Although there are many PCD phenotypes (Bush et al., 2007), only a few are commonly observed. They include the lack of outer dynein arms, a combination of missing inner and outer arms, isolated missing inner arms, or lack of inner arms combined with a radial spoke defect. Rare cases have described a transposition error of the central microtubular pair or a lack of the central microtubular pair. These structural abnormalities are associated with abnormal ciliary movement, which can range from no movement to ineffective movement, such as a windshield wiper or an eggbeater movement (Schidlow & Steinfeld, 2005). Subsequently, the ineffective ciliary movement results in poor airway clearance of mucus, which then leads to recurrent bacterial infections of the airways.

KS is a distinct phenotype of PCD, which comprises situs inversus totalis, recurrent sinusitis, and bronchiectasis (Dell, 2008). KS accounts for half of PCD cases (Schidlow & Steinfeld, 2005). Situs inversus, the complete reversal of the abdominal and thoracic organs so that they appear as a mirror image when examined or visualized on a radiograph, is thought to be due to the dysfunction of embryonic node monocilia (Nonaka et al., 1998). More specifically, Nonaka et al. (1998) identified the KIF3B gene, which aids in the creation of motile cilia that participate in the left to right asymmetrical arrangement through nodal flow inside the yolk sac cavity. Nonaka et al. (1998) also observed that in normal mice, nodal cilia are motile in a counterclockwise direction, causing motion of the extra embryonal fluid in a leftward direction. Thus, the rotating movement of the nodal cilia creates asymmetry in the body (Afzelius, 1999), and if these cilia are defective, the normal asymmetry of the internal organs is not established. Furthermore, almost all males with KS are infertile due to the abnormal movement of spermatozoa (Schidlow & Steinfeld, 2005).

Signs and Symptoms

Associated findings of PCD differ among age groups. Prenatal presentation is described as the presence of mirror image organ arrangement on antenatal ultrasound (Bush et al., 2007). Presentation during the newborn period includes rhinorrhea from the first day, respiratory distress or neonatal pneumonia without any predisposing cause, and positive screening due to positive family history (Bush et al., 2007). Signs and symptoms of PCD that are common in any pediatric age group include rhinitis, cough, and otitis media with effusion (Bush et al., 2007).

Since classical findings (rhinorrhea, recurrent ear infections, cough, and recurrent pneumonia) associated with PCD are common childhood complaints, the diagnosis may be delayed unless a thorough review of history is obtained. Coren, Meeks, Morrison, Buchdahl, and Bush (2002) reported that the median age of diagnosis of patients with PCD was 4 years with a range from 0 to 14 years. Twelve out of the 55 children had bronchiectasis, chronic lung damage, at the time of diagnosis, even though 6 of the children with bronchiectasis had neonatal symptoms and 7 had abnormal situs. In addition, 8 of the 12 had a chronic productive cough. Of the 25 children with abnormal situs and a history of neonatal symptoms, only 13 were diagnosed before 1 year of age and the other 12 received a diagnosis between 4 and 10 years of life. Similarly, in a retrospective review by Jain et al. (2007), only 8 of the 89 patients were diagnosed as neonates, although 43% of them had a history of neonatal respiratory distress.

PCD should be considered in the differential diagnosis for any child with recurrent or persistent pneumonia especially if bronchiectasis is identified on chest X-ray or high-resolution computed tomography (HRCT). Additionally, PCD should be considered in the differential diagnosis of a child with poorly controlled asthma, persistent ear discharge despite myringotomy tubes, or a child with hearing loss (Barbato et al., 2009; Bush et al., 2007).

Although adolescents and adults may have a history of childhood symptoms, they may not be identified as having PCD until they present with fertility issues such as ectopic pregnancy, female subfertility, and male infertility (Bush et al., 2007; Halbert, Patton, Zarutskie, & Soules, 1997). Afzelius and Eliasson (1983) studied fertility among a group of adult males with immotile cilia syndrome and noted that all subjects were infertile. While some had immotile spermatozoa, others had sperms with abnormal appearance, such as coiled sperm tails. In adolescents and adults, additional presenting signs and symptoms include digital clubbing related to chronic pulmonary insufficiency, nasal polyposis, and halitosis from recurrent sinusitis (Barbato et al., 2009).

