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Blood Diseases
General Objective:
Students will be able to describe the most common hematologic dysfunction
among children and their nursing management by the end of this unit.
Specific Objective:
The student will be able to:
1. Recognize with the common types of anemia.
2. Apply nursing process for child with anemia (iron deficiency anemia).
3. Recognize with the common defects in hemoglobin formation such as sickle
cell anemia and B- Thalassemia.
4. Apply nursing process for sickle cell anemia and B- Thalassemia.
5. Recognize with the common defects in hemostasis such as hemophilia and
idiopathic thrombocytopenic purpura.
6. Recognize with the common type of neoplastic disorders such as leukemia
and Hodgkin’s disease.
7. Apply nursing process for leukemic child.
1
Anemias
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:
1. 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).
2. 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:
1. Defect in cell membrane: Hereditary erythrocytosis.
2. Defect in cell enzyme: G6PD deficiency.
3. Defect in cell hemoglobin: Sickle cell anemia, Thalassemia.
 Extracorpuscular causes:
1. Immune: blood transfusion.
2. Non- immune: - burns.
- Snake venoms.

3. 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).
2
N.B.: The morphologic classification provides an orderly method for ruling out
certain diagnoses when establishing a cause for a particular anemia. However, the
etiologic approach provides direction for planning nursing care.
Pathophysiology of Anemias:
The basic physiologic defect caused by anemia is a decrease in the oxygencarrying capacity of blood and consequently a reduction in the amount of oxygen
available to the cells. When the anemia has developed slowly, the child usually
adapts to the declining hemoglobin level, and most children seem to have a
remarkable ability to function quite well despite low levels of hemoglobin.
Assessment of Anemia:
The clinical manifestations of anemia are mostly attributable by hypoxia of the
tissue regardless of its cause.
General Manifestation:
I.



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:
II.








Headache.
Dizziness.
Night headache.
Irritability.
Slowed thought processes.
Decreased attention span.
Apathy.
Depression.
Shock [blood loss anemia]:
III.




Poor peripheral perfusion.
Skin is moist and cool.
Low blood pressure.
Increased heart rate.
Nursing Management of Anemias:
1.
2.
3.
4.
5.
It depends on the type and cause of anemia which include:
Complete nursing assessment (nursing history and physical examination).
Preparation for laboratory studies.
Reduction for the need of oxygen.
Administration of oxygen therapy.
Administration of transfusion therapy.
 Preventing adverse reaction.
 Managing adverse reaction.
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6. Observation for manifestation of infection and complications.
7. Education for parents and child.
Anemia Caused by Nutritional Deficiency
Iron Deficiency Anemia
It is caused by lack of adequate of supply of dietary iron; it is mostly prevailed
from 6-24 months of age.
Etiology:
1. Inadequate supply of iron:
a- Deficient dietary intake:



Rapid growth rate.
Excessive milk intake, delayed addition of solid foods.
Poor general eating habits.
b- Inadequate iron stores at birth:



Low birth weight, premature, multiple births.
Severe iron deficiency in mother (hemoglobin level below 9 g/dl).
Fetal blood loss at or before delivery.
2. Impaired absorption:
a- Presence of iron inhibitors.


