Prognosis.: Most children recover from primary TB infection and are often unaware of its presence. However, very young children have a higher incidence of disseminated disease. TB is a serious disease during the first 2 years of life, during adolescence, and in children who are HIV positive. Except in cases of tuberculous meningitis, death seldom occurs in treated children. Antibiotic therapy has decreased the death rate and the hematogenous spread from primary lesions.

Prevention.: The only definite means to prevent TB is to avoid contact with the tubercle bacillus. Maintaining an optimal state of health with adequate nutrition and avoiding fatigue and debilitating infections promote natural resistance but do not prevent infection. Pasteurization and routine testing of milk and elimination of diseased cattle have reduced the incidence of bovine TB.

Limited immunity can be produced by administration of BCG (bacille Calmette-Guérin), a live vaccine containing bovine bacilli with reduced virulence (attenuated). In most instances, positive tuberculin reactions develop after inoculation with BCG. The distribution of BCG is controlled by local or state health departments, and the vaccine is not used extensively, even in areas with a high prevalence of disease. BCG vaccination is not generally recommended for use in the United States. However, it may be recommended for long-term protection of infants and children with negative TST who are not infected with HIV and who (1) are at high risk for continuing exposure to persons with infectious pulmonary TB or (2) are continuously exposed to persons with TB who have bacilli resistant to both INH and rifampin when the child cannot be removed from the environment or given antituberculosis drug therapy (American Academy of Pediatrics, 2006b).

Prevention.: Nurses are in a position to teach prevention in a variety of settings. 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. Parents should be cautioned about behaviors that their children might imitate (e.g., holding foreign objects, such as pins, nails, and toothpicks, in their lips or mouth). (Prevention based on the child’s age is discussed in Chapters 10 and 12.)

Prognosis.: In spite of advances in understanding and treating ARDS and ALI, mortality in children ranges from 22% (after severe trauma) to 88% (after bone marrow transplant) (Flori, Glidden, Rutherford, and others, 2005; Frankel and DiCarlo, 2004). The precipitating disorder influences the outcome; the worst prognosis is associated with uncontrolled sepsis, bone marrow transplantation, cancer, and multisystem involvement with hepatic failure. Children who recover may have persistent cough and exertional dyspnea.

 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 to 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, and others, 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. Exposure to tobacco smoke is a significant contributing factor in the development of asthma in infants and small children (Sharma, Hansel, Matsui, and others, 2007). Recommendations for controlling allergens are found in the Family-Centered Care box.

Skin testing identifies specific allergens so steps can be 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) will decrease the frequency of asthma episodes. Dehumidifiers or air conditioners control nonspecific factors that trigger an episode, such as extremes of temperature.

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, and others, 2004; Sharma, Hansel, Matsui, and others, 2007).

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. 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 (HFAs); the purported advantages include delivery of more fine particles and less oral deposition (Pongracic, 2003). The FDA mandated December 2008 as the targeted cessation date for MDIs containing CFCs to be produced and sold (US Food and Drug Administration, 2005). 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 very young children who have difficulty using MDIs or other inhalers can receive their asthma medications via a nebulizer. When this device is used, the medication is mixed with saline (also available in premixed form) and nebulized with compressed air. Children are instructed to 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 to 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 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 of time (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. These medications 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 indicate that the use of inhaled corticosteroids at recommended doses 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; it 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 exercise-induced bronchospasm. 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). It is believed that 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 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-dosage inhaled corticosteroid to improve lung function and asthma symptoms and decrease the need for a short-acting β₂-agonist. There is some evidence that this combination may actually enable the practitioner to lower the corticosteroid dose and manage asthma symptoms just as effectively (Mintz, 2004). Inhaled β-adrenergic agents should not be taken more than three or four times daily for acute symptoms.

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 exercise-induced bronchospasm. Salmeterol is not used in children less than 12 years of age, and it is not used to treat acute symptoms or exacerbations.

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, since 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 [Singulair]) 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 zafirkulast is approved for children 7 years and older.

