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- Sickle cell- African American
- Thalassemia- African American & Mediterranean
- Tay-Sachs- Eastern European Jewish (Ashkenazi Jews)
- Pernicious anemia- African American & Scandinavians
Mild states of anemia
Hb 10-14 (may exists without causing symptoms) s/s palpitations, dyspnea, diaphoresis
- Hb 6-10
- cardiopulmonary symptoms occur and pt may experience them while resting, as well as with activity
- has many clinical manifestations involving multiple body systems
Iron deficiency anemia
- due to poor diet, malabsorption, blood loss, hemolysis(destruction of RBC) duodenum removal, pregnancy, menstruation, alcoholism & dialysis.
- s/s Pallor, tongue & lip swelling, headache, tingling sensation, burning of the tongue
Collaborative Care for Iron deficiency anemia
- Give daily dose of oral iron up to 150-200mg a day
- Give 1hr. before or after meals, or with orange juice
- It stains pts teeth so give through a straw to back of teeth
- Give stool softeners or laxatives for constipation
- Give IM no more than 2ml upper outer quadrant of buttocks19-20 gauge needle and no massage is necessary.
- Give iron 2-3mo & monitor liver
Inadequate production of normal hemoglobin. Due to an absent or reduced globulin protein. Growth, both physical & mental is often retarded. Small & pale cells. The symptoms develop in childhood & cause growth & developmental deficits.
caused by impaired DNA characterized by the presence of large RBC's. Cobalamin & folic acid deficiency (vit B12)
is a disease in which the gastric mucosa is not secreting IF(intrinsic factor) because antibodies being directed against the gastric parietal cells & IF itself. Begins usually after 40. Pt is unable to absorb vit B12 if intrinsic factor is lacking.
Collaborate care for Pernicious anemia
Regardless of how much is ingested, the pt is not able to absorb vit B12 if intrinsic factor is lacking, therefore increasing cobalamin does not correct anemia. Tx of choice is parenteral or intranasal administration of vit B12. Pt needs IM daily for 2wks until hematocrit is normal, then monthly FOR LIFE!
Folic Acid deficiency
Treated by replacement therapy. Dose is 1mg per day by mouth. In malabsorption up to 5mg per day may be required. The duration depends on the reason for deficiency. The pt should be encouraged to eat foods containing large amounts of folic acid.
Nursing management for Megablastic anemia
the nurse should ensure that injuries are not sustained because of the diminished sensations to heat and pain resulting from the neurologic impairment. Protect pt from falls, burns & trauma.
Anemia of chronic disease
is associated with an underproduction of RBC's & mild shortening of RBC's survival, caused by chronic inflammatory, autoimmune, infectious, or malignant diseases. Normal folate & cobalamin levels, elevated serum ferratin & increased iron storage.
is a disease in which the pt has peripheral blood (pancytopenia) decrease of all blood cell types & hypocellular bone marrow.
Nursing & collaborative care for aplastic anemia
actions are directed at preventing complications from infections & hemorrage. Tx of choice is blood transfusion from a matched donor (HSCT)Hematopoietic stem cell transplant.
Acute blood loss anemia
There is a sudden reduction in the total blood volume that can lead to hypovolemic shock. The #of RBC's available to carry oxygen is significantly diminished. The major complication in blood loss is shock.
Collaborative care for blood loss
- replace blood volume to prevent shock
- identify the source of the hemorrhage & stop the blood loss.
- pt might need supplemental iron, oral or parenteral iron preparations are given
a condition caused by the destruction or hemolysis of RBC's at a rate that exceeds production. The main focus is to maintain renal function.
Sickle cell disease
inherited, autosomal resesive disorder characterized by the presence of an abnormal form of hemoglobin in the erythrocyte. It causes the erythrocyte to stiffen & elongate taking on a sickle shape in response to low oxygen level. Usually identified in childhood. It is an INCURABLE disease that is fatal by middle age from renal & pulmonary failure.
Sickling episodes are trigered by:
viral or bacterial infections, high altitude, emotional or physical stress, surgery, blood loss, dehydration, increased hydrogen ion concentration (acidosis) increased plasma osmolarity, decrease plasma volume, & low body temperature.