Phytates, phosphates, or oxalates.
Gastric alkalinity.
b- Malabsorptive disorders.
c- Chronic diarrhea.
3. Blood loss:
ab-
Acute or chronic hemorrhage.
Parasitic infestation.
4. Excessive demands for iron:
abc-
Prematurity.
Adolescence.
Pregnancy.
5. Inability to form hemoglobin:
ab-
Lack of vitamin B12 (pernicious anemia).
Folic acid deficiency.
Pathophysiology:
Iron is required for the production of hemoglobin. One molecule consists of
proteins (globin) combined with four molecules of a pigmented compound
(heme). Each molecule of heme contains one atom of iron. When iron stores are
deficient; the production of hemoglobin is reduced. Consequently, the main effect of
iron deficiency is decreased hemoglobin and reduced oxygen-carrying capacity of the
blood.
4
Assessment:
The most significant clinical manifestations of iron deficiency anemia are:
 Porcelain- like or waxy pallor.
 When the hemoglobin level is below 5.0- 6.0 mg/ dl the infant or child
becomes irritable, anorexic and possibly constipated.
 Cardiac dilatation and Tachycardia develop.
 Systolic murmurs may be present.
 The spleen may be enlarged.
 Growth and motor development may be slowed.
 Cardiac failure may occur.
 Intellectual and neurologic functioning, specially the short attention
span, level of learning and degree of alterness may be affected.
Diagnostic Evaluation:
 Through dietary history.
 Complete blood picture.
 Stool examination for parasitic infestation and occult blood.
 In addition to the complete blood count which shows indirectly the level
of iron deficiency on the RBCs. Other tests which measure the amount of
circulating iron are:
1. Serum iron concentration (SIC): Measure the amount of
circulatory iron which is 70 ug / dl in infants and slightly higher in
older children. They are highest in the morning.
3. Total iron binding capacity (TIBC): Measure the amount of transferring,
or iron binding globulin, which is necessary for the transport of iron in
the blood stream. The (TIBC) is elevated above 350 ug / dl (6 months to
2 years) or 450 ug/ dl (children older than 2 years). This elevated
capacity represents the body’s compensatory mechanism to absorb
more oxygen during the states of deficiency.
The combination of a reduced (SIC) and elevated (TIBC) is a diagnostic
in hypochromic, microcytic anemia caused by inadequate intake and
absorption of iron.
Treatment:
 Prevention is the primary goal and is achieved through optimum
nutrition and appropriate iron supplementation.
 Increase the amount of supplemental oral iron the infant or child
receives which done through dietary counseling of parents and
administration of oral iron supplements.
 Parenteral iron therapy is indicated in rare cases.
 Blood transfusion in case of hemoglobin less than 4 gm/dl.
 Treatment of etiologic factor e.g. corrects diet and treat ancylostoma.
Prognosis:
It is very good, however inadequate response can be achieved if prescribed
medication is not given correctly, or poorly absorbed, or if there is unrecognized
continuing blood loss.
5
Prevention:
An important nursing goal is parent education about that:
 Formulas for full-term infants should contain at least the lower level of
iron found in breast milk. Infant should receive 1mg/kg/24 hours of iron.
 Formulas for infants at risk for iron deficiency, such as low birth weight
newborns should be supplemented with iron 2-3 mg/kg/24hours of iron.
 The dietary intake of iron can be increased for formula feed-infants by
given iron fortified commercial formula.
 Breast fed infants should supplement with iron-fortified cereal or iron
drops at about 4-6 months of age.
 When solid foods are introduced nurse should instruct parents; that
milk consumption be limited to about 500ml /day.
Nursing Consideration:
 Since interviewing parents and child is very important. The nurse should
take care of the following points:
1. Nutrition, especially dietary intake of iron.
2. Past history of chronic recurrent infection.
3. Eating habits, particularly pica and ingestion of lead-based paint or
other toxic agents;
4. Bowel habits and presence of frank blood in stools or black, tarry
stools.
5. Familial history of hereditary disease such as sickle cell disease or
thalassemia.
 Prepare child for laboratory tests: The nurse should be responsible about
the following:
1. Explaining the significance of each test, particularly why the tests
are not done at one time.
2. Physically being with the child during the procedure.
3. Allowing the child to play with the equipment on a doll.
 Decrease the child oxygen needs: Decrease in oxygen carrying capacity
of the RBCs is the main pathology. So the nurse is responsible about the
following:
1. To assess the child’s daily living activities and play tolerance.
2. Necessary adjustment is made to avoid undue exertion
3. Observe vital signs and behavior during periods of rests.
4. Observe vital signs and behavior during periods of activities and
compare between the two levels.
 An essential nursing responsibility is instructing parents in the
administration of iron. Oral iron should be given in three divided doses
between meals.
 Acidific fruits or juice should be taken with medication that aids in
absorption.
 An adequate dosage of oral iron turns the stools to greenish black, so the
nurse should advise parents that this is normal and it’s absence may be a clue
to poor administration of iron.
 Vomiting and /or diarrhea are not uncommon complications of iron
therapy. If the parents report these symptoms, the iron can be given with
meals and the dosage reduced and then increased gradually.
6
 Liquid preparations of iron may temporarily stain the teeth. If possible, the
medication should be taken through a straw or given through a syringe or
medicine dropper placed toward the back of the mouth. Brushing the teeth
after administration of drug lessens the discoloration.
 Iron preparations for I.M. injection should be injected deeply into a large
muscle mass using the z-tract method, and the injection site should not be
massaged after injection to minimize skin staining and irritation.
Diet:
A primary nursing objective is to prevent nutritional anemia through family
education.
 The nurse discusses with the parents the importance of nursing iron-formula
and the introduction of solid food at the appropriate age.
 Diet education of teenage girls is particularly prone following weight
reduction diets. So, emphasize the importance of taking the adequate iron in
diet.
N .B.: Because iron ingested in excessive quantities is toxic, parents should
keep no more than a one-month supply in the home and store safely away from
reach of children.
7
Anemias Caused by Defect in
Hemoglobin Formation
I.
II.
Sickle cell anemia (SCA).
Thalassemia.
I. 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.
8
Pathophysiology:
The course of the disease is secondary to:
1. Repetitive occlusion of the small blood vessels by sickled erythrocytes as it
can’t squeeze itself through the small diameter of blood vessel and become
permanently trapped.
2. Increased RBCs destruction caused by inflexibility and fragility of the sickle
cells.
3. As a result there will be increase in blood viscosity, circulatory stasis, and
hypoxia, (which lead to further sickling).
4. If occlusion is extensive, tissue necrosis and extreme pain occur (crisis).
5. If the process is repeated, progressive organ failure results.
Assessment:
 The clinical manifestation of sickle cell disease are not apparent at birth
because of the high levels of fetal hemoglobin (HB F) prevent excessive
sickling of the RBCs, sickling may become apparent clinically after 5-6
months of age.
 Clinical manifestations are caused by: Ischemia resulting from severe
hemolytic anemia and chronic vaso-occlusive episodes.
 The history is taken after admission to the hospital or clinic; it may be
found that the infant or child usually leads to a reasonably normal life.
 However there may be complaints such as:
- Weakness.
- Anorexia and vomiting.
- Fever.
- Pain in the large joints of the extremities, in the back and
abdomen.
- Growth in height and weight may be retarded.
- Sexual maturation may be delayed in older children.
- Female adolescents may have decreased fertility.
- Male adolescents may have priapism (persistent abnormal
erection of the penis accompanied by pain and tenderness), which
may lead to impotence.
Physical Assessment:
1. Heart:
Impressive murmur, cardiac decompansation, heart failure.
2. Lung:
Pneumonia and emboli in pulmonary vessels by occluded mass of sickled
RBCs.
3. Spleen:
Splenomegaly decreased phagocytic function, which leads to infection and
traumatic rupture of enlarged spleen, internal hemorrhage.
4. Liver:
Hepatomegaly, chronic production of bilirubin, jaundice and gallstone.
5. Kidney:
Inability to concentrate urine, hematuria, polyuria and enuresis.
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6. Central nervous system:
Neurologic symptoms, e.g. weakness, headache, vision disturbances, aphesia,
convulsion, stroke hemiplegia and death.
7. Skeletal muscles:
Symmetrical painful swelling of hands and feet (hand –foot syndrome) during
infancy. Kyphosis may occur and chronic hypoxia can leads to osteomyelitis.
Clinical manifestations and Pathophysiology can be summarized in the
following figure:
10
Diagnostic Evaluation:







Complete blood picture.
Sickling cell.
Hemoglobin electrophoresis to detect abnormal Hgb.
Serum bilirubin.
Liver function test.
Sedimentation rate.
X- ray for skull and long bone.
Treatment:
There is no convential’s cure for SCA.
The aims of medical therapy are:
1. To prevent the progress of pathologic sequels.
2. To treat the emergencies of sickle cell crisis.
3. Prevention consists of promoting adequate oxygen and maintaining
hemodiluation.
4. Medical management of crisis is usually directed at supportive and
symptomatic treatment.
The main objectives of medical treatment are:
1.
2.
3.
4.
5.
6.
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 Considerations:
Nursing care of the child with SCA is largely related to teaching and supporting
the family and providing comfort and pain relief to the child during a vaso-occlusive
crisis.
Nursing Diagnosis:
 Impaired physical mobility related to tissue ischemia, generalized
weakness.
 High risk for infection related to poor tissue oxygenation.
 Altered family processes related to child with chronic condition.
For sickle crisis:
 Pain related to ischemia (sickle cell crisis).
 Altered tissue perfusion related to impaired arterial blood flow.
Planning:
The primary nursing goals are as follows:
1. Educate family and child (when appropriate) regarding the sickling
phenomenon and possible consequences.
2. Promoting supportive therapies during crisis.
3. Recognize other complications.
4. Encourage screening and genetic counseling.
5. Help child and parents to adjust to a lifelong potentially fetal hereditary
disease.
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Implementation:
N .B.: In relieving pain use hot application and don’t use cold application where it
leads to more vasoconstriction and ischemia and more pain.
1. Minimize Deoxygenation: Anything that increases cellular metabolism
also results in tissue hypoxia.
2. Promote Hydration: Hemodiluation is very important in preventing
sickling, and also it delays the vaso-occlusive-hypoxic- ischemic cycle. (The
nurse should calculate the daily requirement of fluids).
3. Minimize Crisis: The nurse should teach parents sings and symptoms of
the crisis, and to seek medical advise promptly. And also isolate the child from
any source of infection.
4. Promote Supportive Therapies during Crisis: Through managing
pain, fever.
5. Encourage Screening and Genetic Counseling.
6. Explain the Disease.
7. Support Family:
Report sings of the following immediately:
 Chest Syndrome:

-
Severe chest pain, sometimes spreading to abdomen.
Fever of 38.8°C (102°F) or higher.
Very congested cough.
Dyspnea, tachypnea.
Retraction.
Stroke:
-
Jerking or twitching of the face, legs, or arms.
Convulsions or seizures.
Stranger, abnormal behavior.
Inability to move any arm and /or a leg.
Stagger or an unsteady walk.
Stutter or slurred speech.
Weakness in the hands, feet, or legs.
Changes in vision.
Severe, unrelieved headache.
Severe vomiting.
Evaluation:
The effectiveness of nursing intervention is determined by continual
reassessment and evaluation of care based on the following observational
guidelines:
1. Observe child for any evidence of sickling; monitor preventive strategies and
therapies.
2. Interview family regarding genetic counseling.
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3. Interview family regarding their understanding of the disease, the sickling
phenomenon, and its consequences.
4. Interview and observe child and family regarding the way in which the disease
has affected their lives.
Expected Outcome:
Is described in the following plan:
Nursing Diagnosis:
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.
Patient Goal 3:
Will remain free of infection.
Nursing Intervention:
 Stress importance of adequate nutrition; routine immunization, including
pneumococcal and meningococcal vaccines; protection from known sources of
infection; and frequent health supervision.
 Report any sing of infection to practitioner immediately to avoid delay in
treatment.
 Promote compliance with antibiotic therapy both to prevent and to treat
infection.
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Expected Outcome:
Child remains free of infection.
Patient Goal 4:
Will experience decreased risks associated with a surgical procedure.
Nursing Intervention:
 Explain reason for preoperative blood transfusion (given to increase
concentration of HgbA).
 Keep child well hydrated to prevent sickling.
 Decrease fear through appropriate preparation, since anxiety increases oxygen
needs.
 Administer pain medications to keep child comfortable and reduce stress
response.
 Avoid unnecessary exertion to avoid additional oxygen needs.
 Promote pulmonary hygiene postoperatively to prevent infection.
 Use passive range of motion exercises to promote circulation.
 Administer oxygen, if prescribed, to saturate hemoglobin.
 Monitor for evidence of infection to avoid delay in treatment.
Expected Outcome:
Child undergoes a surgical procedure without crisis.
Nursing Diagnosis:
Pain related to tissue anoxia (vaso-occlusive crisis).
Patient Goal 1:
Will have no pain or pain relieved to level acceptable to child.
Nursing Intervention:
 Plan preventive schedule of medication around the clock, not as needed, to
prevent pain.
 Recognize that various analgesics, including opioids, and medication
schedules may need to be tried to achieve satisfactory pain relief.
 Avoid administration of meperidine (Demerol) because of increased risk of
normeperidine-induced seizures.
 Reassure child and family that analgesics, including opioids, are medically
indicated, high doses may be needed, and children rarely become addicted,
because needless suffering may result from their unfounded fears.
 Apply heat to affected area because it may be soothing.
 Avoid applying cold compresses because this enhances sickling and
vasoconstriction.
Expected Outcome:
Child will experience no or minimal pain.
Nursing Diagnosis:
Altered family processes related to a child with potentially life-threatening
disease.
Patient Goal 1:
Will receive education regarding disease.
Nursing Intervention:
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 Teach family and older children characteristics of basic defect and measures to
minimize complications of sickling.
 Stress importance of informing significant health personnel of child’s disease to
ensure prompt and appropriate treatment (e.g. for pain).
 Explain signs of developing crisis, especially fever, pallor, respiratory distress,
and pain, to avoid delay in treatment.
 Reinforce basics of trait transmission and refer to genetic counseling services
for family to make informed reproductive decisions.
 Teach parents to be an advocate for their child to secure the best care.
Expected Outcome:
Child and family demonstrate an understanding of the disease, its etiology and
its therapies.
Patient Goal 2:
Will receive adequate support.
Nursing Intervention:



Refer to special organization and agencies for ongoing support.
Refer child to comprehensive sickle cell clinic for ongoing care.
Be especially alert to family’s needs when two or more members are affected.
Expected Outcome:
Family takes advantage of community services (specify).
Child receives ongoing care from appropriate facility.
II. 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).
Pathophysiology:
 Normal postnatal hemoglobin (Hgb A) is composed of two α and two βpolypeptide 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.
 This excess α-chains deposited in RBCs lead to defective production of
Hemoglobin (ineffective erythropoiesis) because most cells produced are so
poorly hemoglobinized that never leave the marrow.
 Those that do leave the marrow survive only for a short time, which lead to
hemolysis in circulation and chronic hemolytic anemia.
 Marrow expansion continuous, leading to frontal bossing and typical
maxillary prominence. Hepato-splenomegaly and lymph adenopathy also
occur.
 Decrease Hgb level lead to tissue hypoxia and repeated infection.
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Assessment:
 Diagnosis is made most frequent during the first year of life, perhaps as
early as 3 months but more commonly by 10-12 months of age. The diagnosis
is rarely made during the newborn period, because (Hgb F) production is
sufficient and change to adult hemoglobin is not complete until 3-4 months
after birth.
 The clinical manifestations are caused by:
1. Absent of defective synthesis of (Hgb A),
2. Inadequately structured RBCs,
3. Decreased lifespan of RBCs.
 Assessment Criteria:
1. Anemia:



Unexplained fever.
Poor feeding.
Markedly enlarged spleen.
2. Progressive Anemia: Signs of Hypoxia:




Headache.
Bone pain.
Decreased exercise tolerance.
Anorexia.
3. Other Symptoms:



Frequent epistaxis.
Hemochromatosis.
Hemosiderosis.
4. Other Features:



