Dolphy's disease: No laughing matter

The demise of Dolphy last July triggered a genuine national mourning in honor of a true icon of the Philippine entertainment industry. It was reported that he died of multiple organ failure following recurrent episodes of pneumonia, brought about by a chronic obstructive pulmonary disease (COPD).

The most common risk factor for COPD is cigarette smoking. During Dolphy’s golden era, smoking was common. Medical science neither knew enough nor did it provide the needed health warnings on the dangers of smoking. Truth to tell, at that time, it was fashionable to smoke, and scenes of glamorous stars smoking were commonly seen on the big screen  in both local and Hollywood movies.

COPD takes your breath away

COPD refers to a group of lung diseases that limits airflow as you exhale and makes it increasingly difficult to breathe out. COPD affects thousands of Filipinos and is usually attributed to exposure to tobacco smoke and airborne irritants over a period of time. Most often, it occurs in long-term or former smokers. Left untreated and allowed to progress, COPD literally takes your breath away.

Each time you inhale, air travels through your windpipe into airways in your lungs called bronchial tubes (see illustration). These airways branch many times in your lungs, dividing into thousands of smaller, thinner tubes called bronchioles. At the end of each bronchiole are clusters of air sacs (alveoli). Inside the walls of the alveoli, tiny blood vessels (capillaries) absorb inhaled oxygen and release carbon dioxide so it can be exhaled.

Healthy bronchioles and alveoli are stretchy, so when you breathe in, each little air sac fills up like a balloon, and when you exhale, each deflates. Although a slight decline in lung function is part of normal aging, it’s a different scenario for lung damaged by COPD. The tubes can be thickened or plugged with mucus, and the air sacs can be destroyed and very floppy. As a result, the lungs can’t expel air well, so they become less efficient. This process gradually becomes worse over time  more slowly if you stop smoking and faster if you continue.

Risk factors

The risk of developing COPD is mainly related to your lung’s exposure to irritants. As previously noted, tobacco smoke tops the list, but there are others  dust, secondhand smoke, air pollution, and industrial gases, vapors, and fumes. Indoor fires for heating and cooking are a major cause in some countries.

Signs and symptoms of COPD are usually slowly progressive. They may include shortness of breath, wheezing, chest tightness, and sputum or phlegm production. COPD includes both chronic bronchitis and emphysema  most people with COPD have both. Chronic bronchitis is defined by the presence of a cough and sputum production. Other characteristics include inflammation and thickening of airways.

Emphysema is defined by chronic damage to the alveoli. The chronic injury  usually from smoking  destroys the inner walls of alveoli clusters, reducing the surface area available to exchange oxygen for carbon dioxide and allowing them to over expand. Normally, the alveolar walls are stretchy. Emphysema causes them to lose elasticity, so they fail to contract during exhalation, trapping air in the lungs. The result is shortness of breath because the chest muscles have to work harder to breathe in and out.

Pulmonary function tests are key in diagnosing COPD and its stage. Spirometry uses a machine (spirometer) to measure how much air you can blow out of your lungs, and how quickly you can blow air out. Spirometry is an important test for current or former smokers who have COPD symptoms. Spirometry is also used to track how well treatment is working.

Your doctor may recommend getting a chest x-ray or a computerized tomography (CT) scan to look for other problems. A CT scan may be helpful to screen for early lung cancer, which is a risk for smokers, especially those with COPD. A blood test may be done to see how capable your lungs are of supplying oxygen to your blood and removing carbon dioxide. Sputum (phlegm) can be examined under a microscope for cancer cells, but that’s not recommended as a screening tool.

Treatment options

Treatment focuses on minimizing further damage, controlling symptoms, and preventing sudden worsening of COPD, called an exacerbation. Foremost is eliminating exposure to the irritant that’s damaged your lungs. If you smoke, it’s very important to stop in order to keep your COPD from getting worse. After smoking cessation, you’ll likely have fewer symptoms and your lung function may improve slightly. Vaccination against pneumonia and an annual influenza vaccine are strongly recommended for anyone who has COPD. Flu shots reduce the risk of respiratory hospitalization for up to half. Adopting a physically active lifestyle is of critical importance  research demonstrates that people with COPD who exercise do better overall. Studies show that people with COPD who walk more than two hours a day have a much easier time managing their COPD.

