Cystic fibrosis

81 Cystic fibrosis






Assessment


Initially involves overall appraisal, including monitoring general activity, physical findings, nutritional status, and chest x-ray examination.




Signs and symptoms:


Vary widely, as does the severity of involvement of specific organ systems. Patients tend to have periods without acute symptoms and then periods with acute exacerbation of symptoms. The first clinical manifestation may be meconium ileus in a newborn, or the patient may not have symptoms for months or years.


Most of the usual symptoms are caused by the following:









Diagnostic tests


CF has been called the “great imitator” because signs of chronic respiratory infection and FTT are symptoms of many other childhood conditions.










DNA analysis of chorionic villi or amniotic fluid:


Can establish prenatal diagnosis.





Nursing diagnosis:


Ineffective airway clearance

related to thick, tenacious mucus in airways


Desired Outcome: Immediately following treatment/interventions, child expectorates mucus and exhibits improved airway clearance as evidenced by improved breath sounds and heart rate (HR) and respiratory rate (RR) within child’s normal limits.











































ASSESSMENT/INTERVENTIONS RATIONALES
Assess HR, RR, and breath sounds. This assessment establishes baseline data from which to compare later findings. With ineffective airway clearance, the child will have increased HR and RR. Breath sounds may be decreased with little air movement because of the blocked airway, or adventitious sounds may be increased because of mucus in the airway.
Assist child with sputum expectoration (may be done by respiratory therapist):  

Assessment before and after treatment monitors effectiveness of treatment.

This position facilitates maximum inhalation of medication and improves effectiveness of cough to clear secretions out of airways.

This treatment opens bronchi and loosens secretions. It usually causes considerable coughing followed by expectoration of mucus and sometimes vomiting from excessive coughing. Scheduling in relation to meals is essential to provide maximum benefit of treatment and prevent interference with nutrient ingestion. Treatment before breakfast helps loosen secretions that built up overnight. Treatment before bedtime helps clear secretions that would otherwise provide a medium for bacterial growth.

This helps to loosen secretions, which will facilitate their expectoration. This treatment is performed at least 2-4 times/day for maintenance or routine daily care. Method used depends on age of child, effectiveness of technique, child’s/parent’s ability to perform/tolerate technique, and preference of child/parent.

Chest percussion loosens secretions, and postural drainage facilitates drainage of secretions so that they can be expectorated.

This handheld pipelike device has a plastic mouthpiece on one end that child breathes into. On the other end of the pipe a stainless steel ball rests inside a plastic circular cone. Exhaling into the device vibrates the airways, thereby loosening mucus from the airway walls and accelerating airflow, which facilitates upward movement of mucus so that it can be more readily cleared. This device is very effective and gives child control because it can be used without assistance of others.

This inflatable vest fits like a life jacket and is connected by tubes to a generator. The vest inflates and deflates rapidly, applying gentle pressure to the chest. It provides high frequency chest wall oscillation to help loosen secretions and increase mucus expectoration.

For infants/young children or if there is a large volume of mucus, assistance may be needed to clear secretions from the airway. However, children usually cough sufficiently after nebulizer treatment and chest physiotherapy to clear secretions independently.
Ensure that child is receiving at least maintenance fluids. Hydration thins and loosens secretions for easier expectoration.
Administer dornase alfa (Pulmozyme) as prescribed. This medication thins mucus, which will facilitate expectoration.




Nursing diagnosis:


Impaired gas exchange

related to airway obstruction occurring with air trapping in alveoli and airways narrowed by tenacious mucus


Desired Outcome: Within 2 hr following treatment/intervention, child has adequate gas exchange as evidenced by O2 saturation greater than 92% (or consistent with child’s baseline).


































ASSESSMENT/INTERVENTIONS RATIONALES
Along with vital signs, assess respiratory status q2-4h, or more frequently as indicated by child’s condition. Increased HR and RR would occur with impaired gas exchange, as would chest retractions, increased work of breathing (WOB), nasal flaring, and use of accessory muscles of respiration. These are signs of respiratory distress necessitating prompt intervention/treatment.
Ensure continuous monitoring of pulse oximetry readings; report low value (usually 92% or lower). Decreased O2 saturation can indicate need for initiation of/increased O2.
Monitor for behavioral indicators of hypoxia. Restlessness, mood changes, and/or change in level of consciousness are early signs of O2 deficiency.
Be alert to changes in child’s skin color. Cyanosis of the lips and nail beds is a late indicator of hypoxia and a signal of the need for prompt treatment/intervention.
Position child in high Fowler’s position and/or leaning forward. These positions promote comfort and optimal gas exchange by enabling maximal chest expansion.
Deliver O2 along with humidity via most appropriate delivery system and at rate prescribed. The child’s developmental age helps determine the most effective delivery system and flow rate (e.g., nasal cannula for infants with liter flow rate less than 4). Humidity use replaces convective losses of moisture.
Monitor child on O2 delivery closely. O2-induced CO2 narcosis is a hazard of O2 therapy in the child with chronic pulmonary disease. If O2 saturation is consistently greater than 96%, for example, it is likely that the flow rate can be decreased slowly by small increments.
Encourage games or physical exercise appropriate to child’s condition (e.g., blowing bubbles or walking) but avoid overexertion. Breathing more deeply facilitates clearing of mucus and improves oxygenation.
Provide neutral thermal environment for child. This is a room temperature in which the body does not have to use as much energy to stay warm or cool off, thereby enabling child to use energy to grow or heal. With decreased energy demands, more O2 is available to ensure these needs are met.
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Jul 18, 2016 | Posted by in NURSING | Comments Off on Cystic fibrosis

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