Cystic Fibrosis Flashcards
(45 cards)
What does PEP stand for, and what is it?
Positive Expiratory Pressure - a patient will inhale air at a normal flow, then resistance as they exhale will mean slower and more forceful, pushing secretions out
What is the pathophysiology of cystic fibrosis?
Defect of the CFTR gene - cells unable to transport chloride ions.
Mucous-secreting cells: prevents chloride from being secreted -> intracellular levels increase -> water not attracted to mucus to thin it out -> mucus abnormally thick -> build up of mucus
Sweat-producing cells: chloride ions not reabsorbed -> accumulate in sweat
What is the name of the test performed on newborn babies that screens for cystic fibrosis?
Guthrie Heel Prick test
What are three ways a diagnosis of cystic fibrosis can be confirmed?
- Sweat test (chloride levels >60mmol/L = diagnosis very likely) - gold standard
- DNA testing (gene mutation analysis)
- Stool test (enzyme: elastase)
How does a person inherit CF?
It is an autosomal recessive disorder - inherited when a person receives a mutated copy of the CFTR gene from both parents.
On what # chromosome is the CFTR gene?
7
What organs/organ systems are affected by cystic fibrosis?
- Respiratory system
- Gastrointestinal system
- Liver
- Endocrine system
- Reproductive organs
How does CF affect the respiratory system?
- Abnormal, sticky secretions
- Recurrent infection and inflammation
- Wheezing
- Chronic cough
How does CF affect the gastrointestinal system?
- Malnutrition due to malabsorption of fat and fat-soluble vitamins
- Poor weight gain (failure to thrive)
- Frequent, foul-smelling, greasy bowel movements
- Stomach aches
- Excessive wind
- Liver disease
- Intestinal obstruction (constipation)
- Meconium ileus (neonatal intestinal obstruction)
Signs and symptoms of cystic fibrosis:
- Salty tasting skin
- Slow weight gain or failure to thrive despite good appetite
- Wheezing, coughing, pneumonia
- Abnormal bowel movements
How does CF affect the reproductive system?
Men: blockage of or underdeveloped vans deferens (fertility problems, but not impotent)
Women: irregular menstrual cycles due to nutritional issues (but have healthy, fertile eggs)
What are some indications of infection in CF?
- increased, moist, or more productive cough
- yellow-green sputum
- wheeze
- SOB
- decreased exercise tolerance
- lethargy
- loss of appetite
What S&S might you expect as disease progresses?
- Respiratory distress
- Finger clubbing
- Chest deformity (hyperinflation, flattening of diaphragm)
- Reduction of function
- Respiratory failure
What proportion of Caucasian NZers are thought to be carriers of the CF gene?
1 in 25
The absorption of what vitamins is impaired (due to blocked ducts in the pancreas)?
Vitamin A, D, E, & K
What are six bacteria that can cause lots of trouble for CF patients?
- Pseudomonas aeruginosa
- Staphylococcus aureus (incl. Methicillin-resistant - MRSA)
- Burkholderia cepacia
- Aspergillus
- Non-tuberculous mycobacteria
- Influenza
Why are people with CF recommended to stay at least 6 feet from each other?
Cross-infection - more likely to pass bacteria on to each other
What is common medical treatment for CF?
Antibiotics and inhalation therapy
How are antibiotics commonly administered for CF?
- Antibiotic route (oral, nebuliser, dry powder inhaler, TOBI Podhaler)
- IV (peripheral cannula, PICC, portacath)
Types of inhalation therapy for patients with CF?
- Bronchodilators eg salbutamol (Ventolin)
- Preventers eg Seretide
- Hypertonic saline (before and during airway clearance techniques)
- Bronchitol
- Pulmozyme
- Nebuliser
- Trikafta
When should bronchodilators be used?
- PRN
- Before airway clearance techniques
- Before hypertonic saline
Will a lung transplant cure CF?
No - as CF still affects organ systems other than the lungs. Median survival post transplant ~8.5 years. Transplanted lungs susceptible to infection and rejection
How can CF affect the endocrine system?
Can lead to CF related diabetes in adolescents/adulthood - due to scarring of exocrine pancreas -> disruption to insulin secreting endocrine cells
What is described as the ‘cornerstone of CF treatment’?
Airway clearance
