NCM 112 - HEMATOLOGIC MOD.pdf

UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and E...

UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture [TRANS] UNIT XX: HEMATOLOGICAL DISORDERS  Skin may appear hyperpigmented from melanin deposits Module 2A (and occasionally hemosiderin, an iron-containing pigment) Hematological Disorders or appear bronze in color.  4 Cardiac dysrhythmias and cardiomyopathy Outline o Diseased cardiac muscle which will result to dyspnea in cardiateration can cause a 1. Differentiate the various hypoproliferative hematological m and edema. Total Iron disorders based on their causes. FBS Bypass Normal -10-100 mgld) Endocrine dysfunction is manifested as hypothyroidism, Surgery 2. Compare and contrast the physiologic mechanisms and pancreas that does not diabetes, hypogonadism (testicular atrophy, diminished clinical manifestations. libido, and impotence) does not eredt matras la bayag produce enough insulin 3. Identify the appropriate medical management and transport glucser insider > -  the cell nursing interventions for patients with bleeding and Cirrhosis is common in later stages of the disease, thrombotic disorders. shortens life expectancy, and a risk factor for hepatocellular carcinoma. terminal disease > - 1. Hereditary Hemochromatosis Hospital + 10 : 08 pm = 6 : 00 am ↳ 8 hours fasting  A genetic condition (autosomal recessive) most common HBAIc the genetic disorder among whites, in which excess iron is measures average - Plasmalian glucose level for 3 months a absorbed from the GI tract. to liver myocardium testes thyroid andpancreas > - , , , o Iron is an important component of hemoglobin, the mutation in both substance in red blood cells that carries oxygen from alleles of the HFE gene your lungs to transport it throughout your body. causes increased o However, too much iron can affect other organs which intestinal absorption of leads to organ failure = hemochromatosis dietary intake o Normally, the GI tract absorbs 1-2 mg of iron daily/per day coming to the food that we intake. To persons with hereditary hemochromatosis, the rate of iron absorption becomes significantly increased as compared to iron deficiency anemia. CTTO: https://harvardmedicine.files.wordpress.com o If iron increasingly absorbed in the body, excess iron is stored in your organs, especially your liver, spleen, Assessment and Diagnostic Findings heart, joints, skin, and pancreas.  Elevated serum iron level and high transferrin saturation. o Excessive deposition of iron in the organs causes o Transferrin is the transport protein of iron. damage to the organs (becoming dysfunctional and  CBC values typically normal. affects the appearance) and can lead to organ failure.  Liver biopsy, is a former definitive test but testing for the o Too much iron can lead to life-threatening associated genetic mutation (i.e., C282Y homozygosity) is conditions, such as liver disease, heart problems and now more commonly used. diabetes. o Liver biopsy check liver for deposits of abnormal o Genetic testing is done for detection. deposits of iron. Hemochromatosis Medical Management  Removal of excess iron via > venipuncture (aspirate therapeutic phlebotomy.- blood to lessen in blood, iron  Results in a decrease of 200 to 250 mg of iron.  Phlebotomy is required, at 1 to 2 units weekly initially of iron deposits  After 1 to 3 years, the frequency of phlebotomy can often be further reduced to prevent re- accumulation of iron deposits.  Phlebotomy is necessary only every 1 to 4 months until the CTTO: https://healthjade.net serum ferritin levels are maintained at 50 mcg/L. Clinical Manifestations Nursing Management  Accumulation of iron in body organs occurs gradually.  Limit their dietary intake of iron and iron supplements. Not iron to take food rich in green leafy vegg - o The onset is insidious.  Limit vitamin C intake. malunggay liver (meat) ,  Weakness Liver is for detoxification. o Vitamin C increases the absorption of iron in the  Lethargy body. Medications may also affect liver function -. o Iron rich foods like red meat, green leafy vegetables  Arthralgia (joint pain) (malunggay, pechay, and kangkong).  Weight loss  Instruct to avoid insults to the liver. overdosage of medications (hepatotoxic >  - Loss of libido Closs of sexual drive) o Lifestyle like alcohol consumption.  Advice serial screening tests for hepatoma (e.g., monitoring alpha-fetoprotein). to monitor liver" effects Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 1  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture  Monitor for signs of organ dysfunction, particularly the Organs that will be critical for the blood supply are the endocrine and cardiac systems. kidneys.  Children of patients who are homozygous for the HFE gene mutation should be screened for the mutation (since Medical Management this is genetically acquired).  When secondary polycythemia is mild, treatment may not  Patients who are heterozygous for the HFE gene do not be necessary; when treatment is necessary, it involves develop the disease but need to be advised that they can treating the primary condition. transmit the gene to their children.  Therapeutic phlebotomy o To reduce blood volume and viscosity – done on 2. Polycythemia regular basis.  Increased volume of RBCs.  Hyperproliferative disorders of the blood. 3. Bleeding Disorders  Hematocrit is elevated more than 55% in males, more than  Failure of normal hemostatic mechanisms can result in 50% in females. bleeding.  The increased RBCs will have a very viscous blood  Commonly provoked by trauma (because of accident e.g., because the cellular component (RBCs) is many in aneurysm) but can occur spontaneously. proportion to the liquid component of the blood.  When the source of platelet or coagulation factor  Can be temporary or chronic. abnormalities (deficient), the site of bleeding can be anywhere in the body but when the source is vascular Classification abnormalities, the site of bleeding may be more localized.  When there is bleeding, the bone marrow may be A. Primary Polycythemia stimulated to increase platelet production (help seal off  Also called polycythemia vera. injury in the blood vessel).  