Prevention: Small children should not be allowed access to small objects that they might place in their nose or mouth. Anticipatory guidance for parents of small children is essential. Nurses are in a position to teach prevention in a variety of settings (see Community Focus box). They can educate parents singly or in groups about hazards of aspiration in relation to the developmental level of their children and encourage them to teach their children safety. Caution parents about behaviors that their children might imitate (e.g., holding foreign objects, such as pins, nails, and toothpicks, in their lips or mouth). (Chapters 12 and 14 discuss prevention based on the child’s age.)

Pathogenesis: The severity of the lung injury depends on the pH of the aspirated material, the presence of bacteria, and the volatility and viscosity of the substance. Irritation from aspiration during swallowing, vomiting, or gastric lavage may also cause pulmonary involvement. Pathologic changes include signs of inflammation (edema, hyperemia, infiltration of polymorphonuclear cells); vascular thrombosis and hemorrhage; and necrosis of bronchial, bronchiolar, and alveolar tissues. Other reactions are bronchospasm, atelectasis, emphysema, pulmonary hemorrhage, necrosis, surfactant impairment, and pulmonary edema. Even in small amounts, hydrocarbons spread over the surface of tissues and the lungs and interfere with gas exchange. Aspiration of inert fluids may not produce a chemical or bacterial pneumonia, but these fluids can decrease lung compliance and cause hypoxemia.

Clinical Manifestations: Acid aspiration may produce immediate pulmonary symptoms that worsen over the first 24 hours. Coughing and vomiting, which occur almost immediately after ingestion, contribute to the aspiration. Central nervous system symptoms include agitation, restlessness, confusion, drowsiness, and coma. The temperature is elevated (37.8° to 40° C [100° to 104° F]). (See Ingestion of Injurious Agents, Chapter 16.)

After swallowing, coughing, and choking, the child becomes short of breath, and older children complain of dyspnea. There are varying degrees of cyanosis, tachycardia, tachypnea, nasal flaring, and retractions. Intercostal retractions, grunting, cough, and fever may appear within 30 minutes or be delayed for a few hours. Localized areas of dullness are felt on percussion, and moderately intense wheezes and crackles are heard. Severe injury causes hemoptysis, pulmonary edema, severe cyanosis, and death within 24 hours of aspiration.

Therapeutic Management: Inducing the child to vomit is contraindicated because of the renewed danger of aspiration. Hydrocarbons are readily absorbed by the gastrointestinal tract and excreted by the lungs. Bronchitis or pneumonia usually develops early (within the first 24 hours) but may be delayed. Recovery from pulmonary involvement occurs in most instances despite a severe clinical course. Death is generally the result of hepatic failure complicated by pulmonary factors. Treatment is the same as for any lower respiratory tract inflammation and consists of high humidity, supplemental oxygen, hydration, and treatment of any secondary infection. Endotracheal intubation may be required if the child develops respiratory failure.

Prognosis: In spite of advances in understanding and treating ARDS-ALI, childhood mortality rates of 18% to 49% have been reported (Albuali, Singh, Fraser, et al, 2007; Randolph, 2009), and prolonged respiratory failure requiring an average of 10 to 16 days on mechanical ventilation is common (Randolph, 2009). The precipitating disorder influences the outcome. The worst prognosis is associated with profound hypoxemia, uncontrolled sepsis, bone marrow transplantation, cancer, and multisystem involvement with hepatic failure. Children who recover may have persistent cough and exertional dyspnea.

Heat Injury: Heat causes thermal injury to the upper airway, but because air has low specific heat, the injury goes no farther than the upper airway. Reflex closure of the glottis prevents injury to the lower airway. Heat may reach the middle airway occasionally, but it rarely penetrates to the lungs.

Chemical Injury: The combustion of materials such as clothing, furniture, and floor coverings can generate a wide variety of gases. Acids, alkalis, and their precursors in smoke can produce chemical burns. These substances can be carried deep into the respiratory tract, including the lower respiratory tract, in the form of insoluble gases. Soluble gases tend to dissolve in the upper respiratory tract.

Synthetic materials are especially toxic, producing gases such as oxides of sulfur and nitrogen, acetaldehyde, formaldehyde, hydrocyanic acid, and chlorine. Heated plastics are the source of extremely toxic vapors, including chlorine and hydrochloric acid from polyvinylchloride, and hydrocarbons, aldehydes, ketones, and acids from polyethylene. Irritant gases such as nitrous oxide or carbon dioxide combine with water in the lungs to form corrosive acids. Aldehydes cause denaturation of proteins, cellular damage, and edema of pulmonary tissues. Chemical burns to the airways are similar to burns on the skin, except they are painless because the tracheobronchial tree is relatively insensitive to pain.

Inhalation of small amounts of noxious irritants produces alveolar and bronchiolar damage that can lead to obstructive bronchiolitis. Severe exposure causes further injury, including alveolocapillary damage with hemorrhage, necrotizing bronchiolitis, and inhibited secretion of surfactant, with resultant atelectasis.

Systemic Injury: Gases that are nontoxic to the airways (e.g., carbon monoxide, hydrogen cyanide) can cause injury and death by interfering with or inhibiting cellular respiration. Carbon monoxide is a colorless, odorless gas with an affinity for hemoglobin 230 times greater than that of oxygen. When carbon monoxide enters the bloodstream, it readily binds with hemoglobin to form carboxyhemoglobin (COHb). Because carbon monoxide combines more readily and is released less readily than oxygen, very low levels of tissue oxygen must be reached before appreciable amounts of oxygen are released from the hemoglobin. Therefore tissue hypoxia reaches dangerous levels before oxygen is available to meet tissue needs.

Accidental carbon monoxide poisoning is most often a result of exposure to fumes from heaters or smoke from structural fires, although poorly ventilated recreational vehicles with improperly operated or maintained gas lamps or stoves and cooking in underventilated areas with charcoal grills or hibachis are also frequent causes. Intentional carbon monoxide poisoning may occur in an attempted suicide in the vehicle parked in a closed garage for a long period. Accidental carbon monoxide poisoning may also occur in a vehicle with inadequate vented exhaust which leaks into the vehicle’s passenger (or closed truck bed) compartment. Carbon monoxide is produced by incomplete combustion of carbon or carbonaceous material, such as wood or charcoal.

The signs and symptoms of carbon monoxide poisoning are secondary to tissue hypoxia and vary with the level of COHb. Mild manifestations include headache, visual disturbances, irritability, and nausea, whereas more severe intoxication causes confusion, hallucinations, ataxia, and coma. Carbon monoxide may increase cerebral blood flow, increase cerebral capillary permeability, and increase cerebrospinal fluid pressure, all of which contribute to the central nervous system signs observed. The bright, cherry red lips and skin often described are less common than pallor and cyanosis.

• A continuous care approach with regular visits (at least every 1 to 6 months) to the health care provider is necessary to control symptoms and prevent exacerbations.

• Prevention of exacerbations includes avoiding triggers, avoiding allergens, and using medications as needed.

• Therapy includes efforts to reduce underlying inflammation and relieve or prevent symptomatic airway narrowing.

• Therapy includes patient education, environmental control, pharmacologic management, and the use of objective measures to monitor the severity of disease and guide the course of therapy.

Allergen Control: Nonpharmacologic therapy is aimed at the prevention and reduction of exposure to airborne allergens and irritants. House dust mites and other components of house dust are frequent agents identified in children allergic to inhalants. The cockroach, another common household inhabitant, is an important allergen in many locations. Exterminating live cockroaches, carefully cleaning kitchen floors and cabinets, putting food away after eating, and taking trash out in the evening are essential measures to control cockroaches. The mouse allergen is the most recent allergen to be identified in the homes of inner-city children with asthma. The role of cat and dog dander in allergen-induced asthma has also been studied. Sensitized persons should carefully evaluate having such pets in the household; there are inconclusive data on cat dander, but there is some evidence that dog dander either has no effect or may be protective (Sharma, Hansel, Matsui, et al, 2007). Additional sources of pollutants include ozone, particulate matter produced by tobacco smoke, wood-burning stoves, pesticides, lead, mold spores, nitrogen dioxide, and sulfur dioxide; these are believed to contribute to asthma morbidity in children and should be avoided or minimized (Diette, McCormack, Hansel, et al, 2008). Exposure to tobacco smoke is a significant contributing factor in the development of asthma in infants and small children (Sharma, Hansel, Matsui, et al, 2007).

Skin testing can identify specific allergens. Steps are then taken to eliminate or avoid them. Often, simply removing the offending environmental allergens or irritants (e.g., removing carpeting from the home of a child sensitive to mold and dust particles) decreases the frequency of asthma episodes. Dehumidifiers or air conditioners control nonspecific factors, such as extremes of temperature, that trigger an episode. Avoiding known outdoor allergens such as tree, grass, and weed pollen when these are high may reduce asthma exacerbations as well. Additional suggestions include:

Despite the proven association between the incidence of asthma and exposure to these residential hazards, little evidence-based research demonstrates an overall reduction in symptoms, even with significant interventions aimed at environmental (housing) modifications such as removal of carpeting, cleaning, and extermination (Sandel, Phelan, Wright, et al, 2004; Sharma, Hansel, Matsui, et al, 2007) (see Complementary and Alternative Therapy box).

