Cystic Fibrosis (CF)

CysticFibrosis (CF)
What is it?
*Mucoviscidosis, autosomal- recessive inherited disease
Signs and Symptoms:
*Dysfunction of- mucus producing exocrine glands
*High level of sodium (Na) and chloride (Cl) in tears, saliva, sweat
*Highly viscous secretions in pancreas, bile ducts, small intestine, bronchi
*Meconium ileus sign in newborn infants
Who does is affect?
*1 in 2,000 live births
-80-90% have pancreatic insufficiency
-85% growth retardation
-Average Life Span 24 years (improving up to 30-40 now)
-Death often from malnutrition, lungs collapse, cor pulmonale, bronchopneumonia
-Lung transplantation with some good results
What can I do?
*See your Physician
*See a Registered Dietitian for help with meal planning
*See FFF Registered Dietitian on line for help
-Fats may not be well tolerated
-Lactose intolerance is common
-Use soft foods if chewing causes fatigue
-Encourage fish- for the fatty acids
*Achieve desirable weight, avoid aneroxia due to respiratory distress
*Protein- for growth, resistance to infection, development
*Replace electrolytes as loss in vomiting