Manifestations in the oral cavity in diseases of

Manifestations in the oral cavity in diseases of the blood system in children. . Mosby items and derived items © 2005, 2001 by Mosby, Inc.

• Disorders related to the blood and blood forming organs in childhood encompass a wide range of diseases and pathologic states. • Since the blood is a multipurpose fluid involved in the functions of so many tissues and organs, either primary or secondary changes in the blood are reflected in the essential functions of these structures. • Disorders of blood and blood forming organs in childhood include the anemias, defects in hemostasis, and the immunologic deficiency diseases. Neoplastic disorders of the hematopoietic system are also included. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Lecture Objectives Upon completion of this chapter, the reader will be able to: • Discuss the anatomy and physiology of the hematological system. • Identify differences between child and adult hematological systems. • Explain and identify different types of anemia and discuss the etiology, treatment, and nursing care of those types. • Identify several types of coagulation disorders and discuss the etiology, treatment, and nursing care of those disorders. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Anatomy The hematologic system consists of: • the blood • and blood forming (hemopoietic) tissues: – Red bone marrow (myeloid tissue) – Lymph nodes – Spleen. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Anatomy Blood is composed of two components: • a fluid portion called plasma • a cellular portion known as the formed elements of the blood The two components are approximately equal in volume. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Anatomy • Plasma is about 90% water and 10% solutes. • The principal solutes are: – the proteins: albumin, globulin, and fibrinogen. • The cellular elements are: – red blood cells (erythrocytes), – white blood cells (leukocytes), – platelets (thrombocytes). Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Hematopoiesis (blood-formation) • All of the formed elements of the blood, except to some extent the agranulocytes, are believed to be formed in myeloid tissue during postnatal life. • During embryonic development the mesenchyme, spleen, liver, thymus, and yolk sac serve as additional sites of blood cell formation. • In certain blood disorders these sites, particularly the spleen, can be stimulated to produce blood cells, and constitute extramedullary hematopoiesis. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Hematopoiesis (blood-formation) • Each blood cell originates from a primordial (primitive) cell called a blast, or stem, cell in the bone marrow. This hemocytoblast in turn gives rise to the erythroblast, myeloblast, monoblast, lymphoblast, and megakaryoblast. • Reticuloendothelial system consists of widely dispersed cells of mesodermal origin that line the vascular and lymph channels. These cells, called reticular cells, are capable of: – phagocytosis (ingestion and digestion of foreign substances), – formation of immune bodies, – differentiation into other cells, such as hemocytoblasts, myeloblasts, or lymphoblasts. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Blood functions: • transporting substances needed for cellular metabolism in the tissues; • regulating acid base balance; • protecting against infection and injury. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Differences between child and adult hematological systems • In infants and young children all of the bone contains red marrow (so called because of its color from formation of erythrocytes). • At the end of adolescence, only the ribs, sternum, vertebrae, and pelvis continue to produce blood cells. The remainder of the bone marrow becomes yellow from deposition of fat. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Regulation of erythrocyte production • The usual life span of the mature erythrocyte is 120 days. • They are removed from circulation, mainly by the spleen. • Because RBCs are not capable of replication, the bone marrow releases new, immature erythrocytes called reticulocytes to replace the RBCs removed from circulation. • The hemoglobin is broken down into the iron containing pigment hemosiderin and the bile pigments biliverdin and bilirubin. • The basic regulator of erythrocyte production is believed to be tissue oxygenation. • In states of tissue hypoxia, erythropoietin (also called erythropoietic stimulating factor or hemopoietin) is released by the kidneys into the blood stream. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Age peculiarities of blood: • Smaller than in adults general volume of blood. • Higher than in adults relative blood volume: versus 50 80 m. L/kg in adults • RBC volume varies with age. • Immediately after birth there is a period of inactive erythropoiesis during which the iron obtained from catabolized RBCs is stored as hemosiderin in the bone marrow and the liver tissue. • These stores are greatest at 4 to 8 weeks of age and function to protect the infant from anemia. • Premature infants use up these stores within 6 to 12 weeks, whereas the iron stores of full term infants last up to 20 weeks. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Erythrocyte (RBC) count – age reference cord 5 7 × 1012 cells/L 1 3 days 6 7 × 1012 cells/L 1 wk 1 yr 2. 7 4. 5 × 1012 cells/L 2 12 yr 3. 9 5. 3 × 1012 cells/L 12 18 yr: Male 4. 5 5. 5 × 1012 cells/L Female 4 5 × 1012 cells/L Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Age peculiarities of blood newborn: • Short life span of erythrocytes 12 40 days versus 120 days • Erythrocytosis 6 7 × 1012 cells/L • Anisocytosis (presence of different forms of RBCs inside vessels) • Hemoglobin level 180 240 g/L • Hb F – 63 92 % versus 5 % adults • Color index (CI) 1. 1 1. 3 (degree of saturation of Hb in one erythrocyte) • Erythrocyte sedimentation rate 0 2 mm/hour • Leucocytosis 11 33 × 109 cells/Liter • Presence of extramedullar sources of hemopoiesis. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Age peculiarities of blood – child 1 mo – 1 yr: • Decreasing Hb level to 120 110 g/L • Erythrocyte level decreases, as well, to 4 3. 5 × 1012 cells/L • Reticulocyte count is up to 10 % • Colour index is low: 0. 8 0. 7 • Leucocytosis 10 12 × 109 cells/L appears after: – adding new food in menu – during crying – emotions • 1 st cross of WBC count – 5 mo • Platelets have gigantic forms Mosby items and derived items © 2005, 2001 by Mosby, Inc.

