Pediatric Nursing

Islamic University

Nursing College

DR. Areefa El-Bahri (Alkasseh) Assistance Prof. of MCH

Chapter I

Introduction

• Definition of Pediatric

Nursing

• It is the art and science of giving nursing care to children from birth through adolescent with emphasis on the physical growth, mental, emotional and psycho-social development.

• Aims of Pediatric Nursing

• The main aims of pediatric nurse are to:

• Preventing child’s disease or injury.

• Assisting children, including those with a permanent disability

• Achieve and maintain an optimum level of health and development.

• Treating and rehabilitating children whom have health deviations.

Specialists of this field are known as pediatric nurse. In comparison to other fields of nursing practice pediatric nursing is very wide in scope.

In adult nursing there are different specialty fields like. - Cardiac - Mental health - Emergency care nursing

Pediatric nursing or child health nursing is a specialized nursing practice branch which deals with the health of babies, young children and

adolescents.

A pediatric nurse has to undergo special training in

child health and child behavior after their under

graduate studies where they are trained in child health

both in theory and clinical practices.

Pediatric nursing also focuses on the healthy growth

and development of a child not only at a physical level

but also at mental level.

In pediatric nursing education and awareness of the family

members specially mothers is a very critical and important

aspects because child health is a continuous process and it

requires that families should be educated on how to take

care of child and provide them a healthy growth

opportunity.

Scope of Pediatric Nursing

Practices

• 1) Health care giver • 2) Health teaching • 3) Support /Counseling • 4) Therapeutic role • 5)Coordination/Collaboration • 6) Health care planning • 7) Ethical decision making • 8) Research • 9) Family advocacy

Nurses have the responsibility

in providing nursing

interventions either in

ambulatory or institutional

settings.

Ambulatory setting: such as

home, schools and or

physician’s clinic where

children and their parents have

heath or counseling needs.

Institutional settings are mainly

hospitals (general or specialized

hospitals) where care is

provided to sick children and

their parents.

Role of the Pediatric Nurse

• All children needs:-

• 1. To be free from discriminations.

• 2. To develop physically and mentally, morally and spiritually in

freedom and dignity.

• 3. To have a name and nationality

• 4. To have adequate nutrition, housing, recreation and medical

services.

• 5. To receive love, understanding and maternal security

• 6. To receive an education and develop his /her abilities.

• 7. To be first to receive protection and relief in disaster

• 8. To be protected against all forms of neglect, cruelty and

exploitation.

• 9. To be brought up in a spirit of understanding tolerance,

friendship among peoples.

Child rights

Nursing Assessment Stage:

• Assessing patient health status

(physical and emotional health)

accurately.

• The child and family members must

be included in the assessment.

• Interviewing parents involves more

than just fact gathering; this initial

contact establishes the nature of future

contacts and begins development of a

trusting relationship with the nurse.

• Begin the interview with an

introduction; explain the nurse’s role

and the purpose of the interview to

establish a clear nurse to child/parent

relationship.

• Treat the child/adolescent and

parent as partners equal

• Identifying wellness, actual and potential individualized child/family needs and problems in order to accomplish mutual goals.

• The accuracy of the diagnosis is depends on the comprehensiveness of the available data base.

• Nursing care of infants and children is consistent with the definition of nursing as ―the diagnosis and treatment of human responses to actual or potential health problems‖.

Nursing Diagnosis Stage:

. Planning Stage: • After nursing diagnosis has been established, the

paediatric nurse collaborates with the patient to establish mutual patient-center goal.

• Essential steps of planning phase are prioritizing the nursing diagnosis and selecting nursing intervention that will be help the patient to achieve the goals.

Implementation Stage:

This is the stage of put the plan into action (goal directed care) The nurse initiates and completes the intervention designed to help patient to achieve the goals and demonstrate the specified outcome criteria.

. Evaluation Stage: • This stage involves reassessing child status to determine

the progress toward the goals. • To evaluate the progress and the effect of nursing

intervention, the nurse should compare the current status, abilities and knowledge with the previous assessment.

Chapter 2 Health during Infancy & Childhood

CHILD HEALTH NURSING: Pediatric nursing also focuses on

the healthy growth and development of a child not only at a physical level but also at mental level.

education & awareness of the family members specially mothers is a very critical & important aspects because child health is a continuous process & it requires that families should be educated on how to provide them a healthy growth opportunity.

• In neonatal pediatric nursing a nurse has to take care of the most basic requirement of the patient which needs a very hard and in depth training process.

• Pediatric nursing also incorporates emergency medical management and intensive care for the children of different age groups.

Pediatric Nursing

• Concerns with care of infants and children:

• Preventing disease or injury

• Assisting children, including those with permanent disability or health problems to achieve and maintain an optimum level of health and development.

• Treating or rehabilitating children with health deviations.

Characteristics of a Pediatric Nurse:

• Communicate well with children.

• Be honest& Establish a trust relationship with children & their parents.

• Altruistic (acting of the good/concern for the welfare of others)

• Knowledgeable about diseases and medication (teacher).

• Familiar with community resources and organization (networker).

• Role model and child & family advocate.

• Recognize uniqueness.

• Develop partnership with parents (mutual respect and sharing the decision making role).

• Coordinating the health care team.

Assessment of health of children

Mortality rate.

Morbidity rate.

The assessment of the two variables can reflect:

1- Causes of deaths.

2- Age group at risk for hazards and diseases.

Statistics • Incidence: ex. Number of new cases of DM over specific period

of time.

• Prevalence: ex. Number of old & new cases of DM over specific

period of time.

• Mortality: Number of individuals who died over specific period

of time per 1000 . ( death certificates)

• Infant mortality rate (IMR): Number of deaths per 1000 live

births during first year of life in specific year. Two type of IMR:

Neonatal mortality: From birth to 28 days of life.

Postnatal mortality: From one month to 12 months of life.

Decrease IMR due to

Good infection control.

Improve nutritional conditions.

New generation of antibiotic and

antimicrobial.

Advanced care of preconception

care, prenatal, natal and

postnatal care.

Improve the health and education

of the population as general.

Respiratory diseases.

Infections.

Low birth weight.

Congenital

malformations.

Sudden infant death.

Main leading causes of deaths in Palestine

Causes of Deaths

1. Infants: 0-12 months:

A. Prenatal condition:

Aspiration pneumonia.

Cord compression.

Difficult labor.

B. Congenital malformations: Teteralogy of fallout.

Tracheoesophageal fistula.

Others.

C. Infection & sepsis.

Neonate baby

Low immunity

2. Childhood:

Accidents.

Cancers.

Congenital anomalies (Cystic fibrosis, Thalassemia, etc…).

Infections. (Respiratory tract & Gastrointestinal)

Malnutrition.

3. Adolescents:

o Accidents and suicide.

Morbidity in Children

• Morbidity in children definition:

Any illness sever enough to limit activity or require

medical attention. Classified as:

Acute (respiratory. infections, injuries).

Chronic or disability.

Social illnesses (behavioral, psychosocial &

educational problems( poverty, violence and

school failure)

Chapter 3

Respiratory System Disorders

01/02/1441

DR. Areefa Albahri

• Functions of Respiratory System:

• Taking in O2 and breathing out the CO2

• Maintenance of acid-base balance

• Regulation of H2O and heat balance

• Production of speech

• Facilitate the sense of smell

DR. Areefa Albahri

Respiratory system during childhood years: Infants are obligatory nasal breathers and they use their abdominal muscle for breathing Use more accessory muscles for breathing At 2yrs the Rt bronchus become shorter, wider & more vertical Walls of the airways are small in size and have less cartilage thus lung collapse after expiration is easier.

Assessment of Respiratory Function:

• Inspection breathing:

– Rate

– Regularity

– Symmetry of movement

– Depth

– Effort and the use of

accessory muscles (during

sleep/quietly awake)

• Auscultation: airway patency &

adventitious sounds

• Palpation & percussion: painful

area and tissue density

• Normal breath sounds:

• Bronchial sounds

• Over trachea, bronchi

• Loud, harsh and high

pitched

• Longer in expiration

• Vesicular sounds: • Air moving in and out

alveoli

• Quiet and low pitched

• Longer in inspiration

• Bronchovesicular sounds:

• Near main stem bronchi

• Moderately pitched

• Equal in inspiration and expiration phases

Adventitious sounds:

– Crackles (Rales)

– Result of air passing through fluid in

small airways

– Simulated by rubbing a few strands of

hair between fingers next to the ear

– Most common during inspiratory phase

Associated with COPD and pulmonary

edema

– Wheezes:

• Air passing through narrowed

small airways

• In expiration

• Associated with asthma

– Pleural friction rub:

• Pleural surfaces rubbing across

each other

• Grating sound

• Associated with inflammation of the pleura

• Noisy Breathing:

• Snoring: Indicates partial

obstruction of the upper

airway that causes vibration

of the air as it passes the

nasopharynx and

oropharynx. May cause

sleep apnea.

• Stridor: A harsh, continuous

crowing sound. Mostly

occurs during inspiration.

Croup (Laryngotracheobronchitis).

• Wheezing: Whistling/musical sound.

Indicates a narrowing of the

airways. Mainly heard during

expiration. Accompanied by

tightness of the chest and

labored breathing. Mostly

caused by asthma.

Respiratory Distress: • Clinical Manifestations:

– Increased RR > 60 breath/min; Restlessness & apprehension

– Retractions:

• Chest sinking with each breath

• Mostly observed anteriorly at lower costal margins (Subcostal retraction)

– Cyanosis around the mouth, fingernail

– Grunting during expiration

– Nasal flaring indicates labored breathing

– Wheezing & Cough

– Clubbing caused by chronic hypoxia

– Use of accessory muscles of respiration

– A child with RD should be kept NPO to Decrease the work of breathing and

prevent aspiration

• Neonatal Respiratory Distress Syndrome (RDS)

• RDS is caused by immature lungs anatomy & physiology or genetic problem

with lungs

• Mainly due to Insufficient surfactant (production of surfactant starts around 26

weeks of gestation)

• Deficient surface area for gas exchange

• Pulmonary capillary bed is deficient

– Neonatal RDS mostly seen in neonates born before 28 wks of gestation

– Sometimes called Hyaline membrane disease

Clinical manifestations:

• Tachypnea > 60

breaths/minute

• Grunting on expiration

• Retractions of the chest wall

• Auscultation reveals

decreased breath sounds

and audible rales (crackles)

• Pallor/cyanosis at room air

• Increase in respiratory effort

(flaring and dyspnea)

• Flaccidity

• Apnea

• Hypotension

• Prolonged capillary refill

– Pulmonary function tests (forced vital capacity, tidal volume, functional residual volume

– Tracheal aspiration

– Bronchoscopy

– Blood gas (pulse oximetry, ABG)

– Sputum Studies

– Lung biopsy

Diagnostic Procedures

Therapeutic Techniques:

• Chest tubes

• Tracheostomy

• O2 Therapy

• Nebulizer

• Postural drainage

• Chest Physiotherapy (CPT)

• Suctioning of Airways

• Thoracentesis

• Mechanical Ventilation

• O2 Therapy:

• Delivered by mask, nasal

cannula, tent, hood, face tent or

ventilator. Should be ordered by

physician

• Should be humidified before

administered to the patient

• Tracheostomy: – It is a surgical opening in the

trachea (2-4 tracheal rings)

– Temporary tracheostomy

(epiglottitis, croup, foreign

body aspiration)

