Sickle cell anemia

POLYCYTEMIA VERA
Polycythemia vera is a bone marrow disease that leads to an abnormal increase in the number of blood cells (primarily red blood cells).
Lab. & Diagnostic Exams: Risk Factors:

Pathophysiology:

Erythrocyte elevation predominates Hematocrit elevation can exceed to 60% Spleen resumes its embryonic function of hematopoises & enlarges

o o o o

Thromboses Smoking Obesity Poorly controlled hypertension

Bone marrow biopsy Complete blood count with differential Comprehensive metabolic panel Erythropoietin level Genetic test for the JAK2V617F mutation Oxygen saturation of the blood Red blood cell mass Vitamin B12 level

Bone marrow become fibrotic Nursing Management: Advised patients with a history of significant bleeding to avoid aspirin and aspirin containing medications. Minimizing alcohol intake should be also emphasized to further diminished the risk of bleeding. Instruct the patients that he must avoid iron supplements, including those within multivitamins supplements Explain to avoid vigorous toweling after bathing.

Manifestations:

Breathing difficulty when lying down Dizziness Excessive bleeding Headache Itchiness Shortness of breath Fatigue

POLYCYTEMIA VERA
Polycythemia vera is a bone marrow disease that leads to an abnormal increase in the number of
blood cells (primarily red blood cells).

Risk Factors: Pathophysiology:

Manifestations: Breathing difficulty when lying down Dizziness Excessive bleeding Headache Itchiness Shortness of breath Fatigue

Nursing Management:

HEMOPHILIA
Hemophilia is a group of hereditary bleeding disorder characterized by a deficiency in a blood-clotting factor.
TYPES OF HEMOPHILIA HEMOPHILIA A This is a classic hemophilia and is the most common type;the missing clotting factor VIII HEMOPHILIA B: Christmas disease;the missing clotting factor is factor IX HEMOPHILIA C: The missing clotting factor is factor IX
PATHOPHYSIOLOGY

SIGNS AND SYMPTOMS

Defect in the intrinsic hemostatic mechanism and fibrin thread formation. Gene deletions and point mutations. Cascade of interactions involving clotting proteins, phosphor-lipids, and calcium. Blood coagulation

1. Newborn bleeding signs: 2. Nose bleeding (epistaxis) 3. Skin sign: ecchymotic patches 4. Spontaneous hematuria 5. Anemia 6. Hemarthrosis (hemorrhages in the joints causing pain, swelling and limited movement) 7.

NURSING INTERVENTIONS

HEALTH TEACHINGS
Explain how to care for, administer, store and reconstitute the replacement factor. Inform the child and family that superficial injuries are treated with ice and pressure. Teach parents how to immobilize the joint, pack it in ice, and administer replacement factor. Explain the possible side effects of therapy . Demonstrate passive ROM exercises. Emphasize avoidance of aspirin and aspirincontaining compounds. Provide diet information because weight increase can impose further stress on joints.

HEMOPHILIA
1. Monitor bleeding and maintain bleeding precautions. 2. Prepare to administer replacement factor as prescribed. 3. Monitor for joint pain, immobilize the affected extremity if joint pain occurs. 4. Assess neurological status. 5. Monitor urine for hematuria. 6. Control joint bleeding by immobilization, elevation and the application of ice, in addition apply pressure for superficial bleeding.

DIAGNOSTIC FINDINGS
low factor VIII in hemophilia A , low factor IX in hemophilia B, prolonged partial thromboplastin time impaired prothrombin consumption

MEDICATIONS

Antifibrinolytic medicines tranexamic acid aminocaproic acid Desmopressin Prophylactic

Aplastic anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells.[1] The condition, per its name, involves both aplasia and anemia. Typically, anemia refers to low red blood cell counts, but aplastic anemia patients have lower counts of all three blood cell types: red blood cells, white blood cells, and platelets, termed pancytopenia.

Exams and Tests 1. 2. Signs and Symptoms malaise, pallor and associated symptoms such as palpitations, Thrombocytopenia (low platelet counts), leading to increased risk of hemorrhage, bruising and petechiae, Leukopenia (low white blood cell count), leading to increased risk of infection and Reticulocytopenia (low reticulocyte counts). Nursing Interventions 1. 2. 3. Maintain established rapport Monitor vital signs, especially temp. Stress proper hand washing techniques by all caregivers between therapies 3. Bone marrow aspirate and biopsy History of iatrogenic exposure to cytotoxic chemotherapy X-rays, computed tomography (CT) scans, or ultrasound imaging tests: enlarged lymph nodes (sign of lymphoma), kidneys and bones in arms and hands (abnormal in Fanconi anemia) Chest X-ray: infections Liver tests: liver diseases Viral studies: viral infections Vitamin B12 and folate levels: vitamin deficiency Blood tests for paroxysmal nocturnal hemoglobinuria Test for antibodies: immune competence

4. 5. 6. 7. 8. 9.

