Cystic Fibrosis Cystic fibrosis (CF) is a genetic disorder that impacts the lungs, pancreas, liver, and intestine. Individuals with CF will have difficulty breathing, sinus infections, infertility, and poor growth. The disease is caused by a mutation of a gene that is responsible for the regulation of sweat, digestive fluids, and mucus. A healthy person will possess two sets of this gene. The person diagnosed with CF will not have the gene at all. Without this gene, thick and viscous secretions impact various organs. A fibrosis is a scarring of tissue and in the case of cystic fibrosis, a cyst formation and scarring most often occurs inside the pancreas. Symptoms Medical examiners look for poor growth patterns, poor weight gain, chest infections, coughing, shortness of breath, and salty-tasting skin. Males may report infertility due to the absence of a vas deferens gland.  In some cases, cystic fibrosis may create an inability of blood to coagulate effectively. Neonatal stages of development have shown babies that lack the ability to absorb vitamin K resulting in a deficiency of coagulating agents in the blood. Children with this dysfunction will present to the doctor with unexplained bruising. A clogging of the airways takes place with CF due to excessive mucus. The inflammation resulting from this gradually and steadily produces an inability to get necessary oxygen. Pneumonia may develop in mid stages and in later stages the actual structure of the lungs will change. In final stages, the individual may cough up blood and develop high blood pressure. Eventually, lack of oxygen will facilitate heart and respiratory failure. The pancreas provides important digestive juices that break down food for nutrition. In people with CF, these juices are blocked from entering the intestine and result in damage to the pancreas. The pancreatic ducts become blocked which in turn, increases atrophy and scarring of the inside of the pancreas. The systemic effect of this dysfunctional action is an inability to absorb necessary nutrients. The same dysfunctional pattern may occur in the liver where bile ducts are blocked and bile remains in the liver. This may lead to scarring of the liver and cirrhosis. Ultimately, the liver will be incapable of removing toxins from the blood and will not make necessary proteins. Liver disease will occur, ultimately resulting in death. Diabetes may result from CF as insulin is not adequately created in the islets of Langerhans inside the pancreas. Treatment Treatments for CF have improved over the last several decades with most efforts being directed toward increasing levels of adequate nutrition, fighting lung infections, and enhancing overall organ function. The disease has not been eradicated, but treatments are directed toward the lungs, pancreas, liver, reproductive organs, digestive system, and psychological counseling. Antibiotics and technology designed to help people breath better play a major role in treating the symptoms of CF. Transplants, stem cell research, and gene therapy assist patients who have been diagnosed with CF.
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