Hematologic Disorders By Jeanie Ward

Iron Deficiency Anemia

Iron Deficiency Anemia Reduction in the number of RBC’s caused by: Decreased iron intake Blood loss Increased internal demands during growth periods

Iron Deficiency Anemia Manifestations: Pallor; Pale mucus membranes Enlarged spleen and heart Poor muscle tone with decreased activity Fatigue Diagnosis low hemoglobin (<11g/100mL) Low hematocrit (<33%) RBC are small and hypochromic Serum iron levels are low ( 30μg/mL)

Iron Deficiency Anemia Treatment Oral iron supplements Ascorbic acid (vitamin C) Diet high in iron Iron-fortified formula or supplements Limit cow’s milk to 24oz/day for children >12 months Increase age-appropriate iron-rich foods Why is ingestion of ascorbic acid important?

Sickle Cell Disease

Definition A group of genetic disorders characterized by production of elongated, crescent shaped erythrocytes, or abnormal form of hemoglobin, designated as sickle hemoglobin (HbS) in the place of the normal hemoglobin.

Etiology and Pathophysiology Autosomal recessive inherited disease Occurs almost exclusively among African Americans Defect of beta chain of hemoglobin Disease usually not apparent until 6 months of age – fetal hemoglobin contains a gamma, not a beta chain. Hemoglobin changes from fetal form to adult of HgbA at that time.

Normal vs Sickle Hemoglobin Sickle-shaped Hard (like piece of wood) Often get stuck in small blood vessels Lives for 20 days or less Disc-shaped Soft (like bag of jelly) Easily flows through small blood vessels Lives for 120 days

Genetics Both Parents Have Sickle Cell Trait 25% will be normal 25% will have Sickle cell Disease 50% will have the Trait

What causes the cell to sickle? Low Oxygen tension (less than 60%-70%) Low blood pH (acidosis) Increased blood viscosity (dehydration, fever, hypoxia)

Result of Sickling Process The sickled RBC’s do not move freely through the microcirculation. Blockage in vessel / blood flow halts Tissue distal to the blockage becomes ischemic Acute pain, cell destruction, tissue death

Assessment

General Manifestations Chronic anemia (Hgb. 6-9 g/dl.), pallor, weakness. Unable to do physical activities because of lack of stamina Fatigue, malaise Anorexia Jaundice Possible delayed sexual maturation Marked susceptibility to sepsis Possible growth retardation – with long bones disproportionately long.

Types of Sickle Cell Crisis Aplastic Crisis Sequestration Acute Vaso-Occlusive

Vaso-Occlusive Crisis Occurs from the pooling of many of the new sickled cells in vessels with resulting tissue hypoxia May last from one day to several weeks

Manifestations of Vaso-Occlusive Crisis Bone Pain Most outstanding symptom Mainly in the bones of the extremities and joints, but can occur anywhere Abdominal and back pain is common Related to necrosis of bone tissue Parents may notice this with refusal to move an extremity, crying out with joint movement or joint touched.

Hand and foot Syndrome Dactylitis Painful swelling of the hands and /or feet. May be the first symptom of crisis Warmth in affected areas. Fever

Cerebrovascular accident Vaso-occlusion in the brain results in cerebral infarction which causes variable degrees of neurologic damage Hemiplegia Aphasia Seizures Vision changes Headaches Alterations in level of consciousness

Acute Chest Syndrome Blockage of blood vessels in the chest leads to Pneumonia and acute chest syndrome Chest pains Fever, cough Dyspnea, retractions Leading cause of death in SCD.

Priapism Persistent painful erection of the penis occurring when penile blood flow is obstructed.

Hepatomegaly Hematuria Enlarged liver with jaundice and hepatic coma. related to damage to the kidneys Hematuria

Aplastic Crisis

Aplastic Crisis Diminished RBC production resulting in severe anemia Manifested by: Malaise, lethargy Headache Pallor fainting

Aplastic Crisis Profound anemia- low RBC count is lifelong. The average RBC life is down to an average of 10-20 days in SCA. Jaundice, elevated bilirubin Reticulocytosis Bone marrow producing 3-4 times more RBC’s than normal

Sequestration Crisis

Acute Sequestration Crisis Sickled cells become trapped in the spleen obstructing blood flow with pooling and enlargement of the spleen. Leads to shock and hypovolemia Circulatory collapse and death can occur in less than 30 minutes

Spleen Function Acts as a filter against foreign organisms that infect the bloodstream. Filters out old RBC’s from the bloodstream and recycles them Minor Role: Manufacturers RBC’s toward end of fetal life and after birth, taken over by bone marrow. Acts as a blood reservoir.

