eMedicine Specialties > Pediatrics: General Medicine > Pulmonology
Bronchiectasis: Treatment & Medication
Updated: Sep 8, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
In addition to the treatment of an identified underlying disorder in patients with bronchiectasis, therapy is guided at reducing the airway secretions and facilitating their removal through cough. Pharmacotherapy may be used to enhance bronchodilation and to improve mucociliary clearance. Antibiotics can be used to prevent and treat recurrent infections. Secretions can be mobilized with chest physiotherapy and mucolytic agents. Occasionally, surgery may be considered. The goal of therapy is to mobilize secretions and to reduce the infectious and inflammatory load, thereby preventing progression of airway destruction.
Randomized trials of these treatment options lack proper control groups. In children, many of the therapies have been used in cystic fibrosis (CF). However, non-CF bronchiectasis may not always respond the same as CF. The markers used to assess therapy effectiveness have included the volume of sputum production and the clearance of a radiolabeled aerosol from the lung. More meaningful studies that focus on measures such as rate of respiratory exacerbations and quality of life, improvement in lung function and improvement in radiographic findings are needed.
- Anti-inflammatory agents: Randomized placebo-controlled trials of inhaled corticosteroids in patients with non-CF bronchiectasis showed no significant improvement in lung function. Inhaled corticosteroids may have a role in regulating the host response and halting inflammatory damage to the lung. In children with underlying asthma, it is important to continue inhaled corticosteroids on a chronic basis.
- Bronchodilators: Bronchodilators are indicated when bronchial hyperreactivity is evident and are used to improve ciliary beat frequency and, thus, facilitate mucus clearance. However, no randomized studies have validated their usefulness in the management of bronchiectasis. Furthermore, some patients with bronchiectasis experience paradoxic bronchoconstriction with beta-agonist therapy. This is likely secondary to loss of airway tone due to beta-agonist relaxation of bronchial smooth muscle superimposed on already weakened bronchial cartilage in the bronchiectatic airway. Therefore, assessing bronchodilator response before beginning such therapy is critical.
- Mucolytics: Mucolytic drugs aim to improve tracheobronchial clearance via alteration of sputum consistency. Recombinant DNase did not show significant benefit in non-CF bronchiectasis. This is presumably due to a lesser component of neutrophils in the airway than in CF.
- Antibiotics
- A Cochrane review revealed that long-term therapy with antibiotics is effective in reducing sputum volume and purulence but has limited impact on the frequency of exacerbations and the natural history of the disease process. Also, chronic antibiotic use may result in the emergence of resistant organisms.
- Some clinicians treat bronchiectasis with prolonged oral antibiotics on a rotating basis. This is falling into disfavor, as it is in CF. Broad-spectrum antibiotics can be given for a month, followed by a second broad-spectrum drug, followed by a third, and so forth. Another option is to use alternating antibiotics for 7-10 days, with antibiotic-free periods of 7-10 days between each course.
- Davies and colleagues, and Anwar and colleagues suggested that long-term triweekly therapy with azithromycin can be helpful in patients with bronchiectasis.15,16 This has also been helpful in CF. Macrolide antibiotics have anti-inflammatory effects, which have been helpful in CF and in non-CF bronchiectasis.
- Rosen and associates concluded that antibiotics are important parts of therapy during exacerbations of bronchiectasis, with the selection of agents based on culture results.17
- Inhaled tobramycin was associated with decreased Pseudomonas aeruginosa load in sputum, improved lung function, and fewer hospitalizations. However, Rosen and colleagues concluded that inhaled tobramycin is not indicated in non-CF bronchiectasis, unless Pseudomonas is detected in the sputum or bronchoalveolar lavage samples.17
- Chest physiotherapy: Manual and mechanical interventions such as chest percussion, vibration, postural drainage, cough-assist devices, and airway oscillation (ie, flutter) are used to facilitate mucous expectoration. The goal is to facilitate effective airway clearance. Remember that these devices serve as adjuncts to the cough, which is the most effective and efficient manner of clearing the airway.
