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cystic fibrosis
pn 141 test 2 book:burke pg 561
| Question | Answer |
|---|---|
| what is it | an inherited disorder of childhood that causes excess mucous secretions |
| what system of the body does it damage most | the lungs |
| the genetic defect of CF causes an excess of what | excess mucous production in the lungs |
| the mucous plugs what up | small airways |
| the plugging of the airways leads to what | atelectasis, infection and bronchiectasis, and ariway dilation |
| what happens to the lungs | they become scarred and stiff |
| what disorders develop over time | COPD, pulmonary HTN, R sided CHF |
| what is pulmonary HTN | increased pressure in the pulmonary vascular system |
| what happens with sweat | there is an increased secretion of sodium and chloride in it |
| what enzyme are they lacking | a pancreatic enzyme |
| s/s of it | dyspnea, chest congestion, chronic cough productive of large amounts of thick sticky sputum, barral chest and clubbing of fingers and toes |
| s/s of digestive problems | abd. pain, steatorrhea |
| what is steatorrhea | bulky foul smelling stook |
| what are treatment goals of CF | preventing and treating respiratory complications and maintaining adequate nutrition |
| diagnostic tests: what one Dx it | pilocarpine iontophoresis sweat chloride test |
| diagnostic tests: pilocarpine iontophoresis sweat chloride test- what itis | sweat is collected and analyzed for sodium and chloride |
| meds: what enzyme is given to improve airway clearence; what does it do | dornase alfa; it liquefies mucus |
| meds: why are antibiotics given | prophylactically to prevent recurrent resp. infections |
| what transplants help | lung transplants |