Cystic Fibrosis

Cystic Fibrosis

1. pathophysiology

   • *Genetic -chromosome 7-Protein CF transmembrane conductance regulator (CFTR)-localizes to epithelial surface of airways, GI, liver ducts, pancreas and sweat glands.  CFTR-regulates Na & Cl in and out of cells. Mutations alter channels become blocked. Leads to low sodium content leading to thick sticky mucous which plugs ducts in organs, resulting in scarring thus leading to organ failure.  High concentrations of Na & Cl in sweat of patient result from decreased Cl reabsorption. 
   • Clinical Manifestations
     • Pt s/sx: increased congestion x1 week. Febrile at 37.9, and abnormal lab values. O2 89% 
     • Meconium ileus
       • When meconium ileus is present in the newborn may be indicative of diagnosis of CF.  -Abdominal distention, vomiting, failure to pass stools, rapid development of dehydration
     • Respiratory
       • initial signs: Wheezing respirations, dry, nonproductive cough  delayed:increased dyspnea, paroxysmal cough, evidence of obstructive emphysema and patchy areas of atelectasis.  Progressive: Overinflated, barrel shaped chest, cyanosis, clubbing of fingers & toes, repeated episodes of bronchitis ad bronchopneumonia. 
     • GI Manifestations
       • Large, bulky, loose, frothy, extremely foul smelling stools. Voracious appetite (early), loss of appetite (late), weight loss, marked tissue wasting, growth failure, distended abdomen, thin extremities, sallow skin, evidence of deficiency in fat soluble vitamins A,D,E & K, Anemia. 
   • Lab values
     • WBC-15000-Elevated  Hgb-18-Elevated  Hct-51-Elevated Platelets-250,000 Glucose-130-elevated K+ 4 therapeutic  Na+ 130-decreased  Cl 90-decreased  BUN 26-elevated Cr 0.7-therapeutic 
   • Diagnostics
     • Chest XR- Consolidation of right lower lobe and middle lobes. 
     • PFT -decreased in vital capacity 

2. Complications

   • CFRD-CR related diabetes  Bone disease liver disease pneumothorax hemoptysis  clubbing of fingers & toes  respiratory failure  multisystem organ faillure 

3. 3. Ineffective breathing pattern r/t tracheobroncial obstruction

   • Goal: Repairing or maintaining a normal breathing pattern, achieving maximum lung function.  interventions: Fowler/semi-fowler position to enable lung expansion and ease breathing. Teach deep breathing techniques/diaphragmatic breathing. Observe VS.  Monitoring respiratory rate, and quality of respirations. 

4. 4. Risk for infection

   • Goal: prevent or reduce risk of infection.  interventions: assess causative/contributing factors. Reduce existing risk factors by preventing HAIs with PPI. Promote wellness. 

5. 2. Impaired gas exchange r/t airway obs by nasal obs

   • Goal: Maintaining adequate oxygenation/ventilation.  Interventions: Maintain a patent airway. Proper positioning-high fowlers, sitting or leaning forward. monitoring of VS, ABG, O2.  Provide supplemental oxygen monitoring closely for CO2 necrosis. Motivation exercise appropriate physical condition of the patient to clear accumulated long secretions & improve endurance. 

6. 1. Ineffective Airway Clearance r/t excess tenacious mucus

   • Goal: Patient airway will remain patent. pt will not aspirate.  Interventions: Monitor respiratory status.  Utilize SVN treatments and O2 therapy.  Perform CPT 3-4 times/day.  Suction when necessary such as inability to cough.