...Section 3 T.A. - Sheree Speckman November 15, 2011 Introduction: Cystic Fibrosis is one of the most common chronic lung diseases in children and young adults. It is an inherited deadly disorder that affects Caucasians in the United States. Cystic fibrosis of CF is a disease that is caused by a defected gene, which causes the lungs to build up with an abnormally thick and sticky fluid, or mucus. This mucus affects the lungs obstructing breathing along with also affecting the pancreas, causing poor absorption of nutrients. For my family Cystic Fibrosis is a well-known disease, because my Aunt Rae Ellen died of it back in 1962. The history of Cystic Fibrosis dates back into the mid-17th century where it was first known as a child who had become bewitched. Advances in cystic fibrosis began to pick up in the 1940s. Children with CF are normally diagnosed by the age of 2 and now can live to around the age of 40. There are many different symptoms that come along with this disease. There are different symptoms related to newborns, bowel functions, lungs, and later in life. The most common symptoms seem to be coughing or increased mucus in the lungs and salty-tasting skin. People with cystic fibrosis have mutations on the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which reduces the ability of chloride ions to travel across cell membranes. There is no way to prevent Cystic Fibrosis but there are ways to deal with it and have a quality life with it....
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...Cystic Fibrosis Cystic fibrosis is a recessive, genetic illness, which affects the work of lungs, liver, intestine, and pancreas. This disease causes malfunctioning of the chloride and sodium transportation across the body and results in the thick and viscous secretions. Nowadays, cystic fibrosis affects thousands of people, especially in Europe and the US. Although, cystic fibrosis is a rare disease, it is quickly spreading all over the world and leads to the general shortage of life. In fact, it is a serious disease, which calls for an immediate diagnosis and appropriate treatment. History of the Disease Cystic fibrosis was discovered in the first half of the 20th century. However, without doubts, it emerged thousands of years before. The researchers surmise that cystic fibrosis appeared about 3000 BC, as a result of migration and gene mutation. During the 16-17th centuries, the salty taste of the skin (which is the symptom of cystic fibrosis) was considered a sign of bewitchment. Dr. Dorothy Andersen was the very first to describe the peculiarities and main characteristics of this disease. Before that investigation, the humanity had already known some facts and elements of cystic fibrosis. The reason for such late examination of this disease can be explained with the lack of required tools and means. Even nowadays, there are still many researches, aiming at providing objective information about cystic fibrosis and applying to the methods of the newborn and antenatal...
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...Cystic Fibrosis Cystic Fibrosis (CF) is a life threatening disorder that causes severe damage to lungs and digestive system. CF affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery but, in CF a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant the secretions plug up tubes, ducts and passage ways especially in lungs and pancreas. CF is most common in white people of Northern European ancestry, but also occur in Hispanics, African Americans, and some Native Americans. (Mayo Clinic). More than 70% of patients are diagnosed by age two. The predicted median age of survival for persons with CF is in late 30’s. Symptoms of CF can be found in many ways, from very salty- tasting skin, persistant coughing at times with phlegm, frequent lung infections, wheezing, or shortness of breath, poor growth or weight gain in spite of good appetite, or frequent greasy, bulky stools or difficulty in bowl movements. Although CF requires daily care most people with condition of CF are able to attend school and work. (CFF2013) CF is diagnosed by many ways, all states screen newborns for CF using genetic testing or blood test. The blood test shows whether a newborns pancreas is working properly. Another way is sweat test, this test is most useful for diagnosing CF, a sweat test measures amount of salt in sweat. Doctors triggers sweating on small patch of skin on an arm or leg, he or she...