Anticholinergics (atropine and ipratropium [Atrovent]) may also be used for relief of 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. Ipratropium, when used in combination with albuterol, has been shown to be effective during acute severe asthma in significantly improving lung function and reducing hospitalizations in children coming to the emergency department (Liu, Spahn, and Leung, 2004).

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. The drug is expensive (Courtney, McCarter, and Pollart, 2005), however, 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.

The use of complementary and alternative medicine (CAM) in children with asthma is reported by several sources; most common are the use of herbal products, breathing techniques, homeopathy, and acupuncture (Slader, Reddel, Jenkins, and others, 2006). The use of CAM should be carefully evaluated in conjunction with other therapies in the overall management of asthma.

Exercise.: 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 is rare 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, since 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. Participation should be evaluated on an individual basis. Appropriate prophylactic treatment with β-adrenergic agents or cromolyn sodium before exercise will usually permit full participation in strenuous exertion.

Chest Physical Therapy.: 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 are of value in preventing overinflation and improving efficiency of the cough. However, CPT is not recommended during acute, uncomplicated exacerbations of asthma.

Hyposensitization.: The role of hyposensitization in childhood asthma has become 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 is reinstituted. Hyposensitization injections should be administered only with emergency equipment and medications readily available in the event of an anaphylactic reaction.

Prognosis.: 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 (Ross, Mjaanes, and Lemanske, 2003). Many children who outgrow their exacerbations continue to have airway hyperresponsivenesss and cough as adults. Furthermore, airway hyperresponsiveness in adults appears to be associated with decreased lung function.

Although deaths from asthma have been relatively uncommon, especially in the young age-groups, since the 1980s the rate of death from asthma has increased steadily in the United States and other countries. Data indicate a significant increase in asthma symptoms, emergency department visits, and hospitalization among children ages birth to 4 years of age in 2003 to 2004. Most asthma deaths for the same period were in children ages 11 to 17 years, with significant increases seen in non-Hispanic African-Americans (Linzer, 2007). 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, Spahn, and Leung, 2004).

The adolescent age-group appears to be the most vulnerable, with the greatest increase 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.

Status Asthmaticus.: Children who continue to display respiratory distress despite vigorous therapeutic measures, especially the use of sympathomimetics (e.g., albuterol, epinephrine), are considered to be in status asthmaticus. The condition may develop gradually or rapidly, often coincident with complicating conditions, such as pneumonia or a respiratory virus, that 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 been shown to result 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 acts to decrease 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; heliox can be delivered via a nonrebreathing face mask from premixed tanks, which may be blended in a stand-alone unit or within a ventilator. Heliox 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 (Liu, Spahn, and Leung, 2004). 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. Inhaled magnesium sulfate used in addition to a β₂-agonist for acute asthma attacks has also been effective in treating acute asthma exacerbation (Blitz, Blitz, Beasley, and others, 2005).

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 a 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.

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) (see Family-Centered Care box, p. 787). Parents are cautioned to avoid exposing a sensitive child to excessive cold, wind, or other extremes of weather; smoke; sprays; or other irritants. Foods known to provoke symptoms should be eliminated from the diet.

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).

Relieve Bronchospasm.: Parents and older children are taught 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. A variety of easy-to-use, inexpensive PEFMs are available for use in the home and at school to assess changes in pulmonary function (see Family-Centered Care box). 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 peak flow meter over time, and they should bring their peak flow meter 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, and others, 2007).

Children who use a nebulizer, MDI, Diskus, or Turbuhaler to deliver drugs need to learn how to use the device correctly. A study of school-age children with asthma indicated that only 7% of these children had effective MDI skills (Winkelstein, Huss, Butz, and others, 2000). The MDI device (Fig. 23-5) 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 coordinate breathing should use spacers. 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. Spacers also prevent yeast infections in the mouth when corticosteroids are inhaled via an MDI.

The child and parents also need to be cautioned 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. Parents are cautioned 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. 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 the written action plan, emphasizing that the child and family determine the success of the plan, not the health professionals.