Spleen in the Infant Soft, purplish-red organ that lies under the diaphragm on the left side of the abdominal cavity Filters old blood and clears bacteria

Teach Parents How to Measure Spleen A tongue depressor can be used to measure and track spleen size Place tip on left nipple and make mark where the spleen tip is felt

Treatment for Sequestration Crisis Intravenous fluids Blood transfusion Spleen removal

Effects of Chronic Crisis States on the Child’s Body Spleen – more susceptible to infection. Gradual fibrosis and scarring with reduction in spleen activity. Asplenia. Liver – enlarged, firm, tender Brain – single episode of sickling CVA, seizures Heart – enlarges and murmurs develop Lungs – pulmonary edema and stasis Kidneys – hematuria, unable to concentrate urine Bone Infarcts – hands and feet swell.

Effects of Chronic Crisis States on the Child’s Body Eyes – bleeds in retina. Leg Ulcers Are seen in 10-15% of older children May start as simple insect bite or cut that does not heal With poor circulation develops into leg ulcer Treated with dressings, leg elevation and elastic stockings.

Diagnostic Tests CBC – low hgb and hct Hemoglobin electrophoresis – used to determine type of hemoglobin. When you pass an electric charge through a solution of hemoglobin, distinct hemoglobins move different distances, depending on their composition. This technique differentiates between normal hemoglobin (A), Sickle hemoglobin (S).

Goals in Care The is NO CURE for the disease Treat the Symptoms Relieve the pain Provide oxygenation Adequate hydration

Pain Management Give analgesics as ordered Schedule nursing care to allow for optimal rest Position joints with pillows Application of warmth Promote circulation through passive ROM

Oxygenation Assess Oxygen saturation- pulse oximetry. Should be 95% or >. Administer Oxygen for short period of time to keep saturation levels up. Bedrest to minimize energy expenditure and oxygen consumption Administer blood transfusions

What are Complications associated with frequent blood transfusions? Allergic reaction Circulatory overload Iron Overload Give Desferal, an iron-chelating agent, to decrease the iron levels

Oxygenation Prevent infection, dehydration Avoid emotional stress, overfatigue Avoid prolonged exposure to heat and cold Avoid low oxygen environment

Hydration/ Electrolyte Replacement Encourage fluid intake, at least the minimum amount of fluid required daily for that child’s weight. (1-2x maintenance) Record I & O. Monitor electrolyte balance Give parents written instructions on specific amount of daily intake needed. Assess signs of dehydration (decreased urination, thirst) Teach parents measures to prevent dehydration such as avoiding heat / stress.

Prevent Infection With the spleen damaged, has greater chance of getting sepsis. Avoid know sources of infection Assess Vital Signs and report elevated temperatures. Treat with Ibuprofen, NOT ASA. Place on prophylactic antibiotics such as Penicillin VK 250 mg twice a day, through age 5 yrs

Prevent Infection Immunizations Hepatitis B series Recipient of blood and blood products. Pneumococcal Pneumovax for now at age 24 months and 5 years, with an improved vaccine coming on the market soon for administration to infants. Meningococcal vaccine H. influenzae vaccine

Child / Parent Teaching Assess baseline knowledge and teach accordingly: Causes of disease and consequences, genetic counseling Situations that cause sickling Signs of developing crisis, infection, When to call the doctor Keep in school, promote normal g&d as much as possible Allow for decreased endurance - let the child set his or her own pace during strenuous exercise, and to take rest breaks when fatigue Explain some complications: CVA, anemia, swollen spleen, liver problems Support child and family

Critical Thinking Which of the following nursing diagnoses should receive priority during a vaso-occlusive crisis in a 14 year old with sickle cell anemia? A. Alteration in comfort B. Ineffective individual coping C. Decreased cardiac output D. Ineffective airway clearance

Critical Thinking Which of these instructions should the parents of a child who has recovered from a sickle cell crisis receive? A Isolate child from known sources of infection B Avoid contact with all children C Restrict child’s intake during the night D Reinforce the basics of trait transmission

Hemophilia

Hemophilia A group of bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation of the blood. Hemophilia A – most common bleeding disorder. Deficiency of factor VIII. Hemophilia is an hereditary condition in which the mother may pass the defect to her male child. With deficiency of coagulation factor VIII, blood does not coagulate as it should.