– Long term tracheostomy for

ventilation Care after the

surgery

• Hemorrhage,

edema, aspiration

– Regular care:

• Airway patency

(remove secretions)

• Humidification

• Cleansing

Pharmacology: Respiratory System Disorders:

Bronchodilators:

Reverse bronchoconstriction

thus opening the air

passages in the lungs by

acting:

Stimulating beta-adrenergic

sympathetic nervous system

receptors

Short acting beta agonists

(ventolin); A quick relief of

acute exacerbations and for

the prevention of exercise-

induced airway constriction

Long acting (Foradil) directly

relaxing bronchial smooth

muscles ( Aminophylline,

Theophylline)

Major Side Effect:

Dizziness

(decrease in

BP), CNS

stimulation,

Palpitation, GI

irritation

Mucolytic Agents and Expectorants:

To liquefy secretions in the respiratory tract, thus

promoting a productive cough

Mucolytic directly breaking up mucous plugs (inhalation)

Expectorants increase respiratory tract secretions (oral)

Major side effect

GI irritation

Skin rash

Oropharyngeal irritation

Corticosteroids:

Anti-inflammatory effect & decrease edema

Control Asthma and improve pulmonary function

Inhaled preparation (Fluticasone) oral (Prednisone), IV

(hydrocortisone)

Major side effect

Inhaled: oropharyngeal candiadiasis, growth retardation and

osteoporosis

Oral: with long-term use immunosuppression, increase Wt,

osteoporosis, gastric ulcer,

– Antitussives:

– To suppress the cough reflex

– Preparation with Narcotic (Codeine) without Narcotic

(Tessalon)

– Major side effect

– Drowsiness

– Nausea

– Dry mouth (anticholingeric effect of antihistamine in

combination products)

– Antihistamines:

– To relieve symptoms of common cold and allergies

– Act by blocking the action of histamine at receptor sites,

Major side effect

– Drowsiness, Dizziness

– GI irritation

– Dry mouth (anticholingeric effect of antihistamine in

combination products)

Upper respiratory tract infections ( Resp. system) emergency

• Otitis media

• Croup (laryngotracheobronchitis)

• Epiglottitis

• Respiratory Dysfunction:

• 1. Otitis Media (OM)

– Acute infection of the middle ear

– Prevalent between 6 months to 2 years

– Acute OM rapid short onset

of signs and symptoms

lasting for 3 weeks

– OM with effusion middle ear

inflammation with fluid

present

– Chronic OM may last more

than 3 months

– Prolonged OM may have

consequences:

– Functional: hearing loss

which may affect speech,

language and cognition

development

– Structural: tympanic

membrane retraction which

leads to impaired sound

transmission, perforation ,

infection

Caused by

Streptococcus pneumonia or

Haemophilus influenza Noninfectious caused by blocked

Eustachian tube secondary to

URI, allergy

There is a relationship between

formula-fed infants and OM

Clinical Manifestations:

– Acute OM: – Pain: infant rubs ear or rolls

head from side to side – Temp. 40, Vomiting &

diarrhea – Loss of appetite – Tympanic membrane is bright

red and bulging, no light reflex

– Discharge from the external auditory canal

OM with effusion

No Pain or fever , but fullness in

the ear

Tympanic membrane gray and

bulging

Possible loss of hearing

Treatment:

Antibiotic, antipyretic, analgesic

drugs

Chronic OM: surgery ; insertion

of tympanotomy tube

Nursing care:

Assess pain, S&S of infection, Provide comfort,

prevent complications

Educate family about treatment (ear drop) and possible

complications

2. Croup (Laryngo-tracheobronchitis

(LTB)

• Laryngotracheobronchitis (croup), refers

to the inflammation or irritation of the larynx and trachea and bronchial passageways

• Mostly affect children under 5 years with peak between 6 months to 3 years

• Most common causative agents; – Parainfluenza virus – Respiratory syncytial virus (RSV) – nfluenza A &B

Croup: Signs/Symptoms

Cold” progressing to hoarseness, cough Low grade fever Night-time increase in edema with:

Stridor “Seal bark” cough Respiratory distress Cyanosis Hypoxia Resp. acidosis

Treatment:

Mild Croup

Reassurance, Moist, cool air, Maintain patent airway, Ibuprofen

Severe Croup

Humidified high concentration oxygen

NPO if there is respiratory distress and start IV fluid

For sever cases Nebulized epinephrine or dexamethasone

Possible intubation

– Observation and assessment of respiratory status

– Prepare for possible intubation if the patient develops signs of airway obstructions including:

– Increased HR, RR,

– Retractions,

– Flaring nares

– Increased restlessness

– Provide comfort, avoid eliciting gag reflex (Laryngo-spasm)

• Acute obstructive inflammatory process of

epiglottis

• Mostly occurs in children 2-6 years

• Most often the causative agent is

Hemophilus influenza B

• Abrupt symptoms

• CM:

– Sore throat and pain on

swallowing

– Fever,

– Muffled voice & stridor,

– Tripod sit

– Drooling saliva,

– Irritable and restless & possible

retraction

– Epiglottis red, inflamed, large,

cherry red and edematous

– Airway obstruction leads to

hypoxia and acidosis

Nursing Care Respiratory Dysfunction: 3.

Epiglottitis:

Respiratory Dysfunction: 3. Epiglottitis:

• Acute obstructive inflammatory process of epiglottis • Mostly occurs in children 2-6 years • Most often the causative agent is Hemophilus influenza B • Abrupt symptoms • CM:

– Sore throat and pain on swallowing – Fever, – Muffled voice & stridor, – Tripod sit – Drooling saliva, – Irritable and restless & possible retraction – Epiglottis red, inflamed, large, cherry red and edematous – Airway obstruction leads to hypoxia and acidosis

Treatment:

IV fluid until the patient

can swallow

Antibiotic

Corticosteroids to reduce

the edema

Tracheal intubation in

severe cases

Nursing Care

Reduce the anxiety

Comfortable position

Avoid using tongue

depressor to inspect

epiglottius

Monitor respiratory

status.

•Respiratory distress+ Sore throat + Drooling = Epiglottitis

Complete Airway Obstruction

• Lower respiratory tract infections

• Acute bronchitis • Bronchiolitis/respirat

ory syncytial virus (RSV)

• Pneumonia

Acute bronchitis

Inflammation of trachea , bronchi & bronchioles

Common in children older than 6 yrs

Acute bronchitis usually occurs in association with viral respiratory tract

infection.

Causative agent of acute bronchitis is Mycoplasma pneumonia. Other

causes include chemical agent

CM:

Productive cough

Sometimes retrosternal pain during deep breathing or coughing

It is a self-limited disease (5-10 days)

• Treatment:

• Rest, use of antipyretics, adequate hydration

• Symptomatic treatment

Bronchiolitis/respiratory syncytial virus (RSV)

Infection of the lower respiratory tract

Rarely occurs in children over 2 years old (peak 2-5

months)

Primarily occurs in winter & spring

50% of cases caused by RSV, bacteria also cause

bronchiolitis

The bronchi and bronchioles are inflamed that leads to

obstruction of the airway

Narrowing of the airways during expiration causes

overinflation (emphyasema)

Starts with URT infection then spreads to lower tract

CM: • Earlier S&S; poor feeding and irritability

• Initial S &S; Rhinorrhea, low-grade fever, pharyngitis and

possible OM, conjunctivitis, cough

• Progressive sign increased cough, air hunger, tachypnea,

retractions & cyanosis

• Severe S&S, RR >70, listless, apneic spells

• Symptomatically treatment

• Antiviral medication may be used

• Humidity

• O2

• Fluid & rest

• If the patient is tachypnea NPO

Pneumonia

• Is an inflammation of pulmonary parenchyma

• Types of pneumonia based on the way the child gets the infection or the germ;

– Aspiration pneumonia; occurs when food or drink accidently gets into lungs

– Community Acquired pneumonia

• Most common type

• Caused by viruses, bacteria or chemical irritants

• Mostly occurs in winter and spring

• Fast breathing is a sign of pneumonia;

• 1 wk-2months 60 B/M or more

• 2mon- 12 mon 50 B/M or more

• 1 2months – 5 yrs 40 B/M or more

Severe pneumonia: Wheezes, Stridor, Retractions ,Cyanosis , Nasal flaring Poor feeding , Convulsion

Viral Pneumonia:

• mostly caused by RSV

in children under 5 yrs

– Gradual onset

– Viral infection make the

pt susceptible to bacterial

pneumonia

– Treatment is

symptomatically

• O2

• Comfort

• Fluid

• CPT

• Postural drainage

Bacterial Pneumonia:

• Streptococcus pneumonia is

the most causative bacterium – Others causative agent; group B

streptococcus, hemophilus

influenza type b, group A

streptococcus

– CM:

Productive cough, tachypnea,

fever, ronchi or fine crackles,

chest pain, retraction, nasal

flaring, cyanosis, lethargy

Chest X-ray shows patchy

infiltration

• Irritable , Anorexia, vomiting,

diarrhea and abdominal pain

Treatment:

– Penicillin G ( for allergic pt erythromycin, chloramphenical,

cephalosporin)

– Antipyretic, antitussive (cough)

– Rest

– Increase fluid intake

– O2 may be required for RD children

• Nursing diagnosis: Ineffective breathing pattern R/T inflammatory effects

of pneumonia

• Nursing Care:

• Thorough respiration assessment (signs of RD)

• Provide comfort and O2, Cool humidification

• Encourage cough and deep breathing

• Increase fluid intake & Monitor I &O

• Provide rest & Maintain semi-fowler’s position

• Standard precautions & use of air-borne and droplet precautions

Long-term respiratory dysfunction Asthma Cystic fibrosis

• Asthma: • Chronic inflammatory

disorder of the airway

• Asthma causes recurrent

episodes of wheezing,

breathlessness, chest

tightness & cough

particularly at night or in

the early morning

• Associated with reversible

airflow limitation or

obstruction

• Asthma causes bronchial

hyper-responsiveness to

stimuli

Bronchospasm

Bronchial Edema

Increased Mucus Production

Asthma

Factors aggravate asthmatic exacerbation:

– Allergens (air pollution, dust), Irritants (odor spray, smoking)

– Changes in weather temperature, Cold air

– Environmental changes ( new home)

– Infections

– Animals

– Strong emotions

– Food additives, nuts, dairy product

– Other factors ( menses, pregnancy).

