Chapter 27Chapter 27
The Child with a Condition of theThe Child with a Condition of the
Blood, Blood-Forming Organs, orBlo...
Hematological SystemHematological System
• Occur when blood values exceed or fail to form
correctly or fail to meet normal...
Lymphatic SystemLymphatic System
3Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier ...
Lymphatic SystemLymphatic System (cont.)(cont.)
• Drains regions of the body to lymph node
– Where infectious organisms ar...
Lymphatic SystemLymphatic System (cont.)(cont.)
• Spleen is largest organ of the lymphatic
system
• One of the main functi...
Circulating BloodCirculating Blood
• Consists of two
portions
• Plasma
• Formed elements
– Erythrocytes
– Leukocytes (whit...
AnemiasAnemias
• Can result from many different underlying
causes
– A reduction in the amount of circulating
hemoglobin (H...
Iron-Deficiency AnemiaIron-Deficiency Anemia
• Most common nutritional deficiency of
children
– Incidence is highest durin...
Iron-Deficiency AnemiaIron-Deficiency Anemia (cont.)(cont.)
• Manifestations
– Pallor
– Irritability
– Anorexia
– Decrease...
Iron-Deficiency AnemiaIron-Deficiency Anemia (cont.)(cont.)
• Untreated, iron-
deficiency anemia will
progress slowly
– In...
Nursing TipNursing Tip
• Oral iron supplements should not be given
with milk or milk products because milk
interferes with...
Parent EducationParent Education
• Nurse stresses importance of breastfeeding
for the first 6 months and the use of iron-
...
Sickle Cell DiseaseSickle Cell Disease
• Inherited defect in the formation of hemoglobin
• Sickling (clumping) caused by d...
Transmission of Sickle Cell DiseaseTransmission of Sickle Cell Disease
from Parents to Childrenfrom Parents to Children
14...
Two Types of Sickle Cell DiseaseTwo Types of Sickle Cell Disease
• Sickle cell trait
(asymptomatic)
– Blood of the patient...
ManifestationsManifestations
• Hgb level ranges 6 to 9 g/dL or lower
– Child is pale, tires easily, and has little appetit...
Types of Sickle Cell CrisesTypes of Sickle Cell Crises
• Vaso-occlusive (painful crises)
• Splenic sequestration
• Aplasti...
Health PromotionHealth Promotion
• During sickle cell crisis, anticipate the child’s
need for tissue oxygenation, hydratio...
Therapies and GoalsTherapies and Goals
• Erythropoietin and some chemotherapy
regimens can increase the production of feta...
ThalassemiaThalassemia
• Group of hereditary blood disorders in which
the patient’s body cannot produce sufficient
adult H...
ThalassemiaThalassemia (cont.)(cont.)
• Categorized according to the polypeptide
chain affected
– Beta-thalassemia is the ...
Thalassemia MinorThalassemia Minor
• Also termed beta-
thalassemia trait, occurs
when the child inherits a
gene from only ...
Thalassemia MajorThalassemia Major
(Cooley’s Anemia)(Cooley’s Anemia)
• Child is born with a more serious form of the
dise...
Thalassemia MajorThalassemia Major
(Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.)
• Jaundice that progresses to a muddy...
Thalassemia MajorThalassemia Major
(Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.)
• Bone marrow space enlarges to
compe...
Thalassemia MajorThalassemia Major
(Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.)
• Diagnosis
– Family history of thala...
Thalassemia MajorThalassemia Major
(Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.)
• Goals of therapy
– Maintain hemoglo...
Thalassemia MajorThalassemia Major
(Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.)
• Mainstay of treatment
– Frequent bl...
Thalassemia MajorThalassemia Major
(Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.)
• Nursing measures
– Adhere to the pr...
HemophiliaHemophilia
• One of the oldest hereditary diseases known
to man
• Blood does not clot normally
• Congenital diso...
HemophiliaHemophilia (cont.)(cont.)
• Inherited sex-linked recessive trait
– Defective gene is located on the X, or female...
Hemophilia AHemophilia A
• Caused by a deficiency of coagulation factor VIII,
or antihemophilic globulin (AHG)
• Severity ...
Manifestations of HemophiliaManifestations of Hemophilia
• Can be diagnosed at birth because factor VIII
cannot cross the ...
Manifestations of HemophiliaManifestations of Hemophilia
(cont.)(cont.)
• Anemia, leukocytosis, moderate increase in
plate...
DiagnosisDiagnosis
• Circumstances leading to diagnosis
– Nosebleed that will not stop
– Loss of a deciduous tooth
– Hemat...
Treatment of HemophiliaTreatment of Hemophilia
• If family history exists, a newborn may have certain
procedures delayed t...
Treatment of HemophiliaTreatment of Hemophilia (cont.)(cont.)
• Multidisciplinary approach to assist families to
develop h...
Safety AlertSafety Alert
• Drugs that contain salicylates are
contraindicated for children with hemophilia
38Elsevier item...
