[Exam 4] Chapter 46 – Alteration in Cellular Regulation/Hematologic or Neoplastic Disorder Flashcards
Variations in Anatomy/Physiology, RBC Production: When does this begin in embryo?
8 Weeks gestation. Formed in liver. `
Variations in Anatomy/Physiology, RBC Production: Where is EPO produced at first?
At the liver in the fetus before the kidney eventually takes over
Variations in Anatomy/Physiology, Hemoglobin: What types present in blood?
Hgb A, Hgb F, and Hgb A2. After six months, A2 is predominant one.
Variations in Anatomy/Physiology, Hemoglobin: Fetal hemoglobin puts infant at risk for what?
Anemia, and leading to problems with oxygen-carrying capacity of blood.
Variations in Anatomy/Physiology, Iron: How is iron recieved?
From mother through placenta. Causes anemia within first 2-6 months.
Variations in Adult/Child Cancer: Pediatric cancers arise from what?
Primitive ebryonal (mesodermal) and neuroectodermal tissues, resulting in leukemias, lymphomas, and carsomas.
Variations in Adult/Child Cancer: Where do adult cancers arise from?
Epithelial cells, resulting in carcinomas.
Variations in Adult/Child Cancer: Most common childhood cnacers?
Leukemia, CNS tumors, Lymphoma, neuroblastoma, and Wilms Tumor.
Variations in Adult/Child Cancer: Warning signs of cancer for chilren include?
Blood cell production changes or as a result of compression, infiltration, or obstruction caused by tumor.
Variations in Adult/Child Cancer: Changes in blood cell production may result in what?
Fatigue, pallor, frequent or severe infection and easy bruising.
Variations in Adult/Child Cancer: Cancer usually affects what in children vs adults?
tissues vs organs
Variations in Adult/Child Cancer: Most common sites for adult/child?
Child: Blood, Lymph, Brain, Bone
Adult: Breast, Lung, Prostate, Bowel
Common Medical Txs: What is a clinical trial?
Carefully designed research study that assessess the effectiveness of a treatment as well as its acute and long-term effects on the child
Common Medical Txs: What may a clinical trial include?
Existing medications or treatments in combination with new drugs.
Common Medical Txs, Cancer: Where should child be treated to ensure optimal outcome?
Institution with multidisciplinary cancer care specialists that provide the most advanced care available
Common Medical Txs, Cancer: What is commonly used to treat childhood cancers?
Chemotherapy and radiaiton therapy
Hemoatopoietic stem cell transplantation
Common Medical Txs, Cancer: How does Leukaphresis work?
Whole blood is removed from body, WBCs extraced, and then blood is retransfused into child. For those with WBC > 100,000
Common Medical Txs, Cancer: How does a bone marrow transplant work?
Transfer healthy bone marrow into child with disease. Develops into fuctional cells
Common Medical Txs, Cancer: How does stem cell transplant work?
Removed from donor via apheresis, or stem cells retrieved from umbilcal cord. Then transpllanted into the recipient
Common Medical Txs, Cancer: Why would supplemental oxygen be prescribed?
Hypoxia associated with sickle cell crisis or severe anemia
Common Medical Txs, Cancer: Why would splenectomy occur?
For life-threatening or recurrent splenic sequestration of sickle cell disease
Common Medical Txs, Cancer: How does Radiation therapy work?
Ionizing radiation delivered to cancerous area. Damages cells in locally treated area.
Common Medical Txs, Cancer: Why are central venous catheters used?
Used for adminsitering medications, TPN, or blood productions
Common Medical Txs, Cancer: What is an implanted port?
Needle-accessible port implanted underskin. Has a thin catheter exisitng it that is tunneled into superior vena cava
Common Medical Txs, Cancer: What does Defrasirox do?
Binds with iron, which is removed in feces. Helps with iron toxicity
Common Medical Txs, Cancer: What does Deferoxamine do?
Binds with iron, which is removed in kidneys
Common Medical Txs, Cancer: Why is Factor (VIII or IX) replacement used
REplaces deficient clotting factors HEmophilia
Common Medical Txs, Cancer: What does Hydroxyurea do?
Stimulates development of hemoglobin F in sickle cell anemia
Common Medical Txs, Cancer: Why are chelating agents: Dimercaprol, edetate calcium disodium used?
Remove lead from soft tissues and bone, when lead > 45
Common Medical Txs, Cancer: What doe Allopurinol do?
Decreases production of uric acid
Common Medical Txs, Cancer: What do colony-stimulating factors: darbepoetin alfa do?
Stimulate production of RBC or granulocytes.
Common Medical Txs, Cancer - Chemotherapy: What are the five stages of the cell cycle?
G0 Phase G1 Phase S Phase G2 Phase M Phase
Common Medical Txs, Cancer - Chemotherapy: What occurs in G0 phase?
Resting phase. Lasts from few hours to few years. Cells not dividing
Common Medical Txs, Cancer - Chemotherapy: What occurs in the G1 phase?
Cell makes more protein in preparation for dividing, lasts 18-30 hours
Common Medical Txs, Cancer - Chemotherapy: What occurs in S Phase?
Chromosomes are copies, so that newly formed cells have approrpiate DNA. Lasts 18-20 hours
Common Medical Txs, Cancer - Chemotherapy: What occurs in G2 phase?
Just before cell splits into two cells, lasts 2-10 hours
Common Medical Txs, Cancer - Chemotherapy: what occurs in M Phase?
Mitosis, actual splitting into two new cells. 30 minutes to 1 hour
Common Medical Txs, Cancer - Chemotherapy: Cells most affected by chemotherapy occurs where?
Those in bone marrow, digestive tract, reproductive ssytem and hair follicles
Common Medical Txs, Cancer - Chemotherapy: Adverse effects with this?
Immunosuppression , infection, myelosuppresion, N/V, constipation, alopecia and pain
Common Medical Txs, Cancer - Chemotherapy: Long term complications?
Microdontia, missing teeth, hearing and vision changes
Altered growth
Common Medical Txs, Cancer - Chemotherapy: What is an adjunct therapy to decrease N/V and aversion to chemotherapy?
Acupuncture
Common Medical Txs, Cancer - Radiation Therapy: What type of radiaiton used?
Either gaamma or particle form.
Common Medical Txs, Cancer - Radiation Therapy: Adverse effects of this?