There is hyperactive bone marrow.  Sometimes, increase in platelets does not result from  Abnormalities in RBC production cause an increase in red increased production but from a loss in platelet pooling cell count. within the spleen.  The RBCs produced are not that normal. Their lifespan Mechanism of Coagulation becomes shorter. The normal lifespan of RBC is 120 days. o The oxygen carrying capacity of RBCs is impaired (oxygenation is not improved)  The shorter the lifespan of RBCs and increased cell production will cause rapid turnover of RBC increases the debris (uric acid (gout) in the joints and other substances that can be attached to RBC). B. Secondary Polycythemia  Caused by excessive production of erythropoietin, hormone necessary for blood cell production in the kidneys. Causes of Secondary Polycythemia  Reduced amount of oxygen  Cigarette smoking, chronic obstructive pulmonary disease (COPD) o Pink puffers  Cyanotic heart disease  Non-pathologic conditions such as living at a high altitude (low oxygen)  Hemoglobinopathies in which the hemoglobin has an abnormally high affinity for oxygen.  Genetic mutations cause abnormally high erythropoietin CTTO: https://www.researchgate.net levels. Clotting Factors  Neoplasms that stimulate erythropoietin production (e.g., Clotting Factors Name renal cell carcinoma) I Fibrinogen  Patients with increased in red blood cell will appear to have II Prothrombin dark red (ruddy) or purplish appearance and have Tissue factor or tissue distended veins. III thromboplastin  May suffer from intense itching because of the dilation of IV Calcium the blood vessel. But the perfusion may be poor because V Proaccelerin the blood flow is slower because of the viscosity of the VI Accelerin or factor 5a blood. Slow movement of blood causes vascular stasis or VII Stable factor or Proconvertin clotting of blood which can result to the formation of VIII Antihemophilic factor A thrombus – cause poor perfusion and even infarction. Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 2 Extrinsic pathway known as the tissue factor pathway The tissue factors are released from the damaged vascular wall The tissue factor activates the factor VIi to VIiA Intrinsic pathway begins with the exposure of blood to the collagen from the underlying damaged endothelium. (sa blood vessels na) This activates the plasma factor xii to xii a is a serine protease, it activates the factor xi to xia Any deviation from the clotting factor will cause deviation to clotting. 2) conversion of prothrombin to thrombin Vitamin K is required for the synthesis of prothrombin 3) conversion of fibrinogen into fibrin  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture Christmas factor or IX antihemophilic factor B X Stuart prower factor Plasma thromboplastin XI antecedent XII Hageman factor XIII Fibrin stabilizing factor  All these factors work together to be able to form the final product fibrin clot.  Any problem in these clotting factors can cause bleeding to the patient. Clinical Manifestations CTTO: https://www.ncbi.nlm.nih.gov  When there is bleeding even in small capillaries, observation for petechiae, small pinpoint red dots that can be seen on the skin surface or mucus membrane and can occur all throughout the body.  Dengue patients, Herman’s sign is an indication that there is already bleeding.  Rambel lids test / torniquet test – old way  Petechiae, often in clusters; on the skin and mucous membranes but also occur throughout the body.  Coagulation factor defects do not tend to cause superficial bleeding, because the primary hemostatic mechanisms are still intact. Instead, bleeding occurs deeper within the body. o Hemorrhages in joints IM bleeding, and hematomas. Medical Management  Based on the underlying cause of the bleeding disorder.  If bleeding is significant, transfusions of blood products CTTO: https://www.toppr.com are indicated. o Fresh whole blood - o Fresh frozen plasma – loss of fluid volume in the blood o Packed RBCs – loss of RBCs o Platelet concentrate – dengue patients  If fibrinolysis is excessive, hemostatic agents such as aminocaproic acid.  Blood typing is necessary to know the compatibility (ABO typing and RH typing). CTTO: https://en.wikipedia.org Nursing Management  Educate to observe themselves carefully and frequently for signs of bleeding. o Black tarry stool indicates upper GI tract bleeding. o Fresh blood stool indicates lower (anus) bleeding.  Emphasize the importance of avoiding activities that increase the risk of bleeding. o NSAIDs cause gastric irritation that can cause bleeding.  Examine the skin for petechiae and ecchymoses and the nose and gums for bleeding.  Hospitalized patients are monitored for bleeding by CTTO: https://www.medicalnewstoday.com testing all drainage and excreta for occult blood as well as obvious blood. Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 3  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture o FOBT – unobservable blood in stool sample. Pathophysiology  Outpatients are often given fecal occult blood screening Autoimmune disorder characterized by a destruction of normal cards to detect occult blood in stools. platelets by unknown stimulus. ↓ A. Thrombocytopenia Antiplatelet antibodies develop in the blood and bind to the patient’s platelets. Causes ↓  Decreased production of platelets within the bone Antibody-bound platelets are then ingested and destroyed by marrow. the reticuloendothelial system (RES) or tissue macrophages.  Increased destruction of platelets. ↓  Increased consumption of platelets (e.g., the use of Body attempts to compensate for this destruction by increasing platelets in clot formation). platelet production within the marrow. ↓ Clinical Manifestations Compensatory mechanism may not be effective as  When the platelet count drops to less than 20,000/mm3 or thrombopoietin levels are not elevated in patients with ITP, and less than 5,000/mm3. as such, platelet production may be diminished.  