Platelet DisordersPlatelet Disorders
• Reduction or destruction of platelets in the
body interferes with the clotting mech...
Idiopathic (Immunological)Idiopathic (Immunological)
Thrombocytopenic Purpura (ITP)Thrombocytopenic Purpura (ITP)
• Acquir...
Manifestations of ITPManifestations of ITP
• Classic symptom is easy bruising
– Results in petechiae and purpura
• May hav...
Treatment of ITPTreatment of ITP
• Neurological
assessments are a
priority of care
• Treatment is not
indicated in most ca...
Complications of ITPComplications of ITP
• Bleeding from the GI tract
• Hemarthrosis
• Intracranial hemorrhage
• Preventio...
Risk for Development of CancerRisk for Development of Cancer
• Genetic and environmental factors play a role
• Exposure of...
LeukemiaLeukemia
• Most common form of cancer in childhood
• Refers to a group of malignant diseases of the
bone marrow an...
LeukemiaLeukemia (cont.)(cont.)
• A malignant disease of the blood-forming organs
that results in an uncontrolled growth o...
LeukemiaLeukemia (cont.)(cont.)
• Leukemia cells can infiltrate the spleen, liver,
and lymph glands, resulting in fibrosis...
Manifestations of LeukemiaManifestations of Leukemia
• Most common symptoms
– Initial phase
• Low-grade fever
• Pallor
• B...
Manifestations of LeukemiaManifestations of Leukemia (cont.)(cont.)
• WBCs not functioning
normally, increases
risk of inf...
DiagnosisDiagnosis
• Based on history and symptoms
• Results of extensive blood tests
– Demonstrate presence of leukemic b...
Treatment of LeukemiaTreatment of Leukemia
• Long-term care given whenever possible in an
outpatient setting
• Bone marrow...
Treatment of LeukemiaTreatment of Leukemia (cont.)(cont.)
• Components of chemotherapy include
– Induction period
– Centra...
Side Effects of ChemotherapySide Effects of Chemotherapy
• Steroids can mask signs of infection, cause
fluid retention, in...
Nursing Care of the ChildNursing Care of the Child
with Leukemiawith Leukemia
• Encourage the child to verbalize feelings
...
Child Receiving a BloodChild Receiving a Blood
TransfusionTransfusion
• Hemolytic reactions caused by mismatched
blood are...
Safety AlertsSafety Alerts
• If a blood transfusion reaction occurs, stop
the infusion, keep the vein open with normal
sal...
Hodgkin’s DiseaseHodgkin’s Disease
• A malignancy of the lymph system that primarily
involves the lymph nodes
– May metast...
Manifestations of Hodgkin’sManifestations of Hodgkin’s
DiseaseDisease
• A painless lump along the neck
• Few other manifes...
Criteria for Staging Hodgkin’sCriteria for Staging Hodgkin’s
DiseaseDisease
Stage Criteria
I Restricted to single site or ...
Treatment of Hodgkin’s DiseaseTreatment of Hodgkin’s Disease
• Both radiation and chemotherapy are used in
accordance with...
Nursing Care of Patients withNursing Care of Patients with
Hodgkin’s DiseaseHodgkin’s Disease
• Mainly directed toward sym...
Emotional Support of the PatientEmotional Support of the Patient
with Hodgkin’s Diseasewith Hodgkin’s Disease
• Support pr...
Chronic IllnessChronic Illness
• Behavior problems are lessened when patients
can verbalize specific concerns with persons...
Developmental DisabilitiesDevelopmental Disabilities
• Children with developmental disabilities may
often be overprotected...
Home CareHome Care
• Home health care and other community
agencies work together to provide holistic
care
• Respite care i...
Providing Home Health CareProviding Home Health Care
• Observe how the parents interact with the child
• Do not wait for t...
Care of the Chronically Ill ChildCare of the Chronically Ill Child
• Focusing on what the child can do and providing
succe...
Facing DeathFacing Death
• The nurse must understand
– The grieving process
– Personal and cultural views concerning that
...
Facing DeathFacing Death (cont.)(cont.)
• The response to a child’s death is influenced
by whether there was a long period...
Facing DeathFacing Death (cont.)(cont.)
• The nurse must
– Show compassion
– Demonstrate a nonjudgmental approach
– Be sen...
Self-ExplorationSelf-Exploration
• How nurses have or have not dealt with their
own losses affects present lives and the
a...
The Child’s Reaction to DeathThe Child’s Reaction to Death
• Cognitive development, rather than chronological
age, affects...
The Child’s Awareness ofThe Child’s Awareness of
His or Her ConditionHis or Her Condition
• Failure to be honest with chil...
Physical Changes of ImpendingPhysical Changes of Impending
DeathDeath
• Cool, mottled, cyanotic skin and the slowing
of al...
Stages of Dying and the Nurse’sStages of Dying and the Nurse’s
RoleRole
• Stages
– Denial
– Anger
– Bargaining
– Depressio...
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  • Define hematopoiesis.
  • Discuss Figure 27-1 on page 626.
