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TL Hematologic

Pediatric Hematologic Disorders

QuestionAnswer
Define iron deficiency anemia. Hemoglobin levels below normal range due to inadequate supply, intake, or absorption of iron
What is the leading hematologic problem in children? iron deficiency anemia
Why do kids need more iron than adults? They are growing
Give four causes of anemia. inadequate stores in fetal development, deficient dietary intake, chronic blood loss, poor utilization of iron in the body
What signs during assessment would signal anemia? pallor, paleness of mucous membranes, fatigue
What does “milk baby” have to do with iron deficiency? too much milk > than 32oz/day
What is pica? eating non food substance like clay or chalk
What lab values indicate iron deficiency anemia (3)? decreased Hgb, low serum iron, elevated total iron binding capacity (TIBC)
What is the normal HGB range for newborns? 14-24 g/dL
What is the normal HGB range for infants? 10-15 g/dL
What is the normal HGB range for children? 11-16 g/dL
Give 2 nursing diagnoses that would be applicable to the child with iron deficiency anemia. Ineffective tissue perfusion related to; Activity intolerance related to
What are the nursing interventions for the child with iron deficiency anemia? provide rest periods, support need to limit activities, administer oral iron as prescribed
What do we need to know and teach about the administration of iron? give on empty stomach if tolerated for better absorption, give with vitamin c, use dropper or straw to avoid discoloring of teeth, stools will be tarry, keep out of reach of other children (toxic in excess), do not give with diary
Name some good food sources for iron. meat, fish, liver, legumes, green leafy veggies, whole grains
What should nurses keep in mind when making dietary recommendations to families? family income, cultural preferences
What referrals could be made to a family of a child with iron deficiency? Nutritionist, WIC
Which age groups are most susceptible to iron deficiency anemia? infants 6 and 24 months, adolescent females
Inherited bleeding disorder hemophilia
What kind of genetic disorder is hemophilia? X-linked recessive chromosome (Mother is carrier. Sons may express disease)
What is missing in Hemophilia? Factor VIII
How much factor VIII activity does a person with hemophilia have? How much factor VIII activity does a person without hemophilia have? 50 -200% activity is normal, Hemophiliacs has 0-25% activity
What are some red flags that may indicate hemophilia? prolonged bleeding after circumcision, prolonged bleeding with minor trauma, hemoarthrosis (most frequent), spontaneous bleeding into muscles and tissues, easy bruising, loss of motion in joints, pain, prolonged PTT, Factor assays <25%
What are 2 nursing diagnoses that would be applicable to a child with hemophilia? risk for injury related to; deficient knowledge home care related to
Name 3 autosomal recessive disorders. sickle cell anemia, cystic fibrosis, and phenylketonuria
What does autosomal recessive mean? The trait is recessive, so the only way it can be transmitted to the offspring is if both parents are carriers of the gene. All children of a couple that both carry the recessive trait are at risk for getting the disorder. There is a 1 out of 4 chance.
How does an x linked disorder like hemophilia affect offspring? X-linked disorder affect male children. Male children have a 50% chance of having hemophilia. Female children have a 50% chance of being carriers.
What are the chances a couple in which the female carries the hemophilia gene will have a child with hemophilia? 25%
What are the nursing interventions for a child with hemophilia? administer blood product & aspirin free pain medication, follow blood precautions (risk for hepatitis), teach home care, refer - genetic counseling, anticipatory guidance for times when increased risk for bleeding occur with normal growth & development
What teaching do we give parents of a child with hemophilia regarding home care? how to recognize early signs of bleeding into joints, treatment for minor bleeds (pressure, splinting, ice), use soft tootbrush, protection – soft toys, padded bed rails, helmet, medic alert bracelet
Inherited autosomal recessive disorder of hemoglobin Sickle cell disease
This autosomal recessive disorder is most common among those of African and Mediterranean descent Sickle Cell
When does sickle cell usually appear? after 6 months of age
What happens in sickle cell disease? HGB S replaces all or part of the normal hemoglobin which causes the red blood cells to sickle when oxygen is released into the tissues. Sickled cells cause traffic jams at capillaries=pain
Why is hydration important in the treatment of sickle cell disease? Dehydration promotes sickling. Hydration promotes hemodilution and circulation of red cells through the blood vessels.
