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tongue, resembling milk curd but not scraping away;

underlying erythematous surface, may be painful
- Skin candidiasis: Severe, bright red, sharply circumscribed
(well defined) rash, most commonly in the diaper area with
intense erythema (Redness), well-demarcated border, and
satellite lesions; rash persists despite usual diaper rash
measures
➢ Mode of Transmission: Generally acquired during birth; rare
person-to-person transmission
➢ Therapeutic management:
- Skin candidiasis: Antifungal drugs like nystatin
(Mycostatin), clotrimazole (Lotrimin), naftifine (Naftin),
ketoconazole (Nizoral), econazole (Spectazole), ciclopirox
(Ciclodan), or miconazole (Zeasorb); nystatin is the
cheapest option
- Oral candidiasis (thrush): Nystatin administered orally
approximately four times a day, after feeding and cleaning
lesions
➢ Prevention: Maintain good hygiene, frequent diaper changes, and
proper cleaning of the affected areas.

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HEMATOLOGIC DISORDER ➔ Bilirubin ● Yellow pigment formed during the

➢ Pediatric Hematologic Disorders are BLOOD-RELATED conditions that breakdown of RBC
specifically affect children.
White Blood Cells ● Leukocytes
BLOOD FORMATION AND COMPONENTS ● Function
Defense against antigen invasion
Red Blood Cells ● Erythrocytes ● WBC Count
● Function Children: 5,000-15,000 cells/µL
To transport oxygen and carry carbon Adult: 4,000-11,000 cells/µL
dioxide away from body cells ● Lifespan
● Formed in the bone marrow under the Approximately 6 hours to unknown
stimulation of Erythropoietin (hormone intervals
formed by the kidneys)
● RBC Count ➔ Neutrophils ● Most abundant
Newborn: 4.1-5.5 million cells/µL ● Responsible for early response to
Adult Male: 4.7-6.1 million cells/µL bacterial infections and are involved in
Adult Female: 4.2-5.4 million cells/µL inflammation
● Lifespan
Adult: 120 days ➔ Lymphocytes ● Includes:
Newborn: Around 60-90 days (due to B cells - produces antibodies that
the presence of fetal hemoglobin target specific pathogens
(HbF) T cells - part of adaptive immune
Infants and Children: becomes similar system and memory cells
to adults because HbF will be - for Cancer cells, foreign cells,
replaced by adult hemoglobin (HbA) and infected cells
NK cells - Natural Killer cells,
➔ Hemoglobin ● component of RBC that indicates how responds more quickly than T cells
well your blood can carry oxygen
● Consists of Globin (a protein) and ➔ Monocytes ● Fight infections and help remove dead or
primarily, Heme (iron-containing pigment) damaged tissues

➔ Eosinophil ● Regulates inflammatory response

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➔ Basophil ● Releases histamine DISORDERS OF BLOOD COAGULATION

➢ Platelets is the key factor for blood coagulation
Platelets ● Thrombocytes
➢ Normal Platelet Level
● Function
● 150,000 platelets/µL
For coagulation and hemostasis
● <40,000 platelets/µL - Thrombocytopenia
● Lifespan
About 7-10 days
➢ Disorders of the RBC includes:
● Platelet Count
● Hemophilias
Adult: 150,000-450,000 platelets/µL
NORMOCHROMIC OR NORMOCYTIC ANEMIAS
COMMON HEMATOLOGIC DISORDERS IN CHILDREN
➢ NORMOCHROMIC OR NORMOCYTIC ANEMIAS
➢ Anemia ➢ Normochromic (normal color)
➢ Sickle Cell Disease ➢ Normocytic (normal cell size)
➢ Thalassemia ➢ The RBCs appear normal but reduced in number
➢ Hemophilia ➢ it occurs because of:
➢ Von Willebrand’s Disease - impaired production of erythrocytes
➢ Immune Thrombocytopenic Purpura - by abnormal or uncompensated loss of circulating RBC
➢ G6PD COMMON TYPES OF NORMOCHROMIC OR NORMOCYTIC ANEMIAS
DISORDERS OF THE RED BLOOD CELLS 1. ACUTE BLOOD-LOSS ANEMIA
● Occurs when a significant amount of blood is rapidly lost,.or
➢ Most RBC disorders are Anemias
● This type of anemia can be life-threatening and requires
● Pediatric Anemia is a condition where a child has a lower
immediate medical attention.cause it may lead to more
than normal number of RBC or hemoglobin levels
severe complication
➢ Disorders of the RBC includes:
● Can affect children of all ages, but infants and young
● Normochromic or Normocytic Anemias
children are particularly vulnerable due to their smaller blood
● Hypochromic Anemias
volume.
● Macrocytic (Megaloblastic) Anemias
Causes:
● Hemolytic Anemias
● Trauma
● Thalassemias
- Injuries such as accidents, falls, or blunt force trauma.
● Polycythemia
● Surgical Procedures

