HEMATO Flashcards

Question

Nursing Implications IDA

Answer 1

Assessment; pay particular attention to milk and iron intake. Determine and eliminate the cause General nursing implications for anemia Provide foods rich in iron Teach parents to administer supplements Administer parenteral iron safely Follow-up care

Answer 2

Best absorbed in an acidic environment (oranges) Give with straw or back in the mouth past the teeth Rinse mouth/ brush teeth after administration. Teach parents; measure accurately, increase fluids and fiber in diet. Avoid antacids, coffee, tea, dairy products, eggs, or whole grains one hour before or two hours after administration. Between meals or with ascorbic acid to facilitate absorption

Answer 3

Nausea Gastric Irritation Constipation Diarrhea Anorexia Staining of teeth Tarry stools Overdose is lethal!!!

Answer 4

Bone Marrow failure; All formed elements of the blood are simultaneously depressed—“pancytopenia”

Answer 5

red bone marrow converted to yellow, fatty marrow.

Answer 6

Primary (congenital) - Fanconi's anemia Secondary (acquired)

Answer 7

Bone marrow transplant Stem cell transplant (HSCT) Immunosuppressive therapy

Answer 8

same as Leukemia

Answer 9

Hemolysis Decreased life span of RBC Hereditary spherocytosis (HS) - Splenectomy/partial splenectomy can correct hemolysis but does not correct underlying defect - Splenectomy rarely before age 5 Aplastic crisis

Answer 10

A group of inherited hemoglobinopathies in which the RBCs do not carry the normal adult hemoglobin, but rather a less effective type. Most common type is Hgb SS or sickle cell anemia

Answer 11

Autosomal recessive hemolytic anemia Most common in persons of African, Mediterranean, Middle Eastern, & Indian decent. 1 in 12 African Americans have SC trait. People with SC trait have < 50% Hgb S 1 in 500 African Americans have sickle cell anemia Usually Asymptomatic until age 6-months In areas of the world where malaria is common, individuals with sickle cell trait tend to have a survival advantage over those without the trait

Answer 12

inherited, autosomal recessive several mutations in the B-globin gene that cause 6th amino acid to be changed from glutamic acid to valine. resultant hemoglobin (called HbS) has abnormal physiochemical properties, and is prone to polymerization with other hemoglobin molecules under conditions of low oxygen tension. number of adverse affects on erythrocytes. The normally freely flowing cytosol of red cells become viscous making the red cell much less deformable and impairing its ability to traverse tight capillary beds. As HbS continues to polymerize the entire RBC is deformed giving the characteristic sickle shape.

Answer 13

Acute Painful Episodes (Sickle Cell Crises) Stroke Sepsis Acute chest syndrome Reduced visual acuity Chronic leg ulcers Delayed growth & development Delayed puberty Priapism Enuresis

Answer 14

Due to the deformed shape, HbS induces RBC membrane damage leading to calcium influx into the cell. Calcium influx leads to crosslinking of the membrane proteins and activating channels that allow for the efflux of potassium and water from the cell. This leads to RBC dehydration exacerbating the sickling.Vaso-occlusive crisis results from the sickle red cells obstructing and reducing blood flow to the vital organs leading to ischemia, necrosis and pain. Repeated episodes lead to bone infarction and necrosis; and bone marrow degenerationoccurs overtime. Long bones are affected most commonly, but pain episodes can affect any bone marrow-containing structure, including the ribs, sternum, vertebral bodies, and skull.Pulmonary fat embolism can be a life threatening complication of bone marrow infarction in patients with SCD and precipitate Acute Chest Syndrome (ACS). Furthermore, hemoglobin released from hemolyzed cells readily binds to and depletes NO, leading to vascular smooth muscle cell contraction and enhanced platelet aggregation. Occlusions lead to further hypoxia within the tissue, setting up conditions for a vicious cycle in which further sickling and hypoxia occur.

Answer 15

Sickle cells are mechanically weak and are prone to intravascular hemolysis. However, the more important mechanism leading to decreased red cell survival time is the extravascular hemolysis that occurs when inflexible cells are trapped in the spleen and phagocytosed by the reticuloendothelial systemBone marrow tries to compensate by increasing RBC production but it cannot match the rate of destruction. A normal RBC survives for an average of 90-120 days, whereas, a sickle cell survives 10-20 days. Complications of increased hemolysis include cholelithiasis; due to excessive bilirubin production.

