chapter 21 hematology and oncology disorders copyright © 2012 by saunders, an imprint of elsevier,...

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

Copyright © 2012 by Saunders, an imprint of Elsevier, Inc. 21-2

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 iron-deficiency anemia

– Reiterate that the condition is not uncommon; attempt to support parents and alleviate feelings of guilt


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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


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-Position-

Statements/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


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


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


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


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


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


Hospice Care

• Offered in home, at a hospice facility, or in a hospital

• Can be life-affirming

• Source of support for families in grief


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


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


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


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


chapter 21 hematology and oncology disorders copyright © 2012 by saunders, an imprint of elsevier,...

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