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- Blood loss during or after surgery. kasi diba may blood loss - Keep the child warm with blankets; place the infant in an
aftfer incubator or under a radiant heat warmer.
● Internal Bleeding ● Monitoring
- Conditions such as gastrointestinal bleeding (e.g., ulcers, - Continuously monitor vital signs.
varices), ruptured spleen, or bleeding disorders. - Assess level of consciousness and signs of hypovolemic
● Obstetric Complications shock
- Hemorrhage during childbirth. ● Education
Signs and Symptoms: anything na may kinalaman sa pagkaubos ng - Educate parents on recognizing signs of severe blood loss
dugo. so nanjan ang: and when to seek emergency care.
● Rapid heart rate (tachycardia) Treatments:
● Low blood pressure (hypotension) ● Control of Bleeding
● Pale, cool, clammy skin - Until blood is available for transfusion, a blood expander
● Dizziness or fainting such as plasma or intravenous fluid such as normal saline or
● Shortness of breath lactated Ringer’s may be given (given to expand blood
● Weakness and fatigue volume and improve blood pressure)
● Abdominal pain or swelling (if internal bleeding) - paki emphasize na NUMBER 1 treatment is addressing the
● Visible bleeding from a wound or orifice underlying cause.
Diagnostic Procedures: - replace lost blood (via Blood transfusion).
● Complete Blood Count (CBC): CBC to measure ● Medications
hemoglobin and hematocrit levels. - Administer medications as ordered to control bleeding and
● Blood Type and Crossmatch: To prepare for potential support blood pressure (e.g., vasopressors, hemostatic
blood transfusion. agents).
● Imaging Studies: SUCH AS Ultrasound, CT scan, or MRI to 2. ANEMIA OF ACUTE INFECTION
identify internal bleeding. ● also known as Anemia of inflammation or Anemia of chronic
● Physical Examination: To assess the extent of blood loss disease
and identify the source. ● common condition in children that occurs as a result of an
● Vital Signs Monitoring: acute or chronic infection
Nursing Interventions: ● when the condition is reversed, blood values will return to
● Position normal.
- Lay the child flat to provide as much circulation as possible Causes:
to the brain cells. for optimal blood flow ● Infections

