1. Care of Patients with Hematological (RBC) Disorders Prepared by: Salima Shams Reviewed Jan, 2016

2. Objectives:  Define Anemia.  Understand the pathophysiology of different types of anemia.  Iron deficiency Anemia  Vitamin B12 Deficiency Anemia  Folic Acid deficiency Anemia  Aplastic Anemia  Sickle cell anemia  Heamolytic anemia  polycthemia

3. Objectives:  Discuss the clinical manifestation, diagnostic studies, and management of a client with above mentioned disorder.  Utilize the nursing process to plan care for patient with above mentioned diseases.

4. RED BLOOD CELLS  The life span is approximately 120 days.  Normal values in males are 4.5-6.5X10 million/mm3 and in female is 3.9-5.5 X10 million/mm3.  The percentage of erythrocytes in whole blood is 45-50 and is expressed as HCT.  They are manufactured in the bone marrow (stem cells) and carry hemoglobin into it.  Erythroblast Normoblast Reticulocyte Erythrocyte

5. Hemoglobin consist of complex protein, globin and an iron containing substance called heme.

6. Function of RBC

8. Red Blood Cells Cont… Vitamin B 12 and folic acid help in maturation of RBC. Erythropoiesis : is a process of formation/maturation of erythrocytes (mainly occurs in bone marrow). Erythropoietin: Enzyme that controls the process of erythropoiesis. RBC degradation take place in spleen. Heme molecule is converted into bilirubin and transported to the liver, where it is converted into water soluble for elimination in the bile.

9. RBC continue:  Erythrocyte large in size …. Macrocytic  Erythrocyte smaller in size….. Microcytic.  Cells that contain normal hemoglobin …. Normochromic.  Cells those are deficient of hemoglobin... Hypochromic.

11. https://www.nlm.nih.gov/medlineplus/ency/an atomyvideos/000104.htm

12. Anemia Definition: deficiency in the RBC. OR Low level of hemoglobin found in the red blood cells. Condition result in reduction in an oxygen carrying capacity of the blood. Causes:  Loss of RBC  Decrease production  Increase destruction.

13. Anemia Loss of RBC: Blood loss (gastritis, hemorrhoids, menstruation, blood vessel rupture). Increase RBC destruction: Abnormal hemoglobin( sickle cell), auto immune antibodies, toxins. Decrease RBC production: (nutritional deficiency or stem cell failure) Iron, Vit B-12 deficiency, folic acid deficiency, thalassemia

15. IRON DEFICIENCY ANEMIA Very common world wide. It is a condition in which total body iron content is low, manifest by small RBC and low Hb levels. Risk Factor: Poverty Pregnancy Childhood Causes:  Low iron diet  Impaired iron absorption  Chronic blood loss (Heavy menstruation, GI bleeding)  Pregnancy

16. Iron deficiency anemia (continue): Diagnostic Tests:  Hb, RBC & Hct ?  Serum iron level?  Ferritin level?  Total iron-binding capacity  Other investigations might needed ( endoscopy, colonoscopy, stool occult blood test)

17. Iron deficiency anemia (continue): Management: correct underline cause (malnutrition, hemorrhage)  High iron diet. (almonds, egg yolk, spinach, liver, beef, beets, cabbage, cucumber etc)  Oral ferrous sulphate supplements (GI disturbances)  Parental iron (hypersensitivity reaction)  Avoid coffee, tea and carbonates.  Encourage cooking in iron pans

18. anemia Vitamin B-12 Deficiency Anemia/Pernicious anemia  It’s a autoimmune disorder characterized by absence of intrinsic factor (a substance needed to absorb vitamin B12 from the gastrointestinal tract).  Deficiency led's to impaired DNA synthesis result in defective and large RBCs (megaloblast ) formation.  Vitamin B12 deficiency anemia is also known as Pernicious anemia.

20. anemia Pernicious anemia/ Vitamin B Deficiency Anemia Cause: beside intrinsic factor deficiency other causes are:  Chronic gastritis  Gastrectomy  Factor is not secreted from the stomach( gastric atropy).  Hereditary/congenital.  Small intestine surgery  Drugs  Diet low in vitamin B12

21. anemia Pernicious anemia Clinical manifestations: In addition to other symptoms of anemia  Paresthesia i.e. tingling, numbness in hands and feet (poor production of myelin at nerve ending)  Confusion, memory loss, slow reflexes  Weak muscles  Difficult walking, poor gait

22. Folic Acid Deficiency Anemia:  Folic acid is also required for DNA synthesis and red cell maturation.  Folic acid is present in our blood and store in a body as compound folate.  Compound largely found in green leafy vegetables.  Compound is especially needed by females during pregnancy and pre pregnancy state. (why ?)  Government intiatives  Both vitamin B 12 deficiency and folic acid led to ( increased MCV and normal MCHC).

