Cystic Fibrosis

Cystic Fibrosis

1. Ineffective airway clearance related to infection as evident by infection

   • Demonstrate absence or reduction in congestion with improvement in breath sounds, improved oxygen exchange and increase in pulse oximetry by third day of hospital admission.
     • To promote Wellness
       • Assess parents knowledge of contributing causes, treatment plan, specific medications and therapeutic procedures.
       • Educate the importance of raising and expectorating secretions versus swallowing them; need to report change in color and amount.
       • Will demonstrate in performing specific airway clearance techniques; forced expiratory breathing, respiratory muscle strength training, chest percussion or use of a vest.
       • Encourage opportunity for rest.
       • Refer family to appropriate support group.
       • Determine the need for specialized equipment.
     • Assess for change in patient condition
       • Auscultate breath sounds and assess air movement.
       • Monitor vital signs, note any changes in blood pressure and heart rate.
       • Observe for signs of respiratory depress such as increased respiratory rate, restlessness, anxiety or use of accessory muscles for breathing.
       • Document response to drug therapy.
       • Obtain sputum specimen
       • Monitor with serial chest x-rays, ABG's, and pulse oximetry reading.
     • Mobilize secretions by performing postural drainage techniques.
       • Encourage coughing and deep breathing exercises.
       • Increase fluid intake
       • Position appropriately with the head of the bed elevated or side lying position.
     • maintain a patent airway.
       • Identify risk that patient has excessive/abnormal mucus production.
       • Assess for ability to protect her own airway.
       • Monitor respirations and breath sounds
       • Evaluate the cough/gag reflex, amount and type of secretions and swallowing ability.
       • Suction nose, mouth and trachea as indicated.
       • Instruct and review breathing exercises, effective coughing and use of adjunct devices
       • Elevate the head of the bed, encourage ambulation, change position every 2 hours.
       • Keep environment allergen free.
       • Administer prescribed neb treatments.
       • Assist with PD and vest CPT

2. Impaired gas exchange related to pneumonia, fluid buildup in the lung tissue, abnormal mucus production as evident by low oxygen saturation.

   • Demonstrate improved ventilation and adequate oxygenation.
   • Verbalize understanding of causative factors and appropriate interventions.
   • Participate in treatment regimen with breathing exercises, effective coughing, and use of oxygen.
   • Assess the causative/contributing factors.
     • Note the presence of gas exchange problems, anxiety, fear, decreased lung compliance, excessive or thick secretions, infection, neuromuscular impairment.
   • Evaluate the degree of compromise.
     • Note respiratory rate, depth and use of accessory muscles,
     • Auscultate breath sounds,
     • Note character and effectiveness of cough.
     • Note reports of restlessness, headache, energy level and activity tolerance.
     • Monitor vital signs and cardiac rhythm.
     • Evaluate pulse oximetry and capnography.
     • Review lab results.
   • Administer oxygen to maintain oxygen saturations above 90%

3. Infection related to: chronic illness, alteration in peristalsis, decrease in ciliary action. As evident by: increase in white blood cell count, fever and consolidation on x-ray.

   • Parents will verbalize understanding of individual causative or risk factors prior to hospital discharge
   • Parents will identify interventions to prevent or reduce risk of infection prior to hospital discharge.
   • Parents will identify techniques and lifestyle changes to promote a safe environment by day 2 of hospital stay.
   • Be afebrile by second day of hospital stay.
   • Assess for causative/contributing factors to infection.
     • Note risk factors for disease such as chronic illness, certain treatment settings or modalities, lifestyle, nutritional status, medications or environmental exposure.
     • Observe for changes in skin color and warmth at IV site.
     • Obtain appropriate fluid specimen for testing.
   • Reduce/correct existing risk factors.
     • Practice and emphasize constant and proper hand hygiene by all caregivers between therapies and clients.
     • Wear gloves when appropriate to minimize contamination of hands. Wash hands after glove removal.
       • Instruct family members to wash hands as well.
     • Provide a clean and well-ventilated environment.
     • Monitor the patient's visitors and caregivers for respiratory illness.
     • Provide for isolation if indicated.
     • Emphasize the use of PPE by staff and family members/visitors.
     • Recommend for routine showers and bathing to help reduce bacterial colonization.
     • Maintain sterile technique for invasive procedures.
     • Choose a proper vascular access device based on anticipated treatment/duration and solution/medication
     • Encourage ambulation, deep breathing, coughing and changing positions.
     • Administer/monitor medication regimen, antibiotics and note the response to determine the effectiveness of therapy or presence of side effects.
     • Administer immunizations if indicated.
     • Encourage parents to keep child away from settings such as daycare until fever free for 24 hours.
     • Maintain adequate hydration.
   • Promote wellness.
     • Review nutritional needs, exercise program and need for rest.
     • Educate parents on techniques to protect the integrity of the skin, care for lesions and prevention of spread of infection.
     • Emphasize the necessity of taking antibiotics as directed. Discontinuing antibiotics too soon can result in return of infection and potentiation of drug-resistant strains.
     • Educate on the importance of not taking 'left over' antibiotics..
     • Discuss precautions if planning travel and need for immunizations and immunizations for other children.

4. Imbalanced nutrition, less than body requirements related to inability to ingest or digest food, inability to absorb nutrients. As evident by low weight per her age.

   • Patient will state foods of interest during hospital stay and eat at least 3 meals with 2 snacks throughout a 24 hour period.
   • Patient will state no complaints of abdominal pain during hospital stay.
   • Patient will not lose weight during hospital stay.
   • Parents will verbalize understanding of the causative factors and necessary interventions to maintain adequate weight prior to hospital discharge.
   • Patient's family will demonstrate appropriate behaviors during hospital stay to maintain adequate weight.
   • Assess causative/contributing factors.
     • Identify the client at risk related to chronic illness, malabsorption syndromes, cystic fibrosis, pancreatic disease
     • Assess for pediatric concerns. Change in nutritional needs related to growth phase, malabsorption problems, diabetes, chronic infection.
     • Determine child's ability to chew, swallow and taste food.
     • Assess teeth and gums for oral health.
     • Determine the parent's understanding of the nutritional requirements.
     • Assess for lifestyle factors that may affect nutrition. Amount of money to purchase food, socioeconomic status, proximity of grocery store, available space for storing food.
     • Explore specific eating habits, the meaning of food and individual food preferences.
       • Need to take pancreatic enzymes with each meal and snacks.
     • Assess for drug interactions, disease effects, allergies, diuretics that may affect appetite, food intake and absorption.
     • Evaluate for cultural, ethnic or religious desires and influences.
     • Review usual activities and exercise program noting repetitive activities.
   • Evaluate the degree of nutritional deficit.
     • Assess weight, measure muscle mass, or calculate body fat.
     • Observe for absence of subcutaneous fat, muscle wasting, loss of hair, fissuring of nails, delayed healing.
     • Auscultate bowel sounds.
     • Assist in nutritional status using screening tools.
     • Review laboratory data: serum albumin/prealbumin, transferrin, amino acids, iron, BUN, glucose, liver function, electrolytes and total lymphocyte count
   • Establish a nutritional plan.
     • Note age, body build, strength, activity level and current condition.
     • Evaluate total daily food intake
     • Collaborate with dietician to set nutritional goals.
     • Increase fat intake.
     • Educate on importance of high calorie diet.
     • Administer pancreatic enzymes with meals or snacks.