4. Monitor visitors/ caregivers of the client. Instruct SO to limit visitors especially those with known contagious illnesses. 5. Maintain aseptic/sterile techniques as much as possible

6.Advise the use of facial mask when going outside the clients room 7. Encouraged early ambulation, deep breathing and turning exercises as

Severe pain:abdomen, joints, flank,legs

Pernicious anemia
(also known as Biermer's anemia, Addison's anemia, or AddisonBiermer anemia) one of many types of the larger family of megaloblastic anemias. It is caused by loss of gastric parietal cells, and subsequent inability to absorb vitamin B12. The loss of ability to absorb vitamin B12 is the most common cause of adult vitamin B12 deficiency.

Pathophysiology

Medication Vitamin B-12 is available for therapeutic use parenterally as either cyanocobalamin or hydroxocobalamin.[4] Both are equally useful in the treatment of vitamin B-12 deficiency, and they are nontoxic (except for rare allergic reactions). Theoretical advantages exist to using hydroxocobalamin because it is retained better in the body and is more available to cells; both chemical forms of cobalamine provide prompt correction.

Manifestations:
Fatigue, low blood pressure, rapid heart rate, high blood pressure, pallor, depression, muscle weakness, and shortness of breath Neuropathic pain Frequent diarrhea Paresthesias Jaundice Glossitis Personality or memory changes]

Health Teachings
Compliance in obtaining adequate vitamin B12 for a lifetime by injection (or possibly orally) is necessary to avoid relapse of pernicious anemia. Explain the need to increase fluid intake to prevent dehydration that results from impaired ability to properly concentrate urine. Teach the patient the potential for bleeding and hemorrhage, and instruction to prevent bleeding and discuss the need for regular

Exams and Tests

CBC Reticulocyte count Schilling test (if pernicious anemia is suspected) Serum LDH Serum vitamin B12 level and serum folate levels Esophagogastroduodenoscopy (EGD) to examine the stomach, or enteroscopy to examine the small intestine may be done. Bone marrow biopsy Physical exam

Nursing Interventions
Assess patient ability to perform ADLs Instruct client to stop activity if palpitations, chest pain, shortness of breath, weakness, or dizziness occur Monitor vital sign (Blood Pressure, pulse, and respirations) during and after activity Suggest client change position slowly; monitor for dizziness. Provide or recommend assistance with activities and ambulation as necessary, allowing client to be an active participant as much as possible.

dental examinations and explain the importance of maintaining regular bowel movements to prevent straining.

SICKLE CELL ANEMIA

SIGNS AND SYMPTOMS Severe pain:abdomen,joints,flank legs Severe chronic anemia Irritability,lethargy, listlessness High fever Anorexia,vomiting Jaundice from red blood cells destruction Enlargement of the liver and spleen(hepatosplenomegaly) Severe hemolytic anemia occurring that results from inheritance of the sickle hemoglobin gene

PATHOPHYSIOLOGY Inheritance o the sickle cell hemoglobin gene Sickle cell hemoglobin acquires a crystal like formation when exposed to low oxygen tension Low oxygen to venous blood

DIAGNOSTIC EXAMINATIONS
Hemoglobin electrophoresis-shows the presence of sickle hemoglobins

MEDICATIONS Analgesic -Morphine -Acetaminophen Hydroxyurea Arginine

NURSING INTERVENTIONS

Erythrocyte containing HBs loses its round, pliable, biconcave disk shaped and becomes deformed, rigid and sickle shaped

HEALTH TEACHING Genetic counseling Prenatal screening Screening of children Prevent sickle cell crisis by preventing hypoxia, infection, and dehydration Avoidance of activities that decrease oxygenation of the blood: -smoking -flying in unpressured airplanes -living in high altitudes -living in high altitudes

Families should be encouraged to contact community sickle cell agencies for follow-up information, new drug
protocols, and psychosocial support. Families should also follow the advances of gene therapy, bone marrow transplantation, and the usage of cord blood stem cells.

1. Institute bed rest to decrease the need for oxygen. 2. Maintain adequate hydration. 3. Administer oxygen as ordered. 4. Admister blood transfusion as ordered,for hemoglobin levels lower than 4 g/dl. 5. Assist the child to assume a comfortable position so that child keeps the extremeties extended to promote venous return. 6. Encourage consumption of a high calorie ,high protein diet, with folic acid supplementation. 7. Keep warm. 8. Administer antibiotics as prescribed to prevent infection.