Download class 17 chapters 21-22

Chapter 21
Hematology and Oncology Disorders
Copyright © 2012 by Saunders, an imprint of Elsevier, Inc.
Iron-Deficiency Anemia
• Description
– Anemia caused by insufficient iron in the body
– Anemia: reduction in amount and size of RBCs
or amount of hemoglobin, or both
• Signs and symptoms
– Pallor, irritability, anorexia, and a decrease in
activity
– A slight heart murmur is heard
– The spleen may be enlarged
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Iron-Deficiency Anemia
• Treatment and nursing care
– Iron, usually ferrous sulfate, is given orally 2 or 3
times a day between meals
– Vitamin C aids in the absorption of iron from diet
• Parent education
– Stress the importance of iron-fortified formula
– Infants should start solid foods by 6 months of age
– Emphasize that both dietary changes and
supplemental iron are necessary to eradicate irondeficiency anemia
– Reiterate that the condition is not uncommon; attempt
to support parents and alleviate feelings of guilt
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Sickle Cell Anemia
• Sickle cell disease (SCD) is an inherited
defect in hemoglobin formation
– Hemoglobin S, sickling type
• There are two types of sickle cell disorders
– Asymptomatic: sickle cell trait
– Severe: sickle cell disease
• Sickle cells tend to clump together;
thrombosis and obstructions are common
– These obstructions may cause infarcts, areas of
dead tissue denied proper blood supply
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Sickle Cell Trait
• The blood of the patient contains a mixture
of normal hemoglobin (A) and sickle
hemoglobin (S)
• Asymptomatic
• Genetic counseling is important; the
patient is a carrier
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Sickle Cell Disease (SCD)
• Description
– An inherited defect in the formation of
hemoglobin
– It occurs mainly in populations of African
descent
– Sickling is caused by decreases in blood
oxygen; may be triggered by dehydration,
infection, physical or emotional stress, or
exposure to cold
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Sickle Cell Disease
• Sickle cell disease
– The child inherits the abnormal gene from each
parent
– Symptoms: dactylitis, increased urination, chronic
anemia, pale, tires easily, and loses appetite
– Sickle cell crisis
• Appears acutely ill with severe abdominal pain
• Muscle spasms, leg pains, or painful swollen joints may
be seen
• Fever, vomiting, hematuria, convulsions, stiff neck,
coma, or paralysis can result, depending on the organs
involved
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Sickle Cell Disease
• Treatment and nursing care
– Bed rest
– Blood transfusions may be given for anemia
– Chelation therapy for iron overload
– Antibiotics are given to all children with fever
– Fluid intake is increased above the maintenance
level for the child’s age
– Analgesics are given for relief of pain
– Children in a severe pain crisis should receive a
continuous intravenous narcotic infusion, and
morphine is the drug of choice
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Sickle Cell Disease
• Treatment and nursing care (continued)
– Surgery
• Splenectomy is indicated in patients with multiple
splenic events
• General anesthesia places sickle cell patients at
greater risk for hypoxia
– Stress of surgery and hypoxia from anesthesia may
precipitate sickle cell crisis
• Medication
– Hydroxyurea, an antineoplastic drug, for adults
– Erythropoietin may enhance effects of hydroxyurea, but
could result in mutation of genes.
