Chronic CoughChristina Sun10.8.15

Why do we cough?

•Protective mechanism•Maintains airway patency•Clears retained secretions

•Innervation▫Afferent pathway: cough receptors CN X

medulla▫Efferent pathway: cerebral cortex/medulla

CN X/SLN glottis, external intercostals, diaphragm

Cough receptors•Pharynx•Larynx•Trachea•Major bronchi•Also in: middle

ear, sinuses, pericardium and diaphragm

Chronic cough

•Cough lasting > 3-4 weeks

Chronic cough cases

•Differential diagnoses for stage of childhood

•Pertinent positives•Pertinent negatives•Most likely diagnosis•Investigations (briefly)

Case 1• ID: 8-month-old girl • CC: cough on and off for 2 months• HPI

▫ Cough is worse with eating and lying down, and at times her cry is hoarse.▫ No wheezing▫ Attends child care; got sent home on several occasions because of persistent

coughing, and sometimes spitting up after eating• PMHx

▫ RSV bronchiolitis at age 4 months, 2 nights in the hospital with supplemental O2

• Meds▫ No meds, no allergies. Immunizations are up to date

• FHx▫ Past asthma in the mother▫ Father smokes, but mother insists that the smoking is exclusively outside

• O/E▫ Afebrile, RR 24, HR 100, spO2 99% RA ▫ HEENT: pale, swollen nasal mucosa with clear discharge; otherwise normal

exam▫ Resp: normal chest shape, no retractions. Occasional bilat. expiratory wheezes▫ CVS and abdo exam findings are normal. No finger clubbing or skin rash

Case 1 – cough in the infant

• Pertinent positives▫ Intermittent cough▫ Hoarse cry▫ Worsens with feedings and

lying down▫ Sick contacts▫ Pale, swollen nasal

mucosa▫ Bilateral expiratory

wheeze▫ Hx of RSV bronchiolitis▫ FHx of asthma▫ Smoking environment

• Pertinent negatives▫ No fever▫ Normal chest shape▫ No cardiac murmur▫ No clubbing▫ No rash

DDx: asthma, aspiration, congenital airway disease, cystic fibrosis, infections, environmental, congenital heart disease

What if…

1. She was born premature▫Bronchopulmonary dysplasia

2. She had frequent pneumonia▫Transesophageal fistula

3. She had failed to gain weight▫Cystic fibrosis

Case 1 - asthma• ID: 8-month-old girl • CC: cough on and off for 2 months• HPI

▫ Cough is worse with eating and lying down, and at times her cry is hoarse.▫ No wheezing▫ Attends child care; got sent home on several occasions because of persistent

coughing, and sometimes spitting up after eating• PMHx

▫ RSV bronchiolitis at age 4 months, 2 nights in the hospital with supplemental O2

• Meds▫ No meds, no allergies. Immunizations are up to date

• FHx▫ Past asthma in the mother▫ Father smokes, but mother insists that the smoking is exclusively outside

• O/E▫ Afebrile, RR 24, HR 100, spO2 99% RA ▫ HEENT: pale, swollen nasal mucosa with clear discharge; otherwise normal

exam▫ Resp: normal chest shape, no retractions. Occasional bilat. expiratory wheezes▫ CVS and abdo exam findings are normal. No finger clubbing or skin rash

Case 2• ID: 3-year-old boy presents to your office in the summer• CC: nonproductive cough for 3 to 4 weeks• HPI

▫ Cough started during the summer; no rhinorrhea, no fever▫ Worse with running▫ Initially had significant night cough, but now mostly resolved▫ No witnessed choking episode▫ Has had colds with cough in the past, but not for such a prolonged time

• FHx▫ No family history of asthma or eczema

• SocHx▫ No reported environmental smoke exposure▫ He has not attended child care

• O/E▫ Afebrile, RR 28, HR 80, spO2 96% RA ▫ Well-nourished, no chest retractions▫ Resp: decreased AE on left lower side of chest, no crackles or wheezes▫ All other exam findings are normal; no finger clubbing or skin rash

Case 2 – cough in the toddler

• Pertinent positives▫ Worsens with activity▫ Decreased air entry to

left lung base

• Pertinent negatives▫ No URI symptoms▫ No wheezing▫ No fever▫ Well-nourished▫ No clubbing▫ No rash▫ No FHx of atopy▫ No sick contacts

DDx: asthma, foreign body, bronchiectasis (CF, ciliary dyskinesia, immunodeficiency, post0-infectious), chronic middle ear disease, allergy, sinusitis