  • Refer to Figure 27-2 on page 626.
  • What are the signs and symptoms of anemia?
  • Discuss the reasons this age group is more susceptible to iron-deficiency anemia.
  • Refer to Nursing Care Plan 27-1 on page 628.
  • Discuss the pathophysiology of the causes of sickling.
    Discuss the complications of hemosiderosis.
    Audience Response Question #1
    A 12-month-old child is admitted to the emergency department in sickle cell crisis. Her symptoms might include:
    1. severe itching, vomiting.
    2. fever, seizures, coma.
    3. abdominal pain, swollen painful joints.
    4. polycythemia, tachycardia.
  • Discuss the chances of a child inheriting sickle cell disease.
  • Discuss the pathophysiology of the pain the child experiences.
  • Describe each crisis listed.
  • Discuss how these nursing interventions assist the child with a sickle cell crisis.
  • Discuss methods which could prevent sickling, dehydration, hypoxia, and infection.
  • What ethnic population is mainly affected by thalassemia? Mediterranean
  • Define milk anemia.
    What are symptoms of thalassemia minor?
  • Discuss Figure 27-5 on page 632.
  • What is the rationale for radiographic studies of the bones?
    Discuss types of blood tests which would be ordered for a child with thalassemia major.
    Describe electrophoresis.
  • What activities would promote growth and development for this child?
  • Discuss signs and symptoms of a child reacting to a blood transfusion.
    What nursing interventions would promote emotional health of the child and the family?
  • Discuss how PTT can detect this disorder.
  • Discuss the pathophysiology of the symptoms listed.
  • What procedures might be delayed for the newborn?
  • Discuss which disciplines would be good resources for the family coping with a child who has hemophilia.
  • Discuss the rationale for avoiding salicylates.
  • Describe petechiae, purpura, ecchymosis, and hematoma.
  • Audience Response Question #1
    Complete the analogy. Hemophilia : actor VIII as ITP : ________.
    1. WBCs
    2. RBCs
    3. hemoglobin
    4. platelets
  • Discuss the procedure for bone marrow aspiration.
  • Discuss a proper neurological assessment.
    What is the rationale for a splenectomy?
  • Discuss the pathophysiology of fibrosis and its effects on these organs.
  • Discuss the pathophysiology of the symptoms listed.
  • Discuss Figure 27-6 on page 635.
  • Discuss what changes could be seen upon X-ray of the long bones.
    What are the effects of chemotherapy on the kidneys and the liver?
  • Discuss who would be involved in the care of a child with leukemia.
    Discuss the education of parents and family in preventing infection.
    What is the rationale for avoiding immunizations during the treatment regimen? The body will not be able to manufacture antigens as expected.
    Give an example of why oral care is important.
  • Discuss each of these stages of the treatment regimen.
  • Audience Response Question #3
    Which intervention is best to help prevent or reduce nausea and vomiting during chemotherapy?
    1. Encourage increased fluid intake.
    2. Administer an antiemetic immediately following treatment.
    3. Promote diversional activities.
    4. Administer an antiemetic 30 minutes before treatment.
  • What are ways to assess for infection?
    Discuss nursing interventions to provide good hygiene.
  • What are some signs of a transfusion reaction? Chills, itching, rash, fever, headache, and pain in the back
    What should the nurse do if a transfusion reaction occurs? The transfusion is immediately stopped, a normal saline line is immediately opened, and the primary caregiver and hospital blood bank are notified.
    What are signs of circulatory overload? Dyspnea, precordial pain, rales, cyanosis, dry cough, and distended neck veins
    If circulatory overload occurs, what are the appropriate actions? Be prepared to administer medications and supplemental oxygen.
  • What information will parents and the family need in caring for this child?
    Discuss Nursing Care Plan 27-2 on pages 639-640.
  • Discuss the nurse’s role in managing a child and family when dealing with a chronic illness.
  • Why is respite care for the family important?
  • Discuss issues the nurse would assess regarding home management of the child’s care.
  • Review Table 27-3 on page 641.
    How can the nurse involve the family in the care of a chronically ill child?
  • Discuss the grieving process.
    Give examples of cultural beliefs regarding death.
  • Give examples of how a nurse could support a parent who is angry at the loss of a child.
  • Review the Health Promotion box on page 643 and Nursing Care Plan 27-3 on pages 644-645.
  • How would the nurse explain the dying process to the parents?
    How will you know if your teaching was effective?
  • Discuss and provide examples of how the nurse can assist an individual through each stage.
