ANEMIA DEFINITION ANEMIA Anemia is defined as reduction in volume of red blood cell count or in concentration of hemoglobin .

The normal level of hemoglobin is generally different in males and females CAUSATIVE FACTORS 1. Impaired production of RBC and Hb : Marrow failure a. Diamond-Blackfan anemia (congenital pure red cell aplasia) b. Transient erythroblastopenia of childhood c. Aplastic crisis caused by parvovirus B19 infection (in patients with an underlying chronic hemolytic anemia) d. Marrow replacement (eg, malignancies, myelofibrosis, osteopetrosis) Impaired erythropoietin production e. f. g. h. Anemia of chronic disease in renal failure Chronic inflammatory diseases Hypothyroidism Severe protein malnutrition

Defect in red cell maturation and ineffective erythropoiesis i. Nutritional anemia secondary to iron, folate, or vitamin B-12 deficiency j. Congenital dyserythropoietic anemia k. Thalassemias l. Erythropoietic protoporphyria 2. Accelerated destruction of blood cells Extracellular causes Mechanical injury (hemolytic-uremic syndrome, cardiac valvular defects) o Antibodies (autoimmune hemolytic anemia) o Infections, drugs, toxins o Thermal injury to RBCs (with severe burns) Intracellular causes o Red cell membrane defects (eg, hereditary spherocytosis, elliptocytosis) o Enzyme defects (eg, G-6-PD deficiency, pyruvate kinase deficiency) o Hemoglobinopathies (sickle cell disease, unstable hemoglobinopathies) o Paroxysmal nocturnal hemoglobinuria
o

3. Blood loss y Obvious or occult site of blood loss: GI tract, intra-abdominal, pulmonary, intracranial (in neonates) y Particular risk of massive hemorrhage (internal or external) for patients with bleeding disorder

y Decrease production of platelets y Increased destruction of platelets y Decreasing number of clotting factors 4. Morphologic classification 5. Physiologic anemia occurs in term infants at ages of 8 to12 weeks hematocritshould not fall below 30 % 6. May result from chronic illness such as rheumatoid arthritis and other inflammatory diseases TYPES OF ANEMIA SEEN IN CHILDREN 1. Iron deficiency anemia : Iron deficiency anemia is acondition in which total body iron content is decreased below normal , affecting hemoglobin synthesis . RBC appear pale and very small 2. 3. 4. 5. 6. Anemia of chronic lead poisioning Megaloblastic anemia Hemoglobinopathies Transient erythroblastopenia Anemia from blood loss or bone

Types of anemia Type Morphological characteristics causes Underlying pathophysiology Microcytic: Iron deficiency; chronic blood loss Microcytic; hypochromic Inadequate diet Blood loss, chronic Insufficient iron stores lead to a depleted RBC mass with subnormal hgb conc, and in turn, subnormal O2 carrying capacity of the blood Macrocytic or megaloblastic; pernicious or folic acid Macrocytic with variation in size,

shape of RBCs Inadequate diet, lack of intrinsic factor for pernicious anemia, impaired absorption Causes Vit B12 deficiency Inhibits cell growth; deformed RBCs with poor O2 carrying capacity Neuro damage occurs bec VB12 impairs myelin formation Deficiency of folic acid results in inhibits cell growth, which have shortened life span Aplastic Normocytic, normochromic RBCs, depletion of leukocytes and platelets drug toxicity, genetic failure, radiation, chemicals, infections Damage of destroyed stem cells inhibit blood cell production Hemolytic Normocytic, normochromic, inc number of reticulocytes Mechanical injury, RBC antigenantibody reaction, chemical reactions Reduced RBC survival Post hemorrhagic; acute hemorrhage

