cystic fibrosis Flashcards
what is cystic fibrosis?
autosomal recessive disorder that causes mucous producing glands to work incorectely
no cure, life limiting
multi-system involvment: RESP+GI (pancreas, liver, intestines) + INTEG + REPROD
what is the pathophysiology?
mucous is supposed to be thin and slippery, in pt w CF, mucous is thick and sticky (tenacious)
dt CFTR gene that alteres cells ability to transport chloride and Na
leading to increased viscosity of airway mucous, abnormal airways clearance and lung disease
what are complications of CF?
- chronic bronchial pneumonia
- obstructive emphysema
- intestinal obstruction of newborn
- malabsorption syndrome
- portal htn
what are GI clinical manifestations of CF? (4)
1) pancreatic insufficiency (lack of digestive enzymes
(blockage of pancreatic duct from mucous)
- enzymes not accessible, stay in pancreas =>
inflammation => fibrosis
- give pancreatic supplements
2) CF related DM (relates to insuff digestive enzymes)
3) meconium ileus (d/t blockage of small intestine with thick, putty like, tenacious meconium) -> can lead to bowel obstruction
4) prolapse of rectum (d/t intestinal obstruction from impacted stools)
what are resp clinical manifestations of CF? (5) what can you do?
blockage of airways =>
1) low O2 and Co2 exchange = hypoxia, hypercapnia, acidosis
2) obstructive pulmonary disease (emphysema, bronchieatasis, pneumonia)
3) pneumothorax (collapsed lung, air in pleural space; pleural space becomes positive when it should be negative)
4) hemoptysis: d/t infection or severe damage to lung tissue (older ppl)
5) susceptible to infection d/t incomplete expectoration (interv: inspirometry) (Pathogens: P. arginosa, S.aureus)
- be careful!! chronic antibiotic use = resistance
*** double lung transplant doesnt cure but controls, prolongs and increases QOL
what are some reprod clinical manifestations of CF? (5)
1) INFERTILITY
2) delayed puberty
3) viscous cervical mucous
4) high risk pregnancies
5) adult male =sterile
what are INTEG clinical manifestations of CF?
1) sweat + tears = salty => electrolyte imbalance + dehydration
what are signs of pneumothorax?
1) tachycardia
2)dyspnea
3) pallor/cyanosis
4) drop in o2
5) fatigue
6) tachypnea
7) dry hacking cough
how do you manage pulmonary problems r/t CF? RATIONALE? (6)
airway clearance therapies:
1) aggressive chest physio (CPT) => to maintain. pulmonary
hygiene
- huffing technique: huff cough is performed by
taking a deep breath and then forcing the air out
of your l lungs with your mouth open like you
would do if you were trying to fog up a mirror. It
may help to hold your breath for three seconds
after inhalation to allow air to get behind or
below mucus.
- postural drainage: use gravity to drain mucus out
of your lungs by changing positions
2) inhaled/aerosol meds (bronchodilators, mucolytic) => to
decrease viscosity of mucous (ex; Pulmozyme)
3) “flutter” device => helps clear mucous
4) regular physical exercise
5) antibiotics (monitor blood serum levels for toxicity)
6) O2 therapy (caution: co2 retention-> hypoxic drive in
COPD pts - pt stops breathinh )
management of GI problems? rationales?
1) pancreatic enzyme supplements
- give w meals and snack to ensure that digestive
enzymes are mixed w food in the duodenum
- amount is adjusted to achieve normal growth and
decreased in steatorrhea
2) well balanced diet (high protein, high cal) => bcz of
impaired intestinal absorption
3) fat soluble vitamins (A,D,E,K)- missable form (diff form)
=> uptake of fat soluble vit is decreased
4) if needed: night time supplement of gastrostomy feed
what are the diagnostic test for CF?
1) Hx of disease in family
2) pt hx
3) increased electrolyte concentration of swear chloride
test (children = 2-3X above normal lvls)
4) chest x-ray (pulmonary involvement)
5) pulmonary fx test
6) stool fat or enzyme analysis
how is the sweat chloride test done in CF?
Chemical (Pilocarpine) applied to a small area (arm or leg) that is stimulated by WEAK electrical current. The sweat produced is collected on filter paper
no post care
what is the pulmonary fx test and how is it done?
it measures air flow rates, calculates lung volumes and capacities
NO metered dose inhalers (bronchodilators), aerosol therapy or bronchodilators 6 hours prior or as per protocol
may be asked to perform an aerobic activity (running, stationary bike putting stress on heart to increase endurance) during procedure
pt with compromised resp. function may be exhausted after this test. Pt. may need meds right away on return (bronchodilators)
how is the stool fat or enzyme analysis performed?
Measure fat in stool (bcz theres a problem w malabsorption of fat)
Collect stool for 24-72 hours, store in freezer
No post test care
