Nursing Care of Children with

Hematologic Alterations
By Hidayatullah,
MSc.N, BSN & DCH
Anemias.
A condition in which the number of RBCs and/or the
hemoglobin concentration is reduced below normal:
0-14 days - Hb<145 g/L
15-28 days - Hb<120 g/L
1mo-1yr - Hb<110 g/L
Anemias.
The basic cause of anemia is either
(1) an increased loss or destruction of red blood cells
(2) an impaired or decreased rate of production.
An etiologic classification is based on the various
conditions that can lead to either of these results.
 Classification of anemias:
 Decreased Production of RBCs:
Iron Deficiency Anemia
Aplastic Anemia
 Increased Destruction of RBCs:
Hemolytic Anemia
Sickle Cell Anemia
Thalassemia
 Blood loss:
Iron Deficiency Anemia
Hemorrhagic Anemia
 Severety:
Mild: Hb 110-90 g/L
Moderate: Hb 89-70 g/L
Severe: Hb < 70 g/L
 Classification of anemias:
Morphologic (describe the size of RBCs):
(1) normocytic,
(2) microcytic,
(3) macrocytic.
According to the amount of hemoglobin in
the cell (describe the color and Hb content
of the cells):
(1) normochromic
(2) microchromic.
 Anemia. Assessment
Take health history:
Careful diet history to identify any
deficiencies,
Evidence of pica – eating clay, ice, paste.
Observe for manifestations of anemia:
Muscle weakness
Easy fatigability:
frequent resting,
shortness of breath,
poor sucking (infants)
 Manifestations of anemia
Pale skin, mucous membranes, lips, nail beds, and
conjunctiva
Waxy pallor seen in severe anemia
Capillary refill – no change color
Rapid, pounding heart beat
CNS manifestations:
Headache
Dizziness
Light-headedness
Irritability
Slowed thought processes, decreases attention span
Apathy
Depression.
 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
 Iron Deficiency Anemia
 Causes

- inadequate supply of iron

- impaired absorption
- blood loss
- excessive demands for iron req’d for
growth
- inability to form Hgb
Iron Deficiency Anemia
 Signs and Symptoms: due to tissue hypoxia > lack of
energy, easy fatigability, pallor
 Diagnosis: CBC with diff, red cell indices (MCV,