23. Anemia resulting from vitamin B12 or folate deficiency are sometimes referred to as “macrocytic” or “megaloblastic ” anemia. Megaloblast RBC

24. anemia Management of Pernicious anemia & Folic Acid Anemia: Diagnostic Tests:  Complete blood counts (CBC).  Vitamin B 12 & Folic acid level  Antibodies to intrinsic factor Management:  Diet high in Vit B12 (red meat (liver), dairy products and eggs)  Encourage client to eat a diet high in folic acid e.g green leafy vegetables, fortified cereals, peas, beans, nuts, melons, lemon, bananas, strawberries, milk, eggs etc.  Vitamin (folic acid and B 12) supplement, vitamin C  Treat underline GI conditions  Inj Vit. B12 monthly for life long

25. APLASTIC ANEMIA  Bone marrow is a sponge-like tissue inside the bones. In aplastic anemia hypoplasia /aplasia of bone marrow( stem cells) occur.  There may be actual reduction in the amount of blood-forming marrow, or the marrow may have a functional defect.  Along with anemia, patient may have decrease WBC (leukopenia) and Platelets (thrombocytopenia). i.e. pancytopenia.

26. APLASTIC ANEMIA Cont…… Causes:  Exposure to toxin (pesticides, and benzene etc)  Exposure to radiotherapy & chemotherapy.  Viral infections e.g hepatitis  Autoimmune disorders, such as lupus and rheumatoid arthritis.  idiopathic, rarely its inheritied S/S ? Diagnostic tests:  Bone marrow aspirate and biopsy. (hypocellularity)  C.B.C (leucopenia, thrombocytopenia,  erthrocytes count)  Check vitamin B12 and folate levels, iron levels.

28. APLASTIC ANEMIA Cont…… Management:  Reverse possible causes  Frequent blood and platelets transfusion.  Medication to stimulate bone marrow  Antibiotics.  Bone marrow transplantation (if possible).

30. APLASTIC ANEMIA Cont…… Teaching:  Frequent hand washing.  Take well cooked diet.  Stay away from people who are sick and avoid large crowds.  Frequently brush your teeth or rinse mouth to reduce the risk of infection in your mouth and throat.  Look for the signs of infections.  Stay away from activities that could result in injuries and bleeding, such as contact sports.

31. RBC Destruction

32. Hemolytic Anemia Definition: RBC’s are destroyed/die at shorter period of time. This result in retention/ overload of iron in body and other product of hemoglobin destruction and increase in erythropoiesis. Etiology:  Account for 5 % of anemias.  It could be hereditary (inherited) or acquired.  RBC destruction occur intravascular (toxic factor or transfusion reaction) or extravascular (RBC are less deformable)

34. Classification Intrinsic (inherited) Hemolytic Anemia:  Sickle cell Anemia (abnormal substitution of amino acid in hemoglobin)  Thalassemia's (polypeptide chains is defective) Extrinsic (Acquired) Hemolytic Anemia:  Drugs  Bacteria  Toxin  Antibodies

35. Movie Time  https://www.dnalc.org/resources/3d/17-sickle- cell.html https://www.dnalc.org/resources/3d/17-sickle- cell.html  http://www.webmd.com/children/video/sickle-cell- treatment

36. Inherited Hemolytic Anemia: Sickle cell Anemia Definition: Sickle cell disease is an inherited disorders of mutant hemoglobin that cause sickling of RBCs. Sickle cell RBC dies with in 10-20 days period. Normal hemoglobin (HbA) replaced with hemoglobin (HbS), Beta chain is mutated. Symptoms could vary from patient to patient.

39. SICKLE CELL DISEASE Pathophsiology: The sickle hemoglobin has the unfortunate property of acquiring a crystal like formation when exposed to low oxygen tension.  The oxygen in venous blood is low enough to cause this change.  The cell containing hemoglobin S becomes deformed, rigid, and sickle shaped when in the venous circulation or exposed to low oxygen conditions.

