UNIT XI Nursing Care Management of Children with Hematologic Disorders Objectives: 1.

be able to describe the major hematologic disorders of childhood 2. be able to assess a child with hematologic disorders 3. be able to formulate nursing diagnosis and interventions for a child with hematologic disorders 4. be able to plan and implement nursing interventions for the child with hematologic disorders 5. be able to evaluate expected outcomes for the child with hematologic disorders Topic and Contents: 1. Structure and Function of the Blood (Pillitteri 13821384) 1.1. Blood formation and Components Blood cell formation begins in the fetal yolk sac as early as 2 weeks intrauterine life By 2 months, the liver and spleen begin forming blood components By 4 months, the bone marrow becomes and remains the active center for the origination of blood cells. Spleen serves for the normal destruction of RBS once their normal life span has passed. Total blood volume in the body is proportional to body weight Blood Plasma is the liquid portion of the blood

Copyright 2003 Pearson Education, Inc. publishing as Benjamin Cummings

1.1.1.

Erythrocytes (RBC) function chiefly to transport oxygen to and carry carbon dioxide from the body formed under the stimulation of erythropoietin (a hormone produced by the kidneys and stimulated when the child has tissue hypoxia) RBC form first as erythroblasts, then mature through normoblast and reticulocyte stages then as mature RBC. has a life span of 120 days because it lacks a nucleus RBC are normally destroyed through phagocytosis by reticuloendothelial cells found in the highest portion of the spleen. Long bones in infants are filled with red marrow for RBC formation Blood production in infants is in the long bones Red marrow is in long bones are replaced with yellow marrow during early childhood RBC production from early childhood is carried to the ribs, scapulae, vertebrae and skull bones. at birth infants normally has 5 million RBCs per cubic millimeter of blood which decreases rapidly in the first months By 3-4 months RBC level is approximately 4.1 million per cubic millimeter of blood. By adolescents it reaches adult value by rising slowly to 4.9 million per cubic millimeter of blood

Hemoglobin the component of RBC which allows them to carry oxygen composed of globin (dependent on nitrogen metabolism for formation) and heme an iron containing pigment

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deficiency of either iron pr nitrogen can interfere with the synthesis of hemoglobin fetal hemoglobin (hemoglobin F) is replaced by adult (hemoglobin A) by 6 months of life hemoglobin level in blood depends on the amount of RBC present Hemoglobin levels is highest at birth (13.7-20.1 g/100ml) by 3 months they reach a low of 9.5-14.5 g/100ml By puberty adult levels are reached (11-16 g/100ml)

Bilirubin After the destruction of RBC in the spleen its protein component are stored in the liver and spleen for future use. Iron will be released to be reused by the bone marrows for new RBC production Heme is converted to protoporphyrin which will be then converted to indirect bilirubin. Indirect bilirubin is fat soluble and needs to be converted by the liver enzyme glucoronyl transferase for it to be excreted as bile as direct bilirubin. liver function is immature infants that the conversion of direct bilirubin cannot be made, therefore indirect bilirubins level rises up to 7mg/100ml it then permeates to the circulatory system (jaundice) excessive hemolysis can also be manifested as jaundice

Copyright 2003 Pearson Education, Inc. publishing as Benjamin Cummings 1.1.2. Leukocytes (WBC) nucleated cells they are fewer compared to RBCs (1 WBC per 500 RBC) their primary function is defense against antigen invasion Two main forms include granulocytes and agranulocytes. granulocytes include: Agranulocytes include: typical WBC count is 5000-10000-cells per cubic millimeter of blood In newborns By 4 years old adult level of granulocytes is reached at 5000-10,000 cells/mm3 granulocytes are the most common WBC WBC are produced in response to need their life span is from 6 hours to unknown intervals

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1.1.3.

