Question

List the main similarities and differences between Cystic Fibrosis & Chronic Obstructive Pulmonary Disorder (COPD)

Answer 1

How is CF similar to COPD?

Like COPD, CF is a chronic obstructive lung disease that progress over time. Yet, while CF is usually diagnosed early in life, COPD is usually not diagnosed until after the age of 40. Also similar is that people living with both diseases are living better and longer than ever before. People are now living with CF well into their 40s and 50s.

Like COPD, the main organ affected is the lungs. Also like COPD, other organs are also affected. It also affects the pancreas. When it’s ducts are blocked, digestive enzymes don’t make it to the intestinal tract. And this causes digestive problems.

They have trouble digesting and absorbing nutrients from the foods they eat. This is especially true with fat, and vitamins A, D, E, and K. So this makes it difficult to maintain a healthy weight. They tend to have “growth failure.” This may also increase the risk of developing diabetes later in life. Some people with COPD also develop diabetes.

People living with CF may also experience problems with their liver, sex organs, and skin. They also may experience problems with their heart, such as cor pulmonale. This is another thing in common with COPD. The heart has to work extra hard to pump blood through diseased lungs. This extra work makes the heart abnormally large (hypertrophied). This can lead to other heart problems.

Sweat glands in people living with CF have extra chloride, sodium, and potassium. This is what makes it salty to the taste. In 1959 a sweat test was developed to definitively diagnose CF. Today, there are four tests that definitively diagnose the disease, including genetic testing.

There is one type of COPD that is also diagnosed by genetic testing. This is a rare type called Alpha-1 Antitrypsin Deficiency. Other than that one type, there is no one specific test for diagnosing COPD. However, a test called pulmonary function test (PFT) showing airflow limitation is now considered the best test for diagnosing COPD.

How are CF and COPD lungs similar?

With COPD, the immune system responds to noxious substances that are inhaled. These chemicals cause airway inflammation. Over time, these chemicals are damaging to lung tissues and cause airway scarring. Mucus has a hard time moving to the upper airway. So, it becomes trapped in the lungs. Both of these effects make airways chronically narrow and obstructed.

With CF, airways are affected by another disease called bronchiectasis. The disease causes tissues lining airways to become chronically dilated and scarred. The mucus becomes thick and sticky. It has a hard time moving to the upper airway. This mucus obstructs airways.

So, both diseases cause obstructed airways. These obstructions slow the flow of air through them, or airflow limitation. The extent of airflow limitation can be determined by performing a PFT. This test can be helpful for diagnosing COPD. However, with both diseases, it can prove helpful for determining the severity of lung disease.

Airflow limitation causes shortness of breath. Trapped mucus may cause coughing. These are the two most common symptoms for both CF and COPD. Other common symptoms include chest tightness and wheezing. Along with these, CF patients may also cough up thick sputum that may appear to be chunky. It may also be of many colors, including green or bloody.

Some of these symptoms may be present on good days. But, both CF and COPD are prone to causing exacerbations or flare-ups. This is when symptoms suddenly get worse. A common cause of flare-ups for both diseases are respiratory infections.

Both diseases may benefit from mucus clearing strategies. Both diseases may also cause low oxygen levels. So, people living with both diseases may benefit from supplemental oxygen. This can help maintain normal oxygen levels. So this is one more thing both diseases have in common.

People living with CF and COPD are both living better and longer than ever before

So, as you can see, CF and COPD are similar in many ways. People with CF are living well into adulthood. Plus, some even go undiagnosed until adulthood. A flare-up may cause them to seek medical attention for symptoms of shortness of breath and coughing. This is why many doctors may also want to rule out CF in those who present in such a way. Such improved diagnostic testing, and improved wisdom and medicine for both diseases have greatly benefited those living with both diseases. This means that people living with both CF and COPD are living better and longer than ever before.

Cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) are chronic pulmonary diseases that affect ~70,000 and 251 million individuals worldwide, respectively. Although these two diseases have distinctly different pathophysiologies, both cause chronic respiratory insufficiency that erodes quality of life and causes significant morbidity and eventually death. In both CF and COPD, the respiratory microbiome plays a major contributing role in disease progression and morbidity. Pulmonary pathogens can differ dramatically during various stages of each disease and frequently cause acute worsening of lung function due to disease exacerbation. Despite some similarities, outcome and timing/type of exacerbation can also be quite different between CF and COPD. Given these clinical distinctions, both patients and physicians should be aware of emerging therapeutic options currently being offered or in development for the treatment of lung infections in individuals with CF and COPD. Although interventions are available that prolong life and mitigate morbidity, neither disorder is curable. Both acute and chronic pulmonary infections contribute to an inexorable downward course and may trigger exacerbations, culminating in loss of lung function or respiratory failure. Knowledge of the pulmonary pathogens causing these infections, their clinical presentation, consequences, and management are, therefore, critical. In this review, we compare and contrast CF and COPD, including underlying causes, general outcomes, features of the lung microbiome, and potential treatment strategies.