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- Bacterial, viral, and fungal infections (Infections cause the Nursing Interventions:
release of inflammatory cytokines that disrupt normal iron ● Monitor VS (To detect signs of cardiovascular compromise,
metabolism and red blood cell production). such as tachycardia and hypotension, which may indicate
● Cancer severe anemia.)
- Leukemia ● Assess for signs of infection (to prevent further
- Lymphoma complications.)
- (Cancers can directly affect bone marrow function and lead ● Encourage rest and energy conservation (Reduces oxygen
to anemia by disrupting red blood cell production) demand and prevents fatigue, allowing the child’s body to
Signs and Symptoms: heal.)
● Fatigue and Weakness (Reduced hemoglobin levels ● Educate Family on Nutritional Needs (Ensuring an adequate
decrease oxygen delivery to tissues, leading to fatigue and intake of iron and vitamins supports overall health and aids
weakness.) in recovery.)
● Pallor (Anemia results in reduced red blood cell count, ● Administer Medications as Prescribed (such as iron
causing the skin and mucous membranes to appear pale) supplements
● Shortness of Breath (Lowered oxygen-carrying capacity of Treatments:
the blood makes it harder to meet the body's oxygen ● Address Underlying Cause
demands, especially during physical activity) - Effective treatment of the infection or inflammatory
● Tachycardia (The heart compensates for decreased oxygen condition can reduce anemia
delivery by increasing the heart rate to maintain adequate ● Nutritional Support
tissue perfusion) - Ensuring adequate intake of iron, vitamins, and proteins
Diagnostic Procedures: supports hemoglobin production and overall recovery.
● Complete Blood Count (CBC): Provides detailed ● Iron Supplementation
information on hemoglobin, hematocrit, and red blood cell - Although less effective during inflammation, iron
indices to confirm anemia and determine its type. supplementation can help once the inflammatory response
● Iron Studies: Measures serum iron, ferritin, and total iron- decreases.
binding capacity to assess iron stores and differentiate 3. HYPERSPLENISM
between types of anemia. ● a condition characterized by an overactive spleen (which
● Inflammatory Markers- Elevated levels indicate the results in the excessive destruction of blood cells.) blood
presence of inflammation, supporting the diagnosis of filters rapidly through the spleen. If the spleen becomes
anemia of chronic disease. enlarged, however, blood cells pass through more slowly,
● Reticulocyte Count: Assesses bone marrow response with more cells being destroyed in the process. The

