Cystic Fibrosis by https://padlet.com/polenskebrooklynn/8afcida33ww4 Pediatrics en-us 2018-09-09 18:13:03 UTC 2018-09-10 15:53:01 UTC hello@padlet.com Disease polenskebrooklynn https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279185555 A genetic disease characterized by progressive obstructive lung disease that is a result from a mutation in the CFTR gene. ]]> 2018-09-09 18:40:37 UTC https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279185555 Age group polenskebrooklynn https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186071 Patients are born with the gene mutation and can live up to 38 years old.]]> 2018-09-09 18:43:31 UTC https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186071 Diagnostics polenskebrooklynn https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186189 Every newborn in the US and some other countries are screened with a sweat test (pilocarpine iontophoresis) is the gold standard for diagnosing CF. This tests fro the amount of chloride in the sweat, CF patients will have more chloride in their sweat. Genetic testing is also used to identify genetic mutation of the CFTR gene]]> 2018-09-09 18:44:13 UTC https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186189 Treatments polenskebrooklynn https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186205 Daily therapy includes airway clearance techniques, exercise, inhalation therapies, and infection control
Airway Clearance 
  • Postural drainage , percussion, and vibration *should not be done with patients that have GERD
    Used mostly in infants
    High-Frequency Chest Wall
  • Oscillation (HFCWO)
    This is used once the patient can fit into a vest size, usually a toddler and into adulthood. Settings are variable with each patient, but should be reevaluated often to make sure the patient is getting the maximum benefit. 
  • Forced Expiratory Technique (FET) Huff breathing that helps create an increased expiratory airflow, mobilizes secretions. Usually introduced at age 4.
  • Active Cycle of Breathing (ACB)
    An activity that has the patient cycle through 3 cycles of breathing 1) normal diaphragm breathing 2) thoracic expansion, usually 3-4 breaths 3) FET. This type of breathing usually gets the patient to cough up mucous.
  • Oscillating PEP and PEP therapies
  • IPV
Exercise
  • Improves minute ventilation and expiratory air flow
Inhaled medicataions
  • Bronchodilators: albuterol and levalbuterol
  • Mucolytics: Hypertonic saline and Dornase alfa
  • Antibiotics: Tobramycin, Aztreonam, and Colistin


]]> 2018-09-09 18:44:27 UTC https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186205 Vent management and settings, weaning considerations polenskebrooklynn https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186228 NIPPV is used in End-stage Lung Disease when they require supplemental O2 during sleep or feel breathless.
Mechanical ventilation and intubation is considered when the patient goes into respiratory failure.]]> 2018-09-09 18:44:45 UTC https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186228 Short and long term outcomes. polenskebrooklynn https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186266 Short term
  • Hemoptysis: common in advanced lung disease
  • Pneumothorax: Small or large, may require a pleurodesis to prevent recurrence .
Long term
  • End-stage Lung Disease: Identified by Increased respiratory compromise with dyspnea and gas exchange abnormalities.  High flow mask may help with breathlessness. NIPPV can treat dyspnea.
  • Lung transplant: used in adolescents with respiratory failure. NOT A CURE.  Contraindications: noncompliant to therapy, have resistant organisms, uncontrolled diabetes, and severe malnutrition.

]]> 2018-09-09 18:45:06 UTC https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186266 Pathophysiology polenskebrooklynn https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186339 The CFTR gene encodes a chlorid channel that resides on the surface of the airway epithelial cells. This results in a lack of chloride secretions in the airway and an excessive removal of sodium from the airway, thus dehydrating the liquid layer of the the epithelial and impairing the mucociliary clearance, causing increased secretions.
Along with the pulmonary issues there is gastrointestinal manifestations that include meconium ileus at birth, that requires a surgical fix, and a pancreatic insufficiency that requires an enzyme replacement whenever the CF patient eats. ]]> 2018-09-09 18:46:00 UTC https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279186339 Signs and Symptoms polenskebrooklynn https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279187298 Pulmonary manifestations include: Obstruction of the small airway by viscous secretions and infection with inflammation that will eventually lead to bronchiectasis and mucous plugging.
Some CF patients have gastrointestinal manifestations like pancreatic insufficiency that result in stools that are greasy, oily, or foul smelling. They also require almost 2x the amount of calories and have trouble gaining weight.]]> 2018-09-09 18:56:21 UTC https://padlet.com/polenskebrooklynn/8afcida33ww4/wish/279187298