• ID: 3-year-old boy • CC: nonproductive cough for 3 to 4 weeks• HPI

▫ Cough started during the summer; no rhinorrhea, no fever▫ Worse with running▫ Initially had significant night cough, but now mostly resolved▫ No witnessed choking episode▫ Has had colds with cough in the past, but not for such a prolonged time

• FHx▫ No family history of asthma or eczema

• SocHx▫ No reported environmental smoke exposure▫ He has not attended child care

• O/E▫ Afebrile, RR 28, HR 80, spO2 96% RA ▫ Well-nourished, no chest retractions▫ Resp: decreased AE on left lower side of chest, no crackles or wheezes▫ All other exam findings are normal; no finger clubbing or skin rash

Case 2 – foreign body aspiration

Foreign body aspiration

Case 3• ID: 15-year-old boy presents to your office midwinter• CC: increased coughing for 6 to 8 weeks• HPI

▫ Cough started with mild nasal congestion and rhinorrhea in the first week▫ Over time became a harsh, barking, repetitive cough that occurs several

times per minute for hours on end▫ Several instances in which the cough has led to vomiting▫ No fever, no wheezing, no further nasal symptoms since the first week▫ Able to sleep comfortably, with no night cough▫ Tends to have spring and fall rhinorrhea

• PMHx▫ Bronchiolitis and eczema in infancy

• O/E (you immediately note a harsh, barking cough, which persists throughout the entire exam)▫ RR 16, HR 70, spO2 99% RA ▫ HEENT: pale nasal mucosa, no subconjunctival hemorrhages, no sinus

tenderness. Tympanic membranes are normal▫ Resp: normal chest shape, lungs are clear bilaterally▫ Other exams findings are normal. There is no cyanosis, clubbing, or eczema.

Case 3 – cough in the adolescent

• Pertinent positives▫ Harsh, barky cough▫ Repetitive (not

paroxysmal)▫ Precipitated by URTI▫ Hx of eczema and

bronchiolitis

• Pertinent negatives▫ No wheezing or

rhinorrhea▫ No fever▫ No night cough

DDx: asthma, infection, smoking, bronchiectasis (CF, ciliary dyskinesia, postinfectious), occupational exposure, sinusitis, postnasal drip, chest tumor, psychogenic cough

• ID: 15-year-old boy presents to your office midwinter• CC: increased coughing for 6 to 8 weeks• HPI

▫ Cough started with mild nasal congestion and rhinorrhea in the first week▫ Over time became a harsh, barking, repetitive cough that occurs several

times per minute for hours on end▫ Several instances in which the cough has led to vomiting▫ No fever, no wheezing, no further nasal symptoms since the first week▫ Able to sleep comfortably, with no night cough▫ Tends to have spring and fall rhinorrhea

• PMHx▫ Bronchiolitis and eczema in infancy

• O/E (you immediately note a harsh, barking cough, which persists throughout the entire exam)▫ RR 16, HR 70, spO2 99% RA ▫ HEENT: pale nasal mucosa, no subconjunctival hemorrhages, no sinus

tenderness. Tympanic membranes are normal▫ Resp: normal chest shape, lungs are clear bilaterally▫ Other exams findings are normal. There is no cyanosis, clubbing, or eczema.

Case 3 – psychogenic cough

Summary: chronic cough

Focused history• Age of child• Nature of cough• Stridor or wheezing• Timing and season• Sputum, presence and

character• Exposure to infection• Response to previous

therapy• Family history of atopy,

asthma, eczema, cystic fibrosis

Focused exam• Nutrition and growth• Upper respiratory tract:

ear, nose, sinuses• Chest: AP thoracic

diameter, lung sounds, cardiac exam

• Extremities: clubbing• Skin: eczema

PAL Case

•You are a doctor in a northern community•Jordan is an 8 year old boy with a chronic

cough productive of mucoid sputum. Lately he has been having problems with increasing duration, frequency, and severity of chest infections

What can it be?

What else do you want to know?

DDx: asthma, foreign body, infection, aspiration, post-infection, CF, ciliary dyskinesia, immunodeficiency, chronic middle ear disease, allergy, sinusitis

PAL Case

•His parents bring him to clinic because the father's great uncle died of recurrent pneumonia as a child in Ireland. They are wondering if this might represent a genetic problem

•He seems to be hungry all the time but is unable to gain weight

•As you begin the examination you observe a blue eyed blond thin child▫Height: 25th percentile ▫Weight: < 5th percentile

Chronic cough in the young childWhat we know• Caucasian• Northern community• Productive cough with

mucoid sputum• Remote FHx of recurrent

childhood pneumonia• Increased chest infections

(duration, frequency and severity)

• Failure to gain weight

What else do you want to know• Respiratory symptoms• Growth curve, nutrition• Atopy• Infection exposure (esp.