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...Cystic Fibrosis Cystic Fibrosis is an inherited lifelong disease that affects more than 30,000 children and young adults in the United States (Kids Health, 2007).This genetic disease which affects the transportation and clearance of electrolytes and fluids across cell membranes from exocrine glands (Yourlunghealth.org, 2004). Exocrine glands are present everywhere in your body that comes into contact with an external environment. There is no cure for cystic fibrosis (Medline Plus, 2009). Cystic Fibrosis affects the lungs, pancreas, digestive tract and reproductive tract (American Lung Association, 2008). Cystic fibrosis mainly affects the lungs and pancreas. It causes the lungs to create thick, sticky mucus blocks in the airway making it very hard for the child to breath and also very easy for them to get infections. This can lead to tissue damage and airway inflammation (Yourlunghealth.org, 2004).Eventually the chest will assume a barrel shape with increase in size in the front and back of the child (Price and Gwin, 2008).In the pancreas mucus blocks prevent enzymes that help to break down food from reaching the intestines and aiding in digestion(Genetics Home Reference, 2009).Since the digestive tract cannot digest food properly this can lead to many problems, such as poor absorption of vitamins and minerals which can ultimately lead to malnutrition (Yourlunghealth.org, 2004). Kids with cystic fibrosis have trouble gaining weight even when they have a normal diet and a healthy...
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...----------------------- Nursing Diagnosis: Ineffective airway clearance R/T increased pulmonary secretions AEB Patient has thick purulent sputum and crackles noted upon ascultation Nursing Diagnosis: Risk for infection R/T tenacious secretions and altered body defenses Medical Management: IV antibiotic treatment with Ceftazidime, CPT, and observation Medical Diagnosis: Cystic Fibrosis Signs & Symptoms: Color pale pink with bluish tinged nail beds. Patient Hx: Patient Initials: J.R. 10 year old white male. No previous injuries, has a history of CF Goal: Client will be able to remove secretions from the airway w/i 10 minutes Goal: Client will remain free of infections during my shift Goal: Client’s nutritional status will improve, and the client will exhibit normal growth within 1 week Nursing Interventions 1) The child should take deep breath and then exhale rapidly while whispering the word huff (forcing expiration) 2) Perform CPT 2 or 3 x’s a day and as needed. Determine child’s respiratory status before and after CPT. 3) Administer ordered bronchodilators or mucolytics in conjunction with any other treatments 4) Elevate head of bed, or support child in an upright position and stay with child during coughing episodes. Nursing Interventions 1) Monitor the child for s/s of respiratory infection (fever, chills, increased respirations, dyspnea, purulent secretions) 2) Advise the family to avoid exposing...
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...Matto Massaad 1/19/16 AP Bio Mr. Duehr Cystic Fibrosis In 2010, two sisters, diagnosed with cystic fibrosis, went on America’s Got Talent to show the world that they would not let this horrible disease drag them down. People said from the start, according to Christina and Ali, “they would never be able to sing.” They were from a family of four children who were all diagnosed with cystic fibrosis. Cystic fibrosis is a single gene disorder. This disease is still very life threatening even though science, medicine, and technology have come a long way since the mid 20th century. In the past fifty years, cystic fibrosis patients have gone from dying as infants to having their average life expectancy be from 3540 years old, (Kaneshiro.) Cystic fibrosis is caused by a mutation of the CFTR gene. This gene makes a protein called CFTR or (cystic fibrosis transmembrane regulator.) This protein balances the salt and water content on epithelial surfaces by providing a channel for the movement of chloride ions in and out of cells, (Genetics and Nutrition.) The CFTR protein can be altered if there is a fatal mutation in the CF gene. Although there are so many different mutations of this gene, the most common is found in almost ninety percent of all cystic fibrosis patients. This mutation is just a simple deletion of three nucleotides. These three nucleotides were to create the amino acid phenylalanine, but with CF, that is never made. The CF protein never makes it to the cell ...
