Webb 1 Andrew Webb Mrs.

Harmon CP English II 29 February 2008 Cystic Fibrosis: The Fight for your Life What kind of disease is Cystic Fibrosis? Is it a disease of the respiratory system? Maybe it’s a disease of the digestive system? Cystic Fibrosis is actually a disease of the respiratory, digestive, reproductive, and endocrine systems (Rosaler 17). If you have Cystic Fibrosis, your children have a twenty-five percent chance of having the disease, a twenty-five percent of not having the disease, and a fifty percent chance of being a carrier of the disease. Both the mother and the father of a child with Cystic Fibrosis have to be carrying the recessive Cystic Fibrosis gene. One out of every thirty-one Americans carries the Cystic Fibrosis gene and doesn’t even know it (Larson 721). One out of every 3,500 American children has Cystic Fibrosis, but with new medical technologies people are living longer than ever. In the past, Cystic Fibrosis has been known by other names such as CF, Cystic Fibrosis of the pancreas, Fibrocystic Disease of the pancreas and, Mucoviscdosis (What 2). People in the sixteenth and seventeenth centuries may have had cystic fibrosis, but they did not know how to treat it. The earliest known case of Cystic Fibrosis was diagnosed by a Spanish professor in 1606 (Rosaler 14). The CFTR gene, whose mutation causes Cystic Fibrosis, has been traced back 52,000 years by geneticist (Rosaler 13).Dr. Francis Collins was part of the team that discovered the CFTR gene in 1989 (Rosaler 10). CFTR means Cystic Fibrosis transmembrane conductance regulator (Cystic 2). You get Cystic Fibrosis from the recessive CFTR gene both your parents have to give you (Larson 720). A person with Cystic Fibrosis should make sure their brothers, sisters, and first cousins are tested to see if they carry the defective gene (Cystic Fibrosis 3). Mutations probably occurred in the CFTR gene

Webb 2 soon after it duplicated (Rosaler 2). The CFTR gene contains instructions on how to carry chloride ions in and out of the cell. A mutated CFTR gene will disrupt the chloride channels not letting the flow of water and chloride ions across the cell membrane. Since the mutated CFTR gene pathways that line the lungs and pancreas are usually covered in thick mucus in patients with Cystic Fibrosis, which interrupts the organs ability to function (What 2). The CFTR gene is made inside the cells in the glands of the respiratory system. The body will try to hold back mucus which eventually will build up and cause the symptoms if Cystic Fibrosis (Cystic Fibrosis 1). The Cystic Fibrosis gene affects people differently. People who are exposed to secondhand smoke have decreased lung ability. The CFTR gene makes it harder to breathe properly for people who are exposed to secondhand smoke (Secondhand 1). Vaccines are needed against all respiratory diseases because your immune system is weakened as a result of Cystic Fibrosis (Larson 721). Other than affecting your lungs Cystic Fibrosis affects your pancreas. Mucus blocks your pancreatic gland and makes it much harder to digest food (Rosaler 19). Cystic Fibrosis also affects the reproductive and endocrine systems making most men infertile because the vas deferens, the tube that carries sperm, is blocked with mucus. Overtime mucus build up can cause permanent damage such as scar tissue (What 1). Options for men who are infertile due to Cystic Fibrosis are insemination from a donor or adoption (Cystic Fibrosis 4). Cystic Fibrosis is the most common and the most lethal disease in the white population. Gallstones are also more present in a person with Cystic Fibrosis than a person without Cystic Fibrosis (Cystic 4). A good diet for people with Cystic Fibrosis is very important. A high protein-low fat diet is recommended. Pancreatic enzymes are needed to be taken with protein like meat to help digestion (Larson 720). For people who suffer from this disease there are organizations that can help. The Cystic Fibrosis Foundation was established in 1955by a group of doctors who had children afflicted with the disease. The Cystic Fibrosis Foundation is responsible for many new treatments for Cystic Fibrosis (Rosaler 33).