Thrombocytes (Platelets) round nonnucleated bodies formed by the bone marrow their may function is for capillary hemostasis and primary coagulation normal range is 1500,000-300,000 per cubic millimeter immature thrombocytes are termed megakaryocytes

1.2. Coagulation a complex series of events including a combination of blood and tissue factors released from the plasma When a blood vessel is injured, vasoconstriction occurs in the area proximal to the injury. Platelets will then adhere to the site and to one another (first stage of blood clotting) Second stage includes: both factors from intrinsic or extrinsic systems combine with platelet phospholipids to form a complete thromboplastin Third stage, thromboplastin converts prothrombin (factor II) to thrombin if ionized calcium id present production of prothrombin and factors VII, IX and X depends on vitamin K stage 3 will be incomplete if any levels of factors VIII through XII, vit. K or calcium are deficient fourth stage, thrombin converts fibrinogen (factor I) to fibrin fibrin strands to form a mesh that forms a permanent protective seal at the site of injury Factor XIII acts to make the clot insoluble and permanent To prevent too much coagulation after the seal is complete, plasminogen is then converted to plasmin near the injury to halt clot formation

Vascular damage

Vascular Spasm

Platelet Plug Formation

Coagulation

2. Assessment and Therapeutic Techniques for Hematologic Disorders (Pillitteri pp.13841837) 2.1. Bone Marrow Aspiration provides samples of bone marrow so that the type and quantity of cells can be determined aspiration sites in children the iliac crest or spines (have larger marrow compartments). In neonates the anterior tibia can be used. For a bone marrow aspiration, a child lies prone on a treatment table

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 conscious sedation will be used to help reduce the childs fear (Will be discussed further in skills laboratory) 2.2. Blood Transfusion used as treatment for many disorders There are a wide variety of forms that can be transfused: Whole Blood Packed RBC Washed RBC Plasma Plasma Factors Platelets WBC Albumin Blood must be infused with a solution as isotonic as possible(normal saline) If blood is given with a hypertonic solution, fluid will be drawn out of the blood causing them to shrink If blood will be infused with a hypotonic solution, fluid will be drawn into the cell and they will burst Packed RBC is the most common form of transfusion in children (minimizes the risk of fluid overload) An infusion of packed RBC can raise the level of hematocrit to 5 points An infusion of platelets will elevate the platelet count by 10,000. Platelets usually last for 10 days, every 10 days we will need to transfuse platelets. Always ensure that a signed consent had been obtained to respect socio-cultural or religious beliefs Obtain baseline vital signs before starting the transfusion. Vital signs monitoring q 15 minutes for the first hour and every half hour for the remainder of the transfusion. (to be discussed further in skills laboratory) 2.3. Stem Cell Transplant the intravenous infusion of hematopoetic stem cells from bone marrow donors are compatible if their Human leukocyte antigen (HLA) system matches that of the recipient Stem cell transplant had become a relatively common procedure for children with blood disorders such as: Stem cell transplantation is more effective if the recipient has not already received multiple blood transfusions (BT causes sensitization to blood products) Types of Stem cell transplantation: Allogenic Transplantation, involves the transfer of from an immune-compatible donor Synergenic Transplantation, transplant where donor and recipient are genetically identical (identical twins) Autologous transplant, the childs own stem cells will be used. There is no guarantee that the graft will be accepted or that improvement will occur (to be discussed further in skills laboratory) 2.4. Splenectomy removal of the spleen to prevent destruction of RBC for patients with sickle-cell anemia and thalassemias done to reduce RBC destruction child will need to have oral penicillin prophylactic for a year or two 3. Disorders of the RBC (Pillitteri pp.1389-1403) 3.1. Normochromic, Normolytic Anemias 3.1.1. Acute Blood Loss Anemia Blood loss sufficient enough to cause anemia Maybe due to: Trauma (accidents) Acute nephritis (blood loss in urine) Placenta previa Abruptio placenta Maternal-fetal or twin to twin transfusion Cesarean birth Intestinal parasites Children will be in shock and pale Tachycardia will appear (heart compensation to circulate blood through the body)

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Rapid breathing due to lack of RBC (compensate for oxygenation) Children will have sternal retractions and cyanosis They will not respond to oxygen therapy Treatment involves control of bleeding, Supine position (improve brain circulation) Keep children warm Blood transfusion will be necessary to provide an immediate increase in the no. of erythrocytes Plasma expander such as Plasma or IV fluids (Ringers Solution) may be given until blood is available Anemia of Acute Infection increased destruction of erythrocytes due to infection or inflammation Common conditions include: Osteomylitis Ulcerative colitis advanced renal disease Management includes treatment of the underlying condition Anemia of Renal Disease there is decrease in erythropoietin production due to loss of kidney function here is decrease stimulation of the bone marrow to produce new RBC management is through administration of recombinant human erythropoietin (increase RBC production) Anemia of Neoplastic Disease Anemia due to invasion of bone marrow by proliferating Neoplastic cells with possible blood loss if platelet production is impaired Management includes, measures designed to achieve remissions of the Neoplastic process Transfusion can increase erythrocyte level

3.1.2.