Repeated lung infections in undiagnosed cases will cause deterioration of lung function as patients age. In a report by Noone et al. (2004), 47 adults with PCD had a mean forced expiratory volume in 1 second (FEV₁) of 60% predicted compared to 31 children younger than 18 years of age who had a mean FEV₁ of 85%. Additionally, Ellerman and Bisgaard (1997) demonstrated deterioration in lung function over time in a prospective study. In this study, children and adults were followed over a 2- to 16-year time period. The forced vital capacity (FVC) of children ranged from 69 to 117%; for adults, the range was 30–104%. The FEV₁ for children ranged from 53 to 107% compared to 16–99% for adults, suggesting that lung function declines over time with progression of disease (Ellerman and Bisgaard, 1997).

Diagnosis

There are a variety of diagnostic modalities for ciliary dyskinesia; however, electron microscopy remains the gold standard for detecting ultrastructural defects in the cilia (Dell, 2008). Other available diagnostic tests include ciliary beat frequency measurement, ciliary beat pattern analysis, measurement of ciliary disorientation under electron microscopy, and cell culture with regrowth of ciliated epithelium in difficult cases (Bush et al., 2007).

Genetic Testing

Genetic testing for the specific mutations of DNAH5 and DNAI1 is also available. However, genetic testing alone is of limited clinical use because testing cannot identify the majority of PCD cases. Mutations in DNAI1, the first gene identified in which mutations were responsible for PCD (Morillas, Zariwala, & Knowles, 2007), accounts for less than 25% of PCD cases (Dell, 2008). More recently, Barbato et al. (2009) listed mutations in DNAHI1, DNA12, KTU, RSPH9, and RSPH4A as being associated with PCD. Other mutations associated with PCD have also been linked to other genetic diseases. The RPGR mutation is also associated with X-linked recessive retinitis pigmentosa and sensory hearing deficits (Barbato et al., 2009). Furthermore, a syndrome of PCD and mental retardation is associated with mutations in OFD1.

Nitric Oxide Measurement

Identifying a biomarker that could be measured noninvasively and reproducibly has been sought after for the PCD population. Although the measuring fraction of exhaled nitric oxide (FeNO) was initially examined, it is the measurement of nasal nitric oxide (NO) that has been shown to be particularly helpful in this disorder. Nitric oxide in the airways is the result of the enzymatic conversion of amino acids by NO synthase (Moncada & Higgs, 1993). An isoform of this enzyme is inducible NO synthase (iNOS). It is found mostly in bronchial epithelium in healthy people as well as in asthmatics (Abba, 2010). Nasal NO is extremely low in subjects with PCD (Karadag, James, Gultekin, Wilson, & Bush, 1999; Lundberg et al., 1994). These low levels of NO are mostly related to reduced activity of bronchial iNOS in patients with PCD (Paraskakis, Zihif, & Bush, 2007; Shoemark & Wilson, 2009). Alveolar NO is lower than bronchial NO in PCD (Paraskakis et al., 2007).

Nasal NO is collected from the upper airway via a catheter that is inserted into a nostril. The nasal air is analyzed via a nitric oxide analyzer that uses ozone-based NO₂ chemiluminescence for direct measurement of nitric oxide (Bush et al., 1998). The preferred method to measure NO in children older than 4–5 years of age is by the single-breath online method (American Thoracic Society & European Respiratory Society, 2005). The duration of exhalation must reach at least 4 seconds. As explained by Leigh, Zariwala, and Knowles (2009), the patient must be able to follow directions and proper technique to close the soft palate to limit nasal NO from the lower airway sample. The use of NO measurement may be a good screening tool, but further study is required for test standardization (Leigh et al., 2009).

Saccharin Test

The saccharin test, used historically, involves placing a saccharin tablet on the inferior turbinate; the patient then sits forward and the timing of the taste of the saccharin is recorded. The patient is asked to sit while looking downward without sniffing, sneezing, coughing, eating, or drinking for the hour-long test. If the patient detects the taste of saccharin after 60 minutes an abnormality is detected. A positive saccharin test is usually followed by further diagnostics and workup, including a biopsy to confirm diagnosis. Given the difficulty to perform, this test does not lend itself well to the pediatric population.

