Week 2 Ch 33 Management of Patients With Nonmalignant Hematologic Disorders

Nursing intervention DIC

-Assessment and interventions should target potential sites of organ damage -Monitor and assess carefully -Avoid trauma and procedures that increase the risk of bleeding, including activities that would increase intracranial pressure

what is neutropenia?

-Decreased production or increased destruction of neutrophils (<2000/mm3) -Increased risk for infection: monitor closely -Absolute neutrophil count (ANC) -Medical management: treatment depends on the cause -Nursing management: patient education, preventing and managing complications -Refer to Chart 33-5

Bleeding Disorders #1

-Failure of hemostatic mechanisms -Causes oTrauma oPlatelet abnormality oCoagulation factor abnormality -Medical management: specific blood products -Nursing management: limit injury, assess for bleeding, bleeding precautions

Disseminated Intravascular Coagulation (DIC)

-Not a disease but a sign of an underlying disorder -Severity is variable; may be life threatening -Triggers may include sepsis, trauma, shock, cancer, abruptio placentae, toxins, and allergic reactions -Altered hemostasis mechanism causes massive clotting in microcirculation. As clotting factors are consumed, bleeding occurs. Symptoms are related to tissue ischemia and bleeding -Laboratory tests -Treatment: treat underlying cause, correct tissue ischemia, replace fluids and electrolytes, maintain blood pressure, replace coagulation factors, use heparin or LMWH

what are clinical manifestations of SCD?

-anemia is always present -Hgb values range from 5-11 -Jaundice is a characteristic and is usually obvious in sclerae -enlargement of bone marrow to compensate for anemia

nursing management of hemophilia

-instruct how to give the factor concentrate at home at the first sign of bleeding -assess bleeding and vital signs -analgesics are common to alleviate pain heat is avoided

what causes iron deficiency anemia?

1. Decreased intake -seen in infants less than 6 months 2. increased demand -in child infant and pregnancy 3. decreased absorption -due to decreased acid or in duodenum -seen in celiac disease and antacids protein pump inhibitors (tums) 4. Increased blood loss -heavy blood loss -peptic ulcer disease

what are the 3 types of sickle cell crisis?

1. acute vaso-occlusive crisis, which results from entrapment of erythrocytes and leukocytes in the microcirculation, causing tissue hypoxia, inflammation, and necrosis due to inadequate blood flow to a specific region of tissue or organ 2. Aplastic crisis results from infection with the human parvovirus. The hemoglobin level falls rapidly and the marrow cannot compensate, as evidenced by an absence of reticulocytes 3. Sequestration crisis results when other organs pool the sickled cells. Although the spleen is the most common organ responsible for sequestration in children, most children with SCD have had a splenic infarction by 10 years of age, and the spleen is then no longer functional. Common organs involved is liver and lung.

hemophilia

A hereditary disease where blood does not coagulate to stop bleeding

what is acute chest syndrome in sickle cell disease patients?

Acute chest syndrome is manifested by fever, respiratory distress (tachypnea, cough, wheezing), and new infiltrates seen on the chest x-ray; it is the main cause of death in young adults with SCD Clinically, the patient's condition can deteriorate rapidly, resulting in respiratory failure. Medical management includes red cell transfusion, antimicrobial therapy, bronchodilators, inhaled nitric oxide therapy, and when respiratory failure ensues, mechanical ventilation.

multiple organ dysfunction syndrome symptoms

An altered mental state; A decrease in renal perfusion (decrease in urine output); Respiratory deterioration; A decrease in cardiac function; Deranged metabolic status; A compromised fluid balance

Dabigatran (Pradaxa)

Anticoagulant

Apixiban (Eliquis)

Anticoagulant, Factor Xa Inhibitor

aplastic anemia

Aplastic anemia is a rare disease caused by a decrease in or damage to marrow stem cells, damage to the microenvironment within the marrow, and replacement of the marrow with fat. Stem cell damage is caused by the body's T cells mediating an inappropriate attack against the bone marrow, resulting in bone marrow aplasia

reproductive problems and SCD

As patients live longer with SCD, the adverse impact on sexual function is now better appreciated. Almost 25% of men with SCD develop hypogonadism low test, libido and other complications can occur. infertility possible. Women may have pain during menstruation, fertility issues not well researched in women, contraception is important when hydroxurea is used to treat SCD as it may be teratogenic.

Nursing management of DIC

Assessment and interventions should target potential sites of end-organ damage. As organs become ischemic from microthrombi, organ function diminishes; the kidneys, lungs, brain, and skin are particularly vulnerable. Be aware of patients who are at risk for DIC and assess for signs and symptoms of the condition Assess for signs and symptoms and progression of thrombi and bleeding

What type of anemia results from red blood cell destruction? A. Bleeding B. Hemolytic C. Hypoproliferative D. None of the above

B.Hemolytic Bleeding results from red blood cell loss. Hemolytic anemia results from red blood cell destruction. Hypoproliferative anemia results from defective red blood cell production

interventions for caring patients with anemias?