Foods known to provoke symptoms should be eliminated from the diet, and parents are advised to read labels on prepared foods and snacks to determine the presence of allergens.

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, 2008). Equipment used for the child, such as nebulizers, must be kept absolutely clean to decrease the chances of contamination with bacteria and fungi.

Breathing exercises and controlled breathing are taught and encouraged for motivated children, and the nurse should 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.

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.^§

Provide 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. The importance of continual observation and assessment cannot be overemphasized.

When β₂-agonists and corticosteroids are given, the child is monitored closely and continuously for relief of respiratory distress and signs of side effects or toxicity. Oral fluid intake may be limited during the acute phase; IV fluid replacement may be required to provide adequate tissue hydration.

Older children may be more comfortable standing (Fig. 23-6), sitting upright, or leaning slightly forward (Fig. 23-7). When possible, the nurse communicates in such a way that a child can reply in a few words to avoid fatigue. Shortness of breath makes talking difficult.

Children with acute asthma are apprehensive and anxious. The calm, efficient presence of a nurse helps reassure them that they are safe and will be cared for during this stressful period. It is important to 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 will also reduce the child’s distress. Anxiety is easily communicated to the child from parents and members of the staff.

Support Child or Adolescent and Family.: 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 in these situations where the child’s or adolescent’s life is potentially in harm’s way 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, parents are encouraged to promote as normal a life as possible for their children.

Management of Pulmonary Problems.: Management of pulmonary problems is directed toward prevention and treatment of pulmonary infection by improving ventilation, removing mucopurulent secretions, and administering antimicrobial agents. Many children will 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. 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, and others, 2007). Fungal colonization with Candida or Aspergillus organisms in the respiratory tract is also common in CF patients.

Prevention of infection involves a daily routine of CPT to maintain pulmonary hygiene. 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. 23-9). It has the advantage of increasing sputum expectoration and being used without an assistant. Hand-held 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. Several studies indicate that 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 daily via nebulization, has resulted in improvements in spirometry, PFTs, dyspnea scores, and perceptions of well-being and has reduced the viscosity of sputum.

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.

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. 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 22). With repeated infection and inflammation, bronchial cysts and emphysema may develop. These cysts may rupture, resulting in a pneumothorax.

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.

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 for prolonged periods has been associated with linear growth restriction, glucose tolerance abnormalities, and cataract formation. Antiinflammatory medications such as ibuprofen are becoming more important in the treatment of CF, but careful monitoring for adverse effects (gastrointestinal bleeding) is essential.

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 a smaller amount is 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.

Children with CF require a well-balanced, high-protein, high-caloric diet (because of the impaired intestinal absorption). In fact, they often require up to 150% of the recommended daily allowances to meet their needs for growth. Breastfeeding with enzyme supplementation should be continued whenever possible for parents who prefer this method and, when necessary, supplemented with a higher-calorie-per-ounce formula. For formula-fed infants, commercial cow’s milk—based formulas are usually adequate, although frequently a partially hydrolysated formula with medium-chain triglycerides (e.g., Pregestimil, Alimentum) may be recommended. Enzymes are mixed into cereal or fruit, such as applesauce. 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 enzymes. When high-fat foods are eaten, the child is encouraged to add extra enzymes. Growth failure despite adequate nutritional support may indicate deterioration of pulmonary status. 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 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.

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 tubes), other laxatives, stool softeners, or rectal administration of meglumine diatrizoate (Gastrografin).

Rectal prolapse occurs in about 20% to 25% of children with CF (McMullen and Bryson, 2004). 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. Further management usually involves attempting to decrease the bulk of daily stools through 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 (McMullen and Bryson, 2004).

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, and others, 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 and older) 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, and others, 2005).