3.1.3.

3.1.4.

3.1.5. Aplastic Anemias Results from depression of hematopoetic activity in the bone marrow. all blood components will be affected Congenital Aplastic Anemia also known as Fanconis syndrome it is inherited as an autosomal recessive trait the child will be borne with other congenital anomalies (skeletal and renal anomalies, hypogenitalism and short stature) between age 4-12 years of age will begin to manifest symptoms of pancytopenia (reduction of all blood components) Acquired Aplastic Anemia Decrease in bone marrow production that can occur if a child is exposed excessively to radiation, drugs or some chemicals. Drugs include CMC, sulfonamides, arsenic, hydantoin, benzene or quinine. Exposure to insecticides can also cause severe bone marrow depression meningococcal pneumonia and other serious infection may cause autoimmunologic suppression of the bone marrow Assessment: Pale Easy fatigueability Anorexia Easy bruising (due to thrombocytopenia) Petechiae Epistaxis Gastrointestinal bleeding Infections (due to leucopenia) Bone marrow aspiration will show a reduced number of blood elements Therapeutic and Nursing Care Management treatment is by stem cell transplantation antihemocyte globulin (ATG) or cyclosporine therapy (used if stem cell transplantation is not available) Packed RBC and Platelet transfusion will be necessary RBC-stimulating factor can also be given Discontinuance of suspected drug or chemical that causes bone marrow dysfunction

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3.1.6. Hypoplastic Anemias results from depression of hematopeietic activity in the bone marrow it can either be congenital or acquired it affects only RBC Congenital Hypoplastic Anemia also known as Blackfan-Diamond Syndrome it is a rare disorder that manifests as early as 6-8 months of life it affects both sexes caused by inherent defect in RBC formation onset is insidious RBC are normochromic and normocytic By age 13 affected children will undergo spontaneous permanent remission If they dont undergo remission they are candidates for stem cell transplantation Acquired Hypoplastic Anemia transient reduction of RBC Long term transfusion of packed RBC will be necessary Children will also undergo hypodermoclysis (an iron chelation therapy) to prevent hemosiderosis (iron deposition in body tissues) utilizing deferoxamine (Desferal) 3.1.7. Hypersplenism Increased destruction of RBC due to a spleen enlargement Blood passes slowly to the spleen causing more RBC and other blood components to be destroyed Any splenic condition can cause this condition Therapeutic and Nursing Care Management Treating the underlying splenic disorder Splenectomy can be performed to reduce RBC destruction (delayed until after 2 years of age) Children needs prophylactic penicillin for 2 years after spleen removal Children will also receive immunization against influenza, pneumococci and H. influenzae 3.2. Hypochromic Anemias 3.2.1. Iron-Deficiency Anemia the most common anemia of infancy and childhood occurs when intake of dietary iron is insufficient Inadequate iron prevents proper hemoglobin formation RBC will be hypocytic (small in size) and hypochromic (pale) and possible poikilocytic (abnormal in shape) occurs most often between ages 9 months and 3 years frequency rises in adolescents, when iron requirements increases for menstruating girls It is also found in overweight teenagers (high carbohydrate, low iron diet) Causes in infants: infants diet lacks iron (develops IDA at 5-6 months) infants of low birth weight have fewer iron stores woman who have iron deficiency at pregnancy will give birth to iron0deficient babies infants born with structural defects of the gastrointestinal system (chalasia or pyloric stenosis) infants with chronic diarrhea (inadequate absorption) Causes in older children: in children older than 2 years, frequent blood loss is the cause maybe due to: - polyps - ulcerative colitis - Chrons disease - protein-induced enterophaties - parasitic infection - frequent epistaxis Adolescent girls will have IDA due to menstrual flow Assessment: Pale mucous membrane (dark skinned infants) Possible enlarged heart Possible enlarged spleen Poor muscle tone Decreased activity Pallor Irritability and fatigue Soft systolic precordial murmur

