Nursing Management

of Children with
Blood Disorders

Dr. Nuhad Al Doori

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Blood

 Blood is the fluid of life

 Blood is composed of:

 Plasma
 RBC
 WBC
 Platelets

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Plasma

 Plasma consists of:

 90% water.

 10 % solutes: albumin, electrolytes and proteins.

 Proteins consist of clotting factors, globulins,

circulating antibodies and fibrinogen.

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Red Blood Cells

 RBC’s travel through the body delivering

oxygen and removing waste.
 RBC’s are red because they contain a protein
chemical called hemoglobin which is bright
red in color.
 Hemoglobin contains iron, making it an
excellent vehicle for transporting oxygen and
carbon dioxide.

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RBC’s

 Average life cycle is

120 days.

 The bones are

continually
producing new cells.

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White Blood Cells

 The battling blood cells.

 The white blood cells are continually on the

look out for signs of disease.

 When a germ appears the WBC will:

 Produce protective antibodies.
 Surround it and devour the bacteria.

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WBC’s

 WBC life span is from a

few days to a few
weeks.
 WBC’s will increase
when fighting infection.

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Platelets

 Platelets are irregularly-

shaped, colorless
bodies that are present
in blood.

 Their sticky surface lets

them form clots to stop
bleeding.

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Blood Values

 CBC with differential and platelet count.

 Hgb:
 Normal levels are 11 to 16 g / dl
 Panic levels are:
 Less than 5 g / dl
 More than 20 g / dl

 Normal hematocrit levels are 35 to 44%.

 Panic levels:
 Hmct less than 15 %
 Hmct greater than 60%

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Hemoglobin and Hematocrit

 Can be used as a simple blood test to screen

for anemia.
 The CBC with differential would be used to
help diagnose a specific disorder.
 A bone marrow aspiration would be the most
conclusive in determining cause of anemia –
aplastic / leukemia.

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Normal Pediatric
Hematology Values
 Birth
- Hbg - mean 16.8 (13.7-20.1)
- HCT –mean 55% (45-65)
- Wbc -mean 18,000( 9000-30,000

 3mos
- Hbg - mean 12.0
- HCT –mean 36%
- Wbc -mean 12,000

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Normal Pediatric
Hematology Values
 6mos - 6 years
- Hbg - mean - 12.0
- HCT –mean - 37%
- Wbc -mean - 10,000

 7-12 yrs
- Hbg - mean - 13.0
- HCT –mean - 38%
- Wbc -mean - 8,000

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Iron Deficiency Anemia

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Iron Deficiency Anemia
 A microcytic, hypochromic anemia
 Caused by inadequate supply of dietary iron
 Decreased ferritin, marrow deplete of stainable Fe2+
 Most common cause of childhood anemia worldwide
 Full-term infants exhaust Fe 2+ reserves by 5-6 months age
 Preterm infants have lower reserves – exhaust by 2-3 months of
age. Premature infants are at risk because of their reduced fetal
iron supply.

Results of iron deficiency anemia:

 1- Decreased hemoglobin formation
 2- Red blood cells with less color & smaller in size (hypochromic
microcytic)
 3- Decreased plasma iron
 4- Decreased body stores
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Iron Deficiency Anemia

 Commonly presents:
- Between 6 months – 3 years
- Ages 11-17 years: periods of rapid growth and
increased Fe 2+ requirements
- Adolescents at risk - poor diets, menstrual losses
(Loss of blood)
- Inadequate iron absorption (Low iron intake)

- Can cause irreversible effects on development if

untreated

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Iron Deficiency Anemia

 Etiology
- Vegan diet (no meat)
- Secondary to poor intake of iron-rich foods ( typically
in bottle-fed infants (6-24 months) receiving large
volumes of cow’s milk)

- Cow’s milk allergy: occult bleeding & protein-losing

enteropathy secondary to inflammation enteropathy;
secondary to inflammatory bowel disease

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IDA: Abnormal Laboratory Values

 Hemoglobin levels less than 8 g/dL

 Decreased levels of Serum Iron or Total Iron

Binding or Serum Ferritin

 Microcytic and hypochromic red blood cells

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IDA: Symptoms

 Associated with low oxygenation of tissue:

 Pallor
 Fatigue
 Blue sclera
 Spoon nails
 Glossitis, stomatitis
 Shortness or breath
 Irritability, apathy, poor concentration
 Intolerance of physical work / exercise