The mechanisms responsible for the obstructive symptoms in

asthma include:

Inflammation and edema of the mucus membrane. Accumulation of tenacious secretions from mucus glands. Spasm of the smooth muscle of the bronchi and bronchioles, which decreases the diameter of bronchioles

CM:

– Asthmatic episode begins with: irritability, restlessness, headache, feeling tired

– Dyspnea

– Cough: hacking (harsh), irritative and nonproductive then cough becomes rattle & productive

– Prolonged expiratory phase with wheezing

– Flaring nares , distended neck veins

– Silent chest (severe obstruction in status asthmaticus)

ALL THAT WHEEZES IS NOT ASTHMA

Management:

Eliminate or avoiding irritant/ offending factors long-term control medication anti-inflammatory (Corticosteroids, Cromolyn sodium), bronchodilators (albuterol) Most long term or quick relief medications administered by inhaler CPT: relaxation and strengthen respiratory muscles

• Status Asthmaticus:

• A severe prolonged asthma exacerbation that has not been broken

with repeated doses of bronchodilators

• It is an emergency and may cause respiratory failure

• Symptoms

• Extreme difficulty in breathing, restlessness and anxiety

• Little or no breath sounds, inability to speak, cyanosis and heavy

sweating

• Management by large doses of corticosteriod and bronchodilators, &

O2

• Hint •

•Asthma attack unresponsive to -2 adrenergic agents

Cystic Fibrosis (CF)

Sweat gland: high Na & Cl (3-5 times higher than normal) sweat chloride test above 60 mEq/L are diagnostic

• Pancreas: Becomes fibrotic, decrease production of pancreatic

enzymes

• Decrease in Lipase cause steatorrhea (fatty, foul, bulky stool)

• Decrease in Trypsin increase nitrogen in stool

• Decrease Amylase cause inability to break down

polysacharides

• DM is late complication

• Rectal prolapse:

• Liver; possible cirrhosis from biliary obstruction

• Early manifestations:

– Meconium ileus in newborn infants

– Baby tastes salty

– Failure to regain normal 10% weight loss at birth

– Presence of cough or wheezing during first 6 months of age

– Sweat chloride test > 60mEq/L

– Stool fat

• Chest X-ray shows patchy atelactesis

• Clinical Manifestations:

• Respiratory tract:

– Increased viscosity of bronchial mucus with incomplete

expectoration causes obstruction and serves as a media for

bacterial growth

– Dry nonproductive cough, Wheezing

– Hypoxia, hypercapnia and acidosis, clubbing of finger

– Barrel-shaped chest and distended neck veins

– Obstruction interfering with expiration (emphysema)

• Scattered atelectasis and emphysema

• GI tract:

– High thick secretion blocked the ducts in pancreas leading

fibrosis, Marked impairment of pancreatic enzymes which

affects digestion of fats and protein thus affecting normal growth

• Increased bulk of feces ( undigested and unabsorbed fat and

protein), Wt loss & FTT

– High incidence of DM in children with CF

– Cardiovascular; Cardiac enlargement

– Reproductive:

– Delayed puberty in females, mostly males are sterile

– Integumentary: salty taste, risk of hypochloremic and hyponatremic

• Cystic Fibrosis (CF): Treatment

• Pulmonary problems:

– Chest physiotherapy

– Bronchodilators

– Antibiotic therapy as indicators

– Gastrointestinal problems:

– Pancreatic enzyme supplements

– Balanced nutritional intake

• Cystic Fibrosis (CF): Nursing Diagnosis

• Ineffective airway clearance R/T secretion of thick tenacious mucus

• Ineffective breathing pattern R/T mechanical tracheobronchial obstruction

• Altered family process R/T situational crisis

• Altered nutrition R/T inability to digest nutrients

• Respiratory Dysfunction

• Physical defects of respiratory tract

Esophageal atresia

Tracheoesophageal fistula

Diaphragmatic hernia

Esophageal Atresia &

Tracheoesophageal Fistula

Most common type proximal

esophagus ends in a blind pouch and

distal esophagus is connected to the

trachea

Usually occurs in low birth weight

May be associated with other

abnormalities

Occurrence is 1 in 800 to 5000 births

Indications:

Excessive salivation (drooling)

in newborn

chocking, coughing, cyanosis (3 Cs) sneezing &

when newborn fed the fluid

returns through the nose and

the mouth

Complication: aspiration and RD

Treatment: surgical repair

Congenital diaphragmatic hernia (CHD)

Absent of the diaphragm on

one side or both

Mostly accompanied with other fetus malformations

CM: difficulty in breathing, tachycardia, cyanosis, concave abd. Abnormal chest development

Tx: Surgical repair

Chapter 8 Gastrointestinal Tract

Disorders

01/02/1441

DR. Areefa Albahri

– Main function GI is turning food into nutrients which can be absorbed by the human body to provide energy needed for survival

– GI Accessory organs that assist the GI tract by secreting enzymes to help break down food into its component nutrients (salivary glands, liver, pancreas and gall bladder)

Gastrointestinal Tract: Assessment

Health history: o Gestational age and birth

weight o Nutritional history (length of

breast feeding, introduction of solid food).

o Neonatal & infancy GI problems

o Family factors (life style, hereditary problems)

o Present changes in child’s life (start schooling, new sibling or death in the family)

o Assessment of the digestive function in a 24hr (food intake and elimination)

Gastrointestinal Tract:

Physical Exam

– Physical parameter (Wt and Ht)

– Skin Color – Inspection of oral cavity – S & S of dehydration – Abdominal and rectal

assessment • Peristalsis • Abdominal Tenderness • Distended abdomen

– Displaced heart (diaphragmatic hernia)

– Hair (loss of pigment or brittle)

• Cleft Lip & Cleft Palate • incomplete fusing of soft and hard

tissues of lip and palate (top of mouth)

respectively. May occur together or

separately, one side or both.

• Among the most common facial

anomalies

• Genetic basis is present (family history

for presence of the defect in other

siblings)

• Incidence rate of cleft lip is 1:7800

• Incidence rate of cleft palate alone in

1:2000

• May result in communication between

the nasal and oral cavities

• Cleft lip may be unilateral or bilateral

• Cleft lip may be accompanied with

cleft palate

• Cleft palate may be present without

cleft lip (non-visible): early sign is

dripping milk from nose .

• Assessment should be focused on;

– In newborn: compromised sucking ability

– Respiratory status

– Family reaction

Complication

Feeding problem

Hearing problem

Speech difficulties

Social

• Management:

– Surgical repair for cleft lip during the first few weeks of life

– Initial repair for cleft palate during 4-6 months of age and the surgical correction between 6-18 months

Nursing care

Provide adequate nutrition and

prevent aspiration and infection

(otitis media)During feeding

Upright position

Feed slowly

Burp frequently

• After the surgical operation:

– Restrains may be necessary to prevent disturbance of the surgical site

– No tooth brushing

– Prevent infection

– Follow-up assessment of

• Growth

• Speech

• Teeth development.

• Nursing diagnosis:

• Altered nutrition R/T physical

defect / difficulty eating

• Risk for aspiration

• Risk for infection

• Risk for impaired verbal

communication

• Altered family process

Hypertrophic Pyloric Stenosis

An overgrowth of the circular muscle of

the pylorus, results in obstruction/

partially / narrowing of the pyloric

sphincter

Cause is unknown, however there is a

hereditary component

The stomach contractions increase in

frequency and force to empty the

stomach content.

Usually develops in the first few weeks

of life

• CM:

• Regurgitation small amounts of milk immediately after

feeding

• Projectile vomiting

• Vomiting may occur during feeding or shortly after feeding

• Vomitus contain NO bile

• Gastritis due to prolonged stay of stomach content

• Wt loss and dehydration

• Metabolic alkalosis( blood pH above 7.45, levels of other

blood components, including salts like potassium, sodium,

and chloride, fall below normal ranges. The level of

bicarbonate in the blood will be high, usually greater than

29 mEq/L. Urine pH may rise to about 7.0 in metabolic

alkalosis.

• Failure to thrive

• Dehydration

• Assessment:

• Olive-like mass at right epigastrium under the

edge of the liver

• Peristaltic waves can be noted after feeding

moving from left to right

• Ultrasoundgraphy

• Treatment is by surgery (Pylorotomy): to allow

better passage of milk

• Nursing Care:

• Monitor intake and output

• Assess vomitus

• Prevent dehydration

• Monitor Wt and Ht

•

Intussusception

• Is an invagination of part of the intestine into an adjacent distal

portion of the intestine.

• Occurs in healthy infants around 6 months of age and rarely occur

before 3 months or after 3-years of age

• More common in male infants

• The cause is unknown.

• The most common type is near the ileocecal valve pushing into the

cecum and onto the colon.

• The involved intestine become inflamed and edematous with

bleeding from the mucosa

• Untreated intussusception can lead to intestinal gangrene, peritonitis and death

• Diagnosis by barium enema ( if there is intraperitoneal air from a

bowel perforation thus enema is contraindicated)

• Assessment is focused on:

– Stool inspection (currant-jelly stools)

– CM such as Pain

– Abdominal palpation

• Early symptoms:

• Crampy abdominal pain and a drawing up of the knees to the chest

• Periods of apathy

• Poor feeding and vomiting

• Late symptoms:

• Worsening vomiting, becoming bilious

• Abdominal distension/ Palpable abd. Mass (sausage-shaped)

• Heme positive stools

• Followed by “currant jelly” stools: Jelly stools due to leaking of blood

and mucus into the intestinal lumen as a result of venous

engorgement

• Dehydration

• If untreated, necrosis and perforation are possible

• Treatment: • Supportive therapy

(Fluid; Antibiotics) • Hydrostatic barium • Operation • Manual • Resection and

reanastamosis

Hirschsprung’s Disease (aganglionic megacolon)

A congenital anomaly resulting from

an absence of ganglion cells in the

colon (lack of nerve ending in the

sigmoid colon)

Autosomal dominant genetic

mutations

More common in male & children

with down syndrome

peristalsis cannot occur

• CM:

– Newborn:

• Failure to pass

meconium after birth

(during the firs t 24

hr)

• Poor feeding and

spitting up

• Visible bowel loops

• Bile-stained vomitus

• Abdominal distention

– Infancy:

• Failure to thrive

• Constipation &

Abdominal distention

• Diarrhea & vomiting/

Explosive watery

stool, Fever

– Childhood (more chronic):

– Constipation

– Ribbon-like & foul smelling

stools

– Abdominal distention

– Palpable fecal masses

– Poorly nourished

– Lethargy, nausea and anorexia

– Treatment by surgery (removal

of non-motile part)

– Colostomy/ileostomy care after

surgery

– After surgery high fiber diet is

established

– Prevent enterocolitis

Celiac Disease • A disease of malabsorption &

abnormal immune reaction to

gluten

• Celiac disease is a

hereditary intolerance of

gluten (protein found in

wheat, oats, rye)

• Gluten protein (gliadin)

causes inflammation and

damages villi in the small

bowel

– Enzyme insufficiency

(peptidase) causes

accumulation of toxic

gluten peptide

– Gluten toxicity results

from alteration in

immunologic response. It

is the second cause of

mal absorption after CF.

• CM: (related to malabsorption and

malnutrition)

– Problem starts after the introduction

of solid food

– Diarrhea; Steatorrhea (stool is bulky,

fatty foul smelling)

– Wt loss

– Weakness

– Abdominal pain & distention

– Bone & joint pain

– Anemia (malabsorption of iron)

– Vit. Deficiency

– Failure to thrive ( without S&S of GI

problems)

– Behavioral changes: irritability,

apathy and uncooperative

Dietary management

a gluten-free diet

In acute phase;

Corticosteroid

Fluid replacement

N/G to decrease the

distention

Anti-tissue transglutaminase antibodies (tTGA) or anti-

endomysium , antibodies, CBC, Serum protein

– Complications: (if not treated) • Iron deficiency anemia

• Osteoporosis • infertility or recurrent miscarriage

• depression or anxiety

• tingling numbness in the hands and feet

• seizures

Inflammatory bowel diseases:

Ulcerative Colitis & Crohn’s Disease

• Inflammatory bowel disease (IBD) refers to chronic conditions that cause

inflammation in some part of the intestines.