Fatigue, N/V, Oral Mucositis, and Alterations in skin integrity.
Common Medical Txs, Cancer - Radiation Therapy: Long term complications of this?
Alteration in growth, hormone dysfunction, hearing/vision alterations, learing problems, and pulmonary fibrosis
Common Medical Txs, Cancer - Hematopoietic Steem Cell Transplant: What is this procedure?
Hematopoietic stem cells are infused IV into child. Follows a period of purging of abnormal cells through chemotherapy. Transplanted cells migrate to the empty spaces in bone marrow.
Common Medical Txs, Cancer - Hematopoietic Steem Cell Transplant: What cancers is this used for?
Leukemia, brain tumors, neuroblastoma and solid tumors
Common Medical Txs, Cancer - Hematopoietic Steem Cell Transplant: What is autologous HSCT ?
Achieved through harvest and treatmetn of the child’s own bone marrow, followed by infusion of treated stem cells.
Common Medical Txs, Cancer - Hematopoietic Steem Cell Transplant: What is Allogenic HSCT?
Transplantation using stem cells from other individual that are harvested from bone marrow. REquires human leukocyte antibody mathching.
Common Medical Txs, Cancer - Hematopoietic Steem Cell Transplant: Complications of HSCT?
infection , electrolyte imbalance , bleeding, ,organ and mucous toxicities.
Common Medical Txs, Cancer - Asssessment: What are the first signs of trouble?
Skin color changes such as pallor, bruising and flushing.
Common Medical Txs, Cancer - Health Hx: Elicit a birth history, which includes what?
Low birth weight, gestational diabetes, and whether Vit. K was given after birth.
Common Medical Txs, Cancer - Health Hx: Explore family history for inherited disorders inlcuding?
Hemophilia, sickle cell disease, or history of cancer.
Common Medical Txs, Cancer - Health Hx: Determine presence of risk factors, which include?
Previous malignancy and treamtent, synthetic chemical exposures, and parental exposure to radiation
Common Medical Txs, Cancer - Health Hx: When eliciting hsitory of present illness, inquire abotut what?
Fatigue/Malaise
Pallor of skin
Unusual bruising, Pain location, onset, duration
Common Medical Txs, Cancer - Inspection/Observation: Examine the oral cavity for what?
Bleeding gums or pale mucous membranes.
Common Medical Txs, Cancer - Inspection/Observation: Document visible massess or asymmetry of what
face, thorax, abdomen, or extremities.
Common Medical Txs, Cancer - Auscultation: Why can absence of lung sounds and murmurs occur?
No breath sounds = Area of lung filled with blood
Murmurs = Due to changes in blood viscosity and volume.
Common Medical Txs, Cancer - Percussion: What should you note here?
Dullness over a mass if present
Common Medical Txs, Cancer - Palpation: Carefully palpate the abdomen for what?
Tenderness, hematomegaly, splenomegaly (increased spleen size) or presence of a mass
Common Medical Txs, Cancer - Labs: The components of the CBC include?
RBC Count Hemoglobin Hematocrit (indrect measures of RBCs) WBC Count Platelet Count Mean Platelet Volume
Common Medical Txs, Cancer - Labs: RBC indices what?
Mean corpuscular volume (Average size of RBC)
MEan corpuscular hemoglobin (calculates value of oxygen carrying capacity)
Mean corpuscular hemoglobin concentration (calculated value that reflects cocnentration of Hgb isnide RBc)
Common Medical Txs, Cancer - Labs: RBC when MCV elevated?
RBCs are larger than n ormal
Common Medical Txs, Cancer - Labs: WBC range in 1 year old vs 18?
1: 5-19
18: 5-10
Common Medical Txs, Cancer - Labs: RBC in 1 year old vs 18
1: 3.9-5.3
18: 4.5-5.5
Common Medical Txs, Cancer - Labs: Hgb in 1 year old vs 18?
1: 9.5-14.1
18: 14-17.4
Common Medical Txs, Cancer - Labs: Hct in 1 year old vs 18?
1: 30-40
18: 42-52
Common Medical Txs, Cancer - Labs: What is the Alpha-Fetoprotein test?
Produced by fetal liver and yolk sac, and normally decreases to very low levels by 1 yer of age. Determiens tumor
Common Medical Txs, Cancer - Labs: How doees bone marrow aspiration/biopsy work?
Needle inserted thorugh cortex of bone into bone marrow, bone marrow aspirated, and cells are evaluated
Common Medical Txs, Cancer - Labs: How does bone scan work?
Administration of IV radionuclide maternal, which is taken up by bone
Common Medical Txs, Cancer - Labs: How does hemoglobin electrophoresis work?
Measures percentage of normal adn abnormal hemoglovin in blood
Common Medical Txs, Cancer - Labs: Why is reticulocyte count done?
Measures amount of reticulocytes (immature RBCs) in hte blood
Common Medical Txs, Cancer - Labs: Why is serum ferritin done?
Measures level of ferritin (major iron storage protein) in the blood
Child with Cancer, Administering Chemo: What precautions should nurse take when administering this?
Double gloves and nonpermeable gowns. If splashing possible, wear a face shield/mask
Child with Cancer, Administering Chemo: Chemotherapy medication dose in children based on what?
body surface area. Using a nomogram
Child with Cancer, Managing Effects of Chemo: What does myelosuppression cause?
Low blood cell counts in all cell lines, placing child at risk for infection and hemorrhage.
Child with Cancer, Managing Effects of Chemo: What may help decrease hair loss?
Cooling the scalp during chemo
Child with Cancer, Managing Effects of Chemo - Prevent Infection: What can be administered to promote neutrophil growth and maturation?
Granulocyte colony - stimulating factor (GCSF)
Child with Cancer, Managing Effects of Chemo - Prevent Infection: If child exposed to chickenpox, what is administered?
Varicella zoster immunoglobulin (VZIG)
If present, give IV acyclovir.
Child with Cancer, Managing Effects of Chemo - Prevent Infection: What to know about nadir?
This is the time after administration of the drug when bone marrow suppression expected to be at the greatest, and neutrophil count is expected to be at lowest. Ranges from 7-28 days after dosing.
Child with Cancer, Managing Effects of Chemo - Prevent Infection: What absolute neutrophil counts should we pay attention to?