Prognosis.: The median predicted survival age for the CF patient in 2005 was 36.5 years (Cystic Fibrosis Foundation, 2006). 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. 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, standard precautions with meticulous hand washing should be implemented 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 the effectiveness of the procedure in relation to treatment goals. The nurse may at times administer aerosol therapy, perform CPT, assist with mucus removal 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 essential to the effectiveness of this treatment.

Supplemental oxygen therapy is administered to the child with mild or moderate respiratory distress, and the child requires frequent assessment of the tolerance to the procedure. Noninvasive pulse oximetry provides valuable data about the patient’s oxygenation status, yet nursing assessments, including observation of respiratory pattern, work of breathing, and lung auscultation, are vital assessments.

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 the child 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 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, and 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. 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. 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 not perceive this therapy as a last-ditch effort but as an adjunct therapy to maintain optimum growth and prevent excessive weight loss (Borowitz, Baker, and Stallings, 2002).

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.

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 a result of the realization of the poor prognosis and the reality of unmet life expectations and goals.

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 care of 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 will 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 and are discussed in Chapter 20.

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 the removal of mucus (CPT, 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 because it reduces tension and usually brings a sense of belonging to the family members; this option, however, 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 can be taught 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.

Families also need information about medications and possible side effects. Children receiving multiple antibiotics may require serum drug levels to ensure therapeutic dosaging.

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 (McMullen and Bryson, 2004). In addition to all the recommended routine immunizations, CF patients should be immunized against influenza starting at age 6 months and followed by a yearly booster (American Academy of Pediatrics, 2006b). 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 18).

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 often 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 18). 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 placed 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 curtailment it places on activities and relationships. The child’s activities are interrupted or built around treatments, medications, and diet. This imposes hardships and influences the child’s quality of life. The child should be encouraged to attend school and to join age-appropriate peer groups to foster a life that is as normal and productive as possible. Sports are often an important part of the child and adolescent’s life; interaction with peers are valuable life experiences, especially to adolescents. The child or adolescent with CF should be encouraged to participate in sports activities inasmuch as physical and pulmonary health allows. Exercise is encouraged to increase pulmonary vital capacity, promote muscle development, and enhance cardiovascular function.

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 sources for counseling. Patients need to be guided into activities that enable them to express anger, sorrow, and fear without guilt (see Ethical Case Study).

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 deleterious 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. Adolescent females may need counseling concerning the use of oral contraceptives and other contraceptive options (McMullen and Bryson, 2004).

Adolescents with CF are encouraged to 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 either by distance learning or by attending a local school. Young people are encouraged to set life goals and live normal lives to the extent their illness allows.

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.

1. Presence or history of a condition that might predispose the patient to respiratory failure

2. Observation of respiratory failure

3. Measurement of ABGs and pH

Open the Airway.: For effective CPR the victim is placed on the back on a firm, flat surface, employing appropriate precautions. With loss of consciousness, the tongue, which is attached to the lower jaw, relaxes and falls back, obstructing the airway. To open the airway, the head is positioned with a head tilt—chin lift maneuver by the lay rescuer. Health professionals should open the airway using either a head tilt—chin lift or jaw thrust maneuver. A head tilt is accomplished by placing one hand on the victim’s forehead and applying firm, backward pressure with the palm to tilt the head back. The fingers of the free hand are placed under the bony portion of the lower jaw near the chin to lift and bring the chin forward (chin lift). This supports the jaw and helps tilt the head back (Fig. 23-11).

The jaw thrust is accomplished by grasping the angles of the victim’s lower jaw and lifting with both hands, one on each side, displacing the mandible upward and outward. The jaw thrust is recommended only for health care workers. In suspected neck injuries the jaw thrust method should be used while the cervical spine is completely immobilized. After a patent airway has been restored by removal of foreign material and secretions (if indicated), and if the child is not breathing, maintenance of the airway is continued and rescue breathing is initiated.

Give Breaths.: To ventilate the lungs in the infant (from birth to 1 year of age), the bag-valve mask (BVM) or operator’s mouth is placed in such a way that both the mouth and the nostrils are covered (Fig. 23-12). Children (over 1 year of age) are ventilated through the mouth while the nostrils are firmly pinched for airtight contact.