• The intestinal walls become swollen, inflamed, and develop ulcers

• IBD can cause discomfort and serious digestive problems

• Symptoms depend on which part of the digestive tract is involved

• Causes of the inflammation in IBD involves a complex interaction of

several factors:The genes the patient has inherited, • The environment and the immune system. antigens in the environment may

cause of the inflammation or they may stimulate the body's defenses to

produce inflammation

Symptoms of IBD:

The symptoms of ulcerative colitis and Crohn's disease are similar:

Abdominal pain or cramping

Diarrhea multiple times per day, Bloody stools

Weight loss, Mouth sores and skin problems, Arthritis

Eye problems that affect vision

• Inflammatory bowel

diseases:

Crohn’s disease

• Crohn's Disease is

characterized by a chronic

inflammatory process that may

affect any segment of the

gastrointestinal tract, from

mouth to anus.

• The inflammatory process

usually extends through all

layers of the intestinal wall

• Skip lesions

• Treated by medication to

decrease inflammation and

usually control the symptoms

but does not provide a cure

• Inflammatory bowel

diseases:

Ulcerative Colitis

• characterized by continuous

inflammation confined to the

large intestine. Inflammation is

limited primarily to the mucosa

and does not extend through

all layers.

• Treated by:

– The primary treatment

options are medications

that decrease the abnormal

inflammation in the colon

lining and control the

symptoms..

– Ulcerative colitis is

potentially curable if the

colon is removed.

• Inborn errors of metabolism

• Galactosemia • Phenylketonuria • Congenital hypothyroidism •

Galactosemia:

Lack of galactose-1-phosphate

uridyl-transferase enzyme.

Inherited as autosomal

recessive.

Failure of conversion of galactose

to glucose, accumulation of

galactose leads to damage of the

liver & brain.

Should be suspected in any infant

who vomits, refuses feeds, fails

to thrive & become jaundiced in

the first week

Long term effect: ovaries damage,

speech delay, learning difficulties

Treatment may include stopping

BF & replacement by a special

low-lactose milk.

Phenylketonuria:

• Autosomal recessive genetic disorder causing mutation of the

gene that producing phenylalanine hydroxylase enzyme that is

necessary to metabolize the amino acid phenylalanine to tyrosine.

• Phenylalanine is converted into phenylketone which is detected in

the urine

• The disease may present clinically with

– Seizures

– Excessively fair hair and skin

– "Musty Odor" to the baby's sweat and urine

– Early cases of PKU were treated with a low-phenylalanine diet.

– Children with PKU must adhere to a special diet low in Phe for

optimal brain development.

– The diet requires severely restricting or eliminating foods high

in Phe, such as meat, chicken, fish, eggs, nuts, cheese, milk &

dairy products. If PKU is left untreated, it can cause problems

with brain development, including mental retardation, brain

damage and seizures.

he disease may present clinically with seizures, hypopigmentation (excessively fair hair and skin), and a "musty odor" to the baby's sweat and urine (due to phenylacetate, a carboxylic acid produced by the oxidation of phenylketone). In most cases, a repeat test should be done at approximately two weeks of age to verify the initial test and uncover any phenylketonuria that was initially missed.

Appendicitis:

• Inflammation of the appendix Caused by an obstruction.

• Fecalith (hard feces)

• Lymphoid obstruction

• Infection

• Swelling of the appendix reduces blood flow causing

• Ischemia

• necrosis

• Perforation (rupture) may occur which may cause peritonitis, sepsis or abscess

• CM: • PAIN (pain is the first sign)

• Starts around the belly and moves to the lower right quadrant of the abdomen

• Right lower quadrant localized tenderness

• Right lower quadrant rebound tenderness

• Fever, nausea, vomiting & diarrhea

• WBC over 15,000

• Elevated C-reactive protein

– CM In neonate (non specific)

• Irritability/ lethargy

• Abdominal distention & abdominal mass

• Vomiting

– In infants and older children • Vomiting & diarrhea; Pain & fever

• S & S of appendix perforation: – Fever

– Sudden relief from pain

– Subsequent increase in pain and rigidity guarding of the abdomen

– Progressive abdominal distention

– Tachycardia, rapid-shallow breathing

– Pallor , chills and irritability

Chapter 4 Cardiovascular disorder

DR. Areefa Albahri

• Cardiovascular disease is a significant cause of chronic

illness and death in children.

• Typically cardiovascular disorders in children are

divided into two major categories:

• Congenital heart disease is defined as structural

anomalies that are present at birth. CHD accounts for

the largest percentage of birth defects

• Acquired heart disease includes disorders that occur

after birth. These disorders develop from a wide range of

causes, or they can occur as a complication of CHD.

Fetal Circulation

– Foramen Ovale:

– Is anatomical opening between the right atrium and

left atrium which closes shortly after birth.

• Higher pressure in the left atrium due to increased

pulmonary blood flow cause the foraman ovale to

close.

– Ductus arteriosus:

A vessel that connects the main pulmonary artery to

the aorta.

The ductus arteriosus should functionally close within

15 hours and structurally within a few weeks (in mature

infants)

Assessment of Cardiac Function:

• History: parents usually

report – Poor weight gain, fatigue during

feeding, sweating with feeding.

– Frequent respiratory infections

– Cyanosis

– Evidence of exercise

intolerance

– A previous cardiac defects in a

sibling

– Maternal rubella infection during

pregnancy,

– Children with chromosomal

abnormalities . A history of viral

infection or toxic exposure

(myocardities)

– A history of streptococcal

infection (Rheumatic fever)

Physical Examination

Vital signs Tachycardia or bradycardia may indicate cardiac disease. Tachypnea may indicate congestive heart failure Hypertension; Differences in BP between the upper and lower extremities may indicate coarctation of the aorta Physical Examination Inspection:

Skin color ( cyanosis, ruddy complexion) Position of comfort Presence of clubbing Lethargy and overall

– Physical Exam; Palpation

– The point of maximum intensity

and the apical impulse

– The presence of a thrill/murmur

should be noted.

– The quality and symmetry of all

pulses

– Warmth of extremities, capillary

refill

– Locating the hepatic and splenic

– Heart sounds, heart rate and

rhythm.

– The presence of additional heart

sounds, such as a murmur, is

noted.

– Lung sounds

Classification of Congenital Heart Disease

A cyanotic

a. Increased pulmonary blood flow

1. Atrial septal defect

2. Ventricular septal defect

3. Patent ductus arteriosus

b. Obstruction to blood flow from

ventricles

1. Coarctation of aorta

2. Aortic stenosis

3. Pulmonic stenosis

Cyanotic

a. Decreased pulmonary blood flow

1. Tetralogy of Fallot

2. Tricuspid atresia

b. Mixed blood flow

1. Transposition of great arteries

2. Truncus arteriosus

3. Hypoplastic left heart syndrome

Types of Defects:-

• 1. Acyanotic Heart Defects: there is no mixing of

unoxygenated blood with systemic circulation (Oxygenated blood), its take one of the following forms:

• a. Dextracardia ―as a part of situs inversus ―

• b. Obstructive lesion as: aortic stenosis, pulmonary stenosis and coarctation of the aorta.

• c. Left to Right shunts as: patent ductus arteriosus, atrial septal defect and ventricular septal defect.

Acyanotic a. Increased Pulmonary Blood Flow 1. Atrial Septal Defect (ASD)

Atrial Septal Defect

• Is an abnormal opening in the septum

between two atrium.

Altered Physiology:

• The pressure in the left atrium is

greater than that in the right one,

which promotes the flow of

oxygenated blood from the left to the

right atrium and thus increasing the

total blood flow through the lung.

• If the pulmonary resistance is great

reversal the shunt with

unoxygenated blood flowing from

the right to left atrium and cyanosis

occur.

CM: – Many children have no

symptoms and seem healthy

– If the opening is large S& S of CHF may develop and increased the risk of

endocarditis Harsh systolic Murmur Atrial dysrhythmias Pulmonary vascular

obstructive diseases and emboli formation later in life from chronic condition

Enlarged right side and increased pulmonary circulation

Treatment of ASD:

ASD may close

spontaneously as the child grows.

Some children may need medication, such as digoxin and diuretics.

Control infection. Catheterization

may be needed to close the septum.

Ventricular Septal Defect (VSD)

– An opening between left ventricle and right ventricle

– Most common congenital heart defect

– Can be classified as: Membranous VSD (located in the upper section; 80% of all VSD cases

• Muscular VSD (located in the lower section; 20% of all cases)

• Altered Physiology: The pressure in the left ventricle is greater than that of the right one promotes the flow of oxygenated blood from the left to the right ventricle increasing the total blood flow through the lungs and thus increased right ventricular and pulmonary arterial pressure.

CM

Fatigue, Sweating, Rapid, heavy, congested breathing,

Disinterest in feeding

Poor weight gain, Murmurs, May lead to hypertrophy and

enlargement of the right side

CHF is common

Treatment: Surgical repair

Patent Ductus Arteriosus (PDA)

• Before a baby is born, the fetus's blood does not need to go to the

lungs to get oxygenated. The ductus arteriosus is a hole that allows

the blood to skip the circulation to the lungs. However, when the

baby is born, the blood must receive oxygen in the lungs and this

hole is supposed to close. If the ductus arteriosus is still open (or

patent) the blood may skip this necessary step of circulation.

CM: Asymptomatic or signs of CHF

Murmurs

A widened pulse pressure & bounding

pulses

At risk for endocarditis & pulmonary

vascular obstructive disease

Treatment: Prostaglandin inhibitor, (eg, non steroid antiinflammatory drugs [NSAIDs])

Surgical repair (ligation)

• Coarctation of the Aorta (COA) – Narrowing anywhere in the aorta – Increased pressure proximal to the defect – Restricts the amount of oxygenated

blood to lower part – LT ventricle has to work harder – Risk for endocarditis – Coronary arteries may narrow due to

high pressure

Altered Physiology: the narrowing of the aorta

obstructs the blood flow through the constricted

segment of the aorta, that increasing the left

ventricular pressure.

Acyanotic Obstruction to Blood Flow from Ventricular

– CM: – High BP and bounding pulse in arms – Low BP & Absent femoral pulse and cool extremities – Headaches, dizziness , fainting & epistaxis – Cramps in the legs – Pt at high risk for hypertension, ruptured aorta and

stroke – Kidneys’ function may be altered ( decrease urine ) – Treatment: – Enlargement of constricted section (surgical or

nonsurgical (Balloon angioplasty)

Balloon angiplasty

II. Aortic Stenosis

• Aortic stenosis (AS) is a narrowing or a stricture of the aortic valve that results in:

• Increase left ventricular pressure to overcome the obstructed valve

• Left ventricular hypertrophy, myocardial ischemia and decreased cardiac output.

• Imbalance between the increased O2 requirements and the amount of O2 supplied.

Clinical Manifestations

• Rarely symptomatic during infancy, in severe cases infant may demonstrate evidence of decreased cardiac output such as faint peripheral pulses or exercise intolerance.

• Older children may experience chest pain, dyspnea and fatigue with exertion.

• Narrow pulse pressure and weak peripheral pulses.

• X-ray may show normal heart to varying degrees of left ventricular hypertrophy.

• A harsh ejection systolic murmur is best heard in the aortic area.

• E.C.G.: left ventricular hypertrophy {T. wave inversion}.

• Diagnosis: Cardiac Catheterization.

• Complications: CHF, MI, bacterial endocarditis and death.

Treatment: Balloon dilatation (via cardiac catheterization)

Valvotomy involves a surgical removal of adhesion that preventing valve

leaflets from opening

Aortic valve replacement

III. Pulmonary Stenosis • Refers to narrowing of the

opening to the pulmonic

valve, that cause obstruction

to blood flow, so the right

ventricle has an additional

work-load, causing the

muscle to thicken, resulting in

right ventricular hypertrophy

and decreased pulmonary

blood flow.

