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Respiratory DisordersRespiratory Disorders
Dr Tanya RobertsonDr Tanya Robertson
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AimsAims
�� Overview of respiratory problems in Overview of respiratory problems in childrenchildren
�� DiagnosisDiagnosis
�� ManagementManagement
�� AsthmaAsthma
�� Cystic fibrosisCystic fibrosis
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Why are children different?Why are children different?
�� Immune statusImmune status
�� Structure and function respiratory Structure and function respiratory
systemsystem
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�� How do you classify respiratory How do you classify respiratory
disorders?disorders?
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�� Upper respiratory TractUpper respiratory Tract
�� Lower respiratory TractLower respiratory Tract
�� AcuteAcute
�� ChronicChronic
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Upper Respiratory TractUpper Respiratory Tract
�� Acute: Croup (Acute: Croup (laryngotracheobronchitislaryngotracheobronchitis))
EpiglottitisEpiglottitis
Inhalation of a foreign bodyInhalation of a foreign body
Retropharyngeal AbscessRetropharyngeal Abscess
�� Chronic: Chronic: LaryngomalaciaLaryngomalacia
Vascular ringVascular ring
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DiagnosisDiagnosis
�� SOBSOB
�� Increased respiratory effortIncreased respiratory effort
�� Small increase in respiratory rateSmall increase in respiratory rate
�� InspiratoryInspiratory symptomssymptoms
�� StridorStridor
�� Drooling Drooling
�� hoarsenesshoarseness
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CroupCroup
�� ParainfluenzaParainfluenza virusvirus
�� URTI/ cold prior to illnessURTI/ cold prior to illness
�� Increased temperatureIncreased temperature
�� Barking coughBarking cough
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Croup ScoreCroup Score
LethargicLethargicAnxious/ Anxious/
aggitatedaggitatedRestless Restless
when when
disturbeddisturbed
NNConscious Conscious
levellevel
CyanosisCyanosisNNNNNNColourColour
SevereSevereModerateModerateMild (Mild (s/cs/c))NoNoRecessionRecession
SevereSevereAt restAt restWhen When
agitated agitated NoNoStridorStridor
3322110 0
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EpiglottitisEpiglottitis
�� H H InfluenzaeInfluenzae type Btype B
�� TemperatureTemperature
�� DroolingDrooling
�� Severe respiratory distress and Severe respiratory distress and stridorstridor
�� Hyperextension of the neck Hyperextension of the neck
�� IrritabilityIrritability
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Management Management
�� Keep the child calmKeep the child calm
�� Do not examine the throatDo not examine the throat
�� Oxygen Oxygen
�� Croup:Croup: DexamethasoneDexamethasone
PulmicortPulmicort nebulisersnebulisers
�� Severe croup:Severe croup: Adrenaline Adrenaline nebulisednebulised
�� EpiglottitisEpiglottitis:: Intravenous Intravenous AmpicillinAmpicillin and and
ChloramphenicolChloramphenicol
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LaryngomalaciaLaryngomalacia
�� StridorStridor appearing after the first few appearing after the first few
days of lifedays of life
�� Floppy larynx collapse of the airway on Floppy larynx collapse of the airway on
inspirationinspiration
�� Most resolve with growth and Most resolve with growth and
development of the airwaydevelopment of the airway
�� Severe cases may require Severe cases may require
tracheostomytracheostomy
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Lower Respiratory Tract Lower Respiratory Tract
�� Acute: InfectiveAcute: Infective
�� Chronic: Asthma Chronic: Asthma
Cystic fibrosisCystic fibrosis
BronchiectasisBronchiectasis
BronchiolitisBronchiolitis obliteransobliterans
CongenitalCongenital
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SymptomsSymptoms
�� SOBSOB
�� CoughCough
�� WheezeWheeze
�� TiredTired
�� Irritability Irritability
�� Decreased feeding Decreased feeding
�� Abdominal painAbdominal pain
�� FeverFever
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SignsSigns
�� TachypnoeaTachypnoea
�� Tachycardia Tachycardia
�� Decreased oxygenationDecreased oxygenation
�� Respiratory distressRespiratory distress
�� Wheeze/Wheeze/crepitationscrepitations/crackles/crackles
�� Decreased air entryDecreased air entry
�� Silent chest!Silent chest!