41. Clinical Manifestations (Adhesion of sickled erythrocytes to endothelium activates inflammatory process)

42. Risk factor  Illness and conditions (hypoxia, dehydration and acidosis)  Acidosis reduce affinity of hemoglobin for oxygen  cold  Stress  physical exertion  infection

43. SICKLE CELL DISEASE Complication:  Sickle cell crisis ( acute, vaso – occlusive crisis)  Multiple organ damage (liver, heart, brain, lungs and other major organ because of sluggish blood flow)

44. SICKLE CELL DISEASE Cont…… Diagnostic studies:  CBC (High white blood cell count, low Hb)  Hb electrophoresis  decrease O2 saturation  Elevated bilirubin  Abdominal ultrasound (spleen enlargement)  X-ray (pneumonia)

45. SICKLE CELL DISEASE Cont…… Management: to prevent sickle cell crisis Pain management Hydration Transfusions Daily folic acid Avoid strenuous exercises Avoid high altitude Avoid too much exposure to the sun Avoid emotional stress and sources of infection Teach relaxation techniques, deep breathing exercise. Genetic counseling ( sickle cell trait).

46. Type of AnemiaDescriptionExamples of Causes Iron Deficiency Lack of iron leads to decreased amounts hemoglobin; low levels of hemoglobin in turn leads to decreased production of normal RBCs Blood loss; diet low in iron; poor absorption of iron Pernicious Anemia and B Vitamin Deficiency Lack of B vitamins does not allow RBCs to grow and then divide as they normally would during development; leads to decreased production of normal RBCs Lack of intrinsic factor; diet low in B vitamins; decreased absorption of B vitaminsintrinsic factor Aplastic Decreased production of all cells produced by the bone marrow of which RBCs are one type Cancer therapy, exposure to toxins, autoimmune disorders, viral infections Hemolytic RBCs survive less than the normal 120 days in the circulation; leads to overall decreased numbers of RBCs Inherited causes include sickle cell and thalassemia; other causes include transfusion reaction, autoimmune disease, certain drugs (penicillin) Anemia of Chronic Diseases Various conditions over the long term can cause decreased production of RBCs Kidney disease, diabetes, tuberculosis or HIV

48. Nursing Diagnosis  Activity intolerance related to imbalance of oxygen supply and demand.  Altered nutrition: less than body requirement related to malabsorption or inadequate intake of vitamins, minerals.  High risk for infection related to myelosuppression  Altered health management or maintenance  Anxiety related to threat to health status.  Self-care deficit related to weakness in managing care.  High risk for falls related to weakness, dizziness.  Ineffective/ comprised family coping  Altered sexuality patterns related to weakness and fatigue.

49. Interventions  Monitor and regulate energy expenditure, planning activity and rest is important.  Provide assistance to perform ADL.  Administer oxygen in acute case. (Not in excessive amount because it suppresses the erythropoiesis).  Blood transfusion.  Assess the activity tolerance in daily basis.  Skin care is essential. It includes assessment proper positioning, bath with warm or tepid water, application of cream or lotion, proper mouthwash, and prevent patient from injury.  Assess for the pain, administered analgesics and non-invasive relaxation method.  Assess cerebral oxygenation, support client and family and provide reassurance.  Proper education of nature of the disorder, signs and symptoms, treatment plan, and plan to modify life style and minimizing risk for complication.

50. Polycythemia Definition: Polycythemia is an abnormally high total red blood cells mass, with a hematocrit greater than 50%. Types of Polycthemia: Relative & absolute. Relative: dehydration, use of diuretics and GI losses. Absolute: rise in Hematocrit because of increase in RBC mass. It accompanied by elevated WBC and platelets count. Results in increase blood viscosity, total blood volume, blood congestion in organs

52. Causes:  Excessive production of erythropoietin  neoplastic tumor  people living at high altitudes  smoker.  This may occur in response to a hypoxic stimulus as in COPD

53. Polycythemia Cont: Clinical manifestations:  Dark flushed skin  Hypertension / heart failure  Dizziness, headache, vertigo  Visual disturbance  Stroke, MI, peripheral gangrene  Enlarge liver and spleen (over work). Diagnostic Studies:  C.B.C. high (HB----18-25gm/100ml, RBC levels).  Hct level greater than 54% in male and 49% in females.  Platelet counts increase  increase WBC count  Increase uric acid level

54. Polycythemia Cont: Medical management: (2 goals)  Reduction in blood volume and viscosity.  Reduction of bone marrow activity.  Phlebotomy  Remove 500 ml-2000ml of blood until HCT reaches 45%,  Subsequent phlebotomies should be done as frequent as necessary for maintaining the Hct at 45%  Myelosuppressive Agents :  Administration of radioactive phosphorus, hydroxyurea

55. Polycythemia Cont…….  Radiation therapy:  To decrease the marrow activity.