Do Hemophiliacs bleed more Profusely than people without Hemophilia?

Answer: The blood of a person with hemophilia does not clot normally. He does not bleed more profusely or more quickly than other people; however, he bleeds for a longer time.

Hemophiliacs bleed mainly from minor cuts True or False

False This is a myth. External wounds are usually not serious. Far more important is internal bleeding (hemorrhaging). These hemorrhages are in joints, especially knees, ankles and elbows; and into tissues and muscles. When bleeding occurs in a vital organ, especially the brain, a hemophiliac's life is in danger.

What Causes a Bleeding Episode? Bleeding is often caused by minor injury - a bump or a slight twist of a joint. However, many hemorrhages, especially among severe hemophiliacs, happen for no apparent reason. This is even truer in joints that have bled often in the past. The more a joint has bled, the easier it bleeds again with no external cause. Even hemorrhages in the brain often have no apparent cause. Brain hemorrhages are the leading cause of death from bleeding in hemophilia. Therefore it is important to recognize the symptoms of a brain hemorrhage very quickly.

Severity Related to the deficiency of AHF

Interviewing the Child with Hemophilia – Subjective Data Recent traumas and measures used to stop bleeding Length of time pressure was applied before bleeding subsided Was swelling increased after surface bleeding subsided Did swelling and stiffness occur without apparent trauma

Classic Sign - Bleeding Assessment Prolonged bleeding anywhere from or in the body. Often diagnosed when infant is circumcised Bleeding in the mouth from a cut, bitten tongue or loss of a tooth (especially in children) Classic Sign - Bleeding

Assessment Surface bruising - Bleeding from trauma into soft tissues and muscles (the ileopsoas muscle around the hip, calf, forearm, upper arm, achilles tendon, buttocks, retroperitoneal. Hematuria Hemarthrosis - bleeding into joints (knees, elbows, ankles, shoulders, hips, wrists in descending order of frequency)

How to Assess Bleeding in a Joint The first sign is a feeling of tightness in the joint but no real pain. The joint feels a little puffy to the touch. As the hours pass, the joint becomes hot to the touch. Fully flexing or extending the joint becomes painful. Weight bearing becomes difficult. By this time, the joint is visibly swollen. As the bleeding continues and the swelling increases, all movement in the joint is lost. The joint becomes fixed in a slightly flexed position in an attempt to relieve the interior pressure in the joint. Pain at this point can be excruciating. The bleeding slows after several days when the joint is so full of blood that the pressure inside the joint cavity is equal to the pressure inside the broken blood vessels. Slowly, the bleeding stops and the long process of absorbing the blood in the joint cavity begins.

Assessment Signs and Symptoms of CNS bleeds CNS bleeding - Major cause of death Signs and Symptoms of CNS bleeds Persistent or increasing headache Repeated vomiting Sleepiness or a change in normal behavior Sudden weakness or clumsiness of an arm or leg Stiffness of the neck or complaints of pain with neck movement Complaints of seeing double The development of crossed eyes Poor balance when walking, a lack of coordination Convulsions or seizures

Assessment of Child Bleeding in the mouth Hemarthrosis Surface bruising

Diagnostic Tests DNA testing for the trait Blood Tests Partial Thromboplastin Time (PTT) – Prolonged Bleeding time – Prolonged > 2 hrs. Platelet count and Prothrombin time – Normal Low levels of Factor VIII

Goals Prevent and control Bleeding Prevent crippling effects / Decrease pain Child/ Parent Teaching

Interventions to Prevent or Control Bleeding The basic treatment to stop or prevent bleeding in people with hemophilia A is: Give Factor VIII replacement therapy via IV. These concentrates come from two sources: human plasma (a component of blood), Fresh whole blood, fresh or frozen plasma may be used. a genetically engineered (artificial) Factor VIII blood product made by DNA technology.

In both cases, the Factor VIII protein is nearly identical to the protein which is lacking in the blood of hemophiliacs. After an infusion of the concentrate, all the proteins needed for clotting are in place. A hemophiliac's blood becomes "normal", at least for a few hours. This allows the time for a clot to form at the site of the damaged blood vessel. Unfortunately, the replacement of the missing clotting factors is not permanent. Half of the clotting factor activity which was infused is removed by the body every 24 hours. This means that within 3 days almost none is left. The hemophiliac's blood is again unable to clot normally.