Answer 16

Encapsulated bacterial infections: Splenic sequestration of sickle cells leads to splenic congestion, as manifested by splenomegaly, and reduced immune function. Since the spleen is important for macrophage phagocytosis of encapsulated bacteria, patients with SCD are prone to bacteremia with pathogens like Streptococcus pneumoniae (S. pneumoniae), Haemophilus Influenzae (H. Influenzae) and Neisseria meningitidis (N. Meningitidis). These pathogens, normally causing localized disease, may cause life-threatening sepsis in patients with sickle cell disease.Furthermore, patients with SCD develop pneumonias, predominantly from atypical organisms, such as, Mycoplasma pneumoniae, Chlamydia pneumoniae and Legionella. Respiratory viruses are also common causes of pulmonary infection, while S. pneumoniae and H. influenza type b are uncommon.Osteomyelitis and septic arthritis can affect minority of patients with SCD due to both, bone damage and poor splenic function. The leading pathogens include Salmonella, S. Aureus and other gram negative bacteria. Autosplenectomy: Continued splenic dysfunction eventually leads to infarction and loss of splenic function, which is referred to as autosplenectomy or functional asplenia. Dysfunctional complement system has been proposed to contribute to the infectious complications in SCD, however, this has yet to be proven.

Answer 17

Neurologic complications are related to vasoocculusive crisis and include strokes and silent strokes.The acute pulmonary complications of SCD, collectively referred to as the acute chest syndrome (ACS). It is defined as the appearance of new infiltrate with pulmonary symptoms, presence of fever, hypoxia and chest pain (minority of patients might not have an infiltrate initially). Precipitants of ACS include infections (see above for details), infarction and/or pulmonary fat embolism.No specific cardiomyopathies are present however, two cardiac complications occur; chamber enlargement related to significantly increased forward output due to chronic anemia and sleep/wake oxygen desaturations and secondly, myocardial infarction from vasoocclusion of the coronary arteries. Rarely, RV dysfunction (Cor pulmonale) may develop due to Pulmonary arterial hypertension.

Answer 18

Acute exacerbations that vary in severity and frequency Precipitating factors - Anything that increases the body’s need for oxygen or alters transport of oxygen - Trauma - Infection, fever - Physical and emotional stress - Increased blood viscosity due to dehydration - Hypoxia - From high altitude, poorly pressurized airplanes, hypoventilation, vasoconstriction due to hypothermia

Answer 19

Vaso-occlusive (VOC) thrombotic Splenic sequestration Aplastic crises Acute Chest Syndrome

Answer 20

AKA painful event/ painful episode Most common type of crisis—very painful Stasis of blood with clumping of cells in microcirculation → ischemia → infarction Signs—fever, pain, tissue engorgement Treatment: Hydration, Pain management, heat

Answer 21

Life-threatening—death can occur within hours Blood pools in the spleen Signs - Profound anemia - Hypovolemia - Shock Treatment: Splenectomy, Transfusions

Answer 22

Diminished production and increased destruction of RBCs Triggered by viral infection or depletion of folic acid Signs include profound anemia, pallor Treatment: Splenectomy, Transfusions

Answer 23

Similar to pneumonia VOC or infection results in sickling in the lungs Chest pain, fever, cough, tachypnea, wheezing, and hypoxia Repeated episodes may lead to pulmonary hypertension Treatment: Adequate pain management, Antibiotics, with cover for atypical organisms, ICU, ventilator support, blood transfusion , exchange transfusion may be indicated if no improvement, bronchodilators if asthma like symptoms

Answer 24

Prognosis varies, highest risk are children under 5-years old from infection Life expectancy currently in the mid-forties No cure (except possibly bone marrow transplants) Frequent bacterial infections may occur due to immunocompromise Bacterial infection is leading cause of death in young children with sickle cell disease Strokes in 5% to 10% of children with disease - Result in neurodevelopmental delay, cognitive delays

Answer 25

Diagnosed with Hgb Electrophoresis; part of the newborn screen, Cord blood in newborns Newborn screening done in 43 states Genetic testing to identify carriers and children who have disease Sickle turbidity test - Quick screening purposes in children older than 6 months Palliative Treatment-Erythropoietin or hydroxyuria ( increase Hgb F production), PCN prophylaxis Transcranial Doppler annually Preventing Acute Painful Episodes by Avoiding known triggers of the sickling phenomenon, infection, & other complications Transcranial Doppler annually to assess intracranial blood flow and determine CVA risk Crisis Management Hyper-transfusion; keep Hgb > 10Frequent transfusion leads to hemosiderosis (iron in tissues) Treat with iron chelation - Parenterally—deferoxamine - Oral—deferasirox or deferiprone - Used alone or in combination + Vitamin C to promote iron excretion Bone Marrow Transplant

