L6-Management of patients with Hematologic disorders PDF

Management of patients with Hematologic disorders Nisha Sivapalan MSN, Lecturer, MACHS 1 Objectives On completion of this chapter, the learner will be able to: 1. Describe hematopoiesis and the processes involved in maintaining hemostasis. 2. Di...

Management of patients with Hematologic disorders Nisha Sivapalan MSN, Lecturer, MACHS 1 Objectives On completion of this chapter, the learner will be able to: 1. Describe hematopoiesis and the processes involved in maintaining hemostasis. 2. Discuss the significance of the health history to the assessment of hematologic health. 3. Specify the proper techniques utilized to perform a comprehensive physical assessment of hematologic function. 4. Explain the diagnostic tests and related nursing implications used to evaluate hematologic function. 5. Identify therapies for blood disorders, including nursing implications for the administration of blood components 2 Anatomic and Physiologic Overview The hematologic system consists of the blood and the sites where blood is produced, including the bone marrow and the reticuloendothelial system (RES). Blood is a specialized organ that differs from other organs in that it exists in a fluid state. Blood is composed of plasma and various types of cells. Plasma is the fluid portion of blood; it contains various proteins, such as albumin, globulin, fibrinogen, and other factors necessary for clotting, as well as electrolytes, waste products, and nutrients. About 55% of blood volume is plasma 3  Blood The cellular component of blood consists of three primary cell types: Erythrocytes (red blood cells [RBCs], red cells), Leukocytes (white blood cells [WBCs]), and Thrombocytes (platelets). These cellular components of blood normally make up 40% to 45% of the blood volume. The primary site for hematopoiesis is the bone marrow 4 5 Hematopoiesis 6 Hemostasis Hemostasis is the process of preventing blood loss from intact vessels and of stopping bleeding from a severed vessel, which requires adequate numbers of functional platelets. 7  Hemostasis In primary hemostasis, the severed blood vessel constricts. Circulating platelets aggregate at the site and adhere to the vessel and to one another. An unstable hemostatic plug is formed. For the coagulation process to be correctly activated, circulating inactive coagulation factors must be converted to active forms. This process occurs on the surface of the aggregated platelets at the site of vessel injury. The end result is the formation of fibrin, which reinforces the platelet plug and anchors it to the injury site. 8 Assessment Health History A careful health history and physical assessment can provide important information related to a patient’s known or potential hematologic diagnosis. Nutritional history , History of Anemia The use of prescription and over-the-counter medications, as well as herbal supplements Past medical, surgical history: Frequent infections, Bleeding disorders, Radiotherapy, Recent trauma causing bleeding, Surgery to GI, Transplant surgery coagulation process (immunosuppression) The onset of a symptom or finding (e.g.,Rapid vs. Gradual; persistent vs. Intermittent) Its severity, and any contributing factors can further differentiate potential causes. 9  Physical Examination The physical assessment should be comprehensive and include careful attention to the skin, oral cavity, lymph nodes, and spleen General appearance-Color: pale (anemia), jaundice (hemolytic anemia), Dyspnea, wheeze, difficulties, Bruising. Ask pt. if tenderness anywhere, so do not over palpate painful Nails Koilonychia (iron deficiency) spoon nail Pale nails (anemia). Hands: Pallor of palmar creases (anemia 2° to blood loss),Pulse: rate (anemia has tachycardia), rhythm, character. Arms : Scratch marks , Palpable purpura Eyes :Conjunctiva: pale (anemia),Sclera: jaundice (hemolytic anemia), hemorrhages Mouth :Gum hypertrophy (leukemia),Gum bleeding, Scurvy ( Vit C. deficiency) Tongue: glossitis (iron deficiency, megaloblastic anemia). 10 Diagnostic Evaluation Hematologic Studies -The most common tests used are the complete blood count (CBC) and the peripheral blood smear. CBC- identifies the total number of blood cells (leukocytes, erythrocytes, and platelets) as well as the hemoglobin, hematocrit (percentage of blood volume consisting of erythrocytes), and RBC indices. Peripheral smear -In this test, a drop of blood is spread on a glass slide, stained, and examined under a microscope. The shape and size of the erythrocytes and platelets, as well as the actual appearance of the leukocytes, provide useful information in identifying hematologic conditions. - 11  Bone Marrow Aspiration and Biopsy To assess the quantity and quality of each type of cell produced within the marrow. These tests are also used to document infection or tumor within the marrow. In adults, bone marrow is usually aspirated from the iliac crest and occasionally from the sternum. Nursing interventions Careful explanation of the procedure An antianxiety agent may be administered A signed informed consent is obtained Assist the patient in maintaining a comfortable position and encourage relaxation and deep breathing throughout the procedure After the marrow sample is obtained, pressure is applied to the site for several minutes Most patients have no discomfort after a bone marrow aspiration, but the site of a biopsy may ache for 1 or 2 days. Warm tub baths and a mild analgesic agent (e.g., acetaminophen [Tylenol]) may be useful. Aspirin-containing analgesic agents should be avoided in the immediate postprocedure period because they