Advantages of Factor VIII concentrate They are very concentrated. A small amount contains enough Factor VIII to control bleeding, even in major surgery. Thus, they are very effective. They are convenient. The concentrates can be stored in a home refrigerator for up to a year or kept at room temperature for 3 to 6 months. They are easy to mix. Five to 10 ml. of sterile water is added to the powdered concentrate. One minute later the mixture is ready to be infused. They are quick to infuse. From beginning to end, the infusion takes about 15 to 20 minutes. They are very safe. Hepatitis is rarely, if ever, transmitted by the concentrates made from human plasma and the concentrates made by genetic engineering are even safer because they are not made from human plasma, which can carry blood-borne infections.

Additional Interventions to Prevent and Control Bleeding Administration of DDAVP (desmopressin acetate) nasal spray used to stimiulate release of factor VIII. Used with mild hemophilia Apply local pressure for 10-15 minutes Elevate joint above level of heart Apply cold compresses to promote vasoconstriction Prophylactic administration of factor VIII

What is prophylactic vs. on-demand therapy? In prophylaxis therapy, hemophiliacs receive factor concentrates several times a week to prevent bleeding. The goal is to keep the levels of Factor VIII or IX in the blood high enough that bleeding does not happen. This therapy is common with children. On-demand therapy is the infusion of factor concentrates immediately after the beginning of a bleed. The goal is to stop the bleeding quickly, before any damage is done to the joint or muscle. Research has shown that prophylaxis therapy gives children the best chance to reach adulthood without damage to their joints.

Interventions to Prevent Crippling effects / Decrease Pain During bleeding episode—elevate joint and immobilize ROM after acute episode Exercise unaffected joints and muscles. Give analgesics before Physical therapy. Do NOT give Aspirin. Watch diet/ weight – excessive weight stresses the joints

What is the crippling Effect of Repeated bleeds into a Joint? Bleeding in joints, especially knees, ankles and elbows can lead to: loss of range of motion muscle loss destruction of the joints themselves

Child / Parent Teaching Measures to prevent injury/ providing a safe environment Early recognition of bleeding episodes Keep current on immunizations Hepatitis B – because recipient of blood and blood products

Child / Parent Teaching How to administer factor VIII. Children often learn how to infuse themselves at the age of eight or ten. Then, the hemophiliac is able to treat himself at home, at school, at camp or on vacation.

Critical Thinking A 12 year old hemophiliac child has been admitted to the hospital for hemarthrosis. Which of these expected outcomes should receive priority in the child’s care? A. Family will receive genetic counseling B. Child will participate in appropriate activities for present condition C. Child and family will seek support from National Hemophilia Foundation D. Maximum function of the joint will be restored

Review Following administration of factor VIII to a nine year old child admitted to the hospital with hemarthrosis, the nurse’s next action would be to: A. Elevate and immobilize the affected joint B. Institute passive range of motion to the affected joint during the acute phase C. Apply warm compresses to the affected joint D. Apply pressure to the area as needed

Childhood Cancers

Cancer Leading cause of death from disease in children Almost all childhood cancers involve blood or blood-forming tissues

Types and Incidence of Cancers

Childhood Cancers Brain tumors – second leading cause of death from childhood cancer. Most are cerebellar and brain stem tumors Lymphomas Non-Hodgkins lymphomas—one-third present with a mass in the neck or mediastinal area. Also have dyspnea, wheezing, abdominal mass or pain and lymphadenopathy. Hodgkin’s disease – arises in single lymph node with painless nodal enlargement, followed by extension to adjacent nodes and into spleen, liver, lungs, bone marrow. Neuroblastoma – malignant tumor arising from sympathetic NS ganglion cells outside the cranium and and can arise from anywhere along the sympathetic nervous system chain. Can also occur in retroperitoneal area, pelvis, neck.

Wilms Tumor – solid tumor of kidney. Rhabdomyosarcoma—malignant tumor of the striated muscle cells. occur in muscles around eye, head, neck, extremities, GU system. Retinoblastoma – intraocular malignancy of the retina of eye. Usually unilateral. If bilateral , hereditary. First sign is white pupil. Others – osteogenic sarcoma/ Ewings sarcoma – tumor of bones of the trunk. Often seen in adolescence growth spurt. Found in distal femur, proximal tibia.