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 Fingernails becomes spoon shaped or depressed Abnormally high milk intake Laboratory studies will reveal a decreased hemoglobin and reduced hematocrit levels Mean corpuscular volume is low Low iron serum level (usually 30ug/100ml) Low serum ferritin levels (usually 10 ug/100ml IDA is usually associated with pica (eating of inedible substances such as dirt) Therapeutic and Nursing Care Management: treatment is focus on treating the underlying cause diet rich in iron and vit. C Ferrous sulfate for 4-6 months is the drug of choice to replace depleted iron stores 3.2.2. Chronic Infection Anemia Due to impaired iron metabolism and impaired RBC production degree of anemia is rarely as severe as that occurring with iron deficiency administration of iron has little effect until the infection is controlled

3.3. Macrocytic Anemias 3.3.1. Anemia of Folic Acid Deficiency Anemia due to deficiency in Folic acid and vit. C There will be an accompanying neutropenia and thrombocytopenia There is increased mean corpuscular hemoglobin and corpuscular volume Bone marrow will contain megaloblasts (due to inhibition of erythrocyte production at an early stage) Treatment id daily administration of oral folic acid. Pernicious Anemia In infants it is due to inability to use vit. B12 found in foods of animal origin, including both cows and breastmilk In adolescents would be at risk if he is in long term, poorly formulated vegetarian diet In adults, cause is due to lack of intrinsic factors Assessment: paleness anorexic irritability chronic diarrhea beefy red tongue ataxia hyporeflexia paresthesia positive Babinski reflex (less noticeable than in adults) Laboratory findings will reveal low serum levels of vit. B12 Therapeutic and Nursing Care Management: temporary injections of vit. B12 if caused by b12 deficient diet lifelong monthly intramuscular injections of vit.B12; if due to lack of intrinsic factors 3.4. Hemolytic Anemias 3.4.1. Congenital Spherocytosis A hemolytic anemia that is inherited as an autosomal dominant trait Occurs most often in white Northern European population Cells are small and defective (abnormalities of protein in the cell membrane Life span of erythrocytes is diminished Abnormal cells swells, ruptures and is destroyed Chronic jaundice and spleenomegaly develops Mean corpuscular concentration is increased (due to small cells) Gallstones may be present Infections may precipitate a crisis or cause bone marrow failure Blood transfusion will be necessary to maintain a sufficient number of circulating erythrocytes until the crisis passes Treatment is generally Splenectomy at 5-6 years of age 3.4.2. Glucose-6-Phosphate Dehydrogenase Deficiency Glucose-6-Phosphate Dehydrogenase (G6PD) is necessary for maintenance of RBC life. lack G6PDresult in premature destruction of RBC The disease is transmitted by a sex linked recessive trait Occurs most frequently in African-American, Asian, Sephardic, Jewish and Mediterranean descent Occurs in two identifiable forms. 3.3.2.

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 3.4.3.

Congenital nonspherocytic hemolytic anemia has hemolysis, jaundice and spleenomegaly and may have Aplastic crises Other children have a drug induced form Blood Smear will show Heinz Smear (oddly shaped particles in RBC) The degree of RBC destruction depends on the drug and the extent of exposure to it Drug induced form is usually self limiting

Sickle Cell Anemia There is the presence of abnormally shaped (elongated) RBCs it is an autosomal recessive inherited disorder on the beta chain of hemoglobin Erythrocytes become elongated and present shaped (sickled) when submitted low oxygen tension, low blood pH or increased blood viscosity RBCs tend not to move freely when they sickle (causes to stasis and further sickling) Infants will manifest clinical symptoms by 6 months when their hemoglobin changes to adult hemoglobin (infants hemoglobin contains gamma chains, adults hemoglobin contain beta chains) Sickle cell occurs almost exclusively among African-Americans Assessment: Hemoglobin electrophoresis is used to diagnose the disorder The disease can also be diagnosed prenatally by chorionic villi sampling fever and anemia will be the initial signs in a child 6 months of age Some infants will have swelling of the hands and feet Protruding abdomen (enlarged spleen and liver) Frequent pneumococcal meningitis and salmonella-induced osteomyelitis (loss of function of spleen to filter bacteria) Icteric sclera Decreased vision (due to small retinal occlusions) Priapism (persistent, painful erections) due to cell clusters in the blood vessels of the penis Sickle Cell Crisis Sickle cell crisis is the term used to denote a sudden, severe onset of sickling Symptoms occur from pooling of many new sickled cells in vessels and consequent tissue hypoxia beyond the blockage (vaso-occlusive crisis) Occurs when a child has an illness causing dehydration or a respiratory infection May also be caused by strenuous exercise Symptoms are sudden, severe and painful which includes: - fever - Icteric sclera - vomiting - spleenomegaly - hepatomegaly - acute back pain - possible kidney infarction - painful swollen hands - acute abdominal pain and tenderness - joint pain and warmth Laboratory will reveal hemoglobin level of only 6-8 g/100ml with elevated WBC count Increased bilirubin and reticulocyte levels A sequestration crisis (severe anemia due to pooling and increased destruction of sickled cells in the liver and spleen) can happen and lead to shock from hypovolemia Hyperhemolytic crisis can occur when there is increased destruction of RBC A Megaloblastic crisis may occur if he child has folic acid or vit. B12 deficiency Aplastic crisis is manifested be severe anemia due to sudden decrease in RBC production (usually occur with infection) Therapeutic and Nursing Care Management Pain relief (acethaminophen) Adequate hydration with intensive intravenous therapy Oxygenation Relaxation and rest Blood transfusion may be necessary Hydroxyurea (antineoplastic agent) can be used to increase production of hemoglobin F in affected children Exchange transfusion (with small amounts of blood) will be made if the above management is not effective After a crisis, management focuses on preventing future occurrences Oral folic acid may be prescribed to rebuild hemolyzed RBC Advise parents on regular health care visit Reinforce to parents the importance of childhood immunization to prevent infections