Drug Therapy: Pharmacologic therapy is used to prevent and control asthma symptoms, reduce the frequency and severity of asthma exacerbations, and reverse airflow obstruction. A stepwise approach is recommended based on the severity of the child’s asthma. Because inflammation is considered an early and persistent feature of asthma, therapy is directed toward long-term suppression of inflammation.

Asthma medications are categorized into two general classes: long-term control medications (preventive medications) to achieve and maintain control of inflammation, and quick-relief medications (rescue medications) to treat symptoms and exacerbations (National Asthma Education and Prevention Program, 2007).

Quick-relief and long-term medications are often used in combination. Inhaled corticosteroids, cromolyn sodium and nedocromil, long-acting β₂-agonists, methylxanthines, and leukotriene modifiers are used as long-term control medications. Short-acting β₂-agonists, anticholinergics, and systemic corticosteroids are used as quick-relief (or rescue) medications. Bronchodilators that relax bronchial smooth muscle and dilate the airways include β₂-agonists, methylxanthines, and anticholinergics that can be used as both quick-relief and long-term medications.

Many asthma medications are given by inhalation with a nebulizer or a metered-dose inhaler (MDI). The MDI should always be attached to a spacer when an inhaled corticosteroid is administered to prevent yeast infections in the mouth. Spacers are also important for children who have difficulty coordinating or learning proper inhalation technique (Pongracic, 2003). The spacer and holder can be equipped with a mask or a mouthpiece (Fig. 32-11) (see Family-Centered Care box, p. 1278). Pharmaceutical companies are currently mandated to produce inhalers that do not contain chlorofluorocarbons (CFCs) as the propellant because CFCs have been linked to damage and depletion of the earth’s ozone level. An alternative propellant to the CFCs is hydrofluoroalkanes; the advantages include delivery of more fine particles and less oral deposition. Several currently available CFC-free MDI devices use dry powder (and also called dry powder inhalers [DPIs]); these include the Diskus inhaler and the Turbuhaler. These devices are breath activated, and the child needs to inhale as quickly and deeply as possible to use them effectively. The Diskhaler and Aerosolizer are similar, but with the Aerosolizer the medication must be loaded into the inhaler before use. Infants and young children who have difficulty using MDIs or other inhalers can receive their asthma medications via a hand-held nebulizer (Fig. 32-12). When this device is used, the medication is mixed with saline (also available in premixed form) and nebulized with compressed air. Children should breathe normally with the mouth open to provide a direct route to the trachea.

Corticosteroids are antiinflammatory drugs used to treat reversible airflow obstruction and control symptoms and reduce bronchial hyperresponsiveness in chronic asthma. A major change in the last two revisions of the National Asthma Education and Prevention Program guidelines (2007) is the recommendation that inhaled corticosteroids be used as first-line therapy in children over 5 years of age. Clinical studies of corticosteroids have indicated significant improvement of all asthma parameters, including decreases in symptoms, emergency visits, and medication requirements (National Asthma Education and Prevention Program, 2007).

Corticosteroids may be administered parenterally, orally, or by inhalation. Oral medications are metabolized slowly, with an onset of action up to 3 hours after administration and peak effectiveness occurring within 6 to 12 hours. Oral systemic steroids may be given for short periods (e.g., 3- or 10-day “bursts”) to gain prompt control of inadequately controlled persistent asthma or to manage severe persistent asthma. These drugs should be given in the lowest effective dose. They have few side effects (cough, dysphonia, and oral thrush), and there is strong evidence that they improve the long-term outcomes for children of all ages with mild or moderate persistent asthma. Evidence from clinical trials that monitored children for 6 years indicates that the use of inhaled corticosteroids at recommended dosages does not have long-term significant effects on growth, bone mineral density, ocular toxicity, or suppression of the adrenal-pituitary axis (National Asthma Education and Prevention Program, 2007). However, primary care providers should frequently monitor (at least every 3 to 6 months) the growth of children and adolescents taking corticosteroids to assess the systemic effects of these drugs and make appropriate reductions in dosages or changes to other types of asthma therapy when necessary. The inhaled corticosteroids include budesonide and fluticasone.

Cromolyn sodium is a nonsteroidal antiinflammatory drug (NSAID) for asthma. It stabilizes mast cell membranes; inhibits activation and release of mediators from eosinophil and epithelial cells; and inhibits the acute airway narrowing after exposure to exercise, cold dry air, and sulfur dioxide. There is no way to reliably predict whether a child will respond to the drug. Cromolyn sodium has minimal side effects (occasional coughing on inhalation of the powder formulation) and may be given via nebulizer or MDI. Nedocromil sodium inhibits the bronchoconstrictor response to inhaled antigens and inhibits the activity of and release of histamine, leukotrienes, and prostaglandins from inflammatory cells associated with asthma. The drug has few side effects and is used for maintenance therapy in asthma. The drug is not effective for reversal of acute exacerbations and is not used in children under 5 years of age

β-Adrenergic agonists (short acting) (primarily albuterol, levalbuterol [Xopenex], and terbutaline) are used for treatment of acute exacerbations and for the prevention of EIB. These drugs bind with the β-receptors on the smooth muscle of airways, where they activate adenylate cyclase and convert adenosine monophosphate (AMP) to cyclic AMP (cAMP). The increased cAMP enhances binding of intracellular calcium to the cell membrane, reducing the availability of calcium and thus allowing smooth muscle to relax. Other effects of the drug help stabilize mast cells to prevent release of mediators. Most β-adrenergics used in asthma therapy affect predominantly the β₂-receptors, which help eliminate bronchospasm. β₁-Adrenergic effects such as increased heart rate and gastrointestinal disturbances have been minimized. These drugs can be given via inhalation or as oral or parenteral preparations. The inhaled drug has a more rapid onset of action than the oral form. Inhalation also reduces troublesome systemic side effects: irritability, tremor, nervousness, and insomnia. Levalbuterol reportedly causes fewer side effects; however, its overall effectiveness in childhood asthma is controversial (Linzer, 2007). The 2007 National Asthma Education and Prevention Program guidelines recommend the addition of a long-acting β₂-agonist to a low- or medium-dose inhaled corticosteroid to improve lung function and asthma symptoms and decrease the need for a short-acting β₂-agonist. This combination may actually allow the practitioner to lower the corticosteroid dose and manage asthma symptoms just as effectively. Inhaled β-adrenergic agents should not be taken more than three or four times daily for acute symptoms without medical supervision. A continuous nebulization therapy with a short-acting β₂-agonist may be used in the acute setting with an acute exacerbation.

Salmeterol (Serevent) is a long-acting β₂-agonist (bronchodilator) that is used twice a day (no more frequently than every 12 hours). This drug is added to antiinflammatory therapy and used for long-term prevention of symptoms, especially nighttime symptoms, and EIB. Salmeterol is not used in children younger than 12 years of age.

Theophylline was used for decades to relieve symptoms and prevent asthma attacks; however, it is now used primarily in the emergency department when the child is not responding to maximal therapy (Linzer, 2007). Therapeutic levels should be obtained with this drug because it has a narrow therapeutic window.

Leukotrienes are mediators of inflammation that cause increases in airway hyperresponsiveness. Leukotriene modifiers (such as zafirlukast [Accolate] and montelukast sodium) block inflammatory and bronchospasm effects. These drugs are not used to treat acute episodes but are given orally in combination with β-agonists and steroids to provide long-term control and prevent symptoms in mild persistent asthma. Montelukast is approved for children 12 months old and older, whereas zafirlukast is approved for children 7 years and older.

Anticholinergics (atropine and ipratropium) help relieve acute bronchospasm. However, these drugs have adverse side effects that include drying of respiratory secretions, blurred vision, and cardiac and central nervous system stimulation. The primary anticholinergic drug used is ipratropium, which does not cross the blood-brain barrier and therefore elicits no central nervous system effects (as does atropine). Ipratropium, when used in combination with albuterol, has been effective during acute severe asthma in significantly improving lung function and reducing hospitalizations in children coming to the emergency department (Liu, Covar, Spahn, et al, 2007).

A fairly new asthma drug, omalizumab (Xolair), is a monoclonal antibody that blocks the binding of IgE to mast cells. Blocking this interaction eventually inhibits the inflammation that is associated with asthma. Because many patients with asthma are atopic and possess specific IgE antibodies to allergens responsible for airway inflammation, this drug is a promising adjunct to the treatment of asthma. It has been approved for use in children 12 years and older. The drug is administered once or twice a month by subcutaneous injection. Efficacy of omalizumab is not immediate, and clinical trials report that response to the drug was not evident before 12 weeks (Strunk and Bloomberg, 2006). Clinical trials of the drug indicate that it can be an effective therapy for patients with symptomatic moderate to severe allergic asthma that is poorly controlled with inhaled corticosteroids. However, it is expensive (Courtney, McCarter, and Pollart, 2005), and there have been reported cases of severe anaphylactic reactions. In early 2007 the FDA added a “black box warning” to the drug, which highlights the risk of anaphylaxis.

