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Cystic Fibrosis
Gina Brandl, RN BSN
Nursing Instructor, Pediatrics
Cystic Fibrosis –
Overview of Disease
• Cystic fibrosis (CF) - inherited autosomal
recessive disorder in children
• Most common cause of chronic lung disease in
children – 30,000 children & adults in US
• Incidence
– Annually 1,000 children diagnosed
– 70% of patients are diagnosed by age 2
– 40% of CF population is age 18 and older
• Median life span
Etiology
• Genetically
transmitted disease
– Genes (DNA)
– From parents
• Gene located on 7th
chromosome
– 2 copies of genes needed
to inherit disease
– Carrier states
(National Institutes of Health, n.d.)
Pathophysiology
• Defective gene
– Related to protein
involved in chloride ion
transport
• Body produces thick,
sticky mucus
– Clogs the lungs
• Stagnant mucus
– Obstructs the pancreas
• Malabsorption &
malnutrition
(MedlinePlus, 2006)
Symptoms
• Symptoms may include
– Meconium ileus
• Appears at birth
– Salty-tasting skin
• When newborn is kissed
– Steatorrhea
• Greasy, bulky and foul smelling
– Poor growth/weight gain in spite of good appetite
– Chronic coughing, at times with phlegm
– Frequent lung infections
Diagnostic Tests
• Sweat Test
– Measures sodium or
chloride in person’s
sweat
– Two samples
• Ensure falsepositive does not
occur
– Not reliable on
newborns
• Genetic Analysis
– Newborn with signs
and symptoms may
confirm diagnosis
with blood test.
– Inherited disease
• Recommend
checking family
members and first
cousins
Common Nursing
Diagnoses
• Ineffective airway clearance r/t tracheobronchial
secretions and obstruction
• 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
Planning & Interventions
•
•
•
•
•
Provide respiratory therapy
Administer medications
Meet nutritional needs
Provide psychosocial support
Discharge planning and home teaching
Treatments
• Aimed at relieving symptoms and
complications
– Antibiotics
• Aerosolized
– Mucus-thinning drugs
• Thins secretions
• Easier to cough up
– Bronchodilators
• Relaxes smooth muscles in the airways
Treatments
– Bronchial airway drainage
• Postural drainage
– Oral enzymes and better nutrition
• High calorie diet
• Special vitamins & pancreatic enzymes
– Lung transplant
– Pain relievers
• Ibuprofen
Expected Outcomes
• The expected outcomes of nursing care include
– Family and/or child demonstrate proficiency in
• Providing pulmonary care
• Reducing pulmonary infections
• Developing a schedule for pulmonary cares that fits into
family needs
– Adequate calories and pancreatic enzymes are consumed
to support growth of the child to stay within
developmental weight ranges
Parent and Child
Education
• Respiratory
infections
– Avoid exposure
• Chest percussion &
postural drainage
• Diet
• Community
resources
• Genetic counseling
• Written
information
– Home care
• Support services
Critical Thinking
Exercise
• A 9 month-old infant has a pattern of recurrent
hospitalizations of respiratory difficulty,
recurrent bronchitis with thick mucus production
and poor weight gain with thin extremities. His
history revealed a meconium ileus at birth.
• VS: 101.8 (F)-144-54. A sweat chloride test
confirms a diagnosis of cystic fibrosis. The
physician suggests genetic testing.
Critical Thinking
Exercise
1.
2.
3.
4.
5.
The family asks why genetic testing is needed. How should the
nurse respond to the parent’s question?
Describe the significance of the sweat chloride test in
relation to the disease process of cystic fibrosis.
What are the issues related to having a child with a chronic
respiratory illness?
The family asks why their child has not been gaining weight.
How would you respond to this question?
Diagnosed during infancy or early childhood, what are the
three major presentations of a child with cystic fibrosis to a
primary care provider?
References
• Cystic Fibrosis Foundation. (2007). About cystic
fibrosis: Frequently asked questions. Retrieved
November 18, 2007, from the website
http://www.cff.org/AboutCF/Faqs/
• Mayo Clinic. (2007). Cystic fibrosis – diseases and
conditions. Retrieved November 23, 2007, from
the website
http://www.mayoclinic.com/health/cysticfibrosis/DS00287/DSECTION=6
References
• MedlinePlus. (2006). Cystic fibrosis. Retrieved
November 18, 2007, from the website
https://www.nlm.nih.gov/medlineplus/ency/imagep
ages/18135.htm
• National Institutes of Health. (2006). What
causes cystic fibrosis?. Retrieved November 21,
2007, from the website
www.nhlbi.nih.gov/.../Diseases/cf/cf_causes.html