Petechiae  Nasal and gingival bleeding Clinical Manifestations  Excessive menstrual bleeding  Many patients have no symptoms, and the low platelet  Excessive bleeding after surgery or dental extractions count is an incidental finding (often less than 30,000/mm3;  Spontaneous, potentially fatal central nervous system or GI less than 5,000/mm3 is not uncommon). hemorrhage  Easy bruising  Heavy menses Assessment and Diagnostic Findings  Petechiae on the extremities or trunk “dry purpura” tend to  Examination of the bone marrow via aspiration and have fewer complications from bleeding than those with biopsy. bleeding from mucosal surfaces, such as the GI tract  Screening for certain diseases. (including the mouth) and pulmonary system (e.g.,  A manual examination of the peripheral smear can easily hemoptysis), which is termed “wet purpura”. determine platelet clumping or use of newer cell counter machines. Assessment and Diagnostic Findings  History and physical assessment Medical Management  Tested for hepatitis C and HIV, if not previously done to  Treatment of the underlying disease. rule out these potential causes.  If platelet production is impaired, platelet transfusions  If bone marrow aspirate is performed, an increase in may increase the platelet count and stop bleeding or megakaryocytes may be seen. prevent spontaneous hemorrhage.  Severity of the thrombocytopenia is highly variable, but a  Splenectomy can be a useful therapeutic intervention for platelet count that is less than 20,000/mm3 is common. excessive platelet destruction occurs but often it is not an option. Medical Management  Primary goal of treatment is a “safe” platelet count. Nursing Management  If the patient is taking a medication known to be associated  Consider the cause of the thrombocytopenia, the likely with ITP (e.g., quinine, sulfa-containing medications), then duration of it, and the overall condition of the patient. that medication must be stopped immediately.  Education is important, as are interventions to promote  Immunosuppressive agents block the binding receptors patient safety, particularly fall prevention in the older adult on macrophages so that the platelets are not destroyed. or patient who is frail.  Splenectomy is an alternative treatment.  Certain monoclonal antibodies (e.g., rituximab) B. Immune Thrombocytopenic Purpura (ITP)  Vincristine is used by blocking the receptors on the  Another name is immune thrombocytopenia. macrophages and therefore inhibiting platelet destruction; it  Affects people of all ages, but it is more common among may also stimulate thrombopoiesis. children and young women.  Use of anti-D (WinRho) in patients who are Rh (D) positive. The actual mechanism of action is unknown. a. Primary ITP  Despite extremely low platelet counts, platelet  Occurs in isolation. transfusions are usually avoided.  Defined as a platelet count less than 100cumm with an inexplicable absence of a cause for thrombocytopenia. Nursing Management  Assess patient’s lifestyle to determine the risk of bleeding b. Secondary ITP from activity.  Often results from autoimmune diseases (e.g.,  Obtain medication history including use of over-the- antiphospholipid antibody syndrome, viral infections, counter medications, herbs, and nutritional supplements. human immunodeficiency virus [HIV] infections, and various drugs). Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 4  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture  Assess for any history of recent viral illness and reports  Two inherited bleeding disorders—Hemophilia A and of headache or visual disturbances, which could be initial Hemophilia B—are clinically indistinguishable, although symptoms of intracranial bleeding. they can be distinguished by laboratory tests.  Patients who are admitted to the hospital with neurologic  Both types of hemophilia are inherited as X-linked traits, assessment incorporated into their routine vital sign so almost all affected people are males; females can be measurements. carriers but are almost always asymptomatic.  Avoid injections, rectal medications and rectal temperature measurements. Hemophilia  Explore the extent the patient experiences fatigue and offer strategies to ameliorate this problem.  Patient education addresses signs of exacerbation of disease (e.g., petechiae, ecchymoses).  Instruct to avoid all agents that interfere with platelet function, including herbal therapies and over-the-counter medications.  Instruct to avoid constipation, the Valsalva maneuver, and vigorous flossing of the teeth. Electric razors should be used for shaving, and soft bristled toothbrushes should replace stiff-bristled ones.  The patient may also be counseled to refrain from vigorous sexual intercourse when the platelet count is less than 10,000/mm3.  Educate patients who are receiving corticosteroids of long term are at risk for complications. 4. Platelet Defects CTTO: https://www.vectorstock.com  Qualitative defects, the number of platelets may be normal but the platelets do not function normally. A. Hemophilia A  Caused by a genetic defect that results in deficient or Assessment and Diagnostic Findings defective factor VIII.  Bleeding time was most commonly used to evaluate platelet function now, a platelet function analyzer is often B. Hemophilia B used.  Also called “Christmas disease.”  Examining the platelet morphology (peripheral smear)  Stems from a genetic defect that causes deficient or evaluation. defective factor IX. Clinical Manifestations  Bleeding may be mild or severe.  Its extent is not necessarily correlated with the platelet count or with prothrombin time activated partial thromboplastin time. Medical Management  If the platelet dysfunction is caused by medication, its use should be stopped, if possible, particularly when bleeding occurs.  If platelet dysfunction is marked, bleeding can often be prevented by transfusion of normal platelets before invasive procedures.  Anti-fibrinolytic agents CTTO: https://istudy.pk Nursing Management Clinical Manifestations  Patients with significant platelet dysfunction need to be  Hemorrhages into various parts of the body. instructed to avoid substances that can diminish platelet  About 75% of all bleeding in patients with hemophilia occurs function, such as certain over-the-counter medications, into joints. some herbal therapies, nutritional supplements, and  Most commonly affected joints are the knees, elbows, alcohol. ankles, shoulders, wrists, and hips.  