Complications

Abnormal cilia, abnormal ciliary motion, and poor mucus clearance result in the collection of mucus in the airway, which becomes infected (Weinberger & Abu-Hasan, 2007). Repeated infection with organisms such as Haemophilus influenzae and Pseudomonas aeruginosa (Schidlow & Steinfeld, 2005) can lead to pneumonia in the majority of patients (Jain et al., 2007). Recurrent pneumonic infections lead to bronchiectasis (Schidlow & Steinfeld, 2005). Bronchiectasis is a chronic condition characterized by dilated bronchi, which have been thickened, inflamed, and provide an excellent milieu for bacterial colonization or overgrowth (Li et al., 2005). Although bronchiectasis can be expected with delayed diagnosis, it has also been reported in young pediatric patients. In a case report by Brown, Pittman, Leigh, Forman, and Davis (2008), a 6-month-old with PCD and a 33-month-old with PCD both had evidence of bronchiectasis on chest CT.

Mild hemoptysis or blood-streaked mucus may result from coughing through an inflamed airway (Emmons, 2009). A more worrisome but very rare complication is massive hemoptysis, where blood loss exceeds 600 mL in 1 day; although rare, it may be life threatening. Massive hemoptysis usually occurs when there is irritation and infection of collateral vessels that may develop in long-term disease and usually requires emergency embolization (Barker, 2002).

Cilial dysfunction results in chronic infection of both the lower and upper airways. Recurrent infection leading to irreversible lung damage results in loss of lung function over time (Schidlow & Steinfeld, 2005). Children with PCD may also experience sinusitis, rhinorrhea, and hearing difficulty due to fluid buildup in the ear (Barnes, 2009). The lack of normal cilia function often results in chronic sinusitis, chronic rhinitis, rhinopolypsis, frontal sinus abnormalities, and nasosinus aplasia (Barbato et al., 2009; Bi et al., 2010).

Management

Appropriate and timely screening is of great importance with PCD. Aggressive treatment and early institution of airway clearance will prevent progressive loss of lung function and development of bronchiectasis (Barbato et al., 2009). Early diagnosis may also prevent unnecessary ear, nose, and throat procedures and thoracic surgery (Bush et al., 1998). Timely audiology exams are necessary to treat deafness (Bush et al., 1998).

Many PCD therapies have been extrapolated from the clinical guidelines compiled for the management of CF, another genetic disorder that is also associated with chronic obstructive lung disease, bronchiectasis, and low nasal NO. However, the evidence to support their use in patients with PCD is not available. These guidelines recommend that diagnostics at follow-up should include pulmonary function tests (PFTs) and sputum cultures every 3–6 months and yearly, or biannual lung imaging (Barbato et al., 2009; Ellerman and Bisgaard, 1997; Leigh et al., 2009). Patients with PCD should receive all childhood immunizations and a yearly influenza vaccine (Schidlow & Steinfeld, 2005).

Pharmacological Treatment

High doses of antibiotics should be used to treat lung infections. Prophylactic antibiotics may be needed if repeated courses of oral antibiotics have been required and the patient is experiencing respiratory or pulmonary function decline (Bush et al., 2007). If P. aeruginosa is isolated on sputum cultures, long-term antipseudomonal nebulized antibiotics to prevent exacerbations should be considered (Barbato et al., 2009; Bush et al., 2007). One such agent, Tobi^®, a nebulized tobramycin preparation specifically designed for inhalation is available in 300-mg vials and is dosed twice a day. In CF patients, the drug is administered in cycles of 28 days on medication followed by 28 days off (Emmons, 2009). In some instances, respiratory symptoms and pulmonary function may not improve despite frequent oral or inhaled antibiotics; in these cases, a course of intravenous antibiotic therapy is recommended (Bush et al., 2007).

Airway Clearance

Although there is no evidence that chest physiotherapy is beneficial in PCD, it is often recommended to promote airway clearance of mucus. There are several options for airway clearance available (Bush et al., 2007). Physical exercise may help with airway clearance (Barbato et al., 2009). In addition, patients may use blowing games (such as blowing bubbles), active cycle breathing (Bush et al., 2007), high-frequency chest wall oscillation (vest therapy), oscillating positive expiratory pressure (PEP) (Acapella^®), and huffing to aid in airway clearance.

For huffing, the patient should inhale deeply and hold for a few seconds, followed by two “huff” coughs. As described by Berman, Snyder, Kozier, and Erb (2008), to use huff coughing, the patient should lean forward and exhale forcefully as if with a huff. This allows airways to stay open while clearing mucus out. To prevent mucus from moving back down into the smaller airways, one should follow with short rapid inhalations or “sniffing” (Berman et al., 2008).