  • Chapter 27 Power Point

    1. 1. Chapter 27Chapter 27 The Child with a Condition of theThe Child with a Condition of the Blood, Blood-Forming Organs, orBlood, Blood-Forming Organs, or Lymphatic SystemLymphatic System
    2. 2. Hematological SystemHematological System • Occur when blood values exceed or fail to form correctly or fail to meet normal standards • During childhood, RBCs are formed in the marrow of the long bones; by adolescence, hematopoiesis takes place in the marrow of the ribs, sternum, vertebrae, pelvis, skull, clavicle, and bone marrow • RBC production is regulated by erythropoietin – Substance is produced by the liver of the fetus – At birth, the kidneys take over this process Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 2
    3. 3. Lymphatic SystemLymphatic System 3Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    4. 4. Lymphatic SystemLymphatic System (cont.)(cont.) • Drains regions of the body to lymph node – Where infectious organisms are destroyed – Antibody production is stimulated • Lymphadenopathy is an enlargement of lymph nodes – Indicative of infection or disease 4Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    5. 5. Lymphatic SystemLymphatic System (cont.)(cont.) • Spleen is largest organ of the lymphatic system • One of the main functions is to bring blood into contact with lymphocytes • Most common pathological condition is enlargement (splenomegaly) • Enlarges during infections, congenital and acquired hemolytic anemias, and liver malfunction 5Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    6. 6. Circulating BloodCirculating Blood • Consists of two portions • Plasma • Formed elements – Erythrocytes – Leukocytes (white blood cells [WBCs]) – Thrombocytes (platelets) • Erythrocytes – Transport oxygen and carbon dioxide to and from the lungs and tissues • Leukocytes act as the body’s defense against infection • Lymphocytes are produced in the lymphoid tissues of the body Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 6
    7. 7. AnemiasAnemias • Can result from many different underlying causes – A reduction in the amount of circulating hemoglobin (Hgb) reduces the oxygen- carrying ability of the blood • An Hgb below 8 g/dl results in an increased cardiac output and a shunting of blood from the periphery to the vital organs • Can result in pallor, weakness, tachypnea, SOB, CHF 7Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    8. 8. Iron-Deficiency AnemiaIron-Deficiency Anemia • Most common nutritional deficiency of children – Incidence is highest during infancy (from 9th to 24th month) and adolescence • May be caused by severe hemorrhage, inability to absorb iron received, excessive growth requirements, or an inadequate diet – Giving whole cow’s milk to infants can lead to GI bleeding, leading to anemia 8Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    9. 9. Iron-Deficiency AnemiaIron-Deficiency Anemia (cont.)(cont.) • Manifestations – Pallor – Irritability – Anorexia – Decrease in activity • Infants may be overweight due to excessive milk consumption • Blood tests – RBC count – Hgb and hematocrit – Morphological cell changes – Iron concentrations • Stool may be tested for occult blood 9Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    10. 10. Iron-Deficiency AnemiaIron-Deficiency Anemia (cont.)(cont.) • Untreated, iron- deficiency anemia will progress slowly – In severe cases, heart muscle becomes too weak to function – Children with long- standing anemia may also show growth retardation and cognitive changes • Treatment – Iron, usually ferrous sulfate, orally 2 to 3 times a day – Vitamin C aids in absorption 10Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    11. 11. Nursing TipNursing Tip • Oral iron supplements should not be given with milk or milk products because milk interferes with iron absorption 11Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    12. 12. Parent EducationParent Education • Nurse stresses importance of breastfeeding for the first 6 months and the use of iron- fortified formula throughout the first year of life • Stools of infants taking oral iron supplements are tarry green • Do not give iron with milk • To increase absorption, give the iron between meals when digestive acid concentration is at its highest 12Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    13. 13. Sickle Cell DiseaseSickle Cell Disease • Inherited defect in the formation of hemoglobin • Sickling (clumping) caused by decreased blood oxygen levels may be triggered by dehydration, infection, physical or emotional stress, or exposure to cold – Membranes of these cells are fragile and easily destroyed – Their crescent shape makes it difficult for them to pass through the capillaries, causing a pileup of cells in the small vessels – May lead to thrombosis, can be very painful • Hemosiderosis (iron deposits into body organs) is a complication of the disease Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 13
    14. 14. Transmission of Sickle Cell DiseaseTransmission of Sickle Cell Disease from Parents to Childrenfrom Parents to Children 14Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    15. 15. Two Types of Sickle Cell DiseaseTwo Types of Sickle Cell Disease • Sickle cell trait (asymptomatic) – Blood of the patient contains a mixture of Hgb A and sickle (Hgb S) – Proportions of Hgb S are low because the disease is inherited from only one parent • Hgb and RBC counts are normal • Sickle cell anemia (more severe) • Clinical symptoms do not appear until the last part of the first year of life – May be an unusual swelling of the fingers and toes – Symptoms caused by enlarging bone marrow sites that impair circulation to the bone and the abnormal sickle cell shape that causes clumping, obstruction in the vessel, and ischemia to the organ the vessel supplies Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 15
