Lung Disease: Pneumonia, Cystic Fibrosis, & Pulmonary Fibrosis 

Millions of people in the United States have lung disease, which means they have difficulty breathing. The term lung disease refers to many breathing disorders. If all types of these disorders are collectively grouped together, lung disease is the third leading cause of death in the United States, according to the National Institutes of Health.
 
Pulmonologists (physicians specializing in lung diseases) treat a range of acute and chronic disorders of the respiratory and pulmonary vascular systems, in association with colleagues in neurology, cancer surgery and transplant medicine. Three of the more common lung diseases treated by the physicians and pulmonologists at Houston Methodist are:

• Pneumonia
• Cystic fibrosis
• Pulmonary fibrosis

Pneumonia
Pneumonia is inflammation of the lungs and a common lung infection caused by bacteria, a virus or a fungus (mold). Pneumonia and its symptoms can vary from mild to severe depending on the infecting organism and how much of the lung is affected and unable to exchange oxygen and carbon dioxide. How much of the lung is compromised can be evaluated by your symptoms, what your doctor hears through the stethoscope when listening to you breathe and the results of a chest X-ray.
 
Treatment depends on the cause of your pneumonia, how severe your symptoms are, your age and overall health. Most healthy people recover from pneumonia in one to three weeks, but pneumonia can be life-threatening, especially in the very young, the elderly and in anyone else with a weakened immune system.

The good news is that pneumonia can be prevented by getting an annual flu shot (as flu often leads to pneumonia), frequent hand washing, and — for people at higher risk of pneumonia complications — getting a vaccine for pneumococcal pneumonia. The various strains of pneumococcus are among the most common causes of lung infection. Unless a person is at high risk of complication (for example, a person with severe COPD), pneumonia is treated with oral antibiotics. If the pneumonia does not improve or worsens, hospitalization and additional tests may be necessary. Severe cases may require hospitalization and a respirator and intravenous antibiotics.

The symptoms of pneumonia can vary, depending on whether your pneumonia is bacterial or viral:

• Cough (with some pneumonias you may cough up greenish or yellow mucus, or even bloody mucus)
• Fever (slight elevation, especially in older patients, to very high)
• Shaking chills or ague
• Shortness of breath (dyspnea)

A healthy young person usually returns to normal activities in a week or so. For middle-aged and older people, the fever may be moderate but recovery may take weeks.
 
Cystic Fibrosis
Cystic Fibrosis (CF) is an inherited disease that causes thick, sticky mucus to form in the lungs and clog the airways, making it hard to breathe. This condition can potentially cause lung damage and affect other organs. Because CF is inherited as a recessive gene, both parents must carry the gene for any children to be affected and even then only one in four will have the disease and half will be carriers.
 
CF can be identified before birth through prenatal screening and after birth through newborn screening. Getting early treatment for CF can improve your child’s quality of life and lifespan. If your child was not screened for CF, be alert to symptoms of CF, as you may not have a family history of the disease.

Patients with cystic fibrosis usually have characteristic symptoms:

• Persistent coughing, wheezing or shortness of breath
• Excessive appetite but poor weight gain
• Greasy, bulky stools
• Salty-tasting skin

Treatment for CF depends on the stage of the disease and the organs involved:

• Pulmonary rehabilitation (including airway clearance techniques to clear mucus from the lungs)
• Inhaled medicines
  • TOBI, an aerosolized antibiotic used to treat lung infections
  • Pulmozyme, a mucus-thinning drug shown to reduce the number of lung infections and improve lung function
  • Bronchodilator medicines to help open the airways
• Oral medicines
  • Azithromycin, an antibiotic that fights bacteria in the lungs
  • Anti-inflammatory medicines such as ibuprofen to help reduce swelling in the airways

In some severe cases of CF lung transplantation may be suggested treatment and only option.

Pulmonary Fibrosis
In pulmonary fibrosis, another common lung disease that makes it difficult to breathe, tissue deep in the lungs becomes thick, stiff and scarred. The scarring is called fibrosis and can get progressively worse. In some cases, the cause of pulmonary fibrosis can be found. However, convincing causes cannot be found in most cases of pulmonary fibrosis and they are labeled idiopathic.
 
Pulmonary fibrosis can develop slowly or quickly. There is no effective treatment to slow or reverse the scarring. Because most people with pulmonary fibrosis do not seek medical help until symptoms begin to limit activities of daily living, those with rapidly progressing illness die within a few years after diagnosis.
 
The symptoms of pulmonary fibrosis are due to poor lung function:

• Shortness of breath
• Dry, hacking cough
• Fast, shallow breathing (panting)
• Gradual unintended weight loss
• Tiredness
• Aching joints and muscles
• Clubbing (widening and rounding of the tips) of the fingers or toes

Helping you stay active by relieving symptoms as much as possible is the goal of therapy.
Anti-inflammatory medications are the basis for medical therapy: 

• Prednisone (an oral corticosteroid)
• Azathioprine and cyclophosphamide, which also suppress the immune system

Treatments that can also improve respiration (oxygen uptake and carbon dioxide excretion) are available:

• Oxygen therapy
• Pulmonary rehabilitation

Lung transplantation may be an option, as well, in some severe cases of pulmonary fibrosis.