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Effort of breathingSubcostal recession
mildmild severesevere
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Severe Severe
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Investigations Investigations
�� Oxygenation Oxygenation
�� CXRCXR
�� BloodsBloods
�� Cough swab/ Cough swab/
sputumsputum
�� NPA/PNANPA/PNA
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BronchiolitisBronchiolitis
�� RSVRSV
�� Decreased feeding Decreased feeding
�� Wheezy coughWheezy cough
�� Respiratory distressRespiratory distress
�� On auscultation crackles and wheezeOn auscultation crackles and wheeze
�� NPANPA
�� Supportive treatmentSupportive treatment
�� PalivizmabPalivizmab
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BronchiolitisBronchiolitis obiteransobiterans
�� Secondary to infection, adenovirusSecondary to infection, adenovirus
�� Bronchioles are partially or completely Bronchioles are partially or completely obliterated by nodular masses which obliterated by nodular masses which contain granulation and contain granulation and fibroticfibrotic tissuetissue
�� Respiratory illness followed by Respiratory illness followed by improvementimprovement
�� Then deterioration with cough, Then deterioration with cough, wheeze , SOB and sputum productionwheeze , SOB and sputum production
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�� CT scan on inspiration and expirationCT scan on inspiration and expiration
�� Management difficultManagement difficult
�� SwyerSwyer James syndrome: unilateral James syndrome: unilateral
hyperlucenthyperlucent lung syndromelung syndrome
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BronchiectasisBronchiectasis
�� Dilatation of the bronchiDilatation of the bronchi
�� Inflammatory destruction of bronchial and Inflammatory destruction of bronchial and peribronchialperibronchial tissuetissue
�� Accumulation of Accumulation of exudativeexudative material in material in dependent bronchidependent bronchi
�� Distension of dependent bronchiDistension of dependent bronchi
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Causes:Causes:
�� Cystic fibrosisCystic fibrosis
Pulmonary infectionPulmonary infection
Recurrent AspirationRecurrent Aspiration
Primary Primary CiliaryCiliary DyskinesiaDyskinesia
Immune DeficiencyImmune Deficiency
Inhalation of a foreign bodyInhalation of a foreign body
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SymptomsSymptoms
�� Cough, productiveCough, productive
�� Recurrent chest infectionsRecurrent chest infections
�� Poor weight gainPoor weight gain
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Diagnosis + Treatment Diagnosis + Treatment
�� CT scanCT scan
�� Cough swabsCough swabs
�� PhysiotherapyPhysiotherapy
�� AntibioticsAntibiotics
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Cystic Fibrosis Cystic Fibrosis
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Times Nov 2006Times Nov 2006
�� Mr Brown, who is expected to succeed Tony Blair as prime ministeMr Brown, who is expected to succeed Tony Blair as prime minister, learnt of r, learnt of the condition of his fourthe condition of his four--month old son Fraser soon after his birth following a month old son Fraser soon after his birth following a series of blood tests.series of blood tests.
�� It is the second devastating tragedy to hit the Browns. In 2002 It is the second devastating tragedy to hit the Browns. In 2002 they lost their they lost their first baby, Jennifer Jane, who was born seven weeks premature anfirst baby, Jennifer Jane, who was born seven weeks premature and suffered d suffered a brain haemorrhage shortly before she died. a brain haemorrhage shortly before she died.