» Trials with children between ages 5-15
– Stem cell transplantation, ongoing investigations
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Hemophilia
• Description
– Blood does not clot normally, and even the
slightest injury can cause severe bleeding
– Factor VIII deficiency, or hemophilia A, is
approximately 4 times more common than
factor IX deficiency, hemophilia B
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Hemophilia
• Signs and symptoms
– The slightest bruise or cut causes extensive
bleeding
– Hemarthrosis: effusion of blood into a joint cavity
• Treatment and nursing care
– Administration of highly purified or recombinant
factor VIII concentrates to treat bleeding episodes
or anticipated bleeding episodes
– Teach the patient and family how to prevent
bleeding episodes
– Gene therapy continues to be explored
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Oncology
• Study of cancer
• Neoplastic disorders are the leading
cause of death from disease in children
over age 1
• Almost half of childhood cancers involve
the blood or blood-forming organs
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Leukemia
• Description
– A malignant disease of the blood-forming organs
of the body that results in an uncontrolled growth
of immature WBCs (blasts)
– Almost 80% of childhood cases are acute
lymphoid leukemia (ALL)
• ALL has a survival rate of ~85%
– Prognosis has many factors: age at diagnosis,
initial WBC count, structure of leukemic cells,
their reaction to chemical agents, their genetic
makeup, type of cell-surface antigens they exhibit
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Leukemia
• Signs and symptoms
– Low-grade fever, pallor, a tendency to bruise, leg
and joint pain, listlessness, and enlargement of
the lymph nodes
– Abdominal pain, often attributed to other illnesses
or even constipation, is common
– Petechiae and purpura
– Anorexia, vomiting, weight loss, and dyspnea
• Diagnosis
– Made on the basis of health history, symptoms,
and blood tests
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Leukemia
• Treatment and nursing care
– Three phases of ALL treatment: induction,
consolidation, and maintenance
– Chemotherapy
• Methotrexate is useful in maintaining remission
• Intrathecal chemotherapy is given for central
nervous system (CNS) prophylaxis
• Antibiotics are administered to prevent or control
infection, and transfusions of whole blood or
packed cells are given to correct anemia
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Leukemia
• Treatment and nursing care (continued)
– Bone marrow transplants and immunotherapy
• Bone marrow transplantation is not recommended
for children with acute lymphoblastic leukemia
(ALL) during the first remission
• It is a consideration for children with acute
myelogenous leukemia (AML) during their first
remission and for children with ALL who have had
a relapse
• Immunotherapy strengthens the immune response
of the patient to cancer cells
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Leukemia
• Treatment and nursing care (continued)
– Nursing care
• Physical/psychological needs vary in intensity
according to progression of disease
• Give patients permission to discuss their concerns,
which decrease feelings of isolation
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Leukemia
• Treatment and nursing care (continued)
– Preventing infection
• When fever occurs, broad-spectrum antibiotics are
begun until the offending agent is identified
• In most hospitals, patients are placed in a private
room for their own protection
• The nurse limits visitors and any auxiliary or
medical personnel who appear unhealthy
• Fresh flowers or plants are not permitted if the
child is neutropenic
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Leukemia
• Treatment and nursing care (continued)
– Managing bleeding
• The nurse observes the patient’s skin for petechiae
and ecchymosis
• Nosebleeds are common and are treated with
application of cold and pressure
• The nurse assesses for symptoms of hemorrhagic
cystitis and gastrointestinal (GI) bleeding
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Leukemia
• Treatment and nursing care (continued)
– Transfusions
• Platelets and packed red blood cells (RBCs) may
be given to patients with anemia and
thrombocytopenia
• Signs of transfusion reaction include chills, itching,
rash, fever, headache, pain in the back
– Tumor lysis syndrome
• Tumor cells are lysed and intracellular contents are
dumped into extracellular fluid; kidney failure can
result from trying to excrete the by-products
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Leukemia
• Additional nursing care considerations
– Elimination
• Constipation is a common side effect of chemotherapy
– Skin and hair care
• The skin should be bathed daily and whenever
necessary
• The child’s hair is combed daily and whenever
necessary; hair loss (alopecia) from drug therapy is not