Below 500 places child at greatest risk. Below 1500 warants evaluation
Child with Cancer, Managing Effects of Chemo - Prevent Infection: What precautions will be followed if absolute neutrophil count depressed?
Place in private room
VS every 4 horus
S and S every 8 hours.
Restrict sick visitors.
Do not permit raw fruits or veggies
Child with Cancer, Managing Effects of Chemo - Prevent Hemorrhage: What should you assesss for?
Petechiae, purpura, bruising, or bleeding. Do not perform any activites that may promote bleeding
Child with Cancer, Managing Effects of Chemo - Prevent Hemorrhage: What to do if bone marrow aspiration must be done?
Apply pressure dressing to the site to prevent bleeding.
Child with Cancer, Managing Effects of Chemo - Prevent Anemia: How to prevent this?/
Encourage to eat appropriate diet with iron. Administer EPO as ordered.
Child with Cancer, Managing Effects of Chemo - Mxing N/V, Anorexia: How to prevent nausea?
Administering antiemetic meds prior to chemotherapy for first 1-2 days.
Child with Cancer, Mxing Effects of Chemo - Mxing N/V, Anorexia: What may increase nausea?
Bright lights and noise.
Child with Cancer, Care to Child W/ Hemoatopoietic Stem Cell Transplant - Pretransplant Phase: When does this occur?
7-10 days before procedure. Will maintian isoltion in hospital
Child with Cancer, Care to Child W/ Hemoatopoietic Stem Cell Transplant - Pretransplant Phase: What medications will hbe administered
Gammaglobulin, acyclovir, or antibiotics to prevent infection
Child with Cancer, Care to Child W/ Hemoatopoietic Stem Cell Transplant - Postttransplant Phase: Monitor for symptoms of GVHD including
severe diarrhea and maculopapular rash, progressing from redness or sesquamation of skin. Administer cyclosporine.
Child with Cancer, Care to Child W/ Hemoatopoietic Stem Cell Transplant - Supportive Care: What is done during this phase?
Infections monitored. PRBCS or platelet or GCFS administed.
Child with Cancer, Promoting Growth: What may contribute to constipaiton?
Vinca alkaloids and opioids, as well as decreased activity level
Iron-Deficiency Anemia: When does this occur?
When the body does not have enough iron to produce Hgb. Decrease in Hgb results in decrease in oxygen-carrying capacity, resulting in weakness and fatigue
Iron-Deficiency Anemia, Therapeutic Mx: WHat is usually given?
Iron supplements like ferrous sulfate or ferrous fumarate. 3mg once or twice daily
Iron-Deficiency Anemia, Therapeutic Mx: What needs to be done in more severe cases?
Blood tranfusions may be indicated.
Iron-Deficiency Anemia, Health Hx: Signs and symptoms of this may include?
Irritability, headache, dizziness, weakness, SOB, pallor, and fatigue
Iron-Deficiency Anemia, Health Hx: Risk factors for this?
Maternal anemia
Poorly controled diabetes
Cows milk consumption before 12 months
Lack of iron supplementation
Iron-Deficiency Anemia, Health Hx: How much iron is recommended in 6-12 months vs 14-18 years?
3 mg versus 15 mg
Iron-Deficiency Anemia, Physical Exam: Observe for signs that include?
conjunctivae, oral mucosa, palms, and soles of pallor.
Iron-Deficiency Anemia, Labs: Labs will show what?
Decreased Hgb and Hct, and decreased serum iron level
Iron-Deficiency Anemia, Nursing Mx - Promoting Safety: What neurologic changes can occur?
This is due to decreased oxygen supply to brain. Leads to fatigue and inability to eat enough.
Iron-Deficiency Anemia, Nursing Mx - Dietary Interventions: What should infants be fed?
Only formulas that are fortified with iron, beginning at 4-5 months.
Iron-Deficiency Anemia, Nursing Mx - Dietary Interventions: For children over 1, limit cows milk intake to what
24 oz per day.
Iron-Deficiency Anemia, Nursing Mx - Dietary Interventions: What foods contain high iron?
Red meats, tuba, salmon, eggs, enriched grains, dried beans and peas, and dried fruits
Iron-Deficiency Anemia, Nursing Mx - Txing about Iron Supplement Admin: Begins with infants how
use of formula fortified with iron.
Iron-Deficiency Anemia, Nursing Mx - Txing about Iron Supplement Admin: How should liquid be placed?
Behind the teeth, as this liquid form can stain the teeth.
Iron-Deficiency Anemia, Nursing Mx - Txing about Iron Supplement Admin: What can this cause?
Constipation.
Lead Poisoning: Lead exerts toxic effects on what body parts?
Bone marrow, erythroid cells, nervous system and kidneys
Lead Poisoning: Complications of this?
Behavioral problems and learning difficulties, encephalopathy, seziures, and brain damage
Lead Poisoning: Therapeutic mx?
Chelation therapy (removal of heavy metals from the body via cleatign agents, orally or IV)
Lead Poisoning, Nursing Assessment: Subtle signs of this?
Anorexia, fatigue, or abdominal pain.
Lead Poisoning, Nursing Assessment: What level of lead indicates this?
Anything that is higher than 10
Lead Poisoning, Nursing Mx: Screening should occur when?
6,9,12,18 months
2-6 years
Aplastic Anemia: What is this?
Failure of bone marrow to produce cells characterized by bone marrow aplasia and pancytopenia (decreased numebr of all blodo cells)
Aplastic Anemia: What occurs in the inherited type?
Presents as congenital bone marrow failure; the best known is Fanconi anemia.
Aplastic Anemia: What occurs in severe form?
Granulocyte count less than 500, platelet count les than 20,000, and reticulocyte less than <1%
Aplastic Anemia: What occurs in nonseveree aplastic anemia?
Granulocyte count around 500, platelet count over 20,000 and reticulocyte count over 1%
Aplastic Anemia: Complications of this?
overwhelming infection, hemorrhage and eath
Aplastic Anemia: Therapeutic mx?
Hematopoietic stem cell transplantation from human leukocyte antigen (HLA)
Aplastic Anemia, Nursing Assessment: Determine history of exposure to what?
Myelosuppressive medications or radiation therapy.
Aplastic Anemia, Nursing Assessment: Note history of what?
epistaxis, gingival oozing, or increased bleeding with menstruation.