How does sickle cell disease cause anemia? Cells can return to normal shape after sickling but they don’t live as long (<40 days) leading to anemia.
What are the systemic effects of sickle cell disease? Tissue ischemia causes widespread pathologic changes in spleen, liver, kidney, bones, and central nervous system
What is the pathophysiology that leads to widespread pathologic changes caused by Sickle Cell Anemia? Sickled cells block capillary blood supply resulting in tissue damage and necrosis. Scar tissue replaces healthy tissue with repeated damage.
HgbAS sickle cell trait – heterozygous gene
HgbSS sickle cell disease – homozygous gene
HGBS disease and traint – abnormal hemoglobin
What is the function of Hgb electrophoresis? distinguishes sickle cell trait from sickle cell disease
What are some physical manifestations that could signal sickle cell disease? frequent infections (nonfunctional spleen), tiredness, chronic hemolytic anemia, delayed physical growth, leg ulcers (adolescents), Cerebro-Vascular Accidents
What is the current treatment for kids with SCD who have had a cerebral vascular accident? What is the rationale for this therapy? Chronic blood transfusion therapy. Aproximately 70% of Kids who have had a CVA and don’t receive treatment suffer repeat CVAs resulting in progressively greater brain damage.
Sickle cell crises characterized by distal ischemia and pain. Vaso-occlusive (VOC)
Sickle cell crises caused by a pooling of blood in the liver and spleen with decreased blood volume and shock Sequestration crisis
Sickle cell crisis characterized by diminished RBC production Aplastic Crisis
Sickle cell crises characterized by anemia, jaundice and reticulocytosis hyperhemolytic crisis
Define Acute Chest Syndrome. a complication of sickle cell anemia clinically similar to pneumonia
When can a child with Leukemia go back to school? When the absolute neutrophil count is >500
What are the classic signs of sickle cell crisis? fever, severe abdominal pain, hand-foot syndrome; painful edematous hands and feet, athraligia
What are 3 nursing diagnoses that would apply to a child with sickle cell disease? Acute pain; risk for infection; deficient knowledge (crisis prevention)
What teaching can we provide families to help prevent the development of a sickle cell crises? Avoid hypoxia by: avoid strenuous exercise, high altitudes; prevent infections and seek early intervention at first sign of infection, use prophylactic penicillin if prescribed, hydration 125ml/kg/day, don’t withhold fluids at night for enuresis
What do we do for a child hospitalized with a vaso-occlusive crises? administer IV fluids at 1-2 times the maintenance levels and electrolytes as prescribed to hydrate and treat acidosis, monitor I&O, blood products as prescribed, analgesics/morphine, warm compresses, antibiotics for infection as prescribed
Why don’t we give iron to a child experiencing anemia due to sickle cell disease? What supplement is given? Why? Because anemia is not due to iron deficiency we don’t give iron supplements. We give folic acid to stimulate RBC synthesis.
Why are pneumococcal, meningococcal, yearly influenza, and Haemophilus B vaccines especially important for a child with sickle cell disease? Infection results in sickle cell crises, increased susceptibility to all types of infection (functional asplenia), and infection is the leading cause of death in children with sickle cell disease.
Why doesn’t sickle cell disease become evident in infants? Infants have infant hemoglobin – HbF up to 80%. As long as the hemoglobin is predominantly HbF sickling does not occur.
Newborn screening is mandatory in most states for SCA. What test is used? Electrophoresis of Hgb
What is the quick screening test for SCA that can be done with a finger prick and read in 3 minutes? sickledex
Does Sickledex diagnose SCA? No, it must be followed by hgb electrophoresis.