Webb 3 The Symptoms of Cystic Fibrosis can be very severe. They range anywhere from a cough to vomiting, yet everyday people with cystic fibrosis live normal life’s. People with Cystic Fibrosis have extremely salty sweat glands (Larson 1). A few symptoms of Cystic Fibrosis are wheezing, shortness of breath, trouble gaining weight, dry and salty skin, and asthma (Rosaler 4). Males with Cystic Fibrosis are less affected than women with the same disease (Cystic 6). Since women are affected more by Cystic Fibrosis a pregnancy may be life-threatening for both the mother and the baby (Cystic Fibrosis 5). If a women with Cystic Fibrosis does conceive a child they can have their amniotic fluid tested to find out if there are decreased enzymes, if so their child may have Cystic Fibrosis (Cystic Fibrosis 3). A blockage of the meconium ileus, occurs right after birth in babies that have Cystic Fibrosis. Babies have symptoms such as: excessive diarrhea, wheezing, vomiting, and cough with mucus. Children have some different symptoms in babies. They are: fever, poor growth, poor appetite, and malnutrition (Cystic Fibrosis 2). Mucus in babies or a child’s lungs could cause an infection in your lungs. Mucus can also block the duct from your pancreas to your small intestine (Campbell 1). When more mucus than is needed is in an airway of a patient with Cystic Fibrosis infection is common (Rosaler 26). The severity of Cystic Fibrosis symptoms varies among people (Cystic Fibrosis 2). In some patients the immune system in patients with Cystic Fibrosis is completely unaffected, but in others they have a hard time beating the common cold (Cystic 6). Although there is no cure for Cystic Fibrosis there are ways you can treat the symptoms. Lung problems can be treated with antibiotics which can help a person with Cystic Fibrosis (Cystic Fibrosis 3). Vitamin A, D, E, and K supplements are prescribed to patients who have Cystic Fibrosis to help aid digestion (Cystic Fibrosis 4). Doctors also recommend that a person with Cystic Fibrosis have a high protein, high carbohydrate diets so they can maintain their weight. Foods like French fries, hamburgers, and hot dogs would be acceptable (Rosaler 31). A pancreatic enzyme may need to be taken with protein

Webb 4 like meat to help digestion (Larson 720). Inhalers allow liquid medication to get to the lungs quicker and better. When you can get the medication to the lungs better that means the patient doesn’t have to take as much medicine which means they will experience fewer side effects (Rosaler 41). A device that gets liquid medication in the form of a mist into the patient’s airways is called a nebulizer. A nebulizer will loosen mucus that builds up in the patients airways (Rosaler 35). There are some tests you can take as an adult to find out if you have Cystic Fibrosis. One test, called the cheek test, involves testing white blood cells for the CFTR mutant gene. Another test, and the most common one, is called the sweat test. It measures the amount of salt or sodium in your skin and if you have more than the normal amount you probably have Cystic Fibrosis (Cystic Fibrosis 3). A treatment for Cystic Fibrosis is slow in the works but a cure will hopefully be found soon (Rosaler 37). Sometimes, even with treatment Cystic Fibrosis will take a person’s life. The average survival rate of a person with Cystic Fibrosis is thirty-six years of age and usually men live longer than women (Cystic 5). Cystic Fibrosis almost never affects African, Asian, or Jewish children (Larson 1). The actual cause of death in people with Cystic Fibrosis is reparatory infection and the enlargement of the right side of the heart (Cystic Fibrosis 1). In a person with Cystic Fibrosis the lungs are normal at birth but because of the disease they become infected shortly thereafter (Cystic 3). As a last resort a person with Cystic Fibrosis could get a lung transplant, but the transplant only has a survival rate of about fifty-five percent over three years (Cystic Fibrosis 5). Gene therapy is a new approach to treating cystic fibrosis. One form of gene therapy is to use a virus to override the Cystic fibrosis mutation. Viruses can’t reproduce on their own. They have to make viruses attack other cells and take them over in order to replicate. Viruses are not harmless creatures, scientist have to figure out a way to trick viruses into carrying good genes without causing disease. Viruses are made to change the host cells make-up and if they have a healthy copy of the CFTR gene the disease may be able to be reversed (Rosaler 46). Another form of gene therapy is to use a liposome. A

Webb 5 liposome is a fatty substance that will naturally coat a cell. If you coat the gene with leptosomes it can begin to repair the mutated gene (Rosaler 48). Even though gene therapy has the potential to cure Cystic Fibrosis there are some obstacles. One obstacle is that the body sees all viruses as enemies and the immune system immediately attacks it before the good virus can ever reach a diseased cell (Rosaler 49). Cystic Fibrosis is a disease that affects millions of people and there is no way to control if you get the disease or not. You have a one in four chance of getting the disease if both your parents are carriers. The symptoms can be handled so that you can live an ordinary life and new treatments such as gene therapy give Cystic Fibrosis patients hope for a cure in the near future.

Sign up to vote on this title
UsefulNot useful