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Cystic Fibrosis - Coggle Diagram
Cystic Fibrosis
Treatments
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Airway clearance technique of coughing and huffing to help loosen and get rid of the thick mucus in the lungs
Inhaled medicines that include antibiotics to thin the mucus, open the airways, and fight infections
Pancreatic enzyme supplement in capsules that have small beads containing digestive enzymes to help improve absorption of nutrients
Fitness plan to improve lung function, energy, and overall health
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Some inhaled medications are bronchodilators such as albuterol, hypertonic saline to mobilize mucus and improve airway clearance, and pulmozyme to thin mucus
A fitness plan could include walking, jogging, swimming, dancing, or other muscle strengthening activities
3 main types of modulators include potentiators, correctors, and amplifiers
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Correctors help CFTR protein form the right 3-D shape so that it is able to move to the cell surface and stay there longer
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Incidence/Prevalence
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Occurs in 1 in 2,500 to 3,500 white newborns
Less common in other ethnic groups: 1 in 17,000 African Americans and 1 in 31,000 Asian Americans
More than 30,000 people are living with CF in the United States
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There are approximately 1,000 new cases diagnosed each year
More than 70,000 people with CF worldwide
Pathogenesis
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CFTR protein produced by this gene regulates the movement of chloride and sodium ions across epithelial cell membranes
When mutations occur within the genes, the sodium ion transport is defective which then results in a buildup of thick mucus throughout the body
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Decreased mucociliary clearance (self clearing mechanism of the airways) combined with altered ion transport can allow bacterial colonization of the respiratory tract
The most common types of bacteria found in the respiratory tract are pseudomonas, Haemophilus influenza, and Staphylococcus aureus
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Diagnostics
Sweat test to measure the amount of chloride in the sweat. People with cystic fibrosis have a high level of chloride in their sweat
Newborn screening is a blood test from the baby to check the levels of a chemical made by the pancreas called immunoreactive trypsinogen (IRT)
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IRT is an enzyme precursor that is normally made in the pancreas; In cystic fibrosis, IRT can be secreted at higher levels into the bloodstream due to blockages in the pancreatic ducts
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Risk Factors
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If both parents are carriers of the CFTR gene: 25% chance of inheriting two copies and having CF; 25% chance of inheriting no mutations and not being affected; 50% chance of inheriting one copy and being a carrier
Possible environmental factors such as tobacco smoke and bacterial infections can lead to the progression of CF
Possible socioeconomic factors such as educational attainment, living/working conditions, family environment, social support, and material well being can all lead to the progression of CF
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Clinical Manifestations
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Digestive
Foul-smelling, greasy stools
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Intestinal blockage, particularly in newborns
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