Some children with severe asthma and a history of severe life-threatening episodes may need a primary care practitioner prescription for an EpiPen (subcutaneous injectable epinephrine) (Liu, Covar, Spahn, et al, 2007).

Magnesium sulfate, a potent muscle relaxant that acts to decrease inflammation and improves pulmonary function and peak flow rate, may be used in pediatric patients treated in the emergency department with moderate to severe asthma. The drug is administered intravenously at 25 to 75 mg/kg (Liu, Covar, Spahn, et al, 2007).

Chest Physiotherapy: CPT includes breathing exercises and physical training. These therapies help produce physical and mental relaxation, improve posture, strengthen respiratory musculature, and develop more efficient patterns of breathing. For the motivated child, breathing exercises and controlled breathing help prevent overinflation and improve efficiency of the cough. However, CPT is not recommended during acute, uncomplicated exacerbations of asthma (National Asthma Education and Prevention Program, 2007) (see Bronchial [Postural] Drainage, Chapter 31).

Hyposensitization: The role of hyposensitization in childhood asthma is somewhat controversial. In the past, immunotherapy was used for seasonal allergies and when single substances were identified as the offending allergen. It is not recommended for allergens that can be eliminated, such as foods, drugs, and animal dander.

The National Asthma Education and Prevention Program guidelines (2007) recommend immunotherapy for asthma patients in the following situations:

Injection therapy is usually limited to clinically significant allergens. The initial dose of the offending allergen(s), based on the size of the skin reaction, is injected subcutaneously. The amount is increased at weekly intervals until a maximum tolerance is reached, after which a maintenance dose is given at 4-week intervals. This may be extended to 5- or 6-week intervals during the off-season for seasonal allergens. Successful treatment is continued for a minimum of 3 years and then stopped. If no symptoms appear, acquired immunity is assumed; if symptoms recur, treatment begins again. Hyposensitization injections should be administered only with emergency equipment and medications readily available in the event of an anaphylactic reaction.

Exercise and Exercise-Induced Bronchospasm: EIB is an acute, reversible, usually self-terminating airway obstruction that develops during or after vigorous activity, reaches its peak 5 to 10 minutes after stopping the activity, and usually stops in another 20 to 30 minutes. Patients with EIB have cough, shortness of breath, chest pain or tightness, wheezing, and endurance problems during exercise, but an exercise challenge test in a laboratory is necessary to make the diagnosis.

The problem occurs rarely in activities that require short bursts of energy (e.g., baseball, sprints, gymnastics, skiing) and more common in those that involve endurance exercise (e.g., soccer, basketball, distance running). Swimming is well tolerated by children with EIB because they are breathing air fully saturated with moisture and because of the type of breathing required in swimming.

Children with asthma are often excluded from exercise by parents, teachers, and practitioners, as well as by the children themselves, because they are reluctant to provoke an attack. However, this practice can seriously hamper peer interaction and physical health. Exercise is advantageous for children with asthma, and most children can participate in activities at school and in sports with minimal difficulty, provided their asthma is under control. Evaluate participation on an individual basis. Appropriate prophylactic treatment with β-adrenergic agents or cromolyn sodium before exercise usually permits full participation in strenuous exertion.

Status Asthmaticus: Status asthmaticus is a medical emergency that can result in respiratory failure and death if unrecognized and untreated. Children who continue to display respiratory distress despite vigorous therapeutic measures, especially the use of sympathomimetics (e.g., albuterol, epinephrine), are in status asthmaticus. The condition may develop gradually or rapidly, often coincident with complicating conditions, such as pneumonia or a respiratory virus, which can influence the duration and treatment of the exacerbation.

Therapy for status asthmaticus is aimed at improving ventilation, decreasing airway resistance and relieving bronchospasm, correcting dehydration and acidosis, allaying child and parent anxiety related to the severity of the event, and treating any concurrent infection. Humidified oxygen is recommended and should be given to maintain an oxygen saturation greater than 90%. Inhaled aerosolized short-acting β₂-agonists are recommended for all patients. Three treatments of β₂-agonists spaced 20 to 30 minutes apart are usually given as initial therapy, and continuous administration of β₂-agonists may be initiated. A systemic corticosteroid (oral, IV, or IM) may also be given to decrease the effects of inflammation. An anticholinergic such as ipratropium bromide may be added to the aerosolized solution of the β₂-agonist. Anticholinergics have resulted in additional bronchodilation in patients with severe airflow obstruction. An IV infusion is often initiated to provide a means for hydration and to administer medications. Correction of dehydration, acidosis, hypoxia, and electrolyte disturbance is guided by frequent determination of arterial pH, blood gases, and serum electrolytes.

Additional therapies in acute asthma attacks include the use of IV magnesium sulfate, a potent muscle relaxant that decreases inflammation and improves pulmonary function and peak flow rate among pediatric patients treated in the emergency department with moderate to severe asthma. Heliox may be administered to decrease airway resistance and thereby decrease the work of breathing; it can be delivered via a nonrebreathing face mask from premixed tanks, which may be blended in a stand-alone unit or within a ventilator. It may be used in acute exacerbations as an adjunct to β₂-agonist and IV corticosteroid therapy to improve pulmonary function until the two latter medications have time to take full effect in decreasing bronchospasm; the effects of heliox are usually seen within 20 minutes of administration, whereas other drugs may take longer to exert the desired effect. Ketamine, a dissociative anesthetic, is believed to cause smooth muscle relaxation and decrease airway resistance caused by severe bronchospasm in acute asthma (Linzer, 2007); it may be administered as an adjunct to other therapies mentioned previously.

Antibiotics should not be used to treat acute asthma attacks except when a bacterial infection resulting from another condition such as pneumonia or sinusitis is present (National Asthma Education and Prevention Program, 2007).

A child suspected of having status asthmaticus is usually seen in the emergency department and is often admitted to a pediatric intensive care unit for close observation and continuous cardiorespiratory monitoring. A key component in the prevention of morbidity is helping the child, parents, teachers, coaches, and other adults recognize features of deteriorating respiratory status, use the correct rescue drugs effectively, and immediately place the child with deteriorating respiratory status into the care of trained health care professionals instead of waiting to see if the asthma gets better on its own. The child going into early status asthmaticus is no different from the adult who is having an acute myocardial infarction in terms of needing trained medical assistance before the condition deteriorates to irreversible respiratory failure and possible death. Community education regarding asthma recognition and management is an important component of nursing care.

Prognosis: According to recent Centers for Disease Control and Prevention (2009c) data, 9.1% of U.S. children ages birth to 17 years, or 6.7 million children, were reported to have asthma. Although deaths from asthma have been relatively uncommon since the 1980s, the rate of death from asthma increased steadily in the United States until it peaked in the mid-1990s. Asthma-related deaths decreased between 1996 and 2005 by approximately 3.9% per year (Akinbami, Moorman, Garbe, et al, 2009). Data for the year 2008 indicate a significant increase in asthma symptoms, emergency department visits, and hospitalization among boys from birth to 4 years of age. Mortality and morbidity for asthma are especially high among African-American children, whose hospitalization and death rates are three times higher than those of Caucasian and Hispanic children (Liu, Covar, Spahn, et al, 2007). Most asthma deaths in children occur in the home, school, or community before lifesaving medical care can be administered.

The outlook for children with asthma varies widely. Some children’s asthma symptoms may improve at puberty, but up to two thirds of children with asthma continue to have symptoms through puberty and into adulthood. The prognosis for control or disappearance of symptoms varies in children from those who have rare and infrequent attacks to those who are constantly wheezing or are subject to status asthmaticus. In general, when symptoms are severe and numerous, when symptoms have been present for a long time, and when there is a family history of allergy, there is a greater likelihood of a poor prognosis. Risk factors that may predict persistence of symptoms into childhood (from infancy) include atopy, male gender, exposure to environmental tobacco, and maternal history of asthma. Many children who outgrow their exacerbations continue to have airway hyperresponsiveness and cough as adults. Furthermore, airway hyperresponsiveness in adults appears to be associated with decreased lung function.

The adolescent age-group appears to be the most vulnerable, with the greatest increase in mortality from the condition occurring in children 10 to 14 years of age. No reliable data exist to explain this increase. Factors that have been postulated include exposure of atopic persons to more allergens (particularly in large urban centers), change in severity of the disease, abuse of drug therapy (toxicity), failure of families and practitioners to recognize the severity of asthma, and psychologic factors such as denial and refusal to accept the disease.

Risk factors for asthma deaths include early onset, frequent attacks, difficult-to-manage disease, adolescence, history of respiratory failure, psychologic problems (refusal to take medications), dependency on or misuse of asthma drugs (high use), presence of physical stigmata (barrel chest, intercostal retractions), and abnormal PFTs.

Acute Asthma Care: Children who are admitted to the hospital with acute asthma are ill, anxious, and uncomfortable. The progression or resolution of status asthmaticus is variable. Continual observation and assessment are essential (see Nursing Care Plan).