Nursing care for pt with (SCD) sickle cell disease
Minimizing end organ damage, there is no specific tx, but they can avoid altitudes, maintain adequate fluid intake, treat infections promptly. Pain meds are also given(PCA)Demerol is contraindicated due to accumulation of toxic metabolite wich can cause seizures.
Acquired hemolytic anemia
- Isoimmune reactions: occur when antiboides develop against antigens from another person of the same species.
- Autoimmune reactions: result when individuals develop antibodies against their own RBC's.
- Supportive care ma include administering corticosteroids & blood products or removing the spleen.
is an autosomal recessive disease characterized by increased intestinal iron absorption & as a result, increased tissue iron deposition. have high concentrations up to 50g when norms are 2-6g. Physical examinations reveal an enlarged liver & spleen & pigmentation changes in the skin.
Nursing care for Hemochromatosis
remove excess iron from the body & minimize any symptoms the pt may have. Iron removal is achieved by removing 500ml of blood each wk for 2-3yrs until iron stores in the body are depleted. Dietary modifications may reduce iron accumulations, avoid Vit.C & iron supplements, uncooked seafood & iron rich foods. Most common causes of death are cirrhosis, liver failure, hepatic carcinoma & cardiac failure.
Polycythemia(Polycythemia vera & Secondary polycythemia)
is the production & presence of increased numbers of RBC's. Blood circulation is impaired as a result of the increased blood viscosity(hyperviscosity) & volume (hypervolemia) Splenomegaly & hepatomegaly are common.
Nursing care for Polycythemia
- Phlebotomy is tx of choice. Reduce the hematocrit & keep it less than 45%-48%
- Iron supplement should be AVOIDED! Maintain adequate Oxygenation, stop smoking, avoid high altitudes, measure I&O's, assess nutritional status, active ROM must be initiated to prevent thrombus, which is the major mortality cause.
is a reduction of platelets below 150,000 (150x109/L) prolonged bleeding from a minor trauma to spontaneous bleeding without injury.
Immune Thrombocytopenic Purpura (ITP)
Idiopathic thrombocytopenic purpura
is an autoimmune disease, platelets are coated with antibodies, when they reach the spleen they are recognized as foreign & are destroyed by the macrophages, usual survival rate of normal platelets is 8-10days, but these last 1-3days.
Thrombotic Thrombocytopenic Purpura (TTP)
is an uncommon syndrome characterized by hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever(in absence of infections) & renal abnormalities. Often referred to as TTP-HUS. Its a medical emergency because bleeding and cloting occur simultaneously.
Heparin induced Thrombocytopenia & Thrombosis Syndrome (HITTS)
also called white clot syndrome. It can be mild (type I) or severe (type II) an estimate % of pts on Heparin develop HITTS. The major clinical problem is venous Thrombosis, artirial vascular infarcts resulting in skin necrosis, stroke, & end organ damage, such as to kidneys. Heparin must be discontinued. Coumadin may be started when platelet count has reached 100,000
Clinical manifestations for pts with thrombocytopenia
Bleeding. Nosebleeds, gingival bleeding & large bullous hemorrhages may appear on the buccal mucosa. Bleeding into the skin by petechiae, purpura, or superficial ecchymoses(bruising) major complication is hemorrhage. when the count drops below 20,000 spontaneous life threathening hemorrhages can occur.
Hemophilia & Von Willebrand Disease
(Hemophilia A:Factor VIII)
(Hemophilia B:Factor IX)
is a sex-linked recessive genetic disorder caused by defective or deficiency coagulation factor. Slow, persistent, prolonged bleeding from a minor or small cut.(several hrs. or days) uncontrollable bleeding from dental procedures, nosebleed, GI bleeding from ulcers & gastritis, brusing, pain, paralysis.
Disseminated Intravascular Coagulation (DIC)
is a serious bleeding & thrombotic disorder. Proguse bleeding that results from the depletion of platelets & clotting factors. Its not a disease, its an abnormal response to an underlying disease & can lead to multiorgan failure. It can range from easy bruising to hemorrhage, and hypercoagulability to thrombosis.
decrease in the total WBC count. Signs of inflammation, redness, or heat are absent, no pus, pts are at high risk of infections. A low grade fever is of great significance.
