Cystic Fibrosis - Past, Present and Future

            Cystic fibrosis is an autosomal recessive genetic disorder that, according to the Heart, Lung and Blood Institute, affects over 10 million Americans (2011). According to Peter Crosta, the disease is present from birth (2009). This essay will outline the complications of CF, living with the disorder and the prognosis associated with it. .
             To begin with, this genetic disease is acquired when two "defective copies of the cystic fibrosis transmembrane conductance regulator," or CFTR for short, are inherited from each parent (Crosta, 2009). Because it is a recessive gene disorder, if two carriers of the disease produce offspring their children will have a 25% chance of not inheriting the disease, a 50% chance that they will be a carrier and a 25% chance that they will inherit the disease (Lee, Marks and Shiel, 2012). .
             CFTR is the gene in our bodies that produces our mucus, sweat and stomach acids (Crosta, 2009). The bodies of these people with the disorder go into overdrive and produce "abnormally thick and sticky" mucus, which builds up in their lungs making it extremely difficult to breath (Zieve and Hadjiliadis, 2011). In the tubes leading out of the pancreas, where digestive enzymes are produced, abnormally high amounts of mucus are accumulated in patients with cystic fibrosis, making it difficult for these enzymes to reach the small intestine (National Heart, Blood and Lung Institute, 2011). Thus, people who suffer from cystic fibrosis have difficulty gaining and maintaining weight and as a result, grow more slowly than the average person. Also, with their secretory glands producing abnormal amounts of mucus, the sweat of patients with this disease tends to be saltier (National Heart, Blood and Lung Institute, 2011). As a result of these factors, people with this disease are more prone to lung infections, like pneumonia, which is one of the main causes of death for people with CF.