Theories of Etiology Intrauterine carcinogens Physical carcinogens Viruses Immune defects Genetics Discovered gene for leukemia on chromosome 22

Warning Signs of Childhood Cancer C = continual unexplained weight loss H = headaches with vomiting (early morning) I = increased swelling of pain in joints L = lump or mass D = development of whitish appearance in pupil R = recurrent or persistent fevers, night sweats E = excessive bruising or bleeding N = noticeable paleness or tiredness

Diagnostic Tests Biopsy Blood Tests CBC Uric Acid Bone Marrow Aspiration PET, SPECT MRI, CT, ultrasound

Interventions Radiation therapy Chemotherapy Surgery Bone Marrow and Stem cell transplantation

Radiation Therapy Changes the DNA component of a cell nucleus The cell cannot replicate which Inhibits further cell division and growth

Effects of Radiation Therapy Radiation sickness- anorexia, nausea, vomiting Treated with antiemetics (Zofran or Anzimet). Cool cloth to forehead, provide distraction, accurate I&O. Fatigue allow for naps an rest periods (coordinate care), encourage parent to cuddle in bed with child, pillow, blankets, favorite toys Skin reactions –erythema, tenderness

Bone marrow suppression – anemia, neutropenia, thrombocytopenia May be on reverse isolation Mucositis- inflammation of mucus membranes mainly the mouth Offer soft foods, and cold foods. Frequent mouth care. Lidocaine oral to swish in mouth (older child) Long term – depends on part of body receiving radiation

Chemotherapy There are several categories of antineoplastic drugs used in treating childhood cancers. Scheduled at set times and days and by different predetermined routes. May remain in hospital for few days at first, then later report on specific day for therapy. Children and Parents must be taught about what to do and not to do during therapy.

Side effects and Toxic Reactions to Chemotherapy Bone Marrow Suppression – neutropenia, anemia, thrombocytopenia Place in reverse isolation, keep anyone exposed to a virus away from patient. Monitor temperature Should not receive live-virus vaccines Bleeding Tendency (thrombocytopenia) Apply pressure to puncture site No contact sports Check urine and stool for blood Give stool softeners. WHY? Soft objects in mouth

Nausea, vomiting, diarrhea, anorexia Malaise and fatigue Encourage video games, movies, etc Allow visits from friends Nausea, vomiting, diarrhea, anorexia Give anti-emetics Small frequent meals Monitor for dehydration Altered mucous membranes Stomatitis Rectal ulcerations

Side effects of chemotherapy Renal involvement Uric acid levels rise as a result of breakdown of cells. The renal tubules causing renal failure. If kidney affected/damaged- chemo drugs will not be excreted as usual and may limit drugs given. Body Image changes Alopecia Pain

Nursing Responsibilities Related to Chemotherapy Know OSHA guidelines for administering antineoplastic agents Confirm all measurements and calculations Double-check ordered dosages Obtain complete blood count within 48 hours of chemotherapy administration Note white blood cell and platelet levels before chemotherapy begins Know side effects of chemotherapeutic agents and ways to alleviate these effects

Nursing Responsibilities Related to Chemotherapy (Cont’d) Ensure patency of IV tubing by checking for blood return Ensure needle placement for implantable infusion device Give vesicants (agents that can cause tissue necrosis) only through a fresh IV site Have emergency drugs available

Review of Common Side Effects of Chemotherapy and Radiation Bone marrow suppression Alopecia Malaise/fatigue Nausea Vomiting Anorexia Stomatitis Radiation side effects Skin reactions Fatigue Bone marrow suppression Nausea Vomiting Anorexia Mucositis

Surgery Curative Remove the tumor and cancerous tissue Palliative Relieve complications due to the cancer

Bone Marrow and Stem Cell Transplantation The goal of therapy is to administer a lethal dose of chemotherapy and radiation therapy that will kill the cancer and then re-supply the body with bone marrow and stem cells to reconstitute immunologic function. Healthy bone marrow or stem cells are infused into the bloodstream and migrate to the marrow space to replenish the patient’s immunologic function and help kill remaining cancer cells.

Types of Transplantations Syngeneic bone marrow comes from identical twin Allogeneic bone marrow comes from matched sibling (one in four chances) or someone who is histocompatible. Autologous own bone marrow. May be harvested at time of remission in preparation for relapse or when bone marrow is free of malignant cells. Also being used so toxic doses of chemotherapy and radiation can be administered and the bone marrow rescued.

