Non Cystic Fibrosis Bronchiectasis: How to Proceed?

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1 Non Cystic Fibrosis Bronchiectasis: How to Proceed? Dr Ankit Parakh MD, DNB, MNAMS, RCPCH Fellow (UK), European Diplomate of Pediatric Respiratory Medicine ERS Fellowship Pediatric Medicine & NIV (GOSH, London, UK) APSR fellowship Sleep Medicine (Brisbane, Australia) Consultant Pediatric Pulmonologist BLK Super Specialty Hospital Formerly Assistant Professor Kalawati Saran Children s Hospital

2 Plan of the Talk.. Clarifying the terms: Wet cough, protracted bacterial bronchitis, suppurative lung disease, bronchiectasis When to investigate? Etiology Diagnosis: Imaging Specific Investigations for etiological diagnosis Treatment

3 What Is Wet Cough? Even when airway secretions are present, young children rarely expectorate sputum. Hence wet/moist cough is the preferable term rather than productive cough. Pediatr Pulmonol. 2008; 43:

4 Definitions Protracted Bacterial Bronchitis History of isolated chronic moist cough with no specific cough pointers beyond 3-4 weeks AND Response to antibiotic treatment with resolution of the cough within 2 weeks Chronic suppurative lung disease Persistent chronic wet cough >6 months but without confirmatory c-hrct findings. Bronchiectasis Persistent or progressive condition characterized by dilated thick-walled irreversibly damaged bronchi.

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6 Which children should be investigated for bronchiectasis/csld? Chronic productive or moist cough every day for >8 weeks or less than <8 weeks but worsening British Thoracic Society guideline for non-cf bronchiectasis. Thorax Jul;65 Suppl 1:i1-58.

7 Which children should be investigated for bronchiectasis? Symptoms attributed to asthma that are atypical or which respond poorly to conventional treatment Cough variant asthma Localised chronic bronchial obstruction. Long standing foreign body British Thoracic Society guideline for non-cf bronchiectasis. Thorax Jul;65 Suppl 1:i1-58.

8 Which children should be investigated for Post severe pneumonia bronchiectasis? Incomplete resolution of symptoms, physical signs or radiological changes. Recurrent pneumonia Persistent and unexplained physical signs or chest radiographic abnormalities. British Thoracic Society guideline for non-cf bronchiectasis. Thorax Jul;65 Suppl 1:i1-58.

9 Which children should be investigated for bronchiectasis? Respiratory symptoms with any clinical features of CF, PCD or immunodeficiency. British Thoracic Society guideline for non-cf bronchiectasis. Thorax Jul;65 Suppl 1:i1-58.

10 Etiology of Bronchiectasis in Children POST Infections Muco-Ciliary Disorders Immuno-Deficiency Causes Severe Necrotizing Pneumonia Viral pneumonia (measles, adenovirus), Post-pertussis Post Tubercular Cystic Fibrosis Primary Ciliary Dyskinesia HIV XLA, CVID Hyper IgM Job s Syndrome Important Clinical Findings Allergic/Fungal Allergic Bronchopulmonary Aspergillosis Past Asthma/CF Temporal relationship of identified infections to the onset of chronic respiratory symptoms Stool issues/ftt Chronic Upper airway symptoms (Rhinorrhea, otitis) Serious, persistent or recurrent infections, particularly involving multiple sites, or infections with opportunist organisms Other/Rare Congenital disorders, Alpha-one Antitrypsin def, Ataxia-Telengectasia

11 Etiology of Bronchiectasis in Children Kapur N. Paediatr Respir Rev Jun;12(2):91-6.

12 Diagnosis and Investigations To prove the diagnosis of CSLD: Chest Xray HRCT Chest To prove the etiology of CSLD: Specific Investigations: sweat test, PCD studies, Immunological tests etc To evaluate the airway microbiology: Sputum/Bronchoscopy BAL To evaluate disease extent/progression: Lung Function/follow up Radiology

13 Imaging of diseases of chest Hansell 5 th ed Plugged & thickened centrilobular bronchioles ( treein-bud sign) Nontapering or flaring of bronchi Crowding of bronchi with associated volume loss Areas of decreased attenuation reflecting small airways obliteration Signet Ring Sign Mucus-filled dilated bronchi ( flame and blob sign); Large Airways 25

14 Signet Sign Dilated bronchus Bronchial artery 40

15 Etiology of Bronchiectasis in Children POST Infections Muco-Ciliary Disorders Immuno-Deficiency Causes Severe Necrotizing Pneumonia Viral pneumonia (measles, adenovirus), Post-pertussis Post TB Cystic Fibrosis Primary Ciliary Dyskinesia HIV XLA, CVID Hyper IgM Job s Syndrome Radiological Pointers Usually in location of Pneumonia or Tuberculosis Can be more diffuse with viral Pan Bronchiectasis Sinusitis Small airway disease Non-Specific Can involve one or all lobes Allergic/Fungal Allergic Bronchopulmonary Aspergillosis Central Bronchiectasis, Bronchoceles, HAM, Tree-in-bud

16 Investigations Etiology POST Infections Muco-Ciliary Disorders Immuno-Deficiency Causes Severe Necrotizing Pneuminia Viral pneumonia (measles, adenovirus), Post-pertussis Post TB Cystic Fibrosis Primary Ciliary Dyskinesia HIV XLA, CVID Hyper IgM Job s Syndrome Investigations History and temporal relation Sweat Test, Genetics VideoMicroscopy, EM, Genetics HIV Testing Ig Profile CD3, CD4, CD8, CD19 etc Allergic/Fungal Allergic Bronchopulmonary Aspergillosis Serum IgE Aspergillus Specific IgE/IgG Radiological Findings Other/Rare