Surgical Care
- Pulmonary segmental resection may be beneficial when damage is severe and well localized. Documenting severe abnormalities in ventilation and/or perfusion to the affected portion of the lung, such as with a lung scan, is often helpful.
- Prior to the proliferation and availability of broad-spectrum antimicrobials both Field and Clark demonstrated a gradual symptomatic improvement of some children who did not undergo surgical therapy for bronchiectasis.3,1
- In 1993, Lewiston recommended that surgery be delayed, unless symptomatically necessary, until the patient is aged 6-12 years because of the possibility for clinical improvement. Surgery is also delayed in children with stable disease that can be controlled with medical therapy.
- Otgun and associates, in a retrospective study, concluded that the decision for bronchiectasis surgery should be made in cooperation with the chest disease unit.18 Furthermore, anatomic localization of disease should be mapped with radiography and scintigraphic studies. Otgun and associates found the morbidity and mortality rates to be within acceptable ranges. In unilateral bronchiectasis, total excision and pneumonectomy, as opposed to leaving residual disease, was found to be well tolerated and most beneficial to the child.
- For patients with severe progressive disease, transplantation has worked as well as in subjects with other lung diseases.
Consultations
- Routine care of children with bronchiectasis is successfully provided by a primary care physician.
- A pediatric pulmonologist should be initially consulted for all infants and children with bronchiectasis. The subspecialist should be an integral part of the child's care and should manage most of the pulmonary aspects of the bronchiectasis and the underlying disease.
- If recurrent aspiration is a contributing factor, a pediatric gastroenterologist should have input into the child's care.
- Pediatric immunologists should help manage children with HIV infection or immunoglobulin deficiencies.
- If the child has an underlying rheumatologic disorder, a pediatric rheumatologist should be consulted on a regular basis.
- Physical therapists or respiratory therapists are important and helpful in the chest physiotherapy techniques. Whether manually performed or performed with one of the mechanical devices, the procedure needs to be thoroughly learned and periodically reviewed with the therapist.
Activity
- No specific activity limitations are necessary.
- Exercise generally promotes increased mucociliary clearance, which may enhance airway clearance in patients with bronchiectasis. However, exercise-induced dyspnea may require further investigation using exercise testing to evaluate for limitation or hypoxemia.
Medication
Treatment for bronchiectasis consists of antibiotics for infections and bronchodilators for any airway hyperreactivity, with the caveat above. Antibiotic therapy agents should be tailored to the results of sputum culture.
Increasingly, anti-inflammatory agents are prescribed, such as inhaled or oral corticosteroids. Although beta agonists may improve ciliary function and airway clearance, ensure that they are not adversely affecting lung function. If no significant bronchodilator response is observed, the ciliary effects by themselves are not great enough to warrant such therapy.
Inhaled hypertonic saline has been helpful in patients with cystic fibrosis (CF) and some studies suggest a benefit in patients with non-CF bronchiectasis, as well.
Corticosteroids, inhaled
These agents may be beneficial in treating chronic inflammation in bronchiectasis. They elicit anti-inflammatory and immunosuppressive properties and cause profound and varied metabolic effects. They also modify the body's immune response to diverse stimuli.
Fluticasone (Flovent HFA, Flovent Diskus)
Inhibits bronchoconstriction mechanisms, produces direct smooth muscle relaxation, and may decrease the number and activity of inflammatory cells, in turn decreasing airway hyperresponsiveness. Available as aerosol, Flovent HFA (44, 110, or 220 mcg/actuation), also available as Flovent powder for inhalation (Diskus) that delivers 50 mcg/actuation, 100 mcg/actuation, or 250 mcg/actuation.
