Chronic Cough/Bronchiectasis – Pediatrics

Pathophysiology: Recurrent pneumonia and lower respiratory tract infections cause airway damage that leads to "ectasia" and loss of elasticity of bronchi. Loss of muco-ciliary function leads to difficulty clearing secretions.
Risk Factors:

• Prematurity
• Immunocompromise
• Early recurrent respiratory infections
• Previous injury
• Environmental effects (lack of piped water, household crowding, woodstove use)

Demographics: Alaska YK Delta: 1 in 63 children, Central Australia: 1 in 68, U.S.: 1 in 250,000.
Signs/Symptoms: Chronic wet cough, respiratory exacerbations with fever, crackles, wheezing
Diagnosis: Progression of disease from protracted bronchitis to chronic suppurative lung disease (3 episodes at least 3 months each) to CT scan confirmed Bronchiectasis
Management:

• Reduce infection-inflammation: treat early and exacerbations 'aggressively' with antibiotics, airway hygiene clearance, vaccinations
• Improve other factors contributing: attention to nutrition, detect complications, pollutants
• Systemic care: regular review, multi-disciplinary care, education, enhance self care and management
• Collaboration with Seattle Children's Hospital Pulmonologists who generally see patients in a Bethel Field Clinic 3-4 times a year
• Special attention in adolescents to transferring to adult care.

Critical Times for Affected Patients: exacerbations – antibiotic treatment important, prophylaxis if frequent exacerbations, transition to adulthood critical – poorly managed bronchiectasis can lead to early COPD and death

Resources/References

• Bronchiectasis: Prevention and Management 2016 (PowerPoint Presentation by Rosalyn Singleton, MD)
• Munro, et al. Burden of Bronchiectasis in Indigenous Peoples- How Can it be Improved?. Current Pediatric Reviews. 2009, 5, 00-00.
• Singleton RJ et al. Indigenous children from three countries with non-cystic fibrosis chronic suppurative lung disease/bronchiectasis. Pediatr Pulmonol 2014.
• Redding et al. Respiratory Exacerbations in Indigenous Children From Two Countries With Non-Cystic Fibrosis Chronic Suppurative Lung Disease/Bronchiectasis. CHEST 2014:146;762-4
• Lewis, T. et al. Prevalence of Asthma and Chronic Respiratory Symptoms Among Alaska Native Children. CHEST. 125(5):1665-1673, May 2004. doi.org/10.1378/chest.125.5.1665
• Healthy Homes Baseline Characteristics: Singleton R, Salkoski AJ, Bulkow L, Fish C, Dobson J, Albertson L, Skarada J, Kovesi T, McDonald D, Hennessy TW, Ritter T. Housing characteristics and indoor air quality in households of Alaska Native children with chronic lung conditions. Indoor Air 2017 Mar;27(2):478-486. DOI: 10.1111/ina.12315 Epub 2016 Jul 18
• Healthy Homes Results: Singleton R, Salkoski AJ, Bulkow L, et al. Impact of Home Remediation and Household Education on Indoor Air Quality, Respiratory Visits, and Symptoms in Alaska Native Children. Int J Circumpolar Health 2018;77(1) DOI: 10.1080/22423982.2017.1422669
• Kinghorn B, Singleton R, Mccallum GB, Bulkow L, Grimwood K, Hermann L, Chang AB, Redding G. Clinical course of chronic suppurative lung disease and bronchiectasis in Alaska Native children. Pediatric Pulmonology 2018;1-8. https://doi.org/10.1002/ppul.24174
• Sibanda D, Singleton R, Clark J, et al. Adult outcomes of childhood bronchiectasis. Int J Circumpolar Health. 2020;79(1):1731059. https://www.ncbi.nlm.nih.gov/pubmed/32090714
• YKHC Clinical Guideline for Bronchiectasis
• Free Indoor Air Toolkit Program - 2026 Flyer

YKHC Clinical Guidelines
Common/Unique Medical Diagnoses