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...BI 112 Genetic Paper Cystic Fibrosis Cystic fibrosis is a disease that can be genetically passed down to offspring. According to the cystic fibrosis foundation’s website, “it affects the lungs and digestive system of about 30,000 children and adults in the United States,” and “70,000 worldwide” (www.cff.org). The online website lab tests online, records that cystic fibrosis “is a relatively common disease caused by mutations in the gene located on chromosome 7” (www.labtestsonline.org). This chromosome contains a protein called cystic fibrosis transmembrane regulator or CFTR and a mutation in this gene “lead to absent or defective CFTR production causing cystic fibrosis (www.labtestsonline.org). There are some very specific signs and symptoms of this disease. Some of the most common symptoms are: “very salty tasting skin, persistent coughing at times with phlegm, frequent lung infections, wheezing or shortness of breath, poor growth/weight gain in spite of a good appetite, and frequent bulky tools or difficulty in bowel movements” (www.cff.org). It is also reported by the American lung association’s website that it “causes thick, sticky mucus to form in the lungs, pancreas and other organs. In the lungs, this mucus blocks the airways, causing lung damage and making it hard to breathe. In the pancreas, it clogs the pathways leading to the digestive system, interfering with proper digestion” (www.lungusa.org). It is important to be genetically tested to see if there...
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...mastered by birth. There’s nothing like not being able to breathe to persuade people to appreciate this simple, necessity. Cystic Fibrosis (CF) affects more than 30,000 children and adults in the United States, and 70,000 worldwide. (#7 about CF) Cystic Fibrosis is a chronic, autosomal recessive genetic disorder (#11 page 2). There is no cure for cystic fibrosis; as a matter of fact, it is a progressive disease and will only worsen over time.(#7 about CF). Everyone has a slippery substance (mucus) that lubricates and protects the linings of the airways, digestive system, reproductive system, and other organs and tissues in the body. Patients with CF have mucus that is abnormally thick, and sticky that no longer acts as a lubricant. This unhealthy mucus blocks the airways in the lungs causing permanent damage and loss of function, over time the digestive tract, mainly the pancreas is being clogged by this thick and sticky mucus, leading to malabsorption of food and nutrition in the intestine (#7 How CF may affect the body). Overall, understanding that Cystic Fibrosis is a genetic disorder with many complications, requiring constant testing to manage and treat the disease, while learning to live with CF to improve the quality of life. When it comes to cystic fibrosis, family matters, especially Mom and Dad. Every child inherits two CFTR (Cystic Fibrosis Transmembrane Conductance Regulator) genes, one from each parent. CF occurs when a child inherits a faulty CFTR gene from...
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...Cystic Fibrosis The television must be turned up again to do another breathing treatment or it is time to get tapped before bed. I watch in awe as my little cousin sits there with a shaking vest on, and I wonder what is going on. If it’s time for another meal or if he just wanted a snack, he cannot forget to take his enzymes that he needs to aid in the digestion of food. I again start to wonder what is going on inside and how this little boy ended up with this disease, so I began my search in trying to learn as much about Cystic Fibrosis as I can. As of 2014, Cystic fibrosis (CF) infects about 70,000 people around the world and is known as the most common life shortening disease (Bell 2014). CF is the result of an offspring receiving two recessive alleles from each parent which comprise of a mutation inside the cystic fibrosis...
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...Adult Client with Childhood Disease-Cystic Fibrosis Candace Tiley GCU RN-BSN Health Assessment NRS-434V Kathy Karlberg August 01, 2013 Adult Client with Childhood Disease-Cystic Fibrosis CLC GROUP ASSIGNMENT-SPEAKER NOTES Candace Tiley #1- A clinical description and definition of the disease Definition: Cystic Fibrosis is one of the major life-threatening disorders that affect vital organs such as the lungs, pancreas, liver, and intestines. It is a genetic disorder of the exocrine glands, also called the secretory glands, which are the glands that produce and secrete mucus and sweat. CF affects all races but primarily affects Caucasians of European descent. This disease affects about 30,000 people in the United States and 70,000 people worldwide. In the U.S. nearly 5% of the population carries the defective CFTR gene. There are a high number of asymptomatic carriers. (Scott, 2013) Cystic Fibrosis causes severe damages to cells of the body that produce mucus, sweat, and digestive juices. Under normal conditions, these secretions from the body are usually thin and slippery. However, in people diagnosed with CF, the defective gene causes the secretions to become thicker and stickier. This leads to a plugging up of tubes, ducts and passageways, particularly in the lungs, sinuses, pancreas, intestines, hepato-biliary tree, and the vas deferens. (Hopkin, 2009) Cystic Fibrosis is characterized by abnormal movement of chloride and sodium ions across the epithelial...
