it requires an increased intake of oxygen, and it makes symptoms worse. At the same time, exercise is essential for maintaining lung function, for reducing stress, and for maintaining overall health and well-being.

  If you're already exercising, don't stop. And if you're not currently physically active, consider starting with a moderate workout, such as riding a stationary bike or walking.

  If you've been prescribed oxygen for regular use, be sure to use it when you exercise. You might also ask your doctor for a referral to an exercise physiologist, who can design an exercise program specifically for you.

• Eat well. People with lung disease may lose weight both because it's uncomfortable to eat and because of the extra energy it takes to breathe. Yet a nutritionally rich diet that contains adequate calories is essential. The type of food you eat, the time of day and the size of portions can all play a role in getting the nourishment you need.

  Because it's often easier to breathe when your stomach isn't completely full, you may want to eat smaller meals throughout the day rather than two or three large ones. You might also try choosing lighter fare, such as fruit and salads, rather than rich or fatty foods, which take more energy to digest. A dietitian can give you further guidelines for healthy eating.

• Enroll in a pulmonary rehabilitation program. These programs vary widely, but in general they focus on improving your ability to exercise and carry out normal activities, managing shortness of breath with breathing techniques, improving your appetite and nutritional status, dealing with the difficult psychological aspects of living with lung disease, and improving overall quality of life.

Complications

Scar tissue formation in your lungs can lead to a series of increasingly serious complications, including:

• Low blood oxygen levels (hypoxemia). Because interstitial lung disease reduces the amount of oxygen you take in and the amount that enters your bloodstream, you're likely to develop lower than normal blood oxygen levels. Lack of oxygen can disrupt your body's basic functioning, and severely low levels can be life-threatening.
• High blood pressure in your lungs (pulmonary hypertension). Unlike systemic high blood pressure, this condition affects only the arteries in your lungs. It begins when the smallest arteries and capillaries are compressed and obliterated by scar tissue, causing increased resistance to blood flow in your lungs. This in turn raises pressure within the pulmonary arteries. Pulmonary hypertension is a serious illness that becomes progressively worse and that eventually may prove fatal.
• Right-sided heart failure (cor pulmonale). This serious condition occurs when your heart's lower right chamber (right ventricle) — which is less muscular than is the left — has to pump harder than usual to move blood through obstructed pulmonary arteries. Initially, your heart tries to compensate for the increased workload by thickening its walls and dilating the chamber of the right ventricle to increase the amount of blood it can hold. But this measure works only temporarily, and eventually the right ventricle fails from the extra strain.
• Respiratory failure. Often in the end stage of chronic lung disease, respiratory failure occurs when blood levels of oxygen become dangerously low or, as in the case of emphysema, carbon dioxide levels become excessively high. Severely low blood oxygen can lead to heart arrhythmias and unconsciousness, and high carbon dioxide levels to sleepiness and confusion. Eventually, respiratory failure may prove fatal.

Tests and diagnosis

Identifying and determining the cause of interstitial lung disease can be extremely challenging. An unusually large number of disorders fall into this broad category. What's more, the distinction between interstitial lung disorders with identifiable causes and those with no known cause isn't always clear, and the nomenclature and classification systems of both have historically been confusing and controversial.

In addition, the signs and symptoms of a wide range of medical conditions — among them chronic obstructive pulmonary disease (COPD), heart failure and asthma — can mimic interstitial lung disease, and doctors must rule these out before making a definitive diagnosis.

To help cut through the confusion and rule out other possible illnesses, doctors normally begin by taking a comprehensive medical history, focusing especially on occupational exposure to lung-damaging toxins, on medications and on the presence of health problems commonly associated with lung disorders.

But although a medical history and physical exam can be useful in ruling out certain conditions, they can't accurately diagnose interstitial lung disease. Instead, doctors normally rely on tests such as:

• Chest X-ray. Although this is often the first test given in cases of suspected lung problems, a chest X-ray isn't as effective as a CT scan in diagnosing interstitial lung disease. It can, however, help eliminate conditions that cause signs and symptoms similar to those of interstitial lung disease, including emphysema and a collapsed lobe of one of the lungs.
• High-resolution computerized tomography (HRCT) scan. Whereas a traditional chest X-ray produces two-dimensional images of your lungs, a computerized tomography scan uses an X-ray-sensing unit and a large computer to create cross-sectional images that are far more detailed. A high-resolution CT scan goes even further, showing lung tissue in great detail and providing more information than conventional CT scans do.
• Pulmonary function tests (PFTs). These noninvasive tests check how well your lungs function. For the test, you're usually asked to blow into a simple instrument called a spirometer, which measures how much air your lungs can hold and the flow of air in and out of your lungs. As scarring becomes worse, you're able to take less air in and blow less out. This part of the test takes just a few minutes. Full PFTs, which give far more information and take longer, can measure the amount of gases exchanged across the membrane between your alveolar wall and capillary membrane.
• Exercise tests. Because symptoms of interstitial lung disease are worse when you're active, your doctor may assess your lung function while you exercise, usually on a stationary bike or treadmill. Although specific tests vary, your blood pressure and blood oxygen levels are usually monitored as the difficulty of the exercise increases.
• Bronchoscopy (transbronchial biopsy). In many cases, interstitial lung disease can be definitively diagnosed only by examining a small amount of lung tissue (biopsy). In a transbronchial biopsy, your doctor passes a flexible, fiber-optic tube (bronchoscope) through your mouth into your lungs and removes one or more tissue samples, each about the size of the head of a pin. These are then examined in a laboratory. Bronchoscopy is performed on an outpatient basis using local anesthetic.
• Bronchoalveolar lavage. In this procedure, your doctor injects salt water (saline) through a bronchoscope into a section of your lung, and then immediately suctions it out. The withdrawn solution contains cells from the air sacs. Although bronchoalveolar lavage samples a larger area of the lung than other procedures do, it may not provide enough information to diagnose a specific interstitial lung disease. Instead, doctors often use it to check the progress of a lung disorder or to help determine the best treatment.

• Video-assisted thoracoscopic surgery. When less invasive tests don't yield a specific diagnosis, a thoracic surgeon may perform a surgical lung biopsy. In this procedure, a flexible tube with a camera (endoscope) is inserted through a small incision between your ribs, allowing the surgeon to view your lungs on a video monitor. Surgical instruments are then inserted through another incision, and the surgeon removes thumbnail-sized tissue samples from two or three sites in your lungs.

  Because video-assisted thoracoscopic surgery allows a surgeon to make small incisions in your chest wall rather than a long cut between your ribs, you're likely to have less pain and to heal more quickly than you are with traditional open lung surgery. Risks of the procedure include infection, bleeding, an air leak in the lung wall and pneumonia.