can aggravate or potentiate bleeding Potential complications are bleeding and infection, allergic reaction to anesthesia, persistent pain in the biopsy site Watch video: https://www.youtube.com/watch?v=EYd7OnCt7ug 12 Therapeutic Approaches to Hematologic Disorders Splenectomy-The surgical removal of the spleen Therapeutic Apheresis - blood is taken from the patient and passed through a centrifuge, where a specific component (platelets or leukocytes or plasma) is separated from the blood and removed. The remaining blood is then returned to the patient Hematopoietic Stem Cell Transplantation -stem cells are obtained from a donor whose cells match those of the patient(allogeneic transplant) or the patient’s own stem cells are harvested and then used (autologous transplant). Therapeutic phlebotomy is the removal of a certain amount of blood under controlled conditions. **Splenectomy** is indicated in conditions like hypersplenism, where the spleen excessively removes blood cells, leading to anemia or thrombocytopenia. It's also performed in certain hematologic malignancies, such as lymphoma, and for splenic rupture. **Therapeutic Apheresis** is indicated for autoimmune disorders (e.g., myasthenia gravis), hematologic conditions (e.g., sickle cell disease), and hyperlipidemia. It selectively removes harmful blood components, improving symptoms and preventing disease progression. **Hematopoietic Stem Cell Transplantation** is indicated for severe hematologic diseases like leukemia, lymphoma, and aplastic anemia. It can restore healthy blood cell production through allogeneic or autologous transplants. **Therapeutic Phlebotomy** is indicated for conditions like hemochromatosis and polycythemia vera, where excess blood volume or iron needs to be reduced to prevent complications. It helps manage symptoms and reduce risks associated with high blood cell counts. 13  Blood Transfusion Administration of blood and blood components requires knowledge of correct administration techniques and possible complications. Preprocedure 1. Confirm that the transfusion has been prescribed. 2. Check that patient’s blood has been typed and cross-matched. 3. Verify that patient has signed a written consent form per institution or agency policy and agrees to procedure. 4. Explain procedure to patient. Instruct patient in signs and symptoms of transfusion reaction (itching, hives, swelling, shortness of breath, fever, chills). 5. Take patient’s temperature, pulse, respiration, blood pressure and assess fluid volume status (e.g., auscultate lungs, assess for jugular venous distention) to serve as a baseline for comparison during transfusion. 6. Note if signs of increased fluid overload present (e.g., heart failure), contact primary provider to discuss potential need for a prescription for diuretic, as warranted. 7. Use hand hygiene and wear gloves in accordance with standard precautions. 8. Use appropriately sized needle for insertion in a peripheral vein. Use special tubing that contains a blood filter to screen out fibrin clots and other particulate matter. Do not vent blood container 14  Procedure 1. Obtain packed red blood cells (PRBCs) from the blood bank after the IV line is started. (Institution policy may limit release to only 1 unit at a time.) 2. Double-check labels with another nurse or physician to ensure that the ABO group and Rh type agree with the compatibility record. Check to see that number and type on donor blood label and on patient’s medical record are correct. Confirm patient’s identification by asking the patient’s name and checking the identification wristband. 3. Check blood for gas bubbles and any unusual color or cloudiness. (Gas bubbles may indicate bacterial growth. Abnormal color or cloudiness may be a sign of hemolysis.) 4. Make sure that PRBC transfusion is initiated within 30 minutes after removal of PRBCs from blood bank refrigerator. 5. For the first 15 minutes, run the transfusion slowly—no faster than 5 mL/min. Observe patient carefully for adverse effects. If no adverse effects occur during the first 15 minutes, increase the flow rate unless patient is at high risk for circulatory overload. 6. Monitor closely for 15–30 minutes to detect signs of reaction. Monitor vital signs at regular intervals per institution policy; compare results with baseline measurements. Increase frequency of measurements based on patient’s condition. Observe patient frequently throughout the transfusion for any signs of adverse reaction, including restlessness, hives, nausea, vomiting, torso or back pain, shortness of breath, flushing, hematuria, fever, or chills. Should any adverse reaction occur, stop infusion immediately, notify primary provider, and follow the agency’s transfusion reaction standard. 7. Note that administration time does not exceed 4 hours because of increased risk of bacterial proliferation. 15 8. Change blood tubing after every 2 units transfused to decrease chance of bacterial contamination  Postprocedure 1. Obtain vital signs and breath sounds; compare with baseline measurements. If signs of increased fluid overload present (e.g., heart failure) consider obtaining prescription for diuretic as warranted. 2. Dispose of used materials properly. 3. Document procedure in patient’s medical record, including patient assessment findings and tolerance to procedure. 