CM: Mild cases are asymptomatic Cyanosis in severe cases Murmurs Cardiomegaly (chest X-ray) Treatment: Depends on the degree of the stenosis may wait for a few years Relieve stenosis by balloon angioplasty or surgical valvotomy

• Cyanotic Congenital Heart Disease

• I. Teratology of Fallot (TOF)

• The most common type of cyanotic heart

disease, its involve four anatomical

abnormalities:

• 1- Pulmonary stenosis.

• 2- Ventricular septal defect {VSD}.

• 3- Overriding of the aorta (enlargement

of the aortic valve)

• 4- Right ventricular hypertrophy.

• Altered Physiology:

• Pulmonary stenosis: unoxygenated

blood is shunted from the right ventricle

(from the VSD) directly into the aorta.

• The right ventricle is hypertrophied

because of high right ventricular

pressure.

• Clinical Manifestation:

• Cyanosis: not cyanotic at birth

{left to right shunt}, may starts

later, may be at 1-2 years, first

observed with exertion or crying,

then cyanotic even at rest.

• Clubbing of fingers.

• Squatting posture is assumed to

relief stressed heart by trapping

blood in lower extremities.

• Slow weight gain.

• Hypoxia spells (Tet spells

characterized by sudden cyanosis

and syncope)

• Complication: CHF, endocarditis, CVA and

iron deficiency anemia. • Treatment: • Improve oxygenation • Emergency for Tet spells:

beta-blockers as propranolol, but acute episodes may require rapid intervention with morphine are required.

• Surgical • Complete intracardiac repair of VSD and PA

stenosis. • Repair the VSD with a patch. • removing the excessive muscle tissue will help to

function correctly. • Pacemaker wires are placed temporarily because

of the potential for postoperative ventricular arrhythmias.

• Individual chamber pressures are then measured before the chest is closed. The pressure readings help to determine how effective the surgery was.

• II. Transposition of the Great

Arteries (TGA)

• TGA occurs when the aorta arises

from the right ventricle and the

pulmonary artery from the left

ventricle.

• Incompatible with life unless the

infant has an associated defect

which allows the blood to

communicate between the

pulmonary and systemic

circulation.

• Clinical Manifestation:

• Marked cyanosis since birth.

• Failure to thrive, Fatigability and

dyspnea with subcostal retractions

at rest

• Cardiomegaly, early clubbing of

fingers and C.H.F.

Prognosis: without surgical treatment, 85 % die in the first 6 months of age.

• Treatment:

• Management of C.H.F.

• Palliative procedures: creation of A.S.D. with a balloon catheter during catheterization or surgical creation of A.S.D.

• Complete correction: by cardiopulmonary bypass.

• III. Tricuspid Atresia

• Is a condition in which there is a complete absence of

the tricuspid valve. Therefore, there is an hypoplastic of

right ventricle. Its associated with:

• Atrial septal defect.

• There is usually a V.S.D. allowing some blood to enter

the underdeveloped right ventricle.

• Pathophysiology:

• Blood from the systemic circulation is shunted from the right atrium

through an interatrial communication to the left atrium, to the left

ventricle.

• Pulmonary blood flow is established either through PDA or VSD.

• Clinical Manifestation:

• Cyanosis {marked since birth}, dyspnea on feeding and may hypoxia

spells.

• Early clubbing of fingers and failure to thrive.

• Right heart failure may occur.

• Treatment: • Palliative procedures: to increase the pulmonary blood flow (Anastomosis

between the ascending aorta and right pulmonary artery).

• Fontan Procedure: is a palliative surgical procedure used in children with complex congenital heart defects. It involves diverting the venous blood from the right atrium to the pulmonary arteries without passing through the morphologic pulmonary ventricle

• Diagnostic Evaluation for Congenital Heart Defects.

• 1. Chest x- ray shows enlargement of the affected chambers of the

heart.

• 2. Cardiac catheterization: visually demonstrate the defect,

demonstrate blood oxygenation in heart chambers.

• 3. E.C.G.

• Assessment:

• Take careful health history including evidence of: poor weight gain,

unusual posturing or poor feeding

• Exercise intolerance – frequent respiratory tract infections

• Perform physical assessment with special emphases on: color,

pulse (apical and peripheral), respiration, blood pressure,

examination and auscultation of chest and manifestations of CHD.

Hypoplastic Left Heart Syndrome (HLHS)

– Is a combination of abnormalities of the heart and the great vessels

– In HLHS most structures on the left side of the heart are small & underdeveloped

– Mitral valve, left ventricle, aortic valve, & aorta are involved.

– The newborn develops symptoms shortly after delivery

CM Cyanosis, Sweating, cool skin Increased RR & HR, heavy breathing

• Treatment is by series of surgical repair

Acquired Cardiovascular Disorders

• 1. Rheumatic Heart Disease (RHD).

• 2. Congestive Heart Failure (CHF)

Rheumatic Heart Disease (RHD).

• Permanent damage to the heart valves is caused by RF (rheumatic

fever)

• It develops after an infection of the upper RT with group A β-hemolytic streptococci

• The antigens of group A Streptococci bind to receptors in the heart, muscle, brain & synovial joints, causing an autoimmune response

• The antigens produced by Streptococci are similar to the body’s own antigens thus antibodies may attack healthy body cells

• RF forms Aschoff bodies (inflammatory lesions) that causes swelling and alterations in the connective tissue

• RF major criteria: • Rheumatic Carditis • Polyarthritis • Erythema marginatum • Subcutaneous nodules • Chorea

Rheumatic Carditis.

Involves endocardium, pericardium &myocardium

CM: Valvulitis , Myocardities,

Pericarditis: chest pain, pericardial friction rub, Murmur, Tachycardia especially during sleep

– Polyarthritis: • Joints such as knees, elbows, hips, shoulders & wrists • Joints are swollen, hot, red & painful

– Erythema marginatum: • Erythematous macule mostly found on the trunk & extremities

– Subcutaneous nodules: • Nontender swellings mostly found on the bony prominences areas

such as feet, hands vertebrae

– Chorea:

• Sudden & aimless irregular movements of the extremities

• Involuntary facial, grimaces

• Speech disturbances

• Emotional liability

• Muscle weakness

– Diagnostic test:

• Throat culture-group

a beta hemolytic

streptococcal

• Increased ESR

• Increased in WBC

• Increased in C-

reactive protein

Treatment:

Bed rest until ESR decreases

Antibiotics (penicillin,

erythromycin) x 10 days

Reduce inflammation

(Salicylates: aspirin)

Corticosteroids (if not

responding to aspirin alone)

Phenobarbital for chorea

Treatment of heart failure

Prophylactic antibiotics (benzathine

penicillin G) for 5 years or until 18 to

prevent recurrence

Prevention of RHD by Treating

streptococcal throat infections

with a full course of antibiotic Complications of RHD:

Mitral valve damage Congestive heart failure

Chapter 5 Hematologic disorder

DR. Areefa Albahri

Blood Components

1. Plasma:

– The liquid part of blood. All the blood cells are suspended in this liquid.

– Contains dissolved salts (electrolytes) and

– proteins (Albumin, fibrinogen &globulin)

– helps keeps blood vessels from leaking and carries hormones and drugs to different parts of the body.

• Antibodies (immunoglobulin's)

• Prevents blood vessels from collapsing and clotting by keeping them filled and circulating

– Plays a role in warming and cooling the body

– Plasma contain all blood component except RBC, WBC, Platlet)

2. Red Blood Cells – Erythrocytes: Make up 40% of

the blood’s volume

– Produced in the bone marrow

– Contain hemoglobin, a protein that gives blood its red color and enables it to carry oxygen.

3. White Blood Cells – Leukocytes: Fewer in number

than RBCs (1:660)

– Primary responsibility: Defend the body against infection

4. Platelets – Thrombocytes: cell-like particles

smaller than RBCs and WBCs.

– Help with clotting process by gathering at bleeding site and clumping together to form a plug that helps seal the blood vessel.

Problems of Erythrocyte Production

• Anemia :

• reduction of RBC volume or Hgb concentration below normal

Classifications:

1. etiology/Pathophysiology

causes of RBC/Hgb depletion

2. Morphology – changes in RBC size, shape, and color

• Causes of Anemia

• Nutritional deficiency – iron, folate, B12

• Increased destruction of RBCs – sickle

cell anemia

• Impaired or decreased rate of

production – aplastic anemia

• Excessive blood loss - hemophilia

Anemia

Classified by Etiology or patho-physiology

Nutritional Deficiency

Iron Deficiency

Anemia

Increased Destruction of

RBCs

Sickle Cell Anemia Thalassemia

α- Thalassemia

β- Thalassemia

Impaired or Decreased rate of Production

Aplastic Anemia

Excessive Blood Loss

• Iron Deficiency Anemia: (IDA)

• IDA is caused by an inadequate

supply of dietary iron

• People at risk of IDA:

– Poor children between 6-24

months of age

– Adolescents (rapid growth and

poor eating)

– Preterm infants (reduced fetal

iron supply and storage)

• Normal values of iron:

– Newborn 17-44 mol/L ( 100-

250 mcg/dl)

– Infant 7- 18 mol/L ( 40-100

mcg/dl)

– Child 9-21 mol/L ( 50-120

mcg/dl)

CM: Underweight (some are chubby-

milk baby)

Palor, poor muscle development

Prone to infection

Due to leakage of plasma

proteins infants with IDA may

have edema & growth retardation

Serum-iron concentration (SIC)

Total Iron Binding Capacity

(TIBC)

Hct & MCV (70 for infant & 75

for older children)

Normal RBC, & normal to slightly

reduced Retic. count

• Management:

• Screening at 6 months, 12, 18, 24 then during adolescence period

• Nutritional counseling

• Iron-rich food

• Decrease milk intake

• Iron supplement

• Starts at 4 months (preterm infants at 2 months)

• Give with citrus juice

• Use a straw to avoid staining the teeth

• Use Z-track method for IM

• Educate the mother (appearance of black stool, possible

constipation..)

• Sickle Cell Disease:

Causes: genetic transmission.

• Hgb A is partly or completely

replaced by Hgb S

• With dehydration,acidosis,

hypoxia, and temp elevations,

Hgb S “sickles”

• Autosomal recessive involved

deforming the shape of the RBCs

(sickling)

– Sickle cell trait (carrier) has

30-40 of abnormal Hgb (HbS)

always asymptomatic

– Sickle cell anemia

(homozygosity (affected)of

sickle hemoglobin/HbSS) has

80-100 HbSS

Pathophysiology:

- vaso-occlusion from sickled

RBCs

- increased RBC destruction

- splenic congestion and

enlargement

- hepatomegaly, liver failure

- renal ischemia, hematuria

- osteoporosis, lordosis,

kyphosis

- cardiomegaly, heart failure,

stroke

Sickled cells are More rigid

Fragile & rapidly

destroyed (life span 10-

20 days)

1/5 as soluble as the

normal RBC

Has less O2 concentration

Loss the ability to flow

easily in the tiny

capillary

Factors increase the sickling

Low O2, Acidosis, Infection

Dehydration, Cold, Fever

Vigorous exercise

Emotional and physical stress

High altitude

When sickled RBCs increased they will

aggregate causing obstruction and

cutting down the blood flow to the

affected tissue leading to various

complications.

