Transcript الشريحة 1

KING
SAUD UNIVERSITY
NURSING COLLAGE
MASTER DEGREE
1431-1432H
Nursing management for acute disease
in:
Respiratory system
Endocrine system
Hematology system
Immunology system
Done by : maha alonazi
OBJECTIVE
At the end of the lecture the master student well
be able to :
 Know the most common of acute respiratory
disease .
 Identify nursing management for RD .
 Know the most common of acute endocrine
disease .
 Identify nursing management for ED .
 Know the most common of acute hematology &
immunology disease .
 Identify nursing management for HD & ID .

OUTLINE
RESPIRATORY DISEASE :
 Anatomy of respiratory system .
 Croup.
 Acute epiglottitis
 Broncholitis .
 Pneumonia.
 Aspiration pneumonia .
 ARDS
 Asthma.
 ENDOCRINE DISEASE :
 Anatomy of endocrine system .

Diabetes Mellitus
 Diabetic ketoacidosis .
 Diabetes Insipidus.
 SIADH .
 Hyperthyroidism.
 Hypothyroidism.
 Gigantism
 HEMATOLOGY & IMMUNOLOGY DISEASE :
 Anatomy of immune & hemato system .

Anemia
 Sickle cell disease
 DIC
 Hemophilia
 Leuckemia
 Thalasmia

ANATOMY OF RESPIRATORY SYSTEM
Upper airway .
 Lower airway .
 Thoracic cavity .

UPPER AIRWAY
Ideally above larynx is the upper airway
 Practically speaking Nose, Larynx (Glottic opening
and vocal
cords) and trachea (Up to Carina).

The upper airway is responsible for
warming, humidifying and filtering air before
it reaches the trachea.
 Nose
 Pharynx
 Larynx
LOWER AIRWAY (LUNG)

Trachea

Lung

Conducting Airways

Gas exchange units (Alveoli)
THORACIC CAVITY

Diaphragm

The chest wall
SPECTRUM OF RESP. DISEASES
Upper AW - Stridor, croup,
laryngomalacia
Lower AW
-
Asthma, bronchiolitis
Lung Parenchymal
edema
Pleural
-
- Pneumonia, Pulmonary
Empyema, Pneumothorax
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WHEN WILL YOU SUSPECT UAW DIS.?
Stridor
 Neck retraction
 Suprasternal retraction
 Sniffing position

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ACUTE LARYNGOTRACHEOBRONCHITIS
(CROUP)
Definition : inflammatory swelling of the
submucosa in the subglottic area & trachea
,bronchi ,bronchioles
 Commonest cause of acute stridor usually occure
for child from age 3 month to 5 yrs .
 causes : Viruses: Parainfluenza. RSV .
 Sing & symptoms:
 barking cough.
 Hoarseness.
 stridor.
 Low fever.

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(CROUP)
MANAGEMENT
Mild: Stridor at rest, cough
Minimal handling
 Moderate to severe: Stridor at rest.
Racemic epinephrine nebulization
Dexamethazone IV or IM, also oral or Nebulized
Budesonide
 Severe: Impending respiratory failure
Intubation
for 2-3 days. Improves in a week.
 Majority may not need intubation

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X-ray Neck
in croup
Sharpened
pencil
appearance
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NURSING MANAGEMENT
provide medication as order .
 Provide cool & humidified oxygen as needed
 Check vital sign .
 Monitor pulse oximetry.
 Minimal handling.
 Provide quit environment .
 Enteral feeding may be considered in pt. with
respiratory distress .

Acute epiglottitis
Definition :Is severe life threatening rapidly
progressive infection of the epiglottis &aryepiglottic
folds & surrounding tissue .
 Usually occurs in children aged 2-6 yrs but can
occurs any any age.

Caused: before the use of the HiB vaccine
 ( haemophilus influanzae type B) was the moust
commonly identified cause of acute epiglotittis ,the
usual cause in the vaccinated child is now
streptococcus pyogenes, S.pneumoniae ,or
staphylococcus aureus .

Sing & symptom :
 Sudden onset of high fever.
 dysphagia.
 drooling,.
 muffled voice.
 soft stridor.
 inspiratory retractions.

Normal Lateral
neck
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ACUTE EPIGLOTTITIS - MANAGEMENT
Intubation by a skilled person
 IV Ceftriaxone
 O2 and other supportive management
 Usually extubatable in 3-4 days
 Antibiotics for 10 days.

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NURSING MANAGEMENT
Close monitoring .
 Arm restraint to prevent self extubation .
 Decreased movement of the ETT in the larynx .
 Deliver oxygen as necessary .

CROUP
EPIGLOTTITIS
Age: Younger infants
 No fever
 Harsh stridor

Parainfuenza virus
 Usually no antibiotics,
no intubation

Age: older children
 High fever, toxic
 Soft stridor, Drooling,
muffled
 H influenza(Bacteria)
 Needs antibiotics and
intubation

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DO
AND
DON’T
IN
UAW DIS.
Allow the patient in his position of comfort
 Do not separate the child from mother
 Do not force the child to lie down
 Do not make the child cry?
 Do not send the child for X-ray without
accompanied by medical team
 Continuously monitor for the need for intubation.

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BRONCHOLITIS

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Definition : acute inflammatory disease of the
lower tract that result in obstruction of small
airways .
Causeas : viral infection .
Sing & symptom :
Cough.
Wheezing .
Prolong expiration.
Irritability .
Low grade fever .
Tachypnea .
Retraction .
NURSING MANAGEMENT
Maintained fluid & nutritional requirement
 Monitor child closely for respiratory failure.
 provide medication as order .
 Provide oxygen as needed or M.V if respiratory
failure ,hypoxemia or apnea are developed .
 Check vital sign .
 Monitor pulse oximetry
 Prevent nosocomial infection by good HW.

PNEUMONIA
Pneumonia is infection of the lung caused most often by
bacteria or viruses .
 Mode of transmission:
1-Inspiration.

2-Aspiration.
3-Circulation.
 Bypass of nasal defense
 Pulmonary aspiration (CNS, GER, TEF)
 Abnormal secretions or mucociliary clearance
 Underling chronic disease/nutrition
 Defect in the immune system

Sign & symptom :

Chills, fever and cough
Stuffy nose
Irritability
Resp distress: expiratory grunting, nasoflaring,
retraction, tachypnea, tachycardia.
Cyanosis, air hunger, and occasionally apnea

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NURSING MANAGEMENT
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Monitor V\S ,ABG level ,oxygen saturation .
Administered nebulization & oxygen at
concentration as prescribed .
Placed in high fowler position .
Rendered chest physiotherapy .
Encourage pt. to deep breathing & cough .
Encourage pt. to increased fluid intake to liquefy
secretion .
Assess lung sound .
Administered antibiotic as ordered .
Avoid contact with people with URTI .
ASPIRATION PNEUMONIA
Definition :inflammation caused by pulmonary
aspiration of gastric fluid produced direct injury to the
mucosal surface of the respiratory tract .
 Causes :see table
 Sing & symptom :
 Cough ,fever .
 acute dyspnea.
 wheezing , crackles or absent breathing sound in the
effected lobes ,
 cyanosis, retraction , tachypnea .

