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Resp Disease
Cystic Fibrosis
| Question | Answer |
|---|---|
| What is Cystic Fibrosis | It is a hereditary disease characterized by lung congestion and infection along with malabsorption of nutrients by the pancreas |
| What happens in cystic fibrosis | mucus clogs the lungs leading to chronic respiratory infections and mucus obstructs the ducts of the pancreas preventing digestive enzymes from reaching the intestines |
| What system is CF a dysfunction of | Exocrine |
| What happens to the Electrolyte in sweat? | they increase concentrations |
| What cause the thick secretions in CF | the disruption of the chloride transport, Na is absorped in the airways, pancreas and bile ducts |
| Which ethnicity is mostly like to be a carrier of the CFTR gene | White |
| What would the xray look like with a pt with CF | translucencies Enlarged heart flatten diaphragm |
| How is CF diagnoses | the sweat chloride test |
| What two infections together do we mainly see in CF | Staph aureus + Pseudomonas |
| What are some common respiratory problems do CF pt see | Atelectasis bronchial hyperreactive/ asthma hemoptysis pneumothorax pulmonary hypertension Respiratory failure sleep breathing problems symptomatic rhinosinusitis |
| What are some problems other than respiratory that a CF pt must see | anemeia Appendicitis Cystic fibrosis related diabetes (CFRD) -scarring of the pancreas. 75% of adult CF Pt. Gallbladder disease GERD Malnutrition, growth failure Infertility Pancreatitis Meconium ileus |
| How many positive test must you have to diagnose CF | two positive |
| What are some signs/symptoms a pt with CF might have | ↑ Sweat Chloride Salty sweat, residue on skin/clothes Abdominal Distention/cramping fatty stools Malnutrition even with ravenous appetite(FTT) Once pulmonary involvement – advanced COPD Hypercapnia and severe hypoxemia when the disease is very adva |
| What is a normal routine management for a CF pt | pulmozyme Sympathomimetics P & PD – most pts. Consider most important – PEP, Flutter, vest hypertonic saline xanthines- another way to bronchial dilate then steroids and ibuprofen- reduced 50% FEV1 |
| What are some other treatments | lung transplant gene replacement Vitamins and food supplements O2 PRN |
| What is the prognosis for a CF pt | no cure 32 2000 36.5 2009 |
Created by:
laney21882
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