• CM:

– Pallor, weakness, easy fatigable, Jaundice

– Growth abnormalities and delayed puberty

– Tachycardia and later cardiomegaly

• Diagnostic

Assessment:

– Prenatal diagnosis at 9-11 wk of gestation

– Hgb electrophoresis

Medical management Supportive/symptomatic rx. of crises - bed rest - hydration - electrolyte replacement - analgesics for pain - blood replacement - antibiotics - oxygen therapy

Sickle Cell Crisis:

• Vaso-occlusive crisis

(pain crisis)

• Splenic Squestration

crisis

• Aplastic crisis

• Vaso-occlusive crisis

(VOC) (thrombotic)

• stasis of blood in small

capillary causes distal

ischemia and infarction

Signs:

Low grade-fever Pain Tissue engorgement Hand-foot-syndrome (dactylitis) Mostly occurs in 6 months to 2 years children. Caused by infarction of short tubular bones Painful, swelling of soft tissue over hand and feet, Resolves spontaneously within days or weeks Splenic Squestration crisis

Sudden pooling and trapping of blood

within the spleen and impaired blood

flow to the liver causes enlargement

of the spleen, liver failure and

circulatory collapse. The acute form

occurs most commonly in children

between 2 months and 5 years of age

– CM

• Pain in the left side of the

abdomen

• Big spleen

• Irritability

• Unusual sleepiness

• Pale & weakness

• HR

• Death can occur in less than thirty

minutes

• Treatments

• Intravenous fluids to maintain

vascular volume

• Cautious blood transfusion for

treating anemia.

• Splenoectomy if indicated

Aplastic crisis

Diminished RBCs production

usually triggered by a viral or

other infection may lead to bone

marrow failure & life threatening

anemia. Packed RBC transfusion

is indicated

Complications of Sickle Cell

Disease:

Acute chest syndrome Similar to pneumonia Seizure Weakness in arms and legs Speech problems Loss of consciousness Infection Major cause of death in children Sepsis, meningitis and If Temperature > 38.5 Co it is an

emergency and if Temp is over 40 should start antibiotics

Palliative care

of sickle anemia

– Analgesics

– Fluid

– Rest

– Avoid stress

– Maintain Hgb around 12 by administering packed RBCs

– Vaccination (pneumococcus, Hep B, H. influenzae B)

– Prophylactic antibiotics (penicillin)

– Help pt to copy with the disease (normalcy of life and support group)

– Family education

Thalassemia (Cooley’s Anemia)

• Hereditary disease; structural abnormalities of hemoglobin production.

– Reduced synthesis either of α or β –globin chains of hemoglobin

– RBCs are small, pale, fragile and prone to early destruction

• β - gene is present but with different level of β globin protein production

• Severity of the disease depends on the type of inherited genes

– Thalassemia minor/trait (one abnormal gene) • Has one normal β gene

• RBCs are small / mild anemia

• Affected person has no symptoms

• Mistakenly diagnosed as iron-deficiency anemia.

• Thalassemia intermediat (two abnormal genes)

• Varying level of anemia but no need for blood transfusion (starts

from below 7 to 10gm/dl)

• CM: small stature, poor Wt gain, susceptibility to infection, bone

deformities (face), splenomegaly, weak bone

• Treatment decision depends on the severity of clinical

manifestations

• Splenectomy could be done to slow down RBCs destruction and

slow bone marrow production of RBCs and avoid possible bone

deformities

• Thalassemia major (two abnormal genes)

• Severe anemia

• Eventually require blood transfusion

• Blood transfusion lead to iron overload and chelation therapy is needed (Desferal) to prevent organ injury.

Diagnostic assessment

Hgb electrophoresis RBCs = (Hgb low, MCV low, MCH low, MCHC

normal/low, Serum iron High.

Prenatal diagnosis

Thalassemia stimulate

erythropoiesis

Hyperplastic bone marrow

Skeletal changes

Defective RBCs

Hemolysis

Extramedullary hemopoiesis

Splenomegaly

lymphadenopahty

• Treatment

– Depends on the severity of the disease

– For moderate forms may need blood transfusion occasionally (

during infection, stress)

– Thalassemia major

• Blood transfusion (often 2-4 weeks)

• Chelation therapy (Deferoxamine)

– 40 mg/kg/day over 10-12 hr (5 days/wk)

– Should not take Vit and iron

– Adverse effects such as loss of night/color vision, loss of

high frequencies hearing and rickets

• Bone marrow transplantation

• Surgery ( splenomegaly)

• Annual flu shot and vaccination

• Folic acid

• Gene therapy

• Enhance the production of fetal Hgb after birth reduce the severity

Aplastic Anemia • Normochromic, normocytic • is an unusual blood disorder in which the BM fails to produce blood

cells (Pancytopenia). • The failure could be acquired or congenital (Fanconi anemia) • Incidence rate 1 per 100,000 • Poor prognosis, mortality over 70% and 50% die within 6-months to

a year.

Possible causes Drugs, Radiation/chemotherapy

Severe infection (hepatitis/sepsis)

Leukemia /lymphoma, Idiopathic (unknown)

CM Low Hgb , few or no Retic, Neutropenia and thrombocytopenia

Acquired Aplastic Anemia

• Severe form – Neutropenia < 500, Platelets < 20,000, Retic < 1%

– Decrease cellularity in the Bone Marrow (BM).

– Treatment by bone marrow transplantation

– 20% of severe case live for one year

• Congenital Aplastic Anemia

• Autosomal recessive disorder

• Accompanied congenital abnormalities of the musculoskeletal and genitourinary systems, microcephaly

• Diagnostic test – Bone marrow : fatty substance replaced the precursors of mature

hematologic elements

• Bone Marrow transplant is the only cure for aplastic anemia – Steroid and androgens therapy

Hemophilia:

• Disease transmitted in an X-linked recessive manner • Deficiency of one of the clotting factor VIII (8) (hemophilia A most

common 80%) factor IX ( 9) (hemophilia B) • VIII and IX present but in a diminished capacity to perform its

function in the coagulation cascade; no fibrin clot can be formed • Both types are different but have same symptoms and treatment • Most cases diagnosed in toddlerhood

• Severity of Hemophilia depends on the amount of clotting factors in the blood

– Severe Hemophilia with the factor VIII or IX is less than 1% causing 2-3%

bleeding episodes/week

– Moderate Hemophilia factor VIII or IX deficiency is 1-5% causing 1-2

bleeding episodes/year

– Mild 5-50%

• Usually causes problems with bleeding only after serious injury, trauma,

or surgery.

• In many cases, mild hemophilia B is not discovered until there is

unusual bleeding after an injury, surgery or tooth extraction.

• It may be discovered in adulthood

• CM: – Bruises

– Easily bleeds (from

nose & mouth)

– Bleeding in the brain

• May lead to severe

complications

(blindness)

Most common cause of death in children with hemophilia

Blood in urine and stool

Hemo-arthrosis is oozing of blood into

the soft tissue most common affected

are knees

Causes pain, Swelling, Redness

Hemarthrosis may cause

destruction to the joints

• Diagnostics

– Prolonged activated partial thromboplastin time (PTT),

normal Prothrombin time (PT)

– Deficiency in clotting factors

– Genetic testing

– Amniocentesis

• Treatment is to prevent complications:

– Subcutaneous immunizations

– Avoid contact sports, but encourage activities such us

swimming are recommended

– Immobilization /surgery to the affected joins

– Rehabilitation of the affected joints

– Factor replacement before any dental surgeries & proper

dental hygiene

– Blood transfusion when necessary.

• Nursing diagnosis:

– Risk for injury

– Pain

– Impaired physical mobility

• Knowledge deficient

Nursing Care: Control localized bleeding Pain management No rectal temperature Avoid aspirin products Replacement of clotting factor If joint is involved

Elevate the affect joint Immobilize

Ice packs • Complications: – Intracranial hemorrhage – Osteoporosis – Intestinal obstruction – Paralysis – Airways obstruction – Hepatitis/AIDS

Chapter 6

Musculoskeletal (MS)

DR. Areefa Albahri

• Musculoskeletal (MS) • The main organs and tissues that

are part of the musculoskeletal system in humans are

• The cartilages • The bones • The muscles

• Main functions of MS are:

– To support & protect vital organs (the brain, heart and lungs)

– To keep structure and maintenance of the body spatial conformation.

– Allows the body to move (walking, standing, bending). Because soft tissues are resilient in children, dislocations and sprains are less common than in adults

– Nutrient storage (glycogen in muscles, calcium and phosphorus in bones).

Musculoskeletal: Physical Assessment

• Inspect child undressed • Observe child walking • Spinal alignment • Range of Movement (ROM) • Muscle strength • Reflexes

Fracture types

Fracture: Clinical Manifestations

– Swelling, pain or

tenderness

– Diminished functional

use of the affected

part; Bruising, severe

muscular rigidity

– Sometimes crepitus

– Less frequent

neurological and

vascular damage

(ischemia), which can

be assessed using 5

Ps

– Pain

– Pallor

– Pulselessness

– Parasthesia

– Paralysis

Fracture: Therapeutic Management

• Cast: fiberglass or plaster

application to immobilize

affected body part

• Tractions :Is the direct

application of force to produce

equilibrium at the fracture site

• Distraction: involves the use of

an external device to separate

opposing bones to encourage

regeneration of new bone &

used to immobilize fractures or

correct defects when the bone is

rotated or angled

• Fracture: Cast

• Risk for altered tissue

perfusion R/F pressure from

cast

– Keep extremity elevated to

decrease edema.

– assess circulation Q 15

minutes after applying the

cast then hourly

– assess skin warmth and 5 Ps

– Risk for impaired skin

integrity R/F pressure from

cast

– Cast edges must

smoothed/covered

– Cast remains in place for 4-8

weeks

– Discourage itching under the

cast

• Possible concerns

– Unusual odor under the cast

– Drainage from cast

– Tingling, numbness and swelling

– Loose or cracked cast

– Unexplained fever

– Unusual irritability and pain

– Discoloration of finger or toes

• Fracture: Traction

• traction refers to the practice of exerting a slow, gentle pull on a fractured or

dislocated body part. The purpose is to guide the part back into place and to

produce equilibrium at the fracture site

• . Traction may also be used to stretch the neck and prevent painful muscle

spasms.

• Manual traction: applied by hand is used during cast

– Skin traction: pull applied directly to the skin surface and indirectly to the

skeletal structure

– Skeletal traction: pull applied directly to the skeletal structure by a pin,

wire, or tongs

• Purposes of Traction: – To realign bone fragments & treat dislocation – To provide rest for an extremity & help prevent or improve

contracture deformity. – To allow preoperative or postoperative positioning and

alignment. – To provide immobilization of affected body part – To reduce muscle spasm so that bones can be realigned

• Nursing Care of traction: – Regular assessment of 5 Ps – Skeletal traction is never released by the nurse (do not move

the weights) – Assess blood pressure – Skin care for the child’s back, elbows and heels

– Fracture Complications:

• Circulatory impairment:

– Careful assessment of the pulses, skin color, and temperature is crucial

– Nerve compression syndromes (e.g., carpal tunnel syndrome, tarsal tunnel syndrome)

– Sensory testing with touch and pinprick

– Evaluating motor strength by asking the child to move the unaffected joint distal to the injury

• Compartment syndromes

• Is a tissue ischemia due to a compression of nerves, blood vessels, and muscle inside a closed space (compartment) within the body due to increased pressure in that part

• The most frequent causes are:

• Tight dressings or casts, hemorrhage, trauma, burns, and surgery

• Signs & symptoms include:

• Motor weakness and pain that does not decrease with medication

• The muscle may feel tight or full

• Burning sensation

• Epiphyseal damage: leads to unequal growth

• Infection: osteomyelitis (potential problem in open fractures, from pressure ulcers, or when bone surgery)

• Pulmonary emboli: blood, air, or fat emboli may produce a life-threatening vascular obstruction and ischemia.