The volume of air in an infant’s lungs is small, and the air passages are considerably smaller, with resistance to flow potentially higher than in adults. The rescuer should deliver small puffs of air and assess the rise of the chest to ensure that overinflation does not occur. A gentle rise of the chest is a sufficient indicator of adequate inflation. If air enters freely and the chest rises, the airway is assumed to be clear. Breaths should be given slowly with sufficient volume to make the chest rise.

Pulse Check.: After an initial two breaths, the health care provider palpates the pulse to ascertain the presence of a heartbeat. The carotid is the most central and accessible artery in children over 1 year of age. However, the infant’s short and often fat neck makes the carotid pulse difficult to palpate. Therefore in the infant it is preferable to use the brachial pulse, located on the inner side of the upper arm midway between the elbow and the shoulder (Fig. 23-13). Absence of a carotid or brachial pulse is considered sufficient indication to begin external cardiac massage. Lay rescuers are not taught to check the pulse but are taught to look for signs of circulation (e.g., normal breathing, coughing, or air movement) in response to rescue breaths.

Chest Compression.: External chest compression consists of serial, rhythmic compressions of the chest to maintain circulation to vital organs until the child achieves spontaneous vital signs or ALS can be provided. Chest compressions are always interspersed with ventilation of the lungs.^* For optimal compressions, it is essential that the child’s spine is supported on a firm surface during compressions of the sternum and that sternal pressure is forceful but not traumatic. For a small infant the hard surface can be the rescuer’s hand or forearm, with the palm supporting the infant’s back. The child’s head is positioned for optimal airway opening using the head tilt—chin lift maneuver. It is essential to prevent overextension of the head of small infants, since this tends to close the flexible trachea.

The placement of the fingers for compression in infants is at a point on the lower sternum just below the intersection of the sternum and an imaginary line drawn between the nipples (Fig. 23-14). Compressions on the child 1 to 8 years of age are applied to the lower half of the sternum (Fig. 23-15). Sternal compression to infants is applied with two fingers on the sternum, exerting a firm downward thrust; for children, pressure is applied with the heel of one hand or two hands, depending on the child’s size. Current American Heart Association (2005) guidelines include the addition of the two-thumb technique for chest compressions for infants when two health care providers are present. In the two-thumb technique, one of the two rescuers places both thumbs side by side over the lower half of the infant’s sternum; the remaining fingers encircle the infant’s chest and support the back. The two-thumb technique is not taught to lay rescuers and is not practical for the health care provider working alone.

The depth of compression is adapted to the child’s size. The location, rate, and depth for children older than 8 years of age are the same as for adults.

Lone-rescuer CPR is continued at the ratio of two breaths to 30 compressions for all ages until signs of recovery appear. These signs include palpable peripheral pulses, return of pupils to normal size, the disappearance of mottling and cyanosis, and possibly return of spontaneous respiration. When two rescuers are present, they should deliver two breaths to each 15 compressions. The lay rescuer is not taught two-rescuer CPR according to the new guidelines (American Heart Association, 2005).

Medications.: Medications are an important adjunct to CPR, especially cardiac arrest, and are used during and after resuscitation in children. Medications are used to (1) correct hypoxemia, (2) increase perfusion pressure during chest compression, (3) stimulate spontaneous or more forceful myocardial contraction, (4) accelerate cardiac rate, (5) correct metabolic acidosis, and (6) suppress ventricular ectopy (Table 23-3).

Appropriate fluid therapy is initiated immediately in the hospital or by EMS personnel during transport (see Parenteral Fluid Therapy, Chapter 22, and Shock, Chapter 25). A complete supply of emergency medications is kept and maintained in all EMS vehicles and on all hospital units. The supply is checked on a regular basis (usually once on each 8- or 12-hour shift). When administering drugs during CPR (or a “code”), use a saline flush between medications to prevent drug interactions. Document all drugs, dosages, and the time and route of administration.