CYSTIC FIBROSIS
LAUREN BURNETT
NUR3215 EBP
OBJECTIVES
By completion of this presentation, learners should be
able to:
• Describe the effects Cystic Fibrosis has on the body
• Generate a nursing plan for patients with Cystic
Fibrosis
• Differentiate between different manifestations of
cystic fibrosis
• Compare and contrast between different
treatment options for Cystic Fibrosis
WHAT IS IT?
• A genetic disorder of the
exocrine glands that
results from defective
sodium and chloride
transport
• Primarily affects
respiratory system and GI
tract
EPIDEMIOLOGY
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30,000 people in the US
70,000 people worldwide
100 new cases a year
Median age of diagnosis – 6 months
Median age of survival – mid 30’s
Primarily affects Caucasians
ETIOLOGY
• Mutation in the Cystic Fibrosis Transmembrane
Conductive Regulator (CFTR) gene
• CFTR gene is responsible for production of a protein
that regulates how salt and water are carried
across membranes
PATHOPHYSIOLOGY
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Alteration in chloride, salt, and water transport
Goblet cells increase in size and number
Mucus becomes excessively thick and sticky
Mucus clogs the airways (and other organ systems)
PATHOPHYSIOLOGY
• Bacteria grow inside mucus
• WBC responds to infection, further aggravating airway
• Frequent, persistent infections occur, damaging the
lungs
• Respiratory failure and possible death
MANIFESTATIONS
• Respiratory manifestations are most common
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Persistent cough (maybe with purulent, green sputum)
Multiple respiratory infections
Possible wheezing
Bronchiectasis
Hemoptysis
Tachypnea
Shortness of breath
Loss of appetite/weight loss
SYSTEMIC MANIFESTATIONS
• Gastrointestinal
• Cramps, pain, pancreatic abnormalities
• Endocrine
• Glucose intolerance, polydipsia, polyphasia, polyuria
• Reproductive
• Male sterility, pregnancy complications, delayed sexual
development
DIAGNOSIS
• Newborn testing – heel stick
• Based on clinical symptoms
• Verified by diagnostic tests
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Sweat Chloride test
CXR
Pancreatic enzymes
DNA analysis to detect CFTR
gene mutation
HYPERINFLATION WITH
BRONCHIECTASIS
MANAGEMENT
• Overall goal –
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Control/prevent infection
Maintain clear airways
Improve nutrition
Psychosocial support
Improve quality of life
• There is NO cure to treat all patients at this time
• Early detection and diagnosis is crucial to delay
progression
NURSING MANAGEMENT
• Assessment –
• History, symptoms, activity, nutrition intake, medication use
• Diagnosis –
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Ineffective airway clearance
Ineffective breathing pattern
Risk for infection
Imbalanced nutrition
• Goals of Care –
• Maintain adequate airway
• Reducing risk factors for infection
• Patient Education
MANAGEMENT
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Prophylaxis against infection
Early antibiotic therapy when symptoms arise
Chest physiotherapy
Surgery
• Removal of nasal polyps
• Lung lobectomy
• Lung transplant
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Bronchodilators
Tobramycin
Azithromycin
DNase
IS THERE A CURE?
• Ivacaftor (Kalydeco)
A CFTR potentiator
FDA approved for treatment of CF
Only for 1 mutation type (there are over 1,500)
Reduces viscosity of mucus
Improves respiratory function and reduces risk of
exacerbations
• Significant reduction in sweat chloride test
• 10 adverse effects on label
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DRY POWDER TOBRAMYCIN
Aminoglycoside antibiotic used for years as a nebulizer
Very effective against Pseudomonas aeruginosa
Now available as a dry powder to be inhaled (TIP)
Before TIP (inhaled version), nebulizers were only option due
to high dosage required
• Higher incidence of cough
• Faster administration time, more convenient and portable
• Greater patient satisfaction than nebulizer treatments
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SO WHAT?
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Patient Education
TB screening
Immunizations
Advocate for the patient
NCLEX QUESTION
A patient comes into the ER complaining of a chronic
cough. Upon assessment, the nurse learns this patient
has a history of CF and is also experiencing shortness
of breath . Which manifestation of CF should the
nurse suspect?
A. Respiratory infection
B. Hemoptysis
C. Bronchiectasis
D. Emerging nasal polyps
NCLEX QUESTION
A patient comes into the ER complaining of a chronic
cough. Upon assessment, the nurse learns this patient
has a history of CF and is also experiencing shortness
of breath . Which manifestation of CF should the
nurse suspect?
A. Respiratory infection
B. Hemoptysis
C. Bronchiectasis
D. Emerging nasal polyps
NCLEX QUESTION
The nurse is teaching a mother of a child with CF
about dietary improvements to make. Which type of
diet would be an appropriate suggestion?
A. High-calorie, High-protein diet
B. Low-calorie, High-protein diet
C. High-carb, High-fiber diet
D. Low-protein, Low-fiber diet
NCLEX QUESTION
The nurse is teaching a mother of a child with CF
about dietary improvements to make. Which type of
diet would be an appropriate suggestion?
A. High-calorie, High-protein diet
B. Low-calorie, High-protein diet
C. High-carb, High-fiber diet
D. Low-protein, Low-fiber diet
REFERENCES
• Brashers, V. L. (2012). Alterations in Pulmonary Function. In S. E. Huether, & K. L. McCance, Understanding
Pathophysiology (pp. 685). St. Louis, Missouri: Elsevier.
• Brashers, V. L., & Gott, K. K. (2012). Alterations in Pulmonary Function in Children. In S. E. Huether, & K. L.
McCance, Understanding Pathophysiology (pp. 718-720). St. Louis, Missouri: Elsevier.
• Cashion, A., Graff, C., Graves, R., & Wraa, C. (2014). Genetics. In R. Holleran, K. S. Osborn, A. B. Watson, & C.
E. Wraa, Medical-Surgical Nursing: Preparation for Practice (pp. 142). Upper Saddle River, New Jersey: Pearson.
• Chiu, H., Song, j. C., & Yoon, J. (2012). Ivacaftor: A New Emerging Treatment Option in the Management of
Cystic Fibrosis. Formulary, 47, 132-134, 139-140. Retrieved from
http://eds.a.ebscohost.com/eds/pdfviewer/pdfviewer?sid=d06260d9-e989-4f9d-8070ac489c1bfb9f%40sessionmgr4003&vid=1&hid=4203
• McKeage, K. (2013). Tobramycin Inhalation Powder: A Review of Its Use in the Treatment of Chronic
Pseudomonas aeruginosa Infection in Patients with Cystic Fibrosis. Drugs, 73, 1815-1827. doi:
10.1007/s40265-013-0141-0
• Rhoades, C., & Wraa, C. (2014). Caring for the Patient with Lower Airway Disorders. In R. Holleran, K. S.
Osborn, A. B. Watson, & C. E. Wraa, Medical-Surgical Nursing: Preparation for Practice (pp. 768-769). Upper
Saddle River, New Jersey: Pearson.