WBC differential count during childhood Eosinophils Basophils Neutrophils “bands” Neutrophils “segs” 3 d 5 d 3 yr 5 yr 12 yr 1 4 % 3% 1 4 % 0 0. 5 % 3 5 % 3 5 % 54 62 % 39 42 % 24 30 % 39 42 % 54 62 % Lymphocytes 11 25 % 45 % 63 % 45 % 25 33 % Monocytes 6 10 % 6 10 % Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Age peculiarities of blood – child 1 mo – 1 yr: • Increasing of Hb level to 130 140 d/L till 15 years • Erythrocyte level 4. 5 5. 0 × 1012 cells/L • Reticulocytes are about 0. 5 5 % • Leukocyte level 7 9 × 109 cells/L • Second WBC count cross in 5 years of age Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Examination: subjective data There are some weighty clinical signs that directly show on blood disorders. They are: • Hemorrage (bleeding); • Hematoma; • Enlargement of lymph nodes; • Pallor; • Ossalgia (pain in bones). Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Examination: subjective data Pica – character sign of iron deficient anemia. Eating clay, ice, paste. Other complaints: • Often headaches, • Dizziness, light headednass, • Slowed thought processes, dicreases attention span, • hyperthermia, • Apathy, • Shortness of breath, • Easy fatigability. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Anemias. • A condition in which the number of RBCs and/or the hemoglobin concentration is reduced below normal: – 0 14 days Hb<145 g/L – 15 28 days Hb<120 g/L – 1 mo 1 yr Hb<110 g/L Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Anemias. The basic cause of anemia is either (1) an increased loss or destruction of red blood cells (2) an impaired or decreased rate of production. An etiologic classification is based on the various conditions that can lead to either of these results. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Classification of anemias: • Decreased Production of RBCs: – Iron Deficiency Anemia – Aplastic Anemia • Increased Destruction of RBCs: – Hemolytic Anemia – Sickle Cell Anemia – Thalassemia • Blood loss: – Iron Deficiency Anemia – Hemorrhagic Anemia • Severety: – Mild: Hb 110 90 g/L – Moderate: Hb 89 70 g/L – Severe: Hb < 70 g/L Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Classification of anemias: • Morphologic (describe the size of RBCs): – (1) normocytic, – (2) microcytic, – (3) macrocytic. • According to the amount of hemoglobin in the cell (describe the color and Hb content of the cells): – (1) normochromic – (2) microchromic. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Anemia. Assessment • Take health history: – Careful diet history to identify any deficiencies, – Evidence of pica – eating clay, ice, paste. • Observe for manifestations of anemia: – Muscle weakness – Easy fatigability: • frequent resting, • shortness of breath, • poor sucking (infants) Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Manifestations of anemia • Pale skin, mucous membranes, lips, nail beds, and conjunctiva – Waxy pallor seen in severe anemia – Capillary refill – no change color • Rapid, pounding heart beat • CNS manifestations: – Headache – Dizziness – Light headedness – Irritability – Slowed thought processes, dicreases attention span – Apathy – Depression. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Severe anemia • Impaired healing and loss of skin elasticity • Thinning and early greying of the hair • Abdominal pain, nausea, vomiting, anorexia • Low grade fever Mosby items and derived items © 2005, 2001 by Mosby, Inc.

