Most Common Pediatric Respiratory Diseases

Cystic Fibrosis

Cystic fibrosis is a chronic multisystem Disorder in which is characterized the Dysfunction of exocrine gland.
Cystic fibrosis is an autosomal recessive Disorder
That affects epithelial cells of the respiratory, gastrointestinal, reproductive , integumentary system and leads to abnormal exocrine gland se oration.
Exocrine gland secretes the – mucus, sweat, juices.
Mucus produced by Exocrine gland is abnormally thick causing obstruction of small passage of any system.
Increase sodium and chloride in sweat and saliva is the best Diagnostic test of cystic fibrosis.

Etiology of Cystic fibrosis

Mutation in the cystic fibrosis gene on 7^th chromosome
Abnormal CFTR protein (Cystic fibrosis transmembrane regulator)
Defective chloride transportation
Excessive mucus production
Infection, Atelectasis, Bronchiectasis
Dilation of Distal airway
Chronic hypoxemia
An abnormal elevation of sodium and chloride

Most common features of cystic fibrosis

Pancreatic enzyme Deficiency caused by blockage of Duet
Chronic progressive lung Disease Due to repetitive inflection
Dysfunction of sweat gland, due to Increase Na⁺ and Cl^–

Clinical Manifestations of Cystic fibrosis

effect of cystic fibrosis on the respiratory system

Productive cough and wheezing
Excessive thick mucus production
Obstruction of the respiratory tract due to thick mucus
Repetitive infection in respiratory tract
Coughing and shortness of breath
Chronic hypoxia and clubbing of fingers
Hypertrophy of the muscles of the pulmonary artery
Pneumothorax is a common problem with emphysema
Barrel chest shape
High resonance sound in lungs
Dyspnea and cyanosis
Chronic bronchitis

effect of cystic fibrosis on GI system

The earliest manifestation is = meconium ileus in a neonate
Intestinal obstruction – due to thick intestinal secretion
Nausea , vomiting , abdominal pain and distension
Malnutrition
Diabetes mellitus due to fibrosis
Acute pancreatitis
Steatorrhea
Failure of thrive and rectal prolapsed
Stool are frothy and foul smelling
Hypoalbumia

effect of cystic fibrosis on the Reproductive system

Delay puberty in girls
Reduce fertility chance in females
In male, High chance of infertility

effect of cystic fibrosis on the Integumentary system

Salty test on kiss the child
High concentration of sodium and chloride in sweat
Dehydration due to excessive water loss by sweat
Electrolyte Imbalance

Diagnosis test for cystic fibrosis

Sweat chloride test –

these tests require the sweat

Provide the pilocarpine drug for production the of sweat
Collect the sweat of more than 75mg
Normal sweat chloride concentration is less than 40meq/liter (40mmol/L)
If sweat chloride concentration is more than 60meq/L, indicates cystic fibrous is present
Child less than 6 months – >60meq/L – Positive

– <30meq/L – Negative

Child more than 6month – >60meq/L = Positive

– <40meq/L = Negative

Chest X-ray – for emphysema and atelectasis
Pulmonary function test
Trypsin and chymotrypsin estimation test in stool
Genetic test and sputum culture test

management of cystic fibrosis

Airway clearance is the 1^st intervention
Provide chest physiotherapy to remove the Excessive mucus from the respiratory tract.
Chest physiotherapy produces the vibration on the lungs segment and drainage out of mucus.
Monitor the lung’s sound and respiratory status.
Chest physiotherapy avoids after the feeding because the chance of vomiting and aspiration.
Chest physiotherapy Done only morning and evening
In sever case, Bronchodilator & system inhalation improve the effects of chest physiotherapy
Flutter mucus clearance Devices also use for remove the thick mucus
Special vest Device use for produce high frequency chest wall for Excrete the mucus
Avoid cough suppressant
But Hemoptysis is present use cough suppressant
Last treatment of cystic fibrosis in respiratory tract is – lungs transplant
Provide the high calorie , high protein , and well balance diet
Monitor weight and for failure to thrive
Provide the pancreatic enzyme supplement to maintain the normal mechanism of digestion
Administer of pancreatic enzyme , with meal before 30mint of food
Provide supplement of vitamin A,D,E,K
Avoid the pancreatic enzyme when patient on NPO.
Monitor the stool pattern and for sign of intestinal obstruction
Enteric coated pancreatic enzyme should not be crushed and chewed
Monitor for constipation , intestinal obstruction and rectal prolapse
Monitor sign of gastroesophageal reflux