Instruct to inform their health care providers (including  Pain in a joint before they are aware of swelling and dentists) of the underlying condition before any invasive limitation of motion. Recurrent joint hemorrhages can result procedure is performed. in damage so severe that chronic pain, ankylosis fixation, 5. Hemophilia or arthropathy of the joint occurs. Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 5  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture  Severe factor deficiency can become crippled by the joint  Written emergency plan that includes what to do in damage before they become adults. specific situations as well as names and phone numbers of  Bleeding can be superficial as hematomas or as deep emergency contacts. hemorrhages into muscle or subcutaneous tissue.  Extent of bleeding must be assessed carefully during  With severe factor VIII deficiency, hematomas can occur hemorrhagic episodes. without known trauma and progressively extend in all  Frequent monitoring of vital signs. directions. When the hematomas occur within muscle,  During bleeding episodes, heat is avoided. particularly in the extremities, peripheral nerves can be  Genetic testing and counseling. compressed.  Over time, this compression results in decreased Take Time to Watch sensation, weakness, and atrophy of the area involved. https://www.youtube.com/watch?v=3bjlQH13lUE  Spontaneous hematuria and GI bleeding can occur. Bleeding is also common in mucous membranes, such as F. Von Willebrand Disease (VWD) the nasal passages, and in soft tissues. The most  Inherited as a dominant trait. dangerous site of hemorrhage is in the head (intracranial or  Common bleeding disorder that affects males and females extracranial). equally.  Surgical procedures typically result in excessive bleeding  Caused by a deficiency of VWF, which is necessary for at the surgical site. Because clot formation is poor, wound factor VIII activity. healing is also poor. Types of VWD Hemophilia Joint and Muscle Bleeds A. Type 1  Most common, is characterized by decreases in structurally normal VW. B. Type 2  Shows variable qualitative defects based on the specific VWF subtype involved. C. Type 3  Very rare (less than 5% of cases) and is characterized by a severe VWF deficiency as well as significant deficiency of factor VIII. Abnormal Differences in Clotting found in Hemophilia and VWD CTTO: https://www.nationwidechildrens.org Medical Management  Recombinant forms of factor VIII and X concentrates are available and decrease the need for using factor concentrates, or, more infrequently, fresh-frozen plasma.  Plasmapheresis or concurrent immunosuppressive agents  Activated prothrombin complex concentrates  Aminocaproic acid  Desmopressin (DDAVP) Nursing Management  Instruct how to administer the factor concentrate at home at the first sign of bleeding.  Instruct to avoid agents that interfere with platelet aggregation.  Dental hygiene is very important as a preventive measure.  Applying pressure to a minor wound may be sufficient to control bleeding if the factor deficiency is not severe.  Nasal packing should be avoided.  Splints and other orthopedic devices may be useful in patients with joint or muscle hemorrhages.  All injections should be avoided. Clinical Manifestations  Invasive procedures should be minimized or performed  Bleeding tends to be mucosal after administration of appropriate factor replacement.  Recurrent nosebleeds  Carry or wear medical identification.  Easy bruising Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 6  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture  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. Assessment and Diagnostic Findings  Normal platelet count but a prolonged bleeding time and a slightly prolonged aPTT.  More important tests include the ristocetin cofactor or VWF collagen binding assay, which measures VWF activity.  Other tests include VWF antigen, factor VIII, and, for patients with suspected type 2 defects, VWF multimers, which measure specific subtypes of VWF. Medical Management  Desmopressin (DDAVP), a synthetic vasopressin analogue, can be used to prevent bleeding associated with dental or surgical procedures or to manage mild bleeding after surgery in those individuals with mild VWD, although it is often ineffective in treating those with type 3 VWD. o Administered as an IV infusion or intranasally. o With major surgery or invasive procedures DDAVP is contraindicated in patients with unstable coronary artery disease, because it can induce platelet aggregation and cause acute coronary syndrome (ACS) o Side effects:  Headache  Facial flushing  Tachycardia  Hyponatremia  Rarely, seizures  Replacement products include Humate-P and Alphanate, which are commercial concentrates of VWF and factor VIII.  Aminocaproic acid  Estrogen–progesterone compounds  Platelet transfusions Take Time to Watch https://www.youtube.com/watch?v=x8TLTTyyPfI Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 7  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture [TRANS] UNIT XX: HEMATOLOGICAL DISORDERS Module 2B Hematological Disorders Outline 1. Differentiate the various hypoproliferative hematological disorders based on their causes. 2. Compare and contrast the physiologic mechanisms and clinical manifestations. 3. Identify the appropriate medical management and nursing interventions for the different hematological disorders. 