    16. 16. ManifestationsManifestations • Hgb level ranges 6 to 9 g/dL or lower – Child is pale, tires easily, and has little appetite • Sickle cell crises are painful and can be fatal – Symptoms: severe abdominal pain, muscle spasms, leg pain, or painful swollen joints may be seen • Fever, vomiting, hematuria, convulsions, stiff neck, coma, or paralysis can result • Risk for stroke as a complication of a vaso-occlusive sickle cell crisis 16Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    17. 17. Types of Sickle Cell CrisesTypes of Sickle Cell Crises • Vaso-occlusive (painful crises) • Splenic sequestration • Aplastic crises • Hyperhemolytic 17Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    18. 18. Health PromotionHealth Promotion • During sickle cell crisis, anticipate the child’s need for tissue oxygenation, hydration, rest, protection from infection, pain control, blood transfusion, and emotional support for this life-threatening illness 18Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    19. 19. Therapies and GoalsTherapies and Goals • Erythropoietin and some chemotherapy regimens can increase the production of fetal Hgb and reduce complications • Routine splenectomy is not recommended because the spleen generally atrophies on its own because of fibrotic changes that take place in patients with sickle cell disease • Prevent infection, dehydration, hypoxia, and sickling Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 19
    20. 20. ThalassemiaThalassemia • Group of hereditary blood disorders in which the patient’s body cannot produce sufficient adult Hgb • RBCs are abnormal in size and shape and are rapidly destroyed; results in chronic anemia • Body attempts to compensate by producing large amounts of fetal Hgb 20Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    21. 21. ThalassemiaThalassemia (cont.)(cont.) • Categorized according to the polypeptide chain affected – Beta-thalassemia is the most common variety; involves impaired production of beta chains – Two forms • Thalassemia minor • Thalassemia major, also known as Cooley’s anemia – Can also occur from spontaneous mutations 21Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    22. 22. Thalassemia MinorThalassemia Minor • Also termed beta- thalassemia trait, occurs when the child inherits a gene from only one parent – Heterozygous inheritance • Associated with mild anemia • Often misdiagnosed as having iron-deficiency anemia • Symptoms minimal – Pale – Possible splenomegaly • May lead a normal life with the illness going undetected • Of genetic importance, particularly if both parents are carriers of the trait Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 22
    23. 23. Thalassemia MajorThalassemia Major (Cooley’s Anemia)(Cooley’s Anemia) • Child is born with a more serious form of the disease when two thalassemia genes are inherited (homozygous inheritance) • Progressive, severe anemia • Evident within the second 6 months of life • Child is pale, hypoxic, poor appetite, and may have a fever 23Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    24. 24. Thalassemia MajorThalassemia Major (Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.) • Jaundice that progresses to a muddy bronze color resulting from hemosiderosis • Liver enlarges and the spleen grows enormously • Abdominal distention is great • Increases pressure on the chest organs • Cardiac failure caused by profound anemia is a constant threat 24Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    25. 25. Thalassemia MajorThalassemia Major (Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.) • Bone marrow space enlarges to compensate for an increased production of blood cells – Hematopoietic defects and a massive expansion of the bone marrow in the face and skull result in changes in the facial contour – Teeth protrude due to an overgrowth of the upper jawbone • Bone becomes thin and is subject to fracture 25Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    26. 26. Thalassemia MajorThalassemia Major (Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.) • Diagnosis – Family history of thalassemia – Radiographic bone growth studies – Blood test – Hemoglobin electrophoresis is helpful in diagnosing type and severity 26Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    27. 27. Thalassemia MajorThalassemia Major (Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.) • Goals of therapy – Maintain hemoglobin levels to prevent overgrowth of bone marrow and resultant deformities – Provide for normal growth and development and physical activity – Prevention or early treatment of infection is important – Some may require a splenectomy due to degree of splenomegaly 27Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    28. 28. Thalassemia MajorThalassemia Major (Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.) • Mainstay of treatment – Frequent blood transfusions to maintain Hgb above 10 g/dL – Because of the number of transfusions, hemosiderosis is seen in the spleen, liver, heart, pancreas, and lymph glands • Deferoxamine mesylate (Desferal), an iron-chelating agent is given to counteract this side effect – A splenectomy may be needed to increase comfort, increase ability to move about, and to allow for more normal growth Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 28
    29. 29. Thalassemia MajorThalassemia Major (Cooley’s Anemia)(Cooley’s Anemia) (cont.)(cont.) • Nursing measures – Adhere to the principles of long-term care – Whenever possible, have the same nurse assigned to the child – Observing the patient during blood transfusions for any adverse reactions – Monitoring vital signs – Providing for the emotional health of the child and family is essential 29Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    30. 30. HemophiliaHemophilia • One of the oldest hereditary diseases known to man • Blood does not clot normally • Congenital disorder confined almost exclusively to males – Is transmitted by symptom-free females 30Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    31. 31. HemophiliaHemophilia (cont.)(cont.) • Inherited sex-linked recessive trait – Defective gene is located on the X, or female, chromosome – Fetal blood samples detect hemophilia • Two most common types – Hemophilia B (Christmas disease [a factor IX deficiency]) – Hemophilia A (a deficiency in factor VIII) • A deficiency in any one of the factors will interfere with normal blood clotting 31Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    32. 32. Hemophilia AHemophilia A • Caused by a deficiency of coagulation factor VIII, or antihemophilic globulin (AHG) • Severity dependent on level of factor VIII in the plasma • Some patients’ lives can be endangered by a minor scratch, while others may simply bruise more easily than the average person • Aim of therapy is to increase level of factor VIII to ensure clotting • This is checked by a blood test call partial thromboplastin time (PTT) Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 32