Normocytic, normochromic, inc number of reticulocytes within 48-72 h Internal or external hemorrhage Reduced circulating blood volume TYPES OF ANEMIA SEEN IN CHILDREN 1. Iron deficiency anemia : Iron deficiency anemia is acondition in which total body iron content is decreased below normal , affecting hemoglobin synthesis . RBC appear pale and very small 2. Anemia of chronic lead poisioning 3. Megaloblastic anemia 4. Hemoglobinopathies 5. Transient erythroblastopenia 6. Anemia from blood loss or bone CLINICAL MANIFESTATION Conditions may be acute or chronic , the more slowly the onset of anemia , the less likely patient will be asymptomatic Early symptoms : listenlessness. Fatigability , anorexia related to decresed energy Late symptoms : pallor, weakness , tachy cardia. Tachypnea, palpitations, jaundice. DIAGNOSTIC EVALUATION Z complete blood count ith indices and reticulocytes vary with types of anemia Z serum iron and total iron binding capacity ratio of less than 0.2 Z serum ferritin less than 12 g/dl Z lead greater than 20 g/dl Z free erythrocyte protoporyphyrin greater than 35g/dl Z B12, B6,folate level may be decreased Z Hb electrophoresis- may show Hbs or other abnormality Z Parvo virus B19 titre may be elevated in transient erythroblatopenia Z Coombs test MANAGEMENT Iron deficiency anemia 1. Oral iron at dose of 3 to 6 mg elemental iron /kg per day given between meals . reticulocyte count should increase in 7 to 10 days 2. Dietary : decrease milk intake to 16 oz per day ; include iron fortified cerals and bread products ; increase consumption of red meat ; include foods rich in vit C 3. IRON rarely given IM Anemia of chronic lead poisioning 1. Early detection of high lead levels through screening questionnaires and blood tests 2. Maintainance of a well balanced diet, high in calcium and vitamin D 3. Administration of chelating agent ethylnediaminetetraacetic acid or diamercaprol according to recommendations of the centre for disease control and prevention 4. Use lead free paints 5. Testing of hose and soil 6. Removal of child iron unsafe environment . Hemoglobinopathies 1. Sicle cell anemia

2. Thalassemia Transient erythroblastpenia of childhood 1. Spontaneous recovery in 4 to 8 wks 2. For Hb levels of less than 5 g/dl or cardiac failure , usually a transfusion of packed RBC s. Anemia from blood loss or bone marrow suppression a. Packed RBC transfusions may be necessary COMPLICATIONS mental sluggishness , as a result of decreased oxygen and energy for normal activity ; usually normal neutral activity ; usually associated with a decreased attention span , decreased intelligence, and lethargy growth retardation related to anorexia and decreased cellular metavolism delayed puberty related to growth retardation cardiac enlargement related to muscular hypertrophy because of increased strain on heart , attempting to compensate for increased oxygen demand by tissues eventually results in heart failure death related to circulatory collapse and shock. NURSING ASSESSMENT Obtain history of potential causes a. dietary history b. family history c. persistent infection, fever d. exposure to drugs , poisions e. pica craving and consuming nonfood items Obtain a baseline assessment a. observe skin and mucous membrane b. observe height and weight and plot on growth curve c. measure vital signs , including blood pressure d. assess child s functional level level of exercise tolerated , mental functioning e. assess attainment of development Observe for fatigue , listenless , irritability Observe for blood loss :bruising , bleeding , hematuria or hematochezia NURSING DIAGNOSIS 1. Fatigue related to decreased ability of blood to transport oxygen to the tissues 2. Imbalanced nutrition : less than the body requirement of recommended daily dietary allowances 3. Risk for infection related to debilitated state 4. anxiety related to hospitalization and painful diagnostic procedures . 5. delayed growth and development NURSING INTERVENTIONS MINIMISE FATIGUE Plan nursing care Observe early signs of fatigue Encourage rest Administer oxygen in upright position Provide finger foods Transfuse packed RBC s as directed NUTRITION Small and frequent feed Iron rich foods and vitamins Give meals with orange juice ( iron better aborbed in acidic) Limit milk to 16 to 24 oz Administer liquid iron with dropper to prevent teeth staining