CAUSES OF ASPIRATION PNEUMONIA
Altered level of consciousness
Drugs, alcohol, anesthesia,
seizures, CNS disorders
Altered anatomy or function of
trachea or esophagus
Traceal or esophageal
abnormalities, ETT,
tracheostomy
Altered function of swallow or
esophageal motility
Loss of normal reflexes which
prevent aspiration of stomach
contents, GER (worse with
neuro or anatomical
impairment)
Inhalation injury
Inhalation of toxic substances
NURSING MANAGEMENT
Monitor V\S ,ABG level ,oxygen saturation .
 Administered nebulization & oxygen at
concentration as prescribed .
 Placed in high fowler position .
 Rendered chest physiotherapy .
 Encourage pt. to deep breathing & cough .
 Encourage pt. to increased fluid intake to liquefy
secretion .
 Assess lung sound .
 Administered antibiotic as ordered .

ARDS
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Definition :acute lung injury & noncardiogenic
pulmonary edema .
Causes :
Gastric aspiration.
Toxic inhalation .
Pulmonary infection .
FBA .
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

Sing & symptom :
Stage 1 :(1-2 day) : mild tachypnea,
hypoxemia,anxiety or restlessness.
Stage2 :(2-3 day) :cyanosis in room air
,tachycardia,retraction .
Stage 3:(3-10 day ):symptom change from distress
to failure (inability to oxygenate &
ventilate,alveolar collapse desaturation ,high
oxygen requirement .
Stage 4 : (after 10 day ):development of
pulmonary fibrosis & progressive impairment of
oxygenation are observed .
NURSING MANAGEMENT
Encourage coughing & deep breathing in awake pt.
 Frequent position change with CPT as needed .
 Reduce abdominal distention by NGT if necessary .
 Check vital sign .
 Monitor pulse oximetry .
 Do VBG & ABG analysis .
 Provide supplemental oxygen for pt. with adequate
ventilation .
 Provide medication such as sedative ,analgesic
,neuromuscular blocking agents ,bronchodilator as
ordered .
 Provide high calories & high protein diet.

ASTHMA (CHRONIC DISEASE )


Definition :obstructive pulmonary disease
characterized by airway inflammation with
mucosal edema,thick secretion that cause
plugging & hyperreactivity of the
tracheobronchial tree that result in
bronchospasm of the smooth muscle .
Causes :inflamatory mediators are thought to
stimulate the vagus nerve ( cholinergic
stimulation )causing smooth muscle constriction
& increased production of mucus.
Sing & symptom:
Tachycardia.
Tachypnea.
Cynosis.
Expiratory wheezing in severe case.
Inspiratory & expiratory wheezing .
Hypoxemia .

NURSING MANAGEMENT
Monitor pattern of breathing & V\S at regular
interval .
 Assess S & S of respiratory distress .
 Administer oxygen & nebulization as indicated .
 Assess lung sound .
 Monitor pulse oximetry & ABG level as indicated
.
 Encourage rest in between activity to prevent
fatigue & encourage deep breathing .



1- Endocrine glands : secrete hormones directly into
the bloodstream ( adrenal , pancreas ,thyroid glands)
 2-Exocrine
glands :secrete biochemical substances
that are released into ducts to be delivered to target
organs ( salivary ,sweat glands )
 Major glands :
 1-Hypothalamus-pituitary complex
.(ACTH,TSH,ADH,Oxytocin )
 2-Thyroid gland.(T3,T4)
 3-Parathyroid gland.(PTH)
 4-Adrenal gland.(sex hor.
Cortisol,epinephrine,norepinephrine
)
 5-Islets of langerhans in the pancreas.insullinbeta cell, glucagon-alpha cell, somatostatin-delta
cells
 6-Gonads.estrogen,progesterone,testosterone .
FIG 1. PITUITARY HORMONES AND THEIR
TARGET ORGANS.
DIABETES MELLITUS
 Definitions




Type I: Insulin-dependent mellitus (IDDM)
It is autoimmune disease that result in the T-cell-mediated
destruction of the beta pancreatic cells .it is the most common
form of diabetes in infants & children & requires insulin
replacement therapy .
Type II: Non-insulin-dependent diabetes mellitus (NIDDM)
Is associated with obesity ,strong family history & older age .it
is not autoimmune process but instead due to insulin
resistance enough insulin is produced to prevent ketoacidosis.
it can treated by oral hypoglycemia agents , diet.exercise .
DIABETES MELLITUS
 Diabetic

ketoacidosis
Is the absence of insulin & cellular uptake of
glucose is inhibited & glucose production by
the liver is increased & glucose utilization
decreased resulting in hyperglycemia .
Etiology :
 is related to inadequate endogenous insulin
secretion . Acute stress, infection ,trauma ,
high dose of steroide .

TABLE 1. SIGNS AND SYMPTOMS
Symptoms
Hyperglycemia
Metabolic acidosis (gap acidosis)
Dehydration, shock
Kussmaul breathing
Cardiac arrhythmia
Sodium imbalance
Potassium imbalance
Mental status changes
Hyperosmolality
Ketonuria
Glucosuria
Management
Fluid
Electrolytes
Insulin
Monitoring

NURSING MANAGEMENT
Monitor V\S & blood sugar level .
 Provide 3 regular diabetic meals with in between
snacks as tolerated .
 Check weight daily \weekly as indicated .
 Observed for circulation on feet .
 Monitor intake & output .
 Check urine for protein & ketones .
 Monitor S\S of hypo & hyperglycemia .
 Administered insulin regimen as prescribed .

TABLE 3. CLINICAL OBSERVATION IN
DEHYDRATION
Examination
Dehydration
Skin turgor
Skin (touch)
Buccal mucosa/lips
Eyes
Tears
Fontanelle
CNS
Pulse rate
Pulse quality
Capillary refill
Urine output
Older Child:
3% (30mL/kg)
6% (60mL/kg)
9% (90mL/kg)
Infant
5% (50mL/kg)
10% (10mL/kg)
15% (150mL/kg)
Mild
Normal
Normal
Moist
Normal
Present
Flat
Consolable
Normal
Normal
Normal
Normal
Moderate
Tenting
Dry
Dry
Deep set
Reduced
Soft
Irritable
Slightly increased
Weak
=2 sec
Decreased
Severe
None
Clammy
Parched/cracked
Sunken
None
Sunken
Lethargic/obtunded
Increased
Feeble/impalpable
>3 sec
Anuric
TABLE 4. INSULIN PREPARATION
Insulin
Onset (hr)
Rapid acting
NovoLog
Humalog
0.17-0.33
0.25-0.5
Short acting
Regular
0.5-1
Intermediate
acting
NPH
Lente
Long acting
Ultralente
Basal
Lantus
1-4
1-4
4-10
5
Peak (hr)
Duration (hr)
1-3
0.5-1.5
3-5
6-8
1-5
3-10
4-14
4-14
10-24
12-24+
8-30
18-36
No pronounced peak
20-24+
SYNDROME OF INAPPROPRIATE
ANTIDIURETIC HORMONE

