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 persons
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 parents 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/imagepages
/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