Small stature.
Delayed sexual maturation.
Protrusion of the abdomen (hepato- spleno- megaly).
5. 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.
Diagnostic Evaluation:
1. Hematologic studies reveal the characteristic changes in RBCs and
immature erythrocytes.
2. Low hemoglobin and hematocrit level are seen in severe anemia.
3. Hemoglobin electrophoresis confirms the diagnosis and radiographs of
involved bones reveal characteristic findings.
Therapeutic Management:
1. Blood Transfusion.
2. Chelation Therapy.
16
The objective of supportive therapy is to maintain sufficient hemoglobin levels
to prevent tissue hypoxia. Transfusions are the foundation of medical management.
Recent studies have evaluated the benefits of maintaining the child’s hemoglobin
level above 10 gm/dl a goal that may require transfusions as often as every 3 weeks.
The advantage of this therapy include:
1. Improved physical and physiological well-being because of the ability to
participate in normal activities.
2. Decreased cardiomegaly and Hepatomegaly.
3. Fewer bone changes.
4. Normal or near normal growth and development until puberty.
5. Fewer infections.
 One of the potential complications of frequent blood transfusions is iron
overload. Since the body has no effective means of eliminating the excess
iron, the mineral is deposited in body tissues. To minimize the development of
hemosiderosis, deferoxamine (Desferal) an iron-chelating agent (Chelation
therapy which cause the exertion of excess iron in urine and coloring it orange
red) is given with oral supplement of vitamin C.
 Splenectomy may be necessary to decrease the disability effects of
abdominal pressure and to increase the life span of supplemental RBCs.
Prognosis:
Most children treated with blood transfusion survive well into adulthood. The
most common cause of death is heart disease, infection and liver disease.
Nursing Consideration:
The objectives of nursing care are to:
1. Observe for complications of multiple blood transfusions.
2. Assist the child in coping with the anxiety.
3. Foster the child and family’s adjustment to a chronic illness.
Basic to each of these goals is explaining to parents and older children the
defect responsible for the disorder and its effect on RBCs. Urine changes to orange
red due to Chelation therapy.
Genetic counseling for the parents and fertile offspring is mandatory and both
prenatal diagnoses using amniocentesis at 10 weeks and screening for thalassemia
trait are available.
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Defects in Hemostasis
Hemostasis is the process that stops bleeding when a blood vessel is injured.
Vascular and plasma clotting factors as well as platelets are required. Although the
coagulation process is complex clotting depends on three factors:
1. Vascular influence.
2. Platelet role.
3. Clotting factor.
Defects in platelets and clotting factors are the most common causes of
bleeding during childhood.
I.
II.
Hemophilia.
Idiopathic Thrombocytopenic Purpura.
I.
Hemophilia A
(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).
Pathophysiology:
 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.
Assessment:
 Males are predominantly affected; females are rarely affected (carriers).
 Positive family history in 70% of affected children.
 Parents will bring their child with complaints of recurrent hemorrhages,
prolonged bleeding, and in newborn: intracranial hemorrhage.
 The frequency and severity of bleeding are related to level of factor VIII
deficiency:
Clinical
severity
Severe
Moderate
Mild
Factor VIII
Activity
Bleeding Tendency
1%
Spontaneous bleeding
without trauma.
Bleeding with trauma.
1%-5%
5%- 50%
Bleeding with severe
trauma or surgery.
18
 The severity as explained above is demonstrated in about 60-70% of
affected children.
 Intramuscular and subcutaneous hemorrhages are common.
 Hemarthrosis (bleeding in joint cavities) especially the knees, elbows, and
ankles, is a most common form of internal bleeding. This will lead to pain,
limping fibrous ankylosis of joints lead to crippling.
 Minor hematoma leads to echymosis and large subcutaneous hematomas.
 Hematomas in spinal cord can lead to paralysis.
 Bleeding from mucous membranes; epistaxis, dental and GIT.
 Hematuria.
 Intracranial hemorrhage can be fetal.
Diagnostic Evaluation:
 Clinical symptoms, laboratory data and genetic history are used to
differentiate hemophilia from other bleeding conditions.
 Laboratory tests include: complete blood account, platelet function test,
clotting studies.
Hemophilia leads to a prolonged partial thromboplastin time (PTT). Measure
each coagulation factor in blood plasma and assist in the identification of the
specific hemophilia.
Therapeutic management:
 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.
 A regular program of exercise and physical therapy is an important aspect
of management. Physical activity within reasonable limits strengthens muscles
around joints, which help retard or confine bleeding in the area.
 Treatment without delay results in more rapid recovery and a decreased
likelihood of complications, therefore most of children are treated at home.
The family is taught the technique of venipuncture and to administer the AHF
(anti-hemophilic factor) to children over 3 years of age. The child learns the
procedure for self-administration at 9 to 12 years of age. Home treatment is
highly successful and the rewards, in addition to the immediacy are less
disruption of the family life, fewer school or work days missed and
enhancement of the child’s self esteem.
Prognosis:
There is no cure for hemophilia, its symptoms can be controlled and its
potentially crippling deformities markedly reduced or even avoided. Today many
children with hemophilia are function with minimal or no joint damage.
19
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 longterm goals:
1. Prevent bleeding.
2. Recognize and control bleeding.
3. Prevent crippling effect of bleeding.
4. Support family and prepare for home care.
