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28 Cards in this Set
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Cystic fibrosis |
Autosomal recessive gene passed from both parents affecting exocrine glands. Lungs reproductive,lungs and skin. How does CF |
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How does CF Affect the pancreases? |
Thick secretions block ducts which can cause diabetes. |
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Cardinal signs in newborns that may have cystic fibrosis? |
Meconium ilues at birth. Or bowel obstruction. |
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Life expectancy with Cystic fibrosis? |
37 years old more known in Caucasian males. |
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How to diagnose cystic fibrosis? |
Sweat test = measure amount of salt
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Levels for sweat test? |
39 or less = negative 40-59= needs further testing 60 or more= positive |
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What’s the mutated gene in cystic fibrosis? |
Cystic fibrosis transmembrane regulator |
CFTR |
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Exocrine glands affected in cystic fibrosis? |
Mucous = thick Tears Sweat = too much salt Enzymes = pancreas |
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Complications cystic fibrosis has on lungs? |
Emphysema Hyperventilation Air trapping Bloody sputum Infection |
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Possible infection in lungs? |
Pseudomonas Stag Aureus *Develops resistance or allergies ** lung transplant ** |
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How does CF Affect upper airway? |
Sinus issues Nasal polys (Snoring, stuffiness) |
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How is GI system affected by cystic fibrosis? |
Unable to digest vitamins, fats and proteins. *Protease *Amylase *Lipase |
PAL |
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S/S GI complications with Cystic fibrosis? |
Weight loss Malabsorption Vit deficiency and protein Delayed puberty **greasy foul stool and bloating |
Vitamin DEKA |
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How is the liver affected in cystic fibrosis? |
Bile duct blocked with thick mucous leads to gallstones. |
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How does CF Affect female reproductive system? |
Thick mucous causes non sperm friendly environment |
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How does CF Affect male reproductive system? |
Vas deferens duct may not fully develop or may be blocked with thick mucous. |
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Integumentary problems with CF? |
Production of too much salt. * electrolyte imbalance * dehydration ** give extra salt during excessive sweating |
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Intervention/ treatment for cystic fibrosis? |
Chest physiotherapy Postural drainage PEP devices Huff coughing Nebulizers **** THESE ARE A HUGE PART OF TREATMENT *** |
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What is chest physiotherapy? |
Drains airways of thick mucous so it can be coughed up * Postural drainage * Percussion/ vibration * Huff coughing |
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How often and when do you do Chest Physiotherapy? |
2-4 times per day 20 mins to 1 hour 1-2 hours between meals * never do before or after meals DO ON EMPTY STOMACH |
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PEP Positive expiratory pressure |
Breathe in and out creates vibration then huff cough EXAMPLE DEVICES: * FLUTTER VALVE * ACAPELLA |
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Diet for cystic fibrosis? |
High calorie protein and fat diet |
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Aerobic exercise for CF? |
Clears secretions Caution: *EXCESSIVE SWEATING AND LOST CALORIES |
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PPE for Cystic fibrosis? |
Wear mask Up to date on vaccines Hand washing Avoid sickness |
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Vitamins to replace with Cystic fibrosis? |
Fat souble *DEKA |
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Pancreatic enzymes needed for cystic fibrosis patients? |
Administer Pancrelipase and pancreatin * PEDS * GIVE WITH ACIDIC FOODS. EX : APPLESAUCE *NEVER CRUSH * TAKE BEFORE OR AFTER MEALS |
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GI prevention with cystic fibrosis? |
Drink water Give mirlax daily *MONITOR BLOOD SUGAR / MAY NEED INSULIN |
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Blockage with cystic fibrosis? |
No bowel movement No gas Increased pain |
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