Adult
110-220 mcg (110 mcg per actuation) inhaled PO via valved holding chamber bid
Pediatric
<4 years: Not established; emerging data suggest administering HFA formulation via holding chamber and mask at 44-88 mcg inhaled PO bid initially
HFA; 4-11 years: 88 mcg inhaled PO bid; higher doses may be required
Powder; 4-11 years: 50 mcg inhaled PO bid; may increase to 100 mcg bid or higher
Adolescents: Administer as in adults
Inhibitors of CYP450 3A4 isoenzyme (eg, ketoconazole) may increase fluticasone concentrations
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Suppression of HPA, linear growth, or Cushing syndrome may occur; caution with untreated systemic infections, ocular herpes simplex, or respiratory tuberculosis; rinse mouth after use to reduce likelihood of PO candidiasis; use with spacer
Budesonide (Pulmicort Flexhaler)
Alters level of inflammation in airways by inhibiting multiple types of inflammatory cells and decreasing production of cytokines and other mediators involved in the bronchospasm. Available as Pulmicort Flexhaler, powder for inhalation (90 mcg/actuation and 180 mcg/actuation, each actuation delivers 80 mcg and 160 mcg respectively) or Pulmicort Respules inhalation susp (0.25 mg/2 mL, 0.5 mg/2 mL, or 1 mg/2 mL). Nebulization has been used in children aged 1-8 y.
Adult
Flexhaler: 1-2 inhalations PO qd (180 mcg/actuation)
Pediatric
Flexhaler:
<6 years: Not established
>6 years: Administer as in adults
Respules:
<1 year: Not established
1-8 years: 0.25-0.5 mg inhaled via nebulizer qd/bid
Inhibitors of CYP450 3A4 isoenzyme (eg, ketoconazole) may increase serum concentrations
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Suppression of HPA, linear growth, or Cushing syndrome may occur; caution with untreated systemic infections, ocular herpes simplex, or respiratory tuberculosis; rinse mouth after use to reduce likelihood of PO candidiasis; use with spacer
Beclomethasone (QVAR)
Decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability.
Adult
40-80 mcg inhaled PO bid initially; may increase up to 320 mcg bid; higher doses may be necessary
Pediatric
<5 years: Not established
5-11 years: 40 mcg inhaled PO bid; may increase to 80-160 mcg bid; higher doses may be necessary
Adolescents: Administer as in adults
Coadministration with ketoconazole may increase plasma levels but do not appear to be clinically significant
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Suppression of the HPA, linear growth, or Cushing syndrome may occur; caution with untreated systemic infections, ocular herpes simplex, or respiratory tuberculosis; rinse mouth after use to reduce likelihood of oral candidiasis; use with spacer
Bronchodilators
These act to decrease muscle tone in the small and large airways in the lungs, thereby increasing ventilation. Spirometry is recommended before and after use of an inhaled bronchodilator before beginning chronic therapy. If no response is noted, or if paradoxic bronchoconstriction occurs, these agents should be avoided.
Albuterol (Proventil HFA, Ventolin HFA, ProAir HFA)
Relaxes bronchial smooth muscle by action on beta2-receptors with little effect on cardiac muscle contractility.
Adult
HFA: 90-180 mcg inhaled PO q4-6h; not to exceed 12 inhalations qd (90 mcg/actuation)
Nebulizer: 2.5-5 mg inhaled via nebulization q4-6h; dilute 0.5 mL (2.5 mg) of 0.5% inhalation solution in 1-2.5 mL of sterile 0.9% NaCl solution or water
Pediatric
HFA:
<12 years: 90-180 mcg inhaled PO qid with tube spacer (90 mcg/actuation)
>12 years: Administer as in adults
Nebulized:
<5 years: 1.25-2.5 mg inhaled via nebulization q4-6h; dilute 0.25-0.5 mL (1.25-2.5 mg) of 0.5% inhalation solution in 1-2.5 mL of sterile 0.9% NaCl solution or water
>5 years: Administer as in adults
Beta-adrenergic blockers antagonize effects; inhaled ipratropium may increase duration of bronchodilatation by albuterol; cardiovascular effects may increase with MAOIs, inhaled anesthetics, tricyclic antidepressants, and sympathomimetic agents
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in hyperthyroidism, diabetes mellitus, or cardiovascular disorders; may cause paradoxic bronchoconstriction
Antibiotics
Systemic and inhaled antibiotics are used in bronchiectatic disease to prevent or treat exacerbations caused by bacterial colonization that result in airway inflammation and injury. Antibiotics are generally chosen based on organisms and sensitivities from sputum cultures. The antibiotics used more commonly are listed below.