Nursing Care Plan—The Child with Asthma

When β₂-agonists and corticosteroids are given, the child is monitored closely and continuously for relief of respiratory distress and signs of side effects (tachycardia, restlessness, irritability, hyperactivity). Although food may not be well tolerated in the acute phase, the child may avoid upset stomach associated with β-agonists by taking small amounts of a food, such as a few crackers, once the respiratory status has stabilized somewhat. Pulse oximetry is monitored along with rate and depth of breathing, auscultation of air movement, adventitious sounds, and any signs of respiratory distress (e.g., nasal flaring, tachypnea, retractions). The child on supplemental oxygen requires intermittent or continuous oxygenation monitoring depending on severity of respiratory compromise and initial oxygenation status. The child in status asthmaticus should be placed on continuous cardiorespiratory (including blood pressure) and pulse oximetry monitoring.

IV access is usually initiated once the child has been placed on oxygen. The child may respond well to topical pain management for the procedure. Oral fluid intake may be limited during the acute phase; IV fluid replacement may be required to provide adequate tissue hydration. Endotracheal intubation equipment should be readily available. Medications administered intravenously are monitored for their desired effect and for any untoward effects.

Children with acute asthma are apprehensive and anxious, and they often hyperventilate as a result of the anxiety. Calm coaching to increase depth and slow rate of respirations while administering oxygen with a simple mask may alleviate the child’s fears. The calm, efficient presence of a nurse helps reassure the child that he or she is safe and will be cared for during this stressful period. Assure children that they will not be left alone and that their parents are allowed to remain with them.

Parents need reassurance and want to be informed of their child’s condition and therapies. They may believe that they have in some way contributed to the child’s condition or could have prevented the episode. Reassurance regarding their efforts expended on the child’s behalf and their parenting capabilities can help alleviate their stress. Efforts to reduce parental apprehension also reduce the child’s distress. Anxiety is easily communicated to the child from parents and members of the staff.

General Care: The nursing care of the child with asthma begins with a review of the child’s health history; the home, school, and play environment; parent and child attitudes about the child’s condition; and a comprehensive physical assessment with focus on the respiratory system. Nursing care of children with asthma involves both acute and long-term care. Nurses who are involved with children in the home, hospital, school, outpatient clinic, or practitioner’s office play an important role in helping children and their families learn to live with the condition. The disease can be managed so that it does not require hospitalization or interfere with family life, physical activity, or school attendance. The nursing process in the care of the child with asthma is outlined in the Nursing Care Plan.

Physical assessment of asthma involves the same observations and techniques described in Chapter 6. In addition, the nurse notes and evaluates physical characteristics of chronic respiratory involvement, including chest configuration (e.g., barrel chest), posturing (tripod), and type of breathing. A history of the current and previous episodes and precipitating factors or events provides important information.

Nurses may perform a variety of functions in asthma care, including asthma education in the primary care setting and in schools and other community settings, care of the child with asthma in the acute care setting, ambulatory care, and intensive care. Nurses also obtain information on how asthma affects the child’s everyday activities and self-concept, the child’s and family’s adherence to the prescribed therapy, and their personal treatment goals. Make every effort to build a partnership between the child and family and the health care team. Communication is an essential part of this partnership, and health care providers should routinely assess the effectiveness of patient-provider communication. In particular, assess the child’s and family’s satisfaction with asthma control and with the quality of care. The nurse should also assess their perception of the severity of the disease and their level of social support.

One of the major emphases of nursing care is outpatient management by the family. Parents are taught how to avoid allergens (especially tobacco smoke), recognize and respond to symptoms of bronchospasm, maintain health and prevent complications, and promote normal activities. The child’s asthma action plan should be reviewed periodically at least every 6 months in children with moderate to severe disease; precipitating factors, illness management, and medication use should be discussed. The nurse should determine any cultural or ethnic beliefs or practices that influence self-management and that may necessitate modifications in educational approaches to meet the family’s needs.

Avoid Allergens: One goal of asthma management is avoidance of an exacerbation. Parents need to know how to avoid allergens that precipitate asthma episodes. The nurse assists the parent in modifying the environment to reduce contact with the offending allergen(s). Caution the parents to avoid exposing a sensitive child to excessive cold, wind, or other extremes of weather; smoke; sprays; or other irritants. Parents should also eliminate from the diet any foods known to provoke symptoms.

Approximately 2% to 6% of children with asthma are sensitive to aspirin; therefore nurses should caution parents to use other analgesic-antipyretic drugs for discomfort or fever and to read package labeling. Although aspirin is rarely given to children in the United States, salicylate compounds are in other common medicines such as Pepto-Bismol. Children with aspirin-induced asthma may also be sensitive to NSAIDs and tartrazine (yellow dye number 5, a common food coloring).

Teach parents to avoid administering aspirin to any child because of its association with Reye syndrome unless specifically recommended by and under the supervision of a health practitioner. Acetaminophen is safe for children and is the analgesic of choice.

Relieve Bronchospasm: Teach parents and older children to recognize early signs and symptoms of an impending attack so that it can be controlled before symptoms become distressing. Most children can recognize prodromal symptoms well before an attack (about 6 hours) and implement preventive therapy. Objective signs that parents may observe include rhinorrhea, cough, low-grade fever, irritability, itching (especially in front of the neck and chest), apathy, anxiety, sleep disturbance, abdominal discomfort, and loss of appetite.

The peak expiratory flow rate (PEFR) measures the maximum flow of air that can be forcefully exhaled in 1 second. PEFR is measured in liters per minute using a peak expiratory flow meter (PEFM). Three zones of measurement are typically used to interpret PEFR. The zone system is patterned after a traffic light to make the categories easy to understand and remember (see Nursing Care Guidelines box). Each child needs to establish his or her personal best value. A personal best value should be established during a 2- to 3-week period when the child’s asthma is stable. During this period the child records the PEFR at least twice a day. After the personal best value has been established, the child’s current PEFR on any occasion can be compared with the personal best value. PEFR monitoring can be used for short-term monitoring, managing exacerbations, and daily long-term monitoring.

Because measurement of PEFR depends on effort and technique, children need instructions, demonstrations, and frequent reviews of technique (see Family-Centered Care box). Each individual child’s PEFR varies according to age, height, sex, and race.

A variety of easy-to-use, inexpensive PEFMs are available for use in the home and at school to assess changes in pulmonary function. In general, children 5 years of age and older are able to use a PEFM successfully. However, young children need to be supervised while they are learning to use their PEFM, and their technique should be checked frequently to ensure it is correct. Children should use the same PEFM over time, and they should bring it for use at every follow-up visit. Using the same brand of meter is recommended because different brands can give significantly different values. The use of a PEFM provides objective monitoring regarding the severity of asthma and can decrease asthma episodes, health care visits, and missed school days (Burkhart, Rayens, Revelette, et al, 2007).

Children who use a nebulizer, MDI, Diskus, or Turbuhaler to deliver drugs need to learn how to use the device correctly. The MDI device delivers medication directly to the airways; therefore the child needs to learn to breathe slowly and deeply for better distribution to narrowed airways (see Family-Centered Care box).

Young children and those who are unable to manipulate the MDI or hold their breath for 10 seconds should use a spacer. In infants and small children a mask may be used to facilitate delivery of the medication. These devices allow the parent or child to deliver the medication from the MDI into the spacer, from which the child then inhales the medication while taking slow steady breaths. Spacers also prevent yeast infections in the mouth when corticosteroids are inhaled via an MDI.

The nurse also needs to caution the child and parents about the adverse effects of prescribed drugs and the dangers of overuse of β₂-agonists. They should know that it is important to use these drugs when needed but not indiscriminately or as a substitute for avoiding the symptom-provoking allergen. Caution parents against purchasing over-the-counter preparations because these medications can place the children at risk for increased dosage of a drug and toxicity.

The family should obtain a PEFM and learn to use this device to monitor the child’s asthma. A written asthma action plan that includes the three peak flow meter zones and the child’s asthma medications may be obtained from the child’s primary care provider (Fig. 32-13). A home asthma action plan may reduce the risk of asthma death by 70% (Liu, Covar, Spahn, et al, 2007). Medications used for asthma exacerbations are also included in the asthma plan. This action plan should be used to make decisions about asthma management at home and at school. The nurse may assist the child and family in preparing this plan, emphasizing that they, and not the health professionals, determine the success of the plan.

Teach parents how to read labels on prepared foods and snacks to determine the presence of allergens.

Maintain Health and Prevent Complications: The child should be protected from a respiratory tract infection that can trigger an attack or aggravate the asthmatic state, especially in young children whose airways are mechanically smaller and more reactive. Annual influenza vaccinations are recommended for children with persistent asthma (American Academy of Pediatrics, 2009b). Equipment used for the child, such as nebulizers, must be kept absolutely clean to decrease the chances of contamination with bacteria and fungi.

Teach and encourage breathing exercises and controlled breathing for motivated children, and provide information concerning activities that promote diaphragmatic breathing, side expansion, and improved mobility of the chest wall. Play techniques that can be used for younger children to extend their expiratory time and increase expiratory pressure include blowing cotton balls or a Ping-Pong ball on a table, blowing a pinwheel, blowing bubbles, or preventing a tissue from falling by blowing it against the wall.