Answer 26

Maintain Hydration Maintain Oxygenation Pain management Support for the Child & Family

Answer 27

Inherited blood disorders of hemoglobin synthesis Classified by Hgb chain affected and by amount of effect Autosomal recessive with varying expressivity - Both parents must be carriers to have offspring with disease

Answer 28

Alpha or beta-globulin chain in Hgb synthesis is reduced or entirely absent. - Large number of unstable globulin chains, - Causes RBCs to be rigid and easily hemolyzed - Severe hemolytic anemia leading to hypoxia Normal RBC size with decreased amounts of Hgb Chronic hypoxia - Headache, irritability, precordial and bone pain, exercise intolerance, anorexia, epistaxis Detected in infancy or toddlerhood - Pallor, FTT, hepatosplenomegaly, severe anemia (Hgb <6)

Answer 29

alpha beta

Answer 30

α chains affected Occurs in Chinese, Thai, African, and Mediterranean people

Answer 31

Occurs in Greeks, Italians, and Syrians b is most common and has four forms - Thalassemia minor - Thalassemia trait - Thalassemia intermediate - Thalassemia major “Cooley anemia"

Answer 32

asymptomatic silent carrier

Answer 33

mild microcytic anemia

Answer 34

moderate to severe anemia + splenomegaly

Answer 35

severe anemia requiring transfusions to survive

Answer 36

Anemia; severe Bronze skin tone from hemosidorosis. (excessive iron storage in various tissues) Growth retardation and delayed puberty Cardiac enlargement with flow murmur leading to heart failure Bone pain, skeletal deformities, fractures Death usually related to heart failure or iron overload

Answer 37

Diagnosed with Hgb Electrophoresis Blood transfusion to maintain normal Hgb levels Side effect—hemosiderosis - Treat with iron-chelating drugs such as deferoxamine - Binds excess iron for excretion by kidney - IV or SQ over 8-10 hours multiple times/wk - May be given at home with IV pump per parents - New oral chelation drugs—deferasirox ;Worldwide use Also give oral vitamin C to facilitate binding of iron

Answer 38

Administer packed Red Blood Cells safely. General measures for anemia Monitor chelation Therapy - N/V/D - Decreased appetite - Rash - Increased liver enzymes - Neutropenia - *excreted via the kidneys so adequate hydration is vital Measures to enhance self-esteem & body image Measures to enhance home health maintenance

Answer 39

1) blood vessels get smaller 2) platelet plug is made: - Adhesion- platelets sticking to wall of torn blood vessel - Activation and secretion- platelets change shape to connect with other platelets - Aggregation- platelets stick to each other and to the blood vessel ALL OF THIS IS TEMPORARY 3) clotting factors- A fibrin clot made from clotting factors is needed to make a fibrin weave to make strong clot or strong seal.

Answer 40

Idiopathic Thrombocytopenic Purpura Hemophilia

Answer 41

the cellular fragments needed to form a clot.

Answer 42

low platelet count

Answer 43

leukemia, aplastic anemia

Answer 44

Sickle cell splenic sequestration crises

Answer 45

Most frequent occurring thrombocytopenia of childhood. Acquired Hemorrhagic disorder Autoimmune destruction of platelets in the spleen May be acute or chronic. Cause unknown - thought to be an immune response following a viral infection (measles, C-pox, or rubella) that produces antiplatelet antibodies. Occurrence ; Caucasian children between 2 & 8 years old Most children recover spontaneously in 6-12 months

Answer 46

Sudden onset of easy bruising, purpura, and petechiae Epistaxis Platelet count less than 20,000 Prolonged bleeding time

Answer 47

Primarily Supportive; depends on platelet count Anti D Antibody- Plasma derived immunoglobulin. One dose over 5-10 minutes IV. Causes transient anemia by clearing the antibody coated RBCs. Intravenous Immune Globulin (IVIG). Course of Steroids (1-2 weeks) Splenectomy and IV Gamma Globulin if chronic

Answer 48

Educate Parents Assess for bleeding - hematomas - bruising - bleeding gums - tarry stools - hematuria - obvious bleeding. Watch for intracranial bleed: - irritability, - change in level of consciousness, - head ache, - slurred speech. Safety measures to prevent trauma Soft toothbrush, restrict activity at the onset until platelet count is up, Avoid unnecessary procedures, needle sticks, suppositories, rectal temps. etc. Apply pressure for 5 minutes after venipuncture. Follow-up care in home or clinic

Answer 49

Seek medical attention for a nose bleed lasting longer than 30-minutes Sit up leaning forward Apply pressure for 10-minutes Apply ice bag to bridge of nose.