4. Monitor patient for response to and effectiveness of procedure. If patient is at risk, monitor for at least 6 hours for signs of transfusion associated circulatory overload (TACO); also monitor for signs of delayed hemolytic reaction. characterized by fluid overload in the circulatory system, leading to symptoms like shortness of breath, high blood pressure, and leg swelling. It typically occurs within 12 hours of a transfusion and is more common in patients with heart or kidney issues Note: Never add medications to blood or blood products; if blood is too thick to run freely, normal saline may be added to the unit. If blood must be warmed, use an inline blood warmer with a monitoring system. 16  Nursing Management for Transfusion Reactions If a transfusion reaction is suspected, the transfusion must be stopped immediately and the primary provider notified. Maintain the IV line with normal saline solution through new IV tubing, given at a slow rate. Compare the vital signs with baseline, including oxygen saturation. Assess the patient’s respiratory status carefully. Note the presence of adventitious breath sounds; the use of accessory muscles; extent of dyspnea; and changes in mental status, including anxiety and confusion. Note any chills, diaphoresis, jugular vein distention, and reports of back pain or urticaria. Notify the primary provider of the assessment findings, and implement any treatments prescribed. Continue to monitor the patient’s vital signs and respiratory, cardiovascular, and renal status. Notify the blood bank that a suspected transfusion reaction has occurred. Send the blood container and tubing to the blood bank for repeat typing and culture. The patient’s identity and blood component identifying tags and numbers are verified. 17  ANEMIA Anemia is a condition in which the hemoglobin concentration is lower than normal It reflects the presence of fewer than the normal number of erythrocytes (i.e., red blood cells [RBCs]) within the circulation. As a result, the amount of oxygen delivered to body tissues is also diminished. Anemia is not a specific disease state but a sign of an underlying disorder. It is by far the most common hematologic condition. Classification of Anemias Hypoproliferative anemia is characterized by the inadequate production of red blood cells (RBCs) due to insufficient activity in the bone marrow. Hypoproliferative anemia - caused by a defect in the RBC production Eg: Iron deficiency anemia, Vitamin B12 deficiency inadequate production of red blood cells (RBCs) by the bone marrow, often due to deficiencies in iron, folate, or B12 Hemolytic anemia- caused by the destruction of RBC Eg: Altered erythropoiesis (sickle cell anemia, thalassemia),Hypersplenism (hemolysis),Drug induced anemia, Autoimmune anemia, Mechanical heart valve related anemia Bleeding – Caused by the RBC loss Eg: Bleeding from gastro intestinal tract (gastric ulcers, hemorrhoids), epistaxis, trauma, genitourinary tract (menorrhagia). 18  Clinical Manifestations An otherwise healthy person can often tolerate as much as a 50% gradual reduction in hemoglobin (e.g., over months) without pronounced symptoms or significant incapacity, whereas the rapid loss of as little as 30% (e.g., over minutes) may precipitate profound vascular collapse in the same person. A person who has become gradually anemic, with hemoglobin levels between 9 and 11 g/dL, usually has fewer or no symptoms other than slight tachycardia on exertion and possibly fatigue Assessment and Diagnostic Findings CBC -hemoglobin, hematocrit, reticulocyte count, and RBC indices Serum iron level, total iron-binding capacity Bone marrow aspiration Complications - heart failure, paresthesias, angina and delirium. Medical Management - correcting or controlling the cause, transfusion of packed red blood cells 19 NURSING DIAGNOSES Based on the assessment data, major nursing diagnoses may include: Fatigue related to decreased hemoglobin and diminished oxygen carrying capacity of the blood Imbalanced nutrition, less than body requirements, related to inadequate intake of essential nutrients Activity intolerance related to inadequate hemoglobin and hematocrit Insufficient physiological or psychological energy to complete daily activities Noncompliance with prescribed therapy 20 Nursing Interventions Assist patient in prioritizing activities and balancing rest and activity. Maintain physical activity to prevent deconditioning. Ensure intake of essential nutrients like iron, B12, folic acid. Inform the patient that alcohol interferes with the utilization of essential nutrients Dietary supplements (e.g., vitamins, iron, folate, protein) may be prescribed. Beware of iron overload in patients with chronic transfusion needs. Patients with chronic transfusion needs, such as those with thalassemia or sickle cell disease, are at risk of iron overload due to the accumulation of iron from repeated blood transfusions. Each unit of transfused blood contains about 200 to 250 mg of iron, and the body lacks a mechanism to eliminate excess iron, leading to its deposition in organs like the heart and liver Manage activity Intolerance with transfusions or IV fluids, as per symptoms. Address issues with medication adherence, such as GI effects of iron supplements. Monitor for signs like paresthesias, difficulty maintaining balance, gait disturbances. 