1. Acquired Coagulation Disorders A. Liver Disease With the exception of factor VIII, most blood coagulation factors are synthesized in the liver. Hepatic dysfunction can result in diminished amounts of the factors needed to maintain coagulation and hemostasis. a. Vitamin K Deficiency Synthesis of many coagulation factors depends on vitamin CTTO: https://quizlet.com/ K. Normal hemostatic mechanisms are altered. The Administration of vitamin K (phytonadione), either orally or inflammatory response generated by the underlying as a subcutaneous injection, can correct the deficiency disease initiates the process of inflammation and quickly. coagulation within the vasculature. Adequate synthesis of coagulation factors is reflected by The natural anticoagulant pathways within the body are normalization of the PT. simultaneously impaired, and the fibrinolytic system is Anticoagulant Therapy suppressed. Anticoagulants Clinical Manifestations Standardized system for reporting PT results by using an Reflected in compromised organ function or failure. international sensitivity index for the reagent used in known Decline in organ function. as the INR (international normalized ratio). The excessive clotting triggers the fibrinolytic system to Vitamin K is administered as an antidote for warfarin release fibrin degradation products, which are potent toxicity. anticoagulants, furthering the bleeding. With significant bleeding, fresh-frozen plasma is needed to Frank DIC may bleed from mucous membranes, replace the vitamin K dependent coagulation factors. venipuncture sites, and the GI and urinary tracts. b. Disseminated Intravascular Coagulation (DIC) Patients typically develop multiple organ dysfunction Not a disease but a sign of an underlying condition. syndrome (MODS). Decrease in the platelet count. Pathophysiology Assessment and Diagnostic Findings Drop in platelet count Elevation in fibrin degradation products and D-dimer Increase in PT and aPTT Low fibrinogen level The International Society on Thrombosis and Haemostasis has developed a highly sensitive and specific scoring system. Medical Management Correcting the secondary effects of tissue ischemia by improving oxygenation, replacing fluids, correcting electrolyte imbalances, and administering vasopressor medications. If serious hemorrhage occurs, the depleted coagulation factors and platelets may be replaced. Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 1  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture Cryoprecipitate is given to replace fibrinogen and factors V warfarin induced skin necrosis. This complication appears and VII; fresh-frozen plasma is administered to replace to result from progressive thrombosis in the capillaries other coagulation factors. within the skin. A controversial treatment strategy is to interrupt the Prompt cessation of warfarin, treatment with vitamin K, and thrombosis process through the use of heparin infusion. infusions of heparin and fresh-frozen plasma are crucial to o Heparin may inhibit the formation of microthrombi and arrest the pathophysiologic process and reverse the effects thus permit perfusion of the organs (skin, kidneys, or of the warfarin. brain) to resume. Treatment with purified protein C concentrate is sometimes indicated. Nursing Management Assess thoroughly and frequently for signs and symptoms D. Protein S Deficiency of thrombi and bleeding and monitor for any progression of Protein S is another natural anticoagulant produced by the these signs. liver. Lab values must be monitored frequently, not only for the APC requires protein S to inactivate certain clotting factors. actual result but to note trends over time as well as the rate When the level of protein S is deficient, this inactivation of change in values. process is diminished, and the risk of thrombosis can be Assessment and interventions should target potential sites increased. of end-organ damage. Like patients with protein C deficiency, those with protein S Suctioning should be performed as gently as possible. deficiency have a greater risk of recurrent venous thrombosis early in life, as early as 15 years of age. More Take Time to Watch than 50% of these thromboses are spontaneous. https://www.youtube.com/watch?v=BdqPhaxpR90 Thromboses most commonly occur in the axillary, mesenteric, and cerebral veins. Warfarin-induced skin 2. Thrombotic Disorders necrosis is possible. Several conditions can alter balance within the normal Acquired protein S deficiency can also occur in pregnancy, hemostasis process, causing excessive thrombosis that DIC, liver disease, nephritic syndrome, HIV infection, and may be arterial (due to platelet aggregation) or venous the use of L-asparaginase (comprised of platelets, red cells, and thrombin). Anticoagulation therapy is necessary. The duration of E. Activated Protein C (APC) Resistance and Factor therapy varies with the location and extent of the V Leiden Mutation thrombosis, precipitating events. APC resistance is a common condition that can occur with other hypercoagulable states. A. Hyperchomocysteinemia APC is an anticoagulant, and resistance to APC increases Homocysteine can promote platelet aggregation. the risk of venous thrombosis. Increased plasma levels of homocysteine are a significant A molecular defect in the factor V gene has been identified risk factor for venous thrombosis (e.g., deep vein in most (90%) patients with APC resistance. thrombosis [DVT], pulmonary embolism [PE]) and arterial Factor V Leiden mutation is the most common cause of thrombosis (e.g., ischemic stroke, ACS). inherited hypercoagulability in Caucasians, but its incidence It can be hereditary, or it can result from a nutritional appears to be much lower in other ethnic deficiency of folate and, to a lesser extent, of vitamins B12 Factor V Leiden mutation synergistically increases the risk and B6, because these vitamins are cofactors in of thrombosis in patients with other risk factors (e.g., the homocysteine metabolism. use of oral contraceptives, hyperhomocysteinemia, B. Antithrombin (AT) Deficiency increased age). AT is a protein that inhibits thrombin and certain coagulation F. Acquired Thrombophilia factors, and it may also play a role in diminishing Acquired thrombophilias are types of clotting disorders that inflammation within the endothelium of blood vessels. do not have inherited/genetic causes. A hereditary condition that can cause venous thrombosis, particularly when the AT level is less than 60% of normal. Etiology AT deficiency is acquired by four mechanisms: Typically caused by either an excess in antibodies that Accelerated consumption of AT cause clotting or because of an increase in clotting factors. Reduced synthesis of AT Antiphospholipid syndrome Increased excretion of AT Malignancy/cancer, particularly stomach, pancreatic, lung, Medication induced and ovarian cancers. C. Protein C