Other management of cystic fibrosis

Monitor bone growth in child
Provide support to the child for improving growth
Psychological support provide to parents
Monitor blood glucose level to assess the Diabetes
Encourage to in depended in the child to self-care as age appropriate
Educate the parents about the home care
The information provided about signs/symptoms, complication,s and follow-up care
Annual influenza vaccine is recommended for children 6 months of age and older

complications of cystic fibrosis

Bronchiectasis (Damaged airway)
Chronic infection
Nasal polyps
Hemoptysis
Pneumothorax
Respiratory failure

KEY POINTS of cystic fibrosis

Which type of glands are affect in cystic fibrosis – Exocrine glands
Cystic fibrosis is a – Autosomal recessive Disorder
Most common test to assess the cystic fibrosis – Sweat chloride test
Earliest manifestation of cystic fibrosis in GI tract abnormality – Meconium ileus (Neonate does not pass the meconium)
Obstruction of respiratory tract with cystic fibrosis due to – Excessive secrete thick mucus
Early manifestation of cystic fibrosis is suggest the parents – Salty test on kiss the child
Normal sweat chloride concentration in the body is – Less then 40Meg/liter
In cystic fibrosis , sweat chloride concentration is – More than 60meq/liter
Which drugs provide , for production the sweat for the test – Pilocarpine
Which intervention is priority in cystic fibrosis – Airway Cclearance
Vitamins are recommended for cystic fibrosis child – Fat soluble vitamin
High concentration of sodium and chloride is suggestive – Cystic fibrosis

Bronchiolitis in Children

Bronchiolitis is an acute infectious inflammatory disease of the upper respiratory tract and lower respiratory tract that result in obstruction of the small airways.
Bronchiolitis is inflammation of the Bronchioles that causes the production of thick mucus that block the bronchioles and bronchi.
RSV (Respiratory syncytial virus) is a most common cause of Bronchiolitis.
RSV is not an airborne infection, it is highly communicate by contaminated hand.
Bronchiolitis Disease commonly occurs in winter and spring season.
Other causative agent are = Pare influenza virus, influenza virus, adenovirus, mycoplasma pneunmonie.

Risk factors of Bronchiolitis

Prematurity
Low birth weight
Age less than 6-12 weeks
Low socio-economic group
Parental smoking
Chronic lungs Disease
Airway Anomalies
Congenital and acquired immunodeficiency disease
CHD and pulmonary hypertensions
Neurological Disease
House holds crowding.

Clinical manifestation of Bronchiolitis

Initial manifestation – Rhinorrhea, Coughing, Sneezing, Wheezing, Eye or ear drainage, intermitted fever and pharyngitis.
Manifestation as Disease progress – Increase coughing and wheezing.

Sign of air hunger
Poor feeding
Tachypnea and irritability
Period of cyanosis.

Manifestation in severe illness – Tachypnea more than 70 breath/mint

Decrease breath sound
Poor air exchange
Apnea episodes.

What is a respiratory syncytial virus (RSV)

If usually present age between 3 to 12 month
Maximum = 6month
Rare case = after 2 years.

Diagnosis of respiratory syncytial virus (RSV)

History collection and Physical Examination
Chest x-ray
Nasopharyngeal swab/nasal wash
ECG , ECHO
Blood gas analysis
Rapid antigen Detection for RSV
Culture test and PCR
Direct or Indirect Immunofluorescence.
Lungs function test and spirometry.

Management of respiratory syncytial virus (RSV)

Maintain the patient airway.
Humidified o₂Delivered via nasal cannula determined by pulse oximetry.
Slightly extended to maintain an open airway.
Assess the sign of Dehydration and provide adequate IV fluids.
Oral fluid is contraindicated in tachypnea.
Provide proper rest and clustering care for the RSV infection child.
Semi flower and neck slightly extended position provides.
Chest physiotherapy provides for postural drainage.
Remove the secretions and fluids in the pulmonary tract.
Medication:- Ribavirin
Ribavirin medication given in aerosol state throws hood and mask.
Ribavirin is a teratogen in pregnancy
Pregnant nurse and other female is away from RSV-infected child
Ribavirin drug dissolves the soft lenses
Contact lenses wearing person not involved in RSV client care.
Avoid the use of Broncho dilator
Aminophylline drug can be use
Avoid the sedative drugs
A pt. of RSV infection should be Isolated from the other infected child
The nurse care the RSV-infected child, should not be involved in the care of another patient
Maintain proper strict hand washing for minimizing the transmission of infection.

