MINDANAO STATE UNIVERSITY Buug Campus Ipil Extension Sanito, Ipil, Zamboanga Sibugay Province

In partial fulfillment of the requirements In

PEDIA ABNORMAL
Presented to the faculty of MSU- CHS

Ms. Tashmera P. Datukali

INSTRUCTOR

Presented By:

Alona Beth P. Datukali February 10, 2012

DATE SUBMITTED:

TOPIC CONTENT
I. ANEMIA DESCRIPTION Anemia is a clinical condition that results from an insufficient supply of healthy red blood cells, the volume of packed RBCs, and/or the quantity of hemoglobin. Hypoxia results because the bodys tissues are not adequately oxygenated. Not a disease itself, anemia reflects a number of underlying pathologic processes leading to an abnormality in RBC number, structure, or function.

II.

RISK FACTORS, ETIOLOGY, AND CLASSIFICATION Anemia can arise from primary hematologic problems or can occur as secondary consequence of defects in other body system. The prevalence of anemia increases with age , adults 65 and older are at risk. Aging cannot be assumed to be the cause of anemia. Anemias are classified by either the etiology or the morphology of the specific anemia. Anemia is caused in one of three ways: 1)decrease production of RBCs, 2) increased RBC destruction(hemolysis), or 3) loss of blood.

DECREASE RBC PRODUCTION DEFECTIVE DNA SYNTHESIS DECREASE HEMOGLOBIN SYNTHESIS Cobalamin/vitamin B12 deficiency Folic acid deficiency Iron deficiency Thalassemia Sideroblastic anemia Aplastic anemia Anemia of leukemia and myelodysplasia Chronic diseases

DECREASED NUMBER OF ERYTHROCYTE PRECURSORS

INCREASED RBC PRODUCTION INTRINSIC Abnormal hemoglobin Enzyme deficiency Membrane abnormalities Physical trauma Antibodies Infectious agents Toxins BLOOD LOSS Trauma Blood vessel rupture Gastritis Hemorrhoids Menstruation

EXTRINSIC

ACUTE CHRONIC

III.

TYPES OF ANEMIA

1. IRON DEFICIENCY ANEMIA DESCRIPTION IDA is caused by an inadequate supply of iron for normal RBC formation. Is a chronic, hypochromic, microcytic anemia resulting from an insufficient supply of iron in the body . Without iron, hemoglobin concentrations in the RBCs is reduced and the cells are unable to oxygenate the bodys tissues adequately, resulting in anemia. RISK FACTORS Prominent in age-groups that experience rapid growth like the toddlers and adolescents. In children, it typically occurs between ages 6 months and 3 years. Older adults more than 65 years of age is also at risk for IDA. Pregnant and breastfeeding women. ETIOLOGY Inadequate dietary iron intake Iron malabsorption Low iron stores at birth Significant blood loss Excessive demands for iron required for growth.

PATHOPHYSIOLOGY STAGE 1: characterized by depletion of hemosiderin, ferritin, and other iron storage Complete blood count compounds in the bone marrow, liver and spleen. STAGE 2: characterized by lack of transport iron resulting in decrease iron saturation transferrin. STAGE 3: characterized by marked deficit in transport iron, inhibiting the normal production of hemoglobin. Erythrocyte protoporphyrin increases, and transferrin receptors become more numerous in response to the iron poor environment.

SIGNS AND SYMPTOMS S/S CHANGE

Dyspnea on exertion, fatigue, pallor, inability to concentrate, irritability, headache, susceptibility to infection Increased cardiac output and tachycardia Nails that are coarsely ridge, spoon-shaped (koilonychia), brittle Sore, red, burning tongue Sore, dry skin at corners of the mouth

Decreased oxygen-carrying capacity of blood caused by decreased hemoglobin levels. Decreased oxygen perfusion Decreased capillary circulation Papillae atrophy Epithelial changes.

LABORATORY AND DIAGNOSTIC STUDY FINDINGS CBC will reveal normal to slightly reduced RBCs, low hemoglobin and hematocrit, reduced mean corpuscular volume and reduced hemoglobin. Erythrocytes protoporphyrin level will be greater than 35. Iron tests will reveal low serum iron capacity, decreased serum ferritin level, and elevated total iron binding capacity. Reticulocyte count may be obtained 10 days after therapy is initiated to evaluate its effectiveness. NURSING MANAGEMENT Assess for fatigue, activity intolerance, and other signs of impaired tissue oxygenation. Emphasized proper administration of oral iron supplements like: 1. give the supplements into 2 to 3 divided doses in a small amount of vitamin c containing liquid between meals to enhance absorption and minimize adverse effects. 2. administer iron with a dropper to an infant or through a straw to an older child. 3. brush the child's teeth after administration to minimize staining. Explain the potential adverse effect of iron, which includes nausea and vomiting, diarrhea and constipation, dark green or black stools and tooth discoloration. Discuss measures for preventing infections, including good hygiene, proper nutrition and adequate rest. Promote an adequate intake of iron-rich foods.

