May, a mother of a 1-month old baby, noticed that her baby has a foul-smelling, bulky stool. Later on, the baby developed a nonproductive cough, and though May has already seek the pediatrician’s advice, the cough continued for 2 months more. The pediatrician ran some tests on the infant, and diagnosed her with cystic fibrosis. Show
What is Cystic Fibrosis?
PathophysiologyCystic fibrosis is caused by defects in the cystic fibrosis gene. https://www.youtube.com/watch?v=BhFpFiZumS0
Statistics and IncidencesCystic fibrosis is an autosomal-recessive disease.
CausesCystic fibrosis is an autosomal recessive disease caused by defects in the CFTR gene, which encodes for a protein that functions as a chloride channel, and also regulates the flow of other ions across the apical surface of epithelial cells.
Clinical ManifestationsThe age at diagnosis varies widely, however, as do the clinical presentation, severity of symptoms, and rate of disease progression in the organs involved. Clinical manifestations vary with the patient’s age at presentation.
Assessment and Diagnostic FindingsThe diagnosis of cystic fibrosis (CF) is based on typical pulmonary manifestations, GI tract manifestations, a family history, and positive sweat test results.
Medical ManagementAs a result of the complex and multisystemic involvement of cystic fibrosis (CF) and the need for care by specialists, treatment and follow-up care at specialty centers with multidisciplinary care teams (ie, cystic fibrosis centers) is recommended.
Pharmacologic ManagementMedications used to treat patients with cystic fibrosis may include pancreatic enzyme supplements, multivitamins (particularly fat-soluble vitamins), mucolytics, antibiotics (including inhaled, oral, or parenteral), bronchodilators, anti-inflammatory agents, and CFTR potentiators.
Nursing ManagementManagement of a child with cystic fibrosis should be holistic and meticulous. Nursing AssessmentThe collection of data on the child with CF varies depending on the child’s age and the circumstances of the admission.
Nursing DiagnosisBased on the assessment data, the major nursing diagnoses are:
Nursing Care Planning and GoalsMain Article: 5 Cystic Fibrosis Nursing Care Plans The major goals for the child include:
Nursing InterventionsImplementation of the following goals include these interventions:
EvaluationGoals are met as evidenced by:
Documentation and GuidelinesDocumentation for a child with CF includes:
Practice Quiz: Cystic FibrosisHere’s a 5-item quiz about Cystic Fibrosis. Please visit our nursing test bank page for more NCLEX practice questions. 1. Betty is a 9-year-old girl diagnosed with cystic fibrosis. Which of the following must Nurse Archie keep in mind when developing a care plan for the child? A. Pulmonary secretions are abnormally thick. 1. Answer: A. Pulmonary secretions are abnormally thick.
2. A 16-year old patient with cystic fibrosis is admitted with increased shortness of breath and possible pneumonia. Which nursing activity is most important to include in the patient’s care? A. Perform postural drainage and chest physiotherapy every 4 hours. 2. Answer: A. Perform postural drainage and chest physiotherapy every 4 hours.
3. The mother of a child with cystic fibrosis tells the nurse that her child makes “snoring” sounds when breathing. The nurse is aware that many children with cystic fibrosis have: A. Choanal atresia. 3. Answer: B. Nasal polyps.
4. The nurse is teaching the mother of a child with cystic fibrosis how to do postural drainage. The nurse should tell the mother to: A. Use the heel of her hand during percussion. 4. Answer: D. Use cupped hands during percussion.
5. Which finding is the best indication that a client with ineffective airway clearance needs suctioning? A. Oxygen saturation. 5. Answer: C. Breath sounds.
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