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Teach parents to recognize symptoms of sickle cell crisis, for them to bring the child to the health facility as soon as the symptoms are present Children can participate in any sport except for contact sports Advise parents on how to prevent dehydration especially during physical activity and during summer

3.5. Thalassemias 3.5.1. Thalassemia Minor (Heterozygous Beta-Thalassemia) characterized by production of both defective beta hemoglobin and normal hemoglobin RBC count is normal, due to normal RBC being produced but hemoglobin concentration will be decreased 2-3 g/100ml below normal levels Blood cells are moderately hypochromic and microcytic (due to poor hemoglobin formation) Children would not have any other symptoms except for pallor Children will require no treatment with normal life expectancy 3.5.2. Thalassemia Major (Homozygous Beta-Thalassemia) also known as Cooleys Anemia or Mediterranean anemia. symptoms would not manifest until child fetal hemoglobin has largely been replaced by adult hemoglobin (usually by the second half of the first year) Effects on body systems include: RBC are hypochromic and microcytic. Fragmented piokilocytes and basophilic sippling (unevenness of hemoglobin concentration) are present hemoglobin level is less than 5 g/100ml while serum iron level is high Assessment: Bone pain (due to bone marrow hypertrophy) Parietal and frontal bossing Protrusion of the upper teeth Broad and flattened base of the nose Slanted eyes with epicanthal fold Marked osteoporotic tissues upon x-ray Hepatosplenomegaly Anorexia and vomiting (due to pressure of enlarged spleen) Epistaxis (due to pancreatic hemosiderosis) Cardiac dilatation with accompanying murmur Arrhythmias and heart failure are frequent causes of death Therapeutic and Nursing Care Management: Digitalis, Diuretic and low sodium diet to prevent heart failure Transfusion of packed RBC every 2-4 weeks (hypertansfusion therapy) to maintain hemoglobin levels Iron chelation therapy (removal of excess irons) Splenectomy may become necessary to reduce discomfort and reduce RBC hemolysis. Marrow transplant to reduce mortality of the disorder Autoimmune Acquired Hemolytic Anemia Autoimmune antibodies (abnormal antibodies of the IgG class) attach themselves to RBCs destroying them or causing hemolysis. Can occur at any age and its origin is idiopathic but is associated with: Malignancy Viral infection Rheumatoid arthritis Systemic lupus erythematosus URTI Measles Varicella Hemolysis usually occur after the administration of drugs such as: quinine phenacetin sulfonamides penicillin Assessment: Insidious onset low grade fever anorexia lethargy

3.5.3.