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overactive spleen then leads to increased destruction of ● Peripheral Blood Smear: Examines the morphology of
RBCs which can cause anemia and may lead to blood cells to detect abnormal shapes and signs of
pancytopenia. hemolysis.
● Pancytopenia - deficiency of all cell elements of blood ● Ultrasound or CT Scan: Imaging studies help assess the
Causes: size of the spleen and identify any underlying structural
● Hemolytic Anemias abnormalities.
- Conditions like hereditary spherocytosis or sickle cell ● Bone Marrow Aspiration and Biopsy: Evaluates bone
disease increase red blood cell destruction, causing marrow function and rules out other causes of cytopenias
splenomegaly and hypersplenism ● Liver Function Tests: Assesses liver function and identifies
● Chronic Infections liver diseases that may contribute to hypersplenism.
- Infections such as malaria or tuberculosis can lead to Nursing Interventions:
chronic splenic enlargement and hypersplenism. ● Monitor VS and Blood Counts (Regular monitoring helps
● Autoimmune Disorders detect early signs of complications such as severe anemia,
- Disorders like systemic lupus erythematosus (SLE) can infection, or bleeding.)
cause immune-mediated destruction of blood cells, leading ● Assess for signs of Bleeding and Infection
to splenic hyperactivity ● Provide Nutritional Support (overall health and recovery)
Signs and Symptoms: ● Administer Medications as Prescribed (such as antibiotics)
● Anemia (Overactive spleen destroys red blood cells, leading Treatments:
to decreased hemoglobin levels and symptoms like fatigue, ● Address Underlying Cause
pallor, and weakness.) - Managing the primary disease can reduce spleen activity.
● Leukopenia ( Excessive destruction of white blood cells by ● Blood Transfusions
the spleen increases susceptibility to infections) - Used in cases of severe anemia to quickly restore
● Thrombocytopenia (Overactive spleen destroys platelets, adequate hemoglobin levels.
leading to symptoms like easy bruising, bleeding gums, and ● Splenectomy
petechiae.) - Surgical removal of the spleen
● Splenomegaly (spleen becomes enlarged due to increased
HYPOCHROMIC ANEMIAS
activity, which can cause abdominal discomfort or pain.)
Diagnostic Procedures: ➢ characterized bycharacterized by red blood cells that are paler than
● Complete Blood Count (CBC): Identifies anemia, normal (Hypochromia) due to a reduced hemoglobin content.
leukopenia, and thrombocytopenia, indicating hypersplenism. IRON DEFICIENCY ANEMIA
● Most common type of Anemia in infancy and childhood
● Due to inadequate iron to produce hemoglobin
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● Often occurs: ● Focuses on lack of iron: Ferrous Sulfate for 4-6 weeks- take
- between the ages of 6 months to 3 years (infants drinks more milk on empty stomach (after meals if gastrointestinal irritation
than eating iron-rich food) arise)- avoid calcium-rich
- Adolescence (increase iron requirements especially for girls) - avoid calcium-rich food
Causes: - inform the patient/family that iron may turn stools black
● Inadequate iron intake ● Educate importance of Iron-rich food (red meat, organ
● poor iron absorption meats)
● chronic blood loss ● Vitamin C (helps in iron absorption)
● infections or inflammation ● reinforce the need for thorough brushing of teeth (to prevent
● infants with anemic mothers staining)
Signs and symptoms
● Pale Conjunctiva/Pallor (Hallmark Sign)
● SOB
● Poor muscle tone (decreased activity)
● Heart and spleen may be enlarged
● Pica (unusual cravings)
● Soft systolic precordial murmur (heart beats faster in an
attempt to supply blood cells with more oxygen)
● Developmental delays
● Restless Legs Syndrome
● Fatigue, irritability, poor appetite
Diagnostic Procedures:
● Complete Blood Count (CBC): Identifies anemia
● Peripheral Blood Smear: to check if the blood cells appear
normal or not.
● Serum Ferritin: measures amount of stored iron (low iron
deficiency)
● Serum Iron and Total Iron-Binding Capacity (TIBC):
Reticulocyte Count: to assess bone marrow response to
MACROCYTIC (MEGALOBLASTIC) ANEMIAS
anemia
Treatment/Nursing Interventions: ➢ characterized by abnormally large
➢ caused by nutritional deficiencies
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2 NUTRITIONAL DEFICIENCIES
Diagnostic ● Complete Blood ● Complete Blood
1. ANEMIA OF FOLIC ACID DEFICIENCY
Procedures Count (CBC): Count (CBC):
2. PERNICIOUS ANEMIA
macrocytic anemia macrocytic anemia
ANEMIA OF FOLIC PERNICIOUS ● Serum Folate ● Serum Vitamin B12
ACID DEFICIENCY ANEMIA Levels: low Levels: low
● Peripheral Blood ● Peripheral Blood
Causes ● Inadequate Dietary ● VitaminB12 Smear: Smear:
intake of folate deficiency due to megaloblastic red megaloblastic red
● Malabsorption intrinsic factor blood cells blood cells
(e.g.,celiac deficiency ● Homocysteine ● Methylmalonic Acid
disease,Crohn’s ● Autoimmune Levels: elevated Levels: elevated
disease) destruction of
● Increased Demand gastric parietal Nursing Interventions ● Monitor vital signs ● Monitor vital signs
(e.g.,during rapid cells and blood counts and blood counts
growth or ● Genetic ● Assess and ● Monitor for and
pregnancy) predisposition educate on dietary manage
intake and neurological
S/Sx ● Fatigue and ● Fatigue and supplementation symptoms
weakness weakness ● Educate on the ● Educate on B12
● Pallor ● Pallor importance of supplementation
● Glossitis (inflamed, ● Glossitis and/or folate-rich foods and dietary sources
swollen tongue) sore, red tongue of vitamin B12
● Poor growth and ● Neurological
developmental symptoms(e.g., Treatments ● Oral folic acid ● Vitamin B12
delays numbness, tingling, supplements injections
● Irritability and difficulty walking) ● Dietary (intramuscular) or
behavioral changes ● Mental Status modifications high-dose oral
changes(e.g., supplements
confusion,memory ● Lifelong B12
loss) supplementation if
intrinsic factor