Characterized by inappropriate,
excessive secretion of ADH

Occurs in the face of low serum Na and
low serum osmolality

Clinical signs and symptoms are
secondary to increased blood volume
and hyponatremia.
PATHOPHYSIOLOGY OF SIADH
ETIOLOGY AND RISK FACTORS
 Conditions
associated with SIADH
Meningitis, Head trauma,
Cerebral tumors, Cerebral
hemorrhage, Chornically ill or
malnourished, Spinal surgery

SIGNS AND SYMPTOMS
Low urine output and the absence of
hypovolemia
Headache, confusion, lethargy, altered
LOC, coma or seizures
Nausea and vomiting
Weight gain
Filling pressures (normal or increase)
Hypertension and tachycardia

MANAGEMENT
Serum sodium: normalized over 24 to 48
hours
 Monitoring: intake & output , specific
gravity, serum electrolytes and
osmolality, & daily weights.

NURSING MANAGEMENT
 Monitor
intake & output
 Monitor specific gravity.
 Monitor serum electrolytes and osmolality
 daily weights.
DIABETES INSIPIDUS
Characterized by a decrease in both urine
concentrating ability and water conservation.
 Types of DI


Central
Common form in children and
critical care environment, deficiency
of ADH due to failure of the
hypothalamus to synthesize or
failure of the posterior pituitary to
secrete or both .

Nephrogenic
Characterized by normal secretion of
ADH by the posterior pituitary but
the distal tubule & collecting ducts
in the kidney are resistant to the
effects of ADH .
DIABETES INSIPIDUS
 Etiologyy

:
Central
Pituitary and head trauma, CNS
infections, cerebral edema, cerebral
hemorrhage or infarct, congenital,
familial, idiopathic
 Nephrogenic
Chronic renal disease, polycystic
kidney disease, pregnancy, starvation

SIGNS AND SYMPTOMS

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Large quantity of dilute urine
Hypernatremia
Serum hyperosmolality
Polyuria
Urine osmolality
Polydipsia
Signs of dehydration
Altered mental status
DIABETES INSIPIDUS
 Management

Central
Use fluid or ADH replacement
therapy


Nephrogenic
Difficult to treat, Sodium-restricted
diet

NURSING MANAGEMENT
Monitor intake and output.
Daily weights.
Frequent hemodynamic.
Neurologic assessments.
Assess signs of dehydration.
Assess urine & electrolytes result .
Monitor specific gravity.

DIFFERENTIATION BETWEEN DI AND
SIADH
DI
SIADH
Serum sodium
>145 mEq/L
<135 mEq/L
Urine sodium
Low
High
Serum osmolality
>300 mOsm/kg
<275 mOsm/kg
Urine osmolality
<300 mOsm/L
>800mOsm/L
<1.005
>1.020
High
Low
Urine specific gravity
Urine output
THYROID GLAND


It is the largest endocrine gland. It lies in front of the
neck; it is formed two lobes connected by an isthmus
which crosses the trachea.
THYROID GLAND
 The
thyroid gland secretes the following
hormones:
1-Thyroxine (tetraiodothyronine = T4) and
(triiodothyronine, = T3) both hormones are iodine
containing amino acid.
 2-Calcitonin: it is a calcium lowering hormone,
polyeptide in nature, secreted from parafollicular cells
of thyroid gland. It lowers the plasma calcium level.


Actions of T4 and T3:
Increase oxygen consumption and enhances the
metabolism of all the body tissues.
 Increase protein synthesis in the kidney, muscle
and liver. They stimulate growth. They increase
activity of most of enzymes in the cells.
 Increase the rate of glucose absorption, uptake,
and utilization by tissues.

Increase the metabolism of fat from its stores and
use its fat for production of energy and it
decreases the level of plasma cholesterol.
 Accelerates the heart rate and it is an
erythropoietic factor.
 It increases appetite, the motility and secretions
of gastro – intestinal tract.
 Are important for normal development and
maturation of Central Nervous System.

HYPERTHYROIDISM
 Hyperthyroidism(Grave’s
disease):
Overactivity of the Thyroid Gland, In
healthy people, the thyroid makes
just the right amounts of two
hormones, T4 and T3.
 Hyperthyroidism
is a condition
caused by the effects of too much
thyroid hormone on tissues of the
body.
CAUSES
 There
are several causes of
hyperthyroidism. Most often, the entire
gland is overproducing thyroid hormone.
This is called Graves' disease.
 Less commonly, a single nodule is
responsible for the excess hormone
secretion. We call this a "hot" nodule.
 Thyroiditis (inflammation of the thyroid)
can also cause hyperthyroidism.
SYMPTOMS AND SIGNS

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
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








Palpitations
Heat intolerance
Nervousness
Insomnia
Breathlessness
Increased bowel movements
Light or absent menstrual periods
Fatigue
Fast heart rate
Trembling hands
Weight loss
Muscle weakness
Warm moist skin
Hair loss
Enlargement of the thyroid gland (goiter).
Protrusion of the eye balls due to excessive growth and
degeneration of tissues behind the eye.
TREATMENT
Anti-thyroid Drugs
 Radioactive Iodine Treatment
 Surgical Removal of the Gland or Nodule

NURSING MANAGEMENT
 Monitor
V\S .
 Assess skin turgor & mucous membrane
for signs of fluid deficit .
 Monitor intake & output .
 Monitor weight daily .
 Provide high calorie food .
 Provide quiet ,calm ,cool environment
 Inspected skin frequently
HYPOTHYROIDISM
condition leading to the deficiency in the
production of thyroid hormone.
 Iodine deficiency is the most common cause of
hypothyroidism worldwide but it can be caused
by any number of other causes such as several
conditions of the the thyroid gland, or less
commonly, the pituitary gland or hypothalamus.

Before Puberty (cretinism):
 Mal development or complete absence of gland in a new
born gives the signs and symptoms of cretinism at the
age of about 6 months when the mother’s milk becomes
deficient in thyroxin, it is characterized by:
 Mental retardation due to defective of nerve fibers,
which lead to delayed mile stones as (sitting walking
and speech).
 Retarded growth, the height is less than one meter.
 Intolerance to cold weather and decrease BMR.
 Abdominal muscle weakness, with protruded viscera
and protruded enlarged tongue.
 Infantile sexual organs.
 Thick skin.
 Raised serum cholesterol level.