Balance physical activity, exercise, and rest Maintain adequate nutrition Maintain adequate perfusion Patient education to promote compliance with medications and nutrition Monitor VS and pulse oximetry; provide supplemental oxygen as needed Monitor for potential complications

what foods hellp with iron intake?

Beef, calf liver, chicken liver, meats, beans, leafy greens, raisins, and molasses and take with vitamin C or orange juice.

menifestations of thrombocytopenia?

Bleeding and petechiae usually do not occur with platelet counts greater than 50,000/mm When the platelet count drops to less than 20,000/mm3, petechiae can appear, along with nasal and gingival bleeding, excessive menstrual bleeding, and excessive bleeding after surgery or dental extractions When the platelet count is less than 5000/mm3, spontaneous, potentially fatal central nervous system or GI hemorrhage can occur.

how is hemophilia inherited?

Both types of hemophilia are inherited as X-linked traits, so most affected people are males; females can be carriers but are almost always asymptomatic

what causes neutropenia?

Decreased Production of Neutrophils -Aplastic anemia, due to medications or toxins -Chemotherapy -Metastatic cancer, lymphoma, leukemia -Myelodysplastic syndromes -Radiation therapy Ineffective Granulocytopoiesis -Megaloblastic anemia Increased Destruction of Neutrophils -Bacterial infections -Hypersplenism -Immunologic disorders (e.g., systemic lupus erythematosus) -Medication induceda -Viral disease (e.g., infectious hepatitis, mononucleosis)

anemia manifestation depends on?

Depends on the rapidity of the development of the anemia, duration of the anemia, metabolic requirements of the patient, concurrent problems, and concomitant features

what are the manifestations of anemias?

Fatigue, weakness, malaise Pallor or jaundice Cardiac and respiratory symptoms Tongue changes Nail changes Angular cheilosis Pica

what is the patho of megaloblastic anemia?

Folic acid deficiency -people who rarely eat uncooked veggies -alcoholism increases requirment -liver disease Vitamin B12 deficiency - faulty GI tract absorption -Crohns disease after GI surgery -Pernicious anemia - lack of intrinsic factor b12 cannot be absorbed

medical management of sickle cell disease

Hematopoietic Stem Cell Transplant -HSCT may cure SCD pharmacological therapy -hydroxyurea, a chemotherapy agent that is effective in increasing fetal hemoglobin; the only fda approved drug -folic acid replacement -acute chest syndrome-> antibiotic therapy transfusion therapy - RBC transfusion is highly effective in acute exacerbation of anemia supportive therapy -pain management is caused by vasocclusive crisis and is common ed visit -nsaid use, cieling effect -hydration is important -fatigue is also a big problem

what are diagnostic test for anemias?

Hemoglobin and hematocrit Reticulocyte count RBC indices Iron studies Vitamin B12 Folate Haptoglobin and erythropoietin levels Bone marrow aspiration

hemophilia A vs B

Hemophilia A is caused by a genetic defect that results in deficient or defective factor VIII. Hemophilia B (also called Christmas disease) stems from a genetic defect that causes deficient or defective factor IX.

hemophilia manifestations

Hemophilia is manifested by hemorrhages into various parts of the body; these hemorrhages can be severe and can occur even after minimal trauma

what is sickle cell disease?

I know it is a genetic disorder that leads to the defect of the RBC. Can inhibit o2 carrying ability, decreased lifespan of RBC, it can lead to abnormal agglutination. can cause pain during flair ups. increase for stroke. rbc can return to normal if enough o2 such as in pulmonary circulation. sickle cell crises is important. Cold can aggrivate it. can be a recessive gene however if a child obtains two abnormal genes they will have the disease.

Immune thrombocytopenia purpura

ITP is a disease that affects people of all ages, but it is more common among children and young women Primary ITP occurs in isolation; secondary ITP often results from autoimmune diseases (e.g., antiphospholipid antibody syndrome), viral infections (e.g., hepatitis C, HIV), and various drugs (e.g., sulfa drugs). Primary ITP is defined as a platelet count less than 100 × 109/L with an inexplicable absence of a cause for thrombocytopenia

Secondary thrombocytosis

Increased platelet production is the primary mechanism of secondary, or reactive, thrombocytosis.

Anemias

Lower than normal hemoglobin and fewer than normal circulating erythrocytes; a sign of an underlying disorder

what is the major goal with patients with anemia?