 Dental stains can be removed by brushing with sodium bicarbonate Be alert for adverse effects of iron supplements gastric distress , colicky pain , diarrhea Advice family that childs stool may turn black or green Avoid tiring activity PREVENT INFECTION Good hygienic practices Avoid exposure to cold and infections Report temperature elevation REDUCES ANXIETY explain procedure allow child to handle equipment used for test and procedure PROMOTE GROWTH AND DEVELOPMENT Age related activities Peer socialization Age appropriate play Periodic growth chart evaluation and developmental testing FAMILY EDUCATION AND HEALTH MAINTAINANCE Stress to the parents on continuing iron therapy Initiate and reinforce good dietary habits Discuss general health measures , including adequate rest , diet , sunshine

Type Microcytic: Iron deficiency; chronic blood loss

Morphological characteristics Microcytic; hypochromic

causes Inadequate diet Blood loss, chronic Inadequate diet, lack of intrinsic factor for pernicious anemia, impaired absorption Causes

Underlying pathophysiology Insufficient iron stores lead to a depleted RBC mass with subnormal hgb conc, and in turn, subnormal O2 carrying capacity of the blood Vit B12 deficiency Inhibits cell growth; deformed RBCs with poor O2 carrying capacity Neuro damage occurs bec VB12 impairs myelin formation Deficiency of folic acid results in inhibits cell growth, which have shortened life span Damage of destroyed stem cells inhibit blood cell production

Macrocytic or megaloblastic; pernicious or folic acid

Macrocytic with variation in size, shape of RBCs

Aplastic

Hemolytic

Normocytic, normochromic RBCs, depletion of leukocytes and platelets Normocytic, normochromic, inc number of reticulocytes Normocytic, normochromic, inc number of reticulocytes within 48-72 h

drug toxicity, genetic failure, radiation, chemicals, infections Mechanical injury, RBC antigen- antibody reaction, chemical reactions Internal or external hemorrhage

Reduced RBC survival

Post hemorrhagic; acute hemorrhage

Reduced circulating blood volume

SICKLE-CELL DISEASE OVERVIEW Sickle-cell disease is a general term for a group of genetic disorders caused by sickle hemoglobin (Hgb S or Hb S). Erythrocytes become elongated andcrescent shaped (sickled) removed from the circulation and destroyed atincreased rates, leading to anemia An autosomal recessive inherited defect The disease ischronic and lifelong. Lifespan is oftenshortened within sufferers living to an average of 40 years. The polymerization of deoxygenated HbS is the primary indispensable event in the molecular pathogenesis of sickle cell disease HbSpolymerization is associated with increased red cell density (dense erythrocytes) as well as red cell membrane damage favoring the generation of distorted rigid sickle cells and contributing to vaso-occlusion and premature red cell destruction (hemolytic anemia). The gene defect is a known mutation of a single nucleotide polymorphism (SNP) (A to T) of the -globin gene, which results inglutamic acid to be substituted by valine at position 6. GAG to GUG codon mutation = LEADINGTO HbS FORMATION Fetal hemoglobin contains a gamma, not a beta chain, the disease usually will not result in clinical symptoms until the childs hemoglobin changes from the fetal to the adult form at approximately 6 months.

