Cystic Fibrosis PPTX

8/3/2019 Cystic Fibrosis PPTX

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Cystic FibrosisCystic Fibrosis(Mucoviscidosis)(Mucoviscidosis)


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Overview:Overview:

What is Cystic Fibrosis?

Cystic fibrosis (also known as CF ormucoviscidosis) is a heterogeneous

recessive genetic disorder with features that reflect mutations in the cystic

fibrosis transmembrane conductance regulator (CFTR) gene.

The name cystic fibrosis refers to the characteristic scarring (fibrosis)and cyst formation within the pancreas.

A chronic, progressive, and frequently fatal genetic disease of the bodys

mucus glands.

Affects the respiratory and digestive systems in children and young adults.

The mucus is abnormally thick and sticky that can block tubes and ductswhich causes infections and altered functions.

It occurs approximately 1 in 2500 births.


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CF

Etiology:

Genetically transmitted disease

Genes (DNA)

From parents

If both parents carry the defective gene, there is a 25% chance thattheir child will have cystic fibrosis; a 50% chance that theirchild not develop the condition but will be a carrier of the CFgene and a 25% chance that their child will neither be a carrier

nor will they have the disease.


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CF

Etiology:

Gene located on 7th chromosome

2 copies of genes needed to inherit disease

Carrier states


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Pathophysiology:

Defective gene

Related to protein involved inchloride ion transport

Body produces thick, sticky mucus

Clogs the lungs

Stagnant mucus Lung Infections

Obstructs the pancreas

Malabsorption & malnutrition

CFTR mutations affect epithelial cells,

thus affecting:

Airways (sinuses, lungs)

Pancreas (endocrine and exocrine)

GI tract (liver/biliary system,

intestines)

Reproductive organs

Skin


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Risk Factors:

Family history. Because cystic fibrosis is an inherited

disorder, it tends to run in families.

Boys with CF may not be able to reproduce because they

have persistent plugging and blocking of the vas deferens

from tenacious seminal fluid. Girls may have such thick cervical

secretions that sperm penetration is limited. Artificial

insemination or in vitro fertilization can be accomplished if

they desire to become pregnant.

Race. Although cystic fibrosis occurs in all races, it is most

common in white people of northern European ancestry.

Okay.


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Signs and Symptoms:

Symptoms may include:

Meconium ileus

Appears at birth

Salty-tasting skin When newborn is kissed

Steatorrhea

Greasy, bulky and foul smelling stool

Poor growth/weight gain in spite of good appetite

Chronic coughing, at times with phlegmFrequent lung infections

Frequent trouble in defecation

Nasal polyps - small, fleshy growths in the nose

THE SYMPTOMS!

ASSESS ALL


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Diagnostic Tests

NewbornScreening Test

- This test checks a bloodsample for a particular componentthat is commonly elevated in

babies who have cystic fibrosis.

Sweat Test

Measures sodium or chloridein persons sweat

Two samples

Ensure false-positive doesnot occur.

Not reliable on newborns.

Genetic Analysis

Newborn with signs andsymptoms may confirmdiagnosis with blood test.

Inherited disease Recommend checking

family members andfirst cousins.

Sputum Tests

Stool Exami

natio

ns

Imaging Tests:

1. X-rays

2. Computerized Tomography(CT)

3. Magnetic Resonance

Imaging (MRI)


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Treatment and Some

Common

D

rugs:

The only way to cure CF would be to use gene therapy to replace

the defective gene or to give the patient the normal form of the

protein before symptoms cause permanent damage.

Gene Therapy

Gene therapy is the use of normal DNA to "correct" forthe damaged genes that cause disease.

In the case of CF, gene therapy involves inhaling aspray that delivers normal DNA to the lungs.

The goal is to replace the defective CF gene in thelungs to cure CF or slow the progression of the disease.


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Treatment and Some

Common

D

rugs:

Treatments aimed at relieving symptoms and

complications:

Antibiotics. These drugs are used to treat and prevent lung

infections. They may be swallowed in pill form, inhaled in a mist

or delivered intravenously.

- Azithromycin

- Augmentin (Amoxicillin and clavulanate potassium).