After Puberty Myxoedema:
This due to atrophy of the thyroid glands or due to
thyroidectomy for any reason. It is characterized by:
 Slow mental activity.
 Increase sensitivity to cold, the BMR decreased (30
to 40).
 The hair is coarse and sparse, dry skin, firm non
pitting oedema.
 Sexual hypo function in female, there is irregular
cycle, in male there is impotence.
 Loss of appetite and decrease motility of the
gastrointestinal tract which leads to constipation.
 Deficient erythropoiesis leading to anaemia.
SING & SYMPTOM



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

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





Fatigue.
Cold intolerance increased sensitivity to cold
Constipation
Depression.
muscle cramps and joint pain
Thin, brittle fingernails
Coarse hair
Decreased sweating
Dry, itchy skin
Weight gain and water retention
Bradycardia.
Thinning of the outer third of the eyebrows (sign of
Hertoghe).
Abnormal menstrual cycles.
CAUSES
Iodine deficiency is the most common cause of
hypothyroidism worldwideor a deficiency in
stimulating hormones from the hypothalamus or
pituitary.
 Congenital hypothyroidism is very rare
accounting for approximately 0.2‰
 Hypothyroidism can result from postpartum
thyroiditis, a condition that affects about 5% of
all women within a year of giving birth.
 Hypothyroidism can also result from sporadic
inheritance, sometimes autosomal recessive.

CLASSIFICATION
Primary: Thyroid gland: The most common forms
include Hashimoto's thyroiditis (an autoimmune
disease) and radioiodine therapy for
hyperthyroidism.
 Secondary: Pituitary gland: Occurs if the
pituitary gland does not create enough thyroidstimulating hormone (TSH) to induce the thyroid
gland to produce enough thyroxine and
triiodothyronine. Although not every case of
secondary hypothyroidism has a clear-cut cause,
it is usually caused by damage to the pituitary
gland, as by a tumor, radiation, or
surgery.[Secondary hypothyroidism accounts for
less than 5% or 10% of hypothyroidism cases.


Tertiary: Hypothalamus: Results when the
hypothalamus fails to produce sufficient
thyrotropin-releasing hormone (TRH). TRH
prompts the pituitary gland to produce thyroidstimulating hormone (TSH). Hence may also be
termed hypothalamic-pituitary-axis
hypothyroidism. It accounts for less than 5% of
hypothyroidism cases.
TREATMENT
 Hypothyroidism
is treated with the
levorotatory forms of thyroxine
(levothyroxine) (L-T4) and
triiodothyronine (liothyronine) (L-T3).
NURSING MANAGEMENT
 Monitor
vital signs .
 Monitor ECG tracing to detect
arrhythmias .
 Provide warm environment.
 Provide high fiber diet .
 Administered prescribed stool softener .
 Administered medication as prescribed .
 Encourage increase intake of fluids .
 Provided warm drink .
GROWTH HORMONE (SOMATOTROPIN)
HGH is synthesized and secreted from the anterior
pituitary gland Effects of growth hormone on the tissues
of the body can generally be described as anabolic
(building up).
 In addition to increasing height in children and
adolescents, growth hormone has many other effects on
the body:
 Increases calcium retention, and strengthens and
increases the mineralization of bone
 Increases muscle mass through sarcomere hyperplasia
 Promotes lipolysis

Increases protein synthesis
 Stimulates the growth of all internal organs
excluding the brain
 Plays a role in homeostasis
 Reduces liver uptake of glucose
 Promotes gluconeogenesis in the liver
 Contributes to the maintenance and function of
pancreatic islets
 Stimulates the immune system

 High
levels of growth hormone may
indicate:
 Acromegaly
 Gigantism
 Growth hormone resistance
 Pituitary tumor.
 Low levels of growth hormone may
indicate:
 Growth hormone deficiency.
 Hypopituitarism.
EXCESSIVE GROWTH HORMONE
SECRETION
Gigantism is the result of excessive growth
hormone secretion that begins in young children
or adolescents. It is a very rare disorder, usually
resulting from a tumor of somatotropes. One of
the most famous giants was a man named Robert
Wadlow. He weighed 8.5 pounds at birth, but by
5 years of age was 105 pounds and 5 feet 4 inches
tall. Robert reached an adult weight of 490
pounds and 8 feet 11 inches in height. He died at
age 22.
 Acromegaly results from excessive secretion of
growth hormone in adults.

GROWTH HORMONE DEFICIENCY
 The
effects of growth hormone deficiency
vary depending on the age at which they
occur. In children, growth failure and
short stature are the major
manifestations of GH deficiency, with
common causes including genetic
conditions and congenital malformations.
It can also cause delayed sexual maturity.
Dwarfism:
 It is deficiency of growth hormone in young age.
The rate of development is decreased so the child
who has reached the age of 10 years has the body
of 4 years. The dwarf is short and mentally
normal.
TREATMENT
 For
excessive secration : drugs like
octreotide (somatostatin agonist) and
bromocriptine (dopamine agonist) can be
used to block GH secretion because both
somatostatin and dopamine negatively
inhibit GHRH-mediated GH release from
the anterior pituitary.
 For deficiency: injection of GH.
NURSING MANAGEMENT
provide medication as order .
 Measure weight & height .
 Check weight daily \weekly as indicated .
 Assess GH level .
 Educate pt. & family about the important of
follow up .

ADRENAL GLAND DYSFUNCTION
Hypo function of adrenal cortex:
 (Addison disease):
 It is due to adrenal cortical insufficiency. It
is characterized by:
 Loss of appetite, vomiting, and diarrhea i.e.
gastro intestinal disturbances.
 Muscle weakness due to decrease of anabolic
effect of androgens.





Decrease sodium and increase potassium in
the blood. (Water moves intracellular, ----- the
blood volume decreases and hypotension occur.
Hypoglycemia.
Decrease sexual function.
The Bronze hyper pigmentation of the skin
due to high level of ACTH secondary to low
level of glucocorticoids. ACTH has melanocytic
stimulating activity.
CUSHING'S SYNDROME


Cushing's syndrome is a hormone disorder caused by
high levels of cortisol in the blood.
This pathology was described by Harvey Cushing in
1932.[The syndrome is also called Itsenko-Cushing
syndrome, hyperadrenocorticism or hypercorticism)
Hyper function of Adrenal Cortex
 (Cushing's Syndrome):
 It is characterized by:
 Wasting and weakness of muscle, (catabolic effect).
 Increase sodium and water retention in the blood
(leading to oedema and hypertension with decrease
level of plasma potassium).
 Redistribution of fat, the extremities are thin, there
are accumulation of fat in the abdominal wall, face
(MOON) and the upper back (buffalo trunk).
 Hyperglycemia.
 Gonadal disturbances.
 The skin is thin.


the hypothalamus releases corticotropinreleasing hormone (CRH), which stimulates the
pituitary gland to release adrenocorticotropin
(ACTH). ACTH travels via the blood to the
adrenal gland, where it stimulates the release of
cortisol. Cortisol is secreted by the cortex of the
adrenal gland from a region called the zona
fasciculata in response to ACTH. Elevated levels
of cortisol exert negative feedback on the
pituitary, which decreases the amount of ACTH
released from the pituitary gland. Strictly,
Cushing's syndrome refers to excess cortisol of
any etiology.
One of the causes of Cushing's syndrome is a
cortisol secreting adenoma in the cortex of the
adrenal gland. The adenoma causes cortisol
levels in the blood to be very high, and negative
feedback on the pituitary from the high cortisol
levels causes ACTH levels to be very low.
Cushing's disease refers only to
hypercortisolism secondary to excess production
of ACTH from a corticotrophic pituitary
adenoma. This causes the blood ACTH levels to
be elevated along with cortisol from the adrenal
gland. The ACTH levels remain high because a
tumor causes the pituitary to be unresponsive to
negative feedback from high cortisol levels.
 Cushing's Syndrome was also the first
autoimmune disease identified in humans.