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...Cystic Fibrosis affects more than 30,000 children and adults in the United States. Cystic Fibrosis, also known as CF, is a life-threatening genetic disease that causes lung infections and limits the ability to breathe. CF also affects other organs as well. People who have CF are born with a defective gene that they must receive a copy from both parents. There are more than 1,800 known mutations of CF. Factors such as age of diagnosis, can affect an individual’s health and the course of the disease. The defective gene causes a buildup of thick and sticky mucus in the lungs, pancreas and other organs. Mucus is normally slimy and a little thicker than water. In the lungs the thick mucus buildup clogs airways and traps bacteria which leads to...
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...have been around for decades now. Its is not anything new. Cystic Fibrosis affects both the lungs and pancreas. Thousands, and millions of people have been diagnosed with CF worldwide. But, what are the causes and effects of Cystic Fibrosis? Cystic Fibrosis is a genetic disease that affects the lungs, pancreas, and gall bladder. CF also affects the respiratory and digestive system. The person with CF would have had to be born with a defective gene, which did not work correctly, causing thick mucus to build up in the lungs, pancreas, and gall bladder. The disease, CF was first described and named by Dorothy Anderson in 1938. Dorothy Anderson was born in Asheville, North Carolina, May 15, 1901. Dorothy had...
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...Treatment: There is no cure for cystic fibrosis, so the only way to treat the disease is to try to control the complications associated with it. One of the treatments is loosening the mucus that has accumulated in the lungs by either clapping the patient on the back and chest with cupped hands or with the use of a vibrating vest that also helps loosen mucus so it can be coughed up and removed from the lungs. If oxygen is low in the blood because of poor lung function, oxygen therapy may be needed. Medications that are used are antibiotics that treat and prevent the recurring respiratory infections that are common in people with cystic fibrosis, drugs that thin mucus and allow it to be coughed up which helps with lung function, oral pancreatic enzymes are used to replace the enzymes being blocked by mucus buildup in the pancreatic duct, so nutrients can be absorbed in the digestive system, and bronchodilators are used to keep airways open to allow as much oxygen in with the least resistance possible. Some other treatments are the use of a feeding tube...
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...Cystic Fibrosis is a disease that for which one in every 25 Americans carry a gene, which means around 12 million Americans are carriers. It is caused by a mutation in the Cystic Fibrosis Transmembrane Conductance Regulator, causing an abnormal amount of mucus to be secreted, settling the lungs and pancreas. The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) is a protein that allows for chlorine ions to be able to escape mucus-producing cells. It helps thin out the mucus to keep the airways clear. When that gene is altered, it causes life-threatening harm to newborn babies, where most of them wouldn’t last longer than a couple of months. Due to the benefits of modern diagnosis and treatment, the life-span of a Cystic Fibrosis affected...
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...Cystic Fibrosis Characteristics Cystic fibrosis is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body. Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are usually thin and slippery, but people with cystic fibrosis the defective gene causes the secretions to become sticky and thick. A patient with cystic fibrosis needs around the clock care. Causes/Risk Factors In cystic fibrosis, a defective mutation in a gene that changes a protein that regulates the movement of salt in and out of cells. May different defects can occur in the gene. The type of gene mutation is associated with the severity of the condition. Children have to inherit one copy of the gene from each parent in order to have the disease. If children inherit one copy, they won't have cystic fibrosis, but they can carry it and pass it on to their children....
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