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 absorbed in the airways, pancreas, and bile ducts
Which ethnicity is mostly like to be a carrier of the CFTR gene White
What would the x-ray 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