Promote Self-Care and Normalization: Self-care and asthma self-management programs are important in helping the child and family cope with asthma. Most asthma self-management programs for children convey several principles. First, asthma is a common disease that can be controlled with appropriate drug therapy, environmental control, education, and management skills. Second, it is much easier to prevent than to treat an asthma episode; adherence to a therapeutic program is necessary to prevent exacerbations. Third, children with asthma can live full and active lives.

Asthma camps provide an opportunity for children with asthma to engage in physical activity while learning about their disease in a controlled environment with their peers and health professionals. Children who attend asthma camps often demonstrate improved asthma self-management skills.

Self-contained programs and brochures for patient education are available from the Asthma and Allergy Foundation of America* and the American Lung Association.† The National Heart, Lung, and Blood Institute‡ provides educational materials for asthma education in the school setting and also copies of the Guidelines for the Diagnosis and Management of Asthma for the practitioner (National Asthma Education and Prevention Program, 2007). Another publication designed for health care practitioners, Pediatric Asthma: Promoting Best Practice, can be obtained from the American Academy of Allergy Asthma and Immunology.§

Child and Family Support: The nurse working with children with asthma can provide support in a number of ways. Many children voice frustration because their exacerbations interfere with their daily activities and social lives. They need education about what to do to prevent an asthma episode. These children also need reassurance from the health team that they can learn to control and cope with their asthma and live a normal life. Be aware of children, especially adolescents, who demonstrate signs of depression and may not comply with therapy as a means of passive suicide.

Children in disruptive family situations (divorce, separation, violence, custodial battles) may disregard daily asthma medication regimen or may be at higher risk as a result of neglect by adults who are in charge of their care. Adolescents struggling with a sense of identity and body image often regard asthma as a condition that will “go away,” especially if there is a time lapse between symptoms, and may abandon the therapeutic regimen. In some cases adolescents find themselves in charge of other siblings in blended family situations and may ignore their own health needs. Referral for counseling and guidance is appropriate when the child’s or adolescent’s life is potentially in danger and the therapeutic regimen for asthma is abandoned due to other crises.

The short- and long-term adaptation of children with asthma often depends on the family’s acceptance of the disorder. The task of living day-to-day with affected children involves the entire family. There are periodic crises and the ever-present threat of a crisis, requiring parental vigilance; sleepless nights; frequent trips to the physician, emergency department, or hospital; and often overwhelming medical expenses. Throughout these stresses, encourage parents to promote as normal a life as possible for their children.

Respiratory Tract: Initial pulmonary manifestations are often wheezing respirations and a dry, nonproductive cough. Eventually diffuse bronchial and bronchiolar obstruction leads to irregular aeration with progressive pulmonary disturbance and secondary infection. The most prominent and constant feature of pulmonary involvement is chronic cough. Dyspnea increases, the cough often becomes paroxysmal, and the mucoid impactions within the small air passages cause a generalized obstructive hyperinflation and patchy areas of atelectasis.

Progressive pulmonary involvement with hyperaeration of functioning alveoli produces the overinflated, barrel-shaped chest in which the anteroposterior diameter approaches the lateral diameter. Bronchiectatic cysts and subpleural blebs in the upper lobes occur in advanced disease and may rupture, causing pneumothorax (Boat and Acton, 2007). When ventilation and subsequent diffusion and gas exchange are significantly impaired, cyanosis and clubbing of the fingers and toes may occur. The child or adolescent has repeated episodes of bronchitis and bronchopneumonia and is subject to chronic nasal congestion, rhinitis, chronic sinusitis, and nasal polyps. The incidence of ENT surgeries is higher in this group of children when compared with the general population.

Gastrointestinal Tract: The earliest postnatal manifestation of CF is meconium ileus, which occurs in 15% to 20% of newborns with the disease (Boat and Acton, 2007). Thick, puttylike, tenacious, mucilaginous meconium blocks the lumen of the small intestine, usually at or near the ileocecal valve, which gives rise to signs of intestinal obstruction, including abdominal distention, vomiting, failure to pass stools, and rapid development of dehydration with associated electrolyte imbalance. Thick intestinal secretions continue to be problematic throughout life. Children of all ages are subject to intestinal obstruction (distal ileum) from heavy or impacted feces. Gumlike masses in the cecum can obstruct the bowel, causing pain, nausea, and vomiting. This is referred to as meconium ileus equivalent. Distal intestinal obstruction syndrome is the name given to a partial or complete intestinal obstruction that occurs in some children with CF.

As the disease progresses, obstruction of pancreatic ducts prevents digestive enzymes (trypsin, chymotrypsin, amylase, lipase) from being released into the duodenum, which prevents conversion of ingested food into compounds that can be absorbed by the intestinal mucosa. Consequently, the undigested food (chiefly unabsorbed fats and proteins) is excreted, increasing the bulk of feces to two or three times the normal amount. The bulky nature of the stools may go unnoticed at first, but usually by 6 months of age the child passes large, loose stools with normal frequency or has chronic diarrhea with unformed stools. As solid foods are added to the diet, the excessively large stools become frothy and extremely foul smelling.

Because so little is absorbed from the intestine, affected children have difficulty maintaining weight despite a healthy appetite and diet. Unable to compensate for the fecal losses, many children lose weight and exhibit marked wasting of tissues and growth failure. The abdomen is distended, the extremities are thin, and the sallow skin droops from wasted buttocks. The impaired ability to absorb fats results in a deficiency of the fat-soluble vitamins A, D, E, and K, which may cause bleeding problems if vitamin K deficiency is significant. Anemia is a common complication. Growth failure may be an initial diagnosis in young children with previously undiagnosed CF. Many older children with CF have an increased prevalence of gastroesophageal reflux.

Another common gastrointestinal complication is prolapse of the rectum, which occurs in infancy and early childhood and is related to large, bulky stools; malnutrition; and increased intraabdominal pressure secondary to paroxysmal cough. Appendicitis, intussusception, and volvulus may also occur more frequently in children with CF (Hazle, 2010).

Reproductive System: Delayed puberty in girls with CF is common even when their nutritional and clinical status is good. CF affects the reproductive systems of both males and females. Females with CF have normal fallopian tubes and ovaries. Fertility can be inhibited by highly viscous cervical secretions, which act as a plug, blocking sperm entry. Women with CF who become pregnant have an increased incidence of premature labor and delivery and low birth weight in the infant. Favorable nutritional status and pulmonary function are positively correlated with favorable pregnancy outcomes. Most adult men (approximately 95%) with CF are sterile, which may be caused by blockage of the vas deferens with abnormal secretions or by failure of normal development of the wolffian duct structures (vas deferens, epididymis, and seminal vesicles), resulting in decreased or absent sperm production.

Integumentary System: The consistent finding of abnormally high sodium and chloride concentrations in the sweat is a unique characteristic of CF. Parents frequently observe that their infants taste “salty” when they kiss them. The chloride channel defect in sweat glands prevents reabsorption of sodium and chloride, which leaves the affected person at risk for abnormal salt loss, dehydration, and hypochloremic and hyponatremic alkalosis during hyperthermic conditions. This is especially important to the infant because of limited fluid stores and the potential for inadequate sodium intake with most commercially prepared infant formulas.

The disease is sometimes expressed in other ways (e.g., hyponatremia caused by massive losses through sweat, especially in high environmental temperatures or febrile episodes). Infants with CF who have growth failure frequently demonstrate hypoalbuminemia resulting from diminished absorption of protein, which in severe cases causes generalized edema.

Management of Pulmonary Problems: Direct management of pulmonary problems toward prevention and treatment of pulmonary infection by improving ventilation, removing mucopurulent secretions, and administering antimicrobial agents. Many children develop respiratory symptoms by 3 years of age. The large amounts and viscosity of respiratory secretions in children with CF contribute to the likelihood of respiratory tract infections. Recurrent pulmonary infections in the child with CF result in greater the damage to the airways; small airways are destroyed, causing bronchiectasis.

The most common pathogens responsible for pulmonary infections are P. aeruginosa, B. cepacia, S. aureus, H. influenzae, E. coli, and K. pneumoniae. P. aeruginosa and B. cepacia are particularly pathogenic for children with CF, and infections with these organisms are difficult to clear. In addition, children with CF who are chronically colonized with these organisms have poorer survival rates than children who are not colonized (Boat and Acton, 2007). Colonization and infection with methicillin-resistant S. aureus (MRSA) has recently emerged as a critical factor in lung infection and pulmonary function in patients with CF. Patients with MRSA required longer hospitalization and multiple antibiotic regimens (Ren, Morgan, Konstan, et al, 2007). Fungal colonization with Candida or Aspergillus organisms in the respiratory tract is also common in CF patients.

Airway Clearance Therapy: Prevention of pulmonary infection involves a daily routine of CPT to maintain pulmonary hygiene. (See Chapter 31.) CPT is usually performed on average twice daily (on rising and in the evening) and more frequently if needed, especially during pulmonary infection. The Flutter mucus clearance device is a small, hand-held plastic pipe with a stainless-steel ball on the inside that facilitates removal of mucus (Fig. 32-15). It has the advantages of increasing sputum expectoration and being used without assistance. Hand-held percussors and mechanical percussors may be used to loosen secretions. Another method to clear mucus is high-frequency chest compression, in which the child temporarily wears a mechanical vest device that provides high-frequency chest wall oscillation. Some children and adolescents with an implantable port may experience localized pain with the vest.