21  Hypoproliferative Anemias Iron Deficiency Anemia Other names, microcytic or hypochromic A condition in which the total body iron content is decreased below a normal level, affecting hemoglobin synthesis. RBCs appear pale and are small. From a diet too low in iron or from the body not absorbing enough iron from the gastrointestinal tract The most common cause is chronic blood loss (GI bleeding) The body store1/4 to 1/3 of its iron, until those stores are depleted that iron deficiency anemia actually begins to develop. Iron deficiency anemia is the most common type of anemia in the world In Saudi Arabia it affects particularly women and children , 38% of the children were anemic, 22.6% were borderline 22 Iron Deficiency Anemia Assessment Findings: Pallor (most common symptom) Glossitis Headache Dizziness Irritability Dyspnea Palpitations Impaired thought processes Angular cheilosis (inflammation of lips: Depression The corners of mouth may be cracked, Parasthesias reddened and painful). Koilonychia (spoon-shaped nails) Weakness and fatigue Pale, dry mucous membranes Sensitivity to cold Stomatitis Pica-Appetite for substances that are not edible (non nutritive) persist for one month - Clay, coal, soil, feces, chalk, paper, soap, mucus, flour, raw potato, raw rice, starch, ice cubes) 23 # Pallor **Scenario:** A 45-year-old male comes in with complaints of a sore, swollen tongue and painful cracks at the corners of his mouth. He also mentions that he has been feeling irritable and has had difficulty concentrating at work. **Question:** Which condition is most likely indicated by these symptoms? - A) Oral thrush - B) Iron deficiency anemia - C) Candidiasis - D) Allergic reaction **Answer:** B) Iron deficiency anemia --- pregnant woman visits the clinic expressing unusual cravings for ice and clay. She also reports feeling weak and fatigued throughout the day. **Question:** What condition might this patient be experiencing? - A) Hyperemesis gravidarum - B) Iron deficiency anemia - C) Gestational diabetes - D) Thyroid dysfunction **Answer:** B) Iron deficiency anemia --- ## #### 5. Impaired Thought Processes and Depression **Scenario:** A 50-year-old woman reports increased feelings of depression and difficulty focusing at work. She has experienced fatigue, dizziness, and has noticed her nails becoming brittle. **Question:** What is the most likely diagnosis for this patient's symptoms? - A) Major depressive disorder - B) Iron deficiency anemia - C) Anxiety disorder - D) Sleep apnea **-support-in-medicine/hospital-medicine/hypo-proliferative-anemias/ 24  Iron Deficiency Anemia Diagnostic Evaluation: Hematology shows decreased Hemoglobin level, hematocrit, iron, ferritin, reticulocytes, red cell indices, transferrin, and saturation; absent hemosiderin; and increased iron- binding capacity. Peripheral blood smear reveals microcytic and hypochromic RBCs. Treatment: Diet high in Iron, roughage, and protein with increased fluids and avoidance of teas and coffee, which reduce iron absorption. Transfusion therapy with packed RBCs, if necessary Drug therapy options: Anti-anemic: ferrous sulfate, iron dextran Vitamins: pyridoxine hydrochloride (vitamin B6), Ascorbic acid (vitamin C), cyanocobalamin (Vitamin B12) 25  Iron Deficiency Anemia Nursing Diagnosis: Imbalanced nutrition: less than body requirements related to inadequate intake of iron. Activity intolerance related to decreased oxygen-carrying capacity of the blood. Ineffective tissue perfusion related to decreased oxygen-carrying capacity of the blood. 26  Iron-Deficiency Anemia Nursing Interventions Assess cardiovascular & respiratory status Monitor vital signs Recognizing sign and symptoms of bleeding Monitor stool, urine and emesis for occult blood Diet teaching—foods rich in iron Provide periods of rest Supplemental iron Discuss diagnostic studies Emphasize compliance Iron therapy for 2-3 months after the hemoglobin levels return to normal 27  Patient Education Food sources high in iron include organ meats (e.g., beef or calf’s liver, chicken liver), other meats, beans (e.g., black, pinto, and garbanzo), leafy green vegetables, raisins, and molasses. Taking iron-rich foods with a source of vitamin C (e.g., orange juice) enhances the absorption of iron. Patients with a history of eating fad diets or strict vegetarian diets are counseled that such diets often contain inadequate amounts of absorbable iron. The nurse encourages the patient to continue iron therapy for as long as it is prescribed even though the patient may no longer feel fatigued. Because iron is best absorbed on an empty stomach, the patient is instructed to take the supplement an hour before meals. Many patients have difficulty tolerating iron supplements because of GI side effects (primarily constipation, but also cramping, nausea, and vomiting). Decreasing the frequency of taking iron supplements, or taking with food can mitigate the GI symptoms, but will diminish absorption of the iron While taking iron supplements, eat foods high in fiber to minimize problems with constipation; add stool softener if needed, Not to take Antacids or dairy products with iron (diminish iron absorption) Remember that stools will become dark in color because of Iron supplements Prevent staining the teeth with a liquid Iron preparation by using a straw or placing a spoon at the back of the mouth to take the supplement. Rinse the mouth thoroughly afterward. 