CAUSES
:
Exogenous vs. endogenous
 Hormones that come from outside the body are
called exogenous.
 hormones that come from within the body are
called endogenous.


The most common cause of Cushing's syndrome
is exogenous administration of glucocorticoids
prescribed by a health care practitioner to treat
other diseases (called iatrogenic Cushing's
syndrome). This can be an effect of steroid
treatment of a variety of disorders such as
asthma and rheumatoid arthritis, or in
immunosuppression after an organ transplant.

Administration of synthetic ACTH is also
possible, but ACTH is less often prescribed due to
cost and lesser utility.
 Endogenous
Cushing's syndrome results
from some derangement of the body's own system
of secreting cortisol. Normally, ACTH is released
from the pituitary gland when necessary to
stimulate the release of cortisol from the adrenal
glands.
 In pituitary Cushing's, a benign pituitary
adenoma secretes ACTH. This is also known as
Cushing's disease and is responsible for 70% of
endogenous Cushing's syndrome.
In adrenal Cushing's, excess cortisol is produced
by adrenal gland tumors, hyperplastic adrenal
glands, or adrenal glands with nodular adrenal
hyperplasia.
 Finally, tumors outside the normal pituitaryadrenal system can produce ACTH that affects
the adrenal glands. This final etiology is called
ectopic or paraneoplastic Cushing's syndrome and
is seen in diseases like small cell lung cancer .

SING & SYMPTOM
rapid weight gain particularly of the trunk and
face "moon face “ with sparing of the limbs
(central obesity ).
 A common sign is the growth of fat pads along
the collar bone and on the back of the neck
(buffalo hump) .
 Other symptoms include hyperhidrosis (excess
sweating),.
 Telangiectasia (dilation of capillaries).

Thinning of the skin (which causes easy bruising
and dryness particularly the hands and other
mucous membranes, purple or red striae (the
weight gain in Cushing's syndrome stretches the
skin, which is thin and weakened, causing it to
hemorrhage) on the trunk, buttocks, arms, legs or
breasts, proximal muscle weakness (hips,
shoulders).
 Hirsutism (facial male-pattern hair growth).
 Baldness and/or cause hair to become extremely
dry and brittle.

In rare cases, Cushing's can cause hypercalcemia
which can lead to skin necrosis (osteoporosis ).
The excess cortisol may also affect other
endocrine systems and cause, for example,
diabetes mellitus
 menstrual disorders such as amenorrhea in
women and decreased fertility in men infertility
due to elevations in androgens.


signs include polyuria (and accompanying
polydipsia), persistent hypertension (due to
cortisol's enhancement of epinephrine's
vasoconstrictive effect) and insulin resistance
(especially common in ACTH production), leading
to hyperglycemia (high blood sugar) and insulin
resistance which can lead to diabetes mellitus.

Patients frequently suffer various psychological
disturbances, for example, euphoria , depression
and anxiety.
Haematology and
Immunology
Cell differentiation from stem cells
Anatomy and Physiology
1- Red blood cells (RBCs, erythrocytes):

Develop from erythroid precursor cells

Reticulocytes represent the stage of maturation

Mature RBCs have a life span of 120 days


Hemoglobin: large, complex, iron-containing protein.
Responsible for the RBCs oxygen-carrying abilities.
RBC: transport oxygen from the lungs to the tissues
Hgb A, normal adult hemoglobin
Hemoglobin F ( fetal Hgb) present in
large concentrations in the fetus,
decrease after birth and minimally
present in children and adults.
Normal RBC production requires iron,
folic acid and Vit B12.

THE IMMUNE SYSTEM
2-Leukocytes (white blood cells)


group that serve to protect the organism.
Neutrophils: the largest component of total
circulating WBCs, 60 – 70%.
 Originate and mature in bone marrow
 Polymorphonucleated (PMNs or poly): mature form
of neutrophil
 Band: unsegmented – appearing nucleus
 Neutrophilia: increased number of circulating
neutrophils
Anatomy
Neutropenia: decreased number of
circulating neutrophils (<1500/mm3)
 WBCs mature in bone marrow for 10 days


Eosinophils: 2 – 5% of the circulating WBC.
Eosinophilia: increased number of eosinophils
 Eosinopenia: decreased number of eosinophils


Basophils: smallest portion of granulocytes, 1% of
WBC
 Basophilia increased number of circulating basophils
 Basopenia decreased number of circulating basophils
Mononuclear phagocytes


Monocytes 3 – 8% of circulating WBCs
 Monocytosis increase in the number of circulating
monocytes
 Monocytopenia decrease in the number of
circulating monocytes
Macrophages: have long life span.
Lymphocytes (lymphoid lineage): primary immune
cells
Lymphocytes: 10 – 40% of the ciculating WBCs
 Lymphocytosis: increase number of circulating
lymphocytes
 Lymphopenia: decrease in number of circulating
lymphocytes
 T lymphocytes or T cells: 65 – 85% of all
lymphocytes
 Clusters of differentiation (CD)
 Helper T cells (CD4)
 Suppressor T cells (CD8)
 Cytotoxic or killer T cells (CD8)

Lymph nodes of the body
Anatomy
B Lymphocytes or B cells: constitute up to 35%
 NK cells constitute 5 – 8% of the total lymphocyte
count
 Memory cells – have CD. Programmed to recognize
the original invading microorganism on subsequent
invasions.
 WBC
 Functions
 Defense: protect the body’s internal environment from
“nonself” antigens or microorganisms invasion.
 Assists the immune system in discriminating “self” from
“nonself” or “altered self”
 Second line of defense: involves the inflammatory
response, phagocytosis .

3-Platelets (thrombocytes)
1.
2.

Megakaryoblasts: mature into megakaryocytes
Characteristics: minute, round or oval discs.
 Platelet count: 150, 000 to 400, 000/mm3.
Maintain normal hemostasis & vascular integrity
when a blood vessel wall is injured.
Anatomy
4-Plasma factors:
1.
Procoagulants: aka plasma clotting factors. Promotes
coagulation.
2.
Anticoagulants: inhibit coagulation
 Circulating anticoagulants: antithrombin III, protein C,
protein S
3.
Coagulation: depends on the balance between the
procoagulants and the anticoagulants
Nomenclature for coagulation factors
Factor
Synonym
I
Fibrinogen
II
Prothrombin
III
Tissue thromboplastin
IV
Calcium
V
Proaccelerin
VI
Not assigned
VII
Proconvertin
VIII
Antihemophilic factor (AHF|)
IX
Plasma thromboplastin component (Christmas factor
X
Stuart factor (Stuart-Prower factor)
XI
Plasma thromboplastin antecedent (PTA)
XII
Hageman factor
XIII
Fibrin-stabilizing factor (FSF)
ANEMIA

Anemia is defined as a reduction below normal
in the volume of red blood cells volume or
hemoglobin concentration.