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pallor Icteric sclera dark stool and urine (due to excess bilirubin) marked jaundice Laboratory findings would reveal RBC that are extremely small and round (Spherocytosis) reticulocyte count is increased Direct coombs test is positive Hemoglobin levels falls to as low as 6 g/100ml Therapeutic and Nursing Care Management: In some children the disease run in a short course with no treatments needed In some a single blood transfusion may correct the problem (usually type O Rhnegative) Observe children during blood transfusion Corticosteroid therapy (to reduce the immune response) Splenectomy will be made if the above management is ineffective Provide parents and the child support 3.5.4. Polycythemia there is an increase in the number of RBCs the condition results from increase erythropoiesis usual cause in childhood is chronic pulmonary disease and congenital heart disease Plethora (marked reddened appearance of the skin) occurs because of the increase in total RBC volume erythrocytes are usually Macrocytic and the hemoglobin content is high CVA the RBC count maybe as high as 23 g/100ml Treatment involves treatment of the underlying cause

4. Disorders of WBC (Pillitteri p.1403) 4.1. Neutrpenia 4.1.1. Reduced number of white blood cells 4.1.2. Caused by a transient phenomenon with non pyrogenic infections as viral disease 4.1.3. Can be treated with some drugs (6-mercaptopurine or nitrogen mustard) 4.1.4. Can have possible white blood cell transfusion and prophylactic antibiotic therapy 4.2. Neutrophilia 4.2.1. Increased number of circulating white blood cells (usually neutrophils) 4.2.2. Usually caused by a response to infection or inflammation 4.2.3. treated with antibiotic therapy to eliminate infectious organisms 4.3. Leukemia 4.3.1. Uncontrolled proliferation of white blood cells (to be discussed further in Unit 15)

4.4. Eosinophilia Increased number of eosinophils Associated whit many allergic disorders (Atopic dermatitis and parasitic invasion) 4.5. Lymphocytosis Increased number of lymphocytes normally occurs in preschool period abnormally elevated in childhood illnesses (pertussis, infectious mononucleosis and lymphocytic leukemia) management is by treating the underlying cause 5. Disorders of Blood Coagulation (Pillitteri pp.1404-1408) 5.1. Purpuras 5.1.1. Idiopathic Thrombocytopenic Purpura (ITP) the result of decrease in the number of circulating platelets in the presence of adequate megakaryocytes the cause is unknown(may be due to increase destruction of platelets due to an antiplatelet antibody) Occurs most approximately 2 weeks after a viral infection such as rubella, rubeola, Varicella or URTI Congenital ITP occurs in infants born by a mother with ITP during pregnancy

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Assessment: first evidenced by miniature Petechiae epistaxis or bleeding in the joint may be present laboratory studies would reveal thrombocytopenia positive tourniquet test would be positive Therapeutic and Nursing Care Management: oral prednisone to reduce the immune response administration of intravenous immunoglobulin (IVIG) or anti-D immunoglobulin (Rhpositive children to supply anti-ITP antibodies platelet transfusion will temporarily increase the platelet count but with limited effect usually ITP in children runs only for 1-3 months a course of immunosuppressive drugs may be attempted for children who develops chronic ITP All children would need to be vaccinated against childhood illnesses 5.1.2. Henoch-Schonlein Syndrome also called as anaphylactoid purpura caused by increased vessel permeability it is generally considered as a hypersensitivity reaction to an invading organism occurs most frequently in children 2 and 8 years of age occurs most frequently in boys than girls there is usually a history of mild infection before the outbreak of symptoms Assessment: there is a usual purpural rash in the buttocks, posterior thighs and extensor surface of the arms and legs rash begins as a crop of urticarial lesions that change to maculopapular lesions lesions become hemorrhagic then fade, leaving behind brown macular spots that remain for several weeks swollen and tender joints abdominal pain vomiting or blood in stool gross or microscopic hematuria can be present (kidney involvement) biopsy would reveal granulocytes in the walls of small arterioles laboratory studies show a normal platelet count sedimentation rate, WBC count and eosinophil count are elevated Therapeutic and Nursing Care Management: treated with oral corticosteroid therapy and mild analgesic for a short period typically the disease would run a course of 4-6 weeks 5.2. Disseminated Intravascular coagulation an acquired disorder of blood clotting that results from excessive trauma or some similar underlying stimulus there is an imbalance between clotting and fibrinolysis activity there is extreme clotting due to endothelial damage which then depletes the availability of clotting factors such as platelets and fibrin from the general circulation it is a common complication seen accompanying bleeding during pregnancy it can occur in children with acute infections Assessment: there is formation of Petechiae and ecchymosis on the skin toes and fingers appear pale, cyanotic or mottled and feel cold (impaired circulation) Neurologic and renal symptoms will occur if coagulation is acute laboratory test would usually show: - thrombocytopenia - large fragmented platelets on blood smear - prolonged prothrombin time and partial thromboplastin time - marked low serum fibrinogen levels (less than 100mg/100ml) - elevated fibrin spilt products Therapeutic and Nursing Care Management: treat the underlying cause intravenous heparin administration helps to interfere with the marked coagulation blood transfusion will be necessary only after heparin administration administration of fresh-frozen plasma, platelets or fibrinogen 5.3. Hemophilias 5.3.1. Hemophilia A (Factor VIII Deficiency) caused by the deficiency of the coagulation factor VIII (antihemophilic factor) it is transmitted as a sex-linked recessive trait (1 in 10,000 white males; US) Females usually have lowered but sufficient levels of factor VIII