Patients with CF have been found to regress when conventional CPT is discontinued. Therefore, although it is time consuming for the child and family, CPT remains the cornerstone of pulmonary therapy. Forced expiration, or “huffing,” with the glottis partially closed helps move secretions from the small airways so that subsequent coughing can move secretions forcefully from the large airways. This maneuver enhances the pulmonary function of patients with CF. Autogenic drainage involves a variety of breathing techniques, which the older child can use to force mucus in lower lobes up into the airways so it can be successfully expelled. Another mucus-clearing technique involves use of a positive expiratory pressure mask; this technique involves breathing into a mask attached to a one-way valve, which creates resistance—as the patient exhales, the airway is kept open by the pressure and mucus is forced into the upper airway for expulsion.

Bronchodilator medication delivered in an aerosol opens bronchi for easier expectoration and is administered before CPT when the patient exhibits evidence of reactive airway disease or wheezing. Another aerosolized medication is recombinant human deoxyribonuclease (DNase, known generically as dornase alfa [Pulmozyme]), which decreases the viscosity of mucus. It is well tolerated and has no major adverse effects; minor reactions are voice alterations and laryngitis. This medication, given once or twice daily via nebulization, has resulted in improvements in spirometry, PFTs, dyspnea scores, and perceptions of well-being and has reduced the viscosity of sputum (Redding, 2009). Nebulized hypertonic saline (6% to 7%) has been shown to be effective in improving airway hydration and increases mucus clearance in patients with CF; this treatment, however, causes bronchospasm and may not be recommended for patients with severe disease (Redding, 2009; Rowe and Clancy, 2006). Clinical trials are in progress to examine the effects of inhaled dry powdered mannitol for improving mucociliary clearance in CF by rehydrating the airway (Minasian, Wallis, Metcalfe, et al, 2008).

Physical exercise is an important adjunct to daily CPT. Exercise stimulates mucus excretion and provides a sense of well-being and increased self-esteem. Any aerobic exercise that the patient enjoys should be encouraged. The ultimate aim of exercise is to increase lung vital capacity, remove secretions, increase pulmonary blood flow, and maintain healthy lung tissue for effective ventilation.

Colonization with P. aeruginosa and B. cepacia signals progressive involvement. Although the bacteria are impossible to eradicate, they can be successfully controlled. Inhaled antibiotics are administered as a prophylactic measure in some centers, but once the organisms have become established, antibiotic therapy is most effective when given intravenously. Patients with CF metabolize antibiotics more rapidly than normal; therefore drug dosage is often higher than would be expected. Depending on its sensitivity, P. aeruginosa is usually treated with aminoglycosides in combination with antipseudomonal β-lactam antibiotics (ticarcillin, piperacillin, ceftazidime). Antibiotic treatment of B. cepacia should be based on susceptibility and synergy testing. The duration of therapy depends on the patient’s response, measured by clinical indicators, including cough, fatigue, and exercise intolerance, in addition to tests such as PFTs, chest radiography, and oxygen and carbon dioxide measurements.

Pulmonary infections are treated as soon as they are recognized. In CF patients characteristic signs of pulmonary infection—fever, tachypnea, and chest pain—may be absent. Therefore a careful history and physical examination are essential. The presence of anorexia, weight loss, and decreased activity alert the practitioner to pulmonary infection and the need for an antibiotic regimen (Boat and Acton, 2007). Aerosolized antibiotics such as tobramycin, ticarcillin, and gentamicin are beneficial for patients with frequent pulmonary exacerbations (Redding, 2009). These medications are usually administered by jet or ultrasonic nebulizers after CPT is performed. This type of delivery system allows for direct antimicrobial application with little systemic absorption. It is not uncommon for the hospitalized child with CF to be placed on as many as two or three antibiotics and one antifungal medication to treat coexisting pulmonary infections.

IV antibiotics may be administered at home as an alternative to hospitalization. The use of peripherally inserted central catheters (PICCs) for the administration of antibiotics in children with CF is a viable option with limited complications and fewer needle punctures to obtain blood specimens and to maintain often lengthy treatment with parenteral antibiotics (Tolomeo and Mackey, 2003). Alternatively, an implanted port offers the advantage of access for blood draws and antibiotic infusion. Patients may receive antibiotic therapy at home and continue daily activities with minimum disruptions. However, when pulmonary function does not improve with outpatient management, hospitalization may be recommended for continued antibiotic therapy and vigorous CPT and postural drainage. Oxygen administration is used for children with acute episodes but must be used cautiously because many children with CF have chronic carbon dioxide retention, and the unsupervised use of oxygen can be harmful. (See Oxygen Therapy, Chapter 31.) With repeated infection and inflammation, bronchial cysts and emphysema may develop. These cysts may rupture, resulting in a pneumothorax (see Box 32-10).

Blood streaking of the sputum is usually associated with increased pulmonary infection and often requires no specific treatment. Hemoptysis greater than 250 ml/24 hr for the older child (less for a younger child) indicates a potentially life-threatening event and needs to be treated immediately. Sometimes bleeding can be controlled with bed rest, IV antibiotics, replacement of acute blood loss, IV conjugated estrogens (Premarin) or vasopressin (Pitressin), and correction of any coagulation defects with vitamin K or fresh frozen plasma. If hemoptysis persists, the site of bleeding should be localized via bronchoscopy and cauterized or embolized.

Children and adolescents with CF should be given the age-appropriate immunizations, including the annual influenza virus vaccine. The trivalent inactivated influenza virus vaccine is appropriate for such individuals.

Treatment of nasal polyps includes intranasal corticosteroids, oral antihistamines, and decongestants. If these measures are ineffective, surgical interventions may be necessary.

Because pulmonary damage in patients with CF is believed to be caused by the inflammatory process that occurs with frequent infections, the use of corticosteroids has been studied; however, treatment with corticosteroids found only a modest efficacy and numerous side effects, including linear growth restriction, glucose tolerance abnormalities, and cataract formation (Boat and Acton, 2007). Antiinflammatory medications such as the NSAID ibuprofen have shown significant benefits particularly in younger patients with mild disease (Boat and Acton, 2007). Long-term daily ibuprofen given in a dose sufficient to achieve a peak plasma concentration between 50 and 100 mcg/ml has been shown to slow the rate of decline in pulmonary function and to decrease the need for IV antibiotics in young patients with mild pulmonary involvement Although this therapy is generally well tolerated, careful monitoring for adverse effects (gastrointestinal bleeding) is essential (Boat and Acton, 2007).

Lung transplantation is a final therapeutic option for many CF patients with severe disease. Heart-lung and double-lung procedures have been successfully performed in children with advanced pulmonary vascular disease and hypoxia; however, whether such procedures significantly improve quality of life and survival rates in children with CF is debated in the current literature. Some experts state that infections such as B. cepacia represent a negative factor for long-term survival after transplant (Kotloff, 2009; Liou, Adler, Cox, et al, 2007). Data show that 5-year survival in adults with CF and without B. cepacia is less than 50% (Kotloff, 2009). The obstacles surrounding this technique are availability of donated organs; complications from surgery; and recurrence of pulmonary infections and obstructive bronchiolitis, which decreases transplanted lung function. Living-donor lobar transplantation is an alternative to cadaveric transplantation for those who might otherwise succumb to pulmonary and cardiac failure while waiting for a transplant (Goldberg and Deykin, 2007).

Management of Gastrointestinal Problems: The principal treatment for pancreatic insufficiency is replacement of pancreatic enzymes, which are administered with meals and snacks to ensure that digestive enzymes are mixed with food in the duodenum. Enteric-coated products prevent the neutralization of enzymes by gastric acids, thus allowing activation to occur in the alkaline environment of the small bowel. The amount of enzymes depends on the severity of the insufficiency, the child’s response to enzyme replacement, and the practitioner’s philosophy. Usually one to five capsules are administered with a meal, and fewer are taken with snacks. Capsules can be swallowed whole or taken apart, and the contents sprinkled on a small amount of food to be taken at the beginning of the meal. The amount of enzyme is adjusted to achieve normal growth and a decrease in the number of stools to one or two per day. Pancreatic enzymes should be taken within 30 minutes of eating. The enteric-coated beads should not be chewed or crushed, since destroying the enteric coating can lead to inactivation of the enzymes and excoriation of oral mucosa. The powder form should be used cautiously because inhalation of the powder may precipitate acute bronchospasm. Enzymes are mixed into cereal or fruit such as applesauce for small children.

One issue of concern with pancreatic enzymes is that generic enzymes are not considered adequate and only proprietary enzymes should be given to children with CF (Stallings, Stark, Robinson, et al, 2008). Because the uptake of fat-soluble vitamins is decreased, water-miscible forms of these vitamins (A, D, E, and K) are given, along with multivitamins and the pancreatic enzymes. When high-fat foods are eaten, the child is encouraged to add extra enzymes.