Procedure First --All potential donors are typed for HLA (human leukocyte antigen) compatibility. Collection of bone marrow is a surgical procedure. The donor undergoes anesthesia for aspiration of the bone marrow The bone marrow is then processed and frozen When patient ready - it is infused into the recipient.

Where is the most common site for bone marrow aspiration in children? Iliac crest

Side effects of Transplantation 1. Graft-Versus-Host Disease (GVHD) – potentially lethal immunologic response of donor T cells against the tissue of the recipient. Signs and symptoms – rash, malaise, high fever, diarrhea, liver and spleen enlargement. Because there is no cure, prevention is essential. Careful tissue typing, irradiation of blood products which helps to inactivate mature T lymphocytes. 2. Rejection of the transplant

Post Therapeutic Disabilities CNS – cognitive disorders, seizures, headaches, coordination problems Bone – asymmetric growth of bones, easy fractures, scoliosis, kyphosis CV – cardiomyopathy (pericardial thickening) , pericardial damage Respiratory – pneumonitis, pulmonary fibrosis GI – enteritis, bleeding, hepatic fibrosis Urinary – hemorrhagic cystitis, reflux Endocrine – decrease in growth, thyroid and gonadal dysfunction Reproductive – decrease sperm Dental - caries Secondary malignancies 

Ask Yourself? What is the most common form of childhood cancer? a. leukemia b. brain tumors c. lymphoma d.osteosarcoma

Leukemia

What is it?

Leukemia A malignancy of the blood forming tissues/ bone marrow in which normal bone marrow is replaced by malignant immature WBC’s.

Pathophysiology of Leukemias The WBC's are produced so rapidly that immature cells (blast cells) are released into the circulation. These blast cells are nonfunctional, can't fight infection, and are formed continuously without respect to the body's needs The blasts cells then invade other organs and interfere with metabolism / function. The production of red blood cells and platelets decreases leading to anemia and thrombocytopenia.

Leukemia

Assessment

Signs & Symptoms Bone marrow Depression results in: Decrease in mature WBC’s - fever Decrease RBC’s, Anemia- pallor, lethargy, anorexia Decreased Platelets/ thrombocytopenia-  bleeding Increase cell metabolism which deprives cells of nutrients Enlargement of organs infiltrated with blast cells results in: Bone pain Spleenomegaly. Hepatomegaly, Nephromegaly Lymphadenopathy CNS infiltration – increased ICP

Diagnostic Tests History and Physical Blood Work Leukocytes are > 10,000 Platelet count, Hgb and Hct low Blast cells appear (where they normally don’t) Bone Marrow Aspiration Used to identify the type of WBC involved, therefore, type of leukemia X-rays of long bones Show lesions caused by invasion of abnormal cells Lumbar Puncture – blast cells in the CSF

Chemotherapy is the most common treatment for Leukemia. Why?

Chemotherapy A combination of antineoplastic drugs are given for about a month A different combination is given for about 2-3 years Advantages of using a combination of drugs: Decrease resistance to one drug Lessening of severe side effects of massive doses of one drug Breakdown of the tumor cell cycle at multiple sites

Chemotherapy Phases 1. Remission induction- most intense treatment. Large doses of antineoplastic drug administered in an effort to destroy as many proliferating cells as possible. Lasts 4-6 wks. About 95% respond. 2. Consolidation- method of destroying leukemic cells in the CNS- for children who have CNS involvement or are high risk. Given Intrathecal. 3. Remission maintenance- drugs given at specific intervals. If remain in remission for 3 yrs, treatment is discontinued. Approx 80% of children who sustain remission for 2-3 yrs continue to remain in remission and appear to be cured.

Administration of Chemotherapy When is intrathecal administration of chemotherapeutic medications required? Intrathecal chemotherapy is instilled in to the spinal canal for cancers that have metastasized to the brain

What is the rationale for the use of cranial radiation in addition to chemotherapy? Bypass the blood brain barrier

Nursing Care Prevent infection (neutropenia, anemia) Pain Relief Nausea and vomiting Mouth discomfort-mucositis / stomatitis Fatigue Alopecia Prevent blood loss-platelet low- nose bleed most common kind of bleed Support child and family Assist with referrals to social services, home health agency, chaplain

Nursing Care Community Resource American Cancer Society Candlelighters Childhood Cancer Foundation® (CCCF) is a national non-profit membership organization whose mission is to educate, support, serve, and advocate for families of children with cancer, survivors of childhood cancer, and the professionals who care for them. http://www.candlelighters.org/ American Cancer Society Make a wish Foundation Leukemia Society Church and Schools

Tumor Lysis Syndrome Caused by the breakdown (lysis) of malignant cells which release intracellular contents into the blood. Intracellular electrolytes overload the kidneys and can lead to kidney failure. Further severe electrolyte imbalances cause metabolic acidosis, hyperkalemia, increase uric acid levels, hypocalcemia and cardiac arrythmias. Treat with IV fluids, electrolyte replacement, allopurinol.