    33. 33. Manifestations of HemophiliaManifestations of Hemophilia • Can be diagnosed at birth because factor VIII cannot cross the placenta and be transferred to the fetus – Usually not apparent in the newborn unless abnormal bleeding occurs at the umbilical cord or after circumcision • Normal blood clots in 3 to 6 minutes – In severe hemophilia, it can take up to 1 hour or longer 33Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    34. 34. Manifestations of HemophiliaManifestations of Hemophilia (cont.)(cont.) • Anemia, leukocytosis, moderate increase in platelets may be seen in hemorrhaging; may also be signs of shock • Spontaneous hematuria is seen • Death can result from excessive bleeding, especially if it occurs in the brain or neck • Severe headache, vomiting, and disorientation may be symptoms Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 34
    35. 35. DiagnosisDiagnosis • Circumstances leading to diagnosis – Nosebleed that will not stop – Loss of a deciduous tooth – Hematomas develop at the injection site of an immunization – Hemorrhage into the joint cavity (considered a classic symptom) • A classic symptom of hemophilia is bleeding into the joints (hemarthrosis) Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 35
    36. 36. Treatment of HemophiliaTreatment of Hemophilia • If family history exists, a newborn may have certain procedures delayed to prevent bleeding and tissue injury • Principal therapy is to prevent bleeding by replacing the missing factor • Recombinant antihemophilic factor, a synthetic product, has eliminated the need for repeated blood transfusions • Desmopressin acetate (DDAVP) is a nasal spray that can stop bleeding • Prophylactic care must be provided prior to planned invasive procedures Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 36
    37. 37. Treatment of HemophiliaTreatment of Hemophilia (cont.)(cont.) • Multidisciplinary approach to assist families to develop healthy coping strategies to deal with a child with a chronic illness • Difficult for parents not to be overprotective • The struggle to protect these children and still foster independence and a sense of autonomy is important therefore; allowing the child to participate in decision-making about their care and focusing on their strengths are helpful 37Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    38. 38. Safety AlertSafety Alert • Drugs that contain salicylates are contraindicated for children with hemophilia 38Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    39. 39. Platelet DisordersPlatelet Disorders • Reduction or destruction of platelets in the body interferes with the clotting mechanisms • Skin lesions common to this type of disorder – Petechiae – Purpura – Ecchymosis – Hematoma 39Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    40. 40. Idiopathic (Immunological)Idiopathic (Immunological) Thrombocytopenic Purpura (ITP)Thrombocytopenic Purpura (ITP) • Acquired platelet disorder that occurs in childhood • Most common of the purpuras • Cause is unknown but is thought to be an autoimmune reaction to a virus • Platelets become coated with antiplatelet antibody, seen as “foreign” and are eventually destroyed by the spleen • ITP occurs in all age groups, with main incidence between 2 and 4 years of age 40Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    41. 41. Manifestations of ITPManifestations of ITP • Classic symptom is easy bruising – Results in petechiae and purpura • May have recent history of rubella, rubeola, or viral respiratory infection – Interval between exposure and onset is about 2 weeks • Platelet count below 20,000/mm3 (normal range is between 150,000 and 400,000/mm3 ) – Diagnosis confirmed by bone marrow aspiration 41Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    42. 42. Treatment of ITPTreatment of ITP • Neurological assessments are a priority of care • Treatment is not indicated in most cases • If indicated, prednisone, IV gamma globulin, and anti-D antibody are some of the treatment options • In cases of chronic ITP, a splenectomy may be required • Drugs to avoid – Aspirin – Phenylbutazone – Phenacetin – Caffeine • Activity is limited during acute states to avoid bruising • Platelets are usually not given because they are destroyed by the disease process 42Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    43. 43. Complications of ITPComplications of ITP • Bleeding from the GI tract • Hemarthrosis • Intracranial hemorrhage • Prevention may be helped by immunizing all children against the viral diseases of childhood 43Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    44. 44. Risk for Development of CancerRisk for Development of Cancer • Genetic and environmental factors play a role • Exposure of the fetus to diagnostic X-rays or therapeutic irradiation for brain tumors, the use of fluoroscopy, ultraviolet (sun) exposure, and some drugs have been associated with the increase in cancer 44Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    45. 45. LeukemiaLeukemia • Most common form of cancer in childhood • Refers to a group of malignant diseases of the bone marrow and lymphatic system • Classified according to what type of WBC affected • Two most common – Acute lymphoid leukemia (ALL) – Acute non-lymphoid (myelogenous) leukemia (AMLL or AML) • Cytochemical markers, chromosome studies, and immunological markers differentiate the two types 45Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    46. 46. LeukemiaLeukemia (cont.)(cont.) • A malignant disease of the blood-forming organs that results in an uncontrolled growth of immature WBCs • Involves a disruption of bone marrow function caused by the overproduction of immature WBCs in the marrow – These immature WBCs take over the centers that are designed to form RBCs, and anemia results • Platelet counts are also reduced • Invasion of the bone marrow causes weakening of the bone, and pathological fractures can occur Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 46