What Is the Diagnosis? While working in the community you encounter 10 -month-old Wendy, who is irritable and appears weak for her age. Her mother states she seems more tired than usual and does not play as much as she used to. She has tried giving Wendy a bottle of milk like she usually does when she appears unhappy, but that does not seem to pacify her. You ask more about dietary habits and find that when Wendy's mother stopped breastfeeding, she started her on a cow's-milk bottle. She usually uses this bottle to calm her and when it is time for meals, Wendy typically does not eat very much. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

o What do you think Wendy's diagnosis is? o o What nursing interventions would be appropriate? Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Aplastic anemia • is a condition wherein injury to or abnormal expression of the stem cells in the bone marrow results in the production of inadequate numbers of erythrocytes, leukocytes, and platelets. • Can be hereditary or acquired. • The hereditary form of aplastic anemia, Fanconi's anemia (FA), is a rare autosomal recessive disorder that develops early in life and is accompanied by multiple congenital abnormalities Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Aplastic anemia • The age of onset of varies. • Typically, the first sign noted is increased bruising caused by the decreased number of platelets. • Vulnerability to infection because of the decreased number of WBCs. • When the RBCs have decreased in number, the signs and symptoms of anemia begin to manifest: – pallor, weakness, and difficulty breathing • Impaired growth and development Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Criteria for severe aplastic anemia Severe Aplastic Anemia • Neutrophils < 2 × 109 cells/L • Platelets < 50 × 109 cells/L • Reticulocytes < 0. 5 % • Bone Marrow Cellularity < 20 % Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Nursing Diagnosis • Ineffective tissue perfusion related to anemia • Imbalanced nutrition: less than body requirements related to inadequate iron intake • Deficient earegiver knowledge related to age appropriate iron intake • Activity intolerance related to decreased oxygen delivery to the tissues Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Goals: 1. 2. 3. Minimize physical exertion and emotional stress: – Anticipate and assist in those activities of daily living that may be beyond child’s tolerance – Provide play activities that promote rest and quiet but prevent boredom and withdrawal – Encourage parents to remain with child, etc. Increase oxygen to tissues: – Position for optimim air exchange – Administer supplemental oxygen if needed Help replace blood elements: – Administer blood, packed cells, platelets as prescribed Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Goals: 4. Promote adequate intake of iron rich foods: – Encourage exclusive breastfeeding of infants for 4 6 months after birth. – Diet rich with food sources of iron: meat, liver, fish, egg yolks, spinach, oysters, peas, green leafy vegetables, nuts, whole grains, iron fortified infant cereal and dry cereal – Feed milk as supplemental food in infant’s diet after solids are begun – Administer iron preparations as prescribed: ь Give between meals ь Administer with fruit juice or multivitamin preparation ь Do not give with milk or antacids ь Give fluid preparations with dropper, syringe, or straw to avoid contact with teeth ь If the child is getting enough iron, stools should be a tarry green color. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Iron Supplements • Although oral iron supplementation is generally perceived as a safe treatment alternative, an overdose can be lethal for children. • Initial signs and symptoms of overdose: – vomiting, – abdominal pain, – bloody diarrhea. • These manifestations are typically followed by shock, lethargy, and dyspnea. Often the child will appear to improve before the onset of severe metabolic acidosis, coma, and death. • Urgent treatment is essential and includes flushing out unabsorbed pills, administration of Desferal, and supportive therapy Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Blood Transfusion Nursing responsibilities • Positively identify donor and recipient blood types and groups before transfusion is begun; verify with one other nurse or physician • Transfuse blood slowly for first 15 20 min; remain with patient • In event of signs or symptoms, stop transfusion immediately, maintain patent IV Line, notify physician • Insert urinary catheter to monitor hourly outputs • Monitor for evidence of shock • Save donor blood to re crossmatch with patient’s blood Mosby items and derived items © 2005, 2001 by Mosby, Inc.