�� Fraser Brown was born in July at Edinburgh’s Royal Infirmary. HeFraser Brown was born in July at Edinburgh’s Royal Infirmary. He weighed a weighed a healthy 7 lb 14 ounces. The condition was picked up in the routihealthy 7 lb 14 ounces. The condition was picked up in the routine series of ne series of blood tests that all babies in Scotland undergo.blood tests that all babies in Scotland undergo.
�� The news shocked Westminster tonight, particularly as it dawned The news shocked Westminster tonight, particularly as it dawned that Mr that Mr Brown would have known of the tragedy in the middle of Labour’s Brown would have known of the tragedy in the middle of Labour’s leadership leadership crisis in the early autumn when he was accused by some MPs of plcrisis in the early autumn when he was accused by some MPs of plotting otting against Mr Blair. against Mr Blair.
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Cystic FibrosisCystic Fibrosis
•• 1in 25 population carrier1in 25 population carrier
•• 1 in 2500 newborn babies have CF1 in 2500 newborn babies have CF
�� 7500 babies ,children and young adults affected7500 babies ,children and young adults affected
�� 1 born almost every day ( 5 a week )1 born almost every day ( 5 a week )
�� 3 young lives lost weekly3 young lives lost weekly
�� Most common life threatening inherited diseaseMost common life threatening inherited disease
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AutosomalAutosomal recessive inheritancerecessive inheritance
chromosome 7chromosome 7
Carrier parent Carrier parent
Affected Carrier Carrier Not ababy baby baby carrier
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�� Cystic Fibrosis Cystic Fibrosis TransmembraneTransmembrane
conductance regulatorconductance regulator
�� Transports salt and water in and out Transports salt and water in and out
of cellsof cells
�� CFTR either faulty or absent CFTR either faulty or absent
(depending on gene defect)(depending on gene defect)
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�� Unable to secrete chlorideUnable to secrete chloride
�� Excess sodium absorbed Excess sodium absorbed
�� Water follows sodiumWater follows sodium
�� Sticky mucus clogs up lungs and Sticky mucus clogs up lungs and
digestive systemdigestive system
�� In sweat glands doesn’t absorb In sweat glands doesn’t absorb
chloridechloride
–– Salty sweatSalty sweat
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Presentation Presentation
�� MeconiumMeconium IleusIleus
�� Recurrent chest infections/ recurrent cough/ productive/”asthma”Recurrent chest infections/ recurrent cough/ productive/”asthma”
�� Failure to thriveFailure to thrive
�� Nasal polypsNasal polyps
�� Rectal Rectal prolapseprolapse
�� Oedema secondary to low albuminOedema secondary to low albumin
�� Prolonged conjugated neonatal jaundiceProlonged conjugated neonatal jaundice
�� Family history of cystic fibrosisFamily history of cystic fibrosis
�� Diarrhoea/ frequent large bulky offensive stoolsDiarrhoea/ frequent large bulky offensive stools
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Diagnosis/sweat testDiagnosis/sweat test
�� Sweat collected from forearmSweat collected from forearm
�� Painless electrical impulses across skinPainless electrical impulses across skin
�� Sweat collected 1g/m2/minSweat collected 1g/m2/min
�� 2/52 baby greater than 3 kg well hydrated2/52 baby greater than 3 kg well hydrated
�� Sweat chloride most accurateSweat chloride most accurate–– >60 >60 mmol/lmmol/l chloride diagnosticchloride diagnostic
–– 4040--60 60 mmolsmmols suggestivesuggestive
–– < 40 < 40 mmolsmmols normal , low possibilitynormal , low possibility
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�� Newborn screening 2007Newborn screening 2007