Classification of Anemias 

Anemias can be classified by two
approaches:
 Etiologic factors.
 Morphologic factors.
 I.
Etiologic Factors:
Anemias resulting from impaired or
decreased production:

The production of the RBCs and Hb can be
impaired by:
 Nutritional deficiency (e.g.folate, iron, B12).
 Bone marrow failure (e.g. irradiation,
malignancy).
 Interference with bone marrow activity (e.g.
infection, chronic diseases).




Anemias resulting from accelerated destruction of
RBCs (Hemolytic):
There are causes that can shorten the life span of the cell,
which can be:
Corpuscular Causes:
 Defect in cell membrane: Hereditary erythrocytosis.
 Defect in cell enzyme: G6PD deficiency.
 Defect in cell hemoglobin: Sickle cell anemia, Thalassemia.

Extracorpuscular causes:

Immune: blood transfusion.

Non- immune: - burns. - Snake venoms.

Anemias resulting from excessive blood loss:

Acute blood loss: (bleeding)


Alteration in vital signs doesn’t occur unless 20% or more of blood
lost occur. Shock occurs with losses of 40% of blood volume.
Chronic blood loss: (occult bleeding, Ancylostoma)
 II.

Morphologic Factors:
The characteristic changes in RBCs’ size,
shape, color is described as below:
 Cell
size: for example, normocytes (normal),
microcytes (smaller than normal), or
macrocytes (larger than normal).
 Shape-
irregularly shape RBCs; for
example, poikilocytes (irregularly shaped cell),
spherocytes (globular cells), and drepanocytes
(sickle cells).
 Staining
characteristics or color:
Reflects the hemoglobin concentration; for
example, normochromic (Sufficient or normal
amount) or hypochromic (reduced amount).
SING & SYMPTOM
General Manifestation:
 Dyspnea on exersion.
 Muscle weakness.
 Easy fatigability.
 Frequent resting.
 Shortness of breath.
 Poor sucking (infants).
 Pale skin: waxy pallor seen in severe anemia.
 Tachycardia.
 Cardiac dilatation gradually develops.

Central nervous system manifestations:
 Headache.
 Dizziness.
 Night headache.
 Irritability.
 Slowed thought processes.
 Decreased attention span.
 Apathy.
 Depression.
 Shock [blood loss anemia]:
 Poor peripheral perfusion.
 Skin is moist and cool.
 Low blood pressure.
 Increased heart rate.

Anemias Caused by Defect in
 Hemoglobin Formation
Sickle cell anemia (SCA).
 Thalassemia.


SICKLE CELL ANEMIA
This disorder is characterized by severe chronic
hemolytic disease resulting from premature
destruction of the brittle poorly deformable
erythrocytes. In which normal adult hemoglobin
A (Hgb A) is partly or completely replaced by
abnormal sickle hemoglobin (Hgb S).
 It is a genetically determined (autosomal
reccesive) disease.

SICKLE CELL ANEMIA

sickle cell anemia, the number of red blood cells
is low because sickle cells don't last very long.
Sickle cells usually die after only about 10 to 20
days. The bone marrow can't make new red blood
cells fast enough to replace the dying ones.
Normal red blood cells are disc-shaped and look
like doughnuts without holes in the center. They
move easily through your blood vessels. Red
blood cells contain an iron-rich protein called
hemoglobin. This protein carries oxygen from the
lungs to the rest of the body.
 Sickle cells contain abnormal hemoglobin called
sickle hemoglobin or hemoglobin S. Sickle
hemoglobin causes the cells to develop a sickle, or
crescent shape.
 Sickle cells are stiff and sticky. They tend to
block blood flow in the blood vessels of the limbs
and organs. Blocked blood flow can cause pain,
serious infections, and organ damage.

CAUSES :
Sickle cell anemia is inherited from both parents.
Sickle cell disease is much more common in
people of African and Mediterranean descent. It
is also seen in people from South and Central
America, the Caribbean, and the Middle East.
 Someone who inherits the hemoglobin S gene
from one parent and normal hemoglobin (A) from
the other parent will have sickle cell trait. People
with sickle cell trait do not have the symptoms of
true sickle cell anemia.

SING & SYMPTOM :













Severe sudden chest pain .
Splenomegaly in the young children .
Impaied growth & development .
Attacks of abdominal pain.
Bone pain.
Breathlessness.
Delayed puberty.
Fatigue.
Fever.
Paleness.
Rapid heart rate.
Ulcers. on the lower legs (in adolescents and adults).
Yellowing of the eyes and skin (jaundice).
The main objectives of medical treatment are:
 Bed rest to minimize energy expenditure and
oxygen use.
 Hydration through oral and intravenous therapy.
 Electrolyte replacement.
 Analgesics for severe abdominal and joint pain.
 Blood replacement to treat anemia.
 Antibiotics to treat any existing infection.
NURSING MANAGEMENT












Administer blood products as ordered .
Improve oxygenation .
Prevent iatrogenic anemia .
Provide adequate nutrition with high iron .
Administered iron replacement as ordered .
Monitor vital signs .
Assess pt. for palpitation , SOB, dizziness.
Administered parenteral vitamin B12 as prescribed .
Assess knowledge of present condition .reinforced
importance of therapeutic home management & OPD
follow up .
Assessed dietary intake . Provided diet high in iron .
Advised to rest in between activities .
Assessed level of fatigue , sleeping pattern , and activity
that causes fatigue
To reduce sickle cell crises, take the following
precautions:

To prevent oxygen loss, avoid:
 Demanding physical activity (especially if the spleen is
enlarged)
 Emotional stress
 Environments with low oxygen (high altitudes,
nonpressurized airplane flights)
 Smoking
 Known sources of infection


To make sure you're getting enough fluids:
 Avoid too much exposure to the sun
 Have fluids on hand, both at home and away
 Recognize signs of dehydration.
To avoid infection:
 Consider having the child wear a Medic Alert bracelet
 Have the child vaccinated as recommended by the
health care provider
 Share the above information with teachers and other
caretakers, when necessary

They should take supplements of folic acid
(essential for producing red blood cells) because
red blood cells are turned over so quickly.
Antibiotics and vaccines are given to prevent
bacterial infections, which are common in
children with sickle cell disease.
 Blood transfusions are used to treat a sickle cell
crisis.