Aplastic Anemia, Nursing Assessment: On physical exam, note what?
ecchymoses, petechiae, or purpura, oral ulcerations and tachycardia
Aplastic Anemia, Nursing Assessment: Lab and diagnostic testing may reveal what?
Guaiac-positive stool
Blood in urine
Severe decrease in absence of hematopoietic cells
Aplastic Anemia, Nursing Mx: What is the most important thing to pay attention to?
Safety. Prevent injury in order to avoid hemorrhage.
Aplastic Anemia, Nursing Mx: What type of transfusions would be administered?
Irradiateed and leukocyte-depleted PRBCs or platelet transfusions
Sickle Cell Disease: What is this?
Occurs most often in Africans, Middle Eastern, Indian.. Have an enlongated RBC with a shortened life span. Become sickled in shape.
Sickle Cell Disease: What is thsi on a DNA level?
Recessive.
Sickle Cell Disease: Complciations of this?
Recurrent vaso-occlusive pain crises, stroke, sepsis, acute chest syndrome, reduced visual acuity and delayed growth and development
Sickle Cell Disease: Why do they have an increased incidence of enuresis (involuntary urination)
because kidneys cannot concentrate urine effectively
Sickle Cell Disease - Patho: What may trigger sickling”?
Any stress or traumatic event, such as infection, fever, aicdosis, dehydration or physical exertion.
Sickle Cell Disease - Patho: What happens to blood as cells sickle?
They become more viscous because the sickled cells clump together and prevent normal blood flow
Sickle Cell Disease - Patho: What to know for pain?
Can occur in any body, but mostly joints. Causes increased metabolic need
Sickle Cell Disease - Patho: What happens if cells clump together in the lungs?
Decreased gas exchange occurs, producing hypoxia leading to more sickling.
Sickle Cell Disease - Therapeutic Mx: This focuses on what?
Preventing sickling crissi and infection as well as other complications.
Sickle Cell Disease - Therapeutic Mx: What does fuctional asplenia cause? (decrease in ability of spleen to fuction properly)
Places child at significant risk for serious infection with organisms
Sickle Cell Disease - Therapeutic Mx: Treatment focuses on what?
Pain control. Oxygen administration necessary to prevent more sickling.
Sickle Cell Disease - Therapeutic Mx: What labs are closely monitored?
Hgb, Hct, and Reticulocytes. Electrolyte analysis also necessary.
Sickle Cell Disease - Nursing Assessment, Health Hx: Inspect for what?
Inspect for pallor, lesions or ulcers.
Sickle Cell Disease - Nursing Assessment, Health Hx, Auscultation: What changes may occur in heart?
HR elevated with pain, hyperthermia, or dehydration.
Sickle Cell Disease - Nursing Assessment, Health Hx, Auscultation: Palpate for what?
Warmth, tenderness and range of motion
Sickle Cell Disease - Nursing Assessment, Health Hx, Auscultation: Why should you immediately report symetric swelling of hands/feet in infant or toddler?
Dactylitis, which is an aseptic infarction occuring in metacarpals and matatarsals
Sickle Cell Disease - Nursing Assessment, Labs: When does screening occur?
At birth, as it is required. If positive, Hgb electrophoresis is performed promptly to confirm the diagnosis.
Sickle Cell Disease - Nursing Assessment, Labs: What common labs would be performed?
Hemoglobin, reticulocyte count
Peripheral blood smear
Platelet count
Abnormal liver function tess
Sickle Cell Disease - Nursing Mx: Care focuses on what?
Preventing vaso-occlusive crises, providing education to family and child, managing pain episodes and providing psychosocial suport
Sickle Cell Disease - Nursing Mx, Educating Family: What should parents be encouraged to do”?
To be tested to determine their carrier status. How isntruct how to administer penicillin.
Sickle Cell Disease - Nursing Mx, Educating Family: Discuss complicatiosn that may occur including what?
Delayed growth and development, delayed puberty, stroke, cholelithiasis, retinopathy, and leg ulcers
Sickle Cell Disease - Nursing Mx, Mxing Pain Crisis: Moderate to severe pain requires what
Opioid medication
Sickle Cell Disease - Nursing Mx, Mxing Pain Crisis: Nonpharmacologic pain management techniques may include what?
Relaxation or hypnosis, music, massage, play, guided imagery or therapeutic touch
Sickle Cell Disease - Nursing Mx, Mxing Sickle Cell Crisis: How should treatment be done”?
By treating underlying conditions like infection or injury.
Sickle Cell Disease - Nursing Mx, Mxing Sickle Cell Crisis: What fluid needs occur?
Deficient fluid volume due to decreasd intake, and kidneys inability to concentrate urine. Increase fluid to decrease viscosity.
Sickle Cell Disease - Nursing Mx, Prevent Infection: What should be given by 2 months of age?
Admin of oral penicillin V Potassium as prophylaxis against pneumococcal infection. Continue until 5 years old.
Thalassemia: What is this?
Affects those of African descent. Autosomal recessive. Have reduced production of normal hemoglobin
Thalassemia: What are the two different types of this?
Alpha = Synthesis of alpha chain of hemoglobin protein is affected.
Beta = Moer often, and divided into three categorie
Thalassemia: What three categories is B divided into?
Thalassemia Minor - leads ot mild microcytic anemia, no tx required.
Thalassemia intermedia = child requires blood transfunsiosn
Thalassemia major = to survive, child requires ongoing medicaition attention
B - Thalassemia Major (Cooley Anemia): What occurs here?
Hemoglobin synthesis is reduced or entirely absent. Unstable globulin chains acumulate, causing RBCs to eb rigid and hemolyzed easily,, resulting in severe hemolytic anemia and chronic hypoxia.
B - Thalassemia Major (Cooley Anemia): How does body respond to increased rate of RBC destruction?
Erythroid activity increased. Causes massive bone marrow expansion and thinning of bony cortex. REsults in growth retardation and pathologic fractures
B - Thalassemia Major (Cooley Anemia): What is hemosiderosis?
Excessive supply of iron. Occurs because of rapid hemolysis of RBCs, decrease in hemoglobin production, and increased absorption of dietary iron.
B - Thalassemia Major (Cooley Anemia): Excess iron deposited into bodies tissue causing what?
Bronze pigmentation on skin. Also bony changes such as frontal bossing and maxillary prominence
B - Thalassemia Major (Cooley Anemia): What can happen if untreated? Txs for this?