�� Genetic testing: Delta F508 x 2 Genetic testing: Delta F508 x 2 75%75%
�� 31 mutations 90% ( 20 common)31 mutations 90% ( 20 common)
�� Carrier cascade testingCarrier cascade testing-- blood or blood or mouth swabmouth swab
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Other investigationsOther investigations
�� Faecal Faecal elastaseelastase
�� CXRCXR
�� Cough swab or sputumCough swab or sputum
�� Lung function testsLung function tests
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Initial treatment Initial treatment
�� PhysiotherapyPhysiotherapy
�� Dietary advice and Dietary advice and creoncreon
�� FlucloxacillinFlucloxacillin
�� Vitamin KVitamin K
�� Vitamin EVitamin E
�� Ketovite/dalavitKetovite/dalavit
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Complications:Complications:
�� LUNGSLUNGS
�� Thick secretionsThick secretions–– Recurrent infectionsRecurrent infections
–– BronchiectasisBronchiectasis
–– HaemoptysisHaemoptysis
�� 85 % have pancreatic insufficiency 85 % have pancreatic insufficiency
�� MalnutritionMalnutrition
�� Constipation/DiarrhoeaConstipation/Diarrhoea
�� DIOS DIOS
�� Liver diseaseLiver disease
�� DiabetesDiabetes
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Others Others
�� Gastro oesophageal refluxGastro oesophageal reflux
�� Stress incontinence Stress incontinence -- recurrent coughingrecurrent coughing
�� Anal Anal prolapseprolapse-- coughing and constipationcoughing and constipation
�� Nasal polyps Nasal polyps
�� SinusitisSinusitis
�� InfertilityInfertility–– Men Almost all infertile due to thick secretionsMen Almost all infertile due to thick secretions
–– Female Weight relatedFemale Weight related
�� Osteoporosis and Osteoporosis and osteopeniaosteopenia
�� Dehydration due to high sweat lossDehydration due to high sweat loss
�� Arthritis Arthritis ––large jointslarge joints
�� DepressionDepression
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Infection Infection
�� Very vulnerable groupVery vulnerable group
�� Infections have great impact on life Infections have great impact on life expectancyexpectancy
�� Separate clinics Separate clinics
�� CF patients separated on ward CF patients separated on ward
�� CF group activities not encouragedCF group activities not encouraged
�� Separate classes at schoolSeparate classes at school
�� Separate judo classes etcSeparate judo classes etc
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�� AsthmaAsthma
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Diagnosis is clinicalDiagnosis is clinical
�� Symptoms:Symptoms:
�� WheezingWheezing
�� CoughCough
�� Difficulty breathingDifficulty breathing
�� Chest tightnessChest tightness
�� Interval symptomsInterval symptoms
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Symptoms Symptoms
�� Frequent and recurrentFrequent and recurrent
�� Worse at night and in the early Worse at night and in the early
morningmorning
�� Worse after or occur in response to Worse after or occur in response to
exercise or other triggersexercise or other triggers
�� History of History of atopicatopic disorderdisorder
�� Family historyFamily history
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SignsSigns
�� HarrisonsHarrisons SulciSulci
�� Signs of respiratory distressSigns of respiratory distress
�� WheezeWheeze
�� Features of Features of atopyatopy
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Features that lower the Features that lower the
chance of asthmachance of asthma
�� Symptoms with colds onlySymptoms with colds only
�� Isolated coughIsolated cough
�� History of a moist coughHistory of a moist cough
�� Prominent dizziness, light headedness, Prominent dizziness, light headedness,
peripheral tingling.peripheral tingling.
