cystic fibrosis pptx
TRANSCRIPT
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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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Nursing Interventions with
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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Nursing Interventions with
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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Nursing Interventions with
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.