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CYSTIC FIBROSIS

(MUCOVISCIDOSIS)SATURDAY GROUP

Canlas, FlorisonCapigon, KarenFurio, Larriza

Gabriel, DaizeleVillanueva, Glady

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Cystic Fibrosis Multi-system disorder of the exocrine

glands, leading to increased productionof thick mucus in bronchioles,smallintestines and pancreatic and bileducts & even causes fertility problems

An autosomal recessive disorder Obstructs small passageways of these

organs

Lungs (bronchioles): atelectasis (lung

collapse) & Emphysema (Overinflation ofthe alveoli)

Pancreatic ducts become clogged,impairing digestion and absorption

Small intestine: absence of pancreatic

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INCIDENCE

1: 30 EUROPEANS

1: 3,000 WHITES

1 : 15,000 BLACKS 1 : 90,000 ASIANS

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Assessment Recurrent Upper Respiratory Infection

(URI) Respiratory impairment: Wheezing, dry

non productive cough, dyspnea &

digital clubbing Thick Sticky Mucus

Decreased digestive enzymes

Steatorrhea fatty, foul smellingstools

Meconium ileus

Decreased absorption of Vitamins

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CFTR Gene

Cystic Fibrosis transmembraneConductance Regulator

Transports chloride ions across the

membranes of the cells in the lungs,liver, pancreas, digestive tract, skinand reproductive tract.

Mutation of CFTR gene causessodium and chloride imbalance whichcan lead to thick-sticky mucus.

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Lungs

Ineffective airwayclearance

Perforation of bronchialwall

Respiratory acidosis

Mucocillaryactivity

Ineffectivecoughing

Bacterial growth

Chronic bronchitis /Pneumonia

Trigger inflammatoryresponse

Dilation ofbronchioles

Lung abcess

Tissuenecrosis

consolidation

fibrosis

Arterialerosion

hypertrophy

Sputumproduction

hyperplasia

Crackles /ronchi

Air trapping

hemoptysis

Dilation ofalvoeli

Narrowing of theairways

cyanosis

hypoxia

Activityintolerance

fatigue

dyspnea

Chestcongestion

Metabolicalkalosis

Hyperinflationof lung

Lung

elasticity

emphysema

Lung stiffening

Alveolarcollapse

atelectasis

hemorrhage

RESPIRATORY DISTRESS == > RESPIRATORY FAILURE

Uncompensated

respiratoryacidosis

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Biliarycirrhosis

AUTODIGESTION

Pancreatitis

fibrosis

Diabetes mellitus

Meconiumilleus

DEATH

Vit A, D, E,K

malabsorption

Inability todigest fat

Inability todigestprotein

Decreased / absentcirculating digestive

enzymes

Pancreatic ductobstruction Bile duct

obstruction

Small intestinepancreas liver

Digestivetract

Inability to thriveMALNUTRITION

diarrhea

Rectal prolapsesteatorrhea

constipation

malabsorption

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ReproductiveSystem

male

Obsturctionof vas

deferens

female

Blocks spermflow

azoospermia

Thick stickycervical

mucus

Blocks spermentry

INFERTILITY

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Diagnostic Procedure

SWEAT CHLORIDE TEST

Pilocarpine iontophoreses Used tostimulate sweat production

Analyzes sodium and chloride contentin sweat

Done 3 4 weeks after birth

- A chloride concentration greaterthan 60meq/L is a diagnostic of cysticfibrosis

- Parents often report that infants taste

salty when kissed

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Diagnostic Procedure

72-hour stool collection (Keep fooddiary)

Analyzes fat & enzyme content

Chest x-ray

To reveal atelectasis & obstructive

emphysema

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Priority NursingDiagnosis

Ineffective airway clearance Impaired gas exchanged Risk for infection Altered Nutrition Altered Bowel Movement Activity intolerance Alteration in Activities of Daily Living

Fear/Anxiety Knowledge deficit Risk for ineffective family coping

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Nursing Management Avoid exposure to respiratory infection

CPT Bronchodilator

Antibiotics / Mucolytics

Monitor hemoptysis

HIGH PROTEIN, CALORIE diet

Supplement w/ VITS ADEK (in H20

Soluble form) Monitor weight

Supplement the childs diet with fluids& salt during extremely hot weather

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Pancreatic Enzyme Pancrealipase (Pancrease,

Coazym,Ultrase) Used in to aid in digestion &

absorption of nutrients;

Administered on or before mealsand snacks to ensure that digestiveenzymes are mixed with food in theduodenum

Enteric coated pancreatic enzymesshould not be crushed or chewed

Dose is adjusted to suit normal growth

& decrease in the number of stools per

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CURRENT TRENDS ANDRESEARCHES IN CYSTIC

FIBROSIS

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Comparison of Hospital andHome Intravenous Antibiotic

Therapy in Adults with CysticFibrosis

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Clinical Manifestations ofCystic Fibrosis Among

Patients With Diagnosis inAdulthood

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Metabolic AlkalosisContributes to Acute

Hypercapnic RespiratoryFailure in Adults Cystic

Fibrosis

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Inhaled Tobramycin ImprovesLung Function and BacterialDensity Associated with

Pseudomonas aeruginosainfections in patients with

Cystic Fibrosis

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The Physical, Psychological,and Social Implications ofCaring for the Pregnant

Patient and Newborn WithCystic Fibrosis