2. SICKLE CELL DISEASE DESCRIPTION It is a lifelong condition that manifests in the first year and persist throughout ones lifespan. Is a group of chronic, severe, genetic, hemolytic diseases associated with Hb which transforms RBCs into a sickle shape when blood oxygenation is decreased. The common SCD is the sickle cell anemia which is an autosomal recessive disorder in which the person is homozygous for Hbs. Unlike normal oval cells, fragile sickle cells can't hold enough hemoglobin to nourish body tissues. Sickle cell trait is the carrier of the disorder. The child may experience periodic, painful attacks called sickle cell crises. ETIOLOGY AND RISK FACTORS SCD is found in races of people from areas of the world where malaria is endemic, including Africa, Mediterranean, Middle East, and India. It is the most common form of anemia worldwide; there are more than 200 million carriers of sickle cell trait worldwide. Whether an individual will have SCA, SCT it will still depends on the Hb genes inherited from each parents.

PATHOPHYSIOLOGY Abnormal Hb S replaces all or part of normal Hb A; under conditions of increased oxygen tension and lowered Ph, RBCs change from round to sickle or crescent shaped. SCD do not slide through vessels as normal cells do. Their angled shape causes clumping, thrombosis, arterial obstruction, increased blood viscosity, hemolysis, and eventually tissue ischemia and necrosis. As sickling progresses, acute and chronic changes develop in various organs and structures.
ORGAN MANIFESTATION IN SICKLE CELL CNS CARDIAC PULMONARY RENAL SPLEEN HEPATIC Thrombosis, cerebral deficits or death Systolic murmur, cardiomegaly, heart failure Hematuria, renal failure Splenomegaly, splenic atrophy Hepatomegaly, gallstones

SKELETAL GENITAL OPTISC DERMIS

Osteomyelitis, osteoporosis Penile priapism Hemorrhage, retinal detachment, retinopathy, blindness Stasis ulcers of extremities

ASSESSMENT FINDINGS o o INFANTS Colic from pain Dactylitis or hand foot syndrome. Splenomegaly TODDLERS AND PRESCHOOLERS Hypovolemia and shock SCHOOL-AGE AND ADOLESCENTS Poor healing of leg wounds from inadequate peripheral circulation of oxygenated blood.

NURSING MANAGEMENT Promote tissue oxygenation Administer appropriate therapeutic measures Provide oral and I.V hydration fluids to increase the fluid volume of blood and to help prevent sickling and thrombosis. Administer electrolyte replacement to counter acidosis caused by hypoxia. Deliver oxygen therapy to promote adequate oxygenation. Institute bed rest and careful organization of the childs activities to minimize energy expenditure. Administer and monitor transfusion to treat anemia and reduce the viscosity of blood. o Relieve pain Provide an around-the-clock regimen of preventive medications. Avoid administration of meperidine(DEMEROL) because of the increased risk of seizures. Reassure the family and child that an analgesic even high doses of an opioid is indicated and the addiction is rare. Apply heat to affected areas; avoid cold compresses, which will enhance vasoconstriction and sickling. Position the child for maximum comfort. Help ensure adequate hydration and nutritionally balanced diet. Prevent infection

Support the child and the family by allowing them to verbalize their fears, concerns, anger and other feelings.

3. APLASTIC ANEMIA DESCRIPTION Is characterized by pancytopenia and bone marrow hypoplasia. ETIOLOGY May be primary or secondary Primary types includes: Fanconi syndrome- is inherited as an autosomal and is associated with cytopenia and multiple congenital anomalies. Blackfan-Diamond syndrome- a rare condition that is characterize by destruction of RBCs and slight decrease in WBCs and platelets Common cause of acquired aplastic anemia include: Idiopathic Radiation therapy Drugs, such as chloramphenicol, methicillin, sulfonamides, thizides and chemotherapeutic agents. Toxic agents such as industrial and household chemicals, including dyes, glue, paint remover, insecticides, petroleum products and benzenes. Infections particularly hepatitis and sepsis. Infiltration and replacement of myeloid tissue Hemolytic deficiencies such as SCD Autoimmune or allergic states.

o o o o o

PATHOPHYSIOLOGY In aplastic anemia, the decreased functional capacity of hypoplastic bone marrow results in pancytopenia. In severe pancytopenia can produce massive bleeding or infection. CLINICAL MINEFESTATION LACK OF RED BLOOD CELLS Pallor Lethargy Tachycardia Shortness of breath on exertion LACK OF WHITE BLOOD CELLS Recurrent infection including opportunistic infections