– Primary symptom is shortness of breath and chest pain.

– Interventions should include:

• Place patient in high fowlers

• Administer O2 and check chest X-ray

Sprained ankle • A soft tissue injury • Management ( in the first 6 to 12 hours) • Controlling the swelling and reducing muscle damage by

“RICE” Rest, Ice, Compression, Elevation

Is an abnormally increased

convex angulation in the

curvature of the thoracic

spine

It can result from

degenerative diseases as

tuberculosis, chronic

arthritis, or developmental

problems

Treatment

Postural exercises

Bracing (Milwaukee)

for more marked

deformity

Surgery

Physical therapy

Kyphosis

Is an accentuation of the lumbar

curvature beyond physiologic limits

It may be a complication of a disease

process, the result of trauma or

idiopathic

Lordosis is a normal observation in

toddlers

In older children is often seen in

association with flexion contractures of

the hip, obesity, congenital dislocated

hip and slipped femoral capital

epiphysis.

Treatment: The Roman chair is a piece of

exercise equipment.

Lordosis

Scoliosis

Is a spinal deformity which may involve

lateral curvature, spinal rotation

causing rib asymmetry, and thoracic

hypokyphosis.

It is the most common spinal deformity.

It can be congenital, or it can develop

during infancy or childhood, but it is

most common during adolescence

(peaks between 8-15 years)

It may be genetic and transmitted as an

autosomal dominant trait

It may be multifactorial

Scoliosis: Types

Non-surgical management aimed to :

Promoting self-esteem and positive body image

Maintain spinal stability

Prevent further progression of deformity until bone growth is

complete and surgical repair can be performed

Mild cases (less than 20%), observation and exercises- swimming is

advised & Long-term monitoring.

Moderate (20-40%), exercises, traction, bracing.

Bracing (Milwaukee brace) is successful in halting or slowing the

progression of curvatures

Severe (more than 40%), bracing until the skeletal system mature

and then surgical intervention

Surgery includes realignment and straightening of the spine with internal

fixation

Developmental/congenital hip dysplasia/dislocation

(DDH/CHD)

• Improper formation and function of the hip socket.

• Cause of DDH is unknown, but there are predisposing factors such as:

– Genetic factors & birth order

– Physiologic factors: maternal hormone

– Mechanical factors: intrauterine position (breech), oligohydraminos, twining and fetus size, delivery type, postnatal positioning, DDH occurs more commonly in females.

Acetabular dysplasia (or

preluxation)

Subluxation_Accounts for the

largest percentage of DDH. It

implies incomplete dislocation. The

femoral head remains in contact

with the acetabulum, but a

stretched capsule and the head of

the femur to be partially displaced.

Dislocation

Femoral head loses contact with the

acetabulum and is displaced posteriorly

and superiorly over the fibro-cartilaginous

rim

DDH/CHD: Degree:

DDH/CHD: diagnosis

Ortolani test

Abduction of the thighs with external rotation.

If the femoral head can be felt to slip forward into the

acetabulum on pressure from behind, it is dislocated (positive

Ortolani sign)

Sometimes an audible “clunk” can be heard.

Barlow test

Pressure from the front

If the femoral head is felt to slip out over the posterior lip of the

acetabulum and immediately slips back in place when pressure

is released, there is dislocation or “unstable” (positive Barlow

sign)

Other signs of DDH are:

Shortening of the limb on the affected side (limping and toe walking

Positive Trendelenburg sign or gait) Asymmetric thigh and gluteal folds Broadening of the perineum(in bilateral dislocation)

Ultrasounds

DDH/CHD: Management • Newborn to six months

– Pavlik harness • The harness is used to maintain

the infant’s hips in flexion and abduction and external rotation

• Pavlik harness device is to be worn continuously. Except bathing

• The child in a Pavlik harness needs special attention to skin care because the infant’s skin is sensitive and the harness may cause irritation.

six months to 8 months

– Gradual reduction by traction is used for approximately 3 weeks.

– If the hip is not reducible, an open operative reduction is performed. Following reduction the child is placed in a hip spica cast for 2-4 months

• Older child:

– Operative reduction

– After cast removal and before

weight bearing is permitted,

range-of-motion exercises &

rehabilitative measures

• The former practice of double-or

triple-diaper for DDH is not

recommended because it

promotes hip extension, thus

worsening proper hip

development

Congenital clubfoot (Talipes)

• Deformity of the ankle and foot

• Categories of Talipes

– Positional clubfoot (transitional, mild or postural),

• may occur from intrauterine crowding

• responds to simple stretching and casting.

– Syndromic (tetralogic ) clubfoot • associated with other congenital

abnormalities such as myelomeningocele,

• more severe form of clubfoot that is often resistant to treatment.

Congenital clubfoot (idiopathic )

• has a wide range of rigidity and prognosis

• Congenital clubfoot: Management

• Goal of management: is Correction of the deformity & Maintenance of the correction until normal muscle is gained

• Management:

– Casts: • Begin immediately or shortly after birth and

continue until marked overcorrection is reached.

• Weekly manipulation and cast changes proceed for the first 6 to 12 weeks of life.

– Surgery: • If casting and manipulation are not successful

• Followed by brace and cast

• Nursing Care: – Observation of skin and circulation (particularly important in

young infants because of their normally rapid growth rate): swelling in the toes, foot temperature

– Parents need to understand the diagnosis, the overall treatment program, the importance of regular cast changes

Juvenile Rheumatoid Arthritis (JRA)

• Is an inflammatory disease of the body joints and sometimes affects blood vessels and connective tissue

• Unknown cause but a slight tendency to occur in families • Peak ages: 2 – 5 years and between 9 - 12 years of age • JRA is similar to the adult disease with some distinguishing features

– Onset before puberty – A negative rheumatoid factor (RF).

Pauciarticular

Polyarticular

Systemic

Juvenile Rheumatoid Arthritis (JRA): Courses

• Juvenile Rheumatoid Arthritis (JRA): Pauciarticular

• 4 or fewer joints are affected

• The most common form of JRA; about half of all children with JRA have this type

• Affects large joints, such as the knees, ankle,wrist

• Girls under age 8 are most likely to develop this type of JRA.

• Some children have special kinds of antibodies in the blood.

– Antinuclear antibody (ANA)

– Rheumatoid factor (RF)

– Eye disease affects about 20 to 30% of children with pauciarticular JRA

– Up to 80% of those with eye disease also test positive for ANA – The disease tends to develop at a particularly early age in these

children – Regular examinations by an ophthalmologist are necessary to

prevent serious eye problems such as iritis or uveitis

• Juvenile Rheumatoid Arthritis (JRA): Polyarticular • 30% of all children with JRA have polyarticular disease • 5 or more joints are affected. The small joints (hands and feet) are most

commonly involved, though large joints may be affected • Symmetrical; that is, it affects the same joint on both sides of the body • Some children have an antibody in their blood called IgM rheumatoid

factor (RF) • These children often have a more severe form of the disease

• Juvenile Rheumatoid Arthritis (JRA): Systemic (Still’s disease) • Joint swelling, & fever and a light skin rash • May also affect internal organs such as the heart, liver, spleen, and

lymph nodes • Almost all children with this type of JRA test negative for both RF

and ANA • Affects 20% of all children with JRA. A small percentage of these

children develop arthritis in many joints and can have severe arthritis that continues into adulthood.

• Juvenile Rheumatoid Arthritis (JRA): Clinical Manifestations

• Joint swelling

• Stiffness is worse in the morning or after a nap

• Pain may limit/loss movement of the affected joint

• Commonly affects the knees and joints in the hands and feet

• One of the earliest signs of JRA may be limping in the morning because of an affected knee.

• Besides joint symptoms, children with systemic JRA have

– A high fever and a light skin rash. The rash and fever may appear and disappear very quickly.

– Swelling in the lymph nodes located in the neck and other parts of the body

– In some cases (< 50%), internal organs (heart and, very rarely, the lungs) may be involved.

• Juvenile Rheumatoid Arthritis (JRA): Diagnosis • Diagnosis of JRA is based on:

– onset – Laboratory tests-- Blood may be taken to test for RF , and

Sedimentation Rate (ESR).

• Positive RF is detected in just 10% of the cases. The RF test helps the doctor tell the difference among the three types of JRA.

• ANA is found in the blood more often than RF, and both (ANA & RF) are found in only a small portion of JRA patients.

• ESR indicates inflammation in the body. Not all children with active joint inflammation have an elevated ESR.

• Lab. tests may include elevated WBCs

Juvenile Rheumatoid Arthritis (JRA): Therapeutic Management

• Preserve joint function,

• Prevent physical deformities, and

• Relieve symptoms

Goals

• Nonsteroidal anti-inflammatory drugs

• Disease-modifying anti-rheumatic drugs

• Corticosteroids

• Biologic agent

• Physical therapy

Treatment

• Reye Syndrome

Free of treatment related harm

• Disease-modifying anti-rheumatic drugs (DMARDs) such as (Cytoxan, Methotrexate…): most given in combination with NSAIDs to slow the progression of JRA.

• Corticosteroids: to control severe symptoms; can interfere with a child's normal growth, a round face, weakened bones, and increased susceptibility to infections.

• Biologic agents: Etanercept (Enbrel) blocks the actions of tumor necrosis factor, a naturally occurring protein in the body that helps cause inflammation.

• Physical therapy: Exercise to maintain muscle tone and preserve and recover the range of motion of the joints; rest of affect body part and heat application.

• Bleeding. • Stomach upset. • Liver problems

• Reye’s Syndrome:

• Is sudden (acute) brain damage (encephalopathy) & liver function problems.

• Abnormal accumulations of fat begin to develop in the liver and other organs of the body, along with a severe increase of pressure in the brain.

• Without proper treatment, death is common within a few days.

• During painful episodes of the disease:

– Proper positioning is important to support and protect affected joints. Isometric exercises and passive range-of-motion exercises will prevent contractures and deformities. Swimming in warm water provides strengthening and range-of-motion exercises while protecting the joints.

• Cerebral palsy (CP)

• It is a group of non-progressive disorders (meaning the brain damage does not

worsen, but secondary orthopedic difficulties are common) of motor neuron

impairment that result in

– Motor dysfunction

– May be accompanied by perceptual problems, language deficits, and

intellectual involvement.

– The disabilities usually result from injury to the cerebellum, basal ganglia,

or motor cortex.

• The exact cause is unknown; it may results from injury to the brain before,

during, or shortly after birth

• Risk factors include:

– Prematurity

– LBW

– Asphyxia

– Infections (meningitis, encephalitis)

– Head injuries

– Metabolic problems such as hyperbilirubinemia and hypoglycemia

– Sever dehydration

• Types of Cerebral Palsy:

• Spastic : (most common type)

– May involve one or both sides

– Hypertonicity with poor control of posture, balance,

– Impairment of fine and gross motor skills

• Dyskinetic/ athetoid:

– Abnormal involuntary movement

– Slow, wormlike, writhing (rolling & twisting) movements that

usually involve the extremities, trunk, neck, facial muscles, and

tongue.

