Cystic Fibrosis Flashcards
Most common lethal, genetic disease in the Caucasian population
Autosomal recessive genetic disease
Cystic fibrosis
what does cystic fibrosis mainly involve?
exocrine glands
cystic fibrosis causes dysfunction of what regulator protein?
CFTS (cystic fibrosis transmembrane regulator protein)
problems with the GI tract with CF
deficient secretion of digestive enzymes fat-soluble vitamin deficiency insulin deficiency intestinal obstruction reflux
presentation of GI issues w/ CF patient.
Steatorrhea
malnutrition (below average for growth curves)
how do you diagnose pancreatic insufficiency
72 fecal fat collection
what causes digestive enzyme deficiencies in CF patient?
mucus plugging and damage to pancreas
what enzymes do you need to give someone with CF GI problems.
lipase, protease, amylase taken with each meal or snack
what can occur at really high doses of pancreatic enzymes?
fibrosing colonopathy
what should you recommend in CF patients with GI issues?
High calorie diet
Fat soluble vitamin replacement
may require extra salt (dumping so much salt out)
therapy for GI obstruction with CF patients.
Electrolyte lavage solutions to reach clear endpoints
Liver dz with CF patients
GGT may be elevated and reflect damage
biliary fibrosis, bile duct proliferation, chronic inflammatory infilatration
therapy for liver dz with CF patient
Ursodeoxycholate
decrease liver inflammation and bile duct proliferation
Respiratory problems with CF
chronic rhinitis, sinusitis, nasal polyps
5 therapies for pulmonary issues with CF
Airway clearance techniques (ACT) Anti-inflammatory agents Chronic antibiotics Mucolytics Bronchodilators
how often is ACT done for patients with CF?
twice a day
gets rid of secretions and improves ventilation
what antiinflammatory therapy do you use for CF patients
NSAIDs
Ibuprofen has shown decreased rate of decline with FEV1
what drug is given 3x a week to CF patients to help with inflammation
Azithromycin
what are some mucolytics for CF
Pulmozyme (most often used)
Mucomyst (n-acetylcystine)
What does Pulmozyme do?
Decreases viscosity of sputum
nebulized for administration
ADRs of pulmozyme
hoarseness
voice alteration
paryngitis
problem with mucomyst
irritating, can lead to additional bronchoconstriction
what does nebulized hypertonic saline do?
Draws water into airways, increases ability to cough out mucus
can cause bronchospasm
what tx for CF patients is also helpful if they have asthma?
Inhaled beta 2 agonists
inhaled corticosteroids
what is the major pathogen in CF patients in first year of life?
Staphylococcus aureus
what organism is found by age 3 in CF patients
Haemophilus influenzae
what organisms is colonized in CF patients by age 5
pseudomonas aerguinosa
what chronic antibiotics
nebulized tobramycin (TOBI) nebulized aztreonam (Cayston)
what do chronic antibiotics help with for CF patients?
prolong time b/w acute exacerbations
how do you do the dosing of the chronic antibiotics.
28 days on, 28 days off
what immunizations should CF patients specifically receive?
Flu vaccine Streptococcus pneumoniae (don't have to wait until 65)
what is the high concentration of in the sweat glands of a patient with CF
Abnormally high concentrations of sodium and chloride in sweat
reproductive problems in males with CF
males have congenital bilateral absence of the vas deferens
reproductive problems of patients with CF
delayed onset of puberty
what supplementation is needed for CF patients for low bone mineral density
calcium and vitamin D supplementation
2 bone and joint problems with CF
low bone mineral density
arthritis
tx of arthritis for patients with CF
short courses of nonsetroidal and steroidal inflammatories
is eradication of P. aerguinosa likely in patients with CF?
No
how to you tx acute pulmonary exacerabtions with CF
aminoglycosides in combo with an antipseudomonal penicillin
dose at upper range
when can you get a lung transplant with CF?
older than 10 years old