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Question diagnosisQuestion diagnosis
�� Symptoms present from birthSymptoms present from birth
�� FH of unusual chest diseaseFH of unusual chest disease
�� Excessive vomitingExcessive vomiting
�� InspiratoryInspiratory stridorstridor
�� Abnormal voice or cryAbnormal voice or cry
�� Failure to thriveFailure to thrive
�� ClubbingClubbing
�� Failure to respond to conventional treatmentFailure to respond to conventional treatment
�� Unexpected clinical findingsUnexpected clinical findings
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Aims of managementAims of management
�� Control of symptomsControl of symptoms
�� Prevention of exacerbations Prevention of exacerbations
�� Achieve best possible pulmonary Achieve best possible pulmonary
functionfunction
�� Minimal side effectsMinimal side effects
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Control of symptoms Control of symptoms
�� No daytime symptomsNo daytime symptoms
�� No night time wakeningNo night time wakening
�� No need for rescue medicationNo need for rescue medication
�� No limitations on activityNo limitations on activity
�� Normal lung functionNormal lung function
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ManagementManagement
�� Step wise approachStep wise approach
�� Start treatment at the most Start treatment at the most
appropriate stepappropriate step
�� Before initiating a new step check Before initiating a new step check
compliance and inhaler techniquecompliance and inhaler technique
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InhalersInhalers
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ColoursColours
�� BrownBrown
�� PurplePurple
�� GreenGreen
�� BlueBlue
�� Grey and greenGrey and green
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Treatment step one: mild Treatment step one: mild
intermittent asthmaintermittent asthma
�� Inhaled Short Inhaled Short
acting B2 agonist as acting B2 agonist as
requiredrequired
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Step 2 :Introduce a regular Step 2 :Introduce a regular
preventerpreventer therapytherapy
�� Regular Regular preventerpreventer therapytherapy
�� Start at dose appropriate to severity of Start at dose appropriate to severity of
diseasedisease
�� Children: 200mcg per dayChildren: 200mcg per day
�� Titrate the dose to the lowest dose at Titrate the dose to the lowest dose at
which effective control of asthma is which effective control of asthma is
maintainedmaintained
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When?When?
�� Frequent exacerbations of asthmaFrequent exacerbations of asthma
�� Using inhaled B2 agonists 3x a week Using inhaled B2 agonists 3x a week
or moreor more
�� Symptoms 3x a week or moreSymptoms 3x a week or more
�� Waking one night a weekWaking one night a week
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Step 3: add on therapyStep 3: add on therapy
�� When taking 400mcg per day of When taking 400mcg per day of
steroid inhalersteroid inhaler
�� Inhaled long acting B2 agonistInhaled long acting B2 agonist
�� LeukotrieneLeukotriene receptor antagonistreceptor antagonist
�� TheophyllinesTheophyllines
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Step 4: poor control on Step 4: poor control on
moderate dose inhaled moderate dose inhaled
steroid and add on therapysteroid and add on therapy
�� Increase the dose of inhaled steroid Increase the dose of inhaled steroid
up to 800 mcg per dayup to 800 mcg per day
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Step 5Step 5
�� Oral steroidsOral steroids
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Aged < 5yearsAged < 5years
�� Step1:inhaled short acting B2 agonistStep1:inhaled short acting B2 agonist
�� Step 2: inhaled steroid 200Step 2: inhaled steroid 200--
400mcg/day400mcg/day
�� Step3: consider Step3: consider leukotrieneleukotriene receptor receptor
antagonistantagonist
�� Step 4: refer to respiratory Step 4: refer to respiratory
paediatricianpaediatrician
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�� Important to recheck compliance Important to recheck compliance
between each step between each step
�� Step down when ableStep down when able
�� No research on howNo research on how
�� Decrease dose by approx 25 % each Decrease dose by approx 25 % each
timetime
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�� ClenilClenil
�� QvarQvar
�� Adrenal suppressionAdrenal suppression
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Deaths from asthmaDeaths from asthma
�� Chronically severe asthmaChronically severe asthma
�� Increasing use of B2 agonistIncreasing use of B2 agonist
�� Inadequate steroid therapyInadequate steroid therapy
�� Repeated attendance at A+ERepeated attendance at A+E
�� Previous admission in last yearPrevious admission in last year
�� Previous near fatal asthmaPrevious near fatal asthma
�� Requiring 3 or more classes of asthma Requiring 3 or more classes of asthma
medicationmedication
�� Non compliance, DNA, obesityNon compliance, DNA, obesity
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�� Questions?Questions?