A PEP mask or mouthpiece or an oscillating PEP device such as the Acapella (Bush et al., 2007; Volsko, DiFiore & Chatburn, 2003) can be used by itself or in conjunction with nebulized medications to optimize medication delivery to the lower airways. With either device, the goal is to generate 10–25 cm of H₂O of end expiratory pressure, which will stimulate mucus to move out of small airways (Pruitt & Jacobs, 2005). PEP allows for a prolonged expiration period to help open distal airways, thereby increasing available air to cough more effectively and move secretions (Pruitt & Jacobs, 2005).

High-frequency chest wall oscillation therapy is also believed to be beneficial in the treatment of PCD. It is delivered via a nonstretchable vest sized to fit the individual patient’s chest. It speeds the clearance of mucus from the peripheral and central airways (Braverman & Stewart, 2007) by delivering oscillating pressures to stimulate the cilia activity (Pruitt & Jacobs, 2005). With rigorous airway clearance, the airways are exposed to irritants for shorter periods of time, thus decreasing inflammation, infections, and bacterial colonization (Braverman & Stewart, 2007).

Although mucolytic agents such as Pulmozyme^® (rhDNAase), Mucomyst^® (acetylcysteine), and hypertonic saline can be used along with airway clearance techniques, there is little to no evidence to support their efficacy in this patient population. There is a report of hypertonic saline and rhDNAse resulting in symptom relief in PCD patients (Desai, Weller, & Spencer, 1995; Ten Berge, Brinkhorst, Kroon, & Jongste, 1999). Saline or hypertonic saline in nebulized form has been reported to increase mucus clearance in PCD patients (Barbato et al., 2009; Bush et al., 2007). Hyperosmolar saline is believed to help clear mucus by increasing the movement of water across the lung surface (Amirav, Cohen-Cymberknoh, Shoseyov, & Kerem, 2009) and has been used with an Acapella or a vest to augment airway clearance. Although it has been used in CF patients for decades, Mucomyst (acetylcysteine) was not found to be useful for the treatment of PCD (Stafanger, Garne, Howitz, Morkassel & Koch, 1988). This finding suggests that assuming therapies used in CF should work in PCD may not be a clinically sound approach to therapy.

Imaging

Signs of chronic lung infections on chest radiographs include atelectasis, infiltrates, and hyperinflation (Schidlow & Steinfeld, 2005), all of which can be observed in patients with PCD, as well as bronchiectasis. Other radiographic findings include focal pneumonitis or irregular opacities (Barker, 2002).

In a recent study by Jain et al. (2007), the radiographs of 55 pediatric PCD patients among a cohort of 89 PCD patients were reviewed for abnormalities. Thirty-one patients had dextrocardia; 27 were remarkable for hyperinflation; 54 had bronchial wall thickening and dilatation; 29 had mottled shadows; and 3 had consolidation or collapse, suggesting that there is a wide variety of potential radiographic findings. Additionally, the presence of nodular-cystic lesions or peribronchial thickening on chest X-rays suggests more advanced lung disease and raises suspicion of bronchiectasis, which should be confirmed with HRCT of the chest (Schidlow & Steinfeld, 2005).

Bronchoscopy

Patients may undergo bronchoscopy for direct visualization of the airways to obtain bacterial or fungal cultures and to mechanically clear airways. Bronchoscopy may also document reversed lung anatomy in patients with situs inversus (Sharma, 2008).

Surgical Treatment

Persistent sinusitis may require endoscopic sinus surgery or removal of nasal polyps (Sharma, 2008). Nasal sprays and saline rinses may be effective treatment for the sinusitis and rhinorrhea prior to surgery (Barnes, 2009).

Lung transplantation may be considered in cases with severe end-stage lung disease where the FEV₁ is <40%. Other surgical interventions may need to be considered such as lobectomy for focal disease where bronchiectasis has caused the lung tissue to lose function and to become unresponsive to conventional medical treatment (Emmons, 2009; Sharma, 2008).

Otolaryngological Treatment

Recently, Campbell, Birman, and Morgan (2009) reviewed the literature on the efficacy of myringotomy tube placement in children with PCD who were experiencing otitis media with effusion. In their review, hearing loss and otitis media with effusion appear to occur in all children with PCD. Moreover, prolonged otorrhea after myringotomy tube placement occurred in 33% of patients with PCD compared to 10–50% occurrence rate in the general population. Lastly, they demonstrated that the largest improvement in hearing after myringotomy tubes were placed occurred in children who had the most significant hearing loss and who had normal hearing on follow-up at 1.5 years.