17 11y boy Presented in June 2012 Recurrent episodes of cough/ fever/fb X 5 years Already has taken ATT twice in 2006 and 2011 for clinical complaints and radiological opacities No clear improvement in either clinical picture Radiological opacities increasing

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22 17 y boy Cough with sputum 15d Fever Hemoptysis few episodes No h/o contact with TB Sputum X 2 specimen negative Mantoux 14 mm

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25 Further Investigations Serum IgE: 2684 Kua/L Aspergillus IgE: 8.29 kua/l (normal <0.35) Aspergillus IgG: (normal <8.00) Treatment Oral steroids + Itraconazole Antibiotics

26 15 y old girl Chronic wet cough since age of 1 year Having exacerbations in between 4-5 per year Child treated for TB 2 years back Diagnosed as case of Atelectobronchiectasis secondary to tuberculosis

27 Admitted with Increased fever/cough/increased sputum purulence and volume Examination: Short stature Delayed puberty Wheezing Liver 3 cm spleen 7 cm

28 Investigations HIV NR Sweat Chloride 32 meq/l Sputum AFB Negative Mantoux negative FOB : bilateral tree shows mucopurulent secretions and few ulcerations at carina and left main bronchus

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30 Investigations Immunoglobin profile IgA not detectable ( mg/dl) IgG ( mg/dl) IgM 3.4 ( mg/dl) CD3: 51.88% (57-85%); 514/microL ( ) CD8: 39.84% (5-23%); 395/microL (90-640)

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32 12y boy Presented to Department of ENT November 2011 Chronic nasal obstruction Nasal discharge 3-4 years Exam: Inferior turbinate hypertrophy with nasal discharge

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34 Severe persistent asthma with rhinosinusitis All modifiable factors checked multiple times Evaluated on difficult asthma protocol

35 March 2012 April 2012 May 2012

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38 Management General approach and treatment of the specific underlying cause Education for patients and parents of children Airway clearance Physiotherapy and exercise Mucolytic and hyperosmolar therapies Airway drug therapy Bronchodilation Anti-inflammatory Antibiotic therapy Surgical management

39 Goals of treatment Identify and treat underlying cause to prevent disease progression. [D] Maintain or improve pulmonary function. [D] Reduce exacerbations. [D] Improve quality of life by reducing daily symptoms & exacerbations. [D] Achieve normal growth and development. [D] Patients with immune deficiency should be under joint care with a clinical immunologist. [D] Patients with CF should be referred to a CF specialist centre. [D] British Thoracic Society guideline for non-cf bronchiectasis. Thorax Jul;65 Suppl 1:i1-58.

40 Airway clearance techniques Passive: Postural drainage with percussion Active: PEP devices, Acapella, Active Cycles of Breathing Techniques The duration and frequency of the airway clearance technique should be specific to the needs of the individual. This may alter with periods of infective exacerbation. Airway clearance therapy should be for min once or twice daily.

41 Airway clearance techniques Postural drainage and percussion (PD&P) Active cycle of breathing technique (ACBT) Autogenic drainage (AD) Positive expiratory pressure (PEP) High frequency chest wall oscillation (HFCWO) Exercises None is superior to the other. Compliance and correct technique is important

42 Definition: Paediatric Bronchiectasis Exacerbation Kapur N et al. Pediatr Pulmonol Jan;47(1): British Thoracic Society guideline for non-cf bronchiectasis. Thorax Jul;65 Suppl 1:i1-58.

43 Treatment of Exacerbations When to Admit? Empirical Antibiotics: What to use? Sputum Microbiology Duration Severe Respiratory Distress Hypoxia Hypercapnic Respiratory Failure Non Improvement with oral Antibiotics Amoxicillin for oral Ceftriaxone/Cefuroxime for IV If can produce sputum, else BAL might be required Organism Targeted Therapy to be used Usually days

44 Chang AB. MJA 2008; 189:

45 Adjuvants Adjuvant Is it helpful? Reference Oral Azithromycin Bronchodilators (LABA) Reduce frequency of exacerbations and improve quality of life No Data. Can consider on the airway hyper-responsiveness Kelly C. Cochrane Database Syst Rev Mar 15;3:CD Goyal V. Cochrane Database Syst Rev Jun 10;(6):CD Inhaled Steroids No studies (acute stage or long term) Kapur N. Cochrane Database Syst Rev May 16;5:CD N-Acetyl Cysteine No significant benefit Tarrant BJ et al. Respirology Aug;22(6): Hypertonic Saline Nebulization (3 % or 7%) Limited Benefit outside CF settings Tarrant BJ et al. Respirology Aug;22(6): RNAse Not Recommended Tarrant BJ et al. Respirology Aug;22(6):

46 Follow Up-3 to 6 monthly Assessment of Severity Identify & manage complications & comorbidities Vaccinations Nutrition Avoid smoke Exacerbation frequency Exercise tolerance Signs of right heart failure Pulse oximetry Spirometry GERD Asthma Sleep- disordered breathing and cardiac complications. Annual Influenza Pneumococcal 13 and 23 valent Adequate growth monitoring and advice Active and passive smoking; biomass fuels Chang AB. MJA 2008; 189:

47 Take Home Messages Recognition of chronic wet cough is important Adequate treatment of PBB/CSLD might prevent bronchiectasis Think about etiology!!! Adequate chest PT and treatment of exacerbations are mainstay of treatment.

48 Thank You!!