Deficiency Medical Management Pharmacologic Therapy Protein C is a vitamin K–dependent enzyme synthesized in Unfractionated heparin the liver; when activated, it inhibits coagulation. Warfarin (Coumadin) When levels are deficient, the risk of thrombosis increases, and thrombosis can often occur spontaneously. Dabigatran (Pradaxa) → new oral direct thrombin inhibitor A rare but significant complication of anticoagulation Nursing Management management in patients with protein C deficiency is Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 2  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture Instruct to avoid activities that lead to circulatory stasis Encourage exercise Educate about medications that alter platelet aggregation, such as low-dose aspirin or clopidogrel (Plavix). Assess for concurrent risk factors for thrombosis and should be avoided such the use of tobacco and nicotine products. Take Time to Watch https://www.youtube.com/watch?v=OduOQN1s12g&list=PLldV TLhTUN98kCWH-ShhOysjSZ4dnDY4c Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 3  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture [TRANS] UNIT XX: ANEMIA Patients with coexistent cardiac, vascular, or pulmonary Module 2C disease may develop more pronounced symptoms of Anemia anemia. Some anemias are complicated by various other Outline abnormalities that do not result from the anemia but are 1. Differentiate the different types of anemia. inherently associated with these particular diseases. 2. Use the nursing process as a framework for care of patients with anemia. Healthy Red Blood Cell Shape and Function 3. Use the nursing process as a framework for care of patients with sickle cell crises. Introduction Anemia may be classified in several ways. A physiologic approach classifies anemia according to whether the deficiency in erythrocytes is caused by: o Defect in their production o Destruction o Blood loss o In hypoproliferative anemias, the marrow cannot produce adequate number of erythrocytes. o Decreased erythrocyte production is rejected by a low or inappropriately normal reticulocyte count. Inadequate production of erythrocytes results from marrow damage due to: o Medications (e.g., chloramphenicol) CTTO: https://sicklecellanemiablog.wordpress.com o Chemicals (e.g., benzene) o Lack of factors (e.g., iron, vitamin B12, folic acid, Assessment and Diagnostic Findings erythropoietin) Evaluation, the hemoglobin, hematocrit, reticulocyte count (immature form of erythrocyte), and red blood cell (RBC) indices, particularly the mean corpuscular volume (MCV) and red cell distribution width (RDW). o If the reticulocyte count is high, the bone marrow is producing immature RBCs that is not being matured. Their function is compromised. o Laboratory tests are important objective data Iron studies Serum vitamin B12 and folate levels Haptoglobin and erythropoietin level Bone marrow aspiration Other diagnostic studies may be performed to determine the presence of underlying chronic illness. Medical Management Erythrocytes that are lost or destroyed may be replaced with a transfusion of packed red blood cells. Take Time to Watch https://www.youtube.com/watch?v=IfmJeWW4OyQ 1. Hypoproliferative Anemias CTTO: https://www.phlbi.org A. Iron Deficiency Anemia Clinical Manifestations Results when the intake of dietary iron is inadequate for Hemoglobin levels between 9 and 11 g/dl usually has few hemoglobin synthesis. or no symptoms other than slight tachycardia on exertion o Iron is important in the formation of RBCs. and possibly fatigue. People who customarily are very active or who have Risk Factors significant demands on their lives are more likely to have Inadequate intake of iron symptoms. Blood loss Patients with hypothyroidism with decreased oxygen needs Most common cause of iron deficiency anemia in men and may be completely asymptomatic. postmenopausal women is bleeding from ulcers, gastritis, inflammatory bowel disease, or GI tumors. Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 1  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture Most common causes of iron deficiency anemia in Preventive education is in menstruating and pregnant premenopausal women are menorrhagia and pregnancy. women. o Because of the demands of the pregnancy. Preventive education. Foods sources high in iron include o Women who are pregnant are advised to take iron organ meats and other meats, beans, leafy green supplement. vegetables, raisins, and molasses. Iron malabsorption (if client undergo gastrectomy or celiac Taking iron-rich foods with a source of vitamin C (e.g., disease) orange juice) enhances the absorption of iron. Encourage the patient to continue iron therapy for as long Iron Cycle and Storage as it is prescribed even though the patient may no longer feel fatigued or other symptoms that may indicate IDA. Instruct to take the supplement an hour before meals. IV supplementation of iron may be used when the patient’s iron stores are completely depleted, the patient cannot tolerate oral forms of iron supplementation. o Iron stores can be found in the liver within a certain amount. Take Time to Watch https://www.youtube.com/watch?v=ZjvslDSPT7o B. Anemias in Renal Disease People with kidney failure will have a deficiency in erythropoietin, a hormone produced by the kidney that stimulates the bone marrow to create RBCs. Do not become significantly anemic until the serum creatinine level exceeds 3 mg/100 mL. o Creatinine and BUN are tests for renal function. This type of anemia is caused by both a mild shortening of erythrocyte lifespan (120 days) and a deficiency of erythropoietin. Patients undergoing long-term hemodialysis lose blood into CTTO: https://slideplayer.com the dialyzer and therefore may become iron deficient. o Included in the maintenance is injection of epoetin that Clinical Manifestations will supplement the deficiency of erythropoietin Smooth, sore tongue production of kidney. Brittle and ridged nails o Injected subcutaneously. Angular cheilosis If untreated, the hematocrit usually falls to between 20% and 30%, although in rare cases it may fall to less than 15%. Assessment and Diagnostic Findings Bone marrow aspiration RBC