Primary Nursing Diagnosis

Ineffective airway clearance related to excess tenacious mucus

OUTCOMES. Respiratory status: Airway patency; Respiratory status: Gas exchange; Respiratory status: Ventilation; Infection control

INTERVENTIONS. Chest physiotherapy; Positioning; Airway management; Surveillance


The major goals of treatment are to improve pulmonary, GI, and pancreatic status. These goals are achieved through a combination of medications, nutrition, and exercise regimens. If antibiotics are given to prevent and treat pneumonia, the physician and pharmacist monitor therapeutic blood levels of the antibiotics to determine the peak and trough levels. To help prevent the recurrence of pneumonia, chest physiotherapy (CPT) is performed in the home or hospital four times a day before meals to avoid emesis or after an aerosol treatment. A ThAIRapy vest, a device that provides high-frequency chest wall oscillations to loosen secretions, may also be used.

Maintain calorie counts on daily meal plans; supplement nutritional needs with high-calorie feedings. A patient may also have nasogastric feedings to which pancreatic enzymes are added to ensure the digestion and absorption of fats, protein, and carbohydrates. The physician may also prescribe total parenteral nutrition and fat-soluble vitamins (A, D, E, and K).

Regular exercise, including mobility and muscle-strengthening exercises should be encouraged on a regular basis. Exercise helps maintain physical wellness and supplements the patient's airway clearance strategies by helping to loosen pulmonary secretions.

Some patients develop right-sided heart failure, and if this occurs, most of them die within a year. They may require the use of home portable oxygen therapy and receive digoxin and/or diuretics. As the disease progresses toward the terminal phase, hemoptysis is present and cyanosis is markedly apparent.

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