Respiratory Disorders. Dr Tanya Robertson

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1 Respiratory Disorders Dr Tanya Robertson

2 Aims Overview of respiratory problems in children Diagnosis Management Asthma Cystic fibrosis

3 Why are children different? Immune status Structure and function respiratory system

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5 How do you classify respiratory disorders?

6 Upper respiratory Tract Lower respiratory Tract Acute Chronic

7 Upper Respiratory Tract Acute: Croup (laryngotracheobronchitis( laryngotracheobronchitis) Epiglottitis Inhalation of a foreign body Retropharyngeal Abscess Chronic: Laryngomalacia Vascular ring

8 Diagnosis SOB Increased respiratory effort Small increase in respiratory rate Inspiratory symptoms Stridor Drooling hoarseness

9 Croup Parainfluenza virus URTI/ cold prior to illness Increased temperature Barking cough

10 Croup Score Stridor Recession No No When agitated Mild (s/c( s/c) At rest Moderate Severe Severe Colour N N N Cyanosis Conscious level N Restless when disturbed Anxious/ aggitated Lethargic

11 Epiglottitis H Influenzae type B Temperature Drooling Severe respiratory distress and stridor Hyperextension of the neck Irritability

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14 Management Keep the child calm Do not examine the throat Oxygen Croup: Dexamethasone Pulmicort nebulisers Severe croup: Adrenaline nebulised Epiglottitis: Intravenous Ampicillin and Chloramphenicol

15 Laryngomalacia Stridor appearing after the first few days of life Floppy larynx collapse of the airway on inspiration Most resolve with growth and development of the airway Severe cases may require tracheostomy

16 Lower Respiratory Tract Acute: Infective Chronic: Asthma Cystic fibrosis Bronchiectasis Bronchiolitis obliterans Congenital

17 Symptoms SOB Cough Wheeze Tired Irritability Decreased feeding Abdominal pain Fever

18 Signs Tachypnoea Tachycardia Decreased oxygenation Respiratory distress Wheeze/crepitations crepitations/crackles Decreased air entry Silent chest!

19 Effort of breathing Subcostal recession mild severe

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22 Severe

23 Investigations Oxygenation CXR Bloods Cough swab/ sputum NPA/PNA

24 Bronchiolitis RSV Decreased feeding Wheezy cough Respiratory distress On auscultation crackles and wheeze NPA Supportive treatment Palivizmab

25 Bronchiolitis obiterans Secondary to infection, adenovirus Bronchioles are partially or completely obliterated by nodular masses which contain granulation and fibrotic tissue Respiratory illness followed by improvement Then deterioration with cough, wheeze, SOB and sputum production

26 CT scan on inspiration and expiration Management difficult Swyer James syndrome: unilateral hyperlucent lung syndrome

27 Bronchiectasis Dilatation of the bronchi Inflammatory destruction of bronchial and peribronchial tissue Accumulation of exudative material in dependent bronchi Distension of dependent bronchi

28 Causes: Cystic fibrosis Pulmonary infection Recurrent Aspiration Primary Ciliary Dyskinesia Immune Deficiency Inhalation of a foreign body

29 Symptoms Cough, productive Recurrent chest infections Poor weight gain

30 Diagnosis + Treatment CT scan Cough swabs Physiotherapy Antibiotics

31 Cystic Fibrosis

32 Times Nov 2006 Mr Brown, who is expected to succeed Tony Blair as prime minister, learnt of the condition of his four-month old son Fraser soon after his birth following a series of blood tests. It is the second devastating tragedy to hit the Browns. In 2002 they lost their first baby, Jennifer Jane, who was born seven weeks premature and d suffered a brain haemorrhage shortly before she died. Fraser Brown was born in July at Edinburgh s Royal Infirmary. He weighed a healthy 7 lb 14 ounces. The condition was picked up in the routine series of blood tests that all babies in Scotland undergo. The news shocked Westminster tonight, particularly as it dawned that Mr Brown would have known of the tragedy in the middle of Labour s leadership crisis in the early autumn when he was accused by some MPs of plotting against Mr Blair.