LACK OF PLATELETS Abnormal bleeding Petechiae and bruising. FANCONY SYNDROME CHILDRENS MAY HAVE Malformed kidneys and heart Microcephaly, micropthalmus, dark pigmentation with spots. NURSING MANAGEMENT Prevent infection Assess for abnormal bleeding tendencies and blood products. Administer prescribed medication and blood products. Provide supportive treatment to prevent or control infection; administer transfusion and steroid or hormone therapy. Monitor for complications of steroid therapy. monitor for complication of androgen therapy signaled by abnormal liver function test results, weight gain, acne, increase hair growth, and deepening of the voice. Monitor for complication of ALG or ATG therapy, which include fever, chills, rash, serum sickness, severe thrombocytopenia, and anaphylaxis. Attempt to eliminate toxic agents from the child's environment. Support the family in coping with the procedures and the uncertain prognosis and potential outcome of aplastic anemia.

4. THALASSEMIA DESCRIPTION Is a group of inherited blood disorders characterized by a deficient synthesis of specific globulin chains of the hemoglobin molecule. It occurs in three major forms thalassemia major, thalassemia intermedia, thalassemia minor. ETIOLOGY AND RISK FACTORS Thalassemia is an autosomal disorder. Frequently found in Mediterranean, African, and Southeast Asian origin. Those who inherit both beta-genes (homozygote) have thalassemia major, which results in life threatening anemia.

PATHOPHYSIOLOGY There is a deficit in the production of specific globulin chains in the hemoglobin. Which results in a compensatory increase in hemoglobin production among other hemoglobin chains, which then become unbalanced, disintegrate and destroy red blood cells.

Compensatory increases in erythropoiesis cannot correct the severe anemia.

CLINICAL MANIFESTATION a. Thalassemia minor commonly produces only mild to moderate anemia that may be asymptomatic and commonly goes undetected. b. Thalassemia major commonly produces clinical manifestation around 6 months of age, after protective effect of hemoglobin F diminishes. EARLY SIGNS Anemia Unexplained fever Poor feeding Poor weight gain Markedly enlarged spleen LATE SIGNS Chronic hypoxia Damage to liver, spleen, heart, pancreas, and lymph glands from hemochromatosis. Slight jaundice or bronze skin color Thick cranial bones with prominent cheeks and a flat nose. Growth retardation Delayed sexual development LONG TERM COMPLICATIONS Results from hemochromatosis with resultant cellular damage leading to: Splenomegaly Skeletal complications Cardiac complications Gallbladder disease Liver enlargement and subsequent cirrhosis Skin changes Growth retardation and endocrine complications.

NURSING MANAGEMENT Assess for manifestations and complications of the disorder. Administer BT, and observed for complications of transfusions; the most common problem is iron overload. S/S includes: abdominal pain, bloody diarrhea, emesis, decrease level of consciousness, shock, metabolic acidosis. Implement iron chelation therapy with desferoxamine, as prescribed, to eliminate excess iron and its adverse effects from deposition in tissues. a. Administered during BT b. Monitor V/S

c. d. e. a. a. b. c. d. e.

Be prepared for an allergic reaction Check visual acuity and hearing Monitor I and O. Provide information and follow-up care if the client requires a splenectomy. Explain that the child should avoid people with active infection. Prepare for bone marrow transplant if needed. Prevent infection Prevent fractures Promote adequate rest by coordinating care Decrease dietary iron supplement. Help the child to cope with the illness by allowing him to verbalize concerns. Support the family Provide child and family health teaching. Discuss the nature of the disease and its management Identify S/S of infection, iron overload Provide instruction for home chelation therapy Review activity restrictions, including avoidance of activities that increase the risk of fractures. Outline dietary restrictions.

TOPIC OUTLINE
I. II. III. ANEMIA DESCRIPTION RISK FACTORS, ETIOLOGY AND CLASSIFICATION TYPES OF ANEMIA 1. IRON DEFICIENCY ANEMIA DESCRIPTION RISK FACTORS ETIOLOGY PATHOPHYSIOLOGY LABORATORY AND DIAGNOSTIC FINDINGS SIGNS AND SYMPTOMS NURSING MANAGEMENT 2. SICKE CELL ANEMIA DESCRIPTION ETIOLOGY AND RISK FACTORS PATHOPHYSIOLOGY ASSESSMENT FINDINGS NURSING MANAGEMENT 3. APLASTIC ANEMIA DESCRIPTION ETIOLOGY PATHOPHYSIOLOGY CLINICAL MANIFESTATION NURSING MANAGEMENT 4. DESCRIPTION ETIOLOGY PATHOPHYSIOLOGY CLINICAL MANIFESTATION EARLY SIGNS LATER SIGNS LONG TERM COMPLICATIONS NURSING MANAGEMENT