Cystic Fibrosis Islamic university Nursing College
Cystic Fibrosis • Inherited—autosomal recessive • Both parents must be carriers • Each child has a 1 in 4 chance of being affected • Affects primarily white children Father Mother (carrier) (carrier) Carrier Unaffected Affected Carrier
Cystic Fibrosis • Multi-system disease—affects exocrine glands • Bronchioles, small intestines, pancreas, bile ducts • Exocrine secretions—thick and tenacious • Abnormal sodium excretion • Sweat Chloride test • Heat Prostration
Cystic Fibrosis • Lungs—Secretions pool in bronchioles leading to infection and atelectasis. • Barrel shape chest • Cyanosis • Clubbing of fingers and toes • Recurrent respiratory infections
Cystic Fibrosis • Pancreas—absence of pancreatic enzymes and malabsorption(lipase for digestion, trypsine for protein, amylase CHO) • Small intestine—Meconium hardens leading to meconiumileus • Stools are bulky and fatty (steatorrhea) Stools may also float due to excess lipid, have an oily appearance and be especially foul-smelling • Large belly, wasted extremities • Fat soluble vitamin deficiencies (A,D,E,K)
Cystic Fibrosis • Pulmonary infections: • Thick mucus which obstruct alveoli, decrease exchange of gazes. • Cough. • Worsening respiratory distress with its symptoms. Recurrent chest infection • Respiratory acidosis (due increase level of CO2) • Clubbing of fingers
Cystic Fibrosis • Males usually sterile due to blocked vas deferens. • Females may have trouble conceiving due to thick mucus in the reproductive tract.
Diagnosis • Sweat test (very high in sodium chloride). • Chest x-ray. • Stool exam & enzyme analysis, absence of pancreatic enzymes.
Cystic Fibrosis • Medical treatment • Bronchodilators • Antibiotics • Pancreatic enzymes • Vitamin supplements • Salt supplements in hot weather? • Complications:Pneumothorax
Cystic FibrosisNursing Interventions • At birth—monitor for 1stmeconium • Newborn screening—blood immunoreactivetrypsinogen • Genetic counseling • Parent Education • High calorie, high protein, low fat diet • How to administer pancreatic enzymes • Protect from infection • Breathing exercises and care
Cystic FibrosisBreathing Exercises • Physical activity • Chest percussion and postural drainage
Cystic FibrosisMedications • Aerosol Bronchodilators—opens lungs • Aerosol DNAse—loosens secretions • Corticosteroids—Anti-inflammatory • Antibiotics—Treats infections • Pancreatic enzymes—Aids in digestion • Water soluble ADEK