unusual
– Controlling nausea and vomiting
• Monitor the child for signs of dehydration
• Administer antiemetic medications as ordered during
chemotherapy
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Leukemia
• Additional nursing care considerations
(continued)
– Nutrition
• Food may not be appealing to children with
leukemia
• A low-salt diet may be ordered during
chemotherapy cycles that include prednisone
• Oral/IV fluids may be necessary
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Wilms Tumor
• Description
– Also known as nephroblastoma
– A renal tumor arising from embryonic tissue
• Signs and symptoms
– A mass in the abdomen is discovered,
generally by the mother or by the physician
during a routine checkup
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Wilms Tumor
• Treatment and nursing care
– The abdomen should not be palpated
because trauma to the mass could release
cancer cells into the system
– Surgery
– Chemotherapy and radiation therapy after
surgery are based on the extent of the tumor
and the histologic appearance of the tumor
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Brain Tumors
• Description
– The 2nd most common type of neoplasm in children
– Most childhood tumors occur in the area of the brain
below the cerebellum
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Brain Tumors
• Signs and symptoms
– Increased ICP (headache, vomiting, drowsiness, and
seizures)
– Early-morning headache relieved by vomiting
– Nystagmus, double vision, strabismus, and decreased
vision
– Ataxia, clumsiness, head tilt, behavioral changes, and
cerebral enlargement, particularly in infants
• Treatment and nursing care
– Multidisciplinary; includes surgery, radiation therapy, and in
some cases, chemotherapy
– Phases of nursing care: diagnosis, preoperative care,
postoperative care, radiation therapy and chemotherapy,
and convalescence
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Brain Tumors
• Treatment and nursing care (continued)
– Radiation therapy
• Radiologist outlines areas to be treated
• Advise the child that he/she will be alone in the room,
but will be able to talk to others
• Avoid tape or lotion to prevent burns
• Effects include headaches, anorexia, nausea and
vomiting, diarrhea, general lethargy, leukopenia,
decreased platelet count, skin breakdown, and hair loss
• Radiation has been shown to impair intellectual
development, affect growth, and interfere with hormone
functions
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Brain Tumors
• Treatment and nursing care (continued)
– Chemotherapy
• Children must understand that the medicine is
designed to make them feel better but may make
them feel worse at first
• Adequate nutrition and hydration are important
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Bone Tumors
• Osteosarcoma
–
–
–
–
Malignant tumor of the long bones
Limping, pain, swelling, no sign of muscular injury
Requires complete surgical resection
Prognosis is poor if there are bone and lung metastases
• Ewing sarcoma
– Long bones and flat bones
– Pain, swelling, limited motion, tenderness
• Possibly fever, weight loss, respiratory distress
– Surgery, chemotherapy, radiation
– Favorable prognosis if tumor is small and nonmetatastic
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Hodgkin Disease
• Description
– A malignant disease of the lymph system that
primarily involves the lymph nodes
– The Reed-Sternberg cell is diagnostic of the disease
• Signs and symptoms
– A painless lump in the cervical area or other lymph
node site (supraclavicular, axillary, inguinal)
– In more advanced cases, there may be high spiking
fever, anorexia, weight loss, night sweats, general
malaise, rash, and itching of the skin
• Treatment and nursing care
– Both low-dose radiation therapy and chemotherapy
are used based on the clinical stage of the disease
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Non-Hodgkin Lymphoma (NHL)
•
•
•
•
Involves B and T lymphocytes
60% of lymphomas in children and adolescents
Favorable prognosis for localized disease
Staging based on number and location of tumors
– Stage I – single tumor, no lymph node involvement
– Stage IV – includes tumors in stages I-III, involvement
of CNS or bone marrow
• Chemotherapy
– Radiation is rarely used
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Self-Exploration at end of life
• Attitudes about life and death affect nursing
practice
• Coping is an active, ongoing process for
nurses
• Constructive outlets are critical for nurses
who care for dying children
• Attending a child’s funeral does not detract
from professionalism
• Crying with the family is acceptable as long
as it does not affect the care the patient
receives
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Legal and Ethical Issues Related to
Death
• Legal issues – laws
– Informed consent, role of a legal guardian, Do
Not Resuscitate (DNR) orders, organ donation,
etc.