and hemoglobin levels Serum iron level and an elevated TIBC, which measures the transport protein supplying the marrow with iron as needed referred to as “transferrin.” o Transferrin is the protein transporter of iron. Medical Management Oral iron preparation – ferrous sulfate, ferrous gluconate, and ferrous fumarate. o We avoid teeth discoloration. o Advise the patient to use straw. o Iron is best absorbed with vitamin C. o Foods that can interfere with absorption: dairy products like milk. IV administration of iron may be needed. Method for injecting iron – z-track to prevent drug leakage CTTO: https://www.niddk.nih.gov into the subcutaneous tissue, helps seal the drug in the muscle, minimizes skin irritation, and prevent skin discoloration. Nursing Management Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 2  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture CTTO: www.vivo.colostate.edu Medical Management Recombinant erythropoietin C. Anemia of Chronic Disease Chronic inflammatory diseases are associated with a normochromic, normocytic anemia. These disorders include: o Rheumatoid arthritis o Severe, chronic infections o Cancers Hemoglobin level rarely falls below 9 g/dl and the bone marrow has normal cellularity with increased stores of iron as the iron is diverted from the serum. Erythropoietin levels are low, perhaps because of decreased production, and iron use is blocked by erythroid cells (cells that are or will become mature erythrocytes). A moderate shortening of erythrocyte survival also occurs. D. Aplastic Anemia 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. o We said that the bone marrow will first produce stem cells (undifferentiated cells) o Two bone marrow: CTTO: https://www.saintlukeskc.org  Red (responsible for hematopoiesis)  Yellow (fat) Assessment and Diagnostic Findings Stem cell damage is caused by the body’s T cells mediating A bone marrow aspiration shows an extremely hypoplastic in inappropriate attack against the bone barrow, resulting in or even aplastic (very few to no cells) marrow replaced with bone marrow aplasia. fats. o The person’s own immune system attacks the bone marrow (autoimmune). Significant neutropenia and thrombocytopenia also occur. Risk Factors Congenital or acquired, but most cases are idiopathic Infections Pregnancy Medications Chemicals or radiation damage Clinical Manifestations Typical complications are infection and the symptoms of anemia (e.g., fatigue, pallor, dyspnea). Purpura (bruising) may develop later. If the patient has had repeated throat infections, cervical lymphadenopathy and other lymphadenopathies. Splenomegaly CTTO: https://study.com Retinal hemorrhages Medical Management Normal and Aplastic Anemia Younger than 60 years, who are otherwise healthy, and who have a compatible donor can be cured of the disease with hematopoietic stem cell transplant (HSCT) Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 3  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture Immunosuppressive therapy, commonly using a CTTO: https://i.ytimg.com combination of antithymocyte globulin (ATG) Supportive therapy plays a major role. Any offending agent Clinical Manifestations is discontinued. The patient is supported with transfusions Symptoms of folic acid and vitamin B12 deficiencies are of PRBCs and platelets as necessary. similar, and the two anemias may coexist. Death usually is caused by hemorrhage or infection. Neurologic manifestations of vitamin B12 deficiency do not occur with folic acid deficiency, and they persist if vitamin Nursing Management B12 is not replaced. Assess carefully for signs of infection and bleeding. Pernicious anemia Monitor for side effects of therapy, particularly for Smooth, sore, red tongue hypersensitivity reaction while administering ATG. Mild diarrhea Patients require long-term cyclosporine therapy, monitored Extremely pale, particularly in the mucous membranes. for long-term effects, including renal or liver dysfunction, Become confused; more often, they have paresthesias in hypertension, pruritus, visual impairment, tremor, and skin the extremities. cancer. Difficulty maintaining their balance because of damage to Educate about the importance of not abruptly stopping their the spinal cord, and they also lose position sense. immunosuppressive therapy. Without treatment, patients can die after several years. Take Time to Watch Assessment and Diagnostic Findings https://www.youtube.com/watch?v=RBOTrr0BKL0 Schilling test → classic method of determining the cause of vitamin B12. E. Megaloblastic Anemias Caused by deficiencies of vitamin B12 or folic acid, identical Medical Management bone marrow and peripheral blood changes occur because both vitamins are essential for normal DNA synthesis. Folate Deficiency Erythrocytes that are produced are abnormally large and Increasing the amount of folic acid in the diet. called megaloblastic red cells. Other cells derived from the Administering 1 mg of folic acid daily. myeloid stem cell (non-lymphoid leukocytes, platelets) are Administered intramuscularly only to people with also abnormal. malabsorption problems. A bone marrow analysis reveals hyperplasia and the Multivitamin preparations supplements. precursor erythroid and myeloid cells are large and bizarre in appearance. Vitamin B12 deficiency Abnormal erythroid and myeloid cells are destroyed within Treated by vitamin B12 replacement. the marrow, so the mature cells that do leave the marrow. Replacement is by monthly intramuscular injections of vitamin B12. a. Folic Acid (Vitamin B9) Deficiency Anemia A small amount of an oral dose of vitamin B12 can be Stored as compounds referred to as folates. absorbed by passive diffusion, even in the absence of intrinsic factor in large doses. b. Vitamin B12 Deficiency Anemia To prevent recurrence of pernicious anemia, vitamin B12 Pernicious anemia. therapy must be continued for life. Causes Nursing Management Inadequate dietary intake is rare but can develop in strict Assessment includes inspection of the skin, mucous vegetarians who consume no meat or dairy products. membranes, and tongue and mild jaundice. Faulty absorption from the GI tract is more common. Vitiligo