Nursing Diagnosis:
 1. Altered nutrition, less than body requirements
related to reported inadequate iron intake,
knowledge deficit regarding iron reach foods.
 Goal:

Patient will receive adequate supply of iron.
 Intervention:
 Provide diet counseling to care giver, especially
in regard to food source of iron (e.g. meat, liver,
fish, egg yolks, green leafy vegetable, nuts, whole
grains, infants cereals and dry cereals).
 Expected Outcomes:
 Child receive at least minimum dailyrequirement
of iron.











Nursing Diagnosis:
2-Risk for injury related to abnormal hemoglobin,
decreased ambient oxygen, and dehydration.
Patient Goal 1:
Will maintain adequate tissue oxygenation.
Nursing Intervention:
Explain measures to minimize complications related to
physical exertion and emotional stress to avoid additional
tissue oxygen needs.
Prevent infection.
Avoid low-oxygen environment.
Expected Outcome:
Child avoids situations that reduce tissue
oxygenation.



Patient Goal 2:
Will maintain adequate hydration.









Nursing Intervention:
Calculate recommended daily fluid intake (1600 ml/m3/day)
and base child’s fluid requirements on this minimum
amount (specify) to ensure adequate hydration.
Increase fluid intake above minimum requirements during
physical exercise/emotional stress and during crisis to
compensate for additional fluid needs.
Give parents written instructions regarding specific
quantity of fluid required to encourage compliance.
Encourage child to drink to encourage compliance.
Teach family signs of dehydration to avoid delay in
rehydration therapy.
Stress importance of avoiding overheating as source of fluid
loss.
Expected Outcome:
Child drinks an adequate amount of fluid and shows
no signs of dehydration.
Nursing Diagnosis:
 3. Anxiety /fear related to diagnostic procedures,
transfusion.
 Goal:

Patient, family will become knowledgeable
about the disorder.
 Intervention:
 Prepare the child for tests.
 Remain with the child during tests and initiation
of transfusion.
 Explain purpose of blood components.
 Expected Outcomes:
 Child and family display minimal anxiety.
 Child and family demonstrate an understanding
of the disorders, diagnostic tests and treatment.

Nursing Diagnosis:
 4. Activity intolerance related to generalized
weakness, diminished oxygen delivery to tissues.
 Goal:

Patient will receive adequate rest.
 Intervention:
 Provide diversional play activities that promote
rest and quite but prevent boredom and
withdrawal.
 Choose appropriate roommate of similar age and
interests who requires restricted activities.
 Plan nursing activities to provide sufficient rest.
 Assist with activities requiring exertion.
 Expected Outcomes:
 Child plays and rests quietly and engages in

THALASSEMIA
Thalassemia is a blood disorder passed down
through families an inherited autosomal
recessive blood disease in which the body makes
an abnormal form of hemoglobin, the protein in
red blood cells that carries oxygen.
 The disorder results in excessive destruction of
red blood cells, which leads to anemia.

CAUSES :
Hemoglobin is made of two proteins: Alpha globin
and beta globin. Thalassemia occurs when there
is a defect in a gene that helps control production
of one of these proteins.
 There are two main types of thalassemia:
 1-Alpha thalassemia : occurs when a gene or
genes related to the alpha globin protein are
missing or changed (mutated).
 2-Beta thalassemia : occurs when similar gene
defects affect production of the beta globin
protein.


Alpha thalassemias occur most commonly in persons
from southeast Asia, the Middle East, China, and in those
of African descent.

Beta thalassemias occur in persons of Mediterranean
origin, and to a lesser extent, Chinese, other Asians, and
African Americans
There are many forms of thalassemia. Both alpha
and beta thalassemia include the following two
forms:
 Thalassemia major
 Thalassemia minor
 Thalassemia major : occurs if the baby receive
the defective gene from both parents.
 Thalassemia minor: occurs if the baby receive the
defective gene from only one parent.
 Persons with this form of the disorder are
carriers of the disease and usually do not have
symptoms.

β-Thalassemia Major(Cooley’s Anemia)
 It is most common form of thalassemia in the
world. More common in countries around
Mediterranean Sea. It is inherited as autosomal
genetic disorder of both genes controlling ß- chain
synthesis (homozygous).
 Normal postnatal hemoglobin (Hgb A) is
composed of two α and two β- polypeptide chains,
in thalassemia major there is a partial or
complete deficiency in the synthesis of β- chain of
Hgb molecule.
 Consequently, there is a tremendous increase in
the synthesis of α- chains and у chains.

SIGN & SYMPTOM


The most severe form of alpha thalassemia major
causes stillbirth (death of the unborn baby during
birth or the late stages of pregnancy).
Children born with thalessemia major (Cooley's
anemia) are normal at birth, but develop severe
anemia during the first year of life.
SINGS & SYMPTOM
Unexplained fever.
 Poor feeding.
 Markedly enlarged spleen.
 Headache.
 Bone pain.
 Decreased exercise tolerance.
 Anorexia.
 Frequent epistaxis.
 Hemochromatosis.
 Hemosiderosis.


Other Features:
 Small stature.
 Delayed sexual maturation.
 Protrusion of the abdomen (hepato- splenomegaly).
 Bone
Change (older children if untreated):
Enlarged head.
 Prominent frontal and parietal bosses.
 Flat or depressed bridge of the nose.
 Enlarged maxilla.
 Protrusion of the lip and upper central incisors
and eventual malocclusion.

TREATMENT :
Patients with thalassemia minor usually do not
require any specific treatment unless they have
very low HB.
 Treatment for patients with thalassemia major
includes chronic blood transfusion therapy.
 iron chelation therapy to remove excess iron
from the body.
 splenectomy .
 Bone marrow transplant may help treat the
disease in some patients especially children.

THERAPEUTIC MANAGEMENT:
Blood Transfusion.
 Chelation Therapy

Nursing Diagnosis:
 Anxiety / fear related to diagnostic procedures,
transfusion.
 Activity intolerance and comfort altered related
to hematomas in tissues and joints.
 High risk for bleeding after minor hematomas
related to deficiency of coagulation factor.
 Pain related to bleeding in joints.
 Impaired physical mobility related to
hemorrhage in joints and tissues.
 Altered family process related to child with
chronic illness.

Planning:
The objectives of nursing care can be divided into
immediate needs and long-term goals:
 Prevent bleeding.
 Recognize and control bleeding.
 Prevent crippling effect of bleeding.
 Support family and prepare for home care.
 Identify persons at risk.