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increased reticulocyte count as the bone marrow

deficiency is
compensates for hemolysis.
present
● Peripheral Blood Smear: Reveals spherocytes, which are
small, round, densely stained red blood cells.
HEMOLYTIC ANEMIAS ● Osmotic Fragility Test: Measures the fragility of red blood
cells
➢ Low number of Erythrocytes
● Eosin-5'-Maleimide (EMA) Binding Test: diagnosis by
➢ RBC breaks down or die faster than normal
identifying deficiencies in red blood cell
3 DISEASE UNDER HEMOLYTIC ANEMIA
● Genetic Testing: Confirms mutations in genes responsible
1. CONGENITAL SPHEROCYTOSIS
for the condition.
● a hereditary disorder
● sphere shaped red blood cells (which is more prone to Hemolysis. Nursing Interventions:
Hemolysis means destruction or breakdown of red blood cells ● Monitor Vital Signs and Blood Counts: Regular
Causes: monitoring helps detect changes in anemia severity and
● Genetic Mutation: Defects in genes encoding red blood cell identify potential complications early.
membrane proteins such as spectrin, ankyrin, band 3, or ● Assess for Signs of Hemolysis: such as jaundice, dark
protein 4.2. urine, and splenomegaly.
● Inheritance Pattern: Typically autosomal dominant or ● Educate Family on Disease Management: Inform families
autosomal recessive about the condition, its implications, and the importance of
Signs and symptoms regular follow-up and treatment adherence.
● Anemia: Due to the premature destruction of red blood cells. ● Provide Supportive Care: This includes blood transfusions
● Jaundice: Resulting from the increased breakdown of red if necessary, to manage severe anemia.
blood cells and the subsequent rise in bilirubin levels. ● Encourage Nutritional Support: A balanced diet to support
● Splenomegaly: Enlarged spleen due to the increased overall health and manage anemia.
workload of filtering abnormal red blood cells. Treatments
● Gallstones: Occur due to chronic hemolysis and excess ● Folic Acid Supplementation: Supports red blood cell
bilirubin production. production and helps manage chronic hemolysis.
● Fatigue and Weakness: Common symptoms of anemia. ● Blood Transfusions: Administered as needed for severe
● Growth Delays: Chronic anemia may affect growth and anemia.
development in children. ● Splenectomy: considered in severe cases or after other
Diagnostic Procedure treatments have failed.
● Complete Blood Count (CBC): Indicates anemia and
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● Cholecystectomy: Removal of the gallbladder if gallstones carrying capacity of the blood.

develop. ○ Shortness of Breath: Caused by severe anemia.
2. G6PD ● Chronic Non-Spherocytic Hemolytic Anemia: Less
● Enzyme Glucose-6-Phosphate Dehydrogenase common but can occur without specific triggers.
● necessary for maintenance of RBC life; lack of the enzyme Diagnostic Procedure
results in premature destruction of RBCs or hemolysis. ● Complete Blood Count (CBC): Indicates anemia and
Causes: increased reticulocyte count.
● Genetic Mutation: Caused by mutations in the G6PD gene ● Peripheral Blood Smear: Shows bite cells and Heinz
located on the X chromosome. bodies, which are red blood cells with denatured hemoglobin.
● Inheritance Pattern: X-linked recessive, ● G6PD Enzyme Assay: Measures the activity of the G6PD
enzyme in red blood cells.
● Genetic Testing: Confirms mutations in the G6PD gene.
● Direct Antiglobulin Test (Coombs Test): Negative, which
helps differentiate G6PD deficiency from autoimmune
hemolytic anemia.
Nursing Interventions:
● Monitor Vital Signs and Blood Counts: Regular
monitoring helps detect changes and manage anemia.
● Avoid Known Triggers: Educate the family about
substances and medications that can trigger hemolysis (e.g.,
certain antibiotics, antimalarials, and foods like fava beans).
Signs and symptoms ● Provide Supportive Care: Includes hydration and blood
● Neonatal Jaundice: Often appears within the first few days transfusions if necessary during hemolytic episodes.
of life. ● Educate Family on Disease Management: Helps families
● Acute Hemolytic Anemia: Triggered by factors such as understand the condition, its implications, and the
certain medications, infections, or ingestion of fava beans importance of avoiding triggers.
(favism). Symptoms include: ● Promote Nutritional Support: Ensure a balanced diet to
○ Pallor: Pale skin due to anemia. support overall health and manage anemia.
○ Dark Urine: Resulting from the breakdown of Treatments
hemoglobin. ● Avoidance of Triggers: The primary strategy to prevent
○ Fatigue and Weakness: Due to decreased oxygen- hemolytic episodes.