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Iron Deficiency Anemia

 Management
 Packed RBC transfusion is given 2-3 cc/kg
 02 is given when tissue hypoxia is severe
 Oral iron therapy – ferrous sulfate 3mg/kg/day for 3 months
 For I.M injection Use 5cm long needle, use the zigzag method to
ensure no seepage of the medicine into the subcutaneous tissue
& irritating it to ensure quick absorption, also the area should not
be massaged for the same reason.
 Change site of injection & encourage the child to walk to increase
the absorption of the Iron.
 I.V Iron transfusion is given for serious infections. Sensitivity test
is done prior to giving Iron injection.
 Close follow up to monitor dietary intake, Hbg, ferritn for response
to RX

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IDA: Management

 Iron supplementation
 Given in a.m. on an empty stomach
 To avoid staining of teeth, give using a syringe,
dropper or straw
 Instruct caretaker that child may have dark-
colored stools

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IDA: Management

 Nutritional counseling: encourage diverse, balanced

diet - iron rich foods /formula
 Infants younger than 12 months should be on
formula until around 12 months of age
 Infants 12 months or older
 Decrease intake of milk
 Introduce solid foods
 Children: iron fortified cereals, foods, meat, green
leafy vegetables
 Teenagers: reduce junk food

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Complications: IDA
 - cognitive Impairment.
 - Poor peripheral perfusion
 - Skin becomes moist and cool
 - Low blood pressure
 - Increased heart rate.

Body response to acute blood loss:

 Plasma is replaced within 1-3 days after acute
hemorrhage showing low concentration of
RBC
 RBCs are replaced with in 3- 4 weeks

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Beta-Thalassemia

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Thalassemia

 Autosomal recessive inheritance

 Genetic defect on chromosome 11
 Affects Asian and Mediterranean people

Thalassemia is classified according to:

1. The hemoglobin chain affected.
2. The amount of the globin chain that is
synthesized.
 If α- chains are affected  α Thalassemia (Chinese,
Thai, and African)
 If β- chains are affected  β Thalassemia (Greeks,
Italians & Syrians)

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Beta - Thalassemia

 Defect in the beta globin gene - ß Hbg

 Beta globin chains are required for
synthesis of hemoglobin A
 Leading to ineffective production and
hemolysis of RBC’s

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Beta-Thalassemia
Mode of transmission
Thalassemia is an autosomal-recessive disorder, this means: Both parents must be
affected as (carriers) to produce a child with β Thalassemia Major.

Heterozygous Parent Aa

Gametes
A a

Heterozygous AA Aa
Parent Aa A Normal Carrier

Aa aa
a Carrier Affected

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Beta-Thalassemia
Pathophysiology and child clinical manifestations

1. Normally the postnatal hemoglobin A (Hgb A) is

composed of: Two α and two β polypeptide chains.

2. The prenatal hemoglobin F (fetal Hgb F) is composed

of: Two α and two γ polypeptide chains, the
characteristic of this Hgb F is its great affinity to O2
even under low PO2 which is the condition of the PO2
during intrauterine life and is no longer needed after
birth because the PO2 level is higher so hemoglobin
must change to the A (Adult Hgb A)

3. In βThalassemia there is partial or complete deficiency

in the synthesis of the β chain of Hgb molecule.
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Beta-Thalassemia
4. Consequently a compensatory increase in the
synthesis of the α-chain and the γ-chain remains
activated resulting in defective Hb formation.
5. This unbalanced polypeptide unit is very unstable;
when it disintegrates, it damages the RBCs
causing severe anemia.
6. To compensate for the hemolytic process there is
excessive production of RBC (Immature and
deformed & life span is shortened 20 – 30 days)
but with blood transfusion bone marrow will be
suppressed.

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Beta-Thalassemia
Excess iron from packed RBC transfusion and
rapid destruction of the defective cells is
stored in various organs leading to
hemosiderosis.
The clinical manifestations are attributable to:
1. Defective synthesis of hemoglobin A.
2. Structurally impaired RBCs.
3. Shortened life span of the RBCs

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RBC Characteristics

 Microcytosis = small in
size

 Hypochromia = decrease
hemoglobin

 Poikilocytosis = abnormal
shape

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Beta-Thalassemia
 Clinical Presentation:
- chronic hypoxia: headache, irritability, jaundice
- delayed growth /development( hypogonadal dwarfism)
- Hepatosplenomegaly
- Progressive Anemia
- Expanded bone marrow cavity
- Pathological fractures common
- Precordial bone pain
- Decreased exercise tolerance listlessness and anorexia.
- Epistaxis
- Hyperuricemia and gout from rapid cellular catabolism are also
seen
- Iron overload from supportive treatment; blood transfusion.

