- 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 7th 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
- Diabetes mellitus due to fibrosis
- Acute pancreatitis
- Failure of thrive and rectal prolapsed
- Stool are frothy and foul smelling
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 1st 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
- 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
- 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 o2 Delivered 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
Foreign body Obstruction at trachea
- Hoarsness of voice and cyanosis
- Audiable slap and palpable thud sound.
Foreign body Obstruction at Bronchus
Foreign body aspiration Late sign
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.