Key Points of Bronchiolitis

Inflammation of Bronchioles that cause obstruction in a small airway is known = Bronchiolitis.
Most common cause of Bronchiolitis = RSV
Which season develops the Bronchiolitis disease condition = Winter season
Bronchiolitis Disease occurs during age of = 3-12month
Priority Nursing management in Bronchiolitis patient = Clear the airway
Which Drugs are use the treat respiratory syncytial virus (RSV) = Ribavirin
Ribavirin Drug teratogen in the = Pregnancy
Which technique use in the hospital to prevent the transmission of RSV = Strict hand washing.

Foreign body Aspiration in children

Foreign body aspiration of a foreign body into the air passes.
Foreign body aspiration commonly occurs in the infant and toddler.
Most inhaled foreign body settle down in the main stem or lobar bronchus.
Large Foreign body aspiration is obstructed the upper airway and cause immediate features.
Small foreign body aspiration is obstructed the Respiratory tract for long time and cause the chronic effects.

Incidence of Foreign body aspiration

Commonly 1-3 years age group affected.
Sex – Male more prominent

Visual foreign bodies are

Organic – Peanuts, Popcorn, Seeds, Hotdogs, Vegetable matter
Inorganic -Toys part, pen tops, tracks, pins, nails, Screw, and bullet and casing

Composition of foreign body

Large foreign body produce clinical feature immediately within 3-5 minutes.
Small foreign body id deposit in secondary bronchus and produce aspiration pneumonia.
Buttons, pins, small parts of toy can cause the irritation and edema in the Respiratory tract.

Anatomical location of Obstruction Foreign body aspiration

Foreign body commonly obstruction the trachea and larynx.
Bronchus is most common site of obstruction, mainly right bronchus have more chances because right bronchus is more horizontal and wide.

Degree of obstruction in Foreign body aspiration

Foreign body aspiration creates the obstruction in inspiration and expiration.
A large foreign body only obstructs the expiration.
Small foreign body do not interfere in inspiration and expiration.

Clinical feature of Foreign body aspiration

The clinical feature of foreign body aspiration depends upon the anatomical location of the obstruction.

Foreign body Obstruction at Larynx

Wheezing sound during expiration

- - Harness of voice
  - Inability to speak
  - Coughing
  - Hemoptysis
  - Dyspnea
  - Cyanosis

Foreign body Obstruction at trachea

Cough,
Dyspnea
Hoarsness of voice and cyanosis
Audiable slap and palpable thud sound.

Foreign body Obstruction at Bronchus

Chocking
coughing
Dyspnea
Hemoptysis

Foreign body aspiration Late sign

Wheezing
Emphysema
Atelactesis.

Diagnosis of Foreign body aspiration

History collection and physical examination.
X-ray (Anterior, posterior and lateral)
Bronchoscopy – To diagnose tracheal obstruction.
Laryngoscope use to find out the obstruction.
Laryngoscope also use in removal of obstruction.
Fluoroscopic Examination

Management of Foreign body aspiration

Early removal of foreign bodies reduces the chance of inflammation and edema.
Below 1-year child – 5 back slaps and 5 Chest thrusts apply
In children, fingers are never inserted to remove foreign bodies because of chance of deep aspiration.
In adults, foreign body remove by Meinlich Maneuver ( abdominal thrush maneuver)
The child does not produce P sound means obstruction is severe
Antibiotics and steroids provide for the treatment of inflammation
Humidified air should be inhaled after the removal of a foreign body.
Treat the foreign body aspiration complication.

Nursing management of foreign body aspiration

Nurse all the small objects should be away from the child
Any person and nurse not set a example by putting any object in the mouth in front of child.
After ingestion the object, Nurse allows a comfortable position.
Nurse never attempt to allowing figure to remove the object.
Nurse Dont need to play with small toys and vegetables, nuts, etc.
Nurse encourage to mother for avoiding the laugh the child during feeding.
Nurse educate the mother never feed child during he is play and running

KEY POINTS Foreign body aspiration

Foreign body aspiration occur which age group children – Infant and toddler.
Which type foreign body aspiration create the aspiratory pneumonia – Small foreign body.
Foreign body commonly obstructed into the –Trachea and Larynx.
Most common site of foreign body obstruction in the bronchus –Right bronchus.
Large foreign body obstruction is interferer in – Expiration.
Audiable slap and palpate thud sounds are occur in the – Tracheal Obstruction
Most common management of foreign body removal in 1 Year children – Give 5 back below between the shoulder.

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