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  • Cystic Fibrosis Outline 2009

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    ipurrrdie
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    This document discusses cystic fibrosis, an autosomal recessive genetic disorder that affects the exocrine glands and causes thick, sticky mucus to build up in the lungs, pancreas, and other organs. It outlines the pathophysiology and clinical manifestations of cystic fibrosis, including increased mucus in the lungs leading to chronic lung infections. The document also discusses strategies for treatment, including airway clearance techniques, antibiotics, pancreatic enzyme replacement, nutrition counseling, and supportive therapies to promote optimal functioning for individuals with cystic fibrosis.

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    This document discusses cystic fibrosis, an autosomal recessive genetic disorder that affects the exocrine glands and causes thick, sticky mucus to build up in the lungs, pancreas, and other organs. It outlines the pathophysiology and clinical manifestations of cystic fibrosis, including increased mucus in the lungs leading to chronic lung infections. The document also discusses strategies for treatment, including airway clearance techniques, antibiotics, pancreatic enzyme replacement, nutrition counseling, and supportive therapies to promote optimal functioning for individuals with cystic fibrosis.

    Copyright:

    Attribution Non-Commercial (BY-NC)
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    Markieren Sie unangemessene Inhalte
    19 Ansichten4 Seiten

    Cystic Fibrosis Outline 2009

    Hochgeladen von

    ipurrrdie
    This document discusses cystic fibrosis, an autosomal recessive genetic disorder that affects the exocrine glands and causes thick, sticky mucus to build up in the lungs, pancreas, and other organs. It outlines the pathophysiology and clinical manifestations of cystic fibrosis, including increased mucus in the lungs leading to chronic lung infections. The document also discusses strategies for treatment, including airway clearance techniques, antibiotics, pancreatic enzyme replacement, nutrition counseling, and supportive therapies to promote optimal functioning for individuals with cystic fibrosis.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Als DOC, PDF, TXT herunterladen oder online auf Scribd lesen
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    von 4

     CYSTIC FIBROSIS Sandra H.

    Reid MSN, RN-C

     Objectives

     Discuss pathophysiology & clinical manifestations of CF

     Formulate collaborative strategies to promote optimal functioning of the child with CF

     Identify learning needs of the family R/T medication administration, therapies & nutritional requirements

    Autosomal Recessive Disorder

    Heredity

     Racial Differences

     ( But …Not apply to individuals)

     From all walks of life

     Most common fatal heredity disease

     30,000 Americans, 1: 3,300 live births

     Disease of the exocrine glands

     Body not brain

    Features

     Viscosity of mucous

     Sweat electrolytes

     Change in saliva

     Abnormalities in autonomic nervous system function

     Abnormal chloride movement, Pancreatic insufficiency

    Effects of Increased Viscosity

     Mechanical Obstruction

     Meconium Ileus & Rectal Prolapse

     Liver

     Biliary obstruction

    • Malnutrition & Cirrhosis

    • Portal hypertension , Esophageal varices & blebs


     Pancreas

     Fibrotic Enzymes Malabsorption

     Pulmonary System

     Life threatening / Variable age of onset

     Patchy atelectasis, Hyperinflation, Emphysema

     O2 – CO2 Exchange

     Hypoxia, Hypercapnia, Acidosis

     Increased Infection

     Reproductive System

     Female

    • Cervical Mucous Plug

     Male

    • Abnormal Wolffian Ducts, > 95% Sterility

    Diagnosis

     Familial history

     Sweat chloride test

     Taste “salty”

     Cl > 60 mEq/L, (norm < 40 mEq/L)

     Na & Cl 3 to 5 x higher

     Lack Pancreatic Enzymes

     Stool Analysis

     Pulmonary Involvement

     Chest X ray, PFT’s

    Clinical Findings

     Infancy

     _______________, _______________, _______________, _______________


     Cardiac Enlargement

     Rt ventricular hypertrophy

     Evidence of Chronic Respiratory involvement

     B_________ shaped chest, Clubbing, Cyanosis / Pallor & Distended neck veins, Fatigue

    Therapeutic Management

     Prevent & Tx. Respiratory Infections

     Improve aeration

    • B_______________

    • Recombinant human deoxyribonuclease

    • E_______________

     Remove secretions

    • CPT – minimum 2x / day

    • Flutter mucus clearance device, Vibration

    • No Antihistamines

     Anti-microbial agents

    • Sputum C&S

    • Central venous access

    • Vaccinate for pneumococcus & influenza

     Oxygen (humidified), Pneumothorax – (s/s?)

     Promote Optimal Nutrition

     High Protein / High Calorie

    • 150%, Normal Fat, ? Salt

     Replace pancreatic enzymes

    • 1-5 Capsules, Sprinkle

     Infancy

     Vitamins, Tube Feedings, PN

     Skin care
     Provide Emotional Support & Counseling

     _________________________

     _________________________

     _________________________

     _________________________

     _________________________

     _________________________

    Prognosis

     Pulmonary involvement

     40 years of advancement & research

     Improved length & quality of life

     Carrier screening

     Gene Therapy / Lung transplants

     Remains progressive & incurable

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