1229 Exam 7: Cystic Fibrosis Flashcards
What is Cystic Fibrosis?
inherited as an autosomal recessive trait, inherited defective gene from both parents
CF characterized by thick, tenacious secretions on the lungs (like concrete), mechanical obstruction from the viscosity of mucous secretions
Patho of CF
Increased viscosity of mucous gland secretions, elevation of sweat electrolytes, increase in several organic and enzymatic constituents of saliva, abnormalities in Automatic nervous system function, reproductive changes
Etiology of CF
1 in 2500 white infants yearly
Biochemical defects
Mutated gene, chromosome 7
Assessment
Meconium ileus, salty tasting skin, profuse sweating, frequent inf?, dry non productive cough, increase amount & thickness of secretions, wheezing & cyanosis, digital clubbing, increased diameter of chest, assess stool for steatorrhea - excessive amt of fat in stool, azotorrhea- excess nitrogen in stool, thin ms wasting, failure to thrive
Lab Studies
Sweat test- Chloride>60 meq is +, NA>90 is abnormal Pilocarpine iontophoresis- "electric" Stool fecal fat/Tyrpsin Pulmonary fct study CXR Upper GI/Small bowel
Complications of CF
heat prostration, respiratory-most serious, GI-bowel and biliary obstruction, rectal prolapse, Endocrine-diabetes, Transplants
Therapeutic Management
Multi system approach Goal of Care: Meds- bronchodilators, adrenergic agonists--Nebulizer Mucolytics- pulmozyme, mucomyst Pancreatic enzymes
Treatments for CF
Chest physiotherapy (CPT) O2 Dietary supplementation Flutter Mucous Clearance Device Preventive actions from flu & pneumonia ThAIR vest
Nursing Interventions
CPT Respiratory assessment Cough/suction q 2 hours Mucolytics/Bronchodilators High Fowler's Handwashing Ht & Wt Calorie counts Family support
Nursing interventions cont..
Pancreatic enzymes High calorie and protein carb diet Salt S/S of infection Water miscible vit's Monitor # of stools daily
Diagnostic Studies
Newborn genetic screening "40 Genetic Test" 24 hrs after birth Strip test - 5 gtts of blood Family hx Absence pancreatic enzymes Elevated immunoreative trypsinogen