Children with CF require a well-balanced, high-protein, high-caloric diet, with unrestricted fat (because of the impaired intestinal absorption). Improved nutrition in children with CF has been associated positively with improved lung function. To meet his or her energy requirements, the patient with minimal pulmonary disease must consume 5% to 10% more than the recommended dietary allowance (RDA); for those with severe lung disease energy requirements may be as high as 20% to 50% or more of the RDA (American Academy of Pediatrics, 2009a). A group of experts recently recommended that children and adolescents with CF 2 to 20 years of age have an energy intake of 110% to 200% of standards for healthy persons (Stallings, Stark, Robinson, et al, 2008). Regular nutritional monitoring should be a standard part of the medical care of the child with CF and should occur every 3 to 4 months (American Academy of Pediatrics, 2009a). Breast-feeding with enzyme supplementation should be continued whenever possible and, when necessary, supplemented with a higher-calorie-per-ounce (e.g., 24 kcal/oz) formula. For formula-fed infants, commercial cow’s milk–based formulas may be adequate to achieve desired growth, but additional caloric intake may be required. In older children with CF three daily meals and three snacks are recommended to meet energy and growth requirements (American Academy of Pediatrics, 2009a).

Growth failure despite adequate nutritional support may indicate deterioration of pulmonary status. Data indicate that better forced expiratory volume in 1 second (FEV₁) status (≥80%) strongly correlates with body mass index percentiles above 50th percentile; therefore the target weight for children with CF 2 to 20 years old should be ideally maintained above the 50th percentile (Stallings, Stark, Robinson, et al, 2008). A persistent weight loss over 6 months to 1 year or persistent malnutrition requires aggressive nutritional therapy to prevent declining pulmonary and general physical health status (American Academy of Pediatrics, 2009a). Patients with CF may experience frequent anorexia as a result of the copious amounts of mucus produced and expectorated, persistent cough, effect of medications, fatigue, and sleep disruption. They may be placed on nighttime (or continuous) supplemental gastrostomy feedings or parenteral alimentation in an effort to build up nutritional reserves if there has been a history of inability to maintain weight. Enzyme supplement is encouraged with gastrostomy feedings; these may be given at the initiation of the infusion, at bedtime, and at the conclusion of the feeding infusion (American Academy of Pediatrics, 2009a).

Meconium ileus and meconium ileus equivalent, or total or partial intestinal obstruction, can occur at any age. Constipation is often the result of a combination of malabsorption (either from inadequate pancreatic enzyme dosage or a failure to take the enzymes), decreased intestinal motility, and abnormally viscous intestinal secretions. These problems usually do not require surgical interventions and may be treated with GoLYTELY or Colyte (osmotic solutions given orally or by nasogastric tube), other laxatives, stool softeners, or rectal administration of diatrizoate meglumine (Gastrografin).

Rectal prolapse occurs in a small number of children with CF. The first episode of rectal prolapse is frightening to both parents and child. Its reduction usually requires immediate guidance and intervention, which is managed by simply guiding the rectum back into place with a gloved, lubricated finger with the child lying on her or his side. Further management usually involves attempting to decrease the bulk of daily stools through pancreatic enzyme replacement.

Children with CF often experience transient or chronic gastroesophageal reflux, which should be treated with the appropriate histamine-receptor antagonist and gastrointestinal motility drug, dietary modifications, and an upright position after feedings or meals (Hazle, 2010).

Management of Endocrine Problems: The management of CFRD is critical in the therapeutic treatment of the child with CF. CFRD presents a combination of insulin resistance and insulin deficiency, with unstable glucose homeostasis in the presence of acute lung infection and treatment. Children with CFRD require close monitoring of blood glucose, administration of oral glucose-lowering agents or insulin injections, and diet and exercise management; children with CF may be at increased risk for glucose management problems as a result of decreased nutrient absorption, anorexia, and severity of pulmonary illness. The prevalence of CFRD increases with age, and there is increased morbidity and mortality among children with CFRD compared to those without (Strausbaugh and Davis, 2007). Microvascular complications such as retinopathy and nephropathy may occur in children and adolescents with CFRD (Schwarzenberg, Thomas, Olsen, et al, 2007). However, ketoacidosis is reported to be rare in individuals with CFRD (Boat and Acton, 2007).

Bone health is of concern in children and adults with CF. The pancreatic insufficiency of CF and chronic steroid use present potential risks for less than optimum bone growth in such children. Assessment of bone health by history and bone mass density evaluation should be considered in assessing the child’s (≥8 years old) health status to detect and prevent osteoporosis or osteopenia (Borowitz, Baker, and Stallings, 2002).

The administration of growth hormone (somatropin [Nutropin]) is being investigated as a nutritional adjunct in children with CF to achieve optimum growth; one small study sample suggests an improvement in CF clinical status (Hardin, Rice, Ahn, et al, 2005). One randomized study indicates that the drug is well tolerated but does not result in short-term improvement of FEV in CF patients (Schnabel, Grasemann, Staab, et al, 2007).

Prognosis: In 1966 the median life expectancy for individuals with CF was 7.5 years. The median survival age for the CF patient in 2008 was 37.4 years, and approximately 45% of all patients with CF are 18 years old and older (Cystic Fibrosis Foundation, 2009b).

Lung, heart, pancreas, and liver transplantation have increased survival rates among some CF patients. Heart-lung and double-lung procedures have been successfully performed in children with advanced pulmonary vascular disease and hypoxia. In 2008 estimates of 5-year survival after transplantation were approximately 60% (Hazle, 2010). The obstacles surrounding this technique are availability of donated organs; complications from surgery; pulmonary infections; and recurrence of obstructive bronchiolitis, which decreases transplanted lung function.

Despite considerable progress and a recent surge in new treatment modalities, CF remains a progressive and incurable disease. The pulmonary involvement ultimately determines the patient’s outcome because pancreatic enzyme deficiency is less of a problem if adequate nutrition is ensured. With advances in technology, parents and adolescents are challenged to set future goals that may include college, careers, social relationships, and marriage. Concurrently they are faced with increasing morbidity and higher rates of CF complications as they grow older.

Hospital Care: Most patients with CF require hospitalization only for treatment of pulmonary infection, uncontrolled diabetes, or a coexisting medical problem that cannot be treated on an outpatient basis. Therefore, when patients with CF are hospitalized, implement Standard Precautions with meticulous hand washing to decrease the nosocomial spread of organisms to the CF patient and between hospitalized CF patients (especially when MRSA is prevalent). Contact Precautions may be required for specific infections.

When the child with CF is hospitalized for diagnosis or treatment of pulmonary complications, aerosol therapy, chest percussion therapy, and postural drainage are instituted or continued. Respiratory therapists often initiate, supervise, and provide these treatments; however, it is the nurse’s responsibility to monitor the patient’s tolerance to the procedure and evaluate its effectiveness in relation to treatment goals. The nurse may at times administer aerosol therapy, perform CPT, assist with airway clearance interventions such as the mechanical vest, and teach breathing exercises. CPT should not be performed before or immediately after meals. Planning CPT so that it does not coincide with meals is difficult in the hospital situation but is essential to the effectiveness of this treatment.

Administer supplemental oxygen therapy to the child with mild or moderate respiratory distress, and assess the child’s tolerance to the procedure. Noninvasive pulse oximetry provides valuable data about the patient’s oxygenation status, but nursing assessments, including observation of respiratory pattern, work of breathing, and lung auscultation, are vital.

One of the nursing challenges in the care of the child with CF is encouraging compliance with the therapeutic medication regimen, which often involves a significant number of medications; pancreatic enzymes; vitamins A, D, E, and K; oral antifungals for Candida infection; antihistamines; antiinflammatory agents; and oral antibiotics. This may be overwhelming to the child. Factor in multiple inhaled bronchodilators, CPT and aerosol treatments, blood glucose monitoring and insulin administration, various other medications, and increased mucus production during the acute phase, and it is not uncommon for the child with CF to rebel and be noncompliant with this regimen.

Gentle coaxing, positive reinforcement, and frank negotiation may be required to enlist cooperation for effective medication compliance. The child’s sleep is disrupted frequently by hospital routines; therefore nursing care should be flexible enough to allow him or her some quiet time without affecting vital care. In some cases a daily schedule of events, including medication administration, CPT, aerosolized therapy, and dressing changes, may need to be mutually developed with the child, nurses, and physician so that the child feels he or she has some control of the care.

The diet for the child with CF represents another challenge; careful planning with a pediatric dietitian and the child’s input may help decrease the loss of appetite and weight loss that are often part of the condition. Patients with CF, especially adolescents, enjoy foods brought from home or an occasional fast food of choice (provided these meet therapeutic requirements). Children in the early stages of CF often have a good appetite. With infection and increased lung involvement, their appetite diminishes, and eventually it becomes a challenge to tempt failing appetites. Age-appropriate nutrition education with specific nutritional goals for CF patients may increase compliance with prescribed enzyme therapy and nutritional supplements (Pitts, Flack, and Goodfellow, 2008).

