class 5 cystic fibrosis Flashcards
jan 23
how is CF inherited
autosomal recessive
what causes CF
a deletion on chromosome 7
this causes a mutation in the cystic fibrosis transmembrane conductance regulator protein (CFTR) which transports chloride out of the cells do no sodium or H2O getting thru too
how is CF diagnoses
-newborn screening
-gene testing
-chloride sweat test (>60 positive, <30 negative)
what happens in the lungs with CF
-increased secretions lead to obstruction, leading to repeated infections, bronchiectasis, and decline in lung function, causing respiratory failure
-compromised mucociliary and cough clearance
what happens in the pancreas and GI with CF
-pancreatic ducts are obstructed by thick secretions, leading to decreased excretion of enzymes into the intestine= dec breakdown of fat and protein
-vitamin ADEK deficiency
-pancreas is autodigested and replaced by scar tissue
-can develop CF-related diabetes
-inc risk for celiac disease & colon cancer
what happens with the liver with CF
-irritation & inflammation of bile ducts
-liver cirrhosis= usually presents earlier in life
-hepatomegaly
infection control for CF patients
-patient-to-patient transmission is an issue in this population
-standard precautions, transmission-based precautions including contact and droplet
-hand & equipment hygiene
-inc risk for infection
objectives of CF care
-focus CF care on lung function, nutritional status, and CFRD
-emphasize early detection for tx
-provide education
treating CF with antibiotics
-tx aggressively to prevent lung decline
-oral/home IV/ inpatient
-with chronic therapy
-azithromycin usually used as chronic abx
-exercise
-antibiotic resistance prevalent
order of medications given
1.bronchodilator
2.mucolytic
3.airway clearance (PEP/percussion/huff and couch)
4.inhaled antibiotics (need clean airway)
5.inhaled antiinflammatories (need clean airway)
what is airway clearance with CF
-daily/multiple times daily
-enhances clearance of mucus after puffer/neb
-postural drainage & percussion
-positive expiratory pressure therapy
-deep coughing
-high-frequency chest wall
nutritional therapy for cf patients
high fat and protein diet
-pancreatic enzyme replacement (PERT)
-nurtitional supplements
-vitamin & mineral supplements
-enteral feeds
-BMI should be 22-23
what is pancreatic enzyme replacement therapy (PERT)
-needed to break down fat, CHO, proteins
-different enzymes break down different nutrients
-takes 45-60min to work, needed before meals/enteral feeds
-lipase, proteases, amylases
Nutrition supplementation with Cf
Used if struggling to gain weight
Oral protein drinks(boost)
Ng and peg tube feeds
Enzymes given before tube feeds too
Fat soluble vitamins with CF
-lvls measured annually
-vit A D E K need fat for absorption
-taken at mean time (fat)