MCH, MCHC), iron studies, physical exam

 Medical Treatment: supplement with ferrous sulfate

(dosages vary with age), dietary counseling

Iron Deficiency Anemia
 Nursing Assessment and Interventions:
- educate parents about nutrition
- explain laboratory testing
- teach parents proper administration
of iron preparations, caution about
high toxicity of iron
Severe anemia
Impaired healing and loss of skin elasticity
Thinning and early greying of the hair
Abdominal pain, nausea, vomiting, anorexia
Low-grade fever
 Aplastic anemia
is a condition wherein injury to or abnormal
expression of the stem cells in the bone marrow
results in the production of inadequate numbers
of erythrocytes, leukocytes, and platelets.
Can be hereditary or acquired.
The hereditary form of aplastic anemia,
Fanconi's anemia (FA), is a rare autosomal
recessive disorder that develops early in life and
is accompanied by multiple congenital
abnormalities
 Aplastic anemia
The age of onset varies.
Typically, the first sign noted is increased
bruising caused by the decreased number of
platelets.
Vulnerability to infection because of the
decreased number of WBCs.
When the RBCs have decreased in number, the
signs and symptoms of anemia begin to
manifest:
pallor, weakness, and difficulty breathing
Impaired growth and development
Criteria for severe aplastic anemia
Severe Aplastic Anemia
Neutrophils < 2 × 109 cells/L
Platelets < 50 × 109 cells/L
Reticulocytes < 0.5 %
Bone Marrow Cellularity < 20 %
Nursing Care
1. Minimize physical exertion and emotional stress:
 Anticipate and assist in those activities of daily
living that may be beyond child’s tolerance
 Provide play activities that promote rest and
quiet but prevent boredom and withdrawal
 Encourage parents to remain with child, etc.
2. Increase oxygen to tissues:
 Position for optimim air exchange
 Administer supplemental oxygen if needed
3. Help replace blood elements:
 Administer blood, packed cells, platelets as
prescribed
4. Promote adequate intake of iron-rich foods:
 Encourage exclusive breastfeeding of infants for 4-6 months after
birth.
 Diet rich with food sources of iron: meat, liver, fish, egg yolks,
green leafy vegetables, nuts, whole grains, iron-fortified infant
cereal and dry cereal
 Feed milk as supplemental food in infant’s diet after solids are begun
 Administer iron preparations as prescribed:
 Give between meals
 Administer with fruit juice or multivitamin preparation
 Do not give with milk or antacids
 Give fluid preparations with dropper, syringe, or straw to avoid
contact with teeth
 If the child is getting enough iron, stools should be a tarry green
color.
 Iron Supplements
Although oral iron supplementation is generally
perceived as a safe treatment alternative, an
overdose can be lethal for children.
Initial signs and symptoms of overdose:
vomiting,
abdominal pain,
bloody diarrhea.
These manifestations are typically followed by
shock, lethargy, and dyspnea. Often the child will
appear to improve before the onset of severe
metabolic acidosis, coma, and death.
Urgent treatment is essential and includes
flushing out unabsorbed pills, administration of
Desferal, and supportive therapy
 Blood Transfusion
Nursing responsibilities
Positively identify donor and recipient blood types and
groups before transfusion is begun; verify with one
other nurse or physician
Transfuse blood slowly for first 15-20 min; remain with
patient
In event of signs or symptoms, stop transfusion
immediately, maintain patent IV Line, notify physician
Insert urinary catheter to monitor hourly outputs
Monitor for evidence of shock
Save donor blood to re-crossmatch with patient’s blood
 BLOOD TRANSFUSION COMPLICATIONS
Hemolytic reactions
Febrile reactions
Allergic reactions
Circulatory overload
too rapid transfusion or excessive quantity of blood
transfused
Air emboli
May occur when blood is transfused under pressure
Hypothermia:
Allow blood to warm at room temperature (1hr)
Hyperkalemia
In massive transfusion or patient with renal problems
 SICKLE CELL ANEMIA
A hereditary disorder in which normal adult

hemoglobin (HbA) is partly or completely replaced by

an abnormal hemoglobin (HbS) causing distortion and
rigidity of red blood cells under condition of reduced
oxygen tension.
Sickle cell anemia is part of a group of diseases called

hemoglobinopathies.
 SICKLE CELL ANEMIA
Sickle cell anemia is an autosomal-recessive
disorder. The inheritance is described as
intermediate because the gene is partially
expressed in the heterozygous state and
completely expressed in the homozygous state.
Sickle cell anemia is found primarily in the
black race, although infrequently it affects
whites.
 SICKLE CELL ANEMIA –
Manifestations:
 Take health history, especially regarding any evidence of sickling
crisis and history of the disease in family members.
 General:
Growth retardation
Chronic anemia
Delayed sexual maturation
Marked susceptibility to sepsis
 Other signs and symptoms.
weakness; anorexia; joint, back, and abdominal pain; fever;
and vomiting.
Chronic leg ulcers are common in adolescents and adults and
are thought to be the result of thrombosis and decreased
peripheral circulation.
SICKLE CELL ANEMIA –
Vaso-occlusive (sickling) crisis
 Pain in area(s) of involvement
 Signs of ischemia:
 Extremities: painful swelling (“hand-foot” syndrome), painful joints
 Abdomen: severe pain resembling acute surgical condition
 Cerebrum: stroke, visual disturbances
 Chest: pseudopneumonia, propracted episodes of pulmonary disease
 Liver: obstructive jaundice, hepatic coma
 Kidney: hematuria
Sequestration Crisis
Pooling of large amounts of blood:
Hepatomegaly
Splenomegaly
Circulatory collapse
 Diagnostic evaluation
For screening purposes:
the Sickledex is commonly used. If the test is
positive, hemoglobin electrophoresis is
necessary to distinguish between those
children with the trait and those with the
disease.
Sickling test (sickle cell slide preparation):
place a drop of blood on a slide and cover it with
a sealed cover slip to produce deoxygenation.
Eventually sickling of the red blood cell occurs.
Hemoglobin electrophoresis
("fingerprinting"). This test is accurate, rapid,
and specific.
 Nursing management

Inc tissue oxygenation and prevent sickling:

Family teach.: avoid strenuous physical
exertion, emotional stress, O2 envir
Promote hydration
Prevent infection
↑ tissue oxygenation: adm O2, promote
circulation through passive range of motion
exercises
Relieve pain
 Sickle Cell Anemia
Causes: genetic transmission, 2 parents with
the trait have 25% chance of having child
with SCD, found primarily in Blacks,
Hgb A is partly or completely replaced by

Hgb S
With dehydration,acidosis, hypoxia, and

temp elevations, Hgb S “sickles”

 Sickle Cell Anemia
Pathophysiology:
- vaso-occlusion from sickled RBCs
- increased RBC destruction
- splenic congestion and enlargement
- hepatomegaly, liver failure
- renal ischemia, hematuria
- cardiomegaly, heart failure, stroke
 Sickle Cell Anemia
Signs/Symptoms:
Exercise intolerance
Anorexia
Gallstones
Chronic leg ulcers
Growth retardation
 Sickle Cell Anemia
Medical management
 Supportive/symptomatic tx of crises
- bed rest
- hydration
- electrolyte replacement
- analgesics for pain
- blood replacement
- antibiotics
- oxygen therapy
 Sickle Cell Anemia
Nursing care:
Minimize tissue deoxygenation
Promote hydration
Minimize crises
Pain management
Administering blood transfusions
Encourage screening and genetic counseling
Parent education
 Hemophilia
 Factor 8 or factor 9 deficiency
prolonged bleeding any where in the body!
 Cause: X-linked recessive disorder, defects in platelets
and clotting factors
 Diagnosis: history of bleeding episodes, evidence of x-

linked inheritence,
 Medical Management: Factor VIII concentrate,

DDAVP (vasopressin)
 Hemophilia
Nursing care:
 Prevent bleeding

 Recognize and control bleeding (RICE)

- Rest
- Ice
- Compression
- Elevation
 Prevent crippling effects of bleeding

 Client education

 Avoid competitive sport

 Idiopathic Thrombocytopenic Purpura
 Causes: acquired hemorrhagic disorder of unknown
origin, probably an autoimmune response to
disease-related antigens
 Diagnosis: platelet count < 20,000, abnl

bleeding time and clot retraction

 Signs and Symptoms: petechiae, bruising, bleeding

from mucous membranes, prolonged bleeding from

abrasions
 Medical management: supportive, steroids, Anti-D

antibody, splenectomy
 Idiopathic Thrombocytic Puerpera
Nursing Considerations:
Client/Parent teaching
No contact sports
No aspirin
Prevent infection
 Blood Transfusion
Complications:
 Hemolytic reactions
- chills, shaking, fever
- dyspnea
- flank pain
- progressive signs of shock
 Febrile reactions
 Allergic reactions
- urticaria, flushing
- wheezing
 Circulatory overload
 Blood Transfusions
Nursing Care
 Take VS BEFORE administering blood
 Check ID of recipient with donor’s blood type
 Administer 50 mL or 1/5 volume SLOWLY – STAY WITH
THE CHILD
 Administer with NS on piggyback set-up
 Use appropriate filter
 Use within 30 mins – infuse within 4 hrs
 If reaction suspected: Stop the transfusion, maintain patent
IV line with NS, take VS, notify practitioner
 B-THALASSEMIA

Group of inherited Autosomal

recessive blood disorder
disorder of Hb% synthesis,
characterized by anemia ,
which is mild and sever
 Types
Thalassemia minor ( mild anemia)
Thalassemia intermediate (severe anemia)
Thalassemia major (requiring transfusion)
SIGN AND SYMPTOMS:
Severe anemia
Headache
Weakness
Hypo chromic
Folic acid deficiency
Bone deformities
 microcytic anemia
Cont…
Frequent epistaxis
Jaundice (palor)
Bone pain
Darkening of the skin
COMPLICATION :
 heart failure
Splenomegally
failure of thrive
liver cirrhosis
Cont…
CCF
choloeithiasis
Death of the patient.

Lab Investigation:
Hgb electrophoresis
CBC.
TREATMENT: iron chelating agent with vitamin C
Folic acid
Maintaining Hb level
Bone marrow transplantation.
Blood transfusion.
Nursing care
Observe for transfusion reaction.
Emotional support .
Genetic counseling .
Inform about disease complication.