When dietary intake fails to meet the child’s needs for growth, enteral feedings or supplements may be considered (Borowitz, Baker, and Stallings, 2002). These feedings may be administered via gastrostomy tube during the night to minimize the disruption of daily activities, including school. Additional pancreatic enzymes are required with supplemental feedings to obtain best results. A skin-level feeding gastrostomy affords the child few activity restrictions and minimum disruption of body image in comparison to a nasogastric tube or conventional gastrostomy tube. The child and parents are encouraged to perceive this therapy not as a last-ditch effort but as an adjunct therapy to maintain optimum growth and prevent excessive weight loss (Borowitz, Baker, and Stallings, 2002). In some cases adolescents are taught to insert a nasogastric tube for nighttime supplemental feedings; the tube may then be removed in the morning.

The child needs support during the many treatments and tests that are a part of the hospitalization. IV fluids, IV antibiotics and antifungals, and blood tests are almost always a part of the acute care treatment, and the child soon associates hospitalization with these stress-provoking procedures.

Providing support to both the child and the family is essential. The progressive nature of the disease makes each illness requiring hospitalization a potentially life-threatening event. Skilled nursing care and sympathetic attention to the emotional needs of the child and family help them cope with the stresses associated with repeated respiratory tract infections and hospitalizations.

The child or adolescent who is immobilized as a result of CF requires the same care and attention as the child with immobility from any other chronic or acute illness, including skin care, bowel management, passive range of motion, and positioning.

Home Care: Most children and adolescents with CF can be managed at home. The goals of care include normalization and daily activities, including school and peer involvement. The care plan should be flexible so that family activities are disrupted as little as possible. Parents may initially require assistance finding and contacting durable medical equipment companies that provide home care equipment. They also need opportunities to learn how to use the equipment and to solve problems they may encounter while delivering therapy at home. The many aspects of home care for the child with CF are similar to those of home care for other children. (See Chapter 25.)

Patients and family members need education about the preferred diet of nutritious meals with tolerated fat, increased protein and carbohydrate, and the administration of pancreatic enzymes. For infants and young children, the enzymes can be mixed with pureed fruit such as applesauce and fed with a spoon. Capsules are usually suitable for older children. It is important to stress to parents that the enzymes, in the amount regulated to the child’s needs, should be administered at the beginning of all meals and snacks.

One of the most important aspects of educating parents for home care is teaching techniques for airway clearance (CPT, mechanical vest, forced expiration) and breathing exercises. The success of a therapy program depends on conscientious performance of these treatments regularly as prescribed. The number of times these therapies are performed each day is determined on an individual basis, and often parents readily learn to adjust the number and intensity of the treatments to the child’s needs. For pulmonary infection, home IV antibiotics may be prescribed. Home IV care may be preferred for willing and competent families, since it reduces tension and usually brings a sense of belonging to the family members; however, this option depends on a number of factors, including availability of an agency with adequate staff to perform multiple daily home antibiotic infusions. With use of the venous access devices such as PICC lines and implanted ports, the parents and child learn the technique of direct administration into the IV line. Around-the-clock administration may be difficult for families and requires certain adjustments such as waking at least once during the night to give the drug.

For the child or adolescent with chronic sinusitis, daily nasal lavage with sterile normal saline may be helpful. Caution parents to avoid commercial saline preparations with benzyl alcohol, since these may burn the nasal mucosa (Manning, 2005). A dental jet irrigator may be used or a bulb syringe to inject the saline into the paranasal sinuses; slightly tilt the child’s head back for instillation, then have the child lean forward to drain the saline into an emesis basin or the bathroom sink. Adolescents need instruction on performing this procedure themselves.

Families also need information about medications and possible side effects. If a child is receiving ibuprofen, serum drug levels need to be monitored closely to establish therapeutic dosages, and observations for side effects such as gastrointestinal irritation are essential.

Children and adolescents with CF should receive routine primary care with special attention to diet, growth and development, and immunizations. Primary care providers should be alert to any weight loss or flattening in the growth curve associated with loss of appetite, which could indicate a pulmonary exacerbation in children with CF (Hazle, 2010). In addition to all the recommended routine immunizations, CF patients should be immunized against influenza starting at age 6 months and followed by an annual booster (American Academy of Pediatrics, 2009b). Anticipatory guidance concerning issues of discipline, how to incorporate aspects of the treatment regimen into the school environment, and delayed pubertal development are also important considerations for the primary care provider.

Home palliative care for the child or adolescent with CF who is in the terminal stages may be carried out with the assistance of hospice. (See Chapter 23.)

The nurse can assist the family in contacting resources that provide help to families with affected children. Various special child health services, many local clinics, private agencies, service clubs, and other community groups offer equipment and medications either free or at reduced rates. The Cystic Fibrosis Foundation* has chapters throughout the United States to provide education and services to families and professionals.

Family Support: The most challenging aspect of providing care for the family of a child or adolescent with CF is meeting the emotional needs of the child and family. The diagnosis, treatment, and prognosis for CF are often associated with many problems and frustrations. The diagnosis can evoke feelings of guilt and self-recrimination in parents.

The long-range problems for an infant, child, or adolescent with CF are those encountered in any chronic illness. (See Chapter 22.) Both the child and the family must make many adjustments, the success of which depends on their ability to cope and also on the quality and quantity of support they receive from outside sources. Combined efforts of a variety of health professionals are needed to provide the most comprehensive services to families. It is often the nurse who assesses the home situation, organizes and coordinates these services, and collects the data needed to evaluate the effectiveness of the services.

The persistent need for treatment several times a day places tremendous strain on the family. When the child is young, a family member must perform postural drainage and CPT. Children often balk at these treatments, and the parents are in the position of insisting on adherence. The stress and anxiety related to this routine may produce feelings of resentment in both the child and the family members. When possible, occasional trusted respite care should be available to allow parents to leave the situation for short periods without undue anxiety about the child’s welfare.

The affected child or adolescent may become resentful about the disease, its relentless routine of therapy, and the necessary restrictions it places on activities and relationships. The child’s activities are interrupted or built around treatments, medications, and diet. This imposes hardships and influences his or her quality of life. The nurse should encourage the child to attend school and join age-appropriate peer groups to live as normally and productively as possible. Sports are often an important part of the child’s and adolescent’s life; interaction with peers is a valuable life experience, especially to adolescents. The child or adolescent with CF should be encouraged to participate in sports activities as much as physical and pulmonary health allows. Exercise is encouraged to increase pulmonary vital capacity, promote muscle development, and enhance cardiovascular function.

Depression, anxiety, and disturbed self-image may occur in children and adolescents with CF; young adults with severe symptoms may be especially prone to depression as they face the poor prognosis and the reality of unmet life expectations and goals. Nurses should monitor adolescents with CF for signs of eating disturbances. One study found that 24% of the adolescents in the study group had eating disturbance, but most did not fit the criteria for anorexia or bulimia (Bryon, Shearer, and Davies, 2008). One study found that psychologic problems, including depression and anxiety, increased in adult CF patients with disease progression (Shanmugam, Bhutani, Khan, et al, 2007).

As the disease progresses, however, family stress should be expected, and the patient may become angry and noncompliant. It is important for the nurse to recognize the family’s changing needs and the grief they may experience as the CF worsens. Families should be made aware of resources for counseling. Patients need to be guided into activities that enable them to express anger, sorrow, and fear without guilt.

Transition to Adulthood: As life expectancy continues to rise for children and adolescents with CF, issues related to marriage, sexuality, childbearing, and career choice become more pressing. Males must be informed at some point that they will often be unable to produce offspring. It is important that the distinction be made between sterility and impotence. Normal sexual relationships can be expected. Female patients may be able to bear children but should be informed of the possible harmful effects on the respiratory system created by the burden of pregnancy. They also need to know that their children will be carriers of the CF gene; therefore genetic counseling for those planning on having children is essential. Adolescent females should be offered counseling concerning the use of oral contraceptives and other contraceptive options (Hazle, 2010).

Adolescents with CF should take personal ownership and management of the illness to maximize their life’s potential. Many adolescents and young persons with the illness enroll in college or vocational and technical training school and complete degrees by either distance learning or attending a local school. Young people should set life goals and live normal lives to the extent their illness allows. Adolescents who have had lung transplantation are encouraged to continue taking antirejection medications even though they may feel as though their respiratory status has improved to the point that these medications are no longer necessary.

Life as an independent adult should be encouraged for children with CF. From the time that children can take partial responsibility for their own care (e.g., CPT and taking enzymes), independence and accountability should be fostered. Although the prognosis for these children has improved, many will need continued support as they cope with the demands of surviving with CF.

Anticipatory grieving and other aspects related to care of a child with a terminal illness are also part of nursing care. For example, it is important to prepare the child and family members for end-of-life decisions and care. Families may need information about specific interventions such as hospice and treatments for pain and dyspnea. (See Chapter 23.)

1. Yes, there are sufficient data to arrive at a possible conclusion in this situation.

2. a. Epiglottitis is a serious obstructive inflammatory process that occurs in children 2 to 5 years of age.

3. The suspicion of epiglottitis constitutes an emergency. The priority for nursing care at this time is to maintain the child’s airway.

4. Yes, the evidence supports the conclusion.