28  APLASTIC ANEMIA 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 Can be acquired or, rarely, congenital, but most cases are idiopathic Certain drugs (e.g., streptomycin and chloramphenicol) and exposure to toxic chemicals/radiation cause bone marrow failure Signs/ Symptoms-Infection and the symptoms of anemia (e.g., fatigue, pallor, dyspnea), Purpura (bruising) Diagnosis- CBC reveals pancytopenia (insufficient numbers of RBCs, WBCs and Platelets), Bone marrow biopsy Treatment: hematopoietic stem cell transplant (HSCT), immunosuppressive therapy, Supportive therapy – transfusion, antibiotics Nursing intervention -Assess carefully for signs of infection and bleeding 29 Megaloblastic Anemias Caused by deficiencies of vitamin B12 or folic acid The erythrocytes that are produced are abnormally large and called megaloblastic red cells. Folic Acid Deficiency Folate is found in green vegetables and liver. Thus, folate deficiency occurs in people who rarely eat uncooked vegetables, in pregnant women, in patients with liver disease, malabsorptive diseases of the small bowel etc Folic Acid required for RBC formation and maturation Causes : ✓ Poor dietary intake ✓ Malabsorption syndromes ✓ Drugs that inhibit absorption ✓ Alcohol abuse ✓ Hemodialysis 30  Folic Acid Deficiency Assessment Findings: Fatigue Weakness Pallor Dizziness Headache Tachycardia Sore tongue Cracked lips Diagnostic Evaluation and Treatment: Vitamin B12 and folic acid level – folic acid will be decreased. Complete blood count (CBC) will show decreased RBC, hemoglobin, and hematocrit with increased mean corpuscular volume and mean corpuscular hemoglobin concentration. Oral folic replacement on daily basis 31  Folic Acid Deficiency Nursing Diagnosis: Imbalanced Nutrition: Less than body requirements related to inadequate intake of folic acid Nursing Intervention: Assess diet for inclusion of food rich in folic acid: beef liver, peanut butter, red beans, oatmeal, broccoli, asparagus. Arrange nutritionist referral Assist alcoholic patient to obtain counseling and additional medical care Teach patient to select balanced diet that includes green vegetables (asparagus, broccoli, spinach), yeast, liver and other organ meats, some fresh fruits; avoid overcooking vegetables. Encourage patient to follow up periodically to monitor CBC 32 33  PERNICIOUS ANEMIA Pernicious anemia is a type of megaloblastic anemia associated with vitamin B12 deficiency. Also called, Addison’s anemia. Vitamin B12 is necessary for normal deoxyribonucleic acid synthesis in maturing RBCs. Pernicious anemia demonstrates familial incidence related to autoimmune gastric mucosal atrophy. Intrinsic Factor (IF) Summary 1. 2. 3. 4. 5. 6. Production: Secreted by parietal cells in the stomach. Vitamin B12 Binding: Binds specifically to vitamin B12, protecting it during digestion. Absorption Facilitation: The IF-B12 complex binds to receptors in the ileum, allowing for active absorption into the bloodstream. Pernicious Anemia Prevention: A deficiency in intrinsic factor can lead to pernicious anemia due to inadequate vitamin B12 absorption. Autoimmune Connection: Autoimmune conditions can impair IF production, resulting in vitamin B12 deficiency. Clinical Relevance: Measurement of intrinsic factor levels or antibodies is important for diagnosing certain anemias and assessing gastrointestinal health. Normal gastric mucosa secretes a substance called intrinsic factor, necessary for absorption of vitamin B12 in ileum. If a defect exists in gastric mucosa, or after gastrectomy or small bowel disease, intrinsic factor may not be secreted and orally ingested B12 not absorbed. 34 Pernicious Anemia Sore, red tongue Mild diarrhea Pale mucous membranes Confusion Paresthesias in extremities Balance issues, and loss of position sense. Symptoms are progressive, with spontaneous partial remissions and exacerbations. Without treatment, patients can die from heart failure secondary to anemia. 35  Pernicious Anemia Diagnostic Evaluation Blood chemistry test results reveal increased bilirubin and lactate dehydrogenase levels (LDH) Bone marrow aspiration specimen shows increased megaloblasts, few maturing erythrocytes, and defective leukocyte maturation Gastric analysis shows hypochlorhydria Hematology shows decreased HCT and Hb levels Romberg Test and/or Schilling Test: a medical investigation used for patients with vitamin B12 deficiency. Upper G.I. endoscopy shows atrophy of gastric mucosa Peripheral blood smear reveals oval, macrocytic, hyperchromic erythrocytes. 36  Pernicious Anemia Treatment: Establishing a diet high in iron and protein and restricting highly seasoned, coarse, or extremely hot foods. Vitamin B12 replacement is used for vitamin B12 deficiency. Vegans can prevent or treat deficiency with oral supplements or fortified soy milk. Monthly intramuscular injections of vitamin B12 are used for deficiencies due to defect in absorption or absence of intrinsic factor. Vitamin B12 replacement leads to increased reticulocyte count and return to normal blood counts. Recovery time is longer for severe neurologic manifestations. Vitamin B12 therapy must be continued for life to prevent recurrence of pernicious anemia. 37  Pernicious Anemia Nursing Diagnosis: Disturbed thought processes related to neurologic dysfunction in absence of vitamin B12. Impaired sensory perception (kinesthetic) related to neurologic dysfunction in absence of vitamin B12. Imbalanced nutrition: less than body requirements Nursing Interventions: Early detection and treatment can lead to reversal of symptoms Assess patient for diminished sensations to heat and pain Compliance with medication regimen Ongoing evaluation of GI and neuro status Evaluate patient for gastric carcinoma frequently Increase dietary intake of foods rich in vitamin B12 if the anemia is the result of a dietary deficiency Eg: Citrus fruits, Dried beans, Green, leafy vegetables, Nuts, Organ meats 38 HEMOLYTIC ANEMIAS In hemolytic anemias, the erythrocytes have a shortened lifespan; thus, their number in the circulation is reduced. Fewer erythrocytes result in decreased available oxygen, causing hypoxia, which in turn stimulates an increase in erythropoietin release from the kidney. Erythropoietin then 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; the majority of the heme is converted to bilirubin, conjugated in the liver, and excreted in the bile 39  SICKLE CELL DISEASE It is a severe hemolytic anemia