and premature graying of the hair. Occurs in conditions such as Crohn’s disease or after ileal Neurologic assessment is important, including tests of resection, bariatric surgery, or gastrectomy. position, vibration sense, and cognitive function, gait and Chronic use of proton pump inhibitors. stability, as well as the need for assistive devices and for Use of the drug metformin. assistance in managing daily activities. Absence of intrinsic factor. Advise to eat small amounts of bland, soft foods. Explain that other nutritional deficiencies. Teach about the chronicity of the disorder and the need for monthly vitamin B12 injections or daily oral vitamin B12 even in the absence of symptoms. If parenteral replacement patients can be taught to self- administer their injections. Take Time to Watch https://www.youtube.com/watch?v=nX7bLq0VSeQ 2. Hemolytic Anemias Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 4  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture Pathophysiology Difference Between Normal Red Blood Cell and Sickle Cell Erythrocytes have a shortened lifespan; thus, their number in the circulation is reduced. ↓ Decreased available oxygen, causing hypoxia, which in turn stimulates an increase in erythropoietin release from the kidney. ↓ Erythropoietin stimulates the bone marrow to compensate by producing new erythrocytes and releasing some of them into the circulation somewhat prematurely as reticulocytes. ↓ If the red cell destruction persists, the hemoglobin is broken down excessively; about 80% of the heme is converted to bilirubin, conjugated in the liver, and excreted in the bile. Hemolytic Anemia CTTO: https://www.differencebetween.com Types of Sickle Cell Crisis a. Acute Vaso-Occlusive Crisis Entrapment of erythrocytes in the microcirculation. b. Aplastic Crisis Cause d by infection with human parvovirus causing the Hgb to fall and marrow unable to compensate. CTTO: https://myhealthonly.net c. Sequestration Crisis A. Sickle Cell Anemia Other organs pool the sickled cells and most commonly affected is the spleen. Pathophysiology Risk Factors Inheritance of the sickle hemoglobin (HbS) gene, which causes The HbS gene is inherited in people of African descent and the hemoglobin molecule to be defective. to a lesser extent in people from the Middle East, the ↓ Mediterranean area, and aboriginal tribes in India. HbS acquires a crystal-like formation when exposed to low If two people with sickle cell trait have children, the children oxygen tension. The oxygen level in venous blood can be low may inherit two abnormal genes and will have sickle cell enough to cause this change; consequently, the erythrocyte anemia. containing HbS loses its round, pliable, biconcave disk shape and becomes dehydrated, rigid, and sickle shaped. Pictorial Representation of the Pattern of Inheritance of SCA ↓ These erythrocytes adhere to the endothelium of small vessels; when they adhere to each other causing blood flow to a region or an organ to be reduced. ↓ If ischemia or infarction results, the patient may have pain, swelling, and fever. Sickling process takes time; if the erythrocyte is again exposed to adequate amounts of oxygen before the membrane becomes too rigid it can revert to a normal shape. For this reason, the “sickling crises” are intermittent. Cold can aggravate the sickling process, because vasoconstriction slows the blood flow. Oxygen delivery can also be impaired by an increased blood viscosity, with or without occlusion due to adhesion of sickled cells. CTTO: https://www.archivesofmedicine.com Clinical Manifestations Jopar Jose C. Ramos | Bachelor of Science in Nursing 3-A 5  UA-CONP: C-NCM112 aidè à vivrè Care of Clients with Problems in Oxygenation, Fluid and Electrolytes, Infectious, Inflammatory, #HelpLive Immunologic Response, and Cellular Aberration (Acute and Chronic) Lecture Symptoms vary and are only somewhat based on the Patients with mild forms have microcytosis and mild amount of HbS. Symptoms and complications result from anemia. chronic hemolysis or thrombosis. Thalassemia major is characterized by severe anemia, Sickled cells are rapidly hemolyzed and thus have a very marked hemolysis, and ineffective erythropoiesis. short lifespan of 10 to 20 days. Usually treated with transfusion of PRBCs. Jaundice is characteristic and is usually obvious in the sclerae. Take Time to Watch Enlargement of the bones of the face and skull. https://www.youtube.com/watch?v=th_GSpXFEp4 Chronic anemia is associated with tachycardia, cardiac c. Glucose-6-Phosphate Dehydrogenase Deficiency murmurs, and often an enlarged heart. The G-6-PD gene produces an enzyme within the Dysrhythmias and heart failure may occur in adults. erythrocyte that is essential for membrane stability. Susceptible to infection. A few patients have inherited an enzyme that is so defective Complications include infection, stroke, renal failure, that they have a chronic hemolytic anemia. impotence, heart failure, and pulmonary hypertension. Most common type of defect results in hemolysis only when Assessment and Diagnostic Findings the erythrocytes are stressed by certain situations, such as Patient with sickle cell trait usually has a normal hemoglobin fever or the use of certain medications. level, a normal hematocrit, and a normal blood smear. In Risk Factors contrast, the patient with sickle cell anemia has a low African Americans and people of Greek or Italian origin are hematocrit and sickled cells on the smear. those primarily affected by this disorder. Confirmed by hemoglobin electrophoresis. Inherited as X-linked defects. Medical Management Drugs have hemolytic effects particularly the antibacterial Treatment for sickle cell anemia is the focus of continued agents: research. o Nitrofurantoin (Macrodantin) and Dapsone o Antimalarial agent (Primaquine) Take Time to Watch o Phenazopyridine (Pyridium) https://www.youtube.com/watch?v=hRnrIpUMyZQ o Rasburicase (Elitek) o Methylthioninium chloride B. Thalassemia Ingestion of fava beans, menthol, tonic water, and some A group of hereditary anemias characterized by Chinese herbs such as Coptis chinensis (chuen lien), hypochromia extreme microcytosis, hemolysis and variable