As otorlaryngolical complications are very common, regular audiology testing and specialty management is necessary. Children with hearing loss may benefit from hearing aids; in some cases, hearing impairment may improve with age (Barnes, 2009).

Nursing Care of the Child and Family

It is important to have a strong relationship with the child with PCD and the family. As with any chronic condition, frequent follow-up is important for improved outcomes in those with PCD.

The nurse’s role is multifaceted and includes patient and parent education, patient advocacy, and assessment of adherence issues with daily therapy. In a study by Pifferi et al. (2010), 71.8% of patients found that their quality of life improved after diagnosis. This suggests that making the proper diagnosis, education, and advocacy can have a positive effect on the patient’s life. However, the burden of daily treatment regimens can be heavy in children with PCD due to multiple medications and required airway clearance. Assessment and reinforcement of adherence with therapy are the cornerstones of improved quality of life and improved outcomes. With time, patients are less likely to perform daily airway clearance therapy (Pifferi et al., 2010); therefore, strategies to improve knowledge and to elucidate the connection between adherence and improved quality of life need to be emphasized.

As the child grows, he or she needs to become responsible for his or her own health care. Nurses can aid in the transition to adult care. The nurse may also be the one to discuss the need for contraception if the patient does not feel he or she can discuss the topic with his or her long-time health-care provider and parents. Although subfertility is common in females and infertility is common in males, a patient may still be at risk for an unplanned pregnancy and sexually transmitted diseases if birth control is not used (Barnes, 2009).

It is also important to assess the home environment of the child with PCD and to identify a responsible adult to participate in the care of that child. Health-care providers need to know if the child and parent understand the proper method for administering medications and for performing airway clearance. As the child grows, it is important for the family to maintain a healthy home environment. Children with PCD and their families should understand that refraining from and avoiding smoking, exposure to common aeroallergens, and other irritants is helpful (Sharma, 2008).

Children with PCD and their families need to be informed and updated on the name, use, and indications of prescribed medications. Some medications may not be stocked regularly in community pharmacies. Therefore, families need to learn how and where to refill them. The use of airway clearance modalities also need to be reviewed with parents and children. The nurse should also provide children and families with anticipatory guidance to assist them in troubleshooting problems that arise in the daily use of their therapies.

Providing families with adequate support outside the clinical setting is an important role for the nurse. Families must be informed and provided with information for when and how to contact their pulmonary provider for signs of respiratory complications such as increased cough, increased sputum production, changes in color of sputum, or wheezing. In addition, providing professional and accurate sources of information is extremely valuable to patients and families. Helpful Web sites include http://www.pcdfoundation.org and, in the United Kingdom, http://www.pcdsupport.org.uk. Relevant medical information is shared on the sites. Families may find others’ experiences with PCD invaluable in helping them navigate and advocate for their child.

Many challenging issues will undoubtedly arise during patient care. PCD is dynamic and requires patient- and family-centered nursing care that addresses issues in a broad and flexible manner.

BRONCHIECTASIS

Epidemiology

The prevalence of bronchiectasis in the United States and in the world is unknown (Barker, 2002; Emmons, 2009). However, between 2001 and 2002, the incidence of non-CF-related bronchiectasis in New Zealand was estimated at 37 per million among children less than 15 years of age (Twiss, Metcalfe, Edwards & Byrnes, 2005). Common childhood illnesses such as pertussis, measles, and tuberculosis place children at risk for developing bronchiectasis (Callahan, 2005). Decreased rates of bronchiectasis in the United States have been attributed to vaccinations for pertussis (Barker, 2002).

Pathophysiology

The lungs are susceptible to infection due to their constant contact with pathogens in the environment (Callahan, 2005) but are usually spared from long-term damage and consequences of this exposure because of robust mucociliary clearance mechanisms and immune responses. However, the airways can fall victim to bacterial and viral infection as well as aspiration events (Callahan, 2005), especially if there is an abnormality in the mucociliary escalator or in host defense. Such exposure and events set the stage for bronchiectasis, which results from repeated infection, inflammation, and destruction of lung tissue (Callahan, 2005). Repeated infections trigger a cycle of inflammation and obstruction causing the muscle layers of the airways to become damaged, leading to destruction of supportive cartilage producing a saccular or dilated airway (Brown & Lemen, 1990).