BLOOD TRANSFUSION COMPLICATIONS • Hemolytic reactions • Febrile reactions • Allergic reactions • Circulatory overload – too rapid transfusion or excessive quantity of blood transfused • Air emboli – May occur when blood is transfused under pressure • Hypothermia: – Allow blood to warm at room temperature (1 hr) • Hyperkalemia – In massive transfusion or patient with renal problems Mosby items and derived items © 2005, 2001 by Mosby, Inc.

SICKLE CELL ANEMIA • A hereditary disorder in which normal adult hemoglobin (Hb. A) is partly or completely replaced by an abnormal hemoglobin (Hb. S) causing distorsion and rigidity of red blood cells under condition of reduced oxygen tension. • Sickle cell anemia is part of a group of diseases called hemoglobinopathies. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

SICKLE CELL ANEMIA • Sickle cell anemia is an autosomal recessive disorder. The inheritance is described as intermediate because the gene is partially expressed in the heterozygous state and completely expressed in the homozygous state. • Sickle cell anemia is found primarily in the black race, although infrequently it affects whites, especially those of Mediterranean descent. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

SICKLE CELL ANEMIA – Manifestations: • Take health history, especially regarding any evidence of sickling crisis and history of the disease in family members. • General: – Growth retardation – Chronic anemia – Delayed sexual maturation – Marked susceptibility to sepsis • Other signs and symptoms. – weakness; anorexia; joint, back, and abdominal pain; fever; and vomiting. – Chronic leg ulcers are common in adolescents and adults and are thought to be the result of thrombosis and decreased peripheral circulation. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

SICKLE CELL ANEMIA – Vaso-occlusive (sickling) crisis • Pain in area(s) of involvement • Signs of ischemia: – Extremities: painful swelling (“hand foot” syndrome), painful joints – Abdomen: severe pain resembling acute surgical condition – Cerebrum: stroke, visual disturbances – Chest: pseudopneumonia, propracted episodes of pulmonary disease – Liver: obstructive jaundice, hepatic coma – Kidney: hematuria Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Sequestration Crisis • Pooling of large amounts of blood: – Hepatomegaly – Splenomegaly – Circulatory collapse Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Diagnostic evaluation • For screening purposes: – the Sickledex is commonly used. If the test is positive, hemoglobin electrophoresis is necessary to distinguish between those children with the trait and those with the disease. • Sickling test (sickle cell slide preparation): place a drop of blood on a slide and cover it with a sealed cover slip to produce deox ygenation. Eventually sickling of the red blood cell occurs. • Hemoglobin electrophoresis ("fingerprinting"). This test is accurate, rapid, and specific. Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Nursing Diagnosis • Risk for injury R/T abnormal Hb, decreased ambient oxygen, dehydration • Altered tissue perfusion R/T generalized sickling • Pain R/T tissue anoxia • Altered family process R/T to a child with potentially life-threatening disease Mosby items and derived items © 2005, 2001 by Mosby, Inc.

Nursing management Goals: • Inc tissue oxygenation and prevent sickling: – Family teach. : avoid strenuous physical exertion, emotional stress, O 2 envir – Promote hydration – Prevent infection – ↑ tissue oxygenation: adm O 2, promote circulation through passive range of motion exercises – Relieve pain Mosby items and derived items © 2005, 2001 by Mosby, Inc.

The end. Q&A? Mosby items and derived items © 2005, 2001 by Mosby, Inc.