– Involvement of the pharyngeal, laryngeal, and oral muscles

causes drooling and dysarthria (imperfect speech articulation)

– Involuntary irregular jerking movements

• Ataxic

– Rapid, repetitive movements performed poorly.

– Disintegration of movements of the upper extremities when the

child reaches for objects. Mixed type/dystonic:

– Combination of spasticity and athetosis.

• Cerebral Palsy Clinical Manifestations:

• Delayed gross motor development.

• Alteration in muscle tone: increased or decreased

resistance to passive movements.

• Abnormal posture :opisthotonic postures (exaggerated

arching of the back) and may feel stiff on handling or

dressing.

• Reflex abnormalities: persistence of primitive infantile

reflexes is one of the earliest clues to CP.

• Possible Associated disabilities and problems:

– Convulsion/seizure

– Visual and hearing impairments

– Communication and speech difficulties

– Some may have varying levels of mental retardation.

• Cerebral Palsy Treatments:

• Goals of Treatment:

– Establish locomotion, communication and self-help.

– Gain optimum appearance and integration of motor functions, Correct associated defects, Adaptation.

• Management:

– Provide safe environment to prevent injury.

– Prevent physical deformities by using braces and provide ROM exercises.

– Appropriate motor activities.

– Medications such as sedatives, muscle relaxants, anticonvulsants.

– Encourage ADLs.

– Occupational to improve small muscles development

– Speech therapy

Chapter 7 neurological disorders

DR. Areefa Albahri

Meningitis • is inflammation of protective membranes that covering brain and spinal cord

(meninges).

• Meningitis may extends to the ventricles and the exudation (fibrin) obstruct the

flow of CSF, Caused by: Virus, Bacteria, Other microorganism, Drugs

• Bacterial Meningitis:

• The most common are group B streptococci during the 1st 2 month of life

• H. influenzae (type B)

• Meningococcal:

– Occurs most often in the 1st year of life

– Tends to occur in epidemics among closed populations

• Streptococcus pneumoniae (pneumococci).

– The most common cause of meningitis in adults

– People at risk: chronic otitis, sinusitis, mastoiditis, CSF leaks

• Bacteria typically reach the meninges by: hematogenous spread from sites

of colonization in the nasopharynx or other site such as lungs

• Bacteria can also enter CSF by direct extension from nearby infections (e.g,

sinusitis, mastoiditis)

• Bacterial Meningitis S &S in Infant:

• Abrupt & nonspecific signs

• Extremely irritable

• Lethargic

• Difficult to comfort ; a high-pitched cry

• Jaundice (a yellowish tint to the skin)

• Stiffness of the body and neck(Nuchal rigidity)

• Fever or lower-than-normal temperature

• Poor feeding/ a weak suck/ vomiting, Bulging fontanelles / Seizures

Other Clinical Manifestations

• Pre-infection; respiratory illness/sore throat

• Fever, headache, stiff neck, vomiting

• Kernig’s & Brudzinski’s

• Seizures

• Cranial nerves abnormalities

Changes in consciousness, irritability

Purpura/petechia (meningococcal meningitis)

The primary diagnostic test for meningitis is

lumber puncture (LP)

Treatment

Antibiotics (Ampicillin/vancomycin)

corticosteroids

Supportive care

Isolation Pressure & color Glucose WBC protein

Normal 100-200mmH2O 50-100 mg/dL

0-5 20-45 mg/dL

BacterialMeningitis

>300Cloudy/milky

< 40 Elevated > 100

Viral/aseptic N or increased N N or mild elevated

N or < 100

• Bacterial Meningitis: Nursing Care

• Isolation (contact isolation )

• Monitor V/S and neuro assessment

• Provide quiet environment

• Control fever and pain • Prevent complications

of increased ICP and dehydration

• Viral Meningitis: • Common in children younger than 4

years

• Mostly caused by entero viruses

• Associated with mumps or herpes

• CM: • Gradual signs of headache, fever,

malaise, vomiting

• Meningeal irritation (signs) develop 1-2 days after the onset of illness

• Treatment:

• Symptomatic (rest, fluids, antipyretic, analgesics)

• Isolation is not necessary

• Signs and symptoms subside within 3-10 days with no residual effects (complications)

Encephalitis • Acute inflammatory disease of the

brain

• Usually viral; Herpes Simplex: most common sporadic type

• Acute febrile illness with symptoms of meningitis AND neurologic signs such as aphasia, seizures, cranial nerve involvement

• Patient may present with fever, facial paralysis, headache, seizures, nausea and vomiting

• CT scan usually initially normal; MRI more helpful

• Death occurs in 70-80% of patients if treatment not begun before patient becomes comatose

• Spina Bifida

• Vertebral column fails to close during intrauterine development with no definitive cause identified

• There are three forms:

– Spinal bifida occulta:

– Meningocele

• Myelomeningocele

Spinal bifida occulta:

Protrusion of the meninges. Meninges consist of: dura mater, arachnoid, and pia mater covered by thin membrane

– No paralysis because spinal cord is not involved.

• Failure of vertebral arch to close, a dimple occurs on the sacral area, may be covered by a tuft of hair.

Meningocele

• Protrusion of the meninges. Meninges consist of: dura mater, arachnoid, and pia mater covered by thin membrane

• No paralysis because spinal cord is not involved.

• Myelomeningocele – Protrusion of the meninges and spinal cord. Covered by

thin membrane – Extent of paralysis depends on the location of the defect. – Results in hydrocephalus.

• Spina Bifida: Prevention & Management

• Encourage folic acid 4mg Po with future pregnancies (conception-6 weeks)

• Primary intervention after birth of infant with meningocele & myelomeningocele is:

– To cover defect with a sterile, saline-soaked dressing to prevent cracking in the sac thus decrease infection

– Prone position keeps pressure off the exposed sac

– Head circumference measurement is essential because hydrocephalus can develop in these infants.

• Surgery: – Primary reason of surgical repair is to prevent infection – Correct defect – Minimize complications such as hydrocephalus

• Encourage parents to become involved with infant care ASAP • Teach parents the techniques of feeding, ROM exercises,

positioning, catheterization, skin care • Explain to the parents about possible complications

Hydrocephalus Impaired circulating and reabsorption of CSF

It can be congenital or acquired

It can be communicating or non-communicating

(obstructive hydro)

One of the most causes of hydr0 is ductal stenosis

Other causes include meningitis, tumors, lesions/

malformation

Hydrocephalus: Clinical Manifestation

Enlarged head (earliest sign)

& Bulging fontenells

Poor feeding & Vomiting

Irritability

Lethargy

Dilated & distended scalp

vein and setting sun eyes

Positive Babinski’s

reflex (fanning of

toes)

In older children

Headache

Changes in

personality

Cognitive

deterioration

Hydrocephalus: Treatment & complication

Treatment

V_P shunt placement

Diuresis

Complication

Infection

Visual problems

Memory problems and

reduced IQ

Seizure Disorders • Seizure is an abnormal unregulated excessive electrical discharge (firing) that

interrupts normal brain function

• This electrical firing may last from a few seconds to minutes

• 50% of seizures the causes are unknown

• Seizures before 2 yrs usually caused by developmental defects, birth injuries or metabolic disorders

• Seizure could partial (affect part of the brain) or generalized

• Seizure may be due to disorder such as epilepsy OR to reversible stressors such as:

– Hypoxia; Hypoglycemia; Fever in children, hypcalcemia, hyponatremia

• Seizure usually lasts 1-2 minutes

• Seizure usually causes

– Alteration in awareness, sensation & emotion

– Involuntary movements

– Convulsion

Types

– Generalized • Absence (petit mal)

• Tonic-clonic (grand mal)

• Atonic

• Myoclonic

• Infantile spasm

– Partial seizures • Simple partial seizures

• Complex partial seizure

• Secondarily generalized partial seizures

• Simple partial seizure – No complete loss of consciousness

– May affect the face or a hand first

– May developed to be generalized seizure

• Complex partial seizure – Starts with aura

– Purposeless movements & unintelligible sounds

– Consciousness is impaired

• Secondary generalized partial seizure – Either simple or complex

partial seizure may develop into a tonic-clonic seizure

Abnormal motor function & loss of consciousness

Types are;

Tonic-clonic (grand mal)

Tonic phase; cry, falling down and stiffness .

Followed by clonic ( jerk rapidly and

rhythmically, bending and relaxing at the

elbows, hips, and knee) contractions of

the muscles

Frothing at the mouth, urinary and fecal

incontinence may occur

• Absence (petit mal) • Mostly for children

between 6-12 years

• Lasts for a few seconds

• Abrupt & brief space of consciousness (staring into space or absence spells

• May occur many times a day

– Atonic seizure: • Complete loss of muscle

tone and consciousness • Risk for head injury

• Myoclonic (contraction and relaxation) – Jerky repetitive movement

of a limb/trunk – Consciousness is not lost – May followed by tonic-clonic

seizure – Infantile spasm – Sudden flexion of the arms

and trunk and extension of the legs

– Occurs during the first 5 yrs

• Status Epileptics

• Tonic-clonic Seizure that is lasting 5-10 minute.

• Epilepsy is 2 or more seizures episodes that are not related to reversible stressors.

• Longer epileptics seizures may cause permanent brain damage (more than 60 minutes.

• Lorazepam (Ativan) or diazepam (Valium) is given intravenously to control generalized tonic-clonic status epilepticus and may also be used for seizures lasting more than 5 minutes.

• Seizure Disorders: Epilepsy • Assessment:

– History: • Duration, frequency, sequential evolution • Longest and shortest interval between seizures • Aura, postictal state • Precipitating factors • Risk factors; CNS infection, drug use withdrawal, head trauma, neurologic disorders

– Physical exam: usually normal between the seizure – CBC, serum glucose, creatinine, electrolytes, CT and MRI, LP, video and EEG

monitoring

• Treatment by anticonvulsant drugs

• Well controlled seizures the drugs can eventually stop

and the child remain seizure free (60%)

• Drugs such as amphetamines can trigger seizures thus

should be avoided

• Avoid activity that the loss of consciousness can be life

threatening such as driving, swimming and climbing or

leaving a child in a bathtub

• Drugs to control seizures:

• Parents should be advised not to stop the anticonvulsant suddenly or without consulting the physician. Such action could result in seizure activity

• Parents should be advised about the side effect of

– Valporic acid (Depakene) such as Thrombocytopenia that causes bruising and bleeding

– Phenytoin (Dilantin); gingival hyperplasia. Good oral hygiene will minimize this adverse effect. Hepatic dysfunction thus serum therapeutic level of phenytoin should be carefully monitored

• Nursing care during a tonic-clonic seizure: – Patent airway: suction and O2 supply

– Prevent injury by removing sharp objects

– Do not put any objects in the child’s mouth

– Loosening clothing around the neck

– Roll the child to the side to prevent aspiration

– V/S (temperature) and neurologic

• Nursing care after tonic cloinc seizure:

– The child may be lethargic and confused

– If patient is febrile sponge bath, Check blood glucose level

• Seizure Disorders: febrile seizure:

• Occurs between 6 months and 6 years

• Is a convulsive event lasts 1-5 minutes due to rapid rise in body temperature

• Usually consists of jerking of extremities, eye rolling, unresponsiveness and sometimes cyanosis

• Sometimes it can be non-convulsive such as loss of tone and consciousness or stiffness of the body

• No brain damage and treatment is unnecessary

– Administer diazepam (may cause apnea)