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 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 meconium ileus – 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 Fibrosis Nursing Interventions At birth—monitor for 1 st meconium – Newborn screening—blood immunoreactive trypsinogen Genetic counseling Parent Education – High calorie, high protein, low fat diet – How to administer pancreatic enzymes – Protect from infection – Breathing exercises and care

Cystic Fibrosis Breathing Exercises Physical activity Chest percussion and postural drainage

Cystic Fibrosis Medications Aerosol Bronchodilators—opens lungs Aerosol DNAse—loosens secretions Corticosteroids—Anti-inflammatory Antibiotics—Treats infections Pancreatic enzymes—Aids in digestion Water soluble ADEK