Management Of Cystic Fibrosis Flashcards
Cystic fibrosis overview
Defect in CFTR proteins and prevents them transporting chloride in and out of cells.
This leads to the following three primary physiological effects:
1) increased chloride and sodium concentration in sweat
2) dehydration of mucus layers and inability to move mucus layers. This leads to chronic infections and destruction of epithelial cells since mucocillary transport is at a halt
3) increased sodium and water reabsorption rates in cells
Primary 4 organ systems affected by cystic fibrosis
Respiratory
- increased recurrent upper and lower respiratory infections (especially pneumonia)
GI
- primarily pancreatic and liver
Integumentary
Reproductive
Pulmonary symptoms related to cystic fibrosis
Recurrrent pneumonia And/or sinusitis
Chronic coughing
Wheezing
Nasal polyp formations
Bronchiectasis
Obstructive lung disease issues on PFTs
Acute pulmonary exacerbation
A consequence of untreated cystic fibrosis
Symptoms
- increased productive coughing
- fever
- change in sputum color
- hemoptysis
- dyspnea
- sinus pain/tenderness
- fatigue/malaise/lethargy
4 most common infectious agents in cystic fibrosis
1) staph aureus
2) haemophilus influenza
3) pseudomonas aeruginosa
* most dangerous*
4) bulkhoideria cepacia
* 3 and 4 are dangerous and harder to cure, but are still rarer than 1 and 2. However cystic fibrosis populations have the highest rates *
GI symptoms related to cystic fibrosis
Pancreatic insufficiency/ pancreatitis
most common and will lead to diabetes
Prolonged neonatal jaundice
Biliary cirrhosis
Meconium ileus
- blockage of the ileum in children
- “soap bubble” signs on xray that are really black
- palpable mass in liver area
Steatorrhea
Malabsorption of fat-soluble vitamins
- A,D,E,K
Integumentary symptoms related to cystic fibrosis
Salty skin
- babies taste salty
Digital clubbing
Reproductive symptoms related to cystic fibrosis
99% of males w/ CF are infertile since the ejaculatory duct and vas deferens are not present as well as hypoplasia of seminal vesicles
Females tend to have difficulty getting pregnant but still can
Diagnosis of cystic fibrosis
Newborn screening
- check for immunoreactive trypsinogen levels (will be really high for CF patients)
Sweat test
- patient oversweats w/ prevalence of stupid high chloride levels
Genetic testing
Treatment of cystic fibrosis
Goals:
- increase life expectancy (right now it hovers around 40)
- improve quality of life
- optimize nutritional status
- optimize pulmonary function
Non pharm:
- OMM (especially rib raising)
- chest physiotherapy
- exercise regiments
Pharm:
- mucinolytics
- Nebulizer
- albuterol
- fat soluble vitamin supplements
- pancreatic enzyme replacements
- antibiotics (only if infection is present)
- NO STEROIDS
- lung transplant (last resort)