that results from inheritance of the sickle hemoglobin gene causes the hemoglobin molecule to be defective. The sickle hemoglobin (HbS) acquires a crystal-like formation when exposed to low oxygen tension Sickled cells are rapidly hemolyzed and thus have a very short lifespan (10 to12 days). If the erythrocyte is again exposed to adequate amounts of oxygen (eg, when it travels through the pulmonary circulation) before the membrane becomes too rigid it can revert to a normal shape. For this reason, the “sickling episodes” are intermittent (discontinuous). Cold can aggravate the sickling process (vasoconstriction slows the blood flow) The term sickle cell trait refers to the carrier state for SC diseases; it is the most benign type of SC disease, in that less than 50% of the hemoglobin within an erythrocyte is HbS. Carried on a recessive gene; a person must inherit the gene from both the mother and the father to actually have the disease 40 Pathophysiology Erythrocyte Decreased containing HbS oxygen level in loses its round, venous blood pliable, biconcave disk shape Adhere to the They’re stiff and Becomes endothelium of sticky and tend to deformed, rigid, small vessels & to form clumps and and sickle shaped each other organ get stuck in blood Result in pain, Decreased blood Ischemia or swelling, and flow to a region infarction fever. 41  Clinical Manifestations Symptoms and complications result from chronic hemolysis or thrombosis. Hemoglobin values are 7 to 10 g/dL Jaundice (obvious in the sclera) Enlargement of the bones of the face and skull (due to The bone marrow expands inchildhood in a compensatory effort to offset the anemia) The chronic anemia is associated with: Tachycardia, cardiac murmurs, and enlarged heart (cardiomegaly), Dysrhythmias and heart failure (in adults) Any organ may be affected by thrombosis (primary sites such as the spleen, lungs, and central nervous system) Sickled Patients are susceptible to infection (pneumonia and osteomyelitis) Complications ✓ Infection ✓ Stroke ✓ Renal failure, impotence ✓ heart failure 42 ✓ Pulmonary hypertension  Sickle Cell Episodes (Crisis) There are three types of sickle cell episodes/crisis in the adult population Very painful acute vaso-occlusive crisis (common) results from tissue hypoxia and necrosis due to inadequate blood flow to a specific region of tissue or organ. 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. Pooling” in this context means that sickled cells accumulate or gather in an organ, obstructing blood flow. Sequestration crisis results when other organs pool the sickled cells. Sequestration means the abnormal trapping or accumulation of red blood cells in specific organs, preventing them from circulating normally in the bloodstream. In children spleen is the most common organ responsible for sequestration, most children with sickle cell anemia have had a splenic infarction by 10 years of age, and the spleen is then no longer functional (auto splenectomy). In adults, the common organs involved in sequestration are the liver and, more seriously, the lungs. 43  Medical Management Aggressive management of symptoms and complication Hematopoietic Stem Cell Transplant(HSCT )may cure SCD. However, this treatment modality is available to only a small subset of affected patients, either due to the lack of a compatible donor or due to severe organ damage (e.g., renal, liver, lung) that may be already present in the patient Pharmacologic Therapy Pain management with NSAIDS-Aspirin, parenteral opioids Hydroxyurea - a chemotherapy agent increases fetal hemoglobin (ie, hemoglobin F) levels in patients with sickle cell anemia, & decreasing the formation of sickled cells daily folic acid replacements Transfusion Therapy -RBC transfusions are highly effective in several situations Exchange transfusion -The patient’s own blood is removed and replaced via transfusion, performed to diminish the risk of increasing the viscosity excessively The objective is to patient’s own blood volume with transfused (non-sickled) packed red cells to a target hemoglobin of 10 g/dL. 44 NURSING DIAGNOSES Acute pain and fatigue related to tissue hypoxia due to agglutination of sickled cells within blood vessels Risk for infection Risk for powerlessness related to illness-induced helplessness Deficient knowledge regarding sickle crisis prevention 45  Nursing Interventions Managing pain Any joint that is acutely swollen should be supported and elevated until the swelling diminishes. Relaxation techniques, breathing exercises, and distraction are helpful for some patients. Physical therapy, whirlpool baths, and transcutaneous electrical nerve stimulation Managing fatigue Assisting the patient to find an appropriate balance between exercise and rest Maximizing nutrition, hydration, healthy sleep cycles Preventing and managing infection Prescribed antibiotics should be initiated promptly Promoting coping skills focuses on the patient’s strengths rather than deficits can enhance effective coping skills Monitoring and managing potential complications Leg Ulcers, Priapism Leading to Impotence 46  Nursing Interventions Provide emotional support to allay the client’s anxiety Refer the client for genetic counseling to decrease his anxiety and help him understand the chances of passing the disease to his offspring. During a crisis Apply warm compresses to painful areas and cover the client with a blanket. Cold compresses and temperature can aggravate his condition Administer an analgesic-antipyretic such as aspirin or acetaminophen for pain relief. (additional pain relief may be necessary during an acute crisis) Maintain bed rest to reduce the heart’s workload and reduce pain Administer blood components (packed RBCs), as ordered, for aplastic crisis caused by bone marrow suppression. Encourage fluid intake to prevent dehydration, which can precipitate a crisis. Administer prescribed I.V. fluids to ensure fluid balance and renal perfusion. Give antibiotics as ordered to treat infections and avoid precipitating a crisis. 