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Beta-Thalassemia

 Clinical Presentation:
 increased hematopoiesis leads to bone marrow
expansion, impaired growth & thinning of cortical bone
 increased red cell destruction leads to increased iron
absorption, splenomegaly & hepatomegaly (then
fibrosis, cirrhosis)
 Heart: arrhythmia, myocarditis, congestive heart failure
 Pancrease: Beta cells are destroyed leading to
diabetes
 Pituitary: growth retardation, hypogonadotropic
hypogonadism
 Parathyroid: hypocalcemia, osteoporosis

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Beta-Thalassemia

 Bone changes in older children:

 Enlarged head
 Prominent frontal and parietal bosses
 Prominent molar eminences
 Flat and depressed bridge of the nose
 Enlarged maxilla Protrusion of the upper lip
and the upper central incisors and eventual
malocclusion
 Mongoloid appearance of eyes

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Beta-Thalassemia
Complications:
 Hemosiderosis: Refers to excessive iron storage in
various tissues of the body especially spleen, liver
lymph glands, heart and pancreas but without
associated tissue injury.

 Hemochromatosis: Refers to excessive iron storage with

resultant cellular damage, the mechanism for tissue destruction
resulting from iron storage is not known. Chronic hypoxia is
believed to be an important contributing factor. Excess
Hemosiderosis iron containing pigment from the breakdown of
hemoglobin- results from low Hb synthesis and high hemolysis
of transfused RBCs.

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Beta-Thalassemia

 Low production of Hb → in high supply of

available iron – and the body responses to anemia
by high absorption of iron from diet.
 Retarded growth and delayed sexual maturity may
be due to pituitary failure, which are caused by
hemochromatosis.
 It is possible that the endocrine glands are
extremely sensitive to iron toxicity.
 It is reported that diabetes a frequent
consequence

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Beta-Thalassemia

 Diagnosis
 RBCs microscopically are:
 Microcytic Hypochromic
 Low Hb and PCV is severe anemia.
- CBC, smear, Hbg electrophoresis

 Hemoglobin electrophoresis: This is the diagnostic test for

Thalassemia.
 This will analyze the quantity and specific hemoglobin variants
found in blood in β Thalassemia Hemoglobin (Hgb F) and
hemoglobin A2 (Hgb A2; a type of normal adult hemoglobin) are
elevated Because neither depends on β-chain polypeptides for
synthesis
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Beta-Thalassemia
Treatment:
- Blood transfusions as needed (to prevent tissue hypoxia by
maintaining sufficient levels of Hb above 10 g/dl over 3 weeks)
- Iron chelation to prevent overload
- Bone marrow transplant
- Supportive treatment

 Liver fibrosis is prevented if Desferal is started as early as 2-

4 years of age, but the growth in height and the bony changes
are not totally reduced.

 In some sever splenomegally cases splenectomy may be

necessary to decrease abdominal pressure and to increase
the life span of the supplemented RBCs, but these children
develop sever tendency to infection. So prophylactic
antibiotics with close medical supervision Is done.

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Beta-Thalassemia
Prognosis:
With blood transfusion and chelating therapy life span is
increased and they can lead an early normal life.

The most common causes of death are:

 Heart disease
 Infection
 Liver disease
 Malignancy
These are secondary to hemochromatosis

Bone marrow transplant is a primary treatment it has 59% -95% cures

In Jordan there is about 1000-1100 case of major thalassemia.
3-4% of the population are carriers of the trait
The cost of treatment /case is 5000 JD/year
The bone marrow transplant cost is 30000-40000JD

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Sickle Cell Anemia

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Sickle Cell Anemia

 Autosomal recessive disorder

 Defect in hemoglobin molecule
 Cells become sickle shaped and rigid
 Lose ability to adapt shape to surroundings.
 Sickling may be triggered by fever and
emotional or physical stress

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Pathophysiology

 When exposed to diminished levels of

oxygen, the hemoglobin in the RBC develops
a sickle or crescent shape; the cells are rigid
and obstruct capillary blood flow, leading to
congestion and tissue hypoxia; clinically, this
hypoxia causes additional sickling and
extensive infarctions.