What are the main goals of therapy for SCA? Prevent sickle cell crises which cause the pathologic sequelae; Treat sickle cell crises
What are 6 objectives in the treatment of sickle cell crises? Rest to reduce energy expenditure & O2 use; hydration oral/IV; electrolyte replacement to treat acidosis; analgesics (severe pain); blood replacement to treat anemia and reduce viscosity; antibiotics for infection
Oral BLANK prophylaxis is recommend by BLANK to prevent pneumococcal sepis in infants with SCA. penicillin; 2 months of age
Oxygen is used to prevent more sickling. Why don’t we use oxygen therapy to reverse sickling? because the oxygen is unable to reach the enmeshed sickled erythrocytes clogged vessels and continued administration suppresses bone marrow RBC production
Why isn’t meperidine recommended for sickle cell disease patients? accumulation of a metabolite of meperidine causes seizures. Patients with SCD are particularly sensitive to seizures from meperidine.
What drug can increase HbF, reduce endothelial adhesion of sickle cells and improve sickle cell hydration? hydroxurea
What must the nurse be alert for when a child is getting a blood transfusion? Signs of transfusion reaction and cardiac function since hypervolemia could place excess workload on the heart.
What are the S&S of Acute Chest Syndrome (ACS)? severe chest, back , or abdominal pain, fever of 101.3 or higher, very congested cough, dyspnea, tachypnea, retractions, declining oxygen saturation
What are the S&S associated with a stroke (CVA)? severe unrelieved headache; severe vomiting; jerking/twitching face, arms, or legs; seizure; strange behavior; inability to move an arm/leg; usteady gait; slurred speech; weakness hands/arms/legs/feet; changes in vision
Define Acute Lymphocyte Leukemia. A cancer of the blood forming organs
BLANK accounts for about 80% of childhood leukemias. Acute lymphocytic leukemia
What happens in Acute Lymphocytic Leukemia? lymphoblasts replace normal cells in the bone marrow and peripheral blood
How is Acute Lymphocytic leukemia classified? according to the WBC it involves: T – lymphocytes, B- lymphocytes, or nulls cells (Neither T or B)
Which type of ALL has the best prognosis? null cell type
Which percentage of kids have with acute lymphocytic leukemia have the null cell type? 75%
What causes the S&S of leukemia? replacement of normal cells by leukemic cells in the bone marrow and extramedullary sites
What are the four phases of treatment of acute lymphocytic leukemia? induction, sanctuary, consolidation, and maintenance
What are the S&S of Acute Lymphocytic Leukemia related to anemia? tiredness, weakness, lethargy, pallor
What are the S&S of Acute Lymphocytic Leukemia related to neutropenia? petechia, bleeding, bruising due to thrombocytopenia, infection, fever
What are the signs and symptoms that cause bone/joint pain due to? leukemic infiltration of bone marrow
What happens to the lymphatic system due to Acute Lymphocytic Leukemia? enlarged lymph nodes, hepatosplenomegaly
What signs and symptoms might we see with Acute Lymphocytic Leukemia if the CNS becomes involved? headache,vomiting
What lab value is diagnostic of Acute Lymphocytic Leukemia? Bone marrow aspiration shows 80-90% immature blast cells
What nursing diagnoses apply to the child with leukemia? risk for infection related to, fear related to, deficient knowledge related to
What are the nursing interventions for a child with leukemia? Private room, reverse isolation, age appropriate explanations of care, tests, tx; examine for infection of skin, needle sticks, dental problems; administer blood products/chemotherapeutic agents as prescribed, monitor/treat side effects of chemotherapy
What care can nurses provide to help with the effects of chemotherapy? antiemetics before tx; Monitor for: fluid balance, signs of infection/bleeding, hepatic toxicity, cardiac toxicity, renal toxicity, neurotoxicity; oral hygiene; small high calorie appealing meals, promote positive body image, prevent infection
Prednisone is often used with antineoplastic drugs for what purpose? to reduce mitosis of lymphocytes
What is the purpose of administering allopurinol with chemotherapy? to prevent renal damage caused by uric acid buildup and cellular lysis
What stage of chemotherapy would utilize Vincristine? induction
What stage of chemotherapy would utilize L- asparaginase? Induction
What precautions should be taken with L-asparaginase? have epinephrine and oxygen readily available to treat anaphylaxis
What stage of chemotherapy would utilize Mercaptopurine (6-mp)? Maintenance
What stage of chemotherapy would utilize Methotrexate? Sanctuary and Maintenance