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 bleeding tendencies in males have varying intensity, from mild to moderate Assessment: first recognized in infants with excessive bleeding after circumcision heavy bruising of the lower extremities (most bumped part of the body) hemarthrosis occurs if there is repeated bleeding in the joints epistaxis are common but is not severe thromboplastin generation test would be abnormal partial thromboplastin test would best reveal low levels of factor VIII

Therapeutic and Nursing Care Management: Administration of factor VIII even for minor abrasions Administration can be by fresh whole blood, frozen plasma or by concentrate of factor VIII One bag of concentrate per 5 kg body weight is sufficient and can give protection for 12 hours Administration of factor desmopressin can be helpful Children with inhibitors to factor VIII can also be given a factor IX concentrate (Proplex or Konyne) if there is formation of antibodies to factor VIII every 6 hours, self administration of epsilon-aminocaproic acid can promote healing 5.3.2. Von Willebrands Disease often referred to as angiohemophilia it is an inherited autosomal dominant disorder affecting both sexes there is inability of the platelets to aggregate and blood vessels cannot constrict there is prolonged bleeding time most hemorrhages occur at the mucus membranes the major problem is epistaxis in children girls will have a heavy menstrual flow bleeding can be controlled with factor VIII replenishment or by administration of desmopressin (stimulates factor VIII release) Hemophilia B (Christmas Disease or Factor IX Deficiency) caused by factor IX deficiency transmitted as a sex-linked recessive trait treated with a concentration of factor IX (available for home administration) Hemophilia C (Factor XI Deficiency) also known as plasma thromboplastin antecedent deficiency transmitted as an autosomal recessive trait occurring in both sexes with only mild symptoms bleeding episodes are treated with desmopressin (DDVAP) or transfusion of fresh blood or plasma.

5.3.3. 5.3.4.

6. Related Nursing Care Management Helping in obtaining specimen for laboratory and diagnostic purposes Provisions of therapeutic playtime for children Provide sterilized play materials Measure to relieve pain due to the disorders, diagnostic and therapeutic managements Provide therapeutic support to both parents and the sick child to raise there self esteem Reduce or control predisposing factors that might cause exacerbation of symptoms Provisions of rest periods Therapeutic measures that will help the child to cope with pain Reinforce to parents the need for follow up check up Provisions to prevent accident and injuries Protect IV insertion sites to prevent numerous insertions Advise the child to use soft bristle toothbrush Assess the childs diet, provide for soft non-irritating foods 7. Diagnostics and Treatment 7.1. Venipuncture (Ladner pp.635-636) 7.1.1. can either be performed by using a sterile needle and syringe or a vacuum tube with a sterile two sided needle 7.1.2. Collected blood is placed in a sterile test tubes (to be discussed further in skills laboratory)

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7.2. Blood Typing and cross matching (Ladner p.649-650) 7.2.1. laboratory tests that identifies the clients blood type and determines the compatibility of blood between potential donor and recipient (to be discussed further in skills laboratory)

7.3. Blood Chemistry (Ladner p.651) 7.3.1. analysis using a chemical test using a sequential multiple analyzer (to be discussed further in skills laboratory)

8. Health Promotion and Risk Management (Pillitteri p.1389) begins with ensuring that families have access to genetic counseling for iron deficiency anemia; this can be treated by giving iron-fortified formula for aplastic anemia, educate parents about the importance of keeping poisons out of the reach of children Use of EMLA cream or topical anesthesia to reduce the pain of venipuncture Use of imagery and distraction techniques can reduce childs fear and apprehension during treatment and procedures Proper nutrition (for anemias caused by nutritional deficiency) 9. Documentation Document pertinent information regarding assessment, health history and physical assessment Document nursing interventions done Document diagnostic and therapeutic interventions given Document all health teachings given Document Patient reaction to the therapeutic and nursing procedures given

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