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● Folic Acid Supplementation: Supports red blood cell

production.
● Blood Transfusions: Administered as needed for severe
anemia.
● Treatment of Infections: Prompt treatment of infections to
prevent hemolytic episodes.
● Hydration and Supportive Care: Important during acute
hemolytic episodes to maintain renal function and overall
health.
Understanding Triggers
● Medications to avoid:
- Certain antibiotics (e.g., sulfonamides, nitrofurantoin)
- Antimalarials (e.g., primaquine)
- Analgesics (e.g., aspirin in high doses) Causes:
● Foods to avoid: ● Genetic Mutation: A mutation in the HBB gene, which
- Fava beans encodes the beta-globin subunit of hemoglobin.
- Other legumes may also need to be avoided in some ● Inheritance Pattern: Autosomal recessive; a child must
cases. inherit two sickle cell genes (one from each parent) to have
● Other Triggers: the disease.
- Infections (e.g., viral or bacterial)
- Certain chemicals (e.g., naphthalene found in mothballs)
3. SICKLE-CELL ANEMIA
● a form of Sickle-Cell Disease
● characterized by the production of abnormal hemoglobin
(hemoglobin S, HbS)
● leading to the formation of sickle-shaped red blood cells.
● These cells can obstruct blood flow and break down
prematurely, causing various complications.

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due to trapped sickle cells, leading to a sudden drop in

hemoglobin
● Stroke: Due to blocked blood flow to the brain
Diagnostic Procedure
● Complete Blood Count (CBC): Shows anemia and
increased reticulocyte count.
● Newborn Screening: Routine screening
● Hemoglobin Electrophoresis: Identifies the presence of
HbS and differentiates between sickle cell trait and disease
● Peripheral Blood Smear: Reveals sickle-shaped red blood
cells
● Genetic Testing: Confirms mutations in the HBB gene
Nursing Interventions:
● Monitor Vital Signs and Blood Counts: Regular
monitoring helps detect changes and manage complications
● Pain Management: Administer analgesics and other pain
relief measures during vaso-occlusive crises
Signs and symptoms ● Hydration: Encourage adequate fluid intake to reduce blood
● Chronic Hemolytic Anemia: viscosity and prevent sickling
- Fatigue and weakness ● Infection Prevention: Administer prophylactic antibiotics
- Pallor and ensure vaccinations are up-to-date
- Jaundice due to the breakdown of red blood cells ● Educate Family on Disease Management: Teach families
- Delayed growth and puberty about the condition, how to recognize early signs of
● Chronic Hemolytic Anemia: complications, and the importance of regular follow-up and
- Episodes of severe pain due to blocked blood flow, often adherence to treatment plans
affecting bones, chest, and abdomen ● Provide Psychosocial Support: Address the emotional
- Swelling of hands and feet (dactylitis) in infants and social needs of the child and family
● Frequent Infections: Due to spleen dysfunction, increasing Complications to WOF:
susceptibility to bacterial infections ● Acute Chest Syndrome: Severe respiratory complications
● Acute Chest Syndrome: Severe chest pain, fever, and requiring immediate medical attention sudden onset of
difficulty breathing caused by sickling in the lungs chest pain, fever, and respiratory
● Splenic Sequestration: Rapid enlargement of the spleen
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● Stroke: Requires prompt recognition and management ➢ These conditions are most common in Mediterranean populations
● Splenic Sequestration: Sudden spleen enlargement and but also affect children of African and Asian heritage.
severe anemia, requiring emergency care 2 TYPES OF THALASSEMIAS
● Gallstones: Due to chronic hemolysis, potentially requiring
Thalassemia Minor Thalassemia Major
surgical intervention
(Heterozygous β- (Homozygous β-
Treatments
Thalassemia) Thalassemia, Cooley
● Hydroxyurea: Increases fetal hemoglobin (HbF) production,
Anemia)
reducing the frequency of Vaso-occlusive crises and acute
chest syndrome Genetic Basis Combination of Inability to produce
- Vaso-occlusive Crisis- pooling of sickled cells in defective and normal normal beta
blood vessels that causes tissue hypoxia beyond the beta hemoglobin. hemoglobin.
blockage
- Sickle-cell crisis- will arise as a sudden, severe Prevalence More common Less common
onset of sickling.
● Blood Transfusions: Used to manage severe anemia and Symptoms Mild anemia, often Severe anemia, pallor,
prevent complications like stroke asymptomatic other irritability, anorexia,
● Pain Management: Includes analgesics and other pain than pallor developmental delays,
relief measures during crises frequent nosebleeds
● Antibiotics and Vaccinations: Prevent infections due to (epistaxis), myocardial
spleen dysfunction fibrosis, diabetes
● Bone Marrow or Stem Cell Transplantation: The only mellitus
potential cure, usually reserved for severe cases with a
Age of Onset Often detected in Symptoms appear after
suitable donor
childhood or the first few months of
● Folic Acid Supplementation: Supports red blood cell
adolescence life when fetal
production
hemoglobin is replaced
● L-glutamine (Endari): An FDA-approved treatment to
by adult hemoglobin
reduce the frequency of pain crises
THALASSEMIAS X-Ray Findings Normal Osteoporotic bone
tissue, risk of fractures
➢ are autosomal recessive anemias resulting from abnormalities in
the beta chain of adult hemoglobin (hemoglobin A) Treatment None usually required Stem cell
➢ absent production of one or more of the globin
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➢ which leads to increased blood viscosity and volume.