Nursing management for Neutropenia
- determine the cause of it
- identify the offending organisms if infection has developed
- institue propylactic, empiric or therapeutic antibiotic
- administer hematopoietic growth factor
- institute protective environment practices such as handwashing, private room, +pressure room, visitor restriction
- avoid tap water, fresh fruits & veggies
Myelodysplastic Syndrome (MDS)
"clonal disorder" because some bone marrow stem cells continue to funciton normal whereas others dont. Manifests as infection & bleeding caused by inadequate numbers of ineffective functioning circulating granulocytes or platelets.
Nursing Management for Myelodysplastic Syndrome
Hematologic monitoring, antibiotic therapy, or transfusions with blood products. Overall goal is to improve hematopoiesis & ensure age-related quality of life.
Leukemia (acute & chronic)
is the general term used to describe a group of malignant disorders affecting the blood & blood forming tissues of the bone marrow, lymph system & spleen. Acute refers to immature cells, Chronic refers to mature forms of the WBC's & the disease onset is more gradual.
Four types of Leukemia
- Acute lymphocytic leukemia(ALL)
- Acute myelogenous leukemia (AML)
- Chronic myelogenous (granulocytic) leukemia (CML)
- chronic lymphocytic leukemia (CLL)
Acute Myelogenous Leukemia (AML)
characterized by uncontrolled proliferation of myeloblasts, the one that comes before granulocytes. There is hyperplasia (enlargement of organ) of bone marrow.
Acute Lymphocytic Leukemia (ALL)
most common in children, immature lymphocytes proliferate (multiply) in the bone marrow. s/s bleeding or fever, weakness, fatigue, bone or joint pain.
Chronic Myelogenous Leukemia (CML)
excessive development of mature neoplastic granulocytes in the bone marrow. Massive numbers travel from the blood to the liver & spleen. Once the acute phase turns into the chronic phase (blastic phase) the pt will only survive for a few months.
Chronic Lymphocytic Leukemia (CLL)
production of iactive but long lived, small mature lymphocytes. Pressure on nerves from enlargement leads to pain & paralysis. Most common in adults.
Hairy Cell Leukemia
Inflitrates the bone marrow & spleen. Has spleomegaly but not hepatomegaly
- Induction therapy-aggressive & seeks to destroy leukemic cells.
- Intensification therapy- is started after remission(higher dosage drugs)
- Consolidation therapy-after remission is achieved. The purpose is to eliminate remaining leukemic cells left behind.
- Maintenance therapy-tx with lower dosesof the same drugs given 3-4 wks for a prolonged period of time.
COAP- acronym of the letters of the drugs used in combination chemotherapy
a malignant condition characterized by rapid increase of abnormal multinucleated cells called Reed-Sternberg cells, which are located in lymph nodes. Occurs in persons 15-35yrs & above 50yrs. Twice as much in men then in women, smokers, genetic, exposure to occupational toxins, HIV.
MOPP (chemotherapy drugs)
ABVD (chemotherapy meds)
Non-Hudgkin's Lymphoma (NHLs)
a group of malignant neoplasms of primarily B- or T-cells origin affecting all ages. Most common in individuals that undewent immunosuppresant medications. Manifestation is painless lymph node enlargement.
Multiple Myeloma (plasma cell myeloma)
neoplastic plasma cells infiltrate the bone marrow and destroy the bone. Survival rate after being diagnosed is 2yrs. Genetic factors & viral infection may influence the risk of developing it.
Collaborative care for Multiple Myeloma
- Ambulation & hydration to help with hypercalcemia, renal damage.
- Weight bearing exercises to help reabsorb some calcium
- Pain management
- Monitor electrolyte balance
- Monitor I & O's
Disorders of the Spleen
- Splenomegaly may be painful, if Splenectomy is performed, monitor for hemorrhage, which could lead to shock, fever & abdominal distention.
- Pts are at high risk of infection.
Administration of Blood
- 23-gauge needle can be used but 16-19 gauge is preferred if rapid transfussions are given
- does not run over 4hrs. & must be used within 30mins. if not returned to lab
- Dextrose or lactated Ringer's should not be used due to hemolysis
- rate of infusion should be 2ml/min
Blood transfusion reactions
- Febrile Reactions- leukocyte incompatibility
- Allergic Reactions- epinephrine or corticosteroids are used to treat reaction
- Circulatory Overload- if a large quantity is infused over a short period of time
- Sepsis- septic shock from bacteria
- Acute lung injury- pulmonary edema
- Delayed Transfusion- infection & iron overload