Cystic Fibrosis Islamic university - PowerPoint Presentation

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Cystic Fibrosis

Islamic university

Nursing College

Cystic Fibrosis

Inherited—

autosomal

recessiveBoth parents must be carriersEach child has a 1 in 4 chance of being affectedAffects 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 tenaciousAbnormal sodium excretionSweat Chloride test

Heat Prostration

Cystic Fibrosis

Lungs—Secretions pool in bronchioles leading to infection and

atelectasis

.Barrel shape chest

CyanosisClubbing of fingers and toesRecurrent 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-smellingLarge belly, wasted extremitiesFat 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

AntibioticsPancreatic enzymes

Vitamin supplementsSalt supplements in hot weather?

Complications: Pneumothorax

Cystic Fibrosis

Nursing Interventions

At birth—monitor for 1

st meconium

Newborn screening—blood immunoreactive trypsinogen

Genetic counselingParent EducationHigh calorie, high protein, low fat dietHow to administer pancreatic enzymesProtect 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 secretionsCorticosteroids—Anti-inflammatoryAntibiotics—Treats infections

Pancreatic enzymes—Aids in digestionWater soluble ADEK