TB)• Review of systems• FHx of autoimmune,

metabolic disorders• Previous investigations• Previous treatments

Physical exam• Vitals• General appearance

▫ Poor growth, thinness• HEENT

▫ Shiners, Dennie-Morgan line▫ Swollen nasal turbinates▫ Nasal obstruction/polyps▫ Cleft palate▫ OM, middle ear effusion, TM scarring

• Resp▫ Increased WOB, retractions, accessory muscle use▫ Chest wall hyperinflation or deformity▫ Abnormal breath sounds

Physical exam• Cardiac

▫ Murmurs, extra heart sounds

• GI▫ Abdominal masses,

bloating▫ Hepatosplenomegaly▫ Rectal prolapse

• Neuro▫ Poor tone, muscle

weakness▫ Cranial nerve deficits

• Periphery▫ Peripheral edema▫ Cyanosis▫ Clubbing

• Derm▫ Rashes▫ Skin infections

• Dysmorphic features

Investigations

•Labs▫CBC + diff, lytes, IgA, IgG levels▫Throat swab, sputum Gram stain and C&S▫Sweat chloride measurement

•Imaging▫Chest x-ray 2 views

•Other tests to consider▫PFT▫Tuberculin test

Definitive tests for CF

• Newborn screening▫ Two serial assays▫ If first assay is abnormal,

retest with a second assay

• IRT assay▫ Serum immunoreactive

trypsinogen▫ Elevated in newborns

with CF• DNA assay

▫ DNA analysis for mutations in CF gene

Next step

•The history and exam are consistent with your thoughts and consequently you order some confirmatory tests. These come back positive in 3 weeks time and so you have Jordan's parents in for a second visit.

•What topics will you touch upon in your session with Jordan and his family?

•Outline short and long term management goals

What is CF?

•Life-threatening, multisystem disorder, caused by gene mutation

•Chronic, progressive, obstructive lung disease

•Plus other systemic manifestations▫Pancreas: enzyme and insulin insufficiency▫Liver: cirrhosis▫Intestines: malabsorption, ileus, obstruction▫Sinuses: sinusitus, polyps▫Sex organs: male infertility

How did my child get CF?

•Autosomal recessive▫Both parents are carriers

•Mostly affects Caucasian newborns▫1 in 3,000 Caucasians▫1 in 17,000 blacks▫1 in 31,000 Asians

Gene mutation in CF

CF and sticky mucus

Normal Na+ channel

CFTRCystic fibrosis

Mutant CFTR

Airway and pancreas

Lung

Sweat chloride

Liver and gallbladder

GI tract

Multidisciplinary approach

•Physicians: respirology, GI, endocrinology•Nurse educator•RTs/chest physio•Dietician•Social worker

Treatment: lung disease

•CFTR modulators▫Ivacaftor: main indication for G551D

mutation; restores mutant CFTR protein function

▫$$$$•Antibiotics

▫Chronic antibiotic treatment not recommended

▫Azithromycin: anti-inflammatory▫Inhaled tobramycin

•Bronchodilators

Treatment: lung disease

•Airway secretion clearance▫Inhaled DNase I (dornase alfa)▫Inhaled hypertonic saline

•Chest physio + airway clearance techniques

•Exercise•Vaccinations

▫Influenza▫Pneumococcus▫RSV (palivizumab/Synagis)

Treatment: pancreatic disease

•Pancreatic enzyme replacement▫Lipase + protease + amylase

•Nutrition support▫Oral supplement (Ensure, Pediasure, etc.)▫Tube feeds (G-tube)

•Fat-soluble vitamin (ADEK) replacement

Prognosis?

•Median survival = 41.1 years▫Can pursue typical childhood activities,

participate in sports and high-level education

•Early diagnosis + multidisciplinary care = better prognosis

•Chronic respiratory infection is the most common complication and most common cause of death

References and resources

•References▫Chronic Cough in Children – Pediatric in

Review, 2013▫Approach to Chronic Cough in Children -

UpToDate▫Cystic Fibrosis – Pediatric in Review, 2009 &

2014▫Cystic Fibrosis - UpToDate

•Resources▫Cystic Fibrosis Canada▫CFTR.info