It is fatal if left untreated. Blood transfusions and chelation therapy has increased the life expenctancy
B - Thalassemia Major (Cooley Anemia), Therapeutic Mx: What does this involve?
Monitoring hemoglobin and hematocrit and transfusing PRBCs at regular intervals. Blood iron levels also monitored.
B - Thalassemia Major (Cooley Anemia), Nursing Assess: Infants diagnosed by 1 nad have history of what?
Pallor, jaundice, failure to thrive, and hepatosplenomegaly
B - Thalassemia Major (Cooley Anemia), Nursing Assess: Lab tests may reveal what?
Hct and Hgb decreased
Peripheral blood smear shows anisocytosis and poikilocytosis (variations in size and shape of RBCs)
Bilirubin elevated
B - Thalassemia Major (Cooley Anemia), Nursing Mx: This is geared toward what?
Supporting the family and minimizing the effects of illness. Includes administering blood transfusions and educating family
B - Thalassemia Major (Cooley Anemia), Nursing Mx - Administering Packed RBC Transfusion: Why is this done?
To maintain adequate level of hemoglobin for oxygen delivery and suppresses erythrocyosis in the bone marrow.
B - Thalassemia Major (Cooley Anemia), Nursing Mx - Administering Packed RBC Transfusion: What happens to excess iron?
/removed by chelation therapy.
B - Thalassemia Major (Cooley Anemia), Nursing Mx - Administering Packed RBC Transfusion: Why would deferoxamine be given?
Binds to iron and allows it to be removed through the stool or urine.
B - Thalassemia Major (Cooley Anemia), Nursing Mx - Educate Family: Educate that what is necessary?
Chelation therapy must be maintained. And hwo to administer deferoxamine SQ with small battery powered infusion pump.
Idiopathic Thrombocytopenia Purpura: What is this?
Immune response following a viral infection that produces antiplatelet antibodies. Destroy platelets which leads to petechiae, purpura, and excessive bruising.
Idiopathic Thrombocytopenia Purpura: What is Petechiae?
Pinpoint hemorrhages that occur anywehre on body and do not blanch with pressure
Idiopathic Thrombocytopenia Purpura: What is purpura?
Larger area of hemorrhage in which blood colelcts under the tissue, they are purplish.
Idiopathic Thrombocytopenia Purpura: When does this develop?
Few weeks after a viral infection and is most coommon in young children. Will recover spontaneously after a few months
Idiopathic Thrombocytopenia Purpura: Complications of this?
Severe hemorrhage and bleeding into vital organs and intracranial hemorrhage
Idiopathic Thrombocytopenia Purpura: What to do if platelet counts below 10,000?
Corticosteroids administered for 2-3 weeks. I
Idiopathic Thrombocytopenia Purpura: What is done in acute or chronic cases?
IV immunoglobulin (IVIG) may be infused for 1-2 days.
Idiopathic Thrombocytopenia Purpura, Nursing Assess: Health history would be what for this child?
Previously healthy child who recently developed increased bruising, epistaxis or bleeding of the gums
Idiopathic Thrombocytopenia Purpura, Nursing Assess: Risk factors to increase this??
Recent viral illness, recent MMR immunization, or ingestion of meds.
Idiopathic Thrombocytopenia Purpura, Nursing Assess: Inspect for what?
Petechiae, purpura and bruising which progresses rapidly within first 24-48 hours.
Idiopathic Thrombocytopenia Purpura, Nursing Assess: Lab findings may show what?
Low platelet count (<50,000) , normal WBC count, and normal Hgb,Hct unless hemorrhage has occured
Idiopathic Thrombocytopenia Purpura, Nursing Mx: Treatment?
Many children require no medical treatment, except obserevation and reevaluate lab values
Idiopathic Thrombocytopenia Purpura, Nursing Mx: Teach family to avoid what
aspirin, NSAIDS, and antihistamines because they preceipitate development of anemia in these kids.
Hemophilia: What is this?
X-Linked recessive disorder resulting in deficiency in one of the coagulation factors in blood. Trabsmited by mother to son.
Hemophilia: Most common type?
Hemophilai A, factor VIII deficiency which is essential for activation of factor X, which converts prothrombin into thrombin causing inability of platelets to be used in clot formation.
Hemophilia: What occurs during a bleeding episode?
Vessels constrict and platelet plug forms, but because fibrin will nto solidify, the bleeding continues
Hemophilia, Therapeutic Mx: Primary goal is what?
Prevent bleeding. Teach child to avoid activites with high potential for injury.
Hemophilia, Therapeutic Mx: What to do if bleeding or injury occurs?
Factor administration prescribed. Once deficient factor replaced, clotting factors rerturn to fairly normal levels for a period of time.
Hemophilia, Nursing Assess - Health Hx: What should you inquire about with past bleeding episodes?
Nature of bleeding episode or bruise. If in GI tract, black tarry stools may be present.
Hemophilia, Nursing Assess - Physical Exam: What could happen if bleeding is not found?
Hypovolemia could follow, leading to shock. Note chest pain and abdominal pain
Hemophilia, Nursing Assess - Labs: Labs include what?
Decreased Hgb and Hct
Hemophilia, Nursing Mx: This focuses on what?
prevent bleeding episodes, manage bleeding episodes, and providing education
Hemophilia, Nursing Mx - Preventing Bleeding: Major bleeds into joints may cause what?
Limit ROM and function, eventually decreasing physical abilities and crippling some boys
Hemophilia, Nursing Mx - Mxing Bleeding Episode: What should be given as prescribed?
Factor VIII replacement . Give by slow IV push and document medication information.
Hemophilia, Nursing Mx - Mxing Bleeding Episode: Why would desmopresin be used?
To stop bleeding.
Hemophilia, Nursing Mx - Mxing Bleeding Episode: What to do if external bleeding develops? if inside?
Apply pressure to the area until bleeding stops. If inside joint, apply ice or cold compress to are and elevate injured extremity.
Leukemia: What is this?
Primary disorder of bone marrow in which normal elements are replaced with abnormal WBCs.
Leukemia: What do lymphoid cells normally grow and develop into?
Lymphocytes
Leukemia: What do myeloid cells normally grow and develo into?
RBCs, granulocytes, monocytes and platelets
Leukemia: Complications of this includes?