• Ethical issues
– Relates to what is moral
– Respect for autonomy, benevolence, veracity,
nonmaleficence, confidentiality, fidelity, & justice
– Ethical principles address the unique needs of the
patient and family
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Palliative Care
• Care and comfort-giving to a dying person
– Focuses on relief of symptoms that cause distress
and detract from enjoyment of life
– The American Nurses Association Code of Ethics for
Nurses does not support euthanasia by nurses
– http://www.nursingworld.org/MainMenuCategories/EthicsStandards/Ethics-PositionStatements/Euthanasia-Assisted-Suicide-and-Aid-in-Dying.pdf
• Symptom relief interventions that risk hastening death are
acceptable (focus is symptom relief)
– Support of the patient and family is multi-disciplinary;
a team approach is most beneficial
• Physician, nurse, social worker, spiritual advisor, child life
specialist
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Child’s Reaction to Death
• Each child approaches death in an individual way,
drawing on limited experience
– Children <5 years old fear separation and abandonment
– 6-12 year olds comprehend more, have more fears
– Teens may displace complex emotions onto hospital staff
• As always when caring for children, honesty and
clarity about procedures in age-appropriate terms is
necessary
• Allow as much control as possible, but don’t offer a
choice when there isn’t one
• Encourage communication
• Many terminally ill children are aware of their
condition, even if it is carefully concealed from them
– Failure to be honest leaves them to suffer alone
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Fears of the Child
• Fear of pain
– “Whatever the experiencing person says it is,
existing whenever he says it does”
– Pain must be properly assessed and managed
– An effective dose provides comfort without
impairing functionality
• May need adjustment as disease worsens or tolerance
develops
– Complementary methods: Relaxation, distraction,
biofeedback, guided imagery
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Fears of the Child
• Fear of being alone
– Encourage parents and family to listen to
children’s concerns
– Children love hearing how they affected a
loved one’s life; they need to know they made
a difference
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Family Roles and Needs
• Encourage expression of emotions, validate family’s
feelings
• Families should be with dying children, even in ICU
• Stages of dying—Kübler-Ross (1969)
– Denial, anger, bargaining, depression, acceptance
• Religious associations can be a source of strength and
support
• Because each spouse is grieving, it is sometimes
impossible for them to support each other
– Fathers may be easily overlooked because of absence
during the day, or a need to conceal emotions from others
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Family Roles and Needs
• Cultural issues
– Nurses should familiarize themselves with
different cultural’s issues regarding death
– Possible conflicts: protecting the dying from
knowing prognosis, refusal of pain
medication, customs pertaining to afterlife
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Hospice Care
• Offered in home, at a hospice facility, or in
a hospital
• Can be life-affirming
• Source of support for families in grief
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Preparing for Death
• Wishes, dreams, and desires can be planned and
accomplished, leaving parents and family with positive
memories – Make a Wish Foundation
– With sudden death, anticipatory grieving and wish
fulfillment rarely occur
• Symptoms of death (See Box 22-3)
– Respiratory discomfort; dyspnea, “death rattle”
– GI discomfort; nausea/vomiting, anorexia, dysphagia,
dehydration, constipation
– Weakness and fatigue
– Skin complications related to decreased activity,
incontinence
• Symptoms are managed to maintain comfort; i.e.,
Pain control, bronchodilators, anxiolytics
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Care After Death
• Time of death
–
–
–
–
–
Absence of respiratory, cardiac, and neurological function
Pupils are fixed and dilated
Body temperature falls
Cool, pale skin
Loss of sphincter control; possible passage of urine/stool
• Viewing
– Nurse bathes and dresses child, cleans environment
– Sometimes parents want to bathe the body
– Parents may wish to be present when the mortician
removes the body
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Family Coping
• Anticipatory grief: Sense of loss and grief before
death
• Bereavement: Reactions during and after the
death of a loved one
• Explain that grief has no time frame and cycles
• Parents and siblings benefit from books about
death
• Memories: album, quilt square, treasure box of
mementos
• Support groups
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Reflection
• Death is normal and unique
• Nurses provide dignity, comfort, support,
guidance, and education throughout the
dying process
– The nurse may have little or no experience with
death
• Through self-reflection when feelings of
conflict arise, the nurse’s ability to identify
whose needs are being met becomes
paramount to providing quality patient care
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