An embryonic tumor of the kidney. Wilm’s Tumor An embryonic tumor of the kidney.

Etiology and Pathophysiology Cause is unknown Originates from immature renoblast cells Tumor is vascular

Assessment Palpable abdominal mass Firm and smooth **The abdomen should not be palpated once the diagnosis is made. Avoid palpating the tumor mass during assessment because of the risk of rupturing the protective capsule. Excessive manipulation can cause seeding of the tumor and spread of cancerous cells Abdominal distention Fever Fatigue Late signs Anemia Hematuria, dysuria Hypertension

Diagnostic Tests Abdominal ultrasound CT, MRI Biopsy

____________ __ ______ ___

Interventions Nephrectomy and removal of lymph nodes Post-op chemotherapy and / or radiation therapy CT every 6 months for 3 years Chest x-ray every 3 months for 3 years

Nursing Care Pre-op Post-op Sign on bed ”Do Not Palpate Abdomen” Child / Parent teaching Post-op Monitor kidney function, Strict I & O Monitor vital signs—B/P and temperature Monitor GI function– assess bowel sounds and stool production NG tube to drainage. Measure abdominal girth.

Clinical manifestations Treatment sequence Ewing’s Sarcoma Clinical manifestations Treatment sequence How does this treatment differ from osteosarcoma? Why is metastases more of a concern with this cancer? Ewing's Sarcoma- malignant, small, round cell tumor usually involving the diaphyseal (shaft) portion of the long bones. (femur, pelvis, tibia, fibula, ribs, humerus, scapula, clavical) pain, edema, fever and elevated WBC count, elevated erythrocyte sedimentation rate, and elevated c-reactive protein. May present with fractures of affected bone resulting from atypical trauma (wouldn't normally cause a broken bone) 1) chemotherapy to reduce the tumor   2) surgical removal of the entire bone or invasive high-dose radiation of the entire bone 3) chemotherapy is always used after initial treatment as undetectable metastases are nearly always present Undetectable masses are almost always present

Clinical manifestations- Treatment sequence- Rabdomyosarcoma Clinical manifestations- Treatment sequence- Chemotherapeutic agents (VAC) Rabdomyosarcoma is the most common muscle cancer in children. What is the sequence of treatment for this cancer? 1) surgery followed by 2)wide-field radiation and chemotherapy What are the most common chemotherapeutic agents used to treat this type of cancer? Vincristine, Actinomycin, Cyclophosphamide (VAC therapy)

Clinical manifestations Treatment sequence Osteosarcoma Clinical manifestations Treatment sequence Why is the sequence of treatment important? Disease Clinical manifestations Treatment Osteosarcoma- rare malignant bone tumor usually located in large bone (femur, tibia, or humerus) Pain and edema to area surrounding malignancy. Pain may refer to hip or back pulmonary metastasis very common 1-Chemotherapy is started before surgery to shrink the tumor   2-surgical removal of tumor (limb-salvage to remove tumor or limb amputation) followed by 3-aggressive chemotherapy to prevent metastasis

Review of Nursing Care: Pre-operative nursing goals for the child diagnosed with cancer- Disturbed sensory perception Anticipatory grieving Risk for infection Post-operative nursing goals- Impaired skin integrity Impaired adjustment- disturbed body image Pain Pre-operative nursing goals: Disturbed Sensory Perception (visual) related to altered acuity- tumor interfering with visual field and damage to nerves Anticipatory grieving (family and child) related to potential loss of significant person Knowledge deficit R/T disease process & treatment modality Risk for infection- related to inadequate defenses following chemotherapy What areas of post-operative assessment require priority attention from the nurse? risk for infection, impaired skin integrity, impaired adjustment, disturbed body image, pain