Metastasis (spread of cancer to other sites) to the blood, bone, CNS, spleen, liver, and other organs.
Leukemia: Late effects include what?
Problems with neurocognitive function and ocular, cardiovascular, or thyroid dysfunction
Acute Lymphoblastic Leukemia (ALL): What is this?
More common in white children. Affects T, B, Early Pre-B or Pre-B Cells.
Acute Lymphoblastic Leukemia (ALL): Prognosis is based on what?
WBC at diagnosis, and type fo cytogenetic factors and immunophenotype, age of diagnosis,
Acute Lymphoblastic Leukemia (ALL): Generally the higher the WBC count indicates what?
The worse the prognosis.
Acute Lymphoblastic Leukemia (ALL): Which childeren have the best prognosis?
Between 1-9 years old with WBC count less than 50,000
Acute Lymphoblastic Leukemia (ALL): Complciations of this?
Infection, hemorrhage, poor growth and CNS, bone, or testicular involvement
Acute Lymphoblastic Leukemia (ALL): What may improve the long-term growth fo children with this?
growth hormone replacement therapy using recombinant human growth hormone
Acute Lymphoblastic Leukemia (ALL) - Patho: Wht occurs in here?
Abnormal lymphoblasts abound in blood-forming tissues
Acute Lymphoblastic Leukemia (ALL) - Patho: How are the lymphoblasts here?
Fragile and immature, lacking the infection-fighting capabilities of the normal WBC. Growth excessive and replace normal cells.
Acute Lymphoblastic Leukemia (ALL) - Patho: Since the needs of these lymhoblasts are excessive, what happens to the body?
Normal body cells deprived of needed nutrients and result in fatigue, weight loss, growth arrest or muscle wasting.
Acute Lymphoblastic Leukemia (ALL) - Patho: What happens to bone marrow?
unable to maintain normal levels of RBCs, WBCs, and platelets so anemia and thrombocytopenia result.
Acute Lymphoblastic Leukemia (ALL) - Patho: If spread to lymph nodes, what happens?
Cause diffuse lymphadenopathy, or the liver and spleen resulting in hepatosplenomegaly
Acute Lymphoblastic Leukemia (ALL) - Patho: What happens if spread to CNS?
vomiting, headaches, seizures, coma, vision alteraitons or cranial nerve palsies may occur.
Acute Lymphoblastic Leukemia (ALL) - Therapeutic Mx: This focuses on what?
Giving chemotherapy to eradicate the leukemic cels and restore normal bone marrow function.
Acute Lymphoblastic Leukemia (ALL) - Therapeutic Mx: Wht is provided at each stage of treatment?
CNS prophylaxis. Without it, would spread to CNS.
Acute Lymphoblastic Leukemia (ALL) - Therapeutic Mx: What are teh stages of this?
Induction - Remission in 3-4 weeks
Consolidation - Strengthen remission
Maintenancce - eliminate all residual leukemic cels (2-3 years)
Acute Lymphoblastic Leukemia (ALL) - Nursing Assess, Health Hx: THis includes what
Fever, recurrent infection, fatigue, pallor, unsuual bleeding, or abdominal pain.
Acute Lymphoblastic Leukemia (ALL) - Nursing Assess, Health Hx: Risk factors fo rthis?
Male, 2-5 years, caucasian race, or down syndrome
Acute Lymphoblastic Leukemia (ALL) - Nursing Assess, Physical Exam: What should be performed here?
Take temperature
Look for petechiae, purpura or unsual bruising
inspect skin for infections
Acute Lymphoblastic Leukemia (ALL) - Nursing Assess, Labs: What labs would be performed?
CBC, with low Hgb and Hct, decreased RBC count, and decreased platelet count.
Peripheral blood smear
Bone marrow aspiration (greather than 25% lymhoblast)
Acute Lymphoblastic Leukemia (ALL) - Nursing Mx: This focuses on what?
Managing disease complications such as infection, pain, anemia, bleeding, and hyperuricemia.
Acute Lymphoblastic Leukemia (ALL) - Nursing Mx: What do children require with severe enamia or low platelet counts with active bleeding?
Blood product transfusion
Acute Lymphoblastic Leukemia (ALL) - Nursing Mx, Reducing Pain: Most common areas of pain?
Head and neck, legs and adomen.
Acute Lymphoblastic Leukemia (ALL) - Nursing Mx, Reducing Pain: What can reduce pain?
Distraction techniques.
Using EMLA cream prior to venipuncture, port access, and lumbar puncture.
Acute Myelogenous Leukemia: What does this peak incidence wise?
Duruing the adolescent years.
Acute Myelogenous Leukemia: What does this affect?
Myeloid cell progenitors and precursors in bone marrows, resulting in malignant (invasive and fast-growing) cels.
Acute Myelogenous Leukemia: Complciations of this?
Treatment resistanc,e infection, hemorrhage, and metastasis.
Acute Myelogenous Leukemia: induction phase of AML requires?
intense bone marrow suppressiona nd prolonged hospitalization.
Acute Myelogenous Leukemia, Nursing Assess:Common signs?
REcurrent infection, fever, or fatigue.
Acute Myelogenous Leukemia, Nursing Assess: Risk factors?
Hispanic race, previous chemotherapy and genetic abnormalities such as downs syndrome.
Acute Myelogenous Leukemia, Nursing Assess: Physical exam will icnlude?
skin pallor and salomon-colored or blue-gray papular lesions.
Hodgkin Disease: WHat does hodgkin disease affect?
lymph nodes located closer to the body surface such as cervical, axillary and inguinal areas
Hodgkin Disease: What differentiates this from other lymphomas?
Presence of Reed-Sternberg cells (giant transofmed B lymphocytes with one or two nuclei.
Hodgkin Disease: What happens as lymph nodes enlarge?
Compres nearby structures, destroying normal cells and invading other tissues.
Hodgkin Disease: This seems to be linked to what?
Epstein -Barr virus infection
Hodgkin Disease: Most commmon at what age
adolecents and young adults, and in those 14 and younger. More common in boys
Hodgkin Disease: How is this classified?
Stages I - IV and also A (asymptomatic) and B (presence of symptoms of fever, night sweats, or weight loss 10%)
Hodgkin Disease: Complications of this?
liver failure and secondary cancer
Hodgkin Disease: Treatment of choice?