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Crescent Shaped Cells

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Body Systems Affected by SS

 Brain: CVA – paralysis - death

 Eyes: retinopathy – blindness
 Lungs: pneumonia
 Abdomen: pain, hepatomegaly, splenomegaly
(medical emergency due to possible rupture
 Skeletal: joint pain, bone pain – osteomyelitis
 Skin: chronic ulcers – poor wound healing

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Vaso-occlusive Crisis

Stasis of blood with clumping of cell in the

microcirculation, ischemia, and infarction
 Most common type of crisis; painful

 Signs include fever, pain, tissue engorgement

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Splenic Sequestration

 Life-threatening / death within hours

 Pooling of blood in the spleen
 Signs include profound anemia, hypovolemia,
and shock
 Abdominal distention, pallor, dyspnea,
tachycardia, and hypotension

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Aplastic Crisis

 Diminished production and increased

destruction of red blood cells
 Triggered by viral infection or depletion of
folic acid
 Signs include profound anemia, pallor

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Nursing Diagnoses

 Altered tissue perfusion

 Pain
 Risk for infection
 Knowledge deficit
regarding disease
process

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Nursing Management – Hospital

 Increase tissue perfusion

 Oxygen
 Blood transfusion if ordered
 Bed rest
 Pain management
 Hydration
 IV fluids as ordered
 Oral intake of fluids

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Nursing Management

• Adequate nutrition
• Emotional Support
• Discharge instructions
• Information about disease management
• Daily folic acid
• Control of triggers
• Prophylactic antibiotics
• Immunizations / Pneumococcal

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Patient Education

 Necessity of following plan of care

 Signs and symptoms of impending crisis.
 Signs and symptoms of infection
 Preventing hypoxia from physical and
emotional stress
 Proving adequate rest

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*Hemophilia*

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Bleeding Disorders*

 Three types of Hemophilia: males only

 Type A most common – factor VIII deficiency
 Type B - lack of factor IX (Christmas Disease)
 Type C – lack of factor XI

Von Willebrand Disease – 1% of population – men

or women – prolonged bleeding time

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Hemophilia Type A*

 Hemophilia type A is the deficiency of clotting

factor VIII.
 A serious blood disorder
 Affects 1 in 10,000 males in the US
 Autoimmune disorder with lowered level of clotting
factor
 All races and socio economic groups affected
equally

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Hemophilia*

 Hemophilia is a sex-linked hereditary

bleeding disorder
 Transmitted on the X chromosome
 Female is the carrier
 Women do not suffer from the disease itself

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Goals of Care*

 Goals of care:
 Provide factor VIII (IX) to aid blood in clotting.
 To decrease transmission of infectious agents in
blood products; hepatitis & AIDS.
 Future: gene therapy to increase production of
clotting factor.

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Symptoms*

 May be mild, moderate or severe

 Bleeding into joint spaces, hemarthrosis
 Most dangerous bleed would be intracranial.
 Circumcision may produce prolonged
bleeding.
 As child matures and becomes more active
the incidence of bleeding due to trauma
increases

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Diagnosis*

 Presenting symptoms
 Prolonged activated
aPTT and decreased
levels of factor VIII or
IX.
 Genetic testing to
identify carriers

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Treatment*

 Products used to treat hemophilia are:

 Fresh frozen plasma and cryoprecipitate which
are from single blood donors and require special
freezing.
 Second generation of factor VIII are made with
animal or human proteins.

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Nursing Diagnoses*

 Risk for injury

 Pain with bleed especially into a joint
 Impaired physical mobility
 Knowledge deficit regarding disease and
management of disease

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Nursing interventions*

 No rectal temps.
 Replace the factor as ordered by physician.
 Manage pain utilizing analgesics as ordered.
 Maintaining joint integrity during acute phase:
immobilization, elevation, ice.
 Physical therapy to prevent flexion contraction and
to strengthen muscles and joints.
 Provide opportunities for normal growth and
development.

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Teaching*

 Avoid aspirin which prolongs bleeding time in

people with normal levels of factor VIII.
 A fresh bleeding episode can start if the clot
becomes dislodged.
 Natural reactions in the body cause the clot
that is no longer needed to “break down. This
process occurs 5 days after the initial clot is
formed.

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Family Education*

 Medic-Alert bracelet
 Injury prevention appropriate for age
 Signs and symptoms of internal bleeding or
hemarthrosis
 Dental checkups
 Medication administration

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Long Term Complications*

 20% develop neutralizing antibodies that

make replacement products less effective.
 Gene therapy providing continuous
production of the deficient clotting factor
could be the next major advance in
hemophilia treatment.

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Thanks for listening

Have a great Day 

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