    47. 47. LeukemiaLeukemia (cont.)(cont.) • Leukemia cells can infiltrate the spleen, liver, and lymph glands, resulting in fibrosis and diminished function • Cancerous cells invade the CNS and other organs – Drain the nutrients – Lead to metabolic starvation of the body 47Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    48. 48. Manifestations of LeukemiaManifestations of Leukemia • Most common symptoms – Initial phase • Low-grade fever • Pallor • Bruising tendency • Leg and joint pain – Listlessness – Abdominal pain – Enlargement of lymph nodes – Anemia severe despite transfusions • Gradual or sudden onset • As it progresses, the liver and spleen become enlarged • Skin may have a lemon- yellow color • Petechiae and purpura may be early objective symptoms • Anorexia, vomiting, weight loss, and dyspnea are also common 48Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    49. 49. Manifestations of LeukemiaManifestations of Leukemia (cont.)(cont.) • WBCs not functioning normally, increases risk of infection • Ulcerations develop around the mucous membranes of the mouth and anal regional – Gums tend to bleed 49
    50. 50. DiagnosisDiagnosis • Based on history and symptoms • Results of extensive blood tests – Demonstrate presence of leukemic blast cells in the blood, bone marrow, or their tissues • X-rays of the long bones show changes • Spinal tap may be done to check for CNS involvement • Kidney and liver function studies are done – The adequacy of their function is essential to the outcomes of chemotherapy 50Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    51. 51. Treatment of LeukemiaTreatment of Leukemia • Long-term care given whenever possible in an outpatient setting • Bone marrow suppression in chemotherapy requires family teaching for infection prevention • Adequate hydration to minimize kidney damage • Active routine immunizations must be delayed while receiving immunosuppressive drugs • Nausea and vomiting are common side effects of chemotherapy; can lead to decreased appetite, weight loss, and generalized weakness • Meticulous oral care is necessary Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 51
    52. 52. Treatment of LeukemiaTreatment of Leukemia (cont.)(cont.) • Components of chemotherapy include – Induction period – Central nervous system prophylaxis for high- risk patients – Maintenance – Reinduction therapy (if relapse occurs) – Extramedullary disease therapy • Bone marrow transplant 52Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    53. 53. Side Effects of ChemotherapySide Effects of Chemotherapy • Steroids can mask signs of infection, cause fluid retention, induce personality changes, and cause the child’s face to appear moon- shaped • Certain chemotherapy agents can cause nausea, diarrhea, rash, hair loss, fever, anuria, anemia, and bone marrow depression • Peripheral neuropathy may be signaled by severe constipation caused by decreased nerve sensations to the bowel 53Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    54. 54. Nursing Care of the ChildNursing Care of the Child with Leukemiawith Leukemia • Encourage the child to verbalize feelings – Giving permission to discuss their concerns will help clear up misconceptions and to decrease feelings of isolation • Frequently observe child for infection • Monitor vital signs and for symptoms of thrombocytopenic bleeding (a common complication of leukemia) • Meticulous mouth and skin care 54Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    55. 55. Child Receiving a BloodChild Receiving a Blood TransfusionTransfusion • Hemolytic reactions caused by mismatched blood are rare • Blood is slowly infused through blood filter to avoid impurities • Medications are never added to blood • Monitor the child for signs of transfusion reaction (most occur within the first 10 minutes of the transfusion) • Circulatory overload is a danger in children Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 55
    56. 56. Safety AlertsSafety Alerts • If a blood transfusion reaction occurs, stop the infusion, keep the vein open with normal saline solution, and notify the charge nurse • Take the patient’s vital signs and observe closely 56Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    57. 57. Hodgkin’s DiseaseHodgkin’s Disease • A malignancy of the lymph system that primarily involves the lymph nodes – May metastasize to the spleen, liver, bone marrow, lungs, or other parts of the body • Presence of giant multinucleated cells called Reed-Sternberg cells is diagnostic of the disease • Rarely seen before 5 years of age, incidence increases during adolescence and early adulthood – Twice as common in boys as in girls 57Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    58. 58. Manifestations of Hodgkin’sManifestations of Hodgkin’s DiseaseDisease • A painless lump along the neck • Few other manifestations • More advanced cases, may be unexplained low-grade fever, anorexia, unexplained weight loss, night sweats, general malaise, rash, and itching 58Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    59. 59. Criteria for Staging Hodgkin’sCriteria for Staging Hodgkin’s DiseaseDisease Stage Criteria I Restricted to single site or localized in a group of lymph nodes; asymptomatic II Involves two or more lymph nodes in area or on same side of diaphragm III Involves lymph node regions on both sides of diaphragm; involves adjacent organ or spleen IV Is diffuse disease; least favorable prognosis 59Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    60. 60. Treatment of Hodgkin’s DiseaseTreatment of Hodgkin’s Disease • Both radiation and chemotherapy are used in accordance with the clinical stage of the disease • Cure is primarily related to the stage of disease at diagnosis • Long-term prognosis is excellent 60Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    61. 