Amoxicillin and clavulanic acid (Augmentin)
Amoxicillin inhibits bacterial cell wall synthesis by binding to penicillin-binding proteins. Addition of clavulanate inhibits beta-lactamase–producing bacteria.
Good alternative antibiotic for patients who are allergic or intolerant to the macrolide class. Is usually well tolerated and provides good coverage against most infectious agents. Not effective against Mycoplasma and Legionella species. For children >3 mo, base dosing protocol on amoxicillin content. Because of different amoxicillin/clavulanic acid ratios in 250-mg tab (250/125) vs 250-mg chewable tab (250/62.5), do not use 250-mg tab until child weighs >40 kg.
Adult
500 mg q8-12h PO for 7-10 d
Pediatric
<3 months: 125-mg/5-mL PO susp based on amoxicillin; 30 mg/kg/d divided bid for 7-10 d
>3 months: If using 200-mg/5-mL or 400-mg/5-mL susp, 45 mg/kg/d PO q12h; if using 125-mg/5-mL or 250-mg/5-mL suspension, 40 mg/kg/d PO q8h for 7-10 d
>40 kg: Administer as in adults
Coadministration with warfarin or heparin increases risk of bleeding; may act synergistically against selected microorganisms when coadministered with aminoglycosides; coadministration with allopurinol may increase incidence of amoxicillin rash; may decrease efficacy of oral contraceptives when concomitantly administered
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Hepatic impairment may occur with prolonged treatment in elderly patients; diarrhea may occur; adjust dose in renal impairment; cross-allergy may occur with other beta-lactams and cephalosporins
Ciprofloxacin (Cipro)
Fluoroquinolone that inhibits bacterial DNA synthesis and, consequently, growth by inhibiting DNA gyrase and topoisomerases, which are required for replication, transcription, and translation of genetic material. Quinolones have broad activity against gram-positive and gram-negative aerobic organisms. Has no activity against anaerobes. Continue treatment for at least 2 d (7-14 d typical) after signs and symptoms have disappeared.
Not DOC in pediatrics because of increased incidence of adverse events, including arthropathy, compared with controls; no data exist for dose for pediatric patients with renal impairment (ie, CrCl <50 mL/min).
Adult
250-750 mg PO q12h
Pediatric
<8 years: Not recommended
>8 years: 500 mg PO q8h or 20-30 mg/kg/d PO divided q8h
Antacids, iron salts, and zinc salts may reduce serum levels; administer antacids 2-4 h before or after taking fluoroquinolones; cimetidine may interfere with metabolism of fluoroquinolones; ciprofloxacin reduces therapeutic effects of phenytoin; probenecid may increase ciprofloxacin serum concentrations
May increase toxicity of theophylline, caffeine, cyclosporine, and digoxin (monitor digoxin levels); may increase effects of anticoagulants (monitor PT)
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Dosage adjustments (adult adjustments)
CrCl (mL/min) <10: 50% of PO or IV dose q12h
HD: 0.25-0.5 g PO or 0.2-0.4 g IV q12h
During peritoneal dialysis: 0.25-0.5 g PO or 0.2-0.4 g IV q8h
In prolonged therapy, perform periodic evaluations of organ system functions (eg, renal, hepatic, hematopoietic); adjust dose in renal function impairment; superinfections may occur with prolonged or repeated antibiotic therapy
Trimethoprim and sulfamethoxazole (Bactrim, Septra)
Synthetic combination antibiotic. Each tab contains 80 mg of trimethoprim and 400 mg of sulfamethoxazole. Rapidly absorbed after oral administration. Mechanism of action involves blockage of 2 consecutive steps in biosynthesis of nucleic acids and proteins needed by many microorganisms.
Coverage for common forms of both gram-positive and gram-negative organisms, including susceptible strains of Streptococcus pneumoniae and Haemophilus influenzae. Indicated in treatment of acute and chronic bronchitic symptoms in patients with bronchiectasis.