transplantation
Causes:
(ultimate cure),
● Genetic Mutation: Most commonly associated with a
digitalis, diuretics, low-
mutation in the JAK2 gene, which leads to uncontrolled
sodium diet to prevent
production of blood cells.
heart failure; regular
Signs and symptoms
blood transfusions
● Ruddy Complexion: Reddish or purplish skin tone due to
(hypertransfusion
increased RBCs.
therapy); iron chelation
● Cyanosis: Bluish discoloration of the skin, particularly in the
therapy (deferasirox or
lips and extremities.
deferoxamine);
● Headaches and Dizziness: Due to increased blood
splenectomy if
viscosity affecting cerebral blood flow.
necessary
● Fatigue and Weakness: Resulting from impaired oxygen
Prognosis Normal life expectancy Grave without delivery to tissues.
treatment; improved ● Pruritus (Itching): Often exacerbated by warm water
with regular medical (aquagenic pruritus).
management and ● Hypertension: Increased blood volume and viscosity can
potential stem cell raise blood pressure.
transplantation ● Thrombosis: Increased risk of blood clots due to
hyperviscosity.
Note:
● Splenomegaly: Enlarged spleen due to increased RBC
Thalassemia Minor: Mild, often asymptomatic condition with normal life
destruction.
expectancy. Requires no treatment, but iron supplements should be
● Difficulty Breathing: Due to decreased oxygen delivery.
avoided.
● Epistaxis: Frequent nosebleeds due to increased blood
Thalassemia Major: Severe condition with significant symptoms and
volume and pressure.
complications affecting multiple body systems. Requires ongoing medical
management and has a grave prognosis without treatment, although
survival and quality of life can be significantly improved with proper care.
POLYCYTHEMIA VERA
➢ A rare myeloproliferative disorder where the bone marrow produces
too many RBCs.
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● Monitor Vital Signs and Blood Counts: Regular

