Cystic Fibrosis

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Author:
japanice27
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306857
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Cystic Fibrosis
Updated:
2015-08-26 07:44:05
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cysticfibrosis
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pedia
Description:
review about CF
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  1. CYSTIC FIBROSIS
    • -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.
  2. S/SX:
    • 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
    • -dyspnea
    • -cyanosis
    • -clubbing of the fingers and toes
    • -barrel chest
    • -repeated episodes of bronchitis and pneumonia

    • GIT:
    • -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.
  3. DX TEST:
    • 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.


    NB SCREENING: immunoreactive trypsinogen analysis and direct DNA analysis for mutant genes.

    CXRAY: (+)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).
  4. INT:
    • 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.

    • GIT:
    • -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.
  5. HOME CARE:
    • -child receives immunization at the right time.
    • -annual influenza vaccine is recommended.

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