61. Nursing Care of Patients withNursing Care of Patients with Hodgkin’s DiseaseHodgkin’s Disease • Mainly directed toward symptomatic relief of the side effects of radiation and chemotherapy • Education of patient and family • Malaise is common after radiation therapy, tires easily and child may be irritable and anorexic • Skin in treated area may be sensitive and must be protected against exposure to sunlight and irritation • The patient does not become radioactive during or after therapy 61Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    62. 62. Emotional Support of the PatientEmotional Support of the Patient with Hodgkin’s Diseasewith Hodgkin’s Disease • Support provided should be age-appropriate • Activity is generally regulated by the patient • Appearance of secondary sexual characteristics and menstruation may be delayed in pubescent patients • Sterility is often a side effect of treatment 62Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    63. 63. Chronic IllnessChronic Illness • Behavior problems are lessened when patients can verbalize specific concerns with persons sensitive to their problems • If they feel rejected by and different from their peers, they may be prone to depression • Nurses must develop an awareness of the adolescent’s particular fears of forced dependence, body invasion, mutilation, rejection, and loss of face, especially within peer groups • Important to recognize the adolescent’s need for self-determination Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 63
    64. 64. Developmental DisabilitiesDevelopmental Disabilities • Children with developmental disabilities may often be overprotected, unable to break away from supervision, and deprived of necessary peer relationships • The pubertal process with its emerging sexuality concerns parents and may precipitate a family crisis 64Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    65. 65. Home CareHome Care • Home health care and other community agencies work together to provide holistic care • Respite care is sometimes provided to relieve parents of the responsibility of caring for the child 65Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    66. 66. Providing Home Health CareProviding Home Health Care • Observe how the parents interact with the child • Do not wait for the child to cry out for attention • Watch for facial expression and body language • Post signs above the bed denoting special considerations, such as “never position on left side” • Listen to the parents and observe how they attend to the physical needs of the child • Don’t be afraid to ask questions or discuss apprehensions • Be attuned to the needs of other children in the home 66Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    67. 67. Care of the Chronically Ill ChildCare of the Chronically Ill Child • Focusing on what the child can do and providing successful experiences are more effective than focusing on the disability • Involvement of the entire family with the care of the chronically ill child aids in normal family interaction • Child should be integrated into rather than isolated from the community and society • The wellness of the child should be the center of the child’s life, rather than the disability Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 67
    68. 68. Facing DeathFacing Death • The nurse must understand – The grieving process – Personal and cultural views concerning that process – The views of a parent losing a child – Perceptions of the child facing death 68Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    69. 69. Facing DeathFacing Death (cont.)(cont.) • The response to a child’s death is influenced by whether there was a long period of uncertainty before the death or whether it was a sudden unexpected event 69Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    70. 70. Facing DeathFacing Death (cont.)(cont.) • The nurse must – Show compassion – Demonstrate a nonjudgmental approach – Be sensitive and effective in the provision of care – Facilitate the grief process by anticipating psychological and somatic responses while maintaining open lines of communication – Support the family’s efforts to cope, adapt, and grieve – Know that hostility is a normal response and may drive away those who do not understand its normalcy in the acute grieving process 70Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    71. 71. Self-ExplorationSelf-Exploration • How nurses have or have not dealt with their own losses affects present lives and the ability to relate to patients • Nurses must recognize that coping is an active and ongoing process • An active support system consisting of nonjudgmental people who are not threatened by natural expression of feeling is crucial 71Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    72. 72. The Child’s Reaction to DeathThe Child’s Reaction to Death • Cognitive development, rather than chronological age, affects the response to death • Children younger than 5 years of age are mainly concerned with separation from their parents and abandonment • Preschool children respond to questions about death by relying on their experience and by turning to fantasy • Children do not develop a realistic concept of death as a permanent biological process until 9 or 10 years of age 72Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    73. 73. The Child’s Awareness ofThe Child’s Awareness of His or Her ConditionHis or Her Condition • Failure to be honest with children leaves them to suffer alone, unable to express their fears and sadness or even to say goodbye 73Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    74. 74. Physical Changes of ImpendingPhysical Changes of Impending DeathDeath • Cool, mottled, cyanotic skin and the slowing of all body processes • Loss of consciousness, but hearing may still be intact • Rales in the chest may be heard, which are caused by increased pooling of secretions in the lungs • Movement and neurological signs lessen 74Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc.
    75. 75. Stages of Dying and the Nurse’sStages of Dying and the Nurse’s RoleRole • Stages – Denial – Anger – Bargaining – Depression – Acceptance • Nurse’s Role – Listen – Provide privacy – Provide therapeutic intervention – Provide information – Use appropriate phrases and open- ended statements Elsevier items and derived items © 2011, 2007, 2006 by Saunders, an imprint of Elsevier Inc. 75
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