47  THALASSEMIA 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) ✓ Destruction of blood elements (hemolysis) ✓ Variable degrees of anemia The thalassemias occur worldwide, but the highest prevalence is found in people of Mediterranean, African, and Southeast Asian ancestry Thalassemias are classified into two major groups according to which hemoglobin chain is diminished: alpha or beta beta-thalassemias are prevalent in people from Mediterranean regions & Middle East or Asia 48 THALASSEMIA Pathophysiology Thalassemias are associated with defective synthesis of hemoglobin; the production of one or more globulin chains within the hemoglobin molecule is reduced. When this occurs, the imbalance in the configuration of the hemoglobin causes it to precipitate in the erythroid precursors or the erythrocytes themselves. This increases the rigidity of the erythrocytes and thus the premature destruction of these cells Clinical manifestations- severe anemia, marked hemolysis, and ineffective erythropoiesis Treated by- regular transfusion therapy, Regular chelation therapy (reduce the complications of iron overload) 49  LEUKEMIA The term leukocytosis refers to an increased level of leukocytes (white blood cells [WBCs]) in the circulation. Typically, only one specific cell type is increased. Because the proportions of several types of leukocytes (e.g., eosinophils, basophils, monocytes) are small, an increase in other types can be great enough to elevate the total leukocyte count, particularly the neutrophil or lymphocyte. Although leukocytosis can be a normal response to increased need (e.g., in acute infection), the elevation in leukocytes should decrease as the physiologic need decreases. A significant cause of persistent leukocytosis is hematologic malignancy (i.e., leukemia). Leukemias are a group of hematological malignancies involving abnormal overproduction of leukocytes, usually at an immature stage, in the bone marrow 50 LEUKEMIA The two major types of leukemia - lymphocytic (involving abnormal cells from the lymphoid pathway) and myelocytic or myelogenous (involving abnormal cells from the myeloid pathways). Leukemia may be acute, with a sudden onset, or chronic, with a slow onset and persistent symptoms over a period of years. Leukemia affects the bone marrow, causing anemia, leukopenia, the production of immature cells, thrombocytopenia, and a decline in immunity. The cause is unknown. Risk factors include genetic, viral, immunological, and environmental factors and exposure to radiation, chemicals, and medications, such as previous chemotherapy. 51  LEUKEMIA: CLASSIFICATION Acute or Chronic -based on the time it takes for symptoms to evolve and the phase of cell development that is halted Acute leukemia – ✓ The onset of symptoms is abrupt, often occurring within a few weeks. ✓ Most leukocytes are undifferentiated cells or are blasts stem cells, are cells that have not yet developed into a specific ✓ Progresses very rapidly; death occurs within weeks to months cell type. They have the potential to differentiate into various cell types Chronic leukemia- ✓ Symptoms evolve over a period of months to years ✓ The majority of leukocytes produced are mature ✓ Progresses more slowly; the disease can extend for years 52  LEUKEMIA Assessment Findings: Overt bleeding (nosebleeds, gum bleeding, rectal bleeding, hematuria, increased menstrual flow) and occult bleeding (e.g., as detected in a fecal occult blood test) Prolonged bleeding after minor abrasions or lacerations Anorexia, fatigue, weakness, weight loss Symptoms of Anemia Anemia Fatigue: Persistent tiredness and weakness. Pallor: Pale skin and mucous membranes. Dyspnea on exertion: Shortness of breath during physical activity. Ecchymosis, petechiae Elevated heart rate: Palpitations or tachycardia. Dizziness: Lightheadedness or fainting spells. Symptoms of Thrombocytopenia Elevated temperature Bleeding: Overt bleeding (nosebleeds, gum bleeding, rectal bleeding) and occult bleeding (detected via tests). Bruising: Unusual bruising (ecchymosis, petechiae). Prolonged bleeding: Extended bleeding after minor injuries. Enlarged lymph nodes, spleen, liver Increased menstrual flow: Heavy periods in women. Symptoms of Leukemia Fever: Elevated temperature often without clear cause. Pallor and dyspnea on exertion Enlarged lymph nodes, spleen, liver: Swelling in these areas due to leukemic infiltration. Bone pain and joint swelling: Discomfort in bones and joints. Weight loss and anorexia: Decreased appetite leading to significant weight loss. Headache Headaches: Frequent headaches due to various factors including anemia or leukemic infiltration. Increased susceptibility to infections: Frequent or severe infections due to compromised immunity. Bone pain and joint swelling Palpitations, tachycardia, orthostatic hypotension Normal, elevated, or reduced white blood cell count Decreased hemoglobin and hematocrit levels, and platelet count Positive bone marrow biopsy identifying leukemic blast–phase cells 53  LEUKEMIA Diagnostic Evaluation: Bone marrow biopsy reveals a large number of immature leukocytes Hematology shows decreased hematocrit (HCT), hemoglobin (Hb), Red blood cells (RBCs), and platelets and increased erythrocyte sedimentation rate (ESR), immature WBCs and prolonged bleeding time. Treatment: High-protein, high vitamin and mineral diet, involving soft, bland foods in small frequent feedings. Stem cell transplant Transfusion of platelets, packed RBCs and whole blood. Alkylating agents: busulfan, chlorambucil, cyclophosphamide Antitumor antibiotics: doxorubicin, plicamycin, daunorubicin, mitoxantrone, idarubicin Antimetabolites: fluorouracil, methotrexate, cytarabine, 6-mercaptopurine, 6-thioguanine, fludarabine Antineoplastic (mitotic inhibitors): vinblastine Corticosteroid: prednisone Hematopoietic growth factor: epoetin alfa Monoclonal antibodies: rituximab, alemtuzumab Nitrosourea: carmustine 54  LEUKEMIA :Nursing Diagnoses Risk for infection, bleeding, or both Impaired oral mucous membranes due to changes in epithelial lining of the GI tract from chemotherapy or prolonged use of antimicrobial medications Imbalanced nutrition: less than body requirements related to hypermetabolic state, anorexia, mucositis, pain, and nausea Acute pain and discomfort related to mucositis, leukocyte infiltration of systemic tissues, fever, and infection Fatigue and activity intolerance related to anemia, infection, inadequate nutrition, and deconditioning Risk for imbalanced fluid volume related to renal dysfunction, diarrhea, bleeding, infection, increased metabolic rate, hypoproteinemia, and need for multiple intravenous medications and blood products Self-care deficits (bathing, dressing, toileting) due to fatigue and malaise Anxiety and grieving due to uncertainty about future, anticipatory loss, and altered role functioning Risk for spiritual distress Deficient knowledge about disease process, treatment, complication management, and self-care measures 55  Nursing Interventions Preventing/Managing Infection and Bleeding: Managing Mucositis: Improving Nutritional Intake: Disease increases metabolic rate/nutritional requirements. Pain from stomatitis reduces intake. Encourage/provide mouth care before/after meals; administer analgesics before eating. Use oral anesthetic agents carefully to avoid biting tongue/buccal mucosa. Effective antiemetic therapy to manage nausea. Small, frequent, soft-texture, moderate-temperature feedings. Avoiding uncooked fruits or vegetables and those without a peelable skin Consider low-microbial diets; use nutritional supplements. Monitor daily body weight, intake/output. Calorie counts, formal nutritional assessments, parenteral nutrition may be needed. 56  Nursing Interventions Easing Pain and Discomfort: Acetaminophen for fever, but increases diaphoresis. Use cool water sponging, avoid ice packs. Frequent changing of bedclothes. Gentle back/shoulder massage. Mucositis management with oral hygiene and patient-controlled analgesia. Address psychological suffering with active listening and potential counseling referrals. Decreasing Fatigue and Activity Intolerance: Balance activity and rest. Use HEPA filter masks for ambulation outside despite neutropenia. Encourage use of stationary bicycles; sitting up in a chair can improve circulation. Physical therapy can be beneficial. Hospitalization may cause sleep deprivation; implement strategies for uninterrupted sleep while administering medications. 57 Nursing Interventions Maintaining Fluid and Electrolyte Balance: Monitor intake/output and daily weights. Assess for dehydration and fluid overload. Pay attention to pulmonary status and dependent edema. Monitor lab results (electrolytes, BUN, creatinine, hematocrit). Replace electrolytes, especially potassium and magnesium. Patients on amphotericin or certain antibiotics are at risk for electrolyte depletion. Improving Self-Care: Perform hygiene measures if patient cannot. Encourage patient to maintain mobility and function to preserve self-esteem. Provide empathetic listening and reassure that deficits are temporary. Assist with resuming self-care as patient recovers. Coordinate with home care services for catheter management. 58 Nursing Interventions Managing Anxiety and Grief: Provide emotional support and discuss the uncertain future. Assess how much information the patient wants about the illness, treatment, and complications. Identify priorities to explain procedures and self-care expectations. Address depressive symptoms and help patients grieve losses (family roles, professional roles, physical functioning). Encourage time to adjust to major life changes and role restructuring. Support patient in identifying options and making important decisions. 59  REFERENCES ▪ Smeltzer, S.C., & Bare B.G. (2018). Brunner & Suddarth’s textbook of medical surgical nursing. 15th ed. Philadelphia: Lippincott. ▪ Clinical Handbook for Brunner & Suddarth's Textbook of Medical-Surgical Nursing,14th Ed. 2018 Author(s): Lippincott Williams & Wilkins ISBN/ISSN978149635514 ▪ Medical-Surgical Nursing Assessment and Management of Clinical Problems, 12th Edition, 2020. Author(s): Sharon Lewis, Linda Bucher, Margaret Heitkemper, Mariann Harding, Jeffrey Kwong, and Dottie Roberts. eBook ISBN: 9780323371438 Imprint: Mosby Published Date: September 2016 Page Count: 1776 ▪ Doenges, M., Moorhouse, M., Murr, A. (2019). Nursing Care Plans: Guidelines for individualizing client care across the life span 10th edition. Philadelphia: F.A. Davis company. ▪ Vallerand, A. and Sanoski, C. (2019) Davis’s Drug Guide for nurses, 17th ed. D.A. Davis ▪ Adams, M, Holland, L, & Urban, C. (2017). Pharmacology for Nurses: A Pathophysiological Approach, Second Canadian Edition, 5th edition, Pearson ▪ Berman A. , Snyder, S. , & Frandsen, G. (2021). Kozier & Erb's Fundamentals of Nursing: concepts, process and practice. 11th ed. 60 61