SICKLE-CELL TRAIT Both parents with the disease will have both normal adult and hemoglobin S and be carriers (heterozygous) of the sickle- cell trait. heterozygous form of disease in w/c the affected individual has both normal (Hb A) & sickle (Hb S) hemoglobin 25% - 50% of hemoglobin is abnormal. No symptoms SICKLE CELL ANEMIA- also known as hemoglobin SS disease, is the homozygous form in which the affected individual has predominantly sickle hemoglobin (Hb SS). CHARACTERISTICS OF SICKLED CELLS Signs and symptoms of sickle cell anemia usually show up after an infant is 4 months old and may include: Anemia. Sickle cells are fragile. They break apart easily and die, leaving you chronically short on red blood cells. Red blood cells usually live for about 120 days before they die and need to be replaced. However, sickle cells die after only 10 to 20 days. The result is a chronic shortage of red blood cells, known as anemia. Without enough red blood cells in circulation, your body can't get the oxygen it needs to feel energized. That's why anemia causes fatigue. Episodes of pain. (Vaso-occlusive) Periodic episodes of pain, called crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. Pain can also occur in your bones. The pain may vary in intensity and can last for a few hours to a few weeks. Some people experience only a few episodes of pain. Others experience a dozen or more crises a year. If a crisis is severe enough, you may need hospitalization so that pain medication can be injected into your veins (intravenously). Hand-foot syndrome. Swollen hands and feet may be the first signs of sickle cell anemia in babies. The swelling is caused by sickle-shaped red blood cells blocking blood flow out of their hands and feet. Jaundice. Jaundice is a yellowing of the skin and eyes that occurs because of liver damage or dysfunction. Occasionally, people who have sickle cell anemia have some degree of jaundice because the liver, which filters harmful substances from the blood, is overwhelmed by the rapid breakdown of red blood cells. In people with dark skin, jaundice is visible mostly as yellowing of the whites of the eyes. Frequent infections. Sickle cells can damage your spleen, an organ that fights infection. This may make you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia antibiotics to prevent potentially life-threatening infections, such as pneumonia. Acute splenic sequestration or pooling of blood The spleen is an organ in the abdomen that filters out abnormal red blood cells and helps fight infection. Sometimes, the spleen traps many cells that should be in the bloodstream and it grows large. This causes anemia. Blood transfusions may be needed until the body can make more cells and recover. If the spleen becomes too clogged with sickle cells, it cant work normally. It begins to shrink and stop working. Delayed growth. Red blood cells provide your body with the oxygen and nutrients you need for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers. Vision problems. Some people with sickle cell anemia experience vision problems. Tiny blood vessels that supply your eyes may become plugged with sickle cells. This can damage the retina the portion of the eye that processes visual images. Cerebrovascular accident. blockage of major blood vessels-Sickle-shaped red blood cells may stick to the walls of the tiny blood vessels in the brain. This can cause a stroke. This type of stroke occurs mainly in children. The stroke can cause learning disabilities or more severe problems.

Acute Chest syndrome. similar to pneumonia-Acute chest syndrome is a life-threatening condition linked to sickle cell anemia. It's similar to pneumonia and is caused by an infection or by sickle cells trapped in the lungs. People with this condition usually have chest pain, fever, and an abnormal chest x ray. Over time, lung damage may lead to pulmonary arterial hypertension Priapism. Males with sickle cell anemia may have painful and unwanted erections called priapism (PRI-a-pizm). This happens because the sickle cells stop blood flow out of an erect penis. Over time, priapism can damage the penis and lead to impotence. Ulcers on the Legs. Sickle cell ulcers (sores) usually begin as small, raised, crusted sores on the lower third of the leg. Leg sores occur more often in males than in females and usually appear between the ages of 10 and 50. The cause of leg ulcers is not clear. The number of ulcers can vary from one to many. Some heal rapidly, but others persist for years or come back after healing. Multiple Organ Failure. Multiple organ failure is rare, but serious. It happens when a person has a sickle cell crisis that causes two out of three major organs (lungs, liver, or kidney) to fail. Symptoms linked to this complication are a fever and changes in mental status such as sudden tiredness and loss of interest in your surroundings AT RISK FOR SICKLE CELL ANEMIA most common in people whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia. DIAGNOSTIC TESTS Can be diagnose prenatally by chorionic villi sampling or from cord blood during amniocentesis Routine SCA screening of newborns Abnormal hemoglobin forms are detected on hemoglobin electrophoresis, a form of gel electrophoresis on which the various types of hemoglobin move at varying speed Treatments and drugs Bone marrow transplant offers the only potential cure for sickle cell anemia. But, finding a donor is difficult and the procedure has serious risks associated with it, including death. Bone marrow transplantation (BMT) is a special therapy for patients with cancer or other diseases which affect the bone marrow. A bone marrow transplant involves taking cells that are normally found in the bone marrow (stem cells), filtering those cells, and giving them back either to the patient or to another person. The goal of BMT is to transfuse healthy bone marrow cells into a person after their own unhealthy bone marrow has been eliminated. MEDICATIONS Treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications: Antibiotics. Children with sickle cell anemia usually begin taking the antibiotic penicillin when they're about 2 months of age and continue taking it until they're 5 years old. Doing so helps prevent infections, such as pneumonia, which can be life-threatening to an infant or child with sickle cell anemia. Antibiotics may also help adults with sickle cell anemia fight certain infections. Pain-relieving medications. To relieve pain during a sickle crisis, your doctor may advise over-thecounter pain relievers and application of heat to the affected area. You may also need stronger prescription pain medication. Blood transfusions. Blood transfusions carry some risk. Blood contains iron. Regular blood transfusions cause an excess amount of iron to build up in your body. Because excess iron can damage your heart, liver and other organs, people who undergo regular transfusions must often receive treatment to reduce iron levels. Deferasirox (Exjade) is an oral medication that can reduce excess iron levels. It can be used in people older than 2. Supplemental oxygen Breathing supplemental oxygen through a breathing mask adds oxygen to your blood and helps you