Major goals include decreased fatigue, attainment or maintenance of adequate nutrition, maintenance of adequate tissue perfusion, compliance with prescribed therapy, and absence of complications

Major goal with DIC

Major goals may include maintenance of hemodynamic status, maintenance of intact skin and oral mucosa, maintenance of fluid balance, maintenance of tissue perfusion, enhanced coping, and absence of complications

what is Pica?

Patients with iron deficiency anemia may infrequently crave ice, starch, or dirt

ITP manifestations

Petechiae and purpura, progressing to major hemorrhage

DIC Lab value

Platelet Normal 150,000-400,000 DIC: decreased Prothrombin time (PT) Normal 11-12.5 seconds DIC: ↑ Partial thromboplastin time (activated)(aPTT) 23-35 seconds DIC: ↑

Heparin induced thrombocytopenia

Platelet destruction that arises secondary to heparin therapy hypercoagulable state and thrombocytopenia needs to be taken off heparin

multiple organ dysfunction syndrome

Progressive dysfunction of two or more organ systems resulting from an uncontrolled inflammatory response to severe illness or injury

medical management of hemophilia

Replacement of missing clotting factors Desmopressin (DDAVP) IV Increases factor VIII activity by two to four times Used for mild hemophilia Transfusions Prompt intervention to reduce complications Medications Exercise and physical therapy

secondary polycythemia

Secondary polycythemia is caused by excessive production of erythropoietin. This may occur in response to a reduced amount of oxygen, which acts as a hypoxic stimulus, as in heavy cigarette smoking, obstructive sleep apnea (OSA), chronic obstructive pulmonary disease (COPD), or cyanotic heart disease, or with conditions such as living at a high altitude or exposure to low levels of carbon monoxide.

In any condition where you have a problem making hemoglobin what occurs to the RBC?

The RBC ends up microlytic RBC is big with little filling so body responds by cutting the RBC in half and making it smaller

Vitamin K deficiency

The synthesis of many coagulation factors depends on vitamin K. Vitamin K deficiency is common in malnourished patients. Prolonged use of some antibiotics decreases the intestinal flora that produces vitamin K, depleting vitamin K stores. Administration of vitamin K (phytonadione [Mephyton]), either orally or as a subcutaneous injection, can correct the deficiency quickly; adequate synthesis of coagulation factors is reflected by normalization of the PT.

What is thalassemia?

The thalassemias are a group of hereditary anemias characterized by hypochromia (an abnormal decrease in the hemoglobin content of erythrocytes), extreme microcytosis (smaller-than-normal erythrocytes), hemolysis, and variable degrees of anemia. The thalassemias occur worldwide, but the highest prevalence is found in people of Mediterranean, African, and Southeast Asian ancestry

Nursing management of aplastic anemia?

They should be assessed carefully for signs of infection and bleeding Nurses must also monitor for side effects of therapy, particularly for hypersensitivity reaction while administering ATG

thrombocytopenia

Thrombocytopenia (low platelet level) can result from various factors: decreased production of platelets within the bone marrow, increased destruction of platelets, or increased consumption of platelets

Vitamin K and warfarin

Vit K is used to prevent bleeding and/or hemorrhage. Used to correct Warfarin OD. Pts on Warfarin should NOT ingest foods high in Vit K, as the combo may lead to therapeutic failure.

thrombocytopenia management

When selecting nursing interventions, the nurse considers the cause of the thrombocytopenia, the likely duration, and the overall condition of the patient. Education is important, as are interventions to promote patient safety, particularly fall prevention in the older adult or patient who is frail. The interventions for a patient with thrombocytopenia are the same as those for a patient with cancer who is at risk for bleeding

most hemophilia bleeding occurs where?

about 75% of all bleeding in patients with hemophilia occurs into joints

Microlytic anemia

anemia characterized by small RBCs caused by iron deficiency

what is megaloblastic anemia?

anemias caused by deficiencies of vitamin B12 or folic acid, identical bone marrow and peripheral blood changes occur because both vitamins are essential for normal DNA synthesis. In either case, the erythrocytes that are produced are abnormally large and called megaloblastic red cells

Endoxaban (Savaysa)

anticoagulant medication and a direct factor Xa inhibitor. It is taken by mouth.

megaloblastic anemia symptoms

beefy red tongue

von willebrand disease

bleeding disorder caused by a deficiency of von Willebrand factor, a "sticky" protein that lines blood vessels and reacts with platelets to form a plug that leads to clot formation Bleeding tends to be mucosal. Patients commonly have recurrent nosebleeds, easy bruising, heavy menses, prolonged bleeding from cuts, and postoperative bleeding. Massive soft tissue or joint hemorrhages are not often seen, unless the patient has severe type 3 vWD