Implementation:
 1. Prevent Bleeding:
 Prophylactic administration of AHF.
 Appropriate exercises to strengthen muscles and
joints and to allow age appropriate activity.
 To prevent oral bleeding, some readjustment in
terms of dental hygiene may be related to,
minimize trauma to the gums such as use of a
water-irrigating device, softening the toothbrush
in warm water before brushing.
 A peripheral fingerstick is better for blood
samples and subcutaneous route is substituted
for IM whenever possible.
 Neither aspirin nor any aspirin- containing
compound should be used. Acetaminophen
(Tylenol) is a suitable aspirin substitute,

DISSEMINATED INTRAVASCULAR C OAGULATION
DIC
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DIC is aserious bleeding disorder resulting from
accelerated normal clotting with a subsequent
decrease in clotting factors & platelets leading to
uncontrolled bleeding .
 Etiology
: it is always a result of another
disease or condition ,it is often associated with
shock ,infection ,hemolytic processes such as
transfusion of mismatched blood .
 Sing
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& symptom :
1- bleeding: can occur any where in the body following
trauma or normal activity or spontaneously .
2- specific sing & symptom :
Slow,persistent ,prolonged bleeding from minor
injuries .
Uncontrollable hemorrhage subsequent to dental
extraction or irritation of the gums .
Epistaxis especially after facial injury .
Hematuria.
Ecchymosis ( petechiae are rare ).
Bleeding into joints (hemarthrosis )which may lead to
severe joint deformity .
HEMOPHILIA
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Is a disorder of hemostasis of one or more
clotting factor.
 Type
:
1-hemophilia A: deficient in factor VIII,called
classic hemophilia .
 2- hemophilia B: deficient in factor IX : called
christmas disease .
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 Etiology
: sex link recessive traits occur almost
exclusively in male ,female carriers transmit the
disease
Hemophilia (Classic Hemophilia)
 It is one of the commonest (95%)congenital
coagulation disorders due to deficiency or
functional abnormality of factor VIII
demonstrated as a sex- linked ressive trait (male
is diseased and female is carrier).
 Blood clotting occurring in 3 phases, each is
dependent on the preceding phase.
 The basic defect of hemophilia (A) is a deficiency
of factor VIII (antihemophilic factor AHF).
 AHF is produced by the liver and is necessary for
formation of thromboplastin in phase I of blood
coagulation. The less AFH in the blood, the more
severe the disease.
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The frequency and severity of bleeding are
related to level of factor VIII deficiency:
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Clinical severity

Factor VIII Activity
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Bleeding Tendency

Severe
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1% _Spontaneous bleeding without trauma.
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Moderate
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1%-5% _Bleeding with trauma.
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Mild
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5%- 50% _Bleeding with severe trauma or surgery.
 Sing
& symptom :
1-Bleeding
 Skin : petechia ,purpura , hematoma .
 Head :gingival bleeding & epistaxis .
 GI: hematemesis & melena .
 Renal: hematuria .
 Nuro :headache , altered LOC.
 CV: symptom of shock .
 2-platelet cunt is decreased .
 3-prolong coagulation test .
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TEATMENT
The primary therapy for hemophilia is
replacement of the missing clotting factor.
 Vigorous therapy is instituted to prevent chronic
crippling effects from joint bleeding. If
replacement therapy is begun immediately, local
measures such as ice applications and splinting
are seldom needed.
 Other drugs may be included in the therapy
plans depending on the source of the hemorrhage.
Corticosteroids are used to treat inflammation in
the joints; nonsteroidal antiinflammatory drugs
such as aspirin, indomethasin (Indocin) and
phenylbutazona (Burazolidin) should not be used
because they inhibit platelet function.
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NURSING DIAGNOSIS:
Anxiety / fear related to diagnostic procedures,
transfusion.
 Activity intolerance and comfort altered related
to hematomas in tissues and joints.
 High risk for bleeding after minor hematomas
related to deficiency of coagulation factor.
 Pain related to bleeding in joints.
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LEUKEMIA
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It is a malignant disorder involving bone marrow
dysfunction manifested by anemia,
thrombocytopenia, and abnormal proliferation of
white cells, which results in harmful filtration
into tissues. It is the most common malignancy in
childhood (30%), peak incidence 2-8 years of age.
Incidence among males is greater than females’
2:1.
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Etiology:
Although the exact cause is unknown, but multiple
factors believed to be responsible: ionizing
radiation, chemicals (benzene compound, heavy
metals), drugs (Alkylating agents), and
chromosomal abnormalities (Down syndrome),
and familial predisposition.
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Classification:
Leukemia is classified according to its predominant
cell type and level of maturity as described by the
following:
Lympho
for Leukemias involving the lymphoid or
lymphatic system.
Myelo
for those of myeloid (bone marrow) origin.
Blastic and acute
for those involving immature cells.
Cytic and chronic
for those involving mature cells.
In children two forms are generally recognized:
Acute lymphoid leukemia (A.L.L.) and acute nonlymphoid
leukemia (A.N.L.L) or (A.M.L).
A.L.L includes lymphatic, lymphocytic, lymphoblastic and
lymphoblastoid leukemia.
A.N.L.L. type includes granulocytic, myelocytic, monocytic,
myelogenous, monoblastic and monomyeloblastic.
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Common symptoms of chronic or acute leukemia may
include:
Swollen lymph nodes that usually don't hurt.
Fevers or night sweats.
Frequent infections.
Feeling weak or tired.
Bleeding and bruising easily (bleeding gums, purplish
patches in the skin, or tiny red spots under the skin).
Swelling or discomfort in the abdomen (from a
swollen spleen or liver).
Weight loss for no known reason.
Pain in the bones or joints.
TREATMENT
pharmaceutical medications, typically combined
into a multi-drug chemotherapy regimen.
 Some are also treated with radiation therapy.
 In some cases a bone marrow transplant is useful

Nursing diagnosis:
 1. High risk for infection related to depressed
body defenses.
 Goal:

Patient will experience minimized risk of
infection.
 Intervention:
 Place child in private room.
 Advise all visitors and staff to practice hand
washing.
 Screen all visitors and staff for signs of infection.
 Use aseptic technique.
 Evaluate child for any potential sites or
infections.
 Provide nutritionally complete diet for age.
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Nursing diagnosis:
 2. High risk for injury (hemorrhage) related to
interference with cell proliferation.
 Goal:

Patient will exhibit no evidence of bleeding.
 Intervention:
 Use all measures to prevent infection, especially
in ecchymotic area.
 Use local measures to stop bleeding.
 Restrict strenuous activity that could result in
accidental injury.
 Involve child in responsibility for limiting activity
when platelet count drops.
 Administer platelets as prescribed.
 Expected Outcome:
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Nursing diagnosis:
 3. Altered nutrition less than body requirement
related to loss of appetite.
 Goal:

Patient will receive adequate nutrition.
 Intervention:
 Encourage parents to relax pressure placed on
eating.
 Allow child any food tolerated. Improve quality of
food selections when appetite increases.
 Stress expected increase in appetite from steroid.
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Take advantage of any hungry period, sever
small snakes.
 Allow child to be involved in food preparation
and selection.
 Make food appealing.
 Remember usual food practices of children in
each age group.
 Expected Outcome:
 Nutrition intake is adequate.
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REFERENCES
www.nlm.nih.gov/medlineplus/ency/hptt.
 Medical –surgical nursing ,health & illness
prespectives ,seventh edition .
 www.ar.wikipedia.org/wiki/
 www.en.wikipedia.org/wiki/Thalassemia
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