Chemotherapy with combination of drugs. Radiation may be necessary
Hodgkin Disease, Nursing Assess: Common signs include what
recent weight loss, fever, drenching night sweats, anorexia, malaise, fatigue, and pruiritus.
Hodgkin Disease, Nursing Assess: Risk factors?
Epestein-barr virus infection, family hx and genetic immune disorder
Hodgkin Disease, Nursing Assess: How will lymph nodes feel?
Rubbery and tend to occur in clusters (cervical and supraclavicular)
Hodgkin Disease, Nursing Mx: Focuses on what?
Addressing adverse effects of chemotherapy or radiation
Brain Tumors: Where do they arise from?
More thahn half in posterior fossa (infratentorial) and rest are supratentorial in origin
Brain Tumors: Complications of brain tumors?
hydrocephalus, increased intracrnaial pressure, brain stem herniation and negative effects of radiation
Brain Tumors - Patho: Medulloblastoma occurs where and has what characteristics?
Cerebeullum
Invasive, highly maligannt.
Less favorable outcome. Progresses quickly.
Brain Tumors - Patho: Brain stem glioma occurs where and has what characteristics?
Brain stemp
aggressive, difficult to resect, resistant to chemotherapy
Brain Tumors - Patho: Ependymoma occurs where and has what characteristics?
Frequently arises from floor of fourht ventricle
Varying speeds of growth, often causes hydrocephalus
Brain Tumors - Patho: Estrocytome grows where and causes what?
Cerebellum or cerebral hemispheres
Slow course with insidious onset. REsponseive to chemotherapy and retractable
Brain Tumors - Therapeutic Mx: What o children with hydrocephalus require?
A ventriculoperitoneal shunt.
Brain Tumors - Therapeutic Mx: Radiation reserved for who
those older than 2 due to long-term neuronognitive effects
Brain Tumors - Nurse Assessment, Health Hx: Common signs include?
N/V, HEadache, Unsteady Gait, Blurred Vision, Seizures, Motor Abnormality
Brain Tumors - Nurse Assessment, Health Hx - Inspection/Observation: Observe for what?
Nystagmus, sunsettign eyes, head tilt, alterations in coordination, gait disturbance, and alterations in sensation
Brain Tumors - Nurse Assessment, Health Hx - Inspection/Observation: Why should BP be checked?
May decrease with increasing intracranial pressure.
Brain Tumors - Nurse Assessment, Health Hx - Inspection/Observation: Palpate what in infant?
ANterior fontanel for bulging
Brain Tumors - Nurse Assessment, Health Hx - Labs: This includes what??
CT, MRI, PET
Lumbar puncture may show elevation in tumor markers
Brain Tumors - Nurse Assessment, Nursing Mx: This includes what?
Preoperative and postoperative care, as well as interventions to manage adverse effects related to chemo
Brain Tumors - Nurse Assessment, Nursing Mx - Providing PreOp Care: Care here focuses on what?
Monitoring for additional increases in intracranial pressure and avoiding activites that cause transient increases in intracranial pressure.
Brain Tumors - Nurse Assessment, Nursing Mx - Providing PreOp Care: Why would dexamethasone be prescribed?
to decrease intracranial inflammation
Brain Tumors - Nurse Assessment, Nursing Mx - Providing PostOp Care: What can be done to control edema and swelling?
Regulate fluid administration. and Mannitol can be given to decrease cerebral edema.
TO decreaseswelling, give eye lubricant and apply cool compresses.
Brain Tumors - Nurse Assessment, Nursing Mx - Positioning PostOp Care: How should they be positioned?
On the unaffected side of the bed with head flat Do not elevate foot of bed
Osteosarcoma: What is this?
Most common maligant bone cancer in children. Most commonly found in long bones, like humerus, tibia and femur
Osteosarcoma: Complications of this?
Metastasis, lungs, and recurrence of disease within 3 years
Osteosarcoma: Treatment?
Surgical removal of the tumor. Chemo is often done before surgery to decrease size of tumor and after to prevent metastasis. May lose limb.
Osteosarcoma, Nursing Assessment: Health history should be what?
Determining when pain, limp, or limitation of motion was first notices. Dull bone pain may be present for months.
Osteosarcoma, Nursing Assessment: Inspect affected limb for what
erythema and swelling. Palpate for warmth and tenderness and determine size of soft tissue mass.
Osteosarcoma, Nursing Assessment: Labs may include what?
CT or MRI
Bone scan
Ewing Sarcoma: What is this?
Highly malignant bone tumor. Rarer than osteosarcoma, accounting for only 10% of childhood bone tumors.
Ewing Sarcoma: Occurs most frequently where?
Pelvis, chest wal, vertebrae and long bone diaphyses (midshaft)
Ewing Sarcoma: Treatment consists of ?
Radiation, chemotherapy and surgical excision.
Ewing Sarcoma: What may be used for metastatic disease?
Myeloablative chemotherapy (destroys childs marrow) followed by stem cell rescue transplant
Ewing Sarcoma, Nursing Assess: Note hsitory for what?
History of fever, and for history of interrmittent pain that progressively worsens. Will eventually disrupt sleep.
Ewing Sarcoma, Nursing Assess: What diagnostics will eb performed?
CT or MRI scan will reveal the extent of the tumor. Biopsy necessary to establish diagnosis.
Ewing Sarcoma, Nursing Mx: Discourage what before treatment begins?
Active play or weight bearing on affected extremitiy.
Ewing Sarcoma, Nursing Mx: This focuses on what?
Addressing adverse effects of treatment . Give honest and direct answers.
Rhabdomyosarcoma: What is this?
Soft tissue tumor that uses cells that would oridinally form striated msucle.
Rhabdomyosarcoma: Most common location?
Head and neck, genitourinary tract and extremities.
Rhabdomyosarcoma: How does this spread
Through the venous or lymphatic system, with lungs most common site for metastasis.
Rhabdomyosarcoma: Diagnossi made when
between ages 2-5
Rhabdomyosarcoma: Complciations of this?
Metastasis to lung, bone, or bone marrow and direct extension into the CNS, resulting in brain stem compromise or cranial nerve palsy
Rhabdomyosarcoma: Most common treatment for this?
Surgical removal of the tumor. May use radiation and chemotherapy to shrink tumor
Rhabdomyosarcoma, Nursing Assess: When will parents seek medical attention?