5. 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 waterirrigating 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, especially for use
during control of pain at home.
2.Recognize and Control bleeding:
The earlier a bleeding episode is recognized, the more effectively it can be
treated. Supportive measures for bleeding control may be implemented such as:
 Applying pressure to the area for at least 10 to 15 minutes to allow clot
formation.
 Immobilizing and elevating the area above the level of the heart to decrease
blood flow.
 Applying cold to promote vasoconstriction.
20
Therapies for Hemophilia A:
Site of Bleed
Treatment
Joint
Rest
Ice
Elevation
Splint/Ace wrap/ crutches
Physical therapy
Ice
Elevation
Splint/ Ace wrap
Soft tissue
(Substraneous)
Muscle
Mucous
Membrane
(e.g. nose,
mouth)
Rest
Ice
Elevation
Splint/ Ace wrap/ crutches
Physical Therapy
Complete bed rest for iliopsoas muscle
bleed.
Pressure to nares (for nosebleed)
Topical antifibrinolytic agent (epsilon
amino-capronic acid)
Nasal pack (sometimes necessary)
Emergency Treatment of Bleeding from Nose:
 Have child sit up and lean forward (not lying down).
 Apply continuous pressure to nose with thumb and forefinger for at
least 10 minutes.
 Insert cotton or wadded tissue into each nostril and apply ice or cold
cloth to bridge of nose if bleeding persists.
 Keep child calm and quite.
3. Prevent Crippling Effects of Bleeding:
 During bleeding episodes the joints is elevated and immobilized.
 Active range of motion execrsies are usually instituted after acute episode.
N.B.: Passive range-of –motion exercises should never be part of an exercise
regimen after an acute episode, since the joint capsule could easily be stretched and
bleeding could recur. Active range of motion exercises is best so that the patient can
control his or her own pain tolerance.
4. Support the Family and Prepare for Home Care:
The bleeding can be minimized and the child can live as much as normal,
unrestricted life. Children are taught to take responsibility for their disease at an early
age.
5. Identify Persons at Risk:
Genetic counseling is essential as soon as possible after diagnosis.
21
Evaluation: (Expected Outcome)
1. Child exhibits no evidence of bleeding.
2. Child exhibits no evidence of tissue damage.
3. Child and family discuss their feelings and concerns and demonstrate an
understanding of the disease and its therapy (specify knowledge and method
of administration).
4. Family seeks genetic counseling.
II. Idiopathic Thrombocytopenic Purpura
(ITP)
It is an acquired hemorrhagic disorder most commonly occurs in children
between 2-8 years. Characterized by:
 Shortened life span of platelets (< 100.000/ mm 3), which lead to excessive
destruction of platelets (thrombocytopenia).
 This lead to purperic spots (petichia) which is bleeding under skin.
Etiology:
Although the exact cause is not known, it is believed to be due to autoimmune
platelets antibodies of IgG type.
Pathophysiology:
50%of cases are preceded by viral infections: rubella, measles, and viral
respiratory infection. These viruses may combine with platelets making them
antigenic and stimulate the formation of anti-platelets antibodies (autoimmune
reaction). Leading to:
 Increased destruction of platelets.
 Decreased capacity of megakaryocytes (stem cell in bone marrow from
which platelets evolve).
This will lead to bleeding under the skin.
Assessment:
1. Easy bruising:
 Petechiae (purpuric spots).
 Echymosis: (most commonly over bony prominences).
2. Bleeding from mucous membranes:
 Epistaxis.
 Bleeding gums.
3. Internal hemorrhage evidenced by:
 Hematuria.
 Hematemesis.
 Melena.
 Hemarthrosis.
 Menorrhagia.
4. Hematomas over lower extremities.
Diagnostic Evaluation:
22
 Clinical manifestation.
 Platelet count is reduced to below 20.000 mm 3 , therefore tests that depend
on platelet function are abnormal such as the tourniquet test, bleeding time
and clot retraction.
Therapeutic Management:
 Management is primarily supportive, since the course of the disease is selflimited in the majority of cases.
 Activity is restricted at the onset while the platelet count is low and while
active bleeding or progression of lesions is occurring.
 Corticosteroids are employed for children with the high risk for serious
bleeding for chronic with increased bleeding tendencies.
 Administration of I.V. gamma globin has proved successful in increasing the
platelet count of children with chronic disease.
 Splenectomy is reserved for symptomatic children with chronic disease or in
case of hemorrhage.
Prognosis:
The majority of children have self-limited course without major complication.
Some children develop chronic I.T.P. and require ongoing therapy.
Nursing Consideration:
Nursing care is largely supportive. Children and parents need careful
explanations of the rational behind the therapies employed and support in the efforts
to comply.
The nurse should participate in the supportive therapy, which includes:
1. Whole blood transfusion or if severe bleeding occurs, platelet rich plasma is
given to patient.
2. Antibiotic therapy to treatment infection.
3. Bed rest for moderate or severe bleeding.
4. Well balanced diet rich in iron and protein, also vitamins supplement.
5. Splenectomy, if disease continues for 6-12 months.
6. The harmful effect of using aspirin to control pain are critical for these children,
therefore, salicylate substitutes are always used.
7. The nursing considerations of controlling bleeding and preventing bruising are
similar to those discussed in the leukemia. (Nursing diagnosis no 2 and no 7).
Nursing Care Plan for the Child with Anemia
23
Nursing Diagnosis:
1. 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:
2. Activity intolerance related to generalized weakness, diminished oxygen delivery to
tissues.
Goal: (1)
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 activities appropriate to
capabilities.
Goal: (2)
Patient will exhibit normal respirations.
Intervention:


Promotion for optimum air exchange.
Administer supplemental oxygen if needed.
Expected Outcomes:
Patient breath easily, respiratory rate and depth are normal.
Goal: (3)
Patient will experience minimal emotional stress.
Intervention:
 Anticipate child’s irritability and short attention by offering to assist child in
activities rather than waiting for request for help.
 Encourage parents to remain with child.
Expected Outcomes:
Child remains calm and quite.
Goal: (4)
Nurse help replace blood elements.
Intervention:
24

Administer blood, packed cells, platelets as prescribed.
Expected Outcomes:
Child receives appropriate blood elements without incidence.
Nursing Diagnosis:
3. Altered nutrition, less than body requirements related to reported inadequate iron
intake, knowledge deficit regarding iron reach foods.
Goal: (1)
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 daily requirement of iron.
Goal: (2)
Patient will consume iron supplements.
Intervention:
 Administer iron preparation as prescribed.
 Instruct family regarding correct administration of oral iron: [preparation,
gives in divided doses, administer with fruit juice or multivitamins preparation,
do not give with milk or antacids.
 Administer liquid preparation with dropper, syringe or straw to avoid contact
with teeth.
Expected Outcomes:



Family relates a diet history that verifies child’s compliance.
Child is given iron supplement as evidenced by green tarry stools.
Child takes medication appropriately.
25
Leukemias
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.
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.
Pathophysiology:
There will be a bone marrow failure resulted from overgrowth of leukemic cells
in the marrow (immature blast cells).
Both red cells precursors and megakaryocytes from which platelets are formed are
decreased leading to anemia and epistaxis, and easy bruisability.
 Since white blood cells are decreased infection is common.
 Leukemic cells will proliferate (migrate out) of bone marrow and infiltrate in
lymph nodes spleen, liver leads to Hepato-splenomegaly and
lymphoadenopathy.
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.
26
Assessment:
It is according to the pathological effect:
Organ or Tissue
Consequences
Manifestation
1. Decreased RBCs
Bone
Marrow
anemia.
Dysfunction
2. Neutropneia
infection.
3. Decreased
platelets
bleeding tendancy.
4. Invasion of bone marrow
bone weakness.
5. Invasion of periosteum
increase pressure.
1. Infiltration.
Liver, Spleen and 2. Enlargement.
Lymph gland
3. Eventual fibrosis.
1. Increased
intracranial
CNS Meninges
pressure.
Pallor,
exertion.
Fever
fatigue,
dyspnea
Hemorrhage
(Petechia)
Tendency toward fractures.
Pain (pain in joint, episodes of
abdominal pain)
1. Hepatomegaly.
2. Splenomegaly.
3. Lymphoadenopathy.
1. Severe headache.
2. Vomiting.
3. Irritability.
2. Ventricular enlargement.
4. Lethargy.
5. Papilledema.
6. Eventual coma.
3. Meningeal irritation.
7. Pain.
8. Stiff neck and back.
1. Cell deprivation of nutrients 1. Lassitude.
Hypermetabolism
by invading cells.
2. Muscle wasting.
3. Weight loss.
4. Anorexia.
5. Fatigue.
6. Low-grade fever.
Diagnostic Evaluation:
Leukemia is usually suspected by the history, physical manifestations and
peripheral blood smear that contains immature forms of leukocytes, frequently
combined with low blood counts. Definitive diagnosis is based on bone marrow is
hypercellular with primarily blast cells. Once the diagnosis is confirmed, a lumber
puncture is performed to determine if there is any C.N.S. involvement.
27
Therapeutic Management:
Treatment of leukemia involves the use of chemotherapeutic agents with or
without irradiation in three phases:
1. Remission induction therapy: which is determined by the absence of clinical
evidence of disease and the presence of less than 5% blast cells in the bone
marrow.
2. C.N.S. preventive therapy and consolidation, which provides treatment in
those areas of the body normally protected from cytotoxic, drug levels.
3. Maintenance therapy, which serves to maintain the remission phase.
In some children a fourth phase, reinduction therapy is needed to treat a
relapse.
1. Remission Induction:
(Eradicate leukemic cells)
Almost immediately after confirmation of the diagnosis, induction therapy is
begun and lasts 4 to6 weeks. The principal drugs used for induction in A.L.L. are:
 Corticosteroids. (Especially Prednisone).
 Vincrisline Sulfate “ Oncovin”.
 Adramycin.
2.C.N.S. preventive therapy / Consolidation:
(Prevention of meningeal leukemia)
Treatment of the C.N.S consists of prophylactic therapy with intrathecal
chemotherapy. This therapy is reserved for high-risk patients and those with C.N.S.
disease. C.N.S. therapy usually begins during the first 6 to 8 weeks after diagnosis
and is continued for a specific period of time.
Once complete remission is obtained, a period of intensified treatment is
administered to further reduce the leukemia burden (Consolidation).
3.Maintenance therapy:
Maintenance therapy is begun after completion of successful induction and
consolidation therapy to preserve the remission and further lessen the number of
leukemic cells.
4.Reinduction therapy: (Fourth phase)
When the presence of leukemic cells are observed in the bone marrow, C.N.S.
or other sites, the child has relapsed. Therapy for the child who has relapsed
includes reinduction. Other drugs not previously used.
Bone marrow transplantation:
It has been used successfully for treating children who have A.L.L.. and
A.N.L.L. bone marrow transplantation is not recommended for children with A.L.L.
during the first remission because of the excellent results possible with
chemotherapy. Because of the poor prognosis in children with A.N.L.L.
transplantation may be considered during the first remission.
28
Prognosis:
The most important factors for determining long-term survival for children with
A.L.L. in addition to treatment are:
1. The initial WBCs count.
2. The child’s age at the time of diagnosis.
3. The type of cell involved.
4. The sex of the child.
Children diagnosed between 2 and 9 years of age have consistency
demonstrated a better outlook than those diagnosed before 2 or after 10 years
of age, and females appear to have a more favorable prognosis than male.
Nursing care plan for the child with leukemia:
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.
Administer antibiotics as prescribed.
Expected Outcome:
 Child doesn’t come in contact with infected persons or contaminated
articles.
 Child consumes diet appropriate for age.
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:

Child exhibits no evidence of bleeding.
29
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.
 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.
Nursing diagnosis:
4. High risk for fluid volume deficit related to nausea and vomiting.
Goal:
Patient will experience no vomiting.
Intervention:




Administer initial dose of antiememtic before onset of nausea and vomiting.
Avoid foods with strong odors.
Uncover hospital food tray outside of child’s room to reduce food orders.
Mouths care frequently.
Expected Outcome:

Child retains food and fluids.
Nursing diagnosis:
5. Altered mucous membranes related to administration of chemotherapeutic agents.
Goal:
Patient will not develop oral mucostis.
Intervention:
 Inspect mouth daily for oral ulcers, avoid oral temperatures.
 Oral hygiene as soon as a drug is used that caused oral ulcers.
 Use soft sponge toothbrush, cotton tipped applicator or gauze wrapped
finger.
 Administer frequent (at least every 4 hours and after meals) mouthwashes
(normal saline or sodium bicarbonate solution).
 Apply local anesthetics to ulcerated areas before meals and as needed.
 Serve bland, moist, soft diet.
 Encourage fluids; use a straw to help bypass painful areas.
 Report evidence of ulcers to doctor.
 Avoid juices containing ascorbic acid lemon swabs and hot or cold food.
 Administer antiinfective medication as ordered.
30
Expected Outcome:


Mucous membrane remains intact.
Ulcer shows evidence of healing.
Goal: (2)
Patient will not develop rectal ulceration.
Intervention:
 Wash perineal area after each bowel movement.
 Use warm bath or tub baths.
 Apply protective skin barriers (transparent film dressings, occlusive
ointment) to perineal area.
 Observe constipation resulting from child’s voluntary refusal to defecate or
from chemotherapy.
 Avoid rectal temperature and suppositories.
 Record bowel movement, use stool softener to prevent constipation.
Expected Outcome:



Rectal mucosa remains clean and intact.
Ulcerated areas heal without complications.
Child has regular bowel movements.
Nursing diagnosis:
6. Impaired skin integrity related to administration of chemotherapeutic agents.
Goal:
Patient will maintain skin integrity.
Intervention:



Provide skin care especially in mouth and perineal regions.
Change position frequently.
Encourage adequate caloric-protein intake.
Expected Outcome:

Skin remains intact.
Nursing diagnosis:
7. Pain related to diagnosis and treatment.
Goal:
Patient will experience minimal or no pain.
Intervention:
 Avoid excessive noise or light.
 Place all commodities with easy reach.
 Use gentle, minimal physical manipulation.
 Avoid pressure on painful relief from analgesics.
 Experiment with using heat or cold on painful areas (Use cautiously
because of easy skin breakdown).
 Change position frequently, if difficult for child coordinate with pain relief
from analgesics.
 Avoid pressure on bony prominences.
 Administer analgesics as prescribed.
 Avoid aspirin or any of its compounds.
31
Expected Outcome
 Child rests quietly, exhibits no evidence of discomfort verbalizes no
complaints of discomfort.
Nursing diagnosis:
8. Body image disturbance related to loss of hair and moon face.
Goal: (1)
Patient will exhibit positive coping.
Intervention:
 Administer good scalp hygiene.
 Provide adequate covering during exposure to sunlight, wind or cold.
 Stress that hair begins to regrow in 3 to 6 months and may be a slightly
different color or texture.
Expected Outcome:


Child verbalizes concern regarding hair loss.
Child appears clean, well groomed and attractively dressed.
Goal: (2)
Patient will exhibit adjustment to altered facial appearance.
Intervention:



Stress that this reaction is temporary.
Evaluate weight gain carefully.
Encourage early and consistent interaction with peer.
Expected Outcome:

Child resumes former activities and relationship within capabilities.
Nursing diagnosis:
9. Fear related to diagnostic tests, procedures, treatment, and prognosis.
Goal:
Patient will exhibit reduced fear related to diagnostic procedures and tests.
Intervention:




Explain procedure carefully at child’s level of understanding.
Explain responsibility of child.
Provide child with some means of involvement with the procedure.
Implement distracting technique and pain reduction techniques as indicated.
Expected Outcome:


Child readily responds to verbal directives and exhibits less fear.
Child repeats information accurately.
32
Nursing diagnosis:
10. Altered family process related to a child with life threatening disease.
Goal:
Family will receive adequate support.
Intervention:
 Teach parents about disease process.
 Explain all procedures that will be done to child.
 Schedule time for family to be together.
 Help family for future, especially toward helping child live a normal life.
 Encourage family to discuss feelings regarding child’s course prior to
diagnosis and child’s prospects for survival.
 Discuss with family how they will tell child about outcome of the disease.
Expected Outcome:

Family expresses feelings and concerns and spends time with child.
33