Answer 50

A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors

Answer 51

Classic hemophilia Deficiency of factor VIII Accounts for 80% of cases of hemophilia Occurrence—1 in 5000 males

Answer 52

Also known as Christmas disease Caused by deficiency of factor IX Accounts for 15% of cases of hemophilia

Answer 53

X-linked recessive trait Males are affected Females may be carriers Degree of bleeding depends on amount of clotting factor and severity of a given injury Up to one third of cases have no known family history - In these cases disease is caused by a new mutation

Answer 54

Bleeding tendencies range from mild to severe Symptoms may not occur until 6 months of age - Mobility leads to injuries from falls and accidents Hemarthrosis - Bleeding into joint spaces of knee, ankle, elbow, leading to impaired mobility Ecchymosis Epistaxis Bleeding after procedures - Minor trauma, tooth extraction, minor surgeries - Large subcutaneous and intramuscular hemorrhages may occur - Bleeding into neck, chest, mouth may compromise airway

Answer 55

stiffness, tingling, or ache followed by decreased mobility, warmth, redness, swelling, & severe pain.

Answer 56

Prevent Bleeding - infant-padded crib. - Home; no sharp corners, clutter free, soft carpeted floors, use protective equipment, - avoid contact sports and risky activities Replacement of deficient factor - Factor VIII concentrate - purified from large plasma pools or synthetic, long shelf life. Powder that can be mixed just prior to IV administration. Blocks aldosterone so potassium supplements not needed. or - DDAVP (desmopressin) - Synthetic form of vasopressin, increases factor VIII production. Intranasal. 8-10 yo can self-administer Inhibitors- FEIBA (Anti Inhibitor Coagulant Complex) can cause blood clots Regular program of exercise & PT - helps to keep muscles & joints stronger so they have fewer bleeds.

Answer 57

safe environment teach the use of protective equipment avoid aspirin SQ rather than IM, venipuncture rather than finger stick, no suppositories, adolescents need electric razors, avoid contact sports. Soft toothbrush

Answer 58

Pressure X 15 minutes for frank bleeding RICE ; Rest (Immobilize), Ice, Compression, elevation Children are aware of internal bleeding and can tell you. Be suspicious of head ache, slurred speech, altered LOC, black tarry stools.

Answer 59

long term effects of bleeding into joints & muscles = incomplete re-absorption of blood & limited ROM results in bone & muscle changes, flexion contractures, & joint fixation. active ROM after the acute phase physical therapy nutritional counseling

Answer 60

Med Alert bracelet Notify school nurse and teacher.

Answer 61

A hereditary bleeding disorder involving deficiency of von Willebrand factor (a plasma protein and the carrier for factor VIII) von Willebrand factor needed for platelet adhesion Transmitted as autosomal dominant trait Occurs in both males and females The gene for the disease is located on chromosome 12

Answer 62

Easy bruising Epistaxis Gingival bleeding Excessive bleeding with lacerations or surgeries Menorrhagia

Answer 63

Decreased von Willebrand factor levels von Willebrand antigen levels Decreased platelet agglutination Prolonged bleeding time PTT may be normal or prolonged

Answer 64

Infusion of von Willebrand protein concentrate DDAVP infusion before surgery, during menses, or to treat bleeding episode

Answer 65

Avoid aspirin or NSAIDs (increase bleeding time and inhibit platelet function) Manage bleeding episodes with prompt infusion therapy Children with vWD have normal life expectancy if well managed

Answer 66

Occurs in ages 6 months to 16 years Generalized vasculitis of dermal capillaries Extravasation of RBCs, produces petechial skin lesions Inflammation and hemorrhage in GI tract May lead to ongoing nephrotic syndrome

Answer 67

unknown; often follows URI

Answer 68

Most cases resolve without treatment Corticosteroids and anticoagulants for severe or persistent cases Symptomatic treatment Maintain hydration Monitor renal function