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The first reference to this disorder occurred in the middle ages, where it was believed to be a curse and a result of being bewitched as an infant. Those who were ‘cursed’ were described to have ‘salty skin’ and die young, which are common symptom of Cystic Fibrosis. It was not until 1938 that the name “Cystic Fibrosis of the Pancreas” was put to the disorder by American Pathologist Dr. Dorothy Andersen. Her studies of children dying of malnutrition helped other doctors recognized the disorder, referring to it as “mucoviscidosis”, bringing awareness to the abnormal thickness of mucous. Paul Di Sant’Agnese, a doctor in an emergency room in New York City, encountered a strange phenomenon when a heat wave occurred in 1948. Several babies were brought into the hospital suffering from dehydration. Sant’Agnese soon noticed that the infants sweat was salty, which led him to infer that the tales of Cystic Fibrosis in ancient times were true, and this indeed was a real disorder. Through all of these discoveries, it still took doctors until the 1980’s to understand what caused this genetic defect and find where it was present (Nick. National Jewish Health) . Cystic Fibrosis takes place when the CFTR gene (Cystic Fibrosis Transmembrane Conductance Regulator) is mutated. In someone who doesn’t have CF, mucus that is present in the lungs is thin and easy to move. It acts like lubricant and allows bacteria to be coughed up, sneezed out or destroyed by stomach acid. When there is a mutation in this gene, the CFTR is misshaped or nonexistence, making it impossible for chloride ions to enter/exit the cell. This irregular imbalance of ions causes mucus in the lungs to become thick and hard to move. Ultimately, it will collect bacteria and viruses, which can lead to bigger problems such as pneumonia or bronchitis. The lungs are not the only place affected by Cystic Fibrosis; the intestines, pancreas and liver are impacted as well. (Healthline) (National Heart Lung) . In order for Cystic Fibrosis to be diagnosed, there are certain steps that need to be taken. First, a sweat test will be done and if that comes back abnormal, further tests will be issued. Those additional testings will focus on liver disease, malnutrition, signs of airway obstruction, Nasal polyps, and many more. This method was thought up by experts from the US CF Foundation in 1963 (Shaffer. News-Medical) . After following the diagnosis steps, if Cystic Fibrosis is in fact present, then it is possible that symptoms will eventually show/are already showing. There are different types of symptoms that can occur in someone with CF, and those are respiratory and digestive. In respiratory signs, the thick build up of sticky mucus will cause wheezing, repeated lung infection, persistent cough that produces sputum, breathlessness, exercise intolerance, and inflamed nasal passage (stuffy nose). Symptoms that are described as digestive include poor weight gain/growth, intestinal blockage (particularly in newborns), foul smelling, greasy stools and severe constipation. These take place because mucus is clogging up tubes that move digestive enzymes, causing them to not be able to efficiently do their jobs. There are also a few types of complications that can show in those who suffer from this disorder. Respiratory complications include Chronic infections, coughing up blood (hemoptysis), acute exacerbations, growths in nose, damaged airways, pneumothorax, and respiratory failure. If a person with CF experiences nutritional deficiencies, diabetes, intestinal obstruction, and blocked bile ducts are referred to as Digestive complications. The last complication there is with this disorder affects the reproductive system. Men almost always are infertile because their vas defrans (the tube that connects the testes and the prostate gland), like other parts of the body, is blocked by thick mucus or is missing from the reproductive organs entirely. In women, complications are less severe than men, but they may still have trouble conceiving. Doctors should be talked to before women with cystic fibrosis get pregnant because it can worsen their symptoms (Mayo Clinic) .

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