Several groups of medications are used to treat the symptoms and complications of COPD. Some may be taken on a regular basis and others, as needed. These include:

• Bronchodilators. Drugs such as albuterol, ipratropium, and a number of others are used as bronchodilators to help relax muscles around your airways. The various bronchodilators work by different mechanisms and usually come in an inhaler. They can help relieve coughing and make breathing easier. Depending on how severe your COPD is, you may need a short-acting bronchodilator just before activities, a long-acting one for everyday use, or both. Long-acting bronchodilators are typically prescribed if you have moderate to severe COPD.

• Inhaled corticosteroids. These drugs don’t slow decline in lung function but when used selectively, they do reduce airway inflammation. Their use is fundamental in treating all but the mildest cases of asthma. For people whose symptoms indicate both asthma and COPD, inhaled corticosteroids are probably appropriate in most cases. Importantly, inhaled corticosteroids may be prescribed to prevent sudden exacerbations. They can be beneficial for people who have more than one exacerbation a year. Exacerbations are often due to chest colds. In the wake of a severe exacerbation that requires hospitalization, inhaled corticosteroids are usually provided for six months or even longer. The most common side effects of corticosteroids include increased risk of skin bruising, hoarseness and a yeast infection in the mouth, which can be prevented by gargling after using the inhaler.

• Antibiotics. These are used, when necessary, to fight respiratory infections  such as acute bronchitis, pneumonia and influenza  and to prevent flare-ups of COPD in certain situations. They’re mainly recommended for acute exacerbations.

• Supplemental oxygen also may be needed. Some people require constant oxygen, others may need it only during activity or sleep.

• A pulmonary rehabilitation program can be your opportunity to better understand COPD and to adopt a lifestyle that may improve your quality of life and also slow the progression of COPD. Typically, these programs are provided by a team of health care professionals, who combine education with the introduction of a more active lifestyle. Regular exercise improves endurance and the efficiency of your cardiovascular system. Pulmonary rehabilitation is customized to your needs no matter what roadblocks you may have or how severe your disability. It’s effective even for people with severe lung disease, which includes those who are eligible for lung transplantation.

Surgery may be considered if you have severe emphysema that isn’t helped enough with medications alone. Lung volume reduction involves removal of damaged lung tissue. This creates extra space for the remaining lung tissue and diaphragm to work more efficiently. Single- or double-lung transplants may be an option for people with severe emphysema who meets specific criteria. Both of these surgeries are specialized options for some with very severe COPD. New therapies, such as endobronchial valves, are in development but are still experimental.

When things get worse

Even with optimal COPD treatment, you may encounter sudden exacerbations. About half of exacerbations are due to either viral or bacterial infections, though some are due to irritants. Exacerbations are serious threats. Without prompt treatments, one may lead to lung failure and a need for hospitalization. People with only mild to moderate COPD can develop severe exacerbations. People with previous attacks are the most likely to develop recurrent exacerbations.

For some people predisposed to exacerbations, long-term use of an anti-inflammatory antibiotic or a bronchodilator that also acts as an anti-inflammatory may help prevent further recurrences. For instance, roflumilast is in this latter group of drugs, It’s approved to help prevent exacerbations in people whose COPD is primarily due to chronic bronchitis.

If you develop a chest cold or notice more coughing or a change in your mucus, or if you have a harder time breathing, seek medical attention. Treatments for exacerbations usually require antibiotics. Oral steroids are also usually required to treat severe attacks.

COPD is a difficult disease to have. As Dolphy himself would have told you, having COPD is no laughing matter.

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Next month is Breast Cancer Awareness Month. My next column, which will appear on the first Tuesday of the month, October 2, 2012, will discuss the latest advances in the diagnosis and treatment of this dreaded disease. Don’t miss it.