Prognosis of sickle cell disease

children often die at 1 year. crisis at 1-2 years. complications diminish by 30. lifespan about 45-47 y/o. Death is most commonly due to heart disease (32%), lung disease (28%), kidney disease (16%), infection (14%), and neurologic system disease (12%); less commonly, death results from GI tract or liver disease (9%) and cancer (<1%)

what is medical management of anemias?

correct or control cause transfusion of PRBC Treatment : diet, iron or vitamins, transfusions, immunosuppressive therapy, other

Hypoproliferative anemia

defect in production of RBCs Caused by iron, vitamin B12, or folate deficiency, decreased erythropoietin production, cancer include: vIron deficiency anemia vAnemia in renal disease vAnemia of inflammation vAplastic anemia vMegaloblastic anemia

rivaroxaban (Xeralto)

direct factor Xa inhibitor

Hemolytic anemia

excess destruction of RBCs caused by altered erythropoiesis or other causes such as hyperplenism, drug-induced or autoimmune processes, mechanical heart valves may also be caused by blood loss include: Sickle cell disease Thalassemia Glucose-6-phosphate dehydrogenase deficiency Immune hemolytic anemia Hereditary hemochromatosis Others (refer to Chart 33-1)

what assessment information is important in patient with anemia?

health history and physical exam laboratory data presence of symptoms and impacts of those symptos on patient's life nutritional assessment medications cardiac and GI assessment Blood loss: menses, potential GI loss neurological assessment

nursing management of megaloblastic anemia

inspection of the skin, mucous membranes, and tongue The nurse needs to pay particular attention to ambulation and should assess the patient's gait and stability, as well as the need for assistive devices Because mouth and tongue soreness may limit nutritional intake, the nurse advises the patient to eat small amounts of bland, soft foods frequently. The nurse also may explain that other nutritional deficiencies, such as alcohol-induced anemia, can induce neurologic problems.

pulmonary hypertension and SCD

is a common sequela of SCD and is a common cause of death. pt may c/o dyspnea, fatigue, chest pain, or syncope, SPO2 may be normal Doppler echocardiography is used to identify elevated pulmonary artery pressures; High levels of the amino-terminal form of brain natriuretic peptide can serve as a useful biomarker for pulmonary hypertension; CT also useful

DIC clinical manifestation

may bleed from mucous membranes, venipuncture sites, and the GI and urinary tracts. The bleeding can range from minimal occult internal bleeding to profuse hemorrhage from all orifice

is polycythemia a form of anemia?

no. Polycythemia is a situation of excess red blood cells, not a deficiency, which would be called anemia

DIC patho

normal anticoagulant pathways within the body are messed up, and the fibrinolytic system is suppressed so that a massive amount of tiny clots forms in the microcirculation. At first the coagulation time is normal. However, as the platelets and clotting factors form microthrombi, coagulation fails. So the paradoxical result includes excessive clotting and bleeding and organ failure.

platelet defects

quantitative platelet defects (i.e., thrombocytopenia, thrombocytosis) are relatively common; however, qualitative defects can also occur. With qualitative defects, the number of platelets may be normal but the platelets do not function normally. A platelet function analyzer is used to evaluate platelet function; this method is particularly valuable for rapid and simple screening.

primary polycythemia

refers to an increased volume of RBCs. The term is used when the hematocrit is elevated (more than 55% in males, more than 50% in females). Dehydration (decreased volume of plasma) can cause an elevated hematocrit but not typically to the level to be considered polycythemia. Polycythemia is classified as either primary or secondary. Primary polycythemia, also called polycythemia vera, is a proliferative disorder

shilling test

test used to diagnose pernicious anemia by determining if the body properly absorbs vitamin B12 through the digestive tract The patient is given radiolabeled vitamin B12 orally, following an intramuscular (IM) dose of unlabeled vitamin B12 one hour later. The injection is given to ensure that none of the radioactive B12 binds to any vitamin B12 depleted tissues, for example, the liver.

thrombocytosis

thrombocytosis: higher-than-normal platelet count

angular cheilosis?

ulcerated corners of the mouth

restless leg syndrome

uncomfortable sensations in legs causing movement and loss of sleep common in 24% with iron deficient anemia

how to reduce acute chest syndrome in SCD patients?

use incentive spirometer during episodes of vaso-occlusive crisis and red cell transfusion preoperatively.

DIC assessment findings

↓ Temperature, sensation; ↑ pain; cyanosis in extremities, nose, earlobes; focal ischemia, superficial gangrene ↓ Pulses; capillary filling time >3 seconds Hypoxia (secondary to clot in lung); dyspnea; chest pain with deep inspiration; ↓ breath sounds over areas of large embolism gastric pain and heartburn ↓ Urine output; ↑ creatinine, ↑ blood urea nitrogen ↓ Alertness and orientation; ↓ pupillary reaction; ↓ response to commands; ↓ strength and movement ability