About
Genetic disease that causes thick, sticky mucous to impair the function of the lungs and other organs. Typically diagnosed at birth or early childhood.
Assess
Auscultate lungs, color and consistency of sputum, ability to cough up secretions, previous medical and medication history, current disease management strategies, knowledge of illness and progression, pulse-oximetry, respiratory effort. Major diagnostic test is sweat chloride test.
Complications
Respiratory infections- treat promptly to minimize exacerbation of disease state.
Lung transplant surgery
Organ damage and failure
Death
Diagnosis
- Impaired airway clearance related to excessive, thick secretions
- Imbalanced nutrition related to increased work of breathing while eating
- Activity intolerance related to inability to breathe effectively
- Risk for infection related to excessive mucous
Goals
- The client will be able to state rationale for maintenance and prevention therapies.
- The client will be able to identify three symptoms indicating an exacerbation of the disease state.
- The client will be able to identify a support group or additional means of support to help deal with disease chronicity and debilitation.
Intervnetions
- Monitor VS, lung sounds, intake and output, and sputum color and characteristics.
- Monitor dietary intake. Teach client methods to increase nutritional intake.
- Encourage client to discuss disease process, changes in quality of life, and chronicity with health care providers, friends, family, and other persons effected by disease.
- Administer medications, as indicated.
- Consult respiratory to assist with airway clearance techniques for client.
- Teach client maintenance therapies, including chest physiotherapy, positive expiratory pressure, and an exercise program.
- Teach client sign and symptoms to report to physician that may indicate an exacerbation, an infection, or progression of the disease.
- Identify support groups, web-sites, and other useful tools for client to use.
- Discuss client wishes for treatment, encourage client to address living will and code status.
- Monitor for signs/ symptoms of complications.
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