Review of Nursing Care: What is the rationale for treating some cancers with chemotherapy prior to removal of the tumor? What specific teaching should the nurse include as priority regarding chemotherapy? What lab values does the nurse monitor carefully in the client undergoing chemotherapy? Review care of a child during chemotherapy and radiation therapy. a. What is the rationale for treating some cancers with chemotherapy prior to removal of the tumor? to reduce the size of the tumor and prevent seeding (spreading or metastasis of the tumor throughout the body b. What specific teaching should the nurse include as priority regarding chemotherapy? the child will have suppressed immune system and must be protected from infection. - keep away from individuals or situations in which illnesses may be transmitted or the child may come in contact with pathogens (shopping, zoo, family reunions- public restrooms…) c. What lab values does the nurse monitor carefully in the client undergoing chemotherapy? RBC's and WBC's- low white blood cells indicate a need to hold chemotherapy treatment and contact the oncologist .

Review of Nursing Care: What are the priority psychosocial needs of the child/family diagnosed with cancer What are nursing interventions to assist with stressors that influence the child/family’s response to the diagnosis and treatment of cancer. What are the priority psychosocial nursing interventions for the client/family with the diagnosis of cancer? What emotional stressors surround the child/family? fear of loss of the child, guilt if treatment was delayed for any reason, anger at disease process What financial stressors surround the child/family? Treatment, surgery and numerous hospitalizations & medical care are extremely expensive. Parents may be required to miss work to accommodate treatment plan. What social stressors surround the child/family? Separation from extended family and friends as they attempt to protect the child from pathogens. Isolation as they begin to grieve the potential loss of a child. Important to get the family into outreach groups and support groups ASAP to assist with these stressors.  

Death and Children Caring for a child who is dying can be one of the hardest tasks in nursing

Understanding of Death Children under 3 Have no understanding of own impending death May perceive family anxiety, sadness Preschool to 5 years More afraid of separation from parents than of thought of dying. Greatest fear is separation. Envision death as temporary, and have little of adults’ fear of it Think of it as a long sleep, not a final process. Nightmares increase. May feel pain / illness is a punishment for misdeeds or thoughts May ask questions about death In long term illness – may simulate adult response with depression, withdrawal, fearfulness, anxiety

School Age 5-9 Begin to understand that death is permanent May think it is something that only happens to adults Become aware of what is happening to them when their disorder has a fatal prognosis. Concerns center around fear of pain, fear of being left alone and leaving parents and friends. May associate death with sleep and may be afraid to go to sleep without someone near them. May associate death with darkness—want light left on in room

Understanding Death Adolescent, older school age By age 10 have an adults concept of death, realizing that it is inevitable, universal, and irreversible. Have more understanding than adults realize. Understand that death is the cessation of life. Emotional outbursts may reflect anger View death as fearsome and fascinating (increase in adolescent suicide). May feel immune to death and deny symptoms for longer than usual because they believe it is impossible that anything serious could happen to them. Some adolescents consider themselves alienated from their peers and unable to communicate with their parents for emotional support feeling alone in their struggle.

Nursing Care Child Elicit child's understanding of death before discussing Encourage children to express feelings in own way through play, drawings, or verbalization to promote free expression. Provide a safe, acceptable outlet for expressions of feelings Structure care of child to allow child choices and participation in process within constraints of physical condition Help child maintain independence and control; normal ADL as much as possible (set realistic goals) Realize that they will go through the stages of dying: denial, bargaining, anger, depression, acceptance

Nursing Care Parents Spend time with family to listen, answer questions, and provide information. Discuss issues with parents before discussing with child. Provide opportunities for family to express their emotions and deal with their feelings. Parental reactions: continuum of grief process and usually depend on previous experiences with loss, intellectualization. Reactions may depend on relationship with child and circumstances of illness or injury Reactions depend on degree of guilt felt by parents-help them sort out

Assist parents in expressing fears, concerns, grief to enable them to appropriately support child Assist parents to understand sibling' possible reactions to terminally ill child ·   Guilt- believing they caused the problem or illness ·  Jealousy- wanting equal attention from parents ·   Anger- feelings of being left behind Support, enhance parent-child communication, enhance parents' ability to support child Refer to parent, family support groups- not alone, help focus, open communication, provide information

Nursing Care Nurse Nurse needs to care for self. Care of the caregiver is imperative if the nurse is to provide physical and psychosocial care for families at such a difficult time. Caring for dying children and their families can be stressful and emotionally demanding.