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27 Cards in this Set
- Front
- Back
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CYSTIC FIBROSIS
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-INHERITED D/O
-CAUSES DISFUNCTION IN ALL EXOCRINE GLANDS -MOST COMMON INHERITED DISEASE IN CHILDREN (CAUCASIONS) -AUTOSOMAL RECESSIVE TRAIT:BOTH PARENTS MUST BE CARRIERS -2 PARENTS CARRIERS=25 PERCENT CHANCE THAT CHILD WILL HAVE CF |
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WHAT GIVES RISE TO SEVERAL SEEMINGLY UNRELATED CLINICAL FEATURES AS A RESULT OF DYSFUNCTION OF THE EXOCRINE GLANDS
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-A GENETIC DEFECT OF CHROMOSOME 7
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WHAT SYSTEMS ARE AFFECTED WITH CF
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-LUNGS
-GI -**PANCREAS |
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WHAT IS CF CHARACTERIZED BY
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INCREASED PERSPIRATION
-SWEAT HAS UP TO 5X'S AS MUCH SALT TO IT "TASTE SALTY WHEN KISSED" |
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PULMONARY EFFECTS
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-PRESENT IN MOST CHILDREN WITH CF
-PULMONARY COMPLICATIONS ARE MOST SERIOUS(MAY CAUSE DEATH) -VISCOUS MUCOUS COLLECTS IN THE SMALL AIRWAYS (BRONCHIOLES) |
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WHAT DOES THE VISCOUS MUCOUS IN THE AIRWAY CAUSE
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-OBSTRUCTION TO AIRFLOW-->HYPOXIA,HYPERCAPNIA,ACIDOSIS
-AIR TRAPPING -ATELECTASIS -STAGNATION OF SECRETIONS -CYANOSIS,CLUBBING OF NAILS -MUCOUS SO THICK,CANNOT EXPECTORATE -S/S SIMILAR TO COPD |
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WHAT RESULTS AFTER PANCREAS AFFECTED BY CF
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**MALABSORPTION**
-STETORRHEA(FREQUENT,LARGE,LOOSE,FOUL SMELLING,GREASY STOOLS) -PANCREATIC ENZYMES (AMYLASE,TRYPSIN,LIPASE)ARENT AVAILABLE TO BREAK DOWN FATS,CHO,PROT. |
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WHAT SYSTEM IS AFFECTED DURING THE LATE STAGES OF SEVERE DISEASE (DIABETES MELLITUS)
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-ISLETS OF LANGERHANS
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WHAT OCCURS WHEN ISLETS OF LANGERHANS AFFECTED
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-BOWEL OBSTRUCTION (FROM THICK MECONIUM) OR RECTAL PROLAPSE IN CHILD
-OBSTRUCTION OF BILIARY DUCTS FROM MUCOUS (CAUSES CIRRHOSIS AND PORTAL HYPERTENSION) -ANEMIA OF CHRONIC DISEASE |
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SYMPTOMS OF MALABSORPTION
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-FAILURE TO THRIVE
-FREQUENT FROTHY,FOUL SMELLING GREASY STOOLS-**STETORRHEA -WT LOSS DESPITE VORACIOUS APPETITE -SALTY TASTE TO SWEAT |
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WHAT IS CF MISTAKEN FOR
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-WHOOPING COUGH,CHRONIC BRONCHITIS,ASTHMA
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WHY MIGHT WOMEN WITH CF HAVE DELAYED SEXUAL MATURATION
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INFERTILITY IN WOMEN DUE TO THICKENED VAGINAL MUCOUS
-MALES HAVE ATROPHIED OR OBLITERATED VAS DEFERENS |
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WHAT IS THE DEFINITIVE DIAGNOSTIC TEST FOR CF
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THE SWEAT TEST
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PULMONARY S/S
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-TACHYPNEA,WHEEZING
-CHRONIC PRODUCTIVE -DYSPNEA -BARREL CHEST -CLUBBING AT NAIL BEDS FROM HYPOXIA -CYANOSIS |
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G.I. S/S OF CF
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-FAILURE TO THRIVE
-NEWBORN=MECONIUM ILEUS (THICK MECONIUM CAUSING AN INTESTINAL OBSTRUCTION) |
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THE SWEAT TEST
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-SAFE AND RELIABLE IF DONE AT MED CTR
-MEASURES SODIUM AND CHLORIDE IN SWEAT -SIBLINGS OF CHILD W/ CF SHOULD ALSO BE TESTED -AFTER PUBERTY RESULTS MORE DIFFICULT TO INTERPRET |
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NORMAL SODIUM CONCENTRATION=
POSITIVE SWEAT TEST= |
NORMAL SODIUM<40 MEQ/L
POSITIVE SWEAT TEST>60 MEQ/L |
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PANCREATIC ENZYMES
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OBTAIN SPECIMEN BY GASTROSCOPY
-DECREASED OR ABSENT LEVELS SEEN WITH CF |
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FAT ABSORPTION IN STOOLS
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5 DAY COLLECTION
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NURSING DIAGNOSIS
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-INEFFECTIVE AIRWAY CLEARANCE
-ALTERATION IN NUTRITION -KNOWLEGE DEFICIT |
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WHAT IS THE FUTURE THERAPY FOR CF
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GENE THERAPY
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AMILORIDE
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NEW DRUG**
WORKS BY AEROSOL TO THIN RESPIRATORY MUCOUS -USED IN ALL PT'S WITH CF AND PULMONARY SYMPTOMS |
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PULMOZYME (DORMASE)
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THINS SECRETIONS
-IBUPROFEN IN HIGH DOSES DECREASES RATE OF LUNG INFLAMMATION |
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WHAT TO DO FOR PULMONARY
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-EXPECTORANTS (IODIDES)
-BREATHING EXERCISES BID -POSTURAL DRAINAGE -HUMIDITY (AEROSOL OR NEBULIZATION) -INCREASE ORAL FLUIDS -ANTIBIOTICS |
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WHAT TO DO FOR PANCREATIC
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ADMINISTER PANCREATIC ENZYMES WITH MEALS AND SNACKS TO OBTAIN NORMAL BOWEL MOVEMENT AND GROWTH
-PANCREASE,COTAZYM-S |
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DIETARY MANAGEMENT FOR CF
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NEEDS TO EAT MORE THAN NORMAL CHILD
-HIGH PROTEIN,HIGH CALORIE,HIGH SALT -MODERATE FAT -REPLACEMENT OF VIT A,D,E,K |
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LIFE EXPECTANCY FOR CF
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30 YRS
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