Disease Information

Several different diseases can lead to end-stage lung failure. Chronic obstructive pulmonary disease (COPD), Alpha1-antitrypsin deficiency, Interstitial lung diseases, bronchiectasis and cystic fibrosis.

COPD

• The symptoms of COPD can range from chronic cough and sputum production to severe disabling shortness of breath.
• Individuals with COPD increasingly lose their ability to breathe.
• 12.1 million adults ages 25 and older reported being diagnosed with COPD in 2001.
• About 24 million adults have evidence of impaired lung function indicating that COPD is underdiagnosed.
• The prevalence of self-reported COPD is higher in females than males and in whites than blacks.
• About 726,000 hospitalizations for COPD occurred in 2000. More females than males were hospitalized for COPD (404,000 vs. 322,000).

Alpha-1 Antitrypsin Deficiency

• It is estimated that there are about 100,000 Americans today who were born with Alpha-1 deficiency. Of this group, Alpha-1 related emphysema may afflict a majority of these individuals.
• The first signs of Alpha-1 related emphysema often appear between ages 20 and 40.
• The earliest symptom is usually shortness of breath following activity, as well as decreased exercise capacity and wheezing.
• Both the early age at which the disease is present and the fact that the disease most frequently appears in the lower rather than the upper lung regions helps distinguish Alpha-1-related emphysema from other types of emphysema.
• Alpha-1 deficiency is often under diagnosed or misdiagnosed.
• As many as 3% of individuals with chronic obstructive lung disease (COPD) have undiagnosed Alpha-1 deficiency.
• If AAT deficient individuals also smoke, their risk of developing emphysema is greatly increased.
• Worldwide, it is estimated that 116 million people are carriers of the disease.

Interstitial Lung Diseases

• Interstitial Lung Disease (ILD) is a general term that includes a variety of chronic lung disorders like: idiopathic pulmonary fibrosis, sarcoidosis, eosinophilic granuloma, Goodpasture’s syndrome, idiopathic pulmonary hemosiderosis and Wegener’s granulomatosis.
• When a person has ILD, the lung is affected in three ways.
• The lung tissue is damaged in some known or unknown way.
• The walls of the air sacs in the lung become inflamed.
• Scarring (or fibrosis) begins in the interstitium (or tissue between the air sacs), and the lung becomes stiff.
• About 100,000 hospitalizations per year for individuals with ILD
• About 15 % of individuals hospitalized are seen by a Pulmonologist

Bronchiectasis

• Bronchiectasis is commonly misdiagnosed as asthma or pneumonia.
• Bronchiectasis can occur as part of a birth defect, such as primary ciliary dyskinesia or cystic fibrosis.
• About 50% of all cases of bronchiectasis in the U.S. result from cystic fibrosis.
• Bronchiectasis can also develop after birth as a result of injury or other diseases, like tuberculosis and influenza.
• Bronchiectasis can occur at any age, but most often begins in childhood.
• Symptoms of bronchiectasis include coughing, fever, weakness, weight loss, and fatigue.
• Doctors can detect bronchiectasis with a chest x-ray, breathing tests, sputum culture or computed tomography (CT) scan.

Cystic Fibrosis (CF)

• CF is the most common fatal genetic disease among Caucasians.
• The disease is recessively inherited, meaning both parents need to have the defective gene.
• Approximately 30,000 Americans have CF and about 12 million carry the gene but are not affected by it.
• Though the disease is not exclusive to certain races, it is more prevalent among those of Northern European descent.
• CF patients often have respiratory problems which include bronchitis,  bronchiectasis   (enlargement of bronchial tubes),  pneumonia , sinusitis (inflammation of sinuses), nasal polyps (growths inside nose), or  pneumothorax  (collapsed lung).
• Symptoms of CF include frequent wheezing or pneumonia, chronic cough with thick mucus, persistent diarrhea, salty-tasting skin, and poor growth.

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