- Inhaled TOBI- (tobramycin solution for inhalation) is a widely

used antibiotic treatment. TOBI can be effective against the

most common source of chronic lung infections, a bacterium

called Pseudomonas aeruginosa.


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Treatment and Some

Common

D

rugs:

- Cayston (aztreonam for inhalation solution) also is used to

improve respiratory symptoms in people with CF who

have Pseudomonas aeruginosa.

Mucus-thinning drugs. Drugs that reduce the stickiness of

your mucus make it easier to cough up the mucus, which

improves lung function.

- Pulmozyme - to thin mucus so people can cough it out

easier.

Bronchodilators. Medications such as Albuterol

delivered by an inhaler or a nebulizer help keep your airways

open by relaxing the muscles around your bronchial tubes.


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Treatment and Some

Common

D

rugs:

Bronchial airway drainage

Postural drainage

Oral

enzym

es a

nd b

ette

rn

utrition

High calorie diet

Special vitamins & pancreatic enzymes

Lung transplant

Pain relievers

- Ibuprofen


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Nursing Diagnosis

Ineffective airway clearance r/t tracheobronchial

secretions and obstruction.

Risk for impaired skin integrity r/t acid stools. Imbalanced nutrition: less than body requirements

r/t inability to digest food or absorb nutrients.

Risk for infection r/t chronic pulmonary disease

Interrupted family processes r/t chronic illness.


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Nursing Interventions with

ExpectedO

utcome

s:

Provide respiratory therapy

(For Ineffective airway clearance)

1.P

osition with open airway2. Chest physiotherapy:

flutter valve device provides high frequency oscillation to the

airway as they exhale through mouthpiece.

positive expiratory pressure therapy exhale through flow

resistor which provides positive expiratory pressure. This is

repeated until coughing yields expectoration of secretions. high frequency chest compression vest high frequency chest

well oscillation to increase airway velocity creating cough like

shear forces and decrease viscosity of secretions.

Expected Outcome: Child will maintain open airway,

easy work of breathing, and respiratory rate withinparameters of age.


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ExpectedO

utcome

s:

AdministerMedications1. Medicine to treat infections

-Antibiotics (such as ciprofloxacin [Cipro] and tobramycin [TOBI])

2. Medicines to open breathing tubes in the lungs or keep them open- Bronchodilators (such as albuterol or salmeterol)

-Anticholinergics (such asAtrovent)

3. Medicines to control the amount and thickness ofmucus

- DNase (such as Pulmozyme)

- Mucolytics (such as acetylcysteine)

- Saltwater solution (hypertonic saline)

4. Medicines to reduce inflammation- Nonsteroidal anti-inflammatory drugs (NSAIDs) (such as Motrin)

- Membrane stabilizers (such as cromolyn)

- Corticosteroids (such as prednisone, Medrol, orFlovent Diskus)

5. Medicines to replace theeffect ofdigestiveenzymes

- Enzyme replacement therapy (such as Creon orPancrease)


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ExpectedO

utcome

s:

Meet Nutritional Needs

(For Imbalanced Nutrition: less than body

requirements)1. Calorie counts to ensure adequate intake(may need more than 20-50%

more than RDI)

2. Assist family in choosing, protein- rich food, and other fat snacks to

optimize growth.

3. Encourage supplements.

4. Continuous monitoring including Weight, Height, BMI, Ideal Body

Weight, Skin Fold Thickness, and Upper arm circumference.

Expected Outcome: Child will maintain adequate nutritional

intake and weight gain will occur.


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ExpectedO

utcome

s:

Provide Psychosocial Support

- ForPsychological and Emotional Well-being.

- ForP

hysical andM

ental Development.

Discharge Planning and Home Teaching

- After discharge, educate the parent for:

Respiratory infections

Avoid exposure

Chest percussion & postural drainage

Diet

Community resources

Genetic counseling


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Evaluation:

Childs height and weight follow percentile growth curves; quantity of

stool decreases; signs and symptoms of vitamin deficiency are

absent.

Childs skin does not exhibit areas of erythema or ulceration; rectalprolapse is not present.

Family members state they have adequate resources to cope with

current circumstances.

Childs vital signs are stable.

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Cystic Fibrosis PPTX