Adult
160 mg TMP/800 mg SMZ PO q12h for 10-14 d (ie, 1 double-strength tab q12h)
Pediatric
<2 months: Contraindicated
>2 months: 8 mg/kg TMP and 40 mg/kg SMZ PO per 24 h, administered in 2 divided doses q12h for 10 d
May increase PT when used with warfarin (perform coagulation tests and adjust dose accordingly); coadministration with dapsone may increase blood levels of both drugs; coadministration of diuretics increases incidence of thrombocytopenia purpura in elderly patients; phenytoin levels may increase with coadministration; may potentiate effects of methotrexate in bone marrow depression; hypoglycemic response to sulfonylureas may increase with coadministration; may increase levels of zidovudine
Documented hypersensitivity; megaloblastic anemia due to folate deficiency; age <2 mo
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Do not use during last trimester of pregnancy because of potential toxicity to newborn (eg, jaundice, hemolytic anemia, kernicterus)
Dosage adjustments (adult adjustments)
CrCl (mL/min) 80-50: Recommended IV dose q18h
CrCl 50-10: Recommended IV dose q24h
CrCl <10: Not recommended
HD: 4-5 mg/kg after HD
During peritoneal dialysis: 0.16-0.8 g q48h
Discontinue upon first appearance of skin rash or sign of adverse reaction; obtain CBC counts frequently; discontinue therapy if significant hematologic changes occur; goiter, diuresis, and hypoglycemia may occur with sulfonamides; prolonged IV infusions or high doses may cause bone marrow depression (if signs occur, give 5-15 mg/d leucovorin); caution in folate deficiency (eg, chronic alcoholics, elderly patients, patients receiving anticonvulsant therapy, patients with malabsorption syndrome); hemolysis may occur in individuals who are G-6-PD deficient; patients with AIDS may not tolerate or respond to TMP-SMZ; caution in renal or hepatic impairment (perform urinalyses and renal function tests during therapy); give fluids to prevent crystalluria and stone formation
Inhaled antibiotics
These agents are indicated in bronchiectasis, specifically in patients with CF for P aeruginosa –positive sputum cultures.
Tobramycin for inhalation
Aminoglycoside specifically developed for administration with a nebulizer system. When inhaled, it is concentrated in airways, where it exerts antibacterial effect by disrupting protein synthesis.
Active against a wide range of gram-negative organisms, including P aeruginosa. Indicated for the treatment of patients with CF and P aeruginosa infection.
Adult
300-mg dose administered via a nebulizer
Recommended treatment regimen is repeated cycles of 28 d of the medication q12h, followed by 28 d off
Pediatric
<6 years: Not established
>6 years: Administer as in adults
May increase renal clearance of urate and lower serum uric acid levels; may interfere with urine laboratory tests for 5-hydroxyindoleacetic acid and urine testing for catecholamines
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
When prescribing medication that may suppress cough, important to identify cause of the cough and that suppression does not increase risk of clinical or physiologic complications
More on Bronchiectasis |
| Overview: Bronchiectasis |
| Differential Diagnoses & Workup: Bronchiectasis |
 Treatment & Medication: Bronchiectasis |
| Follow-up: Bronchiectasis |
| Multimedia: Bronchiectasis |
| References |
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References
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Further Reading
Keywords
bronchiectasis, bronchiectasia, destruction of airways, inflammation of the airway, bronchial dilatation, cystic fibrosis, CF, tuberculosis, pneumonia, cylindrical bronchiectasis, varicose bronchiectasis, saccular bronchiectasis, focal bronchiectasis, diffuse bronchiectasis, human immunodeficiency virus, HIV, primary ciliary dyskinesia, adenovirus, measles, Mycobacterium avium, or Aspergillus fumigatus, recurrent pneumonia, bronchitis, asthma, gastroesophageal reflux disease, allergic bronchopulmonary aspergillosis, pertussis, tracheoesophageal fistula, Marfan syndrome, Bruton agammaglobulinemia, Mounier-Kuhn syndrome, Williams-Campbell syndrome, connective tissue disorder, rheumatoid arthritis, systemic lupus erythematosus, treatment, diagnosis