There are a number of clinical entities that have been associated with bronchectasis including (1) CF (Callahan, 2005); (2) immunodeficiency states including IgG and IgA deficiency, hypogammaglobulinemia, common variable immunodeficiency, and complement pathway defects (Brown & Lemen, 1990); (3) congenital lung malformations, such as congenital lobar emphysema, airway malacia, Williams–Campbell syndrome, and tracheomegaly (Stafler & Carr, 2010); (4) aspiration syndromes, such as recurrent aspiration from abnormal swallow in patients with neurological impairment, foreign body aspiration, and untreated gastroesophageal reflux (Eastham et al., 2004; Stafler & Carr, 2010); and (5) congenital abnormalities, such as tracheoesophageal fistula, or laryngeal cleft (Eastham et al., 2004; Stafler & Carr, 2010).

Signs and Symptoms

Children may present with symptoms of a chronic productive cough, shortness of breath, or chest pain (Callahan, 2005; Brown & Lemen, 1990). Occasional presenting symptoms are dyspnea and hemoptysis (Brown & Lemen, 1990). Signs of bronchiectasis may include persistent crackles, wheeze, and digital clubbing (Brown & Lemen, 1990; Callahan, 2005).

Diagnosis

Chest radiographs may show bronchial dilation, volume loss, or bronchial wall thickening (Callahan, 2005). Parallel linear densities associated with bronchiectasis are referred to as the “tram track” sign (Callahan, 2005). After chest X-rays are obtained, a high-resolution chest CT scan (HRCT) should be obtained to confirm diagnosis. On an HRCT, the classical sign of bronchiectasis is the “signet ring,” which refers to the bronchial/pulmonary artery ratio greater than 1, suggesting a dilated airway. Depending on the underlying cause, more diagnostic tests are often needed. For example, if reflux is suspected, a barium swallow may be necessary. Bronchoscopy may be used to rule out a retained foreign body, bronchomalacia, or other abnormal airway anatomy, to obtain sputum cultures, or to detect fat-laden macrophages associated with aspiration (Stafler & Carr, 2010). Other laboratory tests that might be required include a sweat test to rule out CF, a ciliary biopsy to rule out PCD, and total serum immunoglobulin levels, antibody responses to tetanus, pneumococcal or H. influenzae vaccinations, or C3 and C4 complement levels to rule out immunodeficiency, which may predispose patients to recurrent infection and bronchiectasis (Stafler & Carr, 2010).

Complications

Repeated infections in the bronchiectatic areas of the lung are common and may become a focus of recurrent lung infection. Progressive lung damage can lead to chronic respiratory insufficiency, hypercapnia, and pulmonary vascular disease (Chang & Redding, 2006) if bronchiectasis is not treated. Subsequently, it can lead to end-stage pulmonary failure in adults (Stafler & Carr, 2010). The severity and the progression of the disease process have changed with the use of newer and inhaled antibiotics (Chang & Redding, 2006).

Management

The treatment of bronchiectasis depends on the underlying cause. If the cause of the bronchiectasis is due to a noninfectious chronic condition such as CF or PCD, treatment is directed at the underlying problem. A sputum culture should be obtained to guide the decision for appropriate antibiotic therapy (Callahan, 2005). If the patient presents with acute symptoms such as fever, cough, increased sputum production, and a decline in PFTs, the treatment will include aggressive airway clearance and oral or intravenous antibiotics depending on the clinical scenario (Callahan, 2005). Immunoglobulin replacement therapy, along with aggressive antibiotic therapy, may be used for children with immunodeficiency problems (Stafler & Carr, 2010).

Nursing Care of the Child and Family

Nursing care of the child with bronchiectasis includes encouraging adherence to therapies such as antibiotics and airway clearance. Children with bronchiectasis and their families need to be informed and updated on the name, use, and indications of the medications prescribed. For children who use airway clearance regularly, a review of airway clearance techniques should be performed on a regular interval. A portion of this review may be provided directly from a manufacturer but should always be reviewed by the care team. Families should receive anticipatory guidance to help them troubleshoot problems that arise in the daily use of these therapies. Supportive care may include the administration of intravenous antibiotics.

Providing families with adequate support outside the clinical setting is an important role for the nurse. Families must be informed and provided with information for when and how to contact their pulmonary provider for signs of respiratory complications such as increased cough, increased sputum production, changes in the color of sputum, or wheezing. This ensures the institution of early interventions that will help protect the airways from damage over time.

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