[Cystic fibrosis patient post lung transplantation]

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Literature Review

Exploring the experience of cystic fibrosis patient post lung transplantation.

Cystic Fibrosis

First identified in 1938, cystic fibrosis (CF) is the most common inherited life-ending disease found in Caucasians, with an estimated frequency of one in approximately 3,000 live births. Since its initial discovery, scientists have learned that the genetic defect is transmitted as an autosomal recessive trait and that the CF defect impacts a single gene on chromosome 7, named the cystic fibrosis transmembrane regulator (CFTR) (Rozas, Goldman, 2002, 87).

Once a uniform death sentence during childhood, because of enhancements in the care of patients with CF, the diagnosis now holds greater promise for a productive and longer life, particularly when care is received through an advanced, evidence-based medicine CF specialty care center (Rosenstein, Cutting, 2008, 58). CF patients born in the 1990s are predicted to survive a median of greater than 40 years;this compares favorably to the early mid-20th century, when diagnosis during childhood was once predictive of death within six months.

The hallmark of CF is impacted mucus and the list of commonly affected organs includes those in the respiratory tract (nose, sinuses, and lungs); digestive system (intestines, biliary system, and pancreas); and reproductive system.

Although most commonly seen in descendants of European ancestors, CF has been reported in African British, as well as in natives of Middle Eastern countries, India, and Pakistan. The frequency of this genetic defect in nonwhites is 1:9,500 Hispanics; 1:11,200 Native British; 1:15,000 African British; and 1:31,000 Asian British (Ong, Mehta, Ogston, Mukhopadhyay, 2008, 18).

Because of the inherited genetic defect in the CFTR, CF patients' epithelial cells cannot pump adequate water into their mucus secreting cells, leading to development of secretions that are much thicker and stickier than normal; these thick, tenacious secretions effectively act to block mucus glands throughout the body. The obstruction of mucus glands and small airways in the lungs leads to further retention of mucus and associated chronic infections (McCormick, Green, Mehta, Culross, Mehta, 2002, 58). Such chronic infections in the lungs, along with related complications, are ultimately the cause of death in 90 percent of CF patients.

Because of chronic infections and the need for repeated administration of antibiotics, bacteria in CF patients are typically highly resistant to commonly administered antibiotics. This antibiotic resistance makes it much more difficult to find the right combination of antibiotics to treat infections. As the repeated infections become increasingly difficult to clear, CF lung disease progresses to the development of bronchiectasis (a condition where the airways lose their supportive structure, become extraordinarily dilated, and retain even more mucus). Development of bronchiectasis makes the CF patient more prone to lung collapse (pneumothorax), another respiratory complication of CF (Lai, Kosorok, Sondel, 1988, 47).

Symptoms

Respiratory symptoms include cough, shortness of breath, wheezing, and chest pain. When an acute infection occurs, patients may develop fever, muscular pains, fatigue, lack of appetite, and weight loss. Patients may also develop infections and symptoms of sinusitis, bronchitis, and nasal polyps.

Gastrointestinal symptoms may be present at the time of ...