Overview/pathophysiology

Cystic fibrosis (CF) is a chronic, progressive multisystem disease in which there is dysfunction of the exocrine (mucus-producing) glands and abnormal transport of sodium and chloride across the epithelium. This results in abnormally thick secretions, causing obstruction of the small passageways of many organs. CF is an autosomal recessive hereditary disease with more than 1400 gene mutations (Cystic Fibrosis Foundation [CFF], 2010). This is why there is such a wide variation in clinical manifestations.

In the recent past, CF was described as the most common lethal genetic illness in white children. The median life expectancy has improved dramatically from 7.5 years in 1966 (Hockenberry, 2007) to 37.4 years in 2008 (CFF, 2010). The median life expectancy has increased 5.4 years over the past 9 years because of specialized care and new CF therapies.

About 30,000 people in the United States (70,000 worldwide) have CF, and more than 45% are 18 yr or older. About 1000 new cases are diagnosed each year with over 70% diagnosed by age two. More than 10 million Americans are asymptomatic genetic carriers, which is one in every 31 Americans. CF is most often seen in Caucasians but it can affect all races (CFF, 2010).

Health care setting

Primary care, with possible hospitalization for CF exacerbation or other complications

Assessment

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

Diagnostic tests

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

Newborn screening (NBS) panel:

CF has been added to the panel in all 50 states as of January 1, 2010 and is done at the same time as phenylketonuria and other screening tests. The two tiered process follows:

1. Immunoreactive trypsinogen test:

Enables early detection of CF and is done several days after birth. Though many newborns have a positive NBS for CF, less than 5% of all newborns have CF. If positive, referral should be made to a CF-accredited care center for further evaluation. It is confirmed by a “sweat test” or mutation analysis (i.e., genetic testing). The combination of these two tests is sensitive 90%-100% of the time.

2. Pilocarpine iontophoresis (quantitative sweat chloride test or “sweat test”):

Production of sweat is stimulated with a special device, and the sweat is collected and measured. Diagnosis is made with sodium and chloride levels greater than 60 mEq/L (levels of 40-60 mEq/L are considered suggestive and should be repeated) and the presence of clinical symptoms or a family history of CF. Sweat test is not usually done before 4-6 wk of age because the infant has decreased sweat production until then.

Or

3. Polymerase chain reaction (pcr) for gene mutations (genetic testing):

Helps determine if child is a carrier or has CF.

Chest x-ray examination:

Shows characteristic patchy atelectasis and chronic obstructive emphysema.

Pulmonary function tests (after 5-6 yr old):

Assess degree of pulmonary disease and response to therapy and help distinguish between restrictive and obstructive pulmonary disease. In the presence of CF, the test will show decreased vital capacity (VC) and tidal volume, increased airway resistance, increased residual volume, and decreased forced expiratory volume in 1 sec (FEV₁) and FEV₁/VC ratio.

Pulse oximetry:

Reveals decreased oxygen saturation.

Complete blood count:

Increased white blood cells with increased neutrophils on differential count will be present with infection.

Sputum culture:

For identification of infective organisms and sensitivity of these organisms. Many resistant organisms develop because of the frequency of respiratory infections.

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):
– Assess HR, RR, breath sounds, and O2 saturation before nebulization and after chest physiotherapy.	Assessment before and after treatment monitors effectiveness of treatment.
– Position child in an upright sitting position, ensuring that he or she does not slouch.	This position facilitates maximum inhalation of medication and improves effectiveness of cough to clear secretions out of airways.
– Administer nebulization (albuterol) as prescribed 1 hr before or 2 hr after meals.	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.
– Perform chest physiotherapy after nebulizer treatment. Examples follow.	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 and postural drainage for 20-30 min	Chest percussion loosens secretions, and postural drainage facilitates drainage of secretions so that they can be expectorated.
– Mucus clearance device (e.g., flutter valve) used for 5-15 min	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.
– Airway clearance system (e.g., the Vest)	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.
– Suction as necessary.	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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