They find a asymptomatic mass and seek medical attention.
Rhabdomyosarcoma, Nursing Assess: Risk factors include?
Parental smoking, exposure to environmental chemicals, family history of cancer.
Rhabdomyosarcoma, Nursing Assess: Labs include?
CT/MRI
Open biopsy of primary tumor
Bone marrow aspiration and biopsy, bone scan, and skeletal surveyr
Wilms Tumor: What is this?
Common renal tumor. Between 2-3 years. Usually affects only one kdiney. Rapid growth. Metasis occurs via direct extension or through blood stream.
Wilms Tumor: Most commonly metstasizes where?
To the perirenal tissues, liver, diaphragm, lungs, or abdominal muscles.
Wilms Tumor: Complciations of this?
Metasis or complications from radiation therapy such as liver, renal damage, female sterility, bowel obstruction, pneumonia or scoliosis
Wilms Tumor, Therapeutic Mx: What is the treatment of choice?
Surgical removal of tumor and affected kidney (nephrectomy). Radidation and chemo may also occur before/after surgery
Wilms Tumor, Nursing Assess, Health Hx: What should you inquire baout?
When mass was discovered. Note abdominal pain and history of constipation, vomiting, anorexia, weight loss, or difficulty breathing.
Wilms Tumor, Nursing Assess, Physical Exam: What should be done here?
Measure BP, isnpect abdomen for asymmetry or visible mass. Ausculate lungs.
Wilms Tumor, Nursing Assess, Physical Exam: Why should you avoid palpating abdomen after intiial assessment preop?
Highly vascular and soft, so excessive handling may result in tumor seeding and metastasis
Wilms Tumor, Nursing Assess, Labs: This includes?
Renal or Abdominal Ultrasound
CT/MRI Scan of Abdomen
CBC, BUN, Creatinine
Urinalysis
Retinoblastoma: What is this?
Highly malingant tumor. Accounts for 5% of blindness in children.
Retinoblastoma: Nonhereditary retinoblastoma may be associated with what?
Advanced paternal age and always presents with unilateral involvement.
Retinoblastoma: Hereditary retinoblastoma is inherited via what
autosomal dominant mode
Retinoblastoma:
Complications include what?
Spread to the brain and the opposite eye, as well as metastasis to lymph nodes, bone, bone marrow,q and liver as well as metastasis to lymph nodes , bone, and bone marrow.
Retinoblastoma: Goals of treamtnet?
Eradicate the tumor, preserve vision, and provide good cosmetic outcome.
Retinoblastoma: May be treated how?
Radiation, chemotherapy, laser surgery, cryotherapy, or combination of these treatments.
Retinoblastoma: What treatment is done in case of massive tumor with retinal detachment?
enucleation (removal of eye)
Retinoblastoma, Nursing Assess: Parents first notice what?
Cats eye reflex or whitewash glow to affected pupil.
Retinoblastoma, Nursing Assess: Obtain health history, determine when what symptoms began?
strabismus, orbital inflammtion, vomiting, or headache
Retinoblastoma, Nursing Assess: Diagnostic evaluation includes?
Epthalmologic examination under anesthesia. CT, MRI, or ultrasound will help visualize tumor.
Retinoblastoma, Nursing Mx: Dressing changing for socket included what
sterile saline rinses or antibiotic ointment application.
Retinoblastoma, Nursing Mx: Followup will icnldue what?
Eye exams eery 3-6 months until age six, and then annually.
A child on the pediatric unit has morning laboratory results of Hgb 10.0, Hct 30.2, WBC 24,000, and platelets 20,000. What is the priority nursing assessment?
Assess for pallor, fatigue, and tachycardia. Monitor for fever. Assess for bruising or bleeding. Determine intake and output.
Assess for bruising or bleeding
A child with hemophilia fell while riding his bicycle. He was wearing a helmet and did not lose consciousness. He has a mild abrasion on his knee that is not oozing. He is complaining of abdominal pain. What is the priority nursing assessment?
Perform neurologic checks. Assess ability to void frequently. Carefully assess his abdomen. Examine his knee frequently.
carefully assess his abdomen
A 14-year-old with thalassemia asks for your assistance in choosing her afternoon snack. Which choice is the most appropriate?
peanut butter with rice cake small spinach salad apple slices with cheddar cheese small burger on wheat bun
apple slices with cheddar cheese
The nurse is caring for a child who has just been admitted to the pediatric unit with sickle cell crisis. He is complaining that his right arm and leg hurt. What is the priority nursing intervention?
Administer pain medication every 3 hours intravenously until pain is controlled. Perform passive range of motion of the arm and leg to maintain function. Try acetaminophen for pain first, moving up to opioids only if needed. Use narcotic analgesics and warm compresses as needed to control the pain.
Administer pain medication every 3 hours intravenously until pain is controlled.
A 5-year-old has been diagnosed with Wilms tumor. What is the priority nursing intervention for this child?
Educate the parents about dialysis, as the kidney will be removed. Measure abdominal girth every shift. Avoid palpating the child’s abdomen. Monitor BUN and creatinine every 4 hours.
Avoid palpating the child’s abdomen
A child with leukemia has the following AM laboratory results: Hgb 8.0, Hct 24.2, WBC 8,000, platelets 150,000. What is the priority nursing assessment?
Monitor for fever. Assess for bruising or bleeding. Determine intake and output. Assess for pallor, fatigue, and tachycardia.
Assess for pallor, fatigue, and tachycardia
A child with leukemia received chemotherapy about 10 days ago. She presents today with a temperature of 100.4°F, an absolute neutrophil count of 500, and mild bleeding of the gums. What is the priority nursing intervention?
Administer IV antibiotics as ordered. Provide vigorous oral care frequently with a firm toothbrush. Monitor pulse and blood pressure for changes. Administer packed red blood cell transfusion
administer iv antibiotics as ordered
A child with cancer is receiving chemotherapy, and his mother is concerned that the nausea and vomiting associated with chemotherapy are reducing his ability to eat and gain weight appropriately. What is the most appropriate nursing action?
Administer an antiemetic at the first hint of nausea. Offer the child’s favorite foods to encourage him to eat. Start antiemetic drugs prior to the chemotherapy infusion. Maintain IV fluid infusion to avoid dehydration.
Start antiemetic drugs prior to the chemotherapy infusion
