Respiratory DisordersRespiratory Disorders

Dr Tanya RobertsonDr Tanya Robertson

AimsAims

�� Overview of respiratory problems in Overview of respiratory problems in childrenchildren

�� DiagnosisDiagnosis

�� ManagementManagement

�� AsthmaAsthma

�� Cystic fibrosisCystic fibrosis

Why are children different?Why are children different?

�� Immune statusImmune status

�� Structure and function respiratory Structure and function respiratory

systemsystem

�� How do you classify respiratory How do you classify respiratory

disorders?disorders?

�� Upper respiratory TractUpper respiratory Tract

�� Lower respiratory TractLower respiratory Tract

�� AcuteAcute

�� ChronicChronic

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

DiagnosisDiagnosis

�� SOBSOB

�� Increased respiratory effortIncreased respiratory effort

�� Small increase in respiratory rateSmall increase in respiratory rate

�� InspiratoryInspiratory symptomssymptoms

�� StridorStridor

�� Drooling Drooling

�� hoarsenesshoarseness

CroupCroup

�� ParainfluenzaParainfluenza virusvirus

�� URTI/ cold prior to illnessURTI/ cold prior to illness

�� Increased temperatureIncreased temperature

�� Barking coughBarking cough

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

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

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

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

Lower Respiratory Tract Lower Respiratory Tract

�� Acute: InfectiveAcute: Infective

�� Chronic: Asthma Chronic: Asthma

Cystic fibrosisCystic fibrosis

BronchiectasisBronchiectasis

BronchiolitisBronchiolitis obliteransobliterans

CongenitalCongenital

SymptomsSymptoms

�� SOBSOB

�� CoughCough

�� WheezeWheeze

�� TiredTired

�� Irritability Irritability

�� Decreased feeding Decreased feeding

�� Abdominal painAbdominal pain

�� FeverFever

SignsSigns

�� TachypnoeaTachypnoea

�� Tachycardia Tachycardia

�� Decreased oxygenationDecreased oxygenation

�� Respiratory distressRespiratory distress

�� Wheeze/Wheeze/crepitationscrepitations/crackles/crackles

�� Decreased air entryDecreased air entry

�� Silent chest!Silent chest!

Effort of breathingSubcostal recession

mildmild severesevere

Severe Severe

Investigations Investigations

�� Oxygenation Oxygenation

�� CXRCXR

�� BloodsBloods

�� Cough swab/ Cough swab/

sputumsputum

�� NPA/PNANPA/PNA

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

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

�� 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

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

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

SymptomsSymptoms

�� Cough, productiveCough, productive

�� Recurrent chest infectionsRecurrent chest infections

�� Poor weight gainPoor weight gain

Diagnosis + Treatment Diagnosis + Treatment

�� CT scanCT scan

�� Cough swabsCough swabs

�� PhysiotherapyPhysiotherapy

�� AntibioticsAntibiotics

Cystic Fibrosis Cystic Fibrosis

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.

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

AutosomalAutosomal recessive inheritancerecessive inheritance

chromosome 7chromosome 7

Carrier parent Carrier parent

Affected Carrier Carrier Not ababy baby baby carrier

�� 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)

�� 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

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

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

�� 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

Other investigationsOther investigations

�� Faecal Faecal elastaseelastase

�� CXRCXR

�� Cough swab or sputumCough swab or sputum

�� Lung function testsLung function tests

Initial treatment Initial treatment

�� PhysiotherapyPhysiotherapy

�� Dietary advice and Dietary advice and creoncreon

�� FlucloxacillinFlucloxacillin

�� Vitamin KVitamin K

�� Vitamin EVitamin E

�� Ketovite/dalavitKetovite/dalavit

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

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

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

�� AsthmaAsthma

Diagnosis is clinicalDiagnosis is clinical

�� Symptoms:Symptoms:

�� WheezingWheezing

�� CoughCough

�� Difficulty breathingDifficulty breathing

�� Chest tightnessChest tightness

�� Interval symptomsInterval symptoms

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

SignsSigns

�� HarrisonsHarrisons SulciSulci

�� Signs of respiratory distressSigns of respiratory distress

�� WheezeWheeze

�� Features of Features of atopyatopy

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.

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

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

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

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

InhalersInhalers

ColoursColours

�� BrownBrown

�� PurplePurple

�� GreenGreen

�� BlueBlue

�� Grey and greenGrey and green

Treatment step one: mild Treatment step one: mild

intermittent asthmaintermittent asthma

�� Inhaled Short Inhaled Short

acting B2 agonist as acting B2 agonist as

requiredrequired

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

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

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

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

Step 5Step 5

�� Oral steroidsOral steroids

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

�� 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

�� ClenilClenil

�� QvarQvar

�� Adrenal suppressionAdrenal suppression

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

�� Questions?Questions?