breathe easier. It may be helpful if you have acute chest syndrome or a sickle cell crisis. Hydroxyurea (Droxia, Hydrea). This prescription drug, normally used to treat cancer, may be helpful for adults with severe disease. When taken daily, it reduces the frequency of painful crises and may reduce the need for blood transfusions. It seems to work by stimulating production of fetal hemoglobin a type of hemoglobin found in newborns that helps prevent the formation of sickle cells. There is some concern about the possibility that long-term use of this drug may cause tumors or leukemia in certain people. EXPERIMENTAL TREATMENTS 1. Gene therapy. Because sickle cell anemia is caused by a defective gene, researchers are exploring whether inserting a normal gene into the bone marrow of people with sickle cell anemia will result in the production of normal hemoglobin. Scientists are also exploring the possibility of turning off the defective gene while reactivating another gene responsible for the production of fetal hemoglobin a type of hemoglobin found in newborns that prevents sickle cells from forming. 2. Butyric acid. Normally used as a food additive, butyric acid may increase the amount of fetal hemoglobin in the blood. 3. Clotrimazole. This over-the-counter antifungal medication helps prevent a loss of water from red blood cells, which may reduce the number of sickle cells that form. 4. Nitric oxide. Sickle cell anemia causes low levels of nitric oxide, a gas that helps keep blood vessels open and reduces the stickiness of red blood cells. Treatment with nitric oxide may prevent sickle cells from clumping together. 5. Nicosan. This is an herbal treatment in early trials in the U.S. Nicosan has been used to prevent sickle crises in Nigeria. 6. Decitadine. This medicine increases hemoglobin F levels (this type of hemoglobin carries more oxygen). It may be a good choice instead of hydroxyurea. LIFESTYLE AND HOME REMEDIES Take folic acid supplements daily, and eat a balanced diet. Bone marrow needs folic acid and other vitamins to make new red blood cells. Drink plenty of water. Staying hydrated helps keep your blood diluted, which reduces the chance that sickle cells will form. Avoid temperature extremes. Exposure to extreme heat or cold can trigger the formation of sickle cells. Reduce stress. A sickle crisis can occur as a result of stress. Exercise regularly, but don't overdo it. Talk with your doctor about how much exercise is right for you. Use over-the-counter medications with caution. Some medications, such as the decongestant pseudoephedrine, can constrict your blood vessels and make it harder for the sickle cells to move through freely. Fly on airplanes with pressurized cabins. Unpressurized aircraft cabins may not provide enough oxygen. Low oxygen levels can trigger a sickle crisis. Additionally, be sure to drink extra water when traveling by air, as pressurized cabins can be dehydrating. Avoid high-altitude areas. Traveling to a high-altitude area may also trigger a crisis because of lower oxygen levels.
CLINICAL MANIFESTATION Conditions may be acute or chronic , the more slowly the onset of anemia , the less likely patient will be asymptomatic Early symptoms : listenlessness. Fatigability , anorexia related to decresed energy Late symptoms : pallor, weakness , tachy cardia. Tachypnea, palpitations, jaundice.

