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Leading up to
Characterized by:
The DEFECT in CF seems to be in abnormal chloride movement.
Pharmacologic Treatment
Broken into 3 sections :
1) Management of Pulmonary Problems (prevention and treatment of pulmonary infection)
2) Management of GI problems (pancreatic insufficiency)
3) Management of Endocrine problems (insulin resistance and insulin deficiency)
Nursing Interventions
Resources
-Assess pulmonary (focus on lung sounds, cough and fatigue)
**watch for any evidence of growth failure**
(weight loss, muscle wasting pallor, anorexia, decreased activity)
-Monitor pt tolerance and effectiveness of treatment
**stress treatment compliance
Holistic Interventions
Pulmonary Medications
GI Medications
Endocrine Medications
IV antibiotics
-Tobramycin, ticarcillin and gentamicin
**2-3 antibiotics and 1 antifngal medication to treat coexisting pulmonary infections
-PICC may be used for home administration
Aerosol Therapy
~ Bronchodilator (opens bronchi for easier expectoration. admin before percussion and postural drainage when pt exhibits evidence of reactive airway disease or wheezing)
~ Human Deoxyribonuclease (decreases the viscosity of mucus)
~ Nebulized hypertonic saline (improves airway hydration and increases mucus clearance)
-Family support
~Recommend support groups
~Need to be very involved in care...educate about medications, equipment (PICC, ACT), diet, signs and symptoms of infections, and the importance of physical activity
-"Play Periods"
include PT
-Assess for signs of depression, anxiety and disturbed self image
-Flexible care plan so that child can have as normal a life as possible
-Provide O2 in acute situations
-Administer pancreatic enzymes as prescribed 30 min within eating
(to ensure that digestive enzymes are mixed with food in the duodenum)
-Vitamin A, D, E, K
(due to inadequate uptake of fat-soluble vitamins. growth failure despite adequate nutritional support may indicate deterioration of pulmonary status. )
-Polyethylene-glycol electrolyte solution (GoLYTELY) via NG tube for constipation as prescribed
-Histamine-receptor antagonist and motility medications for GERD
- TPN
USE CAUTIOUSLY -- CF pts have chronic CO2 retention
- administer insulin PRN
pt present with a combination of insulin resistance and insulin deficiency with unstable glucose homeostasis in the presence of acute lung infection and treatment.
**may be at increased risk for glucose management problems as a result of decreased nutrient absorption, anorexia, and severity of pulmonary illness.
***with repeated infection and inflammation, bronchial cysts and emphysema may develop. If the cysts rupture --> pneumothorax