monitoring helps detect changes and manage complications.
● Hydration: Encourage adequate fluid intake to reduce blood
viscosity.
● Educate Family on Disease Management: Provide
information on the condition, potential triggers, and the
importance of regular follow-up and adherence to treatment
plans.
● Prevent Thrombosis: Encourage mobility and, if necessary,
administer anticoagulants as prescribed.
● Supportive Care: Manage symptoms such as itching with
appropriate skin care measures and medications.
● Provide Psychosocial Support: Address the emotional
and social needs of the child and family.
Treatments
● Phlebotomy: Regular removal of blood to decrease RBC
count and blood volume, reducing blood viscosity.
● Hydroxyurea: A medication used to reduce RBC production.
Diagnostic Procedure ● Aspirin: Low-dose aspirin to reduce the risk of thrombosis.
● Complete Blood Count (CBC): Elevated RBC count, ● Interferon-alpha: Used in some cases to reduce blood cell
hemoglobin, and hematocrit levels. production.
● JAK2 Mutation Testing: Identifies the presence of the ● JAK2 Inhibitors: Emerging treatments targeting the
JAK2 V617F mutation or other related mutations. underlying genetic mutation.
● Bone Marrow Biopsy: To assess bone marrow activity and ● Management of Complications: Such as controlling
rule out other disorders. hypertension and treating any thrombotic events promptly.
● Erythropoietin Levels: Typically low in polycythemia vera
HEMOPHILIA
as the body tries to compensate for excessive RBC
production. ➢ are inherited disorders of blood coagulation.
● Oxygen Saturation: To rule out hypoxia-related secondary ➢ disorder in which the blood does not clot due to insufficient clotting
polycythemia. factors
Nursing Interventions:

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● Bruising and Petechiae: Small red or purple spots caused

by bleeding into the skin.
● Organ Dysfunction: Due to microvascular thrombosis (e.g.,
renal failure, respiratory distress, hepatic dysfunction).
● Shock: Hypotension and decreased organ perfusion.
● Thrombosis: Blood clots in major organs causing organ
ischemia and infarction.

Disseminated Intravascular Coagulation

● is a complex, acquired disorder
● characterized by systemic activation of blood coagulation
● leading to widespread formation of blood clots in small blood
vessels throughout the body
Causes:
● Infections: Severe bacterial, viral, or fungal infections (e.g.,
sepsis, meningitis).
● Trauma: Major injuries, burns, or head trauma.
● Malignancies: Certain cancers (e.g., leukemia).
● Obstetric Complications: Severe complications during
childbirth (e.g., abruptio placentae).
● Vascular Disorders: Conditions like vasculitis. Diagnostic Procedure
● Severe Inflammatory Responses: Conditions like ● Complete Blood Count (CBC): Low platelet count
pancreatitis. (thrombocytopenia)
● Liver Disease: Severe hepatic dysfunction. ● Coagulation Studies: Prolonged prothrombin time (PT),
Signs and symptoms prolonged partial thromboplastin time (PTT), low fibrinogen
● Bleeding: From multiple sites, including mucous levels.
membranes, gastrointestinal tract, urinary tract, and surgical ● D-dimer Test: Elevated levels indicating increased
wounds. fibrinolysis.
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● Fibrin Degradation Products (FDPs): Elevated levels. ● Supportive Care: Mechanical ventilation, renal replacement
● Peripheral Blood Smear: Presence of schistocytes therapy, and other supportive measures for organ
(fragmented red blood cells). dysfunction.
Nursing Interventions:
● Monitor Vital Signs and Hemodynamic Status: Frequent
assessment to detect signs of bleeding, organ dysfunction,
and shock.
● Administer Blood Products: Platelets, fresh frozen plasma,
and cryoprecipitate as prescribed to replace depleted
clotting factors.
● Ensure Adequate Oxygenation: Provide supplemental
oxygen or mechanical ventilation if necessary.
● Monitor and Manage Bleeding: Apply pressure to bleeding
sites, avoid invasive procedures when possible, and use soft
toothbrushes.
● Administer Medications: Heparin (controversial, used in
specific cases to inhibit ongoing coagulation),
antifibrinolytics if indicated.
● Support Family and Provide Education: Explain the
condition, treatments, and provide emotional support.
Treatments
● Treat Underlying Cause: Address the triggering condition
(e.g., antibiotics for infection, surgical intervention for
trauma).
● Blood Product Replacement: Transfusions to replace
platelets, fresh frozen plasma, and cryoprecipitate.
● Medications: Heparin in some cases to prevent further clot
formation, though controversial; antifibrinolytic agents if
hyperfibrinolysis is present.

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