33 Cystic Fibrosis 1in 25 population carrier 1 in 2500 newborn babies have CF 7500 babies,children and young adults affected 1 born almost every day ( 5 a week ) 3 young lives lost weekly Most common life threatening inherited disease

34 Autosomal recessive inheritance chromosome 7 Carrier parent Carrier parent Affected Carrier Carrier Not a baby baby baby carrier

35 Cystic Fibrosis Transmembrane conductance regulator Transports salt and water in and out of cells CFTR either faulty or absent (depending on gene defect)

36 Unable to secrete chloride Excess sodium absorbed Water follows sodium Sticky mucus clogs up lungs and digestive system In sweat glands doesn t absorb chloride Salty sweat

37 Presentation Meconium Ileus Recurrent chest infections/ recurrent cough/ productive/ asthma Failure to thrive Nasal polyps Rectal prolapse Oedema secondary to low albumin Prolonged conjugated neonatal jaundice Family history of cystic fibrosis Diarrhoea/ frequent large bulky offensive stools

38 Diagnosis/sweat test Sweat collected from forearm Painless electrical impulses across skin Sweat collected 1g/m2/min 2/52 baby greater than 3 kg well hydrated Sweat chloride most accurate >60 mmol/l chloride diagnostic mmols suggestive < 40 mmols normal, low possibility

39 Newborn screening 2007 Genetic testing: Delta F508 x 2 75% 31 mutations 90% ( 20 common) Carrier cascade testing- blood or mouth swab

40 Other investigations Faecal elastase CXR Cough swab or sputum Lung function tests

41 Initial treatment Physiotherapy Dietary advice and creon Flucloxacillin Vitamin K Vitamin E Ketovite/dalavit

42 Complications: LUNGS Thick secretions Recurrent infections Bronchiectasis Haemoptysis 85 % have pancreatic insufficiency Malnutrition Constipation/Diarrhoea DIOS Liver disease Diabetes

43 Others Gastro oesophageal reflux Stress incontinence - recurrent coughing Anal prolapse- coughing and constipation Nasal polyps Sinusitis Infertility Men Almost all infertile due to thick secretions Female Weight related Osteoporosis and osteopenia Dehydration due to high sweat loss Arthritis large joints Depression

44 Infection Very vulnerable group Infections have great impact on life expectancy Separate clinics CF patients separated on ward CF group activities not encouraged Separate classes at school Separate judo classes etc

45 Asthma

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47 Diagnosis is clinical Symptoms: Wheezing Cough Difficulty breathing Chest tightness Interval symptoms

48 Symptoms Frequent and recurrent Worse at night and in the early morning Worse after or occur in response to exercise or other triggers History of atopic disorder Family history

49 Signs Harrisons Sulci Signs of respiratory distress Wheeze Features of atopy

50 Features that lower the chance of asthma Symptoms with colds only Isolated cough History of a moist cough Prominent dizziness, light headedness, peripheral tingling.

51 Question diagnosis Symptoms present from birth FH of unusual chest disease Excessive vomiting Inspiratory stridor Abnormal voice or cry Failure to thrive Clubbing Failure to respond to conventional treatment Unexpected clinical findings

52 Aims of management Control of symptoms Prevention of exacerbations Achieve best possible pulmonary function Minimal side effects

53 Control of symptoms No daytime symptoms No night time wakening No need for rescue medication No limitations on activity Normal lung function

54 Management Step wise approach Start treatment at the most appropriate step Before initiating a new step check compliance and inhaler technique

55 Inhalers

56 Colours Brown Purple Green Blue Grey and green

57 Treatment step one: mild intermittent asthma Inhaled Short acting B2 agonist as required

58 Step 2 :Introduce a regular preventer therapy Regular preventer therapy Start at dose appropriate to severity of disease Children: 200mcg per day Titrate the dose to the lowest dose at which effective control of asthma is maintained

59 When? Frequent exacerbations of asthma Using inhaled B2 agonists 3x a week or more Symptoms 3x a week or more Waking one night a week

60 Step 3: add on therapy When taking 400mcg per day of steroid inhaler Inhaled long acting B2 agonist Leukotriene receptor antagonist Theophyllines

61 Step 4: poor control on moderate dose inhaled steroid and add on therapy Increase the dose of inhaled steroid up to 800 mcg per day

62 Step 5 Oral steroids

63 Aged < 5years Step1:inhaled short acting B2 agonist Step 2: inhaled steroid mcg/day Step3: consider leukotriene receptor antagonist Step 4: refer to respiratory paediatrician

64 Important to recheck compliance between each step Step down when able No research on how Decrease dose by approx 25 % each time

65 Clenil Qvar Adrenal suppression

66 Deaths from asthma Chronically severe asthma Increasing use of B2 agonist Inadequate steroid therapy Repeated attendance at A+E Previous admission in last year Previous near fatal asthma Requiring 3 or more classes of asthma medication Non compliance, DNA, obesity

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68 Questions?