DIAGNOSTIC EVALUATION          complete blood count ith indices and reticulocytes vary with types of anemia serum iron and total iron binding capacity ratio of less than 0.2 serum ferritin less than 12 g/dl lead greater than 20 g/dl free erythrocyte protoporyphyrin greater than 35g/dl B12, B6,folate level may be decreased Hb electrophoresis- may show Hbs or other abnormality Parvo virus B19 titre may be elevated in transient erythroblatopenia Coombs test

MANAGEMENT Iron deficiency anemia 1. Oral iron at dose of 3 to 6 mg elemental iron /kg per day given between meals . reticulocyte count should increase in 7 to 10 days 2. Dietary : decrease milk intake to 16 oz per day ; include iron fortified cerals and bread products ; increase consumption of red meat ; include foods rich in vit C 3. IRON rarely given IM Anemia of chronic lead poisioning 1. Early detection of high lead levels through screening questionnaires and blood tests 2. Maintainance of a well balanced diet, high in calcium and vitamin D 3. Administration of chelating agent ethylnediaminetetraacetic acid or diamercaprol according to recommendations of the centre for disease control and prevention 4. Use lead free paints 5. Testing of hose and soil 6. Removal of child iron unsafe environment . Hemoglobinopathies 1. Sicle cell anemia 2. Thalassemia Transient erythroblastpenia of childhood 1. Spontaneous recovery in 4 to 8 wks 2. For Hb levels of less than 5 g/dl or cardiac failure , usually a transfusion of packed RBC s. Anemia from blood loss or bone marrow suppression a. Packed RBC transfusions may be necessary COMPLICATIONS  mental sluggishness , as a result of decreased oxygen and energy for normal activity ; usually normal neutral activity ; usually associated with a decreased attention span , decreased intelligence, and lethargy  growth retardation related to anorexia and decreased cellular metavolism  delayed puberty related to growth retardation  cardiac enlargement related to muscular hypertrophy because of increased strain on heart , attempting to compensate for increased oxygen demand by tissues eventually results in heart failure

 death related to circulatory collapse and shock.  NURSING ASSESSMENT Obtain history of potential causes a. dietary history b. family history c. persistent infection, fever d. exposure to drugs , poisions e. pica craving and consuming nonfood items Obtain a baseline assessment a. b. c. d. e. observe skin and mucous membrane observe height and weight and plot on growth curve measure vital signs , including blood pressure assess child s functional level level of exercise tolerated , mental functioning assess attainment of development

Observe for fatigue , listenless , irritability Observe for blood loss :bruising , bleeding , hematuria or hematochezia NURSING DIAGNOSIS 1. Fatigue related to decreased ability of blood to transport oxygen to the tissues 2. Imbalanced nutrition : less than the body requirement of recommended daily dietary allowances 3. Risk for infection related to debilitated state 4. anxiety related to hospitalization and painful diagnostic procedures . 5. delayed growth and development NURSING INTERVENTIONS MINIMISE FATIGUE y y y y y y Plan nursing care Observe early signs of fatigue Encourage rest Administer oxygen in upright position Provide finger foods Transfuse packed RBC s as directed

NUTRITION      Small and frequent feed Iron rich foods and vitamins Give meals with orange juice ( iron better aborbed in acidic) Limit milk to 16 to 24 oz Administer liquid iron with dropper to prevent teeth staining

   

Dental stains can be removed by brushing with sodium bicarbonate Be alert for adverse effects of iron supplements gastric distress , colicky pain , diarrhea Advice family that childs stool may turn black or green Avoid tiring activity

PREVENT INFECTION Good hygienic practices Avoid exposure to cold and infections Report temperature elevation REDUCES ANXIETY  explain procedure  allow child to handle equipment used for test and procedure PROMOTE GROWTH AND DEVELOPMENT     Age related activities Peer socialization Age appropriate play Periodic growth chart evaluation and developmental testing

FAMILY EDUCATION AND HEALTH MAINTAINANCE  Stress to the parents on continuing iron therapy  Initiate and reinforce good dietary habits  Discuss general health measures , including adequate rest , diet , sunshine