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- -progressive, incurable d/o, and resp failure is the most common death; organ transplantation to increase survival rates.
- -chronic multisystem d/o (autosomal recessive trait d/o)
- -exocrine gland dysfunction
- ~the mucus produced by the exocrine gland is abn thick, tenacious and copious, causing obstruction of the small passageway of the affected organs (resp, GIT and reproductive -common sx are associated with pancreatic enzyme deficiency, and pancreatic fibrosis caused by duct blockage, progressive chronic lung disease as a result of infection, and sweat gland dysfunction resulting in increased sodium and chloride sweat concentrations.
- RESPIRATORY SYSTEM:
- -(+) stagnation of the mucus in the airway: bacterial colonization and destruction of lung tissue.
- -airway obstruction: (+)emphysema and atelectasis.
- -chronic hypoxemia: (+) contraction and hypertrophy of muscle fibers in pulmonary arteries and arterioles leading to pulmonary hypertension and cor pulmonale
- -progression: pneumothorax frm ruptured bullae and hemoptysis frm erosion of the bronchial wall
- -wheezing and cough
- -clubbing of the fingers and toes
- -barrel chest
- -repeated episodes of bronchitis and pneumonia
- -NB: meconium ileus (is a bowel obstruction that occurs when the meconium in your child's intestine is even thicker and stickier than normal meconium, creating a blockage in a part of the small intestine called the ileum.)⇒EARLIEST MANIFESTATION
- -intestinal obstruction (distal intestinal obstructive syndrome) caused by thick intestinal secretions can occur; s/sx: pain, abdominal distention, n&v
- -stools: frothy and foul-smelling
- -deficiency in vitamins ADEK, which can result in easy bruising, bleeding and anemia.
- -malnutrition and failure to thrive
- -(+)hypoalbuminemia (diminished protein absorption⇒generalized edema).
- (+)rectal prolapse (this result from large, bulky stools and increased intraabdominal pressure).
- -(+)pancreatic fibrosis (is a progressive inflammatory disease of the pancreas)⇒@ risk for DM
- INTEGUMENTARY SYSTEM:-high Na and chloride in sweat
- -infants taste "salty: when kissed
- -dehydration and electrolyte imb occur, esp during hyperthermic conditions.
- REPRODUCTIVE SYSTEM:-delay puberty in girls
- -fertility can be inhibited by the highly viscous cervical secretions, which act as a plug and block sperm entry.
- -males⇒sterile, but not impotent, caused by a block in the vas deferens by abnormal secretions or by failure of normal development of duct structures.
- QUANTITATIVE SWEAT CHLORIDE TEST-(+)
- ↳ production of sweat is stimulated, sweat is collected (>75mg is needed), and sweat electrolytes are measured.
- ↳ normal: <40mEq/L
- ↳ (+) >60mEq/L
- ↳ infants: >40mEq/L (dx in infants younger than 3 mos of age)
- ↳ Chloride concentration of 40-60mEq/L are highly suggestive of CF and require a repeated test.
immunoreactive trypsinogen analysis and direct DNA analysis for mutant genes.
(+)atelectasis (partial or complete collapse of the lung) and obstructive emphysema (the air sacs of the lungs are damaged and enlarged).
PULMONARY FXN TEST:
(+)abn small airway fxn.
STOOL, FAT, ENZYME ANALYSIS:
72-hr stool sample is collected to check fat or enzyme (trypsin) content, or both (food intake is recorded).
- RESP. SYSTEM:
- -GOAL: px and tx pulmonary infection
- -improve aeration, remove secretions and giving antibiotic meds.
- -monitor: lung sounds and (+) cough
- -chest physiotherapy: on awakening and in the evening; frequent during pulmonary infection; done everyday to maintain pulmonary hygiene; DO NOT PERFORM BEFORE AND AFTER A MEAL.
- -FLUTTER MUCUS CLEARANCE DEVICE: removal of mucus; store away from small children; steel ball poses a choking hazard.
- -POSITIVE EXPIRATORY PRESSURE MASK: forces secretions to the upper airway for expectoration.
- -teach child huffing (forced expiratory technique) to mobilize secretions.
- -BRONCHODILATOR MEDS BY AEROSOL: opens the bronchi for easier expectoration; give BEFORE CP when the child has reactive airway dse or is wheezing).
- -MEDS: decrease viscosity of the mucus.
- -O2: acute episodes; WOF: O2 necrosis (n&v, malaise, fatigue, numbness, tingling of extremities, substernal distress) bec a child may have carbon dioxide retention.
- -lung transplantation: occassionaly performed.
- -high calorie, high protein and well-balanced diet to meet energy and growth needs.
- -multivitamins and vitamins ADEK
- -(+)severe lung dse: energy requirement must be as high as 20-50% or more.
- -monitor: weight and failure to thrive; stool patterns (intestinal obstruction)
- -(+)pancreatic insufficiency: replace pancreatic enzymes (given 30mins of eating and given with all meals and snacks); DO NOT GIVE IF CHILD IS NPO.
- -consuming high-fat foods: additional enzyme is given
- -ENTERIC-COATED PANCREATIC ENZYME should NOT be crushed or chewed; capsules can be taken apart and can be sprinkled on a small amt of food,.
- -WOF: constipation, intestinal obstruction and rectal prolapse.
- -WOF: signs of GERD; (+)GERD: place infant in an upright position and sit upright after eating.
- -monitor: glucose levels and signs of DM
- -adequate salt and fluid intake (esp on hot weather or if a child has a fever).
- -monitor bone growth
- -monitor for signs of retinopathy or nephropathy.
- -child receives immunization at the right time.
- -annual influenza vaccine is recommended.